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Meniere disease

Last updated: May 3, 2021

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Meniere disease (endolymph hydrops) is a disorder of the inner ear caused by impaired endolymph resorption. The exact etiology of endolymph malabsorption is unknown but viral infections, autoimmunity, and allergies are thought to play a role. Meniere disease most commonly manifests in adults between 40–50 years of age. Clinical manifestations include recurrent episodes of peripheral vertigo, fluctuating unilateral sensorineural hearing loss (SNHL), and unilateral tinnitus (referred to as the Meniere triad); horizontal nystagmus or horizontal rotatory nystagmus may also be present. The episodes fluctuate in severity, typically lasting from 20 minutes to 12 hours. The diagnosis is based on characteristic clinical features and low to mid-frequency SNHL on audiometry. Treatment is symptomatic. Vestibular suppressants (e.g., benzodiazepines and first-generation antihistamines) may be used during acute vertigo attacks. Lifestyle modifications (e.g., avoidance of allergens, low-sodium diet) and vestibular rehabilitation therapy can help minimize the risk of recurrence. Diuretics should be considered in patients with frequent attacks. Interventional therapy (e.g., chemical vestibular ablation with intratympanic gentamicin, intratympanic steroids) or surgical vestibular ablation (e.g., labyrinthectomy, vestibular neurectomy) is reserved for patients with intractable symptoms that significantly impact their quality of life.

  • Sex: [1]
  • Onset: 20–60 years of age
  • Peak incidence: 40–50 years
  • Prevalence: : 50–200 in 100,000 individuals in the US

Epidemiological data refers to the US, unless otherwise specified.

  • Idiopathic
  • Several etiologies have been proposed, including:
    • Viral infections [2]
    • Autoimmune disease
      • Inner ear autoimmune disease
      • Systemic autoimmune disease [2]
    • Allergies [1]

All patients with Meniere disease have impaired endolymph resorption that results in endolymph hydrops; however, not all patients with endolymphatic hydrops have symptoms of Meniere 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 Meniere disease, the concentration of potassium in the perilymph increases.

Meniere disease characteristically manifests as recurrent episodes of acute, unilateral symptoms that last from minutes to hours. [1]

  • Meniere triad [1]
  • Additional symptoms: may be present in some patients
    • Nausea and vomiting
    • Ear fullness
    • Spontaneous horizontal or horizontal rotatory nystagmus [4][5][6]
      • Seen in some patients during an acute episode of Meniere disease
      • The direction of nystagmus is variable [5]
  • Triggers: Definitive triggers of Meniere disease are not known to exist [7]
  • Progression
    • Episodes fluctuate in severity and typically last from 20 minutes to 12 hours
    • Periods of remission between attacks vary from months to years.
    • In 10–25% of patients, the disease becomes bilateral. [1]

  • Lermoyez syndrome [4]
  • Drop attacks (Tumarkin otolithic crisis) [4]
    • An uncommon feature that may occur in advanced­ Meniere disease, characterized by suddenly falling to the ground without warning
    • There is no loss of consciousness.
    • Falls may have severe to life-threatening adverse consequences (e.g., TBI, hip fractures).
    • Difficult to treat but may resolve spontaneously

Meniere disease is diagnosed based on the characteristic clinical features and demonstrable low- to mid-frequency SNHL on audiometry. Specialized tests (e.g., vestibular function testing, electrocochleography) are reserved for patients with atypical symptoms or before attempting ablative therapies. Neuroimaging should be considered if central vertigo is suspected. [1]

Diagnostic criteria for Meniere disease [1]

  • Definite Meniere disease: must include all of the following criteria
  • Probable Meniere disease: Patients that meet all of the above criteria but do not demonstrable hearing loss on audiometry.

Subjective audiometry [1]

Asymmetric fluctuating hearing loss is a characteristic feature of Meniere disease. Subjective audiometry may be normal at the time of testing because the attacks of Meniere disease are episodic. [1]

Additional evaluation

Vestibular function tests and electrophysiologic testing [1]

Not routinely recommended

  • Indications
    • Atypical symptoms
    • Identifying the affected ear
    • Before vestibular ablative procedures.
  • Modalities and supportive findings

Imaging [1][9]

Imaging studies are not routinely indicated in patients with suspected Meniere disease.

  • Indications
    • To rule out differential diagnosis in patients with atypical symptoms (e.g., sudden SNHL, nonfluctuating SNHL)
    • Before ablative therapies
  • Preferred modality: MRI internal auditory canal and posterior fossa (without and with IV contrast)
  • Supportive findings: endolymphatic space distention (endolymphatic hydrops) in the cochlea and vestibule [9][10]

Always consider vestibular migraine as a differential diagnosis of Meniere disease.

The differential diagnoses listed here are not exhaustive.

There is currently no definitive cure for Meniere disease. Treatment is directed toward symptomatic management and prevention of recurrence. Interventional therapy or surgery is reserved for patients with intractable symptoms that significantly hinder their quality of life. [1]

Acute therapy [1]

Vestibular suppressants are the treatment of choice for an acute vertigo attack in Meniere disease.

Chronic use of vestibular suppressants is contraindicated because of their potential to inhibit central compensation, which could elicit gait and postural instability.

Recurrence prevention [1]

  • Lifestyle modifications [1][14]
    • Stress reduction
    • Low-sodium diet (1500–2300 mg per day)
    • Identification and avoidance of dietary and environmental triggers (e.g., caffeine, alcohol, nicotine, and stress).
  • Vestibular rehabilitation and physical therapy
    • Indications: chronic imbalance , incomplete central vestibular compensation after ablative therapy
    • Options: vestibular habituation exercises (e.g., Cawthorne-Cooksey exercises) and physical exercises that increase stability and help with balance and walking
  • Other:
    • Allergen testing, avoidance, and treatment [1]
    • Patients should be educated about avoiding known triggers and the natural course of the disease, including recurrence and worsening of SNHL with each attack.

Maintenance therapy [1]

Patients with frequently recurring episodes of Meniere disease may be considered for chronic pharmacotherapy.

Interventional therapy [1]

Positive pressure pulse generator devices (e.g., Meniett device) are no longer recommended for Meniere disease.

Surgical intervention [1]

  • Labyrinthectomy (Hearing-sacrificing surgery): destruction and removal of the labyrinth through the mastoid
  • Vestibular neurectomy (Hearing preservation surgery): selective transection of the vestibular nerve within the middle cranial fossa via a craniotomy
  • Endolymph drainage procedures (e.g., sacculotomy , cochleosacculotomy , endolymph sac decompression ): No longer recommended as they are of doubtful clinical benefit [1][18]
  1. Basura GJ, Adams ME, Monfared A, et al. Clinical Practice Guideline: Ménière’s Disease. Otolaryngol Head Neck Surg. 2020; 162 (2_suppl): p.S1-S55. doi: 10.1177/0194599820909438 . | Open in Read by QxMD
  2. A. Greco, A. Gallo, M. Fusconi, C. Marinelli, G.F. Macri, M. de Vincentiis. Meniere's disease might be an autoimmune condition?. Autoimmun Rev. 2012; 11 (10): p.731-738. doi: 10.1016/j.autrev.2012.01.004 . | Open in Read by QxMD
  3. Wu V, Sykes EA, Beyea MM, Simpson MTW, Beyea JA. Approach to Ménière disease management.. Can Fam Physician. 2019; 65 (7): p.463-467.
  4. Nakashima T, Pyykkö I, Arroll MA, et al. Meniere's disease.. Nat. Rev. Dis. Primers. 2016; 2 : p.16028. doi: 10.1038/nrdp.2016.28 . | Open in Read by QxMD
  5. Martinez-Lopez M, Manrique-Huarte R, Perez-Fernandez N. A Puzzle of Vestibular Physiology in a Meniere’s Disease Acute Attack. Case Reports in Otolaryngology. 2015; 2015 : p.1-5. doi: 10.1155/2015/460757 . | Open in Read by QxMD
  6. Hirai C, Yamamoto Y, Takeda T, et al. Nystagmus at the Onset of Vertiginous Attack in Ménièreʼs Disease. Otol Neurotol. 2017; 38 (1): p.110-113. doi: 10.1097/mao.0000000000001255 . | Open in Read by QxMD
  7. Kirby SE, Yardley L. Physical and Psychological Triggers for Attacks in Ménière’s Disease: The Patient Perspective. Psychother Psychosom. 2012; 81 (6): p.396-398. doi: 10.1159/000337114 . | Open in Read by QxMD
  8. Güneri EA, Çakır A, Mutlu B. Validity and Reliability of the Diagnostic Tests for Ménière's Disease.. Turkish archives of otorhinolaryngology. 2016; 54 (3): p.124-130. doi: 10.5152/tao.2016.1697 . | Open in Read by QxMD
  9. Sharma A, Kirsch CFE, Aulino JM, et al. ACR Appropriateness Criteria® Hearing Loss and/or Vertigo. J Am Coll Radiol. 2018; 15 (11): p.S321-S331. doi: 10.1016/j.jacr.2018.09.020 . | Open in Read by QxMD
  10. Attyé A, Dumas G, Troprès I, et al. Recurrent peripheral vestibulopathy: Is MRI useful for the diagnosis of endolymphatic hydrops in clinical practice?. Eur Radiol. 2015; 25 (10): p.3043-9. doi: 10.1007/s00330-015-3712-5 . | Open in Read by QxMD
  11. Walls R, Hockberger R, Gausche-Hill M. Rosen's Emergency Medicine - Concepts and Clinical Practice. Elsevier Health Sciences ; 2013
  12. de Goffau MJ, Doelman JC, van Rijswijk JB. Unilateral sudden hearing loss due to otosyphilis.. Clinics and practice. 2011; 1 (4): p.e133. doi: 10.4081/cp.2011.e133 . | Open in Read by QxMD
  13. Klemm E, Wollina U. Otosyphilis: report on six cases.. J Eur Acad Dermatol Venereol. 2004; 18 (4): p.429-34. doi: 10.1111/j.1468-3083.2004.00939.x . | Open in Read by QxMD
  14. 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 . | Open in Read by QxMD
  15. Sepahdari AR, Vorasubin N, Ishiyama G, Ishiyama A. Endolymphatic Hydrops Reversal following Acetazolamide Therapy: Demonstration with Delayed Intravenous Contrast-Enhanced 3D-FLAIR MRI. AJNR Am J Neuroradiol. 2015; 37 (1): p.151-154. doi: 10.3174/ajnr.a4462 . | Open in Read by QxMD
  16. Murdin L, Hussain K, Schilder AG. Betahistine for symptoms of vertigo. Cochrane Database Syst Rev. 2016 . doi: 10.1002/14651858.cd010696.pub2 . | Open in Read by QxMD
  17. Adrion C, Fischer CS, Wagner J, Gürkov R, Mansmann U, Strupp M. 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 : p.h6816. doi: 10.1136/bmj.h6816 . | Open in Read by QxMD
  18. Alarcón AV, Hidalgo LO, Arévalo RJ, Diaz MP. Labyrinthectomy and Vestibular Neurectomy for Intractable Vertiginous Symptoms.. International archives of otorhinolaryngology. 2017; 21 (2): p.184-190. doi: 10.1055/s-0037-1599242 . | Open in Read by QxMD
  19. Flores García ML, Llata Segura C, Cisneros Lesser JC, Pane Pianese C. Endolymphatic Sac Surgery for Ménière's Disease - Current Opinion and Literature Review.. International archives of otorhinolaryngology. 2017; 21 (2): p.179-183. doi: 10.1055/s-0037-1599276 . | Open in Read by QxMD
  20. 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.
  21. 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.
  22. Karatas M. Central vertigo and dizziness: epidemiology, differential diagnosis, and common causes.. Neurologist. 2008; 14 (6): p.355-364. doi: 10.1097/NRL.0b013e31817533a3 . | Open in Read by QxMD
  23. Dieterich M. Central vestibular disorders. J Neurol. 2007; 254 (5): p.559-568. doi: 10.1007/s00415-006-0340-7 . | Open in Read by QxMD
  24. Minor LB, Schessel DA, Carey JP. Ménière's disease. Curr Opin Neurol. 2004; 17 (1): p.9-16.
  25. Coelho DH, Lalwani AK. Medical management of Ménière's disease. Laryngoscope. 2008; 118 (6): p.1099-1108. doi: 10.1097/mlg.0b013e31816927f0 . | Open in Read by QxMD
  26. 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): p.181-185. doi: 10.1016/s0194-5998(95)70102-8 . | Open in Read by QxMD
  27. Tao Le, Vikas Bhushan, Deol M, Reyes G. First Aid for the USMLE Step 2 CK, Tenth Edition. McGraw-Hill Education ; 2018
  28. 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): p.403-404. doi: 10.1177/0194599816628524 . | Open in Read by QxMD