Friedman Adva B, Guillory Ryan, Ramakrishnaiah Raghu H, Frank Robert, Gluth Michael B, Richter Gresham T, Dornhoffer John L
Department of Otolaryngology/Head and Neck Surgery, University of Arkansas for Medical Sciences, 4301 West Markham, Little Rock, AR 72205, USA.
Int J Pediatr Otorhinolaryngol. 2013 Jul;77(7):1128-31. doi: 10.1016/j.ijporl.2013.04.016. Epub 2013 May 20.
To explore the etiology of pediatric unilateral severe-to-profound sensorineural hearing loss (USPSNHL) and the risk of hearing loss in the contralateral ear.
Pediatric patients with USPSNHL referred to a tertiary children's hospital for unilateral bone-anchored hearing aid implantation were identified for study. Clinical charts, audiograms, and radiographic imaging of the temporal bones were reviewed.
Eighty-four children with USPSNHL were reviewed (40 male, 44 female). The etiology of USPSNHL could be identified in 35 patients (41.7%), with perinatal events (16.2%) and family history of hearing loss (10.0%) being the most common identifiable risk factors. Forty percent of children with available newborn hearing screens passed in both ears but later developed USPSNHL. Progressive unilateral hearing loss leading to USPSNHL was found in 21% percent of patients. No patient experienced worsening hearing in the contralateral ear (mean follow-up=31.9 months). Of the 49 temporal bone scans available for review, 40.8% demonstrated aberrant inner ear anatomy, including semicircular canal dysplasia (10.2%), cochlear aperture stenosis (10.2%), hypoplastic cochlea (8.1%), enlarged vestibular aqueduct (14.3%), incomplete partition I/II (6.1%), and anomalous internal auditory canal (2.0%).
Pediatric USPSNHL can be attributed to a variety of sources with a cause identifiable in approximately 40% of patients. Temporal bone CT scan acquisition appears to be relatively high yield in this patient population, but only rarely do results have clinical implications. Early follow-up suggests that this population of children with USPSNHL does not appear to have a significantly increased risk for hearing loss in the contralateral ear.
探讨儿童单侧重度至极重度感音神经性听力损失（USPSNHL）的病因及对侧耳听力损失的风险。
纳入在一家三级儿童医院接受单侧骨锚式助听器植入的USPSNHL患儿进行研究。回顾临床病历、听力图和颞骨影像学检查结果。
共纳入84例USPSNHL患儿（男40例，女44例）。35例（41.7%）患儿的USPSNHL病因可明确，围产期事件（16.2%）和听力损失家族史（10.0%）是最常见的可识别风险因素。40%通过双耳新生儿听力筛查的儿童随后出现了USPSNHL。21%的患者存在导致USPSNHL的进行性单侧听力损失。没有患者对侧耳听力恶化（平均随访31.9个月）。在49例可供回顾的颞骨扫描中，40.8%显示内耳解剖结构异常，包括半规管发育不全（10.2%）、耳蜗孔狭窄（10.2%）、耳蜗发育不全（8.1%）、前庭导水管扩大（14.3%）、不完全分隔I/II（6.1%）和内耳道异常（2.0%）。
儿童USPSNHL可归因于多种原因，约40%的患者病因可明确。颞骨CT扫描在该患者群体中的诊断率相对较高，但结果具有临床意义的情况很少。早期随访表明，USPSNHL患儿对侧耳听力损失风险似乎没有显著增加。
