A 51-YEAR-OLD WOMAN presented with a history of otosclerosis and bilateral stapedectomies that she had undergone approximately 20 years earlier, with minimal benefit. Since then, she experienced a slow deterioration of hearing in both ears. She noted no family history of otosclerosis. Otoscopic examination revealed normal, mobile tympanic membranes in both ears. A Weber test demonstrated localization to the left ear, and the results of Rinne tuning fork tests were equivocal. The patient's audiogram revealed severe to profound sensorineural loss in the left ear, with thresholds ranging from 70 dB at 250 Hz to 105 dB at 4000 Hz and no response at 6000 or 8000 Hz. The right ear demonstrated no response. Speech awareness at 85 dB was observed for the left ear only. Tympanograms were type A bilaterally. Acoustic reflexes were absent.