Type 1 Usher syndrome is caused by an autosomal recessive gene. Two genes that cause it are USH 1B on the long
arm of chromosome 11 and USH 1C on the short arm of chromosome 11. (Only one defective gene is needed to obstain Type 1 Usher
syndrome). Usher syndrome effects the eyes and ears through a single gene.
Type 1 Usher syndrome causes ineffective semicircular canals in the ear, making balance difficult. However it
also prevents dizziness. This causes difficulty in learning to crawl and walk. Those with Type 1 Usher syndrome are also
extremely hard of hearing and cannot distinguish speech. Most use sign language as a means of communication. Retinis
Pigmentosa also occurs due to Type 1 Usher syndrome. Night blindness also occurs at infancy because the rods are effected
first. Then vision becomes tunnel vision as the cones begin to ruin in the late childhood or early teenage years, and eventually
blind spots appear until central vision loss occurs. This is compensated for by scanning or moving the eyes rapidly until
vision is lost. Also the vision that is seen may be a smaller view, but it can still be perfect vision.
Because Retinitis pigmentosa and loss of hearing are rarely found together, having both points to Usher's
syndrome. An ENG which detects balance problems and an ERG which detects retinitis pigmentosea are tests used to diagnose
Usher's Syndrome early on.
The treatment available for Usher's syndrome is solely educational programs that allow those with Usher's syndrome
to carry out normal tasks and deal with their disabilities. In cases that are not as severe devices that aid in hearing may
work. Basically, teaching those with Usher's syndrome to live productively with their disabilities is the main treatment.
