Ménière's disease

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at September 28, 2016
StartMénière's disease

Ménière's disease is an inner ear disorder that causes vertigo and fluctuating hearing loss that progresses to permanent hearing loss and tinnitus. While the disorder can occur at any age, it most often begins between 20 and 50. Also known as idiopathic endolymphatic hydops, Ménière's disease is the most common cause of dizziness from the inner ear

Contents

Definition & Facts

Ménière's disease is a set of symptoms that are episodic, lasting 20 minutes to 4 hours and progressively worsening over time. These episodes include vertigo, or a sudden sensation of spinning and dizziness that starts and stops without warning, along with hearing loss, ringing or buzzing in the ears, and a feeling of fullness in the affected ear.

In most cases, Ménière's disease only affects one ear, but about 15% of patients have symptoms in both ears. Men and women are equally affected, but the disease affects every person differently. Ménière's disease often leads to permanent hearing loss in one or both ears. According to the National Institute on Deafness and Other Communication Disorders (NIDCD), about 615,000 people in the United States have Ménière's disease and another 45,000 new cases are diagnosed each year.

Symptoms & Complaints

Ménière's disease usually presents as a set of symptoms that come in episodes. These episodes often include vertigo (or a dizzy, spinning sensation); hearing loss; a buzzing, ringing, or roaring sound in the ear (tinnitus); and an odd feeling of fullness.

These episodes may last anywhere from 20 minutes to 4 hours. Hearing loss may begin as intermittent and occur at the same time as vertigo. During these attacks, loud sounds will be distorted. The hearing loss usually involves lower pitches, but as the disease progresses it can affect all pitches. Hearing loss usually becomes permanent, but this may take months or many years after symptoms first develop.

The tinnitus may only occur during the attacks or it may be persistent. For most people, only one ear is affected, but about 15% of patients report the symptoms in both ears. People with Ménière's disease are more susceptible to issues like psychological stress and fatigue, both of which can increase the frequency of symptom attacks. 

Causes

The cause of Ménière's disease is unknown, but it is believed to be the result of an abnormality in the amount of fluid in the inner ear. This theory does have at least some validity as this has shown up on autopsies. An excess accumulation of fluid may be caused by inadequate absorption by the body or excess production of this fluid. In some people, especially among those for whom symptoms are in both ears, autoimmune diseases or allergies may play some role in the development of Ménière's disease.

Any factors that affect the fluid in the ear can contribute to Ménière's disease, including: improper drainage that may be related to an abnormality of the ears, abnormal immune response, traumatic head injury, genetic factors, genetic factors, migraines, and viral infections. Ménière's disease does appear to run in family history families, and there may be genetic mutations that cause abnormalities in the regulation or the amount of fluid in the inner ear. 

Diagnosis & Tests

A physician may suspect Ménière's disease when a patient has vertigo attacks, especially when accompanied by hearing loss. Diagnosis begins by taking a history of the duration, frequency, severity, and type of attacks, the duration of any hearing loss and whether it has changed, and whether the patient has had tinnitus (ringing of the ear) in one or both ears.

Several diagnostic tests may be used to test for Ménière's disease. A hearing test will be used to indicate a sensory type of hearing loss in the affected ear or ears. An electronystagmography (ENG) test can be used to evaluate the patient's balance. Other tests may include an electrocochleography to check for electrical activity in the inner ear, an auditory brainstem response (ABR) test that checks the brain pathways and nerves, and magnetic resonance imaging (MRI) and computed tomography (CT) scans to rule out tumor.

There is no definitive test for Ménière's disease, but diagnosis will be made based on a patient's medical history, family history, the presence of tinnitus, two or more episodes of vertigo that last at least 20 minutes each, temporary hearing loss, and a feeling of fullness in the ear.

Treatment & Therapy

There is no cure for Ménière's disease, but vertigo attacks can be controlled well using several treatment options. The standard treatment for Ménière's disease includes a low sodium diet and diuretics (water pills), anti-vertigo medication, and intratympanic injections of dexamethasone or gentamicin. In some cases, low-salt diet and diuretics alone can control symptoms. Intratympanic injections involve injecting medication into the space of the middle ear through the eardrum to the point of the ear bones. This treatment is done in an otolaryngologist's office and involves inserting a thin tube in the eardrum or making a temporary opening.

Gentamicin is used to alleviate dizziness, but it does have carry a risk of causing hearing loss. Corticosteroids do not carry this risk, but they are less effective. Air pressure pulse generators are mechanical pumps that can be applied to the ear canal three times a day for about five minutes per session. A ventilating tube is inserted through the eardrum to allow pressure to be transmitted across the membrane and adjust the air pressure inside the inner ear.

In a very small number of people, surgery may be recommended. This is only advised when vertigo is not controlled by other treatment options and becomes disabling. Several surgical procedures may be performed, including endolymphatic sac shunt; selective vestibular neurectomy, or cutting the vestibular nerve; and labyrinthectomy which may be used for those who have already lost their hearing. This last procedure is only considered when the patient already has damaged hearing in the affected ear but severe vertigo.

Prevention & Prophylaxis

There is no known way to prevent Ménière's disease, but several steps can be taken to reduce the frequency of Ménière's disease episodes. Avoiding alcohol, smoking, caffeine, stress, and excessive salt seem to reduce the frequency of episodes in most people.

A proper diet and regular sleep are also beneficial as stress can worsen symptoms. Physical activity and regular exercise are important but not to the point of excessive fatigue which can trigger symptoms.