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A bstract Background: To review the use of electroretinography and identify common reasons for referral and diagnoses at a tertiary paediatric hospital. Design: Retrospective cohort study. Participants: Three hundred and eighteen patients (male 195, female 123) aged <18 years with 388 electroretinograms were included. Methods: All electroretinograms performed at the Royal Children's Hospital, Brisbane from 1998 to 2005 were reviewed. Normative data and electroretinograms from patients aged ≥18 years were excluded. Main Outcome Measures: Reasons for referral and diagnoses were determined from each patient's first electroretinogram. Concordance between the first electroretinogram diagnosis and clinical referral was reviewed to determine whether the electroretinogram was normal, inconclusive, confirmed, excluded, or changed the clinical diagnosis or provided a new diagnosis. Results: The median age at the time of the first investigation was 3.78 years (range 2.6 weeks to 17.5 years). The most common reasons for referral were nystagmus ( n = 93), decreased vision ( n = 33) and sensorineural deafness ( n = 29). After one electroretinogram, 51% were normal ( n = 162) and 15% were inconclusive ( n = 49). The most common electroretinography diagnosis was cone rod dystrophy. The first electroretinogram for each patient confirmed the clinical suspicion in 17.6% ( n = 56) and excluded it in 23% ( n = 73) of cases. The electroretinogram resulted in a changed diagnosis in 0.9% ( n = 3) and provided a new diagnosis in 15.1% ( n = 48). Overall, the first electroretinogram was considered useful in 85% cases ( n = 269). Conclusions: Electroretinography is a valuable investigation for evaluating paediatric eye disease and in this series confirmed, excluded, changed or provided a new diagnosis in 85% of cases. [ABSTRACT FROM AUTHOR]