Ménière's disease

Overview

Ménière's disease is a chronic disorder of the inner ear that affects balance and hearing. It is characterized by a classic triad of symptoms: episodic vertigo, fluctuating sensorineural hearing loss (typically in one ear), and tinnitus (ringing or buzzing in the ear), often accompanied by a feeling of aural fullness or pressure. The condition was first described by French physician Prosper Ménière in the 19th century.

Ménière’s disease most commonly affects adults between the ages of 40 and 60 and is typically unilateral (affecting one ear), although bilateral cases can occur. The unpredictable and recurrent nature of the symptoms can have a significant impact on quality of life, especially due to the severity of vertigo attacks and progressive hearing loss.

Causes

The exact cause of Ménière's disease is not fully understood, but it is associated with endolymphatic hydrops, which refers to an abnormal accumulation of fluid (endolymph) in the inner ear’s labyrinth. This buildup disturbs the normal balance and hearing signals sent from the inner ear to the brain. Contributing factors and potential causes include:

• Abnormal fluid drainage: Blockage or anatomical malformation of the endolymphatic sac or duct

• Autoimmune response: Some researchers suggest an autoimmune component affecting the inner ear

• Genetics: Familial cases suggest a hereditary predisposition in some individuals

• Viral infections: Past infections like herpes simplex may play a role in triggering inner ear inflammation

• Allergies: Some studies link Ménière’s to systemic or local allergic reactions

Despite these associations, the disease mechanism remains idiopathic in many patients.

Symptoms

The hallmark symptoms of Ménière's disease are episodic and affect both auditory and vestibular systems. The severity and frequency of episodes can vary significantly among individuals.

1. Vertigo

• Sudden, spontaneous episodes of spinning or dizziness

• Usually last from 20 minutes to several hours (but not more than 24 hours)

• Often accompanied by nausea, vomiting, and imbalance

• Attacks may be unpredictable and disabling

2. Hearing Loss

• Fluctuating sensorineural hearing loss, often affecting low frequencies initially

• Hearing may improve between episodes but worsens over time

3. Tinnitus

• Persistent or intermittent ringing, buzzing, or roaring sound in the affected ear

4. Aural Fullness

• Sensation of pressure or fullness in the ear, similar to being underwater

Additional symptoms may include balance problems between episodes, anxiety due to the unpredictability of attacks, and progressive hearing impairment in the long term.

Diagnosis

Diagnosis of Ménière's disease is clinical, based on a detailed patient history, physical examination, and audiologic testing. Diagnostic criteria are defined by the American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS).

1. Clinical Criteria

• Two or more episodes of spontaneous vertigo lasting 20 minutes to 12 hours

• Documented sensorineural hearing loss on at least one occasion

• Tinnitus or aural fullness in the affected ear

• No other identifiable cause for the symptoms

2. Audiometric Testing

• Pure tone audiometry confirms low- to mid-frequency sensorineural hearing loss

3. Vestibular Testing

• Electronystagmography (ENG) or videonystagmography (VNG) may reveal reduced vestibular function on the affected side

4. Imaging

• MRI: Performed to exclude other causes such as acoustic neuroma or multiple sclerosis

5. Additional Tests

• Electrocochleography (ECoG) may help detect endolymphatic hydrops

Since Ménière’s disease is a diagnosis of exclusion, ruling out other vestibular or neurological conditions is essential.

Treatment

There is no cure for Ménière’s disease, but treatment focuses on reducing the frequency and severity of attacks, managing symptoms, and preserving hearing. Treatment options include lifestyle modifications, medications, and, in refractory cases, surgery.

1. Lifestyle and Dietary Changes

• Low-sodium diet: Reducing salt intake to minimize fluid retention in the inner ear

• Avoiding triggers: Such as caffeine, alcohol, and tobacco

• Stress reduction: Through relaxation techniques or therapy

2. Medications

• Diuretics: Such as hydrochlorothiazide to reduce fluid accumulation

• Vestibular suppressants: Like meclizine or diazepam during acute vertigo attacks

• Antiemetics: Such as promethazine to control nausea

• Betahistine: A vasodilator used in some countries to reduce vertigo frequency (not FDA-approved in the U.S.)

3. Intratympanic Therapy

• Steroid injections: Into the middle ear to reduce inflammation and preserve hearing

• Gentamicin injections: An aminoglycoside antibiotic that ablates vestibular hair cells to control severe vertigo (used when hearing is already significantly impaired)

4. Surgical Options (for Refractory Cases)

• Endolymphatic sac decompression: Aims to relieve fluid pressure while preserving hearing

• Vestibular nerve section: Cutting the nerve to eliminate vertigo while preserving hearing (requires craniotomy)

• Labyrinthectomy: Removal of the balance organ; used when hearing is nonfunctional

5. Vestibular Rehabilitation

• Exercises to improve balance and reduce dizziness between episodes

Prognosis

The course of Ménière’s disease is highly variable. While some patients experience frequent and disabling episodes, others have only occasional attacks or periods of remission. Over time:

• Vertigo episodes may decrease in frequency but be replaced by chronic imbalance

• Sensorineural hearing loss may become permanent and worsen gradually

• Emotional and psychological impacts, such as anxiety and depression, may occur due to the unpredictability of symptoms

With appropriate management, many individuals can control their symptoms and maintain a good quality of life. Continued research may lead to improved diagnostic tools and therapeutic options in the future.