Definition and Overview
Ménière's disease is an inner ear disease that causes repetitive attacks of rotary vertigo, fluctuating sensorineural hearing loss, tinnitus, and aural fullness due to endolymphatic hydrops, which is excessive accumulation of endolymph fluid in the inner ear.
It was first described by French physician Prosper Ménière in 1861. The prevalence is estimated to be about 50 to 200 per 100,000 people, and it occurs most often in people in their 30s and 60s. It occurs approximately 1.3 to 1.9 times more often in women than in men.
Diagnostic criteria
Diagnostic criteria presented by the Bárány Society and the International Headache Society (IHS) (2015):
Definite Ménière's disease:
1. Two or more attacks of spontaneous rotatory vertigo (lasting 20 minutes to 12 hours) 2. Low-frequency or low-mid-frequency sensorineural hearing loss on the affected side, confirmed by hearing testing at least one time before, during, or after the attack 3. Variable auditory symptoms on the affected side (decreased hearing, tinnitus, aural fullness) 4. Not explained by another vestibular disorder
Probable Ménière's disease:
1. Two or more attacks of vertigo or dizziness (20 minutes to 24 hours) 2. Variable auditory symptoms on the affected side 3. Not explained by another vestibular disorder
Causes and Mechanisms
The exact cause of Meniere's disease has not yet been fully identified. Although endolymphatic hydrops is anatomically based, there are cases where there are no symptoms even if endolymphatic hydrops is present, making it difficult to explain it as a single cause.
Endolymph circulation disorder
The endolymph fluid of the inner ear is produced in the stria vascularis and absorbed in the endolymphatic sac. When this circulation is disrupted, endolymph pressure rises and endolymphatic edema is formed. Endolymphatic sac dysfunction, inner ear blood flow disturbances, and immune-mediated damage are involved.
related factors
- Autoimmunity: Patients with Meniere's disease have a high rate of accompanying autoimmune diseases (especially thyroid diseases).
- Viral infection: There is a hypothesis that the herpes simplex virus may latently infect the inner ear and cause attacks.
- Migraine: Meniere's disease and migraine often occur together, and differentiation from vestibular migraine is necessary.
- Heredity: If there is a family history, the risk of developing the disease is high.
- Psychological/autonomic nervous system: Stress and autonomic dysfunction can cause or worsen attacks.
clinical features
pre-attack phase
Prodromal symptoms such as increased fullness and tinnitus may occur hours to days before the attack.
attack stage
A sudden onset of rotational dizziness is accompanied by nausea, vomiting, and sweating. Attacks last from 20 minutes to several hours (up to 12 hours). During this period, hearing loss, tinnitus, and fullness of the ear become more severe. During a attack, a rare Tumarkin sudden drop attack may occur, causing sudden collapse.
Post-attack stage
After the attack passes, fatigue may persist and it may take several days for balance to be fully restored.
long term course
Initially, hearing is restored between attacks, but with repeated attacks, hearing is gradually lost starting from the low frequency range. In approximately 80-90% of patients, only one ear is affected, but in 10-20% of cases, it progresses bilaterally.
diagnostic tests
- Pure tone audiometry: It is characterized by variable sensorineural hearing loss in the low frequency region (250-1000 Hz).
- MRI of the internal auditory canal: Research is underway to directly identify endolymphatic hydrops using gadolinium-enhanced MRI.
- Electrocochleography (ECochG): Increased SP/AP ratio suggests endolymphatic hydrops.
- Electronystagmus test (ENG)/video head impulse test: assessment of vestibular function.
- Auditory evoked potentials (ABR): Used to rule out retrolabyrinthic lesions.
treatment
life management
A low-salt diet (1,500 to 2,000 mg of sodium per day) is the most important lifestyle management method. Reduce caffeine, alcohol, and nicotine and maintain regular sleep. Stress management is also important in preventing attacks.
medication
Acute attack phase: - Antihistamines (meclizine, dimenhydrinate) - Benzodiazepines (diazepam) - Antiemetic (metoclopramide)
Preventive treatment: - Diuretics (hydrochlorothiazide, acetazolamide): For the purpose of reducing endolymph production. - Betahistine: Used to improve blood flow in the inner ear, but evidence is limited.
Intratympanic injection treatment
Intratympanic steroid injection: Aimed at suppressing inner ear inflammation and preserving hearing.
Intratympanic gentamicin injection: chemically blocks vestibular function on the affected side using vestibular neurotoxicity. It is effective in controlling dizziness (about 70-90%), but there is a risk of hearing loss.
surgical treatment
If the patient does not respond to drug treatment, endolymphatic sac decompression, vestibular nerve transection, and labyrinthectomy are considered.
life guide
- Limit your sodium intake. Reduce processed foods, pickles, and salty foods.
- Limit caffeine (coffee, energy drinks, black tea) and alcohol intake.
- Regular sleep and stress management are important in preventing attacks.
- Recognize attack prodromal symptoms (fullness of the ear, worsening tinnitus) and ensure a safe environment.
- Driving during a attack is absolutely prohibited. If there are occupational hazards, occupational safety measures are necessary.
- If you experience changes in your hearing, get your hearing tested periodically.