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下颌面骨发育不全(Mandibular facial dysostosis)首由Barry于1889年报告,1900年Treacher Collins报告2例相似病例而被名为Treacher Collins综合征。至今已报告200例以上,且认为是常染色体显性遗传病。Henner(1960)将伴有小耳与外耳道闭锁患者分为3级:1.耳廓形态大小正常,外耳道狭小,鼓膜活动,乳突常气化和听力损失50～60分贝的传音聋;2.不同程度小耳,外耳道闭锁或狭窄,听骨常畸形,乳突气化而中耳大小正常;3.中耳明显缩小或丧失及咽鼓管未发育,余同Ⅱ级。对畸形者可
Mandibular facial dysostosis was first reported by Barry in 1889 and Treacher Collins reported two similar cases in 1900 and was named Treacher Collins syndrome. To date, more than 200 cases have been reported and are considered autosomal dominant. Henner (1960) will be accompanied by small ears and external auditory canal atresia patients are divided into three levels: 1. Auricle shape and size of the normal, small external auditory canal, tympanic membrane activity, mastoid often gasification and hearing loss of 50 to 60 dB of acoustic deafness; Different degrees of small ears, the external auditory canal atresia or stenosis, cataract, mastoid gasification and the size of the middle ear; 3. The middle ear was significantly reduced or lost and eustachian tube is not developed, with the same level Ⅱ. Deformity can be