audiology - ENT and Audiology News

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ENT
audiology
news
www.entandaudiologynews.com
IAPO
Exclusive
Content
18-20 November
2011
Sao Paulo, Brazil
Included inside: articles from the recent paediatric
issue (July/August 2011)
Plus added bonus paediatric articles published since 2009
welcome
to your IAPO 2011 Exclusive ENT & audiology news content
featuring articles from the recent paediatric issue (July/August 2011)
PLUS added bonus paediatric articles published since 2009
Paediatric articles published in the JULY/AUGUST 2011 issue
ENT FOCUS
5
The Scope of Paediatric ENT
19
Iain Bruce
8
Paediatric ENT: why choose this subspecialty?
Congenital Midline Nasal Masses Compared
Mary-Louise Montague
22
Sujata De
Virus Infection of the Paediatric Airway and
Chronic Disease Effects
Adam J Donne and Michael P Rothera
10
Combining Adult and Paediatric Practice – is it
still feasible?
25
Konstance Tzifa and Kate Hanvey
Fiona B MacGregor
12
Failed Extubation in Children: assessment and
management options
The Rehabilitation of the Deaf Child
27
Serious Complications of Acute Otitis Media:
mastoiditis and intracranial sepsis
William PL Hellier
Joe Grainger and Michael Kuo
31
15
17
Paediatric Nasal Emergencies
Reassembling the Auditory World in Children
with Cochlear Implants
Tom Beech and Ann-Louise McDermott
Blake C Papsin, Sharon L Cushing and Karen A Gordon
Neck Abscesses in Children
34
The Future of Paediatric ENT Surgery
Gavin Morrison
Louise Melia and Haytham Kubba
AUDIOLOGY MATTERS
38
Paediatric Audiology
45
Josephine Marriage
39
41
Post Newborn Hearing Screening ABR: quality
assurance and the role of peer review
Priya Singh
Guy Lightfoot, Graham Sutton and Sally Wood
Amplification Options for Mild Bilateral
Hearing Loss & Unilateral Hearing Loss in
Children: a literature overview
Helping Families Accept Technology
Cherilee Rutherford
Jane R Madell
43
Unilateral Hearing Loss in Early Childhood: what
are we doing about it?
48
Auditory Verbal Therapy Provision in the UK
52
Paediatric Tool Development: developing a rationale for empowering children with hearing loss
Elizabeth Tyszkiewicz
Melanie Gregory
BONUS ARCHIVE: Paediatric articles published since 2009
ENT FOCUS
AUDIOLOGY MATTERS
55
69
European Society of Paediatric Otolaryngology
John Graham
May/June 2009
57
Doris-Eva Bamiou and Tony Sirimanna
Jan/Feb 2009
Paediatric Audiology at Alder Hey Children's
NHS Foundation Trust
72
Ezeddin El Tabal
Sept/Oct 2009
58
Imaging the Paediatric Airway
75
The Challenges Ahead in Paediatric Audiology
Jane Madell
Mar/Apr 2009
Bone Anchored Hearing Aids in Children
78
Patrick Sheehan
Mar/Apr 2010
65
Managing Children with Auditory Processing
Disorders in the Educational Environment
Pauline Grant
Jan/Feb 2009
Laura Coleman, Helen Williams, Kate Parkes and Michael Kuo
Jan/Feb 2010
62
A Clinical Service For Children and Adults With
Suspected APD
New Developments in Hearing Aids for Children
and Adults
Josephine Marriage
May/June 2009
Epidemiology of Paediatric Sleep Disordered
Breathing
80
Michelle Wyatt
Jul/Aug 2010
Cochlear Implantation in Early Childhood: what
is happening in the long-term?
Sue Archbold
Jul/Aug 2009
83
New Frontiers: auditory brainstem results in
adults and children
Martin O’Driscoll
Jul/Aug 2009
87
An Approach to the Dysmorphic Child with
Deafness
Melissa Lees
Sept/Oct 2010
PLUS
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features
Paediatric ENT
Paediatric ENT: why choose this subspecialty?
Combining Adult and Paediatric Practice – is it still feasible?
Failed Extubation in Children: assessment and management options
Congenital Midline Nasal Masses Compared
Virus Infection of the Paediatric Airway and Chronic Disease Effects
The Rehabilitation of the Deaf Child
Serious Complications of Acute Otitis Media: mastoiditis and intracranial sepsis
Reassembling the Auditory World in Children with Cochlear Implants
The Future of Paediatric ENT Surgery
Mr Iain Bruce, MD,
FRCS(ORL-HNS),
Consultant Paediatric
Otolaryngologist.
Correspondence
Royal Manchester
Children’s Hospital,
Manchester, UK.
E: [email protected]
Declaration of
Competing Interests
None declared.
he range of services provided by
paediatric
ENT
surgeons
continues to evolve with the
requirement to manage isolated pathologies and the ENT manifestations of other
disease processes (for example nasal polyposis in cystic fibrosis) and syndromes (for
example OSA in Mucopolysaccharidosis).
The majority of secondary level ENT problems in childhood continue to be managed
successfully in District General Hospitals,
under the guidance of lead clinicians for
paediatric ENT. This article will focus on
tertiary level services provided by dedicated paediatric ENT surgeons, often but
necessarily exclusively, working in children’s
hospitals or large teaching hospitals. An
effective working relationship between
tertiary and secondary level paediatric ENT
services is obviously important if the
highest possible standard of care is to be
provided.
Paediatric ENT as a subspeciality is
unique as it necessitates the management
of complex otology, rhinology and head
and neck problems, with a consultant
often being skilled in each of these areas
(Table 1). Therefore, there is often an
overlap with the skill set of adult subspecialists, with this providing a potential
source of strength as it facilitates the
recruitment of the most appropriate local
team to ensure effective management of
certain pathologies. The amount of input
from adult subspecialists will vary
between hospitals, but an effective relationship is important. This interaction is
most noticeable when presented with a
child with a head and neck malignancy.
Although the child may initially be
reviewed by a paediatric ENT surgeon or
paediatric oncologist, thyroid or salivary
gland malignancy will often be best
managed by a cancer surgeon. Children
will also be referred with secondary level
ENT problems complicated by significant
comorbidity.
T
The management of childhood airway
obstruction is a significant component of a
paediatric ENT surgeon’s workload. The
ability to perform a diagnostic rigid airway
endoscopy (MLB, DLTB) is a basic requirement of a paediatric ENT surgeon. A wide
variety of airway pathologies may be
encountered as illustrated in Figure 1.
Management may include endoscopic
surgical treatment (for example subglottic
cysts), a surgical airway (tracheostomy) or
an open airway augmentation procedure
(for example laryngotracheal reconstruction with a costal cartilage graft). Access to
the airway may be further complicated by
abnormalities such as micrognathia in
Treacher Collins syndrome. The management of an airway emergency requires
advanced airway skills and close cooperation with the paediatric anaesthetist.
The management of childhood chronic
otitis media, with or without cholesteastoma, is particularly challenging. Effective
management of this clinical problem
involves disease control or eradication, but
an increasing emphasis is also being placed
on the rehabilitation of hearing loss once
the disease has been treated, to limit the
effect on educational performance.
Collaboration with paediatric audiologists
is important and bone anchored hearing
aids (BAHA) and middle ear implants
(MEI) may also be indicated. Particular
childhood pathologies present specific
otological problems, such as hearing loss in
children with cleft palate, craniosynostoses,
microtia and canal atresia. Although the
majority of cochlear implants in children
are performed by subspecialist otologists,
paediatric ENT surgeons now perform
cochlear implantation in several centres in
the UK. An established paediatric ENT /
anaesthesia team may be more appropriate
when the child also has complex comorbidity, particularly involving the airway.
Paediatric ENT surgeons will manage
congenital head and neck anomalies
An effective working relationship between tertiary and secondary level
paediatric ENT services is obviously important if the highest possible
standard of care is to be provided
ENT & audiology news | www.entandaudiologynews.com
5
feature
Figure 1: Laryngeal pathology in children.
Subglottic haemangioma
Epiglottitis
Laryngeal cleft
MPS deposits
Post-intubation changes
Foreign body
Supraglottic cyst
RRP
Laryngomalacia
Subglottic stenosis
Subglottic cyst
Collaborative working with paediatricians, neurologists, intensivists,
anaesthetists, audiologists, respiratory physicians and cardiologists is an
essential and rewarding aspect of working as a paediatric ENT surgeon
6
feature
Table 1. Summary of childhood tertiary level ENT problems.
including lymphovascular malformations
(for example cystic hygroma), branchial
abnormalities (for example branchial
sinuses), thyroglossal cysts and
teratomas. Treatment will often
comprise surgical resection but may also
include airway management in large
cystic hygromas and teratomas.
Various congenital abnormalities may
present with nasal obstruction or a mass
in early childhood. Neonates are obligate
nasal breathers and airway obstruction in
this period may present as an airway
emergency. Pathologies may include
choanal atresia, nasal dermoids, gliomas,
teratomas and encephaloceles. The
surgical approach will be dictated by the
nature, site and extent of the disease and
the options include endoscopic
transnasal, transpalatal, and combined
transnasal and transcranal in conjunction
with a neurosugeon.
A paediatric ENT surgeon must be
able to manage the complications of
childhood head and neck sepsis. This will
include deep neck space infections,
orbital complications of rhinosinusitis
and the intracranial and intratemporal
complications of acute and chronic otitis
media. A paediatric ENT surgeon must
have demonstrable skills in this area that
constitutes, along with airway obstruction, a significant component of on-call
commitment.
As paediatric ENT departments
expand the services provided to children
are becoming increasingly comprehensive. To meet these varying clinical
demands paediatric ENT surgeons are
often required to have undertaken a
paediatric ENT fellowship during their
training. Collaborative working with
paediatricians, neurologists, intensivists,
anaesthetists, audiologists, respiratory
physicians and cardiologists is an essential and rewarding aspect of working as a
paediatric ENT surgeon. n
Anatomical sites
Pathologies
Neck
Deep neck space infections
Congenital abnormalities
Thyroglossal cysts
Branchial abnormalities
Lymphovascular abnormalities
Teratoma
Malignancies
Rhabdomyosarcomas
Thyroid
Salivary gland
Airway
Oropharygeal
OSA
Micrognathia
Adenotonsillar hypertrophy (for example MPS,
Down syndrome)
Macroglossia (Beckwith Weidermann syndrome)
Lingual thyroid
Supraglottic
Laryngomalacia
Vallecular cysts
Cystic hygroma (microcystic)
RRP
Glottic
Vocal cord palsy
Laryngeal webs
RRP
Post-intubation injury
Laryngeal clefts
Subglottic
Subglottic stenosis (congenital or acquired)
Haemangioma
Subglottic cysts
Trachea and bronchus
Tracheobronchomalacia
Foreign bodies
Ears
OME in children with comorbidity
Chronic otitis media
Complications of AOM and COM
Microtia and atresia
Surgical rehabilitation of moderate to profound hearing loss
Bone anchored hearing aids
Middle ear implants
Cochlear implants
Nose
Congenital nasal obstruction and masses
Choanal atresia
Nasal dermoid
Glioma
Teratoma
Encephalocele
Craniosynostosis
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7
Paediatric ENT: why choose
this subspecialty?
Miss Sujata De,
FRCS(ORL-HNS),
ENT Surgeon.
Correspondence
Alder Hey Children’s
Hospital, Eaton Road,
Liverpool, L12 2AP, UK.
E: Su.de@
alderhey.nhs.uk
Declaration of
Competing Interests
None declared.
he vast majority of ENT surgeons
have some element of paediatric
practice – indeed our ‘bread and
butter’ has historically comprised
grommet insertion and adenotonsillectomy. The old adage ‘children are not little
adults’ remains true and Paediatric ENT as
a distinct subspecialty was born of a
necessity to recognise this in ENT.
Essentially paediatric ENT is the practice
of ENT surgery in children. This encompasses the common procedures as
mentioned above but also the management of conditions that are unique to
infants and children such as congenital
anomalies and airway disorders that are
sequelae of prolonged intubation and / or
prematurity. In any case children are not
little adults and so it is only fair that their
ENT needs are addressed accordingly.
Paediatric ENT is a relatively young
subspecialty in its own right. It is only in
the last 20 years or so that it has had its
own subspecialty association – BAPO in
the UK, ESPO in Europe, and ASPO in the
United States. As a result the paediatric
ENT community is a relatively small one
with the resulting ease of exchange of
ideas, and rapid uptake of innovation. An
example of this is the speed at which the
treatment of subglottic haemangioma has
been revolutionised by the use of propranolol. Paediatric ENT has been incorporated into the assessment and examination process of most ENT training
programmes. Specialist experience can be
T
8
gained by seeking fellowships both in the
UK and abroad.
There are two types of paediatric ENT
surgeon; one who works in general ENT
practice but who perhaps takes on the
role of paediatric lead in a District General
Hospital (DGH). A large proportion of
children who require an ENT opinion or
management can be seen and managed in
a DGH. This paediatric ENT surgeon in
this case would be responsible for
ensuring that children are treated
according to the highest standards
possible and to create referral pathways
with the local tertiary unit for conditions
that cannot be dealt with at a DGH either
because of lack of suitable anaesthetic
cover or lack of nursing / surgical expertise
or ICU backup.
The other type of paediatric ENT
surgeon works in a tertiary paediatric
hospital and in addition to dealing with
common childhood ENT disorders in
both ‘normal children’ and children with
other challenging conditions, also
manages conditions specific to children.
The tertiary ENT consultant is a ‘generalist’ in that he / she will have to manage all
ENT conditions in children. These are fairly
evenly spread between otology, rhinology,
laryngology and head and neck surgery.
One can therefore put to use all the skills
accumulated during years of ENT training.
There are specialist areas such as paediatric
airway, congenital anomalies, cochlear
implantation that require specific skills and
feature
expertise. In addition, a lot of collaboration is required
particularly in managing children with challenging conditions such as Down’s syndrome, craniofacial conditions
and neurological conditions. One has the great privilege of
working closely with paediatricians, neonatologists,
geneticists and intensivists. Of course the main advantage
of being a paediatric ENT surgeon is the opportunity to
work with children and to make a real difference to the
quality of life of individuals who have the whole of their
lives ahead of them. I still remember the surgeon who took
out my tonsils. Trivial operation maybe, but he’s the
person who inspired me to be a surgeon!
One of the challenges of paediatric practice is the lack
of a strong evidence base for much of our practice. This
has led some to question whether what we do is necessary
at all!1 Clinical/ pharmaceutical research in children is obviously much more difficult than in adults. We sometimes
have to rely upon evidence from adult research to guide
our management principles. Even good research
conducted in children has to be interpreted carefully as
conclusions cannot be generalised across the board. For
example OME poses a very different management challenge in a two year old when compared to a 13 year old
with Down’s syndrome!
Of course life is not without its challenges and working
with infants and children inherently means having to deal
with their parents and carers. Infants and younger children
cannot communicate and are therefore completely reliant
on their parents or carers to express their needs and
concerns. Taking a history therefore is a challenge as the
parent or carer may bias the explanation of symptoms
towards a course of action that they prefer. At some point
in time (and this varies from child to child), the child
becomes quite capable of expressing him / herself; and
then the consultation can be like a game of ping pong
with the child claiming one thing while the parents claim
another. It is the clinician’s job to decipher the real story
and to make an appropriate decision that is in the child’s
best interest. Most of the time managing parents and their
expectations is a positive part of the experience but there
will be occasions when there is disagreement between you
and the parents as to what is in the child’s best interests.
Most conflict can be resolved with good communication,
careful explanation and if necessary mediation by another
member of the team.
It is always unfortunate when management plans do
not result in a favourable outcome. It sometimes becomes
necessary particularly in children with complex morbidity
to take a step back and decide whether less intervention is
a kinder course of action. The loss of a young life is particularly harrowing. Thankfully, in paediatric ENT mortality is
rare but it does occur and can be heartbreaking.
On the whole paediatric ENT is a very rewarding
subspecialty, one that makes a difference to its patients
and their families, and one that challenges one’s clinical
skills, both outside and inside the operating theatre. n
References
1. Spence D. Bad medicine: paediatric ear, nose and throat surgery. BMJ
2010;341:c6560.
British Association for Paediatric Otorhinolaryngology [www.bapo.org.uk]
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9
Combining Adult and
Paediatric Practice – is it still
feasible?
Fiona B MacGregor,
MBChB, FRCS,
FRCS(ORL),
Consultant
Otolaryngologist and
Honorary Senior Lecturer.
Correspondence
Gartnavel General
Hospital, Great Western
Road, Glasgow, G12
0YN, UK.
E: Fiona.MacGregor@
ggc.scot.nhs.uk
O
ne of the factors that originally drew me to ENT surgery over 20 years ago was
the opportunity to work with both adults and children. Indeed, most consultants would see adults and children in the same clinic and combined operating
lists were commonplace. Practice has changed since then and children are usually seen in
dedicated paediatric clinics by ENT surgeons with a paediatric subspecialty interest.
Operations are carried out on paediatric lists with the involvement of paediatric anaesthetists, nurses and other healthcare professionals. Almost all would agree that this is a
significant improvement in the delivery of ENT care for the paediatric population.
Declaration of
Competing Interests
None declared.
There has been
much debate
about paediatric
ear disease and
who should
manage it
Royal Hospital for Sick Children, Glasgow.
10
Specialising solely in paediatric otolaryngology was unheard of until around 20 years
ago. Now, some children’s hospitals in the
UK are staffed by a number of ENT surgeons
whose sole practice is paediatric. However,
there remain a significant number of
surgeons who work between the adult and
children’s sectors both in teaching and
district general hospitals.
Generally speaking, specialist paediatric
ENT surgeons will turn their hands to all
things paediatric – choanal atresia,
subglottic stenosis, thyroglossal cysts and
even cholesteatomas. In the adult sector,
ENT surgeons are often specialised in terms
of otology, rhinology, or head and neck
surgery. Partly as a consequence of this many
ENT surgeons have become reluctant to
cover children in the on call setting – often
citing a concern about managing the challenge of the difficult airway in a child. This
concern has resulted in debate, disagreement and even downright disorder in some
ENT departments in the UK!
Some would argue that having some
experience in adult ENT surgery can
enhance a surgeon’s paediatric practice and
vice versa. Others believe that it is in the
interest of both children and clinicians if the
two are entirely separate.
At The Royal Hospital for Sick Children,
Glasgow, I work with four full time paediatric
ENT colleagues in addition to an associate
specialist and a consultant audiological
physician. My practice is split approximately
30% paediatric and 70% adult with a
specialist interest in head and neck surgery,
both benign and malignant, and voice disorders. Three of my colleagues share the
complex paediatric airway work and hence I
do little in the way of assessment and intervention in the paediatric airway. I cover both
adults and children when I am on call and
for me (and hopefully my patients) this
works, but it has its challenges. In this article
I hope to illustrate the advantages and
disadvantages of a split workload through
my own experiences.
I will start with one of the most
contentious issues…
On call
The common paediatric emergencies
include post-tonsillectomy bleeds, tonsillitis,
neck abscesses, a variety of foreign bodies in
the nose and ear, traumatic injuries and less
commonly periorbital cellulitis and
mastoiditis. Inhaled foreign bodies are
uncommon and are usually referred to
specialist paediatric centres.
A few of the larger paediatric ENT departments in the UK have separate adult and
A child-friendly environment in a
paediatric centre.
feature
paediatric on call rotas. However, this is more
difficult to organise in smaller centres where
there may be only two or three ENT
surgeons with specialist paediatric skills.
Working a ‘1 in 2’ or ‘1 in 3’ is no longer
accepted by most and a compromise is
usually sought. In Glasgow we now have a
hybrid rota with three consultants covering
both adults and children, a further four
colleagues covering children only and the
remainder covering adults only. Does this
work? Well, it’s not perfect. It’s difficult to
make a swap when you cover both rotas and
forward planning is essential. It is no doubt
busier for those of us who cover both rotas
and it is not possible to be in two places at
once. Personally, I have to ensure that I maintain my core skills in paediatric emergencies
(for airways please read on).
Some major ENT centres remain
committed to involve all consultants in
both the paediatric and adult rotas. There
is certainly the very reasonable argument
that consultants should endeavour to
maintain their core skills in all subspecialties including paediatrics. After all we still
expect otologists to deal with epistaxis,
rhinologists to manage neck trauma and
head and neck surgeons to drain a mastoid
abscess in the emergency situation.
Fortunately most of us are in the position
that we have helpful colleagues who can
assist us when we encounter challenging
patients.
To maintain skills in paediatric airway
intervention I would suggest attending the
occasional specialised paediatric airway list
and instructional paediatric airway course.
It is necessary to understand how to
perform a tracheostomy on a young child
and to recognisie the differences from the
adult procedure, in addition to being
comfortable in assembling a paediatric
bronchoscope.
From a personal point of view I very
much enjoy seeing routine paediatric ENT
patients and it is a refreshing contrast to my
adult head and neck workload in the West
of Scotland!
Head and neck surgery
Fortunately, the majority of head and neck
pathologies in children are benign –
congenital cysts and sinuses, cervical
lymphadenopathy and so on; interesting
and rewarding to manage. Malignancies in
the head and neck region in children are
rare and I have found my experience in the
adult sector to be invaluable in this situation. Regularly performing for example
thyroidectomies and neck dissections in
adults is extremely useful when faced with
the rare thyroid carcinoma in a child.
Having access to the adult head and neck
managed clinical network has been invaluable when considering the treatment of
rare head and neck tumours, perhaps
more often seen in adults. For instance I
managed a 12 year old boy with a laryngeal
synovial cell sarcoma who ultimately
required a total laryngectomy. Decision
making was gratefully shared with the
network and my adult head and neck
team including the theatre staff and
speech and language therapist were invaluable in the management and rehabilitation
of this young man.
Airway
I work in the recognised national paediatric
airway centre for Scotland and the vast
majority of paediatric airway work is
performed electively. Three of my
colleagues take on most of this work and I
am fortunate that they make themselves
available to deal with the problems that
sometimes arise.
Otology
General paediatric ENT surgery
The vast majority of children seen in ENT
clinics have problems related to tonsillitis,
middle ear effusions, sleep disordered
breathing and rhinitis. This is of course
managed extremely well by the majority of
general ENT surgeons in teaching and
district general hospitals. Indeed, the
limiting factor in continuing to see children
outwith specialist centres is often down to
the anaesthetists and their desire to maintain their paediatric anaesthetic skills. No
doubt this has been frustrating for many
capable surgeons and has resulted in an
increased workload of routine ENT procedures in specialist centres. But that is a
subject for another day…
There has been much debate about paediatric ear disease and who should manage it.
Traditionally, adult otologists have provided
this service to paediatric hospitals but the
workload has in general been taken up by a
number of paediatric surgeons with a
particular otological subspecialty interest.
What is well recognised is that surgery
should be performed by those familiar with
the anatomical and pathological variations
in young children – and that surgery should
be something that he or she performs on a
regular basis.
In Scotland we have one national
cochlear implant centre which is situated
at Crosshouse Hospital in Ayrshire. There
the surgeons operate on both adults and
children. However, a small number of the
very young and children with complex
problems are operated on by these same
surgeons at Yorkhill because of the availability of paediatric ICU and specialist anaesthetists and paediatricians. This situation
works well for us and we have a good relationship with our implantation colleagues.
The insertion of bone anchored hearing
aids and aural reconstructions have often
been performed by otologists working in
the adult sector but an increasing number
of paediatric surgeons have developed a
specialist interest and expertise in this field.
Rhinology
Apart from the snotty nose, adenoidal
hypertrophy and allergic rhinitis much of the
pathology found is unique to paediatrics –
choanal atresia, nasal dermoids, encephaloceles and so on. Functional endoscopic
surgery is rarely required (for example cystic
fibrosis) and one could argue that this might
be an instance where the adult rhinologist
may have specific skills to offer.
Logistics
Working between the two sectors usually
means working at more than one site. Not
uncommon these days but there are logistical challenges associated with having two
secretaries, two IT systems, two destinations
for postal mail, and the supervision of two
surgical teams. In addition, I find myself asked
to commit to teaching, training and administration commitments in both sectors...
Summary
In practice most UK paediatric ENT centres
are staffed by a combination of those who
work solely with children assisted by a variable number of surgeons who have a
combined adult / paediatric practice. The
challenge of providing an adequate paediatric on call rota continues. Surgical expertise
should be provided by those with specialist
paediatric skills but that does not necessarily
mean these skills cannot be provided by a
surgeon who also works in the adult sector.
In my opinion and from personal experience combining adult and paediatric practice is feasible. I find it enjoyable and
rewarding despite the challenges. It does
however depend on having a good working
relationship with your colleagues and I am
indebted to mine.
I thank my paediatric colleagues
throughout the UK who have provided me
with information regarding their local
paediatric services and their (differing!)
opinions on the feasibility of combined
practice. n
11
Failed Extubation in Children:
assessment and management
options
Mr Joe Grainger,
FRCS,
ENT Surgeon.
D
espite neonatologists’ and paediatric intensivists’ best efforts to reduce
laryngeal injury during periods of intubation, approximately 5% of children
spending time on the Paediatric Intensive Care Unit (PICU) will require an
unanticipated reintubation within 48-72 hours of being extubated. One of the roles of
the paediatric ENT surgeon is to assist in the assessment and management of the child
who ‘fails’ extubation, usually following critical illness or major surgery. This article aims
to provide an overview of the assessment of these children and look at some of the
current management strategies.
Mr Michael Kuo,
FRCS,
Children’s Ear, Nose &
Throat Surgeon.
Correspondence
Birmingham Children’s
Hospital, Steelhouse
Lane, Birmingham,
B4 6NH, UK.
Declaration of
Competing Interests
None declared.
Initial assessment
Although the child who has failed extubation is likely to have been reintubated long
before you as an ENT surgeon arrives on
PICU, there is a lot of information to be
gleaned from both the staff on the unit
and the parents of the child. The history
surrounding the intubation episode will
give you a significant insight into the likely
causes of the failure and whether or not an
endoscopic examination of the larynx is
likely to be helpful.
What do you want to know?
A reasonable approach would be to find
out about three areas: the child’s history
prior to intubation, the reason for the intubation and what happened around the
time of the trial of extubation.
Were there problems during the pregnancy or following delivery? If the child
spent time on the neonatal unit, try and
ascertain if they were intubated and if so
how long for. Parents frequently get
confused between intubation for ventilation and the passing of a nasogastric tube
so try and be as clear as possible. Did the
child have noisy breathing of any kind and
was this ever investigated? If they are
slightly older, have they had issues such as
recurrent croup as this may suggest underlying airway disease?
Was this admission and intubation
related directly to an airway problem or
was it for another reason? Frequently, children who fail extubation are not known to
have a pre-existing airway problem – they
may have been intubated for surgery or for
a medical reason such as severe sepsis.
These children may have an underlying
mild airway problem that has been
12
exacerbated by intubation, or may have
developed an airway issue solely as a
result of the intubation episode. Upper
airway factors such as micrognathia will
also have a role and an assessment of the
oropharnx should form part of the assessment process.
What happened during the admission
and around the time of extubation is
crucial. A knowledge of the child’s ventilatory requirements and oxygen requirements will indicate whether there is an
underlying chest problem. Ideally, prior to
extubation, the child should require an
FiO2 of less than 0.35 and minimal pressure
support. High pressures and a large oxygen
requirement may suggest a lower respiratory tract problem. The endotracheal tube
size and whether there is an air leak will give
an indication as to the size of the
subglottis, although with the increasing use
of low pressure cuffed paediatric tubes, this
information is becoming less useful.
Overall, prior to extubation the child
should be as well as possible from a cardiac
and respiratory point of view in addition to
having as little sedation on board as
possible.
A good relationship between the ENT
surgeon and PICU is vital. Whilst in the past
it was commonplace for intensivists to
attempt extubation multiple times before
contacting their local ENT surgeon, it is
now becoming more common to involve
the ENT surgeon at a much earlier stage.
A further trial of extubation may be
appropriate if there are factors that can be
corrected or improved. However, repeated
extubation and reintubation cycles should
be avoided as these are likely to result in
further laryngeal damage. Twenty-four to
feature
Table 1. Common causes of failure to extubate.
Respiratory
Cardiac
Neurological
Above the larynx
choanal atresia
Pierre-Robin sequence
Laryngeal
laryngomalacia
bilateral vocal cord immobility
granulation tissue/oedema
subglottic cysts
subglottic stenosis
Below the larynx
repeated extubation
and reintubation
cycles should be
avoided
tracheomalacia
bronchomalacia
Figure 1: Intubation related granulation tissue of
the glottis – small areas will usually resolved
spontaneously following extubation.
Figure 2: Subglottic cysts.
48 hours of dexamethasone (100 micrograms / kg 8 hourly) along with anti-reflux
medication (ranitidine 4mg / kg 8 hourly or
omeprazole 750 micrograms / kg 12
hourly) may facilitate extubation by
reducing oedema and is generally recommended.
Airway endoscopy
Assuming that the child's cardiorespiratory
status was optimal for their trial of extubation, an airway endoscopy in theatre is
likely to be required if the child needs reintubation. This allows the diagnosis to be
established, appropriate treatment to be
commenced and provides reassurance to
those looking after the child. However, if
there are factors that can be corrected,
these should be addressed prior to considering endoscopy. It is always preferable to
perform an assessment on an airway that
has not been recently intubated.
A formal endoscopic assessment of the
airway is performed following removal of the
endotracheal tube in the operating theatre.
An assessment of vocal fold mobility should
be made. The Storz ventilating bronchoscope provides a safe means of assessing the
airway whilst at the same time facilitating
ventilation if necessary. The summary of the
potential causes of failed to extubation can
be found in Table 1 although this list should
not be regarded as exhaustive.
Figure 4: Early soft subglottic stenosis.
Figure 3: Image of the airway seen in Figure 2,
following endoscopic deroofing of cysts.
laryngeal rest. The child is intubated for a
period of around two weeks without a
further trial of extubation during this
time. A smaller tube size is utilised and
anti-reflux treatment is optimised.
Histological studies have shown that
mucosal ulceration may heal without
stenosis and therefore, laryngeal rest may
represent an alternative option to the
cricoid split. However, a two week period
of intubation on PICU is not without its
problems and this option is therefore used
infrequently.
Removal of granulation and deroofing of cysts
Figure 5: Balloon dilatation of the subglottis for
a soft granular stenosis.
Initial management
Once the diagnosis has been established,
treatment can be tailored accordingly. The
therapeutic options include the following:
A period of 'laryngeal rest'
Providing there is no established firm
subglottic stenosis, minor ulceration and
oedema may settle with a period of
Intubation related granulation tissue and
cysts are a common finding (Figures 1 and
2). Small areas of granulation can be
removed endoscopically following the
application of topical epinephrine.
Subglottic mucous retention cysts can be
carefully de-roofed aiming to leave an area
of undamaged mucosa in between cysts to
prevent circumferential fibrosis (Figure 3).
Balloon dilatation
Extensive granulation and soft stenosis
around the endotracheal tube responds
well to balloon dilatation (Figures 4 and 5).
Radial dilatation crushes granulation and
reduces damage to normal mucosa.
Balloon dilatation may also be used where
13
feature
Figure 6: Suggested algorithm for the management of children who have failed attempted extubation.
there is an element of firmer stenosis after
radial division of the fibrous tissue along
with local infiltration of corticosteroid.
Cricoid split
The anterior cricoid split was first described
by Cotton and Seid in 1980. It is best utilised
in neonates that have failed extubation
because of significant oedema and developing subglottis stenosis as an alternative to
tracheostomy. The cricoid ring is divided
anteriorly, allowing an age appropriate
endotracheal tube to be passed and any
glottic oedema to disperse. The larynx is
then rested for five to seven days before a
further trial of extubation. The original
procedure was described without a graft,
however, both thyroid and hyoid cartilage
are readily available in the operative field
and may be used as in a laryngeal reconstruction to hold open the cricoid ring. Up
to 90% of children will be successfully extubated after an anterior cricoid split, thus
avoiding the need for tracheostomy.
The cricoid ring can also be split endoscopically using a sickle knife. This technique is best performed by those with
significant endoscopic airway experience in
order to prevent complications and ensure
adequate division.
Tracheostomy
Whilst a tracheostomy may be regarded as
a failure by some, for children who have
14
significant chest disease, severe laryngeal
disease, bilateral vocal cord immobility or
have failed extubation attempts multiple
times, it provides a means of providing a
secure airway that can be managed on a
ward and avoid the associated morbidity
of prolonged ventilation. A tracheostomy
enables the larynx to settle and enables
airway surgery to be performed electively.
facilitate successful extubation. Therefore,
unless there are correctable cardiorespiratory issues, diagnostic endoscopy should
be utilised early in cases of extubation
failure in order to prevent further mucosal
damage. A suggested algorithm for the
management of children who have failed
extubation is shown is Figure 6. n
Subsequent management
Even following successful extubation, the
subglottis will continue to evolve over the
following weeks as areas of ulceration heal
and any residual granulation tissue fibroses.
Subsequent follow-up of these children is
therefore required until their airway is
stable in order to identify developing
stenosis. Many will have sustained significant mucosal damage and may require
more definitive surgery including laryngotracheal reconstruction.
Summary
‘Failure of extubation’ may be due to many
causes and it is important that cardiorespiratory function is optimised prior to
further attempts. A detailed history
relating to the child’s preintubation status,
their reason for intubation and the events
surrounding their extubation attempt are
important to establish. The paediatric ENT
surgeon has, at their disposal a number of
endoscopic and open techniques to
FURTHER READING
Albert D. Post intubation laryngotracheal stenosis. In Pediatric ENT. Edited
by Graham JM, Scadding GK, Bull PD).
Berlin: Springer; 2007:223-7.
Baisch SD, Wheeler WB, Kurachek SC,
Cornfield DN. Extubation failure in
pediatric intensive care: incidence
and outcomes. Pediatr Crit Care Med
2005;6(3):312-8.
Cotton RT, Seid AB. Management of
the extubation problem in the premature child. Anterior cricoid split as
an alternative to tracheostomy. Ann
Otol Rhinol Laryngol
1980;89(6 Pt 1):508-11.
Eze NN, Wyatt ME, Hartley BE. The
role of the anterior cricoid split in
facilitating extubation in infants.
Int J Pediatr Otorhinolaryngol
2005;69(6):843-6.
Lusk RP. Congenital anomalies of the
larynx. In Ballenger’s
Otorhinolaryngology. Edited by Snow
JB, Wackym PA. Connecticut:
BC Decker Inc; 2009:815-28.
Miss Ann-Louise
McDermott,
BDS, FDSRCS, PhD,
FRCS(ORL-HNS).
N
asal and sinus problems are very common in children however clinicians should
be mindful that they do have a risk of serious morbidity and even mortality if not
managed appropriately. The following article highlights the main paediatric emer-
gencies and their management.
1. Choanal atresia
Mr Tom Beech,
MSc, FRCS(ORL-HNS).
Correspondence
Miss Ann-Louise
McDermott, BDS,
FDSRCS, PhD,
FRCS(ORL-HNS),
Department of Paediatric
Otorhinolaryngology,
Hospital, Steelhouse
Lane, Birmingham,
B4 6NH, UK.
Declaration of
Competing Interests
None declared.
Although nasal and
sinus problems are
very common in
children, clinicians
should be mindful
that they do have a
risk of serious
morbidity and even
mortality if not
managed
appropriately
Choanal atresia was first described by
Roederer in 1775 and is now accepted as one
of the more common congenital abnormalities of the nose.1 It is thought to affect 1:8,000
live births and females are more commonly
affected. Choanal atresia may be bilateral or
unilateral. The unilateral case may not
become apparent until later childhood and
may only cause difficulties during breast
feeding when the unaffected side is occluded
by contact with the breast.
Association with other malformations is
reported and CHARGE syndrome is the most
widely reported. Paediatric assessment and
screening for CHD 7 mutation is recommended for infants with bilateral choanal
atresia. Bilateral choanal atresia typically
presents as a respiratory emergency very soon
after birth as neonates lack the ability to
mouth breathe.
Previous diagnostic tests have included bilateral failure of nasal misting on a metal spatula
and failure to pass a soft suction tube through
each nasal passage. The gold standard test
however is computerised tomography (CT).
This confirms the diagnosis, indicates whether
the atresia is bony, membranous or mixed and
furthermore the thickness and relation to other
anatomical structures can be assessed.
The airway needs securing in the first
instance, usually by means of a endotracheal
airway although modifications of a nipple
from a feeding bottle have been described.2
Definitive surgery is recommended in the first
week of life to create bilateral choanal patency.
Orogastric tube feeding is necessary if surgery
is delayed. Endoscopic intranasal choanotomy
techniques are the most commonly
performed and the removal of the posterior
vomer is common practice.
Surgery for choanal atresia has a significant
risk of re-stenosis. It is reported to be higher in
children with CHARGE syndrome. It is estimated that a mean of 1.5 to 3 operations are
required per patient.2,3 The application of
mitomycin C and the use of choanal stents are
still popular with many surgeons in an
attempt to reduce the re-stenosis risk.
2. Anterior pyriform aperture
stenosis
Congenital nasal pyriform aperture stenosis is
an unusual cause of nasal emergency
presenting as respiratory distress in neonates.
The problem is caused by bony overgrowth of
the nasal process of the maxilla. The clinical
presentations mimic those of bilateral choanal
atresia and a CT scan is required to confirm the
diagnosis. A pyriform aperture width of 11mm
or less associated with a single central incisor, in
a term baby is diagnostic.4,5
Early management is to secure the neonatal
airway. Bony overgrowth of the pyriform aperture is removed via a sublabial surgical
approach. The use of nasal stents is common in
the postoperative period. Not all neonates
require surgery. Some may be managed conservatively with a simple nasal airway.
3. Sinusitis and its complications
Rhinosinusitis is very common in children, and
often more extensive than seen in adults. It
also has the tendency to resolve spontaneously as the child grows and develops.
Acute rhinosinusitis commonly follows an
URTI and can have severe complications with
spread of infection locally into the orbit with
threat to vision and also intracranial spread
with a mortality rate quoted as high as 2030%.3 A child presenting with periorbital
cellulitis requires an urgent ENT and ophthalmological examination.
The presence of proptosis, chemosis, restriction of eye movement, red colour desaturation
and reduced visual acuity should alert the clinican to the urgency of the situation.
Nasal decongestion and broad spectrum
antibiotic therapy should be commenced
immediately. It is difficult to clinically identify
whether cellulitis is pre- or postseptal. An
urgent CT scan with intravenous contrast of
the paranasal sinuses, orbits and brain is the
investigation of choice.
15
feature
Urgent drainage of the affected sinuses
should be performed and orbital infection
may be drained either by an endoscopic and
/ or external approach.
For those children with intracranial
complications, close cooperation with
neurosurgical team is necessary. Early surgical
drainage of the involved sinuses is necessary
and should ideally be performed at the time
of the neurosurgical drainage procedure.
4. Nasal foreign bodies
This group of patients constitutes the largest
component of paediatric nasal emergencies.
Typically the history includes nasal obstruction, foetor and unilateral rhinorrhoea. The
important factor is the nature of the foreign
body. Ideally the foreign body should be
removed in the outpatient setting at the
time of presentation however some children
require a general anaesthetic.
Controversy exists regarding the true risk
of aspiration of nasal foreign bodies if they are
not removed immediately. Review of the literature reveals very little evidence to support
this aspiration risk.6 Inert and nontraumatic
nasal foreign bodies are managed on a semielective basis however foreign bodies such as
the ‘button’ battery are urgent. The button
battery rapidly causes tissue destruction
when placed in a damp environment such as
the nose, and results in extensive soft tissue
destruction which in turn leads to nasal
deformity. Examination of both nostrils is
advised after foreign body removal to ensure
no other foreign matter is present.
5. Epistaxis
This a very common problem in childhood
but it is often self limiting and of short duration. Epistaxis is most commonly seen in children between the age of three and eight years.
Clinicians should have a high index of suspicion for any child that presents with excessive
bleeding that fails to settle conservatively.
Epistaxis may be the first sign of underlying
systemic disease or tumours such as an
angiofibroma. The management of paediatric
epistaxis depends on the age of the child.
Adequate resuscitation is the first line of
treatment for epistaxis. Unlike adults
however, it is rarely required in children.
Digital pressure should be applied to
control the bleeding. Topical application of
cotton wool soaked in local anaesthetic to
the nose is often not tolerated by younger
children. Silver nitrate topical nasal cautery
may be used. Soft and malleable (resorbable)
nasal packs such as Nasopore may also be
considered.
In children known to have a bleeding
diathesis, cautery and nasal packing is best
avoided wherever possible and haematology
16
Figure 1: Axial CT scans demonstrating bilateral bony and membranous choanal atresia.
Figure 2: Axial CT scans illustrating Pyriform Aperture Stenosis and a midline central incisor.
Figure 3. Sublabial surgical approach to widen the
nasal pyriform aperture.
Figure 4: Axial CT scan (without contrast) showing
subperiosteal collection within the right orbit and
complete opacification of the right ethmoidal air cells.
advice regarding administration of appropriate clotting factors should be sought.
6. Nasal trauma and septal
haematoma
Paediatric nasal trauma is a common presentation to the Accident and Emergency
department but not all cases are then referred
to ENT. In the face of any paediatric nasal
trauma it is important for the nasal septum
to be properly examined for any signs of a
septal haematoma. Failure to identify such an
injury may result in cartilage necrosis, abscess
formation and later a saddle deformity.
The external structure of the nose in
young children is predominantly cartilaginous and the nasal bones are soft. Fracture of
the nasal bones is therefore very rare particularly in children under the age of six years and
displacement is a more common finding.
Manipulation is usually all that is required for
such displacements.
A septal haematoma (both unilateral or
bilateral) requires urgent evacuation if the
cartilage is to be saved and abscess formation
is to be avoided. Broad spectrum antibiotic
therapy is advised for five to seven days. n
References
1. Devgan BK, Harkins WB. Congenital choanal atresia.
Twenty years experience. Int Surg 1977;62(8):397-9.
2. Froehlich P, Ayari-Khalfallah S: Management of
Choanal Atresia. In: Pediatric ENT. Edited by Graham
JM, Scadding GK, Bull PD. New York: Springer;
2007;291-4.
3. Samadi DS, Shah UK, Handler SD. Choanal atresia: a
twenty year review of medical co morbidities and surgical outcomes. Laryngoscope 2003;113(2):254-8.
4. Belden CJ, Mancusso AA, Schmalfuss IM. CT features
of congenital nasal piriform aperture stenosis: initial
experience. Radiology 1999;213(2):495-501.
5. Rollins N, Booth T, Biavati M. Case 40: congenital pyriform aperture stenosis. Radiology 2001;221(2):392-4.
6. Qureshi AA, Lowe DA, McKiernan DC. The origin of
bronchial foreign bodies: a retrospective study and literature review. Eur Arch Otorhinolaryngol
2009;266(10):1645-8.
Louise Melia,
MBChB, MRCS,
ST5 ENT,
Royal Hospital for Sick
Children, Yorkhill,
Glasgow, G3 8SJ, UK.
C
ervical lymphadenopathy in children is extremely common, and neck infections account for a significant proportion of acute paediatric otolaryngology
admissions and therefore resource allocation. Although a high proportion of
acute lymphadenitis resolve with antibiotic therapy, a significant number will progress
to a suppurative collection requiring surgical incision and drainage, as well as intravenous antibiotics and prolonged hospital stay.
Superficial lymph node abscess
Haytham Kubba,
Otolaryngologist,
Royal Hospital for Sick
Children, Yorkhill,
Glasgow, G3 8SJ, UK.
Correspondence
E: louisemelia07@
gmail.com
Declaration of
Competing Interests
None declared.
Acute lymphadenitis is common in children,
and usually presents as a swollen lymph
node or nodes after an upper respiratory
tract infection (URTI). Superficial lymph
node abscesses tend to present with a triad
of neck swelling, neck pain and pyrexia. In
addition, patients may have poor oral
intake. Children may have signs or symptoms of sepsis, and may be dehydrated, and
this needs addressed immediately.1
Lymph node abscesses are usually of
bacterial aetiology, with infections potentially arising from the oropharynx or anterior nares. The most frequently isolated
organism is Staphlococcus aureus.2,3
Treatment for superficial neck abscesses
is surgical and medical. Small abscesses of
less than 3ml volume can be treated with
intravenous antibiotics only. Initial surgical
incision and drainage with cultures is
reserved for patients presenting with
typical rapid onset neck mass and symptoms consistent with abscess such as fever
and increased white blood cell count.4
The main issue for the surgeon is to
distinguish those children who require incision and drainage from those who can be
managed conservatively. Clinical assessment for fluctuance, and ultrasound for
liquefaction are the usual means for doing
this, although both are fallible.
Peritonsillar abscess
This can be difficult to diagnose. In a frightened, toxic child it may not be possible to
examine the oropharynx due to trismus and
poor co-operation. An experienced ultrasonographer may get surprisingly good
views of the tonsil to allow diagnosis. Some
cases will improve with intravenous antibiotics, but most will need to proceed to
surgery with a presumptive diagnosis only. A
senior anaesthetist and a gas induction are
mandatory. The trismus will usually relax
once the child is anaesthetised. At this point,
a tonsillectomy is probably the most reasonable course of action if quinsy is confirmed.
Floor of mouth
Infection in the floor of mouth is usually
secondary to a dental source, and can
present with swelling in the submandibular,
submental or submasseteric regions
(Figures 1a and 1b). Life threatening airway
compromise can develop rapidly and intubation or tracheostomy may be needed.
Management should include drainage of
the abscess as well as treatment of the
primary infective source, therefore a dentist
or maxillofacial surgeon should be involved.
Antibiotics must provide anaerobic cover.
Deep neck space infections
Figure 1a: Floor of mouth infection.
Figure 1b: Intraoperative image of floor of
mouth infection.
Deep neck infections spread along the
fascial planes and spaces of the head and
neck region. Despite the widespread use of
antibiotics for the early treatment of
cervical infections and worldwide
17
feature
Figure 2: CT scan of large parapharyngeal
abscess.
improvements in dental care and oral
hygiene, deep neck infections remain relatively frequent. Life threatening complications such as airway damage, jugular vein
thrombosis, mediastinitis, pericarditis, pneumonia, and arterial erosion may develop
because of delays in diagnosis and treatment.5
Abscesses in the retropharyngeal space
often present as airway emergencies in
infants. They develop from suppuration in
retropharyngeal lymph nodes. The presentation is similar to epiglottitis with pyrexia,
drooling and soft stridor. Drainage can be
achieved transorally, but an experienced
anaesthetist is essential to secure the airway.
Infection in the parapharyngeal space can
be due to dental infection or tonsillitis as well
as the usual URTI. Presentation can be insidious with little external swelling in the neck
and only ‘soft’ signs such as low grade
pyrexia, malaise and torticollis. CT scanning is
helpful in confirming the diagnosis and planning the surgical approach (Figure 2).6 An
external approach allows a drain to be placed
to prevent reaccumulation. In some cases,
depending on the position of the abscess in
relation to the great vessels and oropharyngeal mucosa, it may be possible to drain
intraorally and avoid a scar, but the risk of
reaccumulation is higher.
Atypical mycobacterial infection
This is surprisingly common and often misdiagnosed. The organisms (M. bovis, M. avium
intracellulare, and so on) are widely present
in the soil and in the limescale deposits in
your shower head. They enter via oral ingestion and pass to the regional lymph nodes,
typically parotid and submandibular nodes.
The child presents with a neck lump, which
develops violet-red skin discolouration, with
eventual skin breakdown (Figure 3a). A sinus
discharges for months before healing with a
puckered scar (Figure 3b). This whole process
can take up to three years to complete.
18
Figure 3a: Typical appearance of atypical
mycobacterial infection.
The diagnosis is clinical on the basis of the
characteristic skin appearance in a child who
remains systemically well and apyrexial and
whose history is longer than would be
expected for a bacterial abscess (usually a few
weeks). Aspiration is controversial: while it
may provide a sample for Ziehl-Neelsen
staining, this comes at the cost of potentially
causing skin breakdown and a discharging
sinus, compromising the ultimate cosmetic
result. Incision and drainage should clearly be
avoided for the same reason. Culture takes six
weeks and is only positive in a minority of
cases. A chest x-ray is mandatory, but tuberculin testing is only necessary for children at
high risk of TB.
Some children will resolve spontaneously
in a short time, or respond to prolonged
treatment (3-9 months) with antibiotics.
Single-agent regimens (clarithromycin,
ciprofloxacin) and multi-agent regimens have
been used (clarithromycin plus ethambutol,
isoniazid or rifabutin). How many children
will settle with antibiotics is currently
unknown. For the remainder, surgery should
be considered if it will shorten the duration
of the disease and if the cosmetic result is
likely to be better than that resulting from
natural resolution.
If antibiotics are to be used, follow-up
should be early and frequent to identify
those who are not resolving: in these children surgery should be done early to prevent
skin loss and optimise the cosmetic
outcome. Surgical excision is the treatment
of choice if the skin is at risk of breakdown or
if the disease progresses despite antibiotic
therapy. Surgery is difficult due to the large
mass of matted nodes and overlying collarstud abscesses in the subcutaneous plane. A
block dissection of lymph nodes will suffice
for limited disease but a partial parotidectomy and supraomohyoid neck dissection
are not uncommonly required for extensive
disease. Discoloured skin can usually be
saved if it is in a cosmetically obvious place
Figure 3b: Burnt-out atypical mycobacterial
infection allowed to run its course without
treatment over 2.5 years – early surgery is
recommended to avoid this sort of cosmeticallyobvious scarring.
(such as the cheek) by curetting the subcutaneous abscess from the deep surface of
the dermis. The skin colour will eventually
return to normal. In such cases, we would
suggest clarithromycin for three months
postoperatively, but there is currently no
evidence to support this. In cases where the
skin has already broken down, skin must
either be excised or the disease left alone to
run its course.
Conclusion
Neck abscesses in children can present in a
variety of forms. Clinical assessment should
be used in conjunction with ultrasound and
CT scanning to determine those children
that require surgical management with incision and drainage. It is vital that the airway
remains safe throughout, and that an experienced anaesthetist is involved in cases where
there is any concern. Dentists or maxillofacial
surgeons need to be involved if there is any
suspicion of a primary dental source. n
References
1. Butler KM, Baker CJ. Cervical lymphadenitis.
Paediatric Infectious Diseases. Philadelphia: WB
Sauders Company; 1992:220-30.
2. Simo R, Hartley C, Rapado F, Zarod AP, Sanyal D,
Rothera MP. Microbiology and antibiotic treatment
of head and neck abscesses in children. Clin
Otolaryngol Allied Sci 1998;23(2):164-8.
3. Inman JC, Rowe M, Ghostine M, Fleck T. Pediatric
neck abscesses: changing organisms and empiric
therapies. Laryngoscope 2008;118(12):2111-4.
4. Dodds B, Maniglia AJ. Peritonsillar and neck abscesses
in the pediatric age group. Laryngoscope
1988;98(9):956-9.
5. Marioni G, Staffieri A, Parisi S, Marchese-Ragona R,
Zuccon A, Staffieri C, Sari M, Speranzoni C, de Filippis
C, Rinaldi R. Rational diagnostic and therapeutic
management of deep neck infections: analysis of 233
consecutive cases. Ann Otol Rhinol Laryngol
2010;119(3):181-7.
6. Wetmore RF, Mahboubi S, Soyupak SK. Computed
tomography in the evaluation of pediatric neck
infections. Otolaryngol Head Neck Surg
1998;119(6):624-7.
Congenital Midline Nasal
Masses Compared
Mary-Louise
Montague, MBChB
(Hons), FRCS ORLHNS,
Consultant
Otolaryngologist,
Honorary Senior Clinical
Lecturer, University of
Edinburgh,
Edinburgh, UK.
Correspondence
Department of ENT
Surgery, The Royal
Hospital for Sick
Children, 9 Sciennes
Road, Edinburgh, EH9
1LF, UK.
E: mary-louise.montague
@luht.scot.nhs.uk
Declaration of
Competing Interests
None declared.
N
asal dermoid cysts, encephaloceles and gliomas are the most frequently
seen congenital midline nasal masses. Overall congenital midline nasal
masses are rare. Although rare they are clinically important because of their
potential for connection to the central nervous system. Their potential to cause disfig-
urement, destruction of the cranial base and meningitis should not be underestimated.
This article compares the embryological development, clinical presentation, optimal
imaging, complications and surgical management of nasal dermoid cysts, encephaloceles and gliomas.
Embryology
Congenital midline nasal masses are
believed to share similar embryogenetic
origins. A failure of the normal separation
of the different germ cell layers is considered to be causative. Consequently, all
patients with congenital midline nasal
masses must be considered as potentially
having an intracranial extension.
During formation of the skull base and
nose the mesenchymal structures are
formed from several centres which eventually fuse and ossify. Before fusion there are
recognised spaces between these elements
– the fonticulus frontalis, prenasal space,
and foramen cecum (Table 1). They are
important in the development of congenital midline nasal masses.
During development dura projects
through the foramen cecum and attaches
to skin. This connection is normally lost
when dura separates from nasal skin and
retracts through the foramen cecum. If skin
maintains an attachment to the underlying
fibrous tissue, nasal capsule, or dura,
epithelial elements may be pulled into the
prenasal space with or without dural
connection, this predisposing to dermoid
cyst formation.1
The hypothesis for the formation of
gliomas is similar to that of nasal dermoids.
They are thought to develop from extracranial rests of glial tissue persisting after
abnormal closure of the fonticulus frontalis.
A second theory hypothesises that they may
represent encephaloceles that have lost their
cerebrospinal fluid (CSF) connection.
Encephaloceles are thought to develop
as a result of abnormal closure of the
fonticulus frontalis resulting in herniation
of meninges with or without brain tissue.
Clinical presentation
Of the three anomalies, nasal dermoid
cysts are the most common accounting for
approximately 60% of all congenital
midline nasal lesions.2 The mode of presentation and other characteristics of these
lesions are compared in Table 2. A positive
Furstenberg test refers to expansion of a
lesion with crying, Valsalva manoeuvre, or
compression of the ipsilateral jugular veins.
Optimal imaging
All suspected congenital abnormalities of
the nose require radiological evaluation,
the objective being to confirm the clinical
diagnosis, delineate any intracranial
involvement and detect associated abnormalities. It is crucial not to biopsy or excise
any intra- or extranasal mass in infants and
children prior to complete work-up,
Table 1. Potential spaces important in the development of congenital
midline nasal masses.
●
●
●
Fonticulus frontalis – space between the frontal and nasal bones which
eventually fuses with the foramen cecum to create a separation between
intracranial and extracranial structures
Prenasal space – space between the nasal bones and the nasal capsule (the
precursor of the septum and nasal cartilages)
Foramen cecum – formed by articulation of a small notch at lower end of
the frontal crest of the frontal bone with the ethmoid
19
feature
Table 2. Nasal dermoid cysts, encephaloceles and gliomas compared.
Nasal Dermoid Cyst
Nasal Encephalocele
Nasal Glioma
Sex predilection
Occur equally
between sexes
More frequent in males
Occur equally between sexes
Presentation
Slow growing midline
mass, midline sinus or
combination of these
Midline mass
Firm mass
Nasal obstruction
Slow growing firm, non
pulsatile unilateral nasal
Hypertelorism
Polyp – reddish coloured
sometimes with telangiectasias
Hair protruding from
sinus tract/cyst
pathognomonic
Site
Broad nose
Intermittent discharge of
sebaceous material
CSF Rhinorrhoea
Nasal obstruction
Inflammation/abscess
Recurrent meningitis
CSF Rhinorrhoea
Anywhere from glabella
to philtrum of lip. Lower
1/3 of nasal bridge most
common
Root of nose or inferior to
nasal bones
60% Extranasal – near
root of nose
30% Intranasal – typically
medial to middle turbinate
10% Combined
a
c
Transillumination
No
Yes
Compression
May be fluctuant
-ve Furstenberg test
Soft and compressible
+ve Furstenberg test
Non compressible
-ve Furstenberg test
Contents
Skin and dermal
elements including hair
follicles, sweat glands
and sebaceous glands
Meninges with or without
brain tissue
Benign masses of glial
tissue containing large
aggregates of astrocytes
and fibrous connective
tissue enveloping blood vessels
Intracranial extension
Up to 20-45%
All
15%-20%
Frequency of associated
abnormalites
40% associated with
craniofacial
malformations
30-40%
Generally isolated anomalies
b
d
Figure 1: CT and MRI demonstrating a nasal dermoid cyst with
intracranial extension. (a) Axial CT showing separation of the nasal
bones. (b) Widening of the foramen cecum on coronal CT. (c & d) T1
and T2-weighted sagittal MR views respectively showing multiple
cysts extending intracranially with attachment to dura.
20
No
including imaging, due to the risk of CSF leakage and meningitis if there is an
intracranial connection.
The complimentary roles of high resolution computed tomography
(HRCT) and multiplanar MRI in congenital midline nasal masses are much
quoted in the literature.3 But MRI is undoubtedly the best imaging modality
with the distinct advantages of more precise soft tissue imaging, direct
sagittal imaging planes and the possibility to generate multiple tissue
contrasts. Many infants and children will require general anaesthesia for
adequate MRI evaluation but this is justified in the knowledge that surgical
planning is optimised.
Common CT findings in nasal dermoid cysts are bifid nasal bones, a
thickened or bifid nasal septum, and a circular translucency under the
nasal bones. The presence of a widened foramen cecum or a bifid crista
galli on CT suggests intracranial extension of the dermoid. Non visualisation of an intracranial mass on CT does not exclude the possibility of
intracranial extension. False positives may also be seen with CT and may
be accounted for by anatomic variants and incomplete ossification of the
crista galli and foramen cecum (14% of children younger than one year of
age). Nasal dermoids appear strikingly hyperintense on T1-weighted MRI
due to their fat content (Figure 1).
feature
Figure 2: A midline nasal dermoid cyst
presenting with local infection and abscess
formation extending superolaterally. The dorsal
midline pit or sinus is apparent.
Figure 3: Intranasal glioma obstructing the right nasal cavity of a neonate. This had no intracranial
attachment and was removed endoscopically with a microdebrider. A nasopharyngeal airway was
required preoperatively.
Nasal gliomas are usually isodense on
CT. Calcifications are reported rarely and
cystic changes occasionally within them.
On MRI nasal gliomas are usually hyperintense on T2-weighted images with variable
intensity on T1-weighted images.
Because of its high soft-tissue resolution MRI is particularly useful in imaging
encephaloceles. MRI demonstrates the
pathway of intracranial herniation and
the presence of associated anomalies
more clearly than CT. MRI will also differentiate encephalocele from glioma if an
adjacent communicating CSF space is
present.
The lack of ionising radiation associated
with MRI is especially important in children
who may on occasion require follow-up
imaging.
causing remodelling and deformity of the
nasal bones as well as progressive facial
disfigurement. Obstruction of the nasal
passage and nasolacrimal duct can occur
causing respiratory distress in the neonate
and epiphora on the affected side respectively (Figure 3).
Complications
Prompt diagnosis and management is
essential in light of the potential for
complications.
Dermoid cysts
Failure to recognise and treat this condition promptly may lead to progressive
enlargement of the cyst and bony and
cartilaginous distortion of the nose. There
is also potential for primary or recurrent
infection that may progress to loss or scarring of overlying skin, meningitis, brain
abscess, cavernous sinus thrombosis, or
periorbital cellulitis (Figure 2).
Encephaloceles and gliomas
Untreated these may be complicated by CSF
rhinorrhoea, meningitis and intracranial
abscess. Both may also expand gradually
Surgical management
Treatment of congenital midline nasal
masses requires early, well-planned, careful,
complete surgical resection.
Nasal dermoid cysts must be removed
intact as even a small epithelial remnant
can lead to recurrence. The external
rhinoplasty approach has largely replaced
midline vertical and lateral rhinotomy
incisions.4 It provides excellent exposure
of the nasal dorsum and allows sinus
tracts to be followed and removed in
their entirety by providing an adequate
angle of approach to and visualisation of
the anterior skull base. More control can
be exerted during osteotomies and the
aesthetic outcome is vastly superior
compared to a midline dorsal incision.
Cartilage grafts are occasionally required
for dorsal augmentation. For dermoids
with substantial intracranial components
a combined procedure with a neurosurgeon may be required.
A neurosurgical approach combined with
either an external rhinoplasty approach or
lateral rhinotomy is generally recommended
for encephaloceles and gliomas with
intracranial extension. A number of cases of
successful endoscopic microdebrider excision of small gliomas with and without
evidence of intracranial extension have now
also been reported. n
It is crucial not to biopsy
or excise any intra- or
extranasal mass in
infants or children prior
to complete work-up,
including imaging, due
to the risk of CSF
leakage and meningitis
if there is an
intracranial connection
References
1. Charrier JB, Rouillon I, Roger G, Denoyelle F, Josset
P, Garabedian EN. Craniofacial dermoids: an
embryological theory unifying nasal dermoid sinus
cysts. Cleft Palate Craniofac J 2005;42(1):51-7.
2. Brown K, Brown OE. Congenital Malformations of
the nose. In: Pediatric Otolaryngology Head &
Neck Surgery, 3rd edition. Edited by Cummings
CW. St Louis: Mosby; 1998:92-8.
3. Barkovich AJ, Vandermarck P, Edwards MS, Cogen
PH. Congenital nasal masses: CT and MR imaging
features in 16 cases. Am J Neuroradiol
1991;12(1):105-16.
4. Bilkay U, Gundogan H, Ozek C, Tokat C, Gurler T,
Songur E, Cagdas A. Nasal dermoid sinus cysts and
the role of open rhinoplasty. Ann Plast Surg
2001;47(1):8-14
5. Agirdir B, Derin A, Ozbilim G, Ozçaglar H.
Endoscopic management of intranasal glioma. J
Pediatric Surg 2004;39(10):1571-3.
21
Virus Infection of the
Paediatric Airway and Chronic
Disease Effects
Adam J Donne, PhD,
FRCS(ORL-HNS),
Honorary Senior
Lecturer.
W
ithin the realms of Paediatric ENT arguably the most important airway
infecting viruses are the types that result in bronchiolitis and Recurrent
Respiratory Papillomatosis (RRP). Both Respiratory Syncytial Virus (RSV)
and Human Papillomavirus (HPV) can result in chronic conditions. This article reviews
some of the evidence published on the effect of these viruses upon the paediatric
airway. This article does not intend to explore all viral aetiologies of bronchiolitis but
rather indicate the relative complexity of this condition.
Chronic airway disease implies a group of airway diseases, typically chronic obstrucMichael P Rothera,
Royal Manchester
Children's Hospital,
Oxford Road, Manchester,
M13 9WL, UK.
Correspondence
Adam J Donne, PhD,
FRCS(ORL-HNS),
Honorary Senior Lecturer,
Alder Hey Children’s NHS
Foundation Trust,
Alder Hey, Eaton Road,
Liverpool, L12 2AP, UK.
E: ajdonne@
doctors.org.uk
Declaration of
Competing Interests
AJD has spoken
/lecturered at events
sponsored by Sanof
Pasteur MSD.
22
tive pulmonary airway disease. RSV may indeed result in chronic airway disease
however this article focuses on the longer term viral effects. HPV infections can result
in a disease of chronic duration Recurrent Respiratory Papillomatosis.
RSV
who have a predisposed risk. A 20 year
prospective study reports that croup before
two years of age generates an increased risk
of asthma until adulthood independently of
development of atopy.3 Viruses are not the
only risk for bronchiolitis as passive smoking
is a significant risk.4
RSV usually causes simple coryzal symptoms
but it can result in bronchiolitis and pneumonia. RSV infection is common however,
premature babies are more at risk as they
have weaker immunity and a possibility of
chronic lung disease. Chronic lung disease of
prematurity is also termed bronchopulmonary dysplasia. It is thought to result from
damage to lung tissue especially in the very
premature hence low birth weight babies
requiring mechanical ventilation. RSV is associated with an increased rate of asthma.
Term babies who have RSV as infants are ten
times more likely to have bronchodilators by
eight to 10 years of age.1 This has been
confirmed in other studies that identified
asthma was present in 30% of children previously hospitalised for RSV compared to 3%
in age and gender-matched controls.2
Controversy exists as to whether RSV causes
asthma or simply highlights the children
Croup (laryngotracheobronchitis) is characterised by a barking cough, stridor and
hoarseness. Numerous definitions appear
to exist in the published literature but they
are based upon the clinical picture. Croup
is caused by a viral infection resulting in
swelling of the subglottis and on a chest
radiograph would classically give a church
steeple outline in the subglottis (Figure 1).
On endoscopy the subglottic inflammation is visible (Figure 2). Croup is most
commonly caused by parainfluenza virus
type 1 and 2, other causes include RSV,
Figure 1: Image of church steeple sign on chest
radiograph is characteristic of croup.
Figure 2: Endoscopic image demonstrating laryngeal
inflammation and reduced subglottic airway.
Croup
feature
coronavirus, adenovirus, metapneumovirus, etc.5 The severity of croup can be
classified by the Westley score into mild,
moderate or severe;6 though this is
primarily of research value.
Recurrent croup is poorly defined.
Originally, it was thought that recurrent
croup did not have a viral aetiology
however RT-PCR studies indicate that the
viral aetiologies are similar. Up to 6% of
children under four years suffer from recurrent croup.7 The causes of recurrent croup
include: Subglottic stenosis,8 Asthma 17%9
and GERD 60%.9 However, inhaled steroids
and GERD therapy result in an improvement in 77%.9 The rate of subglottic
stenosis in reports on recurrent croup does
vary and may reflect referral practices.
Recurrent Respiratory
Papillomatosis
RRP is caused by HPV, predominantly types
6 and 11. This condition affects children
and adults and typically has a more locally
aggressive nature in children but very rarely
undergoes malignant transformation. The
likelihood of transformation has resulted in
the terminology low risk (6 and 11) and
high-risk (types 16 and 18) of malignant
change. RRP is extremely difficult to treat
due to the recurrent nature and the varied
activity over the course of the disease.
Many fundamental questions about RRP
remain unanswered, perhaps the most
important being why so many people are
infected (albeit transiently) by HPV but so
few develop disease. There is geographical
variation in HPV prevalence from cervical
smear study data, however the prevalence
varies according to age and there in now a
recognised U-shaped age-dependent
distribution curve for prevalence.10 The
prevalence for the UK is approximately 520% depending upon age group.
Why does HPV manifest as RRP?
This may be due to a problem with host
immunity or HPV factors / cofactors which
are currently undefined.
Detection of HPV has improved over
the last 20 years. Originally techniques were
in situ hybridisation methods but PCR
techniques are more sensitive however
even these reply upon searching for a
specific DNA sequence. This explains why
additional HPV types have only recently
been identified in RRP tissue. Could it be
that different HPV have different prognostic potential? Many now believe that
HPV 11 causes more aggressive disease
than HPV6 though age of onset is more
important than either 6 or 11.11 There are
19 HPV6 variants and 10 HPV11 variants
Figure 3: Endoscopic image of Recurrent
Respiratory Papillomatosis.
and no commercially available test
attempts to identify these.12 It is highly
probable that the variants are important
and differ in activity. Indeed in vitro studies
go some way to prove this to be the case
for some variants.13
Clinical management
The endoscopic appearance of RRP is characteristic (Figure 3). There is difficulty in
defining how frequently to perform MLTB
surgery and papillomatous tissue clearance.
Obviously the first surgery depends upon
the extent of disease and potential for
airway compromise at presentation. The
second surgery should probably be around
four to six weeks later. This will allow an
assessment of the aggressiveness of the
condition, though each patient’s condition
does vary throughout its course.
There are numerous methods for papillomatous clearance but cold techniques
allow less collateral thermal damage hence
better voice quality outcomes than laser
techniques14 and the more laser procedures
the worse the voice quality.15 This, plus the
addition of more controllable techniques
such as small laryngeal microdebriders,
means that lasers seem to be used less
frequently now even though they do offer
a very precise area to be targeted.
Coblation is a relatively new method which
allows covalent bonds within tissue to be
broken down due to generation of a flux of
sodium ion yet the temperature generated
is not greater than 60°C. Irrespective of the
method used best practice should include
a tissue biopsy at every surgery as the
naked eye cannot differentiate between
papillomatosis and early carcinoma. This is
the safest way to ensure that an emerging
carcinoma is not missed.
Adjuvant therapy
There have been many adjuvant therapies
used, most with no direct scientific foundation for their use in RRP. The most
contemporary is cidofovir which is a nucleoside analogue which, on entering the cell
undergoes two stage phosphorylation
process. At this point the molecule resembles a nucleotide and can then be incorporated into expanding DNA. Scientific
studies have shown that cidofovir has
effects against HPV 16 (high risk)16 but no
study has demonstrated such an effect
with HPV6 or 11 (low risk).17 It was reasonable to assume that cidofovir may have
been effective and the patient is more
complex than a simple cell model.
However, evidence does show that cidofovir in altered human cells that contain an
HPV 6 gene (E6) results in changes in gene
expression and results in an environment in
which malignancy may develop.18 The cells
that did not contain HPV did not demonstrate the same gene expression. So the risk
of malignancy is cidofovir plus HPV.
Rodent studies have shown cidofovir to
be carcinogenic though this was not
found to be the case in primates.19 There
are no absolutely convincing reports of
carcinoma following cidofovir exposure
though it may be that the effects have not
been fully appreciated. For example
smoking is thought to be very carcinogenic yet only causes cancer in 10-15%.20
Radiation causes thyroid cancer but
anything from three to 60 years post exposure.21 It could be that cidofovir has a low
potency with a long duration before
malignant effect is seen. The in vitro work
demonstrated that at high enough doses
cidofovir killed all cells. However, at lower
(sub-lethal) doses a genetic environment
was created in which malignant transformation might be possible.18 HPV exists not
only at the point of the papillomas but
also other areas of the neighbouring
airway tissue and trachea.22 There must be
a lowering gradient of drug the further
away from the cidofovir injection site.
Indeed, it is conceivable that HPV infected
(but otherwise normal in appearance)
trachea might develop a malignancy if
exposed to this low dose. Indeed, such a
report does exist.23
At the very least when taking consent
for cidofovir administration to control RRP
a risk of ‘unquantifiable malignant potential’ should be identified to the patient.
Summarising the outcomes of cidofovir
treatments from numerous published articles it does appear that cidofovir has some
positive effect24 but it is not a cure and the
administration regime should be carefully
controlled. In vitro work has indicated that
sub-lethal doses appear to stimulate
growth of cells containing HPV genes and
this observation has been made clinically
with too long an interval between cidofovir administrations.25
23
feature
trials and initial post-marketing surveillance
conducted in several continents show
both vaccines to be safe.”27
Current research
Figure 4: Gardasil vaccine.
Alpha-interferon has historically been
used and summarising the results would
indicate that the numbers undergoing
some element of resolution is less than
with cidofovir. Interferon works by stimulating the immune response against virus
infections. The side-effects of alpha-interferon were frequently nausea and vomiting
with a potential for hepatic derangement.
The usage of an adjuvant should only be
considered when there have been at least
four interventions per year to control the
airway (RRP task force guideline).
Prevention is better than cure and it
seems appropriate to offer a prophylactic
vaccine if possible. Unfortunately the
current UK HPV vaccination programme
administers Cervarix which is effective
against high risk HPV whereas Gardasil is
also effective against HPV 6 and 11 (Figure
4). From the perspective of RRP it seems
more appropriate to offer Gardasil. There
have not been studies to evaluate the
effect of Gardasil directly upon the rate of
RRP due to numerous problems, not least
the relative rarity of the condition. Genital
warts (condylomata accuminata) are
caused by the same HPV types 6 / 11 and
it has been shown that Gardasil did reduce
the development of genital warts.26 The
implication is that Gardasil would do the
same for RRP. Given the potential concerns
of cidofovir safety it is reassuring that the
WHO reports that, “Data from clinical
Dr Farrel Buchinsky in Pittsburg, USA is
working to identify the genetic susceptibility links to the development of RRP. He
requires blood and tissue samples from
patients for this major NIHR funded study.
Ethical approval has been granted for the
first UK site and involvement is encouraged
to make this important study a success. For
information contact Adam Donne at the
email address provided above.
Conclusion
Viral infections are common and potentially important in childhood. They may
have long-term effects. Increasing numbers
of viruses are being found to result in
bronchiolitis and the balance of evidence
would suggest this is important in the
development of asthma. HPV is remarkably
common also yet predominantly transient.
The factors that make HPV result in disease
are not understood. There is no antiviral
agent which effectively targets HPV 6 / 11.
Vaccines and further research provide
hope for the future. ■
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The Rehabilitation of the Deaf
Child
Konstance Tzifa,
MPhil, FRCS(ORL-HNS),
DLO,
Consultant ENT Surgeon,
Hospital, Birmingham, UK.
R
ehabilitation usually refers to restoration of a skill that is lost. In paediatric deafness the ability to hear may have not been there in the first place, therefore the
term ‘habilitation’ is more appropriate because the skill needs to be taught. ‘Aural
habilitation’ for children with congenital or acquired deafness depends on many factors
relevant to the onset and type of deafness, and also on the mode of communication.
Kate Hanvey,
Specialist Speech and
Language Therapist,
The Midlands Hearing
Implant Programme –
Children’s Service,
Birmingham Children's
Hospital NHS Foundation
Trust, Birmingham, UK.
Correspondence
E: Konstance.tzifa@
bch.nhs.uk
E: Kate.hanvey@
bch.nhs.uk
Declaration of
Competing Interests
None declared.
The importance of a critical learning period for
language development is well accepted. This
learning must occur very early in life to avoid
greater social, educational and psychological
disadvantages. Delays in diagnosis of deafness
can be detrimental for the child’s language
development. Therefore, the focus for clinicians
is early diagnosis and intervention.
For most children with normal hearing,
listening and spoken language develops in line
with other developmental milestones. This is
not the case for most children who have a
severe to profound hearing loss. Where
listening skills are impaired, the development
of spoken language becomes a challenge.
Communication through visual language is
limited by the skills of the parents and primary
carers. Therefore the following are essential.
1. Early identification of hearing impairment
in infants and children
2. Determine aetiology and progression of
hearing loss
3. Provision of amplification, sound philosophy
4. Early educational options
Infants can be fitted with hearing aids as soon
as the hearing loss is confirmed, even prior to
age three months. Binaural amplification and
education facilitates the acquisition of speech
and language by the child with profound
hearing loss.1 The use of binaural hearing aids
for amplification in children is well accepted.2
Monitoring of the child wearing hearing
aids and hearing aid assessment is essential
because changes in hearing performance are
commonly seen in children using hearing
aids. There are many causes for changes in
hearing performance including malfunctioning hearing aids, additional acquired
cause of hearing loss and progression of the
existing hearing loss.
For children with profound hearing loss,
where hearing aids offer little or no benefit, a
cochlear implant is the device of choice.
Cochlear implant candidates should receive
them as early in life as possible. In the UK, most
centres aim for around 10 to 12 months of age.
Since the first two implantations by Graeme
Clarke3 and his group at the University of
Melbourne in Australia, experience of cochlear
implantation and rehabilitation for children has
increased and proved cochlear implantation to
be a remarkable achievement in habilitation of
deafness and speech and language development. How the brain learns to interpret signals
from the cochlear implant as meaningful is an
amazing process.
Hearing aids amplify sound to the cochlea.
Cochlear implants replace the cochlea function through electrical stimulation of the
hearing nerve. Hearing aids rely on existing
hair cells. Implants restore the sensation of
hearing, whereas hearing aids exploit existing
hearing.
Fitting of optimal amplification is just the
beginning of the parental journey in helping
their hearing impaired child develop competent and effective communication. The task
that is faced by parents cannot be underestimated. To illustrate this, in a recent presentation, Carol Flexer4 shared some startling
research conducted with normally hearing
children. By the age of four years, children in
‘professional’ families have heard 46 million
words spoken whereas children from ‘welfare’
families have heard 13 million words. This
puts children from welfare families at such a
disadvantage, the gap cannot be closed.5 For a
congenitally profoundly deaf child awaiting
cochlear implant fitting, a conservative
assumption is that they will have already lost
12 months of listening experience and once
fitted, their hearing is not comparable to their
normal-hearing peers. Yet with carefully
structured guidance and support, it is
possible for some hearing impaired individuals to close the gap.
One may assume that if optimal amplification is fitted ‘early enough’, then therapy and /
or early educational intervention may not be
required. However the following issues
remain:6
25
feature
1. Reduced ‘listening time’ during the day
during times when amplification cannot
be worn (for example sleeping, bathing
and so on), by comparison with 24/7
listening in normal hearing
2. Reduced opportunities for incidental
learning through overhearing (90% of
learning and knowledge in children is
incidental), due to background noise
and distance listening
3. Learning to listen begins after a period
of sensory deprivation and therefore the
early building blocks of communicative
interactions that come through hearing,
have not been established.
Of course, all children and their families are
different. There are several factors that
affect outcomes of cochlear implantation
and also play an important role in rehabilitation and education. A significant proportion of children who are born with a
profound hearing loss will have additional
needs.7 These sometimes impede learning
and are often related to the primary aetiology. Awareness of the cause of deafness
and any additional impairment is important
for counselling as this may impact on the
communication options chosen by parents
or recommended by professionals. There
may be individual family circumstances that
make it difficult to access or take on board
the support offered. In particular, in the
early stages following diagnosis, parents
may need time to grieve, to adjust and
consider the options on behalf of their
child. Therefore there is no unique intervention option that suits all, circumstances
change over time and children and their
families require a highly individualised
programme which has been tailored to
their needs.8
Ninety to 95% of children with hearing
impairment are born into families where
both parents have normal hearing.9,10 Many
parents from this group would like their
children to learn to listen and talk as their
primary means of communication, to
attend a mainstream school and to have
the same opportunities available to them as
their normal-hearing peers. Although not
without its challenges, this is now a realistic
desire for many, particularly for children
who do not have significant additional
needs above and beyond their hearing
impairment. Auditory-Verbal Therapy and /
or Auditory-Oral programmes can support
parents who have chosen this route. It
should be noted that these approaches are
not the same, and sources of further information can be found at the end of this
article. A high quality service should
26
provide support to parents and families to
guide, coach, engage and empower them
with confidence, skill and knowledge to
develop their child’s auditory brain for
listening and talking.
Total Communication is a philosophy
that includes a wide range of communication strategies using hearing, vision and
touch. It appears to be most commonly
interpreted to mean that individuals are
using some sign language in addition to
spoken language. Unfortunately, the result
can be an impoverished rather than
enhanced communicative exchange
whereby the child does not receive a good
language model in either modality.11
However a total communication approach
does have a place. Some parents find it
particularly useful to help establish basic
meaningful communication in the absence
of appropriate amplification (for example
whilst waiting for a cochlear implant in
toddlers) and some also like to use a total
communication approach as a way of
keeping options open. In addition, some
children require a degree of sign language to
augment their spoken communication and
for these individuals sign support may be
required throughout their educational
career. Choosing a total communication
route as a long-term option implies that all
family members and other key carers will
learn to sign to a competent level in order
to provide enriched models of language.
Sign Bilingualism aims to develop
competent and age appropriate language
using a formal sign language system (such as
British Sign Language). Functional understanding and use of written and spoken
language is also encouraged. Within the UK,
Sign Bilingual programmes are usually delivered in some Special Schools for the Deaf
and are therefore not usually available until
a child is of Nursery age (around three or
four years old). Parents who have little or no
experience of deafness and sign language
should be encouraged to think about the
long-term implications of choosing this
option for their child, as it is absolutely
essential that all family members and other
significant carers become fluent in the Sign
Language used in the school. Learning sign
language to a fluent level requires significant
financial investment and takes several years.
Without this, their children may become
isolated from the rest of the family and may
grow up being unable to communicate
effectively within the family unit.
Regardless of the amplification device
and communication modality, the aim of
an early intervention programme should be
to prevent significant language delay and
educational underachievement, and habilitate through a normal developmental
sequence, rather than to rehabilitate disordered communication that has developed
over time as a consequence of hearing loss.
Not all roads lead to the same destination.
Choices made when the child is 12 months
old may not fit with the parents’ long-term
hopes, dreams and wishes for their child.
Parents should therefore be encouraged to
obtain information from a range of sources
in order to come to an informed decision
about the choices they make on behalf of
their child. n
Further information
National Deaf Children’s Society, Communicating with
your deaf child
[http://www.ndcs.org.uk/family_support/
communication_m/communicating_with_your_
deaf_child/]
Carol Flexer homepage [http://www.carolflexer.com/]
Auditory Verbal Lounge
[http://auditoryverballounge.co.uk/]
Auditory Verbal [http://www.auditoryverbal.org.uk/]
Deaf Education Through Listening and Talking
[http://www.deafeducation.org.uk/]
References
1. McConnel F, Liff S. Symposium on sensorineural
hearing loss in children: early detection and intervention. The rationale for early identification and
intervention. Otolaryngol Clin North Am
1975;8(1):77-87.
2. Downs MP. Amplification in the habilitation of the
young deaf child. In Early management of hearing
loss. Edited by Mencher GT, Gerber SE. New York,
NY: Grune & Stratton; 1981;199-224.
3. Clark G, Tong Y, Black R, Foster I, Patrick J, Dewhurst
D. Multiple electrode cochlear implant. J Laryngol
Otol 1983;91:41-4.
4. Flexer C. Cochlear implantation and neuroplasticity:
linking auditory exposure and practice. Presented at
the British Cochlear Implant Group annual conference, Nottingham, UK; 2011.
5. Hart B, Risley TR. The social world of children: learning to talk. Baltimore: Brookes Publishing Company;
1999.
6. Tyszkiewicz E, Stokes J. Paediatric habilitation. In
Cochlear Implants A Practical Guide, Second edition. Cooper HR, Craddock LC. London: Whurr
Publishers; 2006.
7. Condon M-C. Unique challenges, children with
multiple handicaps. In Pediatric Amplification,
Omaha. Edited by Feigin JA, Stemachowicz PG. Boys
Town National Research Hospital; 1991.
8. Wyatt JR, Niparko JK. Evaluation of the benefit of
the multichannel cochlear implant in children in
relation to its cost. In: Cochlear Implant
Rehabilitation in Children and Adults. Edited by
Allum DJ. San Diego, CA: Singular: 1996;20-30.
9. Mitchell RE, Karchmer MA. Chasing the mythical
ten percent: Parental hearing status of deaf and hard
of hearing students in the United States. Sign
Language Studies 2004;4(2):138-63.
10. NDCS factsheet. Statistics on childhood deafness in
the UK; 2003.
11. Wilcox S. American deaf culture. Silver Spring, MD:
Linstok Press; 1989.
Serious Complications of
Acute Otitis Media: mastoiditis
and intracranial sepsis
Mr William PL
Hellier, MBChB,
FRCS(ORL-HNS),
Consultant ENT Surgeon.
Correspondence
Department of ENT,
Southampton University
Hospital and Royal
Hampshire County
Hospital, Hampshire,
SO16 6YD, UK.
Declaration of
Competing Interests
None declared.
A
cute Otitis media (AOM) is one of the most common diseases diagnosed in
the paediatric population. Rates of AOM vary between countries, but
approximately 30-60% of children will have an episode in their first year of
life, and up to 70-80% by the time they are three years old. The greatest risk of AOM
occurs in the second six months of life.1 In the USA it is the most common indication
for antibiotic therapy.
Mastoiditis
Most cases of AOM will involve infection of
both the middle ear cleft and mastoid air
cells with purulent exudate. However in
certain serious episodes, possibly due either
to mastoid obstruction because of mucosal
oedema leading to mucopus under pressure, or possible bacterial virulence, bacteria
may penetrate the middle ear or mastoid
mucosa. This leads to an infective osteitis or
acute mastoiditis, often with involvement of
the periosteum either by direct spread or via
venous channels. If the infiltrative and
destructive infective process continues,
progression to coalescent mastoiditis may
occur with breakdown of the normal
mastoid architecture, associated, or
followed by spread beyond the mastoid
either intra or extra cranially.
Serious complications of AOM are
unusual but are of great interest to the
otolaryngologist, as they will need management under the care of the ENT surgeon.
Serious complications are classically divided
into extracranial / intratemporal complications and Intracranial complications. These
are detailed in Table 1.
Mastoiditis is the most common of the
serious complications of AOM. The classic
presentation is that of a child with a history
of AOM who develops a reddened, tender
posterior auricular sulcus often with a
degree of effacement, and protrusion of the
auricle. This occurs due to the underlying
osteitis and periosteitis. If left untreated this
will progress and pus will collect under the
periosteum forming a subperiosteal abcess
or infection may spread further, potentially
intracranially.
There have been a number of studies that
have examined the incidence of mastoiditis
in different populations. In Norway the incidence from 1999-2005 of mastoiditis was
found to be 4.3-7.1 / 100,000 in the two to
16 year old age group, but 13.5-16.8 in the
under two year old population.2 In the UK a
Table 1. Complications of Acute Otitis Media
Intratemporal Complications
Acute Mastoiditis
Facial paralysis
Labyrinthitis
Sensorineural hearing loss
The significance
of mastoiditis is
that it is the
‘staging post’ to
intracranial
complications
Petrositis / Gradenigo’s Syndrome
Extratemporal Complications
Subperiosteal mastoid abscess
Bezold’s abscess
Intracranial Complication
Extradural abscess
Subdural abscess
Cerebral / cerebellar abscess
Sigmoid sinus thrombosis
Otitic hydrocephalus
Meningitis
27
feature
Figure1: Mastoiditis. CT scan showing
opacification of the right middle ear and
mastoid, post-auricular swelling with abscess
formation.
recent study has estimated the incidence as
0.15 / 1,000 child-years.3 Other studies have
quoted incidences of between 1.1 and 12 /
100,000. Most have found higher rates in the
under two years of age group.4 This is unsurprising as this group is when this incidence
of AOM is at its greatest. Certainly this
reflects the experience from my own unit
where the vast majority of AOM complications have been in this age group. The incidence of mastoiditis after AOM has also
been studied and, in the UK this was found
to be approximately 2.4 cases of mastoiditis
per 10,000 cases of AOM (0.024%), but this
was higher in children not treated with
antibiotics (0.038%).3
Historically Rudberg in 19545 reported
mastoiditis occurring in 17 % of cases of
AOM. Palva in 1959 described the incidence
of mastoiditis after AOM in Finland as 0.3%.6
Palva reported again in 19857 showing an
incidence of mastoiditis of 4 / 100,000,
which he described as a marked reduction,
and which he attributed to the increased
use of antibiotics. However there may have
been other factors such as a change in living
standards over this time, which may have
also influenced the rate of AOM. There has
been some debate over the recent years
whether there has been an increase in the
incidence of mastoiditis, as there has been a
growing trend for not prescribing antibiotics
for AOM. Van Buchem in 1985 published a
paper in the BMJ examining this subject,8
finding only a small effect of antibiotic use
on the rate of mastoidtitis. However children under two were excluded from the
study, and this is the most common age
group to suffer from complications of
AOM. Other recent studies have shown a
reduced incidence of mastoiditis in countries where antibiotic prescribing is higher
(rates in Holland where antibiotic use is low
are 3.8 / 100,000, and 1.2-2.0 / 100,000 in
28
countries with high rates of prescribing).9
There have been a number of papers that
have suggested no change in mastoiditis
rates (including a recent publication from
the UK comparing 1991-1998 and 19902006),3 but a few suggesting a rise in cases
over the last few years.10,11 The picture is
therefore a little mixed. This issue is further
confused however when one examines the
literature, as in most series approximately 4060% of children admitted with a complication of AOM had already been started on
antibiotics by their primary care physician for
the preceding AOM. Although 50% of children have had no prior treatment, it would
seem a high proportion of cases of complications have occurred despite oral antibiotic
therapy. This could be related to the virulence of the bacteria involved, the incorrect
antibiotic or poor patient compliance.
Bacteriology
Reviews of the bacteriology of mastoiditis
show that in a high proportion of cases,
40-50%, no growth will be found on microbiological swab analysis.4,12-14 This is possibly
due to the previous administration of
antibiotics. Of those cases with positive
microbiology, in most series Streptococcus
pneumoniae is the most frequent bacteria
isolated. Strep. pyogenes, Staphylococcus
aureus and Haemophilus influenzae are the
next most common, with Pseudomonas
aeruginosa and a mixture of anaerobes and
other bacteria found infrequently.
Antibiotic therapy for mastoiditis or a
complication of AOM must therefore be
targeted at these organisms.
Intracranial complications
The most concerning sequelae of AOM are
undoubtedly those of intracranial septic
complications, where there may be high
morbidity and potentially mortality. The
significance of mastoiditis is that this is the
‘staging post’ to intracranial complications.
In all series intracranial sepsis occurs in nearly
all cases as a combination with or after
mastoiditis (the only exception is meningitis
which may occur without signs of
mastoiditis). This is the reason mastoiditis
must be treated seriously and with urgency.
Intracranial complications occur due to
direct spread of infection from the mastoid
ostetitis, but also via a septic thrombophlebitis of the local venous system or
mastoid emissary veins. Inflammation of the
neighbouring middle or posterior fossa dura
may occur with localised abscess formation.
Thrombophlebitis from the inflamed dura
may lead to intracerebral or intracerebellar
abscess collections. The walls of the sigmoid
sinus are formed by dural folds, infection or
inflammation of which leads to localised
mural thrombus formation that may
progressively reduce or totally obstruct
venous flow. Propagation of this sigmoid
sinus thrombus (SST) may extend into the
internal jugular vein, the transverse sinus, or
other venous sinuses. This thrombus may
become infected leading to frank pus, or
cause the obstruction of the cerebral venous
drainage system leading to hydrocephalus
(otitic hydrocephalus). In the preantibiotic
era the mortality of sigmoid sinus thrombosis
and intracranial abscess was almost 100%,
however in modern times this mortality has
fallen sharply but death still occurs.
The incidence of intracranial complications after mastoiditis varies between
modern series but is in the order of 4-20%.12,13
There seems to be reasonably even distribution between extra and intradural abscesses
and infective SST. However there may well
be a combination of these complications.
Management
The management of a child with
mastoiditis has changed over the years. In
the preantibiotic era surgery was the mainstay of treatment with mastoidectomy
with myringotomy. In the antibiotic era
there has been a marked change in
management. A number of recent series
have shown that the percentage of children with mastoiditis who need to
undergo mastoidectomy has fallen to 1634.5%.15 The majority of children with
mastoiditis, especially if early in its course,
will resolve with intravenous antibiotics +/myringotomy. Some studies16,17 have shown
that myringotomy does not always need to
be performed but should be considered if
the tympanic membrane is still intact to
allow middle ear drainage, and also to
collect microbiological samples. If a subperiosteal mastoid abscess is present at
admission or develops subsequently, this
will need drainage.15 Usually this will be
performed by formal surgical incision, but
some series have described management
with simple needle aspiration in addition
to myringotomy and antibiotics.17,18
Careful observation of a child with
mastoiditis is needed because if the condition does not improve or deteriorates,
further intervention with cortical mastoidectomy may be needed. This will probably be
required if the child has developed an
intracranial or severe extracranial complication. However there are a number of children
where the decision to perform a mastoidectomy is more finely balanced. If there is a
subperiosteal abscess, the child is systemically
feature
Figure 2: Sigmoid Sinus Thrombosis. Axial CT
scan with contrast showing enhancement of the
right sigmoid sinus walls due to inflammation
with no luminal flow due to thrombus. This is
often called the Delta Sign.
Figure 3: Cerebellar Abscess. Axial MRI scan
showing opacification of the left middle ear and
mastoid with a large cerebellar abscess.
Figure 4: Sigmoid Sinus thrombosis. MRA
showing no flow in the left sigmoid sinus
(arrow).
unwell or there is failure of resolution over 2448 hours, many otologists would argue that
cortical mastoidectomy, with drainage of the
abscess, should be carried out. However
some series have managed such patients
conservatively, and in the modern day era of
subspecialisation not all otolaryngologists
may be entirely comfortable exploring the
mastoid of a 14 month old child. There have
been a number of studies that have looked at
features of mastoiditis that may predict for
the need for mastoidectomy (high white cell
count and CRP seem to be predictive), but
none have shown any absolute indications,
and there still remains a degree of clinical
judgement. There is also no information that
suggests whether a complete cortical
mastoidectomy or a purely drainage procedure is most appropriate.
Some children with AOM and mastoiditis
will have established intracranial sepsis when
they present to secondary care, others will
develop this during the course of their
disease. The classic symptoms of intracranial
complications are an unwell child, with high
fever (with spiking pyrexias, the so called
‘picket fence’ chart), often drowsy or
lethargic and possibly with neurological
signs. Children presenting in this way, or
whose mastoiditis is not resolving need radiological investigation to assess the temporal
bone and intracranial compartment. In the
era of broad spectrum antibiotics, it must be
remembered that not all children with an
intracranial complication will present with
classic signs however, and a high index of
suspicion, and a low threshold for radiological investigations is important.
CT scanning with contrast will give good
bony information, and can show intracranial
sepsis. MRI scanning gives far more brain
and dural detail and will show peri-dural
infection, cerebritis or cerebral abscess.
Adding MRA gives more information about
the sigmoid sinus,19,20 especially showing
thrombus formation or cerebral sinus occlusion (Figures 2, 3, 4).
Treatment of the intracranial complication
will depend upon its nature but may need
involvement of the neurosurgeons or paediatric neurologists. Extradural abscesses may
be drained by complete cortical mastoidectomy with drilling down of the posterior or
middle fossa plate to expose the extradural
pus and exteriorise this into the cortical
cavity. Subdural abscesses may be drained in
a similar way but with needle aspiration
through the exposed dura in combination
with the neurosurgeons. Intracerebral or cerebellar abscesses are often drained by the
neurosurgery team via a separate burr hole. A
common finding in these cases is generalised
dural inflammation with granulation tissue.
All intracranial complication will need a
prolonged course of intravenous antibiotics.
Sigmoid sinus thrombosis (SST) presents
a condition which is not usually managed
by the neurosurgeons, as it is more often
seen in children with clotting derangements and presents usually to the paediatric neurologists where it is managed
medically, typically with anticoagulation.
The treatment of SST secondary to AOM is
different however and has slowly changed
over the years. In the pre- and early antibiotic era thrombus extension and septic
emboli were not uncommon, and internal
jugular vein ligation (IJVL) with sigmoid
venotomy, thrombus removal and packing
were performed regularly. More modern
series have shown that IJVL is now rarely
needed, and surgical treatment has
become more conservative, although there
is still discussion over the optimum
management.19-24 Cortical mastoidectomy
can be indicated and it is important to fully
expose the sigmoid sinus walls and some of
the posterior fossa dura. This allows
drainage of any perisinus collections or
granulation tissue.21 Many papers have
suggested that placing a needle into the
sinus gives information about whether
there is any blood flow, but this may be
unnecessary as MRA now gives excellent
preoperative information regarding blood
flow and sinus patency. A number of series
have indicated that in the presence of
thrombus, the sinus should be opened and
a thrombectomy performed.22-24
Recent studies however have shown that
this may not be needed, and if the thrombus
is noninfected, the sinus can be left intact
and recannalisation may occur in a number
of cases.19-21 If there is infection of the intrasinus thrombus then the sinus must be
opened and the pus drained. My own experience from a number of cases of SST is that
with thrombus alone the dural walls of the
sigmoid sinus were thickened with granulations and could be left in situ, but in three of
the cases where the thrombus had become
infected the dural walls had already thinned
and the area of the sinus was filled with
purulent material and gas which was easily
opened and drained.
If a SST has occurred there is debate in
the literature whether anti-coagulation is
needed.20,21,24 A number of earlier case series
routinely anticoagulated such children.
However there have been reports of haemorrhagic complications,25 and recently
there have been a number of series where
anticoagulation has not been instituted,
without any progression of the thrombus
or septic emboli. 20-22 The need for
anticoagulation needs consideration and
29
feature
discussion with the paediatric neurology
team, but the indication is certainly not
absolute. n
References
1. Teele DW, Klein JO, Rosner B. Epidemiology of otitis
media during the first seven years of life in children
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Infect Disease 1989;160(1):83-94.
2. Kvaerner KJ, Bentdal Y, Karevold G. Acute mastoiditis
in Norway: No evidence for an increase. Int J Paed
Otorhinolaryngol 2007;71(10):1579-83.
3. Thompson PL, Gilbert RE, Long PF, Saxena S,
Sharland M, Wong ICK. Effect of antibiotics for
acute otitis media on mastoiditis in children: A retrospective cohort study using the United Kingdom
genral practice research database. Pediatrics
2009;123(2):424-30.
9. Van Zuijlen DA, Schilder AGM, Van Balen FAM,
Hoes AW. National differences in incidence of acute
mastoiditis: relationship to prescribing patterns of
antibiotics for acute otitis media? Pediatr Infect Dis J
2001;20(2):140-4.
10. Finnbogadottir AJ, Petersen H, Laxdal P,
Gudbrandsson F, Gudnason P, Haraldsson A. An
increasing incidence of mastoiditis in children in
Iceland. Scand J Infect Dis 2009;41(2):95-8.
11. Benito MB, Gorricho BP. Acute mastoiditis: Increase
in the incidence and complications. Int J Paed
12. Dhooge IJM, Albers FWJ, Van Cauwenberge PB.
Intratemporal and intracranial complications of acute
suppurative otitis media in children: renewed interest.
Int J Paed Otorhinolaryngol 1999;49 Suppl 1:S109-14.
17. Taylor MF, Berkowitz RG. Indications for mastoidectomy in acute mastoiditis in children. Ann Otol,
Rhinol, Laryngol 2004;113(1):69-72.
18. Lahav J, Handzel O, Yehuda MP. Postauricular needle
aspiration of subperiosteal abscess in acute mastoiditis. Ann Otol Rhinol Laryngol 2005;114(4):323-7.
19. Bales BB, Sobol S, Wetmore R, Elden LM. Lateral
sinus thrombosis as a complication of otitis media:
10-year experience at the children’s hospital of
Philadelphia. Paediatrics 2009;123(2):709-13.
20. Chistensen N, Wayman J, Spencer J. Lateral sinus
thrombosis: A review of seven cases and proposal of
a management algorithm. Int J Paed Otorhinolaryngol
2009;73(4):581-4.
21. Wong I, Kozak FK, Poskitt K, Ludemann JP, Harriman
M. Pediatric lateral sinus thrombosis: Retrospective
case series and literature review. J Otolaryngol
2005;34(2):79-85.
4. Leskinen K, Jero J. Complications of acute otits
media in children in southern Finland. Int J Paediatr
13. Luntz M, Brodsky A, Nusem S, Kronenberg J Luntz
M, Brodsky A, Nusem S, Kronenberg J, Keren G,
Migirov L, Cohen D, Zohar S, Shapira A, Ophir D,
Fishman G, Rosen G, Kisilevsky V, Magamse I,
Zaaroura S, Joachims HZ, Goldenberg D. Acute mastoiditis – the antibiotic era: a multicenter study. Int J
Paed Otorhinolaryngol 2001;57(1):1-9.
5. Rudberg RD. Acute otitis media: comparative therapeutic results of sulphonamide and penicillin administered in various forms. Acta Otolaryngol Suppl
1954;113:1-79.
14. Quesnel S, Mguyen M, Pierrot S, Contencin P,
Manach Y, Couloinger V. Acute mastoiditis in children: a retrospective study of 188 patients. Int J Paed
23. Manolidis S, Kutz JW Jr. Diagnosis and management
of lateral sinus thrombosis. Otol Neurotol
2005;26(5):1045-51.
6. Palva T, Pulkkinen K. Mastoiditis. J Laryngol Otol
1959;73:573-88.
15. Tamir S, Shwartz Y, Peleg U, Shaul C, Perez R, Sichel
JY. Shifting trends: mastoiditis from a surgical to a
medical disease. Am J Otolaryngol 2010;31(6):467-71.
24. Bradley DT, Hashisaki GT, Mason JC. Otogenic sigmoid sinus thrombosis: What is the role of anticoagulation? Laryngoscope 2002;112(10):1726-9.
16. Geva A, Oestreicher-Kedem Y, Fishman G,
Landsberg R, DeRowe A. Conservative management
of acute mastoiditis in children. Int J Paed
25. Shah UK, Jubelirer TF, Fish JD, Elden LM. A caution
regarding the use of low-molecular weight heparin
in pediatric otogenic lateral sinus thrombosis. Int J
Paed Otorhinolaryngol 2007;71(2):347-51.
7. Palva T, Virtanen J, Makinen J. Acute and latent mastoiditis in children. J Laryngol Otol 1985;99(2):127-36.
8. Van Buchem FL, Peeters MF, Van ‘t Hof MA. Acute
otitis media: a new treatment strategy. BMJ
1985;290(6474):1033-7.
22. Lee HL, Choi JC, Park K, Choung Y. Managements
for lateral sinus thrombosis: does it need ligation of
internal jugular vein or anticoagulants? Eur Arch
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30
Reassembling the Auditory
World in Children with
Cochlear Implants
Blake C Papsin, MD.
I
t has been over twenty years since cochlear implants (CI) were first approved for
use in children with sensorineural hearing loss (SNHL) and at the Hospital for Sick
Children in Toronto, we recently implanted our 1,000th CI. While our under-
standing of CI in children has evolved dramatically, we still cannot fully predict or
explain their benefits and limitations for any one child. Clearly there are many research
questions left to answer. Improved surgical techniques have minimised complications,
Sharon L Cushing, MD.
allowing us to reduce the age of implantation and CI is now routinely performed bilaterally on infants with safe, reliable and incredibly sophisticated devices.1 Likewise criteria
for implantation have evolved and we now provide CI to individuals not originally
considered to be candidates. Implanted children are developing linguistic skills on par
with their peers, albeit with the aid of significant intervention from auditory verbal
therapists. The outcome is magnificent when we measure speech perception in quiet
or even with controlled background noise. By contrast, important questions regarding
auditory plasticity and how children with early onset SNHL function in the real world
Karen A Gordon, PhD.
Correspondence
Blake C Papsin, MD,
Department of
Otolaryngology – Head
and Neck Surgery,
Hospital for Sick
Children,
555 University Avenue,
Elm Wing 6103-C,
Toronto, ON, Canada,
M5G 1X8.
E: blake.papsin@
utoronto.ca
Declaration of
Competing Interests
None declared.
remain. Whilst we were once thrilled with any improvements in hearing following CI,
our present objectives are no less than to restore ‘normal’ auditory function. This brief
communication will not act as a review of the current literature, but rather takes advantage of this rare opportunity to speculate on what we think is fundamental to CI
outcomes in children based on questions we are presently asking in our laboratories.
The auditory system, stimulated by either
normal hearing or auditory prostheses,
does not act in isolation. There is ample
evidence that the auditory pathways can
be modulated by other sensory and
arousal mechanisms.2 As humans, we
need to take in multiple, diverse inputs,
focus on those which are important at
that time, and decide how to integrate
and respond to this information. The
perception of the signal is as important as
the context in which it resides. For
example, a siren in most cases elicits a
basic instinct to run for the exits.
However, when a siren is heard in Bob
Dylan’s Rainy Day Women No. 12 & 35
(which opens his 1966 album Blonde on
Blonde), our reaction is completely
different. Clearly the context in which
sound is presented is key to understanding this input and deciding how to
react to it.
In the lab, we start to see the limitation of
CI when we ask recipients to reassemble
auditory information by judging the
emotion in a spoken sentence. We found
that these children are less accurate and
Figure 1: Plain radiograph demonstrating bilateral
cochlear implants with the electrode array within
the cochlea (white arrowhead).
respond more slowly than their normal
hearing peers when doing this task.3
Increased reaction times strongly suggest
that additional processing time is required to
reconstruct the auditory environment and
react to it.
Along the same line, we have been
studying musical perception in children
with CI and have found that their ability
lags far behind their normal hearing
31
feature
The human brain rapidly assembles and processes multi-sensory
information for both survival and enjoyment. Assembly of the auditory
environment in children with significant hearing loss is best achieved by
early cochlear implantation, ideally performed bilaterally, to encourage
auditory development which is as close to normal as possible
Figure 2: Histologic section of the cochlea with cochlear implant electrode array in situ in the basal turn (white arrow). Photo courtesy of Cochlear Limited.
peers. These deficits reflect the poor
temporal information provided by the
CI.4 Despite these limitations, however,
children with CI are still able to distinguish between happy and sad music and
that they love to listen to music.3 Again,
we see that they compensate for limited
input and enjoy using what they have.
Measuring progress
It is important to note that our ‘standard’
battery of speech perception tests
provides a narrow view of auditory
perception. Such measures do not elucidate how children use their CI in daily
life as they develop socially, scholastically, preparing for employment and
32
adulthood, nor the complex neural
network involved in hearing. Although
the fundaments of auditory perception
can be examined in the laboratory by
asking listeners to identify the properties
of simple sounds, more complex and ‘real
world’ auditory processing is often left
unexamined. The use of complex stimuli
and listening tasks can help define the
limits of hearing in children using CI and
allow us to study how these children
might compensate to meet these
listening challenges. It follows then that
the real test of CI effectiveness should be
how well the sensory input it provides
steers the contextual reassembly of the
auditory environment.
It is reasonable to assume that hearing
is compromised when auditory input is
limited or degraded. Yet, the auditory
pathways appear remarkably adept at
using whatever information it receives. To
our initial surprise, even children with
significant anatomic malformations of
the cochleae perceive reasonably well in
test environments.5 This speaks to the
complexity and redundancy that must
exist in the auditory system which, when
fully functional, is able to perceive subtle
cues in speech such as innuendo and
sarcasm. Efforts to 1) increase the
amount of information made available to
the auditory system, 2) improve the
ability to extract information from the
feature
environment and / or 3) promotes
normal development of the auditory
system, would enhance the ability to
reconstruct the auditory environment for
CI users. These three principles should
provide the motivation for improved
implant technology and novel methods
to test CI outcomes in the future.
Reorganisation of the hearing
network
Is it naïve to think that one or even two CIs
could provide sufficient information for
children to reassemble the auditory environment and make important decisions
about how to interact or react? To answer
this, we need to understand how the
underlying mechanisms of auditory
perception develop. It is clear that the
normal interplay between different brain
areas is altered in congenital deafness,
resulting in abnormal reorganisation.6
Although we would like the early restoration of audition through CI to eliminate
any changes away from normal, we realise
that reorganisation likely begins with the
onset of deafness and is already underway
even when we provide CIs in early infancy.7
Given that the CI cannot fully mimic the
normal hearing ear, we do not know
whether CI use can halt or reverse the
effects of deafness in early development.
It is important to remember that the CI
may not be treating the same deafness or
the same effects of deafness in all children. We have recently shown a high
degree of heterogeniety in cortical
responses in children at initial CI use.
Interestingly, responses look more
uniform in children with biallelic GJB-2
mutations. 8 This suggests that the
etiology of the hearing loss may define
the state of the auditory pathways at the
time of CI and perhaps even predict how
the auditory system will respond to CI
stimulation. This issue could become
more important as more discrete and
focused stimulation of the auditory nerve
is provided through advances in technologies.
Binaural input
In addition to the possible limitations of
CI stimulation on auditory development,
we must acknowledge the implications of
unilateral auditory deprivation through
the provision of a single sided CI. We are
finding that the auditory cortex becomes
reorganised after long periods of unilateral CI use (no contralateral hearing aid).
This reorganisation persists even when a
second CI is provided and the bilateral
implants are used for three to four years.9
Our recent data shows that the auditory
brainstem is able to detect interaural level
and timing cues after sequential bilateral
implantation (long inter-implant delay),
but that binaural input is not normally
processed in the cortex. Perhaps this is
why these children are unable to perceive
changes in interaural timing cues in a
behavioural lateralisation task.10 Even with
these abnormalities, we see that bilateral
implantation has been beneficial,
providing an improvement in spatial
hearing for sound localisation and
detecting speech in noise.11 Although
these skills are far from normal in this
group, the benefits attest to the remarkable ability of the child to integrate and
use whatever information is available to
make sense of the world around them.
Auditory support of non-auditory
tasks
The auditory environment is often used
to support non-auditory tasks. Normal
binaural auditory processing allows us to
construct a spatial organisation of sounds
in our environment and directs our visual
attention to the sound source. These
instinctive reactions allow us to navigate
safely through traffic or locate a friend in
a crowd and it is these fundamental
hearing skills which we should strive to
restore for children with SNHL and CI. Up
until recently, most individuals received a
single CI which precludes binaural
processing, challenging sound localisation. The advent of bilateral, and particularly simultaneous bilateral CI, allows us
to re-approximate, at a minimum, the
physiology of binaural sound input with
the aim of restoring binaural processing
and localisation and our ongoing studies
suggest that we are making progress.
Another example of how auditory
information supports non-auditory tasks,
is the statistically significant improvement
in balance function (on the BruininksOseretsky Test of Motor Proficiency 2
balance subset) observed when the CI is
used during the balance task.12 These data
provide a strong case for improved
balance function using the reconstructed
auditory environment as an additional
source of sensory input.
Conclusion and future directions
Of late, our thinking is less about the
implant and more about plasticity, and
current efforts are focused on improving
the child’s ability to correctly reassemble
all the inputs that contribute to their
auditory environment. Can implant recipients detect emotion in music and
speech? Can they understand sarcasm?
Can they perceive innuendo? These critical aspects of the auditory environment
allow the listener more than just survival;
they comprise the ‘fine points’ of listening
and allow maximal enjoyment of the
auditory world. Can the implant recipient
reassemble the auditory environment
accurately and quickly enough to allow
the full use of short-term memory and
how does that affect the ability to learn?
Should different strategies be employed
in educating children with CI? The technology, though magnificent, has been
reasonably static and the real gains in our
opinion have been in understanding how
the human uses this limited information
so completely in an attempt to accurately
reassemble the auditory world that
surrounds them. n
References
1. James AL, Papsin BC. Cochlear implant surgery at
12 months of age or younger. Laryngoscope
2004;114(12):2191-5.
2. Shimojo S, Shams L. Sensory modalities are not
separate modalities: plasticity and interactions. Curr
Opin Neurobiol 2001;11(4):505-9.
3. Hopyan T, Gordon KA, Papsin BC. Identifying emotions in music through electrical hearing in deaf
children using cochlear implants. Cochlear Implant
Int 2011;12(1):21-6.
4. Rubinstein JT. How cochlear implants encode
speech. Curr Opin Otolaryngol Head Neck Surg
2004;12(5):444-8.
5. Papsin BC. Cochlear implantation in children with
anomalous cochleovestibular anatomy.
Laryngoscope 2005;115(1 Pt 2 Suppl 106):1-26.
6. Rapin I. Consequences of congenital hearing loss –
a longterm view. J Otolaryngol 1978;7(6):473-83.
7. Gordon KA, Valero J, Jewell SF, Ahn J, Papsin BC.
Auditory development in the absence of hearing in
infancy. Neuroreport 2010;21(3):163-7.
8. Gordon KA, Tanaka S, Wong DD, Stockley T,
Ramsden JD, Brown T, Jewell S, Papsin BC. Multiple
effects of childhood deafness on cortical activity in
children receiving bilateral cochlear implants simultaneously. Clin Neurophysiol 2011;122(4):823-33.
9. Wong D, Gordon KA. Hemispheric Lateralization of
Evoked Potentials in Bilaterally Implanted Cochlear
Implant Users. In: 32nd Annual Midwinter Meeting
of the Association for Research in Otolaryngology.
Baltimore, MA; 2009.
10. Salloum CA, Valero J, Wong DD, Papsin BC, van
Hoesel R, Gordon KA. Lateralization of interimplant timing and level differences in children who
use bilateral cochlear implants. Ear Hear
2010;31(4):441-56.
11. Litovsky RY, Johnstone PM, Godar S, Agrawal S,
Parkinson A, Peters R, Lake J. Bilateral cochlear
implants in children: localization acuity measured
with minimum audible angle. Ear Hear
2006;27(1):43-59.
12. Cushing SL, Chia R, James AL, Papsin BC, Gordon
KA. A test of static and dynamic balance function
in children with cochlear implants: the vestibular
olympics. Arch Otolaryngol Head Neck Surg
2008;134(1):34-8.
33
The Future of Paediatric ENT
Surgery
Gavin Morrison,
MA, FRCS,
Otolaryngologist.
Correspondence
Evelina Children’s
Hospital, Guy’s &
St Thomas’s NHS
Foundation Trust,
London, SE1 7EH, UK.
E: gajm@
gavinmorrison.com
Declaration of
Competing Interests
None declared.
increasing
numbers of young
children requiring
surgery are being
transferred to
regional tertiary
centres for both
emergency and
elective surgery,
without this
transfer being
planned,
managed or
resourced
34
I
wish to discuss the future provision of ENT services within the UK. To determine where we are going, it should be helpful to look at where we have come
from and at recent trends. Many factors will influence how paediatric ENT
services are delivered across the UK. These include changes in medical education
and surgical training, a reduction in experience resultant from years of compliance
to the European Working Time Directive (EWTD), healthcare politics, driven by
central government, and perhaps most importantly, what is happening to the allied
specialties with whom we work so closely. Specifically, we will be driven by the
changes we have seen in anaesthetic practice, over the past decade or more.
Look back in anger
The way we live now
Since 1948, the NHS has tried to address
healthcare inequalities. The model of our
NHS ‘free at the point of delivery’ but
centrally funded out of taxation and
thus politically directed makes it vulnerable to the constant shifting of party
politics.
During my training and consultant
years, I have witnessed endless initiatives,
frameworks, plans and guidelines for the
provision of health delivery and medical
education. The 1983 ‘Griffiths Report’,
the 1991 The Patients’ Charter and
‘Designed to Care’ – the White Paper of
1997 all had their merits. Through The
NHS Plan (2000) we witnessed competition and the internal marketplace, fundholder GPs came and went, yet the
Wanless Report of 2002 further
increased the power and influence of
primary care. More personally we have all
been influenced by Action on ENT,
MMC, The New Consultant Contract,
PMETB, PBR, Patient Choice / Choose
and Book, NICE and more. The current
political will is going to see GP consortia
handling ‘real’ budgets to buy care (from
‘any willing provider’) on behalf of their
local communities; the abolition of all
primary care trusts and of strategic
health authorities, and the creation of a
new NHS Commissioning Board. A
similar American Model showed mixed
success over a 20 year period and only a
small proportion of these groups have
survived.1 We are about to boldly go
where no man has gone before with the
‘starship’ of the Health and Social Care
Bill to guide us.2
Are we in a position to influence the
future of Children’s ENT healthcare? We
should be, as only the paediatric ENT
surgical body itself has the understanding and experience to see what is
really needed, and what is practicable.
Above all, the need should be patient
focused.
The 1989 NCEPOD Report3 highlighted that surgeons and anaesthetists
should not undertake occasional paediatric practice. The concept of a
‘minimum case load’ was introduced, as
was the increasing centralisation of
paediatric services. In 1989, over 80% of
consultant anaesthetists had anaesthetised one or more infants of less than
six months of age, within that year. Nine
years later, that number had fallen to
40%. Evidence from Hospital Episode
Statistical data (HES) confirms the
perception that children’s surgery has
shifted from District General Hospitals
to specialist centres. In 2005, The Royal
College of Surgeons Children’s Surgical
Forum reported on trends in children’s
surgery, in England.4 Marker operations
for the study included grommets, tonsillectomy and adenoidectomy. Over the
10 year period from 1994 to 2004, the
percentage of procedures undertaken in
DGHs dropped from almost 80% to just
over 60%, with the specialist centres
showing the inverse of these percentages. The evidence is strong that there
has been a significant shift in children’s
ENT surgery from DGHs into specialist
centres, and a reduction in overall
number of procedures. Trends in general
feature
paediatric surgery will also influence paediatric ENT. In 2006, a joint statement on
general paediatric surgery provision in
DGHs was made on behalf of the relevant
colleges.5 It confirmed that increasing
numbers of young children requiring
surgery are being transferred to regional
tertiary centres for both emergency and
elective surgery, without this transfer being
planned, managed or resourced. A future
was foreseen in which almost all children
under five years and most under eight,
presenting with paediatric surgical emergencies, would be transferred from a DGH
to a specialist centre for treatment. The
development of paediatric surgical regional
networks between groups of hospitals was
envisaged. A three centre model for paediatric surgery, comprising small DGHs, intermediate centres which were large DGHs
and some university hospitals, and thirdly,
specialist or tertiary centres was proposed.
This same model could be applied with
benefit to paediatric ENT services.
A recent review of children’s surgery6
highlighted minimum ages for emergency
or urgent surgery to be under five or even
under eight years of age. If these ages
applied to paediatric ENT surgery many
children would be disadvantaged as they
could not receive ENT procedures locally
and the specialist centres do not have sufficient resources to treat more three to eight
year olds.
Let us now consider how anaesthetic
services are being driven in the UK. The
Royal College of Anaesthetists (RCoA) in
their publication ‘Raising the Standard: a
Compendium of Audit Recipes’7
confirmed that avoidable deaths were
identified in the under-10 age group. They
proposed that a target for best practice
should be that a consultant anaesthetist or
equivalent be expected to hold core
competencies in the management of
simple elective procedures in fit children
who are ASA 1 or 2 down to at least five
years of age. The more up to date RCoA
publication of April 2010, ‘Guidance on the
Provision of Paediatric Anaesthesia
Services’,8 broadly suggests that children
with significant acute or chronic medical
problems, those undergoing more
complex procedures, neonates and small
infants should be referred to specialist units
or tertiary paediatric centres. Nevertheless,
DGHs should have arrangements for
treating simple surgical emergencies and
should be able to resuscitate and stabilise
seriously ill children of all ages, prior to
their transfer. All consultant anaesthetists
with a CCT or equivalent should be
competent to provide care for common
Table 1: ASA Physical Health
Classification
•
ASA 1 is a normal healthy
patient in all respects
•
ASA 2 has no functional
limitations, but may have a
well controlled disease in one
body system, or mild obesity.
These are the patients who can
be treated in the DGH setting
with an age limit to be agreed
•
ASA 3 patients have some
functional limitation and
controlled disease in more than
one body system or in one
major system
•
ASA 4 has poorly controlled
severe disease with possible
risk of death
•
ASA 5 is not expected to
survive 24 hours without
surgery and has imminent risk
of death or multi-organ failure
•
ASA 6 is declared brain dead
for organ donorship
surgical conditions, both elective and
emergency, for children aged three years
and older. Day care surgery was considered
appropriate for children for non-complex
surgery in the healthy child with no comorbidities.
In 2008, when President of BAPO, Peter
Robb discussed and reported on the provision of ENT services highlighting that ENT
training differs from general surgical
training.9 Paediatric subspecialist work is
embedded into both the syllabus and the
final intercollegiate examination in ENT.
Thus our consultants across the UK have
experience and the expertise to manage
younger children.
In December 2010 The RCS of England
published a survey10 in which of 305 DGHs,
less than half were actually able to provide
an emergency paediatric surgical service.
Many of the Trusts faced problems
sustaining anaesthetic services for children
and 33% of hospitals were unable to anaesthetise children under the age of three.
The London Specialised Children’s
Services Review, commissioned by the
NHS11 is suggesting organisation and coordination of currently fragmented services
across London, where 28 hospitals provide
children’s services, yet the number treated
per annum ranged from 14 to 2,795! The
key recommendations for change are to
focus a wide range of specialised children’s
services in fewer hospitals, and to establish
networks that co-ordinate specialised
services in a defined area. The 8,000,000
population would support only two
networks. Similarly, the ongoing ‘Safe and
Sustainable NHS Review’ is dictating on the
future of children’s congenital heart
services and neurosurgery, in England.12 The
result will be fewer tertiary specialised
services, each with the potential for the
best of outcomes.
Back to the future
I believe that the same principles currently
being applied in other paediatric surgical
specialties will, and should be, taken up by
paediatric otolaryngology. The model of
regional networks is essential but will in
fact be driven by changes in anaesthetic
practice.
We are a strong sub-specialty group with
the highest levels of training. Both our
current close attention to agreed guidelines
for Tonsillitis and OME, and our representation of the specialty at the international
paediatric ENT meetings demonstrates that
we have much to be proud of. Nevertheless,
co-ordinated and resourced planning
should enhance our delivery of safe care.
I envisage a model in which paediatric
ENT care continues to be delivered across
the range of NHS settings. Primary care and
community based ENT delivery could have
an important place for certain outpatient
services. Secondary care provision in hospitals should continue to provide the
majority of routine paediatric ENT surgery
in the over three year olds if possible.
However, the routine paediatric ENT
procedures will probably only be undertaken in secondary care DGHs where the
children are ASA 1 or 2 and over the agreed
age. If the anaesthetists’ current training
guidelines are adopted, then children from
three years and over should be accepted. If
the trends in general paediatric surgery
wield undue influence, then the cut-off age
could become five (or even eight). This
would disadvantage many; children would
have to travel great distances for their ENT
surgery and a much greater increase in the
resources at the bigger centres would be
required. The model of smaller (intermediate secondary care) and larger DGHs or
university hospitals, networking together, is
the right one. Those larger centres perhaps
with maternity services and higher dependency critical care, will certainly be able to
routinely undertake the slightly more
complex and younger child. The tertiary
centres will inevitably continue to receive
more and more of the specialist problems,
35
feature
the paediatric ENT emergencies and small
children. Thus any young child with a
potentially life threatening airway problem,
and indeed many of the more complicated
septic conditions such as neck abscesses,
mastoiditis and complicated sinusitis, will
not meet the criteria of being ASA 1 or 2,
and all those under three will need to be
retrieved or transferred to the tertiary
centres. The ASA (American Society of
Anaesthesiologists) classification system,13
together with a younger age cut off point,
does provide a simple and practical means
of determining healthcare policy. All children considered ASA 3 or above are likely
to require transfer to tertiary or specialist
centres.
I envisage that we need in the region of
a dozen tertiary centres across the UK.
Currently, we have at least nine very strong
providers of tertiary paediatric ENT care.
The few major centres that are weaker
should be actively developed with new
recruitment and funding. These tertiary
centres should all undertake the full range
of paediatric ENT surgery and are also
largely well positioned to encompass
paediatric cochlear implantation. A smaller
subset of these regional tertiary units,
which might be termed quaternary, should
provide for paediatric oncology, neurosurgery and the most demanding of the
airway problems such as complex low
tracheal work and other rarer congenital
disease.
I believe this model would be successful,
but there are numerous problems,
including training issues, ENT on-call rotas
and who should undertake the paediatric
ENT surgery in the DGH setting. To
achieve a critical load, the ENT surgeon
should perform an operating list at least
once a fortnight and ideally, weekly. The
other non-paediatric ENT operators
should not undertake occasional surgery
on children, but the whole department
will need to be updated in emergency
stabilisation and resuscitation of paediatric
life-threatening complications, for on-call
to be manageable. CME and revalidation
could valuably include mandatory annual
update training in these areas, run by the
regional tertiary centres. Within the
expanded tertiary centres, the emergency
workload will be much greater than our
current representation of 30%. To accommodate this really requires a significant
expansion in consultants and facilities and
would allow the emergence of stand alone
paediatric ENT on call rotas. Training of all
ENT surgeons in the UK already robustly
includes paediatric otolaryngology. It
36
could be enhanced if all trainees are
obliged to spend six months in a tertiary
unit and if post-CCT fellowships were to
be undertaken by all who intend to undertake DGH paediatric ENT surgery as the
local lead clinician.
There is a need for organised planning of
these resources. If the principles of the
pathways for care are agreed, then appropriate central funding of highly specialised
services, together with the primary care
commissioners, should allow a successful
and financially viable integrated networked
service. The difference between ‘high
volume, low tariff’, ENT procedures and
‘low volume, high tariff ones’, needs to be
recognised and the tariffs adjusted to
ensure the viability of hospital services. The
specialist tertiary centres will be undertaking remarkably little in the way of
routine care and the low volume airway
cases as well as emergency care in the
under threes will need to attract much
larger tariffs or the units will become
unsustainable.
I personally believe that there is no place
for paediatric ENT services in the treatment centres (CATS/ICATS/SPMS). To
meet the exacting criteria of the healthcare
commission and to have facilities for safe
resuscitation and emergency management
and transfer, would be very costly. Current
tariffs make it non-viable and would see
both educational and financial destabilisation of NHS secondary care. Similarly the
private sector is unlikely to wish to
compete for NHS surgery on cost.
The trends we have seen for a reduction
in the overall numbers of tonsils and
grommet surgery, across the UK, will
almost inevitably continue because of
financial impecunity. Whilst academic
debate may still rage about the value of
these operations, the prospect for the next
two public spending reviews looks tight for
the NHS up to perhaps 2017, and increased
healthcare rationing in some form seems
inevitable.
This future as I have envisaged it is realistic. It would provide a structure for a safe
and enhanced delivery of care to children
across the whole spectrum of ENT conditions. It would allow for closely controlled
audit, but has not addressed the need for
paediatric ENT research in the UK.
Currently, no structure exists for the
choice to become a primary researcher in
paediatric ENT as a career. I would love to
see a future in which this deficit is
addressed. That would truly enhance
paediatric ENT care into the far future –
the final frontier. n
References
1. O’Dowd A. GP consortiums will need first class
management support. BMJ 2011;342:d337
2. Health & Social Care Bill, 2010-2011
[http://www.dh.gov.uk/en/Publicationsandstatistics
/Legislation/Actsandbills/index.htm]
3. National confidential enquiry into patient outcome
and death, 1989.
[http://www.ncepod.org.uk/1989.htm]
4. Trends in Children’s Surgery 1994-2005: Evidence
From Hospital Episode Statistics Data.
[http://www.bipsolutions.com/docstore/pdf/
16070.pdf]
5. Joint statement on General Paediatric Surgery on
behalf of the Association of Paediatric
Anaesthetists, the Association of Surgeons of GB
and Ireland, the British Association of Paediatric
Surgeons, the Royal College of Paediatrics and
Child Health and the Senate of Surgery for GB and
Ireland, 2006.
[http://www.rcseng.ac.uk/service_delivery/
children2019s-surgical-forum/
the-csf-e-newsletters/documents/
Aug%2006%20Joint%20statement%20GPS.pdf]
6. Pye JK. Survey of General Paediatric Surgery
Provision in England, Wales and Northern Ireland,
on behalf of the Association of Surgeons of Great
Britain and Ireland. Ann R Coll Surg Engl
2008;90(3):193-7.
7. ‘Raising the Standard: a Compendium of Audit
Recipes’ The Royal College of Anaesthetists
[http://www.rcoa.ac.uk/index.asp?PageID=125]
8. Guidance on the Provision of Paediatric
Anaesthesia Services, The Royal College of
Anaesthetists 2010
[http://www.rcoa.ac.uk/docs/GPAS-Paeds.pdf]
9. Robb,P. Provision of Children’s ENT Services, 2008
[http://www.rcseng.ac.uk/service_delivery/
children2019s-surgical-forum/
the-csf-e-newsletters/documents/PJR%20Newsletter
%20Childrens%20ENT%20March%2008.pdf]
10. Royal College of Surgeons of England, General
paediatric surgery service provision Survey
summary, 2010 [http://www.rcseng.ac.uk/publications/docs/
general-paediatric-surgery-service-provision-survey]
11. The London Specialised Children’s Services Review,
2011 NHS Commissioned.
[http://www.londonhp.nhs.uk/
wp-content/uploads/2011/03/
FINAL-London-specialised-childrens-services
-review-summary-guidance.pdf]
12. National Paediatric Surgery Reviews: Safe and
Sustainable 2011
[http://www.specialisedservices.nhs.uk/
safeandsustainable]
13. American Society of Anaesthesiologists Physical
Status Classification System
[http://www.asahq.org/For-Members/
Clinical-Information/ASA-PhysicalStatus-Classification-System.aspx]
audiology matters
Guest edited by Josephine Marriage
Post Newborn Hearing Screening ABR: quality assurance and the role of
peer review
Helping Families Accept Technology
Auditory Verbal Therapy Provision in the UK
Unilateral Hearing Loss in Early Childhood: what are we doing about it?
Amplification Options for Mild Bilateral Hearing Loss & Unilateral Hearing
Loss in Children: a literature overview
Paediatric Tool Development: developing a rationale for empowering
children with hearing loss
In this issue...
Josephine Marriage,
PhD,
UCL Ear Institute,
332 Grays Inn Road,
London, WC1X 8EE, UK.
am delighted to have been asked to edit this section on
Paediatric Audiology for ENT & audiology news. The implementation of newborn hearing screening programmes
(NHSP) around the world has reduced the age of diagnosis of
hearing loss for children and improved potential outcomes for
speech and language for these children. The fact that the UK was
the first country to introduce universal coverage of newborn
screening is testament to the innovative nature of its audiology
base, but paediatric audiology is now at a cross roads in the UK.
Screening is only the first step of the complex process of defining
extent and type of hearing loss and expanding family choices for
intervention. Guy Lightfoot gives a candid reflection of the pitfalls
and challenges of evaluating hearing levels on which options for
hearing aid amplification are based and the need for continuous
training and peer-review to develop clinical skills in recording and
interpreting ABR traces. While nationally-based policies and regulation are possible within an integrated healthcare system, the
lessons for constantly evolving effective practice are equally
important for all those engaged in assessing ABRs in infants.
The benefits offered by early hearing aid fitting to provide
neural stimulation from the first months of life need to be
matched by exposure to meaningful, socially-relevant auditory
communication. The use of hearing aids in infancy relies primarily
on parents, who may be struggling to consolidate the life changes
inherent with the arrival of a new baby with the devastating identification of hearing loss. Jane Madell has outlined her excellent
thoughts on helping to support the use of technology by parents
and other family members at this crucial time.
As non-verbal responses of an infant with hearing loss are not
the same as those of a typically hearing infant there is a need to
support the auditory-based communication dynamic. It is an
equally critical component in realising the benefits of early identification of hearing loss. Without this support David Luterman’s
comment that “Screening efforts have far out stripped our habilitation efforts, leaving parents with diagnosis but without
support” has a ring of truth. Parents are entitled to consider all
options in finding a technique or strategy that addresses their
own goals and wishes for their child. If the onus of responsibility
for use of amplification is conferred on the parents, they are
equally central to communication choices. Elizabeth Tyszkiewicz
provides an overview of the Auditory Verbal Therapy (AVT)
approach which, although widely available in other countries
with early screening programmes, until recently has only been
available through non-government providers in the UK. The
adoption of AVT at Manchester and Birmingham Cochlear
implant programmes marks an important stage in expanding
choices for auditory-oral communication in the UK.
One of the incidental challenges that has materialised from
NHSP is the identification of unilateral hearing loss, a proportion
I
38
of which will develop into bilateral hearing loss. Here, Priya Singh
provides an up-to-date and comprehensive review of the known
impact of unilateral hearing loss (UHL) in childhood, reviewing
the impact of UHL on susceptibility to speech delay, educational
underachievement and social disadvantage. This perspective is
far removed from the traditional advice proffered that ‘one ear is
enough’. Cherilee Rutherford then goes on to describe the developing range of amplification technologies appropriate for children with UHL emphasising that careful assessment and
management consideration for each individual case is required
in the same way as for bilateral hearing loss.
It is intriguing to note that this collection of articles has been
provided by seven people who were trained at universities in four
of the five continents of the world. A global resource now exists for
paediatric audiology, a necessary requirement if the challenges and
complexities in this specialised area of audiology are to be faced
head-on. Amplification and implantable devices are more
complex, and both the human interface between technology and
fundamental communication needs and the range of assessment
techniques available requires constant up-skilling and new patterns
of training. It is deeply unsettling therefore to report that the
training of graduate audiologists in the UK is regressing back to a
diagnostic science curriculum, to be combined with vision and
diagnostic neurosciences delivered by means of a three-year
training, in-service programme. The current economic climate and
Government cuts have reduced resources within the NHS UK
healthcare system, the universities and social support for deaf
people to the point that recent advances in hearing healthcare are
in real peril.
The apparent solution to these challenges – a fundamental
regression of audiology primarily to that of a diagnostic healthcare
science – will not only cost more to deliver but (in terms of the
actual spend required to produce graduates) it will likely generate
a large pool of poorly-trained healthcare workers, possessing only
low-level, generic qualifications and little currency within the international team of paediatric audiology professionals. Audiologists
are autonomous practitioners for long-term management of
hearing and balance impairments. These needs are not, and never
will be, met solely by the fitting of a hearing aid, as will become
apparent to the increasing proportion of the population with
hearing loss. There is no possibility that generically-trained
‘neurosensory’ diagnosticians will work in vision science one week,
diagnostic neuroscience the next and audiology the following
week, and still be able to fulfil the needs of infant, teenage, adult
and elderly hearing impaired people. As with many politicallycontrived schemes, this programme for the modernisation of
scientific careers will be soon be found to be unfit for purpose but,
for the coming decade of children born with hearing loss and their
families, the future looks opaque indeed. n
Post Newborn Hearing Screening
ABR: quality assurance and the
role of peer review
Guy Lightfoot
he English Newborn Hearing
Screening Programme (NHSP)
began in 2001 and implementation was completed across England in
March 2006. The NHSP Programme Centre
then embarked upon a Quality Assurance
programme to assess performance against
the agreed Quality Standards.1 The Quality
Assurance programme encompassed early
audiological assessment, habilitation and
early intervention as well as screening
activity. This QA process began to identify
problems with the quality of ABR assessment; some audiology departments’ standards of performing and interpreting ABR
tests were so poor that there were serious
risks of misdiagnosis. Indeed in some cases
the ABR service had to be suspended and
was only reinstated when further training,
supervision and ongoing quality assurance
had been implemented.
These errors are usually an example of
unconscious incompetence – people
thinking that they know what they are
doing, when they do not. This tends to
arise when staff do not understand the
underlying concepts of the task (were
T
Graham Sutton
Sally Wood
Correspondence
English Newborn
Hearing Screening
Programme Clinical
Group
Declaration of
Competing Interests
GL has acted as an
expert witness for the
plaintiff in legal cases
involving misdiagnosis
associated with poor
ABR practice. In all cases
to date, the defendant
has settled out of court.
never properly trained), do not update
their knowledge (CPD), or do not invite
external scrutiny of their work (peer
review). This is often linked to managerial
pressure on staff to accept new responsibilities without the necessary specialist
training or support. Another contributory
factor can be how often tests are
performed. Too small a catchment population and / or too great a number of staff
doing the work may result in an individual
performing tests too infrequently to
develop or maintain the required degree of
competence.
To address this urgent training need a
series of ABR refresher days was initiated to
disseminate the important principles
needed to ensure good ABR practice,
together with individual training visits and
mentoring of some departments. These
refresher days continue and are a good
means for testers to check their understanding of the latest guidance and to
benchmark their own standards.
Figures 1 and 2 give a ‘before and after’
example of ABR waveforms from one
department that had a ‘serious incident’
for a peer
review system to
work effectively
Figure 1
practitioners
must feel
confident and
comfortable in
submitting their
own results for
review
Figure 2
39
feature
One important weakness of any regional peer review group is that it can be
only as good as its best contributor
and subsequently had NHSP specialist
training and mentoring. Both cases were
discharged but the child shown in Figure 1
was actually profoundly deaf. Aficionados
of ABR will be able to identify the specific
errors made in this example. Such cases are
rare but do continue to occur.
Figure 2 is an example of the same
department’s current work, with unequivocal 4kHz clear responses down to
discharge level. Like many other departments, this centre also routinely tests at
1kHz although this exceeds the NHSP
requirement for discharge. It helps keep skills
sharp for those cases that require multifrequency assessment prior to amplification.
NHSP guidance for conducting and
interpreting ABR tests has been updated2,3
based on certain important requirements
designed to minimise the scope for serious
misdiagnosis.
One significant development included
in these recent documents is the move
away from clicks toward 4kHz tone pips as
the primary stimulus upon which
discharge is based. This was a move that
NHSP resisted for some time because
frequency-specific ABR responses are
smaller than click responses and this
presents a more challenging task, with
greater scope for interpretive error. We did
not want to compound existing errors of
procedure and interpretation by making
the task more difficult. However, this
change has now been introduced because
the skills of the majority of ABR staff have
improved to the required level.
An ABR discussion forum was added to
the NHSP web site so that interesting and
difficult cases could be submitted, ideas
shared and expert opinion given.
So what about peer review?
A pilot scheme in which all ABR test results
were reviewed at a regional (Strategic
Health Authority) level has been undertaken, after first training local ABR staff to
review tests to standards described in
current NHSP guidance. An infrastructure
including the anonymous distribution of
tests to reviewers and central support with
a team of national ‘expert’ reviewers was
necessary. This approach did demonstrably
improve practice but was clearly too
expensive and unwieldy to roll out as a
national model. The feedback provided by
the reviewers to testers was constrained by
40
the reviewing spreadsheet used and was
sometimes perceived as stark, impersonal
and negative with therefore limited effectiveness in terms of a catalyst for change. A
full report on this pilot is in preparation.
More attractive and manageable is the
establishment of regional peer review
groups. Some areas have successfully used a
regional peer review model for some time.
There are a number of models that could be
used, ranging from the independent review
of every case by local peers, to the occasional group meeting over coffee to discuss
‘difficult cases’. The former is labour intensive
and in practice simply doesn’t happen; the
latter is probably not very effective in raising
and maintaining standards.
In our opinion for a peer review system to
work effectively practitioners must feel
confident and comfortable in submitting
their own results for review. There must be
systematic selection of cases for review
otherwise there is a risk that only the best
results will be submitted for review. There
needs to be a systematic approach to the
review (we have a new spreadsheet that can
be used to structure the review) and individuals must be prepared to openly critique
the work of other (sometimes more senior)
clinicians, and we sometimes observe an
understandable reluctance to do this.
Other features of a peer review model
include:
● rotation of reviewers with sufficient
cover for holiday and sickness
● a quick (typically 48-72 hours) review
turnaround time for specific cases to
allow the feedback to be used in the
management of that case
● use of a standard review format plus
phone discussions between reviewer
and tester when helpful
● regular meetings between reviewers to
check and benchmark their own practice
● access to NHSP experts for disagreements, queries and periodic moderation
● annual report to include an audit and
review of arrangements and results of an
external moderation
One important weakness of any regional
peer review group is that it can be only as
good as its best contributor and errors
could continue to occur if poor methods
or standards are considered acceptable by
all participants. For this reason, all peer
review groups should have both an initial
training element and periodic independent
external moderation.
We would also like to strongly suggest
there needs to be a system of formal assessment and accreditation of competence of
individuals before they start routinely
performing newborn ABRs. This is equally
important for those performing VRA and
other behavioural tests on young children.
We hope that the professional bodies, DH
and HEIs will move rapidly towards such a
scheme.
Getting the right balance between
conflicting practical and technical requirements is difficult. NHSP are currently
helping establish a number of regional peer
review groups and will continue to learn
what works and what doesn’t.
Closing remarks
To use a motoring analogy, the UK still has
an ‘MOT’ test to check that cars are roadworthy. Many countries have abandoned
this idea, arguing that it is actually more cost
effective to allow accidents to happen than
to pay for the infrastructure around the
MOT. In pure financial terms that is probably correct. That is also what most countries have, by default, with their current
postscreening ABR test services: no systematic check of performance, with occasional
misdiagnoses leading to substantial financial
settlements. NHSP believe that our profession can and should do better than that; we
also think it is important to consider the
cost to the child of a missed opportunity to
optimally develop language. QA and peer
review may be somewhat inconvenient and
have financial implications but they are the
right thing to do. This is what marks us out
as professionals. n
References
1. NHSP Quality Standards. Newborn Hearing
Screening Programme (England)
[http://hearing.screening.nhs.uk/standardsandprotocols]
2. Guidance for Auditory Brainstem Response testing in
babies. Version 1.1 April 2010. Newborn Hearing
Screening Programme (England)
[http://hearing.screening.nhs.uk/
audiologicalassessment]
3. Guidelines for the early audiological assessment and
management of babies referred from the newborn
hearing screening programme. Version 2.5 March
2011 Newborn Hearing Screening Programme
(England) [http://hearing.screening.nhs.uk/
audiologicalassessment]
Helping Families Accept
Technology
Jane R Madell, PhD,
CCC A/SLP, ABA, LSLS
Cert AVT,
Director, Paediatric
Audiology Consulting,
Correspondence
450 West 23rd Street
New York, NY 10011
E: Jane@
JaneMadell.com
W
ith the technology that is currently available, almost every child should be
able to hear sufficiently to use audition to develop listening and spoken
language. However, children will only be able to use audition to develop
spoken language if they wear the technology which means that parents have to under-
stand and accept the need for full time use of technology. Why then, do parents have
difficulty accepting technology?
Declaration of
Competing Interests
None declared.
It is not really about the technology.
Today’s parents are comfortable with technology – they use cell phones, computers
and so on. But hearing aids are different.
Accepting hearing aids means accepting
the hearing loss. To succeed in getting kids
to use hearing aids full time, families must
accept the hearing loss. For each family, we
need to know what is interfering with the
family accepting the hearing loss. Why can
some families move ahead through their
sadness and fit hearing aids and start
therapy, while other families seem paralysed and cannot move ahead. While there
is a little time to spare, we need to get
hearing aids on relatively quickly to help
children become auditory learners and
develop an auditory brain.
Our ability to assist parents in accepting
hearing loss is closely tied to how we, as
clinicians, feel about hearing loss.
Although I feel sad for the family when I
diagnose a hearing loss, I am very optimistic about what is possible for a child
identified with hearing loss in 2011. I know
technology available today will enable
virtually every child to hear and learn,
especially when paired with auditory
based early intervention services. Part of
counselling families is passing on optimism about the future of children with
hearing loss. If we are not optimistic, we
do families a disservice. The first reason is,
because we will not provide them with
the hope to move on. Secondly, we may
not push as hard as we might to be sure
41
feature
Children will only be able to use audition to develop spoken language if
they wear the technology which means that parents have to buy into the
need for full time use of technology
kids are hearing as well as they can,
accepting ‘good enough’ in place of
‘excellent’. We can become more optimistic by getting to know families who
have kids who are successful, by talking to
and listening to kids who have had the
advantage of current technology, by
meeting today’s young adults, who did
not have the advantage of current technology, and are still successful. The more
optimistic we are, the more optimistic we
can help families become.
therapists in understanding performance
and planning remediation.
Keeping the technology on the
head
Parents need to believe that
technology will help
Parents need to understand that the ears
are the pathway to the brain, and that
audition is critical for auditory brain
development which results in both
spoken language and academic learning.
They need to understand that children
learn language by overhearing. Unless
they are hearing soft speech all day long,
they are going to miss out on incidental
learning. They need to know that auditory access leads to reading. Parents are
anxious that their children should be able
to fulfill their potential and be successful.
Helping them understand the relationship between hearing, language, reading
and learning is critical in helping them
understand the importance of wearing
technology on a full time basis.1
The technology needs to be
working optimally
The most encouraging thing for a parent
is seeing the child respond to sound. On
the other hand, if a child does not seem
to be responding, it is difficult for parents
to fight with children to keep the equipment on. When a parent first sees a child
alert, turn to sound, or search for a
sound, they feel hopeful – “maybe this
will be okay,” and they can work on being
sure the technology is kept on.
How do we know if technology is
working optimally? It is not guesswork.
We need to test. With tiny ones, we start
off with an ABR, but we need to move to
behavioural testing quickly. ABR cannot
42
be used to monitor hearing aid performance but behavioural testing can.
Behavioural observation audiometry
(BOA) can be used to test infants under
six months of age,2,3 VRA and
Conditioned Play can be used for older
children. Obtaining aided thresholds and
detection levels for the Ling sounds with
hearing aids will let us, and parents, know
if the hearing aid is providing sufficient
gain throughout the frequency range.
Moeller et al. (2010)4 have reported that
40% of children are not receiving sufficient gain from their hearing aids. Real
Ear testing tells us what is reaching the
ear drum, but not what is reaching the
auditory brain. While Real Ear testing is
critical, it is not sufficient. Aided thresholds can be accurately obtained by
starting below threshold and presenting
short duration warble tones or noise
bands. This should not set off compression and should provide reliable results. If
the child is not hearing softly enough,
hearing aid settings can be adjusted to
provide sufficient hearing to receive all
auditory information. Once a child is old
enough, speech perception testing
should be added to the test protocol at
normal and soft conversational levels in
quiet and in competing noise.5 This information is useful both for parents and for
Parents sometimes have difficulty
keeping technology on. It is important to
know why in order to make the appropriate suggestions. Children sense their
parent’s discomfort and may remove
hearing aids for that reason. Parents need
to understand that they must give a very
clear message to their children indicating
that the hearing aids may not be
removed. If the child takes it off, it has to
go right back on. Children should be
using the hearing aids full time after two
weeks. Parents need to understand that it
will be a difficult two weeks but once
accomplished, the child is on his / her
way. Keeping the child entertained helps.
Children are less likely to take technology
off while they are being read to and more
likely to remove it when alone in the back
seat of the car (FM can help in that situation). A bonnet covering the ears and tied
under the chin will help in keeping
hearing aids on during the early weeks.
Being optimistic about what is possible
for children with hearing loss, will help
parents be optimistic. Our job is to keep
them from giving up. n
References
1. Robertson L. Literacy and Deafness: Listening and
Spoken Language. Plural Publishing; San Diego:
2009.
2. Madell JR. Using Behavioral Observation
Audiometry to Evaluate Hearing in Infants from
Birth to 6 Months. In Pediatric Audiology:
Diagnosis, Technology, and Management. Edited
by Madell JR, Flexer C. New York; Thieme: 2008.
3. Madell J. Behavioral evaluation of hearing in
babies: Yes, you can! AudiologyOnline Recorded
Course 12919, eLearning library; 2009.
[http://www.audiologyonline.com]
4. Moeller PM. Outcomes of children with Hearing
Loss. ASHA Conference, Boston; 2010.
5. Madell JR. Evaluation of Speech Perception in
Infants and Children. In Pediatric Audiology:
Diagnosis, Technology, and Management. Edited
by Madell JR, Flexer C. New York; Thieme: 2008.
Auditory Verbal Therapy
Provision in the UK
Elizabeth
Tyszkiewicz,
Auditory Verbal
Therapist.
Correspondence
Children's Service,
Midlands Hearing
Implant Programme,
Optegra Building, Aston
University, Birmingham,
B4 7ET, UK.
E: Elizabeth.Tyszkiewicz
@bch.nhs.uk
A
uditory verbal therapy, is “an individual, auditory, developmental programme,
implemented by the child’s family in close collaboration with a therapist, with the
goal of achieving age appropriate spoken language ability, and full social partici-
pation throughout childhood and beyond.” (AVUK, 2003). It has particularly urgent rele-
vance now, as NHSP and early amplification fitting presents support services with what
Carol Flexer, Distinguished Professor Emeritus, University of Akron and Northeast Ohio
AuD Consortium (NOAC), calls ‘a new paradigm’:
“Rapid infant brain growth requires prompt intervention, typically including amplification
Declaration of
Competing Interests
None declared.
and a program to promote auditory skill development. In the absence of sound, the brain reorganises itself to receive input from other senses, primarily vision: …this reduces auditory
neural capacity.” (Carol Flexer, 2011).
The parent is at the centre of each AVT session (photo by kind permission of Auditory Verbal UK).
AVT is labour
intensive,
requiring
whole-family
commitment
and active
participation
Auditory Verbal Therapy (AVT) practice
developed in North America, and is well
established in Australia. It increasingly
informs mainstream thinking about service
provision for children with early-identified
hearing impairment in UK and Europe, as
well as many other countries around the
world. AVTs are licensed practitioners in
Audiology, Speech and Language Therapy
or Education, who have undergone a three
year post-qualification training and
mentoring programme. The knowledge
base for this programme of study encompasses a range of domains, including parent
guidance, education and support, child
development, spoken language communication, auditory functioning, hearing and
hearing technology, strategies for listening
and spoken language development. A key
element of the practice is the provision of
regular, individual hour-long parent and
child sessions, until the child is shown to
have attained age-appropriate spoken
language ability, or makes the transition to
another, more appropriate programme.
The approach is founded on the principle
that language is learned by all children from
the main carers, usually the parents. The
therapist provides an intensive, individual
coaching programme, through which they
gradually gain confidence and expertise to
become the main ‘agent of change’ for the
listening and spoken language development of their child with impaired hearing.
Families who research options when they
find out their child has impaired hearing
often identify AVT as relevant to them, but
have difficulty in finding a suitably qualified
43
feature
therapist. Until recently, the limited resources
available in the UK were all in the independent sector, either as private practitioners, or
within voluntary sector organisations.
Now, two of the Cochlear Implant
programmes in the UK offer follow-up from
Certified AVTs – others employ therapists
who have an interest, or have undergone
training in AVT. There is considerable interest
from both specialist educators, and speech
language therapy services, as they are
increasingly asked to provide for the children
identified via the NHSP. However, there is still
no therapy offered in NHS departments
which fit non-implantable hearing technology, and which cater for the majority of
children with permanent hearing impairment in the UK.
In Auditory Verbal Therapy, an informed
choice to ‘opt in’ is involved, from the
family’s and the therapist’s point of view. You
don’t find yourself in an AVT programme by
default, or by accident.
Especially in the early years, AVT is labour
intensive, requiring whole-family commitment and active participation. It sets clear
short and long-term goals for the child, and
constantly generates new diagnostic information which must be incorporated into
day to day living. The child’s family must
regularly attend AVT sessions, and also,
inform and enlist the support of the wider
team, working to ensure the child has access
to mainstream education. People often
imagine there is ‘homework’ for the parents.
In fact, it is more a matter of learning to
interact with the child at all times in a way
that stimulates the development of the
‘auditory brain’ through spoken language
embedded in play, problem solving, and the
most apparently mundane activities of daily
living. It takes far more energy than the
already considerable demands of bringing up
young children.
For the therapist, the programme means
hour-long, packed and very detailed
sessions, tailored to the learning needs of
the specific family and child. Although good
therapy is often mistaken by observers for a
relaxed and playful time, each session
contains careful planning, goal-setting, and
targeted activities. Regular individual assessment and reporting are crucial, and there is
hands-on coaching for parents throughout
the session. The emphasis is strongly on
audition, with a consequent need to
monitor / maintain technology, feeding
back immediately to audiologist or ENT
specialist when hearing is not optimal. Each
session yields diagnostic information which
must be turned into action to be implemented. The therapist must also inform,
44
and enlist the support of the team around
each child in the school / local area. At the
early stages, the therapist, working against
the adults’ natural belief that information
must be made visually easy for the child, has
to make changes in the learning environment and ‘kickstart’ the neural connections
between sound and meaning. It is crucial
that the family feels successful, and sees
progress as a result of their effort.
Sometimes the steps are very small, and the
therapist has a crucial role in helping them
to observe changes in their child’s learning.
All the above are in conflict with trends in
public sector service delivery, which are
away from individually tailored care, and
towards generic ‘advisory’ programmes, or
group-based interventions.
You cannot ‘grow’ an early developing
auditory brain in a group. If true spoken
language competence is to emerge, the child
needs an optimised version of the opportunities given to the typically hearing
newborn: individual, highly audible, and
exactly tailored to move the learning on to
the next developmental stage. Where there
is no hearing difficulty, parents provide all
this without a second thought. In the presence of hearing impairment, and using
hearing technology, AVTs are there to map
the route, support the process, and measure
the outcome at regular intervals to ensure
learning is on track.
Within an NHS service such as the
Midlands Hearing Implant Programme
Children’s Service in Birmingham, the caseload is extremely diverse, in terms of social
circumstances, hearing history, and the presence of complicating factors in addition to
hearing impairment. While families make an
informed choice to have a cochlear implant
for their child, they may not wish, or be able,
to opt into an intensive AVT follow-up
programme. Other family pressures or social
needs may come first, or, indeed, they may
have other goals for their child than those
defined by AVT, which aims for fluent
spoken communication and mainstream
schooling. Flexibility and adaptability are
also key features of AVT practice in the
course of the diagnostic assessment.
Hearing impairment doesn’t mean there are
no other complications which interfere with
learning. It may be necessary for the AVT
practitioner to become the ‘hearing adviser’
on a multidisciplinary team which addresses
the child’s predominant needs. A great
strength of AVT is its rigorous use of
ongoing diagnostic observation and formal
assessment in the domains of audition,
speech, language, cognition and social interaction. There is very little ‘watchful waiting’
in AVT practice. The clear understanding of
each child’s latent auditory potential, and of
the typical developmental trajectory, leads
to active investigation of the reasons why
milestones are not reached in a timely
fashion. Sadly, there is inequity of access to
services, not only because certified therapists are still rare (totalling approximately
ten in the UK) but because some people
now believe that AVT is ‘only for children
with cochlear implants’, as this is the group
that most readily gains access to AVT
services in the public sector.
Ideally all children with impaired hearing
would be offered the strong diagnostic
benefits of AVT to optimise use of hearing
technology, inform management, and identify complicating factors. If paediatric
hearing aid departments had a staff AVT, a
missing piece of the NHSP puzzle might be
slotted into place: intervention would begin
on the day of amplification fitting, and
dovetail smoothly with follow-up from the
local multidisciplinary team.
The need for quality intervention to
ensure that children with impaired hearing
fulfil their true listening and speaking potential has colossal training implications. The
‘paradigm change’ in the last few years
means that not enough people know how
to harness the tremendous human potential of the next generation of young children
with impaired hearing, or what it is reasonable to expect from them and from their
families. On the other hand, there is a
groundswell of interest and initiative which
bodes well for the future: some trainee audiologists receive a grounding in the theory
and practice of Auditory Verbal Therapy,
special interest groups (SIGs) meet regularly
to learn and network, short courses and
training days are offered in the public and
voluntary sectors, web based resources are
easily accessible (see references below), and
a Post-Graduate Diploma course is offered
at Aston University in Birmingham. n
References
Dornan D, Hickson L, Murdoch B, Houston T,
Constantinescu G. Is Auditory-Verbal Therapy Effective
for Children with Hearing Loss? The Volta Review (Fall)
2010;110(3):361-87.
Goldberg DM, Flexer C. Auditory-verbal graduates:
outcome survey of clinical efficacy. J Am Acad Audiol
2001;12(8):406-14.
Hogan S, Stokes J, White C, Tyszkiewicz E, Woolgar A.
An evaluation of Auditory Verbal therapy using the
rate of early language development as an outcome
measure. Deafness Educ Int 2008;10(3):143-67.
Alexander Graham Bell Association for the Deaf and
Hard of Hearing [www.agbell.org]
Auditory Verbal UK [www.avuk.org]
Equal Voice for Deaf Children [www.evdcweb.org]
Unilateral Hearing Loss in Early
Childhood: what are we doing
about it?
Priya Singh, AuD,
Senior Clinical Lecturer,
Correspondence
UCL Ear Institute, 332
Grays Inn Road, London,
WC1X 8EE, UK.
T
he impact of unilateral hearing loss (UHL) on children was recognised and
reported by Bess, Tharpe and colleagues in 1986. Since then, evidence has
continued to emerge to change the popularly held misconception that UHL
is a benign condition that requires little more than identification. The introduction of
newborn hearing screening programs (NHSP) has allowed cases of UHL to be identified
Declaration of
Competing Interests
None declared.
earlier in life than was previously the case. However, there are no recommendations for
specified early intervention for these children. Management therefore varies from one
centre to the next, from no intervention to amplification with or without support
services. The purpose of this review is to remind ourselves of what we know, what we
don’t know and to ensure that we make best use of the early identification to start
management as soon as possible.
Benefits of binaural hearing
Research has enlightened thinking on the
benefits of binaural hearing in all listening
environments. Studies on binaural listening
and amplification have shown favourable
effects from binaural summation on speech
understanding and ease of listening. Studies
on the head shadow effect have demonstrated that children with UHL had poorer
speech discrimination scores than normal
hearing peers regardless of which ear the
signal and noise were directed at (that is,
monaural direct or indirect). This has implications for the child in a classroom, or
kindergarten, from poor signal to noise
ratios, even when the child has preferential
seating arrangements. It is known that children with UHL require a higher signal to
noise ratio than adults when listening to
speech in noise. When assessing the impact
of the squelch effect, it is necessary to look
specifically at noise effects in children as
they are less efficient in suppressing background over the primary signal in order to
improve speech perception. When the
negative impact of background noise is
coupled with reverberation and poor classroom acoustics as is the commonest
context for learning speech, language or
academic skills, it is clear that there are good
theoretical reasons for there being a functional negative impact from UHL in these
areas of childhood learning.
Howard et al. (2010) measured listening
effort using a dual task paradigm in a
typical classroom and found that while
children were able to maintain performance on the primary task, the higher
listening effort required greater cognitive
resources, and that performance decreased
on the secondary task. Tharpe’s studies on
dual task paradigm (2002) found that even
children with normal hearing had difficulties with these tasks.
Impact of UHL on speech and
language development, and
educational achievement
The literature on speech and language
development shows mixed outcomes,
with some studies reporting delay in
speech and language development in children with UHL and others not. A number
of studies showing disadvantage experienced by this population are given in Table
1 below. The majority of the studies were
on school aged children, and it is important to note that in most it is not known
whether the hearing losses were congenital
or acquired or whether pre- or postlingually acquired, which may impact on
the findings. Further, these early studies
were conducted before the introduction of
newborn hearing screening, implying later
identification rather than late onset of the
hearing loss.
children with UHL require a higher signal to noise ratio than adults
when listening to speech in noise
45
feature
Table 1. Studies looking at speech and language development in UHL vs normal hearing children.
Study and Year
Severity of UHL
Number of subjects
Speech / Language Problems
Klee & Davis-Dansky (1986)
>= 40dB
25
Few differences on 6 standardised language
subtests (6-13 years)
Borg et al. (2002)
>= 20dB
58
Delayed language development
Kiese-Himmel (2002)
>= 30dB
31
Normal first words but delayed acquisition
of 2-word phrases
Colorado HIP (2002)
Varied
15
Significant or borderline language delay in
33%
Colorado Study II (2003)
30-120dBHL
155
8% received speech and language therapy,
and in total 53% had other interventions
Table 2. Impact of UHL on education and academic development.
Study and year
Severity of UHL
Number
Educational Findings
Bess and Tharpe (1986)
>= 45dB HL
60
51% satisfactory, 35% repeated a grade,
13% needed assistance
Bovo et al. (1988)
Profound
115
22% failed a grade, 12% needed assistance
Oyler et al. (1988)
>= 25dB HL
54
27% failed a grade, 41% needed assistance
Watier-Launey et al. (1998))
>= 20dBHL
175
40.4% repeated a grade compared to 16%
in general population
Hallmo et al. (1986)
>= 20dB HL
56
School results & linguistic development
normal (NB: no testable or repeatable
measures used, no comparison group)
One of the challenges in evaluating the literature with respect to UHL in
children is the lack of a universally accepted definition
Table 2 above summarises the findings of
studies regarding the impact on educational
and academic outcomes for children with
UHL. Many of these studies show that a
significant proportion of the children with
UHL repeated a grade, with an additional
number requiring additional educational
support. The failure rate at school in the Bess
and Tharpe (1986) study found that children
with UHL were 10 times greater than the
failure rate of the general school population,
which was only 3.5%. Waber-Launay et al.
(1998) had a larger number of children in
their study and had a similar finding, with
40% of the children with UHL failing a grade
compared to 16% of the normal hearing
population.
It is important to note that in the
majority of the studies listed in Tables 1
and 2, the findings were similar irrespective
of the degree of UHL, thus suggesting that
even mild unilateral loss can have a functional impact on achievement and should
not be ignored. This may be shown to
relate to reduced neural inhibition within
the brainstem pathways, thereby changing
the input to the ‘normal hearing’ ear, in
future physiological research.
46
Behaviour at school and quality of
life considerations
In addition to educational achievement,
behaviour at school is another factor for children with UHL. Brookhauser (1991), in a five
year study of 391 children with UHL, found
that 59% had academic and behavioural
problems. Behavioural issues in school were
also noted by Bess and Tharpe (1984) and
Stein (1983) who reported that 24% of the
group of 19 had behavioural problems in
spite of adequate grades. These issues could
be related to frustration and fatigue created
by the concentration level necessary for UHL
children in poor listening conditions,
resulting in a lack of co-operation and
perceived inattention at school.
If we are to be truly comprehensive in the
assessment and management of children
with UHL, then their mental well-being must
also be considered. Studies on children with
bilateral hearing loss demonstrated lower
scores on tests which assess quality of life
(QoL) than normal hearing individuals. The
negative emotional and social impact of
UHL in adults has been documented. At
present there is limited data available
regarding the QoL in children with UHL,
which requires further investigation and
consideration.
Co-exisiting intrinsic and extrinsic
factors
UHL has not been fully explored with
respect to other co-existing factors like
cognitive deficits, developmental disabilities,
and demographic factors such as socioeconomic status, level of language stimulation at home, child care quality and
common childhood diseases like otitis
media with effusion (OME). This co-existence may create a negative synergy, and
these children are at greater risk for speech
and language delays during the critical
period of development, which then impacts
on educational progress.
Challenges in evaluating the
evidence base
One of the challenges in evaluating the
literature with respect to UHL in children is
the lack of a universally accepted definition.
Bess et al. (1998) provided a definition
which was adopted by the CDC in 2005
but there are a number of other definitions
used in research for example Niskar et al.
feature
(1998) and BAAP (2009). These differences
probably contribute to the high variability
in the prevalence figures for children with
UHL (Ross et al., 2010). From an estimation
of 1 to 3 per 1,000 newborns and 1 to 5%
of school aged children having UHL, Davis
(2005) reports a prevalence of 0.7/1,000
identified with UHL in the NHSP. It must be
noted however that there is the possibility
of underestimation of true prevalence
figures not solely due to definitions, but
also the types of losses included in these
figures, poor follow-up rates, acquired aetiologies and newborn screening programs
not being specifically designed to identify
UHL. It is important to recognise the
importance of new skills in applying appropriate masking in follow-up testing with
both objective and behavioural testing. In
addition, late identification has led to
causes of the hearing loss not being fully
investigated, thus making it difficult to
know whether the losses are congenital or
acquired and, more importantly, which
losses are likely to deteriorate or progress
to bilateral hearing loss.
Clinical strategy for children with
UHL
With newborn hearing screening
programmes (NHSP) identifying UHL in
early life and in greater numbers than was
the case a decade ago, audiology teams are
in a better position to intervene early on. Full
evaluation of the aetiology of UHL ensures
that risk of progression to bilateral HL is identified, and can be addressed in the treatment
planning. This allows parents to be part of
the decision-making and management for
their children to minimise speech / language
and education delays. There needs to be ongoing audiological assessment to monitor
progressive and fluctuating losses and to
identify cases that may become bilateral as
soon as possible, usually associated with
enlarged vestibular aqueduct syndrome
(EVAS). It seems that early neural stimulation
of the auditory system through both ears
applies just as much to UHL as to bilateral
HL. Full and effective masking to define true
hearing levels in the impaired ear, are critical
in deciding on the potential benefit of
amplification, which has no benefit on a
profoundly deaf ear.
Management options, including amplification, acoustic modifications and FM
systems, must all be considered and trialled
where appropriate. Amplification trials
should not be based merely on degree of
loss and speech perception scores, but
quality of life as well. Appropriately high
expectations can play a very important
role and as such the intermittent use of
amplification may have to be considered if
there is the potential for improving academic and QoL outcomes for these children
in certain situations. In addition, functional
auditory assessments for both the child
and the parents can contribute valuable
information for rehabilitation, together
with speech / language assessment and
monitoring. Even if we are uncertain of the
benefits of the intervention strategy, we are
obligated to consider them and evaluate
outcomes before they are discounted,
ensuring that informed choices are being
made for the child with the family. A study
conducted by the NHSP team in the UK
(Bears study) recognised that the impact of
diagnosis of UHL in early life could be just
as devastating for families as for those with
bilateral hearing loss, which lead into a
structured intervention plan.
Borg E, Risberg A, McAllister B, Undemar BM, Edquist G,
Reinholdson AC, Wiking-Johnsson A, WillstedtSvensson U. Language development in hearing-impaired
children. Establishment of a reference material for a
'Language test for hearing impaired children. Int J Pediatr
Conclusion
McKay S. To aid or not to aid: children with unilateral
hearing loss; 2002. [http://www.audiologyonline.com/
articles/pf_article_detail.asp?article_id=357]
The negative impact of UHL on children’s
speech and language development and
academic outcomes is now well recognised. With the NHSP detecting cases of
UHL in early life, there is a clear need to
define the population, to identify risk
factors for speech, language, educational
and social delay in UHL and to establish
consistency, timely and effective management for each case aiming to reduce the
gap between detection and appropriate
intervention. The management plan starts
at diagnosis and continues into school and
beyond. We have an obligation to
contribute to policy across audiology
teams and to be the advocates for these
children and families, improving their QoL
and levelling the playing field for equal
opportunities and choices into adulthood.
The current evidence base for UHL is clear;
UHL is not benign and therefore should
not be treated as such. Our approach to
management of UHL in children may be
expressed by a quotation by
Michaelangelo: “The greater danger for
most of us lies not in setting our aim too
high and falling short; but in setting our
aim too low, and achieving our mark.”n
Further reading
Davis A, Devoe S, Robertson H. UK Trial of Early amplification in children with unilateral or mild hearing impairments. National workshop on mild and unilateral hearing loss, Colorado, CDC and Marion Downs Centre;
2005.
Watier-Launcey C, Soin C, Manceau A, Ployet MJ.
Necessity of auditory and academic supervision in
patients with unilateral hearing disorders: retrospective
study of 175 children. Ann Otolaryngol Chir Cervicofac
1998;115(3):149-55.
Ross DS, Visser SN, Holstrum WJ, Qin T, Kenneson A.
Highly variable population-based prevalence rates of
unilateral hearing loss after the application of common
sense definitions. Ear Hear 2010;31(1):126-33.
Fitzpatrick EM, Durieux-Smith A, Whittingham J.
Clinical practice for children with mild bilateral and unilateral hearing loss. Ear Hear 2010;31(3):392-400.
Bess FH, Tharpe AM. Case history data on unilaterally
hearing-impaired children. Ear Hear 1986;7(1): 20-6.
Lieu JE, Tye-Murray N, Karzon RK, Piccirillo JF. Unilateral
hearing loss is associated with worse speech-language
scores in children. Pediatrics 2010;125(6):1348-55.
Kiese-Himmel C. Unilateral sensorineural hearing
impairment in childhood: analysis of 31 consecutive
cases. Int J Audiol 2002;41(1): 57-63.
Lieu JE. Speech-Language and educational consequences of unilateral hearing loss in children. Arch
Otolaryngol Head Neck Surg 2004;130(5):524-30.
Lieu JE. Children with unilateral hearing loss. Semin Hear
2010;31(4):275-88.
Kenworthy OT, Klee T, Tharpe AM. Speech recognition
ability of children with unilateral sensorineural hearing
loss as a function of amplification, speech stimuli and
listening condition. Ear Hear 1990;11(4):264-70.
Brookhauser PE, Worthington DW, Kelly WJ. Unilateral
hearing loss in children. Laryngoscope 1991;101(12, part
1):1264-72.
Oyler RF, A. Oyler AL, Matkin ND. Unilateral hearing
loss: demographics and educational impact. Language,
Speech and Hearing Services in Schools 1988;19:201-10.
Bovo R, Martini A, Agnoletto M, Beghi A, Carmignoto
D, Milani M, Zangaglia AM. Auditory and academic
performance of children with unilateral hearing loss.
Scand Audiol Suppl 1988;30:71-4.
Borton SA, Mauze E, Lieu JE. Quality of life in children
with unilateral hearing loss: a pilot study. Am J Audiol
2010;19(1):61-72.
Stein DM. Psychosocial characteristics of school-age
children with unilateral hearing losses. J Acad Rehabil
Audiol 1983;16:12-22.
Klee TM, Davis-Dansky E. A comparison of unilaterally
hearing-impaired children and normal hearing children
on a battery of standardized language tests. Ear Hear
1986;7(1):27-37.
Tharpe AM. Minimal hearing loss in children: the facts
and the fiction. Sound foundation through early amplification, Chicago; 2007.
Tharpe AM. Unilateral hearing loss in children: a mountain or a molehill? Hear J 2007;60(7):10-17.
Tharpe AM. Unilateral and mild bilateral hearing loss in
children: past and current perspectives. Trends Amplif
2008;12(1):7-15.
Bess FH, Tharpe AM. Unilateral hearing impairment in
children. Pediatrics 1984;74:206-16.
Holstrum WJ, Gaffney M, Gravel JS, Oyler RF, Ross S.
Early intervention for children with unilateral and mild
bilateral degrees of hearing loss. Trends Amplif
2008;12(1):35-41.
Yoshinaga-Itano C. Johnson CD, Carpenter K, Brown AS.
Outcomes of children with mild bilateral hearing loss and
unilateral hearing loss. Semin Hear 2008;29(2):196-210.
47
Amplification Options for Mild
Bilateral & Unilateral Hearing Loss
in Children: a literature overview
Cherilee Rutherford,
AuD,
Lecturer and Director of
the MSc Advanced
Audiology & Audiology
Short Courses.
Correspondence
The Ear Institute,
University College
London, 332 Gray’s Inn
Road, London, WC1X
8EE, UK.
E: cherilee.rutherford@
ucl.ac.uk
Declaration of
Competing Interests
None declared.
T
he past few years has shown a growing interest from professionals in the diagnosis and management of children with minimal hearing loss, including mild
bilateral (MBHL) and unilateral hearing loss (UHL). In addition to moderate
and more severe losses, MBHL and UHL are sometimes identified through universal
newborn hearing screening programs, but more often these type of hearing losses are
identified later during the school age years. The impact of moderate and severeprofound hearing loss on language, communication and academic development is well
documented and subsequent management in terms of amplification, parental guidance,
and early communication intervention is well established. The management involved
with MBHL and UHL is less clear and many questions remain unanswered. In 2005 the
Centre for Disease Control and the Marion Downs Hearing Centre hosted a national
workshop on mild and unilateral hearing loss to consolidate current knowledge
regarding prevalence, screening, diagnosis, amplification, outcomes, and early intervention. This review will attempt to (1) highlight the differences between MBHL, UHL, and
single-sided deafness (SDD) and, (2) summarise the amplification options as part of the
holistic treatment approach for children with MBHL or UHL.
Definitions of MBHL, UHL & SDD
Historically there has been no consensus
on the definitions of MBHL and UHL and
terms like ‘slight’, ‘mild’, and ‘minimal’ were
used to describe a variety of permanent,
sensorineural, conductive, and unilateral
hearing losses ranging from 16-45dB HL.
For the purpose of this review the definitions of MBHL and UHL will be used as
proposed by the 2005 CDC National
Workshop:
MBHL: “Calculated or predicted average
pure tone air conduction (PTA) thresholds
for both ears at 500Hz, 1kHz, 2kHz
between 20 and 40dB HL or PTA thresholds greater than 25dB HL at two or more
frequencies above 2kHz (that is, 3, 4, 6,
8kHz)”.
UHL: “Calculated or predicted average
PTA threshold in the low frequencies
(500Hz, 1kHz, and 2kHz) of any level
greater than 20dB HL, or, in the high
frequencies, PTA thresholds greater than
25dB HL at two or more frequencies above
2,000Hz in the affected ear. PTA average
thresholds in the good ear should be less
than or equal to 15dB HL”.
Ross et al. (2008)1 described terms such
as ‘mild’, ‘slight’, ‘minimal’ as confusing and
indicated that the terminology could be
misleading as to how serious the effects of
these types of hearing losses potentially
could be.
Single-sided deafness (SDD): Sometimes
the terms UHL and SDD are used interchangeably and again these can be
confusing. Although there is currently no
clinical consensus, the more typical reference to SDD is in the context
of
permanent,
severe-to-profound
sensorineural hearing loss in the poor ear,
with normal hearing in the other ear.2,3
Hearing aids
Hearing aid fitting to children with MBHL
presents parents and clinicians with quite a
few dilemmas. Does the benefit of amplification outweigh the cost associated with
...terms such as ‘mild’, ‘slight’, ‘minimal’ as confusing and indicated
that the terminology could be misleading as to how serious the effects of
these types of hearing losses potentially could be
48
feature
it? Should children be fitted monaurally or
binaurally? What type of ear mould
coupling is preferred and which dynamic
features are proven to deliver the best
outcomes? Would non-amplification be
detrimental to their development and when
is the best time to provide hearing aids?
More importantly, what are the benefits of
amplification for this group? Davis, Reeve &
Hind (2001)4 suggested that benefit assessments for amplification should include
acceptance of amplification, use of amplification, development of speech, language
and communication, quality of life for the
child, and quality of family life. These are
questions that clinicians are faced with in
everyday practice and unfortunately the
evidence base is currently not yet there to
support decision making. While the clinical
community is awaiting the results of
research efforts in this field, clinicians are
encouraged to follow existing guidelines
(American Academy of Audiology
Paediatric Amplification Protocol, 2003;
JCIH 2007 Position Statement)5,6 until more
evidence becomes available.
McKay, Gravel, & Tharpe (2008)7
suggested that the lack of acoustic stimulation could lead to auditory deprivation.
Children who have been wearing amplification for longer were found to have better
speech recognition abilities. They propose
that, in a conservative effort to reduce poor
speech recognition in the future, hearing
aids should be considered and offered to
children with MBHL or UHL. Following
current best practice guidelines, this means
that a binaural fitting would be recommended for MBHL patients. According to
the American Academy of Audiology
Pediatric Amplification Protocol (2003),5
children with MBHL should be considered
for amplification and / or FM systems.
Decisions about trialling hearing aids and /
or FM systems should be made with the
family and child’s preferences in mind, and
considering the child’s educational and
communicative development. When the
decision has been made to fit hearing aids,
standard good practice guidelines should be
followed, including the use of probe microphone measures and existing prescriptive
methods such as the Desired Sensation
Level (DSL i/o). Several input levels should
be measured to prevent over-amplification
in this population. Probe microphone
measures should also be performed at every
appointment to accommodate the child’s
growing ear canal that could influence gain
settings on the instrument. RECD’s should
be used when possible. When a child rejects
the use of hearing aids, it may be that some
acoustic cues have been reduced by the
fitting of a full shell earmould. Open fittings
should be considered to maintain the
natural quality of the child’s own voice
perception, although an external vent may
be more suitable for infants with small ear
canals.
McKay, Gravel, & Tharpe (2008)7
provided these practical guidelines on
hearing aid features and settings for children
with MBHL:
1. Hearing instrument features need to be
considered for each patient individually.
2. Feedback cancellation may not be necessary due to limited gain settings, but it
may be required in certain conditions
such as when the child is wearing a hat or
lying down.
3. Other features such as multiple memories
and directional microphones should be
considered in light of the child’s ability to
manipulate the controls and choosing the
correct situation to make the adjustment
in. The use of automatic microphone
switching could be beneficial. Alternatively,
the hearing aid could be set to directional
mode during instruction time.
Tharpe, Eiten & Abbot Gabbard
(2008)8 suggested further that:
1. Linear amplification with low threshold
compression could be appropriate on the
basis that children with MBHL have a
larger dynamic range of hearing so WDRC
is not needed.
2. Omni-directional microphone setting
should be used for infants / toddlers who
are mostly in the home environment
perhaps with auto switching for schoolage children with the option to manually
override.
3. Clinicians should carefully consider the
internal noise floor in certain hearing aids
that may interfere with speech perception.
The issue of binaural interference (poorer
bilateral performance with asymmetric
auditory input) may be a problem in children with UHL who have been provided
with a hearing aid on the poor ear and
therefore should be monitored closely by
the clinician.
FM systems
FM is a type of wireless technology that helps
with speech understanding in noise. The
person speaking typically wears a transmitter
microphone and speech signals are then sent
directly to the listener via harmless radio
waves to an FM receiver which the user wears
as part of a small behind-the-ear hearing aid.
It is well known that children with HI
need a much better signal to noise ratio
(SNR) than normally hearing (NH) children
for optimum speech understanding in background noise. FM systems provide the best
improvement in SNR (perhaps as much as
12-18dB) over directional hearing aid microphones (Hawkins, 1984).9 As a general
guiding principle, Hawkins recommended
that HI children in mainstream education
should have the best sound quality from
amplification and SNR as reverberation
times and SNR in typical classrooms are
often outside recommended optimal levels.
There is a common perception among
professionals that FM use is reserved for
more severe-profound hearing losses and
that the high cost of personal FM systems
often discourage patients from making use
of it. Perceptions like these need to be challenged by evidence about the real world
benefits of FM use in children with milder
losses. A combination of speech perception
scores, functional measures, and survey data
could be used to demonstrate the efficacy
of such an approach to amplification.
When should an FM system be offered to
a child with MBHL? Tharpe, Eiten & Abbot
Gabbard (2008)8 suggested that candidacy
be considered when a child performs more
poorly than normative data in speech in
noise tasks, struggles academically, shows
listening fatigue, or struggles to listen in
noisy situations. This decision needs to be
made while taking the parents’, teacher’s
and, importantly, the child’s views into
account. A monaural FM fitting configuration is recommended, leaving the other ear
unoccluded and free for surround listening
when the speaker is not using a microphone.8 Hawkins (1984)9 recommended
that the teacher’s transmitter microphone
should be set to directional mode as the
standard configuration (this would lead to
an additional 3dB advantage). McKay et al.
(2008)7 pointed out that sound level FM /
infrared devices could potentially benefit all
children in a classroom (HI and NH), as well
as the teacher (less vocal strain), but it might
not be sufficient for the HI child who may
still require an ear level FM system in addition. It is recommended that this be
provided on a trial basis and that an assessment tool such as the SIFTER is used to
monitor functional listening. Tharpe & Bess
(1999)10 outlined some of the advantages of
soundfield FM: (1) The child is not being
isolated and labelled as hearing impaired; (2)
Reasonable cost – ear level FM systems are
more expensive than soundfield systems; (3)
Normal hearing children could also benefit
from SNR improvements.
For infants and toddlers there is currently
no evidence to suggest benefit from FM
systems, but theoretically they should
benefit from an improved SNR. Guidance
49
feature
should be provided on when to use FM
with infants & toddlers, for example in the
stroller, in the car, in day care or preschool
during instruction time.7
CROS aids, Bone anchored
solutions and Transcranial
amplification
CROS aids may be considered as an option
for older children with SDD as they are more
able than younger children to monitor their
environment, change their head position,
and change settings on the device to
accommodate changes in the listening environment.3 Limited outcome data exist for
the use of bone anchored devices in children. The two studies found in the literature
had typically small sample sizes, and
reported outcomes for mostly teenage children. The data, however small, does seem to
indicate some benefit for users post-fitting
with increases in speech performance in
noise and listening abilities.11,12 Transcranial
devices like Transear™ and Transcranial
CROS work on the principle of providing
stimulation to the better cochlea through
placement on / in the poorer ear using a
BTE, ITE or CIC device. Again, these technologies are showing some promise but
currently have very limited outcome data to
support an evidence-based recommendation to parents of children with SDD. The
interested reader may refer to
www.transear.com and Valente & Oeding
(2010)13 for a more detailed review on these
devices.
Cochlear implants
Some preliminary data for cochlear
implants as a treatment option for SDD are
starting to emerge,14-16 but these are based
focus groups of
parents indicated
they want to
know as soon as
possible of any HI,
regardless of the
degree of loss
50
on adult patients and predictions about
the applicability to the paediatric population may be premature. In the UK, this
approach is not currently supported by the
NICE (National Institute of Clinical
Excellence) guidelines, which governs decision making regarding implantation, but it
is an interesting concept to watch for the
future.
No discussion of treatment options for
hearing impairment however can be
complete by simply referring to technology
alone, and must always be accompanied by
early intervention considerations, parental
counselling and other rehabilitative efforts
to ensure optimal outcomes for the HI
child and his / her family.
Early intervention
Davis, Reeve & Hind (2001)4 noted that in
focus groups, parents indicated they want
to know as soon as possible of any HI,
regardless of the degree of loss. This could
have important implications for screening
practices and equipment / algorithms to
detect mild losses and more international
surveys should be done to add data to this
issue highlighted by parents. These authors
also suggested such children be monitored
for progressive HL and that genetic counselling may also have to be part of the
management process. Davis et al. (2001)4
suggested management of mild HL in four
areas: (1) Family support, including education; (2) Ongoing assessment and review,
mainly audiological monitoring of hearing
loss; (3) Communication including the
encouragement of early communication,
speech and language development; (4)
Management choices such as amplification
and FM device usage.
McKay, Gravel & Tharpe (2008)7 recommended that if parents preferred to follow
a ‘wait & see’ approach before fitting
amplification, this time should be used to
educate them on assessing auditory behaviours, managing the acoustic environment
for optimal communication, and to stimulate and model good communication.
Referral to early intervention services
should be made and speech & language
development should be proactively monitored.
Parental counselling
A list of recommended activities and practices for informational counselling is
attached in Appendix A. A downloadable
reference document for parents on functional auditory measures is also available in
Appendix B. Another excellent resource for
parents has been developed by McKay
(2010),3 entitled Infants and Toddlers with
UHL – A Parent’s Guide, and is well worth a
read.
Tharpe, Eiten & Abbot Gabbard (2008)8
noted that children typically have large
RECDs and therefore gain predictions may
only show a few decibels. The counselling
implication is therefore a discussion on
the cost effectiveness of wearing hearing
aids. Perhaps counselling the family on
effective communication strategies
should take greater prominence. Hearing
aids as a treatment option can then again
be considered later on. Part of the counselling discussion should also incorporate
awareness training of hearing conservation and noise protection, and the benefits of binaural hearing (localisation,
binaural squelch, binaural summation,
head shadow effect). n
Appendix A
Suggested Early Intervention Strategies
for children and families with MBHL or
UHL.17
● As a minimum, family members must
receive information about MBHL or
UHL. Provide information to help
them observe their child, provide ideas
for early intervention activities, and
help them monitor their child for signs
of progressive hearing loss or bilateral
hearing loss.
● Audiologists must provide information
regarding functional auditory skills,
provide hearing aids and / or FM systems on a trial basis, help the family
●
●
with use of amplification and developing communication skills in more challenging acoustic environments.
Create or modify the acoustic environment to suit learning: reduce background noise, distance from the
teacher / speaker, reduce reverberation,
use carpets / drapes, cover feet of
chairs with felt, ensure good lighting,
keep the child’s interest by adjusting
activities, help family read infant cues
to stimulate reciprocal communicative
behaviour.
Put parents of children with MBHL /
UHL in touch with each other to share
experiences.
feature
●
●
●
●
Provide informational support to the teacher / caregivers regarding the effects of MBHL / UHL.
Provide visual information in addition to auditory
information to aid understanding. Write instructions
down. Help child to see speaker’s lips so they can
benefit from lipreading.
Make eye contact and obtain child’s attention before
speaking to him / her. Speak a little bit louder.
Maintain face-to-face contact with family / caregivers
(not just ‘phone calls / emails) to ensure the importance of the situation in perceived.
Appendix B
Incorporating functional auditory measures into paediatric practice (Tharpe & Flynn)
http://www.oticonusa.com/eprise/
main/SiteGen/Uploads/Public/
Downloads_Oticon/Pediatrics/
Inc_Functional_Measures_Guide.pdf
References
1. Ross DS, Holstrum WJ, Gaffney M, Green D, Oyler RF, Gravel JS. Hearing
screening and diagnostic evaluation of children with unilateral and mild
bilateral hearing loss. Trends Amplif 2008;12(1):27-34.
2. Lieu JEC. Unilateral hearing loss in children. Semin Hear 2010;31(4):275-89.
3. McKay S. Audiological management of children with single-sided deafness.
Semin Hear 2010;31(4):290-312.
4. Davis A, Reeve K, Hind SB. Children with mild and unilateral hearing loss.
In A Sound Foundation Through Early Amplification – Proceedings of the
Second International Conference; 2001:179-186.
5. American Academy of Audiology. Pediatric Amplification Protocol; 2003.
[http://www.audiology.org/resources/documentlibrary/Documents/peda
mp.pdf]
6. Joint Committee on Infant Hearing (JCIH) Year 2007 Position Statement:
Principles and Guidelines for Early Hearing Detection and Intervemtion
Programs.
[http://www.soundbeginnings.org/download/JCIH_Executive_Summary.p
df]
Now you can
Subscribe Securely
online.
Subscribe to
ENT& audiology
news
for 1 year
(6 issues)
7. McKay S, Gravel JS, Tharpe AM. Amplification considerations for children
with minimal or mild bilateral and unilateral hearing loss. Trends Amplif
2008;12(1):43-54.
8. Tharpe AM, Eiten L, Abbot-Gabbard S. Hearing technology. Semin Hear
2008;29(2):169-77.
9. Hawkins DB. Comparisons of speech recognition in noise by mildly tomoderately hearing impaired children using hearing aids and FM systems. J
Speech Hear Disord 1984;49(4):409-18.
10. Tharpe AM, Bess FH. Minimal progressive and fluctuating hearing losses in
children. Characteristics, identification & management. Pediatr Clin North
Am 1999;46(1):65-78.
11. Christensen L, Dornhoffer JL. Bone anchored hearing aids for unilateral
hearing loss in teenagers. Otol Neurotol 2008;29(8):1120-2.
and receive
2 issues
FREE
12. Christensen L, Richter GT, Dornhoffer JL. Update on bone anchored hearing aids in pediatric patients with profound unilateral sensorineural hearing
loss. Arch Otolaryngol Head Neck Surg 2010;136(2):175-7.
13. Valente M, Oeding K. Transcranial contralateral routing of the signal as a
fitting option for patients with single-sided deafness. Semin Hear
2010;31(4):366-77.
14. Anrdt S, Aschendorff A, Laszig R, Beck R, Schild C, Kroeger S, Ihorst G,
Wesarg T. Comparison of Pseudobinaural Hearing to Real Binaural Hearing
Rehabilitation After Cochlear Implantation in Patients With Unilateral
Deafness and Tinnitus. Otol Neurotol 2011;32(1):39-47.
15. Baguley DM. Cochlear Implants in Single-Sided Deafness and Tinnitus.
Semin Hear 2010;31(4):410-3.
16. Buechner A, Brendel M, Lesinki-schiedat A, Wenzel G, Frohne-Buechner C,
Jaeger B, Lenarz T. Cochlear Implantation in Unilateral Deaf Subjects
Associated With Ipsilateral Tinnitus. Otol Neurotol 2010;31(9):1381-5.
17. National Workshop on Mild and Unilateral Hearing Loss: Workshop
Proceedings. Breckenridge (CO). Centres for Disease Control and
Prevention (2005).
scan the QR code above for your instant link
51
Tool Development: developing
a rationale for empowering
children with hearing loss
Melanie Gregory,
Staff Audiologist.
Correspondence
Ida Institute, Egebaekvej
98, DK-2850, Naerum,
Denmark.
A
s an educational institute, we design and create processes and tools to
enhance communication between the patient and hearing care professional
and to facilitate a patient-centred approach to rehabilitation. In this article we
outline the Ida institute’s approach to innovation and tool development for professionals working with children with hearing loss and their families and describe a proto-
Declaration of
Competing Interests
None declared.
The Ida Institute is a
non-profit making
educational organisation,
funded by the Oticon
Foundation, whose
mission is to ‘foster a
better understanding of
the human dynamics
associated with hearing
loss’.
W: www.idainstitute.com
type of a new tool that we are developing to empower older children with hearing loss
within their own rehabilitation process. In January 2011 the Ida Institute held a paediatric focus group in Skodsborg, Denmark. The purpose of this group was to address the
following question: “What are the unique challenges facing professionals who work with
children with hearing loss and their families? How can existing Ida Institute tools be
adapted or improved to address these challenges?”
A co-creative process to develop
new tools
Real life
knowledge and
experience of the
field is a key
element for the
innovation
process as it is
the starting
point for
developing a
shared
understanding
of the dilemmas
experienced by
the participants
52
The focus group was designed as a cocreative laboratory, which means we bring
professionals together from around the
world to engage with each other and to
bring their expertise, knowledge, ideas and
everyday clinical experience to the innovation process. The process has three interdependent stages:
1. To develop a shared understanding of
the topic
2. To explore the challenges experienced by
hearing care professionals who work
within the area of paediatric audiology
3. To create ideas that can be transformed
into a tangible form for tools that
address these dilemmas.
The aim is to identify a specific clinical
dilemma or challenge and to develop ideas
into a practical tool that can be used in a clinical situation. The tool could be a board game,
a metaphor, a new process, a service on the
internet… the possibilities are limitless.
Developing the rationale for the
paediatric tool
Understanding the challenges
experienced by paediatric
audiologists
Eileen Rall, PhD described the holistic role
of the paediatric audiologists when she
said: “Audiologists not only help enhance
communication by improving access to
sound through technology, but also have a
role to play in promoting the healthy
development of the child, as hearing can
impact on the social, emotional and
cognitive development of the child. Part of
our role can be seen as helping parents to
understand the importance of healthy
attachment within the family system,
supporting parents through their feelings
associated with grief and helping parents
to understand the impact of hearing loss
on communication.”
“Some of the challenges facing paediatric
audiologists are: limited training opportunities for student audiologists within the field
of paediatrics, quality assurance is
frequently focused on protocols, rather
than the real life experience of the patient
and an ‘expert’ model does not always lead
to a satisfying experience of audiological
services for parents or their children.”
Josephine Marriage, Skodsborg, 2011.
Real life knowledge and experience of
the field is a key element for the innovation
process as it is the starting point for developing a shared understanding of the
dilemmas experienced by the participants.
Real life case examples were assigned
before the focus group, allowing group
discussions in a closed web forum and
during group sessions in the focus group.
Three specific challenges emerged:
1. The management of hearing loss
in paediatrics carries a unique
responsibility
The impact of hearing loss goes beyond
the ear and can impact on other areas of
development with a profound impact on
the family system. Therefore there is a need
feature
Figure 1: Collage of ideas.
Figure 2: A tool draft presented during role play.
to not only support the family in management of the hearing loss, but to understand how the family is integral to the
overall development of the child.
Often the technology or tools used in
paediatric audiology are adapted from
adult technologies, with little consultation
of professionals at an early stage in the
development process and therefore these
technologies do not fully address the
needs of the paediatric population.
2. Effective communication with
children and families is critical
The first appointment with a family is the
beginning of a long journey and participants
felt it was imperative to start the journey well
together. It is difficult to get a complete
picture of daily life for a child from within the
clinical setting. Participants wanted to be
able to set the scene for positive, proactive
interaction despite significant appointment
time constraints. Their goal was to enable
the child to express his / her own needs and
to participate in decision making.
3. Managing hearing loss is a
dynamic process
There are challenges in understanding the
changing circumstances of the family and
different cultural backgrounds, alongside the
current needs of children with hearing loss
through different developmental stages.
They wanted to be better equipped to see
the whole child within his or her own
personal context.
the dilemmas was developed.
Transforming ideas into tools
Participants presented their new tool
prototype using role play, which was
filmed so that the rest of the group could
review and evaluate each tool prototype.
The tool drafts that were presented
displayed some common themes and
patterns. Some of the patterns included
the use of pictures to represent and
describe the individual daily routines of
children with figures of family members
and friends and objects that could be used
by the child.
Reflections from the tool draft
presentations
We interviewed each group after the role
play presentation so that they could
describe what happened when they used
the tool. These are some of the reflections
about the new tool drafts from participants:
“By using open-ended questions and the
situations, we became more child-focused.
The appointment is more focused on the
child’s own routine and daily activities.”
“The audiologist spent more time
listening”
“There was a balanced three-way
dialogue between the audiologist, child
and parent”
“The child had a voice”
“The parent, child and audiologist
problem solve together”
Figure 3: The My World Tool prototype.
Exploring the challenges and
possible new solutions
During this phase live theatre and role play
were used based on clinical cases to further
explore the dilemmas and to enact some
clinical scenarios to explore the use of new
or different approaches to those they
might usually employ. Through reflection
on ethnographic videos and discussion of
case examples, a deeper understanding of
“It felt safe and empowering to manipulate the figures and objects”
“I can concretely show what happens to
me in daily life”
A new tool prototype
Through a combination of the design
elements, a new clinical tool emerged and
was subsequently developed into an applicable prototype named ‘My World’.
It is a booklet with schematic room
drawings in which the child manipulates
items relevant to different scenarios and
shows the challenges or positive events
linked to the situations of his / her real
world experience. The My World framework allows the child to express himself /
herself in pictures of daily events,
including challenges and successes. My
World is still in the development phase,
and will be completed in cooperation
with audiologists in the UK, Australia and
the USA.
Conclusion
From the point of view of the participants,
the rationale for developing the tool in this
format was to facilitate a child-centred
dialogue reflecting the child’s own experience and so focus on important hearing and
communication issues for the child at that
time.
The tool prototypes aimed to engage the
child as an active participant giving him or
her an equal voice with the parent and the
audiologist.
The tool gave a window for the parent
and audiologist to understand the child’s
experience of daily life.
Each situation described by the child
became an opportunity to understand the
communication, social interaction and
emotional response to that situation for
the child. In addition, the description
provided the possibility to review effective
communication strategies and to problemsolve together. n
53
features
European Society of Paediatric Otolaryngology
May/June 2009
Paediatric Audiology at Alder Hey Children’s NHS Foundation Trust
Sept/Oct 2009
Jan/Feb 2010
Bone Anchored Hearing Aids in Children
Mar/Apr 2010
Epidemiology of Paediatric Sleep Disordered Breathing
Jul/Aug 2010
The European Society of
Pediatric Otolaryngology
Mr John Graham,
Consultant
Otolaryngologist.
Correspondence
Mr John Graham,
Royal National Throat
Nose and Ear Hospital,
Gray's Inn Road,
London
WC1X 8DA, UK.
E: john.graham10@
virgin.net
Declaration of
Competing Interests
None declared.
Acknowledgements
I am very grateful to
Carel Verwoerd and Chris
Raine for input to this
article and to Chris for
the photograph of the
ESPO Board.
Evolution of ESPO 1968-2010
ESPO was born in 1968, when Carlo Gatti
Manacini, Renato Fior and Guilio
Pestelozza, three ENT surgeons in the
north of Italy, admired the existence of
paediatric ENT as a separate subspecialty
in Poland and other Eastern Bloc nations
and decided to put their own primary
interest
in
paediatric
otolaryngology on a
more formal footing.
They organised a
meeting of likeminded paediatric
otolaryngologists
in Sirmione, home of
the Latin poet Catullus,
on Lake Garda. The
outcome of this meeting was that
the European Working Group in Pediatric
Otorhinolaryngology was formed. This fairly
informal group grew and recruited
members from other European countries,
including the United Kingdom, where
Robert Pracy in Liverpool and John Evans
at Great Ormond Street were already
established in the field of paediatric ENT.
The First International Congress of
Pediatric ENT was organised by the
working group, again in Sirmione, in April
ESPO fosters strong links between British
otolaryngologists and our colleagues on the
continent and between specialists in
‘Western’ Europe and our colleagues in the
previous Eastern Bloc, who have
contributed greatly to ESPO throughout its
life and are now well placed to take a
leading role in its future
1977 and attracted 400 delegates. John
Evans volunteered to host the next
meeting in Bath, UK in 1981 and many of
us who had recently been appointed to
consultant jobs in the UK checked in,
greedy for advice from experts like Sylvan
Stool, Robin Cotton, John Evans and
others on how to do a safe tracheotomy
in a neonate weighing 1,200
grams and how to
correct the laryngeal
stenosis produced
as a complication
of the new lifesaving method of
intubating
and
ventilating premature
infants; as well as the new
methods of objective hearing tests
in infants. Cochlear implants were still
largely in the future, but we were aware of
their potential.
Subsequently the main rôle of the
European Working group was to hold four,
then two yearly meetings, to introduce
colleagues working in this expanding field
to each other and encourage and present
research, and to expose younger surgeons
to teaching from experienced colleagues.
The attendance at these meetings grew
from the original 400 in Sirmione, and a
more formal organisation, ESPO, was
founded during the meeting hosted in
Rotterdam, the Netherlands by Carel and
Jetty Verwoerd in 1994. At subsequent
congresses in Helsinki (1998), Oxford (2002)
and Paris (2006) as many as 1,200 delegates
registered. Prof Robert Ruben, from New
York, attended the first Sirmione meeting in
1977 and two years later founded the
International
Journal
of
Pediatric
Otorhinolaryngology; Bob Ruben has
remained a strong supporter of ESPO and
always encourages authors to publish the
papers they have read at ESPO meetings.
Structure and functions of ESPO
ESPO is run by its board, consisting of past
(me), present (Noel Garabedian) and
future (Javier Cervera) presidents, a General
Secretary (Carel Verwoerd), the Treasurer
(Chris Raine, who else?) and the chair of
the Members committee (Jetty VerwoerdVerhoef). ESPO Council has representatives
from 23 European countries. Peter Robb
55
feature
and David Albert are the current UK reps.
The national societies of paediatric ENT in
Europe: Dutch-Flemish, Hungarian, Italian,
Polish, Spanish, Greek, French and British
(BAPO) are all associated with ESPO. The
next president after Javier will be Anne
Schilder from Utrecht, who will organise
the 2012 congress in Amsterdam, the
Netherlands. We also have liaison delegates
from the US (ASPO), South America
(IAPO) and India (APOI). The Japanese
society (JSPO) has also contributed to
meetings. Noel Garabedian is our current
liaison link with ASPO.
Carel Verwoerd, from Rotterdam, took
over the job of General Secretary of ESPO
in 2001 from the founding General
Secretary, Renato Fior and acts as a point
of liaison with other organisations. Javier
Cervera, from Madrid will be the next
President and he and Manuel Manrique
will host the next meeting in Pamplona in
Spain: June 5th-8th, 2010 (please note this
in your diary NOW). We visited Pamplona
and found it an excellent city to spend a
few days; friendly and small enough to
allow exploration on foot. Good range of
hotels and excellent food and drink. The
new conference centre seems the right size
for an ESPO meeting. The St Firmin festival,
when bulls are let loose in the streets of
Pamplona, is held a month after the
congress, in July, perhaps just as well. The
congress website can be found at:
www.espopamplona2010.com.
What of the current role of ESPO?
I’m glad to say that ESPO does not have
anything as tiresome as a Mission
Statement, however it does have clear
objectives, which are set out in the website:
to disseminate knowledge of ENT disorders in children and their treatment,
● to
encourage co-operation across
national boundaries and establish links
between organisations involved with
the care of children with ENT disorders,
● to promote clinical and basic research.
Although ESPO does not have funds to
pay for research, it can put researchers in
touch with others in similar fields in
other countries,
● to hold regular scientific congresses,
● to promote training programmes and
help define standards for professional
practice in Paediatric ENT.
The ESPO website www.espo.eu.com has
more details of the Society’s basic structure and functions, and the membership
form.
●
Why join ESPO?
• ESPO members normally join because
they have a clear interest in paediatric
ENT. Many are members of their
national paediatric otolaryngology societies. In fact the UK has the highest
number of members of any country.
This is partly because of the success of
BAPO, partly due to effective recruiting
by Chris Raine and partly because of:
• £££ / €€€ You get a discount on the
registration fee for all ESPO meetings.
You also get the International Journal of
Pediatric Otorhinolaryngology at the
significantly reduced subscription of
€120 a year.
• The membership subscription is
absurdly low, at €25 for one
year, ---50 for two years. The application form is on the ESPO website.
• You can attend, at a reduced rate (see
The ESPO Board. (L-R): John Graham, Jetty Verwoerd-Verhoef, Chris Raine, Noel Garabedian (President),
Carel Verwoerd, Javier Cervera.
previous column) an ESPO congress
every two years, meet old friends and
update yourself from experts in the
rapidly advancing fields of paediatric
ENT.
What’s the use of attending conferences?
Some hospital managers certainly doubt
the value of ‘jaunts for doctors’. One of our
own dear managers provided the view that
she didn’t “see the point of people being
paid to have a holiday”, before disappearing out of contact on a series of totally
essential management away days…
My view is that medical conferences
remain extremely valuable, even when
they take us away from the important
business of removing myriad tonsils to
boost hospital income from the UK’s
primary care trusts. Web searches, books
(even Pediatric ENT: edited by Graham,
Scadding and Bull; now in paperback –
order your copy today!), emails and telecongresses cannot really replace live
presentations that include time for indepth questions and discussion and total
immersion in a topic, with unlimited time
for sharing problems and setting up lifelong professional links that a three day
meeting provides. Our own paediatric
ENT association, BAPO, was founded as a
spin-off from the Ghent ESPO meeting in
1990 when Peter Bull and I locked all the
British delegates into a lecture room, only
letting them out after we had all agreed to
found our own society; the final details
being sorted out during lively discussions
with David Proops in the bar of the crosschannel ferry going home.
Conclusion
ESPO is a lively organisation with excellent
links across all European countries. In
particular it fosters strong links between
British otolaryngologists and our
colleagues on the continent and between
specialists in ‘Western’ Europe and our
colleagues in the previous Eastern Bloc,
who have contributed greatly to ESPO
throughout its life and are now well
placed to take a leading role in its future.
ESPO is also highly respected and well
placed to influence training and the
organisation of services in paediatric ENT
in Europe. Join now! n
FURTHER INFORMATION
Website: www.espo.eu.com
www.espopamplona2010.com
56
feature
Paediatric Audiology at Alder Hey Children's
NHS Foundation Trust
Apart from the medical staff, the department is backed up by
non-medical audiologists. The full spectrum of audiological
services is offered here. Since I have been in this department, I have
been involved in testing and managing the hearing of all age
groups of children including those who have severe learning
disabilities, syndromes, and neurological difficulties. As a Staff
Grade in a busy teaching hospital, I have made a significant contribution to providing a comprehensive range of out-patient services
for the children. My experience has allowed me to contribute to
the development and improvement of the Audiology Service in
the region and I have set up a peripheral clinic at May Logan in
South Sefton which I run independently. I have one clinic per
month for fast-track assessment of children hearing after meningitis and I am the key person to see the children referred to audiology for targeted follow-up from Newborn Hearing Screening. I
also share one clinic per week with an ENT Consultant to assess
the children who have hearing problems and need surgical intervention. In addition, I have made a considerable contribution to
the teaching programme of the medical and audiological
students. n
Dr Ezeddin El Tabal,
Staff Grade in Paediatric Audiology,
Alder Hey Children's Hospital,
Merseyside, UK.
reprints available
>
>
>
>
Peer reviewed
ABPI compliant
Translations available
Multiple country coordination
For more information and quotes, contact
[email protected]
matters
57
Laura
Coleman, MRCP,
Specialist Registrar in
Radiology
M
ultidisciplinary co-operation in the assessment of the paediatric airway is
essential to the safe management of infants and children with respiratory
compromise. This article is not intended to be a comprehensive atlas of
airway anomalies but seeks to illustrate some of the situations where high quality
imaging is central to diagnosis and management.
General considerations in
radiology
Helen Williams,
FRCR,
Radiologist
Kate Parkes,
Radiology Clinical
Systems Manager
Michael Kuo, FRCS,
Children’s Ear, Nose and
Throat Surgeon
Correspondence
Michael Kuo,
Children’s Ear,
Nose and Throat
Surgeon,
Birmingham
Steelhouse Lane,
Birmingham
B4 6NH, UK.
Declaration of
Competing Interests
None declared.
58
Many aspects of current imaging, such as
Computed Tomography (CT) and
Magnetic Resonance Imaging (MRI),
require a degree of patient co-operation
and control in order to obtain adequate
images. However, with a young child, especially those with developmental delay, this
may not be possible. Therefore sedation
and general anaesthesia have to be considered. Sedation can produce excellent
results, avoiding invasive procedures, but
requires careful monitoring at all times
(respiratory rate, oxygen saturation). It
poses the risk of destabilising the airway,
increasing the risk of aspiration and causing
respiratory compromise. Therefore only
suitable candidates must be chosen and
resuscitation equipment needs to be close
by. Multidetector spiral CT is often used in
paediatric imaging due to its faster acquisition times, reduction in ionising radiation
exposure and ability to produce better 3D
image reconstructions. Faster scanning
helps to reduce movement artefact which
is of particular benefit with a paediatric
population who can't necessarily follow
instructions and co-operate with, for
example, breath-holding.
If there is any risk of destabilising the
airway in a child, especially one with known
or suspected airway abnormality, then
general anaesthesia is by far the safest
option. It is important to remember that
neonates and young infants are obligate
nasal breathers, so any form of nasal airway
obstruction can lead to acute respiratory
compromise. Airway control aided by
general anaesthesia is best considered on a
planned basis rather than as an emergency
procedure in the CT or MRI suite. It should
be carried out and closely supervised by a
fully trained paediatric anaesthetist. Care
must be taken to use MRI compatible
equipment when necessary.
Foetal imaging
Rapid improvements in ultrasound technology, particularly in improved image
resolution, have facilitated the diagnosis of
more complex disorders . Of particular relevance to the airway are the diagnoses of
neck / thoracic masses causing compression of the airway and disease entities such
as laryngeal atresia, both needing immediate intervention at birth. Apart from
ultrasound visualisation of mass lesions,
polyhydramnios picked up on foetal ultrasound may be an indirect indicator of
possible foetal airway abnormalities,
prompting further investigation.
Where a mass lesion is detected in the
neck, foetal MRI can be used to further
define the mass, contributing to the differential diagnosis as well as defining its relationship to the airway (Figure 1). This aids
the obstetrician and the paediatric airway
surgeon in counselling the prospective
parents particularly with respect to airway
Figure 1: Sagittal T2 weighted foetal MRI image
showing mass in the anterior neck.
feature
Figure 2A: Axial CT scan showing congenital
piriform aperture stenosis.
Figure 2B: Coronal CT scan of same patient
showing single incisor associated with
congenital piriform aperture stenosis.
management during delivery and the
appropriateness of an ex-utero intrapartum therapy (ExIT) procedure.
Neonates are obligate nasal breathers for
the first few months of life and significant
nasal obstruction results in respiratory
distress, apnoea and cyanosis. Less acutely,
it may manifest itself as difficulty feeding
and a failure to thrive. The paediatric
otolaryngologist may be asked to review a
child in whom it has been impossible to
pass a nasogastric tube down one nostril to
assess for unilateral choanal atresia. The
availability of fine neonatal endoscopes
(1.8mm) allows endoscopic examination,
but CT remains the modality of choice for
defining bony anatomy and is able to highlight the absence of nasal bones as well as
the presence and thickness of bony atresia
plates. Three-dimensional CT imaging can
be used for planning nasal construction /
reconstruction surgery in those paediatric
patients who are developmentally normal
or near normal.
Multiplanar CT or MRI is used to assess
the intracranial contents in order to aid
prognosis. MRI provides more detailed
neuroanatomy and is able to confirm the
presence or absence of the olfactory nerves.
Congenital anterior piriform aperture
stenosis / central midline incisor syndrome
arises from overgrowth of the nasal process
of the maxilla, narrowing the nasal piriform
aperture and is an uncommon cause of
upper airway obstruction in the neonate.
Clinically, there is a narrowing of the anterior choana. CT demonstrates a narrowed
anterior nasal cavity: piriform aperture
<8mm diameter including nasal septum
and single anterior nare width <2mm
(Figures 2A and 2B). Features which may
also be present include a hypoplastic triangular-shaped palate, bony overgrowth of
the maxillary nasal process, incisor abnormalities – a single central midline incisor or
partial central incisor fusion. It is important
to image the intracranial contents of these
patients due to the risk of other midline
abnormalities. An MRI head scan in these
patients will identify the presence or
absence of associated intracranial midline
abnormalities including holoprosencephaly, posterior pituitary ectopia, medial
deviation of the cavernous carotids and
Chiari I malformation.
Posterior choanal stenosis / atresia is
caused by defective development of the
oronasal membrane at the level of the
Figure 3A: Bilateral mixed bony-membranous
choanal atresia.
Figure 3B: These images show coronal / axial images
of unilateral left bony choanal atresia.
Nasal airway pathology of infancy
choanae.
Computed
posterior
Tomography is the imaging modality of
choice in determining whether there is
choanal atresia or just stenosis, whether it is
unilateral or bilateral and for surgical preparation, whether an atresia is membranous,
bony or mixed. Sedation is contraindicated
in infants with bilateral disease and airway
intubation under general anaesthesia is
essential. The patient needs to have nasal
suction and instillation of vasocontrictive
nasal drops pre-scan. High resolution axial
scans with multiplanar reconstruction are
obtained. Axial and coronal views help to
demonstrate the presence or absence of a
complete bony bridge. Computed
Tomography features of posterior choanal
stenosis / atresia include a narrowed or
funnel shaped posterior nasal cavity,
medial deviation of the posterior maxilla,
lateral deviation and thickening or splitting
of the posterior vomer, fusion or bridging
of the vomer to the maxilla, complete or
partial obstruction of the posterior
choanae by a bony bridge or soft tissue
membrane (choana <3.5mm diameter), airfluid level within the obstructed nasal
cavity, elevation of the ipsilateral hard
palate and hypoplasia of the inferior
turbinates. Observation of these features
prepares the surgeon for the operative
approach.
Where choanal atresia is diagnosed, high
resolution CT imaging of the petrous bone
should be performed at the same time, if
possible, especially in infants with bilateral
disease due to the high incidence of
CHARGE syndrome in this population
(coloboma, heart defects, atresia choanae,
retarded growth / development, genital
hypoplasia, ear anomalies)(Figures 3A and
3B).
Radiology and the assessment of
stridor in children
The majority of children presenting with
stridor will require direct visualisation of
59
feature
Figure 4: Plain radiograph showing the ‘steeple’
sign in acute laryngotracheobronchitis.
Figure 5: Inspiratory and expiratory chest radiographs showing air-trapping in the left lung on
expiratory film.
the airway with either flexible or rigid
endoscopy. However, radiology provides a
useful adjunct to this and can give invaluable additional information during both
diagnosis and follow-up which aids the
appropriate management of the patient.
the glottic and subglottic regions, dilatation of the piriform sinuses and
ballooning of the pharynx (Figure 4).
Plain films of the neck can show radioopaque inhaled foreign bodies. When
there is clinical suspicion of an inhaled
foreign body, inspiratory and expiratory
chest radiographs should be obtained
and may help to assess for indirect signs
of a foreign body in the airway such as airtrapping or lung collapse / consolidation
(Figure 5).
Plain radiography
Frontal (anteroposterior) and lateral views
of the neck help to demonstrate airway
calibre and outline important anatomical
landmarks. However, they can be
misleading as small variations in patient
position can dramatically alter the appearances on the film and radiographs are not
performed as a routine in clinical practice.
The high-kV ‘Cincinnati’ view is not used as
frequently today as in the past due to the
improvement in image quality obtained
with modern-day computed radiography.
Plain radiographs are helpful in demonstrating adenoidal hypertrophy and the
effect on the nasopharyngeal airway in
obstructive sleep apnoea. The upper limit of
normal adenoid size is 12mm AP diameter.
Enlarged palatine tonsils may also be seen.
Plain film imaging of the child with acute
stridor has fallen out of favour because it is
considered dangerous, especially out of
hours, to take a child with a marginal
airway away from the resuscitation room
and into an environment which the child
may find intimidiating. The diagnosis of the
child with acute stridor is primarily a clinical one. Acute epiglottitis in children has
largely been eradicated in this country
courtesy of the HIB vaccination
programme but it remains a cause of
stridor and odynophagia in adults. Acute
epiglottitis is primarily a clinical diagnosis
and requires prompt treatment. Plain radiography can aid the clinical diagnosis of
acute laryngo-tracheo-bronchitis / ‘croup’
demonstrating the characteristic ‘steeple’,
‘pencil tip’ or ‘wine-bottle’ sign caused by
narrowing of the subglottic airway on
frontal views. Additional features include
loss of soft tissue distinction in the glottic
region on lateral films, ill-defined haziness
at the soft tissue – air interfaces between
60
movements of the vocal cords can be
assessed during deep inspiration and
vibration movements during phonation.
The resting position of the vocal folds can
also be seen (Figure 6).
Ultrasonography can also contribute
to the ‘work-up’ of babies with a laryngeal
web, subglottic haemangioma and
congenital subglottic stenosis, but
endoscopy remains the principal tool for
investigation of these conditions.
Fluoroscopy
Ultrasound of the neck
Ultrasound of the neck is useful in
assessing cystic and solid masses which
may be causing extrinsic compression on
the airway. It is also of particular use in
imaging the larynx in children, which can
be inadequately assessed using CT or MRI
due to poor soft tissue contrast. It
provides a non-invasive and dynamic
mode of imaging without radiation exposure. More recently, laryngeal ultrasound
has been used to monitor infants with
vocal fold paralysis. Abduction-adduction
Contrast swallow is a useful dynamic
study but it involves ionising radiation. It
can aid in the diagnosis of aspiration,
laryngeal cleft, tracheomalacia and
tracheo-oesophageal fistula. While pH
studies are the investigation of choice for
the definitive diagnosis of gastrooesophageal reflux, contrast swallow
examinations can demonstrate reflux into
the oesophagus if it occurs during the
study although in itself, the contrast
swallow is a poor test for detecting reflux.
Extrinsic compression from vascular
Figure 6: Laryngeal ultrasound of a child with a right vocal fold paralysis. The right vocal fold is abducted.
feature
Figure 8: Three-dimensional reconstruction of CT thorax
demonstrating tracheal stenosis.
All children with
significant stridor
should undergo cross
sectional imaging
with CT or MRI
under general
Figure 7: Lateral contrast swallow view
showing indentation in oesophagus created
by an aberrant subclavian artery.
Figure 9: Three-dimensional MIP (maximum
intensity projection)
post-contrast MRI volume image of the thorax
showing double aortic arch.
anaesthesia
structures can also be appreciated
although further imaging, usually with
MRI, is required (Figure 7).
Cross-sectional imaging
Children with significant stridor requiring
cross-sectional imaging with CT or MRI
should be imaged under general anaesthesia. As an adjunct to airway endoscopy,
CT is particularly useful where a tight
stenosis prevents safe endoscopic examination distal to the stenosis. In these situations, multidetector CT, especially with
3D reconstruction can define both the
luminal diameter as well as the length of
the stenosis. Computed Tomography is
also beneficial in the further assessment of
extra-luminal and thoracic lesions and
tumour staging. It is fast and readily accessible but has its limitations due to ionising
radiation and requirement for iv contrast
which may be contraindicated in certain
patients. In the future, spiral CT under
controlled ventilation may be used to
acquire virtual endoscopy images of the
airway. This may help to replace the more
invasive diagnostic methods but the additional ionising radiation exposure needs to
be considered. At present, this remains a
research tool in development (Figure 8).
MRI is able to easily demonstrate
anatomy in the neck and mediastinum in
any plane without exposure to ionising
radiation or necessarily to contrast media.
However, image quality is affected by
movement, such as breathing or pulsation
artefact from vascular structures so alterations in technique such as respiratory
triggered scans are often required. MRI
provides detailed imaging of soft tissue
structures and vascular anatomy. It has
now become accepted as the single most
valuable modality for assessing airway
compression, accurately and non-invasively demonstrating the level, severity and
cause of airway compression (Figure 9).
Summary
High quality imaging is vital to complement endoscopy in the assessment of the
infant with a compromised airway. Safe
imaging in the presence of airway
compromise requires expertise from radiographers and radiologists but also good
communication between the airway
surgeon and the radiologist to ensure that
the optimal modality is employed to give
the necessary clinical information with
minimal risk. n
FURTHER READING
King SJ, Boothroyd AE: Pediatric ENT Radiology. Berlin, Germany: Springer-Verlag;
2003.
Johnson K, Williams H, Foster K, Miller C. Paediatric Radiology. Oxford Specialist
Handbook in Paediatrics. Oxford, UK: Oxford University Press; 2009.
61
Bone Anchored Hearing Aids
in Children
Patrick Sheehan,
BCh, MPhil,
FRCS(ORL-HNS),
Otolaryngologist.
oday, the Bone Anchored Hearing Aid (BAHA) is a well described and accepted form of
T
auditory rehabilitation. In the adult population, the technique is a relatively uncomplicated, and is usually performed as a one-stage procedure under local anaesthesia with
few complications.
Ann-Louise
McDermott,
FDS, RCS, PhD,
FRCS(ORL-HNS),
Otolaryngologist.
Correspondence
Mr Patrick Sheehan,
Otolaryngologist,
Royal Manchester
Oxford Road,
Manchester,
M13 9WL, UK.
E: Patrick.Sheehan@
manchester.ac.uk
Declaration of
Competing Interests
Patrick Sheehan has
been reimbursed by
Cochlear Ltd, the
manufacturer of Baha,
for attending several
conferences.
In the paediatric population, however, it is not so
simple. Firstly, general anaesthesia is preferred
and two-staged surgery is generally necessary. As
many children who need a BAHA often have a
significant medical history and / or are
syndromic, the procedure may carry more risk.
Secondly, there are issues of soft tissue and skull
thickness, which vary greatly in young children
and may significantly affect the surgical procedure itself as well as any subsequent complication rates.
The aims of the BAHA in the paediatric population is to restore hearing, maximise auditory
pathways and improve speech and language
development as soon as possible in order for the
child to achieve his / her full potential.
Indications
In the early days of BAHA surgery, the indications
were predominantly for chronic suppurative
otitis media. Now BAHA is the hearing aid of
choice for hearing loss associated with congenital ear deformities. The recent role of BAHA in
unilateral hearing loss has further expanded the
indications. Indication for paediatric BAHA are:
1.
2.
3.
4.
5.
6.
7.
8.
9.
Congenital conductive hearing loss,
Congenital aural atresia,
Congenital microtia,
Chronic suppurative otitis media,
Persistent otitis media with effusion,
Chronic otitis externa,
Unilateral profound hearing loss,
Failure with conventional aids,
Trauma to external ear.
Congenital conductive hearing loss is usually
associated with syndromal children such as
Treacher Collins syndrome, Goldenhaar’s
syndrome and Oto-renal syndrome. Children
with these syndromes have benefited from
BAHA's.
The use of BAHA in adults with single sided
deafness (SSD) has initially been very positive
giving the patient a perceived improvement in
directionality of sound particularly in noise situations or background noise. Small studies on
older children with SSD have supported the
62
adult results,1 however, studies on children with
unilateral congenital ears showed that many of
these children have excellent directional hearing
and objective studies on speech in noise showed
no significant improvement after BAHA fitting.2
Overall, there is no convincing evidence for early
intervention in those children with unilateral
congenital ears.3 However it is good clinical practice to involve the child’s carers in the decision
process when considering aiding the unilaterally
deafened child.
The child
A large proportion of children with congenital
ear abnormalities have associated syndromes
and complex medical conditions. This may in
turn make the surgical process more difficult.
These children require two general anaesthetics
and many of them may pose intubation difficulties. These children should ideally be treated in
specialised childrens’ centres where there are
dedicated multidisciplinary teams.4
BAHA has been shown to be very successful
in children with Down syndrome and also in
children with severe learning difficulties who find
conventional hearing aids difficult to tolerate
(see article on page 55).
The Baha® Softband
Currently, the Baha® Softband is the most
appropriate means to provide aural rehabilitation to very young children. The use of the
BAHA on a test band is now the gold standard
for audiological preoperative assessment in
children.
In those children with conductive hearing loss
in whom a BAHA is being considered for aural
rehabilitation a four week trial of a Baha®
Softband will allow the child to experience the
hearing benefits of BAHA. This trial leads to
better informed decision making between child,
parents and audiologists.
Timing of surgery
Another reason to use the Baha® Softband in
younger children is the higher rate of fixture loss.
Fixture failure rates of children below the age of
five years and those 5-10 years are 40 and 8%
feature
Table 1: Overview of paediatric bone anchored hearing aid surgical outcomes.
Year
2009
2008
2008
2007
Number of children
182
93
39
40
2007
2005
2002
2001
71
41
51
19
Age years (mean)
6.8
9.3
3.3
3.2
7.6
9.6
8.4
7.6
11.2
Skin complications %
19
9.4
44
NA
NA
37
2.4
3.8
16
Revision soft tissue. Surgery per patient
7.7
10.9
14.5
7.5
Fixture loss %
14
16.3
40
17.5
25.9
17.1
NA
10.5
25
9.1
21.5
5.3
Adapted with permission from a table in De Wolf et al. 2008.6
respectively. Those children over the age of
10 years have 1% fixture loss.4 The child’s
activities, medical history and social
settings must all be considered.
There is no fixed age for BAHA surgery
but the older the child, the thicker the
calvarial bone, the longer the fixture that
can be placed and the better the longterm
results are. The use of the Baha® Softband
until age four years is preferred. There are
studies reporting successful implantation
in under three year olds but other factors
such as prolonged osseointegration times
are practised.5
The site of fixture placement should be
in bone that is of sufficient thickness at the
time of surgery, to support a fixture of
3mm or more. Thus to obtain the best
results, surgery is recommended after the
age of four years.
One or two stage surgery
Two-stage surgery is standard practice for
children. This allows for better osseointegration period in the water-rich / mineral
deprived bone of children and also allows
for ease of surgery and the osseointegration period requires no aftercare / maintenance for child or carer.
Surgical technique
Various soft tissue reduction techniques
have been described in children. The surgical
principles are to use either a free or pedicled
split skin graft to produce an area of skin
surrounding the abutment that is hairless
and tightly bound to the periosteum,
encroaching on neither the abutment, nor
the sound processor. The introduction of
the dermatome to produce suitably thin
skin around the abutment has shown to give
reproducible results. The linear incision technique recently described by the Nijmegen
team6 which does not require a skin graft but
just undermines the surrounding skin is
reported to have fewer complications.
The ideal time for soft tissue reduction
and the skin graft is following the planned
period of osseointegration. At this second
stage of BAHA surgery, the fixtures are
uncovered after all the soft tissue work is
completed and the abutment is attached
to the osseointegrated fixture. The child
can attached the BAHA processor about
one week after the second stage surgery.
The position of the BAHA is important
in the child with congenital atresia if autologous reconstruction is to be considered at
a later stage. More posterior placement of
the fixture is recommended to avoid
compromising the healthy tissues at the
site of the future reconstructive surgery.7
Another point of interest is the use of a
‘sleeper’ fixture. This is usually an ipsilateral
fixture close to the primary fixture. All children under the age of 10 years should
ideally have a sleeper fixture due to the
increased rates of fixture failure and
trauma.
Recent suggestions have been to place
the sleeper fixture on the contra lateral side
in potential bilateral cases, thus reducing
the overall costs and allowing the option of
bilateral BAHA use.8
Fixture size
It has been suggested that 2mm of cortical
bone thickness is necessary for implantation for BAHA. There is no consensus of
the size of fixture that is used in children.
Very often the dura is exposed when using
the guide drill. Some surgeons described
the placement of a 3mm fixture flush with
the outer bone table (dura depressed by
the fixture) then a longer period of
osseointegration time before Stage II.
Other surgeons routinely use a 4mm
fixture even if calvarial bone is very thin. No
countersink is created and the fixture is
either left proud of the outer bone table
and bone dust augmentation used or a
very gentle displacement of the dura is
carried out by placing the fixture at low
torques of 20Nm2 and in some cases by
hand without the use of the handpiece.
Most centres practise a three to four
month period between surgeries to allow
for osseointegration unless there were difficulties encountered at the first procedure
such as inadequate bone thickness in
which cases the intervening time is
increased to six months.
Complications
It is well established that BAHA surgery in
children is associated with higher complication rates.4-6 These will include:
Failure of osseointegration
There is a significantly higher rate of fixture
failure particularly in the very young.6 Most
authors agree that the majority of fixture
failures occur in the first year following
surgery.4,6
Soft tissue complications
Increased soft tissue complications are well
recognised in children4,6 and are a significant problem for centres dealing with
paediatric BAHA. Children rely greatly on
their carers for the maintenance and
hygiene of the soft tissues around the abutment.
Many of these children have a significant
medical condition, some of which are associated with underlying behavioural and
developmental delay. This would make
maintenance of their BAHA more challenging. Furthermore, as the child grows
the fixture and abutment may become
buried by new cortical bone and for some
children there is marked hypertrophy of
the skin surrounding the abutment
requiring skin reduction. Therefore the
aftercare of children with BAHA may be
longterm.
Table 1 illustrates an overall summary of
reported surgical outcomes from
Paediatric BAHA centres.
Trauma
Finally, trauma is a challenge to any paediatric BAHA team: Damage to the device
and / or the abutment appears to be not
63
feature
uncommon. Clinicians involved in paediatric BAHA provision must be aware that
unusual and unexpected complications
can occur long after the surgery is
complete and this highlights the importance of a multidisciplinary team in the
longterm management of these children.
Quality of life
A great deal of research has been undertaken by the majority of paediatric BAHA
centres into establishing whether the
provision of a BAHA improves the quality
of life of the child.3,9
The overwhelming consensus from all
published literature is that there is a significant improvement in quality of life
reported by the children / carers despite
the associated increased morbidity in the
younger age groups.
The measure of success of a BAHA in a
child should be reflected by the number of
hours / days per week that the child wears
his / her BAHA. The recent literature clearly
reflects success rates of 96% and above in
all the larger paediatric centre.4,9
Conclusion
References
The bone anchored hearing aid in children
is an effective means of aural rehabilitation
in children with conductive hearing loss
who otherwise cannot benefit from
conventional hearing aids. The main indication remains in those children with
chronic ear disease that require hearing
amplification. Their role in the child with
congenital atresia is widely supported by
many units around the world. The BAHA
system has been shown to improve the
overall quality of life of the child. Although,
it is a well tolerated and relatively ‘simple’
procedure it is not without risk. A multidisciplinary team approach combined with
careful patient selection, the counselling of
the child and carer and an understanding
of the problems that present in children
should result in a successful outcome for
the child. Although various surgical techniques are described the predominately
important consideration is attention to
detail in the surgery. This management
approach consistently gives a good audiological and quality of life outcome as has
been reported in the literature. n
1. Christensen L, Dornhoffer JL. Bone anchored hearing
aids for unilateral hearing loss in teenagers. Otol
Neurotol 2008;29:1120-2.
2. Kunst SJ, Leijendeckers JM, Mylanus EA. BAHA system application for unilateral congenital conductive
hearing impairment: audiometric results. Otol
Neurotol 2008;29:2-7.
3. Snik AFM, Leijendeckers J, Hol MKS. The bone
anchored hearing aid for children: recent developments. Int J Audiol 2008;47:554-9.
4. McDermott A-L, Williams J, Kuo MJ, Reid AP, Proops
DW. The Birmingham Paediatric Bone Anchored
Hearing programme: a 15 year experience. Otol
Neurotol 2009;30:173-83.
5. Davids T. Bone anchored hearing aids in infants and
children younger than 5 years. Arch Otolaryngol Head
Neck Surg 2007;133:50-5.
6. de Wolf MJ, Hol MK, Huygen PL, Mylanus EA,
Cremers CW. Nijmegen results with application of a
bone anchored hearing aid in children: simplified surgical technique. Ann Otol Rhinol-Laryngol
2008;117:805-14.
7. Zeitoum H, De R, Thompson SD, Proops DW.
Osseointegrated implants in the management of
childhood ear abnormalities. J Laryngol Otol
2002;116:87-91.
8. Bernstein JM, Sheehan PZ. An approach to bilateral
bone anchored hearing aid surgery in children: contra lateral placement of sleeper fixture. J Laryngol Otol
2009;123:555-7.
9. McDermott A-L, Williams J, Kuo MJ. Quality of life in
children fitted with a bone anchored hearing aid.
Otol Neurotol 2009;30:344-9.
For a quick link to www.entandaudiologynews.com
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64
Epidemiology of Paediatric
Sleep Disordered Breathing
Michelle Wyatt,
MA, FRCS(ORL-HNS),
ENT Surgeon.
Correspondence
Michelle Wyatt, MA,
FRCS(ORL-HNS),
ENT Surgeon,
Great Ormond Street
Hospital,
London, WC1N 3JH, UK.
Declaration of
Competing Interests
None declared.
he concept of sleep-disordered
breathing (SDB) in the paediatric
population was introduced by
Guilleminault in 1976 to reflect the difference
between the well-recognised concept of
obstructive sleep apnoea seen in adults and
the broader spectrum of different issues seen
in children. SDB encompasses primary
snoring, upper airways resistance syndrome
(UARS), obstructive hypoventilation (OH)
and obstructive sleep apnoea (OSA) on a
continuum of worsening levels of respiratory
disturbance.
Classically primary snoring is noise generated by the vibration of the soft palate during
sleep. There is no associated alteration in
sleep quality. In UARS there is an increase in
negative intrathoracic pressure during inspiration with resulting arousals and sleep fragmentation. The American Thoracic Society
defines OSA as a ‘disorder of breathing during
sleep characterised by prolonged upper
airway obstruction and / or intermittent
complete obstruction (obstructive apnoea)
that disrupts normal ventilation during sleep
and normal sleep patterns’.1 Obstructive
hypoventilation is seen by this organisation as
part of OSA. Interestingly the latest
International Classification of Sleep Disorders2
uses respiratory related arousals (seen in
UARS) in the diagnosis of OSA and so UARS
itself may also soon fall under the OSA terminology umbrella. The prevalence of OSA
could therefore be underestimated as those
with UARS and OH have been excluded from
the majority of research studies to date.
Another difficulty in reporting the true
epidemiology of paediatric SDB is the fact
that different studies use different methods of
data collection and different diagnostic
criteria to define the problem. Generally the
two measures most frequently used in assessment are the Apnoea Hypopnoea Index
(AHI) and the Respiratory Disturbance Index
(RDI). The former refers to the number of
obstructive apnoeas and hypopnoeas per
hour of sleep and the latter includes these
two plus the number of respiratory related
arousals per hour of sleep. An AHI or RDI of
between 1-5 suggests a child has OSA.
T
General prevalence of SDB
Primary snoring in the UK has a quoted prevalence of 12.1%.3 A meta-analysis of 41 studies
from around the world showed a prevalence of
7.45%.4 For OSA, figures of between 0.7 and 1.8%
are quoted for Europe3,5 and 1.2% for the USA.6
Specific epidemiological factors
Gender
It has generally been felt that gender did not
affect the prevalence of SDB. However, a
number of studies have shown that male sex is
associated with an increased incidence of SDB,
with rates of between 50-100% quoted.4 These
authors suggest that failure by others to detect
a difference related to small sample sizes. The
gender difference was initially felt to be more
notable when older children were studied. It
was therefore proposed that this variance was
due to the hormonal and physiological changes
seen at puberty. However, further analysis
shows that differences are still present in
cohorts reviewed before hormonal changes
occur and so other factors must also be
involved.
Ethnic origin
SDB is known to be higher in children of AfroCaribbean origin. One study in American
adolescents showed it to be twice as likely,7 and
another looking at eight to eleven year olds
showed it to be four to six times more likely.8
This may reflect craniofacial differences and / or
upper airway lymphoid tissue size variation.
Age
There is varying evidence regarding effect of
age on the prevalence of SDB with some
studies showing no differences in SDB as the
child grows. However, an Australian review of
985 children did show a significant decrease in
snoring prevalence between the ages of four
and twelve9 and others have shown that problems become more common again in the
older teenager.10 More severe OSA and a
higher incidence of central apnoea has been
detected in those less than three years of age,
leading to the concerns regarding careful
postop monitoring in this group.11 Of note
over 40% of neonates are known to have
obstructive or mixed apnoeas, which are
more frequent between the ages of two to
seven weeks than from eight to 28 weeks.12
SDB was three to five times more likely in children of eight to eleven years who were born
prematurely.8
65
feature
Obstructing adenoids (left) viewed through a flexible endoscope. Large tonsils (right) classical of the upper
airway lymphoid hyperplasia particularly seen in children of Afro-Caribbean origin.
Obesity
The impact of increasing levels of obesity on
the numbers of those with significant SDB is
followed with great interest. Again difficulties with accurate and consistent data
collection generate their own issues. The use
of Body Mass Index (BMI), for example, can
only be in the context of age and sex specific
growth charts. Increased weight has been
shown in a number of studies to be a risk
factor for OSA.13 One study has shown that
heavier babies at two to four months old
have more issues with snoring.14 A review of
adolescents showed a 3.5 fold increase in
the risk of OSA syndrome for each standard
deviation increase in BMI Z-score.15 (The Zscore has been introduced to monitor
changes in patients with a BMI above the
99th percentile or below the 1st percentile.)
In obese children, less adenotonsillar
enlargement is required to affect sleep
quality to the same degree compared with
non-obese individuals.16 For the overweight
teenager pharyngeal adipose tissue and
decreased respiratory capacity are also felt
to be important issues. Obese children are
more likely to have persistent OSA and poor
quality of life scores after adenotonsillectomy.17
OSA in pre-school children is
often due to large tonsils and
adenoids. Note mouth
breathing is a common feature.
66
Craniofacial anomalies
Children with craniofacial anomalies have an
increased incidence of SDB. Reasons for this
are multifactorial with issues occurring at a
number of levels including the nose, post
nasal space, tongue base, pharynx or
mandible.
Abnormal polysomnography (PSG) with
evidence of OSAS was obtained in 57% of
children with Down syndrome with a figure
of 80% seen if those with an elevated arousal
index were also included.18 The contributing
factors to this are pharyngeal hypotonia,
midfacial / mandibular hypoplasia and
macroglossia / glossoptosis. Adenotonsillectomy therefore does not have the same high
incidence of effective intervention as it does
in the general population. Two studies show
persistent issues post surgery in around two
thirds of individuals.19,20
A review of 95 children with achondroplasia showed clinical evidence of
obstructive sleep apnoea in 38%, with
adenotonsillectomy being an effective intervention. However, 18% of these children
who had Ts and As required further
therapy.21 Contributing factors which lead to
OSA have been shown on radiological
studies and include significant midface
OSA in Down Syndrome occurs for a
number of reasons, including
macroglossia (illustrated here).
A two-year-old boy with Apert
syndrome with associated mid
face hypoplasia. His severe
obstructive sleep apnoea is
successfully managed with a
nasopharyngeal airway.
Baby with Pierre Robin Sequence
illustrating a small mandible. Image
provided courtesy of the Cleft Lip and
Palate Association (CLAPA):
www.clapa.com
narrowing, retrusion of the chin and
increased lower facial height.22
A small jaw, as seen in Pierre Robin
Sequence, is also associated with SDB. Thirty
one of 52 children (60%) in a Mexican study
were shown to have OSAS.23 Treatments
aimed at bypassing the obstruction at the
mandibular level have good outcomes.
Neuromuscular disorders
These children are particularly vulnerable to
SDB and its complications. OSAS is more
likely as, in addition to the more usual causes
such as adenotonsillar hypertrophy, there is
often low muscle tone in the tongue and
pharynx resulting in significant upper airway
obstruction. The neuromuscular condition
can affect the diaphragm and other respiratory muscles. This results in sleep related
hypoventilation, which along with OSAS,
can lead to an increased severity of hypoxaemia during sleep compared to those with
either condition alone. There is also an
increased incidence of central apnoea with
reduced central neural chemoresponsiveness.24 Particular care is required for these
individuals if surgery is planned.
Summary
Although there is considerable evidence as
to which population groups are affected by
SDB, there are limited data as to how the
natural history progresses in an individual.
Conclusions in the review by Lumeng and
Chervin4 suggest that:
1. Children with abnormal PSG are likely
to continue with problems for several
years if untreated,
2. A significant number of primary snorers
will see spontaneous resolution in a few
years and
3. A small number of children without
problems will commence primary
snoring as they get older.
The detrimental impact of SDB on the
general health of the paediatric population
feature
is becoming increasingly obvious as time
goes on. An understanding of those most
at risk allows focus when resources are
limited. However, consideration should
always be given to the potential diagnosis,
as evidence suggests that even primary
snoring can have neurobehavioural consequences and obstructive issues can exist in
the absence of obvious snoring. n
References
1. American Thoracic Society. Standards and indications for cardiopulmonary sleep studies in children.
Am J Respir Crit Care Med 1996;153:866-78.
2. American Academy of Sleep Medicine. International
classification of sleep disorders:diagnostic and coding
manual. Second edition. Westchester, IL: American
Academy of Sleep Medicine; 2005.
3. Ali NJ, Pitson DJ, Stradling JR. Snoring, sleep disturbance and behaviour in 4-5 year olds. Arch Dis
Child 1993;68:360-6.
4. Lumeng JC and Chung RD. Epidemiology of
Pediatric Obstructive Sleep Apnea. Proc Am Thorac
Soc 2008;5:242-52.
5. Brunetti L, Rana S, Lospalluti ML, Pietrafesa A,
Francavilla R, Fanelli M, Armenio L. Prevalence of
OSA syndrome in a cohort of 1207 children of
Southern Italy. Chest 2001;120:1930-5.
6. Bixler EO, Vgontzas AN, Lin H-M, Liao D, Calhoun
S, Vela-Bueno A et al. Sleep Disordered Breathing in
children in a general population sample: prevalence
and risk factors. Sleep 2009;32:731-6.
7. Johnson EO, Roth T. An epidemiologic study of
sleep disordered breathing symptoms among adolescents. Sleep 2006;29:1135-42.
8. Rosen CL, Larkin EK, Kirchner HL, Emancipator JL,
Bivins SF, Surovec SA, Martin RJ, Redline S.
Prevalence and risk factors for sleep-disordered
breathing in 8-11 year old children: association with
race and prematurity. J Pediatr 2003;142:377-82.
9. Zhang G, Spickett J, Rumchev K, Lee AH, Stick S.
Snoring in primary school children and domestic
environment: a Perth school based study. Respir Res
2004;5:19.
10. Cordo GM, Forastiere F, Agabiti N, Pistelli R,
Dell’Orco V, Perucci CA, Valente S. Snoring in 9 to
15 year old children: risk factors and clinical relevance. Pediatrics 2001;108:1149-54.
11. Don DM, Geller KA, Koempel JA, Ward SD. Age
specific differences in pediatric OSA. Int J Pediatr
Otorhinolaryngol 2009;73:1025-8.
12. Kato I, Franco P, Groswasser J, Kelmanson I, Togari
H, Kahn A. Frequency of obstructive and mixed
sleep apneas in 1023 infants. Sleep 2000;23:487-92.
13. Shine NP, Coates HL, Lannigan FJ. Obstructive sleep
apnoea, morbid obesity and adenotonsillar surgery:
a review of the literature. Int J Pediatric
14. Kelmanson IA. Snoring, noisy breathing in sleep
and daytime behaviour in 2-4 month old infabts.
Eur J Pediatric 2000;159:734-9.
15. Kohler MJ, Thormaehlen S, Kennedy JD, Pamula Y,
van den Heuvel CJ, Lushington K, Martin AJ.
Differences in the association between obesity and
OSA among children and adolescents. J Clin Sleep
Med 2009;5:506-11.
16. Dayyat E, Kheirandish-Gozal L, Sans Capdevila O,
Maarafeya MM, Gozal D. OSA in children:relative
contributions of body mass index and adenotonsillar hypertrophy. Chest 2009;136:137-44.
17. Mitchell RB Boss EF. Pediatric OSA in obese and
normal weight children: impact of adenotonsillectomy on quality of life and behaviour. Dev
Neuropsychol 2009;34:650-61.
18. Shott SR, Amin R, Chini B, Heubi C, Hotze S, Akers
R. Obstructive sleep apnea: should all children with
Down syndrome be tested? Arch Otolaryngol Head
Neck Surg 2006;132:432-6.
19. Merrell JA, Schott SR. OSAS in Down syndrome:
T&A versus T&A plus lateral pharyngoplasty. Int J
Pediatr Otorhinolaryngol 2007;71:1197-203.
20. Shete MM, Stocks RM, Sebelik ME, Schoumacher
RA. Effects of adenotonsillectomy on polysomnography patterns in Down syndrome children with
obstructive sleep apnea: a comparative study with
children without Down syndrome. Int J Pediatr
21. Sisk EA, Heatley DG, Borowski BJ, Leverson GE,
Pauli RM. Obstructive sleep apnea in children with
achondroplasia: surgical and anesthetic considerations. Otolaryngol Head Neck Surg 1999;120:248-54.
22. Sleep disordered breathing in children with achondroplasia. Part 2 Relationship with craniofacial and
airway morphology. Int J Pediatr Otorhinolaryngol
2006;70:453-61.
23. Bravo G, Ysuna A, Arrieta J, Pamplona MC.
Videonasopharyngoscopy is useful for identifying
children with Pierre Robin sequence and severe
obstructive sleep apnoea. Int J Pediatr
24. Alves RS, Resende MB, Skomro RP, Souza FJ, Reed
UC. Sleep and neuromuscular disorders in children.
Sleep Med Rev 2009;13:133-48.
Writing for ENT & audiology news
If you have attended a meeting or conference recently, and would
like to write for our Newsround section, please get in touch!
Meeting reports should be 250-300 words long, with the main
message of ‘what you missed if you didn’t attend’ focusing on:
•
•
•
•
Get in touch
Who were the key speakers?
What were the highlights?
Were there any controversies?
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Please email [email protected]
for conference report requirements
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67
audiology matters
A Clinical Service for Children and Adults with Suspected APD
Jan/Feb 2009
Managing Children with Auditory Processing Disorders in the Educational
Environment
Jan/Feb 2009
The Challenges Ahead in Paediatric Audiology
Mar/Apr 2009
New Developments in Hearing Aids for Children and Adults
May/June 2009
Cochlear Implantation in Early Childhood: what is happening in the longterm?
Jul/Aug 2009
New Frontiers: auditory brainstem results in adults and children
Jul/Aug 2009
An Approach to the Dysmorphic Child with Deafness
Sept/Oct 2010
A Clinical Service For
Children and Adults
With Suspected APD
Dr Doris-Eva
Bamiou, MD,
MSc(distinction)
PhD. DoH HEFCE
Senior Lecturer
(UCL Ear Institute).
Consultant in
Audiovestibular
Medicine.
H
earing impairment may have a profound effect upon an individual’s ability
to function in his personal, social, and occupational life. In the last five to six
decades, clinicians have become increasingly aware that hearing difficulties
can be caused by disordered processing of the sound signal within the brain across all
age ranges. The following article will highlight key theoretical considerations and clinical
issues regarding the diagnosis and management of patients with suspected disordered
auditory processing.
Dr Tony Sirimanna,
MB, BS, FRCS,
DLO(UK), MS,
MSc, FRCP,
Consultant
Audiological
Physician.
Correspondence
Dr Doris-Eva Bamiou
Consultant in
Audiovestibular
Medicine,
National Hospital for
Neurology and
Neurosurgery & Great
Ormond Street Hospital
for Children.
Email: d.bamiou@
ucl.ac.uk
Declaration of
Competing Interests
Doris-Eva Bamiou is
the elected Chair of
the Steering
Committee of the
Auditory Processing
Disorders Interest
Group of the British
Society of
Audiology. Dr
Bamiou runs clinics
for adults with APD
at the National
Hospital for
Neurology and
Neurosurgery and
for children with
APD at Great
Ormond Street
Hospital.
What is the current scientific
theoretical background that may
inform setting up a clinical service
for auditory processing disorders
(APD)?
Recent advances in auditory neuroscience
have enhanced our understanding of how
the brain extracts biologically relevant
meaning from the pressure and frequency
waves representing sounds in the cochlea.
Auditory processing is thought to be subserved by an initial ‘decomposition’ of the
auditory signal in the cochlea, accurate transmission of the information contained in the
initial activity patterns and further analysis
(which includes summation, subtraction or
correlation) of the transmitted signal by the
‘central processor’ in the brain. At the level of
the auditory cortex, sounds are represented
as ‘auditory objects’ rather than in terms of
their acoustic features (Nelken 2008).1
Functional organisation of the auditory cortex spans a continuum, from basic sensory
processing in primary cortical areas, to polymodal integration in non-primary (or association) areas, to behavioural modulation in
the limbic system and multisensory processing in multisensory subregions (Winer et al.,
2006).2 Neural representation of sound is
influenced by task-specific demands, expectations, and higher order top down effects
on multiple levels of the auditory pathway.
For example, auditory attention may modulate cochlear activity as reflected on otoacoustic emission recordings in a frequencyspecific manner, while attentive expectation
(such as when listening to a known melody
with silent gaps) helps to shape cortical
responses (as the auditory cortex will be
active even during the silent gaps in the
melody) (reviewed in Fritz et al., 2007).3 The
common representation between auditory
function and known cognitive, emotional
and visual centres may help explain how
other non-auditory factors influence and
modulate the perception of sounds.
Audiologists have long been aware that a
normal functioning ear does not guarantee
normal hearing and conversely, that patientor parent- (in the case of a child) reported
auditory difficulties such as difficulties with
speech in background noise may not always
arise because of problems within what is traditionally regarded as the auditory domain.
Our current understanding of the intricate
process of audition has not as yet entirely
translated into the clinic in the form of gold
standard tests for auditory processing, but
may help inform our clinical practice and
decision making.
What drove the development of the
APD service?
The development of the paediatric APD service at Great Ormond Street Hospital
(GOSH) and of the adult APD service at the
National Hospital for Neurology and
Neurosurgery (NHNN) was driven by realisation of the need and, in recent years, by a
rapidly increasing patient as well as professional demand. Children with suspected
APD may experience language or learning
difficulties, may be perceived as unruly, due
to his / her poor listening skills, or may be
perceived as not fulfilling their potential.
Adults with APD (and this includes a significant proportion of the aging population)
may similarly experience difficulties in their
personal, social and professional environments, and may be perceived as unsociable
or uncooperative.
Are there controversies surrounding
APD?
A great many, including what exactly constitutes an APD, with some professionals
69
feature
being still unconvinced that it exists as a
separate clinical entity, poor understanding of the boundaries and overlap
between APD and language or other
developmental disorders, and lack of uniform accepted guidelines regarding testing
and management of APD. Auditory processing disorders are not formally recognised in current editions of any of the formal classification manuals, such as the
Diagnostic and Statistical Manual of
Mental Disorders (DSM-IV). It is beyond
the scope of this paper to argue for or
against its existence – both authors use
the term APD as referring to ‘disordered
auditory processing’ and distinguish APD
from language processing disorder that
extends from the point at which speech
sounds are categorised into psycho-linguistically distinct percepts.
What is the diagnostic test
protocol at GOSH and NHNN, on
what premises was it chosen and
how is the diagnosis of APD made?
The clinical presentation of APD in both
children and adults may include difficulties with localisation and distinction / discrimination of sounds. Understanding
speech in background noise and fast / distorted speech, auditory memory or musical perception may be impaired, to name
but a few patient- and parent-reported
symptoms, and there may be additional
social, emotional, language or educational
sequelae. This clinical presentation is characteristic but non specific to APD, in that
other higher order disorders (for example
of language, attention, cognition and so
on) may also be implicated. The APD
Interest group of the British Society of
Audiology4 thus recommends that the
assessment for APD should be multidisciplinary and requires a test battery
approach. The assessment should include:
Detailed audiometry to define hearing
thresholds and to assess cochlear and
auditory nerve function
● Specific central auditory tests, both
non-speech and speech, which should
assess >2 different aspects of auditory
processing
● Tests of language, cognition and memory (conducted by the appropriate certified professionals) to assess the effect of
higher order factors on the patient’s
presentation.
It is essential to ensure that the child's (or
adult’s) attention is optimum at all times
during behavioural testing.
At GOSH, paediatric patient referrals
are only accepted if they have had a recent
speech and language and cognitive psychology assessment, especially if there are
concerns about language or cognitive
function, while adult patients at the
NHNN are referred for cognitive neuropsychometry and speech and language
assessment after they have been seen in
the clinic. The audiological test battery in
both settings has been informed by the
BSA recommendations, by previous recommendations of the American Speech
Language and Hearing Association (ASHA
1996, 2007)5,6, as well as by what tests are
available for clinical practice with ageappropriate normative data. This battery
includes:
a. baseline audiometry (including tympanometry, acoustic reflexes, otoacoustic
emissions and suppression, speech discrimination in quiet and auditory brainstem evoked responses)
b. central auditory tests (obtained from
Audiology Illustrated7 or from Auditec8)
Dichotic digits (dichotic listening)
Frequency and duration pattern tests
(temporal sequencing)
Gaps in noise and / or random gap
detection tests (temporal resolution)
●
Children with suspected APD may
experience language or learning difficulties,
may be perceived as unruly, due to their poor
listening skills, or may be perceived as not
fulfilling their potential
70
Masking level difference (Binaural
integration)
Low redundancy speech tests such as
filtered words or words presented in a
background of babble (from the SCAN
C or A by Keith from Tartan Products9,
in the paediatric setting, and a homedeveloped test delivered via the Matlab
platform at the NHNN)
c. Selected cases may have additional
tests, such as a formal assessment of
musical skills (Montreal battery for the
evaluation of amusia by Peretz et al.
200310 for adults, or Gordon’s musical
aptitude profile for children from GIA
Publications11), electrophysiology such
as mismatch negativity or other auditory event related potentials and imaging
as needed. The Newcastle Auditory test
Battery (NAB, Griffiths et al., 2001)12 and
the IMAP test battery (see Melanie
Ferguson’s article in same issue) may
also be applied on patients for research
purposes.
Diagnosis of APD is made upon the findings of abnormal results in at least two
tests, one of which is non-speech, and by
exclusion of other potential confounders
as per the basic audiology, speech and language and cognitive assessments.
How can APD patients be
managed?
Diagnosis of APD helps to explain the
patient’s difficulties and to provide the
adult or child sufferer and their families
with advice that may help. This diagnosis
will also help provide some guidance for
the child’s teachers as to how to provide
optimum support that will help the child
to achieve his / her best.
Intervention for children and adults
with APD is based on the patient’s test
deficits and complaints, and implemented
by a range of professionals. This may
include (Bamiou et al., 2006)13:
a. Modifying the educational environment, including provision of personal or
soundfield FM systems (that is, wireless
devices that receive, amplify and transmit the sound to the listener’s ear),
speaker / teacher based adaptations
and environmental modifications in
order to reduce ambient noise and
reverberation (that is, multiple reflections of sounds within a room) as per
current UK building regulations for
schools
b. Auditory training (AT). This aims to
improve auditory processing by tapping
into the brain’s potential for plasticity,
but it may also enhance or facilitate
other higher order / top-down processes
feature
such as auditory attention or memory. Auditory training
can be implemented by means of commercial computerised game type programmes, or conducted in the form
of ‘games’ or exercises and can be aimed at improving
specific deficits detected on testing for example interhemispheric exercises, phonemic discrimination
c. Compensatory linguistic and other strategies may also
be beneficial. However, although there is some initial
evidence that such interventions help both children
and adults with APD, there is a great need for robust
randomised controlled trials to assess whether a specific type of intervention is more beneficial than other
types of non-specific input.
What are the priorities for the future?
There is a pressing need for reliable diagnostic tools and
for uniform diagnostic criteria for APD in order to facilitate research, which would translate into evidence-based
clinical practice (Bamiou and Luxon, 2008).14 This has
been acknowledged by several multiprofessional
consensus conferences, both in the UK and abroad (for
example BSA 2007, ASHA 2005). In the UK, the British
Society of Audiology established a multidisciplinary APD
Interest Group in October 2003 with an elected steering
committee (of which the first author is current chair and
the second author is past chair), which aims to define
guidelines and identify research needs via a multidisciplinary forum (please see the website on how to become
involved if interested15). The clinician who deals with the
assessment of suspected APD cases is faced with challenges and scientific uncertainties. However, this relatively
new field is rapidly expanding, as basic scientific findings
are being translated into clinical practice, and clinical
questions and ambiguities are identified and addressed by
scientific studies. n
References
1. Nelken I. Processing of complex sounds in the auditory system. Curr
Opin Neurobiol 2008 (Epub ahead of print)
2. Winer JA. Decoding the auditory corticofugal systems. Hear Res
2006;212:1-8.
3. Fritz JB, Elhilali M, David SV Shamma SA. Auditory attention —focusing the searchlight on sound. Curr Opin Neurobiol 2007;17:437-55.
4. Auditory Processing Disorder (APD) Steering Committee British
Society of Audiology. Interim Position Statement on APD
[http://www.thebsa.org.uk/apd/BSA_APD_Position_statement_Final_
Draft_Feb_2007.doc]
5. American Speech and Language Hearing Association. Central auditory
processing: current status of research and implications for clinical practice. Am J Audiol 1996;5:41-54.
6. American Speech-Language & Hearing Association (ASHA), 2005.
(Central) Auditory Processing Disorders. http://www.asha.org/members/deskref-journals/deskref/default
7. Audiology Illustrated [http://www.audiologyillustrated.org/]
8. Auditec [http://www.auditec.com/]
9. Tartan Products [http://www.capdtest.com/default.cfm]
10. Peretz I, Champod S, Hyde K. The Montreal Battery of Evaluation of
Amusia. Ann NY Acad Sci 2003;999:58-75.
11. GIA Publications [http://www.giamusic.com/products/P-musicaptitudeprofile.cfm]
12. Griffiths TD, Dean JL, Woods W, Rees A, Green GGR. The Newcastle
Auditory Battery (NAB). A temporal and spatial test battery for use on
adult naive subjects. Hear Res 2001;154:165-9.
13. Bamiou DE, Campbell N, Sirimanna TS. Management of Auditory
Processing Disorders. Audiological Medicine 2006;4:46-56.
14. Bamiou DE, Luxon LM. Auditory processing
disorders – an editorial. BMJ 2008;337:a2080.
Forward
Features
for 2012
January | February
SPECIAL FOCUS
The Voice
AUDIOLOGY MATTERS
New Perspectives in Ménière’s
Disease
March | April
SPECIAL FOCUS
Implantable Devices
AUDIOLOGY MATTERS
Ototoxicity
May | June
SPECIAL FOCUS
Sinus/Allergy
AUDIOLOGY MATTERS
Hyperacusis
July | August
SPECIAL FOCUS
Endoscopic Ear Surgery and
Tele-health
AUDIOLOGY MATTERS
Tele-audiology
September | October
SPECIAL FOCUS
The Thyroid and Parathyroid
AUDIOLOGY MATTERS
Diagnostics
November | December
SPECIAL FOCUS
Laryngeal Cancer
AUDIOLOGY MATTERS
World Audiology
Topics are subject to change.
15. British Society of Audiology [http://www.thebsa.org.uk/]
71
Auditory Processing
Disorders in the
Educational Environment
Pauline Grant,
Advisory Teacher of
the Deaf, London
Borough of Harrow.
ncreasing numbers of children are being identified with auditory processing diffi-
I
culties and there is currently a degree of uncertainty about how they can be best
Correspondence
Pauline Grant,
Teachers’ Centre,
Tudor Road,
Harrow,
HA3 5PQ, UK.
Email: pauline.grant
@harrow.gov.uk
Declaration of
Competing Interests
None declared.
supported in school – and indeed, who should be responsible for providing it. In
my local authority of Harrow, Greater London, teachers of the deaf have taken the lead
and offer training and support to schools. We also acknowledge the importance of
working closely with our colleagues in speech and language therapy and educational
psychology and we are working together to produce an information leaflet which will
explain the condition, offer general teaching and learning strategies and explain our different support roles. This leaflet will go out to all Harrow schools and perhaps to other
professionals – such as GPs. We hope that this will reduce the levels of confusion around
referrals, intervention and who is responsible for what.
At the moment it is all rather muddled,
with schools learning that a child may have
a problem from a number of sources.
Raising awareness through training is
essential and recent local sessions have
been very well attended. In October, I
offered a Special Educational Needs Joint
Initiative for Training (SENJIT) half day on
this topic, held at the Institute of Education
in London, which attracted almost a hundred delegates representing twenty-five
local authorities.
Intervention and training in
schools
It is important that teachers and support
staff understand the difference between
hearing and listening (auditory processing).
Katz, Stecker & Henderson1 defined auditory processing as:
“What we do with what we hear – the
ability of the brain (the central nervous system) to process incoming auditory signals.”
Teachers often say to me, “I know he can
hear me, so it isn’t as if he isn’t listening!”
Figure 1: How do schools find out?
72
feature
Hearing is passive, but listening is an active
behaviour which takes much longer to
develop. Carol Flexer, PhD, states that in
normal listening development, it is not until
between the ages of eleven and fifteen years
that higher level listening skills, such as listening in background noise and filling in
the gaps of missed auditory information,
develop. In other words, children’s listening
skills are different to adults.
I am indebted to a young man I was
asked to see around fourteen years ago. He
was thirteen and his teachers thought he
had a hearing loss. He didn’t, but he clearly
had real trouble listening in conflicting
background noise. He sparked my interest
in auditory processing disorders (APD) and
I still use his definition of his own auditory
processing problems:
“Miss – I’m fine in here with you, it’s
quiet. But in the classroom, sometimes it’s
like I’m listening to scribble.”
For children with APD, hearing is not the
problem; but listening is, and some listening
situations will always cause difficulty. The
educational implications may be devastating. In a mainstream school, listening is the
primary method by which children absorb
new information – it is the cornerstone of
learning. Yet schools can be very noisy
places – particularly in the early years, when
we expect children to lay the foundations
of reading (phonics) and to learn to listen
to the teacher and each other – often in
conflicting background noise greater than
80dBA. Prof Bridget Shield (South Bank
University) and Prof Julie Dockrell (Institute
of Education, London) are researching the
effects of noise conditions on academic
performance in London primary schools.
They have found that performance in verbal and speed tasks is significantly reduced
in ‘babble’ conditions.
Table 1 illustrates the difficulties in a typical classroom caused by a diminishing signal over distance, competing against background noise.
Children in the middle or back rows will
miss important information spoken by the
teacher.
Schools have a responsibility to provide a
good acoustic learning environment, but,
unfortunately, this is not always the case.
Although the basic acoustic requirements
for a school are met, they are not wholly
satisfactory, even in new builds. In many
schools, children are taught in mobile classrooms or classrooms with high ceilings,
hard surfaces and lots of glass, resulting in
high levels of reverberation – a cacophony
of sound bouncing around the room creating a stressful environment for teacher and
pupil. It is no wonder that many children
either ‘switch off’ because listening is too
tiring, or else behave poorly because they
miss important information and fall behind.
Teachers of the Deaf have always known
the importance of good classroom
acoustics for children with a hearing loss –
if it is too noisy, they cannot learn to listen
effectively through hearing aids or cochlear
implants. But a good listening environment
is essential for all children – not just those
with APD or deafness. The National Deaf
Children`s Society has produced a useful
handbook – ‘The Acoustic Toolkit’ which
provides an overview for non specialists on
the school listening environment – as well
as more technical advice aimed at teachers
of the deaf, educational audiologists and
acoustical engineers.2
Our responsibility as educators is, first
and foremost, to acknowledge the problem. Once students realise that his / her difficulties are understood and that there are
strategies that will help them minimise the
effects of APD, they become more secure
learners.
It is not until between
the ages of eleven and
fifteen years that
higher level listening
skills, such as listening
in background noise
and filling in the gaps
of missed auditory
information, develop
What does educational
intervention need to address?
Dr Dilys Treharne (Department of
Communication Sciences at Sheffield
University) describes the following areas:
● Separating a target sound from background noise
● Auditory closure
● Memory: sequential and processing
● Recognising sounds
● Sequencing verbal concepts
● Rhythm
/ pattern perception and
prosody
● Processing at speed
● Integrating speech with movement
So, for example, children who have difficulty
separating a target sound from conflicting
background noise, will find it hard to function in classrooms that have poor acoustics,
or have a persistent buzz from overhead
projectors or computers. Environmental
noises from outside the classroom – grass
cutting or traffic – may be so distracting
that it is impossible to focus on what the
teacher is saying. Similarly, during group
work, it may be impossible to take part in
or understand a discussion against the general babble. Students can be helped to
develop this skill by being asked to follow
directions in different listening environments. Increasing levels of background
noise can be introduced – for example low
level music or radio talk shows. Young children might be asked to follow directions to
complete a picture, older students will be
asked to show his / her comprehension of a
piece of text read to them while background noise is present. Without this skill,
note taking in high school or college will be
impossible.
In Harrow, we advise schools on activities
to address particular areas of difficulty that
can be carried out by a teaching assistant
for a short period each day. We also provide
parents with some games and activities that
can be done at home. The activities should
be enjoyable and not cause additional anxiety – there must be no sense that the child
Table 1: Illustrates the difficulties in a typical classroom caused by a
diminishing signal over distance, competing against background noise.
Average classroom noise – 50 – 70 dBA
Adults need a +6dB advantage. Young children need +16dB
Distance from teacher
6 feet
12 feet
24 feet
Teacher’s voice
65dB
59dB
53dB
Noise
60dB
60dB
60dB
Signal to noise
+5dB
-1dB
-7dB
Dr Tony Sirimanna – Great Ormond Street Hospital, UK.
73
feature
has ‘failed’. Many schools will already have
resources to develop listening skills which are
perfectly suitable and can be adapted to
meet individual needs.
It is also helpful if the child is ‘cued in’ to
the lesson (what the lesson will be about
and the sequence of the tasks to be set).
Teachers often put this on the board, which
is excellent practice. Many children also find
it helpful to be given a glossary of terms and
vocabulary for each subject so that they are
prepared in advance for unfamiliar words;
their meaning, their sound, how they look
on the lips and what they look like on the
page. This will give them confidence to participate in the lesson and not feel that they
are in a constant state of ‘catch up’.
General class management advice is
offered:
●
●
●
●
●
Preferential seating
Encouraging ‘good listening, good
looking’
Checking understanding
Written information to consolidate verbal instructions
Reducing background noise. Rooms with
poor acoustics can be improved by carpets, wall hangings and displays, cork
notice boards, soft reading areas with
bean bags and making sure that rubber
tips are in place on chairs and tables.
● Making sure that lip reading conditions
are favourable.
Once strategies are in place and there is a
high level of awareness of APD in the school,
it may be appropriate for the child to try an
assisted listening device. This is a discreet ear
level fm receiver that is fitted to the child’s
ear – usually the right. The teacher wears a
transmitter and microphone so that his / her
voice is transmitted directly to the child’s ear
and the effects of background noise are minimised. It is fairly costly and there is some
debate about who should fund it, but some
parents have purchased it and it may also be
possible to have one on loan to try.
Some schools opt to fit classroom sound
field systems. Again the teacher wears a
microphone and fm transmitter, which
transmits to four speakers in each corner of
the room. These can improve listening conditions for all the children, but they only
work optimally if the room acoustics are fairly good to start. If the acoustics are poor,
then the voice coming out of the speakers
simply adds to the general noise in the
room.
In summary, strategies and listening exercises, together with sensitive handling of the
effects of APD will certainly help, but the
message needs to be sent loud and clear to
education authorities that schools must
ensure that they provide a good listening
and learning environment. Without it, children will not develop good listening skills,
they will miss information, fall behind and
risk educational failure. The issue of poor
acoustics in many classrooms – particularly
in old buildings or ‘temporary’ mobile classrooms – needs to be addressed as a matter
of urgency. Not just for those with APD, but
for all children. To quote John Erdreich, PhD:
“We would never teach reading in a classroom without lights. Why then do we teach
in acoustical darkness? Speaking to a class,
especially of younger children, in a room
with poor acoustics, is akin to turning out
the light.” n
References
1. Introduction to central auditory processing. In
Central Auditory Processing: a transdisciplinary view
Edited by: J Katz, NA Stecker, D Henderson. Mosby
Year Book Inc; St Louis, USA: 1992.
2. National Deaf Children’s Society [www.ndcs.org].
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74
The Challenges Ahead in
Jane R Madell, PhD,
CCC A/SLP, ABA,
LSLS, Cert AVT
Director, Hearing and
Learning Centre and
Co-Director, Cochlear
Implant Centre.
here are a number of challenges facing paediatric audiology in the next few
T
years. Some revolve around changes in the way audiologists are being trained,
and others around reimbursement for services. When the decision was made
to move to the AuD as the basic degree for audiologists in the USA, many looked
Correspondence
Jane R Madell,
The Ear Institute at
The New York Eye and
Ear Infirmary,
New York City,
NY, USA.
Declaration of
Competing Interests
None declared.
forward to what another year of training would provide in increased skills for audiologists. However, the move away from graduate programs which offered degrees in both
audiology and speech-language pathology significantly changed the philosophy of
many audiology training programs. In many graduate programs, audiologists are
getting extensive training in the technical aspects of the profession and less in the habilitation / rehabilitation aspects. As a result, many audiologists are having problems
meeting the needs of providing services to infants and children with hearing loss.
Working with babies
Most audiologists are comfortable using
auditory brainstem response (ABR) and
auditory steady state response (ASSR)
testing to diagnose hearing loss in infants
but are not comfortable obtaining behavioural testing for infants. While electrophysiologic testing is useful, it is not a
direct measure of hearing. If a hearing loss
is identified using ABR, and the need for
hearing aids is determined, the audiologist
sets hearing aids using real ear measures.
While the information provided with real
ear measures tells us how much sound is
being presented to the ear, it does not tell
us what the child is actually hearing. The
audiologist has used a protocol that is not
a direct measure of hearing (ABR) and an
indirect measure of how much sound a
hearing aid is providing (real ear measurements) to provide critical information
about whether we are providing sufficient
Figure 1: Test results
comparing BOA, VRA, and
conditioned play audiometry
for a child at eight weeks,
eight months, and 2.5 years.
75
feature
Figure 2: BOA evaluation of an eight week old infant identifying a moderate hearing loss and aided thresholds for each ear.
auditory access for a child to use audition
to develop speech and language.
We cannot ask infants to report what
they hear so it is essential that audiologists
have an accurate way of determining what
the child is actually hearing. We cannot use
ABR to monitor performance with hearing
aids. If we are going to effectively fit infants
with advanced technology, we need to be
able to measure how well they are hearing
with it. Without behavioural testing, it is
not possible to determine if the infant is
receiving sufficient benefit from the
hearing aids. Appropriately fit technology
may be the most important thing we can
do for the children we work with – ‘what
we hear is what we say’. For children over six
months of age many audiologists are
comfortable using visual reinforcement
audiometry to monitor both hearing levels
and gain obtained from amplification. But
if we are to meet our goals of fitting
hearing aids appropriately before six
months of age, it will be necessary for audiologists to gain competence testing infants
using behavioural observation audiometry
(BOA). As cochlear implants (CI) are being
fitted at younger ages, with many infants
being implanted at six to eight months, we
absolutely must know that the child has
received a sufficient hearing aid trial to be
able to comfortably report that hearing
aids are not providing sufficient benefit
and that the child is a candidate for a CI.
With BOA we can determine if a child is
receiving sufficient gain and hearing at
sufficiently soft levels. BOA can be accurately obtained by observing changes in
76
sucking.1-3 Figure 1 demonstrates test
results obtained for a child using BOA at
eight weeks, visual reinforcement audiometry (VRA) at eight months, and conditioned play audiometry at 2.5 years. The
results demonstrate that BOA is a reliable
test protocol.
Figure 2 demonstrates test results for an
eight week old who had hearing loss identified by ABR, confirmed using BOA, and
benefitted from hearing aids measured
using BOA. By using this protocol, the audiologist and the family can feel comfortable
that the child is hearing at sufficiently soft
levels to enable him / her to develop
speech and language skills. Developing the
ability to accurately monitor hearing in
infants using behavioural techniques can
significantly improve the services available
to infants.
Auditory habilitation /
rehabilitation
As audiology focuses more on the technical aspects of the profession, fewer audiologists are interested and involved in auditory habilitation / rehabilitation. Auditory
habilitation is frequently allocated only a
portion of a course in graduate school.
Much of audiology training takes place in
hospitals where medical audiology is the
primary service. Exposure to selection and
dispensing of hearing aids and cochlear
implants is frequently limited. When audiologists are involved in hearing aid
dispensing, patients are frequently fitted
and sent off to adjust to the hearing aids or
cochlear implants on their own. Children
may be referred for speech-language
therapy but in many cases, the speechlanguage pathologists are not trained in
auditory habilitation. It is assumed that
wearing the hearing aids or cochlear
implants will be enough to provide auditory access. No matter how good the technology is, children (and adults) with
hearing loss are listening through a
damaged auditory system. We should not
expect that they will develop maximum
auditory skills without specific training.
They need to be specifically taught to learn
to listen and to use auditory information to
make fine distinctions between similar
sounding phonemes (for example s / sh,
f / th). Children who are taught to use audition have been shown to have significantly
better speech and language skills than children taught using other methods.4-6
Audiologists need to know more about
development of auditory skills so they can
monitor the progress of their young
patients and either provide appropriate
therapy or know when to refer them on to
good auditory therapists. For this to
happen, auditory habilitation / rehabilitation will need to become a more significant part of the graduate school
curriculum and internships in this area will
need to be expanded.
Staffing in paediatric audiology
Staffing in paediatric centres is becoming
a concern. There are not a sufficient
number of clinicians available to work in
the area of paediatric audiology and reimbursement for paediatric audiology
feature
editorial board
Appropriately fitted
technology may be the
Editor
most important thing we can
do for the children we work
Mr Raymond Clarke, BSc, DCH, FRCS, FRCS (Orl),
ENT Consultant, Royal Liverpool University Hospital and Royal Liverpool
Children’s Hospital, Liverpool, UK. Email: [email protected]
Section Editors and Specialist Advisors
Section Editors
– Audiology Matters
with - what we hear is what
we say
Dr David Baguley, PhD, MBA,
Cambridge University Hospitals NHS
Foundation Trust, Hills Road,
Cambridge, CB2 2QQ, UK.
Email: [email protected]
Amanda Casey,
Director of Audiology Programmes,
Aston University, Birmingham, UK
services does not necessarily cover expenses of running
paediatric centres. Many people graduating with AuD
degrees in the USA report that they have spent a significant
amount of money getting educated and want to work in a
situation in which they can feel comfortable about their
earning capacity. This is not likely to happen in a paediatric
audiology program and so they are choosing to work in
ENT offices or in private practices dispensing hearing aids.
Paediatric centres have a difficult time bringing in sufficient
money to meet their expenses so salaries are often less that
they are in some other practices. This problem is a difficult
one to resolve. Audiology services for children take more
time and require more staff. Reimbursement for an audiological evaluation is the same for a short test for an adult
and a long test requiring two clinicians for a child. It is
possible that in some practices where both adults and children are seen this works out, but it does not work out in
paediatric centres where all patients require additional time
and additional staff. For children identified with hearing loss
and their families, having services provided in a specialty
centre is often the best option. It is essential that audiologists be reasonably reimbursed for the time and effort
involved in providing audiology services to children.
Currently, these centres count on fundraising to cover the
expenses not covered by insurance reimbursement. In difficult financial times, fundraising may not be adequate. If the
problem of providing adequate reimbursement is not
resolved, we risk having even fewer centres able to provide
quality services for children. The negative impact of this for
children with hearing loss is obvious.
Paediatric audiology continues to be a most exciting clinical area. We must continue to help it grow. n
References
1. Madell J. Behavioral Evaluation of Hearing in Infants and Children.
Thieme Medical, New York, USA: 1998.
2. Madell J. Video – Behavioral Evaluation of Hearing in Infants and
Children. Thieme Medical, New York, USA: 1998.
Dr Gareth Smith, MSc, AuD,
Deputy Head Audiology Service
Southend University Hospital,
Southend, Essex, UK.
– Book Reviews
Mr M Shahed Quraishi FRCS,
FRCS(ORL, H&N), Consultant
Otolaryngologist, Thyroid &
Parathyroid surgeon, Clinical Director
for Head & Neck and Special Surgery,
Hon Senior Lecturer in Surgical
Oncology, University of Sheffield,
Doncaster Royal Infirmary, Doncaster,
South Yorkshire, UK.
– Journal Reviews
Mr Liam Flood, FRCS, Consultant ENT
Surgeon, Middlesbrough, UK.
– Newsround
Dr Sunil Narayan Dutt, MS, PhD, FRCS,
DNB, DLO, DORL, Senior Consultant
in Otolaryngology and Head & Neck
Surgery, Bangalore, India.
– Trainee Matters
Nazia Munir, FRCS, DOHNS,
ENT Specialist Registrar, Liverpool, UK.
Jonathan Bernstein, FRCS,
Clinical Research Fellow,
Manchester Royal Infirmary and
Christie Hospital, UK.
Email: jonathan.bernstein@
doctors.org.uk
Specialist Advisors
– Audiology
Prof Mark E Lutman,
Prof of Audiology,
Southampton, UK.
– How I Do It
Mr Kim Ah-See, MD, FRCS(ORL),
Consultant Otolaryngologist, Head
and Neck Surgeon, Aberdeen, UK.
– Internet Review
Mr Tunde A Odutoye, FRCSEd,
Consultant Head and Neck Surgeon,
London, UK.
Mr David Pothier, TWJ Fellow in
Neurotology, University Health
Network, Canada.
– Hearing Industry
Karen Finch, RHAD, FSHAA, FRSA,
Managing Director,
The Hearing Care Centre Ltd,
Ipswich, Suffolk, UK.
Email: karenfinch@
hearingcarecentre.co.uk
– Speech and Communication
Mr Paul Carding, PhD,
Head of Speech and Voice
Department,
Newcastle-upon-Tyne, UK.
New Editor for
ENT & audiology news
NEWS
We are delighted to announce that we have appointed
a new Editor and two new Features Editors!
An announcement in the magazine will follow in the
January/February 2012 issue, but for a sneak peek at
our new line-up, visit our website.
3. Madell J, Flexer C. Pediatric Audiology: Diagnosis, Technology and
Management. Thieme; New York, USA: 2008.
4. Moog JS, Geers AE. Epilogue: major findings, conclusions, and implications for deaf education. Ear Hear 2003;24:1215-55.
5. Nicholas JG, Geers AE. Effects of early auditory experience on the spoken
language of deaf childen at 3 years of age. Ear Hear 2006;27:286-98.
6. Ying E. Speech / language / auditory management of infants and children with hearing loss. In: Pediatric Audiology: Diagnosis, Technology
and Management. Thieme; New York, USA: 2008.
77
New Developments in Hearing
Aids for Children and Adults
Josephine
Marriage, PhD,
UCL Ear Institute
Correspondence
Josephine Marriage,
UCL Ear Institute,
Gray's Inn Road,
London, UK.
WC1X 8EE
Declaration of
Competing Interests
None declared.
C
hanges in hearing aid technology over the past decade have been steady.
Hearing research has informed the development of new strategies in hearing
aid design to try and overcome the reduced detection and discrimination of
sounds by the impaired cochlea. The recognition that basilar membrane compression is
compromised in cochlear hearing loss emphasised the importance of compression in
amplification. Thus the first compression hearing aids were analogue, for example the kamp, evolving to digitally programmable, and then to fully digital devices. Since digital
technology has become the norm, there has been a period of steady improvements in
function, represented by increasing the frequency bandwidth for speech amplification,
directional microphone technology and noise reduction. These have been positive
advances, but not really groundbreaking in terms of changing outcomes and acceptance
of hearing aids for either children or adults.
Another
interesting
example of new
applications of
technology for
improving life
quality is the
development of
hearing aids with
background music
to help reduce
tinnitus
awareness and
situation anxiety
78
However the past couple of years have
seen a huge number of new initiatives and
developments, with inspired new concepts
in amplification. Some of this new
creativity has been helped by having
common themes with mobile phone technology. These include improvements in
miniaturisation and an increased expectation for ease of listening in adverse listening
conditions. While it is true that no hearing
aid can overcome the distortion that is due
to the impaired cochlear hearing mechanism, there is a new creativity in using technology to compensate for historical limitations of hearing aid amplification.
As long as hearing aids have been
around audiologists have grappled to find
the compromise between the occlusion
effect arising from a closely fitting
earmould, set against the increased susceptibility to acoustic feedback given by a
more open fitting. Now with the use of
signal processing to control feedback, for
example using signal cancellation and
inversion strategies, a new generation of
open fitting hearing aids are available.
Initially these were constrained to use with
high frequency hearing losses, but now can
be fitted to low, mid and high losses, with
some devices successfully being used with
severe extents of hearing loss.
Open fittings can be done at a single
appointment, there is no occlusion effect,
the aids are lightweight and come in funky
shapes, colours and finishes. They can even
curl around inside the concha with a very
discrete fitting, or reflect the wearer’s self
image as part of a personal or fashion statement. Many of these fittings are equally
appropriate for children with specific
audiometric configurations, as for adults
with acquired high frequency loss.
Another hugely innovative development is the synchronisation of binaural
hearing aids, using wireless technology to
maintain communication for the adaptive
function of two hearing aid devices. This
gives rise to a more stable representation of
the spatial scene for the listener from two
synchronised hearing aids. This gives
perceptual improvements in speech clarity
for the listener, increasing the potential for
higher-level auditory processes for localisation and segregation. Wearers report better
perception of sound source movement, for
example being able to hear an oncoming
bicycle from the tyre noise, and being able
to judge its distance.
Perhaps the most difficult nut to crack
has been in developing technologies for
improving understanding of speech in
noise.1 The irony is that most people first
decide to think about a hearing aid
because they can’t follow speech in a
group of people. They usually can follow
one to one conversation. They get a
hearing aid that improves their ease of
listening in one to one situations but is of
little benefit in noisy or group settings.
Many noise reduction approaches have
been explored and continue to be limited
in benefit, partly due to the cochlear
processing mechanism. However a
different approach has been to improve
feature
The be by Resound.
The water resistant Naida, by Phonak.
The Dual and Streamer from Oticon.
transmission of signals from a distance,
through having integrated receivers for
radio aids, wifi and, recently, bluetooth
technology. These systems will undoubtedly have new applications that are unfulfilled at the moment but represent potential for the future.
A new approach for increasing speech
transmission beyond the traditional
hearing aid amplification bandwidth is the
use of frequency compression. The search
for a method of transposing inaudible high
frequency speech information into the
lower residual hearing range has been
pursued for decades (frequency transposition). Recently algorithms have been
produced that show clear research
evidence for improvements in speech
recognition.2 This frequency compression
approach is not only beneficial for people
with restricted hearing in the high frequencies, but also for flat audiometric configurations. The high frequency cues that fall
outside the normal amplification bandwidth therefore have benefits for perception of voiceless fricatives including the allimportant / s / in spoken English.3 The
increased speech clarity improves, among
other things, the ability to hear the regional
accent of the speaker with all the connotations that accent has for relating to the
person behind the speech. The effect on
localisation skills has not yet been evaluated. This particular innovation has applications for severe and profoundly impaired
adults and children, and may be an alternative consideration for some people who
were potentially candidates for cochlear
implantation.
Cochlear implants (CI) have been very
effective in developing new ways of
extracting a finite number of features of
speech to give the extraordinary performance that can be achieved now, when
the CI intervention is timely and individualised. Imagine the celebration there will be
for an equivalent device that can restore
visual perception, as CI have been able to
supplement auditory perception. The
constraints of the impaired natural cochlea
are still the constraints of hearing aid technology. As there is better understanding of
the function of the inner and outer hair
cells in the cochlea, coupled to creative use
of acoustic signal processing, we may
expect to see more radical ways of
sculpting amplification around the specific
impairment of function rather than the
profile of the audiogram (for example,
Moore, 2004).4
As technology provides new and better
solutions for hearing impaired people,
more people will use hearing devices and
the stigma that was traditionally associated
with hearing aids will fade. (Remember
when everyone had contact lenses, and
now it seems the majority of middle-aged
adults wear fashion-statement glasses).
Another interesting example of new applications of technology for improving life
quality is the development of hearing aids
with background music to help reduce
tinnitus awareness and situation-anxiety
that can exacerbate hyperacusis, or aversion to specific sounds.
While these innovations in effective
hearing aid technology can provide new
hearing opportunities, the challenge for all
of us is to use hearing aid technology as
part of the wider context of rehabilitation
of a hearing impaired individual within his /
her own community.
This article does not aim to cover all the
new innovations in technology that are
breaking for hearing aid products at
present, it aims to give some examples of
the excellent and focused commitment to
hearing aid production by the hearing aid
manufacturers and their research and
development (R&D) teams. One device
that has been on the wish list for children
and parents for many years is a waterproof
hearing aid, that can be used for swimming, water play and beach holidays. An
analogue version has been available for
many years, but with a linear amplification
output and it is no longer available in the
UK. Clearly the waterproofing has been
shown to be effective, it just needs
applying to the better quality modern
digital devices that we are blessed with
today. Come on you hearing aid manufacturers, see if you can’t sort this out for the
next generation of children. They will be
able to express their own thanks to you. n
References
1. Turner CW, Henry B. Benefits of amplification for
speech recognition in background noise. J Acoust
Soc Am 2002;112:1675-80.
2. Bagatto MP, Scollie SD, Glista DA, Pasa V, Seewald
R. Case study outcomes of hearing impaired listeners using non-linear frequency compression technology. Audiology Online 2008. [http://www.audiologyonline.com/articles/article_detail.asp?article_id
=1990]
3. Stelmachowicz PG, Pittman AL, Hoover BM, Lewis
DE and Moeller MP. The importance of high-frequency audability in the speech and language
development of children with hearing loss. Arch
Otolarygol Head Neck Surg 2004;130:556-62.
4. Moore BCJ. Dead regions in the cochlea: conceptual foundations, diagnosis and clinical applications.
Ear Hear 2004;25:98-116.
79
Cochlear Implantation
in Early Childhood:
what is happening in the long term?
Sue Archbold, MPhil,
The Ear Foundation,
Nottingham, UK.
Correspondence
Sue Archbold,
The Ear Foundation,
Nottingham, UK.
E: Sue@
earfoundation.org.uk
Declaration of
Competing Interests
The Ear Foundation
receives educational
grants from Advanced
Bionics, Cochlear Europe,
and MED-EL UK.
Note
This article is based on a
presentation given at
the Royal Society of
Medicine, February 5th
2009, as the invited
Graham Fraser Memorial
Lecture.
O
ver the last twenty years cochlear implantation in children has developed
from being considered experimental and highly controversial to being the
accepted management for profoundly deaf children. In most developed
countries the majority of deaf children now receive implants, and developing countries
are making great strides in implant provision too. Over 80,000 children have implants
worldwide, and are being implanted increasingly early in life, following progress in
screening programmes and the earlier diagnosis of deafness. More recently, bilateral
implantation has been increasingly considered as the appropriate way forward for children. As experience has grown, and technology developed, expectations have changed
far beyond what was envisaged possible in the early history of childhood implantation.
Early expectations in children
When implantation began in children in the
1980s, it was largely carried out on those
who had been deafened, typically by meningitis, and those who gained no benefit at all
from conventional hearing aids. Early expectations were that cochlear implantation
would provide environmental sound awareness and cues for lipreading. Outcomes have
far exceeded these expectations, providing
deaf children with the ability to understand
conversation without lipreading, and to
acquire new spoken language, thus encouraging implantation in those who were born
deaf and in those with complex needs. What
do we know of the long-term outcomes in
these groups? What issues remain?
Outcomes from implantation in
children
Outcomes in children were initially
measured in terms of speech perception and
production; little information was initially
gathered about the effects of implantation
on the development of language, on education and on psycho-social functioning1 and
these areas were largely under-researched.
However, the effect of implantation in early
childhood on these areas takes years to
emerge, and is complex to investigate, with
many confounding variables. Changing technology and candidacy sets challenges for
researchers.2
How to manage the effect of profound
deafness from early childhood on the development of language has long been the
subject of controversy; whether to use sign
language, signed support or spoken
language for example. The effect of cochlear
implantation on the development of spoken
80
language far exceeded expectations: so
much so that two researchers of deaf education who had been conservative about
implantation, commented:
“Spoken language development of deaf children may be more possible today than ever
before. …we are now presented with the
opportunity to learn from earlier mistakes
and misunderstandings and to synthesise
the best ideas from the past with the technological, programming and social
advances of today. …….we may finally be
able to fulfil the promise of effective support
for speech and spoken language with
hearing loss.”
Marschark and Spencer, 2006, p17.3
Increasingly, implantation in the first year of
life is enabling the development of early
communication skills and spoken language
to those levels expected of hearing children4,5; outcomes not foreseen ten years ago.
The effect of this progress on levels of educational attainments, particularly literacy, are
now being reported in the long term.
Educational outcomes
Traditionally, reading has been a challenge for
deaf children, and studies show that little has
changed since the seminal study of Conrad
(1979)6 showing deaf children leaving school
with the reading age of an eight year old. A
number of studies demonstrate improvements in reading progress for children with
implants. For example, Geers7 found over
half of children tested aged 8 / 9 years of age,
scored within the average range for hearing
children (n=181). Vermuelen8 found the
reading comprehension of children with
implants was significantly better than those
without (n=50). Archbold et al.9 found that
feature
age at implant was a significant factor: those
implanted below 42 months were reading at
age-appropriate level five years later.
Few studies have looked at a broader
range of educational outcomes, but Stacey
et al.10 in a study of 2,853 children, including
468 with implants, found improvements in
educational attainments when the group
with cochlear implants were compared with
those with hearing aids. Thoutenhoofd,11
exploring comprehensive Scottish data,
showed that children with implants educationally outperformed those with hearing
aids, particularly in Maths.
What about the effect of cochlear
implantation on educational decisions of
communication mode and educational
placement, which have been taken as
measures of success? We know that more
children with implants go to mainstream
schools than to special schools, when
compared with groups with hearing aids12
and that children with implants are more
likely to use spoken language than to use
sign language.13 Watson et al.14 found children implanted young were likely to move
to using spoken language, whether or not
sign support had been used prior to implantation. She went on to show15 that from
responses by 142 parents, 120 indicated a
change in communication choice after
implantation, with 113 towards spoken
language and seven towards signed communication. A further in-depth study by
Wheeler et al.16 showed that parents viewed
communication as a journey – while the
goal after implantation was spoken language
most were pragmatic about communication, recognising that some sign or gestural
support can be useful at different times.
A study of 29 young people aged 13-19
years in the UK by Wheeler et al.16 revealed
that 76% considered the cochlear implant
helped understanding in the classroom
compared with 7% who felt that signed
support was useful. All viewed their implants
positively, and all but two wore them all the
time. Other long-term studies17,18 showed
benefits over ten years after implantation in
terms of high levels of usage, and reported
benefits in spoken language and education.
The reality?
While outcomes from implantation have far
exceeded expectations, there are a few
reality checks to be made. Research in
outcomes from deaf children is made
complex by the lack of heterogeneity in the
group, and research into those with
implants has an increased number of variables to account for; type of device, the
effectiveness of programming for example.
Marschark et al.19 commented on the need
to control variables such as age at implantation, language and reading skills before
implantation, and consistency of implant
use. Thouthenhoofd et al.1 made similar
comments: the variability in outcomes from
implantation is known to be large.2
Comprehensive studies into long-term
outcomes from implantation are rare: to
date Beadle et al.17 and Uziel et al.18 are
unusual in their completeness and length of
follow-up. Others report on skewed populations and omit to report on those lost to
follow-up. In the study of Stacey et al.10 those
with implants were younger as a group and
from more affluent families, known factors
for success. In Holland, Damen et al.20 found
that children with implants did less well than
hearing children in mainstream schools, as
did Mukari et al.21
In the report of Thoutenhoofd11 those
with implants were doing better than those
with hearing aids, but not as well as their
hearing peers. Similarly, the Vermuelen8
study reported that those with implants
were still delayed compared with hearing
peers. In the large US Geers et al.7 study,
when they retested the group at aged 15 / 16
years of age, some had made little progress in
reading skills in the intervening years. They
concluded:
“early cochlear implantation had a longterm positive impact on auditory and verbal
development, but did not result in ageappropriate reading levels in high school for
the majority of students.”
Geers et al., 200822
At high school level, linguistic demands are
high and children with implants may find
this challenging, particularly in poor acoustic
conditions and demanding learning situations. Increasing numbers of reports reveal
difficulties in the more subtle linguistic skills,
which are important at this stage. A study
asking the young people themselves what
they found in secondary schools revealed
the challenges they meet in the more
demanding conditions they face there. For
example, two mainstream students with
excellent speech perception scores:
“I need one person at a time, just one person
at a time not all the same time, who, over
my head, I lose control. It is quite hard to
work… other people talk too fast, then walk
off” Mainstream student (14)
“Group work is the biggest pain you could
ever possibly imagine. Small group is all right
but big group or class discussions they are
the worst.” Mainstream student, (14)
(Report to RNID, 2009)23
The changing educational needs of those
with implants in high school or secondary
school do not appear to be being met.
Continuing to provide similar support to
that previously provided for profoundly deaf
children is not appropriate.
What are the long-term needs after
implantation?
In a European study carried out by Archbold
& Wheeler (submitted) of the long-term
needs of children with implants, training for
local professionals and long-term management were by far the most common issues
to be raised by parents and professionals.
They commented that early support was
not continuing, that new teachers need
training in managing the technology and
how to maximise its use, particularly in
mainstream schools. They also had concerns
about support into adulthood for children.
Comments included:
“Need to realise that CI is not a quick ‘fix’
and the children still need support in whatever educational provision they are in
whether signing or oral, mainstream or
special”
“Most day to day support is from parents
and teachers – they need to be well
informed”
“Our son had implant at brilliant centre,
and was sent to school with little knowledge… we have been fighting for an education which will help him to develop his CI
use best”
(Archbold & Wheeler, submitted)
A study of parents in the UK revealed similar
comments. One parent when asked about
the follow-up care after implantation
commented:
“It is dreadful in my experience. It is incomprehensible that my daughter could receive
£60K worth of technology and no clear plan
about how to enable her to make best use
of it. Complete waste of resources with lots
of people 'involved' in her care but doing
very little of any use other than endless
assessment. Thank goodness for the voluntary sector. CI centre care great at technical
support”
(Report to NDCS, 2009)24
Comment
The long-term outcomes from early implantation in children have transformed the
opportunities for developing spoken
language and for its consequent effects on
education in ways which were unforeseen
ten years ago. Evidence about linguistic and
educational outcomes inevitably takes years
to accrue, and in the meantime technology
and candidacy has moved on. The research
evidence produced here is based largely on
populations which were older at time of
implantation than is currently the case, who
were using older technology, had less
residual hearing and typically were wearing
81
feature
Our latest addition
only one implant, rather than two. There is every likelihood
that future outcomes will be even more encouraging: those
involved in cochlear implantation are required to collect
rigorous data, collaborating with those in other associated
fields such as cognition and linguistics, ensuring that the data
collected is robust, inclusive, and long-term. However, this will
only be possible when long-term management is secure with
an informed community-based infrastructure to support it. n
References
1. Thoutenhoofd E, Archbold SM, Gregory S, Lutman ME, Nikolopoulos TM,
Sach TM: Paediatric Cochlear Implantation. England: Whurr; 2005.
2. Kirk KI, Choi S. Clinical investigations of cochlear implant performance. In
Cochlear implants: principles and practice (2nd edition). Edited by: Niparko
JK. Philadelphia; Lippincott Williams & Wilkins. 2009:191-222.
3. Marschark M, Spencer P. Historical and Theoretical Perspectives. In
Advances in Spoken Language in Deaf and Hard of Hearing Children. Edited
by: Marschark M, Spencer P. New York: Oxford University Press; 2006:3-21.
4. Dettman SJ, Pinder D, Briggs RJ, Dowell RC, Leigh JR. Communication development in children who receive the cochlear implant younger than 12
months: risks versus benefits. Ear Hear
2007;28:11S-18S.
5. Tait ME, Nikolopoulos TP, Lutman ME. Age at implantation and development
of vocal and auditory preverbal skills in implanted deaf children. Int J Pediatr
6. Conrad R. The Deaf School Child. London: Harper Row, 1979.
7. Geers AE. Predictors of reading skill development in children with early
cochlear implantation. Ear Hear 2003;24:59S-68S.
8. Vermeulen AM, Van Bon W, Schreuder R., Knoors H, Snik A. Reading comprehension of deaf children with cochlear implants. J Deaf Stud Deaf Educ 2007;12:283-302.
9. Archbold S, Harris M, Nikolopoulos TP, O’Donoghue G, White A, Lloyd
Richmond HL. Reading abilities after cochlear implantation: the effect of age
at implantation on outcomes at five and seven years after implantation. Int J
Pediatr Otorhinolaryngol 2008;72:1471-8.
10. Stacey P, Fortnum H, Barton G, Summerfield A. Hearing-impaired children in
the United Kingdom, I: Auditory performance, communication skills, educational achievements, quality of life, and cochlear implantation. Ear Hear
2006;27:161-86.
11. Thoutenhoofd E. Cochlear implanted pupils in Scottish schools: 4-year school
attainment data 2000-2004. J Deaf Stud Deaf Educ 2006;11:171-88.
12. Archbold SM, Nikolopoulos TP, Lutman ME, O’Donoghue GM. The educational settings of profoundly deaf children with cochlear implants compared
with age-matched peers with hearing aids: implications for management. Int J
Audiol 2002;41:157-61.
13. Archbold SM, Nikolopoulos TP, Tait M, O’Donoghue GM, Lutman ME,
Gregory S. Approach to Communication, Speech Perception and Intelligibility
after Paediatric Cochlear Implantation. Br J Audiol 2000;34:257-64.
14. Watson LM, Archbold SM, Nikolopoulos TP. Children’s communication mode
five years after cochlear implantation: changes over time according to age at
implant. Cochlear Implants Int 2006;7: 77-91.
15. Watson LM, Archbold S, Hardie T, Wheeler A. Parents’ views on changing
communication after cochlear implantation. J Deaf Stud Deaf Educ
2007;13:104-16.
16. Wheeler A, Archbold S, Gregory S, Skipp A. Cochlear implants: the young
peoples’ perspective. J Deaf Stud Deaf Educ 2007;12:303-16.
17. Beadle EAR, McKinley DJ, Nikolopoulos TP, Brough J, O’Donoghue GM,
Archbold SM. Long-term functional outcomes and academic-occupational status in implanted children after 10 to 14 years of cochlear implant
use. Otol Neurotol 2005;26:1152-60.
18. Uziel AS, Sillon M, Artieres F, Pison JP, Daures JP, Mondain M. Ten-year follow-up of a consecutive series of children with multichannel cochlear
implants. Otol Neurotol 2007;28:615-28.
19. Marschark M, Rhoten C, Fabich M. Effects of cochlear implants on children’s reading and academic achievement. J Deaf Stud Deaf Educ 2007;
12:269-82.
20. Damen GW, Langereis MC, Snik AF, Chute PM, Mylanus EA. (2007).
Classroom performance and language development of CI students placed
in mainstream elementary school. Otol Neurotol 2007;28:463-72.
21. Mukari S, Ling L, Ghani H. Educational performance of pediatric cochlear
implant recipients in mainstream classes. Int J Pediatr Otorhinolaryngol
2007;71:231-40.
22. Geers A, Tobey E, Moog J, Brenner C. Long-term outcomes of cochlear
implantation in the preschool years: from elementary grades to high school.
Int J Audiol 2008;47 Suppl 2:S21-30. 2008;
23. Wheeler A, Archbold S, Gregory S. Supporting Children with Cochlear
Implants in Secondary School. Report to RNID; 2009.
24. NDCS report: Children with cochlear implants: what do they need and
what do they get? 2009.
82
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New Frontiers: auditory
brainstem implant results
in adults and children
Martin
O’Driscoll, MSc,
Consultant Clinical
Scientist (Audiology)
Correspondence
Martin O’Driscoll,
Head of Audiology,
Manchester Cochlear
Implant Programme,
Ellen Wilkinson Building,
University of
Manchester,
Manchester,
M13 9PL, UK.
E: martin.odriscoll@
manchester.ac.uk
Declaration of
Competing Interests
None declared.
Acknowledgements
Many thanks to Lise
Henderson, Deborah
Mawman, Adam Walker
and Prof Richard
Ramsden.
Background
Cochlear implantation is a remarkably effective treatment for many people with severe
to profound sensorineural hearing loss. For a
successful outcome, the cochlear implant
(CI) requires a cochlea structure and an
intact auditory (VIII) nerve to allow the electrical signal to be transmitted to the central
auditory system. For some causes of hearing
loss, the auditory nerve or the cochlea is
compromised to such an extent that a CI is
not a viable option. The auditory brainstem
implant (ABI) has been developed from CI
technology and is indicated for totally deaf
people who have anatomical abnormalities
of the cochlea or dysfunction of the auditory nerve. About 1,000 people have
received an ABI worldwide, with the majority
suffering from neurofibromatosis type 2
(NF2). NF2 is a genetic condition that affects
about one in 40,000 people and is characterised by the presence of bilateral vestibular
schwannomas. The presence of these
tumours, or the surgery to remove them,
usually results in total deafness. Other more
recent indications for ABI are for children
with cochlear or auditory nerve agenesis or
hypoplasia (absent or malformed cochleas
or VIII nerves) or for children and adults for
whom cochlear implant surgery is complicated by the presence of total cochlea ossification following meningitis.1,2
The Nucleus ABI consists of an electrode
array which is paddle-shaped with 21 small
disc electrodes (Figure 1). The array is
inserted on to the surface of the cochlear
Figure 1: The Nucleus ABI internal implant. (Image courtesy of Cochlear Ltd).
nucleus of the brainstem in the lateral recess
of the fourth ventricle. The device stimulates
the auditory pathway at a higher level than
the damaged cochlea or VIII nerve. During
surgical placement of the ABI, electrodes on
the array are stimulated and an electrically
evoked auditory brainstem response
(EABR) is recorded to aid the surgeon in the
optimal placement of the device on the
cochlear nucleus. The EABR recorded via ABI
stimulation usually consists of anything from
one to four peaks (Figure 2). In comparison
to a standard acoustic ABR we do not see
waves I or II as these are generated within the
auditory pathway before the cochlear
nucleus.
Post-operatively, the ABI is set up in a
similar manner to a CI with individual electrodes stimulated to establish threshold (T)
and comfortably loud (C) levels of response
which are then used to form a MAP or
Programme within a conventional speech
processor. There are, however, notable differences between the programming of a CI and
ABI:
Stimulation of the ABI electrodes may
produce undesirable side-effects by activating neural tissue that is not auditory.
Patients report a variety of non-auditory
sensations from dizziness to sensations in
muscle groups down the body ipsi-lateral to
the side of the implant.1,2 Patient feedback is
important in establishing the non-auditory
sensations, and electrodes that produce
these side-effects are deactivated and not
used in the take home MAP.
The CI takes advantage of the well
defined tonotopic map within the cochlea
in which stimulation of electrodes positioned in the basal end of the cochlea will
elicit a higher pitch sensation than those
positioned in the apical end. In contrast, the
tonotopic map within the cochlear nucleus
runs parallel and obliquely through the
nucleus3,4 and the ABI positioned on the
surface does not stimulate neural structures
in such a clear, tonotopically ordered way. To
optimise the clinical fitting of the ABI, the
clinician must rely on the patient to report
the relative pitch perceptions produced
when each electrode is stimulated in order
to create a patient specific tonotopic map.
Speech perception outcomes with an ABI
are relatively poor compared to those
83
feature
Figure 2: Example of an intra-operative, three peak EABR.
Figure 3: Environmental sound discrimination with the ABI in adults and older children (n = 23). Boxplots
indicating median value with inter-quartile range and the upper and lower range of measures.
reported in multichannel CI users.5 Possible
reasons for this may be due to the presence
of non-auditory side-effects limiting the
overall number of channels that can deliver
useful frequency information coupled with
poor frequency specificity delivered by a
restricted tonotopic range. In addition, the
debilitating disease process of NF2 may take
its toll. Colletti and his colleagues from
Verona have described outcomes of ABI in
non-tumour patients compared with the
traditional tumour (NF2) patients.6 The nontumour patients had post-meningitic
cochlea ossification, advanced otosclerosis
or post-traumatic avulsion of both cochlear
nerves. Some of these subjects achieved
good speech discrimination without
lipreading and they compared well with the
best CI results. The presence of the tumour
in NF2 was believed to have damaged
84
specialised cells in the cochlear nucleus
important for speech perception leading to
relatively poor outcomes in the NF2
patients. Coletti was also the first to report
the use of ABI for children born deaf with
cochlear nerve aplasia or hypoplasia.7,8 In a
series of five children aged between 14
months and four years of age, the ABI was
reported to give between eight and 21 electrodes that produced an auditory sensation.
The longer term outcome and potential of
ABI in these children is not yet reported.
Manchester ABI experience:
candidacy and device fitting
Older children and adults
Prof Richard Ramsden performed the first CI
in Manchester just over 20 years ago and
under his direction the implant programme
has grown to support over 1,000 adults and
children. The first ABI was performed in 1994
and now Manchester is the largest centre for
ABI in the UK supporting 50 older children
and adults with the Nucleus ABI system. All
but two of these patients have NF2, one has
biltateral vestibular schwannoma not related
to NF2 and another has otosclerosis with a
previous removal of a CI following infection.
The patients range in age from 12 years to 73
years with a mean age of 30 years. Nine
patients had their implant inserted during
removal of their first vestibular schwannoma
whilst retaining useful hearing in their
contra-lateral ear. Typically, these patients do
not use the ABI until the hearing has deteriorated or the tumour removed from the
contra-lateral ear. Four of these subjects are
now using their ABI.
Programming the ABI and fitting the
external speech processor occurs when the
patient is well enough and about eight
weeks post tumour removal. Initial fitting
takes place within the hospital clinic. ECG
monitoring is performed due to the small
risk that heart rhythm could be disturbed by
stimulation of the vagus nerve by an ABI
electrode. In practice, this has never been
observed within our series of patients. The
levels of electrical stimulation required to
produce auditory sensations through an ABI
are higher than that typically found in
patients with a CI and wider stimulus pulse
widths and greater amplitudes of electrical
current are usually seen. Depending on the
stimulus levels required, either a Freedom
behind-the-ear or SPRINT body worn
processor is used. The T and C levels are
measured and the patient asked to report
any non-auditory sensation. The electrodes
with auditory sensations are placed in pitch
ranked order which is determined behaviourally using a midpoint comparison procedure.4 Once pitch ranked, the electrodes
producing auditory sensations are used in
the patient’s take home fitting. Further
appointments for fine tuning and intensive
auditory rehabilitation take place over a nine
month period with annual appointments
thereafter.
Young children
The incidence of cochlear nerve aplasia or
hypoplasia is not certain, however, most
paediatric cochlear implant teams will have
been referred children who have absent
cochlear nerves. The only hearing option for
these children is an ABI. With the experience
gained from both a large paediatric CI and
relatively large ABI programme and after
careful consideration of the ethical issues
involved, the team in Manchester decided to
feature
the rest. They are, however, patients that
report clear and consistent pitch variation
across the electrode array.
Figure 4: CUNY sentences discrimination in three conditions: lipreading (LR) alone, LR and ABI together
and ABI alone in adults and older children (n = 23). Boxplots indicating median value with inter-quartile
range and the upper and lower range of measures, except for outliers more than 1.5 box lengths from
the box edge.
offer ABI to three congenitally deaf children
with cochlear nerve aplasia or hypoplasia.
The children’s families were carefully counselled regarding the risks of surgery and the
uncertainty about whether the ABI could
provide sufficient information for the development of speech and language. The children, all boys, received their implants in late
2006 when they were aged between three
years seven months and four years nine
months.
The initial behavioural activation of the
ABI for these children was performed with
ECG monitoring in a hospital setting by clinicians experienced in programming speech
processors for children with cochlear
implants. Children were encouraged to
stand and they were closely observed to
note any body sway, induced nystagmus or
signs of discomfort. If a clear behavioural
reaction was observed, such as smiling,
stilling, looking up or pointing to the ear, a
conditioned response using play audiometry
was attempted. Where possible, the stimulus
level was reduced to find the T level. From
experience gained in setting speech processors for children with cochlear implants, the
C level was set at or just below the first stimulus level that provoked a clear behavioural
response.
During this initial programming, bipolar
electrode combinations that were found
to produce EABR intra-operatively were
stimulated. Any electrodes that produced
clear non-auditory responses were not
used in the final take home fitting for the
child. Electrodes were pitch ordered using
the theoretical physiological distribution
of pitch within the cochlea nucleus with
low pitch relating to the proximal end of
the electrode array and high pitch to the
distal end of the array. Following the initial
device fitting, further appointments for
fine tuning and habilitation using an audi-
tory verbal approach were offered on a
regular basis.
Outcomes
Adults and older children
Six patients (12%) experienced non auditory
sensations on all electrodes on the array and
were not able to use the ABI. On average, the
number of electrodes eliciting auditory
sensations was 11 (range: 0 to 21). The most
common non-auditory sensation was dizziness which was reported by 53% of patients
for at least one electrode. Four patients
chose to not use their device due to
perceived poor sound quality and lack of
benefit and five patients continue to have
some useful hearing in their contralateral ear.
Audiological results are available for 23
patients.
Figure 3 shows the results from a standardised, recorded test of 20 environmental
sounds in the auditory only condition.
Results are displayed over time up to 21
months post activation of the implant and
then the best percentage score obtained for
patients at any time after 36 months of
implant use. Figure 4 shows results from
standardised, recorded CUNY sentences in
three conditions: lipreading (LR) alone, LR
and ABI together and ABI alone. Again,
results are presented over time with the
result after 36 months representing the best
score obtained. It may be seen from both
Figures 3 and 4 that most progress with the
ABI occurs within the first nine months and
that the ABI acts as an aid to lipreading and
gives an awareness of environmental sounds.
Of interest, however, is that some patients
are able to achieve significant open set
discrimination with the ABI (note outliers in
the 36 m +, CUNY sentences, ABI only).
These patients have NF2 and it is not entirely
clear why they score so much higher than
Younger children
The children have had their ABI for just over
two years. Parents report that the children
use their ABIs on a full time basis and will ask
for their speech processors. Parents are able
to recognise if there is a fault with equipment
through the changes in their child’s
responses to sound, but only two of the
three children can reliably report when their
equipment is faulty.
Over time there have been improvements
in each child’s auditory awareness but there
are marked differences in the auditory abilities of the children. Whilst all three are able
to detect the Ling sounds (oo, m, ah, ee, sh,
s) and, therefore, have access to all the
components of spoken English, their ability
to discriminate and imitate these speech
sounds varies significantly.
The Meaningful Auditory Integration
Scale (MAIS) has been administered at
regular intervals to monitor the progression
in auditory development. The MAIS gathers
information from families regarding their
child’s early auditory responses through a 10
item questionnaire. Parents are asked to rate
the frequency of a range of auditory behaviours from Never = 0 to Always = 4. The
maximum score that can be achieved is 40.
The scores are outlined in the Table 1.
In addition, Child 1 has scored 82% on the
Iowa speech perception test at two years
post-implant.
All of the children use sign language as
their main mode of communication and
they are educated either in schools for the
deaf or hearing impaired units attached to
mainstream schools.
Child 1 recognises a wide range of environmental sounds and responds consistently
to his name. He is able to understand a
number of spoken everyday phrases without
lip patterns, he uses his voice consistently in
conjunction with sign and has a small
spoken vocabulary that would be recognisable to listeners familiar with the speech of
hearing impaired children.
Child 2 responds consistently to a range of
environmental sounds and his name. He
understands speech only with the support
of lip patterns, he uses his voice more consistently in conjunction with sign. The majority
of his speech attempts are unlikely to be
recognisable without the support of sign
language.
Child 3 responds consistently to a limited
number of environmental sounds (dogs
barking, phone ringing). His parents report
85
feature
Table 1. MAIS scores over a 12 month period for 3 children with an ABI.
4 months
6 months
12 months
Child 1
17
32
40
Child 2
12
19
20
Child 3
15
17
Not available
he will turn to voice in low levels of background noise but he does not understand
any spoken language without the support
of sign. He has become more vocal since
receiving his ABI but has no recognisable
spoken words.
Conclusion
Clearly there is only a small number of
children with an ABI and we are in the
early stages of establishing whether an ABI
provides sufficient auditory information
to allow the acquisition of meaningful
speech and language. The three children
presented here may be considered quite
old when they had their implant and the
outcomes for them are typical of those
seen in children who are implanted late
with a CI. To achieve the maximal potential with an ABI we will probably need to
capture the benefits of auditory plasticity
and provide the implant at a younger age.
This will, of course, present even greater
challenges in the programming of the
device. To this end we have been investigating the use of evoked potentials, both
brainstem and cortical, to assist in the
setting up of the device. To offer the ABI
to even younger children will call upon
the highest levels of skill and experience
from the implant team.
Apart from the notable exceptions, the
results of the ABI for older children and
adults, mostly with NF2, are poor
compared with typical results from CI
users. It is important to emphasise,
however, that from the recipients’ point
of view, the ABI is the only hearing option
available and the benefits achieved with
lipreading and awareness of environmental sounds are regarded as a worthwhile connection to the hearing world. n
References
1. Colletti V, Carner M, Miorelli V, Guida M, Colletti
L, Fiorino F. Auditory brainstern implant (ABI): new
frontiers in adults and children. Otolaryngol Head
Neck Surg 2005;133:126-38.
2. Nevison B, Laszig R, Sollmann WP, Lenarz T,
Sterkers O, Ramsden R, Fraysse B, Manrique M,
Rask-Andersen H, Garcia-Ibanez E, Colletti V, von
Wallenberg E. Results from a European clinical
investigation of the Nucleus (R) multichannel
auditory brainstem implant. Ear Hear 2002;23:17083.
3. Illing RB. Maturation and plasticity of the central
auditory system. Acta Oto-Laryngologica
2004;124:6-10.
4. Long CJ, Nimmo-Smith I, Baguley DM, O'Driscoll
M, Ramsden R, Otto SR, Axon PR, Carlyon RP.
Optimizing the clinical fit of auditory brain stem
implants. Ear Hear 2005;26:251-62.
5. Di Nardo W, Fetoni A, Buldrini S, Di Girolamo S.
Auditory brainstem and cochlear implants: functional results obtained after one year of rehabilitation. European Archives Of Oto-Rhino-Laryngology
2001;258:5-8.
6. Colletti V, Shannon RV. Open set speech perception with auditory brainstem implant?
Laryngoscope 2005;115:1974-8.
7. Colletti V, Fiorino F, Carner M, Miorelli V, Guida M,
Colletti L. Perceptual outcomes in children with
auditory brainstem implants. International
Congress Series 2004;425-28.
8. Colletti V, Carner M, Fiorino F, Sacchetto L, Miorelli
V, Orsi A, Cilurzo F, Pacini L. Hearing restoration
with auditory brainstem implant in three children
with cochlear nerve aplasia. Otol Neurotol
2002;23:682-93.
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86
An Approach to the
Dysmorphic Child
with Deafness
Melissa M Lees,
MBBS, DCH, MRCP,
FRACP, MSc, MD,
Consultant in
Clinical Genetics
Correspondence
Melissa M Lees,
Great Ormond Street
Hospital NHS Trust, Great
Ormond Street Hospital,
London, WC1N 1EH, UK.
E: Melissa.lees@
gosh.nhs.uk
A
child with a hearing loss may have a number of unusual clinical features,
which may help to identify the aetiology of the hearing loss. These features
may form part of a recognisable pattern of anomalies, known as a syndrome.
There are over 200 syndromes with deafness as a feature listed on the Winter-Baraitser
Dysmorphology Database (WBDD). Alternatively, the features may not be recognised as
part of a known condition, and may be the result of an underlying chromosome imbalance, congenital infection, teratogenic effects or other unspecified cause.
down a
Dysmorphology is the study of physical
characteristics in order to diagnose an
underlying disorder. Reaching a diagnosis
gives the family and health professional an
insight into the natural history of that condition, information regarding appropriate
monitoring, management and the inheritance pattern, in addition to access to
patient or parent support groups. While
technology in the genetic laboratories is
increasingly improving our ability to make a
precise syndromic diagnosis, the diagnosis is
usually clinically made or at least suspected,
with expensive genetic tests supporting the
clinical diagnosis only where indicated.
Although the clinician may recognise that
the child or individual may appear ‘different’
from other family members, characterising
the features can be difficult. This article aims
to help guide the clinician through the
somewhat daunting task of assessing the
dysmorphic child with deafness.
particular
History
Declaration of
Competing Interests
None declared.
Whilst the
physical signs
might lead you
diagnostic
pathway, the
findings must be
taken in
conjunction with
the history of
prenatal, prenatal
and postnatal
factors
As with the majority of disciplines within
medicine, the evaluation of a child with deafness and dysmorphic features starts with a
thorough history. A detailed family history
should include three generations where
possible, with specific questions to determine whether relatives may be similarly
affected or have more subtle signs of a
related condition. For example in Stickler
syndrome where the proband may have a
sensorineural hearing loss, the mother may
have had a cleft palate repair. It is helpful to
document any consanguinity, as rare recessive conditions are more common in the
offspring of a consanguineous union.
Information regarding the pregnancy
should be sought, specifically asking about
drugs, medications and alcohol (teratogenic
effects), maternal diabetes or epilepsy, fevers
and rash (possible congenital infection), or
pv loss (vascular compromise in hemifacial
microsomia). Information about the delivery
including mode, gestation and size of the
baby are relevant. Perinatal birth asphyxia,
prematurity and sepsis (meningitis, ototoxicity of aminoglycosides) may all contribute
to hearing loss in a child.
Developmental delay is commonly found
in children with dysmorphic features and a
careful developmental history should be
taken to try and establish whether developmental delay is present, and if so whether
this is global, affecting all areas of development, or more specific such as with speech
or motor development. Poor head control
and delayed walking may be early signs of
vestibular dysfunction, and therefore very
relevant in a child with a hearing loss.
Depending on the age of the child, the
progress of the individual at school is important, noting any additional support received
within the educational environment.
Information on the social integration and
behaviour is helpful.
Significant medical events such as an
admission to hospital for meningitis are
clearly fundamental to a thorough history.
Physical examination
Facial gestalt
Sometimes a child may present with easily
and quickly recognisable features, such as the
facial features seen in trisomy 21, where the
diagnosis can be made by the assessment of
the overall picture of the face. This is also
known as the ‘facial gestalt’.
More commonly, however, although
there may be unusual features present, the
clinician will need to work through the
features individually, before being able to put
the various findings together to consider if a
common link can be found.
87
feature
Head and neck
The head should be measured and the shape
evaluated, by looking from all angles,
including from above. An unusual head shape
may suggest premature fusion of the cranial
sutures (craniosynostosis syndromes include
Apert,
Crouzon,
Saethre-Chotzen
syndromes). Microcephaly is seen in many
conditions including congenital infections.
missing teeth, which may suggest an ectodermal dysplasia. Is there gum hypertrophy? Is
there a cleft palate (Treacher Collins, some
craniosynostosis conditions).
Neck
It is important to look at the neck for any
remnants of a branchial sinus (branchio-otorenal syndrome) or thyroid enlargement
(Pendred syndrome).
Face
Does the face appear symmetrical (hemifacial
microsomia)? Can the face be described as
long, round, or triangular in shape? Is there
undervelopment of part of the face such as
hypolastic zygomatic arches in Treacher
Collins syndrome, or mid-face hypoplasia
(Stickler syndrome)? The shape of the forehead is important when considering certain
conditions for example skeletal dysplasias.
Ears
Do the ears appear normally formed and
positioned? Are they simple in shape or
particularly prominent or cupped? Are the
lobes unusual? Note the presence or otherwise of preauricular pits or tags, post-auricular
skin
defects
(branchio-oculo-facial
syndrome), and ear creases (Beckwith
Wiedemann syndrome).
Eyes
When looking at the eyes the following
should be considered: do the eyes appear
close together or widely spaced, are the globes
prominent or do the eyes appear small, are
epicanthic folds present, do the palpebral
fissures slant up or down, do the eyebrows
meet or are they sparse, are there colobomas
of the iris or eyelid, are the eyes the same
colour?
An example of the importance of the eyes
is in the diagnosis of the type of Waardenburg
syndrome. White forelocks and heterchromia
are common to both type 1 and 2, but
synophrys and telecanthus (lateral displacement of the inner canthi) are seen in type 1.
Nose
Is the nasal bridge wide or flat, or high? Is the
tip of the nose broad or upturned, or bifid?
Looking at the philtrum – is this long, smooth
and featureless, or are the philtral pillars wellpronounced?
Growth
Evaluation of growth is an important part of
the assessment of any child with dysmorphic
features. If short, is this disproportionate with
short limbs?
Skin
The skin provides many clues towards underlying aetiology of a complex disorder. Areas of
hypopigmentation may be seen in
Waardenburg syndrome, café au lait patches
in neurofibromatosis, and multiple lentigines
in Leopard syndrome.
Digits
The hands again provide many diagnostic
clues. Palmar creases may be single (trisomy
21) or deep (mosaic trisomy 8). The digits
may be short (skeletal dysplasias) or long
(22q11 deletion). Fifth finger clinodactyly is
present in trisomy 21, Silver-Russell syndrome.
Fingers or toes may be webbed and the
pattern of the webbing may be important for
example 1/2 toe webbing in triploidy, 4/5
syndactyly in oculo-dental-digital syndrome.
Are the joints hyperextensible, seen in Stickler
syndrome, or are there joint contractures
(facio-audio-symphalangism).
Further evaluation
Clinical photographs are an extremely useful
tool in the assessment of a dysmorphic child,
as these may be further assessed outside the
clinic setting, shared with other colleagues,
presented at a conference or to a web-based
discussion board for a wider diagnostic
opinion, and comparison to published cases
of overlapping conditions.
Many facial features are part of a continuous trait such as distance between eyes,
Assessment pathway
Three generational pedigree
Mouth
Pre- peri- and postnatal history
Is the mouth wide (macroglossia may be part
of branchial arch defects)? Is there evidence of
a cleft lip repair? Are the lips thin (fetal alcohol
syndrome) or full and everted (Coffin Lowry
sydrome), or is the cupids bow prominent?
Are the teeth normally formed or are there
small, underdeveloped, unusually shaped or
Developmental history
88
Physical examination including
growth parameters
Clinical photographs
Books, literature and web-based
resources
broadness of forehead, and so on, and
comment on these may be subjective.
However there are standard values for many
measurable features, which can then be
plotted on a chart to determine the significance, although in a busy clinical this may not
be practical.
Whilst the physical signs might lead you
down a particular diagnostic pathway, the
findings must be taken in conjunction with
the history of prenatal, perinatal and postnatal
factors and supported by appropriate investigations.
In some cases, where a facial gestalt has
been recognised, a clinical diagnosis can be
made which may or may not need confirmation by a laboratory test. In many cases
further assessments are necessary to gain
sufficient information to make a diagnosis. For
example an ophthalmic assessment may
identify vital clues in the diagnostic search
(retinopthy in congenital infections, high
myopia in Stickler syndrome, coloboma in
CHARGE), a renal ultrasound scan in a patient
and sometimes other family members may
confirm a clinically suspected diagnosis of
branchio-oto-renal syndrome, or an echocardiogram where a clinical diagnosis of Noonan
syndrome is being considered.
Interpretation of features
When faced with a child with complex difficulties, in spite of an excellent and detailed
history and documentation of the physical
features, no diagnosis may have come to light.
This may be because the collection of signs
and symptoms do not fall within a recognised
pattern, and may represent a subtle chromosome imbalance or a ‘new’ syndrome.
Alternatively the features may be part of a
recognised pattern but one which is not
familiar to the clinician, and in this situation
the information needs to be further evaluated
using additional resources.
There are a number of excellent reference
books, where the chapters are divided
according to the main features of the group of
conditions, such as external ear malformations. The physical differences identified can
also be entered onto a searchable database
such as the WBDD. The various ‘handles’ may
lead to a number of suggested diagnoses,
which match to some extent the features
identified, which can then be evaluated individually on their own merit as to whether the
patient may or may not have the condition in
question. Some features may be considered
strong ‘handles’, where the particular feature is
only present in a few conditions for example
heterochromia iridis, where others are more
non-specific for example single palmar
creases, and therefore less diagnostically
helpful. A number of key features can be
feature
searched on, making any ‘strong handle’ a mandatory feature.
Using such a tool, a number of diagnostic suggestions are
made, which the clinician can consider in turn, deciding
whether it remains a diagnostic possibility. If a specific
syndrome is being considered, greater evaluation of the literature may be necessary, which may help to clarify whether this
is indeed the correct diagnosis or whether additional investigations may help to confirm or refute the diagnosis.
Unfortunately this is not currently freely available but may be
available within your institution.
It is important to be aware that making a diagnosis prematurely with insufficient evidence is unwise as it may be incorrect and removing a diagnostic label once applied can be very
difficult and distressing for the patient and their family.
Genetic testing
Any child with deafness with additional features such as
dysmorphic features and / or developmental delay should
have a karyotype performed. Some centres are now offering
array CGH (comparative genome hybridisation) as the first
line investigation for chromosomal copy number variation,
which identifies smaller areas of chromosome imbalance than
was previously available through conventional G-banding
techniques. Where a clinical diagnosis is suspected and the
molecular basis known it may be possible to confirm the
diagnosis by DNA analysis. Each individual case needs to be
assessed as to whether the additional information would be
beneficial to the health professional and family, as the costs
involved are often significant. If the condition is rare there may
be a research group working on that disorder which would be
happy to receive a DNA sample. When blood is being taken,
it is often helpful to store a DNA sample in case additional
DNA tests are warranted at a later stage.
Role of the clinical geneticist
It is often both interesting and challenging for the ENT surgeon
and audiologist to try and pinpoint the precise cause of the
hearing loss in a child with complex difficulties or dysmorphic
features. Often, however, in spite of following the above and
obtaining a detailed family history and careful physical examination, no diagnosis has been made. Referral to a clinical
geneticist either to confirm the clinical suspicion or to help
evaluate the clinical picture further may be appropriate. The
geneticist will also be able to suggest appropriate additional
investigations, and use their specific expertise in the assessment of a dysmorphic child to help make a diagnosis, in addition to providing the parents with genetic counselling. n
Further reading
Winter-Baraitser Dysmorphology Database (WBDD), London Medical
Databases [www.lmdatabases.com]
Syndromes of the head and neck, 4th edition. Edited by: Gorlin RJ, Cohen
MM Jr, Hennekam RCM. New York: Oxford University Press; 2001.
Hereditary Hearing Loss and Its Syndromes. Edited by: Gorlin RJ, Toriello HV,
Cohen MM Jr. Oxford University Press; 1995.
Oxford Handbook of Genetics. Bradley-Smith G, Hope S, Firth HV, Hurst J.
Oxford University Press; 2009.
Website resources
Online Mendelian Inheritance in Man (OMIM) provides a catalogue of
human single gene disorders [www.ncbi.nlm.nih.gov/omim]
GeneTests·GeneClinics provides excellent reviews of a broad range of genetic
diseases and syndromes [www.geneclinics.org]
National Organization for Rare Diseases (NORD) provides information and
links to support groups [www.rarediseases.org]
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Correspondence
T: +44 (0)131 557 4184
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Mr Doug Elder,
ENT & audiology news:
a global readership thanks
to our sponsored distribution
partners
ENT & audiology news has one of the largest international circulations among the ENT and
hearing healthcare field globally. Each issue of the magazine has an approximate print run of
16,900 copies, increasing to around 18,000 during the conference season. The magazine is distributed to 140 countries worldwide. We are very proud of this global circulation, which is thanks,
in part, to the unique sponsored distribution scheme we operate in conjunction with the
companies listed on page 93. In this article we thank all those companies – our Distribution
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and to explain how the distribution scheme works.
Subscriptions
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from some of our existing Distribution Partners, who have illustrated a number of benefits of
distributing the magazine.
Since both ENT and audiology
are covered in the magazine, we,
as the distributor, feel that we are
bridging the gap between the
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The readers embraced the magazine warmly as it was bringing
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critical review for the magazine – managing to contact each
other via their contact emails published in the magazine!
Euthymiades Audiology Centre, Cyprus,
a distributor since 2008
We have distributed ENT & audiology news for a long time in
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Dr Janet Leung (pictured)
explains “Receiving the latest
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Widex Hong Kong, distributors for more than 10 years
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Fanne Azarakhsh Co., Iran, a distributor for almost 7 years
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Mr Ganesh, Hansaton Acoustics, India
90
Establishing close relationships with ENT doctors is always one
of Quang Duc Hearing Co Ltd's focuses. ENT & audiology news
has played a valuable part to help us build that link. Our doctors
remember us, and refer to us new customers who need our
services.
Quang Duc Hearing Services Co., Ltd, Vietnam,
a distributor since 2010
distribution
Why do companies undertake
Sponsored Distribution?
Our Distribution Partners say there are a number of benefits for
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distributing ENT & audiology news our partners can:
● Supply a useful, informative, unbiased and educational resource
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receive a regular copy of the magazine
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as an ambassador for local ENT and audiology professionals
Finally, with a reason to communicate with doctors every two
months, our Distribution Partners get a sprinkling of that indefinable magic that has made ENT & audiology news a worldwide
success for two decades. When a market is proving ‘hard to crack’,
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New partners welcome
We particularly welcome enquiries from companies that might
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Distributing such a high profile specialised magazine enabled
Jordan Hearing Aids to do much more within its outreach initiatives in an environment where access to specialists' news and trends
is challenging. We thank ENT & audiology news for their pivotal role
in keeping the ENT care community in touch with the global news.
Raed Billeh, General Manager, Jordan Hearing Aids
In some countries different companies work together, sharing
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by professional interest for instance, with one company delivering
to otolaryngologists and another to audiologists or hearing
healthcare practitioners.
Costs of being a Distribution Partner
As our Distribution Partners help us to reach the widest possible
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A big THANK YOU from the Publisher
This feature is an expression of appreciation of all our
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and sometimes their ingenuity – in ensuring the international
circulation of the magazine. If you already avail of this service,
maybe you would like to let them know that you appreciate it
too. If you do not get the magazine from one of our Distribution
Partners, check out the list on page 15 and apply to join a
scheme. Or if none exists for your country contact Ms Nova
McMillan, Distribution Co-ordinator, to organise a quote, or Mr
Doug Elder for a personal subscription.
With every issue we include a cover letter detailing our products which are advertised inside, and giving some information
on the other ENT products we supply. We are sure the distribution of the magazine has been a considerable help in
achieving our aim of becoming the first port of call for Irish
ENT surgeons when they are in the market for any new equipment or instruments.
Tekno Surgical, Republic of Ireland
We choose to distribute the magazine because we want to take on
our shoulders the responsibility of community services for people in
Kuwait and Oman. One of the most important ways is to increase
awareness and knowledge of the medical field. The magazine is one
of the best informative and accredited sources and so we decided
to sponsor distribution to serve all our ENTs and audiologists.
Hassans Optician Co., a distributor since 2003
ENT & audiology
news is distributed
to physicians and
audiologists across
Israel with great
success. We have
been
receiving
excellent feedback
regarding
the
magazine’s range of topics. The request for new subscriptions
continues to grow and for this reason we intend to continue
and support its circulation around Israel.
Ursula Bayer,
MED-EL’s Marketing Project Manager reads the latest issue.
ENT & audiology news is highly valued in our team as it gives us
an outstanding overview about current developments both in
scientific research and in the hearing device industry. The ‘News
Update’ section regularly informs us about product news of
hearing device manufacturers, the ‘Newsround’ section gives a
perfect insight into hearing events all over the world.
Steiner Hearing Instruments, Israel
MED-EL, Austria
91
distribution
Through delivery of the
magazine we not only
enhance our professional
reputation
amongst customers
and hospital doctors,
but we also support
development of China’s
domestic audiology, to cultivate new audiologists, open their
minds and eyes to new areas and the latest technology.
Starkey China, a distributor since 2001
ENT & audiology news will help us to
grow our businesses. Readers are very
positive about the contents of the
magazine. There are so many articles and
so much information on ENT. The magazine is a very good tool to develop relationships with ENT doctors, audiologists
& dispensers.
Global otorhinolaryngology information can be easily understood and is found useful – we distribute to ENT Professors at
the University Hospital in Osaka. As a result of distributing ENT
& audiology news, our sales of hearing aids and otorhinolaryngology inspection machines have gone well.
Toshin Jitsugyo Co. Ltd, Japan, a distributor for 10 years
Widex India, a distributor since 2001
Our readers declare they are very satisfied with the range of
topics which are presented in the publication. It is a well known
magazine in medical society and for that reason we intend to
support its circulation in Greece.
We continually strive to bring
new and innovative products
to the ENT surgeons. Lanoy
Medical has issued complimentary issues to ENT practitioners in South Africa and
Namibia for over a decade. The
surgeons are kept informed of all that is happening in the field of
ENT surgery, and distributing ENT & audiology news keeps us in
continual contact with our client base.
Lanoy Medical, South Africa
Uni-Pharma S.A., Greece a distributor since 2003
Twenty years on
Now in its twentieth year, it may come as a surprise for
readers to hear that when ‘ENT News’ first launched, we only
expected to circulate the magazine to the UK’s ENT profession. Demand quickly arose however from the audiology and
hearing healthcare professions, as well as from associated
specialists such as oral and maxillofacial surgeons, voice scientists and head and neck cancer nurses. The cross-over
between ENT and the related specialties quickly became
apparent, and the magazine began to cater to this wider
market, ultimately resulting in the magazine’s name change to
ENT & audiology news.
Growth of the international circulation
Within months of the magazine’s launch we began to
receive subscription requests by ‘phone, fax and post from
specialists based overseas. Equally surprised and delighted
by these requests, we soon learned that doctors from overseas would come across the magazine at meetings, or when
visiting colleagues in the UK. Its mixture of events, reviews
and news of the latest industry developments was a hit with
doctors at home and abroad. One Italian doctor, who
‘phoned to arrange a subscription for his friend as a birthday
gift, explained that the short reviews and attractive layout
92
made the magazine accessible and easy to read for readers
whose first language was not English.
The birth of the Sponsored Distribution scheme
As processing new subscriptions began to take up more and
more resources, a time-saving solution was needed to meet
the international demand for the magazine.
Many of the early subscription requests came from the
Netherlands and from constructive discussions, EnterMed
BV became our first official sponsored distributor. This was
quickly followed by Widex who have for many years distributed ENT & audiology news far and wide. The resulting
contribution to the education of, and communication with
ENTs, audiologists and related specialists from Egypt to
Hong Kong and from Brazil to Libya has been invaluable,
and has also ensured the goodwill of the profession
towards those that distribute the magazine around the
globe.
In the following months companies throughout Europe,
The Middle East, Australia and New Zealand came on board
to replicate the scheme. The cumulative result today is that
ENT & audiology news has the largest international circulation
of any magazine in its field. n
international circulation
ENT & audiology news now averages 18,500 readers worldwide thanks, in part, to the following companies who sponsor distribution of the
magazine to the profession outside the UK. Individual readers based in the countries listed below may apply for a complimentary copy of the
magazine from one of our Distribution Partners.
Log onto www.entandaudiologynews.com for direct links to the companies listed below.
Australia
HAC Acoustic Technologies,
Libya
South Africa
Arthrocare (Australasia) Pty Ltd,
TF3 Kokesh Tower,
18 Kodambakkam High Road,
Chennai, India, 600034,
Widex Libya,
Lanoy Medical,
PO Box 10, Jaafer Ben Abitaleb St,
Menshia 7, Tripoli, Libya.
Tel: +218 927310296.
PO Box 3342,
Halfway House, 1685, Johannesburgh.
Tel: +27 (0)11 466 1820,
Fax: +27 (0)11 466 1825,
Building 3, Unit 3, 49 Frenchs Forest Road,
Frenchs Forest, NSW 2086, Australia.
Tel: +61 2 8422 1100,
Fax: +61 2 8422 1102,
Austria
Med-El Medical Electronics,
Furstenweg 77a, A-6020, Innsbruck, Austria.
Tel: +43 (0)512 28 88 89 0,
Fax: +43 (0)512 29 33 81.
Bahrain
Delmon Optical and Audiological
Centre,
PO Box 5201, Manama, Bahrain.
Tel: +973 17 294171,
Fax: +973 17 294476.
Brazil
Indonesia
Melawai Hearing Aid,
Jalan Melawai Raya 192, Jakarta Selatan,
Indonesia.
Tel: +62 21 530 6956 or +62 23 532 1111,
Fax: +62 21 548 0677.
Web: www.widex.co.id or
www.melawai.com
Iran
Fann Azarakhsh Co Ltd,
Malta
No6, 2nd Floor,
15 Ave, Gandi St, Tehran, Iran.
Tel: +98 21 88 77 12 83,
Fax: +98 21 88 77 8473,
Ireland
Rua 24 de Maio 77-2, Amdarconj 202,
Villa Buarque, Sao Paulo Cep 01041-907,
Brazil.
10 Fonthill Business Park,
Dublin 22, Ireland.
Tel: +353 1 675 4800,
Starkey (Suzhou)
Hearing Technology Co, Ltd.
C2,128 Hongye Road, Suzhou Industrial
Park, Jiangsu Province, 215006.
Tekno Surgical Ltd,
Israel
Steiner Hermina Hearing
Instruments Ltd,
Croatia
10 Abbas St, Haifa 31094, Israel.
Tel: +972 485 215 14,
Bontech Research Co doo
Italy
A.B.Simica 48, 21000 Split, Croatia.
Tel: +385 21 371 505
Web: www.bontech.hr
Seda S.p.a.
Cyprus
Euthymiades Audiology Centre Ltd,
Euthymiades Building, 8, Methonis,
1070 Nicosia, Cyprus,
Tel: +357 22 377177, Fax: +357 22 377161,
Web: www.euthymiades.com.cy
Denmark
Widex A/S
-Via Tolstoj, 7, 20090 Trezzano
sul Naviglio (MI), Italy.
Tel: +39 02 48 42 41,
Fax: +39 02 48 42 42 90,
Web: www.seda-spa.it
Japan
Starkey Japan Co, Ltd.
5-2-20 Nakamachidai, Tsuzuki-ku,
Yokohama, Kanagawa 224-0041.
Tel: +81 45 942 6593, Fax: +81 45 942 7158,
Web: www.starkey-japan.co.jp/
Toshin Jitsugyo Co Ltd,
Nymoellevej 6,
DK-3540 Lynge, Denmark.
Tel: +45 44 35 56 00, Fax: +45 44 35 56 01.
2-4-7 Nishihonmachi,
Nishi-Ku, Osaka, 5500005, Japan.
Egypt
Azuma Hearing Aid Center Co, Ltd,
Widex Egypt,
25 El-Khalifa El-Maamon St, 2nd floor,
Flat No 11, Roxy, Cairo, Egypt.
Tel: +202 2918421, Fax: +202 6905758.
Greece
Uni-Pharma SA,
14 KLM National Road,
145 64 Kifissia, Athens, Greece.
Tel: +30 210 8072512 or +30 210 8072534,
Fax: +30 210 8078907,
Hong Kong
Widex Hong Kong Hearing & Speech
Centre Ltd,
Room 1101-2, Hang Shing Bldg, 363 Nathan
Road, Yaumatei, Kowloon, Hong Kong.
Tel: +852 2771 0501, Fax: +852 2388 4627,
India
Widex India Private Limited,
DLF Infocity, Tower 'D', 2nd Floor,
Chandigarh Technology Park,
Chandigarh,-160 101 (INDIA),
Tel: + 91 172 5000201,
Fax: + 91 172 5042075,
Top Hearing Centre LOT SB6 to SB9
Lower Ground Floor, Bangunan Cahaya
Suria, Jalan Tun Tan Siew Sin (Jalan Silang),
50050 Kuala Lumpur, Malaysia.
Tel: +60 3 207 80204, Fax: +60 3 207 23632,
Malaysia: Email: [email protected]
or [email protected]
Web: www.widex.com.my
Centro Auditivo Widex-Brasitom
Ltda,
China
Malaysia
2-2-18Sakai-Cho, Niihama,
Ehime 7920812, Japan.
Tel: 0897 35 2277.
Beacon Healthcare Ltd,
‘Sea Breeze’, Qawra Coast Road,
Qawra SPB 05, Malta.
Tel: +356 576 171, Fax: +356 419 300,
New Zealand
Widex New Zealand Ltd,
PO Box 46-323, Herne Bay, Auckland,
New Zealand.
Tel: +64 9 360 3412, Fax: +64 9 360 3426,
Nigeria
Otana Hearing and
Edu-Health Services,
Otana House, 69 Wawuna Street
(off Obi Wali Road), Rumugibo,
PO Box 8867, Port Harcourt, Nigeria.
Tel: +234 803 339 7678,
Fax: +234 803 505 6602,
Sri Lanka
D S Jayasinghe Opticians (Pvt) Ltd,
41 Symonds Road, Colombo 10,
Sri Lanka.
Tel/Fax: +94 1 449780 or +94 1 446518,
Sudan
Sudanese Hearing Centre,
Altayar Gameel Street Cross al said Abdel
Rahman Square 6 Home 3,
PO Box Albarari Khartoum, Albarari.
Tel: +249 11 79 76 78,
Fax: +249 11 79 67 67,
Taiwan
Melody Medical Instruments Corp,
2F-1, No191, Section 4,
Chung-Hsiao East Road, Taipei,
Taiwan 106.
Tel: +886 (2) 2741-9117 x216,
Fax: +886 (2) 2741-9139,
Pakistan
Thailand
Rehabilitation Centre for Hearing
Impaired,
D MED Hearing Center Co, Ltd.
Ghafoor Plaza, 289 Ferozepur Road,
Lahore, Pakistan.
Tel: +92 42 588 3773, Fax: +92 42 585 5585,
Web: www.widex.com/pk
Phillippines
Ledesma Audiological Center,
Lower Level Makati Cinema Square, Pasong
Tamo Street, Makati City, Phillippines.
Tel: +63 2 818 8564, Fax: +63 2 811 1255,
Web: www.ledesma.com.ph
Portugal
Casa Sonotone,
326,326/3-4 Sukhothai Rd. Suanchitladda,
Dusit, Bangkok 10300.
Tel: +66 (0)2668 1300 3,
Fax: +66(0)2668 1304.
Web: www.widex.co.th or
www.dmedhearing.com
Turkey
Starkey Turkey
– Golden Star Ltd,
Halaskargazi Cad, Teyyareci,
M Alibey Sk. No1/2 80260 Sisli,
Istanbul, Turkey.
Tel: +90 212 230 4532 or
+90 212 232 1755
or +90 212 232 3287,
Fax: +90 212 230 4502.
PO Box 2208, Amman 11181 – Jordan,
4th Circle, Ibn Khaldoun St,
Building 77.
Tel: +962 6 461 1331
Praça da Batalha 92-1, 4000 Porto, Portugal.
Tel: +351 223 3930 60,
Fax: +351 223 323 091.
Acustica Medica
Rua Conde De Arnoso
No 5-2 Piso, 1700-112, Lisbon.
Kenya
Romania
Widex Emirates Hearing Care,
Beam Hearing Centre,
Sonorom SRL,
Adalyn flats – 4B, Ngong’ Roads,
PO Box 72073, Nairobi, Kenya.
Tel: +254 2 722 861,
Str. Unitatii 130, Bloc B4, Ap. 106,
Sector 3, Bucuresti 74355, Romania.
Tel: +40 1 337 0942/335 7562,
Fax: +40 1 337 0942,
PO Box 26402, Sharjah,
United Arab Emirates.
Tel: +971 6 5744734,
Fax: +971 6 5744735,
Saudi Arabia
Vietnam
Jordan
Jordan Hearing Aids Man Co,
Korea
Starkey Korea,
656-766 Sungsoo, 1-Ka 2-Dong,
Sungdong-Ku, Seoul, 133-112, Korea.
Tel: +82 2 465 0999, Fax: +82 2 465 1012,
Web: www.starkey.co.kr
Al-Madina Hearing Aids Centre,
Kuwait & Oman
Slovenia
Hassan’s Optician Co,
Neuroth sluš ni aparati d.o.o.
PO Box 1139, Safat 13012, Kuwait.
Tel: +965 242 1818, Fax: +965 242 1815.
Tbilisijska 59, 1000 Ljubljana, Slovenia,
www.neuroth.si
Old Airport Road, PO Box 6812,
Riyadh 11452, Saudi Arabia.
Tel: +966 1 478 8809,
United Arab Emirates
Quang Duc Hearing Services Co, Ltd.
384 (new no.1056) CMT 8,
Ward 4, Tan Binh District,
Ho Chi Minh City, Vietnam
Tel: +84 8 3844 6415 / 3948 5919
Fax: +84 8 3811 9668
Web: www.qdhs.vn
93
contributors
Graham Sutton
Dr Ezeddin El Tabal
Michelle Wyatt
Blake C Papsin
Jane R Madell
Laura Coleman
Dr Doris-Eva Bamiou
Mary-Louise Montague
Sharon L Cushing
Elizabeth Tyszkiewicz
Helen Williams
Dr Tony Sirimanna
Mr Joe Grainger
Adam J Donne
Karen A Gordon
Kate Parkes
Pauline Grant
Michael Kuo
Michael P Rothera
Gavin Morrison
Cherilee Rutherford
Jane R Madell
Ann-Louise McDermott
Konstance Tzifa
Josephine Marriage
Melanie Gregory
Patrick Sheehan
Sue Archbold
Mr Tom Beech
Kate Hanvey
Guy Lightfoot
Mr John Graham
Ann-Louise McDermott
Martin O’Driscoll
Melissa M Lees
Mr Iain Bruce
Louise Melia
Miss Sujata De
Haytham Kubba
Fiona B MacGregor
Mr William PL Hellier
Priya Singh

audiology - ENT and Audiology News

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