原发性Q-T间期延长综合征是以阵发性晕厥;Q-T间期延长;室性心律失常;猝死;可能伴有先天性聋哑为主征的一组综合征。伴聋哑者,称为Jrervell-Lange-Nielson综合征。多有家族性,属常染色体隐性遗传。不伴聋哑者,属常染色体显性遗传,称为Romano-Ward综合征。本文所报告的一例是有家族性和先天性聋哑的Q-T间期延长综合征。患儿,男,3岁,朝鲜族,85年8月30日突然晕厥收入院。一年前开始突然晕厥,已发着7次。晕厥时面 Primary Q-T prolongation syndrome is paroxysmal syncope; Q-T interval prolongation; ventricular arrhythmia; sudden death; may be associated with congenital deaf and dumb-based syndrome syndrome. With deaf people, called Jrervell-Lange-Nielson syndrome. More familial, autosomal recessive inheritance. Without deaf who are autosomal dominant inheritance, known as Romano-Ward syndrome. An example reported here is Q-T prolongation syndrome with familial and congenital deafness. Children, male, 3 years old, Korean, August 30, 85 sudden syncope income hospital. Suddenly began to faint a year ago, has sent seven times. Syncope time