Vestibulotomy for Unilateral Congenital Oval Window Atresia With Aberrant Facial Nerve in an 8-Year-Old Child: A Case Report

Conductive hearing loss in children with a normal tympanic membrane may result from congenital middle ear anomalies that pose diagnostic and surgical challenges. Here, we report a case of an 8-year-old girl with unilateral conductive hearing loss associated with complex middle ear malformation that was successfully treated with vestibulotomy. She presented with right-sided hearing loss that was identified during a school screening. Otoscopic examination findings were unremarkable. Pure-tone audiometry revealed significant conductive hearing loss, with an air-conduction (AC) threshold of 72 dB HL and a bone conduction (BC) threshold of 23 dB HL. Temporal bone computed tomography demonstrated right-sided oval window atresia with an aberrant facial nerve course and an abnormal stapes shadow. Bilateral lateral semicircular canal aplasia was also identified as an associated anomaly. Exploratory tympanotomy of the right ear confirmed complete oval window atresia and a detached stapes footplate floating within the middle ear cavity. A fenestration was created at the presumed oval window using a CO2 laser, followed by placement of a piston prosthesis. Postoperatively, hearing improved significantly, with an AC threshold of 20 dB HL and a BC threshold of 15 dB HL, and remained stable over the 3-year follow-up period. This case highlights vestibulotomy as an effective surgical option for pediatric patients with congenital oval window atresia and an aberrant facial nerve course.