tv [untitled] December 28, 2010 7:30am-8:00am PST
girl: when i became, like, 16 years old, i realized, you know, there was something different about me. my friends were going out at night, and i didn't like going out at night at all. there was just something that was holding me back. second girl: when i'm walking down the hallway at school here, i'm always running into people by accident. i bump right into them. woman: i tri t to find out as much as i could about what it is and what it could possibly mean. man: when it's dark at night, my parents wouldn't permit me to go out. second man: i don't feel i have problem being deaf because i have an established communication system
using sign language. third girl: well, i have been able to listen and speak, and to me, that's the greatest thing in the world. narrator: these individuals have a genetic disorder called usher syndrome. each was born with hearing loss, often complete deafness, and all have retinitis pigmentosa, a condition that causes slow, progressive loss of vision.
the typical case, ok, is one, a son or a daughter, originally diagnosed as having a hearing loss and then eventually within several years later, usually as a preteen or teen, diagnosed with the retinitis pigmentosa, and the family is thunderstruck because there's nobody else in the family with a hearing loss. there's nobody else in the family with any kind of visual problems other than grandma, who had cataracts. so, how could this happen? narrator: paul molloy has usher syndrome. he is profoundly deaf and legally blind. he and his wife, who is also deaf, have two children who are hearing and sighted.
paul lives on long island and commutes daily to manhattan, where he works at columbia university. paul learned he had usher syndrome while attending college when he was 19 years old. at this point in his life, he has only a pinhole of vision left and uses a cane to assist him with travel. with usher syndrome, your vision is always changing, especially as you get older. some of these changes will have a significant impact on your life. i call these changes hits. for example, in your teen years, you start to experience night blindness where you lose the ability to travel independently or safely at nighttime. narrator: lauren is 13