Susan Wiley

Language performance in children with cochlear implants and additional disabilities

Quantify post‐cochlear implant (CI) language among children with disabilities and determine the role of nonverbal cognitive quotients (NVCQ) in predicting language.

Children with cochlear implants and developmental disabilities: A language skills study with developmentally matched hearing peers

The number of children receiving cochlear implants (CIs) with significant disabilities in addition to their deafness has increased substantially. Unfortunately, children with additional disabilities receiving CIs have largely been excluded from studies on cochlear implant outcomes. Thus limited data exists on outcomes in this population to guide pre-implant counseling for anticipated benefits. The study objectives were: (1) evaluate differences in post-cochlear implant language skills between children with cochlear implants and developmental disabilities and age/cognitively matched controls; (2) quantify possible discrepancies between language level and cognitive level. Fifteen children with a developmental disability who received a CI were matched 1:1 on nonverbal cognitive ability and age to hearing controls. Language was evaluated using Preschool Language Scale-IV and reported as language quotients. Multivariable mixed models for matched pairs analyzed differences in language levels between groups. No significant differences were seen between CI and control groups regarding insurance, maternal education, or family income level. Results of the multivariable models indicated that compared to matched controls, the CI group had significantly lower mean receptive (24.6 points, p=0.002) and mean expressive (21.9 points, p=0.001) language quotients after controlling for confounders such as number of therapies and weekly hours in therapy. Significant discrepancies between language level and cognitive level were seen among CI participants only. Compared to age- and cognitively matched controls, children with CIs had significantly lower language levels with delays disproportionate to their cognitive potential. Mechanisms behind this performance-functional gap need to be understood to deliver appropriate intervention strategies for this special population.

Auditory Skills Development among Children with Developmental Delays and Cochlear Implants

Objectives: We sought to understand auditory skills outcomes in young children with cochlear implants and developmental delay. Methods: Children who received cochlear implants at less than 36 months of age were identified via chart review. Their postimplant auditory skills outcomes were measured with the Auditory Skills Checklist. Results: Of 35 children who received cochlear implants before the age of 36 months, 14 children (40%) had additional disabilities or some form of developmental delay. The 12-month postimplant data indicated progress in all groups of children. Children with additional disabilities had the same rate of auditory skills progress as children with no additional disabilities (β = 9.3 versus 9.3; p = 0.5). However, the children with additional disabilities tended to start at a lower baseline skills set (approximately 6 points lower) on the Auditory Skills Checklist. For children with average developmental quotients (at least 80), the rate of progress was twice that of children with a developmental quotient of less than 80, irrespective of a developmental disability (β = 9.9 versus 4.8; p = 0.03). Children with a developmental quotient of less than 80 were less likely to gain skills in discrimination and identification after the first postimplant year. Conclusions: Children with additional disabilities make progress in auditory skills, but may not develop higher auditory skills of identification and comprehension within the first 6 months after implantation. Categorizing children according to a cognitive developmental quotient may provide more predictive ability than does categorizing them by disability type.

Findings from multidisciplinary evaluation of children with permanent hearing loss

OBJECTIVES To describe clinical findings from a multidisciplinary program for children with permanent hearing loss (PHL). METHODS Retrospective chart review at a tertiary care childrens hospital. PATIENTS Two hundred patients charts were selected from the population of 260 children with permanent hearing loss presenting between July 2005 and December 2006. MAIN OUTCOME MEASURES PHL etiology; radiographic findings; clinical findings by genetics, ophthalmology, developmental pediatrics, speech pathology, and aural rehabilitation. RESULTS Etiology of hearing loss was determined in 60% of subjects. Genetic causes of hearing loss were identified or presumed (positive history of first degree relative with hearing loss) in 27% of the children. Structural ear anomalies were found in 20% of children. Among the 36% of children with CNS imaging, abnormal findings were noted in 32%. There were a high rate of ophthalmological findings (53%) among children seen by ophthalmology (n = 105). Neurodevelopmental evaluations were completed in 58% of subjects and clinically significant findings were noted in 68%. Of the 61% of children who receiving received speech/language evaluations, 77% required intervention. Over half of the 40% of subjects who had an aural rehabilitation evaluation needed therapy. There were not significant differences in rates of findings for children with mild or unilateral hearing loss as compared to children with more severe degrees of hearing loss. CONCLUSIONS Interdisciplinary medical evaluation of children with PHL allows for the identification and treatment of clinically significant ophthalmologic, neurodevelopmental, genetic, and speech/language disorders. A high rate of CNS and temporal bone abnormalities were identified. These findings provide an understanding of the importance of considering thorough medical and developmental evaluations among children who are deaf/hard of hearing.

Autism spectrum disorders in 24 children who are deaf or hard of hearing

OBJECTIVES Approximately 4% of children who are deaf or hard of hearing have co-occurring autism spectrum disorder (ASD). Making an additional diagnosis of ASD in this population can be challenging, given the complexities of determining whether speech/language and social delays can be accounted for by their hearing loss, or whether these delays might be indicative of a comorbid ASD diagnosis. This exploratory study described a population of 24 children with the dual diagnosis of ASD and hearing loss. METHODS Children completed a comprehensive ASD evaluation using standardized autism diagnostic instruments (Autism Diagnostic Observation Schedule, language and psychological testing). Children with permanent hearing loss who had a developmental evaluation between 2001 and 2011 and were diagnosed with an ASD based on the results of that evaluation were included. Information on communication modality, language and cognitive abilities was collected. RESULTS The median age of diagnosis was 14 months (range 1-71) for hearing loss and 66.5 months (range 33-106) for ASD. Only 25% (n=6) children were diagnosed with ASD ≤ 48 months of age and 46% by ≤ 6 years. Twelve (50%) children were diagnosed with ASD, 11 were diagnosed with pervasive developmental disorder not otherwise specified and 1 child had Aspergers. Most (67%) had profound degree of hearing loss. Fourteen (58%) children had received a cochlear implant, while 3 children had no amplification for hearing loss. Nine (38%) of the 24 children used speech as their mode of communication (oral communicators). CONCLUSIONS Communication delays in children who are deaf or hard of hearing are a serious matter and should not be assumed to be a direct consequence of the hearing loss. Children who received cochlear implants completed a multidisciplinary evaluation including a developmental pediatrician, which may have provided closer monitoring of speech and language progression and subsequently an earlier ASD diagnosis. Because children who are deaf or hard of hearing with ASD are challenging to evaluate, they may receive a diagnosis of ASD at older ages.

Auditory Skills Checklist: Clinical Tool for Monitoring Functional Auditory Skill Development in Young Children with Cochlear Implants

Objectives: The Auditory Skills Checklist© (ASC) was developed to address the need for tools to evaluate functional auditory skill progress in very young children with sensorineural hearing loss. We describe the development, validation, and utility of the ASC for use in young children with cochlear implants. Methods: Using the ASC, we measured auditory skills in 37 subjects who received cochlear implants at no more than 36 months of age. Repeated measures analysis was conducted to determine expected auditory skill development after implantation. Interrater reliability was tested on a small subset. The ASC was compared to the Infant-Toddler Meaningful Auditory Integration Scale (IT-MAIS) to determine its validity in measuring functional auditory skills. Results: The ASC had excellent internal consistency (Cronbachs alpha, 0.98) and interrater reliability (intraclass correlation coefficient, 0.99), and was highly correlated with the IT-MAIS (r = 0.90). According to the repeated measures analysis, children who received a cochlear implant at 36 months of age or earlier were expected to increase their ASC score by 8 points every 3 months (beta coefficient, 8.3; p < .001). Conclusions: The ASC is a clinically relevant and easily administered tool for assessing the functional auditory skills of young children with a cochlear implant. By assessing auditory skill development over time with the ASC, we can better realize expectations for a particular child based on his or her age, hearing loss level, and management strategies in place.

Longitudinal functional performance among children with cochlear implants and disabilities: A prospective study using the Pediatric Evaluation of Disability Inventory

OBJECTIVE Functional outcomes are important in children with cochlear implants (CI) and additional disabilities as studies on auditory skill and speech/language development may not identify functional benefits from implantation. This study sought to measure functional performance skills of young children with developmental disabilities post-CI. METHODS Eight children with cognitive disabilities undergoing cochlear implantation were enrolled in a prospective study of language and functional abilities; 6 with 1 year follow-up were included in the analysis. Functional performance was measured using Pediatric Evaluation of Disability Inventory (PEDI), providing standardized (mean: 50) and scaled scores (range: 0-100) of functional domains: Self-Care, Mobility and Social Function. The PEDI was administered pre-implant, 6 and 12 months post-implantation along with language testing at the same intervals. RESULTS All children had cognitive disability; 5 also had motor delay. The ages at CI ranged from 13.8 to 134 months. For functional abilities, children did not make significant changes in domain-specific standard scores over 1 year. Children made progress in scaled scores by 1-year post-implant. The largest increase for all domains occurred in the first 6 months (7-11.5 point increase). For language abilities, children made a median 5.5-month increase in receptive language age (p=0.06) and 5-month increase in expressive language age (p=0.03) in the first year post-CI with no change in language quotients. Receptive language level was significantly (p<0.05) associated with increasing scores in the domains of Self-Care and Social Function. CONCLUSIONS This is the first study to measure daily functional abilities in children with implants and disabilities using a standardized tool. Although our small group of complex children did not have an increase in standard scores (gap-closing trajectories), they made progress in skill development on scaled scores. Receptive language appears to play a key role in social functioning in this population. Functional assessments are informative for treatment planning and identifying specific areas to target intervention.

Access to cochlear implant candidacy evaluations: Who is not making it to the team evaluations?

The objective of this study was to investigate trends in the referral process among pediatric cochlear implant candidates. Medical and audiologic charts between 2003 and 2005 were reviewed, and children five years and younger with moderately-severe or worse sensorineural hearing loss were included. Of the 105 audiograms meeting the inclusion criteria, 69% were referred for a cochlear implant, and 52% were considered as definite candidates for an implant by audiologists with expertise in cochlear implant technology. Children referred for an implant, compared to children who were not referred, were more likely to have married parents (91% vs. 70%, p=0.02) and more likely to have private insurance (56% vs. 29%, p=0.02). Multivariable regression results were consistent with the unadjusted findings regarding marital status, but not insurance status. Children with sensorineural hearing loss are inconsistently referred to cochlear implant teams despite similar audiologic findings. To reach the Healthy People 2010 goals, this disparity should be addressed. A further understanding of the population of children not referred is important in diminishing inconsistencies and understanding barriers to care.

Needs of Parents of Children Who Are Deaf/Hard of Hearing With Autism Spectrum Disorder

Little is known about children who are deaf or hard of hearing (D/HH) with a coexisting autism spectrum disorder (ASD). The objective of our study was to understand the needs of children who are D/HH with coexisting ASD. We posed questions for group discussion about diagnostic process, impact of dual diagnosis on communication, and helpful resources. Four parents of three children participated. Challenges in the diagnostic process included the challenges in the appropriateness of the evaluation tools and the limited expertise of the professionals performing the evaluations. Broad-based special educational settings were perceived as helpful. Families described a range of broad-based communication strategies (spoken, sign, and written language, augmentative communication approaches). Families prioritized a focus on behavior and day-to-day functioning over academic performance. Families recognized the lack of professionals who understand ASD and deafness but have found the internet and technology as a mechanism to connect to information and families with similar needs.

Functional performance among children with cochlear implants and additional disabilities

Abstract Objective To assess daily functional skills among young cochlear implant (CI) recipients with additional disabilities. Methods Children with CI and developmental disabilities and a hearing control group containing similar ages and similar disabilities were enrolled in a cross-sectional study of language and functional skills. Daily functional skills were measured using the Pediatric Evaluation of Disability Inventory (PEDI), which provides standard and scaled scores in Self-Care, Mobility, and Social Functioning domains. Language was assessed using the Preschool Language Scales, 4th edition which provides standard scores and age equivalents. Results Fourteen children with CIs and seven hearing controls were included in this analysis. Most children in this study (90.5%) had cognitive disabilities and 57% had motor disabilities. Compared with the hearing controls, children with CIs were less likely to have functional independence in any PEDI domain. Children with CI had significantly lower median social functioning standard scores compared with controls (17.3 vs. 27.5, P = 0.009). After controlling for nonverbal cognitive abilities and language level, the difference was no longer significant (19.1 vs. 24, P = 0.3). Among children with CI, age at implant and duration with device were not associated (P > 0.4) with PEDI scores. Discussion Although children with CI and developmental disabilities had similar functional skills as hearing children with similar disabilities regarding the PEDI domains of Self-Care and Mobility, they had lower Social Function standard scores. This lower social functioning among children with implants may be related to lower language levels and possible language deficits seen in the CI group. Regular assessments of functional abilities coupled with language abilities are essential in providing every child with the opportunity for maximizing the potential for independence.