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Massive fibrous dysplasia of the temporal bone

ALTHOUGH fLbro-osseous dysplasias of the maxilla are not uncommon, those of the temporal bone are very rare. The case herein reported is one of massive involvement of the entire temporal bone and somewhat different from cases described in the past. A female, aged 30 years, attended the Outpatient Department complaining of: (1) Diffuse swelling over the mastoid and temporal region, extending up to the root of the zygoma on the left side. (2) Complete loss of hearing. The patient could not remember precisely the duration of her complaint, but it extended back for many years. She had no other complaints. A history of otorrhoea from the involved ear was the only significant feature in her past history, but the discharge ceased in childhood. She has three children and the delivery in each case was normal, thus excluding any suggestion of a contracted pelvis. General examination of the patient showed her to be of short stature, being only 4ft. 4m. in height; otherwise she was a perfectly healthy female. Local examination of the pharynx, larynx, nose and right ear showed no abnormality. Left ear—On local examination there was a diffuse, hard, smooth bony swelling over the entire temporal bone extending from the tip of the mastoid process, over the mastoid bone, to the squamous part of the temporal bone, occluding the external auditory meatus and extending to the pre-auricular area at the level of the root of the zygoma (Fig. 1). Occlusion of the external auditory meatus was complete and even a pointed needle could not be introduced further. The swelling did not seem to extend beyond the temporal bone to any other cranial bones. There was neither tenderness, nor crepitus nor eggshell crackling, over the diseased area. She was totally deaf in the left ear. Tuning forks of 256, 512, 1024, 2048 frequencies could not be heard by air or bone conduction. With a 512 frequency tuning fork (on the incisor teeth) the Weber response was lateralized towards the right ear. A pure tone audiogram showed no hearing in the left ear, but the right ear was normal. Skiagrams of the temporal bone showed a markedly dense marble-like opacity of the entire bone (Figs. 2, 3 and 4). The external as well as the internal auditory meatus could not be demarcated or visualized. The dense opacity was shown to be confined to the temporal bone only, and it was limited by the occipito-mastoid and temporo-parietal suture lines. Not a single air cell could be made out. The rest of the cranial vault showed some degree of osteo-porosis.