• Xue Gao, Juan Zhao, Guan-Hua Li, Xi Wang, Wei Wang, Xing Liu, Min Liu, Meng-Meng Guo, Zhen-Dong Wang, Ya-Yan Lu, Jia Li, Yong Feng, Kun Yang, Jin-Cao Xu, and Pu Dai.
• College of Otolaryngology Head and Neck Surgery, Chinese PLA General Hospital, Chinese PLA Medical School, Beijing, China.
• Medicine (Baltimore). 2023 Feb 17; 102 (7): e33000e33000.

Rationale And Patient ConcernsCongenital hearing loss is often caused by an inner ear malformation, in such cases, the presence of other anomalies, such as microtia, and venous anomalies of the temporal bone and laryngomalacia makes it challenging to perform cochlear implantation surgery.DiagnosesThis study reports the case of a 28-month-old girl with congenital profound hearing loss, laryngomalacia, and malformed inner ear, who received cochlear implantation surgery. The bony structure, vessels and nerves were first assessed through magnetic resonance imaging and computed tomography before exploring the genetic basis of the condition using trio-based whole exome sequencing. Perioperative evaluation and management of the airway was then performed by experienced anesthesiologist, with the surgical challenges as well as problems encountered fully evaluated.InterventionsCochlear implantation was eventually performed using a trans-mastoid approach under uneventful general anesthesia.OutcomesDue to the small size of the cochlea, a short electrode FLEX24 was inserted through the cochleostomy.LessonsConsidering the high risk of facial nerve injury and limited access to the cochlea when patients present significant bony and venous anomalies, cochlear implantation in such patients require careful preoperative evaluation and thoughtful planning. In these cases, airway assessment, magnetic resonance venography, magnetic resonance arteriography, and magnetic resonance imaging and computed tomography can be useful to minimize the risks. Intraoperative facial nerve monitoring is also recommended to assist in the safe location of facial nerve.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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