Purpose
To evaluate rod-isolated, cone-isolated, and combined rod and cone flicker electroretinograms (ERGs) as a possible means to identify electrophysiological abnormalities in carriers of X-linked retinoschisis (XLRS).
Methods
Full-field ERGs were recorded from 6 carriers of XLRS (ages 34 to 66 years) and 8 normally-sighted subjects (ages 27 to 59 years) under rod-isolated (ERGR), cone-isolated (ERGC), and combined rod and cone (ERGR+C) conditions. ERGs were obtained using a 4-primary LED-based ganzfeld photostimulator and standard recording techniques. The 4 primaries were modulated sinusoidally in phase to achieve combined rod and cone activation (ERGR+C) or in different phases to achieve ERGR and ERGC by means of triple silent substitution. After 30 minutes of dark adaptation, 8 and 15 Hz ERGR, ERGC, and ERGR+C responses were obtained at a mean luminance level of 24 scot. cd/m2. Standard ISCEV ERGs were also obtained from each subject
Results
The ISCEV and 15-Hz flicker ERGs were generally within the normal range for the carriers. The 8-Hz ERGR, ERGC, and ERGR+C amplitudes were also generally normal. In contrast, the carriers had ERGR, ERGC, and ERGR+C timing abnormalities, with phase advances beyond the range of normal for the ERGR (4 carriers), ERGC (4 carriers), and ERGR+C (3 carriers). Only one carrier had normal 8-Hz responses under all conditions.
Conclusions
The 8-Hz ERG timing abnormalities in 5 of 6 carriers indicate that retinal function is not necessarily normal in carriers of XLRS. The 8-Hz flicker ERG may be useful for studying retinal function in these individuals.