Aims Compared with adults, paediatric cochlear implant (CI) recipients more often have additional disabilities, syndromes and inner ear malformations. The aim of the talk to highlight the inner ear abnormalities and outcomes with CIs Methodology Retrospective reviews of CI recipients at NextSense (SCIC) for the cochlear nerve dysplasia (CND), CHARGE syndrome, Large vestibular aqueduct syndrome(LVAS) and large internal auditory meatus (LIAM) cohorts. Retrospective international collaboration for the common cavity cohort (CC) Results Children with CND, may have further investigations with transtympanic electrically evoked auditory brainstem response looking for evidence of neural transmission. In our study of 50 children with CND, with 71 CIs, half the children were able to develop some open set verbal understanding and three quarters used some spoken language. Additional disabilities and syndromes were present in approximately half of the children. In children with CHARGE syndrome and CIs, outcomes are variable with anatomy abnormalities encountered. In our study reporting on 10 children: all had small mastoids and absent lateral semicircular canals, seven with no or abnormal round windows, six with abnormalities of the facial nerve and CND was present in the congenital profoundly deaf children. In LVAS we reported on 162 CI users, 130 children and 32 adults, outcomes had a lower quartile of 85% on BKB at 12 months’ post-CI. Outcomes were reported in seven children with CC, these were variable, but improved over time and CAP scores of five or more were possible for some. In LIAM was reported in five children, and associated with previous episodes of meningitis in two children due to CSF leakage. Conclusion Children with abnormal inner ear anatomy can benefit from CIs. Careful counselling is needed regarding variable outcomes.