People with Usher 3 are born with either normal hearing or a partial hearing loss. At some stage, usually in early adulthood but sometimes in children or young people, they then develop a visual impairment called Retinitis Pigmentosa (RP) and, at roughly the same time, their hearing starts to deteriorate. Balance can also become affected. Because Usher Type 3 is rare in the UK (probably no more than 10% of the total Usher population), diagnosis of the condition can be quite delayed.
What does this mean?
Problems with vision are first noticed at night followed by increasing difficulty with side or peripheral vision. At times this may lead to bumping into or falling over people and objects. People may notice difficulty moving into bright light from a darker place or vice versa. The RP with Usher Type 3 is more variable than with the other Types and this can mean that central vision is affected earlier than with Types 1 and 2.
However, there is the additional problem that the hearing is also changing. This presents the person with Usher Type 3 with the challenge of continuously adapting to two changing senses.
What does the future hold?
Because the RP with Usher Type 3 is so variable for person to person, it is impossible to predict how much sight and hearing one might have at any given stage in life. There is no treatment available for Usher at the moment. Research is taking place in a number of universities in Europe and the USA, which is looking into the types of Usher, the genes that are involved and what messages the genes are passing to the cells. Even though the challenges of Usher Type 3 can be particularly tough, much can be done to help people with this Type make the best use of their vision and hearing.
