Navigation-Guided Cochlear Implantation in Complex Congenital Ear Anomalies: Pierre Robin Syndrome With External Aural Atresia and Middle Ear Agenesis.

Cochlear implantation in patients with complex craniofacial anomalies such as Pierre Robin syndrome poses significant surgical challenges, particularly in the presence of external aural atresia and middle ear agenesis. The authors present the case of a female patient diagnosed with Pierre Robin syndrome at 7 weeks of age, who exhibited glossoptosis, cleft palate, micrognathia, and multiple systemic anomalies. Audiological evaluation confirmed profound bilateral sensorineural hearing loss, and imaging revealed severe malformations of the external, middle, and inner ear. Sequential bilateral cochlear implantation was planned. At 20 months of age, left-sided cochlear implantation was successfully performed using anatomic estimation despite the absence of conventional landmarks. A second attempt for right-sided implantation was made at 26 months; however, the surgery was aborted due to the inability to localize the cochlea. At 4 years of age, right-sided implantation was successfully achieved using a navigation-guided transmastoid approach. High-resolution preoperative imaging with fiducial markers enabled accurate intraoperative localization, allowing for safe electrode insertion despite the complex anatomy. Postoperative follow-up demonstrated favorable auditory and language development. This case highlights the value of advanced surgical planning and navigation-assisted techniques in overcoming anatomic challenges in cochlear implantation for patients with severe congenital ear anomalies.