What is and who can be affected by Ménière’s disease

Ménière’s disease is a multifactorial disorder of the inner ear characterized by recurrent, spontaneous attacks of rotatory vertigo, often with nausea and vomiting, accompanied by hearing loss, tinnitus and aural pressure in the affected ear. They usually last between 20/30 minutes and 1/2 hour.

Hearing loss is initially fluctuating, recovering after the vertigo attack. However, it progressively becomes less variable and evolves into a moderate-severe hearing loss. Tinnitus usually becomes permanent, increasing prior to vertigo attacks.

The course of the disease is highly variable. Patients usually present several attacks of vertigo followed by long asymptomatic periods. In a significant percentage of patients, vertigo attacks may subside within 2 to 8 years, but in some patients the disease becomes disabling.

The disease owes its name to the French physician Prosper Ménière, who was the first to describe its symptoms in 1861. The incidence of Ménière’s disease is estimated at 8/46 cases per 100,000 inhabitants per year. It occurs between 30 and 70 years of age, and is more common in women. Its prevalence clearly increases with age.

Ménière’s disease is associated with migraine, irritable bowel disease, psoriasis and rheumatoid arthritis.

What causes Ménière’s disease?

The etiology of Ménière’s disease is not known. Autoimmune, genetic and local damage factors have been invoked.

How is Ménière’s disease diagnosed?

The International Classification of Vestibular Disorders of the Bárány Society bases the definitive diagnosis of Ménière’s disease on a compatible clinical history, with two or more episodes of vertigo (lasting between 20 minutes and 12 hours) with fluctuating aural symptoms (tinnitus, hearing loss or otic fullness), together with an audiometrically documented low or mid-frequency sensorineural hearing loss.

Although Ménière’s disease is strictly defined clinically, not all data are always present or are not all present simultaneously. Certain tests can help us to clarify an uncertain diagnosis or to rule out other processes:

• Electrocochleography: provides us with electrophysiological evidence of endolymphatic hydrops or dilatation of the vestibular organ that characterizes the pathophysiology of this disease.
• The Video-Head Impulse Test (v-HIT), vestibular evoked myogenic potentials (VEMPs) or caloric tests help us to identify the affected side and to quantify vestibular damage.
• MRI with gadolinium is indicated when we want to rule out causes related to the central nervous system, although recently we have been able to visualize hydrops using specific sequences.

With which processes should a differential diagnosis be made?

The symptomatic triad of vertigo, hypoacusis and tinnitus is not exclusive to Ménière’s disease. In early stages it can be confused with third window syndrome, tumors of the cerebellopontine angle and even with tubaritis.

When there is frank vertigo, we must rule out peripheral (labyrinth fistula, infectious labyrinthitis, otosclerosis, vertebrobasilar insufficiency, benign paroxysmal positional vertigo) or central (multiple sclerosis, Susac’s syndrome) processes.

What is the treatment of Meniere’s disease?

The goals of treatment are to reduce the number and intensity of vertigo and to improve the associated hearing loss and tinnitus. The treatment of Meniere’s disease is usually staggered, depending on the frequency and intensity of symptoms.

1. Medical treatment.

1. Betahistine: has a positive effect in reducing vertigo symptoms, although meta-analysis studies to date give it low quality evidence.
2. Diuretics: the most commonly used are hydrothiazide and acetazolamide, in order to reduce endolymphatic fluid pressure. There is no quality evidence to support or reject their use.
3. Intratympanic corticosteroids: the injection of corticosteroids through the tympanic membrane under local anesthesia achieves high concentrations of the drug in the inner ear fluids and avoids the undesirable effects of its systemic administration. There are studies with sufficient scientific quality that support its use.
4. Intratympanic gentamicin: gentamicin is an antibiotic with toxicity for vestibular cells, so its intratympanic injection aims at total or partial ablation of vestibular function. It achieves a high rate of complete or substantial control of vertigo, with very low risk to hearing.

2. Surgical treatment.
   1. Labyrinthectomy: involves surgical ablation of the inner ear. It is indicated in patients with a disabling course who present with poor hearing.
   2. Vestibular neurectomy: involves the section of the vestibular nerve, respecting the cochlear nerve. Indicated in patients with a disabling course who still have useful hearing.

3. Rehabilitative treatment. Vestibular rehabilitation is indicated in patients with advanced disease who present instability or imbalance. It is also useful for recovery after surgical treatments.

For more information, consult an ENT specialist.