抄録
The temporal bone findings in a case of Cornelia de Lange syndrome were reported.
Cornelia de Lange syndrome is characterized by peculiar facies such as synophrys, anteverted nostrils, low set ears and micrognathia, and retardation of mental and physiological development, microbrachycephaly, micromelia, and so on.
The pathologic findings of the temporal bones were as follows; 1) shortened cochlea, 2) maldevelopment of the posterior semicircular canal, 3) malformations of the auditory ossicles, 4) mesenchymal tissue in the tympanic cavity, 5) wide angle of the facial genu, 6) large bony defect of the facial canal.
