USHER Syndrome

The major symptoms of Usher syndrome are hearing impairment and retinitis pigmentosa, an eye disorder that causes a person's vision to worsen over time. Some people with Usher syndrome also have balance problems. There are three general types of Usher syndrome - Usher syndrome type 1 (USH1), Usher syndrome type 2 (USH2), and Usher syndrome type 3 (USH3). USH1 and USH2 are the most common types.

Approximately 3-6 percent of all deaf children and perhaps another 3-6 percent of hard-of-hearing children have Usher syndrome. In developed countries such as the United States, about 4 babies in every 100,000 births have Usher syndrome.

Presently there is no cure for Usher syndrome. Researchers are currently trying to locate the genes that cause the syndrome and identify the function of those genes. This research will lead to improved genetic counseling and early diagnosis, and may eventually expand treatment options. The genes that cause Usher syndrome are MY07A, USH1C, CDH23, PCDH15, and SANS, which cause USH1; USH2A, which causes USH2; and USH3A, which causes USH3.

For further information we recommend to read:

The Foundation Fighting Blindness > Macular Degeneration : Usher Syndrome > What is Usher syndrome?