More Than Hearing Loss

Study Participants

Children less than 6 years of age who had received a cochlear implant by the age of 3 years.

Study Objective

The objective of the study was to understand the post-implant language skills of children with developmental disabilities. Children with implants were matched to a control group of hearing children with similar ages and disabilities.

This was the first study to use this sort of comparison group as most studies will look at outcomes in typically developing children who are deaf or hard of hearing.

Findings

There were several important findings from this study.

1. Language scores were higher when sign language was incorporated into the test results.
2. Cognitive abilities of the child were the strongest predictor of language outcomes in this group of children. In fact, cognitive abilities in this population were more important than the age at which a child had received the cochlear implant.
3. Even though children made language gains post-implant, children with additional disabilities were performing well below their cognitive ability when compared to hearing children of similar ages and disabilities. This finding has compelled us to better understand why language levels remain lower.

Study Related Publications

Meinzen-Derr, J, Wiley, S, Grether, S, Choo, D. Language performance in children with cochlear implants and additional disabilities. Laryngoscope. (120) 405-413. 2010.

Meinzen-Derr, J, Wiley, S, Grether, S, Choo, D. Children with cochlear implants and developmental disabilities: a language skills study with developmentally matched hearing peers.Research in Developmental Disabilities. (32) 757-767. 2011.

Study Participants

Deaf children with additional disabilities who had received a cochlear implant.

Study Objective

The study’s objectives were to evaluate language and functional outcomes of deaf children with additional disabilities who had received a cochlear implant.

We aimed to:

1. Describe the natural history of language and functional skill progression of deaf children with disabilities who receive cochlear implants.
2. Assess the impact of a cochlear implant on language and functional competencies among children with disabilities.

This was the first study to measure daily functional abilities in children with implants and disabilities using a standardized tool.

Findings

Our findings indicated that children with additional disabilities and a cochlear implant made progress in social communication skills, self-care skills, and mobility. However, improvements in language comprehension were needed for this progress to occur, as language plays a key role in social functioning.

In fact, our findings indicated that cognitive and language abilities together explained the majority of the functional skill abilities that we measured. A detailed functional portfolio can be helpful in structuring or modifying interventions.

We have used the findings from this cochlear implant study to expand to children with all degrees of hearing loss.

Study Related Publications

Wiley, S, Meinzen-Derr, J, Grether, S, Choo, D, Hughes, M. Longitudinal functional performance among children with cochlear implants and disabilities: a prospective study using the Pediatric Evaluation of Disability Inventory. International Journal of Pediatric Otorhinolaryngology. (76) 693–697. 2012.

Study Participants

Children between the ages of 6 months and 8 years with dual sensory impairment (vision and hearing loss) who had received a cochlear implant were recruited from across the United States to participate in an evaluation of language skills. 115 children were recruited as study subjects from a variety of settings, including state deaf-blind programs, Part C (Early Intervention) programs, and clinical cochlear implant centers across 27 states within the United States.

The International CHARGE Syndrome Conference, a meeting that includes families and children with CHARGE syndrome was also used as a forum to inform and families about the study for recruitment.

About the Study

2007-2009—Phase 1—recruited children with dual sensory impairment who had already received a cochlear implant.
2009-2011—Phase 2—prospectively enrolled children who were in the process of receiving a cochlear implant. Children in this group completed a pre-cochlear implant evaluation as well as assessments at 6 months, 12 months, and 18 months following implantation.

Study Objective

This is the first study of this magnitude to report on a consistently and reliably administered language evaluation developed for vision impairment in children and adapted for children with dual sensory impairment post-implantation. The children included in this study were quite heterogeneous and complex (only 14% with isolated vision and hearing loss), which is not uncommon in children with dual sensory impairment.

Approximately 20% of children with dual sensory impairments reach higher level receptive (following simple and more complex directions) and expressive language abilities (speaking in simple or complex sentences) post-implant. The remainder continues to perform at pre-linguistic levels of communication. Higher developmental quotients are strongly associated with higher levels of communication. This is not particularly surprising if children have neurologically or cognitively based limitations, since this would also impact their likely language progress. There are a number of children who can hear and see but remain at pre-linguistic levels of communication based on the severity of their disabilities.

Submitted for publication

(Use of the Ages and Stages Questionnaire in Young Children who are Deaf/Hard of Hearing)

Study Participants

Children with sensorineural hearing loss have a high rate of additional disabilities beyond those expected by the impact of hearing loss on language and are identified later than children without hearing loss. Identifying these developmental delays and allowing earlier implementation of interventions specific to these concerns would improve care.

Study Objective

The aim of this study was to determine whether the Ages and Stages Questionnaire© (ASQ) can identify unmet needs or unidentified developmental delays among young children with bilateral sensorineural hearing loss.

Findings

While 32% of our population had a developmental delay outside the domain of communication, the ASQ had poor sensitivity on the overall score without the communication domain as well as for fine motor, cognitive, personal-social domains. It had good sensitivity for the domains of communication and gross motor skills and good specificity (ranging 83-85%) on specific domains as well as for the overall score (70%).

The ASQ does not provide an effective mean for identifying additional developmental concerns in young children with bilateral sensorineural hearing loss. Further studies are needed to determine how to identify additional disabilities in a timely manner among young children with hearing loss.

Study submitted for publication.

Our early studies focused on qualitative benefits of cochlear implants in children with complex medical and developmental needs. Through a series of parent interviews of 20 children who were deaf with an additional disability who received a cochlear implant, we had a better understanding of a broader range of positive cochlear implant outcomes for children with additional disabilities.

Findings

After receiving a cochlear implant, parents reported their children having improved awareness of the environment and connectedness, and communication skills. All families said that if they were to make the decision again, they would choose to have their child implanted.

Study Related Publications

Wiley, S, Jahnke, M, Meinzen-Derr, J, and Choo, D. Perceived qualitative benefits of cochlear implants in children with multi-handicaps.International Journal of Pediatric Otorhinolaryngology. 69, 791—798. 2005.

Study Participants

Children with developmental delays who received a received a cochlear implant.

About the study

We looked at children with developmental delays to determine how they gained auditory (listening) skills after they received a cochlear implant. The children with delays made about half the rate of progress of children with implants who were typically developing. We used the Auditory Skills Checklist, which uses a combination of therapist observations and parent observations to understand how a child listening skills are progressing.  The form is used in every phase from detection to discrimination to identification and finally to comprehension.

The Auditory Skills Checklist is available for use at no cost and has been used in many settings (early intervention, educational settings, therapy settings) and across the United States.

Findings

We learned a few key points that have helped us better understand children with a dual diagnosis of hearing loss and disabilities.

1. Children with additional disabilities make measureable listening skill progress post-implant.
2. The rate of listening skill progress was about half that of children without disabilities. This means that it might take twice as long to gain the same skills as a child without disabilities who received a cochlear implant.
3. In the first 6 months after receiving an implant, children may not develop the higher order skills of identification and comprehension, though this is highly dependent upon the cognitive ability of the child (and not the disability diagnosis or label).

Study Related Publications

Wiley, S, Meinzen-Derr, J, Choo, D. Auditory skills development among children with developmental delays and cochlear implants. Annals of Otology, Rhinology & Laryngology. 117:711-718. 2008.