Cochlear implantation via the retrofacial route in a child with a malformed inner ear: A clinical capsule report.

OBJECTIVE

To report the technical feasibility and early outcome of cochlear implantation using a retrofacial approach (RFA) in a child with an inner ear malformation and limited access to the round window.

METHODS

A 3-year-old child with bilateral profound sensorineural hearing loss presented with an incomplete partition type I (IP-I) malformation characterized by a cystic cochlear cavity, enlarged vestibule, and hypoplasia of the lateral and posterior semicircular canals. Preoperative imaging suggested limited exposure of the round window through the facial recess. Intraoperative assessment indicated that conventional posterior tympanotomy would provide limited and tangential access to the round window. Cochlear implantation was therefore performed using a RFA with management of a cerebrospinal fluid gusher.

MAIN OUTCOME MEASURES: Electrode insertion, intraoperative safety, postoperative imaging findings, and early auditory rehabilitation outcomes.

RESULTS

Complete electrode insertion was achieved. Intraoperative telemetry responses were obtained from several electrodes, indicating neural responsiveness. Postoperative imaging confirmed intracochlear placement. No facial nerve dysfunction or surgical complications occurred. Early auditory responses were observed.

CONCLUSIONS

The RFA may represent a useful alternative in selected pediatric patients with IP-I malformation when access to the round window through the facial recess is limited. Careful imaging analysis, intraoperative assessment, and surgical planning remain essential.