Computed tomography (CT) was used to study 25 patients with congenital conductive hearing loss and normal external auditory canals. Deformities were subdivided according to ossicular, fenestral, and cholesteatomatous origin. Isolated ossicular deformities were found in 14 patients (five bilateral), cholesteatoma in eight, oval-window nondevelopment (with ossicular deformity) in one, and normal studies in two (congenital stapes fixation at the level of the annular ligament). Ossicular deformities may be subdivided into incudostapedial disconnections into incudostapedial disconnections (most common), malleoincudal fixations, and stapes fixations. Most are due to developmental anomaly of the first or second branchial arch. The stapes has a dual origin (second arch and otic capsule). A cholesteatoma is defined as congenital only if there is no history of otitis and the tympanic membrane is intact. In this series, six were in the middle ear proper, and two were within the attic beyond otoscopic view. Their CT appearance, with one exception, was essentially identical to that of acquired lesions.