meniere's disease

1. MDDr. AJAY MANICKAM
JR- DEPT OF ENT ,RG KAR MC
eniere’s isease

2.  Idiopathic endolymphatic hydropsIdiopathic endolymphatic hydrops

Dr. Robert Prosper Meniere, 1861Dr. Robert Prosper Meniere, 1861 (vertigo, imbalance, inner ear)(vertigo, imbalance, inner ear)

Portmann, 1926Portmann, 1926 (endolymphatic sac, decompression in human)(endolymphatic sac, decompression in human)

Hallpike & Cairns, 1938Hallpike & Cairns, 1938 (endolymphatic hydrops)(endolymphatic hydrops)
 ““Recurrent, spontaneous episodic vertigo; hearing loss; aural fullness; and tinnitus. EitherRecurrent, spontaneous episodic vertigo; hearing loss; aural fullness; and tinnitus. Either
tinnitus or aural fullness (or both) must be present on the affected side to make thetinnitus or aural fullness (or both) must be present on the affected side to make the
diagnosis.” – AAO-HNS, 1995diagnosis.” – AAO-HNS, 1995
 A primary otologic disorder, often overdiagnosedA primary otologic disorder, often overdiagnosed

3. Fluctuating
sensorineural hearing loss
Episodic vertigo
Roaring tinnitus
Aural fullness

4. EpidemiologyEpidemiology
 40-60 years (range: 4-90)40-60 years (range: 4-90)
 Female:male = 1.3Female:male = 1.3
 Primarily affects the CaucasiansPrimarily affects the Caucasians
 15/1,00,000 in the US, 157/1,00,000 in the UK15/1,00,000 in the US, 157/1,00,000 in the UK
 High prevalence in India, but less diagnosedHigh prevalence in India, but less diagnosed
 Familial predisposition; ~50% have positive family historyFamilial predisposition; ~50% have positive family history

5. VertigoVertigo

Sensation of motion when no motion is occurring relative to Earth’s gravitySensation of motion when no motion is occurring relative to Earth’s gravity (AAO-HNS)(AAO-HNS)
 Meniere’s disease: ~10% of all causes of vertigoMeniere’s disease: ~10% of all causes of vertigo
 Most distressing, affecting QOL the mostMost distressing, affecting QOL the most
 RotationalRotational

RecurrentRecurrent (2 or more episodes)(2 or more episodes)
 SpontaneousSpontaneous
 EpisodicEpisodic
 20 minutes – 24 hours20 minutes – 24 hours

6. Hearing lossHearing loss
 SensorineuralSensorineural
 Low frequenciesLow frequencies
 Fluctuating; audiometrically documented on at least 1 occasion; returns to the baselineFluctuating; audiometrically documented on at least 1 occasion; returns to the baseline
 UnilateralUnilateral (subsequent contralateral involvement in 2-78% cases, average ~20-30%; 7.6 years)(subsequent contralateral involvement in 2-78% cases, average ~20-30%; 7.6 years)
 Increases in severity with time; flattening of curveIncreases in severity with time; flattening of curve
 Distortion (disharmonic diplacusis), intolerance to loud noisesDistortion (disharmonic diplacusis), intolerance to loud noises

7. Tinnitus and aural fullnessTinnitus and aural fullness
 Low pitched, rumbling/roaringLow pitched, rumbling/roaring
 The severity is proportional to the degree of hearing lossThe severity is proportional to the degree of hearing loss
 Less intrusive (Less intrusive (cf.cf. noise exposure, presbyacusis, migraine, panic attack)noise exposure, presbyacusis, migraine, panic attack)
 Vertigo and hearing loss are more pressing symptoms, hence is not the principalVertigo and hearing loss are more pressing symptoms, hence is not the principal
presenting complaintpresenting complaint
 <33% patients present with the complete triad<33% patients present with the complete triad
 Associated with aural fullness/pressure in the affected site; may/may not return toAssociated with aural fullness/pressure in the affected site; may/may not return to
baselinebaseline
 Tinnitus and aural fullness build up gradually before precipitation of a vertigo attackTinnitus and aural fullness build up gradually before precipitation of a vertigo attack

8. Etiopathogenesis of Meniere’s
disease

10. Allergy
Autoimmunity
Viral infection
Genetic factors
Anatomic factors
Vascular events
Posterior fossa tumors
Allergy
Autoimmunity
Viral infection
Genetic factors
Anatomic factors
Vascular events
Posterior fossa tumors
Altered endolymph homeostasis
Altered endolymph flow
Altered endolymph composition
Altered secretory function of sac
Immunoreactivity of sac
Altered endolymph homeostasis
Altered endolymph flow
Altered endolymph composition
Altered secretory function of sac
Immunoreactivity of sac
Endolymphatic hydropsEndolymphatic hydrops
Events within the membranous labyrinth
Asymptomatic state
Allergy, viral, others

11. Patholophysiology
unknown
Multifactorial

12. Diagnosis of Meniere’s
disease

13. Differential diagnoses of Meniere’s diseaseDifferential diagnoses of Meniere’s disease
 Secondary endolymphatic hydropsSecondary endolymphatic hydrops
otic syphilisotic syphilis
delayed endolymphatic hydropsdelayed endolymphatic hydrops
Cogan’s syndrome (interstitial keratitis)Cogan’s syndrome (interstitial keratitis)
recurrent vestibulopathyrecurrent vestibulopathy
 Other forms of hydropsOther forms of hydrops
Larmoyez syndromeLarmoyez syndrome
cochlear hydropscochlear hydrops
vestibular hydropsvestibular hydrops

14. Larmoyez syndromeLarmoyez syndrome
 Rare variant of Meniere’s diseaseRare variant of Meniere’s disease
 Symptoms arise in reverse orderSymptoms arise in reverse order
 Progressive hearing lossProgressive hearing loss →→ episodic vertigoepisodic vertigo →→ recovery of hearingrecovery of hearing
 MigraineMigraine

15. ClinicalClinical

16. Diagnosis of exclusion

17. Investigations pertaining to Meniere’s diseaseInvestigations pertaining to Meniere’s disease
 Pure tone audiometryPure tone audiometry
 Transtympanic electrocochleography (ECoG)Transtympanic electrocochleography (ECoG)
 Electronystamography (ENG), videonystagmography (VNG)Electronystamography (ENG), videonystagmography (VNG)

18. Treatment of Meniere’s
disease

19.  On-and-off phenomenon of the disease processOn-and-off phenomenon of the disease process
 Unpredictability of the clinical courseUnpredictability of the clinical course
 Need for individualizationNeed for individualization
 Evolutionary pattern: the intrinsic nature of the diseaseEvolutionary pattern: the intrinsic nature of the disease
No definite treatment protocol
As good as placebo effect?
No proved cure

20. Treatment of Meniere’s diseaseTreatment of Meniere’s disease
 Assurance and re-assuranceAssurance and re-assurance
 ConservativeConservative (medical)(medical)
 SurgicalSurgical (non-destructive and destructive)(non-destructive and destructive)

21. Conservative managementConservative management
 Lifestyle managementLifestyle management
Avoidance of anxiety (with/without administration of anxiolytics or sedatives)Avoidance of anxiety (with/without administration of anxiolytics or sedatives)
Proper sleep and regular mealsProper sleep and regular meals
Salt restriction with intake of plenty of waterSalt restriction with intake of plenty of water
Exercises for stress reduction and conditioningExercises for stress reduction and conditioning
Improving the Quality of Life

22. Conservative managementConservative management
 Salt restrictionSalt restriction (2-2.5 gm; no added salt in food)(2-2.5 gm; no added salt in food) + plenty of water + diuretics+ plenty of water + diuretics
(acetazolamide/hydrochlorthiazide + triamterine/K+ supplementation)(acetazolamide/hydrochlorthiazide + triamterine/K+ supplementation)
decreases the endolymph volume by indirect, unrecognized mechanism
keeps [AVP] low, preventing action of ADH on their receptors in the sac wall
direct effect on ion transport in the stria vascularis and spiral ligament
Decreasing the fluid content

23. Conservative managementConservative management
 BetahistineBetahistine (releases histamine, increases cochlear blood flow; vestibular sedation through H3-antagonism)(releases histamine, increases cochlear blood flow; vestibular sedation through H3-antagonism)
 CO2 inhalationCO2 inhalation
 Exercises for stress reduction and conditioningExercises for stress reduction and conditioning
 Nicotinic acids, papaverineNicotinic acids, papaverine
 LipoflavonoidsLipoflavonoids (eriodictyol glycoside)(eriodictyol glycoside)
Vasodilatation
Decreased blood flow in the inner ear
Aimed at increasing the inner ear circulation
No convincing association with the pathophysiology of the Meniere’s disease

24. Conservative managementConservative management
 Oral/IV steroidsOral/IV steroids
 Intratympanic steroids (dexamethasone)Intratympanic steroids (dexamethasone)
 Methotrexate, cytoxan, allergic desensitizationMethotrexate, cytoxan, allergic desensitization
Altering the immune reactivity Refractory to other medical treatment
Sudden decline in hearing
Bilateral Meniere’s disease
Suspected autoimmune inner ear syndrome

25. Conservative managementConservative management
 AntihistaminicsAntihistaminics (Meclizine)(Meclizine)
 AmitriptylineAmitriptyline
 BenzodiazepinesBenzodiazepines (diazepam, clonazepam, lorazepam)(diazepam, clonazepam, lorazepam)
Vestibular suppressants
Anticholinergic effect
Meant to cover brief (acute) period of vertigo (up to 2 weeks; “crisis intervention”)
Psychological and physical dependence
Impaired compensation, prolonged symptoms, suboptimal results

26. Conservative managementConservative management
 Streptomycin or gentamicinStreptomycin or gentamicin
 Gentamicin favored; streptomycin has narrow therapeutic window and less availableGentamicin favored; streptomycin has narrow therapeutic window and less available
 Gentamicin preferentially toxic to the vestibular hair cells (?), and also to the dark cellsGentamicin preferentially toxic to the vestibular hair cells (?), and also to the dark cells
Intratympanic aminoglycoside injection
Chemical ablation of vestibular end-organ with lesser chance of hearing loss
Also decreases endolymphatic fluid volume by action on dark cells
Mainly for unilateral cases

27. Combined intratympanic aminoglycoside & steroid therapyCombined intratympanic aminoglycoside & steroid therapy
 The “sub-ablative” therapyThe “sub-ablative” therapy
 Low dose aminoglycoside + high dose dexamethasoneLow dose aminoglycoside + high dose dexamethasone
 Combined advantages of both the agentsCombined advantages of both the agents
 Improves QOLImproves QOL
 A substitute of surgeryA substitute of surgery

28. Medical therapy Surgery
Intermediate
• Persistent episodic vertigo
refractory to medical treatment
• > once/month
Intratympanic drug delivery
Vertigo control rate ~90%
~10% of remaining cases

29. Endolymphatic sac surgeryEndolymphatic sac surgery
 Mechanical decompression of the endolymphatic sacMechanical decompression of the endolymphatic sac
 EffectiveEffective vertigo controlvertigo control as an outpatient procedure with little morbidityas an outpatient procedure with little morbidity
 Drainage of endolymph from the sac to the mastoid cavity through a plasticDrainage of endolymph from the sac to the mastoid cavity through a plastic
shunt/prosthesis (Portman, ’27)shunt/prosthesis (Portman, ’27)
 Fick procedure, Cody tack, cryosurgery, USG, otic-periotic shuntFick procedure, Cody tack, cryosurgery, USG, otic-periotic shunt
 Improves the function of the sac, induction of temporary subclinical labyrinthitisImproves the function of the sac, induction of temporary subclinical labyrinthitis
 Not aimed at improvement of hearingNot aimed at improvement of hearing

30. Vestibular nerve sectionVestibular nerve section
 The erstwhile “gold standard” surgery for control of intractable vertigo (not imbalance)The erstwhile “gold standard” surgery for control of intractable vertigo (not imbalance)
 Selective de-afferentation of the irritable vestibular function, with preservation of theSelective de-afferentation of the irritable vestibular function, with preservation of the
cochlear nervecochlear nerve
 Vestibular nerve isVestibular nerve is sectionedsectioned proximal to the geniculate ganglion, andproximal to the geniculate ganglion, and a portion of itsa portion of its
length removedlength removed
 Retrolabyrinthine, retrosigmoidRetrolabyrinthine, retrosigmoid
 Done unilaterally, with preserved (serviceable) hearingDone unilaterally, with preserved (serviceable) hearing
 Favored optionFavored option only whenonly when
unilateral diseaseunilateral disease
preserved ability of vestibular compensationpreserved ability of vestibular compensation
no central causes attributable to the vertigono central causes attributable to the vertigo
no other CNS disorders (CVA, MS, degenerative disorders)no other CNS disorders (CVA, MS, degenerative disorders)

32. Vestibular nerve section: complicationsVestibular nerve section: complications
 Chronic imbalanceChronic imbalance
 Chances of sensorineural hearing loss (~15%)Chances of sensorineural hearing loss (~15%)
 CSF leakCSF leak
 MeningitisMeningitis
 Facial nerve injuryFacial nerve injury
 Higher costHigher cost

33. LabyrinthectomyLabyrinthectomy
 Destruction of the vestibular portion of the inner ear sacrificing the cochlear functionsDestruction of the vestibular portion of the inner ear sacrificing the cochlear functions
 Done in casesDone in cases
with no serviceable hearing (stage 4)with no serviceable hearing (stage 4)
failure of all other treatment optionsfailure of all other treatment options
elderlyelderly
 Results in total hearing lossResults in total hearing loss
 Chemical labyrinthectomyChemical labyrinthectomy
intratympanic perfusion ofintratympanic perfusion of high dosehigh dose aminoglycosides (e.g., 120 mg/ml streptomycin)aminoglycosides (e.g., 120 mg/ml streptomycin)
 Surgical labyrinthectomy (with instillation of aminoglycoside)Surgical labyrinthectomy (with instillation of aminoglycoside)

34. Outcomes of different therapiesOutcomes of different therapies
Procedures Success rate Placebo
Vestibular nerve
section
~ 95%
~ 55%Intratympanic
gentamicin
perfusion
70-90%
ESS ~ 80-90%
Destructive
procedures
> 90% -

35.  Clinical diagnosisClinical diagnosis
 Diagnosis of exclusionDiagnosis of exclusion
 Essentially a vestibulocochlear diseaseEssentially a vestibulocochlear disease
 A disease in process of evolutionA disease in process of evolution
 Vestibular irritabilityVestibular irritability →→ vestibular suppression: a gradual “burningvestibular suppression: a gradual “burning
out” processout” process

36. Thank you