OBJECTIVE To describe presenting symptoms, evaluation findings, and surgical management of cranial base hyperostosis in patients with Camurati-Engelmann disease (CED). DESIGN Retrospective study and literature review. SETTING The Mayo Clinic, Rochester, Minnesota. PATIENTS A total of 306 patients diagnosed as having CED, including 12 primarily evaluated at our institution between 1968 and...

We report the progressive otic capsule demineralization around the membranous labyrinth and facial nerve in an adult patient with osteogenesis imperfecta tarda. Whereas the initial CT scan showed bandlike, undermineralized pericochlear areas, 2 years later, repeat CT performed because of hearing deterioration showed progression of these findings to the promontory, the round window niche, and th...

Bone remodelling is a complex mechanism regulated by osteoclasts and osteoblasts perturbation of this process leads to the onset diseases, which may be characterised altered bone erosion or formation. In review, we will describe some formation-related disorders as sclerosteosis, van Buchem disease, hypophosphatasia Camurati–Engelmann disease. past decades, research focused on these rare offered...

We report a 45-year follow-up of a patient with Engelmann's disease previously described in 1950, showing progression of the disease with unique involvement of the femoral capital epiphyses. The case is compared with others to add some information about the later stages of a disease which is not fully understood.