Bilateral Congenital Stapes Footplate Fixation With Absence of the Stapedial Tendon

Congenital stapes footplate fixation (CSFF) is a rare cause of nonprogressive conductive hearing loss in pediatric patients. The concurrent absence of the stapedial tendon is exceptionally uncommon, and bilateral involvement has not been previously reported. This article describes a case of a 15-year-old male with a history of left hemifacial microsomia, bilateral microtia, and complex congenital heart disease who presented with longstanding bilateral conductive hearing loss with air-bone gap of 64 dB and 58 dB on the right and left, respectively. Surgical exploration revealed bilateral CSFF with absent stapedial tendons, with associated malformed ossicles including shortened and thinned incudal long processes with fibrous nonunion to malformed stapes suprastructures. After undergoing staged bilateral stapedotomy, postoperative audiometry demonstrated substantial improvement with air-bone gap reduced to 19 dB on the right and 15 dB on the left. Speech reception thresholds improved to 25 dB on the right and 15 dB on the left, representing functional hearing restoration. This case represented the first reported instance of bilateral CSFF with absent stapedial tendons. Surgical exploration remains essential in select patients, and stapedotomy can result in meaningful hearing restoration even in the setting of complex congenital ossicular anomalies.