Usher Syndrome
Usher Syndrome Causes
Usher syndrome is inherited. Meaning, it is passed from parents to their children through genes. It is inherited as an autosomal recessive trait, which means that either mother or father can have the disorder and pass it on to their offspring.
Usher Syndrome Definition
Usher syndrome is probably the most common condition that affects both hearing and vision. Usher syndrome also has three clinical types: type 1, type 2, and type 3. The most common types in the United States are type 1 and type 2. They account for approximately 90 to 95 percent of all cases of children who have Usher syndrome.
Usher Syndrome Diagnosis
Diagnosis of Usher syndrome involves evaluation of the three senses namely vision, hearing and balance. The common tests are ERG, audio/hearing evaluation, and electronystagmogram (ENG).
Usher Syndrome Symptoms and Signs
Usher syndrome has several symptoms but the most common are hearing loss and an eye disorder called retinitis pigmentosa, or RP. The disorder causes night-blindness and a loss of peripheral vision (side vision) through the progressive degeneration of the retina. As the disorder progresses, the field of vision narrows. Said condition is known as ?tunnel vision??until only central vision (the ability to see straight ahead) remains. Most Usher syndrome patients also have severe balance problems.
Usher Syndrome Treatment
There is currently no cure for Usher syndrome. Early identification for now, is the best treatment since it allows children to be involved in education programs as soon as possible. The use of hearing aids, assistive listening devices, cochlear implants, or other communication methods such as American Sign Language; orientation and mobility training; and communication services and independent-living training that may include Braille instruction, low-vision services, or auditory training, are also considered as essential aspects of treatment. Though not a cure, a high dose of vitamin A palmitate may slow down the progression of RP. This is according to a long-term clinical trial supported by the National Eye Institute and the Foundation for Fighting Blindness.