American Hearing Lecture on Meniere`s Disease

American Hearing Lecture
on Meniere’s Disease
Richard J. Wiet, M.D., F.AC.S.
chicagoear.com
EAR INSTITUTE OF CHICAGO
Outline
 Case discussion
 Introduction (History, Definitions, Subtypes)
 Pathophysiology
 Clinical Diagnosis
 Treatment options
Case Example : typical pt
 A 42 year old woman gives a history of
unilateral hearing loss… in left ear:
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Periodic fluctuating hearing loss
Room spinning one to two hours 2 x a week
Nausea and vomiting
Occurs several times monthly, intensity varies
American Academy :1995 Criteria for
the diagnosis of Meniere’s disease
Definition:
Introduction - History
 Prosper Meniere
 1861 – Memoire sur des
lesiones de l’orielle interne
dominant lieu a des symptomes
de congestion cerebrale
 Suggested that, the ear:
peripheral end organ, could
be cause of vertigo
Introduction - History
 1920 Portman performed endolymphatic sac experiments
 1938 Hallpike and Cairns demonstrated hydrops
histologically
 1965 Kimura and Schuknecht developed model of
hydrops in the guinea pig
Subtypes , Meniere’s
 Tumarkin
Tumarkin’ss otolithic crisis
 Sudden, spontaneous fall to the ground without prior
warning
 Often little vertigo, nausea or vomiting, sweating,
disequilibrium, rare..loss of consciousness
 Otolith dysfunction
Subtypes
 Lermoyez syndrome
 Increasing tinnitus, aural fullness and hearing
relieved by an episodic attack of vertigo
Introduction - Definitions
 Meniere’s disease = Idiopathic
 Meniere’s syndrome = Secondary
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Otitic syphilis
Cogan’s disease
Autoimmune
Multiple Sclerosis
Tumors of endolymphatic sac
Epidemiology
 Meniere’s disease has a prevalence of about 200
cases/100,000 persons in the United States, or in
other words, about 0.2 % of the population
 The prevalence increases with age, rather
linearly
Age of Onset
Not adjusted for
population from which
sample was taken
Sporadic Meniere's Disease Age of Onset N=332
140
120
Number
100
80
60
40
20
0
11-20y
21-30y
31-40y
41-50y
Age
51-60y
61-70y
Epidemiology
Female preponderance
Bilaterality in Meniere’s
 610 patients followed for at least 5 years, 31.8%
developed bilateral disease
 Over half of those developing bilateral disease did so
within 5 years
 Morrison reported that bilaterality increased with time.
42.5% occurred within 20 yrs
Molecular Epidemiology
 Familial occurrence of Meniere’s disease in 10%20% of patients
 Possible association with certain HLAs
 B8/DR3 & Cw7
Summary Thus Far
 Meniere’s disease consists of episodic bouts of vertigo
associated with tinnitus and hearing loss
 Incidence is about 10-200/100,000 per year
 Onset most commonly in middle age
 About 1/3 have bilateral disease
 Some sort of familial aggregation
Pathophysiology
 Central dogma for Meniere’s syndrome
“endolymphatic hydrops generates the clinical
symptoms of Meniere’s syndrome”
 That dogma….Increasingly questioned :
 10% of population has hydrops on autopsy, only
0.2% of population has Meniere’s during life
 Patients have Meniere’s but no hydrops on autopsy
Meniere’s Inner Ear
Cochlea (Normal->Hydrops)
Pathophysiology
Hydropic Cochlea
Normal Cochlea
Alternative Pathophysiologies
 Hydrops (conventional explanation), with drainage
pathways explanation (plugged up plumbing)
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But now….
Cytokine release (autoimmune disorders)
Viral relapse (e.g. Herpes)
Debris accumulation in endolymphatic system
III. Clinical Signs and Symptoms:
 Onset
 Duration
 Frequency
 Provocateurs / Mollifiers
 Quality (imbalance, spatial disorientation )
Signs: Acute vertigo
Horizontal rotary nystagmus, fast phase to left
Vertigo Attacks
 What’s happens?
 Membrane Ruptures
 Mixture of
Endolymph (high K+)
& Perilymph (low K+)
Auditory clinical features
 Diplacusis - the perception of a single auditory
stimulus as two sounds, as a result of cochlear
pathology
 Recruitment - May cause moderately loud
sounds to be perceived as uncomfortably loud
 Due to alteration in hair cell function
Physical exam
 Otologic exam
 Focused Neurologic exam
 Cranial Nerves
 Cerebellar testing
 Special Maneuvers for dizziness
 Spontaneous nystagmus
 DHP with Frenzel lenses
 Ocular exam (if indicated by history)
III. Diagnosis
 Clinical History - diagnosis of Meniere’s disease
is one of exclusion, and a careful history is the
most important guide to a correct diagnosis
 Audiometric Findings – Low frequency
fluctuating SNHL (Early)
Audiometry (Normal Hearing)
Early Meniere’s
Advanced Meniere’s
Consider Ménière's in persons with:
 Episodic Vertigo
 Monaural fullness
 Fluctuating hearing
 Multi-frequency tinnitus
What Else Could It Be?
 Main Differential Diagnosis:
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Migraine
Perilymphatic Fistula
Acoustic Neuroma
Autoimmune inner ear disease
Cogan’s syndrome (slit lamp exam)
Very rarely…tumor of the endolympatic sac
Symptom
Migraine-Associated
Vertigo
Ménière Disease
Vertigo
May last >24 hr
Lasts <24 hr
Sensorineural hearing loss
Very uncommon; when present, often
low frequency; very rarely progressive;
may fluctuate in cases of basilar
migraine
Nearly always progressive; most often
unilateral; may be bilateral; fluctuation is
common
Tinnitus
May be unilateral or bilateral; rarely
obtrusive
May be unilateral or bilateral; often of
significant intensity
Photophobia
Often present; may or may not be
associated with dizziness
Never present unless a concurrent
history of migraine exists
Migraine-Associated Vertigo Robert A Battista, MD, FACS ,
www.E-medicine.com
Exclusionary testing
(to rule out alternatives)
 MRI scan
 BAER (ABR) testing (if no MRI scan)
 Blood testing
Diagnosis - Imaging
 MRI
performed to rule out retrocochlear pathology and MS:
 Diffusion-weighted MR to evaluate vascular changes
 Vertigo is presenting symptom for MS in 7%10% of patients
 Because of slow growth of acoustic neuroma,
typically causes unsteadiness, not vertigo
Must rule-out Posterior fossa
Neoplasms !
Adenoma of the endolymphatic sac. Hassard AD, Boudreau
SF, Cron CC. J Otolaryngol. 1984 Aug;13(4):213-6.
(10 % of von Hipple Lindau will have this diagnosis)
Laboratory Testing
 Heat-shock protein 70, also known as Anti-68KD (anticochlear antibody) – somewhat controversial at present.
 ANA
 ESR
 FTA-Abs
 +/- Lyme titer, TSH, lipids, CBC
Heat-shock protein 70 (Anti68KD)
Can be found in 30-45% of pts with MD
Present in 25% of healthy controls
Positive in 89% of patients with auto-immune
inner ear disease
Treatment Options
 Medical
 Surgical
 +/- Physical therapy
 (not for acute exacerbations phase)
Treatment typical Ménière's
Disease
Intratympanic gentamicin
Level II/III
(30%)
Level I
(70%)
Endolymphatic sac surgery
Destructive procedures
Low salt diet
Diuretics
Vestibular Suppressant
Steroids (Oral/ Intratympanic)
Balance Rehab
Medical Treatments
 Salt restriction (<2 gram)
 Diuretic (usually dyazide)
 Vestibular suppressants
 Meclizine and relatives
 Valium and relatives
 Antiemetics (e.g. phenergan)
 Steroids (oral vs. transtympanic)
 Various other Rx -- ? Placebos ?
Placebos ?
 Betahistine (Serc is non-FDA approved)
 Vitamins (lipoflavinoids)
 Allergy treatments
 Migraine prophylactic treatments
 Meniett device ?
Meniett™ Treatment ?
Safe

Simple

Portable

Effective ?
Short term results with Meniett
 Randomized, dbl-blinded, placebo controlled multicenter study
 62 patients, active unilat MD, uncontrolled with diet /
meds (instructed to continue baseline Rx)
 Device used TID
 Assesed subjective rating of vertigo, activity and
tinnitus, fullness and hearing
 Surveys, audios and ECOG conducted baseline, 2 and
4 mos….early result promising
Surgical Treatments
 Non-destructive procedures:
 Endolymphatic shunt, or decompression
 Destructive procedures:
 Trans-tympanic Gentamicin
 Vestibular nerve section
 Labyrinthectomy
Destructive procedures
 Selective ablation of vestibular function with hearing
preservation:
 Gentamicin …first
 Vestibular nerve section
 Complete inner ear destruction (hearing loss)
 Labyrinthectomy
 Indications for VNS or labyrinthectomy :
 Driving for a living?
 Drop Attacks
 Intolerable quality of life
Transtympanic Gentamicin
 Titration to effect method gave best vertigo
control (first onset of Spont. Nystag.)
 Multiple daily injections resulted in greater HL
Intratympanic perfusion
considerations
 How does it work ?
 Anatomy, end organ targets
 Diffusion concentrations, time factors, comparison to
PO, IV, IM administration
 Indications
 For which disease processes ?
 Evidence to support efficacy
 Complications
How does Gentamicin work?
 Semi-selective vestibulotoxin
 Targets dark cell of stria vascularis and planum
semilunatum of the SCC (↓ endolymph
production), may destroy type I cells of ampulla
 May interfere with active transport system
crucial for maintaining ionic balance of
endolymph
Inner ear diffusion
 Substances permeate RW into scala tympani
(peri-lymph)
 Possible active transport into endolymph where
concentrations have been found to be higher
Inner ear diffusion
 Consequense of non-uniform distribution:
 Possible protective affect of low-mid freq from
Gentamicin
 Possible decrease efficacy to these regions in steroid
perfusions
 Which substances to perfuse for Meniere’s ?
Gentamicin efficacy
 Currently it is widely used
 What is the best way to give it to minimize
hearing loss ?
 Dosing regimen
 Concentration
 Delivery (transtympanic bolus, micro-wick etc)
Our technique :
 0.3 ml of gentamycin (vial is 40 mg/ml)…so..@12mg
 23 gague needle used after anesthesia to TM
 One injection…helps about 60 % of patients..some 3
After…Lange et al..Laryngoscope 2004
Our Data :
Control of Vertigo
100.00
90.00
82.50
80.00
70.00
63.75
60.00
50.00
40.00
30.00
20.00
10.00
0.00
Complete (Class A)
Complete to substantial (Class A + B)
% of Patients
Sample Size
(Useable Data)
Complete(
Class A)
Complete to
Substantial
(Class A +B)
Average Follow-up
Time (Months)
95 (80)
63.75
82.50
6.76
Our Data
4 Frequency PTA
100.00
90.00
80.00
PTA (dB)
70.00
56.29
57.99
Baseline
Follow-up
60.00
50.00
40.00
30.00
20.00
10.00
0.00
Baseline
Follow-Up
PTA (dB)
SD
n
PTA
(dB)
SD
n
Average Follow-up
Time (Months)
56.29
15.02
88
57.99
19.44
75
4.96
Our Data
Word Recogntion Score
100.00
90.00
80.00
WRS (%)
70.00
61.41
60.83
Baseline
Follow-up
60.00
50.00
40.00
30.00
20.00
10.00
0.00
Baseline
Follow-Up
WR (%)
SD
N
WR
(%)
SD
n
Average Follow-up
Time (Months)
61.41
27.84
82
60.83
28.82
72
5.01
Vestibular nerve section
 Can spare hearing
 90% effective as hearing and vestibular nerves
may be intermingled medially
 Intrinsically riskier than gentamicin as
requires general anesthesia, and typically a
neurosurgical approach.
Vestibular neurectomy : indications :
 Reasonable hearing is present … all cases &
 Especially with “drop attack vertigo”
 Indicated in certain individuals :
 Life style demands it…”driving”, “fireman”
 Drop attack vertigo
Labyrinthectomy:
 Hearing poor…trans mastoid approach
 Drill out labyrinth, remove all neuroepithelium
 Should be 90 % effective
 Intrinsically ablates residual hearing
 Unsteadiness after procedure (requires hospitalization)
 Can be rehabilitated with cochlear implant
Cochlear implantation after labyrinthectomy. Facer et al. Am J
Otol May 2000
Conclusions
 Meniere’s is a dibilitating disease that still affects
thousands of Americans / year
 The otolaryngologist (otologists) are most
familiar with current therapies for this problem
 It (Meniere’s) has been often called …the “great
masquerader”…as other dx may be the same
And finally
 The majority of patients today can be treated
conservatively
 Those that fail medical therapy are immensely
helped with intratympanic therapy, with a low risk
to hearing loss
 There still remains a role for surgery, VNS most
effective when hearing present