2. Menière’s disease is a disorder characterized
by spontaneous attacks of vertigo, with
associated fluctuating sensorineural hearing
loss, tinnitus and aural fullness. It is a
common cause of the syndrome of
spontaneous vertigo.
3. INCIDENCE
Wide variation
Reports vary from 10.7 per 100,000 in the Japanese
population to a high of 513 per 100,000 in the
population
The frequency of bilateral disease is unclear, and the
incidence in published reports is from 2% to 78%.
5. Peripheral
o Semicircular canals-crista ampullaris and the cupula
o Utricle and saccule(otolith organs) –maculae
Central
Vestibular nuclei in pons and medulla
7. FLUIDS OF LABYRINTH
1. Perilymph
• Between bony and membranous
labyrinth
• Composition similar to
cerebrospinal fluid
• Rich in sodium,low potassium
• Derived from CSF or blood
vessel
2. Endolymph
• In membranous labyrinth
• high potassium, low Na
• Secreted by stria-vascularis
• Believed to be absorbed by
endolymphatic sac
8. ENDOLYMPHATIC CIRCULATION
Predominantly derived from stria vascularis
Small amount contributed by planum semilunatum and dark
vestibular cells
2 types of circulation
1. Longitudinal pattern (slow process) Both occur
2. Radial flow pattern (rapid process) concurrently
9. LONGITUDINAL PATTERN
Produced by Stria Vascularis of
the cochlea
Scala media
Saccular duct
Vestibular Labyrinth
Endolymphatic sac-where
it is absorbed
Ductus reuniens
Vestibular aqueduct
11. VESTIBULAR NERVE CONNECTIONS
Afferents
Peripheral receptors
Cerebellum
Reticular formation
Spinal cord
Contralateral vestibular nuclei
Efferents
Extra ocular motor nuclei
Motor part of spinal cord
cerebellum
12. Autonomic nervous system
Vestibular nuclei of opposite side
Cerebral cortex-temporal lobe
Distal processes of bipolar neurons of
vestibular ganglion innervate the sensory
epithelium while the central processes
aggregate to form vestibular nerve.
14. Dr. Prosper Menière(1799-1862)-
1. French physician from Paris
2. First described the symptom complex of the eponymous
disease in 1861
3. Proposed the pathologic site to be in the labyrinth.
Menière, along with Flourens in 1842; recognized that
vertiginous symptoms could originate in the inner ear;
however, ascribing this to hemorrhage into the inner ear,
which Menière himself believed to be the
pathophysiology, has proven to be erroneous.
Interestingly, the patient he used in his initial description
probably had leukemia and not the disease that now
bears his name.
1871: Knapp’s gave the first insight into the true
pathophysiology of Menière disease with the hypothesis
that inner ear hydrops was similar to glaucoma.
In 1927, Guild
1. produced the first description of the longitudinal flow
of endolymph,
2. accurately identified the stria vascularis as the
principal source of endolymph and the endolymphatic
sac as the site of endolymphatic ‘outflow’.
15. Dr.Georges Portmann- first endolymphatic
sac drainage operation for the treatment of
vertigo in 1926
1928: Dandy proposed selective vestibular
nerve section via a suboccipital approach
and treated over 600 patients.
1938: Hallpike and Cairns described
endolymphatic hydrops as the principal
pathologic feature of Menière disease.
1965: Kimura and Schuknecht produced
the first consistent animal model of
endolymphatic hydrops.
Understanding of this disease has
advanced considerably since these initial
descriptions, yet the cause of the
underlying hydrops remains elusive and
controversial despite 70 years of active
research.
16. The famous painter Vincent Van Gogh is believed to have been suffering from
Menière disease. It is thought that the severe symptoms of uncontrolled Menière
disease caused his psychological turmoil and finally, his famous episode of auricular
amputation.
Vincent Van Gogh (1853-1890)
18. Menière disease appears to be one member of the
group of disorders of the inner ear linked by the
common pathophysiological condition of
endolymphatic hydrops.
The low-tone fluctuating hearing loss, together
with the fact that glycerol may temporarily
improve the hearing, suggests the presence of
endolymphatic overpressure in Menière disease.
Endolymphatic hydrops is a physical distortion in
the membranous labyrinth.
Endolymphatic hydrops occurs due to –
1. Overproduction or
2. Inadequate absorption
Prevelant theory: endolymphatic malabsorption in
longitudinal flow.
It mainly affects the cochlea and the saccule (pars
inferior).
Pars superior: utricle and semicircuar canal are
less involved.
19. Temporal bone studies of otosclerosis have implicated bony narrowing of
the vestibular aqueduct and duct obstruction, explaining the co-existence
of otosclerosis and endolymphatic hydrops.
Other findings in humans with endolymphatic hydrops include:-
1. Hypoplasia of vestibular aqueduct
2. Narrowing of endolymphatic duct
3. Perisaccular fibrosis
4. Loss of epithelial integrity
5. Atrophy of the sac
6. Positive immunofluorescent staining for immunoglobulins of the sac wall
20. • Bowing of the reissner’s membrane into the scala vestibule and distention of
the saccule.
• Enlargement of endolymphatic sac occurs at the expense of perilymphatic
space.
• In the cases reviewed by Paparella; enolymphatic space budge occurred in
the region of helicotrema in half of the cases and saccule bulged against the
footplate in 60% of cases.
21. Rupture in the
membranous labyrinth
Leakage of neurotoxic
endolymph( rich in
potassium) into the
perilymph
Sustained
depolarization and
inactivation of the hair
cells and neurons of
the VIIIth nerve
Healing of membrane
rupture and return of
normal perilymph and
endolymph barrier
Return of normal ear
function
MENIÈRE ATTACKS
22. The chronic deterioration in inner ear function
presumably is the effect of repeated exposure to
the effects of the potassium.
24. • Endolymphatic hydrops is characteristic histological
sign.
• Structural defects or holes in the wall of the
membranous labyrinth that may be covered with new
membranes. Such healed discontinuities seem
consistent with a rupture theory.
• Under light microscopic: histopathological studies
fail to show loss of sensory hair cells in the inner ear
except in advanced cases in which degenerative
changes seem to occur.
• Under electron microscopic: reduction in the
number of afferent nerve endings and synapses in the
cochlea, but there are no reports regarding the
vestibular hair cells.
• Tsuji et al.:-
1. using a new optical technique; Nomarski differential
interference contrast microscopy on 20 mm celloidin
sections, studied the vestibular sensory regions in 24
Menière temporal bones.
2. Type I hair cell densities within normal limits, but a
significant loss of type II hair cells in all sensory
regions of the vestibular organ compared with age
matched controls. A significant loss of vestibular
ganglion cells was also found.
This new technique allows quantitative
measurements in vestibular otopathology and may be a
valuable tool in the future.
Light microscopy showing rupture of the
membranous labyrinth in the semicircular
canal from a patient that suffered from
Menière’s disease. The rupture is healed by
a herniating membrane
25. HENNEBERT’S SIGN
Subjective vertigo and tonic eye deviation and nystagmus observed during a
pressure-induced excursion of the footplate
Fibrous adhesions may form between the saccule and the undersurface of stapedial
footplate
False positive fistula test
Hennebert’s sign also found in congenital syphilis ( Hypermobile stapes footplate)
28. Idiopathic
Predisposing factors
Age –before 50;males>females
More in developed countries
Urban people
Emotional stress,pre menstrual period,later stage of pregnancy
High salt diet
Sodium and water retention
endocrine –hypothyroidism,hypoadrenalism,hypopituitarism,diabetes,
hyperlipoproteinemia
Increased sympathetic stimulation ischaemia of stria vascularis fluid
transudation
29. AETIOLOGICAL FACTORS
1.Genetic-
•Autosomal Dominant
•Both sporadic and familial cases are seen
•Possible locus lying between HLA-C and HLA-A loci oon short arm of chromosome 6
•HLA DRB1*1602 subtype of HLA-DR2
2. Anatomical-
•Small vestibular aqueduct
•Significant reduction of the volume of the rugose portion of the endolymphatic sac.
3. Traumatic
•Either physical or acoustic
•Trauma may produce biochemical dysfunction in the cells of the membranous
labyrinth or it may simply cause the release of debris into the endolymph which could
then obstruct the endolymphatic duct and sac.
4. Viral Infection
•Neurotropic viruses
•Herpes simplex type 1
•Enterovirus
30. 5. Allergy
• Both food and inhalant allergen were implicated
6. Autoimmunity
• Cirulating immune complexes
• Antibodies against mesenchymal elements of normal inner ear
• Antibodies against Type II collagen
7. Ischemia of the endolymphatic sac or inner ear. Such a common vascular
mechanism may link migraine and Meniere disease.
8. Psychosomatic and Personality features
• Emotion is a precipitating factor
It is possible that Meniere disease is precipitated by a variety of
events that include autoimmune, viral, traumatic, vascular/ischemic, and even
congenital anatomic and molecular variations; these may act as triggers for the later
development of symptomatic hydrops.
31. AETIOLOGICAL FACTORS IN SECONDARY
ENDOLYMPHATIC HYDROPS
Developmental insult
Abnormal metabolic and endocrine states
Syphillis
Chronic otitis media
Viral infection
Autoimmunity
Otosclerosis
Abnormal fluid balance
Leukaemia
33. More prevalent among whites
Equal gender distribution.
Peak age of onset: fourth and fifth decades, although presentation of the disease can
occur at almost any age.
Familial tendency
Right and left ear affected equal frequency
Menière’s disease is characterised by attacks of
1. Recurring attacks of vertigo (96.2%)
2. Tinnitus (91.1%)
3. Ipsilateral hearing loss (87.7%)
Attacks are often preceded by an aura that consists of a sense of
fullness in the ear, increasing tinnitus, and a decrease in hearing. However, onset may be
sudden with little or no warning. Acute attacks typically last from minutes to hours, most
commonly 2 to 3 hours. Attacks longer than a day are unusual, and if present, this should
cast doubt on the diagnosis.
34. VERTIGO
Episodic vertigo
Associated with nausea and vomiting
Begins suddenly with severe spinning sensation,
accompanied by pallor, diaphoresis, nausea,
diarrhoea and vomiting.
Severe at the beginning of the attack
Head movement exacerbates symptoms
During attack patient has a normal level of
consciousness, orientation and no focal neurological
symptoms like diplopia, dysarthria, paraesthesia, or
muscular weakness.
Nystagmus associated with vertigo may cause
visual blurring
Following its onset, the vertigo typically increases
in intensity over a period of minutes and then
usually lasts for several hours.
Silverstein and colleagues found that vertigo ceased
spontaneously in 57% of patients in 2 years and in
71% after 8.3 years.
35. Horizontal nystagmus is the cardinal finding
A typical attack has three phases, each defined by the direction of the
spontaneous nystagmus.
First phase / Irritative phase:
1. Nystagmus, usually horizontal or horizontal-torsional, beats towards the affected
ear
2. Lasts for a very short time around 20 sec.
3. Membrane rupture rising perilymphatic potassium excitatory effect in
first order vestibular neurons
Second phase /Paretic phase:
1. Nystagmus beats away from the affected ear
2. Lasts several hours, sometimes even a day or two.
3. As the concentration of potassium increased Blockage of action potentials
Third phase /Recovery phase:
1. Nystagmus again beats towards the affected side
2. Lasts about as long as the second phase.
3. Due to vestibular adaption
36. TULLIO’S PHENOMENON
• Subjective imbalance and nystagmus observed in response
to loud sound, low frequency noise exposure.
• It occurs in Superior canal dehiscence, perilymph fistula,
Menière’s syndrome,post fenestration surgery and
vestibulofibrosis
37. HEARING LOSS
Sensorineural hearing loss
Fluctuating and progressive
Unilateral
Early in the disease:
1. Low frequency fluctuating hearing loss
2. Second early pattern is low frequency hearing
loss in concert with high frequency hearing
loss (inverted ‘V’ shaped audiogram) centered
at 2kHz.
Hearing loss tends to flatten with time and
variability decreases
Profound deafness is the end point only rarely
in the progression of Menière’s disease(1%-
2% of cases)
Diplacusis (a difference in the perception of
pitch between the ears)
Recruitment
38. TINNITUS
Variable in character
First symptom of the disorder and it may begin with the first attack
Always present during spell( if patient is able to listen for it), often
present between attacks
Continuous or intermittent
Non-pulsatile
Variously described as whistling or roaring
Pitch-corresponds to region of most severe hearing loss
Severity loosely related to the severity of hearing loss
EARLY- tinnitus becomes loud when the hearing is reduced and then
becomes softer as the hearing improves
LATER- tinnitus is constant and more distracting between attacks. Hence,
tinnitus is the patient’s primary complaint in the later stages.
39. AURAL FULLNESS
Pressure sensation is limited to the ear
But some patients may consistently feel pressure elsewhere in the
head and neck with attacks
40. SOMATOPSYCHIC EFFECTS
Secondary agoraphobia produced by frightening vertigo,
particularly elderly
In the early stages of the disease, most patients are well between
attacks. As the disease progresses, patients may have persistent
hearing loss, tinnitus and postural imbalance between the attacks of
vertigo.
41. TUMARKIN’S CRISIS/ DROP ATTACKS
Sudden unexplained falls without vertigo or loss of consciousness
Occurs in 2% of cases
Patients describe a sensation of being pushed, or thrown to the ground by
some external force, or a sudden illusion of movement of the enviorment.
Acute dysfunction in the otolithic organs
Sudden changes in the output of gravity reference information from the
saccule and utricle results in inappropriate postural adjustment via the
vestibulospinal tract.
Usually occur in patients in later stages of disease.
Some patients may have only one or two of these attacks during the course
of their illness, while others have repeated attacks.
43. Symptoms arise in the reverse order
Tinnitus and hearing loss precede and worsen with the onset of
vertigo. However, when the vertiginous episode occurs, the tinnitus
and hearing loss dramatically resolve.
These patients often give a history of migraine
The temporal bone studies of one individual with such attacks noted
hydrops and membrane ruptures isolated to the basal turns of the
cochlea and the saccule
LERMOYEZ SYNDROME
44. COCHLEAR MENIÈRE’S DISEASE
Also known as Menière’s disease without vertigo, atypical Menière’s
disease, cochlear hydrops
Sudden development of unilateral hearing loss and tinnitus
Fluctuating and progressive sensorineural hearing loss
Audiometric tests are typical of Menière’s disease
80% go on to develop classical Menière’s disease
Due to obstruction to the ductus reuniens, causing hydrops confined
to cochlear duct
45. VESTIBULAR MENIÈRE’S DISEASE
Also known as Menière’s disease without deafness, vestibular
hydrops, recurrent vestibulopathy
Intermittent episodic vertigo
Auditory function normal, vestibular testing shows abnormalities
identical to Menière’s disease
20% develop auditory symptoms of classical Menière’s disease
No pathological correlation
Utriculo- endolymphatic valve is deficient
46. DELAYED ENDOLYMPHATIC HYDROPS
Recurrent attacks of rotatory vertigo in an ear that has been
previously deafened
First described by Nadol, Weiss and Parker
Causes : viral etiology, heavy noise exposure
Young age affected most commonly
49. AMERICAN ACADEMY OF OTOLARYNGOLOGY–HEAD AND
NECK SURGERY CRITERIA FOR MENIÈRE’S DISEASESE VERITY
In 1996, the Committee on Hearing and Equilibrium reaffirmed and clarified the 1985
guidelines, adding initial staging and reporting guidelines.
Vertigo
a. Any treatment should be evaluated no sooner than 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
c. Numeric value scale
0: Class A: Complete control of definitive spells
41 to 80: Class B: Limited control of definitive spells
81 to 120: Class C: Insignificant control of definitive spells
> 120: Class D
Class E: Secondary treatment initiated
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
50. Hearing
a. Hearing is measured by a four-frequency pure tone average (PTA) of 500
Hz and 1, 2, and 3 kHz
b. Pretreatment hearing level: worst hearing level during 6 months prior to
surgery
c. Posttreatment hearing level: poorest hearing level measured 18 to 24
months after institution of therapy
d. 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% speech
discrimination improvement
iii. Worse >10-dB PTA worsening or >15% speech discrimination
worsening
In 1996, the Committee on Hearing and Equilibrium reaffirmed and clarified
the guidelines, adding initial staging and reporting guidelines.
Initial Hearing Level
Four-Tone Average (dB)
Stage 1: ≤25
Stage 2: 26-40
Stage 3: 41-70
Stage 4: >70
51. Functional Level Scale
Regarding my current state of overall function, not just during attacks:
1. My dizziness has no effect on my activities at all.
2. When I am dizzy, I have to stop for a while, but it soon passes, and I can resume
my activities. I continue to work, drive, and engage in any activity I choose
without restriction. I have not changed any plans or activities to accommodate my
dizziness.
3. When I am dizzy, I have to stop what I am doing for a while, but it does pass,
and I can resume activities. I continue to work, drive, and engage in most
activities I choose, but I have had to change some plans and make some allowance
for my dizziness.
4. I am able to work, drive, travel, and take care of a family or engage in most
activities, but I must exert a great deal of effort to do so. I must constantly make
adjustments in my activities and budget my energies. I am barely making it.
5. I am unable to work, drive, or take care of a family. I am unable to do most of
the active things that I used to do. Even essential activities must be limited. I am
disabled.
6. I have been disabled for 1 year or longer and/or I receive compensation because
of my dizziness or balance problem.
53. CONDITIONS WITH VERTIGO WITHOUT
AUDITORY SYMPTOMS
1. Vestibular neuronitis:-
• Due to change in the vestibular output of one inner ear
• Severe vertigo
• Patient very ill initially
• Symptoms subside over 24-48 hours
• ENG: reduced caloric response on the affected side and a paralytic nystagmus
• Lack of auditory symptoms, aural fullness distinguish from Menière’s disease
2. Benign paroxysmal positional vertigo
• Evoked by changes of head position
• Latent period of a few seconds followed by severe vertigo lasting less than a
minute
• Dix-Hallpike testing is positive
• Rotatory nystagmus with fatiguability
54. CONDITIONS WITH AUDITORY SYMPTOMS
WITHOUT VERTIGO
1. Sudden deafness
• Hearing loss develops more quickly usually across a the frequency spectrum
• Aural fullness is absent
2. Vestibular schwannomas (acoustic neuroma)
• Progressive sensorineural hearing loss and tinnitus
• Patients complain of dysequilibrium
• Rotatory nystagmus uncommon
• Pure tone audiometry shows high frequency loss
• Absent stapedial reflex or marked stapedial reflex decay
• Diagnosis by MRI with gladolinium DTPA enhancement
55. CONDITIONS WITH COMBINATION OF AUDITORY
SYMPTOMS AND VERTIGO
1. Cogan’s syndrome
• Presence of interstitial keratitis
2. Craniovertebral junction abnormalities
• Also known as craniocervical dysplasia or basilar impression
• Vascular or neural malformations resulting in pressure over brainstem
• Symptoms resemble Menière’s disease
• Presence of other malformation or disease process such as rheumatoid arthritis
3. Vertebrobasilar insufficiency
• Transient vertigo or disequilibrium following certain head movements
• Auditory symptom unusual
• Focal neurological symptoms may occur
4. Migraine
• Most commonly in women
• Basilar migraine ma present as Menière’s disease
5. Non-specific cochleovestibulopathies
• Progressive, non fluctuating unilateral hearing loss with attacks of vertigo and/ or disequilibrium
• Abnormal ENG
56. Infective erosion of labyrinth
Recognised by otoscopic evidence of middle ear disease
58. 1. Careful detailed history
2. Full neuro-otological examination
3. General examination:-
• Normal stance and gait witout spontaneous nystagmus between attacks
4. Positional testing rarely provokes either vertigo and nystagmus
5. Otoscopy: Normal tympanic membrane
6. Tuning fork test: show sensorineural hearing loss (Rinnie- positive;
Weber- lateralised to better ear)
59. ASSESSMENT OF COCHLEAR FUNCTION
Pure Tone Audiometry
1. Sensorineural hearing loss predominantly low tone in early stages
2. Menière’s disease will most often demonstrate a flat audiogram (42%) followed
by a peaked pattern (32%), a downward sloping pattern (19%), and a rising pattern
(7%).
3. Most patients have good air and bone conduction thresholds at 2000Hz
4. Serial audiometry over time my demonstrate fluctuation in the degree of
sensorineural haring loss
5. Fluctuations are mainly seen in the frequency range of 250-1000 Hz, usually
within an average amplitude of 20-30dB.
Speech audiometry
1. Recruitment : positive
2. SISI (short increment sensitivity test): score is better than 70% in two thirds of
the patient
3. Tone decay test: no tone decay
60. Audiogram in Menière’s disease showing a 60 dB
low-frequency hearing loss with normal acoustic
reflexes (Z), indicating a fully recruiting cochlear
hearing loss.
Audiogram after four months of treatment with
a rigorous low-sodium diet shows normal pure-
tone thresholds.
61. Electrocochleography
1. It refers to the measurement of electrical events generated either within the cochlea or
by primary afferent neurons.
2. Changes characteristic of, and probably diagnostic of endolymphatic hydrops
3. Broadening of SP/AP waveform (normal-1.2 to 1.8ms, widening of greater than 2ms
is significant), due to a relative enhancement of summating potential(SP). (AP-
action potential of eight cranial nerve)
4. The summating potential (SP), as recorded by electrocochleography in Menière’s
patients, is larger and more negative.
5. This reflects the distension of the basilar membrane into the scala tympani, which
causes an increase in the normal asymmetry of basilar membrane vibration
6. Normal SP/AP fund in 20% of the cases
7. In the ear with hydrops the ratio is often as high as 30%
8. It is not a definitive test, however, because ratios are elevated in 62% of patients with
Meniere disease and in 21% of control subjects.
63. Electrocochleogram (ECOG) in Menière’s
disease. Transtympanic electrocochleogram of
a patient, showing large negative summating
potentials (SP) in response to 16 ms, 1 kHz and
2 kHz tone-bursts at 80 and 100 dB nHL. The
mean absolute negative SP levels in patients
with definite Menière’s disease, who have
subjective thresholds below 40 dB nHL, are as
follows: 2 kHz, 100 dB 46 mV; 2 kHz, 80 dB
44 mV; 1 kHz, 100 dB 44 mV. These results
show that tone burst ECOG responses are more
likely to be abnormal in patients with
Menière’s disease than are click-evoked
responses
64. DEHYDRATING AGENTS
• Dehydration of patients with Menière’s disease using parenteral glycerol produces
significant improvement of hearing thresholds,particularly in cases where hearing
loss is still fluctuant
• Glycerol is incompletely metabolized in the body when given in high doses, and acts
as an osmotic diuretic. The osmotic effect of glycerol is thought to reduce the
endolymphatic hydrops and intralabyrinthine pressure, resulting in more symmetrical
basilar membrane vibration.
• Procedure of ‘glycerol test’: P.T.A. and speech audiogram is done prior to the test.
Glycerol (1.5ml/kg) mixed with isotonic solution is given orally. Audiological tests
repeated after 2 hr.
• Test is positive if
1. Pure tone threshold improves > 12dB
2. Speech Discrimination Score increases > 12%
3. S.P./ A.P. ratio in E.Co.G decreases >15%
65. • The reported sensitivity and specificity of the test vary widely. Klockhoff
reported a 60% sensitivity in cases of known Meniere disease.
• Psychologic factors are also significant, which has led some to question
the usefulness of the test.
• Side effects include headache, nausea, and drowsiness.
66. Acetazolamide, a carbonic anhydrase inhibitor, has been used to increase the
cochlear endolymphatic hydrops, a sort of ‘reverse glycerol test’
Documentation of deterioration in pure tone thresholds and in speech
discrimation scores, as well as significant increase in the enhancement of the
negative summating potential supports diagnosis of Menière’s disease
Fewer side effects than glycerol but increase in pathological condition of the
cochlea makes it open to critisim.
67. ASSESMENT OF VESTIBUAR FUNCTION
VIDEONYSTAGMOGRAPHY
Recording of eye movements after caloric and rotational stimulation is a
commonly available and reliable method of assessing vestibular function.
Caloric test used to localize the involved ear, and a significant caloric response
reduction is found in 48% to 73.5% of patients with Menière’s disease.
Commonest pattern is canal paresis, but a directional preponderance towards the
normal ear, or a combination of reduced canal sensitivity and directional
preponderance may be found
HEAD-THRUST TESTING
Popularized by Halmagyi and Curthoys
Very sensitive test for unilateral vestibular dysfunction.
However, in Menière’s disease, the asymmetry is subtle and is only present in
29% of those who have the disease.
69. METABOLIC AND SCREENING TESTS
To rule out cause of secondary endolymphatic hydrops
1) Complete blood count (CBC)
2) Erythrocyte sedimentation rate (ESR)
3) Urea, electrolytes
4) Veneral disease research laboratory test (VDRL)
5) Treponema pallidum haemagglutination test (TPHA)
6) Random serum glucose (fasting glucose)
7) Glucose tolerance test
8) Cholesterol, triglycerides(fasting lipid profile)
9) Thyroid function tests
10) Immunoglobulin assays, autoantibody screening
70. VESTIBULAR EVOKED MYOPOTENTIALS
Vestibular-evoked myopotentials (VEMPs) are generated by playing loud clicks
in the ear, which moves the stapes footplate and stimulates the saccule.
This is the start of a disynaptic pathway that passes through the vestibular nuclei
and then to synapses that relax the sternocleidomastoid muscle.
The saccule is the second most common site affected by hydrops, which has
caused VEMPs to be investigated as a possible diagnostic tool.
Normal ear: best response is near 500 Hz.
Ears affected by Meniere disease: have elevated VEMP thresholds with flattened
tuning, and the interaural amplitude difference in the response has been
implicated as a staging tool for Meniere disease.
Most reliable finding : cervical VEMP has reduced amplitudes.
Although these tests show differences between populations, they currently have
limited diagnostic value because of the large variation in individual responses.
72. Therapy is aimed at the reduction of symptoms, and the optimal
curative treatment should stop vertigo, abolish tinnitus, and reverse
hearing loss
GENERAL MEASURES
1. Reassurance
2. Smoking cessation
3. Avoid excess water intake
4. Avoidance of alcohol,caffeine,stress
5. Mental relaxation techniques
6. Avoiding activities requiring good body balance
74. SYMPTOMATIC RELIEF DURING ACUTE
ATTACK
1. Rest and assurance with psychological support
2. Bed rest to minimise movements
3. Vestibular suppressants:
• Phenothiazines such as prochlorperazine and perphenazine,
• Antihistamines such as cinnarizine, cyclizine, dimenhydrinate,
promethazine hydrochloride,
• Benzodiazepines such as lorazepam and diazepam
75. PROPHYLAXIS BETWEEN ATTACKS
DIETARY MODIFICATION AND DIURETICS
• Salt restriction and diuresis may be the best initial therapy for Meniere disease.
• Goal: to reduce endolymph volume by fluid removal and/or reduced production.
• Despite the popularity of these treatments, neither salt restriction nor diuretic use
has had its efficacy confirmed by double-blind placebo-controlled studies.
• The best designed diuretic study to date, a cross over placebo-controlled study of
Dyazide.
• Carbonic anhydrase inhibitors, such as acetazolamide, were initially recommended
because of presence of carbonic anhydrase in the endolymph producing dark cells
and stria vascularis. However, their use has not shown to be clinically superior to
other diuretics and the immediate effect of acetazolamide is to increase hydrops
and hearing loss, caution should be taken while using this drug
Hyperosmolar dehydration
76. Vasodilators
• Betahistine, an oral preparation of histamine, has proven
effective in the treatment of Meniere disease in placebo-
controlled studies
• Other vasodilators include papaverine, isoxsupride,
nylidrin, dipyridamole, amyl nitrite, nitroglycerine,
nicotinic acid, carbon dioxide and thymoxamine.
Hearing loss is rehabilitated using hearing aid
77. LOCAL OVERPRESSURE THERAPY
A relatively recent approach to decrease hydrops
Pulsing pressure to the middle ear
The mechanism of vertigo reduction is unclear, but it may facilitate endolymph
absorption.
Since 2000, THE MENIETT DEVICE (Medtronic, Minneapolis, MN) has been approved
for use by the United States Food and Drug Administration.
Handheld air-pressure generator
Self-administeration
Therapeutic pulse pressure is delivered in complex pulses of up to 20 cm of water
delivered over 5 minutes, and the device requires a ventilation tube to be placed in the
tympanic membrane prior to starting therapy.
79. Surgical treatment in Meniere disease is reserved for 10-20% of patients who fail
conservative medical management
ENDOLYMPHATIC DECOMPRESSION
• Sacculotomy
Fick’s sacculotomy
Puncture of saccule through stapes footplate
• Cody’s tack procedure
Placing stainless steel tack on stapes footplate
Cause periodic decompression of saccule when it gets distended
• Cochleosacculotomy
Fracture dislocation of spiral lamina
Permanent fistulisation of cochlear duct
80. ENDOLYMPHATIC SAC SURGERY
Surgical decompression of the endolymph for Meniere disease
was first described by Portmann in 1926.
Variations of endolymphatic sac surgery hav
1. Simple decompression,
2. Wide decompression that includes the sigmoid sinus,
3. Cannulation of the endolymphatic duct,
4. Endolymphatic drainage to the subarachnoid space,
5. Drainage to the mastoid,
6. Removal of the extraosseus portion of the
A variety of prostheses have also been proposed, from simple
silastic sheets to tubes and one-way valves designed to allow
flow selectively in either the mastoid or subarachnoid direction.
81. Simple mastoidectomy
Identification of the middle and posterior fossa dural plates, sinodural angle,
sigmoid sinus, antrum, the horizontal semicircular canal and incus
Identification of the facial nerve leaving intact a thin bony covering from the
horizontal canal to the stylomastoid foramen. (The endolymphatic sac lies on
the dura medial to the fallopian canal and the retrofacial air cells.)
Identification of the posterior semicircular canal
Removal of the posterior fossa dural plate between the sigmoid sinus and the
posterior canal.
The endolymphatic sac is located by tracing an imaginary (Donaldson’s) line
through the horizontal semicircular canal, perpendicular to and bisecting the
posterior semicircular canal. The upper edge of the endolymphatic sac is
usually located just inferior to this line.
The precise management of the sac subsequent to its identification varies
according to which procedure is conducted.
Decompression of the sac by removal of all bone of the posterior fossa dural
plate completes the procedure.
Shunting of the sac can be performed either into the mastoid or the
subarachnoid space
82. Endolymphatic sac procedure.
A, A standard simple mastoidectomy is
performed. The middle and posterior fossa dura
plates, sinodural angle, sigmoid sinus, and
antrum are identified. The horizontal canal and
incus are then identified as well as the digastric
ridge. The facial nerve is skeletonized from the
horizontal canal to the stylomastoid foramen;
copious irrigation is used to keep the nerve cool.
Facial nerve monitoring can be beneficial. The
retrofacial cell tract is opened.
B, The posterior semicircular canal is identified
and the posterior fossa dura plate is removed
between the sigmoid sinus and the posterior
canal.
C, The upper edge of the endolymphatic sac is
identified; it generally lies at or below
Donaldson’s line (a line extended posteriorly
along the long axis of the horizontal canal that
bisects the posterior semicircular canal).
83. Paparella technique for endolymphatic mastoid shunting. A
T-shaped piece of silicone is coiled and placed into a
lateral incision in the endolymphatic sac to create a
drainage path to the mastoid cavity
84. Endolymphatic subarachanoid shunt
A. After exposing and opening the lateral
wall of the endolymphatic sac, the medial
wall of the sac is incised to open the
lateral prolongation of the basal cistern.
Dissection in the cistern is carried out
bluntly to avoid venous injury
B. A silicon(Silastic) shunt is inserted to
maintain drainage path between the
endolymphatic sac and the basal cistern.
The lateral endolymphatic sac should be
carefully closed with a fascia graft to
prevent CSF leak.
86. Retrosigmoid approach to nerve section. The cerebellum is retracted medially giving a
view of the superior and inferior vestibular nerves
A. The posterior fossa exposed and nerves are identified
B. The superior vestibular nerve is separated from the more anterior facial nerve
C. The superior vestibular nerve has been sectioned
87. Middle fossa approach to vestibular
nerve section
A, The temporal portion of the squamosa
is identified through a standard vertical
middle fossa incision. After the bone flap
is removed, the dura is elevated from
posterior to anterior, exposing the floor
of the temporal fossa.
B, Using suction irrigation and a
diamond burr, the arcuate eminence is
identified, as is the meningeal artery.
C, The facial nerve has been identified
and traced into the internal auditory
canal. The superior semicircular canal
has been “blue lined.” The internal
auditory canal has been skeletonized and
opened; the superior and inferior
vestibular nerves are then avulsed.
88. INTRATYMPANIC INJECTION/ CHEMICAL
LABYRINTHECTOMY
Commonly performed with either dexamethasone or gentamicin for control of vertigo symptoms
Gentamicin has a vestibulotoxicity that is high relative to its cochleotoxicity; thus it can be used to control
vestibular symptoms while sparing hearing.
Administered through a tympanostomy tube, or it can be directly injected through the tympanic membrane.
Peripheral vestibular deficits are evident on head-thrust testing after even a single dose of gentimicin
Single-injection regimen with additional doses only if needed to control symptoms (titration therapy).
The risk of hearing loss with gentamicin using many current protocols is similar to that found in the natural history
of Meniere
Gentamicin was found to be superior to dexamethasone for vertigo control in a randomized controlled
Intratympanic injection of dexamethasone is considered by many to be a reasonable procedure to offer when
vertigo is intractable but the patient still has some functional hearing.
The mechanism for steroid effect on vertigo symptoms is not currently clear, although some evidence suggests that
Meniere disease has an autoimmune component that steroids may address.
Dexamethasone injections may need to be repeated every 3 months to maintain freedom from vertigo symptoms,
although the optimal dosing frequency is variable and unknown. Concentrations used have varied from 2 to 24
mg/mL, but 10 mg/ mL is typical.
89. LABYRINTHECTOMY
The most destructive procedure for treatment of Meniere disease is
labyrinthectomy
Uniform destruction of hearing and vestibular function.
Ideal candidates have no functional hearing and have failed more
conservative treatments, such as gentamicin injection.
Despite this morbidity, the procedure has a higher rate of vertigo
control than vestibular neurectomy and has been reported to improve
quality of life in 98% of patients.
approaches
1. Transmastoid exposure
2. Transcanal approach.
91. OUTCOMES AND COMPLICATIONS
The natural history of Menière's disease is variable.
Attacks may occur days, months or even years apart, with little or no
warning.
Some patients have a single bout of attacks lasting only a few months
and never develop any permanent loss of auditory or vestibular function.
Others have a relentlessly progressive course and continue to have
vertigo attacks, along with continual tinnitus and no useful hearing in
one ear.
Others still are fortunate and have no further vertigo attacks and little
tinnitus – so called burnt-out Menière's disease.
In most patients, only one ear is affected in the early stages of Menière's
disease. Unfortunately, the second ear eventually becomes involved in
about half of the patients with this condition
