1984～1988年期间南京医学院生物学教研室与第一附属医院耳鼻咽喉科对我省某县某地区(该地区包括六个乡,178000多人口)方氏大家系遗传性聋进行了现场调查研究。确证方氏家系在该地已衍生六代,其中一、二代均死,第三代仅少数人存活,而四、五、六代人口代代增多,目前人数已达683人(血缘关系508人,配偶175人),80％以上均生活在当地。确定该家属中遗传性聋患者137例,发病年龄分布在0～53岁。患者均为单纯性聋,具有早发倾向。遗传方式为常染色体显性遗传,但为不规则显性遗传,外显率为79.10％。已完成科研总结报告,内容包括:一、绪言;二、遗传性研究报告:(一)家谱分析,(二)皮纹学研究,(三)染色体及姐妹染色单体交换的观察分析,(四)ABO血型鉴定;三、基因分析和听力学调 From 1984 to 1988, Department of Otolaryngology, Department of Biology, Nanjing Medical College and First Affiliated Hospital of Ophthalmology conducted a field survey on the hereditary deafness of a large family in a certain county of our province (including six towns and more than 178000 population) . It is confirmed that Fang’s family has been derived from this family for six generations, of which one or two are dead, the third generation only a few survive, and the fourth, fifth and sixth generation population increased from generation to generation, the current number has reached 683 (kinship 508 Person, spouse 175), more than 80% live in the local. To determine the families of hereditary deaf patients in 137 cases, the age of onset in 0 to 53 years old. Patients are simply deaf, with premature tendency. Genetic means of autosomal dominant inheritance, but irregular dominant, penetrance was 79.10%. Scientific research summary report has been completed, including: I. Introduction; 2. Hereditary research reports: (a) genealogical analysis, (b) dermatoglyphics, (c) observation and analysis of chromosome and sister chromatid exchange ) ABO blood type identification; Third, gene analysis and audiology tune