Observational Study to Preliminarily Characterize the Audiological Profile of Children with Down Syndrome

Purpose: Down syndrome occurs in one of 700 births, and high rates of hearing loss are reported in this population. This puts children with Down syndrome at risk for communication, learning, and social development difficulties, compounding known language and cognitive vulnerabilities in this population. The purpose of this study was to comprehensively characterize audiological profiles in children with Down syndrome, including the use of extended high-frequency sensitivity and speech intelligibility index assessment. Method: Participants were 18 children with Down syndrome between 5 and 17 years of age. Audiological profiles were characterized using behavioral audiometry, tympanometry, and wideband acoustic immittance (WAI). Audibility was characterized using the speech intelligibility index. Results: Of the participants successfully completing behavioral audiometry, hearing loss of a moderate or greater degree was observed in one or both ears for 46% of the participants at conventional audiometric test frequencies and 85% of the participants at frequencies above 8 kHz. Seven children met criteria for amplification based on the speech intelligibility index, but only two wore hearing aids. Abnormal middle ear function was found in approximately 50% of the participants for whom WAI or tympanometry were successfully measured. Conclusions: Consistent with prior research, high rates of hearing loss and middle ear dysfunction were observed. The high prevalence of hearing loss above 8 kHz suggests the importance of including extended high-frequency assessment in audiologic characterization of children with Down syndrome. Few children meeting audibility-based guidelines for amplification wore hearing aids, putting them at additional risk for speech/language and educational difficulties.