ABSTRACT
The purpose of the study is to characterise retinal function using light-adapted electroretinograms (ERGs) in a series of young children with ONH, congenital dysplasia of retinal ganglion cells. ERGs were recorded with chloral hydrate sedation in 27 children with ONH (18 with bilateral and 9 with unilateral ONH, age 4-35 months) and an adult reference population (n = 12). Stimuli included ISCEV standard flash, oscillatory potentials (OPs) and standard flicker as well as a light-adapted luminance-response series (photopic hill). The disc diameter to disc macula (DD:DM) ratio was measured from fundus photographs. The results are eyes with ONH, classified by DD:DM, were severe (