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In a 35 year-old female diaphyseal hyperostosis of femur and tibia indicative of a Camurati-Engelmann syndrome (progressive diaphyseal dysplasia) were discovered during routine phlebography. This rare disorder belongs to the group of osteochondrodysplasias and affects primarily the diaphyses of long bones. Sporadic as well as familial cases have been observed. Although the cause and pathogenetic mechanism of the disease are unknown, an autosomal dominant inheritance is being discussed. Diagnosis was based on radiological findings, which are presented along with the scintigraphic appearance of the disease.

In Camurati-Engelmann disease HR-CT can impressively demonstrate the involvement of the skull base and also correlate well with the clinical symptoms of deafness, vestibular disturbances and facial paralysis. Since conservative treatment with corticosteroids, calcitonin and diphosphonates is hardly successful and compression of the cranial nerves practically absent, surgical treatment with decompression should be considered. Differential diagnosis against other bone affections with deafness depends on age, typical x-ray findings, local distribution and clinical signs.

Camurati-Engelmann disease is a rare progressive bone dysplasia; involvement of the skull base can lead to deafness, vestibular disturbances, facial paralysis and damage to the optic nerves. Treatment with corticosteroids, calcitonin and diphosphonates promises only very limited success. Conservative treatment of compression of the cranial nerves is almost ineffective, but the aim of surgical treatment is decompression of involved nerves. The differential diagnosis depends on radiological findings and clinical symptoms. Follow-up depends on radiographic examination and skeletal scintigraphy for showing the extent of the disease. CT may help in demonstrating compression of cranial nerves and define the indications for surgical decompression.