menieres disease

1. Menie`re’s disease
Dr. Krupal A. Pardhi
PG Resident II Year
Dept. of E.N.T. & H.N.S
SKNMC & GH,Pune

2. Introduction
• It’s an idiopathic endolymphatic hydrops & a
disorder of the inner ear.
• It was first described by Prosper Meniere in
1861
• DEFINITION- Menie`re’s disease is a disorder
characterized by spontaneous attacks of
vertigo, with associated fluctuating
sensorineural hearing loss, tinnitus, and aural
fullness.

3. Etiology
• Exact cause of the disease is not yet known.
• Viral: The causative roles of viruses (HSV,
cytomegalovirus or VZV) remain uncertain.
• „Hereditary: Familial occurrence in 10–20% cases.
There is an autosomal dominant mode of
inheritance.
• Autoimmune: Certain genetically acquired major
histocompatibility complexes specifically human
leukocyte antigens (HLA) B8/DR3 and Cw7 have
been associated with Meniere’s disease.

4. Etiology & pathogenesis
• overproduction or malabsorption of endolymph,
result in endolymphatic hypertension.
• Endolymphatic hypertension,in turn, results in
gross enlargement of the membranous labyrinth
– so-called endolymphatic hydrops.
• Defective absorption by endolymphatic sac-
-Poor vascularity of sac
-Less absorptive tubular epithelium
- increased perisaccular fibrosis

5. Overaccumulation of endolymph
at the expense of perilymphatic
space results in the distortion of
membranous labyrinth.
Alterations in the size of
endolymphatic duct and sac
along with reductions in tubular
specializations of the lining of
these structures have been
observed.

7. • Delayed endolymphatic hydrops can occure after
many years in
- syphilis
- mumps,
- Cogan's syndrome,
- trauma and even
- chronic suppurative otitis media,
• This produce the clinical picture of Meniere's disease -
so-called Meniere's syndrome.
• Sometimes the attacks of vertigo can occuer in the
other, previously normal, ear this is known as
contralateral delayed endolymphatic hydrops,

8. CLINICAL MANIFESTATIONS
A typical attack has three phases, each defined by
the direction of the spontaneous nystagmus.
• 1.Irritative phase- the nystagmus, usually
horizontal or horizontal-torsional,beats towards
the affected ear; this phase usually lasts less than
an hour.
• 2.Paretic phase- the nystagmus beats away from
the affected ear; this phase lasts several hours,
sometimes even a day or two.
• 3.Recovery phase-the nystagmus again beats
towards the affected side; this phase lasts about
as long as the second phase.

9. The SNHL in Meniere’s disease typically is-
• fluctuating and progressive.
• It often occurs with the sensation of fullness or pressure in the
ear.
• A pattern of low-frequency fluctuating loss and a coincident
nonchanging, highfrequency loss is described,
• a “peaked” or “tent-like” tracing on the audiogram is obtained.
• This peak classically occurs at 2 kHz.
• Over time, the hearing loss normalizes across frequencies and
becomes less variable.
• In only 1% to 2% the hearing loss progress to profound
deafness.
• Additional features include Diplacusis (43.6%) , a difference in
the perception of pitch between the ears recruitment (56%).

10. Tinnitus
• tends to be nonpulsatile
• 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.

11. Audiogram in Meniere's disease.
This patient, a 14-year-old female, began to have attacks of acute
spontaneous vertigo with right-sided aural fullness and then tinnitus. (a)
Audiogram on presentation shows a 60 dB low-frequency
Hearing loss with normal acoustic reflexes (Z), indicating a fully
recruiting cochlear hearing loss. (b) Audiogram after four months of treatment
with a rigorous low-sodium diet shows normal pure-tone thresholds.

12. • In the early stages of the disease, most patients
are well between attacks.
• As the disease progresses, persistent hearing loss,
tinnitus and postural imbalance persist.
• Some patients, particularly those in the later
stages of the disease, develop drop attacks
(Tumarkin or otolithic crises),
• recurrent vestibulopathy and atypical Meniere’s
disease describe the condition on persons with
less than the classic triad of hearing loss, vertigo,
and sensation of aural fullness or tinnitus.

13. Tumarkin or otolithic crises
• Are thought to occur as a result of
acute otolithic dysfunction.
• The patient simply drops to the ground
without warning and can sustain a
fracture or other serious injury.
• The patient may describe a sensation
of being pushed to the ground by some
external force during these attacks.
• There is no associated vertigo or loss of
consciousness.
• Some patients may have only one or
two of these attacks during the course
of their illness, while others have
repeated attacks.

14. Lermoyez syndrome
• Described an unusual clinical presentation in
which tinnitus and hearing loss precede the
onset of vertigo.
• When the vertiginous episode occurs, the
tinnitus and hearing loss dramatically resolve.
• The temporal bone studies in a patient with
such attacks noted hydrops and membrane
ruptures isolated to the basal turns of the
cochlea and the saccule.

15. AAO-HNS Criteria for Meniere’s Disease Diagnosis
Major Symptoms
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
• No neurologic symptoms with vertigo
Deafness
• Hearing deficits fluctuate
• Sensorineural hearing loss
• Hearing loss progressive, usually unilateral
Tinnitus
• Variable, often low-pitched and louder during attacks
• Usually unilateral
• Subjective

16. Diagnosis
Possible Meniere’s disease
- Episodic vertigo without hearing loss or
- Sensorineural hearing loss, fluctuating or fixed, with
dysequilibrium, but without definite episodes
- Other causes excluded
Probable Meniere’s disease
- One definitive episode of vertigo
- Hearing loss documented by audiogram at least once
- Tinnitus or sense of aural fullness in the presumed
affected ear
- Other causes excluded

17. • Definite Meniere’s disease
-Two or more definitive spontaneous episodes of vertigo
lasting at least 20minutes
- Audiometrically documented hearing loss on at least one
occasion
- Tinnitus or sense of aural fullness in the presumed affected
ear
- Other causes excluded
• Certain Meniere’s disease
- Definite Meniere’s disease, plus histopathologic
confirmation

18. • Glycerol Dehydration test: Ingestion of glycerol
1.5 g/kg mixed with equal volume of water is
followed by serial audiograms over 3 hours.
- Positive test: 25 dB shift at three consecutive
frequencies, or 16% improvement in speech
discrimination.
• Glycerol testing is not commonly used today
because of headaches as a frequent side
effect.

19. Investigations
• The caloric test
The caloric test often can localize the involved
ear.
• it reveals a canal paresis on the affected side
(most common) but sometimes there is
directional preponderance to healthy side or a
combination of both.

20. Investigations
Electrocochleography
• The summating potential (SP) is larger and more negative due
to the distension of basilar membrane into scala tympani.
• Action potential (AP) of CN VIII: Reduction in amplitude.
• The most commonly used value is the ratio of amplitudes of
the SP and the eighth cranial nerve action potential (AP), the
SP/AP ratio : Increases.
• It is most sensitive and specific for Meniere's disease when
tone-burst and click stimuli are used
• Giving patients 4 g of oral sodium chloride for three days prior
to the electrocochleogram may increase the sensitivity of the
test.

22. Treatment
• Therapy of Meniere’s disease is aimed at the
reduction of its associated symptoms.
• Currently, almost all proven therapies are
directed at relieving vertigo, which usually is
the most distressing symptom.

23. Acute Attack
• Reassurance and bed rest
• Vestibular sedatives: They are given parenteral if vomiting
precludes oral use. They take care of vertigo and anxiety and
include prochlorperazine (Stemetil), promethazine theoclate
(Avomine), dimenhydrinate (Dramamine), and diazepam. Atropine
can also be very effective.
• Vasodilators:
– Inhalation of carbogen (5% CO2 with 95% O2) causes cerebral
vasodilation and improves labyrinthine circulation.
– Histamine diphosphate 2.75 mg in 500 ml glucose slow IV drip
helps in managing acute episodes but is contraindicated in
asthmatics and should not be given empty stomach. The adverse
side effects include tachycardia, disturbed cardiac rhythm,
hypotension, hyperthermia and bronchospasm.

24. Chronic phase
Dietary Modification and Diuretics
• Salt restriction and diuresis may be the best initial therapy
for Meniere’s disease.
• Carbonic anhydrase inhibitors such as acetazolamide were
recommended on the basis of the localization of carbonic
anhydrase to the dark cells and the stria vascularis.
• However, these agents have not proved to be clinically more
effective than other diuretics.
• Despite the lack of hard evidence of their efficacy, a low-salt
diet and diuresis constitute an appropriate and effective
treatment for Meniere’s disease with a low risk of side effects
• Frustenberg Regimen -
1. Low salt diet
2. Diuretics + Pot. chlor
3. High protein

25. Vasodilators
• Betahistine is an oral preparation of histamine.
• It has proved to be effective in treatment of
Meniere’s disease in a placebo-controlled study.
Symptomatic Treatment
• Antivertiginous medications, antiemetics,
sedatives, antidepressants, and psychiatric
treatment have been reported to be beneficial in
reducing the severity of the vertigo and vegetative
symptoms and in improving tolerance of
Meniere’s symptoms.

26. Local Overpressure Therapy
• A relatively recent approach to decrease hydrops by
pulsing pressure in the middle ear.
• overpressure in the middle ear was reported to
decrease Meniere’s symptoms during acute vertigo
attacks.
• Since 2000, the Meniett device has been approved
for use by the U.S. Food and Drug Administration
(FDA).
• The pressure is delivered in complex pulses of up to
20 cm of water, over a 5 minute period.
• 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
• Long-term treatment with the Meniett device has
shown results similar to the natural course of
Meniere’s disease.
• simple placement of a ventilation tube with no
additional therapy also has been reported to control
vertigo symptoms

27. Intratympanic Injection
• The term chemical labyrinthectomy often is applied to
intratympanic gentamicin treatment,
• Gentamicin has a vestibulotoxicity that is high relative to its
cochleotoxicity;
• The gentamicin can be administered through either a
tympanostomy tube or directly injected through the tympanic
membrane.
• The risk of hearing loss with gentamicin using many current
protocols is similar to that with the natural history of
Meniere’s disease.
• Intratympanic injection of dexamethasone is a reasonable
procedure to offer when vertigo is intractable
• Dexamethasone injections may need to be repeated every 3
months to maintain the patient free of vertigo symptoms.

28. Surgical treatments
Endolymphatic sac surgery
• may result in a reduction in the frequency, duration and intensity of
vertigo attacks.
• it is not always effective in stopping the vertigo attacks
• no benefits for the auditory symptoms.
Selective vestibular neurectomy
• effective in stopping vertigo attacks in most patients
• Vestibular nerve section by Middle Fossa Approach or
Retrolabyrinthine Approach
• hearing loss and facial paralysis is potential complications.
surgical labyrinthectomy
• can stop the vertigo attacks at the expense of losing any
• remaining hearing on that side.

29. • Sacculotomy
-puncture of the stapes footplate with a sharp
needle to rupture the underlying saccule.

30. • Endolymphatic sac surgery
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.
• 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

31. 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

32. Endolymphatic subarachnoid
shunt. A, After opening the lateral
wall of the endolymphatic sac, an
incision is made into the medial
wall and the posterior fossa to
open into the lateral prolongation
of the basal cistern.

33. B, A silicone (Silastic) shunt tube
is inserted to maintain a
drainage route between the
endolymphatic sac and the basal
cistern. A muscle plug is usually
sutured over the lateral aspect
of the endolymphatic sac to
facilitate closure and prevent
cerebrospinal fluid leak.

34. Paparella’s technique of endolymphatic mastoid shunting. A “T”-shaped piece of
silicone (Silastic) is inserted into the open endolymphatic sac to form a permanent
fistula between the lumen of the sac and the mastoid cavity.

35. Surgical labyrinthectomy
• Transcanal Labyrinthectomy
• Transmastoid Labyrinthectomy

36. Schuknecht’s transtympanic labyrinthotomy for
Meniere’s disease.

37. Transmastoid labyrinthectomy.
A, A postauricular incision is
made.
B, A complete mastoidectomy is
performed. The fossa incudis,
digastric ridge, and vertical
portion of the facial nerve are
identified. The facial recess is
opened.
C, The incudostapedial joint is
disarticulated and the incus
removed. The semicircular canals
are then opened, beginning with
the horizontal canal and
continuing to the posterior and
finally the superior canals.

38. A, The ampullae and neuroepithelium of the three semicircular canals
are exposed, as are the saccule and utricle.
B, The neuroepithelium is removed under direct visualization.

39. Thank you