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.
Clinical and genetic features of nonsyndromic autosomal dominant sensorineural hearing loss: KCNQ4 is a gene responsible in Japanese
