両側耳小骨奇形を伴つたTreacher Collins症候群の1例

TREACIIER-COLLINS SYNDROME WITH MALFORMATION OF THE EAR OSSICLES, A CASE REPORT

　Ⅰ．緒言

　先天性上顎骨および下顎骨の発育不全については，Treacher Collins症候群，Berry症候群，Fanceschetti症候群，Mandibulofacial Dysostosis第1鰓弓症候群，第1，第2鰓弓症候群などの命名があり，それぞれに多少のnuanccの違いがあるようである。今回，私どもは両側耳小骨奇形を伴つたTreacher Collins症候群と思われる1症例を経験し，両側手術による聴力改善に成功したので，その概要を，発生に関する文献的考察を加えて，ここに報告した。

　A case of malformation of the middle ear ossicles, Treacher-Collins syndrome, is reported with a review of literature.

　The malformations were revealed during the course of operation performed for hearing improvements. The processes of the malleus were prolonged on both sides. The movement of the incus was limited on the right side, while the left was entirely missing except for a rudimental remnant of the structure.

　Besides these malformations the patient showed a facial malformation which the authors believe, that pointed to developmental aberration of the first and second brachial arches.