Congenital ossicular anomalies are important, often-missed causes of pediatric conductive hearing loss that may occur in isolation or as part of a syndrome. Accurately identifying and describing ossicular anomalies is important for determining treatment options and surgical planning. We review ossicular development, anatomy, and CT imaging findings of both nonsyndromic and syndromic congenital anomalies, including branchio-oto-renal syndrome, Treacher Collins syndrome, CHARGE (Coloboma of the eye, Heart defects, Atresia of the choanae, Retardation of growth and/or development, Genital and/or urinary abnormalities, and Ear abnormalities and deafness) syndrome, 22q11.2 deletion syndrome, hemifacial microsomia, Cornelia de Lange syndrome, and cleidocranial dysostosis. Learning Objective: Review normal anatomy and development of the ossicles, and identify imaging features of various congenital ossicular anomalies.
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