EAR-SURGERY IN TREACHER-COLLINS-SYNDROME

The autosomal dominant hereditary Treacher Collins syndrome manifests itself phenotypically in dysmorphogenesis of particularly the first, b ut also the second branchial arch system. Consequently, 50% of patient s with Treacher Collins syndrome have a congenital, generally pure con ductive hearing loss resulting from a major or minor ear anomaly. The outcome of surgery to improve patients' hearing varies and is sometime s even disappointing. Thorough analysis of 33 cases (39 operated ears) and the strict application of a classification for the anomaly to eac h ear enabled us to gain insight into the most suitable surgical polic y and to form a prognosis for reconstructive ear surgery.