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自1957年 JerveIl 等首次报导耳聋-心脏综合征以来,已有一些家族性 Q-T 间期延长的报导,许多患者发生突然死亡。除了有心电图 Q-T 间期延长,晕厥发作并最终导致室性心律失常外,先天性耳聋也是其特征之一,本文报导姊妹二人均有 Q-T 间期延长。例1 女,13岁,以往体健,在学校中发生晕厥,晕厥之前有轻度眩晕感及恶心,发作时有惊厥及尿失禁,起病前无过度体力活动或情绪波动等诱因,其家
Since the first report of deafness-heart syndrome by Jerve et al. In 1957, there have been some reports of prolonged Q-T prolongation in the family and many have undergone sudden death. In addition to prolongation of the Q-T electrocardiogram, episodes of syncope and consequent ventricular arrhythmias, congenital deafness is also one of the hallmarks of this phenomenon. It is reported herein that both sisters have Q-T prolongation. Example 1 Female, 13 years old, formerly healthy, syncope in school, slight dizziness and nausea before syncope, convulsions and urinary incontinence in seizures, no excessive physical activity or mood swings before onset,