Living with Usher - Facts and Fiction

Facts and fiction about Usher syndrome

I got Usher from my mother or my father.


Usher is a recessive genetic condition. This means that both parents either have Usher, or carry a specific gene for Usher, in order for you to be affected.

I have Usher because my mother was ill during pregnancy.
When a woman has an infection such as rubella during pregnancy, this may cause sight and hearing problems in some children. Usher is different. It is not caused by illness during pregnancy. It is a genetic condition.

I have Usher. If I have children, they will also have Usher.
It is possible, but very unlikely
In order for your children to have Usher, your partner must either have the same type of Usher as you or be a carrier of the same gene. A child will not get Usher syndrome from only one parent so it is very unlikely that your children will be affected. Anyone with Usher thinking about having a family may wish to get advice from a genetic counsellor. 

There is no cure for Usher.
At present there is no cure for Usher and no way of preventing the condition. However, much research is taking place and as doctors and scientists start to understand more about the condition and the genes that cause it, treatment may become available. This may take several years. Researchers are currently trying to find treatments to stop retinitis pigmentosa (RP) from getting worse.

People with Usher will definitely go blind.
Usher is an individual condition – everyone is different. The extent to which vision changes and the time this takes is variable, but is probably linked to the type of Usher you have. Many people with Usher have tunnel vision and problems seeing at night, but their central vision may remain good for a long time. This means that while they may have difficulty with mobility, they can still see to communicate, read and do other tasks.
A person with Usher may be registered bind (or severely sight impaired) because of their tunnel vision, but may still have useful central vision.
An ophthalmologist (eye doctor) will be able to tell you more. 

People with Usher syndrome may get other eye problems.
Some people with Usher may get other eye problems as well as RP. Such problems can include cataracts or macular oedema. 
When the clear lens in the front of the eye becomes cloudy, this is called a cataract.  Although cataracts can affect anyone, people with Usher are more likely to develop them at an earlier age - maybe in their thirties or forties.  Symptoms of a cataract include increased problems with glare, “halos” around, for example, street lamps and an increased fogginess of vision.  Cataracts can be surgically removed which will usually improve vision.  However, the symptoms of the underlying RP will remain.
Macular oedema is rarer than cataracts. It occurs when part of the retina swells with fluid. This may be treated by medication but success is not guaranteed. The swollen part of the retina will not function properly and will cause some loss of sight in that part of the visual field.
It is important to have your eyes checked regularly and to report any problems to your ophthalmologist.  If you develop eye problems suddenly, go straight to your nearest hospital accident and emergency department.
Sense and the National Deaf Children’s Society have published useful guidance for families and professionals about how the vision of children with hearing impairments can be assessed and looked after. For a copy of Vision Care for your Deaf Child contact Sense or visit our website.

People with Usher will always be dependent on other people.
Many people with Usher lead independent and fulfilling lives. They may go to college or university, have a partner and a family and have a job or career. However, they may require additional support at times - for example a sign language interpreter, or a sighted guide when travelling after dark. 

People with Usher cannot go out on their own due to their poor eyesight.
Many people with Usher travel independently. As their vision changes, some people benefit from using a mobility aid, such as a red and white cane, to help them move around safely and independently. Your local Rehabilitation Worker with Visually Impaired People (ROVI) will be able to provide advice and safety tips and also give you a mobility aid if you agree that this will help.
It is your choice whether you use an aid.

Some people with Usher should not drive.
It is dangerous to drive if you cannot see well at night, have tunnel vision or your eyes take time to adapt between different lighting conditions.
The eye clinic or the Driver and Vehicle Licensing Agency (DVLA) can provide more information and also advise on whether you are legally entitled to drive. People who continue to drive after they have been told to discontinue driving will invalidate their insurance in the event of an accident and are also putting other road users, and themselves, at risk of injury or death.

People with Usher must give up their job when their sight deteriorates. 
Access to Work is a scheme operated by the government to support disabled people to get employment, or stay in their job if their condition changes. Depending on the assessment, funding can be available to pay for equipment, such as a special computer, or to pay for support, such as a BSL interpreter or a Communicator Guide. Employers must make “reasonable adjustments” in the workplace to enable the disabled person to do their job. Disability Employment Advisors (contactable through the local Job Centre) can provide more information. 

People with Usher cannot care for themselves or look after their own children.
Most people with Usher continue to care for their children, prepare meals, clean their home and manage their own personal care (washing and dressing). If any of this becomes difficult, the person (or their partner or family) may ask for an assessment by their local Social Services or Adult Services Department. They may be eligible for aids or adaptations to help (for example, a vibrating baby monitor) or additional support (for example, a Communicator Guide or a home care worker).

Updated: February 2016
Review due: February 2018

First published: Thursday 4 February 2016
Updated: Monday 16 January 2017