Dr T Balasubramanian MS DLO www.drtbalu.com
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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 www.drtbalu.com
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1.
1747 – Antonio Scarpa described anatomy of membranous labyrinth
2.
1861 – Prosper Meniere described the classic features of
Meniere’s disease & attributed it to labyrinthine causes
3.
1871 – Knappin theorized that dilated membranous labyrinth to be the cause of this disorder
4.
1927 – Guild described endolymphatic ciruclation
5.
1938 – Hallpike and Portmann described pathology of Meniere’s disease by studying temporal bones.
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1.
150 years have passed since this syndrome was described
2.
Amount of literature accumulated has virtually doubled
3.
Only consensus reached so far is that its cause is multifactorial
4.
Not all individuals with histological features of Meniere’s disease manifested the classic clinical features (? Unknown factors protecting the individuals)
5.
Surgical destruction of sac ameliorates symptoms. (? What role does sac play exactly in endolymphatic circulation) www.drtbalu.com
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1.
Inner ear contains two types of fluids (perilyimph and endolymph separated by membranous labyrinth.
2.
Perilymph is similar in composition to CSF (Containing high Na and low K ions)
3.
Endolymph similar in composition to intracellular fluid (Containing low Na
and high K concentration). It is secreted by stria vascularis www.drtbalu.com
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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 www.drtbalu.com
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1.
Secretory function
2.
Absorptive function
3.
Immune / defense function www.drtbalu.com
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1.
Aquaporins
2.
Glycoproteins like Saccain
3.
Endolymph
4.
Glycoproteins act as a driving force for longitudinal flow www.drtbalu.com
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1.
Longitudinal flow
2.
Radial flow
3.
Dynamic flow www.drtbalu.com
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1.
Was first proposed by Guild
2.
Striavascularis is the principal source
3.
This is a slow process
4.
Elimination occurs at the endolymphatic sac level www.drtbalu.com
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1.
First proposed by Lawrence
2.
This is a combination of both longitudinal and radial flow patterns www.drtbalu.com
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1.
This is active process (energy consuming)
2.
Production occurs from dark vestibular cells & planum semilunatum
3.
Absorption occurs at the striavestibularis www.drtbalu.com
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1.
This is a small membranous bulb located where the endolymphatic duct enters the vestibule
2.
This is where the volume of circulating endolymph is monitored
3.
Monitoring the volume of endolymph is not possible by sac because it will be interfered by CSF pressure and pressure exerted by lateral sinus www.drtbalu.com
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1.
Composition of endolymph is maintained by stria vascularis by controlling the influx of water
2.
Normally endolymph is a biological puddle with very little radial / longitudinal flow
3.
Only under exceptional circumstances like increased endolymphatic fluid volumes does radial / longitudinal movement towards sac occurs
4.
Under normal circumstances radial flow alone is sufficient to maintain endolymph fluid balance and the longitudinal flow due to saccmechanics is not necessary
5.
The longitudinal flow is restricted by the isthmus portion of the duct which acts like the constriction seen in the hour glass www.drtbalu.com
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1.
Small amounts of excess endolymph can be cleared by radial flow
2.
Larger volumes need longitudinal flow for their clearance
3.
Endolymphatic sinus temporarily accommodates excess endolymph till the sac is ready for it
4.
Endolymphatic valve of Bast isolates pars superior and prevents endolymph from draining out of the utricle www.drtbalu.com
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1.
Genetic causes
2.
Infection
3.
Otosclerosis
4.
Trauma (physical / acoustic)
5.
Syphilis
6.
Miscellaneous – Allergy, tumors, leukemia and autoimmune disorders www.drtbalu.com
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1.
Classical Meniere’s disease
2.
Vestibular Meniere’s disease – vestibular symptoms and aural pressure
3.
Cochlear Meniere’s disease – cochlear symptoms and aural pressure
4.
Lermoyez syndrome – Reverse Meniere’s
5.
Tumarkin’s crisis – Utricular Meniere’s www.drtbalu.com
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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 www.drtbalu.com
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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 4 th and 5 th decades of life
 Bilateral Meniere’s syndrome is seen in 5% of these patients www.drtbalu.com
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1.
Endolymphatic hydrops causes distortion of membranous labyrinth
2.
Pressure building up in the scala media may cause mirco ruptures of membranous labyrinth
3.
This would account for the episodic nature of the attacks
4.
Healing of these ruptures causes resolution of the disorder www.drtbalu.com
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1.
Episodic vertigo rotatory in nature
2.
Ipsilateral hearing loss
3.
Aural fullness
4.
Roaring tinnitus
5.
Diplacusis www.drtbalu.com
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1.
Stage I – Patient has solely cochlear symptoms
2.
Stages II – IV – Patients have progressively more cochlear and vestibular symptoms
3.
Stage V – End stage Meniere’s disease (dead ear) www.drtbalu.com
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1.
Irritative nystagmus during the first 20 mins of attack
2.
Paralytic nystagmus follows
3.
Later recovery nystagmus starts www.drtbalu.com
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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 www.drtbalu.com
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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 www.drtbalu.com
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1.
Sensori neural in nature
2.
Fluctuating and progressive
3.
Affects low frequencies
4.
Mild low frequency conductive hearing loss (rare)
5.
Profound sensori neural hearing loss (End stage) www.drtbalu.com
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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 www.drtbalu.com
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1.
This is abnormal growth in the perceived intensity of sound
2.
This is usually positive in patients with Meniere’s disease
3.
ABLB is the test used to look for the presence of recruitment
4.
This test is really time consuming www.drtbalu.com
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1.
Increased summating potential / action potential ratio. 1:3 is normal
2.
Widened summating potential / action potential complex. A widening of greater than 2 ms is significant
3.
Small distorted cochlear microphonics www.drtbalu.com
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1.
Not mandatory for diagnosis of Meniere’s disease
2.
Caloric test is still performed
3.
It is low frequency stimulation (0.003 Hz) of lateral canal
4.
Caloric asymmetry will point to the diseased ear
5.
20% difference between the two ears (Jongkee’s formula) is significant www.drtbalu.com
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1.
Vestibular evoked myogenic potential
2.
Measures the relaxation of sternomastoid muscle in response to ipsilateral click stimulus
3.
Brief high intensity ipsilateral clicks produce large short latency inhibitory potentials (VEMP) in the toncially contracted Ipsilateral sternomastoid muscle
4.
This test is due to the presence of vestibulo collic reflex
5.
Afferent arises from sound responsive cells in the saccule, conducted via the inferior vestibular nerve.
6.
Efferent is via vestibulo spinal tract
7.
Normal responses are composed of biphasic (positive-negative) waves
8.
VEMP reveals saccular dysfunction www.drtbalu.com
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1.
Glycerol
2.
Frusemide
3.
Isosorbide
4.
Tests are positive if there is pure tone improvement of 10dB or more at two / more frequencies between 200-2000Hz www.drtbalu.com
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1.
First introduced by Klockhoff and Lindblom – 1966
2.
Glycerol is administered in doses of 1.5 mg/kg body wt in empty stomach
3.
Serum osmolality should increase at least by 10 mos/kg
4.
Side effects include Headache, Nausea, vomiting, drowsiness
5.
PTA is performed 2-3 hours after administration
6.
False positivity is rare
7.
Positivity depends on the phase of the disease www.drtbalu.com
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1.
Dietary management
2.
Physiotherapy
3.
Psychological support
4.
Pharmacological intervention www.drtbalu.com
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1.
Intravenous fluids – dehydration
2.
Vestibular suppressants – May delay recovery / rehabilitation process
3.
Corticosteroids – May help if tinnitus and deafness are debilitating www.drtbalu.com
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1.
Frustenberg diet
2.
2 grams / 24 hours (restricted salt intake)
3.
Life style modification www.drtbalu.com
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1.
Diuretics play a vital role in alleviating acute symptoms
2.
This has been in use since 1930’s
3.
Thiazide group of drugs are commonly used
4.
Frusemide may be used to alleviate acute symptoms
5.
Clear scientific evidence is lacking regarding the usefulness of diuretics
(cochrane review) www.drtbalu.com
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1.
Cochlear vascular insufficiency has been proposed as one of the mechanism of Meniere's disease
2.
Betahistine is supposed to cause vasodilatation of cochlear blood vessels
3.
Betahistine has weak H1 agonistic property and considerable H3 antagonist properties
4.
It reduces the frequency & intensity of vertigo. Has minimal effect on tinnitus
5.
Doesn’t help much with hearing loss (Cochrane review) www.drtbalu.com
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1.
Immune modulating effects
2.
Improves fluid dynamics of inner ear due to mineralocorticoid effects
3.
Vertigo was controlled on an immediate basis
4.
Methylprednisolone has the best effect as it penetrates the round window better
5.
Silverstein microwick can be used for intratympanic drug administration www.drtbalu.com
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1.
Isordil
2.
ϒ – globulin
3.
Urea
4.
Glycerol
5.
Lithium
6.
Anticholinergics – Glycopyrrolate 1-2 mg /day
7.
Antidopaminergics – Droperidol 2.5 – 10 mg orally / day
8.
Leuprolide acetate – Blocks normal sex hormone production
9.
Innovar – A combination of droperidol and fentanyl can be used to suppress vestibular symptoms (can replace endolymphatic sac surgery)
10.
Hyperbaric oxygen therapy www.drtbalu.com
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1.
Stress reduction
2.
Patient education
3.
Hearing aids – can be used to suppress troublesome tinnitus
4.
Tinnitus retraining www.drtbalu.com
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1.
Meniett Device
2.
Low pressure pulse generator
3.
Vibrations are transmitted via external auditory canal
4.
Vibrations alter inner ear fluid dynamics by their effects on the oval and round windows
5.
Exact mechanism of action is not known
6.
It is totally non invasive
7.
This device is portable www.drtbalu.com
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1.
Diagnosis should be confirmed
2.
Ventilation tube should be inserted
3.
Patient should be trained for self administration of the treatment
4.
Usually administered thrice a day about 5 mins each time
5.
Treatment lasts for 5 weeks www.drtbalu.com
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1.
Classic unilateral Meniere’s disease
2.
Intense vestibular / cochlear symptoms
3.
Failed medical therapy
4.
Over 65 years of age
5.
Imbalance / aural fullness / tinnitus after gentamycin treatment www.drtbalu.com
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1.
Perilymph fistula
2.
Acoustic neuroma / brain tumor
3.
Retrocochlear damage
4.
Low pressure hydrocephalus www.drtbalu.com
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1.
Vestibulotoxic effects are put to therapeutic use.
2.
Sensation of vertigo reduced while hearing is preserved
3.
Streptomycin / gentamycin are predominantly Vestibulotoxic
4.
Intratympanic administration is preferred www.drtbalu.com
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1.
Fixed dose protocol is used
2.
40 mg/ml gentamycin is buffered with soda bicarb (pH6.4) final concentration
26.7mg/ml.
3.
T tube grommet inserted into the postero inferior quadrant of ear drum. A mcirocatheter is inserted through the grommet
4.
1ml of gentamycin solution is injected into the middle ear cavity via the microcatheter
5.
Three injections are given per day in outpatient setting
6.
Injections are given for 4 days
7.
After injection patient should lie supine with the infiltrated ear up for 30 mins
8.
Vertigo usually develops between 2-4 days after cessation of treatment www.drtbalu.com
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1.
Sac enhancement procedure
2.
Sac decompression procedure
3.
Labyrinthine ablative procedures www.drtbalu.com
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1.
External shunts – Drains the sac into mastoid cavity / subarachnoid space
2.
Internal shunts – Drains excessive endolymph into the perilymphatic space
(cochleosacculotomy / labyrinthotomy) www.drtbalu.com
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1.
Helpful in treating debilitated patients
2.
Involves disruption of osseous spiral lamina
3.
Angular pick introduced via round window towards oval window. It will accommodate 3 mm long pick
4.
After perforation the pick is withdrawn and the round window is sealed by fat www.drtbalu.com
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1.
Labyrinthectomy
2.
Translabyrinthine vestibular neurectomy
3.
Retrolabyrinthine vestibular neurinectomy
4.
Retrosigmoid vestibular neurinectomy
5.
Middle cranial fossa vestibular neurinectomy www.drtbalu.com
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