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L’ostéogenèse imparfaite, cause rare de surdité chez l’enfant

Authors :
Mnari, W.
Hafsa, C.
Salem, R.
Maatouk, M.
Golli, M.
Source :
Archives de Pédiatrie. Nov2008, Vol. 15 Issue 11, p1663-1666. 4p.
Publication Year :
2008

Abstract

Summary: We report a case of bilateral hearing loss in a child, caused by osteogenesis imperfecta and we evaluate CT scan findings. A 12-year-old child consulted for bilateral hearing loss. A computed tomography scan of the petrous temporal bone showed bilateral extensive unmineralized bone involving the cochleae, vestibules, and semicircular canals extending to the internal auditory canals. Osteogenesis imperfecta of the temporal bone is a genetic connective tissue disorder with increased bone fragility, low bone mass, and other extraskeletal manifestations. Hearing loss is rare in the first 2 decades of life, but it is one of the frequent features of this disorder in adult patients. Thin-section CT scans of the temporal bone show a remarkable proliferation of unmineralized bone involving the otic capsule. This demineralization is similar to that observed in the cochlear form of otospongiosis. [Copyright &y& Elsevier]

Details

Language :
French
ISSN :
0929693X
Volume :
15
Issue :
11
Database :
Academic Search Index
Journal :
Archives de Pédiatrie
Publication Type :
Academic Journal
Accession number :
35231486
Full Text :
https://doi.org/10.1016/j.arcped.2008.08.018