Otology Panel – Difficult Otologic Cases

ASK THE EXPERTS: OTOLOGY
William H. Slattery III, MD
Los Angeles, CA
Wayne K. Robbins, DO
Grand Blanc, MI
Richard Goode, MD
Palo Alto, CA
Mark E. Reader, DO
Porterville, CA
Kami K. Fehlig, AuD
Spokane, WA
Mark J. Van Ess, DO
Springfield, MO
Case 1 - BG

42 Y/O white male sales manager seen by a local
ENT 7/2000 w/ complaint of change in hearing in
left ear
Progressed over period of weeks w/ periods of
fluctuation
 Associated w/ left tinnitus but no vertigo or disequilibrium
 Had been treated w/ course of oral prednisone w/out
improvement in hearing



PMHx IgA nephropathy
Social Hx: Denies tobacco & EtOH

MRI ICAs w/ w/o contrast


“negative”
Lab work

Electrolytes, and Thyroid panel – normal
Case BG

10 / 2000 after first evaluation
 ENG
showed 18% Left weakness
 No ECoG available
 All labs normal (ANA, Sed Rate, VDRL, TSH, Lipids,
BUN, CREAT and UA)


No hearing changes on Prednisone
Two weeks after starting on Dyazide Pt presents for
audiogram due to improved hearing
 SRT
improved from 65 dB to 35 dB primarily in low
frequencies
Case BG

7 / 2001 Pt seen by otology only pertinent finding
was right-beating nystagmus on head shake
 Audiogram
had deteriorated
 ELS surgery was offered but not recommended based
on results

12 / 2002 BG seen after a first bout of acute
vertigo that lasted 8 hours and improved using
antivert from PCP
 Pt
noted significant improvement in hearing after acute
vertigo attack
 Restarted on Dyazide and discontinued antivert
 Follow up ENG shows 33% Left weakness
Case BG


From 2/ 2003 thru 6/ 2006 BG was stable with
continued mild (mostly subjective) fluctuation in
hearing, aural fullness and tinnitus but no vertigo
6/ 2006 Several episodes of vertigo of shorter
duration aggravated by flying
 Pos
fistula test Left
 CT scan negative for canal dehiscence
 ME exploration neg for fistula
 BAHA placed for single sided deafness left ear

3/ 2008 persistent vertigo w/ flying
 Treatment?
BG MRI
July 2000
March 2008
BG MRI
Right Intra-labyrinthine Acoustic Neuroma
July 2000
March 2008
Case Presentation

HPI
 51
y/o female Hx “right ear surgery” 11/29/2011 for
cholesteatoma and meningoencephalocele
 Presents 09/05/2012 w/ c/o AD non-fluctuating, nonprogressive hearing loss present since initial surgery unchanged
 Denies
 Pt
otorrhea, otalgia or vertigo
had been scheduled for “2nd look” and staged OCR
but had not proceeded w/ surgery
PMH/PSH

PSH

AD T-mast CWI/Reconstruction w/o OCR w/ TM cartilage
graft w/ repair of meningoencephalocele (middle fossa
craniotomy)




AD cholesteatoma, Incus & Malleus Erosion – (removed),
meningoencephalocele (middle fossa craniotomy – bone repair
tegmen defect)
Planned – 2nd look T-plasty w/ OCR by primary surgeon – pt did not
undergo procedure
T&A, hysterectomy, appendectomy, hip
PMH

Asthma, OSAS, DM II, HTN, Depression
Physical Exam

HENT:
 AD:
EAC w/o mass/lesion or abnormality, TM intact,
thickened graft, unable to visualize through TM
(suspect cartilage graft beneath TM), poor
movement to pneumatoscopy
 AS: EAC unremarkable, TM intact, ME clear
 Tuning fork: 512 Hz
 AD
– BC > AC
 AS – AC > BC
 Webber lateralized right
Last audiogram May 2011 *
Speech Reception Threshold and Word Recognition: live voice
Right Ear: 40 dB HL with 100 % word recognition at 75 dB HL with 45 dB masking
Left Ear: 5 dB HL with 100 % word recognition at 40 dB HL with no dB masking
Cholesteatoma

Imaging
 Role



of CT & MRI
 Role
of adhesive
barrier(s)
 Silastic,
Facial nerve monitor
Canal Wall
Staging/”2nd look”

gelfilm, other
Ossicular chain
reconstruction
 Primary
or staged
Case 3

65 y/o Caucasian male
 B/L
slowly progessive, non-fluctuating hearing loss >
20 years
 Patient wears bilateral hearing aids for > 15 years
 Denies
vertigo or otalgia
 Admits to right sided otorrhea
 Previous bilateral ear surgery
PSH/PMH

PSH
 B/L
ear surgery
 Left
- canal wall down Tympanomastoidectomy
 Right - tympanomastoidectomy (canal wall intact)
 CABG

PMH
 CAD,

COPD, HTN, hypercholesterolemia
Allergies
 Levaquin
Physical Examination

HEENT
 Right
ear: Chronically thickened TM w/ granulation
tissue and poor movement w/ pneumatotoscopy.
Unable to visualize middle ear space
 Left
ear: Canal wall down cavity w/ intact TM and
ossicular chain. Middle ear clear of effusion. TM
retracted superiorly onto malleus head, incus body
and long process down onto stapes and facial nerve
Audiometric Testing

Speech Reception Threshold
 Right:

Speech Awareness
 Right

90 dB HL; Left: 85 dB HL (live voice)
at 60 dB HL; left at 75dB HL
Word Recognition Testing
 Right:
36 % at 95 dB HL (0 dB masking)
 Left 36 % at 100 dB HL (0 dB masking) (live voice)
AzBio sentences were completed at 60 dB using recorded
voice and patient’s own hearing aids. He obtained a score of
14%.
Case 3

? Implant candidate?

Choice of ear(s)?

Further considerations/work-up?

Choice of device?
Vaccine Recommendations
http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5231a5.htm#tab
http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5909a2.htm#tab1
http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5909a2.htm#tab2
Case 4

HPI
 63
y/o cauc male c/o 2 month duration AS tinnitus
 Constant,


 (+):
“high frequency”, non-pulsatile
Intensity 8-9 of 10 – non- fluctuating
Aggravating factors – noise exposure, stress
B/L, gradual-onset, slowly worsening, non-fluctuating HL,
present for years, significant noise exposure, AS aural
fullness and subjective decrease in left sided hearing
sensitivity
 (-): dizziness/vertigo, headaches, otalgia/otorrhea, previous
ear surgery
History

PMH
 Polio,
Hepatitis, Measles, Mumps as child
 Osteoarthritis

PSH
 Hip

(2007), “surgery for polio” (1950’s, 1960’s)
Allergies
 NKMA

Meds
 Alendronate
ginger root
Sodium, Flonase, fish oil, MVI, milk thistle,
Physical Exam

HEENT
 Unremarkable
 EOMI,
PERRL, no nystagmus
 EAC patent, TM’s intact, ME clear bilateral,
pneumatotoscopy w/ good TM mvt w/o vertigo/nystagmus

Slight temporary decrease in intensity of tinnitus w/ positive
pressure pneumatotoscopy – lasting 10 – 20 seconds duration
 Tuning


forks
512 Hz – AC > BC bilateral (Rinne Positive)
512 Hz – Webber midline
Testing
Testing

Audiogram, Tympanogram
 Stapes


reflex
Intact Ipsi & Contralateral Intact
Neurotologic Evaluation
 IR
Video Oculography

No evidence spontaneous nystagmus, gaze evoked nystagmus, post
head-shake nystagmus or nystagmus noted after rapid positioning w/
Dix-Hallpike postitioning tests

Vestibular occular reflex (VOR) present & symmetric bilateral
Case 4

Other testing of value?
 If
yes, which test(s)
Diagnosis

Assessment
 AU
SNHL – High Frequency Mild
 Unilateral
2

tinnitus, AS
months duration, very bothersome to patient
Recommendations for patient?
Recommendation(s)

Further testing
 MRI


of IAC’s w/ gadolinium  “unremarkable”
Discussed observation, noise protection, +/amplification
Other options?
Recommendation(s)

Positive pressure pneumatotoscopy  decreased
intensity of tinnitus and “normal” MRI of IAC’s, no
change on repeat audiogram 4 weeks initial follow
up
 Trial

IT Dexamethasone (24 mg/ml) perfusion
Single IT treatment – topical phenol
Follow-UP

1 week follow-up post IT dex

Significant decrease in intensity of tinnitus



From initial 8-9 of 10 to 2-4 of 10
Decrease in aural fullness w/ subjective improvement in hearing –
audiogram unchanged
6 week follow-up following 4 IT Dexamethasone treatments


Symptoms stable (decreased: intensity of tinnitus – 2 of 10 to  not
noticeable – and aural fullness resolved), Cochlear hydrops???
@ 1 year post treatment – symptoms remain improved
Case 5

50 y/o cauc. Female
L
sided (unilateral), persistent, non-pulsatile tinnitus
 Present
x 3-4 years
 Slowly increasing in intensity

Now 6-7 of 10 in annoyance/intensity
Case 5

HPI
 Unilateral
tinnitus
 Denies
hearing loss, vertigo, imbalance, aural fullness,
otalgia, otorrhea or previous ear surgery

PMH/PSH
 Unremarkable

Soc Hx
 Works
at casino blackjack dealer, + smoker, +EtOH
Case 5

Stapedial reflexes
 Present
– ipsilateral and contralateral

Other testing of value?

MRI of IACs with and without contrast
Case 6

HPI

50 y/o cauc female w/ 1 month history episodic whirling
vertigo minutes to hours in duration. ~ 1-2 episodes/wk.
Most recent episode ~ 24 hours prior to examination. Tx w/
valium, promethazine PRN.

(+): 6 + episodes disabling vertigo associated w/ gradually
worsening AS HL (worse following each episode vertigo over last
month), AS tinnitus, AS aural fullness, nausea & vomiting, moderate
improvement in symptoms w/ valium & phenergan

(-): otorrhea, otalgia, headaches, loss of consciousness,
syncope/near-syncope
History

PMH: Allergic rhinitis
 Allergies
 NKMA
 Medications
 Allegra,
Valium, Promethazine
 PSH
 Foot

surgery
Soc Hx
 Married,
Denies EtOH, tobacco, caffeine
Physical Exam

HEENT
 EOMI,
PERRLA, No nystagmus (w/ fixation)
 EAC
patent, TM intact, ME clear AU, pneumatotoscopy –
good TM mvt, negative fistula test
 512
Hz tuning fork
 Weber
lateralized AD, Rinne AC > BC (positive) AU
Physical Exam

Neurotologic Evaluation
 IR
Video Oculography (removal visual fixation)

No evidence spontaneous nystagmus

Horizontal right beating gaze evoked nystagmus present w/ rightward gaze


Not present in centric or left-ward gaze
Horizontal right beating post head-shake nystagmus

Greater in intensity than nystagmus elicited by right-ward gaze
Testing

Further testing?
 EcOG,
ABR, VNG, VEMP, DVA, VAT, etc.?
 Lab
testing?
 MRI
of IACs w contrast?
Testing

Stapes reflex
 Ipsi

& contralateral intact
MRI of IACs w/ gadolinium contrast
 “Unremarkable”

No evidence intravestibular/intracochlear lesion, no evidence IAC
mass/lesion, no intracranial mass/lesion
Assessment

Diagnosis
 AS
Meniere disease
 Acute
unilateral vestibular weakness, AS
Management

Treatment options
 “Lifestyle”/medical
 Diuretics,
 IT
v. “surgical”?
Betahistine, etc.?
therapy?
Management

IT dexamethasone 24 mg/ml
 Single
trans-tympanic injection, valium PRN
 Initial
improvement immediately following 1st treatment w/
relief vertigo attacks, aural fullness & tinnitus x 3 days 
return of symptoms
Management

Following 1st perfusion recurrent vertigo, HL, aural
fullness, tinnitus returned
 2nd
– 4th dexamethasone perfusion (q weekly)
 Vertigo

relief x 4 weeks
Recurrent episodes intense vertigo lasting seconds in duration
made worse w/ positional changes/rolling over in bed
Repeat Examination

Neurotologic Evaluation

IR Video Oculography (removal visual fixation)

No evidence spontaneous nystagmus

No gaze evoked nystagmus w/ right-ward or left-ward gaze

Horizontal Left-beating post head-shake nystagmus

Dix-Hallpike testing


Head turned left  Horizontal, left beating (geotrophic) nystagmus
Direction changing w/ head turned right 

Horizontal, right beating (geotrophic) nystagmus – less intense than w/ head
turned left
Management

Recurrent vertigo following series of 4
dexamethasone perfusions
 Positional
 MD
in nature
vs. ? Other etiology
 Repeat
testing/further testing?
Management

AS HSC BPPV - cupulolithiasis
 Positioning
 Log


maneuvers
roll
Positional vertigo resolved @ 1 week follow up
AS MD
 Observe
Repeat Examination

2 weeks post AS HSC BPPV treatment
 Patient
presents again c/o repeated episodes vertigo
(hours in duration) associated w/ aural fullness, HL,
tinnitus, not associated w/ changes in position/rolling
over in bed
 Currently
nauseated w/ decreased hearing, increase in
aural fullness/tinnitus and vertigo
Physical Exam

Neurotologic Evaluation
 IR

Video Oculography (removal visual fixation)
Spontaneous horizontal, left-beating nystagmus (increasing in intensity
w/ removal visual fixation)

Increasing in intensity w/ gaze to left and decreasing in intensity w/
gaze to right
Assessment

AS MD - worsening
 Post

Dexamethasone perfusion x 4
AS HSC BPPV – cupulolithiasis
 Resolved
Management

Further testing?

Repeat Dexamethasone perfusion?

ELS, VNS, Gent, other?
Management

AS gentamicin perfusion
 0.2
7
ml 80 mg/ml trans-tympanic x 1
minutes supine
1 week f/u Post Gent Perfusion

Pt denies vertigo, c/o disequilibrium
 Hearing
improved, aural fullness resolved, tinnitus
improved
 Neurotologic
exam (IR video oculography)

Spontaneous horizontal, right-beating nystagmus

Horizontal right-beating nystagmus increasing in intensity w/ rightward gaze

Less in left-ward gaze
3 Month Follow – up

No further episodes vertigo
 Disequilibrium
 Playing
 Aural
resolved
volleyball & running on treadmill
fullness resolved
 Minimal,
constant tinnitus present AS (2-3 of 10 down
from 8-9 of 10 @ greatest intensity)
 Hearing
improved
1 year follow - up

Patient w/ recurrent episodes of
“dizziness”/imbalance – several per month, lasting
seconds in duration
 Fluctuating
aural fullness and HL left ear
 Tinnitus present and unchanged
 Continues PO Maxzide

Also w/ c/o nasal itching, nasal drainage,
obstruction, sneezing, watery eyes, etc.
Follow up

Exam unchanged
 No
nystagmus post head-shake testing

Hearing unchanged on repeat audiometric testing

Treatment
 Role
of allergy testing/treatment