Ella+Miller-Ferr.+Bio

**Usher Syndrome** // A genetic condition that causes hearing and vision loss. //  **Description**: Usher syndrome is the most common condition that affects both hearing and vision. A syndrome is a disease or disorder that has more than one feature or symptom. **Initial Symptoms:** Lack of balance, hearing and sight are all common signs of Usher Syndrome. Most children are diagnosed once these childhood developments do not show up. ** Common Treatments: ** Some ophthalmologists believe that a high dose of vitamin A palmitate may slow, but not halt, the progression of retinitis pigmentosa. **Side effects of disease:** Children with type 1 Usher syndrome are profoundly deaf at birth and have severe balance problems. Many of these children obtain little or no benefit from hearing aids. Children with type 2 Usher syndrome are born with moderate to severe hearing loss and normal balance. Although the severity of hearing loss varies, most of these children can benefit from hearing aids and can communicate orally. The vision problems in type 2 Usher syndrome tend to progress more slowly than those in type 1 **Prevention and control:** Currently, there is no cure for Usher syndrome. The best treatment involves early identification so that educational programs can begin as soon as possible. **What chromosome is the disease associated with:** 11 genetic loci (a segment of chromosome on which a certain gene is located) have been found to cause Usher syndrome, and nine genes have been pinpointed that cause the disorder. **How is the disease inherited**: Usher syndrome is inherited, which means that it is passed from parents to their children through genes. To have Usher syndrome, a person must receive a mutated form of the Usher syndrome gene from each parent. If a child has a mutation in one Usher syndrome gene but the other gene is normal, he or she is predicted to have normal vision and hearing. **Populations with predispositions to the disease:** There is an unusually high incidence of Usher syndrome in the French-Acadian population that was resettled in Louisiana after France ceded Canada to Great Britain. **Extra fact:** Approximately 3 to 6 percent of all children who are deaf and another 3 to 6 percent of children who are hard-of-hearing have Usher syndrome. In developed countries such as the United States, about four babies in every 100,000 births have Usher syndrome.

__**Work Cited**__

"Usher Syndrome." National Institute on Deafness and Other Communication Disorders [NIDCD]. National Institue of Deafness, 13 Dec. 2009. Web. 01 Feb. 2012. [].

"Fine Mapping of the Usher Syndrome Type IC to Chromosome 11p14 and Identification of Flanking Markers by Haplotype Analysis." Molecular Vision. 16 Apr. 2007. Web. 01 Feb. 2012. [].

"Usher Syndrome (Usher's Syndrome) Causes, Symptoms, Diagnosis, and Treatment on MedicineNet." Medicinenet.com. Medical Association of America, 24 June 2011. Web. 01 Feb. 2012. .