menniere's disease

1. MENIERE’S DISEASE
PRESENTER : DR SHWETA SHARMA

2. • DEFINITION
• REVIEW OF LITERATURE (HISTORY)
• INCIDENCE
• REVIEW OF ANATOMY
• AETIOPATHOLOGY
• DIAGNOSIS
• MANAGEMENT

3. DEFINITION
Cause unknown

4. HISTORY
150 years & still elusive…..
• Prosper Meniere first described the symptom complex
in 1861 and proposed the pathologic site to be in the
labyrinth.
• In 1871 Knapp advanced the hypothesis that hydrops
was similar to ocular glaucoma : aural glaucoma
• In 1938 Hallpike and Cairns described the underlying
pathology endolymphatic hydrops but the precise
etiology still remains elusive

5. INCIDENCE
• The disease seems to be more prevalent among
whites, with a variable female-to-male ratio
( ranging from 1:1, 1.3:1, 2:1 ; female
preponderance ).
• The peak age at onset is in the fourth and fifth
decades of life, although presentation can be at
almost any age.
• The incidence of bilateral disease probably is in
the range of 19% to 24%.

6. • Familial occurrence of Meniere’s disease has
been reported in 10% to 20% (An AD mode)
Migraine is strongly associated in such cases.
• The incidence is elevated in persons with
specific major histocompatability complexes
(MHCs).
• Human leukocyte antigens (HLA) B8/DR3 and
Cw7 have been associated with Meniere’s
disease.

7. REVIEW OF ANATOMY

9. ENDOLYMPHATIC SYSTEM
ED short single lumen tube ~ 2 mm
Isthmus is the narrowest 0.09 mm x 0.2 mm
Intraooeous 6 – 15 mm L x 3 – 15 mm W
Extraosseous 10 – 15 mm L x 5 – 7 mm W

10. Functions of ENDOLYMPHATIC sac
1. Resorption of the water content of endolymph
2. Ability to participate in some ionic exchanges with
endolymph
3. Removal of metabolic and cellular debris, including otoconia
4. Immunodefense function ( perisaccular )
5. Inactivation and removal of viruses
6. Secretion of Glycoproteins to attract extra fluid
(Glycoproteins act as a driving force for longitudinal flow)
1. Secretion of Saccin to increase Endolymph production

14. AETIOPATHOLOGY

15. Aetiology
• Anatomical-abnormalities
• Genetic- AD
• Immunological - immune complex deposition
• Viral-serum IgE to herpes simples virus types I and II,
Epstein-Barr virus and CMV
• Vascular-associated with migraines
• Metabolic-potassium intoxication

17. PATHOPHYSIOLOGY

19. DRAINAGE THEORY

20. CLINICAL PRESENTATION

21. Clinical presentation
• The typical history consists of recurring attacks of
vertigo (96.2%) with tinnitus (91.1%) and ipsilateral
hearing loss (87.7%).
• Attacks often are preceded by an aura consisting of
a sense of fullness in the ear, increasing tinnitus, and
a decrease in hearing.
• cochlear and vestibular Meniere’s disease

22. • Otolithic crisis of Tumarkin
• Lermoyez syndrome

23. Physical Examination
• Examination results vary, depending upon the phase
of disease.
• During an acute attack, the patient has severe
vertigo.
• Spontaneous nystagmus directed toward affected ear
is typical during an acute attack.
1. Irritative nystagmus during the first 20 mins of attack
2. Paralytic nystagmus follows
3. Later recovery nystagmus starts

24. Hearing Loss and Tinnitus
• Sensorineural hearing loss is fluctuating and progressive, with the
sensation of fullness or pressure in the ear.
• In only 1% to 2% of patients does the hearing loss progress to
profound deafness.
• Additional features include diplacusis, a difference in the
perception of pitch between the ears (43.6%) and recruitment
(56%) .
• Tinnitus tends to be nonpulsatile and variously described as
whistling or roaring. It may be continuous or intermittent. Tinnitus
often begins, gets louder, or changes pitch as an attack approaches.
Frequently a period of improvement follows the attack.

25. • The Romberg test generally shows significant instability
and worsening when the eyes are closed.
• The Weber tuning fork test usually lateralizes away from
the affected ear.
• The Rinne test usually indicates that air conduction
remains better than bone conduction.
• Complete neurologic evaluation is important. New-onset
vertigo might be an early sign of stroke, migraine, or
brainstem compression that may require emergent
evaluation and care.

26. Particularly helpful to document present hearing acuity and to
detect future change.
-The patient may not notice a loss at specific frequencies.
Low-frequency or mixed low- and high-frequency
insufficiency may be observed.
- Typically, the lower frequencies are affected more
severely. This is due to preferential sensitivity of the apex to
the hydrops.
- Multiple hearing tests, which document fluctuating
hearing loss, are helpful in diagnosing Ménière.
Audiometry

27. A pattern of low-frequency fluctuating loss and a coincident nonchanging, high-frequency loss is
described, resulting in a “peaked” or “tent-like” tracing on the audiogram. This peak classically
occurs at 2 kHz.

28. Audiogram typical of early Meniere's disease on the right side
(x=left, o=right). There is a low-tone sensorineural hearing loss.

29. Audiogram typical of middle-stage Meniere's disease, again on
the right side. Hearing is reduced at all frequencies, but more so
at high and low frequencies.

30. Audiogram typical of late-stage Meniere's disease, again on the
right side. Hearing is flat, and unaidable on the right side.

31. Recruitment
•Alternate binaural loudness balance test (ABLB)
•Short increment sensitivity index (SISI)

32. Imaging Studies
• Magnetic resonance imaging
- R/o abnormal anatomy or mass lesions. Specifically,
acoustic neuromas or CP angle lesions. Other lesions, such
as multiple sclerosis or Arnold-Chiari malformations, also can
be ruled out.
• CT scans reveal dehiscent superior semicircular
canals and/or widened cochlear and vestibular
aqueducts

33. DIAGNOSIS

34. AAO-HNS Criteria for Meniere’s Disease
Diagnosis
Major Symptoms
1.Vertigo
– Recurrent, well-defined episodes of spinning or rotation • Duration from 20 minutes to
24 hours.
– Nystagmus associated with attacks
– Nausea and vomiting during vertigo spells common
– No neurologic symptoms with vertigo
1.Deafness
– Hearing deficits fluctuate
– Sensorineural hearing loss
– Hearing loss progressive, usually unilateral
1.Tinnitus
– Variable, often low-pitched and louder during attacks
– Usually unilateral
– Subjective

35. • Possible Meniere’s disease
1. Episodic vertigo without hearing loss or
2. Sensorineural hearing loss, fluctuating or fixed, with dysequilibrium, but
without definite episodes
3. Other causes excluded
• Probable Meniere’s disease
1. One definitive episode of vertigo
2. Hearing loss documented by audiogram at least once
3. Tinnitus or sense of aural fullness in the presumed affected ear
4. Other causes excluded
• Definite Meniere’s disease
1. Two or more definitive spontaneous episodes of vertigo lasting at least 20
minutes
2. Audiometrically documented hearing loss on at least one occasion
3. Tinnitus or sense of aural fullness in the presumed affected ear
4. Other causes excluded
• Certain Meniere’s disease
– Definite Meniere’s disease, plus histopathologic confirmation

36. AAO-HNS Criteria for Meniere’s Disease
Severity
Vertigo
a. Any treatment should be evaluated after at least 24 months.
b. Formula to obtain numeric value for vertigo:
ratio of average number of definitive spells per month after therapy divided by
definitive spells per month before therapy (averaged over a 24-month period) ×
100 = numeric value.
Numeric value scale Control Class
0 Complete control of definitive
spells
A
0-40 Limited control of definitive
spells
B
41-80 Insignificant control of
definitive spells
C
81-120 D
>120 Secondary treatment initiated E

37. Disability
a. No disability
b. Mild disability: intermittent or continuous
dizziness/unsteadiness that precludes working in
a hazardous environment.
c. Moderate disability: intermittent or continuous
dizziness that results in a sedentary occupation
d. Severe disability: symptoms so severe as to
exclude gainful employment

38. Hearing
• Hearing is measured using a four-frequency pure-tone average
(PTA) of 500 Hz, 1 kHz, 2 kHz, and 3 kHz.
• Pretreatment hearing level: worst hearing level during 6 months
before therapy
• Post-treatment hearing level: poorest hearing level measured 18-24
months after institution of therapy
• Hearing classification:
i. Unchanged: ≤10-dB PTA improvement or worsening or ≤15% speech
discrimination improvement or worsening.
ii. Improved: >10-dB PTA improvement or >15% discrimination
improvement
iii. Worse: >10-dB PTA worsening or >15% discrimination worsening

39. • In 1996, the Committee on Hearing and
Equilibrium reaffirmed and clarified the
guidelines, adding initial staging and reporting
guidelines:
• Initial Hearing Level
Stage Four tone average
1 < 25
2 26-40
3 41-70
4 >70

40. Differential diagnosis
Central
•Acoustic neuroma
•Multiple sclerosis
•Vascular loop compression
syndrome
•Aneurysm
•Vascular insufficiency
•Arnold-chiari malformation
•Cerebellar or brainstem
tumors
•Cervical vertigo
•Transient ischemic attacks/
CVA
Peripheral
•BPPV
•Labyrynthitis
•Perilymphatic fistula
•Otosclerosis
•Migraine induced vertigo
Metabolic
•Diabetes
•Hyper/hypothyroidism
•Syphilis
•Cogan’s syndrome
•Anemia
•Autoimmune disorders

41. MANAGEMENT

42. Other SPECIAL INVESTIGATIONS
• Electronystagmography
• Head Thrust Testing
• Electrocochleography
• Dehydrating Agents
• Vestibular evoked Myopotentials

43. Electronystagmography
• Electrooculographic recordings of eye movements
after caloric and rotational stimulation are a
commonly available and reliable method of assessing
vestibular function.
• The caloric test often can localize the involved ear. A
significant caloric response reduction is found in 48%
to 73.5% of patients with Meniere’s disease.
• As many as 25% of meniere’s disease patients have
no abnormalities

44. Head Thrust Testing
• The head thrust test popularized by Halmagyi
is very sensitive for detection of unilateral
vestibular dysfunction.
• However, in Meniere’s disease, the
asymmetry is subtle and present in only 29%
of the patients.

45. Electrocochleography
• The summating potential (SP) is larger and more negative(( due to
distention of the basilar membrane into the scala tympani, causing an
increase in the normal asymmetry of its vibration).
• Ratio of amplitudes of the SP and the 8th
cranial nerve action potential
(AP), the SP/AP ratio.
• The SP becomes relatively larger in hydrops; accordingly, the SP/AP ratio
increases; SP:AP > 0.45
• It is not a definitive test, because ratios are elevated in 62% of patients
with Meniere’s disease and in 21% of control subjects.

47. Dehydrating Agents
• Glycerol (1.5ml/kg body weight) : half an hour to one hour intervals with
atleast 5dB improvement in three consecutive frequencies.
• Improvement seen with audiometrics, reduction in SP negativity (as
recorded with ECoCh), or a change in the gain of the vestibulo-ocular
response to rotational stimulation.

48. Vestibular Evoked Myopotentials
• VEMPs are generated by playing loud clicks in the ear, which move the
stapes footplate and stimulate the saccule. This is the start of a disynaptic
pathway that passes through the vestibular nuclei and then to synapses
that relax the sternocleomastoid muscle.
• The saccule is the second most common site affected by hydrops, which
has caused VEMPs to be investigated as a potential diagnostic tool. In the
normal ear, the best response is near 500 Hz.
• Ears affected by Meniere’s disease have elevated VEMP thresholds with
flattened tuning.
• The interaural amplitude difference in the response has been implicated
as a staging tool for Meniere’s disease.

50. TREATMENT
• Dietary modifications and diuretics
• Vasodilators
• Symptomatic treatment
– Antiverginous medications
– Antiemetics
– Sedatives
– Antidepressants
– Psychiatric treament
• Local overpressure therapy

51. Dietary modifications
• Low sodium (1500 mg) diet (1 month trial)
• Furstenberg diet
• Restriction in caffeine, nicotine and alcohol.

52. Medications

53. • Salt wasting diuretic such as diazide (1 month trial)
• Betahistine (2 week trial, often combined with
verapamil)
– Relaxes the precapillary sphincters and thus improves the
microcirculation of inner ear
– Antivertigo action due to imhibition of massive impulses to
the polysynaptic lateral vestibular nucleus)
• Verapamil 30 mg SR (one month trial -- dyazide is
stopped)
• Lipoflavins and vitamins ( hypothetical importance )

54. Local overpressure therapy
• Since 2000, the Meniett device has been approved
for use by the U.S. Food and Drug Administration
(FDA).
• A randomized controlled trial demonstrated that
patients using the Meniett device experienced a
significant decrease in vertigo symptoms for the first
3 months of therapy but that findings afterward
were similar to those with placebo.

55. Restoring balance in the hydrodynamic system of
the inner ear by applying low-pressure pulses to the
middle ear

57. Surgical treatment
• Intratympanic injection
• Endolymphatic sac surgery
• Nerve section
• Labyrinthectomy

58. Intratympanic Injection
• Intratympanic injection commonly is performed with either dexa-
methasone or gentamicin for control of vertigo symptoms.
• The term chemical labyrinthectomy often is applied to intratympanic
gentamicin treatment, but it may not be an appropriate assessment of the
effect of gentamicin on the labyrinth in titrated therapy.
• Instillation of aminoglycosides into the middle ear was described by
Schuknecht in 1957 with streptomycin injection through a microcatheter
placed through the tympanic membrane.
• Control of vertigo was achieved in these patients, but severe hearing loss
in the treated ear also occurred in most patients.

59. Dexamethasone
• To be offered when vertigo is intractable but the patient still has some
functional hearing.
• The risk of hearing loss or other complications from the steroid injection
appears to be low.
• A small randomized trial has shown complete resolution of vertigo
symptoms in 82% of patients receiving dexamethasone, compared with
57% receiving saline injection.
• Dexamethasone injections may need to be repeated every 3 months to
maintain the patient free of vertigo symptoms, although the optimal
dosing frequency is variable and unknown.
• Concentrations used have varied from 2 to 24 mg/mL.

60. Shea gave:
After giving puncture in the TM, with needle being just above the round window
0.5ml of hyaluronidase & 1 ml of 16mg dexamethasone mixed together; injected in ME
Pt instructed to lie down for 3 hrs with injected ear up, entire process repeated 3
consecutive days

61. Gentamicin
• Has a vestibulotoxicity that is high relative to its cochleotoxicity;
accordingly, it can be used to control vestibular symptoms while sparing
hearing.
• The gentamicin can be administered through either a tympanostomy tube
or directly injected through the tympanic membrane.
• Peripheral vestibular deficits are evident on head thrust testing after even
a single dose of gentamicin

62. • 40 mg/ml gentamycin is buffered with soda bicarb (pH6.4) final concentration 26.7mg/ml.
• 0.7ml of gentamicin injection mixed with 0.3ml of sodium bicarbonate solution
• Three injections are given per day in outpatient setting
• Injections are given for 4 days
• After injection patient should lie supine with the infiltrated ear up for 30 mins

63. • The current trend is away from multiple doses of
gentamicin and toward a single-injection regimen,
with additional doses only if needed to control
symptoms (so-called titration therapy).
• The risk of hearing loss with gentamicin using many
current protocols is similar to that with the natural
history of Meniere’s disease.

64. Nerve section
1.Translabyrinthine vestibular neurectomy
2.Retrolabyrinthine vestibular neurinectomy
3.Retrosigmoid vestibular neurinectomy
4.Middle cranial fossa vestibular neurinectomy

65. SUMMARY

66. Prognosis
• Prognosis is variable, since the disease pattern
of exacerbation and remission makes evaluation
of treatment and prognosis difficult to predict.
– In general, Ménière symptoms tend to stabilize
spontaneously with time. With regard to vertigo, about
half of patients stabilize over several years.
– Patients tend to "burn out" over time and with residual
poor balance and hearing.