Meniere*s Disease

Report
Dr T Balasubramanian MS DLO
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Meniere’s disease is defined as a symptom complex associated with:
1. Roaring tinnitus
2. Sensorineural hearing loss (Low frequency)
3. Vertigo (episodic)
4. Fullness of the ear
5. These symptoms are associated with dilated membranous
labyrinth filled with endolymph
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1747 – Antonio Scarpa described anatomy of membranous
labyrinth
1861 – Prosper Meniere described the classic features of
Meniere’s disease & attributed it to labyrinthine causes
1871 – Knappin theorized that dilated membranous labyrinth to
be the cause of this disorder
1927 – Guild described endolymphatic ciruclation
1938 – Hallpike and Portmann described pathology of Meniere’s
disease by studying temporal bones.
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150 years have passed since this syndrome was described
Amount of literature accumulated has virtually doubled
Only consensus reached so far is that its cause is multifactorial
Not all individuals with histological features of Meniere’s disease
manifested the classic clinical features (? Unknown factors
protecting the individuals)
Surgical destruction of sac ameliorates symptoms. (? What role
does sac play exactly in endolymphatic circulation)
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Inner ear contains two types of fluids (perilyimph and endolymph
separated by membranous labyrinth.
Perilymph is similar in composition to CSF (Containing high Na and low K
ions)
Endolymph similar in composition to intracellular fluid (Containing low Na
and high K concentration). It is secreted by stria vascularis
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Duct begins at ductus reuniens
Duct is a single lumen tube
about 2 mm long
The duct narrows at the isthmus
which lies at the level of
vestibular aqueduct
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Secretory function
Absorptive function
Immune / defense function
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Aquaporins
Glycoproteins like Saccain
Endolymph
Glycoproteins act as a driving force
for longitudinal flow
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Longitudinal flow
Radial flow
Dynamic flow
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Was first proposed by Guild
Striavascularis is the principal source
This is a slow process
Elimination occurs at the endolymphatic
sac level
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First proposed by Lawrence
This is a combination of both
longitudinal and radial flow patterns
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This is active process (energy consuming)
Production occurs from dark vestibular cells &
planum semilunatum
Absorption occurs at the striavestibularis
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This is a small membranous bulb located where the
endolymphatic duct enters the vestibule
This is where the volume of circulating endolymph is monitored
Monitoring the volume of endolymph is not possible by sac
because it will be interfered by CSF pressure and pressure
exerted by lateral sinus
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Composition of endolymph is maintained by stria vascularis by controlling
the influx of water
Normally endolymph is a biological puddle with very little radial /
longitudinal flow
Only under exceptional circumstances like increased endolymphatic fluid
volumes does radial / longitudinal movement towards sac occurs
Under normal circumstances radial flow alone is sufficient to maintain
endolymph fluid balance and the longitudinal flow due to saccmechanics is
not necessary
The longitudinal flow is restricted by the isthmus portion of the duct which
acts like the constriction seen in the hour glass
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Small amounts of excess endolymph can be cleared by radial flow
Larger volumes need longitudinal flow for their clearance
Endolymphatic sinus temporarily accommodates excess endolymph till
the sac is ready for it
Endolymphatic valve of Bast isolates pars superior and prevents
endolymph from draining out of the utricle
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Genetic causes
Infection
Otosclerosis
Trauma (physical / acoustic)
Syphilis
Miscellaneous – Allergy, tumors, leukemia and autoimmune disorders
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Classical Meniere’s disease
Vestibular Meniere’s disease – vestibular symptoms and aural pressure
Cochlear Meniere’s disease – cochlear symptoms and aural pressure
Lermoyez syndrome – Reverse Meniere’s
Tumarkin’s crisis – Utricular Meniere’s
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This is a variant of Meniere’s disease. It is characterized by sudden sensori
neural hearing loss which improves during or immediately after the attack of
vertigo.
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This variant is characterized by abrupt falling attacks of brief duration
without loss of consciousness. This is caused due to an enlarging utricle
due to excess endolymphatic volume. Utricular crisis is used to indicate
this condition.
In the later disease stages the valve of Bast remaining patent may cause
sudden drainage of endolymph from the utricle due to longitudinal flow
resulting in these drop attacks
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Roughly 1 in 1000 individuals are affected
Constitutes 10% of all patients attending vertigo clinic
Female preponderance
Rare in children under the age of 10
Commonly begins between 4th and 5th decades of life
Bilateral Meniere’s syndrome is seen in 5% of these patients
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Endolymphatic hydrops causes distortion of membranous labyrinth
Pressure building up in the scala media may cause mirco ruptures of
membranous labyrinth
This would account for the episodic nature of the attacks
Healing of these ruptures causes resolution of the disorder
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Episodic vertigo rotatory in nature
Ipsilateral hearing loss
Aural fullness
Roaring tinnitus
Diplacusis
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Stage I – Patient has solely cochlear symptoms
Stages II – IV – Patients have progressively more cochlear and
vestibular symptoms
Stage V – End stage Meniere’s disease (dead ear)
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Irritative nystagmus during the first 20 mins of attack
Paralytic nystagmus follows
Later recovery nystagmus starts
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 Possible Meniere’s disease:
Episodic vertigo of Meniere’s type without documented hearing loss
Fluctuating hearing loss with disequilibrium but without definite episodes
 Probable Meniere’s disease:
One definitive episode of vertigo
Audiometrically documented hearing loss at least during one attack
 Definitive Meniere’s disease
Two or more definitive episodes of spontaneous vertigo one atleast lasting for
20 mins.
 Audiometrically documented hearing loss at least on one occasion
Tinnitus and aural fullness in the treated ear
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 Sensori neural hearing loss combined with:
Tinnitus now / in the past
Vertigo attacks (at least two present now or in the past)
Exclusion of other pathology following Groningen protocol
 Hearing loss:
Sensori neural in nature
No demonstrable conductive element
Hearing loss of 20 dB or more at one of the usually measured
audiometric thresholds
 Vertigo:
Paroxysmal rotatory dizziness, accompanied by nausea / vomiting
At least two episodes should be reported during a course of illness.
One of the attack should last at least for 5 mins
In between attacks there may be periods of unsteadiness
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Sensori neural in nature
Fluctuating and progressive
Affects low frequencies
Mild low frequency conductive hearing loss (rare)
Profound sensori neural hearing loss (End stage)
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Roaring in nature
Could be continuous / intermittent
Non pulsatile in nature
Frequency of tinnitus corresponds to the region of cochlea which has suffered
the maximum damage
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This is abnormal growth in the perceived intensity of sound
This is usually positive in patients with Meniere’s disease
ABLB is the test used to look for the presence of recruitment
This test is really time consuming
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Increased summating potential / action potential ratio. 1:3 is normal
Widened summating potential / action potential complex. A widening of
greater than 2 ms is significant
Small distorted cochlear microphonics
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Not mandatory for diagnosis of Meniere’s disease
Caloric test is still performed
It is low frequency stimulation (0.003 Hz) of lateral canal
Caloric asymmetry will point to the diseased ear
20% difference between the two ears (Jongkee’s formula) is significant
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Vestibular evoked myogenic potential
Measures the relaxation of sternomastoid muscle in response to ipsilateral click
stimulus
Brief high intensity ipsilateral clicks produce large short latency inhibitory
potentials (VEMP) in the toncially contracted Ipsilateral sternomastoid muscle
This test is due to the presence of vestibulo collic reflex
Afferent arises from sound responsive cells in the saccule, conducted via the
inferior vestibular nerve.
Efferent is via vestibulo spinal tract
Normal responses are composed of biphasic (positive-negative) waves
VEMP reveals saccular dysfunction
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Glycerol
Frusemide
Isosorbide
Tests are positive if there is pure tone improvement of 10dB or more
at two / more frequencies between 200-2000Hz
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First introduced by Klockhoff and Lindblom – 1966
Glycerol is administered in doses of 1.5 mg/kg body wt in empty stomach
Serum osmolality should increase at least by 10 mos/kg
Side effects include Headache, Nausea, vomiting, drowsiness
PTA is performed 2-3 hours after administration
False positivity is rare
Positivity depends on the phase of the disease
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Dietary management
Physiotherapy
Psychological support
Pharmacological intervention
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Intravenous fluids – dehydration
Vestibular suppressants – May delay recovery / rehabilitation process
Corticosteroids – May help if tinnitus and deafness are debilitating
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Frustenberg diet
2 grams / 24 hours (restricted salt intake)
Life style modification
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Diuretics play a vital role in alleviating acute symptoms
This has been in use since 1930’s
Thiazide group of drugs are commonly used
Frusemide may be used to alleviate acute symptoms
Clear scientific evidence is lacking regarding the usefulness of diuretics
(cochrane review)
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Cochlear vascular insufficiency has been proposed as one of the
mechanism of Meniere's disease
Betahistine is supposed to cause vasodilatation of cochlear blood
vessels
Betahistine has weak H1 agonistic property and considerable H3
antagonist properties
It reduces the frequency & intensity of vertigo. Has minimal effect on
tinnitus
Doesn’t help much with hearing loss (Cochrane review)
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Immune modulating effects
Improves fluid dynamics of inner ear due to mineralocorticoid effects
Vertigo was controlled on an immediate basis
Methylprednisolone has the best effect as it penetrates the round window
better
Silverstein microwick can be used for intratympanic drug administration
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Isordil
ϒ – globulin
Urea
Glycerol
Lithium
Anticholinergics – Glycopyrrolate 1-2 mg /day
Antidopaminergics – Droperidol 2.5 – 10 mg orally / day
Leuprolide acetate – Blocks normal sex hormone production
Innovar – A combination of droperidol and fentanyl can be used to
suppress vestibular symptoms (can replace endolymphatic sac surgery)
10. Hyperbaric oxygen therapy
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Stress reduction
Patient education
Hearing aids – can be used to suppress troublesome tinnitus
Tinnitus retraining
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Meniett Device
Low pressure pulse generator
Vibrations are transmitted via external
auditory canal
Vibrations alter inner ear fluid dynamics by
their effects on the oval and round
windows
Exact mechanism of action is not known
It is totally non invasive
This device is portable
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Diagnosis should be confirmed
Ventilation tube should be inserted
Patient should be trained for self administration of the treatment
Usually administered thrice a day about 5 mins each time
Treatment lasts for 5 weeks
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Classic unilateral Meniere’s disease
Intense vestibular / cochlear symptoms
Failed medical therapy
Over 65 years of age
Imbalance / aural fullness / tinnitus after gentamycin treatment
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Perilymph fistula
Acoustic neuroma / brain tumor
Retrocochlear damage
Low pressure hydrocephalus
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Vestibulotoxic effects are put to therapeutic use.
Sensation of vertigo reduced while hearing is preserved
Streptomycin / gentamycin are predominantly Vestibulotoxic
Intratympanic administration is preferred
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Fixed dose protocol is used
40 mg/ml gentamycin is buffered with soda bicarb (pH6.4) final concentration
26.7mg/ml.
T tube grommet inserted into the postero inferior quadrant of ear drum. A
mcirocatheter is inserted through the grommet
1ml of gentamycin solution is injected into the middle ear cavity via the
microcatheter
Three injections are given per day in outpatient setting
Injections are given for 4 days
After injection patient should lie supine with the infiltrated ear up for 30 mins
Vertigo usually develops between 2-4 days after cessation of treatment
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Sac enhancement procedure
Sac decompression procedure
Labyrinthine ablative procedures
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External shunts – Drains the sac into mastoid cavity / subarachnoid space
Internal shunts – Drains excessive endolymph into the perilymphatic space
(cochleosacculotomy / labyrinthotomy)
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Helpful in treating debilitated patients
Involves disruption of osseous spiral lamina
Angular pick introduced via round window towards oval window. It will
accommodate 3 mm long pick
After perforation the pick is withdrawn and the round window is sealed by
fat
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Labyrinthectomy
Translabyrinthine vestibular neurectomy
Retrolabyrinthine vestibular neurinectomy
Retrosigmoid vestibular neurinectomy
Middle cranial fossa vestibular neurinectomy
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