Chole R A, Henry K R
Ann Otol Rhinol Laryngol. 1985 Jul-Aug;94(4 Pt 1):366-72.
Otosclerosis is an inherited disease in which abnormal bone growth results in ossicular fixation and hearing loss. Although the disease affects up to 10% of humans, it has not been observed in other animals. The LP/J inbred mouse has been found to develop abnormal bony lesions of the middle ear which resemble human otosclerosis. In this study of 113 temporal bones from LP/J mice, we found that the lesions develop after puberty and involve only the ossicles and the otic capsule. The most frequent site of involvement was the incus (46.9%), followed by the anterior crus of the stapes (31.3%), the malleus (14.2%), the otic capsule (14.2%), and the stapes footplate (8%). Cochlear hair cell loss was progressive throughout the lifespan of these animals. Although this disease is not identical in histologic appearance to human otosclerosis, understanding its disease process may shed light upon the pathophysiology of the human disease.
耳硬化症是一种遗传性疾病，异常的骨质生长导致听骨固定和听力丧失。尽管该疾病影响了多达10%的人类，但在其他动物中尚未观察到。已发现LP/J近交系小鼠会出现类似于人类耳硬化症的中耳异常骨病变。在这项对113块LP/J小鼠颞骨的研究中，我们发现病变在青春期后出现，仅累及听骨和耳囊。最常受累的部位是砧骨（46.9%），其次是镫骨前脚（31.3%）、锤骨（14.2%）、耳囊（14.2%）和镫骨底板（8%）。在这些动物的整个生命周期中，耳蜗毛细胞损失呈进行性。尽管这种疾病在组织学外观上与人类耳硬化症并不相同，但了解其疾病过程可能有助于揭示人类疾病的病理生理学。
