A Case of Auditory Ossicular Malformation with Fixation of the Stapes Suprastructure Accompanied by Congenital Cholesteatoma

We present a rare case of isolated congenital fixation between the immature suprastructure of the stapes and the facial canal, associated with congenital cholesteatoma. A 7-year-old girl showed left conductive hearing loss on pure-tone audiometry. CT showed abnormal calcification in the tympanic cavity. During surgery, we observed that the suprastructure of the stapes was an immature dumbbell-shaped bone fixed on the horizontal portion of the facial canal and separated from the footplate, which displayed good mobility. A stapedial tendon was present. After resection of the immature bone from the suprastructure, a congenital open-type cholesteatoma was noted on the footplate. The congenital cholesteatoma was extirpated and type IV tympanoplasty was performed using Apaceram®-T.