Usher Syndrome

What is Usher Syndrome?

Usher Syndrome is a genetic condition which causes hearing loss from birth, and a later progressive loss of vision. The condition was named after CH Usher, a British opthamologist. There are three types of Usher Syndrome - I, II and III.

Symptoms

Each type presents slightly differently – hearing problems will vary and some people will also experience difficulties with balance. In all cases sight deteriorates as a result of Retinitis Pigmentosa (RP). This is the name given to a group of hereditary eye disorders. They damage the retina, the light sensitive tissue lining the back of the eye, in which the first stages of seeing take place. . As a result, deaf people in their teens and twenties may seem clumsy and, for instance, bump into lamp posts or fall down steps.

The first sign of RP is usually night blindness or temporary blindness when going from a dark to a bright place, for example moving outdoors. As RP progresses, the field of vision narrows until only the central vision remains – this is known as ‘tunnel vision.’ The degree of sight loss and the rate of decline vary depending on the individual. Most people with RP retain some useful, central vision into old age.