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Archives de pédiatrie
Volume 15, n° 11
pages 1663-1666 (novembre 2008)
Doi : 10.1016/j.arcped.2008.08.018
Received : 25 November 2007 ;  accepted : 7 August 2008
L’ostéogenèse imparfaite, cause rare de surdité chez l’enfant
Osteogenesis imperfecta and hearing loss in childhood

W. Mnari , C. Hafsa, R. Salem, M. Maatouk, M. Golli
Service d’imagerie médicale, CHU F-Bourguiba Monastir, 5000 Monastir, Tunisie 

Auteur correspondant. App. 64, 18, rue du Général-Sarrail, 94000 Créteil, France.

Nous rapportons 1 cas rare de surdité bilatérale précoce chez 1 enfant atteint d’ostéogenèse imparfaite, en précisant l’apport capital de la tomodensitométrie pour le diagnostic. Un enfant âgé de 12 ans, atteint d’ostéogenèse imparfaite, consultait pour une surdité bilatérale. La tomodensitométrie des rochers mettait en évidence de multiples hypodensités préstapédiennes étendues à la lumière endocochléaire, avec d’autres foyers hypodenses du labyrinthe osseux au voisinage du vestibule et du conduit auditif interne. La perte progressive de l’audition est une manifestation souvent tardive de l’ostéogenèse imparfaite, elle est rare chez l’enfant. La tomodensitométrie des rochers, utilisant des coupes fines millimétriques, met en évidence de multiples foyers de déminéralisation touchant le labyrinthe osseux d’une façon variable, pouvant faire discuter d’autres affections déminéralisantes du rocher comme l’otospongiose.

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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.

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Mots clés : Ostéognèse imparfaite, Surdité, Rochers, Tomodensitométrie

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