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Children less than 6 years of age who had received a cochlear implant by
the age of 3 years.
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
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.
There were several important findings from this study.
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.
Deaf children with additional disabilities who had received a cochlear implant.
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:
This was the first study to measure
daily functional abilities in children with implants and disabilities using a
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.
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
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.
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
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)
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.
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.
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.
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
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.
Wiley, S, Jahnke, M, Meinzen-Derr, J, and
Choo, D. Perceived qualitative benefits of cochlear implants in children with
Journal of Pediatric Otorhinolaryngology. 69, 791—798. 2005.
Children with developmental delays who received a received a cochlear
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.
We learned a few key points that have helped us better understand children with
a dual diagnosis of hearing loss and disabilities.
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.
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