Marres H A, Cremers C W, Marres E H
University Hospital Nijmegen, Institute of Otorhinolaryngology, Department of ENT, The Netherlands.
Rev Laryngol Otol Rhinol (Bord). 1995;116(2):105-8.
12 patients suffering from a Treacher-Collins syndrome, or mandibulo-facial dysostosis, were operated on in the Nijmegen University Hospital between 1960 and 1990. An early diagnosis is generally reached when there is a congenital atresia of the auditory canal. Auditory rehabilitation with a conventional prosthesis of the bone or a BAHA is preferable to surgical reconstruction. In minor cases, deafness must be screened as early as possible, with a bone hearing aid prosthesis. Surgical exploration can be performed at best as the age of 10, but the chances of success are less than those of other functional reconstructions because of the associated malformation.
1960年至1990年间,奈梅亨大学医院对12名患有特雷彻-柯林斯综合征(又称下颌面骨发育不全)的患者进行了手术。当出现先天性耳道闭锁时,通常能做出早期诊断。使用传统骨假体或骨锚式助听器进行听觉康复比手术重建更为可取。在轻症病例中,必须尽早使用骨助听器假体进行耳聋筛查。手术探查最好在10岁时进行,但由于存在相关畸形,成功的几率低于其他功能重建手术。