The role for cochlear implants in microtia and congenital aural atresia: A case report and a review of literature

Sensorineural hearing loss resulting from microtia and aural atresia is rare due to different embryologic origins of the external and inner ear. Consequently, cochlear implants are seldom performed in patients with microtia and/or aural atresia. Herein we discuss an 8 year-old boy with congenital aural atresia and microtia who underwent cochlear implant surgery for profound hearing loss with good results. A literature review was performed and discussed. Pre-operative planning with high resolution computed tomography and facial nerve monitoring are crucial in identification and preservation of the facial nerve due to the high rate of aberrant anatomy. Careful incision placement is required particularly if pinna reconstruction is considered. Literature review highlighted various surgical approaches as well as different modalities used for intra-operative facial nerve monitoring. Only one patient had intra-operative complications. Cochlear implant is a viable solution in patients with external ear malformations and severe-profound sensorineural hearing loss. Adequate planning and counselling are essential due to the challenges that may occur in microtia and aural atresia.