Best's macular dystrophy with a macular hole.

The occurrence of a macular hole with Best's dystrophy is an extremely rare finding with only one reported case in the literature. We wish to report two cases with typical Best's dystrophy in one eye and a macular hole in the contralateral eye. In one case the Pattern Reversal VER (PVER) was also recorded and graphed as an amplitude-check size function curve. The PVER changes in this case were grossly subnormal in both the eyes, suggesting a significant degree of bilateral macular impairment. This implies that a significant amount of functional impairment occurs at the vitelliform stage itself, though how this stage progresses to a macular hole is unclear.