Wolfram syndrome: 3 cases.

Wolfram syndrome is a rare disorder defined by the occurrence of diabete me llitus, diabete insipidus, optic atrophy and deafness; DIDMOAD is a commonl y accepted acronym. We report 3 further cases. two girls and one boy. Their age ranged from 12 to 17 years, The diagnosis was based on the presence of juvenile diabete mellitus, bilateral optic atrophy, urologic signs, with u rinary tract dilation; and deafness in two cases. The ophthalmic signs of Wolfram syndrome are progressive decrease in visual acuity, constriction of the peripheral visual field with or without centra l scotoma, color vision disturbances and bilateral optic disc atrophy. Diab etic retinopathy is a rare complication. The other clinical features are discussed so as to differentiate between Wo lfram syndrome and other optic atrophies associated with diabete mellitus. We discuss of the pathogenic hypothesis including the mitochondrial dysfunc tion.