Impact of Co-Occurring Birth Defects on the Timing of Newborn Hearing Screening and Diagnosis

Abstract

Purpose Early detection of hearing loss in all newborns and timely intervention are critical to children's cognitive, verbal, behavioral, and social development. The initiation of appropriate early intervention services before 6 months of age can prevent or reduce negative developmental consequences. The purpose of this study was to assess, using large, population-based registries, the effect of co-occurring birth defects (CBDs) on the timing and overall rate of hearing screening and diagnosis. Method The authors linked statewide data from newborn hearing screenings, a birth defects registry, and birth certificates to assess the timeliness of newborn hearing screening and diagnosis of hearing loss (HL) for infants with and without CBDs in 485 children with confirmed HL. Results Nearly one third (31.5%) of children with HL had 1 or more CBDs. The presence of CBDs prolonged the time of the initial infant hearing screening, which contributed to further delays in the subsequent diagnosis of HL. Conclusions Better coordination of HL assessment into treatment plans for children with CBDs may enable earlier diagnosis of HL and provide opportunities for intervention that will affect long-term developmental outcomes for these children.

An Analysis of Risk Factors in Unilateral Versus Bilateral Hearing Loss

“…Congenital hearing loss has a prevalence ranging from 0.97 to 2.0 per 1000 newborns. [1][2][3] Unilateral hearing loss (UHL) is more prevalent than bilateral hearing loss (BHL) among children aged 6 to 19 years. 4 Children with UHL often went undiagnosed and unnoticed until they reach elementary school.…”

Implementation and results of bedside hearing screening with automated auditory brainstem response in the neonatal intensive care unit

“…The Joint Committee on Infant Hearing (JCIH; including representatives of the American Speech‐Language‐Hearing Association, the American Academy of Otolaryngology‐Head and Neck Surgery, the American Academy of Audiology, the American Academy of Pediatrics and the Directors of Speech and Hearing Programs in State Health and Welfare Agencies) has advised to conduct universal early hearing screening in children, aiming at optimal chances for normal speech and language development in children with congenital hearing disorders (7,8). Early (before 6 months of age) identification and therapeutic intervention results in better language outcomes and positively influences social, emotional and gross motor development (9–16). However, in addition to age at diagnosis, many other factors play a role in determining final communication skills in children with hearing loss.…”

“…The presence of other disabilities may weaken the impact of early identification and intervention (9). Moreover, the presence of multiple co‐occurring birth defects may be associated with delays in initial hearing screening and confirmatory diagnosis (11). In 120 children with bilateral hearing loss, Kennedy et al.…”

Effects of Policy Changes to Universal Newborn Hearing Screening Follow-Up in a University Clinic

“…Factors contributing to the low rates of timely diagnosis as well as loss to follow-up and documentation rates include parental noncompliance in scheduling, middle ear fluid, infants having other medical conditions, and distance from the testing facility (Munoz, Nelson, Goldgewicht, & Odell, 2011); information gaps between hospitals, parents, physicians, and audiologists as well as a limited number of audiologic testing facilities (Shulman et al, 2010); and cooccurring birth defects, which are present in nearly one third of infants identified with hearing loss (Chapman et al, 2011). Significant variability in wait times for scheduling the diagnostic testing across facilities has been identified as an additional challenge, as longer wait times impact the ability to obtain accurate test results during natural sleep (Munoz et al, 2011).…”