Variability of Vestibular Function in Pediatric Patients with Auditory Neuropathy Spectrum Disorder

Background/Objectives: Auditory neuropathy spectrum disorder (ANSD) is a well-established hearing disorder in the pediatric population and is estimated to account for at least 10% of children with sensorineural hearing loss. Compared to auditory function, vestibular function in children with ANSD has not been well described in the past. The purpose of this study is to examine vestibular testing results in children with ANSD and to better characterize vestibular dysfunction in these children. Methods: A retrospective review of vestibular laboratory testing results was conducted in pediatric patients diagnosed with ANSD. Vestibular evaluation included vestibular evoked myogenic potential (VEMP), rotary chair test, video head impulse test (vHIT), and videonystagmography (VNG). Results: A total of 30 pediatric patients with ANSD were identified, including 18 boys and 12 girls, with a mean age of 4.5 years. Bilateral ANSD was found in 24 cases, while 6 cases were unilateral. Etiologies of ANSD included a history of hyperbilirubinemia in infancy, cochlear nerve dysplasia, genetic-related conditions, etc. Vestibular dysfunction was found in 12 cases, as indicated by at least one abnormal outcome in VEMP, vHIT, or rotary chair testing. Nineteen children were cochlear implant candidates and eventually underwent unilateral or bilateral implantation. Conclusions: Vestibular dysfunction is significant in pediatric patients with ANSD, and vestibular outcomes appear to be related to underlying etiologies. Formal vestibular evaluation is necessary to identify such vestibular losses, and these findings will be helpful to guide clinical management and rehabilitation strategies for these children.