Meniere’s Disease Mr Philip Clamp – Consultant in ENT Surgery. University Hospitals Bristol

• Unclear pathophysiology. Associated with endolymphatic hydrops. 1 • Defined by 2015 clinical criteria . Episodic , loss and /pressure. • Differential diagnosis is vestibular migraine (20 x more prevalent, may be co-existent). • Management of acute attacks involves vestibular sedative medication. • Disease modifying treatment includes dietary modification, betahistine, thiazide diuretics, intratympanic steroid injection and endolymphatic sac surgery. • Ablative treatment includes intratympanic gentamicin injection, labyrinthectoy and vestibular nerve section.

• The evidence base for treatment is generally poor, with a lack of placebo/control data.

Background Meniere’s disease (MD) is an idiopathic condition characterised by episodes of vertigo, and tinnitus or pressure. The aetiology is unknown and multiple theories have been proposed; abnormal fluid regulation, channelopathies, immune mediated, allergy/inflammation, vascular , genetics and environmental factors.

Diagnosis The condition is defined by 2015 clinical criteria1: • “Definite” o 2+ attacks of vertigo 20 min to 12 hours o SNHL >30 dB, 2 contiguous freq below 2KHz, during/after attack o Fluctuating aural symptoms (hearing loss, tinnitus or fullness) o No other cause identified

• “Probable” o 2+ attacks of vertigo or dizziness 20 min to 24 hours o Fluctuating aural symptoms (hearing loss, tinnitus, fullness) o No other cause identified Tests: • Pure tone audiogram: essential in order to diagnose associated low frequency hearing loss. • MRI: may be useful (especially in refractory cases or before ablative treatment) to rule out unusually such as acoustic neuroma/CPA tumours and endolymphatic sac tumour. • Vestibular tests: lack overall sensitivity and specific for MD, but may help determine if the pathology is vestibular or not. Considered mandatory prior to ablative treatment to ensure contralateral is functioning.

Major differential diagnosis is vestibular migraine. This is 20 times more prevalent and may be co-existent with MD.

Treatment Acute attacks are managed with oral or buccal prochlorperazine (Stemetil™, Buccastem™) Vestibular rehabilitation / physiotherapy may aid compensation from chronic loss of vestibular function2 Audiological support (hearing therapy, hearing aids, cochlear implants) may help hearing function

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Long term management involves an attempt to reduce frequency and severity of attacks3

• Dietary modification (low salt, low caffeine, low alcohol) o Lacks high quality evidence but usually recommended due to minimal risk4,5. • Betahistine o Lacks high quality data. Recent RCT (with placebo arm) shows no benefit6,7. • Thiazide diuretics o Lacks high quality data. Historic RCTs show possible benefit8,9. • Intratympanic steroid injections o Recent RCT show favourable results; equivalent to intratympanic gentamicin but without risk of hearing loss10. • Intratympanic gentamicin injections o Supported by evidence with good control of vertigo10,11. o Some risk of hearing loss (up to 25% in some studies but much less in others) • Endolymphatic sac decompression o Preserves hearing. High control rate (80%). o Unclear from studies if this is placebo effect12. • Labyrinthectomy or vestibular nerve section o Usually reserved for highly refractory cases. Good control rate (90%+) o Labyrithectomy sacrifices hearing. o Vestibular nerve section involves intra-dural approach with small risk to hearing and facial nerve o No controlled studies12 Other considerations Meniere’s disease appears to have a high placebo / no treatment control rate13. The condition usually “burns out” after many years. This may result in drop attacks (Tumarkin otolithic crisis). e-lefENT / additional reading http://www.e-lefent.org.uk/the-learning-zone/node/3382

References 1. Lopez-Escamez JA, Carey J, Chung WH, et al. Diagnostic criteria for Meniere's disease. J Vestib Res. 2015;25(1):1-7. 2. McDonnell MN, Hillier SL. Vestibular rehabilitation for unilateral peripheral vestibular dysfunction. Cochrane Database Syst Rev. 2015;1:CD005397. 3. Nevoux J, Barbara M, Dornhoffer J, Gibson W, Kitahara T, Darrouzet V. International consensus (ICON) on treatment of Meniere's disease. Eur Ann Otorhinolaryngol Head Neck Dis. 2018;135(1S):S29-S32. 4. Hussain K, Murdin L, Schilder AG. Restriction of salt, caffeine and alcohol intake for the treatment of Meniere's disease or syndrome. Cochrane Database Syst Rev. 2018;12:CD012173. 5. Luxford E, Berliner KI, Lee J, Luxford WM. Dietary modification as adjunct treatment in Meniere's disease: patient willingness and ability to comply. Otol Neurotol. 2013;34(8):1438-1443. 6. Adrion C, Fischer CS, Wagner J, et al. Efficacy and safety of betahistine treatment in patients with Meniere's disease: primary results of a long term, multicentre, double blind, randomised, placebo controlled, dose defining trial (BEMED trial). BMJ. 2016;352:h6816. 7. James AL, Burton MJ. Betahistine for Meniere's disease or syndrome. Cochrane Database Syst Rev. 2001(1):CD001873. 8. Crowson MG, Patki A, Tucci DL. A Systematic Review of Diuretics in the Medical Management of Meniere's Disease. Otolaryngol Head Neck Surg. 2016;154(5):824-834. 9. Thirlwall AS, Kundu S. Diuretics for Meniere's disease or syndrome. Cochrane Database Syst Rev. 2006(3):CD003599. 10. Patel M, Agarwal K, Arshad Q, et al. Intratympanic methylprednisolone versus gentamicin in patients with unilateral Meniere's disease: a randomised, double-blind, comparative effectiveness trial. Lancet. 2016;388(10061):2753-2762. 11. Pullens B, van Benthem PP. Intratympanic gentamicin for Meniere's disease or syndrome. Cochrane Database Syst Rev. 2011(3):CD008234. 12. Pullens B, Verschuur HP, van Benthem PP. Surgery for Meniere's disease. Cochrane Database Syst Rev. 2013(2):CD005395. 13. Hamill TA. Evaluating treatments for Meniere's disease: controversies surrounding placebo control. J Am Acad Audiol. 2006;17(1):27-37.

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