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Hearing loss due to cranio-metaphysial dysplasia
D W Morgan1, C Aldren, T J Hoare
1Royal National Throat, Nose and Ear Hospital, London.
The Journal of Laryngology and Otology
|October 1, 1990
Abstract:
Craniometaphysial dysplasia is a rare cause of conductive hearing loss. It is a genetic disorder of bone due to decreased osteoclastic activity in the endosteal and periosteal regions of craniofacial and long bones. The characteristic temporal bone abnormalities are attic fixation, abnormally shaped ossicles and obliterated foramina ovale. Computerised tomography is helpful in predicting operative findings; surgery should be reserved for those with a severe hearing loss.