Kokai H, Oohasi M, Kikuchi H, Iida K, Ishikawa K, Kobashi M, Takizawa M, Ootani H, Terayama Y
Azabu Triology Hospital, Sapporo.
Nihon Jibiinkoka Gakkai Kaiho. 1996 Jun;99(6):910-7. doi: 10.3950/jibiinkoka.99.910.
Paget's disease of the bone is a chronic, progressive disease of unknown etiology characterized by abnormal bony resorption and deposition. It is a common skeletal disease in Europe and North America, while in Japan it is very rare. Paget's disease of the temporal bone has been reported to cause hearing loss frequently. We report a 50-year-old woman with Paget's disease who had progressive bilateral sensorineural hearing loss and right vestibular dysfunction. CT and 3 Dimensional CT (3D-CT) images demonstrated resorption of the entire bony labyrinth and its surroundings on both sides and that the temporal bone elsewhere remained intact. Bone scans revealed disease symmetrically in the bilateral otic capsules. Previous studies indicated that the bone changes in Paget's disease in the petrous pyramid begin in areas best supplied with marrow tissue and that the otic capsule is relatively spared until advanced changes are present in the remainder of the petrous pyramid. But, this patient mainly had foci in the bilateral otic capsules and the pattern was similar to cochlear otosclerosis. 3D-CT was useful for differentiation of Paget's disease and cochlear otosclerosis. The pattern of the affected areas indicated that this is a very rare situation even in the reports of Europe and North America, where the disease is rather common.
骨佩吉特病是一种病因不明的慢性进行性疾病，其特征为异常的骨质吸收和沉积。它是欧美常见的骨骼疾病，而在日本则极为罕见。据报道，颞骨佩吉特病常导致听力丧失。我们报告一例50岁患有佩吉特病的女性，她患有进行性双侧感音神经性听力损失和右侧前庭功能障碍。CT和三维CT（3D-CT）图像显示双侧整个骨迷路及其周围骨质吸收，而其他部位的颞骨保持完整。骨扫描显示双侧耳囊对称受累。先前的研究表明，岩骨佩吉特病的骨质改变始于骨髓组织供应最好的区域，并且在岩骨其余部分出现晚期改变之前，耳囊相对 spared。但是，该患者主要病变位于双侧耳囊，其模式类似于耳蜗性耳硬化。3D-CT有助于鉴别佩吉特病和耳蜗性耳硬化。受累区域的模式表明，即使在欧美这种该疾病相当常见的地区的报道中，这也是一种非常罕见的情况。
