Table 1

Summary of clinical findings

FamilyCase*Age (years)VARefractive errorCone ERG
*Cases 1 and 2 are brothers, cases 4 and 5 are sisters.
A186/12 RE+2.0/+1.50×90Absent cone ERG
6/18 LE+2.5/+1.75×90
A266/18 RE+3.0/+1.00×90Absent cone ERG
6/18 LE+3.5/+1.25×90
B386/12 RE+1.25/+1.5×90Reduced cone b:a wave ratio
6/12 LE+1.25/+2.0×90
C4146/24 RE−11.0/−2.00×30Markedly reduced cone ERG
6/12 LE−8.5/−3.00×155
C566/24 RE−8.0/−1.50×20Markedly reduced cone ERG
6/24 LE−9.5/−2.00×110
D686/24 RE−13.0/−1.5×170Delayed and mildly reduced cone ERG
6/36 LE−13.0/−1.25×10