As a different class of animal model from mice, pig is being highly valued in areas including comparative biology, developmental biology, medical genetics and animal husbandry. Our representation describes a variation of the unique Rongchang pig strain-Rongchang hereditary pedigree connected to hearing defects . The mutation sites, pathology and pathogenesis are very similar to those in human Waardenburg syndrome type 2A. Application of this pedigree, in the field of hearing research, benefits the study on treatment of neurosensory hearing loss with stem cell transplantation, early genetic intervention, auditory device implantation, etc. Moreover, it provides an ideal model of large mammals with respect to hearing medicine sciences.%猪作为继小鼠之后的又一类模式动物，正受到包括比较生物学、发育生物学、医学遗传学、畜牧学等在内的多领域科学家的高度关注。本文主要介绍我国特有的荣昌猪品系中的一个变异群体－“荣昌猪遗传性听力缺陷家系”，其突变位点，病理表现和发病规律与人类Waardenburg综合征2A型极为相似。此家系在听觉医学研究中的应用为研究感音神经性耳聋的干细胞移植，早期基因干预治疗、听觉植入等创造了条件。为听觉医学领域的相关研究提供了理想的大型哺乳动物模型。
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