Deafness progressive cataract autosomal dominant

Cochleosaccular degeneration with progressive cataracts


Type of disease: Rare conditions

Deafness progressive cataract autosomal dominant is a rare inherited disorder. People with this disorder have progressive hearing loss as well as progressive cataracts. The type of hearing loss is sensorineural and is due to the breakdown overtime to parts of the cochlea. The cochlea is the snail-shaped structure which aids in hearing by changing the vibrations we hear into signals that can be sent to the brain through our nerves. As the cochlea degenerates, hearing loss develops. Eventually the individual will become completely deaf. There are a number of options available for individuals with deafness or sensorineural hearing loss, including hearing aids and/or cochlear implants. Parents should seek information about the range of options open to their child before deciding which options may work best for their child and family. Specialists may include an otologist, an audiologist, a speech-language pathologist, a clinical geneticist, and a genetic counselor. Support groups are also good resources of support and information. Cataracts are the clouding of the lens in the eye and it can occur in one or both of the eyes. In a normal eye, the lens is transparent and focuses light passing through the eye onto the retina, a light sensitive tissue in the back of the eye. The retina then converts the light into nerve signals, which are sent to the brain to produce a sharp image. Cataracts cause the lens to be cloudy and as a result, produce blurred images. Symptoms of a cataract include blurry vision, glares surrounding light, poor night vision, and double vision. A physician can detect cataracts through a comprehensive eye exam. Treatment for cataracts usually involves surgery to remove the old lens and replace with an artificial lens.

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