Meniere’s Disease

Dr. Vishal Sharma

Introduction

• Described by Prosper Meniere in 1861

• Vertigo + Deafness + Tinnitus + Aural fullness

• Etiology: endolymphatic hydrops (Hallpike, 1938)

due to ed absorption of endolymph or

ed production of endolymph

• Especially involves cochlear duct & saccule

Prosper Meniere`

Normal membranous labyrinth

Endolymphatic Hydrops

Normal membranous labyrinth

Endolymphatic Hydrops

Pathogenesis

1. Endolymphatic hydrops rupture of membranous

labyrinth potassium rich endolymph mixes with

perilymph sustained inactivation of hair cells &

neurons of vestibulo-cochlear nerve bathed in

perilymph deafness + vertigo + tinnitus

2. ed Sympathetic activity ischemia of cochlear &

vestibular end organs deafness + vertigo

Etiology of Primary Meniere’s

disease

A. Idiopathic

B. Increased production of endolymph:

Allergy

Sodium & water retention

Autoimmune

Viral infection

sympathetic activity ischemia of stria

vascularis fluid transudation

Endocrine Hypo (thyroidism, pituitarism,

adrenalism), Diabetes, Hyperlipoproteinemia

C. Decreased absorption of endolymph:

Small size of

endolymphatic sac / duct

Obstruction of endolymphatic sac / duct

Ischaemia of endolymphatic sac

Inner ear trauma

Secondary Meniere Syndrome

Clinically resembles Meniere’s disease. Seen in:

· Syphilis

· Otosclerosis,

· Cogan syndrome (interstitial keratitis)

· Post-stapedectomy

· Paget’s disease

Clinical Features

• 30 - 60 years, more in males, unilateral

1. Vertigo:

Sudden onset, episodic, rotatory, 30 min - 24 hr,

along with nausea, vomiting & diaphoresis.

85 % pt have positional vertigo

• Vertigo caused by loud, low frequency sound

Tulio phenomenon

Clinical Features

2. Deafness:

Accompanies vertigo, improves after vertigo

attack, sensori-neural, fluctuant, progressive

• Intolerance to loud sound (due to recruitment)

• Distortion of sound frequency, called diplacusis

binauralis dysharmonica

Clinical Features

3. Tinnitus:

Low-pitch, roaring, non-pulsatile,

continuous / intermittent. Increased during

vertigo attacks

4. Aural fullness:

Fluctuating, not relieved by swallowing

5. Emotional upset, anxiety, agoraphobia

AAO-HNS Diagnosis Criteria (1995)

A. Vertigo: Spontaneous, > 2 episodes lasting > 20 min

B. Audiogram documented sensori-neural deafness

C. Tinnitus or Aural fullness in diseased ear

D. Other cases excluded

E. Staging as per pure tone average (500 - 3000 Hz):

1 = < 25 dB 2 = 26 - 40 dB

3 = 41 - 70 dB 4 = > 70 dB

Meniere’s disease variants

• Lermoyez’s reverse Meniere syndrome:

Deafness vertigo improvement in hearing

• Tumarkin’s sudden drop attack:

Pt falls without vertigo / loss of consciousness

• Meyerhoff’s oculo-vestibular response:

Vertigo due to opto-kinetic stimulus

• Cochlear hydrops: deafness & tinnitus only

• Vestibular hydrops: vertigo only

E.N.T. Examination

• Otoscopy: normal tympanic membrane

• Nystagmus: irritative paralytic recovery

• False +ve fistula sign (Hennebert sign): in 30% pt

• Rinne test: positive (A.C. > B.C.)

• Weber test: lateralizes towards better ear

• A.B.C. test: decreased in diseased ear

• Irritative nystagmus: occurs immediately with

onset of an attack, for 20 seconds, toward

diseased ear, due to initial excitation of action

potential by increasing potassium in perilymph

• Paralytic nystagmus: occurs minutes into an

attack, toward healthy ear, due to blockade of

action potential by increased K+ in perilymph

• Recovery nystagmus: occurs hours later, toward

diseased ear, due to vestibular adaptation

Pure Tone Audiometry

Rising curve in early stageLow frequency SNHL due to more fluid accumulation

in apical portion of scala media

Inverted curve

Low + high frequency sensori-neural deafness

Flat curveUniform sensori-neural deafness

Down sloping curve

Further SNHL in high frequency

Other Audiological Tests

• Speech Audiometry: Score = 50 - 80 %

• A.B.L.B.: Recruitment present

• S.I.S.I.: positive (> 70 % score)

• Tone Decay Test: negative (decay < 20 dB)

Laddergram in A.B.L.B.

Electro-cochleography

Electro-cochleography findings in Meniere’s disease

• Summation potential : compound action

potential ratio > 30 %

• Widened SP-AP waveform (> 2msec)

• Distorted cochlear micro-phonics

SP – AP Waveform

Cochlear Microphonics

Normal

SP/AP > 30 %

Distorted CM

Bithermal Caloric TestI/L canal paresis in 75 % cases

Bithermal Caloric TestC/L directional preponderance

Glycerol Test (confirmatory)

• Do P.T.A. & speech audiogram. Glycerol (1.5 ml /

Kg), mixed in lime juice given orally. Repeat

audio tests after 2 hrs. Test is positive if:

• Pure Tone threshold improves > 10 dB

• Speech Discrimination Score increases > 15 %

• S.P. / A.P. ratio in E.Co.G. decreases > 15 %

Other Investigations Full blood count + ESR

Urea, electrolytes

RBS, FBS

Fasting lipid profile

Thyroid function test

VDRL, TPHA

Immunological assay, antibody screening

Treatment of Acute attack

Reassurance Bed rest + head support

Inj. Prochlorperazine (Stemetil):

12.5 mg I.V., T.I.D. – Q.I.D.

Inj. Promethazine (Phenergan):

25 mg I.V., T.I.D. – Q.I.D.

· Inj. Diazepam (Calmpose):

5 mg I.V. stat

Non-surgical treatment

Discussion: Reassurance. Avoid tea, coffee,

colas, chocolate, allergens, stress, smoking,

alcohol, flying, diving, heights.

Diet: Low salt (1.5 g/day), less fluids. Exercise.

Vestibular Depressants: Cinnarizine, Diazepam,

Prochlorperazine, Dimenhydrinate

Non-surgical treatment

Cochlear VasoDilators: Betahistine, Xanthinol

nicotinate, Carbogen (5 % CO2 + 95 % O

2), L.M.W.

Dextran, Histamine drip.

Diuretics: Thiazide + Triamterene

Dexamethasone / Ig G: decreases auto-immunity

Dehydration by hyperosmolar fluids

Hormone replacement therapy

Meniett Device

Low pressure pulse

generator. Pressure

pulses transmitted to

round window via

grommet displace

endolymph relieve

endolymph hydrops.

Used for 5 min, TID.

Meniett Device

Surgical treatment of Meniere’s disease

A. Hearing preservation + Balance preservation:

1. Endolymphatic sac decompression / shunting

2. Sacculotomy by puncture of footplate

3. Cochlear duct piercing via round window

B. Hearing preservation + Balance ablation:

1. Chemical labyrinthectomy 2. Vestibular neurectomy

3. Vestibular end organ destruction by USG / cryoprobe

C. Hearing ablation + Balance ablation:

1. Section of 8th nerve 2. Total labyrinthectomy

Decompression Surgery

1. Endolymphatic sac decompression (Portmann)

2. Endolymphatic sac shunting: into sub-

arachnoid space or mastoid cavity

3. Sacculotomy:

Fick’s needle puncture of footplate

Cody’s tack puncture of footplate

4. Cochlear duct piercing via round window

Decompression Surgery

Endolymphatic sac decompression

Georges Portmann

Sac shunting into mastoid

Sac shunting into subarachnoid

Fick’s needle puncture of footplate

Chemical Labyrinthectomy

Trans-tympanic drug injection

Intra-tympanic drug instillation via grommet

Intra-tympanic drug instillation via Silverstein

micro wick

Trans-tympanic drug perfusion

Drug used: Gentamicin (vestibulo-toxic)

Trans-tympanic injection

Intra-tympanic drug instillation

Grommet in P.I.Q.

Trans-tympanic gentamicin

• 26.7 mg/ml solution used

• 0.75 ml solution instilled in affected ear (via

grommet) 3 times daily for 4 consecutive days

• After instillation, pt to lie supine with affected ear

up for 30 min & not swallow anything

• Vertigo control = 94%. Hearing unchanged or

improved = 74%. Hearing worsened = 26%.

Silverstein micro wick

Trans-tympanic drug perfusion

Trans-tympanic Dexamethasone

Mechanism of action:

reducing inflammation

control of auto-immune injury

Solution strength: 0.25 mg/ml

Dose: 5 drops every alternate day for 3 months

Vestibular Surgery

• Denervation of vestibule by vestibular

neurectomy via middle cranial fossa

• Destruction of vestibule (via round window or

lateral semicircular canal) by:

Cryo-probe

Ultrasound probe

Vestibular Neurectomy

Vestibular Destruction

Ultrasound Probe

Total Destructive Surgery

Destroys both cochlear & vestibular functions.

Done in pt with severe deafness.

Types of surgery are:

• Section of vestibular + cochlear nerves

• Trans-mastoid total labyrinthectomy

Total Destructive Surgery

Total Labyrinthectomy

Vestibule + semi-circular canals exposed

Total Labyrinthectomy

Vestibule + ampullae opened to show neuro-epithelium

Total Labyrinthectomy

Neuro-epithelium destroyed

Treatment Ladder

Vertigo Control Level Score

Average vertigo spells per month post-treatment (24 mth)

= ------------------------------------------------------------------------- X

100

Average vertigo spells per month pre-treatment (6 mth)

Score 0 = Complete control = Level A

Score 1 - 40 = Substantial control = Level B

Score 41 - 80 = Limited control = Level C

Score 81 - 120 = Insignificant control = Level D

Score > 120 = Worse = Level E

Severe vertigo requiring other treatment = Level F

Hearing level reporting

• Pure Tone Average taken for 0.5, 1, 2 & 3 KHz

• If multiple pre and post levels are available,

worst is always used

• PTA is considered improved / worse if a 10 dB

difference is noted

• Speech Discrimination Score is considered

improved / worse if a 15% difference is noted

Prognosis• 60% have complete control of vertigo & 40%

have good hearing, without any treatment

• Medical & surgical therapies show high levels of

improvement with placebo

• Results vary greatly between different series

• Average result: Level A + B = 60 - 80%

Level C = 20 - 30%

Level D + E + F = 10 - 20%

Thank You