The Electroretinogram in Albinos and Carriers of the Ocular Albino Trait

Abstract

Albinism is a hereditary anomaly of pigmentation caused by the insufficient formation of melanin pigments. There is marked variation in the degree of this insufficiency. In the most severe form the lack of pigmentation is noted throughout the entire body (universal or generalized albinism), and in the mildest form, in the eye alone (ocular albinism).¹ The ocular involvement is usually more extensive in the universal albinism, as evidenced by lighter appearance of the fundus, greater photophobia, and lower visual acuity. The ocular involvement may also vary with age. An increase in pigmentation occurs in most albinotic eyes (and in the skin and hair of universal albinos) with advancing age.¹ This pigmentary increase is accompanied by a decrease in photophobia and nystagmus and is often characterized by improvement in visual acuity. The genetic factors include a usual recessive inheritance in universal albinism and an intermediate sex-linked inheritance in the