Prior to the era of molecular diagnosis, it was known that vision loss in the SCAs could result from primary optic neuropathies or, less commonly, retinal degeneration.5 Detailed studies of vision in the different SCA genotypes have not yet been performed, although it is well established that SCA7 is the only genotype in which retinal degeneration commonly occurs.5 In a prior series of patients with genetically confirmed SCA1, decreased visual acuity, dyschromatopsia, and optic atrophy were reported, but no other funduscopic abnormalities were noted.4 All 6 patients in that series had attenuated oscillatory potentials and some had decreased b-waves,4 possibly indicating inner retinal dysfunction. Another report described a patient with genetically confirmed SCA1 who had progressive vision loss and a pigmentary macular dystrophy,6 similar to that described in SCA7.5 Full-field ERG revealed photoreceptor dysfunction and genetic testing had negative results for SCA7, suggesting that a pigmentary macular dystrophy can occur in SCA1.6 Our patient with genetically confirmed SCA1 had progressive binocular central vision loss and subtle funduscopic changes suggestive of retinal degeneration, without optic atrophy. Full-field ERG revealed rod and cone dysfunction. The presence of vision loss in other family members with cerebellar ataxia and presumably SCA1 suggests that the vision loss was a manifestation of SCA1 and not due to a second pathology. Our findings therefore suggest that vision loss in SCA1 can be due to rod-cone dystrophy and should prompt evaluation by ERG, even in the absence of obvious retinal changes.