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DEAF & HARD-OF-HEARING

AUDIOLOGISTS' CORNER :: USHER SYNDROME

WHAT IS USHER SYNDROME?

Usher Syndrome is a genetic disorder characterized by serious hearing impairment, present at birth or shortly thereafter, and progressive vision loss due to Retinitis Pigmentosa (RP). Researchers believe that the loss of hearing is sensorineural deafness and is due to a problem with the sensory (nerve) cells in the cochlea of the inner ear. The vision loss begins later in life, but usually before adolescence. There is no way of knowing the time of onset or the rapidity of the vision loss. Usher Syndrome is a very difficult disease to describe because each case is so different. The changes in hearing and vision are not the same for all people who have Usher Syndrome.


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