What is Usher syndrome?
Usher syndrome is an inherited disorder that involves both a hearing impairment and a vision impairment called retinitis pigmentosa. Retinitis pigmentosa is a progressive degeneration of the retina that causes night blindness and reduction of peripheral vision (side vision). Some people also have problems with balance.
Usher syndrome is passed from parents to their children through genes.
What are the different types of Usher syndrome?
There are 3 types of Usher syndrome:
US type 1 (US1) characteristics include:
Totally deaf from birth
Do not usually benefit from hearing aids
Severe balance problems
Vision problems begin by age 10
Blindness eventually occurs
US type 2 (US2) characteristics include:
Moderate to severe hearing problems
Usually benefit from hearing aids
Use speech to communicate
Retinitis pigmentosa begins in teenage years
US type 3 (US3) characteristics include:
Born with normal hearing
Hearing problems develop in teenage years
Near normal balance
Deafness by late adulthood
Retinitis pigmentosa begins around puberty
Blindness by mid-adulthood
How is Usher syndrome diagnosed?
Special tests assist in the diagnosis of Usher syndrome, including:
Electronystagmography (ENG) to find balance problems
Electroretinography (ERG) to find retinitis pigmentosa
Audiologic (hearing) evaluation and testing
Treatment for Usher syndrome
Specific treatment for Usher syndrome will be determined by your health care provider based on:
Your age, overall health, and medical history
Extent of the disease
Your tolerance for specific medicines, procedures, or therapies
Expectations for the course of the disease
Your opinion or preference
At present, there is no known cure for Usher syndrome. The best treatment, however, is early identification so that education programs can begin as soon as possible. Treatment may include:
Devices, like hearing aids
Orientation and mobility training
Independent living training
Low vision services
Last Reviewed Date: 04/29/2015
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