Eトリソミー症候群の側頭骨病理所見

抄録

The histopathological changes in the temporal bones from a 12-day-old girl with E trisomy are reported. A review of the literature revealed thirteen previous histopathological reports on the temporal bones in E trisomy. Findings varied widely from normal cases to those with severe malformations of the auditory structures. In the present case, a great difference was observed between the right and left ears. The right ear showed only minor anomalies, such as a wide angle of first genu of the facial nerve, wide dehiscence of the Fallopian canal, large marrow cavities of the incus and malleus, and residual mesenchyme in the round window niche. However, the left ear showed additional severe anomalies, such as a bulky and deformed stapes, a small oval window, flattened crista of the superior semicircular canal, and absence of the bony portion of the posterior semicircular canal which was involved by outpouching of the large vestibule among other anomalies.