Prevalence and surgical management of cholesteatoma in Down Syndrome children.

OBJECTIVE: The objective was to report the outcomes of surgical management of cholesteatoma in Down Syndrome (DS) children and to determine the prevalence of cholesteatoma in DS children at three academic tertiary care centers. METHODS: A retrospective chart review was performed of all DS children diagnosed with cholesteatoma from three tertiary care academic centers. Demographic data, comorbidities, imaging findings, surgical management, and pre- and postoperative pure tone averages were collected. RESULTS: The prevalence of cholesteatoma in DS patients at these three institutions was calculated to be 8/802 (0.998%), 4/448 (0.893%), and 4/1016 (0.394%), with an average prevalence of 0.762%. Of the 16 patients with cholesteatoma identified, the average age of diagnosis was 10.8 years, and 5/16 (31%) had at least three sets of pressure equalizing tubes (PETs) placed. Of the 13 patients with preoperative CTs, 4/13 (30.8%) had blunting of the scutum, 4/13 (30.8%) had tegmen involvement, 1/13 (7.7%) had erosion of the semicircular canal, 2/13 had facial nerve dehiscence, and 7/13 (53.8%) had ossicular chain erosion. For surgical management, disease control was attained in 4/16 children with tympanoplasty only and canal wall up (CWU) tympanomastoidectomy in 11/16 patients. Available pre- and postoperative audiograms were evaluated; however, there was insufficient audiological data to contribute to outcomes. CONCLUSION: Cholesteatoma occurs in nearly 1% of DS patients, and the disease tends to be advanced with tegmen involvement in nearly a third of patients, and ossicular erosion seen in more than half the patients. A CWU procedure can be successful in managing DS patients with cholesteatoma when consistent long term follow-up is planned. Close monitoring and a high index of suspicion for cholesteatoma are essential to prevent delays in diagnosis and treatment of DS patients with a history of multiple sets of PET placement.

Assessment and Treatment of Behavioral Disorders in Children with Hearing Loss: A Systematic Review.

OBJECTIVE: There is evidence that children who are deaf and hard of hearing (DHH) have a higher incidence of behavioral disorders. Assessment of behavioral health in this population is often complicated by language developmental delays, which may result in unrecognized and untreated behavioral problems. The purpose of this study is to assess the association of behavioral disorders among children who are DHH and to explore behavioral interventions for children in this population. DATA SOURCES: PubMed, CINALH, PsychINFO, and Web of Science. REVIEW METHODS: Search terms included the following: problem behavior, child behavior disorders/diagnosis, child behavior disorders/psychology coupled with hearing loss, cochlear implants, hearing aids, or deafness. Studies from the last 30 years (1985-2016) were included. The articles were reviewed independently by 3 reviewers. RESULTS: Thirty-six articles met criteria. There was an association between internalizing behaviors and hearing loss among children, which may persist after cochlear implantation. These problems may be more pronounced for children with additional disabilities. Conduct and hyperactivity disorders as well as emotional and executive function problems among children who are DHH may be related to poor language development. There was limited evidence regarding interventions to address the behavioral disorders of DHH children. CONCLUSIONS: There is a significant body of evidence demonstrating behavioral problems among DHH children but a lack of clear understanding of the mechanisms involved. There is limited evidence on interventions to address the behavioral problems of DHH children. Future research is warranted to mitigate the long-term effects of disruptive behavior among these children.

Barriers to Rehabilitation Care in Pediatric Cochlear Implant Recipients.

OBJECTIVE: The purpose of this study was to assess barriers to rehabilitation care for pediatric cochlear implant (CI) recipients. STUDY DESIGN: Cross-sectional questionnaire study. SETTING: Tertiary medical center. PATIENTS: Parents of children who received a CI from October 1996 to June 2013. MAIN OUTCOME MEASURE(S): Factors related to access to and barriers in audiology and speech therapy services, factors related to CI use, and performance with CI using the Parents' Evaluation of Aural/Oral Performance of Children (PEACH). RESULTS: Thirty-five parents participated in the study (21 rural residents and 14 urban residents). Distance was a significant barrier to audiology services for rural participants compared with urban participants (p = 0.01). Consistent CI use was complicated by mechanical complications or malfunction in 70% of rural children compared with 33% of urban children (p = 0.05). Only 10% of rural children were able to access speech therapy services at diagnosis compared with 42% of urban children (p = 0.04). Low socioeconomic (SES) status and Medicaid insurance were associated with a lack of local speech therapists and medical/mechanical CI complications. Higher parental educational attainment was associated with higher PEACH scores in quiet conditions compared with families with lower parental education (p = 0.04). CONCLUSIONS: Rural children are often delayed in receipt of CI rehabilitation services. Multiple barriers including low SES, insurance type, and parental education can affect utilization of these services and may impact the recipient language development. Close follow-up and efforts to expand access to care are needed to maximize CI benefit.