Purpose To assess the feasibility, sensitivity, and specificity of nonsedated handheld cone flicker electroretinogram (ERG) as a screening tool to detect retinal dysfunction in children with nystagmus. Methods Pediatric patients at a tertiary referral center from December 2015 to July 2016 were enrolled and placed into three age-matched groups: normal, nystagmus with a retinal dystrophy, and nystagmus without a retinal dystrophy. Unsedated 30 Hz cone flicker ERG responses were obtained using a handheld device (RETeval) from both eyes of each patient using skin electrode sensors after pupillary dilation. Results A total of 71 children were enrolled; amplitudes and implicit times were successfully obtained in 65 (92%): 31 (mean age ± SD, 5.6 ± 2.7 years; range, 1-12 years) without nystagmus and 34 with nystagmus. Nystagmus patients were grouped by those with (n = 15; mean age, 8.5 ± 4.5 [range, 2-17 years) and without (n = 19; mean age, 4.3 ± 3.0 [range, 6 months-10 years]) a retinal dystrophy. The patients with retinal dystrophies had significantly smaller amplitudes and prolonged or nonmeasurable implicit times than the other two groups (P < 0.001). Among nystagmus patients, amplitude was able to discriminate between those with and without retinal dystrophies with area under curve of 0.986 (SE = 0.016; P < 0.001). An amplitude <5 μV in combination with an implicit time of >33 ms warrants further evaluation. Conclusions Unsedated handheld cone flicker ERG is a feasible screening test that effectively detects retinal dysfunction in children with nystagmus. In conjunction with clinical findings, the test helps reduce the need for sedated ERG in children.
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health