Children with unilateral hearing loss (UHL) have difficulty hearing in noisy environments and localizing sounds, impacting learning and social opportunities across contexts. Using a visible device like a cochlear implant (CI) may improve functioning but can also create psychological risk. Audiological measures alone are insufficient for predicting social, emotional, educational, adaptive, and quality-of-life post-operative outcomes, which are highly variable in this population and can also be impacted by a secondary diagnosis. Extending beyond audition to consider the “whole child” through neuropsychological evaluation may produce a sharper picture of potential outcomes, with or without surgical/audiological intervention. Given recent FDA approval for CI in children with UHL, more are receiving this elective surgery despite difficulties predicting who will experience significant benefit. Here we describe neuropsychological profiles of children with UHL who underwent CI candidacy evaluation at a tertiary pediatric hospital.

During pre-operative clinical care, CI candidates completed targeted neuropsychological evaluation to identify patient- and family-level factors that could impact CI use and outcomes from surgery. Cognitive, language, attention/executive, visuoperceptual/visuomotor, academic, adaptive, and emotional/behavioral functioning were assessed. Evaluations integrated history, observations, caregiver report forms, and performance-based test data.

18 individuals were evaluated (age 7-months to 16-years). Most had left-sided UHL (67%) and were male (61%). Known hearing loss etiologies were congenital cytomegalovirus (n=5), enlarged vestibular aqueduct (n=1), traumatic brain injury (n=1), meningitis (n=1), cholesteatoma (n=1), neurofibromatosis type 1 (n=1), and Waardenburg syndrome (n=1). Indices of general cognitive ability were generally low average to average. Patterns of cognitive impairment were not restricted to language-based tasks (e.g., Beery VMI-6 range 56-109, M=89.42, SD=16.27). Standardized parent ratings of everyday executive functioning, social/emotional/behavioral functioning, and adaptive skills were collected. Eight (44%) had a behavioral health diagnosis: Attention Deficit Hyperactivity Disorder (n=2), Global Developmental Delay (n=2), Unspecified Neurodevelopmental Disorder (n=2), Autism Spectrum Disorder (n=1), and Depression (n=1). Thirteen (72%) received or will receive a CI, of whom 38% had a behavioral health diagnosis. Average Area Deprivation Index (a marker of socio-economic status) was lower for individuals who ultimately received CIs (M=18%tile) compared to those who did not (M=25%tile).

There may be increased rates of neurodevelopmental/psychological conditions among children with UHL, especially when the etiology involves the central nervous system. Albeit preliminary, results align with findings from bilateral hearing loss samples. Findings highlight the importance of routine neuropsychological screening in children with UHL and close interdisciplinary collaboration for optimal outcomes. Socio-economic disparities among those who do and do not receive CI need further exploration as those who did not receive CIs tended to be from less resourced neighborhoods. Additional research is warranted to understand the full range of risk and protective factors for children with UHL and how these relate to outcomes for those who opt for cochlear implantation.