Stapedotomy above the facial nerve in a congenitally malformed ear: a case report.

Surgery to correct congenital conductive hearing loss affords an opportunity for the otologic surgeon to drastically alter the patient’s hearing environment and offer a normal level of hearing in lieu of a lifetime of hearing aids. Unfortunately, the spectrum of abnormalities encountered in the congenital middle ear is wide, and the surgeon must be prepared for a variety of tasks. Perhaps the most daunting anomaly is when the course of the facial nerve deviates from its normal route as the potential for injury as a consequence of surgery is increased. We report here the case of a patient whose facial nerve coursed over the promontory between the round and oval windows, in conjunction with a malformed stapes and no identifiable footplate or contact with the oval window niche. This patient had a successful ossiculoplasty with no necessary manipulation of the facial nerve secondary to the extremity of its anomalous course. Computed tomography (CT) obtained preoperatively demonstrated this finding with clarity. This case serves as an example of the many variations described for the course of the facial nerve in the congenitally malformed middle ear and the value of CT in predicting its location throughout the middle ear and mastoid.