A person who is suffering from Usher’s syndrome is born with a certain degree of hearing loss which may vary from person to person. Later in life, the person develops a condition called Retinitis Pigmentosa. Usher´s syndrome may also be referred to as Retinitis Pigmentosa. Usher´s syndrome is a rare disease.
Some of the patients are congenitally deaf and are suffering from progressive visual loss caused by Retinitis Pigmentosa. This syndrome may exist in profoundly deaf children to a degree of 10 per cent.
Retinitis Pigmentosa affects the retina. The retina is the part of the eye which processes light and transmits information through the optic nerve to the brain. As Retinitis Pigmentosa develops, the retina slowly begins to degenerate, which will then affect the vision.
In the first stages, night vision is usually affected, leaving the person night blind. After some time, the daytime vision will be affected, as well.
It is currently believed that there are two main forms of Usher´s syndrome. One of them is characterised by profound deafness and poor balance. Retinitis Pigmentosa is normally present in a child under the age of 10. In another form of Usher´s syndrome, the person will be suffering from partial hearing loss, and Retinitis Pigmentosa will be present before the patient reaches 20 years of age. For people with this kind of Usher´s syndrome, hearing aids can be of great value.