Electrophysiologic findings in bilateral optic nerve hypoplasia

Electrophysiologic studies were performed on 17 patients with bilateral optic nerve hypoplasia. Since most patients were infants, the diffuse light visual-evoked response was more helpful than the visual-evoked response obtained with pattern stimulation. In children over 1 year of age, the amplitude with diffuse light stimulation correlated well with other clinical measurements used to assess visual function. Before 6 months of age, it correlated better with visual prognosis than did clinical analysis. Photopic and scotopic electroretinographic measurements were normal in all except two in whom the scotopic b wave was supernormal.