HYPEROSTOSIS CRANIALIS INTERNA - A NEW HEREDITARY SYNDROME WITH CRANIAL-NERVE ENTRAPMENT

In this report, we describe a curious and apparently unique genetic bone disorder in a white family. The main radiologic features are intracranial hyperostosis and osteosclerosis of the calvaria and the base of the skull; the mandible and skeleton are normal. Recurrent facial palsy is the primary clinical sign, and there is variable simultaneous impairment of the senses of smell, taste, and vision and of cochleovestibular function. This disorder can be classified among the craniotubular hyperostoses. Case Report The proband (III–7 in Fig. 1) was seen at 20 years of age because she had recurrent left facial-nerve palsy of three weeks'. © 1990, Massachusetts Medical Society. All rights reserved.