ELECTROOCULOGRAPHY IN AUTOSOMAL DOMINANT VITREORETINOCHOROIDOPATHY

Thirteen members of a family presumed to be harboring the gene for autosomal dominant vitreoretinochoroidopathy were examined. In four affected members, electro-oculography demonstrated marked reduction of the Arden ratio (range, 1.1 to 1.5; normal, greater-than-or-equal-to 1.8), despite electroretinographic evidence of mildly affected rod function and normal cone function. These findings suggested that a diffuse disturbance of the photoreceptor-pigment epithelium complex may have been present prior to widespread loss of photoreceptor function in the affected members of this family. As in previously described families, the pattern of inheritance appeared consistent with autosomal dominance.