Vestibular function in children with vestibulocochlear nerve aplasia/hypoplasia

Abstract

Background Infants and young children with vestibulocochlear nerve (VCN) hypoplasia/aplasia present with severe hearing loss and are candidates for cochlear implantation (CI). It is unknown whether vestibular function is related to CI outcome and if vestibular tests can guide the operation decision. Aims/Objectives Our aim was to describe the vestibular function in patients with VCN hypoplasia/aplasia before a possible CI. Materials and Methods Forty-two ears in 23 patients were tested between 2019 and 2022 with bone-conducted cervical vestibular evoked myogenic potentials (BCcVEMP), video head impulse test (vHIT) and miniice-water caloric test (mIWC). Results All ears could be tested with at least one vestibular test and 83% could be tested with more than one method. Twenty-nine ears (61%) showed normal function with at least one method. The presence of a normal response to any test doubled the likelihood of a measured hearing threshold after CI, the best predictors being the BCcVEMP and vHIT (p < 0.05). Conclusion Canal function may represent a predictor of auditive pathway integrity with a possible favourable audiological outcome after CI operation Significance Our results demonstrate high vestibular response rates suggesting a functioning pathway despite the radiological diagnosis.