先天性耳聋或聋哑是一种常见的致残疾病，其病因可分为遗传型与非遗传型二类。遗传型中其遗传方式多数为常染色体隐性，少数为常染色体显性或X连锁隐性。我们在遗传咨询门诊中发现两个X连锁隐性感音性聋家系，因较少见故报道如下。病例与家系家系1：先证者，男，15岁，学生，于 Congenital deaf or deaf is a common disabling disease, the etiology can be divided into two types of genetic and non-genetic. Genetics in the majority of its genetic methods are autosomal recessive, a few are autosomal dominant or X-linked recessive. We found in the genetic counseling clinic two X-linked audio-visual hearing-impaired deaf family, as a result of less so reported as follows. Case and family pedigree 1: proband, male, 15 years old, student, at