Usher Syndrome (Usher) is a genetic disorder combining a moderate to profound hearing loss and Retinitis Pigmentosa commonly known as RP, which causes a progressive loss of vision.

There are three main types of Usher Syndrome identified at present.

• Born with a profound hearing loss.
• Balance problems.
• Develop night-blindness in their teens or earlier, due to Retinitis Pigmentosa.

• Born with a moderate to severe hearing loss.
• Develop night-blindness in their twenties, due to Retinitis Pigmentosa.
• No balance problems in childhood.

• Hearing loss that gets progressively worse over time.
• Develop visual impairment around the age of 20-30 years due to Retinitis Pigmentosa.
• May have balance problems later.

People, who have Type 1 Usher will often be sign language users and may attend a school for deaf children.

People who have Type 2 Usher will normally have speech, may or may not use sign language and may attend a mainstream school or school for the deaf children.

Type 3 Usher is very rare.

A small number of people who have type 3 Usher have speech and do not normally sign. They would normally have attended a mainstream school.

RP is an eye condition which causes a gradual loss of vision. The eye becomes less able to adjust to low light, resulting in night blindness. As RP progresses, the field of vision narrows until only central vision remains. This is called "Tunnel Vision". Many people with Usher Syndrome will retain at least some central vision for a long time.