Menieres disease

1. MENIERE’S DISEASE
Dr Mohammed Nishad n

2. INTRODUCTION
• Meniere’s disease is an idiopathic inner ear disorder ch/b recurrent
spontaneous vertigo accompanied by fluctuating or progressive SNHL,
tinnitus , and aural fullness in the affected ear .
• Endolymphatic hydrops
• vertigo episodes are the most incapacitating
• It is a common cause of the syndrome of spontaneous vertigo.
• Despite this well-known symptom complex, it remains a controversial and
often difficult condition to diagnose and treat.

3. INCIDENCE
• Wide variation exists from 17/100000 in the Japanese population to
513/100000 in the southern Finland.
• M:F = no differance
• Most common age at presentation is 2 nd – 6th decade.
• only 1-7 % of MD – In paediatric population .
• Majority - sporadic
• Familial occurrence 5 – 15%.
• In familial cases– Higher prevalance of autoimmune ds ,longer spells
of vertigo, migraine is found to be more strongly associated , B/L MD
more common

4. GENES--MD
AQP2(Aquaporin water channel genes.
KCNE1&KCNE3(Potassium channel genes.
ADD1 (Na –K Pump activity regulator.
 COCH( cochlin protein production) - progressive non syndromic
deafness and b/l vestibular areflexia
 HCFC1 (Host cell factor C1)
 HSPA1A (Single nucleotide polymorphism of HSP 70 gene)
PTPN22 (Lympoid protein phosphatase)
 IL1

5. ANATOMY OF ENDOLYMPHATIC SYSTEM
• Inner ear consists of 2 parts – bony labyrinth & membranous
labyrinth.
• Membranous labyrinth is filled with a clear fluid called endolymph
while space between membranous & bony labyrinths is filled with
perilymph.
• Membranous labyrinth consists of the cochlear duct, the utricle &
saccule, the 3 semi-circular ducts and the endolymphatic duct & sac.

6. • Cochlear duct is subdivided by two
longitudinally running membranes that
separate three chambers, the scala tympani,
scala media and scala vestibuli.
• The scala media is triangular in section, the
other boundaries represented by Reissner's
membrane, which runs obliquely with respect
to the basilar membrane from a ridge of
tissue, the spiral limbus near the modiolus, to
the lateral wall that runs along the inside of
the bony wall.
• The organ of Corti runs in a spiral along the
floor of the scala media, situated on its lower
boundary, an acellular layer called the basilar
membrane.

7. • Reissner’s membrane consists of 2 layers of cells separated by a basement
membrane.
• The layer facing into the scala tympani is
mesothelial cell layer and other layer facing
scala media is the endothelial layer
• Cells within each layer are joined by tight
junctions which act as an impermeable
barrier to ions and small molecules.
• Stria vascularis is present on the lateral wall and consists of 3 layers of cells
composed of marginal cells, intermediate cells and basal cells. These cells
have a variety of ion pumps, enzymes and transport proteins associated
with homeostatic mechanisms for maintaining ionic composition of
cochlear fluids.

8. • Fluid in scala media, endolymph contains high potassium and low sodium
at an unusually high positive electrical potential (+80mV) called the
endolymphatic potential (EP). This contrasts with the scala tympani and
scala vestibuli, both of which are filled with perilymph that has high sodium
and 0mV EP.
• At the apex of cochlea, the 2 outer chambers are joined by an aperture
called helicotrema.
• Maintenance of EP is crucial to hearing.
• As with both the stria vascularis and reissner’s membrane, the cells of the
organ of corti facing scala media are joined by tight junctions. Thus the
whole of the scala media is electrically and chemically isolated from other
scalae, the only communication being through ion channels in the sensory
cells of organ of corti.
• EP is involved in driving currents through transduction channels that are
fundamental to hair-cell function.

9. INNER EAR FLUIDS & THEIR CIRCULATION
Perilymph :
• resembles extracellular fluid
• Rich in Na ions
• Fills space between bony & membranous labyrinth
• Communicates with CSF through aqueduct of cochlea which opens
into scala tympani near the round window
• Formation – 1)filtrate of blood serum formed by capillaries of spiral
ligament 2)direct continuation of CSF & reaches the labyrinth via
aqueduct of cochlea

10. Endolymph :
• Fills entire membranous labyrinth
• Resembles ICF, rich in K ions
• Secreted by secretory cells of stria vascularis of cochlea & by dark
cells.
• Longitudinal flow – cochlea > saccule, utricle > E.duct > E.sac
• Radial flow – secreted by stria vascularis & also gets absorbed by it

11. Composition of inner ear fluids

12. UTRICLE
• lies in the posterior part of bony vestibule
• Receives 5 openings from 3 semi-circular ducts
• Connected to saccule through utriculo-saccular duct
• Sensory epithelium of it is called macula that is concerned with linear
acceleration and deceleration

13. SACCULE
• Lies in the bony vestibule, anterior to utricle & opposite the stapes
footplate
• Its sensory epithelium is called macula
• Exact function of it is not known , probably responds to linear
acceleration & deceleration
• In Meniere’s, distended saccule lies against footplate & can be
surgically decompressed by perforating the footplate

14. SEMICIRCULAR DUCTS
• 3 in number
• Open in the utricle
• Ampullated end of each duct contains a thickened ridge of
neuroepithelium called crista ampullaris

15. ENDOLYMPHATIC DUCT & SAC
• E. duct is formed by union of 2 ducts – one each from saccule &
utricle.
• It passes through the vestibular aqueduct.
• Its terminal part is dilated to form endolymphatic sac, which lies
between the 2 layers of dura on the posterior surface of the petrous
bone.
• E. sac is surgically important. It is exposed for drainage or shunt
operation in Meniere’s disease.

16. BLOOD SUPPLY
• Labyrinthine artery (br of AICA)

17. PATHOGENESIS
• Main pathology is system due to
increased volume
distension of endolymphatic
of endolymph causing endolymphatic
hypertension.
to either increased production or decreased• This may be due
absorption or both.

18. • Defective absorption by endolymphatic sac – causing raised pressure
in the cochlear duct -> Distension of it -> rupture of Reissner’s
membrane -> mixing of endolymph & perilymph -> attack of vertigo.
• Ischaemia of sac is seen in some cases indicating poor vascularity and
thus poor absorption by the sac.
• Vasomotor disturbance – sympathetic overactivity -> spasm of
internal auditory artery -> impaired function of cochlear or vestibular
sensory neuroepithelium -> deafness & vertigo.

19. • Anoxia of stria vascularis capillaries > increased permeability >
transudation of fluid > increased production of endolymph.
• Allergy – inner ear acts as a shock organ due to allergen causing increased
production of endolymph.
• Na & water retention causes excessive fluid to get retained leading to
hydrops
• Hypothyroidism – 3%
• Autoimmune – approx 1 in 3 pts with MD (cross reaction between similar
antigens,acquired intolerance to inner ear antigens,genetic factors etc)
• Bacterial infections like syphilis
• viral –mumps,HSV

22. • Meniere’s syndrome – Many different inner ear or temporal bone
diseases, such as syphilis, mumps, Cogan's syndrome, trauma and
even chronic suppurative otitis media, can, after many years, produce
the clinical picture of Meniere's disease so-called Meniere's
syndrome.
I/L delayed endolymphatic hydrops –
• Patient describes attacks of spontaneous vertigo beginning many
years after the onset of unilateral hearing loss due to delayed
endolymphatic hydrops on the abnormal side.

23. C/L delayed endolymphatic hydrops
• Sometimes attacks of vertigo coincide with onset of fluctuating
hearing loss in the other, previously normal ear which is thought to be
due to development of autoimmunity to inner ear antigens.
• Histopathology reveals pathological changes in deaf ears that are
similar to those known to occur in mumps ,measles,HSV labyrinthitis.
These observations support the proposition that Meniere's disease
may occur as a delayed sequela of inner ear damage sustained during
an attack of subclinical viral labyrinthitis occurring in childhood.

24. CLINICAL FEATURES
• Recurrent attacks of spontaneous vertigo /Low to medium frequency
fluctuating hearing loss, tinnitus and aural fullness. An attack typically
increases in intensity over a period of minutes and then usually lasts
for several hours.
• Hearing loss and tinnitus may persist for days.
• Associated nausea and vomiting.

25. An attack of vertigo
• A typical vertigo attack has 3 phases.
• initial irritative phase due to increased potassium concentration –
nystagmus usually horizontal or horizontal-torsional & beats towards the
affected ear; usually lasts less than an hour.
• second paretic phase nystagmus beats away from affected ear because of
peripheral hypofunction and reduced spontaneous neural activity (due to
blockade of neurotransmitter release) & lasts usually several hours or
sometimes 1 or 2 days.
• Third recovery phase in which nystagmus again beats towards affected site;
brainstem compensation lasts for several hours or sometimes 1 or 2 days.
• Healing of rupture then allows restitution of normal chemical composition
of both fluids terminating an attack and improvement in symptoms.

27. • Diplacusis or double hearing is a phenomenon of altered sound
perception, which differs from that of a presented sound.
• Diplacusis could be manifested as binaural or monaural. In binaural
diplacusis, the same tone is perceived differently in each ear. It is most
frequently reported in Meniere's disease.
• Evaluation –
• Patients typically complain of 'distorted' sounds. Diplacusis can be easily
identified using the tuning fork test, when the patient is able to report
whether the tone presented to each ear sounds different.

28. • Hyperacusis is defined as a reduced tolerance to noise, or an increased
sensitivity to sounds in levels that would not cause discomfort in a normal
individual.
• The essence of hyperacusis is the lack or insufficiency of adaptive
(ihibitory) mechanisms, which normally limit the sound input and prevent
overstimulation of the auditory system.
Recruitment---- Abnormal growth of loudness
Ear which does not hear low intensity sound begin to hear greater intensity
sound as loud
Seen in cochlear lesions

29. CLASSIFICATION
Guidelines were given by AAOHNS.
Definite Meniere’s
• > 2 spontaneous attacks of vertigo each lasting > 20 min
• Hearing loss documented by PTA on atleast 1 occasion
• Fluctuating Tinnitus or aural fullness on affected side
Probable Meniere’s
• 1 Definitive spontaneous attacks of vertigo lasting > 20 min + Hearing loss
documented by PTA + tinnitus + aural fullness
Possible Meniere’s
Episodic spontaneous attacks of vertigo without any documented hearing
loss (vestibular variant)
SNHL , Fluctuating or fixed ,with dysequilibrium but without definitive
episodes ( cochlear variant)
 Certain --Definite MD confirmed by histopathology

30. D/D
 TIA
Vestibular migraine
Vestibular paroxismia
R/c u/l vestibulopathy
 Vestibular schwannoma
 Endolymphatic sac tumour
 Chronic subjective dizziness

31. EXAMINATION
• Otoscopy – no abnormality in TM
• Nystagmus – seen only during acute attack, quick component is
towards unaffected ear
• Tuning fork tests – indicate SNHL. Rinne +, ABC reduced, weber
lateralised to better ear.

32. INVESTIGATIONS
• PURE TONE AUDIOMETRY – SNHL
• Early stages – lower frequencies affected & curve is rising type
• When higher frequencies involved, curve becomes a flat or a falling type.
• SPEECH AUDIOMETRY – Discrimination score is usually 55-85%
between attacks but impaired during & immediately following an
attack.
• Todifferentiate from retrocochlear
• Recruitment positive
• SISI >70%
• Tone decay test – decay < 20dB

33. Audiogram

35. Electrocochleography
• Most sensitive & specific for
Meniere’s when tone-burst and click
stimuli are used, and when the
responses are recorded
transtympanically at the promontory.
• Giving 4g oral NaCl for 3 days prior to
ECoG may increase the sensitivity of
the test.

36. Electrocochleography

37. • SEROLOGY.
Fluorescent treponemal antibody absorption is mandatory in any
patient given the diagnosis of an idiopathic disease since syphilis may
perfectly imitate Meniere’s disease.
Autoimmune inner ear disease may present initially with a Meniere’s
picture. The distinguishing characteristics of an autoimmune inner ear
disease include a more aggressive course and early bilateral
involvement. Autoimmune serologic tests may also be helpful. A
promising test is a Western blot looking for anticochlear antibodies
that can bind to a 68 to 70 kD antigen.

38. • Caloric test
• It shows reduced response on the affected side in 75% of cases.
• Often, 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 canal
paresis on the affected side and directional preponderance on the opposite side.
• Glycerol test
• Glycerol is a dehydrating agent.
• When given orally, it reduces endolymph pressure and thus causes an improvement in
hearing.
• Patient is given glycerol (1.5 mL/kg) with an equal amount of water and a little
flavouring agent or
lemon juice.
• Audiogram and speech discrimination scores are recorded before and 1–2 h after
ingestion of glycerol.
• An improvement of 10 dB in two or more adjacent octaves or gain of 10% in
discrimination score makes the test positive.
• There is also improvement in tinnitus and in the sense of fullness in the ear.
• The test has a diagnostic and prognostic value.
• These days, glycerol test is combined with electrocochleography.

39. Delayed MRI – Gadolinium based
Intratympanic (24 hrs before mri )or i/v (4hrs before
MRI)gadolinium
Gadolinium loads in to the perilymphatic space without
entering the endolymph in normal ears
To exclude CP angle and inner ear pathology,
ELECTRO NYSTAGMOGRAPHY
Can measure residual function and evaluate the
treatment effectiveness to destroy vestibular function

40. VARIANTS OF MENIERE’S DISEASE
• Cochlear hydrops –
• Only cochlear symptoms present
• Vertigo is absent, may appear after several years
• Block at level of ductus reuniens, confining increased pressure to cochlea
only.
• Vestibular hydrops –
• Typical attacks of episodic vertigo
• Cochlear functions normal
• Lermoyez syndrome –
• Symptoms of menier’s appear in reverse order
• Hearing loss -> vertigo attack (hearing recovers by this time)

41. VARIANTS OF MENIERE’S DISEASE
• Drop attacks
• a/k/a tumarkin or otolithic crises
• Reason - acute otolithic dysfunction of utricle or saccule due to changes in
endolymphatic pressure
• Occur in the later stages of the disease
• Patient simply drops to the ground without warning and can sustain a fracture
or a serious injury.
• Sensation of being pushed to the ground by some external force during these
attacks.
• No associated vertigo or loss of consciousness.
• Number of attacks varies

42. STAGING OF MENIERE’S DISEASE
• It is based on average of the pure tone thresholds at 0.5, 1, 2, 3 kHz
(rounded to nearest whole) of the worst audiogram during interval of
6 months before treatment.

43. MANAGEMENT
GENERAL MEASURES
• The treatment of Meniere's disease should be preceded by diagnosis on the basis
of strict criteria and after Exclusion of retrocochlear pathology and of causes such
as Cogan's syndrome or syphilis.
Reasurance. Patients anxiety can be relieved by reasurance and by
expalaing the true nature of disease
Cessation of smoking. Nicotine causes vasospasm. Smoking
should be completely stopped.
• Level of anxiety correlates with severity of symptoms
• Stress may contribute via autonomic mechanisms in the genesis of endolymphatic
hydrops, thus indicating the need for lifestyle adaptations.
• restrict salt intake to 1- 1.5 mg per day(Low salt diet)
• Caffeine free diet,avoid alcohol

44. MEDICAL TREATMENT
Aim – to decrease production or accumulation of endolymph
Drug therapy for Meniere's disease may include:
• acute vestibular symptoms suppression,
• Diuretics (Hydrochlorothiazide , acetazolamide , triamterene )
MOA– Reduce endolymphatic volume and pressure
Osmotic diuretics, oral urea powder twice a week, are rarely used
Diuretics Can cause electrolyte disturbances and drug interaction
• Betahistin (potent H3 antagonist and weak H1 agonist)
16 mg tid
Limited side effect profile

45. Transtympanic low pressure therapy
(MENIETT)
It is observed that intemittent positive pressure deliverd to
inner ear fluid brings relief from symptoms of MD.
 Intemittend positve pressure wave can be delivered through an
instrument called meniett device.
 A prerequisite for this thearpy is insertion of grommet tube so
that device can deliver pressure waves to round window
membrane via grommet tube.

46.  Pressure wave passes through perilymph and causes reduction o
in endolymph pressure by redistributing it through endolymphatic sac
or the blood vessels.

47. Used for 5 minute 3 times a day.
 Patient can self administer the treatment at home.

48. Intratympanic injection of corticosteroids
Mechanism is inner ear homeostasis via transmembrane
water transporters , the aquaporins
Long term Vertigo control in refractory cases without
significant hearing loss

49. Intratympanic injection of AG
• Gentamicin is predominantly vestibulotoxic and acts by destroying the
dark cells of the secretory epithelium, thus decreasing endolymph
production.
• high efficacy in controlling the vertigo of up to 90 percent,
• profound sensorineural hearing loss may develop in 3-30 percent
treated cases.
• This treatment should be reserved for patients who have failed
medical therapy.

50. Other means of treatment.
• Alternobaric oxygen therapy with continuous variations in pressure
• Inhalation of carbogen ( 5% CO2 + 95% O2 ).

51.  1) CONSERVATIVE PROCEDURES:- they are used in case where
vertigo is disabling but hearing is still useful and need to be
preserved.
a) endolymphatic sac decompression
b) endolymphatic shunt operation
c) sacculotomy
d) cochleosacculostomy
e) section of vestiblar nerve
d) ultrasonic destruction of vestibular labyrinth

52. ENDOLYMPHATIC SAC SURGERY
• Endolymphatic sac surgery involves a
mastoidectomy and locating the ES on the
posterior fossa dura.
• The sac is medial to the sigmoid sinus and inferior
to the PSCC.
• Donaldson line:- a imaginary line
passing through horizontal scc and
bisects the posterior scc.this is landmark
for endolymphatic sac,which is located
below and along this line.
• The ES may be decompressed or have a shunt
placed that communicates into the subarachnoid
space or mastoid cavity.

53. 2). ENDOLYMPHATIC SHUNT OPERATION.
A tube is put, connecting endolymphatic sac with subarachnoid
space,to drain excess endolymph.

54. 3) SACCULOTOMY:- (ficks operation)
 It is puncturing the saccule with a needle
through stapes footplate.
 A distended saccule lies against stapes footplate,
can be easily penetrated.

55. VESTIBULAR NERVE SECTION.
• Vestibular nerve section provides a
definitive treatment of unilateral
Meniere’s disease in patients with
serviceable hearing.
• Ninety-five Percent of patients achieve
vertigo control, and hearing is preserved
in over 95% of patients.
• The vestibular neurectomy may be
approached via a retrosigmoid or middle
fossa approach.
• The risk to the facial nerve is less than 1%
in the retrosigmoid approach and less
than 5% in the middle fossa approach.

56. • The advantage of the middle fossa approach is sectioning the
vestibular nerves in the IAC lateral to where they join the cochlear
nerve in the CPA.
• The patients are acutely vertiginous and have nystagmus (fast phase
away from the operated ear) for a few days until central compensation
takes effect.
• The hearing appears to degenerate in the postoperative patient in
accordance with the natural history of Meniere’s disease.

57. DESTRUCTIVE PROCEDURE:- they
totally destroy cochlear and
vestibular function so it is used only
when cochlear function is not
serviceable.

58. LABYRINTHECTOMY.
• A transmastoid labyrinthectomy with fenestration of the bony
semicircular canals and vestibule and removal of the membranous
neuroepithelium provides control of vertigo in nearly all patients with
unilateral Meniere’s disease and poor hearing.
• The rate of control may decline by 10 years owing to development of
vertebral basilar artery insufficiency (aging), poorer vision, and
development of Meniere’s disease in the contralateral ear.
• The complete loss of unilateral vestibular function owing to the
labyrinthectomy leads to unsteadiness in up to 30% of patients.

59. THANK YOU