Peer-reviewed article

Hearing Loss Assessment in Children

Sommerfeldt J, Sutton AE, Kolb CM · 18 Aug 2026

Abstract

Congenital and childhood hearing loss is among the most common sensory disorders, affecting approximately 1 to 3 per 1000 newborns, with prevalence increasing throughout childhood. By adolescence, up to 20% of children demonstrate some degree of hearing loss. Early identification is essential because untreated hearing loss can adversely affect speech and language development, academic performance, vestibular function, psychosocial well-being, and overall quality of life, including increased risks of anxiety, depression, and low self-esteem. Early auditory stimulation during critical periods of neuroplasticity is essential for optimal development of the cortical auditory pathways. Universal newborn hearing screening (UNHS) was endorsed by the National Institutes of Health in 1993 and subsequently formalized through successive Joint Committee on Infant Hearing guidelines position statements, most recently updated in 2019 [JEHDI. Year 2019 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs]. Results from one study found that UNHS protocols resulted in 1 hearing loss diagnosis for every 811 infants screened without high-risk factors. One study found that using Universal Newborn Hearing Screening protocols resulted in one hearing loss diagnosis for every 811 babies screened without high-risk factors. The established 1-3-6 benchmark advocates for screening by 1 month, diagnostic confirmation by 3 months, and intervention by 6 months. The 2019 Joint Committee on Infant Hearing position statement recommends that programs meeting these targets strive for an accelerated 1-2-3 timeline. Despite a national screening rate exceeding 98%, loss to follow-up remains a significant challenge. Furthermore, the deployment of UNHS programs varies by setting and specific circumstances. Program implementation should account for local resource availability, screening accuracy, follow-up systems, and cost-effectiveness considerations. Screening protocols commonly employ automated auditory brainstem response, with implementation varying by institutional resources, patient population, and follow-up infrastructure. Results from one study showed that additional testing on day 7 of life was beneficial for determining the ideal timing of neonatal hearing screening using transient evoked otoacoustic emissions. Hearing loss may be unilateral or bilateral. Although unilateral hearing loss is frequently considered less problematic than bilateral hearing loss, more recent data have demonstrated its substantial clinical and educational significance. Symmetrical auditory input to both ears is essential for the development of binaural hearing pathways early in life. Reduced binaural input impairs sound localization and speech perception in noisy environments through decrements in binaural squelch. Children with unilateral hearing loss have a 10-fold higher risk of grade repetition than children with normal hearing (35% vs 3.5%, respectively). Up to 40% require additional educational assistance. Unilateral hearing loss may also progress to bilateral loss, underscoring the need for ongoing surveillance. Inner ear malformations, including enlarged vestibular aqueduct and Mondini spectrum anomalies, are commonly identified among children with congenital unilateral hearing loss. Additional etiologies include genetic mutations, congenital cytomegalovirus infection, neonatal intensive care unit–related risk factors, ototoxic exposure, and other structural abnormalities.