What is Usher syndrome?

Usher syndrome is an inherited, genetic condition. The main symptoms are hearing loss and sight loss, due to a condition called retinitis pigmentosa (RP) and, in some cases, balance difficulties.

Everybody with Usher syndrome experiences the condition in a different way.

The time of onset, as well as how your sight, hearing and balance are affected, varies from person to person. It also changes over time.

The main symptoms of Usher syndrome are hearing loss and an eye disorder called retinitis pigmentosa (RP).

RP causes nightblindness and a loss of peripheral vision through the progressive degeneration of the retina. As RP progresses the field of vision narrows – a condition sometimes known as ‘tunnel vision’ – until only the ability to see straight ahead remains.

If you have Usher syndrome, you may also have balance problems.

There are three types of Usher syndrome.

Usher type 1

People with Usher type 1 are usually born with profound hearing loss in both ears.

Balance problems are also common. Babies may show delays in sitting and walking, and experience disorientation throughout their life, particularly as their sight changes.

Usher type 1 is also associated with the development of nightblindness. This occurs in the first 10 years of a child’s life, progresses to tunnel vision and further sight problems associated with RP.

A person’s primary communication method is typically British Sign Language (BSL), though this may change as their sight deteriorates.

Usher type 2

People who have Usher type 2 are usually born with a mild to severe hearing loss in both ears. This is typically within the higher frequency ranges.

Type 2 is not associated with balance problems, which is a characteristic that distinguishes it from type 1.

Usher type 2 is characterised, in most cases, by the development of retinitis pigmentosa (RP) in the teens or early twenties.

A person’s primary communication method is usually speech, often assisted by hearing aids and lipreading.

Usher type 3

Usher type 3 is characterised by gradual sight and hearing loss, which occurs later in life after a person has learnt to speak.

Some people may develop severe hearing loss while others may not. 

People with Usher type 3 typically have problems with their balance. It’s common for people to be misdiagnosed as having type 1 if they demonstrate balance problems and severe hearing loss

A person’s primary communication method is usually speech, often assisted by hearing aids and lipreading.

Atypical Usher

Not everyone who has Usher syndrome fits into these three clinical types. People may have varying symptoms that are difficult to diagnose as a specific Usher type. Cases like this are called atypical. 

A 2010 study found that there are approximately 9,750 people in the UK with Usher syndrome.

Communication methods and Usher

The way you communicate will depend on how much your sight and hearing has been affected by Usher syndrome.

From sign language and deafblind manual, to speech and gestures, there are many different formal and non-formal methods of communication, which can be used to express yourself and communicate with other people.

Contact the Sense Usher Service

The Sense Usher Service specialises in Usher syndrome and can support you, your family and professionals.

With the right support, you can overcome the challenges presented by Usher, and live a full, connected and independent life.

Contact us on info@sense.org.uk or usher@sense.org.uk for more information. You can also follow us on Facebook.

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