Usher syndrome

Usher syndrome is a genetic disease causing deaf-blindness. It is essentially retinitis pigmentosa combined with progressive hearing loss.

Usher syndrome is divided into three types, I, II and III. Children with type I syndrome are born nearly deaf, and eyesight usually begins degrading around the age 5-10 years, beginning with night-blindness. If identified at a young age, children usually receive a cochlear implant, and generally learn spoken language. Sign language is also sometimes used, though when vision loss becomes severe one must revert to tactile sign language. Problems with balance are usually present, due to the failure of the hair cells of the inner ear. Type II children are hard-of-hearing, and changes in sight begin usually at the age of 5-7. In the type III syndrome, hearing loss as well as retinitis pigmentosa can occur later in life.