Background: It has been suggested that children with Down syndrome (DS) may display high frequency hearing loss much earlier than those in the general population. The purpose of this study was to investigate the diagnostic accuracy of high-frequency distortion-product otoacoustic emissions (HFDPOAEs) as a screening technique for schoolchildren with DS. Materials and methods:Exactly 25 children with DS (15 males, 10 females, mean age =9.7 years, SD=2.6) were sampled from six special education schools. HFDPOAE results were compared to the reference test results -high-frequency pure tone audiometry (HFPTA) at 8 and 10 kHz.Results: Test performance was found to reach adequate levels (≥0.7) of accuracy for HFDPOAE SNR at 8 kHz and 10 kHz. The hit rate at 8 kHz was average, but moderately high at 10 kHz. True negative rates and positive posterior probabilities for the HFDPOAE test were high, and false alarm rates were acceptably low. However, the false negative rate was average at 8 kHz and negative posterior probabilities were moderately high at both frequencies. Efficiency index values were high indicating that a large proportion of HFDPOAE results were correct when compared with HFPTA results. Conclusions:Initial findings support the potential future use of HFDPOAE for screening children with DS for high-frequency hearing loss.