They also exhibit balance difficulties and learn to walk slowly as children, due to problems in their vestibular system. People with Usher I are born profoundly deaf and begin to lose their vision in the first decade of life. Usher syndrome is named after Scottish ophthalmologist Charles Usher, who examined the pathology and transmission of the syndrome in 1914. Type I is most common in Ashkenazi Jewish and Acadian populations, and type III is rarely found outside Ashkenazi Jewish and Finnish populations. The occurrence of Usher syndrome varies across the world and across the different syndrome types, with rates as high as 1 in 12,500 in Germany to as low as 1 in 28,000 in Norway. ![]() These mutations are inherited in an autosomal recessive pattern. All three subtypes are caused by mutations in genes involved in the function of the inner ear and retina. Usher syndrome is classed into three subtypes (I, II and III) according to the genes responsible and the onset of deafness. It is a major cause of deafblindness and is at present incurable. Usher syndrome, also known as Hallgren syndrome, Usher–Hallgren syndrome, retinitis pigmentosa–dysacusis syndrome or dystrophia retinae dysacusis syndrome, is a rare genetic disorder caused by a mutation in any one of at least 11 genes resulting in a combination of hearing loss and visual impairment. ![]() ![]() The genes implicated in Usher syndrome are described below. Usher syndrome is inherited in an autosomal recessive pattern.
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