Conclusion: The psychometric characteristics of the Congenital Aural Atresia Questionnaire (CAAQ) are proposed to be reliable and sensitive to determine clinical changes in a patient's quality of life (QOL) and the questionnaire has potential for clinical application. Objective: The aim of this study was to develop a quantifiable, self-assessed, and health-related QOL questionnaire for patients with congenital aural atresia and to explore its reliability and validity. Methods: An initial QOL questionnaire for patients with congenital aural atresia was constructed according to widely used available QOL questionnaires. A total of 140 patients with congenital aural atresia were recruited into this study. After pretest and item sifting, the QOL questionnaire was constructed, and its reliability and validity were evaluated. Results: An 18-item QOL questionnaire for patients with congenital aural atresia was constructed, which included 3 principal domains: symptoms and impact (physical, 8 items), mental status (psychological, 6 items), and social interaction (4 items). The retest reliability was 0.878; split-half reliability coefficient and Cronbach's alpha coefficient were 0.927 and 0.899, respectively. The factor analysis results indicated satisfactory construct validity.