THE ELECTROOCULOGRAM IN HETEROZYGOTE CARRIERS OF USHER SYNDROME, RETINITIS-PIGMENTOSA, NEURONAL CEROID-LIPOFUSCINOSIS, SENIOR SYNDROME AND CHOROIDEREMIA

Electrooculographic studies were performed in 77 carriers of tapetoret inal dystrophies: Usher syndrome (2o), retinitis pigmentosa (32), neur onal ceroid lipofuscinosis (6), Senior syndrome (2), and choroideremia (I7). The carriers were matched for sex and age with normal controls. In carriers of Usher syndrome the E O G Lp/Dt ratio was significantly lowered with 30% of the recordings having a subnormal value. There wa s a trend in carriers of retinitis pigmentosa to a subnormal E O G. In contrast to previous studies there was no decrease in the E O G Lp/Dt ratio in carriers of neuronal ceroid lipofuscinosis. Two carriers of Senior's syndrome had a normal E O G. Carriers of choroideremia did no t differ significantly from normal controls; however, the Lp/Dt ratio decreased with increasing age. An abnormal E O G may be indicative of the carrier state in relatives of patients with tapetoretinal dystroph ies.