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Meniere's disease is an idiopathic inner ear disorder characterized by vertigo, fluctuating hearing loss, tinnitus, and aural fullness. The causes are not understood. One of the most popular theories suggests the abnormal amount of endolymphatic fluid in the inner ear. Scientists suggest head trauma, a viral infection of the inner ear and a family history of Meniere's disease as main risk factors, although a lot of patients do not have any of these. The illness is unpredictable and incurable, but there are lifestyle recommendations, medications and surgical procedures to facilitate distorting symptoms.
Meniere's disease is a chronic, incurable disorder of the inner ear, defined as “the idiopathic syndrome of endolymphatic hydrops” that consists of spontaneous, episodic attacks of vertigo; fluctuating sensorineural hearing loss; tinnitus; and aural fullness. Meniere disease remains controversial; often it is difficult to diagnose, to determine pathogenesis, and to define optimal treatment.
There are no subtypes for this disease. There are differences between Meniere's disease and syndrome, which is explained in section Pathophysiology.
Etiology is unclear, immune and viral causes have been proposed. Endolymphatic hydrops is present. The mixing of endolymph and perilymph, when there is rupture of the membranous labyrinth, is suspected to be responsible for hearing loss and attacks of vertigo. Rarely it can be due to recent head trauma and syphilis (30 years after the onset of infection).
Possible contributing factors are:
Possible risk factors for the development of Meniere's disease include syphilitic otitis, a viral infection of the inner ear, head trauma, and a family history of Meniere's disease.
Meniere's disease is a long-lasting disease, but treatments and lifestyle changes can help to ease the symptoms. The main mechanism is thought to be a swelling of the endolymphatic compartment (called endolymphatic hydrops) that leads to permanent damage of the inner ear structures. Possible reasons for such swelling include mechanical obstruction of endolymphatic flow or dysregulation of the electrochemical membrane potential between endolymph and perilymph compartments.
If no primary cause is identified, the idiopathic form of the syndrome (triad of typical symptoms—hearing loss, tinnitus, vertigo) is called Meniere's disease. When an underlying cause is found, it is referred to as Meniere's syndrome or secondary Meniere's syndrome. The secondary syndrome can result from delayed endolymphatic hydrops or viral injury to the inner ear structures, autoimmune ear disease, syphilitic otitis, and trauma.
Symptoms vary between the time of an attack.
Before an attack, you may feel aura with underlying symptoms, such as:
If you recognize those symptoms, you should go to a place, which is comfortable and secure.
By the time of an attack, you may feel symptoms such as:
Those symptoms can be accompanied by:
After and between attacks you may feel symptoms, such as:
In general, most of the diagnosis is made clinically based on unilateral hearing loss, tinnitus, ear fullness, and episodic vertigo. There is no single test that can identify Meniere's disease, it can be done by excluding other pathologies.
There are criteria for certain, definite, probable, and possible Meniere's disease.
Certain Meniere's disease: definite Meniere's disease plus histopathological confirmation.
Definite Meniere's disease: two or more episodes of vertigo, each lasting 20 minutes or longer, with tinnitus or fullness in the affected ear; audiometric hearing loss documented on at least one occasion; excluded any other vestibular diagnosis.
Probable Meniere's disease: one definitive episode of vertigo and fulfillment of other criteria.
Possible Meniere's disease: episodic vertigo if the Meniere's type without documented hearing loss.
Neurological examination for balance disorders.
Audiometry during the tinnitus and fluctuation in hearing shows a low-frequency hearing loss while the patient is in the acute stage of vertigo or immediately after it.
Magnetic resonance imaging (MRI) of the head is useful to exclude other pathologies of the central nervous system.
Thyroid function tests can be done to rule out impaired thyroid function as a cause of dizziness.
Blood tests to rule out syphilis, Lyme disease, lupus if there are symptoms suggesting these conditions.
Electronystagmography (ENG) determines the vestibular system pathology by measuring involuntary eye movement while the balance is put under stress.
The purpose of the treatment is to make the attacks lighter and uncommon. Ease the residual symptoms and prevent the progression of hearing loss.
Conservative treatment: dietary modification (low-salt diets, avoidance of any allergens, caffeine, chocolate, alcohol, and smoking), reduce the intake of fluid, diuretics, vestibular sedatives, vasoactive medications.
During the attack, you need to have bed rest, avoiding noise and bright light. If necessary, you may intake medications such as vestibular sedatives or antiemetics.
Vestibular rehabilitation exercises can improve symptoms of vertigo. They help to train the brain to account for the difference in balance between both ears.
A short burst of oral steroids may be helpful for disease exacerbation. Intratympanic steroids also have been used to reduce systemic side-effects.
Transtympanic gentamicin ablation is used to reduce or destroy the remaining vestibular functions of the inner ear, so that signals are no longer sent to the brain.
If there is no improvement of symptoms with conservative treatment, surgery can be the required method, which includes the following options: endolymphatic sac decompression, vestibular neurectomy, and labyrinthectomy. The physician will suggest each method regarding vestibular and hearing function.
In the case of secondary Meniere's syndrome, treatment is underlying to the cause.
Possible complications include the progression of unilateral (rarely bilateral) hearing and vestibular function disorders. Because of the severe vertigo attacks, many patients might develop agoraphobic features (fear and avoid places or situations that might cause panic and feel trapped, helpless or embarrassed). The disease can interrupt quality of life and cause fatigue, emotional stress, depression and anxiety. Altered balance increases the risk of falls and accidents while driving or operating heavy machinery.
There is no known way of prevention for Meniere's disease because it occurs in many people without any risk factors.
There is no known cure for Meniere's disease, but it is not fatal. There is treatment to manage and ease the symptoms. It is difficult to predict the prognosis. The symptoms may disappear in one day and never return. Conversely, they can become more severe and patients may become totally or functionally disabled.
According to the Vestibular Disorders Association, Meniere's disease can develop at any age, the peak incidence is 40 to 50 years of age, it is rare in children and from the sixth decade. There is no gender bias. As reported in the Journal Otolaryngologic Clinics of North America in 2010, the prevalence rates of Meniere's disease varies widely, with estimated as low as 3.5 per 100,000 and as high as 513 per 100,00. The National Institute on Deafness and Other Communication Disorders estimates that about 615,000 people in the U.S. have Meniere's disease and that 450,000 new cases are re-diagnosed each year.
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