Menieres Disease

Chunfu Dai
Otolaryngology Department
Fudan University
 Prevalence
Incidence is 4/100000 in Japan.
15/100000 in US, 46/100000 in
Sweden, 100/100000 in UK.
The large differences are due to
geographic, genetic, ethnic or
environmental factors, or different
diagnostic criteria.
 Background
In 1861,
Meniere described a syndrome
In 1938,
Hallpike and Yamakawa reported the
finding of endolymphatic hydrops in
temporal bone from patients with MD.
 Background
In 1972,
AAOO report also defined two sub-
varieties: cochlear and vestibular MD
In 1985,
AAO-HNS updated the term MD should be
restrictive and include only those cases
with complement of classic symptoms and
findings of the disease presumed to result
from idiopathic endolymphatic hydrops
 Pathogenesis
Radial circulation
Longitudinal flow
 Pathology
Endolymphatic hydrops
The hydrops principally involves the cochlear
duct and saccule but were not obvious in the
utricle and ampullae of semicirclar canals.
The walls of the membranous labyrinth may
show areas of thinning, outpouching and
ruptures
Decrease in the amount of loose connective
tissue around the endolymphatic sac,
hypoplasia of vestibular aqueduct, and
decreased vascularization of the sac and
perisaccular fibrosis were also observe.
Pathology
Vestibular fibrosis
Proliferation of fibrous tissue
within the vestibule is often
observed which sometimes
results in the formation of band
of fibrous tissue between the
under-surface of the footplate
and the utriclar macula
This phenomenon may account
for a positive Henneberts sign,
which observed in about 30% of
ears with MD.
Pathology
Hair cell loss
In most of cases with MD, light Microscope has
failed to demonstrate loss of HC in cochlear and
vestibular organ.
In advanced disease, loss of cochlear HC, atrophy
of support cells, distortion and atrophy of tectorial
membrane and atrophy of cristae were identified.
EM could identified fusion of cilia, disruption of
cuticular bodies and basal ward displacement of
some OHC with loss of contact with the cuticular
plate.
Pathology
Neuronal cell
SGN and Scarpas ganglion
neuron are normal in most
patients
10% of temporal bones showed
an isolated loss of cochlear
neurons in the apex. Loss of
nerve fibers in the osseous
spiral lamina.
EM identified striking reduction
in the number of afferent nerve
endings and afferent synapes
at the base of both IHC and
OHC.
Recurring episode of Vertigo
Whirling vertigo is usually sudden,
Companied with nausea and
vomiting
Persist for several hours, attacks last
longer than 24 h make the diagnosis
of MD doubtful
 Fluctuating Hearing loss
Early fluctuation of low
frequencey hearing loss
and later involvement of
the high frequencies.
An eventual decline over 5-
10 years and subsequent
stabilization with a flat
moderate to severe hearing
loss
speech discrimination
falling to approximately
50%. Making the use of
hearing aid difficult.
There is also diplacusis and
loudness recruitment
 Hearing loss
Hearing loss may be due to the
hydrops in membrane of cochlea,
which lead to restrict movement
of basal membrane.
Neural degeneration of
unmylinated afferent processes at
the EM may explain the clinical
finding of progressive loss of
speech discrimination despite the
presence of intact HC and SGN.
Biochemical and mechanical
theories

Proposed by Lawrence, McCabe and

Schuknecht that a sudden rupture of the
thin distented membranous labyrinth
results in flooding of the perilymphatic
space with potassium-rich neurotoxic
endolymph, causing paralysis of
sensorineuroal structure which in turn
results in a sudden attack of vertigo and
hearing loss.
Aural pressure and fullness
One possibility is that
this might be mediated
by sensory nerves
supplying the middle
ear aspect of the round
and oval windows.
Another possibility is
sensory innervation of
dura mater surrounding
the endolymphatic sac.
 Natural history of MD
Spontaneous remission of vertigo has
been reported in up to 71% of cases
with 8 years.
Spontaneous remission makes assessment
of efficacy of therapy quite difficult.
Up to 50% of patients will eventually
develop symptoms from the opposite
ear. 50% of patients occurred within a
period of 2 year
 Natural history of MD
Lermoyezs syndrome: is characterized by
hearing improvement that accompanies an
acute attack of vertigo. The hearing remains
good for a period of days or weeks only to
gradually diminish before onset of the next
attack
Drop-attacks associated with MD (Tumarkin):
in addition to the typical symptoms, patients
experience abrupt and brief attacks such that
they find themselves thrown to the ground
with no warning and with little or no vertigo
after the initial fall
 Delayed endolymphatic
hydrops
Patients with pre-existent profound
unilateral deafness of long duration.
Symptoms are relieved by
labyrinthectomy in the deaf ear.
The original cause of deafness may
have led to the hydrops.
 Glycerol test
Hearing improvement is considered
suggestive of MD after oral glycerol
administration. Only 60% of MD was positive.
However, negative test result did not exclude
a diagnosis, thereby, implying that the test
was specific but not sensitive.
In hydropic GP and rabbit ears, CAP
thresholds show further worse rather than an
improvement after administration of glycerol.
 Diagnosis
The diagnosis is made on the basis of
history.
Physical examination is usually normal
Many tests are interesting for research
purpose but no test proves or disproves the
diagnosis.
Vestibular, audiogram and ENG are

performed to confirm the presence of

function of the other ear.
 Differentiate diagnosis
Benign paroxysmal positional vertigo
Vestibular neuritis
Vestibular drug toxicity
Labyrinthitis
Hunt syndrome
Cogan syndrome
Delayed endolymphatic hydrops
Perilymph fistula
 Benign proxysmal position
vertigo
Inappropriate excitation of the PSC
Latency (1-4s), transient (<1minute)
with position change
Decreasing with repetitive testing
Mixed vertigo torsional nystagmus
Spontaneous resolution with 1 year
 Benign proxysmal position
vertigo
Causes
Head trauma
Prolonged bedrest
Viral or ischemic labyrinthitis
Older people
Mechanism
dislodged otoconia trapped in PSC from
the utricle.
 Benign proxysmal position
vertigo
Interventions
Spontaneously resolve
Canalith repositioning
Ampullary nerve section
Block posterior semicircular canal
(preventing movement of endolymph in
the canal)
Perilymph fistula
 Treatment
Recent treatments aim at control of
vertigo.
Medicine
Surgery intervention
No treatment has definitively proven to
alter the natural course of disease in
Fluctuative, progressive hearing loss
Tinnitus
Aural fullness
 Treatment
Salt restriction
Sedative
The key is to use sedative briefly
Prolonged use of sedative impair
compensation, prolongs symptoms and
produces a suboptimal result.
Diuretic
 Treatment
Short course of steroid is reasonable
2 tabs qid x7d
2 tabs tidx2d
2 tabs bidx2d
1 tab bidx2d
1 tab dailyx2d
Intratympanic gentamicin injectin
Medical therapy fails
Intratympanic injection is recommended
Less than 5% patients progress to the stage
where destructive treatment is indicated.
 Surgical interventions
Endolymphatic sac
shunt
Vestibular
neurectomy
Labyrinthectomy
 Evaluation of ESS
Portmann first proposed such
procedures 11 years prior to the
description of endolymphatic hydrops.
Definition of clinically significant
hearing change (1995 AAO-HNS
committee)
a change of at least 10 dB PTA
Word recognition score of at least 15%
 Evaluation of ESS
Arguments on hearing outcome (without
control study)
Hearing outcome after ESS is no different than
hearing after vestibular nerve section, or
attributable to natural history alone.
Some author indicated postoperative hearing
stabilizes or improves in 55% to 85% of patients
after ESS follow up greater than 2 years.
Tinnitus relief after ESS was reported in 21-75%
of patients.
 Evaluation of ESS
Thomsens study indicated that
subjective hearing loss to be slightly significantly better
in the ESS group compared with the placebo group.
Objective test showed no significant difference
difference between the two groups.
Above mentioned data reexamed by Welling
supported
ESS is effective in management of MD and refutes the
placebo effect
The actively shunted group have significant
improvement in vertigo, tinnitus, and combined overall
score when compared with the placebo group.
 Evaluation of ESS
Animal and human studies showed the
shunts and similar foreign materials
become encapsulated by fibrous tissue
proliferation which makes it doubtful that
will remain patent.
The pathway by which endolymph from
the cochea might reach the sac is blocked
at multiple location due to collapse of the
endolymphatic lumen.
 Evaluation of ESS
Despite the obvious conflicting
beliefs concerning the benefit of
endolymphatic sac surgery in MD.
Most otologist routinely perform the
procedure.
A success rate approaching 80%, low
complications.
 Otology group in Nashville
Long-term efficacy of ESS
Do not offer the patients reasonable expectation
for long-term, complete control of vertigo (7%).
The theoretic benefit of sac surgery in regard to
the control of tinnitus, fullness, and either
stabilization or improvement in hearing were not
realized.
As a result, ESS is no longer performed.
Suboccipital vestibular nerve section is
primarily recommendation.
 Evaluation of VNS
Surgical approaches include the middle fossa,
retrolabyrinthine and retrosigmoid
/suboccipital routes.
VNS is the gold standard for control of vertigo
spell.
Highly successful (90%) in eliminating vertigo
in patients with MD
No effect on hearing or other MD symptoms
Complications: facial palsy, infection,
deafness.
 Evaluation of VNS
Postoperative caloric test showed a
lower percentage of patients actally
achieve total ablation of vestibular
response.
This is due to the difficult in severing
all vestibular nerve fiber.
The use of intraoperative evoked
vestibular potential may overcome this
problem.
House institution
experiences
ESS is the first line of surgical treatment
for MD unresponsive to medical
treatment ( diuretic and vasodilator
therapy)
In case with disable vertigo unresponsive
to medical treatment or failing ESS, VNS
is recommended and often combine with
primary or revision ESS
House institution
experiences
Concurrent ESS and VNS does not
improvement hearing or tinnitus
outcome over vestibular nerve
section alone
Karolinska hospital policy
Patients with MD still having serviceable
hearing were primarily offered ELS
In total loss of cochlear function or persisting
symptoms after a previous ESS, intratympanic
gentamicin injection was chosen.
In patients with normal cochear function and
non Menieriform peripheral vestibular
dysfunction, vestibular neurectomy was
recommended.
 Karolinska hospital policy
As compared with destructive procedures,
such as labyrinthectomy and neurectomy
that result in severe vertigo
postoperatively.
Intratympanic gentamicin injection seldom
force the patient to require bed rest and
physical inactivity, due to slowly declining
vestibular impairment, patients only feel
slightly unsteadness.
 Labyrinthectomy
Transcanal labyrinthectomy
Semicircular canals remain intact.
Gentamicin us usually placed in the ear as well
Transmastoid labytinthectomy
Drilling the semicircular canals
Opening the vestibule and destroying the
saccule and utricle.
Gentamicin us usually placed in the ear as well