Usher's+Syndrome

What is Usher Syndrome? Usher syndrome is a genetic disease that causes deafness and progressive degeneration of the retina leading to blindness. The disease is mostly found in the Ashkenazi Jewish people. It is rare but not unlikely to get. There is no proven cure for Usher Syndrome, though they are testing to find one. There are three types of Usher Syndrome. The trait that carries Usher Syndrome is recessive, which makes it rare. Also it is not connected to the sex chromosomes so you can get Usher Syndrome whether you are a boy or a girl. 

What are the types of Usher Syndrome?

This is a picture of a person with Usher Syndrome (left) compared to a normal person (right). The optic nerve (arrow) look very pale, the vessels (stars) are very thin and characteristic pigment, called bone spicules (double arrows).

What are the types of Usher Syndrome? There are three different types of Usher Syndrome. A child born with type one is deaf at birth, posses severe balance problems, and have vision problems that begin in the childhood and generally turn into blindness. Plus they have little to no help from hearing aids and retinitis pigmentosa (RP) does not develop until the teen years. Children born with type two of Usher Syndrome have less severe hearing loss compared to type one; they have slower degeneration of vision, and have normal balance. Children born with type three have normal hearing at birth, their hearing and sight worsen over time, by mid-adulthood most effected people will be legally blind and usually need a hearing aid. Also they may develop balance problems later on. In the USA type 1 and 2 are the most common cases. The carrier rate is approximately 1 in 165 for type 1. The carrier rate is approximately 1 in 95 for type 3.


 * **Usher Syndrome: Type 1, 2, and 3** ||
 * || **Type 1** || **Type 3** || **Type 3** ||
 * **Hearing** || Profound deafness in both ears from birth || Moderate to severe hearing loss from birth || Normal at birth; progressive loss in childhood or early teens ||
 * **Vision** || Decreased night vision before age 10 || Decreased night vision begins in late childhood or teens || Varies in severity; night vision problems often begin in teens ||
 * **Vestibular function (balance)** || Balance problems from birth || Normal || Normal to near-normal, chance of later problems ||

What are the symptoms of Usher Syndrome? Some of the major symptoms of Usher Syndrome are hearing loss and an eye disorder called retinitis pigmentosa also known as RP. RP also causes night blindness and loss of peripheral vision. The out come of losing your peripheral vision is something called tunnel vision. Other minor symptoms are speech problems and balance problems. The balance problems are mostly present in the type one and three.

This is what you would see if you had Retintis Pigmentosa:

Finally, this youtube video contains a real life person with Usher Syndrome, it involves basc info and interviews with a person with Usher Syndrome. __[]__

I got my information from [|www.knowyourgenes.org] and [|www.ushersyndrome.nih.gov/whatis]

By: Tori Fusco<33

