Hyperostosis cranialis interna

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Hyperostosis cranialis interna

A rare primary bone dysplasia with increased bone density characterized by slowly progressive endosteal hyperostosis and osteosclerosis exclusively of the skull base and the calvaria resulting in entrapment and dysfunction of cranial nerves I II V VII and VIII. First symptoms often appear during the second decade of life and include disturbances in smell vision facial sensation and expression hearing and balance as well as headaches due to increased ocular and intracranial pressure. After the fourth decade radiological progression is minimal although decreased intracranial volume can lead to death in severe cases.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview.

Reference: Access aggregated data from Orphanet at Orphadata.

Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version November 2023

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Hyperostosis cranialis interna?

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