Behavioral Audiology Procedures in Children With Down Syndrome

Abstract

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.

Identifying reduced hearing in children who have developmental disabilities: Insights for inclusive research practices with electronic health records

“…This issue may be somewhat mitigated by the use of physiologic measures (e.g., auditory brainstem response and otoacoustic emissions), but these procedures are prone to missing some of the above hearing profiles and may require sedation. Challenges in adhering to recommended pediatric audiology diagnostic practices seen here and for other studies ( Findlen and Schuller, 2020 ; Nightengale et al, 2020 ; Trudeau et al, 2021 ), suggests that there is a need for future research and clinical guidance to ensure equitable access for children who have developmental disabilities to a comprehensive hearing assessment in a timely manner.…”

“…Compared to typically-developing, age-matched controls, laboratory studies using clinical methods have confirmed that fewer behavioral thresholds are obtained for infants and children who have multiple conditions ( Gans and Gans, 1993 ); 3- to 10-year-old children who have autism spectrum disorder (ASD; Tharpe et al, 2006 ); or 6- to 72-month-olds with DS ( Greenberg et al, 1978 ). Similarly, retrospective clinical studies confirm that children who have developmental disabilities are at risk for not obtaining behavioral thresholds ( Meagher et al, 2021 ; Trudeau et al, 2021 ), and thresholds were obtained from children who have DS at ages later than recommended ( Nightengale et al, 2020 ). We posit that it is difficult to measure behavioral thresholds in children who have developmental disabilities because clinical methods are based on assumptions of typical child development.…”

Development of Masked Speech Detection Thresholds in 2- to 15-year-old Children: Speech-Shaped Noise and Two-Talker Speech Maskers

“…Documenting performance differences across masker types is expected to advance our theoretical understand of auditory development for children with developmental disabilities. Furthermore, the creation of clinical tools for monitoring hearing abilities in children with developmental disabilities is in critical need, as this population is at increased risk of hearing loss (e.g., Rosenhall et al 1999; Kreicher et al 2018) yet are difficult to test with present behavioral methods (e.g., Gans & Gans 1993; Nightengale et al 2020).…”

Hearing Loss Diagnosis and Management in Adults with Intellectual and Developmental Disabilities

“…These subjective and objective methods for hearing loss screening present various challenges for patients with IDD. Patients may have difficulty communicating their experiences or understanding instructions related to the hearing screen, thus necessitating more objective measures [ 30 ]. Patients may have behavioral issues that can make it challenging to complete a hearing screen.…”