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1957年Jervell和Lange-Nielsen报告一组病人,特点为:①先天性双侧高频性耳聋;②心电图示Q-T间期延长;③因心律失常而发作晕厥、猝死。该组病人发生于挪威的一个家庭中,在兄妹6人中,4人生后一年内就开始发作晕厥,其中3人分别于3、4和9岁时,发作晕厥而猝死。此后屡有报告,称心脏-听力综合征、耳聋-心脏综合征或Jervell和Lange-Nielsen综合征。
1957 Jervell and Lange-Nielsen report a group of patients, characterized by: ① congenital bilateral high-frequency deafness; ② ECG Q-T prolongation; ③ due to arrhythmia and syncope, sudden death. This group of patients occurred in a Norwegian family. Out of 6 siblings, onset of syncope occurred within 4 years after birth, and 3 of them died of syncope at the age of 3, 4, and 9 years. Repeatedly reported thereafter, said the heart - hearing syndrome, deafness - heart syndrome or Jervell and Lange-Nielsen syndrome.