1. Meniere’s Disease
Dr. Vishal Sharma

2. 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

3. Prosper Meniere`

4. Normal membranous labyrinth

5. Endolymphatic Hydrops

6. Normal membranous labyrinth

7. Endolymphatic Hydrops

8. Pathogenesis

9. 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

10. Etiology of Primary Meniere’s disease

11. 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

12.  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

13. Secondary Meniere Syndrome
Clinically resembles Meniere’s disease. Seen in:
Syphilis
Otosclerosis,
Cogan syndrome (interstitial keratitis)
Post-stapedectomy
Paget’s disease

14. Clinical Features
years, more in males, unilateral
1. Vertigo: Sudden onset, episodic, rotatory, 30 min - 24 hr, along with nausea, vomiting & diaphoresis % pt have positional vertigo
Vertigo caused by loud, low frequency sound  Tulio phenomenon

15. 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

16. 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

17. 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 ( Hz):
1 = < 25 dB 2 = dB
3 = dB 4 = > 70 dB

18. Meniere’s disease variants

19. 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

20. 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

21. 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

22. Pure Tone Audiometry

23. Rising curve in early stage
Low frequency SNHL due to more fluid accumulation in apical portion of scala media

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

25. Uniform sensori-neural deafness
Flat curve
Uniform sensori-neural deafness

26. Further SNHL in high frequency
Down sloping curve
Further SNHL in high frequency

27. Other Audiological Tests
Speech Audiometry: Score = %
A.B.L.B.: Recruitment present
S.I.S.I.: positive (> 70 % score)
Tone Decay Test: negative (decay < 20 dB)

28. Laddergram in A.B.L.B.

29. Electro-cochleography

30. Electro-cochleography findings in Meniere’s disease
Summation potential : compound action potential ratio > 30 %
Widened SP-AP waveform (> 2msec)
Distorted cochlear micro-phonics

31. SP – AP Waveform

32. Cochlear Microphonics
SP/AP > 30 %
Normal
Distorted CM

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

34. Bithermal Caloric Test
C/L directional preponderance

35. 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 %

36. Other Investigations Full blood count + ESR Urea, electrolytes
RBS, FBS
Fasting lipid profile
Thyroid function test
VDRL, TPHA
Immunological assay, antibody screening

37. 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

38. 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

39. Non-surgical treatment
Cochlear VasoDilators: Betahistine, Xanthinol nicotinate, Carbogen (5 % CO % O2), L.M.W. Dextran, Histamine drip.
Diuretics: Thiazide + Triamterene
Dexamethasone / Ig G: decreases auto-immunity
Dehydration by hyperosmolar fluids
Hormone replacement therapy

40. Meniett Device
Low pressure pulse generator. Pressure pulses transmitted to round window via grommet  displace endolymph  relieve endolymph hydrops. Used for 5 min, TID.

41. Meniett Device

42. Surgical treatment of Meniere’s disease

43. 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 Vestibular neurectomy
3. Vestibular end organ destruction by USG / cryoprobe
C. Hearing ablation + Balance ablation:
1. Section of 8th nerve Total labyrinthectomy

44. 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

45. Decompression Surgery

46. Endolymphatic sac decompression

47. Georges Portmann

48. Sac shunting into mastoid

49. Sac shunting into subarachnoid

50. Fick’s needle puncture of footplate

51. 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)

52. Trans-tympanic injection

53. Intra-tympanic drug instillation

54. Grommet in P.I.Q.

55. 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%.

56. Silverstein micro wick

57. Trans-tympanic drug perfusion

58. 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

59. 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

60. Vestibular Neurectomy

61. Vestibular Destruction

62. Ultrasound Probe

63. 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

64. Total Destructive Surgery

65. Total Labyrinthectomy
Vestibule + semi-circular canals exposed

66. Total Labyrinthectomy
Vestibule + ampullae opened to show neuro-epithelium

67. Total Labyrinthectomy
Neuro-epithelium destroyed

68. Treatment Ladder

69. 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 = Substantial control = Level B
Score = Limited control = Level C
Score = Insignificant control = Level D
Score > 120 = Worse = Level E
Severe vertigo requiring other treatment = Level F

70. 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

71. 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 = %
Level C = %
Level D + E + F = %

72. Thank You