Abstract
Treacher Collins Syndrome, alternatively called mandibulofacial dysostosis, is an autosomal dominant anomaly of the craniofacial development that occurs with an incidence of 1 in 50,000 live births. Approximately 60% of cases possibly arise as a result of mutations. After assessing HRCT images using Jahrsdoerfer's grading scale, we performed tympanoplasty for two siblings with Treacher Collins Syndrome. The use of Jahrsdoerfer's grading scale provides us a great prediction of postoperative hearing prognosis. However, mobility of stapes are not evaluated by the grading system, so that we have to prepare for stapes surgery.