Loss of electroretinographic oscillatory potentials, optic atrophy, and dysplasia in congenital stationary night blindness.

New criteria for diagnosing congenital stationary night blindness include loss of the oscillatory potentials in the photopic and bright-flash dark-adapted electroretinogram, and atrophy or dysplastic changes, or both, in the optic nerve head. Ten patients (seven male and three female, ranging in age from 6 to 19 years) had typical findings of congenital stationary night blindness including congenital nonprogressive nyctalopia, no pigmentary retinopathy, and full visual fields consistent with myopia. Visual acuities ranged from 20/30 to 20/60, though one patient had a visual acuity of 20/200. Most patients had histories of strabismus. The photopic electroretinograms were subnormal. Of the male patients, five had tilted optic disks with temporal portions of the nerve missing, and two had misshapen nerve heads. The three female patients had pallor of the optic disk without evidence of tilt.