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目的：分析无综合征的遗传性进行性感音神经性聋三个家系的听力学特点及遗传特征。方法：三家系纯音测听３３人，耳聋１４例，声导抗、ＡＢＲ测试４人。对这些家系进行相关资料的调查和听力学检查。结果：三个家系语前聋１个家系，语后聋２个家系，表现为双侧对称性进行性听力下降，Ａ家系耳聋始于１２岁后，Ｂ家系耳聋始于２０岁后，首先是高频区受损，迅速依次向中、低频扩展，Ｃ家系出生后即耳聋，表现为聋哑症。４例８耳声导抗及ＡＢＲ测试均证实为耳蜗性感音性听力损失，三个家系男女均有发病，显示了很高的外显率，全身检查未发现其它部位畸形。结论：三个家系可能为无综合征的常染色体显性遗传性感音神经性聋。
Objective: To analyze the hereditary characteristics and genetic characteristics of three families without hereditary sensory neuropathy deafness without syndrome. Methods: Three pedigree pure tone audiometry 33 people, deafness in 14 cases, acoustic impedance, ABR test 4 people. Investigate and audiologically examine the data of these families. Results: There were 1 pedigree of prelingual deafness and 2 pedigrees of post-lingual deafness in the three families. The performance of bilateral familial hearing loss was deafness. After deafness of family A began after 12 years old, deafness of family B began after age 20, High-frequency area impaired, rapidly in turn to the middle and low frequency expansion, C family deafness after birth, manifested as deaf and dumb. Four cases of 8 otoacoustic impedance and ABR tests were confirmed as cochlear sensory hearing loss, both men and women in three families incidence, showed a high penetrance, the body examination found no other site deformity. Conclusion: The three families may be autosomal dominant sensorineural hearing loss without syndrome.