收集了一个六代遗传性耳聋大家系，进行了病史、体检、纯音听力测试的综合分析，明确了该家系疾病的特征，在此基础上应用文献报道的常染色体显性遗传耳聋相关基因座的多态性标记进行等位基因共享分析，结果表明该家系的致病基因与已报道基因座不相关，连锁分析结果进一步确定这是一个尚未报道的新的基因座。 A six-generation hereditary deafness pedigree was collected for a comprehensive analysis of history, physical examination and pure tone hearing tests, and the characteristics of the pediatric diseases were clarified. On the basis of this, the prevalence of autosomal dominant deafness related loci Polymorphism markers were used for allelic sharing analysis. The results showed that the causative gene of this pedigree was not related to the reported locus. The linkage analysis further confirmed that this gene was a new locus which had not been reported yet.