Cramer H B, Chole R A
Ann Otol Rhinol Laryngol. 1986 Mar-Apr;95(2 Pt 1):169-72. doi: 10.1177/000348948609500212.
The LP/J inbred mouse is the first known animal to spontaneously develop otosclerosis-like bone lesions in the middle and inner ear. These abnormal bone foci resemble human otosclerotic lesions, are inherited, and produce a progressive hearing loss. On the basis of histological evaluation alone, it was unclear whether these otosclerosis-like lesions in LP/J mice develop suddenly or gradually as in human otosclerosis. To study the dynamics of lesion formation, four different colored fluorochrome bone labels were given in sequence to postpubertal LP/J mice and CBA/J mice as normal controls. All of the otosclerosis-like lesions incorporated at least one fluorochrome, and half were labeled with two or more markers indicating continuous lesion growth over many weeks. It appears that otosclerosis-like lesions in LP/J mice develop during early adulthood and progress gradually as in human otosclerosis.
LP/J近交系小鼠是已知的第一种在中耳和内耳自发形成耳硬化症样骨病变的动物。这些异常骨灶类似于人类耳硬化症病变，具有遗传性，并会导致进行性听力损失。仅基于组织学评估，尚不清楚LP/J小鼠中的这些耳硬化症样病变是像人类耳硬化症那样突然发生还是逐渐形成。为了研究病变形成的动态过程，对青春期后的LP/J小鼠和作为正常对照的CBA/J小鼠依次给予四种不同颜色的荧光染料骨标记物。所有耳硬化症样病变至少包含一种荧光染料，半数病变被两种或更多标记物标记，表明病变在数周内持续生长。看来LP/J小鼠中的耳硬化症样病变在成年早期形成，并像人类耳硬化症一样逐渐发展。
