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Hearing loss in children can significantly impact development and quality of life. Screening guidelines are in place to assist providers, with the recommendation to screen all newborns for hearing loss by at least one month, followed by diagnostic audiologic testing by 3 months, and appropriate intervention by 6 months. There are various ways to evaluate a patient with potential hearing loss, from audiologic tests to laboratory and imaging tests. This activity reviews the components frequently utilized in audiologic testing, discusses the indications for laboratory and imaging modalities, and highlights the role of the interprofessional team in evaluating and treating patients with known or suspected hearing loss. Objectives: Identify the screening indications for hearing loss in children. Assess treatment considerations for pediatric patients with hearing loss. Evaluate the most effective testing methods for children of different ages with suspected hearing loss. Access free multiple choice questions on this topic.

Hearing loss is relatively common in newborns and children, with an estimated prevalence of 1.1-3.5 per 1,000 newborns screened.[1][2][3] Up to 20% of children are affected by some degree of hearing loss by age 18, underscoring the need for appropriate diagnostic and intervention techniques to prevent negative sequelae of hearing loss. Undiagnosed and untreated, hearing loss can have significant consequences, including speech and language developmental delays, vestibular dysfunction, increased anxiety and depression, and decreased well-being and self-esteem.[4][5][6] In 1993, the National Institutes of Health published recommendations that all newborns obtain routine hearing screening by 3 months of age. The Joint Committee on Infant Hearing published its first position statement in 1994, with updates made in 2000, 2007, and most recently in 2019. The 2007 guidelines called for newborn hearing screening to be performed in all newborns by 1 month of age, with definitive diagnostic audiometric testing completed by 3 months for those who did not pass, and finally, with the initiation of an appropriate intervention by 6 months of age.[7] These recommendations have been adopted widely in the United States, with recent data indicating that 98% of all newborns completed initial screening for hearing loss within 1 month of birth. This led to the updated recommendation 2019 that screening, diagnostic testing, and intervention be completed in 1 month, 2 months, and 3 months, respectively, wherever possible.

In 1993, the National Institutes of Health published recommendations that all newborns obtain routine hearing screening by 3 months of age. The Joint Committee on Infant Hearing published its first position statement in 1994, with updates made in 2000, 2007, and most recently in 2019. The 2007 guidelines called for newborn hearing screening to be performed in all newborns by 1 month of age, with definitive diagnostic audiometric testing completed by 3 months for those who did not pass, and finally, with the initiation of an appropriate intervention by 6 months of age.[7] These recommendations have been adopted widely in the United States, with recent data indicating that 98% of all newborns completed initial screening for hearing loss within 1 month of birth. This led to the updated recommendation 2019 that screening, diagnostic testing, and intervention be completed in 1 month, 2 months, and 3 months, respectively, wherever possible. Hearing loss may be unilateral or bilateral. Unilateral hearing loss is frequently considered less problematic than bilateral hearing loss, although more recent data has demonstrated its clinical significance. Balanced input to both ears is essential for developing binaural hearing pathways early in life.[8] Without binaural hearing, sound localization is more difficult and can significantly affect patients, especially when trying to hear in the presence of background noise through decrements in binaural squelch.[9] Children with unilateral hearing loss have a 10-fold higher risk of repeating at least 1 grade in school than normal-hearing children (35% vs. 3.5% respectively). Up to 40% require additional educational assistance.[10] Unilateral hearing loss has also been reported to progress into bilateral hearing loss in 7.5 to 11% of cases, demonstrating the importance of active diagnosis and treatment of unilateral and bilateral hearing loss. Cochlear malformations such as Mondini dysplasia or enlarged vestibular aqueduct have been reported to be responsible for greater than 50% of unilateral hearing loss in children.[11]

While there are no significant complications from assessing for hearing loss in children, the primary concern is the risk of false results, both false positives and, more importantly, false negatives. A false positive or a falsely abnormal test may result in additional testing for the patient and added stress or anxiety for parents and caregivers, but ultimately, it will not result in long-term deficits for the patient. On the other hand, false negative or falsely normal testing in a patient with hearing loss may result in a delayed diagnosis of hearing loss. The associated social, intellectual, and developmental consequences of false-negative assessments may negatively affect the child's developmental potential. Complications of the hearing assessment, such as perforation of the tympanic membrane or injury to the ear canal, are rare and typically not long-term. These risks are present whenever the ear is instrumented. The presence of behavioral concerns during evaluation may increase the risk of any of the above complications and make the testing more challenging to perform. Behavioral issues may decrease the accuracy of results.

The assessment and management of hearing loss in children involve a multidisciplinary approach. Healthcare providers should work together as part of a coordinated team to ensure the rapid diagnosis and initiation of appropriate interventions. Communication between specialists is crucial to ensure that appropriate care is provided to each patient and that patients do not become lost to follow-up. Without a patient-centered team approach, efforts to improve the developmental potential of patients with hearing loss falter.