Imaging of congenital temporal bone anomalies

A variety of congenital malformations of the ear and temporal bone may be encountered. Anomalies of the inner ear, middle ear, and external auditory canal and auricle can all occur, and there are a variety of clinical presentations that include conductive and sensorineural hearing loss. Some malformations are part of well-described genetic syndromes, whereas others are sporadic. Cochlear implantation may be helpful in some cases. Knowledge of typical patterns of abnormalities can help guide the clinical workup and management plan. The goal of this review is to familiarize the reader with the role of imaging in some of the commonly encountered congenital malformations of the ear and temporal bone.