Oculo-auriculo-vertebral dysplasia. A temporal bone study of a case of Goldenhar's syndrome

A case of oculo-auriculo-vertebral dysplasia is presented, with histopathological findings in the temporal bone and eye, and some radiological features. The syndrome consists of anomalies of the first and second branchial arch derivatives associated with ophthalmic and vertebral anomalies. Although clinical features and radiological findings of oculo-auriculo-vertebral dysplasia are documented in the literature, there have been very few reports of the histopathological findings of abnormal temporal bones. These vary from our own findings. The prominent features in our case are: a small middle ear cleft, an absence of ossicles, an aberrant facial nerve, and dysplastic, short lateral and superior semi-circular canals, as well as a narrow internal auditory meatus.