Deaf And Blind
By Tyler Brooker, Thu Dec 8th
Usher syndrome - "a genetic disease causing and blindness."A simple definition. In essence, this disease slowly blinds youby progressive retinis pigmentosa, as well as ruin your hearingthrough congenital hearing loss. This particular disease canonly be inherited, and is uncommon becuase it is a recessivetrait. It can occur in an estimated 1 in 11,000 people.
"UsherSyndrome" is not a name which best describes or reflects itssigns and symptoms. It was named this becuase of C.H. Usher, aBritish ophthalmologist. He wrote about the disease when it wasunknown, and described cases in which there was a clear linkbetween congenital deafness and retinis pigmentosa. This waslong ago, and he was ahead of his time, as the paper was writtinin 1914.
This condition has three seperate types; Usher Syndrome I, IIand III. I is the most unfair, as the child is born almostcompletely deaf, and eyesight begins to fail after about age 10.In type II, eyesight does not tend to degrade until later inlife, but the child is born hard of hearing. Type III syndromeis the most common, wherein loss of sight and hearing occursmuch later in life.
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The USH3A gene is responsible for creating a protien that isessential in the development and upkeep of the retina and innerear. Usher syndrome type III is a result of a mutation whithinthis gene, however, the full explanation as to the protiens rolein vision and hearing loss, is yet to be discovered.
About the author:Feel free to reprint this article as long as you keep thearticle, this caption and author biography in tact with allhyperlinks.
Tyler Brooker is the owner and operator of UsherSyndrome - http://www.usher-syndrome.com, which is the bestsite on the internet for all Usher Syndrome related information.
