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Meniere’s Disease Dr. Vishal Sharma. Introduction Described by Prosper Meniere in 1861 Vertigo + Deafness + Tinnitus + Aural fullness Etiology: endolymphatic.

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Presentation on theme: "Meniere’s Disease Dr. Vishal Sharma. Introduction Described by Prosper Meniere in 1861 Vertigo + Deafness + Tinnitus + Aural fullness Etiology: endolymphatic."— Presentation transcript:

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 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. 85 % 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: F luctuating, 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 Inverted curve Low + high frequency sensori-neural deafness

25 Flat curve Uniform sensori-neural deafness

26 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 Normal SP/AP > 30 % 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 % O 2 ), 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 2. Vestibular neurectomy 3. Vestibular end organ destruction by USG / cryoprobe C. Hearing ablation + Balance ablation: 1. Section of 8th nerve 2. 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


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