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Meniere’s disease


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Meniere’s disease

  2. 2. Where do we stand? 1.150 years have passed since this syndrome was described 2.Amount of literature has virtually doubled 3.cause is multifactorial 4.Not all individuals with histological features of Meniere’s disease manifested the classic clinical features
  3. 3. Introduction : History • First described by Prosper Meniere in 1861. • In 1902, Parry performed a CN VIII division for vertigo in a patient with suspected Meniere’s disease. • Portman did endolymphatic sac decompression via a transmastoid approach in 1926. • In 1931,McKenzie performed a selective vestibular neurectomy.
  4. 4. Introduction • Meniere's disease (idiopathic endolymphatic hydrops) is a disorder of the inner ear associated with a symptoms consisting of • spontaneous, episodic attacks of vertigo; • sensori neural hearing loss which usually fluctuates • tinnitus • sensation of aural fullness.
  5. 5. INCIDENCE  Roughly 1 in 1000 individuals are affected  Constitutes 10% of all patients attending vertigo clinic  Female preponderance  Rare in children under the age of 10  Commonly begins between 3th to 6th decades of life  Bilateral Meniere’s syndrome is seen in 5% of these patients
  6. 6. TYPES OF MENIERE'S DISEASE 1.Classical Meniere’s disease 2.Vestibular Meniere’s disease – vestibular symptoms and aural pressure 3.Cochlear Meniere’s disease – cochlear symptoms and aural pressure 4.Lermoyez syndrome – Reverse Meniere’s 5.Tumarkin’s crisis – Utricular Meniere’s 7
  7. 7. LERMOYEZ SYNDROME • This is a variant of Meniere’s disease. It is characterized by sudden sensori neural hearing loss which improves during or immediately after the attack of vertigo.
  8. 8. TUMARKIN’S DROP ATTACKS • abrupt falling attacks of brief duration without loss of consciousness. due to excess endolymphatic volume. Utricular crisis is used to indicate this condition. • In the later disease stages the valve of Bast remaining patent may cause sudden drainage of endolymph from the utricle due to longitudinal flow resulting in these drop attacks
  9. 9. • Several pathophysiological mechanisms are thought to be implicated in the otolithic catastrophe of Tumarkin: • sudden shift of the utricular macula, sudden changes in the endolymphatic fluid pressure, and sudden electrolyte changes secondary to the rupture of the membrane labyrinth. • Thus, the inappropriate stimulation of the otolithic organs might generate a failure of the vestibulospinal reflex with the loss of postural tonus and, consequently, the falling
  10. 10. Etiology
  11. 11. Pathophysiology • Theories behind endolymphatic hydrops – Obstruction of endolymphatic duct/sac – Hypoplasia of endolymphatic duct/sac – Alteration of absorption of endolymph – Alteration in production of endolymph – Autoimmune insult – Vascular origin – Viral etiology
  12. 12. .
  13. 13. Normal membranous labyrinth Dilated membranous labyrinth in Meniere's disease (Hydrops)
  14. 14. PATHOLOGY A. Defective absorption by endolymphatic sac- • Poor vascularity of sac • Less absorptive tubular epithelium • increased peri saccular fibrosis B. Rupture of reissner’s membreane leading to mixing of perilymph & endolymph- Schuknecht • allow leakage of the potassium-rich endolymph into the perilymph, bathing the eighth cranial nerve and lateral sides of the hair cells
  15. 15. Histopathological changes • Loss of shorter stereocilia of outer hair cell first occuring in the apical region • Outer hair cell->inner hair cell->intercellular edema b/w marginal cell->vacuolization- >atrophy of marginal and intermediate cells-> loss of spiral ganglion cell
  16. 16. • High concentrations of extracellular potassium depolarize the nerve cells, causing their acute inactivation. • The result is a decrease in auditory and vestibular neuronal outflow consistent with the hearing loss and features of acute vestibular paralysis seen in a typical Meniere's attack • The chronic deterioration in inner ear function presumably is the effect of repeated exposure to the effects of the potassium
  18. 18. HEARING LOSS 1. Sensori neural in nature 2. Fluctuating and progressive 3. Affects low frequencies 4. Mild low frequency conductive hearing loss (rare) 5. Profound sensori neural hearing loss (End stage)
  19. 19. TINNITUS  Roaring in nature subjective  Could be continuous / intermittent  Non pulsatile in nature  Frequency of tinnitus corresponds to the region of cochlea which has suffered the maximum damage
  20. 20. Diagnosis
  21. 21. Investigations • Tuning forks tests : SNHL • PTA • Speech audiometry • Recruitment test +ve • SISI >70% • Tone decay <20 dB
  22. 22. Investigations • Caloric testing – canal paresis • ENG • Head Thurst test • ECoG – SP is larger & more negative • SP/AP ratio increases > 30% • Dehydration/Glycerol test • VEMP (Vestibular evoked myogenic potentials) elevated threshold
  23. 23. Spontaneous nystagmus • The direction of the observed spontaneous nystagmus varies; it can consistently beat toward the • involved ear (irritative), • away from it (paralytic), or • change from an irritative to a paralytic pattern over time, and thus cannot be used to lateralize the disease.
  24. 24. LOUDNESS RECRUITMENT 1. This is abnormal growth in the perceived intensity of sound 2. This is usually positive in patients with Meniere’s disease 3. ABLB (alternate binaural loudness balance test)is the test used to look for the presence of recruitment 4. This test is really time consuming
  25. 25. ELECTRO COCHLEOGRAPHY 1. Increased summating potential / action potential ratio. 1:3 is normal 2. Widened summating potential / action potential complex. A widening of greater than 2 ms is significant
  26. 26. VESTIBULAR TESTS 1. Not mandatory for diagnosis of Meniere’s disease 2. Caloric test is still performed 3. It is low frequency stimulation (0.003 Hz) of lateral canal 4. Caloric asymmetry will point to the diseased ear 5. 20% difference between the two ears (Jongkee’s formula) is significant
  27. 27. VEMP 1. Vestibular evoked myogenic potential 2. Measures the relaxation of sternomastoid muscle in response to ipsilateral click stimulus 3. Brief high intensity ipsilateral clicks produce large short latency inhibitory potentials (VEMP) in the toncially contracted Ipsilateral sternomastoid muscle 4. This test is due to the presence of vestibulo collic reflex 5. Afferent arises from sound responsive cells in the saccule, conducted via the inferior vestibular nerve. 6. Efferent is via vestibulo spinal tract 7. Normal responses are composed of biphasic (positive-negative) waves 8. VEMP reveals saccular dysfunction
  28. 28. VEMPs
  29. 29. DEHYDRATION TESTS 1.Glycerol 2.Frusemide 3.Isosorbide • the test is considered positive if • (1) there isa 10-dB or more improvement in pure tone thresholds at 2 or more frequencies (250 to 2000 Hz), or • (2) there is a 12% or greater improvement in speech discrimination score.
  30. 30. GLYCEROL TEST 1. First introduced by Klockhoff and Lindblom – 1966 2. Glycerol is administered in doses of 1.5 mg/kg body wt in empty stomach 3. Serum osmolality should increase at least by 10 mos/kg 4. Side effects include Headache, Nausea, vomiting, drowsiness 5. PTA is performed 2-3 hours after administration 6. False positivity is rare 7. Positivity depends on the phase of the disease
  31. 31. Management • Conservative treatments include lifestyle and dietary adjustments,diuretics, and supplemental use of vestibular suppressants. • Invasive or destructive procedures are indicated only in the 5% to 10% of patients with Meniere’s disease who fail conservative and medical measures. • Overall, vertigo control is achieved in more than 99% of patients with Meniere’s disease.
  32. 32. MEDICAL MANAGEMENT 1. Dietary management 2. Physiotherapy 3. Psychological support 4. Pharmacological intervention
  33. 33. Treatment • Medical management – • ACUTE stage : labyrinth sedatives + anti-emetics • Carbogen, Histamine drip • Frustenberg Regimen - 1. Low salt diet 2. Diuretics + Pot. chlor 3. High protein • Beta histine – to relieve vascular ischemia • Stop caffeine, nicotine, alcohol & tobacco
  34. 34. MEDICAL TREATMENT Acute Therapy
  35. 35. TREATMENT OF ACUTE EXACERBATION 1. Intravenous fluids – dehydration 2. Vestibular suppressants – May delay recovery / rehabilitation process 3. Corticosteroids – May help if tinnitus and deafness are debilitating
  36. 36. LOW SALT DIET 1. Frustenberg diet 2. 2 grams / 24 hours (restricted salt intake) 3. Life style modification
  37. 37. ROLE OF DIURETICS 1. Diuretics play a vital role in alleviating acute symptoms 2. This has been in use since 1930’s 3. Thiazide group of drugs are commonly used 4. Frusemide may be used to alleviate acute symptoms 5. Clear scientific evidence is lacking regarding the usefulness of diuretics
  38. 38. BETAHISTINE 1. Cochlear vascular insufficiency has been proposed as one of the mechanism of Meniere's disease 2. Betahistine is supposed to cause vasodilatation of cochlear blood vessels 3. Betahistine has weak H1 agonistic property and considerable H3 antagonist properties 4. It reduces the frequency & intensity of vertigo. Has minimal effect on tinnitus 5. Doesn’t help much with hearing loss
  39. 39. INTRATYMPANIC STEROIDS 1. Immune modulating effects 2. Improves fluid dynamics of inner ear due to mineralocorticoid effects 3. Vertigo was controlled on an immediate basis 4. Methylprednisolone has the best effect as it penetrates the round window better 5. Silverstein microwick can be used for intratympanic drug administration
  40. 40. OTHER TREATMENT MODALITIES (ANCILLARY) 1. Stress reduction 2. Patient education 3. Hearing aids – can be used to suppress troublesome tinnitus 4. Tinnitus retraining
  41. 41. VIBRATOR THERAPY 1. Meniett Device 2. Low pressure pulse generator 3. Vibrations are transmitted via external auditory canal 4. Vibrations alter inner ear fluid dynamics by their effects on the oval and round windows 5. Exact mechanism of action is not known 6. It is totally non invasive 7. This device is portable
  42. 42. VIBRATOR THERAPY STEPS 1. Diagnosis should be confirmed 2. Ventilation tube should be inserted 3. Patient should be trained for self administration of the treatment 4. Usually administered thrice a day about 5 mins each time 5. Treatment lasts for 5 weeks
  43. 43. INDICATIONS FOR VIBRATOR THERAPY 1. Classic unilateral Meniere’s disease 2. Intense vestibular / cochlear symptoms 3. Failed medical therapy 4. Over 65 years of age 5. Imbalance / aural fullness / tinnitus after gentamycin treatment
  44. 44. CONTRAINDICATIONS FOR VIBRATOR THERAPY 1. Perilymph fistula 2. Acoustic neuroma / brain tumor 3. Retrocochlear damage 4. Low pressure hydrocephalus
  45. 45. ROLE OF AMINOGLYCOSIDES 1.Vestibulotoxic effects are put to therapeutic use. 2.Sensation of vertigo reduced while hearing is preserved 3.Streptomycin / gentamycin are predominantly Vestibulotoxic 4.Intratympanic administration is preferred
  46. 46. INTRATYMPANIC GENTAMYCIN 1. Fixed dose protocol is used 2. 40 mg/ml gentamycin is buffered with soda bicarb (pH6.4) final concentration 26.7mg/ml. 3. T tube grommet inserted into the postero inferior quadrant of ear drum. A mcirocatheter is inserted through the grommet 4. 1ml of gentamycin solution is injected into the middle ear cavity via the microcatheter 5. Three injections are given per day in outpatient setting 6. Injections are given for 4 days 7. After injection patient should lie supine with the infiltrated ear up for 30 mins 8. Vertigo usually develops between 2-4 days after cessation of treatment
  47. 47. SURGICAL MANAGEMENT 1.Sac enhancement procedure 2.Sac decompression procedure 3.Labyrinthine ablative procedures
  48. 48. Endolymphatic Sac Surgery – Decompression: Removal of bone overlying the sac – Shunting: Placement of synthetic shunt to drain endolymph into mastoid – Drainage: Incision of the sac to allow drainage – Removal of sac: Excision of the sac
  49. 49. ABLATIVE PROCEDURES 1.Labyrinthectomy 2.Translabyrinthine vestibular neurectomy 3.Retrolabyrinthine vestibular neurinectomy 4.Retrosigmoid vestibular neurinectomy 5.Middle cranial fossa vestibular neurinectomy
  50. 50. SHUNT PROCEDURE 1. External shunts – Drains the sac into mastoid cavity / subarachnoid space 2. Internal shunts – Drains excessive endolymph into the perilymphatic space (cochleosacculotomy / labyrinthotomy)
  51. 51. COCHLEOSACCULOTOMY / LABYRINTHOTOMY 1. Helpful in treating debilitated patients 2. Involves disruption of osseous spiral lamina 3. Angular pick introduced via round window towards oval window. It will accommodate 3 mm long pick 4. After perforation the pick is withdrawn and the round window is sealed by fat
  52. 52. Endolymphatic Sac Surgery
  54. 54. THANK YOU……
  55. 55. ANY QUESTION?????