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Ménière's disease

Definition
Ménière's disease is a chronic disorder of the inner ear characterized by intermittent episodes of vertigo and fluctuating hearing loss.
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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.
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Epidemiology
The prevalence of Ménière's disease in the US is estimated at 190 per 100,000 population.
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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.
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Prognosis and risk of recurrence
The prognosis of Ménière's disease is generally favorable in terms of symptom management.
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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 Physical Therapy Association (APTA 2022), the American Cochlear Implant Alliance (ACI Alliance 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 (AAN 2017).
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Guidelines

1.Screening and diagnosis

Diagnostic criteria
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
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2.Diagnostic investigations

Audiometry: obtain audiometry when assessing patients for the diagnosis ofMénière's disease.
B

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  • MRI

  • Vestibular function testing

3.Diagnostic procedures

Electrocochleography: do not obtain routine electrocochleography to establish the diagnosis ofMénière's disease.
D

4.Medical management

Acute therapy: offer a limited course of vestibular suppressants for the management of acute vertigo attacks in patients withMénière's disease.
B

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  • Maintenance therapy

5.Nonpharmacologic interventions

Dietary and lifestyle changes: advise patients withMénière's disease on dietary and lifestyle modifications that may reduce or prevent symptoms.
B

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  • Vestibular rehabilitation

  • Psychological support

6.Therapeutic procedures

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

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  • Intratympanic corticosteroids

  • Intratympanic gentamicin

  • Hearing aids

  • Cochlear implants

7.Surgical interventions

Tympanostomy: consider performing tympanostomy tube placement in patients withMénière's disease.
E

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  • Labyrinthectomy

  • Endolymphatic sac surgery

8.Patient education

Patient education: educate patients withMénière's disease about the natural history, measures for symptom control, treatment options, and outcomes of this disease.
B

9.Follow-up and surveillance

Follow-up assessment: document resolution, improvement, or worsening of vertigo, tinnitus, and hearing loss and any change in the QoL in patients withMénière's disease after treatment.
B