Hearing Impairment Related to Age in Usher Syndrome Types 1B and 2A

Abstract
THE USHER syndrome was first described in 1858 and characterized as a disorder with bilateral sensorineural hearing loss and visual impairment caused by tapetoretinal degeneration.1 Several authors emphasized a high prevalence of this syndrome in certain families, and a hereditary nature was suspected. von Wibout2 suggested that the syndrome had an autosomal recessive mode of inheritance.