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Ménière's disease

Background

Overview

Definition
Ménière's disease is a chronic disorder of the inner ear characterized by intermittent episodes of vertigo and fluctuating hearing loss.
1
Pathophysiology
The pathophysiology of Ménière's disease is multifactorial, with a characteristic sign being endolymphatic hydrops, a disorder in which excessive endolymph accumulates in the inner ear and causes damage to the ganglion cells.
2
Epidemiology
The prevalence of Ménière's disease in the US is estimated at 190 per 100,000 population.
3
Disease course
Clinically, patients experience intermittent episodes of vertigo lasting from minutes to hours, along with fluctuating sensorineural hearing loss that can progress over time. Tinnitus and aural pressure are also common symptoms. The severity and frequency of these symptoms can vary greatly among patients.
1
Prognosis and risk of recurrence
The prognosis of Ménière's disease is generally favorable in terms of symptom management.
1

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of Ménière's disease are prepared by our editorial team based on guidelines from the American Cochlear Implant Alliance (ACI Alliance 2022), the American Physical Therapy Association (APTA 2022), the American Academy of Otolaryngology-Head and Neck Surgery Foundation (AAO-HNSF 2020), the Danish Health Authority (DHA 2018), and the American Academy of Neurology ...
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Screening and diagnosis

Diagnostic criteria
As per AAO-HNSF 2020 guidelines:
Diagnose definite or probable Ménière's disease in patients presenting with ≥ 2 episodes of vertigo lasting 20 minutes to 12 hours (definite) or up to 24 hours (probable) and fluctuating or non-fluctuating sensorineural hearing loss, tinnitus, or pressure in the affected ear, when these symptoms are not better accounted for by another disorder.
B
Determine if patients meet diagnostic criteria for vestibular migraine when assessing for Ménière's disease.
B
ICVD criteria for Ménière's disease
Episodes duration
≥ 2 spontaneous episodes of vertigo, each lasting 20 minutes to 12 hours
≥ 2 episodes of vertigo or dizziness, each lasting 20 minutes to 24 hours
Aural symptoms
Fluctuating aural symptoms (hearing, tinnitus or fullness) in the affected ear
Audiometry findings
Audiometrically documented low-to-medium-frequency sensorineural hearing loss in one ear, defining the affected ear on at least one occasion before, during or after one of the episodes of vertigo
Differential diagnosis
Not better accounted for by another vestibular diagnosis
Criteria not met
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Diagnostic investigations

Audiometry: as per AAO-HNSF 2020 guidelines, obtain audiometry when assessing patients for the diagnosis of Ménière's disease.
B

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  • MRI

  • Vestibular function testing

Diagnostic procedures

Electrocochleography: as per AAO-HNSF 2020 guidelines, do not obtain routine electrocochleography to establish the diagnosis of Ménière's disease.
D

Medical management

Acute therapy: as per AAO-HNSF 2020 guidelines, offer a limited course of vestibular suppressants for the management of acute vertigo attacks in patients with Ménière's disease.
B

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  • Maintenance therapy

Nonpharmacologic interventions

Dietary and lifestyle changes: as per AAO-HNSF 2020 guidelines, advise patients with Ménière's disease on dietary and lifestyle modifications that May reduce or prevent symptoms.
B

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  • Vestibular rehabilitation

  • Psychological support

Therapeutic procedures

Positive pressure therapy: as per AAO-HNSF 2020 guidelines, do not perform positive pressure therapy in patients with Ménière's disease.
D

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  • Intratympanic corticosteroids

  • Intratympanic gentamicin

  • Hearing aids

  • Cochlear implants

Surgical interventions

Tympanostomy: as per DHA 2018 guidelines, consider performing tympanostomy tube placement in patients with Ménière's disease.
E

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  • Labyrinthectomy

  • Endolymphatic sac surgery

Patient education

Patient education: as per AAO-HNSF 2020 guidelines, educate patients with Ménière's disease about the natural history, measures for symptom control, treatment options, and outcomes of this disease.
B

Follow-up and surveillance

Follow-up assessment: as per AAO-HNSF 2020 guidelines, document resolution, improvement, or worsening of vertigo, tinnitus, and hearing loss and any change in the QoL in patients with Ménière's disease after treatment.
B