Although the Treacher-Collins syndrome is not a new entity and cases have been reported sporadically since Berry in 1888 first reported two cases of congenital coloboma of the lower eyelids, it is surprising that little has been written concerning the otologic aspects of this very interesting, if unfortunate, aggregate of congenital malformations. In the past, most efforts in the treatment of this syndrome have been directed toward cosmetic improvement of the facial appearance while disregarding or minimizing its effects on the ear. To the best of our knowledge, 12 cases of mandibulofacial dysostosis have been treated at either Temple Medical Center or St. Christopher's Hospital for Children since 1959. It is our purpose not only to discuss the syndrome and its general characteristics, but, in presenting seven of these cases, to focus attention on the otologic manifestations, including audiologic and surgical findings as well as methods and results of treatment.