How prevalent is Usher Syndrome?
It has proven difficult to establish accurate figures for the occurrence of Usher Syndrome. Figures for the incidence in Europe are incomplete; in the USA more statistical work has been done but even there the figures are tenuous. About 3 per 100,000 people in the US have Usher. Of all those who have a congenital hearing impairment, 3 to 6% are victims of Usher. Of the 70,000 to 100,000 population who have RP in the US, one out of four has Usher syndrome. Thus, Vernon estimates that there are 17,500 to 25,000 people who will be deaf-blind due to Usher syndrome. As Vernon rightly points out, these figures show that Usher syndrome is all too common among deaf people. Ignorance amongst professional people who deal with deaf children about this syndrome is widespread. This must change. The need for earlier diagnosis is paramount if benefit is to be had from educational guidance, career counselling, psychological coping techniques and genetic counselling.
