Usher's Syndrome: Growing Up with a Deaf and Blind Mom

Growing up, my mother did not hear very well, she never saw anything unless she was staring directly at it either. For a child, this had its advantages and disadvantages. Me and my sisters would always say things under our breath and get away with it. In order to hear someone, my mom would have to stare directly at your mouth to read you lips. Her famous funny quote was; "Turn on the lights I can't hear!" When I was in my teenage years I completely took advantage of her disability. I could walk in the house holding a case of beer and as long as I kept talking to her, she would stare at my mouth and not look down to see what I was holding.

My mother was born with Usher's Syndrome. Usher's Syndrome is an inherited condition that involves loss of hearing and progressive vision loss due to retinitis pigmentosa (RP), which is the degeneration of the retina, the light sensitive tissue in the back of the eye crucial to vision. Usher's syndrome is the major cause of deaf blindness and about 10-15,000 people in the US have it.

Usher's Syndrome is passed down genetically by an autosomal recessive gene. Autosomal means that the gene is not link by sex so both males and females are equally affected. Two copies of the usher's syndrome gene are needed in order for a person to have the syndrome. An individual can have only one copy of the gene from one parent and just be a carrier, without the symptoms.

Basically, your hearing is bad and your vision closes in on you, at least this was the case for my mother. My mother stopped driving at night when I was about 8 or 9, and completely stopped driving when I was 14, she was about 35 then. I think she was driving way past the time she was supposed to but I too would be reluctant to give up that freedom. Her peripheral vision was closing in on her, it was like looking though a tunnel. Slowly her range of vision became smaller and smaller. Her balance has been affected also. A couple years ago a cop saw her walking down the street after dark and asked her is she was drunk, she told him that she could not see, he let her go.

Today my mother is classified as legally blind. When she is looking at my mouth to read my lips she can not see my eyes. She has had a cane for years but rarely uses it. Sometimes she will use the cane at night or if she is going somewhere she is not familiar, and also when she's in a bad mood. She has a sense of humor about it, whenever there is a busy street to cross she will whip out the cane and all the cars will immediately stop. God forbid you hit a blind person, no one will win that case.

For more information on Usher's Syndrome:
National Eye Institute http://www.nei.nih.gov/health/ushers/index.asp
The Foundation Fighting Blindness http://www.blindness.org/content.asp?id=47
Boys Town Research Hospital http://boystownhospital.org/Usher/information.asp