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Visual Electrophysiology Unit


ISCEV standards (http://www.iscev.org/standards/index.html) recommend that ERGs be recorded for five response types:

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In children some of the retinal diseases that give abnormal ERG results are Leber Congenital Amaurosis, Rod Monochromatism, Congenital Stationary Night blindness, Cone dystrophies, Cone-rod dystrophies, Retinitis Pigmentosa and Choroideremia.

Retinal disease may be accompanied by nystagmus (eg. Rod Monochromatism, Cone Dystrophy) or roving eye movements (Leber Congenital Amaurosis). If the child has problems seeing in dim light, this signals an abnormality of the rod system (eg. Retinitis Pigmentosa, Congenital Stationary Night Blindness). If the cone system is affected, the child may have problems with photophobia; this happens in Achromatopsia (complete or incomplete) and in Cone Dystrophies. Also, if cone activity is reduced, then visual acuity is likely to be affected. At The Hospital for Sick Children (Toronto), we receive ERG referrals for children with nystagmus, unexplained visual loss, night blindness, and photophobia.

Multi-focal Electroretinogram (mfERG).
Multifocal ERGs (mfERGs) are useful in children when central retinal defect is suspected.