Bartel-Friedrich S, Fuchs M, Amaya B, Rasinski C, Meuret S, Kösling S
Abteilung Phoniatrie und Pädaudiologie, Klinik und Poliklinik für Hals-Nasen-Ohren-Heilkunde, Kopf- und Halschirurgie, Universitätsklinikum der Martin-Luther-Universität Halle-Wittenberg, Deutschland.
HNO. 2008 Feb;56(2):225-30. doi: 10.1007/s00106-007-1665-y.
The aim of this study was to clarify the clinical course of large endolymphatic duct and sac syndrome (LEDS). Although LEDS is the most common form of radiologically detectable inner ear malformation associated with sensorineural hearing loss (SNHL), it is relatively unknown in the German-speaking countries. We hoped to derive useful pointers for clinical care.
We evaluated the clinical audiometric records and imaging findings of 169 patients with clinically suspected inner ear malformation seen between 1994 and 2003. Following identification of all LEDS cases, we serially investigated the anamnestic and functional data with regard to severity and course.
Among 169 patients, 17 (median age 12 years; 12 females) showed LEDS. A total of 28 ears were affected. Clinically, most cases were of prelingual or perilingual onset and displayed steady or fluctuatingly progressive severe SNHL with emphasis on the high frequencies or deafness. Episodes of sudden hearing loss were relatively frequent. No correlation could be demonstrated between the severity of morphological changes and the degree of SNHL.
Progression of SNHL and episodes of sudden hearing loss were seen mainly in late childhood or adolescence. This should be taken into account when informing and advising patients and planning therapy.
本研究旨在阐明大前庭导水管综合征（LEDS）的临床病程。尽管LEDS是与感音神经性听力损失（SNHL）相关的最常见的可通过放射学检测到的内耳畸形形式，但在德语国家相对鲜为人知。我们希望能得出对临床护理有用的指导意见。
我们评估了1994年至2003年间临床怀疑有内耳畸形的169例患者的临床听力记录和影像学检查结果。在确定所有LEDS病例后，我们对病史和功能数据的严重程度及病程进行了系列研究。
在169例患者中，17例（中位年龄12岁；12名女性）表现为LEDS。共有28只耳朵受累。临床上，大多数病例在学语前或学语期发病，表现为稳定或波动渐进性的重度SNHL，以高频听力损失或全聋为主。突发性听力损失发作相对频繁。形态学改变的严重程度与SNHL的程度之间未显示出相关性。
SNHL的进展和突发性听力损失发作主要见于儿童晚期或青少年期。在向患者告知情况、提供建议及规划治疗时应考虑到这一点。
