Because Usher syndrome affects hearing, balance, and vision, diagnosis of the disorder usually includes the evaluation of all three senses. An ophthalmologist (Eye M.D.) evaluation of the eyes may include a visual field test to measure a person's peripheral (side) vision, an electroretinogram (ERG) to measure the electrical response of the eye's light-sensitive cells, and a retinal examination to observe the retina and other structures in the back of the eye.
Usher syndrome treatment
Currently there is no known cure for Usher syndrome or for retinitis pigmentosa. The best treatment involves early identification so that educational programs can begin as soon as possible, depending on the severity of the vision loss and the age and ability of the child. Treatment may include instruction on reading with Braille and learning to use low-vision devices and techniques. Recently, inherited diseases similar to RP and Usher syndrome have been successfully treated with genetic therapy. It is likely that such treatment may be available for Usher’s in the near future as well.
Some research has shown that Vitamin A may slow the progression of certain forms of RP. Your ophthalmologist can advise you about the risks and benefits of Vitamin A and how much you can safely take. Taking too much vitamin A can be toxic, and evidence of vitamin A’s effect on RP progression is not substantial.