The prevalence of hearing loss in children with congenital diaphragmatic hernia: A longitudinal population-based study.

BACKGROUND: The true prevalence of hearing loss among children with congenital diaphragmatic hernia (CDH) is unknown, with some studies reporting rates up to 60%. PURPOSE: The purpose of this study was to determine the prevalence of hearing loss among children with CDH and compare it to age-matched controls. METHODS: We used population-based datasets to compare the number of hearing loss diagnoses in children younger than 10 years-of-age born between 1992 and 2009 with CDH to date-of-birth matched controls without CDH. Factors associated with CDH disease severity were analyzed to determine their effect on the prevalence of hearing loss. A sensitivity analysis was performed to determine if selection bias of improved care over the course of the study affected hearing loss in CDH patients. The prevalences of hearing loss were compared using Fisher's exact tests and statistical significance was defined as p < 0.05. RESULTS: A total of 529 children, 38 CDH cases and their 491 date-of-birth matched controls, met the inclusion criteria. Hearing loss was found in 7 children with CDH (18.4%) compared to 26 (5.3%) controls; the risk ratio (RR) of hearing loss was 3.48 (95%CI = 1.61-7.49, p = 0.006). There was no association between CDH disease severity and hearing loss. CONCLUSIONS: CDH is associated with hearing loss compared to the general population. Our results suggest that congenital factors may contribute to hearing loss in CDH more than perinatal exposures. LEVEL OF EVIDENCE: 3.