ûInner ear disease • Vertigo • Sensorineural hearing loss • Tinnitus

Inner ear disease
• Vertigo
Recurrent episodes
Jack J Wazen MD FACS
Herbert Silverstein MD FACS
Silverstein Institute
Ear Research Foundation
Sarasota, Florida
Vestibular Meniere’
Meniere’s disease
Recurrent vertigo, no hearing loss, hx of migraine headache,
allergies
Bilateral Meniere’
Meniere’s disease(autoimmune
IED)
Delayed endolymphatic hydrops (DEH)
Trauma or viral deafness. years later develop
DEH in same or opposite ear
Treatment
• Acute attacks
Lorazepam (Ativan 0.5mg) Sublingual
Better than Meclizine
Compazine or phenergan suppository 25mg
Bed rest
Reassurance
• Sensorineural hearing loss
Usually low frequencies early
• Tinnitus
• Aural fullness
Etiology- unknown, allergic, migraine
variant
Inciting causes, stress, excess salt,
seasons, weather, and unknown
Pathology
• Dilatation of the membranous labyrinth
• Excess volume endolymph
increased secretion or
reduced absorption endolymphatic sac
Surgical indications for gentamicin
perfusion with MicroWick
• Recurrent vertigo attacks main complaint
• Unresponsive to medical treatment
• Serviceable hearing in opposite ear
• Minimal unsteadiness
• Unilateral disease
Classical Meniere's’
Meniere's’ disease Vertigo
attacks, low frequency hearing loss,
tinnitus and pressure (Unilateral)
Cochlear Meniere’
Meniere’s disease
• Same as classical, no vertigo
Treatment
• Between attacks
Dyazide qd diamox 250mg bid or hydodiuril 50mg/day
Reduce salt intake
Early casecase- prednisone 60mg/d x 2 weeks
Betahistine 8mg tid.
Allergy work up
Inner ear perfusion Dex 10 mg/cc one month
MethodMethod- Gentamicin
• Surgeon injects the 1st time(69801)
• Patient instills 2 drops into the ear canal
t.i.d.
• Weekly visits to titrate the end point
Air VNG, HT, Ecog
• 2-4 weeks of treatment
224 patients with disabling Meniere
disease treated between May 1996 and
June 2007
•
•
Average 47 months follow up, 22 of 24 patients
(88%) showed unchanged or improved SDS
(statistically significant; p = 0.009)
•
•
Minimum 24 months followfollow-up
No previous otologic surgery for Meniere
disease
Patients with stage 1 Meniere disease treated with low
dose continuous gentamicin (10mg/mL) perfusion with
the MicroWick
•
Stage I Meniere disease (PTA < 25 dB)
•
Appear to have:
Similar vertigo control
Better hearing preservation
Better overall satisfaction than patients with
advanced stage disease
Results
• All 28 patients (normal hearing/mild hearing loss)
improved/resolved - 23 patients (82%)
unchanged - 5 patients (18%)
• Subgroup, 17 patients with normal hearing
improved/resolved in 16 (94%) patients
worsening of symptoms - 0
decreased hearing - 0
Stage 22-4 Meniere disease (PTA > 25 dB)
•
Average 89 months followfollow-up, 116 of 200 patients
(59%) showed unchanged or improved SDS
Mean PTA loss of 11 dB
Seventeen patients (71%) reported complete or
improved vertigo control
•
148 patients (74%) reported complete or improved
vertigo control
Sixteen patients (89%) reported an improved
quality of life (GBI)
•
33 patients (56%) reported an improved quality of life
Mean PTA loss of 8 dB
Low frequency hearing loss
May fluctuate
Tinnitus high or low frequency
Pressure or fullness
Vertigo none or occasional attacks
Conclusions
• Early form of Mé
Méniè
nière disease with fullness
/stuffiness in ear and normal hearing.
• The improvement in a high % of our
patients suggest an inflammatory or
immune mediated etiology
• Early treatment may prevent or delay
onset of full blown Meniere disease
Indications for one month dexamethasone
perfusion
44-10mg/cc 2 drops
t.i.d. with MicroWick
• Unresponsive to medical treatment
• Hx of ulcer, hypertension, diabetes
• Avoid systemic effects of steroids
• SubSub-clinical endolymphatic hydrops
Advantages
• Little systemic side effects
• Can be used when there is diabetes,
hypertension, or ulcers
• Does not cause hearing loss
• If vertigo develops can still use gentamicin
single injections or with MicroWick
Vestibular neurectomy
• Indications
Gentamicin failure
Persistent vertigo attacks
Can undergo general anesthesia
Balance normal between attacks
Unilateral disease usually
Serviceable hearing in opposite ear
Association between vertigo and migraine
•
•
•
•
1873 Liveing
1907 Gowers et al
1926 Symonds et al
1968 Graham et al
1961 Bickerstaff introduced Basilar Migraine
concept
Vestibular migraine, first defined by
Neuhauser et al in 2001, is a condition of
episodic vertigo linked to migraine headache.
Its acute vestibular symptoms are
indistinguishable from MD
Headaches of all types occur in 70-76% of
patients with MD
Migraine specifically occurs in 22-56% of
classical MD
The prevalence of Vestibular Migraine : 13% of the population
Prevalence of Menieres disease is 0.2%
Vestibular neurectomy
• Complications
Infrequent
CSF leakleak- 1%, none since 1988
Infections rare
Meningitis, facial paralysis, deathdeathnone
Imbalance difficultydifficulty- 2%
Second most common cause of recurrent
vertigo after BPPV
Indications for transtrans-canal labyrinthectomy
• Persistent vertigo and poor hearing
• Gentamicin failure and poor hearing
Must remove the Utricle
Prevalence of migraine:
• Males: 4-6%
• Females: 11.2- 17.2% (20% in age 20- 49 years)
6–7% of patients in neurological dizziness
clinics
Prevalence of vertigo and non-vestibular
dizziness: 23- 29.5%
9% of patients in a migraine clinic
Migraine and dizziness correlate more
frequently than could be expected by
chance
The epidemiological link between migraine
and vestibular symptoms and signs
suggests shared pathogenetic
mechanisms
1992 Cutrer and Baloh
•
2 Mechanisms
1. spreading wave of depression and/or
transient vasospasm.
2. neuroactive peptides
Spreading depression theory:
• Stimulus/trigger (chemical, mechanical) results in
a transient wave front that suppresses central
neuronal activity.
• Spreads in all directions.
• Large ion fluxes (↑ K+ intracellular,
↓ CA++ extracellular)
• Reduction in cerebral blood flow
• Aura during spreading wave of cortical depression
Migraine without aura (formally called common
migraine) –At least 5 attacks including:
• Headaches lasting 4-72 hours
• Has at least 2 of the following characteristics:
Unilateral location
Pulsating quality
Moderate or severe
Aggravation by activity
• During headache, at least 1 of the following occurs:
Nausea and/or vomiting
Photophobia and phonophobia
Other disorders excluded by exam or appropriate
diagnostic studies
Migraine headaches begin years before
vertigo usually
There might be a migraine free period in
between
Vertigo may be the initial symptom of
migraine
Positive family history
Recurrent vertigo and migraine headaches
can be independent
Vasospasm of the internal auditory artery causes ischemia
to the labyrinth
Migraine has been shown to lead to isolated infarction of
the inner ear probably through vasospasm of the small
arteries.
Migraine may cause vasospastic microvascular ischemic
damage to the inner ear, hearing loss, and susceptibility to
developing ELH
The role of migraine in SSNHL has been proposed based
on the association of migraine with amaurosis fugax,
hemiplegia, angina, and visual auras.
Migraine with aura (formally called classic
migraine) –At least 2 attacks including:
• 3 of the 4 following characteristics:
One reversible aura symptom indicating focal CNS
dysfunction (i.e., vertigo, tinnitus, decreased hearing,
ataxia, visual symptoms in one hemifield of both
eyes, dysarthria, double vision, paresthesias,
paresis, decreased level of consciousness)
Aura symptom that develops gradually over more
than 4 minutes or 2 or more symptoms that occur in
succession
No aura symptom that lasts more than 60 minutes
unless more than one aura symptom is present
Headache follows aura in less than 60 minutes
Other disorders excluded by exam or appropriate
diagnostic studies
• Long history of motion intolerance during car,
boat or air travel
• The duration of the vertigo is variable:
7% experience vertigo for seconds.
31% for minutes up to 2 hours.
5% for 2-6 hours.
8% for 6-24 hours.
49% for longer than 24 hours.
• Continuous rocking sensation for weeks to
months
Others: Central and peripheral deficits are
secondary to release of neurotransmitters
such as calcitonin- gene-related peptide
during the migraine attack.
Ion-channel dysfunction and calcium
channel disturbances of the inner ear and
its central connections could be implicated
? effect on treatment
Other categories:
• Migraine with prolonged aura - Fulfills criteria for
migraine with aura but the aura lasts more than 60
minutes and less than 7 days
• Basilar migraine (replaces basilar artery migraine) -
Fulfills criteria for migraine with aura but 2 or more
aura symptoms of the following types occur: vertigo,
tinnitus, decreased hearing, ataxia, visual symptoms in
both hemifields of both eyes, dysarthria, double vision,
bilateral paresthesias, bilateral paresis, and decreased
level of consciousness
• Migraine aura without headache (replaces migraine
equivalent or acephalic migraine) - Fulfills criteria for
migraine with aura but no headache occurs
Vertigo usually rotational but could be “to
and fro sensation”
Lightheadedness
Dizziness
Giddiness
Floating sensation
Motion sickness like
Swimming or rocking sensation
Postural imbalance but can walk alone
Common but not severe
Phonophobia (85%), to be differentiated from
Vertigo exacerbated by motion
recruitment and hyperacusis of MD
Mild to moderate loss rarely progresses
Positional vertigo
• Unilateral or bilateral
Aural fullness
Fluctuating hearing loss
Visual vertigo
No diagnostic tests exist!
•
•
diagnosis is made by clinical history
If unclear diagnosis, by therapeutic response to
treatment.
Evidence of peripheral vestibular weakness
favors MD
Progressive SNHL favors MD
No differences with posturography, VEMP,
and oVEMP
Tinnitus
Diagnosis of migrainous vertigo requires
ALL of the following:
1. Lifetime diagnosis of migraine
2. Vestibular symptoms that:
• a. Are intermittent, not constant, AND
• b. Are more than simple dizziness (e.g., vertigo,
illusory motion, or head motion intolerance), AND
• c. Interfere with daily activities, AND
• d. Are not caused by identified pathology
Abnormalities have been seen on :
Caloric testing (directional preponderance)
Ocular-motor testing
Rotational testing (asymmetric nystagmus
responses )
Positional testing
Posturography
Usually do not indicate a definite
peripheral or central vestibular lesion
3. One or more migraine symptoms has
occurred with episodic vestibular attacks:
• a. Migraine headache, OR
• b. Photophobia, OR
• c. Phonophobia, OR
• d. Aura (other than dizziness)
4. No hearing loss or neurologic or otologic
pathology to explain balance abnormalities
(E.g., patients with Meniere's disease would
not be diagnosed with migrainous vertigo)
Symptom
Furman et al developed a diagnostic
algorithm
Definite migrainous vertigo
• meet International Headache Society criteria for
migraine,
• episodic or fluctuating symptoms highly
suggestive of a vestibular disorder
Probable migrainous vertigo
• episodic or fluctuating vestibular symptoms
suggestive of a vestibular disorder
• no known causes of vertigo, and
Ménière Disease
Usually lasts up to 24 hours
May last > 24 hours; prolonged rocking
sensation may persist for weeks or months
Sensorineural
Hearing Loss
Most often unilateral; fluctuating; almost
always progressive
Rarely occurs but if present, rarely progressive;
may fluctuate in basilar migraine
Tinnitus
Unilateral or bilateral; intensity varies
May occur (usually bilateral) but rarely disturbing
Aural Fullness
May occur prior to/during acute attack only
or chronic
Rarely occurs
Phonophobia
May occur after or between acute attacks;
unilateral or bilateral
Often present; usually bilateral
Photophobia
Never present unless concurrent history of
migraine
Often present
Headache
Rarely occurs
May or not be present with vertigo; can occur
with/without aura
not simply dizziness, giddiness, or light-headedness,
• no known causes of vertigo
• experience at least one of a set of migrainous
symptoms during at least two vertiginous attacks.
• symptoms highly suggestive of migraine
Migraine-Associated Vertigo
Vertigo
Diarrhea
Often occurs during acute attack
Nausea/Vomiting
Frequently occurs during acute attacks
May occur with vertigo
History of motion
sickness/ childhood
BPPV
Rarely associated
Frequently associated
Rarely occurs
The 3 broad classes of migraine headache
treatment
• reduction of risk factors
• abortive medications
• prophylactic medical therapy.
In general abortive drugs not effective in treating
migrainous vertigo.
Reduction of risk factors (stress, anxiety,
hypoglycemia, fluctuating estrogen, certain
foods, smoking)
Elimination of birth control pills or estrogen
replacement products
First-line prophylactic
• calcium channel blockers (verapamil 80–120 mg/d)
• tricyclic antidepressants (nortriptyline 10-150mg/d)
• beta-blockers (propranolol 80–320 mg/d).
Second-line treatment includes
•
•
•
•
•
•
Acetazolamide (Diamox®) 250 mg bid
Topiramate (Topamax®) 50 mg bid
Methysergide
Valproic acid.
SSRIs
Gabapentin
•Acute vestibular symptoms are indistinguishable from
MD
•Unilateral auditory sxs (tinnitus, fullness,
hearing loss) most useful clinical
differential characteristic , favoring MD.
•Bilateral auditory sxs are equally
frequent in both groups.
•Patients with MD usually develop unilateral HL
within 1 year of onset of vertigo
•Unilateral SNHL and a hx of moderate to severe
headaches were the most distinguishing features of
MD vs VM.
Effective in fewer than 25-30% of migraine
cases
Offending foods:
• monosodium glutamate (MSG)
• alcoholic beverages (red wine, port, sherry,
scotch, bourbon)
• aged cheese
• chocolate
• Aspartame
Regular sleep
Regular meals
Exercise
Avoiding peaks of stress, troughs of
relaxation
Relaxation training
Biofeedback
Vertigo
• Lorazepam (Ativan®) 0.5 mg tid prn
• Promethazine/pseudoephedrine (25 mg/60 mg twice
daily)
• Dimenhydrinate (Dramamine®)
• Meclizine (Antivert®)
Whitney et al. : vestibular physical therapy
beneficial in 14 patients with either migrainerelated dizziness or dizziness with a history of
migraine.
Significant improvement in both
Treat associated anxiety or panic
disorder
• Behavioral therapy
• Pharmacotherapy
• subjective (Dizziness Handicap Inventory and
Activities- Specific Balance Confidence Scale) and
• Objective (Dynamic Gait Index) measures occurred
after completion of an average of 5.4 physical therapy
treatment sessions over 3.9 months.
Tricyclic antidepressants
Anxiolytic (eg, benzodiazepine)
Cutrer FM, Baloh RW: Migraine-associated dizziness. Headache 1992;
32: 162-3.
Whitney SL, Brown KE, Furman JM. Physical therapy for migraine
related vestibulopathy and vestibular dysfunction with history of migraine.
Laryngoscope 2000;110(9):1528-34.
Neuhauser H, et al: The interrelations of migraine, vertigo, and
migrainous vertigo. Neurology, 2001; 56: 436-41
Reploeg MD, Goebel JA: Migraine-associated Dizziness: Patient
Characteristics and Management Options. Otol Neurotol. 2002;23:364371.
Furman JM, et al. Migrainous vertigo: development of a pathogenetic
model and structured diagnostic interview. Curr Opin Neurology 16:5–13.
2003
Neuhauser HK, Radtke A, von Brevern M, et al: Migrainous vertigo,
Prevalence and impact on quality of life. Neurology 2006;67:1028-1033
Cha YH, Brodsky J, Ishiyama G, et al: The relevance of migraine in
patients with Meniere’s disease. Acta Otolaryngol, 2007; 127: 12411245.
Neuhauser HK. Epidemiology of vertigo. Curr Opin Neurol 20:40–46,
2007.
Eggers, Scott D Z. Migraine-related vertigo: diagnosis and treatment.
Current Pain & Headache Reports. 11(3):217-26, 2007
Karatas . Central Causes of Dizziness. The Neurologist. Volume 14,
Number 6, November 2008
Honakera J, Samyb RN. Migraine-associated vestibulopathy. Current
Opinion in Otolaryngology & Head and Neck Surgery 2008, 16:412–
415
Casani AP, Sellari-Franceschini S, Napolitano A, et al: Otoneurologic
Dysfunctions in Migraine Patients With or Without Vertigo. Otol
Neurotol 30:961-967, 2009.
Brantberg K, Baloh RW: Similarity of vertigo attacks due to Meniere’s
disease and benign recurrent vertigo, both with and without migraine.
Acta Otolaryngol, 2011; 131:722-727.
Zuniga MG, Janky KL, Schubert MC, Carey JP. Can VestibularEvoked Myogenic Potentials Help Differentiate Meniere Disease from
Vestibular Migraine? Otolaryngol Head Neck Surg. 2012;146, 788-796.
Neff BA, Staab JP, Eggers SD,et al. Auditory and Vestibular
Symptoms and Chronic Subjective Dizziness in Patients with
Meniere’s Disease, Vestibular Migraine, and and Meniere’s Disease
with Concomitant Vestibular Migraine. Otol Neurotol 2012;33:12351244