IMPORTANCEIn 2019, the US Food and Drug Administration approved cochlear implantation for children with single-sided deafness (SSD). The absence of robust clinical data specific to pediatric patients to guide shared decision-making and to identify potential advantages is a challenge in family counseling.OBJECTIVE To evaluate the audiological and patient-reported outcomes in children who underwent cochlear implantation for SSD and to assess the association between time of implantation, subjective outcomes, and cochlear implant device use rates.DATA SOURCE MEDLINE, Embase, Scopus, Cochrane, and PubMed were searched for English-language articles that were published in a peer-reviewed journal from database inception to February 18, 2020.STUDY SELECTION Inclusion criteria were designed to capture studies that evaluated pediatric patients (1) younger than 18 years, (2) with a diagnosis of SSD for which they underwent a cochlear implantation, and (3) with at least 1 outcome of interest measured numerically: speech perception, sound localization, device use, and patient-reported outcomes. Of the 526 articles reviewed, 12 (2.3%) met the selection criteria. DATA EXTRACTION AND SYNTHESISThe Meta-analyses Of Observational Studies in Epidemiology (MOOSE) reporting guidelines were followed. Data were pooled using fixed-effect and random-effect models. The following information was obtained from each article: study characteristics, patient characteristics, hearing loss and intervention characteristics, and outcomes. MAIN OUTCOMES AND MEASURESOutcomes were (1) postoperative changes in speech perception (in quiet was measured as a proportion of correct responses, and in noise was measured as decibel signal to noise ratio for speech reception threshold) and sound localization (measured in degree of localization error), (2) patient-reported audiological outcomes (measured by the speech, spatial, and qualities of hearing scale), and (3) device use rates among children who received cochlear implantation for SSD. RESULTSTwelve observational studies that evaluated 119 children (mean [SD] age, 6.6 [4.0] years) with SSD who received a cochlear implant were included. Most children showed clinically meaningful improvement in speech perception in noise (39 of 49 children [79.6%]) and in quiet (34 of 42 children [81.0%]). Long duration of deafness (>4 years in congenital SSD and >7 years in perilingual SSD) was the most commonly proposed reason for lack of improvement. Sound localization as measured by degrees of error from true location (mean difference [MD], -24.78°; 95% CI, -34.16°to -15.40°; I 2 = 10%) improved statistically significantly after cochlear implantation. Patients with acquired SSD and shorter duration of deafness compared with those with congenital SSD reported greater improvements in speech (MD, 2.27; 95% CI, 1.89-2.65 vs 1.58; 95% CI, 1.00-2.16) and spatial (MD, 2.95; 95% CI, 2.66-3.24 vs 1.68; 95% CI, 0.96-2.39) hearing qualities. The duration of deafness among device nonusers was statistically significantly longer than t...
Objective To assess quality of life (QOL) in pediatric patients with sensorineural hearing loss (SNHL) with the Pediatric Quality of Life Inventory 4.0 (PedsQL 4.0) and the Hearing Environments and Reflection on Quality of Life 26 (HEAR-QL-26) and HEAR-QL-28 surveys. Study Design Prospective longitudinal study. Setting Tertiary care center. Subjects and Methods Surveys were administered to patients with SNHL (ages 2-18 years) from July 2016 to December 2018 at a multidisciplinary hearing loss clinic. Patients aged >7 years completed the HEAR-QL-26, HEAR-QL-28, and PedsQL 4.0 self-report tool, while parents completed the PedsQL 4.0 parent proxy report for children aged ≤7 years. Previously published data from children with normal hearing were used for controls. The independent t test was used for analysis. Results In our cohort of 100 patients, the mean age was 7.7 years (SD, 4.5): 62 participants had bilateral SNHL; 63 had mild to moderate SNHL; and 37 had severe to profound SNHL. Sixty-eight patients used a hearing device. Mean (SD) total survey scores for the PedsQL 4.0 (ages 2-7 and 8-18 years), HEAR-QL-26 (ages 7-12 years), and HEAR-QL-28 (ages 13-18 years) were 83.9 (14.0), 79.2 (11.1), 81.2 (9.8), and 77.5 (11.3), respectively. Mean QOL scores for patients with SNHL were significantly lower than those for controls on the basis of previously published normative data ( P < .0001). There was no significant difference in QOL between children with unilateral and bilateral SNHL or between children with SNHL who did and did not require a hearing device. Low statistical power due to small subgroup sizes limited our analysis. Conclusion It is feasible to collect QOL data from children with SNHL in a hearing loss clinic. Children with SNHL had significantly lower scores on validated QOL instruments when compared with peers with normal hearing.
Objective: Endoscopic ear surgery (EES) has been increasingly adopted to enhance visualization during otologic surgery including pediatric cholesteatoma resection. The purpose of this study is to systematically review the current literature reporting rates of residual cholesteatoma to evaluate outcomes of EES in the pediatric population. Data Sources: PubMed, Embase, Cochrane. Study Selection: Using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis recommendations (PRISMA), we systematically reviewed studies that reported outcomes for cholesteatoma surgery in children using the endoscope. Studies were included if they described the use of the endoscope for middle ear surgery and measured outcomes following cholesteatoma resection in pediatric patients. Data Extraction: Identified articles were assessed for study design, sample size, inclusion of pediatric population, the presence of a comparison group, outcomes measured such as: residual disease rate, recurrent cholesteatoma, audiologic outcome, quality of life measures, and complications. Data Synthesis: Fourteen articles in the literature discussed cholesteatoma outcomes in children when the endoscope was employed. Residual disease rate ranged from 0 to 22% when endoscope was used for dissection compared with 11 to 55% when endoscope was used for inspection or not at all. A meta-analysis was conducted on 11 studies. There was a significant risk reduction in residual disease rates for endoscopic dissection when compared with microscope (RR = 0.48, 95% CI = 0.37–0.63, p < 0.001). Conclusion: Among the studies included in this review, EES was associated with lower rates of residual cholesteatoma compared with traditional microscopic techniques. Additional prospective studies are needed to further assess these findings.
Objectives/Hypothesis: In children with mild to moderately severe unilateral hearing loss (UHL), assess whether subject-reported quality of life (QOL) and teacher-and parent-reported perception of listening difficulty are affected by use of a hearing aid (HA) with baseline accommodations, compared to children receiving only baseline accommodations.Study Design: Randomized crossover clinical trial. Methods: Thirty-seven children 6-12 years of age with mild to moderately severe UHL and ≥80% word recognition scores in the poorer hearing ear were randomized into arm 1, using baseline accommodations (frequency-modulated system and strategic seating) for 12 weeks, followed by addition of a HA for 12 weeks. The other participants were randomized into the reverse methodology: arm 2, using a HA in addition to baseline accommodations for 12 weeks, followed by baseline accommodations alone. Surveys of QOL (Hearing Environments and Reflection on Quality of Life) and listening difficulties or challenges with hearing amplification (CHILD and LIFE-R questionnaires) were administered at 6-week intervals. Differences in mean survey scores, percent change, and improvement over time were computed between the two arms and inter-arm intervals. Per-protocol analysis was used.Results: Of the 37 children enrolled, 34 children underwent the study interventions and were included in the analysis, (arm 1 = 20, arm 2 = 14) (mean [standard deviation] age = 8 [1.5] years; 21 boys [61.8%]). Survey scores averaged across both arms during the HA interval (77.79 [15.13]) were significantly higher than during the baseline-only interval (69.67 [14.69], P = .036). There was no significant difference between trial arms in mean scores between the two HA intervals (P = .450) and two baseline-only intervals (P = .539).Conclusions: Hearing-related QOL and listening ability improved in children who met eligibility criteria with mild to moderately severe UHL with HA use compared with baseline accommodations alone. Trial Registration: ClinicalTrials.gov identifier: NCT02269124.
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