Ménière's disease Ménière's disease Ménière's disease (MD) is a disorder of the inner ear that is characterized by episodes of feeling like the world is spinning (vertigo), ringing in the ears (tinnitus), hearing loss, and a fullness in the ear. Typically only one ear is affected, at least initially; however, over time both ears may become involved. Episodes generally last from 20 minutes to a few hours. The time between episodes varies. Over time the hearing loss and ringing in the ears may become constant. The cause of Ménière's disease is unclear but likely involves both genetic and environmental factors. A number
Refsum disease acid metabolism in the liver of mice. Individuals with Refsum disease present with neurologic damage, cerebellar degeneration, and peripheral neuropathy. Onset is most commonly in childhood/adolescence with a progressive course, although periods of stagnation or remission occur. Symptoms also include ataxia, scaly skin (ichthyosis), difficulty hearing, and eye problems including retinitis pigmentosa, cataracts, and night blindness. In 80% of patients diagnosed with Refsum disease, sensorineural hearing loss has been reported. This is hearing loss as the result of damage to the inner ear or the nerve connected to ear to the brain. Refsum disease is a peroxisomal disorder caused by