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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. Supplemental Material: https://doi.org/10.23641/asha.21200422

OBJECTIVE: The purpose of this study was to 1) characterise word recognition in a speech masker for preschoolers tested using closed-set, forced-choice procedures and 2) better understand the stimulus and listener factors affecting performance. DESIGN: Speech recognition thresholds (SRTs) in a two-talker masker were evaluated using a picture-pointing response with two sets of disyllabic target words. ChEgSS words were previously developed for children ≥5 years of age, and simple words were developed for preschoolers. Familiarisation ensured accurate identification of target words before testing. STUDY SAMPLE: Participants were 3- and 4-year olds (n = 21) and young adults (n = 10) with normal hearing. RESULTS: Preschoolers and adults had significantly lower SRTs for the simple words than the ChEgSS words, and lower SRTs for early-acquired than later-acquired ChEgSS words. For both word sets, SRTs were approximately 11-dB higher for preschoolers than adults, and child age was associated with SRTs. Preschoolers’ receptive vocabulary size predicted performance for ChEgSS words but not simple words. CONCLUSIONS: Preschoolers were more susceptible to speech-in-speech masking than adults, with a similar child–adult difference for the ChEgSS and simple words. Effects of receptive vocabulary in preschoolers’ recognition of ChEgSS words indicate that vocabulary size is an important consideration, even when using closed-set methods.

Masked speech recognition is poorer for children who are typically developing compared to adults, particularly when the masker is two-talker speech. Audibility, receptive vocabulary size, and executive function contribute to these age effects, all areas of weakness for children with Down syndrome. This study sought to determine whether these weaknesses put children with Down syndrome (n = 15, 5–17 yrs) at a disadvantage when listening to masked speech compared to age-matched children who are typically developing. Speech-in-noise and speech-in-speech was assessed using an adaptive, forced-choice procedure with a picture-pointing response. Audiological testing and standardized assessments of receptive vocabulary, non-verbal cognition, and executive function were also completed. Overall, children with Down syndrome performed more poorly than their typically developing peers. While the relationship between age and SRTs differs between the noise and speech maskers for children who are typically developing, this interaction was not observed for children with Down syndrome. Controlling for age, SRTs for children with Down syndrome were associated with vocabulary and executive function scores with the noise masker but not the speech masker. Results suggest that masked speech recognition is more challenging and is limited by different factors for children with Down syndrome compared to those who are typically developing.