Being born severe to profoundly deaf or gradually losing hearing, losing vision starting in infancy or later in adolescence, and being born with severe balance problems are the three conditions that people with Usher syndrome look for according to the type (1, 2 or 3) they suffer from.
Usher syndrome is the most common cause of deafblindness in children. It represents between 3 and 6% of all childhood deafness. 50% of people with deafblindness in developed countries are due to this syndrome. Its estimated prevalence ranges between 6-17/100,000.
Usher syndrome is a rare genetic condition first described in 1858. It is an autosomal recessive hereditary disorder, thus inherited from parents to children, which only manifests itself in 25% of cases in which both parents are carriers of the mutation.