Cone function and cone interaction in hereditary degenerations of the central retina

Spectral sensitivity functions and the transient decrease of sensitivity to short wavelengths after the offset of yellow light (transient tritanopia) were measured by increment threshold techniques in patients suffering from hereditary macular degenerations. Color vision defects were determined by arrangement tests and the anomaloscope. Central areolar choroidal dystrophy was found to produce a mild protan defect and to reduce foveal spectral sensitivity throughout the visible spectrum by a factor of 100; it also abolishes transient tritanopia. Electroretinogram (ERG) was normal, electrooculogram (EOG) subnormal. Stargardt's disease, despite numerous fluorescent macular spots, does not abolish transient tritanopia nor does it reduce spectral sensitivity, although scotopic matches were performed on the Nagel anomaloscope. Only in severe, advanced cases was transient tritanopia reduced and spectral sensitivity found to follow the absorption spectrum of rods. Routine ERGs and EOGs were normal. Vitelliform macular degeneration, despite the ophthalmoscopically pronounced dystrophic macula, produced only very small changes in spectral sensitivity and transient tritanopia, although a widened matching range on the Nagel anomaloscope and electrophysiological abnormalities were found. Apparently damage of the retinal circuit which connects long and short wavelength-sensitive cones, caused by hereditary conditions, is different from that caused by retinotoxic drugs.