Management of MINIERE’S DISEASE power point

MANAGEMENT OF MÉNIÈRE’S DISEASE
PART 1: INTRODUCTION + PATHOPHYSIOLOGY (Academic)
Ménière’s disease (MD) is a chronic, idiopathic inner ear disorder characterized by recurrent spontaneous episodic vertigo, fluctuating sensorineural hearing loss (classically low frequency early in disease), tinnitus, and aural fullness. It is a disorder with significant socio-behavioural morbidity because the attacks of vertigo are disabling and unpredictable, impacting employment, driving, quality of life, social functioning and psychological health. It is one of the most clinically and pathologically extensively studied vestibular disorders in Otorhinolaryngology, yet its exact etiology remains unresolved. MD primarily represents a disorder due to dysregulated homeostasis of endolymph volume, classically termed endolymphatic hydrops. The condition was first described by Prosper Ménière in 1861, and remains a cornerstone disease to understand for ENT postgraduates particularly due to its chronicity, multi-disciplinary management requirement, and long-term follow-up complexity.
The prevalence of Ménière’s disease is estimated between 190–513 per 100,000 population globally, though significant regional variability exists, with Japan and Northern Europe reporting higher prevalence. In India, the exact epidemiologic data remains poorly documented due to underdiagnosis and referral pattern bias, but tertiary centres frequently manage these cases and nearly every ENT surgeon in practice will encounter MD. MD is most common in individuals between 30–60 years, though it may occur in all age groups.
Vertigo in MD is episodic, spontaneous and lasts typically 20 minutes to several hours, often associated with nausea and vomiting, and post-episode imbalance and exhaustion. The hearing loss in MD is fluctuating initially, which is a classical diagnostic clue (especially in differential diagnosis against vestibular migraine). Over progression, the loss becomes permanent and progressive. Tinnitus is usually low pitched, roaring type. Aural fullness precedes episodes and is among the earliest symptoms noticed. The disease is unilateral initially in most patients but up to one-third may develop bilateral involvement eventually.

Pathophysiology
The established pathological correlate is endolymphatic hydrops — abnormal distension of the endolymphatic compartment of the membranous labyrinth. Histopathologically, endolymphatic hydrops is characterized by expansion of the scala media and displacement / distension of Reissner’s membrane. The hydrops causes mechanical and ionic dysfunction within the cochlea and vestibular organs, resulting in fluctuating hearing loss and episodic vestibular crisis events. However, hydrops in itself is no longer considered the cause universally — it is considered the final common pathway. Many upstream etiologic mechanisms may feed into the development of hydrops.

Proposed etiological theories include:
Impaired endolymphatic sac physiology
Abnormal Ee

1. MANAGEMENT OF MINIERE’S DISEASE PRESENTER : Dr Kaveri BP MODERATOR: Dr Sana

2. What is MINIERE’S DISEASE ? 1861 - Prosper Miniere 1871- Knapp - Aural glaucoma 1938 - Hallpike and Cairns - endolymphatic hydrops 1902 - Parry performed a CN VIII division 1926 - Portman - endolymphatic sac decompression via a transmastoid approach 1931- McKenzie - selective vestibular neurectomy.

3. DEFINITION ? INCIDENCE ? Female to Male Peak age of Onset Incidence of bilateral disease Familial Occurance - 20% - AD - Specific MHC - HLA B8/DR3

4. Review Of Anatomy Functions of Endolymphatic Sac ?

5. Pathology : Etiology ?

6. Postulated Theories Vasomotor Disturbance Allergy Sodium and Water retention Hypothyroidism Autoimmune - immune complex deposition Viral Etiology - herpes simples virus types I and II, Epstein-Barr virus and CMV Anatomical-abnormalities Vascular-associated with migraines Metabolic-potassium intoxication

7. TYPES Classical Meniere's disease Vestibular Meniere's disease - vestibular symptoms and aural pressure Cochlear Meniere's disease - cochlear symptoms and aural pressure Lermoyez syndrome Tumarkin's crisis

8. Clinical Presentation Recurring attacks of vertigo (96.2%) Tinnitus (91.1%) - Non pulsatile - Whistling or Roaring type - Continuous or Intermittent Ipsilateral hearing loss (87.7%) - Sensorineural hearing loss is fluctuating and progressive, with aural fullness. Attacks often are preceded by an aura -sense of fullness in the ear, increasing tinnitus, and a decrease in hearing.

9. Investigations : Tuning Fork Tests - Rinne’s- AC>BC Weber’s - Lateralises to the better ear Rhombergs - Shows significant instability and worsening when the eyes are closed. Pure tone Audiometry -

10. Loudness Recruitment Electrocochleography

11. VEMP Dehydration Tests Glycerol - 1.5mg/kg - Serum Osmolality increase by 10mos/kg ; PTA after 2-3 hours Mannitol Frusemide Isosorbide

12. LATEST CRITERIA FOR THE DIAGNOSIS OF MINIERE’S DISEASE

13. TREATMENT Salt wasting diuretic such as diazide (1 month trial) Betahistine (2 week trial, often combined with verapamil) Relaxes the precapillary sphincters and thus improves the microcirculation of inner ear Antivertigo action due to imhibition of massive impulses to the polysynaptic lateral vestibular nucleus) Lipoflavins and vitamins ( hypothetical importance)

14. TREATMENT Dietary Modification And Diuretics: Salt Restriction and Diuresis Carbonic anhydrase inhibitors - Acetazolamide - Localization of carbonic anhydrase in the dark cells and the stria vascularis Salt wasting diuretic such as diazide (1 month trial) Betahistine (2 week trial, often combined with verapamil) Lipoflavins and vitamins ( hypothetical importance)

15. TREATMENT Local Over Pressure Therapy - Minnet Device Low Pressure pulses - 20cm of water over 5 minutes Restoring balance in the hydrodynamic system of the inner ear Randomised Control Trial Indications - Classic unilateral Meniere's disease Intense vestibular / cochlear symptoms Failed medical therapy Over 65 years of age Imbalance / aural fullness / tinnitus after gentamycin treatment

16. SURGICAL TREATMENT Intratympanic Injection : Dexamethasone : Gentamicin : In which Patients? Evidence from randomized trial? How Often? What Concentrations?- varied from 2 to 24 mg/mL. Injection of 0.5ml of hyaluronidase & 1 ml of 16mg dexamethasone mixed together - injected in ME. Patient instruction? Vestibulotoxic How to administer? 0.7ml of gentamicin injection mixed with 0.3ml of sodium bicarbonate solution Three injections are given per day in outpatient setting Injections are given for 4 days After injection patient should lie supine with the infiltrated ear up for 30

17. Surgical Treatment Sacculotomy Proposed by Fick in 1964, and consisted of using a needle to puncture the saccule through the stapes footplate. Long-term follow-up of patients so treated has shown an unacceptable degree of hearing loss

18. Surgical Treatment Cochleosacculotomy Fracture dislocation of the osseous spiral lamina (and hence a permanent fistulization of the endolymph-containing cochlear duct)

19. Surgical Treatment Endolymphatic Sac Surgery

20. Steps of Surgery :

21. Steps of Surgery :

22. Endolymphatic Shunting Paparella and Hansen Technique

23. Endolymphatic Subarachnoid Shunt

24. Vestibular Neurectomy RetroSigmoid Approach Steps Of Surgery:

25. Vestibular Neurectomy RetroSigmoid Approach Steps Of Surgery:

26. Vestibular Neurectomy RetroSigmoid Approach Steps Of Surgery:

27. Vestibular Neurectomy Middle Fossa Approach Steps Of Surgery:

28. Vestibular Neurectomy Middle Fossa Approach Steps Of Surgery:

29. Vestibular Neurectomy Middle Fossa Approach Steps Of Surgery:

30. Vestibular Neurectomy Middle Fossa Approach Steps Of Surgery:

31. Vestibular Neurectomy Middle Fossa Approach Steps Of Surgery:

32. Labyrinthectomy Most destructive procedure Who is an Ideal Candidate? Approaches -  Transcanal approach Transmastoid approach - much better exposure and is more popular.

33. Transcanal Labyrinthectomy Limitation - Poor exposure - Therefore complete ablation may not be achieved Technically difficult

34. Transmastoid Labyrinthectomy

35. References Thank You ☺️ Scott Brown’s Otorhinolaryngology and Head and Neck Surgery - 8th Edition Glasscock-Shambaugh surgery of ear 6th edition The Temporal Bone A Manual for Dissection and Surgical Approaches Mario Sanna, M.D.