The prevalence of hearing loss and abnormal middle ear status is high in the pediatric population with DS. Audiologic evaluations should follow the American Academy of Pediatrics practice guidelines to monitor this high-risk population, and amplification should be considered as an appropriate intervention option if repeated audiologic examinations reveal hearing loss.

Purpose Normative data regarding behavioral audiologic testing procedures are based upon the general population and often do not apply to children with Down syndrome (DS). Testing children with DS can be challenging, and outcomes may be unreliable due to their different cognitive demands and delays. The aim of this study was to assess optimal audiologic testing procedures for specific age groups of children with DS. Method This study used a retrospective investigation of 273 children with DS (145 boys, 128 girls; average age at evaluation = 5.92 ± 4.74 years) who received an audiologic evaluation during 2013 as part of their medical care at a large pediatric hospital (satellite facilities included). Results Age ranges for the completion of audiometry procedures in children with DS are provided. Average age to reliably complete behavioral testing in children with DS was delayed by up to 30 months compared to typically developing children. The majority of children with DS achieved at least good-to-fair reliability for audiologic results starting at 16 months (85.7%) and two ear results at 6–10 years (76.1%). Though not statistically significant, the use of a two-tester assistant compared to a single tester appeared to be helpful in obtaining reliable results. Conclusion The results provide a guide to optimal audiologic test procedures for children with DS, as the standard audiologic guidelines for typically developing infants and children do not apply.

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