Division of Pediatric Radiology, Department of Radiology and Radiological Science, Johns Hopkins Hospital, Baltimore, MD 21287-0842, USA.
J Neuroradiol. 2013 Jul;40(3):192-7. doi: 10.1016/j.neurad.2012.10.002. Epub 2013 Feb 18.
This study aimed to determine the spectrum of temporal bone computed tomography (CT) abnormalities in children with conductive hearing loss (CHL) with and without microtia.
From 1993 to 2008, a total of 3396 pediatric records including CHL were reviewed at our institution and revealed 180 cases of persistent CHL, 46 of whom had diagnostic temporal bone CT examinations. All of these examinations were systematically reviewed by two pediatric neuroradiologists, working in consensus, who had 5 and 18 years, respectively, of dedicated pediatric neuroradiology experience.
Of the 46 children, 16 were boys and 30 were girls (age: 0.2-16 years; mean: 5 years). Also, 21 (46%) children had microtia and 25 (54%) children did not, as determined by clinical evaluation. External auditory canal atresia/stenosis (EAC-A/S) was the most common anomaly in both microtia and non-microtia groups. Two or more anomalies were observed in 18/21 children with microtia. The frequency of EAC-A/S was greater in children with microtia versus those without it (86% versus 32%, respectively; P = 0.0003). Syndromic diagnoses were also significantly more frequently made in children with microtia versus those without microtia (76% versus 20%, respectively; P = 0.0001). Temporal bone CT scans were normal in 10 children (22%) with persistent CHL.
Microtia is an important finding in children with CHL. EAC and middle ear/ossicle anomalies were significantly more frequently seen in children with microtia, and multiple anomalies and bilateral microtia were more common in children with syndromic associations. These findings highlight the importance of understanding the embryological development of the temporal bone. The presence of one anomaly should raise suspicion of the possibility of other anomalies, especially in the setting of microtia. Bilateral microtia and multiple anomalies should also raise suspicion of genetic syndromes.
本研究旨在确定伴有和不伴有小耳畸形的传导性听力损失（CHL）儿童的颞骨计算机断层扫描（CT）异常谱。
1993 年至 2008 年，我们机构对包括 CHL 在内的 3396 例儿科病历进行了回顾，发现 180 例持续性 CHL，其中 46 例进行了诊断性颞骨 CT 检查。所有这些检查均由两位儿科神经放射学家进行系统回顾，他们分别具有 5 年和 18 年的儿科神经放射学经验。
在 46 名儿童中，男 16 例，女 30 例（年龄：0.2-16 岁；平均年龄：5 岁）。根据临床评估，21 例（46%）儿童有小耳畸形，25 例（54%）儿童无小耳畸形。外耳道口狭窄/闭锁（EAC-A/S）是小耳畸形和非小耳畸形组中最常见的异常。21 例小耳畸形患儿中有 18 例存在 2 种或以上畸形。小耳畸形患儿的 EAC-A/S 发生率高于无小耳畸形患儿（分别为 86%和 32%；P = 0.0003）。小耳畸形患儿的综合征诊断也明显多于无小耳畸形患儿（分别为 76%和 20%；P = 0.0001）。10 例（22%）持续性 CHL 患儿的颞骨 CT 扫描正常。
小耳畸形是 CHL 儿童的重要发现。EAC 和中耳/听小骨异常在小耳畸形患儿中更为常见，且综合征相关患儿的多畸形和双侧小耳畸形更为常见。这些发现强调了了解颞骨胚胎发育的重要性。出现一种异常应怀疑其他异常的可能性，尤其是在小耳畸形的情况下。双侧小耳畸形和多畸形也应怀疑遗传综合征。
