Meniere’s Disease

1. Meniere’s Disease Dr.azimi

2. definition spontaneous,episodicattacks of vertigo; sensorineural hearing loss which usually fluctuates; tinnitus; and often a sensation of aural fullnaess

3. Epidemiology • 17 /100,000 Japan …… 513/100,000 Finland • More in Whites • M = F • Peak : 4th -5th decade • 19 – 24% bilateral • 10-20 % familial (HLA B8/DR3)

4. Current epidemiology of Meniere'ssyndrom2010 USA • 3.5 in 100,000 to 513 in 100,000 • F /m : 1.89 /1 • Prevalence of meniere’s syndrome increase with age

6. Dilated membranous labyrinth in Meniere's disease (Hydrops) Normal membranous labyrinth

7. Endolymphatichydrops • Meniere’s disease • Trauma • acute otitis media • labyrinthitis, • congenital inner ear deformity • idiopathic processes

8. pathophisiology • Ruptures in the membranous labyrinth • leakage of the potassium-rich endolymphinto the perilymph • bathing the eighth cranial nerve and lateral sides of the hair cells • depolarize the nerve cells, causing their acute inactivation • typical Meniere'sattack • Healing of the membranes • chronic deterioration in inner ear function (repeated exposure to the effects of the potassium)

9. Pathophysiology

10. terminology • Triad : vertigo + HL + Tinitus • Triad + known etiology : meniere’s syndrome • Triad + unknown etiology : meniere’s Disease • Triad + known hydrops etiology : secondary meniere’s

11. Etiology • Immonologic (Abs,HLA,chronic and fluctuating) • Viral (delayed endolyphativhydrops after deafness) • Ischemy of inner ear or endolymphatic sac (linkage of meniere’s & migrain) • Other related processes (trauma, acute otitis media, labyrinthitis, congenital inner ear deformity, idiopathic processes)

12. AA0-HNS criteria

14. Clinical presentation • Vertigo : 96.2% • Tinitus : 91.1 % • Unilateral HL : 87.7% • Sometimes aura : (increased tinitus,HL,aural fullness) • Attacks longer than one day : no meiere!!??

15. Vertigo • Most distressing complaint • Spinning vertigo with horizontal axis • Exacerbated with head motion • nausea, vomiting. diarrhea, and sweating • Between attacks: asymptomatic, periods of dysequilibrium ,lightheadedness, and tilt • Horizontal nystagmus(not useful in determining the involved ear)

16. otolithic crisesofTumarkin • Suddenunexplained falls without loss of consciousness or associated vertigo • 2% to 6% of persons with Meniere's disease • The spells are brief with little associated vertigo • tend ro occur in clusters and then spontaneously remit • abrupt change in otolithic input generates an erroneous vertical gravity reference

17. Hearing Loss • SNHLfluctuating and progressive • sensation of fullness or pressure in the ear • low-frequencyfluctuating loss and a coincident nonchanging, high-frequency loss • "peaked" or "tent-like“ at 2 kHz • profound deafness in 1-2 % • Diplacusis (43.6%) • Recruitment (56%)

18. Hearing assessment in Menière's disease2011 spain • 273 ps. • Follow up 1-30 years. • If surgery was done,data of before the surgery was accessed • Unilateral : low frequency,even in very advanced stage. was accentuated at 5 and 15 years from onset • Bilateral : slightly more severe. a flatter audiometric curve than in unilateral cases • The audiometric curve configuration may be an indicator of future bilateral disease.

19. tinitus • Nonpulsatile • Whistling or roaring • continuous or intermittent • Tinnitus often: • begins, gets louder, or changes pitch as an attackapproaches

20. Clinical characteristics of tinnitus in Ménière'sdiseas2011 • 88 pswere analysed • Avaragetime of the disease: 15.4 • 46% of moderate intensity ( 5/10 analogue-visual scale) & low frequency • unfavourable prognostic factors for decrease in quality of life : history of depression & young age. • patients do not perceive tinnitus as a problem that produces serious impairment in their quality of life

21. Electronystagmography • recordings of eye movements after caloric and rotational stimulation • often can localize the involved ear. • significant caloric response reduction: 48-73.5% • Complete absence of caloric response : 6-11%

22. Electrocochleography • SP : is larger and more negative • increase in SP/AP : reduce the intertest variability, a more linear response • Increase in SP/AP : 62% of meniere & 21% in normal controls

23. Dehydrating Agents • urea, glycerol, furosemide • Improvement has been measured with audiometrics, reduction in SP negativity (as recorded with electrocochleography), or a change in the gain of the vestibula-ocular response to rotational stimulation • Sensivity : variable . 60%

24. Vestibular Evoked Myopotentials • Click stapes movement sacculestimulation vestibular nocleus SCM relaxation • Saccule :2nd most common affected site in hydrops • Affected ear: elevated VEMP thresholds with flattened tuning

25. Treatment

26. Dietary Modification and Diuretics • Salt restriction + diuretics(best initial therapy) • Reduce endolymph volume • Not confirmed • Acetazolamide: • localization of carbonic anhydraseto the dark cells and the striavascularis • Not proved

27. Diuretics in Meniere disease: A therapy or a potential cause of harm?2011 italy • A common treatment • to decrease volume and pressure in the endolymphatic partition of the labyrinth • possibility of an adverse effect: • Abrupt lowerng of BP …exaggerated vasomotor response …. local ischemia….permanent damage. • terminal vascular supply…hemodynamic imblaenc: loss of perfusion

28. vasodilators • At the basis of sterial ischemia • (2010) exact mechanism in unclear • Betahistine: • Oral histamine preparation • Proved to be effective in vertigo • (cochrane 2010) although individual trials had positive results,largrandomised trials is needed (2010) No effect on hearing loss

29. Symptomatic treatment • Anti vertigo • Anti nausea • Sedatives • Antidepressants • Psychiatric treatment

30. Local Overpressure Therapy • Increase in middle ear pressure: relief of meniere’s symptoms • Meniett device: • FDA approved • First; VT insertion • Pressure : 20 cm H2O for 5 min periods • Effective in first 3 months

32. Long-term effects of the Meniett device in Japanese patients with Meniere's disease and delayed endolymphatichydropsreported by the Middle Ear Pressure Treatment Research Group of Japan.2011 • 29 ears of 28 meniereps& 5 ears of 5 ps with DEH • All ,failed medical treatment • 57% of MD & 100% of DEH remained entirely free from vertigo spells • 32% of MD : significant decrease in the frequency of vertigo spells • 25 ears : stable hearing levels • 4 ears : significant hearing improvement. • No complications

33. Intratympanic Injection • Gentamycin: • Vestibulotoxicity more than cochleotoxicity • Elimination of vertigo: 90% ,83% • SNHL : 10%, 3% • Single dose is better (booster dose if needed) • The risk of hearing loss with gentamicin using many current protocols is similar to that with the natural history of Meniere's disease

34. Intratympanicgentamicin in Ménière's disease: our experience.2011 spain • Longitudinal, prospective, descriptive • Completevertigo control :65.6% • complete or substantial control :84.37% • 13 ps (18.3%) suffered significant hearing loss. • a good alternative to more aggressive techniques for the treatment of Ménière's disease which does not respond to medical treatment.

35. Intratympanic Injection • Dexametasone: • Control of vertigo without effect on HL or tinitus • Intarctable vertigo but functional hearing • Success : 83% • Repeated dose every 3 month • Dose : 2-24 mg/ml

36. Intratympanic steroids for Ménière's disease or syndrome.2011 UK • Clinical trial • A single trial containing 22 patients, with a low risk of bias was included. • daily injections of dexamethasone solution 4 mg/ml for five consecutive days • After 24 m. compared placebo with intratympanicdexamethasone • improvement in vertigo (90% vs 42%) • mean vertigo subjective improvement (90% vs 57%) • clinically significant. No complications . • statistically and clinically significant improvement of the frequency and severity of vertigo

37. Endolymphatic Sac Surgery • Simple decompression, • wide decompression that includes the sigmoid sinus, • cannulatingthe endolymphatic duct • endolymphaticdrainage to the subarchnoid space, • drainage to the mastoid • removal of the extraosseousportion of the sac

38. Nerve Section • Approaches: retrosygmoid,suboccipital,middlefossa,retrolabyrinthine • 85-95% relief of vertigo • 80-90% hearing perservation • HL less than gentamycin injection

39. 10-year review of endolymphatic sac surgery for intractable meniere disease2011 London • 1998 – 2007 • 33 ears. • Mean age :49 m/f : 63%-37% • Successfulvertigo control :64.5% (class a-b) • Hearing : • improved 14.8%,the same 51.9%,worsend 33.3% • 3p. (10%) had profound SNHL • 80% of ps,had improved quality of life scales

40. Labyrinthectomy • Nofunctional hearing with resistant vertigo • Best control of vertigo • But should be the last choice

41. Meniere's disease and the use of proton pump inhibitors2010 italy • 2001 – 2006 • 42 ps • 18 patients had used PPI for various reasons for at least 12 consecutive months, whilst 24 patients had never been prescribed them • f/u 29 month • Most of MD patients (72%) using PPI suffered less than one episode of menieric crisis/year. On the other hand patients who had never used a PPI, experienced considerably more episodes only 16.7% having less than one crisis per year. This difference was statistically significant (p<0, 001). • strongly suggest a possible role for proton pumps in the pathogenesis of MD