Electroretinographical Study of the Hypoplasia of the Optic Nerve

Publisher Website
Google Scholar
Abstract
Of 22 sporadic cases of hypoplasia of the optic nerve, 16 were bilateral and 6 unilateral. The sex distribution was equal in the bilateral cases, while all the unilateral cases were females. Visual impairment was severe in all the cases. There was total blindness or only light perception. The pupillary reflexes were absent or very slow. Only 1 case had a rather good visual acuity (1/10) with a constricted visual field. In addition to the typical aspect of hypoplasia of the optic disc, some cases showed an obvious or suspected retinal degeneration, such as a marked choroidal pattern with pigmentary dystrophy of the posterior pole. The electroretinogram (ERG) was normal in most cases, and showed no modification with increasing age. No supranormal recordings were obtained. Of 36 eyes, 12 had a subnormal response. Seven showed an involvement only of the scotopic response, 1 only of the photopic response and 4 of both responses. The reduction of the ERG amplitude was rather mild in most cases. The ERG was never extinguished. The temporal characteristics were normal in all but 2 cases.