Meniere's Disease- What Exactly Is It?

It is a disorder of the inner ear, which is characterized by episodes of vertigo, ringing in the ear (tinnitus), hearing loss, and fullness in the ear.

Epidemiology, Incidence, and Prevalence

The reported prevalence of Meniere disease varies widely, from 15 per 100,000 in the United States to 157 per 100,000 in the United Kingdom.

Causes

The cause of Meniere’s disease is elevated endolymphatic pressure. Causes of elevated endolymphatic pressure include:

1. Metabolic disturbances
2. Hormonal imbalance
3. Trauma
4. Infections, such as otosyphilis and Cogan’s syndrome (interstitial keratitis)
5. Autoimmune diseases, such as systemic lupus erythematosus and rheumatoid arthritis, may cause an inflammatory response within the labyrinth

Signs and Symptoms

1. Recurrent episodes of vertigo, hearing loss and tinnitus
2. These episodes may be accompanied by a headache and a feeling of fullness in the ears
3. People may also have additional symptoms related to irregular reactions of the autonomic nervous system, such as nausea, vomiting, and sweating, which are typical symptoms of vertigo and not of Meniere disease
4. Sensation of being pushed sharply to the floor from behind
5. Sudden falls without loss of consciousness

Complications of Meniere’s disease may include the following:

• Injury due to falls
• Anxiety regarding symptoms
• Accidents due to vertigo spells
• Disability due to unpredictable vertigo
• Progressive imbalance and deafness
• Intractable tinnitus

Differential Diagnosis

• Anterior circulation stroke
• Basilar artery thrombosis
• HIV-1 associated CNS conditions - meningitis
• Hypothyroidism and myxedema coma
• Inner ear labyrinthitis
• Intracranial hemorrhage
• Ischemic stroke in emergency medicine
• Migraine headache
• Multiple sclerosis
• Neurosyphilis
• Polyarteritis nodosa
• Rheumatoid arthritis
• Syncope
• Viral encephalitis
• Viral meningitis
• Wernicke encephalopathy

Investigations

Laboratory studies - No blood test is specific for Meniere’s disease but to exclude obvious metabolic disturbances, infections, or hormonal imbalances:

• Thyroid-stimulating hormone (TSH), T4, and T3 to rule out hyperthyroidism and hypothyroidism
• Glucose level to rule out diabetes
• Erythrocyte sedimentation rate (ESR) and antinuclear antibody (ANA) test to rule out autoimmune disorders
• Complete blood count (CBC) to rule out anemia and leukemia
• Electrolyte levels to rule out salt/water imbalance
• Venereal Disease Research Laboratory test (VDRL) and fluorescent treponemal antibody (FTA-ABS) to rule out neurosyphilis and Lyme disease
• Allergy testing for allergy-mediated Meniere’s syndrome
• C-reactive protein (CRP)

MRI of the brain - Should be done to rule out abnormal anatomy or mass lesions. Specifically, acoustic neuromas or other cerebellopontine angle lesions, multiple sclerosis, or Arnold-Chiari malformations, also can be ruled out. Note that mass lesions rarely are found but are important to exclude.

CT scan of the brain - They are done to detect possible dehiscence of the semicircular canals, congenital abnormalities, widened cochlear and vestibular aqueducts, and subarachnoid hemorrhage.
Audiometry - It is particularly helpful for documenting present hearing acuity and detecting future change.

Management - 

1. Vestibulosuppressants
2. Diuretics and diuretic-like medications
3. Steroids
4. Aminoglycosides
5. Histamine agonists
6. Endolymphatic sac decompression or shunt
7. Labyrinthectomy

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