Usher Syndrome

Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder that causes your vision to worsen over time. It is the most common condition that involves both hearing and vision problems.

There are three types of Usher syndrome:

• People with type I are deaf from birth and have severe balance problems from a young age. Vision problems usually develop by age 10 and lead to blindness.
• People with type II have moderate to severe hearing loss and normal balance. Vision problems develop in the early teens and progress more slowly than in type I.
• People with type III are born with normal hearing and near-normal balance but develop vision problems and then hearing loss.

There is no cure. Tools such as hearing aids or cochlear implants can help some people. Training such as Braille instruction, low-vision services or auditory training can also help.

National Institute on Deafness and Other Communication Disorders

Usher Syndrome on Wikipedia

'''Usher syndrome''' is a genetic disease causing deaf-blindness. It is essentially progressive retinitis pigmentosa combined with congenital hearing loss. It is almost always inherited in an autosomal recessive pattern and is estimated to occur in 1 in 10,000 people. Whilst this is a rare genetic condition it represents the major cause of syndromic deafness with blindness. The condition gets its name from British ophthalmologist, C.H. Usher, who in 1914 wrote a paper describing several cases in which the link between congenital deafness and retinitis pigmentosa was stressed. Usher syndrome is divided into three types, I, II and III. Children with type I syndrome are born profoundly deaf, and eyesight usually begins degrading after the first decade of life, beginning with night-blindness. If identified at a young age, children usually receive a cochlear implant, and generally learn spoken language. Sign language is also sometimes used, though when vision loss becomes severe one must revert to tactile signing. Problems with balance are usually present, due to the failure of the Hair_cells_%28ear%29 of the inner ear. Type II children are hard-of-hearing, and changes in sight usually begin later, first becoming noticable after the second decade of life. In the type III syndrome, hearing loss as well as ''retinitis pigmentosa'' can occur later in life.

Usher syndrome I

External Resources

Usher Syndrome - A description of this disease from Retinal Preservation Foundation of South Africa.

A-Z Deafblindness - An in depth article by Mary Guest, Head of Usher Services at Sense, about Usher syndrome. Includes detailed description of what it is, the symptoms, genetics, transmission, the problems associated with it and what can be done.

Croatian Association for the Deafblind Persons Dodir - About Croatian deafblind people, news, information about deafblindness and Usher syndrome, photo album. [Croatian, English]

Australian DeafBlind Council (ADBC) - Information on Usher's syndrome. Membership includes people with deafblindness, family, carers, support workers, professionals and service organisations.

RetNet - Retinal Information Network - Provides tables of genes causing inherited retinal diseases, such as retinitis pigmentosa, macular degeneration and Usher syndrome, and related information.

Pro Retina Deutschland e.V. Infoseite Augsburg - Selbsthilfevereinigung von Menschen mit Netzhautdegeneration, Retinitis Pigmentosa, Makula-Degeneration, Usher-Syndrom. Regionalgruppe Augsburg.

Retina Suisse - Die Schweizer Selbsthilfeorganisation von Menschen mit Retinitis pigmentosa (RP), Makuladegeneration, Usher Syndrom und anderen degenerativen Netzhauterkrankungen informiert über die Erkrankungen und die Organisation.

Usher UK - Voluntary organisation run mainly by people with Usher syndrome, promoting the interests and well-being of all people who have Usher and their families. Information on activities and events.

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