Ménière's disease is an abnormality of the inner ear causing a host of symptoms, including vertigo, tinnitus, fluctuating hearing loss, and the sensation of pressure or fullness in the affected ear. The disorder usually affects only one ear at onset but the other ear can eventually be affected in up to 30 percent of patients. This syndrome was named after a French physician, Prosper Ménière who first described it in 1861.
The symptoms of Ménière's disease are associated with a change in fluid volume within a portion of the inner ear known as the labyrinth. The labyrinth has two parts: the bony labyrinth and the membranous labyrinth. The membranous labyrinth, which is encased by bone, is necessary for hearing and balance and is filled with a fluid called endolymph. An increase in endolymph causes the membranous labyrinth to balloon or dilate, a condition known as endolymphatic hydrops. The exact cause of hydrops is unknown. It is possible that too much fluid is produced, or that its normal circulation and absorption are blocked. Scientists are investigating several possible causes of the disease, including genetics, anatomy, viral infections and environmental factors.
The symptoms of Ménière's disease are highly variable. The cochlear symptoms include fluctuating hearing loss, tinnitus and ear pressure. In the early stages, a person's hearing tends to recover between attacks but a permanent hearing loss eventually develops which then progressively worsens over time. The vestibular symptoms include attacks of vertigo, nausea, and vomiting, which are usually disabling and last for several hours. Diarrhea and sweating may also occur. Some patients experience milder episodes and become dizzy and imbalanced but don’t vomit. People experience these symptoms at varying frequencies, durations, and intensities. Some may feel mild symptoms only a few times a year while others can be debilitated with imbalance and deafened by frequent attacks.
Meniere’s disease is a clinical diagnosis that sometimes requires time and observation to diagnose. The diagnosis of Ménière's disease begins with a medical history, a physical examination and careful audiometric testing. Documentation of a fluctuating hearing loss is the cornerstone of the diagnosis. Accurate measurement and characterization of hearing loss are of critical importance and audiograms are frequently repeated over time and after attacks to conclude the diagnosis. Other electrophysiological tests of the inner ear can also be helpful. Metabolic conditions and tumors are ruled out with blood work and MRI. Specialized MRI imaging of hydrops is possible in uncertain cases. A VNG test (videonystagmography) can help evaluate the balance status of the inner ear. Other balance tests are sometimes ordered.
There is no known cure for Ménière's disease. The symptoms are often controlled successfully by reducing the body’s retention of fluids through dietary changes (a low-salt and no caffeine diet) and medications. Diuretics are the most frequently prescribed drugs for Meniere’s. Betahistine, allergy medications, steroids and vasodilators are sometimes used. Eliminating tobacco use and reducing stress levels are also important. Steroids are frequently used to try to recover the hearing if there has been a significant recent drop in hearing. These can be taken orally or injected into the ear transtympanically. The administration of the ototoxic antibiotic, gentamycin directly into the middle ear space is very useful to stop vertigo attacks if the hearing is poor. The Meniette device is sometimes prescribed for people with Meniere’s disease who are not good candidates for an endolymphatic sac surgery, or who are medically not suitable for surgery.
Different surgical procedures have been advocated for patients with persistent, debilitating vertigo from Ménière's disease. An endolymphatic sac decompression and shunt insertion can help improve Meniere’s disease in up to 75% of cases. Labyrinthectomy (removal of the inner ear sense organ) can effectively control vertigo, but sacrifices hearing and is reserved for patients with nonfunctional hearing in the affected ear. Vestibular neurectomy, selectively severing a nerve from the affected inner ear organ, usually controls the vertigo while preserving hearing.
Meniere's Disease Surgery
