Cusimano F, Martines E, Rizzo C
Department of Audiology, University of Palermo, Italy.
Int J Pediatr Otorhinolaryngol. 1991 Jul;22(1):49-58. doi: 10.1016/0165-5876(91)90096-t.
Deafness and electrocardiographic changes (prolongation of the Q-T interval and inversion of the T wave) with a clinical picture of syncopal attacks and sudden death, were described as a distinct syndrome by Jervell and Lange-Nielsen in 1957. The syndrome is inherited as an autosomal recessive trait. In this study, all the cases reported since 1957 and their proposed prevalence are reviewed. The authors describe the 4 cases they have studied, all of which presented congenital sensorineural hearing loss and electrocardiographic changes characteristic of the syndrome. The relatively high number of cases they have encountered casts doubt on literature that states that the syndrome occurs more frequently in Northern-European populations. Consequently, it is advisable to perform an electrocardiogram in all children affected by congenital deafness.
1957年，耶尔韦尔和朗格 - 尼尔森将伴有晕厥发作和猝死临床表现的耳聋及心电图改变（Q-T间期延长和T波倒置）描述为一种独特的综合征。该综合征以常染色体隐性性状遗传。在本研究中，对1957年以来报道的所有病例及其推测的患病率进行了综述。作者描述了他们研究的4例病例，所有病例均表现为先天性感音神经性听力损失及该综合征特征性的心电图改变。他们遇到的病例数量相对较多，这对文献中称该综合征在北欧人群中更常见的说法提出了质疑。因此，建议对所有先天性耳聋患儿进行心电图检查。
