Prevalence of Cochlear Nerve Deficiency and Hearing Device Use in Children With Single-Sided Deafness

ObjectiveThis study aimed to assess the prevalence of cochlear nerve deficiency (CND) in a cohort of pediatric patients with single-sided deafness (SSD). A secondary objective was to investigate trends in intervention and hearing device use in these children. Study DesignCase series with chart review. SettingPediatric tertiary care center. MethodsChildren ages 0 to 21 years with SSD (N = 190) who underwent computerized tomography (CT) and/or magnetic resonance imaging (MRI) were included. Diagnostic criteria for SSD included unilateral severe-to-profound sensorineural hearing loss with normal hearing sensitivity in the contralateral ear. Diagnostic criteria for CND included neuroradiologist report of an "aplastic or hypoplastic nerve" on MRI or a "stenotic cochlear aperture" on CT. ResultsThe prevalence of CND was 42% for children with CT only, 76% for children with MRI only, and 63% for children with both MRI and CT. Of the children with MRI and CT, there was a 90% concordance across imaging modalities. About 36% of children with SSD had hearing devices that routed sound to the normal hearing ear (ie, bone conduction hearing device/contralateral routing of signal), while only 3% received a cochlear implant. Approximately 40% did not have a hearing device. Hearing device wear time averaged 2.9 hours per day and did not differ based on cochlear nerve status. ConclusionThere is a high prevalence of CND in children with SSD. Cochlear nerve status should be confirmed via MRI in children with SSD. The limited implementation and use of hearing devices observed for children with SSD reinforce the need for increased support for early and continuous intervention.