Dr T Balasubramanian MS DLO

skillfulbuyerUrban and Civil

Nov 16, 2013 (3 years and 10 months ago)

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Dr T Balasubramanian MS DLO

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Meniere’s disease is defined as a symptom complex associated with:

1.
Roaring tinnitus

2.
Sensorineural hearing loss (Low frequency)

3.
Vertigo (episodic)

4.
Fullness of the ear

5.
These symptoms are associated with dilated membranous
labyrinth filled with endolymph


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1.
1747


Antonio
Scarpa

described anatomy of membranous
labyrinth

2.
1861


Prosper Meniere described the classic features of
Meniere’s disease & attributed it to labyrinthine causes

3.
1871


Knappin

theorized that dilated membranous labyrinth to
be the cause of this disorder

4.
1927


Guild described endolymphatic
ciruclation

5.
1938


Hallpike

and
Portmann

described pathology of Meniere’s
disease by studying temporal bones.


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1.
150 years have passed since this syndrome was described

2.
Amount of literature accumulated has virtually doubled

3.
Only consensus reached so far is that its cause is multifactorial

4.
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)


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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

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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

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1.
Secretory function

2.
Absorptive function

3.
Immune / defense function

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1.
Aquaporins

2.
Glycoproteins like
Saccain

3.
Endolymph

4.
Glycoproteins act as a driving force
for longitudinal flow

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1.

Longitudinal flow

2.

Radial flow

3.

Dynamic flow

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1.
Was first proposed by Guild

2.
Striavascularis

is the principal source

3.
This is a slow process

4.
Elimination occurs at the endolymphatic
sac level

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1.
First proposed by Lawrence

2.
This is a combination of both
longitudinal and radial flow patterns

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1.
This is active process (energy consuming)

2.
Production occurs from dark vestibular cells &
planum

semilunatum

3.
Absorption occurs at the
striavestibularis

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1.
This is a small membranous bulb located where the
endolymphatic duct enters the vestibule

2.
This is where the volume of circulating endolymph is monitored

3.
Monitoring the volume of endolymph is not possible by sac
because it will be interfered by CSF pressure and pressure
exerted by lateral sinus


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1.
Composition of endolymph is maintained by
stria

vascularis

by controlling
the influx of water

2.
Normally endolymph is a biological puddle with very little radial /
longitudinal flow

3.
Only under exceptional circumstances like increased endolymphatic fluid
volumes does radial / longitudinal movement towards sac occurs

4.
Under normal circumstances radial flow alone is sufficient to maintain
endolymph fluid balance and the longitudinal flow due to
saccmechanics

is
not necessary

5.
The longitudinal flow is restricted by the isthmus portion of the duct which
acts like the constriction seen in the hour glass



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1.
Small amounts of excess endolymph can be cleared by radial flow

2.
Larger volumes need longitudinal flow for their clearance

3.
Endolymphatic sinus temporarily accommodates excess endolymph till
the sac is ready for it

4.
Endolymphatic valve of
Bast

isolates pars superior and prevents
endolymph from draining out of the utricle

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1.
Genetic causes

2.
Infection

3.
Otosclerosis

4.
Trauma (physical / acoustic)

5.
Syphilis

6.
Miscellaneous


Allergy, tumors, leukemia and autoimmune disorders


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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

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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.

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This variant is characterized by abrupt falling attacks of brief duration
without loss of consciousness. This is caused due to an enlarging utricle
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

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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 4
th

and 5
th

decades of life



Bilateral Meniere’s syndrome is seen in 5% of these patients


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1.

Endolymphatic
hydrops

causes distortion of membranous labyrinth

2.

Pressure building up in the
scala

media may cause
mirco

ruptures of
membranous labyrinth

3.

This would account for the episodic nature of the attacks

4.

Healing of these ruptures causes resolution of the disorder

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1.

Episodic vertigo rotatory in nature

2.

Ipsilateral hearing loss

3.

Aural fullness

4.

Roaring tinnitus

5.

Diplacusis

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1.

Stage I


Patient has solely cochlear symptoms

2.

Stages II


IV


Patients have progressively more cochlear and
vestibular symptoms

3.

Stage V


End stage Meniere’s disease (dead ear)

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1.

Irritative nystagmus during the first 20 mins of attack

2.

Paralytic nystagmus follows

3.

Later recovery nystagmus starts

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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

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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


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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)

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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

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1.

This is abnormal growth in the perceived intensity of sound

2.

This is usually positive in patients with Meniere’s disease

3.

ABLB is the test used to look for the presence of recruitment

4.

This test is really time consuming

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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

3.

Small distorted cochlear
microphonics




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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

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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

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1.
Glycerol

2.
Frusemide

3.
Isosorbide

4.
Tests are positive if there is pure tone improvement of 10dB or more
at two / more frequencies between 200
-
2000Hz

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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


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1.

Dietary management

2.

Physiotherapy

3.

Psychological support

4.

Pharmacological intervention

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1.

Intravenous fluids


dehydration

2.

Vestibular suppressants


May delay recovery / rehabilitation process

3.

Corticosteroids


May help if tinnitus and deafness are debilitating

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1.

Frustenberg

diet

2.

2 grams / 24 hours (restricted salt intake)

3.

Life style modification

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41

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
(
cochrane

review)

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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 (Cochrane review)


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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

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1.

Isordil

2.

ϒ



globulin

3.

Urea

4.

Glycerol

5.

Lithium

6.

Anticholinergics


Glycopyrrolate 1
-
2 mg /day

7.

Antidopaminergics



Droperidol 2.5


10 mg orally / day

8.

Leuprolide acetate


Blocks normal sex hormone production

9.

Innovar


A combination of droperidol and fentanyl can be used to
suppress vestibular symptoms (can replace endolymphatic sac surgery)

10.

Hyperbaric oxygen therapy



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1.

Stress reduction

2.

Patient education

3.

Hearing aids


can be used to suppress troublesome tinnitus

4.

Tinnitus retraining

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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

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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

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48

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

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1.

Perilymph fistula

2.

Acoustic neuroma / brain tumor

3.

Retrocochlear damage

4.

Low pressure hydrocephalus

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50

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

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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


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52

1.
Sac enhancement procedure

2.
Sac decompression procedure

3.
Labyrinthine ablative procedures

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1.

External shunts


Drains the sac into mastoid cavity / subarachnoid space

2.

Internal shunts


Drains excessive endolymph into the
perilymphatic

space
(
cochleosacculotomy

/
labyrinthotomy
)

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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

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1.
Labyrinthectomy

2.
Translabyrinthine

vestibular
neurectomy

3.
Retrolabyrinthine

vestibular
neurinectomy

4.
Retrosigmoid

vestibular
neurinectomy

5.
Middle cranial fossa vestibular
neurinectomy

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