1.
Acta Otorrinolaringol Esp
; 45(3): 207-13, 1994.
Article
in Spanish
| MEDLINE
| ID: mdl-8068367
ABSTRACT
Camurati-Engelmann's disease (progressive diaphyseal dysplasia) is a rare hereditary condition characterized by symmetrical hyperostosis of the long bones and the skull-base, myopathies and neurological disturbances. So far, little more than one hundred cases have been reported. The diagnosis is based on radiological imaging (traditional X rays and CT scan) and family history. Our patient (a 15-year-old male) had high-grade skull-base hyperostosis producing audiological signs, such as progressive deafness, persistent otorrhea and otalgia with progressive stenosis of the external acoustic meatus.