Usher’s syndrome is characterized by retinitis pigmentosa, sensori neural deafness and vestibulo cerebellar ataxia and mental disorder. Three cases of this syndrome all belonging to the same family has been presented. The available literature briefly reviewed. It was felt that Usher’s syndrome and Siebenmann and Bing’s disease, also characterized by retinitis pigmentosa and sensori neural deafness but without any nedrological manifestations, are two varients of the same condition in view of the closely related clinical picture. Deafness in Usher’s syndrome is probably a local aural manifestation of a congenital weakness of central nervous system due to an identical etiological condition responsible for retinitis pigmentosa.