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

B. Increased production of endolymph:  Allergy 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 stage Low frequency SNHL due to more fluid accumulation in apical portion of scala media

Low + high frequency sensori-neural deafness Inverted curve Low + high frequency sensori-neural deafness

Uniform sensori-neural deafness Flat curve Uniform sensori-neural deafness

Further SNHL in high frequency 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 SP/AP > 30 % Normal Distorted CM

Bithermal Caloric Test I/L canal paresis in 75 % cases

Bithermal Caloric Test C/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 % O2), 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%

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