Treacher-Collins syndrome. Management of major and minor anomalies of the ear

Abstract

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.