Histopathology of the temporal bones in thanatophoric dysplasia.

The pathological findings of temporal bone in two cases of thanatophoric dysplasia were reported. Thanatophoric dysplasia is classified into type 1 and type 2, each of which has been reported to show specific clinical and radiographic-findings. The present study revealed that each type also showed specific characteristic in the structure of the temporal bones. The developmental mechanisms of hearing impairment in this disease were also discussed on the basis of the pertinent literature.