Temporal bone findings in Pierre Robin syndrome.

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.