ObjectiveTo discuss the audiology, imaging characteristics and choice of surgical approach for cochlear implantation in patients with congenital aural bony atresia.MethodsFive cases of congenital aural bony atresia were collected from July 2015 to January 2019. Their clinical data including the history, audiological examination, imaging characteristics and surgical approach were analyzed retrospectively.ResultsAll the patients had severe sensorineural hearing loss. The imaging examination showed aural bony atresia, ossicular chain deformity, abnormal route of facial nerve without any inner ear malformations. Tympanic sinus approach was adopted in all the five patients and posteroinferior to facial nerve in 2. The electrode was inserted through round window membrane in all the patients with smooth implantation process and good reaction after switchon of cochlear devices.ConclusionsFor cochlear implantation in patients of congenital aural bony atresia, tympanic sinus approach should be adopted to expose the round window niche via opening mastoid cavity and tympanic sinus, removing abnormal ossicular chain. If the mastoid segment of facial nerve moves forward and completely blocks the round window niche, the niche can be exposed posteroinferior to the facial nerve.