The cornerstone of successful cochlear implantation has been the presence of a population of cochlear nerve endings which are able to mount a neural response to electrical stimulation. The authors of this paper present their experience of five children with absent or hypoplastic cochlear nerves who received cochlear implants. All children used their implants daily and had some improvement in perception of sound but only one achieved intelligible speech, in common with other similar series. There were no pre-operative imaging or auditory factors that predicted good performance. It appears that a small number of children with apparent cochlear nerve dysplasia may benefit from cochlear implantation, but it is currently impossible to predict which. Further, more sophisticated imaging or physiological tests will probably tell us in the future.