Sex Differences in Congenital Aural Atresia: A Systematic Review and Meta-Analysis

To evaluate sex differences in the diagnosis and management of congenital aural atresia (CAA).

PubMed, SCOPUS, CINAHL, Cochrane Library.

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.

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.

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.