Meniere’s disease


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This presentation discusses Meniere's disease and its current management trends

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  • Schematic of how the endolymphatic sinus detects and regulates endolymph volume status. When endolymph volume is normal (left), pressure elevations in the vestibule (black arrow) produce only small endolymph movements into the sac before the sinus membrane occludes the duct. In contrast, when the endolymphatic sinus is dilated (right), pressure elevations in the vestibule result in a larger volume being forced into the sac before the duct is occluded. The increase in volume delivered to the sac with dilation of the endolymphatic sinus will act to counteract the volume increase, acting to stabilize endolymph volume within a specific range.
  • The excess volume tends to accumulate in the apical end of the cochlea, where the membranes are more lax than elsewhere, even though the endolymph pressure would be similar elsewhere in the cochlea.
  • Meniere’s disease

    1. 1. Dr T Balasubramanian MS DLO 1
    2. 2. Meniere’s disease is defined as a symptom complex associated with:1. Roaring tinnitus2. Sensorineural hearing loss (Low frequency)3. Vertigo (episodic)4. Fullness of the ear5. These symptoms are associated with dilated membranous labyrinth filled with endolymph 2
    3. 3. 1. 1747 – Antonio Scarpa described anatomy of membranous labyrinth2. 1861 – Prosper Meniere described the classic features of Meniere’s disease & attributed it to labyrinthine causes3. 1871 – Knappin theorized that dilated membranous labyrinth to be the cause of this disorder4. 1927 – Guild described endolymphatic ciruclation5. 1938 – Hallpike and Portmann described pathology of Meniere’s disease by studying temporal bones. 3
    4. 4. 1. 150 years have passed since this syndrome was described2. Amount of literature accumulated has virtually doubled3. Only consensus reached so far is that its cause is multifactorial4. Not all individuals with histological features of Meniere’s disease manifested the classic clinical features (? Unknown factors protecting the individuals)5. Surgical destruction of sac ameliorates symptoms. (? What role does sac play exactly in endolymphatic circulation) 4
    5. 5. 1. Inner ear contains two types of fluids (perilyimph and endolymph separated by membranous labyrinth.2. Perilymph is similar in composition to CSF (Containing high Na and low K ions)3. Endolymph similar in composition to intracellular fluid (Containing low Na and high K concentration). It is secreted by stria vascularis 5
    6. 6.  Duct begins at ductus reuniens Duct is a single lumen tube about 2 mm long The duct narrows at the isthmus which lies at the level of vestibular aqueduct 6
    7. 7. 1. Secretory function2. Absorptive function3. Immune / defense function 7
    8. 8. 1. Aquaporins2. Glycoproteins like Saccain3. Endolymph4. Glycoproteins act as a driving force for longitudinal flow 8
    9. 9. 1. Longitudinal flow2. Radial flow3. Dynamic flow 9
    10. 10. 1. Was first proposed by Guild2. Striavascularis is the principal source3. This is a slow process4. Elimination occurs at the endolymphatic sac level 10
    11. 11. 1. First proposed by Lawrence2. This is a combination of both longitudinal and radial flow patterns 11
    12. 12. 1. This is active process (energy consuming)2. Production occurs from dark vestibular cells & planum semilunatum3. Absorption occurs at the striavestibularis 12
    13. 13. 1. This is a small membranous bulb located where the endolymphatic duct enters the vestibule2. This is where the volume of circulating endolymph is monitored3. Monitoring the volume of endolymph is not possible by sac because it will be interfered by CSF pressure and pressure exerted by lateral sinus 13
    14. 14. 14
    15. 15. 1. Composition of endolymph is maintained by stria vascularis by controlling the influx of water2. Normally endolymph is a biological puddle with very little radial / longitudinal flow3. Only under exceptional circumstances like increased endolymphatic fluid volumes does radial / longitudinal movement towards sac occurs4. Under normal circumstances radial flow alone is sufficient to maintain endolymph fluid balance and the longitudinal flow due to saccmechanics is not necessary5. The longitudinal flow is restricted by the isthmus portion of the duct which acts like the constriction seen in the hour glass 15
    16. 16. 16
    17. 17. 17
    18. 18. 18
    19. 19. 1. Small amounts of excess endolymph can be cleared by radial flow2. Larger volumes need longitudinal flow for their clearance3. Endolymphatic sinus temporarily accommodates excess endolymph till the sac is ready for it4. Endolymphatic valve of Bast isolates pars superior and prevents endolymph from draining out of the utricle 19
    20. 20. 1. Genetic causes2. Infection3. Otosclerosis4. Trauma (physical / acoustic)5. Syphilis6. Miscellaneous – Allergy, tumors, leukemia and autoimmune disorders 20
    21. 21. 1. Classical Meniere’s disease2. Vestibular Meniere’s disease – vestibular symptoms and aural pressure3. Cochlear Meniere’s disease – cochlear symptoms and aural pressure4. Lermoyez syndrome – Reverse Meniere’s5. Tumarkin’s crisis – Utricular Meniere’s 21
    22. 22. This is a variant of Meniere’s disease. It is characterized by sudden sensorineural hearing loss which improves during or immediately after the attack ofvertigo. 22
    23. 23. This variant is characterized by abrupt falling attacks of brief durationwithout loss of consciousness. This is caused due to an enlarging utricledue to excess endolymphatic volume. Utricular crisis is used to indicatethis condition.In the later disease stages the valve of Bast remaining patent may causesudden drainage of endolymph from the utricle due to longitudinal flowresulting in these drop attacks 23
    24. 24.  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 4th and 5th decades of life Bilateral Meniere’s syndrome is seen in 5% of these patients 24
    25. 25. 1. Endolymphatic hydrops causes distortion of membranous labyrinth2. Pressure building up in the scala media may cause mirco ruptures of membranous labyrinth3. This would account for the episodic nature of the attacks4. Healing of these ruptures causes resolution of the disorder 25
    26. 26. 1. Episodic vertigo rotatory in nature2. Ipsilateral hearing loss3. Aural fullness4. Roaring tinnitus5. Diplacusis 26
    27. 27. 1. Stage I – Patient has solely cochlear symptoms2. Stages II – IV – Patients have progressively more cochlear and vestibular symptoms3. Stage V – End stage Meniere’s disease (dead ear) 27
    28. 28. 1. Irritative nystagmus during the first 20 mins of attack2. Paralytic nystagmus follows3. Later recovery nystagmus starts 28
    29. 29.  Possible Meniere’s disease: Episodic vertigo of Meniere’s type without documented hearing loss Fluctuating hearing loss with disequilibrium but without definite episodes Probable Meniere’s disease: One definitive episode of vertigo Audiometrically documented hearing loss at least during one attack Definitive Meniere’s disease Two or more definitive episodes of spontaneous vertigo one atleast lasting for 20 mins. Audiometrically documented hearing loss at least on one occasion Tinnitus and aural fullness in the treated ear 29
    30. 30.  Sensori neural hearing loss combined with: Tinnitus now / in the past Vertigo attacks (at least two present now or in the past) Exclusion of other pathology following Groningen protocol Hearing loss: Sensori neural in nature No demonstrable conductive element Hearing loss of 20 dB or more at one of the usually measured audiometric thresholds Vertigo: Paroxysmal rotatory dizziness, accompanied by nausea / vomiting At least two episodes should be reported during a course of illness. One of the attack should last at least for 5 mins In between attacks there may be periods of unsteadiness 30
    31. 31. 1. Sensori neural in nature2. Fluctuating and progressive3. Affects low frequencies4. Mild low frequency conductive hearing loss (rare)5. Profound sensori neural hearing loss (End stage) 31
    32. 32.  Roaring in nature Could be continuous / intermittent Non pulsatile in nature Frequency of tinnitus corresponds to the region of cochlea which has suffered the maximum damage 32
    33. 33. 1. This is abnormal growth in the perceived intensity of sound2. This is usually positive in patients with Meniere’s disease3. ABLB is the test used to look for the presence of recruitment4. This test is really time consuming 33
    34. 34. 1. Increased summating potential / action potential ratio. 1:3 is normal2. Widened summating potential / action potential complex. A widening of greater than 2 ms is significant3. Small distorted cochlear microphonics 34
    35. 35. 1. Not mandatory for diagnosis of Meniere’s disease2. Caloric test is still performed3. It is low frequency stimulation (0.003 Hz) of lateral canal4. Caloric asymmetry will point to the diseased ear5. 20% difference between the two ears (Jongkee’s formula) is significant 35
    36. 36. 1. Vestibular evoked myogenic potential2. Measures the relaxation of sternomastoid muscle in response to ipsilateral click stimulus3. Brief high intensity ipsilateral clicks produce large short latency inhibitory potentials (VEMP) in the toncially contracted Ipsilateral sternomastoid muscle4. This test is due to the presence of vestibulo collic reflex5. Afferent arises from sound responsive cells in the saccule, conducted via the inferior vestibular nerve.6. Efferent is via vestibulo spinal tract7. Normal responses are composed of biphasic (positive-negative) waves8. VEMP reveals saccular dysfunction 36
    37. 37. 1. Glycerol2. Frusemide3. Isosorbide4. Tests are positive if there is pure tone improvement of 10dB or more at two / more frequencies between 200-2000Hz 37
    38. 38. 1. First introduced by Klockhoff and Lindblom – 19662. Glycerol is administered in doses of 1.5 mg/kg body wt in empty stomach3. Serum osmolality should increase at least by 10 mos/kg4. Side effects include Headache, Nausea, vomiting, drowsiness5. PTA is performed 2-3 hours after administration6. False positivity is rare7. Positivity depends on the phase of the disease 38
    39. 39. 1. Dietary management2. Physiotherapy3. Psychological support4. Pharmacological intervention 39
    40. 40. 1. Intravenous fluids – dehydration2. Vestibular suppressants – May delay recovery / rehabilitation process3. Corticosteroids – May help if tinnitus and deafness are debilitating 40
    41. 41. 1. Frustenberg diet2. 2 grams / 24 hours (restricted salt intake)3. Life style modification 41
    42. 42. 1. Diuretics play a vital role in alleviating acute symptoms2. This has been in use since 1930’s3. Thiazide group of drugs are commonly used4. Frusemide may be used to alleviate acute symptoms5. Clear scientific evidence is lacking regarding the usefulness of diuretics (cochrane review) 42
    43. 43. 1. Cochlear vascular insufficiency has been proposed as one of the mechanism of Menieres disease2. Betahistine is supposed to cause vasodilatation of cochlear blood vessels3. Betahistine has weak H1 agonistic property and considerable H3 antagonist properties4. It reduces the frequency & intensity of vertigo. Has minimal effect on tinnitus5. Doesn’t help much with hearing loss (Cochrane review) 43
    44. 44. 1. Immune modulating effects2. Improves fluid dynamics of inner ear due to mineralocorticoid effects3. Vertigo was controlled on an immediate basis4. Methylprednisolone has the best effect as it penetrates the round window better5. Silverstein microwick can be used for intratympanic drug administration 44
    45. 45. 1. Isordil2. ϒ – globulin3. Urea4. Glycerol5. Lithium6. Anticholinergics – Glycopyrrolate 1-2 mg /day7. Antidopaminergics – Droperidol 2.5 – 10 mg orally / day8. Leuprolide acetate – Blocks normal sex hormone production9. Innovar – A combination of droperidol and fentanyl can be used to suppress vestibular symptoms (can replace endolymphatic sac surgery)10. Hyperbaric oxygen therapy 45
    46. 46. 1. Stress reduction2. Patient education3. Hearing aids – can be used to suppress troublesome tinnitus4. Tinnitus retraining 46
    47. 47. 1. Meniett Device2. Low pressure pulse generator3. Vibrations are transmitted via external auditory canal4. Vibrations alter inner ear fluid dynamics by their effects on the oval and round windows5. Exact mechanism of action is not known6. It is totally non invasive7. This device is portable 47
    48. 48. 1. Diagnosis should be confirmed2. Ventilation tube should be inserted3. Patient should be trained for self administration of the treatment4. Usually administered thrice a day about 5 mins each time5. Treatment lasts for 5 weeks 48
    49. 49. 1. Classic unilateral Meniere’s disease2. Intense vestibular / cochlear symptoms3. Failed medical therapy4. Over 65 years of age5. Imbalance / aural fullness / tinnitus after gentamycin treatment 49
    50. 50. 1. Perilymph fistula2. Acoustic neuroma / brain tumor3. Retrocochlear damage4. Low pressure hydrocephalus 50
    51. 51. 1. Vestibulotoxic effects are put to therapeutic use.2. Sensation of vertigo reduced while hearing is preserved3. Streptomycin / gentamycin are predominantly Vestibulotoxic4. Intratympanic administration is preferred 51
    52. 52. 1. Fixed dose protocol is used2. 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 grommet4. 1ml of gentamycin solution is injected into the middle ear cavity via the microcatheter5. Three injections are given per day in outpatient setting6. Injections are given for 4 days7. After injection patient should lie supine with the infiltrated ear up for 30 mins8. Vertigo usually develops between 2-4 days after cessation of treatment 52
    53. 53. 1. Sac enhancement procedure2. Sac decompression procedure3. Labyrinthine ablative procedures 53
    54. 54. 1. External shunts – Drains the sac into mastoid cavity / subarachnoid space2. Internal shunts – Drains excessive endolymph into the perilymphatic space (cochleosacculotomy / labyrinthotomy) 54
    55. 55. 55
    56. 56. 1. Helpful in treating debilitated patients2. Involves disruption of osseous spiral lamina3. Angular pick introduced via round window towards oval window. It will accommodate 3 mm long pick4. After perforation the pick is withdrawn and the round window is sealed by fat 56
    57. 57. 1. Labyrinthectomy2. Translabyrinthine vestibular neurectomy3. Retrolabyrinthine vestibular neurinectomy4. Retrosigmoid vestibular neurinectomy5. Middle cranial fossa vestibular neurinectomy 57