Alport综合征的听力表现及遗传方式

Alport-Syndrome hearing display and the way of heredity

报告５例Ａｌｐｏｒｔ综合征。结合其遗传方式及听力表现进行分析，５例中３例为常染色体显性遗传，２例为Ｘ染色体连锁隐性遗传。本病不只呈一种遗传方式，在不同家系中以不同方式遗传。听力表现：５例均为双侧感音神经性聋，听力损失约４０～７０ｄＢ。听力曲线表现多样，高频下降型２例，上升型１例，呈谷型２例。声导抗测试：镫骨肌声反射Ｍｅｔｚ试验阳性，感音性耳聋可能性较大。根据其听力表现及分析结果，提示听力检查应力求全面，有助于证明耳聋与肾病表现的关系。

cases of Alportds-syndrome have been reported.They were analysed on the basis of way of their heredity and hearing behavior.They are 2 cases of X-linked recessive and 3 cases of autosome dominant inheritance.The different family trees are different in the way of heredity.The results of pure-tone audiometry showed that they all agreed with sensorineural deafness.