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DR.KAUSHIK SUTRADHAR
PGT , DEPTT. OF ENT
is a disorder of the inner ear characterized by
 Acute rotatory vertigo which is unpredictible,
precipitous in nature associated with nausea
and/or vomiting.
 Hearing loss which is unilateral low-tone
fluctuant sensorineural
 Tinnitus
 Aural fullness
 1747 – Antonio Scarpa described anatomy of
membranous labyrinth
 1861 – Prosper Meniere described the classic
features of Meniere’s disease & attributed it to
labyrinthine causes
 1871 – Knappin theorized that dilated
membranous labyrinth to be the cause of this
disorder
 1927 – Guild described endolymphatic ciruclation
 1938 – Hallpike and Portmann described
pathology of Meniere’s disease by studying
temporal bones
Type I
(aka inner)
Type II
(aka outer)
With
Kinocilium
VESTIBULAR HAIR CELLS
• Perilymph is similar in composition to
CSF (Containing high Na and low K ions)
• Endolymph similar in composition to
intracellular fluid (Containing low Na and
high K concentration). It is secreted by
stria vascularis
1.Secretory function
2.Absorptive function
3.Immune / defense function
 Roughly 1 in 1000 individuals are affected
 Constitutes 10% of all patients attending
vertigo clinic
 Female preponderance
 Rare in children under the age of 10
 Commonly begins between 4th and 5th
decades of life
 Bilateral Meniere’s syndrome is seen in 5% of
these patients
 Idiopathic
 Anatomical (small vestibular aqueduct)
 Viral infection (HSV type 1)
 Traumatic (physical, acoustic)
 Allergy
 Autoimmunity
 CSOM
 Syphilis
 Otosclerosis
 Longitudinal flow
 Radial flow
 Dynamic flow
 Was first proposed by Guild
 Striavascularis is the principal source
 This is a slow process
 Elimination occurs at the endolymphatic sac
level
 This is active process (energy consuming)
 Production occurs from dark vestibular cells &
planum semilunatum
 Absorption occurs at the stria vestibularis
 First proposed by Lawrence
 This is a combination of both longitudinal
and radial flow patterns
 Endolymphatic hydrops causes distortion of
membranous labyrinth
 Pressure building up in the scala media may
cause mirco ruptures of membranous
labyrinth
 This would account for the episodic nature of
the attacks
 Healing of these ruptures causes resolution of
the disorder
 Small amounts of excess endolymph can be
cleared by radial flow
 Larger volumes need longitudinal flow for
their clearance
 Endolymphatic sinus temporarily
accommodates excess endolymph till the sac
is ready for it
 Endolymphatic valve of Bast isolates pars
superior and prevents endolymph from
draining out of the utricle
The excess volume
tends to accumulate in
the apical end of the
cochlea, where the
membranes are more
lax than elsewhere,
even though the
endolymph pressure
would be similar
elsewhere in the
cochlea.
1.Stage I – Patient has solely cochlear
symptoms
2. Stages II – IV – Patients have progressively
more cochlear and vestibular symptoms
3. Stage V – End stage Meniere’s disease
(dead ear)
 Classical Meniere’s disease
 Vestibular Meniere’s disease – vestibular
symptoms and aural pressure
 Cochlear Meniere’s disease – cochlear
symptoms and aural pressure
 Lermoyez syndrome – Reverse Meniere’s
 Tumarkin’s crisis – Utricular Meniere’s
 variant of Meniere’s disease
 sudden sensorineural hearing loss, which
improves during or immediately after the
attack of vertigo.
 Cause is sudden spasm of the labyrinthine
artery followed by immediate dilatation
 AKA Tumarkin’s drop attacks
 abrupt falling attacks of brief duration
without loss of consciousness.
 due to an enlarging utricle due to excess
endolymphatic volume
 Sensori neural in nature
 Fluctuating and progressive
 Affects low frequencies
 Mild low frequency conductive hearing loss
(rare)
 Profound sensori neural hearing loss (End
stage)
 Roaring in nature
 Could be continuous / intermittent
 Non pulsatile in nature
 Frequency of tinnitus corresponds to the
region of cochlea which has suffered the
maximum damage
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 spontaneous vertigo
 Audiometrically documented hearing loss at least during one
attack
 Tinnitus and aural fullness in the affected ear
Definitive Meniere’s disease
 Two or more definitive episodes of spontaneous vertigo one
atleast lasting for 20 mins.
Audiometrically documented hearing loss at least on
one occasion.
 Tinnitus and aural fullness in the affected ear
 History
1. Nature of the sensation
2. Timing of the initial spell
3. Frequency and duration of the symptoms
4. Precipitating factors
 Vestibular tests
 Complete Haemogram
 Audiometry
 Loudness recruiment
 VEMP
 Dehydration tests
 Posturography
 Electronystagmography
 This is abnormal growth in the perceived
intensity of sound
 This is usually positive in patients with
Meniere’s disease
 ABLB is the test used to look for the presence
of recruitment
 This test is really time consuming
 Alternate Binaural loudness balance test
 Tone is presented alternately between the two
ears. The level of the tone stays the same in one
ear (i.e. fixed ear) and is varied up / down in the
other ear (i.e. variable ear). The patient is asked to
report when the sound is louder in the right ear,
louder in the left ear, and when it sounds equal in
both ears.
 Loudness balance is said to have been obtained
when the patient indicates that the sound is heard
equally in both ears. The tester then records the
two levels in dB hearing level where the balance
has occurred.
 Increased summating potential / action
potential ratio. 1:3 is normal
 Widened summating potential / action
potential complex. A widening of greater
than 2 ms is significant
 Small distorted cochlear microphonics
 Vestibular evoked myogenic potential
 Measures the relaxation of sternomastoid muscle in
response to ipsilateral click stimulus
 Brief high intensity ipsilateral clicks produce large
short latency inhibitory potentials (VEMP) in the
toncially contracted Ipsilateral sternomastoid muscle
 This test is due to the presence of vestibulo collic
reflex
 Afferent arises from sound responsive cells in the
saccule, conducted via the inferior vestibular nerve.
 Efferent is via vestibulo spinal tract
 Normal responses are composed of biphasic
(positive-negative) waves
 VEMP reveals saccular dysfunction
 Glycerol
 Mannitol
 Frusemide
 Isosorbide
 These tests involve the subject ingesting
glycerol or mannitol and observing for a
change in symptoms and a measurable
improvement in hearing
Tests are positive if there is pure tone
improvement of 10dB or more at two / more
frequencies between 200-2000Hz
 First introduced by Klockhoff and Lindblom –
1966
 Glycerol is administered in doses of 1.5 mg/kg
body wt in empty stomach
 Serum osmolality should increase at least by 10
mos/kg
 Side effects include Headache, Nausea, vomiting,
drowsiness
 PTA is performed 2-3 hours after administration
 False positivity is rare
 Positivity depends on the phase of the disease
 Antibodies to 68-kDa protein has been noted
in many patients with meniere’s disease
the aim is to decrease the production or accumulation of
the endolymph
CONSERATIVE
 Dietary sodium restriction (1mg/day)
 Restriction of caffeine and nicotine like substances
 Diuretics like bendroflurazide,dyazide, chlorthalidone
 Betahistine
histamine analogue with weak H1, H2 agonistic and
moderate H3 antagonistic action
causes improved microvascular circulation in striae
vascularis
inhibition of vestibular nuclei activity
 Calcium agonists
 Steroids
1. Topical application via tympanostomy tubes
2. Shea et al reported 35.4% hearing
improvement and complete vertigo control
in 63.4% cases treated with 16 mg
intratympanic and 16 mg i.v.
dexamethasone for three consecutive days
followed by oral dexamethasone
3. Silverstein microwick can be used for
intratympanic drug administration
 Intratympanic injection of aminoglycosides
a form of chemical labyrinthectomy,
gentamycin therapy ablates the vestibular
“dark cells” of the secretory epithelium thus
decreasing endolymph production
response to this is measured by in response
to rapid, rotatory head thrusts
 Alternobaric oxygen therapy
 Local overpressure therapy by means of Meniett
device which applies intermittent micropressure
to the inner ear via a tympanostomy tube
1. Diagnosis should be confirmed
2. Ventilation tube should be inserted
3. Patient should be trained for self
administration of the treatment
4. Usually administered thrice a day about 5
mins each time
5. Treatment lasts for 5 weeks
1. Classic unilateral Meniere’s disease
2. Intense vestibular / cochlear symptoms
3. Failed medical therapy
4. Over 65 years of age
5. Imbalance / aural fullness / tinnitus after
gentamycin treatment
1. Perilymph fistula
2. Acoustic neuroma / brain tumor
3. Retrocochlear damage
4. Low pressure hydrocephalus
1. Isordil
2. ϒ – globulin
3. Urea
4. Glycerol
5. Lithium
6. Anticholinergics – Glycopyrrolate 1-2 mg /day
7. Antidopaminergics – Droperidol 2.5 – 10 mg orally /
day
8. Leuprolide acetate – Blocks normal sex hormone
production
9. Innovar – A combination of droperidol and fentanyl
can be used to suppress vestibular symptoms (can
replace endolymphatic sac surgery)
 Endolymph decompression
 First described by portmann 1926
 Via the round window by otic-periotic shunt that
perforates the basilar membrane
 Cochleosacculotomy creates a fracture dislocation of
osseous spiral lamina
both these procedures have highdegree of hearing
loss
1. Helpful in treating debilitated patients
2. Involves disruption of osseous spiral lamina
3. Angular pick introduced via round window towards
oval window. It will accommodate 3 mm long pick
4. After perforation the pick is withdrawn and the round
window is sealed by fat
 Simple decompression
 Cannulation of endolymphatic duct
 Endolymphatic drainage to the subarachnoid
space
 Drainage to mastoid
 Removal of extraosseous portion of the sac
1. Labyrinthectomy
2. Translabyrinthine vestibular neurectomy
3. Retrolabyrinthine vestibular neurinectomy
4. Retrosigmoid vestibular neurinectomy
5. Middle cranial fossa vestibular neurinectomy
 Vestibular neurectomy
1. more complete vertigo control than shunt
procedures
2. Lower risk of hearing loss than gentamicin
therapy
3. Middle fossa approach, risk of facial nerve
injury is higher
4. Suboccipital approach
 Labyrinthectomy
1. Transcanal approach
2. Transmastoid approach, more common
 Vestibular rehabilatation
Cawthorne-Cooksey exercises
MENIERE’S DISEASE

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MENIERE’S DISEASE

  • 2. is a disorder of the inner ear characterized by  Acute rotatory vertigo which is unpredictible, precipitous in nature associated with nausea and/or vomiting.  Hearing loss which is unilateral low-tone fluctuant sensorineural  Tinnitus  Aural fullness
  • 3.  1747 – Antonio Scarpa described anatomy of membranous labyrinth  1861 – Prosper Meniere described the classic features of Meniere’s disease & attributed it to labyrinthine causes  1871 – Knappin theorized that dilated membranous labyrinth to be the cause of this disorder  1927 – Guild described endolymphatic ciruclation  1938 – Hallpike and Portmann described pathology of Meniere’s disease by studying temporal bones
  • 4.
  • 5.
  • 6. Type I (aka inner) Type II (aka outer) With Kinocilium VESTIBULAR HAIR CELLS
  • 7.
  • 8. • Perilymph is similar in composition to CSF (Containing high Na and low K ions) • Endolymph similar in composition to intracellular fluid (Containing low Na and high K concentration). It is secreted by stria vascularis
  • 10.  Roughly 1 in 1000 individuals are affected  Constitutes 10% of all patients attending vertigo clinic  Female preponderance  Rare in children under the age of 10  Commonly begins between 4th and 5th decades of life  Bilateral Meniere’s syndrome is seen in 5% of these patients
  • 11.  Idiopathic  Anatomical (small vestibular aqueduct)  Viral infection (HSV type 1)  Traumatic (physical, acoustic)  Allergy  Autoimmunity  CSOM  Syphilis  Otosclerosis
  • 12.  Longitudinal flow  Radial flow  Dynamic flow
  • 13.  Was first proposed by Guild  Striavascularis is the principal source  This is a slow process  Elimination occurs at the endolymphatic sac level
  • 14.  This is active process (energy consuming)  Production occurs from dark vestibular cells & planum semilunatum  Absorption occurs at the stria vestibularis
  • 15.  First proposed by Lawrence  This is a combination of both longitudinal and radial flow patterns
  • 16.
  • 17.  Endolymphatic hydrops causes distortion of membranous labyrinth  Pressure building up in the scala media may cause mirco ruptures of membranous labyrinth  This would account for the episodic nature of the attacks  Healing of these ruptures causes resolution of the disorder
  • 18.  Small amounts of excess endolymph can be cleared by radial flow  Larger volumes need longitudinal flow for their clearance  Endolymphatic sinus temporarily accommodates excess endolymph till the sac is ready for it  Endolymphatic valve of Bast isolates pars superior and prevents endolymph from draining out of the utricle
  • 19. The excess volume tends to accumulate in the apical end of the cochlea, where the membranes are more lax than elsewhere, even though the endolymph pressure would be similar elsewhere in the cochlea.
  • 20. 1.Stage I – Patient has solely cochlear symptoms 2. Stages II – IV – Patients have progressively more cochlear and vestibular symptoms 3. Stage V – End stage Meniere’s disease (dead ear)
  • 21.  Classical Meniere’s disease  Vestibular Meniere’s disease – vestibular symptoms and aural pressure  Cochlear Meniere’s disease – cochlear symptoms and aural pressure  Lermoyez syndrome – Reverse Meniere’s  Tumarkin’s crisis – Utricular Meniere’s
  • 22.  variant of Meniere’s disease  sudden sensorineural hearing loss, which improves during or immediately after the attack of vertigo.  Cause is sudden spasm of the labyrinthine artery followed by immediate dilatation
  • 23.  AKA Tumarkin’s drop attacks  abrupt falling attacks of brief duration without loss of consciousness.  due to an enlarging utricle due to excess endolymphatic volume
  • 24.  Sensori neural in nature  Fluctuating and progressive  Affects low frequencies  Mild low frequency conductive hearing loss (rare)  Profound sensori neural hearing loss (End stage)
  • 25.  Roaring in nature  Could be continuous / intermittent  Non pulsatile in nature  Frequency of tinnitus corresponds to the region of cochlea which has suffered the maximum damage
  • 26. 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 spontaneous vertigo  Audiometrically documented hearing loss at least during one attack  Tinnitus and aural fullness in the affected ear Definitive Meniere’s disease  Two or more definitive episodes of spontaneous vertigo one atleast lasting for 20 mins. Audiometrically documented hearing loss at least on one occasion.  Tinnitus and aural fullness in the affected ear
  • 27.  History 1. Nature of the sensation 2. Timing of the initial spell 3. Frequency and duration of the symptoms 4. Precipitating factors  Vestibular tests  Complete Haemogram  Audiometry  Loudness recruiment  VEMP  Dehydration tests  Posturography  Electronystagmography
  • 28.  This is abnormal growth in the perceived intensity of sound  This is usually positive in patients with Meniere’s disease  ABLB is the test used to look for the presence of recruitment  This test is really time consuming
  • 29.  Alternate Binaural loudness balance test  Tone is presented alternately between the two ears. The level of the tone stays the same in one ear (i.e. fixed ear) and is varied up / down in the other ear (i.e. variable ear). The patient is asked to report when the sound is louder in the right ear, louder in the left ear, and when it sounds equal in both ears.  Loudness balance is said to have been obtained when the patient indicates that the sound is heard equally in both ears. The tester then records the two levels in dB hearing level where the balance has occurred.
  • 30.  Increased summating potential / action potential ratio. 1:3 is normal  Widened summating potential / action potential complex. A widening of greater than 2 ms is significant  Small distorted cochlear microphonics
  • 31.  Vestibular evoked myogenic potential  Measures the relaxation of sternomastoid muscle in response to ipsilateral click stimulus  Brief high intensity ipsilateral clicks produce large short latency inhibitory potentials (VEMP) in the toncially contracted Ipsilateral sternomastoid muscle  This test is due to the presence of vestibulo collic reflex  Afferent arises from sound responsive cells in the saccule, conducted via the inferior vestibular nerve.  Efferent is via vestibulo spinal tract  Normal responses are composed of biphasic (positive-negative) waves  VEMP reveals saccular dysfunction
  • 32.  Glycerol  Mannitol  Frusemide  Isosorbide  These tests involve the subject ingesting glycerol or mannitol and observing for a change in symptoms and a measurable improvement in hearing Tests are positive if there is pure tone improvement of 10dB or more at two / more frequencies between 200-2000Hz
  • 33.  First introduced by Klockhoff and Lindblom – 1966  Glycerol is administered in doses of 1.5 mg/kg body wt in empty stomach  Serum osmolality should increase at least by 10 mos/kg  Side effects include Headache, Nausea, vomiting, drowsiness  PTA is performed 2-3 hours after administration  False positivity is rare  Positivity depends on the phase of the disease
  • 34.  Antibodies to 68-kDa protein has been noted in many patients with meniere’s disease
  • 35.
  • 36.
  • 37. the aim is to decrease the production or accumulation of the endolymph CONSERATIVE  Dietary sodium restriction (1mg/day)  Restriction of caffeine and nicotine like substances  Diuretics like bendroflurazide,dyazide, chlorthalidone  Betahistine histamine analogue with weak H1, H2 agonistic and moderate H3 antagonistic action causes improved microvascular circulation in striae vascularis inhibition of vestibular nuclei activity  Calcium agonists
  • 38.  Steroids 1. Topical application via tympanostomy tubes 2. Shea et al reported 35.4% hearing improvement and complete vertigo control in 63.4% cases treated with 16 mg intratympanic and 16 mg i.v. dexamethasone for three consecutive days followed by oral dexamethasone 3. Silverstein microwick can be used for intratympanic drug administration
  • 39.  Intratympanic injection of aminoglycosides a form of chemical labyrinthectomy, gentamycin therapy ablates the vestibular “dark cells” of the secretory epithelium thus decreasing endolymph production response to this is measured by in response to rapid, rotatory head thrusts  Alternobaric oxygen therapy
  • 40.  Local overpressure therapy by means of Meniett device which applies intermittent micropressure to the inner ear via a tympanostomy tube
  • 41. 1. Diagnosis should be confirmed 2. Ventilation tube should be inserted 3. Patient should be trained for self administration of the treatment 4. Usually administered thrice a day about 5 mins each time 5. Treatment lasts for 5 weeks
  • 42. 1. Classic unilateral Meniere’s disease 2. Intense vestibular / cochlear symptoms 3. Failed medical therapy 4. Over 65 years of age 5. Imbalance / aural fullness / tinnitus after gentamycin treatment
  • 43. 1. Perilymph fistula 2. Acoustic neuroma / brain tumor 3. Retrocochlear damage 4. Low pressure hydrocephalus
  • 44. 1. Isordil 2. ϒ – globulin 3. Urea 4. Glycerol 5. Lithium 6. Anticholinergics – Glycopyrrolate 1-2 mg /day 7. Antidopaminergics – Droperidol 2.5 – 10 mg orally / day 8. Leuprolide acetate – Blocks normal sex hormone production 9. Innovar – A combination of droperidol and fentanyl can be used to suppress vestibular symptoms (can replace endolymphatic sac surgery)
  • 45.  Endolymph decompression  First described by portmann 1926  Via the round window by otic-periotic shunt that perforates the basilar membrane  Cochleosacculotomy creates a fracture dislocation of osseous spiral lamina both these procedures have highdegree of hearing loss 1. Helpful in treating debilitated patients 2. Involves disruption of osseous spiral lamina 3. Angular pick introduced via round window towards oval window. It will accommodate 3 mm long pick 4. After perforation the pick is withdrawn and the round window is sealed by fat
  • 46.  Simple decompression  Cannulation of endolymphatic duct  Endolymphatic drainage to the subarachnoid space  Drainage to mastoid  Removal of extraosseous portion of the sac
  • 47. 1. Labyrinthectomy 2. Translabyrinthine vestibular neurectomy 3. Retrolabyrinthine vestibular neurinectomy 4. Retrosigmoid vestibular neurinectomy 5. Middle cranial fossa vestibular neurinectomy
  • 48.  Vestibular neurectomy 1. more complete vertigo control than shunt procedures 2. Lower risk of hearing loss than gentamicin therapy 3. Middle fossa approach, risk of facial nerve injury is higher 4. Suboccipital approach  Labyrinthectomy 1. Transcanal approach 2. Transmastoid approach, more common

Editor's Notes

  1. Nature: sense of motion can be rotatory, linear, change in orientation relative to vertical vertigo indicates problem in peripheral vestibular system Horizontal movements ndicate scc problems while drop attacks indicate otolith dysfunction Timing: after serious illness -ototoxic drugs , starting or stopping drugs, changing dosage Trauma or infection positional vertigo stapes surgery perilymphatic fistula Girl having menarche might have initial spell of dizziness hormone related Frequency n duration:short term spells >bppv,scc dehiscence,perilymphatic fistula….medium length upto 4 hrs meniers…..longer ones migraine
  2. Alternate Binaural loudness balance test