Meniere's Disease

Meniere's disease (also known as endolymphatic hydrops) is an inner ear disorder characterized by recurrent vertigo (a spinning sensation), deafness, and tinnitus (ringing in the ears). It is uncommon before the age of 40.

Cause

The cause of the disorder is an accumulation of fluid in the labyrinth. The buildup may damage the labyrinth and sometimes the adjacent cochlea.

Symptoms

There is a sudden attack of vertigo, lasting from a few minutes to several hours. This is usually accompanied by nausea, vomiting, nystagmus (abnormal jerky eye movements), and deafness, tinnitus, and a feeling of pressure or pain in the affected ear.

Diagnosis and treatment

Diagnosis is usually made with audiometry or other hearing tests, and a caloric test. Treatment with certain antihistamine drugs, such as cinnarizine, or with betahistine usually relieves the symptoms. Prochlorperazine may be given, either rectally or by injection, for severe attacks.

Ménière’ s disease can also be treated by surgery to the inner ear if symptoms are not controlled by drugs. If deafness eventually becomes total, the other symptoms usually disappear.

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Meniere's disease in detail - technical article

Meniere’s Disease in more detail - technical

History and Pathogenesis

Meniere’s disease or endolymphatic hydrops is an idiopathic inner ear disorder characterized by attacks of vertigo, fluctuating hearing loss, tinnitus, and aural fullness. The recognition that vertigo was linked to the inner ear rather than only to central sources was first clearly described by Prosper Meniere in 1861. In 1938, Charles Hallpike and Hugh Cairns made the most significant advance in understanding the pathogenesis of Meniere’s disease by describing dilation of the saccule and scala media with obliteration of the perilymph spaces of the vestibule and scala vestibuli.

The cause of the endolymphatic hydrops continues to elude us. The cause of Meniere’s disease has been attributed to anatomic, infectious, immune, and allergic factors. The focus of most studies has been the endolymphatic duct and sac (ES), with the basic premise being that there is increased endolymphatic fluid owing to impaired reabsorption of the endolymphatic fluid in the endolymphatic duct and endolymphatic sac (ES). There has been no conclusive proof of an infectious agent related to Meniere’s disease. The role of immune and allergic factors in Meniere’s disease is under active investigation. The ES is able to process antigens and mount a local antibody response. The ES may be vulnerable to immune injury because of the hyperosmolarity of its contents and due to the fenestrations in its vasculature. A significant percentage (50%) of patients with Meniere’s disease have concomitant inhalant and/or food allergy, and treating these allergies with immunotherapy and diet modification has improved the manifestations of their allergy and their Meniere’s disease.

Epidemiology and Natural History

The incidence of Meniere’s disease ranges from 10 to 150 in 100,000 persons per year. There is no gender bias, and patients typically present in the fifth decade of life. Meniere’s disease is characterized by remissions and exacerbations. Longitudinal studies have shown that after 10 to 20 years, the vertigo attacks subside in most patients, and the hearing loss stabilizes to a moderate to severe level (50 dB HL). Meniere’s disease usually affects one ear initially, but the cumulative risk of developing Meniere’s disease in the other ear appears to be linear with time.

Diagnostic Evaluation

Meniere’s disease is a clinical diagnosis. Audiologic assessment initially shows a low-frequency or low- and high-frequency (inverted V) sensorineural hearing loss. As the disease progresses, there is a flat sensorineural hearing loss. With electrocochleography, the cochlear microphonic, summating potential (SP), and eighth nerve action potential (AP) can be measured. In Meniere’s disease, the SP to AP ratio is often increased but lacks the specificity or sensitivity to diagnose Meniere’s disease consistently or predict its clinical course. Vestibular testing (electronystagmography with caloric test-ing) shows peripheral vestibular dysfunction.

Management

The primary management of patients with Meniere’s disease is a low-salt diet (1,500 mg/d) and diuretics (hydrochlorothiazide) if needed. Acute attacks are managed with vestibular suppressants (meclizine, diazepam) and antiemetic medications (prochloperazine suppository). The majority of patients are controlled with conservative management.

Medically refractory patients with serviceable hearing may undergo intratympanic gentamicin therapy, endolymphatic sac (ES) surgery, or vestibular nerve section. Patients without serviceable hearing may undergo intratympanic gentamicin therapy or transmastoid labyrinthectomy.