Walker AM; Patel PA; Nguyen SA; Clemmens CS; White DR · 2026 · Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery
Paper
OBJECTIVE: To evaluate sex differences in the diagnosis and management of congenital aural atresia (CAA). DATA SOURCES: PubMed, SCOPUS, CINAHL, Cochrane Library. REVIEW METHODS: A comprehensive literature search was conducted from inception through January 2026. Eligible studies included patients diagnosed with CAA with data stratified by sex. Meta-analysis of proportions, relative risk (RR), mean difference (Δ), and continuous measures with 95% confidence intervals (CI) were performed using fixed- and random-effects models. RESULTS: Eleven studies met inclusion criteria, encompassing 345 patients with CAA. The mean age was 11.6 years (range 3-54), with a male predominance of 56.2% (CI 50.95-61.38). Most patients were Marx grade III (60%, CI 38.01-78.58) with a mean Jahrsdoerfer score of 8.2 (range 7-10). Bone conduction hearing devices (BCHD) were the most common intervention (n = 105), while canalplasty was less frequent (n = 31). Males underwent canalplasty more often, while females demonstrated higher risk for bilateral disease (RR = 1.57 [1.02-2.42], P =.04) and, when unilateral, right-sided defects (RR = 1.2 [1.01-1.42], P =.03). No significant differences were observed between sexes in Jahrsdoerfer scores, microtia grade, or preintervention audiometry. CONCLUSION: Sex may influence presentation and management in CAA, with females more likely to present with bilateral and right-sided disease and males more often undergoing canalplasty. These findings underscore the need for heightened awareness of sex-specific patterns and highlight the importance of future stratified studies to guide diagnosis, counseling, and treatment selection.
Analysis
This systematic review and meta-analysis evaluates sex differences in congenital aural atresia (CAA), finding that females are more prone to bilateral and right-sided defects, while males undergo canalplasty more frequently.
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