Temporal Bone CT Findings in Cornelia de Lange Syndrome

Axial section of the middle ear of a 2-year-old girl with Cornelia de Lange syndrome. Note the soft tissue filling the tympanomastoid cavity, the dysmorphic ossicle with a large incus body and a malleus head, and a cleft on the incus (arrow) in the patient with Cornelia de Lange syndrome (A) compared with a 2-year-old control with normal hearing (B).