• Clinical science

Ménière disease (Idiopathic endolymphatic hydrops)

Summary

Ménière disease is an idiopathic condition affecting the inner ear, in which impaired resorption of endolymphatic fluid causes it to accumulate in the membranous labyrinth (endolymphatic hydrops). The disease usually manifests episodically with a triad of symptoms: vertigo, hearing loss, and tinnitus. Episodes may last from minutes to hours and decrease in frequency as patients age. Hearing loss tends to worsen with every episode. Diagnostic tests show low-frequency hearing loss, typically with horizontal nystagmus. Acute episodes are treated with bed rest and vestibular suppressants, while long-term prevention therapy focuses on lifestyle changes such as adopting a low-sodium diet and reducing both alcohol consumption and stress. For patients who do not respond to these measures, interventional and/or surgical treatment may be necessary to shut down the vestibular organ.

Epidemiology

  • Sex: >
  • Onset: 20–60 years of age
  • Peak incidence: 40–60 years
  • Prevalence: : 70–90 in 100,000 individuals in the US

References:[1][2]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

  • Idiopathic

References:[3][4]

Pathophysiology

All patients with Ménière disease have impaired endolymph resorption that results in endolymph hydrops; however, not all patients with endolymphatic hydrops have symptoms of Ménière disease. The cause of impaired resorption is unknown. There are currently two main theories about why some patients develop symptoms:

  • Endolymph hydrops: accumulation of fluid in the endolymphatic sac.
  • Rupture theory: fluid accumulation in the endolymphatic sac → tear in the Reissner membrane → increased perilymphatic potassium → depolarization of the afferent acoustic nerve fibers → symptom onset
  • Compression theory: impaired endolymph resorption → compression of the semicircular canals → symptom onset

The endolymph is rich in potassium and perilymph is rich in sodium. In Ménière disease, the concentration of potassium in the perilymph increases!

Clinical features

  • General
    • Recurrent episodes of acute, unilateral symptoms that last from minutes to hours.
    • Frequency of these episodes decreases as patients age.
    • In 30% of patients, the disease eventually affects both sides.
  • Ménière triad
    • Peripheral vertigo
    • Tinnitus
    • Sensorineural hearing loss: Low-frequency loss increases with each episode that may lead to deafness after several years.
  • Additional symptoms

References:[5][6][7]

Diagnostics

References:[3][4]

Differential diagnoses

The differential diagnoses listed here are not exhaustive.

Treatment

References:[3][9][10]

  • 1. Minor LB, Schessel DA, Carey JP. Ménière's disease. Curr Opin Neurol. 2004; 17(1): pp. 9–16. pmid: 15090872.
  • 2. Tao Le, Vikas Bhushan, Deol M, Reyes G. First Aid for the USMLE Step 2 CK, Tenth Edition. New York: McGraw-Hill Education; 2018.
  • 3. Moskowitz HS, Dinces EA. Meniere disease. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. http://www.uptodate.com/contents/meniere-disease. Last updated March 22, 2016. Accessed April 3, 2017.
  • 4. Committee on Hearing and Equilibrium. Committee on Hearing and Equilibrium guidelines for the diagnosis and evaluation of therapy in Menière's disease. Otolaryngol Head Neck Surg. 1995; 113(3): pp. 181–185. doi: 10.1016/s0194-5998(95)70102-8.
  • 5. Furman JM. Pathophysiology, etiology, and differential diagnosis of vertigo. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. http://www.uptodate.com/contents/pathophysiology-etiology-and-differential-diagnosis-of-vertigo. Last updated June 3, 2015. Accessed April 3, 2017.
  • 6. Karatas M. Central vertigo and dizziness: epidemiology, differential diagnosis, and common causes. Neurologist. 2008; 14(6): pp. 355–364. doi: 10.1097/NRL.0b013e31817533a3.
  • 7. Dieterich M. Central vestibular disorders. J Neurol. 2007; 254(5): pp. 559–568. doi: 10.1007/s00415-006-0340-7.
  • 8. Goebel JA. 2015 Equilibrium Committee Amendment to the 1995 AAO-HNS Guidelines for the Definition of Ménière’s Disease. Otolaryngology–Head and Neck Surgery. 2016; 154(3): pp. 403–404. doi: 10.1177/0194599816628524.
  • 9. Coelho DH, Lalwani AK. Medical management of Ménière's disease. Laryngoscope. 2008; 118(6): pp. 1099–1108. doi: 10.1097/mlg.0b013e31816927f0.
  • 10. Kiran Hussain et al. Restriction of salt, caffeine and alcohol intake for the treatment of Ménière's disease or syndrome. Cochrane Database of Systematic Reviews. 2018. doi: 10.1002/14651858.cd012173.pub2.
last updated 02/27/2020
{{uncollapseSections(['jDa_V5', 'kDame5', 'ODaIe5', 'NDa-e5', 'mDaVU5', 'nDa7U5', 'oDa025', 'KDaU25'])}}