Objective: Imaging characteristics and hearing outcomes in children with cochleovestibular or cochleovestibular nerve (CVN) abnormalities. Study Design: Retrospective, critical review. Setting: Tertiary referral academic center. Patients: Twenty-seven children with CVN abnormalities with magnetic resonance (MRI) and/or computed tomography (CT). Study Intervention(s): None. Main Outcome Measure(s): Determine the likely presence or absence of a CNV and auditory stimulation responses. Results: Two of 27 cases had unilateral hearing loss, and all others had bilateral loss. Eleven (46%) were identified with a disability or additional condition. Twenty-two (42%) ears received a cochlear implant (CI) and 9 ears (17%) experienced no apparent benefit from the device. MRI acquisition protocols were suboptimal for identification of the nerve in 22 (42%) ears. A likely CVN absence was associated with a narrow cochlear aperture and internal auditory canal and cochlear malformation. Thirteen (48%) children with an abnormal nerve exhibited normal cochleae on the same side. Hearing data were available for 30 ears, and 25 ears (83%) exhibited hearing with or without an assistive device. One child achieved closed set speech recognition with a hearing aid, another with a CI. One child achieved open set speech recognition with a CI. Conclusions: Current imaging cannot accurately characterize the functional status of the CVN or predict an assistive device benefit. Children who would have otherwise been denied a CI exhibited auditory responses after implantation. A CI should be considered in children with abnormal CVN. Furthermore, imaging acquisition protocols need standardization for clear temporal bone imaging.