Does Usher syndrome always result in blindness?
Very few people with Usher syndrome will become totally blind – that is, have no light perception. Some adults maintain a small degree of central vision throughout their lives while others lose all usable vision. Natural history studies are currently collecting data to map the "typical" progression of vision, hearing and vestibular loss for the various types of Usher syndrome.
Children will begin to develop night blindness in dimly lit areas. Progressive loss of visual fields will affect their ability to see to their right, left, above and below. Typically, central vision for reading and fine detail is functional into the adult years, as long as good lighting is available. It may take a minute or so for individuals with RP to adjust to glare and dramatic changes in lighting. As visual fields narrow, individuals with RP will often stop and scan the environment before entering a building or room. Objects and people at a distance will be easier to discern than those that are near to them.
Sometimes children and adults with Usher appear to be clumsy because of vestibular/balance issues. This can be even more noticeable as visual fields decrease, or in dimly lit areas. Depth perception may be affected, as well as the ability to see monotones such as grey, white and black.
Low vision specialists can suggest optical aids and devices that maximize remaining vision. Orientation and mobility instructors can provide training in safe travel.