Incidence and Types of Pediatric Nystagmus

Purpose To report the incidence, prevalent subtypes, and clinical characteristics of pediatric nystagmus diagnosed over a 30-year period. Design Retrospective, population-based study. Methods Using the Rochester Epidemiology Project, we reviewed the medical records of all children (<19 years) diagnosed as residents in Olmsted County, Minnesota, with any form of nystagmus from January 1, 1976, through December 31, 2005. Results Seventy-one children were diagnosed during the 30-year period, yielding an annual incidence of 6.72 per 100,000 younger than 19 years (95% confidence interval [CI], 5.15–8.28) Infantile nystagmus, onset by 6 months, comprised 62 (87.3%) of the study patients, corresponding to a birth prevalence of 1 in 821. The median age at diagnosis for the cohort was 12.7 months (range, 0 days to 18.6 years) and 42 subjects (59.2%) were male. The main types of nystagmus, in declining order, were nystagmus associated with retinal/optic nerve disease in 23 (32.4%), idiopathic or congenital motor nystagmus in 22 (31.0%), manifest latent nystagmus or latent nystagmus in 17 (24.0%), and 2 (2.8%) each associated with Chiari malformation, medication use, tumor of the central nervous system, and no diagnosis. Developmental delay was diagnosed in 31 (43.6%), strabismus in 25 (35.2%), and amblyopia in 10 (14.1%). Eighty percent had 20/40 (or equivalent) or better vision at presentation in at least 1 eye. Conclusions This study provides population-based data on incidence and clinical characteristics of childhood nystagmus in North America. Idiopathic and nystagmus associated with retinal/optic nerve disease were the most common presentations, with most patients having good vision. Developmental delay, strabismus, and amblyopia were common in this cohort.