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Meniere's disease; labyrinthine hydrops; endolymphatic hydrops

Etiology: 1) hydrops of the membranous labyrinth of the inner ear 2) vestibular & cochlear hair cell injury 3) allergic component in 1/3 of patients Epidemiology: - age of onset 1) usually 20-60 years of age 2) 5th decade of life common 3) may occur at any age - prevalence of 190 per 100,000 in North America [7] Pathology: - excess endolymphatic fluid pressure leading to inner ear dysfunction Clinical manifestations: 1) episodic vertigo a) intermittent attacks b) associated nausea & vomiting c) horizontal nystagmus during attacks c) duration: 15 minutes - 24 hours d) remissions: weeks - years 2) tinnitus a) low pitched b) continuous c) fluctuates in intensity - often worsens preceding an attack d) may be described as roaring [3] 3) hearing loss a) often persistent through remissions b) low-frequencies most affected c) may be unilateral & subjectively described as blocked ears [3] 4) diplacusis 5) recruitment of loudness a) abnormally rapid increase in loudness in affected ear b) loud noises may be painful 6) associated vagal symptoms a) abdominal pain b) bradycardia c) pallor d) sweating 7) generally unilateral a) bilateral involvement suggests allergic component b) Weber test lateralizes to unaffected ear c) patient may describe a blocker ear [3] 8) Rhine test: normal pattern Laboratory: 1) audiometry a) impedance audiometry - recruitment of loudness -> cochlear pathology b) audiogram a) low-frequency hearing loss b) may progress to loss of all tones 2) electronystagmography (ENG) - hypoactive caloric test 3) auditory brain stem response (ABR) - distinguishes cochlear from retrocochlear pathology (acoustic neuroma) 4) magnetic resonance imaging (MRI) 5) exclusion testing a) thyroid function tests b) serologic test for syphilis c) lipid panel Differential diagnosis: 1) allergic reaction 2) hyperthyroidism 3) hypothyroidism 4) viral labyrinthitis (duration of symptoms > 24 hours) 5) benign positional vertigo (duration of symptoms a few minutes, no hearing loss) 6) vestibular neuronitis (no hearing loss & duration of symptoms > 24 hours) 7) acuostic neuroma 8) hyperlipidemia 9) syphilitic vertigo 10) labyrinthine fistula 11) vestibular granuloma 12) temporal bone fracture 13) multiple sclerosis Management: 1) acute attacks a) first line agents - atropine: 0.2-0.4 mg IV - diazepam (Valium): 5 mg IV as needed - meclizine (Antivert): 25-100 mg qd - scopolamine patch (Transderm-Scop) b) alternative agents - dimenhydramine (Dramamine): 50 mg IV, IM q4-6h - promethazine (Phenergan): 25-50 mg IV, IM q4-6h - cyclizine (Marezine): 50 mg PO q4-6h - diphenhydramine (Benadryl): 25-50 mg IV 2) maintenance a) first line agents - meclizine 12.5-25 mg tid or qid - diazepam 1-2 mg bid, tid or qid - thiazide diuretics - hydrochlorothiazide (HCTZ): 25-50 mg qd - in conjunction with low salt diet b) alternative agents - promethazine (Phenergan): 25 mg PO q4-6h - diphenhydramine (Benadryl): 25 mg PO q4-6h 3) vestibular rehabilitation [2] 4) surgery - endolymphatic sac shunt - vestibular nerve section 5) diet: low salt (no proven value) 6) bedrest during acute attacks 7) patient education - avoid ototoxic agents - avoid loud noise - over time, vertigo improves but hearing loss worsens

Related

diplacusis hydrops (dropsy) Rinne test Weber test

General

chronic ear disease vestibular disorder (vestibulopathy)

Database Correlations

OMIM correlations MORBIDMAP 603196

References

  1. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 1071-72
  2. Medical Knowledge Self Assessment Program (MKSAP) 11, 16, 18. American College of Physicians, Philadelphia 1998, 2012, 2018. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. Geriatric Review Syllabus, 8th edition (GRS8) Durso SC and Sullivan GN (eds) American Geriatrics Society, 2013 - Geriatric Review Syllabus, 9th edition (GRS9) Medinal-Walpole A, Pacala JT, Porter JF (eds) American Geriatrics Society, 2016 - Geriatric Review Syllabus, 11th edition (GRS11) Harper GM, Lyons WL, Potter JF (eds) American Geriatrics Society, 2022
  4. Le CH, Truong AQ, Diaz RC. Novel techniques for the diagnosis of Maniare's disease. Curr Opin Otolaryngol Head Neck Surg. 2013 Oct;21(5):492-6. Review. PMID: 23995329
  5. Pierce NE, Antonelli PJ. Endolymphatic hydrops perspectives 2012. Curr Opin Otolaryngol Head Neck Surg. 2012 Oct;20(5):416-9. Review. PMID: 22902416
  6. Basura GJ, Adams ME, Monfared A et al Clinical Practice Guideline: Meniere's Disease. Otolaryngology - Head and Neck Surgery. April 8, 2020 PMID: 32267820 https://journals.sagepub.com/doi/full/10.1177/0194599820909438
  7. Rizk HG, Mehta NK, Qureshi U et al Pathogenesis and Etiology of Meniere Disease. A Scoping Review of a Century of Evidence. JAMA Otolaryngol Head Neck Surg. Published online February 10, 2022 PMID: 35142800 https://jamanetwork.com/journals/jamaotolaryngology/fullarticle/2788899
  8. Basura GJ, Adams ME, Monfared A, et al. Clinical Practice Guideline: Meniers's Disease. Otolaryngol Head Neck Surg. 2020;162(2_suppl):S1-S55 PMID: 32267799
  9. Hoskin JL. Meniere's disease: new guidelines, subtypes, imaging, and more. Curr Opin Neurol. 2022 Feb 1;35(1):90-97. PMID: 34864755 Review. https://journals.sagepub.com/doi/10.1177/0194599820909438
  10. Wu V, Sykes EA, Beyea MM, Simpson MTW, Beyea JA. Approach to Meniere disease management. Can Fam Physician. 2019 Jul;65(7):463-467. PMID: 31300426 PMCID: PMC6738466 Free PMC article. Review.
  11. The Basics: Meniere's Disease [NIDCD Health Information] http://www.nidcd.nih.gov/health/balance/thebasics_menieres.asp