Usher Syndrome

Usher Syndrome is a genetic disease that affects the sight and hearing of children; in its most severe form, children are born profoundly deaf and begin to lose their sight by age ten. People with Usher Syndrome Type III are born with normal sight and hearing, but slowly become blind and deaf. There is no treatment or cure, and unless there is family history of the disease, identifying people who are carriers of the gene is as yet very difficult.MSNBC: Peter Alexander on his sister, who is losing both her sight and hearing (March 20, 2009)

Usher Syndrome is an inherited disease, and passes from parent to child. To inherit Usher, a child must get the recessive gene from both parents. People with an Usher Syndrome mutation in a gene may be carriers, in that they do not show symptoms but may pass it along to their children. Genetic tests for people without family history of Usher Syndrome are not typically done, because the disease is known to be seated in several genes and may be located in others unknown as yet.NIH: Usher Syndrome Genetics Home Reference: Usher syndrome

• Type 1: Children who have Type 1 Usher Syndrome are profoundly deaf at birth and have severe balance problems. They are slow to sit up and may not walk independently until they are 18 months old. Vision problems develop by age 10, starting with night blindness; they rapidly go completely blind. Genetics Home Reference: Usher syndrome

• Type 2: Type 2 Usher Syndrome children are born with moderate to severe hearing loss and normal balance. Vision problems progress more slowly than Type 1.NIH: Usher Syndrome

• Type 3: Children with Type 3 Usher Syndrome have normal hearing at birth. Hearing and sight worsen over time, but the rate of decline varies.NIH: Usher Syndrome Genetics Home Reference: Usher syndrome

The content in this page is not a substitute for professional medical advice. If you think your child has Usher Syndrome, please make sure to consult your doctor.

1. NIH: Usher Syndrome
2. Genetics Home Reference: Usher syndrome
3. MedicineNet: Usher Syndrome
4. Boys Town: Information About Usher Syndrome
5. MSNBC: Peter Alexander on his sister, who is losing both her sight and hearing (March 20, 2009)
6. Emax Health: Study: Clues To Beating Hearing Loss (March 6, 2009)
7. Wikipedia: Usher syndrome

• <iframe height="339" width="425" src="http://www.msnbc.msn.com/id/22425001/vp/29741456#29741456" frameborder="0" scrolling="no"></iframe><style type="text/css">.msnbcLinks {font-size:11px; font-family:Arial, Helvetica, sans-serif; color: #999; margin-top: 5px; background: transparent; text-align: center; width: 425px;} .msnbcLinks a {text-decoration:none !important; border-bottom: 1px dotted #999 !important; font-weight:normal !important; height: 13px;} .msnbcLinks a:link, .msnbcLinks a:visited {color: #5799db !important;} .msnbcLinks a:hover, .msnbcLinks a:active {color:#CC0000 !important;} </style>

  Visit msnbc.com for Breaking News, World News, and News about the Economy