Igarashi M, Filippone M V, Alford B R
Laryngoscope. 1976 Nov;86(11):1679-87. doi: 10.1288/00005537-197611000-00009.
This is the first complete report on the histopathologic study of the temporal bones from an infant with a well-documented Pierre Robin syndrome (micrognathia, glossoptosis and cleft palate), demonstrating multiple middle and inner ear anomalies. The anomalies are basically architectural malformations rather than neutral or end organ developmental anomalies. The anomalies in this case, except for a few points, are somewhat similar in both ears. Multiple anomalies include: abnormal narrowing of the crus commune-utricle junction, superiorly located crus commune and posterior semicircular canal, underdeveloped modiolus, absence of the bony septum between the middle and apical coil (existence of scala communis in left ear), abnormally small internal auditory meatus, and abnormal direction of internal auditory canal, large cartilaginous mass around the superior semicircular canal and in the tympanic end of the fissula ante fenestram, small facial nerve, large facial bony canal dehiscence, anomalic stapes, etc.
这是关于一名患有记录详实的皮埃尔·罗宾综合征（小颌畸形、舌后坠和腭裂）婴儿颞骨组织病理学研究的首份完整报告，该报告展示了多个中耳和内耳异常情况。这些异常基本上是结构畸形，而非神经或终末器官发育异常。除了少数几点外，该病例双耳的异常情况有些相似。多种异常包括：总脚 - 椭圆囊连接处异常狭窄、总脚位置偏高及后半规管、蜗轴发育不全、中圈与顶圈之间骨隔缺失（左耳存在总蜗管）、内耳道异常狭小、内耳道方向异常、上半规管周围及窗前裂鼓室端有大的软骨块、面神经细小、面神经骨管大裂孔、镫骨异常等。
