Objective This study aims to investigate the disease characteristics of patients with Townes-Brocks Syndrome(TBS)and explore appropriate interventions for different types of hearing loss.Methods A comprehensive examination was conducted on TBS patients,summarizing the clinical phenotype,laboratory and imaging abnormalities,as well as evaluating and recording detailed results of hearing-related examinations.Results Based on the evaluation of systemic multi-system abnormalities'tolerability for surgery combined with hearing-related examination results,appropriate interventions were developed.Conclusion The optimal treatment for patients with TBS should be chosen based on the specific clinical phenotype and the degree and type of hearing loss.including hearing AIDS,auditory chain reconstruction surgery,and cochlear implant.