Combined microtia and aural atresia: issues in cochlear implantation.

Objectives: This article presents the first report of cochlear implantation in a patient with congenital aural atresia, microtia, dysplastic cochlea and internal auditory canals, and bilateral profound sensorineural hearing loss (HL). This rare combination requires special management considerations. Preoperative issues include thorough evaluation of computed tomography and magnetic resonance imaging to determine favorable anatomy, cochlear implantation candidacy, and surgical planning. Intraoperative concerns include incision placement, surgical approach to the middle ear, and abnormal facial nerve anatomy. Postoperative use of a special headset combining a microphone and transmitter coil is required. Study Design: Case report and literature review. Methods: The patient's chart was reviewed for diagnostic studies, operative strategy, and postoperative auditory stimulation and testing. A literature review was performed. Results: A 2-year-old male presented with bilateral aural atresia, microtia, and profound sensorineural HL. Imaging studies revealed multiple abnormalities of the cochlea, vestibule, and internal auditory canal, all of which were more favorable on the right side. An incision was designed to accommodate future microtia repair. The cochlear implant was placed without difficulty by way of a facial recess approach to the middle ear. Postoperative results include the detection of Ling sounds and voices in the environment as well as the ability to locate sounds. Conclusions: This is the first report of cochlear implantation in a patient with bilateral aural atresia, microtia, and profound sensorineural HL in conjunction with multiple inner ear abnormalities. Close collaboration among the otologist, neuroradiologist, and plastic surgeon is essential to coordinate surgical management and optimize cosmetic and functional outcomes in this unique population.