1957年,Jervell和Lange-Nielsen首次报道在一挪威家庭中看到的耳聋-心脏综合征,其临床表现为先天性耳聋,Q-T间期延长,心律失常,时有晕厥发作,发作时甚至可至猝死,属常染色体隐性遗传。1963年以后,Romano、Ward和Barlovs等又相继报道不伴先天性耳聋的类似综合征,属常染色体显性遗传。这二类Q-T间期延长综合征,多数是家族性的,但个别也可以是非家族性的。晕厥的直接原因是室性心动过速、心室颤动或心室停搏。本文报道马姓一家中姐弟三人患不伴耳聋的Q-T间期延长综合征。这三人的二个姐 In 1957, for the first time, Jervell and Lange-Nielsen reported deafness-heart syndrome seen in a Norwegian family whose clinical manifestations were congenital deafness, prolonged QT interval, arrhythmia, occasional fainting episodes, and even seizures Sudden death, autosomal recessive inheritance. After 1963, Romano, Ward and Barlovs and so on have reported a similar syndrome without congenital deafness, autosomal dominant inheritance. These two types of Q-T prolongation syndrome, the majority is familial, but individual may also be non-familial. The direct cause of syncope is ventricular tachycardia, ventricular fibrillation or ventricular arrest. This article reports a maternal family of three siblings suffering from deafness without Q-T prolongation syndrome. The three sisters of the two