The aim was to evaluate alterations in Visual-Evoked Potentials (VEP) and E
lectroretinogram (ERG) and discover whether these tests are useful for dete
rmining residual visual acuity in cases where a patient is unable to collab
orate. Flash and, when possible, Transient Pattern Reversal Visual-Evoked P
otentials and Maximal Response ERG were recorded in 8 children (under three
years old) affected by different aspects of optic nerve coloboma. None of
them had visual acuity evaluated because of poor collaboration. All examina
tions were carried out using skin electrodes. Amplitude of the a and b comp
onent of ERG, amplitude, morphology and latency of the major components of
Flash VEP and amplitude and latency of P100 Pattern Reversal VEP were evalu
ated. Four of the patients were examined three years later and visual acuit
y was compared with the previous electrofunctional results. Alterations in
ERG were found only in the case of significant retinal anomalies ((great co
loboma, retinal detachment), huge alterations were found in both Flash VEP
and in Pattern Reversal VEP. The retrospective study of VEP traces and visu
al acuity showed a good correlation between electrofunctional data and visu
al capability. Electrofucntional examinations can identify important conduc
tive retinocortical anomalies. The possibility of correlating electrophysio
logical results with residual visual acuity is important for rehabilitative
management in such optic disc malformations.