Cochlear implants in eight children with Down Syndrome - Auditory performance and challenges in assessment

Objectives: A small proportion of children with Down Syndrome (DS) have severe to profound hearing loss and may potentially benefit from a cochlear implant (CI). Evidence on outcomes in DS is very limited, and there is a need for further investigation to provide a basis for clinical evaluation of candidates and outcomes. This study aims to explore outcomes of CI in children with DS in Norway.

Methods: Data on all children with DS and CI in Norway were collected from the CI registry and patients' records at the national pediatric CI center. Main outcome measures were: use of CI, Category of Auditory Performance (CAP), Speech Intelligibility Rate (SIR), and parents' and caregivers' views of the benefits of CI.

Results: Eight children with DS have received CI in Norway, all bilaterally. The outcomes varied greatly. All children used their CIs, and all of them responded to environmental sounds. Four children reached CAP score 5 after several years of use, (i.e., they understand phrases without lip reading). All children scored at least 2, (i.e., responds to speech sounds). One child reached a SIR score of 3, (i.e. connected speech is intelligible to experienced listeners). The rest of the children reached SIR scores of 1 or 1-2, (i.e., connected speech is unintelligible). Without exception, parents had an entirely positive attitude to their children using a CI. Co-morbidity and middle ear disease frequently delayed implantation.

Conclusions: Our experience with CI in children with DS is positive. However, CI cannot replace other types of communication for these children, and it is important to give parents realistic expectations prior to surgery. Outcomes might be considered limited when evaluated with instruments for testing auditory performance and speech intelligibility constructed for children without additional disabilities. We do not believe that such outcomes reflect the benefit in real life.