Ménière Disease

Ménière disease is a condition characterized by episodes of vertigo, tinnitus, and hearing loss, likely caused by endolymphatic hydrops of the labyrinthine system in the inner ear. The risk factors include a family history of Ménière disease, preexisting autoimmune disorders, allergies, and trauma to the head or ear. A diagnosis is made clinically, by audiometry, by vestibular testing, and occasionally by imaging. Management can be by diet and lifestyle modification, vasodilators, diuretics, antihistamines, benzodiazepines, antiemetics, glucocorticoids, surgical intervention, or hearing aids.

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Overview

Definition

Ménière disease is a triad of episodic vertigo, tinnitus, and hearing loss likely caused by endolymphatic hydrops of the labyrinthine system of the inner ear.

Ménière syndrome is Ménière disease occurring secondary to other inner ear infections.

Epidemiology

  • Occurs at any age
  • Symptoms begin from age 20 to 40. 
  • Incidence ranges from 10 to 150 per 100,000 persons.
  • Bilateral disease occurs in 10%–50% of patients.

Risk factors

  • Family history of Ménière disease
  • Preexisting autoimmune disorders
  • Congenital inner ear malformations
  • Allergies
  • Trauma to the head or ear
  • Syphilis

Pathophysiology

  • Endolymph and perilymph are separated by thin membranes that contain the neural apparatus of hearing and balance.
  • Pressure fluctuations cause stress on the nerve-rich membranes → hearing disturbances, tinnitus, vertigo, imbalance, and a pressure sensation in the inner ear
  • Increase in endolymphatic pressure → breaks in the membrane separating perilymph from endolymph
  • The resultant chemical mixture bathes the vestibular nerve receptors → depolarization blockade and transient loss of function
  • Sudden change in the rate of vestibular nerve firing creates an acute vestibular imbalance.
  • Increased endolymphatic pressure → physical distension → mechanical disruption of auditory and otolithic organs
  • The utricle and saccule are responsible for linear and translational motion detection.
  • Irritation produces nonrotational vestibular symptoms.
  • Also physical distension → mechanical disturbance of organ of Corti → distortion of the basilar membrane and the inner and outer hair cells → hearing loss and/or tinnitus 
  • The apex of the cochlea is wound up tighter than the base → apex more sensitive to pressure changes than the base
The internal ear

Anatomy of the inner ear

Image: “The internal ear” by BruceBlaus. License: CC BY 3.0

Clinical Presentation

  • Vertigo:
    • Perceived rotatory spinning or rocking motion while remaining still
    • Sometimes associated with nausea and vomiting lasting 20 minutes to 24 hours
  • Hearing loss: initially intermittent, then progresses to permanent hearing loss
  • Tinnitus:
    • Perceived noises (usually high-pitched ringing) that are not actually present
    • Fluctuating or constant with variable pitch and intensity

Diagnosis

Diagnostic criteria

Clinical diagnosis made by the presence of:

  • 2 or more spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours
  • Audiometrically documented low- to mid-frequency sensorineural hearing loss in the affected ear
  • Fluctuating aural symptoms (reduced or distorted hearing, tinnitus, or fullness) in the affected ear
  • Symptoms not better accounted for by another vestibular diagnosis

Testing

  • Audiometry
    • For all patients
    • Most common pattern:
      • Low-frequency sensorineural hearing loss 
      • Combined low- and high-frequency sensorineural hearing loss
      • Normal hearing in the middle frequencies
    • With time, hearing “flattens out” with hearing loss across all frequency ranges. 
  • Vestibular testing
    • May be normal early in the course of the disease
    • Abnormal as disease advances on the affected side
    • Include electronystagmography (ENG), rotary chair testing, and computerized dynamic posturography 
  • Imaging
    • MRI of the temporal bone: can identify supporting features (e.g., less prominent endolymphatic duct)
    • MRI/CT brain: CNS lesions

Management

Treatment improves and relieves symptoms but does not correct the underlying pathophysiology.

Goals of treatment

  • Reducing frequency and severity of vertigo attacks
  • Reducing or eliminating the tinnitus and hearing loss associated with attacks
  • Alleviating chronic symptoms of tinnitus and disequilibrium
  • Minimizing disability associated with Ménière disease
  • Preventing progressive hearing loss

Initial diet and lifestyle management

  • Salt restriction
  • Limiting caffeine and alcohol to 1 drink daily
    • Caffeine and nicotine are vasoconstrictors reducing microvascular flow in the labyrinthine system.
    • Alcohol causes fluid and electrolyte shifts, stressing a vulnerable ear.

Vestibular rehabilitation for residual disequilibrium between attacks

  • Used in:
    • Patients with response to surgical or medical treatment with residual disequilibrium
    • Patients with significant balance and disequilibrium symptoms between acute vertigo attacks
  • Does not reduce the frequency or severity of vertigo attacks

Pharmacotherapy for patients with refractory symptoms

  • Goal: Reduce the severity and intensity of attacks.
  • Chronic management:
    • Betahistine: vasodilator (preferred because it is well tolerated)
    • Hydrochlorothiazide: diuretic
    • Furosemide: diuretic
    • Acetazolamide: diuretic
  • Acute management:
    • Antihistamines: reduce swelling and improve blood flow to inner ear
      • Dimenhydrinate
      • Diphenhydramine
      • Meclizine
    • Benzodiazepines: vestibular suppression
      • Alprazolam
      • Clonazepam
      • Diazepam
      • Lorazepam
    • Antiemetics: suppress associated nausea
      • Domperidone
      • Metoclopramide
      • Ondansetron
      • Prochlorperazine
      • Promethazine
  • Refractory, disabling symptoms despite initial pharmacotherapy:
    • Continue betahistine or diuretics, if beneficial.
    • Glucocorticoid therapy for all patients
      • Systemic glucocorticoids: prednisone
      • Intratympanic glucocorticoids: dexamethasone
  • Refractory, disabling symptoms despite glucocorticoid therapy:
    • Destructive therapies: reduce or eliminate signals from the affected labyrinthine system 
      • Intratympanic gentamicin
      • Surgical labyrinthectomy
      • Vestibular neurectomy
    • Nondestructive procedures: reduce fluid accumulation or alter fluid and electrolyte physiology
      • Decompression and/or shunting of the endolymphatic sac
      • Sacculotomy
  • Hearing amplification for hearing loss:
    • Patients with significant binaural hearing loss
    • In early disease, hearing fluctuation leads to frustration with amplification devices and poor patient compliance.

Differential Diagnosis

  • Migraine (migrainous vertigo or vestibular migraine): condition associated with headache and sometimes vertigo. Migraines are usually accompanied by photophobia or phonophobia not seen in Ménière disease. For diagnosis, episodic vestibular symptoms and at least 2 migraine symptoms (migraine headache, photophobia, phonophobia, or visual or other aura) occurring during at least 2 vertiginous episodes should be present. 
  • Vestibular schwannoma: presents with progressive asymmetric hearing loss with occasional fluctuating hearing loss. Patients rarely have true vertigo but often complain of imbalance and disequilibrium. Patients may occasionally have tinnitus and imbalance but no hearing loss. Auditory brainstem response testing and MRI show abnormalities indicating nerve compression of the 8th cranial nerve.  
  • Multiple sclerosis (MS): characterized by disseminated patches of demyelination in the brain and spinal cord. Patients can present with symptoms identical to those of Ménière disease. However, during an attack of MS with vertigo, the nystagmus is typically more severe and longer-lasting than in patients with Ménière disease. Patients with MS also may have additional neurologic complaints. On ENG testing, central abnormalities are often seen in patients with MS but are typically absent in early Ménière disease. In MS, white-matter lesions may be seen on brain MRI and CSF abnormalities may be detected, but such findings are absent in patients with Ménière disease.  
  • Benign paroxysmal positional vertigo (BPPV): condition characterized by perceived rotatory motion. The condition is associated with vertigo related to head movements, lasting seconds to minutes with no aural symptoms. Hearing symptoms are absent, unlike in Ménière disease.

References

  1. Meyerhoff, WL, Paparella, MM, & Shea, D. (1978). Ménière’s disease in children. Laryngoscope. https://pubmed.ncbi.nlm.nih.gov/682806/ 
  2. Basura, G., et al. (2020). Clinical practice guideline: Ménière’s disease executive summary. Otolaryngol Head Neck Surg. 162(4), 415-434. https://pubmed.ncbi.nlm.nih.gov/32267820/ 
  3. Coelho, DH, & Lalwani, AK. (2008). Medical management of Ménière’s disease. Laryngoscope. https://pubmed.ncbi.nlm.nih.gov/18418279/ 
  4. Lustig, LR. (2021). Meniere disease. [Online] MSD Manual Professional Version. Retrieved September 14, 2021, from https://www.msdmanuals.com/professional/ear,-nose,-and-throat-disorders/inner-ear-disorders/meniere-disease
  5. Li, JC. (2020). Meniere disease (Idiopathic endolymphatic hydrops). In Lorenzo, N, et al. (Eds.), Medscape. Retrieved September 14, 2021, from https://emedicine.medscape.com/article/1159069
  6. Koenen, L, & Andaloro, C. Meniere Disease. [Updated 2021 Jul 19]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Retrieved October 5, 2021, from https://www.ncbi.nlm.nih.gov/books/NBK536955/

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