Background: About 25 % children with profound deafness have additional disabilities. For children who cannot be evaluated by speech perception tests, an alternative scale scoring-method was designed by the CI-team at Rikshospitalet, reflecting subjective observations on reactions to sound and the desire to use CI. Study question: To explore the benefit from CI in children with profound hearing loss and complex disabilities. Additionally the impact of cognitive dysfunction on benefit was explored. Population and method: Retrospective review of 127 medical records from all children receiving CI during 2008 – 2011 at Rikshospitalet, Norway. Two scales were used for evaluation. The standard scale from 0-10 (10 normal speech development) was based on speech perception only. When not applicable, the alternative scale from 0-4 (0 = non-user, 4 = great benefit, speech development) was applied. Results: Thirty (24 %) children had complex disability. Of these, nine (30 %) had a cognitive dysfunction. Sixteen (53 %) of the children with complex disabilities could be tested with the normal speech perception test, and their mean scores equaled mean to scores of otherwise “healthy” children with CI (6,6 and 7,6 respectively). The alternative scale was applied for 14 children with complex disorders (nine of them with cognitive dysfunction), the mean score (2,4) indicated that these children also benefited from CI. Forty-two (33 %) children were not yet tested because of young age or testing difficulties.Conclusion: In this study children with complex disabilities without cognitive dysfunction did as well with CI as children without complex disabilities, in terms of speech development. When cognitive dysfunction was present, alternative scale scores indicated a clear, but variable benefit. Further studies on this group of children with CI are important in order to develop appropriate tools for evaluation and prognosis.