What is Meniere's disease?

Ménière's Disease: Understanding and Management

Ménière's disease is an inner ear disorder characterized by spontaneous vertigo attacks lasting 20 minutes to 12 hours, with documented low- to mid-frequency sensorineural hearing loss and fluctuating aural symptoms including tinnitus and ear fullness. 1

Definition and Diagnostic Criteria

Definite Ménière's Disease

• Two or more spontaneous attacks of vertigo, each lasting 20 minutes to 12 hours 1
• Audiometrically documented fluctuating low- to mid-frequency sensorineural hearing loss in the affected ear on at least one occasion before, during, or after one of the episodes of vertigo 1
• Fluctuating aural symptoms (hearing loss, tinnitus, or fullness) in the affected ear 1

Probable Ménière's Disease

• Two or more episodes of vertigo lasting up to 24 hours 1
• Fluctuating or non-fluctuating sensorineural hearing loss, tinnitus, or pressure in the affected ear 1
• Symptoms not better accounted for by another disorder 1

Pathophysiology

• The underlying etiology is not completely clear but has been associated with inner ear fluid (endolymph) volume increases 1
• Endolymphatic hydrops (excessive endolymph accumulation in the inner ear) is a characteristic sign that causes damage to ganglion cells 2
• Multiple biochemical pathways may be involved, including the endocrine system, innate immune response, and autonomic nervous system 3
• Allergens, infectious agents, vascular events, or genetic factors could modify inner-ear homeostasis and trigger the disease 3

Clinical Presentation

• Vertigo: Sensation of self-motion (rotary spinning) or movement of the environment when neither is occurring 1
• Hearing Loss: Often fluctuates from low- to mid-frequency but over time may involve all frequencies 1
• Tinnitus: Ringing, buzzing, or other noises in the ear when there is nothing causing the noise 1
• Aural Fullness: Sensation of ear feeling full or plugged 1
• Drop Attacks (Tumarkin's Otolithic Crisis): Sudden falls without warning or loss of consciousness, may be experienced during later stages of the disease 1
• Imbalance: Sense of unsteadiness or instability, distinct from vertigo; may be ongoing and not episodic 1
• Headache: May occur during attacks 3

Diagnostic Evaluation

Essential Testing

• Audiogram: Clinicians should obtain an audiogram when assessing a patient for Ménière's disease 1
  • Necessary to differentiate probable versus definite Ménière's disease 1
  • Should include pure tone thresholds and measures of speech recognition 1

Optional Testing

• MRI of Internal Auditory Canal and Posterior Fossa: May be offered in patients with nonfluctuating sensorineural hearing loss, tinnitus, or pressure when symptoms are not better accounted for by another disorder 1
  • Primarily to rule out retrocochlear pathology such as vestibular schwannoma 1
  • Not routinely required for all patients with suspected Ménière's disease 1

Tests to Avoid

• Vestibular Function Testing or Electrocochleography: Should not be routinely ordered to establish the diagnosis 1
  • Limited diagnostic value for confirming Ménière's disease 1

Management Approaches

Conservative Measures (First Line)

• Dietary Modifications:
  • Low sodium diet (American Heart Association recommends an "ideal" limitation of 1500 mg daily) 1
  • Limit alcohol consumption 1
  • Limit caffeine intake 1

Medical Therapy

• Vestibular Suppressants: Should be offered for a limited course to manage acute vertigo attacks 1
• Diuretics: May help reduce symptoms by decreasing fluid retention 1
• Intratympanic Steroids: May be offered to patients with active Ménière's disease not responsive to conservative measures 1
• Intratympanic Gentamicin: Should be offered to patients with active Ménière's disease not responsive to nonablative therapy 1
  • Caution: This is an ablative therapy that can affect vestibular function 1

Interventions Not Recommended

• Positive Pressure Therapy: Should not be prescribed for patients with Ménière's disease 1

Rehabilitation

• Vestibular Rehabilitation/Physical Therapy: Should be offered for patients who have failed less definitive therapy and have nonusable hearing 1
• Hearing Aids: May benefit those with usable hearing 1
• Contralateral Routing of Sound (CROS): Option for profound hearing loss in one affected ear 1

Patient Education and Follow-up

• Clinicians should educate patients about the natural history, measures for symptom control, treatment options, and outcomes 1

• Patients should be informed that Ménière's disease:

  • Is an adult-onset disorder (most commonly between 40 and 70 years) 1
  • Has unpredictable vertigo attacks and fluctuations in hearing, tinnitus, and ear fullness 1
  • May result in worsening hearing over time, though hearing may stabilize in some patients 1
  • Can affect the contralateral ear in some patients (25-40% develop bilateral involvement) 3

• Clinicians should document resolution, improvement, or worsening of vertigo, tinnitus, and hearing loss and any change in quality of life 1

Common Pitfalls and Caveats

• Misdiagnosis is common due to overlapping symptoms with other vestibular disorders 1
• Patients with asymmetric hearing loss should be evaluated for retrocochlear pathology if word recognition scores are worse than expected for their pure tone average 1
• Before considering highly invasive and ablative surgical procedures, patients should be informed about the possibility of bilateral disease development 1
• The disease can significantly impact quality of life, causing psychological distress that requires appropriate support and counseling 1

Ménière's disease remains a challenging condition to diagnose and manage, but with proper evaluation and a systematic approach to treatment, most patients can achieve significant symptom control and maintain quality of life.