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.