Menieres

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 Menieres
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 Menieres disease. Seen in:
Syphilis
Otosclerosis,
Cogan syndrome (interstitial keratitis)
Post-stapedectomy
Pagets 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
Menieres disease
variants
Lermoyezs reverse Meniere syndrome:
Deafness vertigo improvement in hearing
Tumarkins sudden drop attack:
Pt falls without vertigo / loss of consciousness
Meyerhoffs 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
Inverted curve
Low + high frequency sensori-neural deafness
Flat curve
Uniform 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 Menieres 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 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 % CO
2
+ 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
Menieres 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:
Ficks needle puncture of footplate
Codys 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
Ficks 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