Mandibulofacial dysostosis: CT evaluation of the temporal bones for surgical risk assessment in patients of bilateral aural atresia.
We present the results of detailed CT investigation of nine patients with mandibulofacial dysostosis (MFD). We also graded the severity of microtia according to Marx's classification system. The results revealed a positive correlation between the severity of microtia and the severity of deformity of the external auditory canal, and attic formation. In 13 ears, the stapes was not adequately visualized to allow any comment on its form. The angle of the first genu of the facial nerve ranged from 55.0 to 123.6 degrees (mean +/-S.D.; 99.5 +/-18.5 degrees ), indicating that this angle is more obtuse in MFD patients than in normal subjects. The eustachian tube and tympanic sinus were present in all the ears examined. None of the patients had mastoid pneumatization. In four ears, the lateral semicircular canal was dysplastic. Eleven ears of six patients scored less than 5 in the grading system developed by Jahrsdoerfer for assessment of the suitability for atresia surgery. These findings indicate that most patients with mandibulofacial dysostosis are poor surgical candidates.
Takegoshi
H
H
Department of Otorhinolaryngology, Faculty of Medicine, University of Tokyo, Hongo 7-3-1, Bunkyo-ku, Tokyo 113-8655, Japan. takegoshi@mb.kcom.ne.jp
Kaga
K
K
Kikuchi
S
S
Ito
K
K
D002675:Child, Preschool; D004431:Ear, External / Q000002:abnormalities* / Q000000981:diagnostic imaging; D007758:Ear, Inner / Q000002:abnormalities / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D008297:Male; D008342:Mandibulofacial Dysostosis / Q000000981:diagnostic imaging* / Q000601:surgery; D018570:Risk Assessment; D013701:Temporal Bone / Q000000981:diagnostic imaging*; D014057:Tomography, X-Ray Computed