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Ushers Syndrome

What is Ushers Syndrome?

Ushers Syndrome is the most common condition affecting both vision and hearing. It is a rare genetic disorder that is the leading cause of deaf and blindness. Devices that magnify and read are most suited to those with Usherss Syndrome.

The eye disease can be divided into three clinical types:

Ushers type I

People with Ushers type 1 are born deaf. Vision normally begins to decline from the age of 10. People with Ushers type 1 often also have balance problems and have difficulty learning to walk.

Ushers type II

People with Usher type II are not born deaf, but are hearing impaired. They have no visible problems with their balance and begin to experience visual impairment after the age of 20.

Ushers type III

People with Ushers type II are not born deaf. They only have to deal with complaints such as hearing loss and visual impairment at a later age.

The Syndrome is a variable condition, where the severity of the abnormality is not necessarily determined by the clinical type one has. Of the people who have Retinitis Pigmentosa, approximately one-sixth have Ushers Syndrome.

The eye condition is a hereditary condition, which means that it is genetically determined. It is a recessive condition, so both parents must have this gene for a child to have it. Being told that vision is affected by Ushers Syndrome can turn the world upside down.

When the first emotions subside, the knowledge that one is not alone may provide support. There are many people in the same situation, some who have just been diagnosed themselves and some who have been living with it for years.

Dealing with Usher Syndrome

Many people with Ushers Syndrome can continue to do most activities. In most cases there is still peripheral vision (vision on the sides). With the use of specific tools it is often possible to continue doing the things one loves.

If you experience visual impairment as a result of Ushers Syndrome, there are aids available that can help with the tasks you have difficulty with. These aids can provide support with tasks that require vision and can help you continue to lead a full and independent life.

Image view without and with Ushers Syndrome
Left: normal vision. Right: view with Ushers Syndrome

What are the symptoms of Ushers Syndrome?

Symptoms include:

What can one expect from Ushers Syndrome?

There is no medical treatment that can prevent it. The best treatment involves early diagnosis so that retraining and therapies can begin as soon as possible. The precise implementation of such programs depends on the extent to which the Syndrome occurs and the severity of the limitations. In addition to these programs, hearing aids, implants, other communication tools, orientation and mobility training and devices for the visually impaired and/or Braille can also be used.

Learn more about Ushers Syndrome

Extensive information is available. This information is intended to inform you about the most important aspects of this eye condition and is not intended to replace the information you receive from your doctor or eye specialist.

More Information

Sense has some great information on Usher’s Syndrome – Usher syndrome – Sense

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