Meniere's Disease

Aka: Meniere's Disease, Meniere Disease, Labyrinthine Hydrops, Endolymphatic Hydrops

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II. Epidemiology

  1. Incidence: 4.3 per 100,000 persons/year
  2. Prevalence: 17-46 per 100,000 persons
  3. Age of onset
    1. Most common at ages 20 to 60 years old
    2. Peaks at 40-60 years old (with a biphasic peak around age 20 years old)

III. Etiology

  1. Unknown
  2. Exacerbated by stress or emotional disturbance
  3. Associated with concurrent infection in 50% cases

IV. Pathophysiology

  1. Swelling of endolymphatic labyrinthine spaces (increased endolymphatic fluid pressure)
  2. Degeneration of the Organ of Corti

V. Symptoms

  1. Prodrome
    1. Headache
  2. Triad
    1. Vertigo
      1. Recurrent, spontaneous episodes that may last minutes to hours, days when severe (may require bed rest)
      2. Diagnosis requires at least 2 episodes lasting 20 minutes
      3. Associated Nausea, Vomiting and Ataxia
    2. Sensorineural Hearing Loss
      1. Fluctuant, typically low pitched Hearing Loss
    3. Tinnitus
      1. "Roaring", low tone Tinnitus (or aural fullness)
  3. Distribution
    1. Typically unilateral, at least initially (but 33% have bilateral disease)
  4. Other symptoms
    1. Episodic fluctuating, ear pressure or aural fullness (inner ear endolymphatic fluid collection)

VI. Signs

  1. Nystagmus (and associated Ataxia)
    1. Nystagmus is only present when Vertigo present
    2. Unidirectional, horizontal or rotary, torsional Nystagmus
  2. Sensorineural Hearing Loss
    1. Early: Low tones affected (low to medium frequency Sensorineural Hearing Loss)
    2. Later: All tones affected
  3. Hyperacusis
    1. Some noises may seem paradoxically louder (auditory recruitment)

VII. Diagnostics

  1. Audiogram

VIII. Management: Acute

  1. Benzodiazepines (e.g. Diazepam)
  2. Antiemetics (e.g. Ondansetron, Prochlorperazine, Promethazine)
  3. Transdermal Scopolamine

IX. Management: Maintenance

  1. As of 2023, evidence is poor for all of the interventions listed
  2. Diuretics
    1. Hydrochlorothiazide or
    2. Hydrochlorothiazide/Triamterene (Dyazide)
  3. Lifestyle changes
    1. Low salt diet (<2 grams daily)
    2. Decrease Caffeine
    3. Smoking Cessation
    4. Limit Alcohol
    5. Vestibular rehabilitation or Exercises may be effective
  4. Symptomatic Medications for acute episodes
    1. See Vertigo Management for acute symptomatic management
    2. Vestibular balance and rehabilitation therapy

X. Management: ENT

  1. Transtympanic injection of Corticosteroids
  2. Ablation of vestibular hair cells (in those who already have Hearing Loss)
    1. Performed with transtympanic injection of Gentamicin
  3. Surgery: (10% of patients with refractory cases)
    1. Labyrinthectomy to decompress endolymphatic sacs (Symptom relief in 66%)
    2. Vestibular Nerve section (Symptom relief in 95%)
    3. No procedure corrects the Hearing Loss

XI. References

  1. Glasscock (1984) Am J Otol 5:536-42 [PubMed]
  2. Knox (1997) Am Fam Physician 55(4):1185-90 [PubMed]
  3. Muncie (2017) Am Fam Physician 95(3): 154-62 [PubMed]
  4. Rogers (2023) Am Fam Physician 107(5): 514-23 [PubMed]

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