Outcomes and benefits of pediatric cochlear implantation in children with additional disabilities: a review and report of family influences on outcomes

Abstract

The number of children with hearing loss with additional disabilities receiving cochlear implantation has increased dramatically over the past decade. However, little is known about their auditory and speech and language development following implantation. The purpose of this review is to evaluate the effects of cochlear implantation on the most common genetic and developmental disorders in children with hearing loss. Benefits of cochlear implantation for children with autism spectrum disorder, developmental delay, CHARGE syndrome, cerebral palsy, learning disorders, Usher syndrome, Waardenburg syndrome, and attention deficit/hyperactivity disorder are reviewed. Our review indicates that children with hearing loss and additional disabilities benefit from cochlear implantation, especially when implanted early. Thus, early interventions seem as important for these children as for deaf children without additional disabilities. Comparisons of outcomes across these disabilities indicate that children with little to no cognitive impairment (eg, Waardenburg sydrome, attention deficit hyperactivity disorder) have better outcomes than those with greater deficits in intellectual functioning (eg, autism, CHARGE syndrome). In addition, parents of children with hearing loss and additional disabilities report higher levels of parenting stress and greater child behavior problems than those without comorbid diagnoses. However, these parents are as sensitive when interacting with their children as parents with typically developing children using cochlear implantation. Given these results, it is critical to evaluate these children’s developmental milestones to provide early implantation and intervention, appropriately counsel families regarding realistic expectations for the implant, and facilitate family adaptation.

Comparisons of IQ in Children With and Without Cochlear Implants: Longitudinal Findings and Associations With Language

“…Data were drawn from the Childhood Development after Cochlear Implantation (CDaCI; NIDCD R01DC004797) study, which is a multi-center, national cohort investigation of the effectiveness of CIs in deaf children in relation to their hearing peers (Fink et al 2007). The larger parent study examined a variety of outcomes in children before and after cochlear implantation, including expressive and receptive language, quality of parent-child interactions, joint attention, psychosocial development, and health-related quality of life (Niparko et al 2010; Cruz et al 2013; Quittner et al 2013; Cejas et al 2015).…”

“…Traditionally, outcomes for children with CIs have focused on speech perception, oral language skills, and literacy while ignoring the important role cognition plays in facilitating these outcomes (Blamey et al 2000; Pyman et al 2000; Sarant et al 2001; Geers et al 2003; Marschark et al 2007; Quittner et al 2007; Niparko et al 2010; Knoors & Marschark 2014). Despite the known benefits of cochlear implantation, there is well-documented variability in these outcomes (Svirsky et al 2000; Niparko et al 2010; Geers & Sedey 2011) that may be related to cognitive abilities (e.g., visual attention) (Smith et al 1998; Cejas et al 2015; Quittner et al 2016). Measures of intelligence are the most broad-based, valid indicators of these developing abilities in children, and are significantly related to language learning, behavioral and social development, and academic functioning (Marschark et al 2007; Barker et al 2009; Wiefferink et al 2012; Hoffman et al 2014, 2015; Quittner et al 2016).…”

Health-Related Quality of Life Instruments for Children With Cochlear Implants: Development of Child and Parent-Proxy Measures

“…Assessing these domains is crucial for deaf children with cochlear implants (CIs), who have deficits in social competence, externalizing behavior, and linguistic skills (Quittner et al 2007;Barker et al 2009;Cejas et al 2014;Hoffman et al 2015). Furthermore, these deficits are more pronounced in CI children with comorbid conditions, who account for 30-40% of the CI population (Johnson & Wiley 2009;Cruz et al 2012;Cejas et al 2015;Inscoe & Bones 2016).…”

Use of Functional Near-Infrared Spectroscopy to Predict and Measure Cochlear Implant Outcomes: A Scoping Review

“…All of the records included in this review recruited CI-users who were otherwise healthy, excluding participants at the recruitment stage for reasons such as cognitive disorder, neurological illness, or brain injury. However, it is well documented that individuals with hearing loss often have other comorbid conditions, such as developmental delay, autism spectrum disorder or cerebral palsy in children [96,97], and cognitive and psychological impairments in adults [98,99]. Whilst the inclusion or exclusion of individuals with such conditions from research studies is justifiable during early work in this field, it will be important to consider at what stage future work will include participants on such grounds and how these decisions might impact the applicability of a future clinical tool.…”