Phelps P D, Poswillo D, Lloyd G A
Clin Otolaryngol Allied Sci. 1981 Feb;6(1):15-28. doi: 10.1111/j.1365-2273.1981.tb01782.x.
Bilateral conductive deafness is common in mandibulofacial dysostosis with or without atresia of the external auditory meatus. This deafness is due to a wide range of deformities of the ossicular chain associated with a characteristic reduction in the size of the middle ear cavity. The attic and antrum are particularly affected and usually have a slit-like appearance on coronal section tomograms. The inner ear is essentially normal. The tomographic appearances of 44 ears from 22 patients with mandibulofacial dysostosis are described as well as the operative findings in 14. These were correlated with induced ear deformities in an animal model that had features characteristic of human mandibulofacial dysostosis. A review of the findings in the animal model suggests a very close correspondence with the human ear anomalies described at radiological investigation, operation and post-mortem examination. The lesions are largely symmetrical and this, with the characteristic slit attic, helps to differentiate mandibulofacial dysostosis from lesions with different aetiology but similar features such as facial microsomia. The prospects for surgical correction of the deafness in mandibulofacial dysostosis range from very good in mild cases with ossicular discontinuity, to poor where there is severe atresia. The importance of tomograms at an early age is stressed.
双侧传导性耳聋在伴有或不伴有外耳道闭锁的下颌面骨发育不全中很常见。这种耳聋是由于听骨链的多种畸形，伴有中耳腔大小特征性减小。上鼓室和鼓窦尤其受累，在冠状位断层扫描上通常呈裂隙状外观。内耳基本正常。描述了22名下颌面骨发育不全患者44只耳朵的断层扫描表现以及14只耳朵的手术发现。这些与具有人类下颌面骨发育不全特征的动物模型中的诱发性耳部畸形相关。对动物模型研究结果的回顾表明，与放射学检查、手术和尸检中描述的人类耳部异常非常吻合。病变大多对称，这一点以及特征性的裂隙状上鼓室，有助于将下颌面骨发育不全与病因不同但特征相似的病变（如面部小畸形）区分开来。下颌面骨发育不全性耳聋的手术矫正前景，从听骨链中断的轻度病例的非常好，到严重闭锁病例的很差不等。强调了早期进行断层扫描的重要性。
