Vestibular function in children with vestibulocochlear nerve aplasia/hypoplasia

• Published in: Acta oto-laryngologica Vol. 143; no. 10; pp. 1 - 866
• Main Authors: Karpeta, Niki, Asp, Filip, Edholm, Kaijsa, Bonnard, Åsa, Wales, Jeremy, Karltorp, Eva, Duan, Maoli, Verrecchia, Luca
• Format: Journal Article
• Language: English
• Published: England 2023
• Subjects: Medicin och hälsovetenskap; Vestibular function; vestibular evoked myogenic potentials; children; cochlear implants; video head impulse test; vestibulocochlear nerve aplasia
• ISSN: 0001-6489; 1651-2251; 1651-2251
• DOI: 10.1080/00016489.2023.2285453

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. Our aim was to describe the vestibular function in patients with VCN hypoplasia/aplasia before a possible CI. 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). 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 (  < 0.05). Canal function may represent a predictor of auditive pathway integrity with a possible favourable audiological outcome after CI operation. Our results demonstrate high vestibular response rates suggesting a functioning pathway despite the radiological diagnosis.