Cochlear implantation (CI), in the past more than 30 years, has become a routine intervention and its a standard of care for children with profound sensorineural hearing loss. The presence of a functional cochlear nerve is a key issue in pediatric CI. There are cases where implant candidacy decisions are difficult to make using existing evaluation procedures and among these cases are children with cochlear nerve deficiency (CND). The aim of this presentation is to highlight the importance of a comprehensive audiological, electrophysiological and radiological preoperative assessment in making a decision for cochlear implantation in prelingually deaf children.