Congenital Aural Atresia: What the Radiologist Needs to Know?

Congenital aural atresia (CAA) is characterized by hypoplasia or aplasia of external auditory canal associated with auricular deformity. It also embodies a spectrum of temporal bone abnormalities including anomalies of middle ear, ossicles, facial nerve, oval window, round window, mastoid pneumatization, temporomandibular joint and occasionally inner ear and vascular anomalies. The management in such patients is variable and consists of rehabilitative hearing aids or surgical correction depending on various patient factors like associated temporal bone abnormalities, degree of hearing impairment and laterality. Surgical management if planned, depends on the extent of associated temporal bone anomalies and hence necessitates comprehensive knowledge about the normal and abnormal anatomy of these structures. High resolution computed tomography (HRCT) is the optimal investigation which provides required anatomical information and plays a pivotal role in deciding the management strategy. This pictorial review aims to review the spectrum of anomalies involving critical structures in CAA on HRCT along with their clinical significance.