Imaging of ear deformities in Treacher Collins syndrome

Conductive deafness is a common feature of the Treacher Collins syndrome. The bilateral, essentially symmetrical hypoplastic deformities of middle and external ears, are characteristic. Improved imaging of the deformities using high resolution computed tomography (HRCT) has accompanied a present reluctance to explore surgically these middle ears because of poor results and the increasing success of bone anchored hearing aids. We describe the results of HRCT in 13 patients with Treacher Collins syndrome. An unpneumatized mastoid and a greatly reduced attico-antral region are almost constant findings, but a proportion of patients have a patent external auditory meatus, and, only in these is exploratory surgery feasible. Correction of ossicular deformities may be possible. Patients with atresia of the external auditory meatus are not suitable for exploration and need bone anchored hearing aids. Imaging for these children should be limited with regard to radiation dose and sedation.