The electroretinogram, findings, in response to a flash stimulus, was recorded from a skin electrode placed on the bridge of the nose in 4465 infants and children seen over a 10 year period. The electroretinogram was combined with a flash visual evoked potential. From this total, the electroretinographic findings in 240 patients, aged 1 day to 17 years, without suspected retinal pathology and with a normal visual evoked potential, were used as controls and normal electroretinographic parameters of different age groups defined. There were 332 patients who showed an absent or very reduced amplitude electroretinogram. They were divided into primarily ocular disorders (n = 195), neurodegenerative disorders (n = 94), and various syndromes (n = 43). Fundus examination did not always show any obvious abnormalities. The use of this simple and reliable technique for recording the electroretinogram made it possible to include this investigation as a routine procedure without the need for sedation in infants and uncooperative children. Electroretinographic studies, especially when combined with visual evoked potentials, and in some cases electroencephalography, may aid diagnosis in a wide variety of paediatric conditions, many of which have genetic implications.
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