• Clinical science

Ménière disease

Summary

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

Epidemiology

References:[1]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

References:[2][3]

Pathophysiology

All patients with Ménière disease have impaired endolymph resorption, the cause of which is not fully understood. However, not all patients with endolymphatic hydrops have symptoms of Ménière disease. There are currently two theories about why some patients develop symptoms:

  • Rupture theory: Fluid accumulation in the endolymphatic sac can cause a tear in the Reissner's membrane, which usually separates the endolymph (rich in potassium) from the perilymph (rich in sodium). The resulting increase in perilymphatic potassium levels depolarizes the afferent acoustic nerve fibers, causing the characteristic symptoms.
  • Compression theory: Impaired endolymph resorption leads to compression of the semicircular canal, which is crucial for balance and hearing.

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

Clinical features

Acute, unilateral symptoms that last from minutes to hours. The frequency of these episodes decreases with increasing 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)
  • Ear fullness
  • Often notable for a horizontal nystagmus

Central vs peripheral vertigo

Central Vertigo (CV)

Peripheral Vertigo (PV)

References:[4][5][6]

Diagnostics

References:[2][3]

Differential diagnoses

See differential diagnoses for vertigo.

The differential diagnoses listed here are not exhaustive.

Treatment

  • Conservative
    • Lifestyle adjustments
      • Avoid dietary and environmental triggers (caffeine, alcohol, and stress)
      • Low-sodium diet
    • Bed rest
    • Medical therapy
  • Interventional
    • Intratympanic gentamicin injection: repeated application of gentamicin to the middle ear cavity through a small incision in the eardrum (paracentesis)
    • Sacculotomy: The endolymphatic sac and duct (part of the vestibular organ) is surgically exposed in order to promote drainage of endolymph.
    • Vestibular neurectomy: Surgical lysis of the vestibular bundle entering the internal auditory canal. In some cases, the procedure is associated with hearing loss.

References:[2][7]

  • 1. Minor LB, Schessel DA, Carey JP. Ménière's disease. Curr Opin Neurol. 2004; 17(1): pp. 9–16. pmid: 15090872.
  • 2. 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.
  • 3. 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.
  • 4. 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.
  • 5. Karatas M. Central vertigo and dizziness: epidemiology, differential diagnosis, and common causes. Neurologist. 2008; 14(6): pp. 355–364. doi: 10.1097/NRL.0b013e31817533a3.
  • 6. Dieterich M. Central vestibular disorders. J Neurol. 2007; 254(5): pp. 559–568. doi: 10.1007/s00415-006-0340-7.
  • 7. Coelho DH, Lalwani AK. Medical management of Ménière's disease. Laryngoscope. 2008; 118(6): pp. 1099–1108. doi: 10.1097/mlg.0b013e31816927f0.
  • Tao Le, Vikas Bhushan, Deol M, Reyes G. First Aid for the USMLE Step 2 CK, Tenth Edition. New York: McGraw-Hill Education; 2018.
  • 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.
  • 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 12/04/2019
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