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Purpose: To determine whether retinal dysfunction in obligate carriers of X-linked retinoschisis (XLRS) could be observed in local electroretinographic responses obtained with the multifocal electroretinogram (mfERG)., Methods: Nine obligate carriers of XLRS (mean age, 46.2 years) were examined for the study. Examination of each carrier included an ocular examination and mfERG testing. For the mfERG, we used a 103-scaled hexagonal stimulus array that subtended a retinal area of approximately 40 degrees in diameter. The amplitudes and implicit times in each location for the mfERG were compared with the corresponding values determined for a group of 34 normally-sighted, age-similar control subjects., Results: Mapping of 103 local electroretinographic response amplitudes and implicit times within a central 40 degrees area with the mfERG showed regions of reduced mfERG amplitudes and delayed implicit times in two of nine carriers., Conclusions: The mfERG demonstrated areas of retinal dysfunction in two carriers of XLRS. When present, retinal dysfunction was evident in the presence of a normal-appearing fundus. Multifocal ERG testing can be useful for identifying some carriers of XLRS.

Purpose: To evaluate acuity and multifocal electroretinogram (mfERG) responses from the macula in affected and unaffected fellow eyes of patients with macular holes., Methods: We tested 10 eyes with macular hole and 10 fellow eyes from 11 patients. We measured local visual acuity thresholds at 27 discrete locations within 21 degrees diameter using the Functional Fundus Imaging System (FFIS), a psychophysical system that measures visual acuity as a function of visual field location, and local ERG responses within 45 degrees diameter using the mfERG., Results: In the affected eyes, the mean FFIS visual acuity thresholds were significantly elevated within the central 21 degrees diameter area, compared to a group of control eyes. No significant differences were found between the acuities of the fellow eyes compared to those of the control group. The amplitudes of the first positive peak of the mfERG were reduced in the central 7.8 degrees in affected eyes. In the central 2 degrees , 4 out of 10 affected eyes showed non-measurable ERG signals. The remaining six eyes showed significantly reduced mean amplitudes, but not delayed implicit times, when compared to the control group. For the fellow eyes, the mean amplitudes of the mfERG and implicit times did not differ from the means of the control eyes., Conclusions: Both local psychophysical and electrophysiological testing demonstrated retinal dysfunction extending beyond the site of the macular holes in some patients (three of the patients had central mfERG amplitudes falling within the normal range).