ppt on meniers disease

1. MENIERE’S DISEASE
PRESENTER
DR. DEEPA SHIVNANI

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. 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. 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. 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

7. 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

8. 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.

9. 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

10. • 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

11. Etiology

12. 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

13. .

14. Normal membranous labyrinth
Dilated membranous labyrinth
in Meniere's disease (Hydrops)

21. 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

23. 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

24. • 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

25. MECHANISM OF MENIERE’S
DISEASE

26. 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)

27. 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

28. Diagnosis

29. Investigations
• Tuning forks tests :
SNHL
• PTA
• Speech audiometry
• Recruitment test +ve
• SISI >70%
• Tone decay <20 dB

30. 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

31. 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.

32. 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

33. 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

34. 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

35. 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

36. VEMPs

37. 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.

38. 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

39. 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.

40. MEDICAL MANAGEMENT
1. Dietary management
2. Physiotherapy
3. Psychological support
4. Pharmacological intervention

41. 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

42. MEDICAL TREATMENT
Acute Therapy

43. 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

44. LOW SALT DIET
1. Frustenberg diet
2. 2 grams / 24 hours (restricted salt
intake)
3. Life style modification

45. 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

46. 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

47. 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

48. OTHER TREATMENT
MODALITIES (ANCILLARY)
1. Stress reduction
2. Patient education
3. Hearing aids – can be used to suppress
troublesome tinnitus
4. Tinnitus retraining

52. 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

53. 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

54. 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

55. CONTRAINDICATIONS FOR VIBRATOR
THERAPY
1. Perilymph fistula
2. Acoustic neuroma / brain tumor
3. Retrocochlear damage
4. Low pressure hydrocephalus

56. 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

57. 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

58. SURGICAL MANAGEMENT
1.Sac enhancement procedure
2.Sac decompression procedure
3.Labyrinthine ablative procedures

59. 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

60. ABLATIVE PROCEDURES
1.Labyrinthectomy
2.Translabyrinthine vestibular neurectomy
3.Retrolabyrinthine vestibular neurinectomy
4.Retrosigmoid vestibular neurinectomy
5.Middle cranial fossa vestibular
neurinectomy

61. 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)

63. 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

64. Endolymphatic Sac Surgery

66. SAC IDENTIFICATION

68. THANK YOU……

69. ANY QUESTION?????