RESUMEN
Conductive hearing loss is a condition that can be corrected surgically in most cases. Stapedotomy is usually performed for patients with congenital ossicular anomaly or fixation. However, otologic surgeons have often encountered difficulty due to the complexity of stapes surgery. Moreover, congenital stapes fixation with anomalous facial nerve and oval window absence is an uncommon and dangerous condition. In such cases, vestibulotomy can be a surgical option to prevent facial nerve damage and improve hearing. The etiology can be explained by the embryological development of the middle ear structures. To prevent facial nerve damage and maximize the efficacy of surgery, the surgeon should have knowledge about this disease and take a computed tomography for double check before stapes surgery. We describe a case of congenital stapes fixation with aberrant facial nerve courses, a 15-year-old female who was treated with vestibulotomy and piston wire insertion.