ENT

Meniere’s Disease: Diagnosis and Management

SUMMARY:

Dizziness is one of the most common chief complaints in the ambulatory setting, making up an estimated 5% of all primary care visits. The differential for dizziness is broad and can range from orthostatic hypotension to hypoglycemia to stroke. Ménière’s disease (MD) is a clinical condition characterized by recurrent bouts of vertigo in association with the hallmark symptoms of tinnitus and hearing loss. Early diagnosis and initiation of MD therapy can improve quality of life, decrease the risk of disability due to vertigo attacks and progressive hearing loss, and lead to decreased health care costs. The American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS) has released a guideline to help doctors identify and treat this chronic illness.

Clinical Presentation

Triad of symptoms: Vertigo | Hearing loss | Tinnitus
Vertigo attacks:
- Last 20 minutes and up to 24 hours
- Can be associated with nausea and vomiting
Hearing loss can fluctuate and typically affects lower frequency hearing early in presentation
Tinnitus can be variable and occur independent of vertigo attacks or hearing loss
Most common in patients between 40 to 60 years old
- <3% of cases estimated to occur in childhood
Similar prevalence rates in men and women
MD usually presents with unilateral ear symptoms that can span decades
- Bilateral ear symptoms on presentation are exceptionally rare and should prompt consideration of another diagnosis
- Bilateral ear symptoms do occur in many patients as their disease progresses within two decades after initial presentation
MD attacks are typically random and episodic (approximately 6 to 11 per year)
Periods of remission may last months to years and symptoms can fluctuate unpredictably
Vertigo attacks often occur at increased frequency during the first few years of illness and then may level off or stop completely

Definitions and Diagnosis

Definitions

Aural Symptoms: Hearing loss | Tinnitus | Fullness
Vertigo: Sensation of self-motion OR the environment moving when neither is occurring
Drop attacks: Sudden fall associated with discrete MD attacks

Diagnosis

The diagnosis is made clinically based on defined criteria
It may take months or years to reach a diagnosis of MD given the relapsing and intermittent nature of its presentation
If MD is suspected clinicians should obtain an audiogram to evaluate for subclinical hearing loss and establish baseline hearing
Doctors may offer MRI to rule out inner ear or retrocochlear lesions in patients who present with asymmetric hearing loss
- Tests that should not be routinely offered include: Vestibular function testing | Electrocochleography
Diagnostic criteria for definite MD
- Two or more spontaneous attacks of vertigo, each lasting 20 minutes to 12 hours
- Audiometrically documented fluctuating low- to midfrequency sensorineural hearing loss in the affected ear on at least 1 occasion
- Fluctuating aural symptoms in the affected ear
- Other causes excluded by other tests
Diagnostic criteria for probable MD
- Two or more episodes of vertigo OR dizziness, each last 20 minutes to 24 hours
- Fluctuating aural symptoms in the affected ear
- Other causes excluded by other tests

Differential Diagnosis

Migraine (e.g., Migrainous vertigo | Vestibular migraine)
- The AAO-HNS recommends vestibular migraine be specifically ruled out in patients presenting with symptoms concerning for MD
- Vestibular migraine is much more common than MD in the general population
- Symptoms of vestibular migraine include: Headache (before or after attacks of vertigo) | Photophobia | Phonophobia
TIA | Stroke
Multiple sclerosis
Otosyphilis
Vestibular neuritis
Acute labyrinthitis
Tumor (e.g., Vestibular schwannoma)
BPPV

Treatment

Goals of MD therapy
- Prevent and/or reduce the frequency of vertigo attacks
- Relieve or prevent hearing loss and other aural symptoms
- Improve quality of life

Non-pharmacologic

Dietary and lifestyle modifications are the first line therapy for treatment of MD and should be continued indefinitely
- Identify and avoid personal triggers (e.g., Alcohol | Caffeine | Nicotine | Stress | High salt intake)
Vestibular rehabilitation/physical therapy should be offered to MD patients with chronic imbalance
- Not indicated for patients with acute vertigo/acute symptoms
Amplification and hearing assistive technology for patients with evidence of hearing loss clinically or on audiogram

Pharmacologic

Acute vertigo symptoms during MD attacks
- Clinicians should offer a limited course of vestibular suppressants (e.g., Benzodiazepines | Antiemetics | Antihistamines)
Chronic management of MD
- Betahistine
- Diuretics (e.g., Furosemide | Hydrochlorothiazide | Triamterene)
Recalcitrant or severe symptoms of MD
- Intratympanic steroids
- Intratympanic gentamicin

Surgical

For patients with persistent severe MD symptoms and nonusable hearing, referral to a clinician who can perform ablative procedures (e.g., labyrinthectomy) is recommended
- These procedures are destructive to the labyrinthine system and should not be used when hearing is preserved

Follow Up

Clinicians should document changes and trajectory of MD’s cardinal symptoms (e.g., Vertigo | Hearing loss | Tinnitus) following initiation of therapy
Continued disabling symptoms despite lifestyle changes, dietary modifications, and pharmacotherapy should prompt investigation into an alternative diagnosis

KEY POINTS:

Ménière’s disease is a chronic relapsing and remitting illness diagnosed by the classic triad of recurrent vertigo in association with tinnitus and hearing loss
Untreated Ménière’s disease can result in significant disability and decreased quality of life
Treatment for Ménière’s disease focuses on identification and avoidance of triggers, but pharmacotherapy and surgical options can help in recalcitrant cases

Learn More – Primary Sources

AAO-HNS: Clinical Practice Guideline: Ménière’s Disease Executive Summary

AAFP Dizziness: A Diagnostic Approach

JAMA: Progression of Symptoms of Dizziness in Ménière’s Disease