Park A H, Kou B, Hotaling A, Azar-Kia B, Leonetti J, Papsin B
Department of Otolaryngology--Head and Neck Surgery, Loyola University Medical Center, Maywood Illinois 60153, USA.
Laryngoscope. 2000 Oct;110(10 Pt 1):1715-9. doi: 10.1097/00005537-200010000-00029.
To determine any factors that could improve the early detection and management of congenital inner ear malformations.
A retrospective review was performed of all patients with a diagnosis of inner ear malformation at Loyola University Medical Center (LUMC) and the Hospital for Sick Children (HSC) between 1987 and 1995. Clinical records and audiometric data were accumulated. One neuroradiologist reviewed every temporal bone computed tomography (CT) scan.
Forty-six pediatric patients with congenital inner ear anomalies evaluated at two tertiary care hospitals.
The average patient age at initial assessment was 25.7 months. A family history of hearing loss was noted in only five patients (12.8%). A major nonotological deformity was seen in 41% of patients. The average hearing threshold was 88 dB. All three patients with sudden hearing loss had vestibular aqueduct enlargement. Two of the three patients with common cavity anomalies had a history of recurrent meningitis. Twenty-seven patients had a vestibular aqueduct deformity, the most frequent radiographic abnormality in the series.
Because inner ear malformation was diagnosed after 24 months of age in a significant percentage of patients, we recommend increased parental education and vigilance by primary care practitioners. Universal newborn screening may be the key to earlier detection of these infants. For children with idiopathic sensorineural hearing loss, we recommend a temporal bone CT scan. Patients with vestibular aqueduct enlargement must be counseled about the risk of progressive sensorineural hearing loss, meningitis, and the need to avoid contact sports. Patients with common cavity abnormalities should be considered for exploratory tympanotomy and also educated about the risk for meningitis.
确定任何可改善先天性内耳畸形早期检测与管理的因素。
对1987年至1995年间在洛约拉大学医学中心（LUMC）和病童医院（HSC）被诊断为内耳畸形的所有患者进行回顾性研究。收集临床记录和听力测定数据。由一名神经放射科医生审阅每例颞骨计算机断层扫描（CT）。
对在两家三级医疗医院评估的46例患有先天性内耳异常的儿科患者进行研究。
初次评估时患者的平均年龄为25.7个月。仅5例患者（12.8%）有听力损失家族史。41%的患者有主要的非耳科畸形。平均听力阈值为88分贝。所有3例突发听力损失患者均有前庭导水管扩大。3例共同腔畸形患者中有2例有复发性脑膜炎病史。27例患者有前庭导水管畸形，是该系列中最常见的影像学异常。
由于相当比例的患者在24个月龄后才被诊断出内耳畸形，我们建议加强对家长的教育，并提高初级保健医生的警惕性。普遍的新生儿筛查可能是早期发现这些婴儿的关键。对于特发性感音神经性听力损失的儿童，我们建议进行颞骨CT扫描。必须向有前庭导水管扩大的患者告知进行性感音神经性听力损失、脑膜炎的风险以及避免接触性运动的必要性。对于有共同腔异常的患者，应考虑进行探索性鼓室切开术，并告知其脑膜炎的风险。
