Children with cochlear implant and additional disabilities benefit from consistent device use.

Objectives: Although the prevalence of additional disabilities (AD) in children with a cochlear implant (CI) is high, children with such disabilities are often excluded from clinical studies, or their specific characteristics are only partially included. The literature shows that several factors need to be considered in evaluating auditory and language development in CI children with AD, including demographic variables as well as the severity and type of disability. Current findings on device use in children show correlations with auditory and language outcome, but little is known about device use specifically in children with AD. The purpose of this study was to determine the auditory and language outcome of CI children with AD and to analyse their datalogging-based daily device use, both 1 year and 2 years after implantation. In addition, any potential correlations between outcome and device use were to be identified.

Methods: A cohort of 32 CI children with 5 different types of AD were included in this retrospective analysis. The children's auditory and language outcome was assessed by the parental questionnaires LittlEARS and ELFRA and by the professional observation tool CAP (Categories of Auditory Performance) 1 and 2 years after implantation. Longitudinal device use was analysed by using the CI system-integrated data-logging; daily duration of CI use, number of coil disconnections and exposure to different listening scenes were recorded.

Results: Overall, the cohort's auditory and language performance showed significant progress over time, while reduced abilities became more obvious after 2 years of CI experience. The mean daily duration of CI use increased significantly from 7.8 ± 2.8 to 8.2 ± 2.7 h after 2 years. High numbers of daily coil disconnections were detected, with a significant mean decrease from 83.4 ± 73.1 to 66.3 ± 54.6 whereas the percentage exposure to different listening environments was widely stable over time. Significant rank correlations were identified between outcomes measured by ELFRA and CAP with daily duration of CI use, numbers of coil disconnections and percentage of exposure to speech-characterised listening scenes.

Conclusion: The auditory and language outcome in CI children with AD is variable, but it progresses over time. Children benefit from a consistent daily device use as well as from a high exposure to speech-characterised environments. Device use should be monitored constantly, with particular focus on daily duration of CI use and, in particular, on the number of coil disconnections if children have a severe motor impairment. Objective data-logging is an important addition to outcome assessment by testing, observations and parental questionnaires. Although assessment in children with AD is a major challenge for professionals, comprehensive assessment is needed to improve cochlear implant services with special adaption to children with AD, and this should include audiological, development-related and psychosocial information. A unified system to classify types of disabilities could help to improve procedures for analysing different outcomes.