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Pagets disease of the bone is a chronic, progresive disease of unknown etiology, characterized by abnormal bony resorption and deposition. It is a common skeletal disease in Europe and North America, while it is very rare in Asia. Pagets disease of the temporal bone has been reported to cause hearing loss, tinnitus, and mild vestibular symptom. We report a 28-year-old man with Pagets disease, which was a first reported case of bilateral sensorineural hearing loss for the first time in Korea. (Korean J Otolaryngol 2002;45:911-4)