Two Cases Usher's Syndrom in two Brothers

ABSTRACT

Usher's syndrome is an autosomal recessively inherited trait that characterized by a congenital nonprogressive sensorineural hearing impairment and progressive night-blinding disorder, retinitis pigmentosa, with cataract, psych psis, speech disorder, mental deficiency, and vestibular ataxia being variable additional findings. We experienced two cases of Usher's syndrome in two brothers, which has the hearing loss and night-blinding disorder that had been developed since in early childhood and 2nd decade respectively. Their ophthalmoscopic retinal and ERG findings are characteritic ones of retinitis pigmentosa. such as 1) bone corpuscle pigmentation in the periphery, narrow arteries, and a waxy, yellowish optic, disc, 2) non-recordable ERG, respectively. Their pure tone automatry confirmed the bilateral sensorineural hearing loss.