Usher Syndrome is a genetic condition which causes deafness from birth and sight loss over a number of years. This sight loss often begins in late childhood and is caused by an eye condition known as Retinitis Pigmentosa (RP). This is a major cause of sight loss in people under 40. The early symptoms include difficulty seeing in the dark and in different lighting conditions. Over time vision gradually deteriorates until tunnel vision develops.

How is it passed on?

Usher Syndrome is an inherited condition which is passed through the family. It is possible to be a carrier of Usher Syndrome but be unaffected by the symptoms. Often parents are unaware that they are carriers until their child is diagnosed as having the condition. For the child to be born with Usher Syndrome, both parents must either be carriers of the faulty genes, or have the same type of Usher Syndrome themselves.

Living with Usher Syndrome

Usher Syndrome is one of the most challenging disabilities that anyone can face. People with Usher are born deaf or hard of hearing, then gradually start to lose their sight in late childhood.

But despite the enormous challenge this brings, people with Usher can achieve remarkable things. With the right support many people can go to college, get jobs, find a partner and enjoy their leisure, just like anyone else.

Learning new skills

With the right training and support, people with Usher Syndrome can be taught to minimise the effects of their new disability. And they can learn new skills which will help them to cope with life when their sight gets worse.

Many people born deaf with Usher will have learned to use Sign Language to communicate. As their sight narrows they will still be able to use and understand sign, provided people sign to them within their remaining visual field. When this remaining vision fails, people may then use tactile, or 'hands-on' signing, by placing their hands over the hands of the signer so that they can feel the signs being used. People born partially hearing with Usher will invariably use speech to communicate.

The Challenge

Since 95% of our understanding of the world comes through what we see and her, people with Usher Syndrome face enormous challenges.

When someone finds out that they have Usher Syndrome the news can be devastating. Many people do not realise they have Usher until they are teenagers and begin to lose 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.

As their field of vision shrinks, people with Usher often feel isolated, not just from the hearing sighted world, but also from their deaf friends. They will often experience communication problems as it becomes increasingly difficult for them to see sign language, or to lipread. Some may have poor balance which can severely affect their freedom to move around safely. As time goes on, it becomes harder to use public transport, to hold down a job and live independently.

How Sense Helps

Sense Usher Services helps people with Usher Syndrome to live as full a life as possible. It also supports their families, carers and the professionals who work with them. The service includes:

Providing advice and support to people with Usher and their families - particularly when the condition is first diagnosed.

Training education, health and social services professionals to understand Usher Syndrome and its effects.

Offering information to people with Usher Syndrome, family and professionals about practical ways to get the most out of life.

Running Ashley House. This is a small family house with low cost adaptations for people with a dual sensory impairment, and is available for short courses, group meetings and assessments.