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