Usher syndrome

Usher syndrome is a major cause of deafblindness in adults. People with Usher have a genetic condition which means they are born deaf or hard of hearing then gradually start to lose their sight.

The sight loss often begins in late childhood and is caused by an eye condition known as Retinitis Pigmentosa (RP). With RP, the retina slowly degenerates and loses its ability to transmit pictures to the brain.

The early symptoms of Usher include difficulty seeing in the dark and in different lighting conditions. Over time vision gradually deteriorates until tunnel vision develops. Many people do not realise they have Usher until they begin to have problems with their sight. They will have grown up as deaf or partially hearing, and coming to terms with losing their sight as well can be very hard.

Often, the communication skills people with Usher Syndrome learned as children may be less effective or ineffective as they gradually lose their sight, therefore they need to learn new skills appropriate for their abilities.