感音神经性聋 (sensorineuralhearingloss ,SNHL)的发病机制目前尚未完全阐明 ,其致病因素和临床表现形式的多样性使得治疗上难以获得确切有效的结果。近年来 ,有遗传学证据表明感音神经性聋的发生与染色体异常有关 ,先天性或后天性聋可由一些特定基因的突变导致[1～ 4 ]。遗传 The pathogenesis of sensorineural hearing loss (SNHL) is not yet fully elucidated, and the diversity of its causative factors and clinical manifestations make it difficult to obtain definitively valid results in treatment. In recent years, there is genetic evidence that the occurrence of sensorineural deafness and chromosomal abnormalities, congenital or acquired deafness can be caused by a number of mutations in specific genes [1-4]. Genetic