Your SlideShare is downloading. ×
Meniere’s disease
Upcoming SlideShare
Loading in...5
×

Thanks for flagging this SlideShare!

Oops! An error has occurred.

×

Introducing the official SlideShare app

Stunning, full-screen experience for iPhone and Android

Text the download link to your phone

Standard text messaging rates apply

Meniere’s disease

4,120
views

Published on

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

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

Published in: Health & Medicine

0 Comments
4 Likes
Statistics
Notes
  • Be the first to comment

No Downloads
Views
Total Views
4,120
On Slideshare
0
From Embeds
0
Number of Embeds
1
Actions
Shares
0
Downloads
187
Comments
0
Likes
4
Embeds 0
No embeds

Report content
Flagged as inappropriate Flag as inappropriate
Flag as inappropriate

Select your reason for flagging this presentation as inappropriate.

Cancel
No notes for slide
  • 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.
  • Transcript

    • 1. Dr T Balasubramanian MS DLO www.drtbalu.com 1
    • 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 www.drtbalu.com 2
    • 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. www.drtbalu.com 3
    • 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) www.drtbalu.com 4
    • 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 www.drtbalu.com 5
    • 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 www.drtbalu.com 6
    • 7. 1. Secretory function2. Absorptive function3. Immune / defense function www.drtbalu.com 7
    • 8. 1. Aquaporins2. Glycoproteins like Saccain3. Endolymph4. Glycoproteins act as a driving force for longitudinal flow www.drtbalu.com 8
    • 9. 1. Longitudinal flow2. Radial flow3. Dynamic flow www.drtbalu.com 9
    • 10. 1. Was first proposed by Guild2. Striavascularis is the principal source3. This is a slow process4. Elimination occurs at the endolymphatic sac level www.drtbalu.com 10
    • 11. 1. First proposed by Lawrence2. This is a combination of both longitudinal and radial flow patterns www.drtbalu.com 11
    • 12. 1. This is active process (energy consuming)2. Production occurs from dark vestibular cells & planum semilunatum3. Absorption occurs at the striavestibularis www.drtbalu.com 12
    • 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 www.drtbalu.com 13
    • 14. www.drtbalu.com 14
    • 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 www.drtbalu.com 15
    • 16. www.drtbalu.com 16
    • 17. www.drtbalu.com 17
    • 18. www.drtbalu.com 18
    • 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 www.drtbalu.com 19
    • 20. 1. Genetic causes2. Infection3. Otosclerosis4. Trauma (physical / acoustic)5. Syphilis6. Miscellaneous – Allergy, tumors, leukemia and autoimmune disorders www.drtbalu.com 20
    • 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 www.drtbalu.com 21
    • 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. www.drtbalu.com 22
    • 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 www.drtbalu.com 23
    • 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 www.drtbalu.com 24
    • 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 www.drtbalu.com 25
    • 26. 1. Episodic vertigo rotatory in nature2. Ipsilateral hearing loss3. Aural fullness4. Roaring tinnitus5. Diplacusis www.drtbalu.com 26
    • 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) www.drtbalu.com 27
    • 28. 1. Irritative nystagmus during the first 20 mins of attack2. Paralytic nystagmus follows3. Later recovery nystagmus starts www.drtbalu.com 28
    • 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 www.drtbalu.com 29
    • 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 www.drtbalu.com 30
    • 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) www.drtbalu.com 31
    • 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 www.drtbalu.com 32
    • 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 www.drtbalu.com 33
    • 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 www.drtbalu.com 34
    • 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 www.drtbalu.com 35
    • 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 www.drtbalu.com 36
    • 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 www.drtbalu.com 37
    • 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 www.drtbalu.com 38
    • 39. 1. Dietary management2. Physiotherapy3. Psychological support4. Pharmacological intervention www.drtbalu.com 39
    • 40. 1. Intravenous fluids – dehydration2. Vestibular suppressants – May delay recovery / rehabilitation process3. Corticosteroids – May help if tinnitus and deafness are debilitating www.drtbalu.com 40
    • 41. 1. Frustenberg diet2. 2 grams / 24 hours (restricted salt intake)3. Life style modification www.drtbalu.com 41
    • 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) www.drtbalu.com 42
    • 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) www.drtbalu.com 43
    • 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 www.drtbalu.com 44
    • 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 www.drtbalu.com 45
    • 46. 1. Stress reduction2. Patient education3. Hearing aids – can be used to suppress troublesome tinnitus4. Tinnitus retraining www.drtbalu.com 46
    • 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 www.drtbalu.com 47
    • 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 www.drtbalu.com 48
    • 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 www.drtbalu.com 49
    • 50. 1. Perilymph fistula2. Acoustic neuroma / brain tumor3. Retrocochlear damage4. Low pressure hydrocephalus www.drtbalu.com 50
    • 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 www.drtbalu.com 51
    • 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 www.drtbalu.com 52
    • 53. 1. Sac enhancement procedure2. Sac decompression procedure3. Labyrinthine ablative procedures www.drtbalu.com 53
    • 54. 1. External shunts – Drains the sac into mastoid cavity / subarachnoid space2. Internal shunts – Drains excessive endolymph into the perilymphatic space (cochleosacculotomy / labyrinthotomy) www.drtbalu.com 54
    • 55. www.drtbalu.com 55
    • 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 www.drtbalu.com 56
    • 57. 1. Labyrinthectomy2. Translabyrinthine vestibular neurectomy3. Retrolabyrinthine vestibular neurinectomy4. Retrosigmoid vestibular neurinectomy5. Middle cranial fossa vestibular neurinectomy www.drtbalu.com 57