Outcomes in Children with Additional Disabilities Following Cochlear Implantation: A Systematic Review

Background: Thirty percent of children with hearing loss have an additional disability. These children may be difficult to test according to standard audiologic behavioral test protocols. Additionally, progress within this population may present differently than in children with no additional disability. Currently, no evidence-based protocol exists for assessing cochlear implant benefit and outcomes in this population. Objective: The purpose of this investigation is to perform a systematic review on the outcomes of cochlear implantation in children with additional disabilities. Specifically, this study focused on areas of function assessed, outcome measures used, and evidence of benefit observed. Methods: A comprehensive search was conducted utilizing the databases MEDLINE/PubMed, OneFile, ProQuest Nursing & Allied Health Source. The keywords used to identify relevant studies included pediatric, special needs, developmental disabilities, functional assessment, outcomes, benefit.” The keywords cochlear implantation or cochlear implant” were present throughout all searches. Results: Included in this study were 24 articles. The results revealed that despite wide variability among the studies, some benefit was observed in children with cochlear implantation and additional disabilities in the areas of auditory skills and speech perception, receptive and expressive language, and adaptive behaviors. Discussion: Many challenges arose when studying this population. Limited experimental control as well as wide variability in disability type were major issues noted throughout this review. However, overall children with cochlear implantation and additional disabilities showed some improvement in all areas, although they still did not perform as well as children with cochlear implantation and no additional disabilities, or normally hearing peers matched according to age and cognitive abilities. Conclusions: Research in this area is challenging due to the limitations involved in the ability to produce randomized, double blind studies to determine value of cochlear implantation in this population. Cognitive ability is a strong, but not the only, predictor of performance. Although on average the lower the cognitive ability, the lower the post implant performance, there was much variability among participants, adding to the challenge of deciding whether to implant such a child. There is some evidence to support the implantation of children with additional disabilities, however, more research is recommended involving more multicenter collaborations to increase the participant pool and to isolate individual disabilities to establish performance. Research should continue to explore use of alternative assessments such as quality of life measures