Purcell Patricia L, Shinn Justin R, Coggeshall Scott S, Phillips Grace, Paladin Angelisa, Sie Kathleen C Y, Horn David L
*Department of Otolaryngology †Division of Pediatric Otolaryngology, Seattle Children's Hospital ‡Department of Biostatistics §Department of Radiology, University of Washington, Seattle, Washington.
Otol Neurotol. 2017 Jul;38(6):e138-e144. doi: 10.1097/MAO.0000000000001452.
To investigate the risk of hearing loss progression in each ear among children with unilateral hearing loss associated with ipsilateral bony cochlear nerve canal (BCNC) stenosis.
Tertiary pediatric referral center.
Children diagnosed with unilateral hearing loss who had undergone temporal bone computed tomography imaging and had at least 6 months of follow-up audiometric testing were identified from a prospective audiological database.
Two pediatric radiologists blinded to affected ear evaluated imaging for temporal bone anomalies and measured bony cochlear canal width independently. All available audiograms were reviewed, and air conduction thresholds were documented.
Progression of hearing loss was defined by a 10 dB increase in air conduction pure-tone average.
One hundred twenty eight children met inclusion criteria. Of these, 54 (42%) had a temporal bone anomaly, and 22 (17%) had ipsilateral BCNC stenosis. At 12 months, rates of progression in the ipsilateral ear were as follows: 12% among those without a temporal bone anomaly, 13% among those with a temporal bone anomaly, and 17% among those with BCNC stenosis. Children with BCNC stenosis had a significantly greater risk of progression in their ipsilateral ear compared with children with no stenosis: hazard ratio (HR) 2.17, 95% confidence interval (CI) (1.01, 4.66), p value 0.046. When we compared children with BCNC stenosis to those with normal temporal bone imaging, we found that the children with stenosis had nearly two times greater risk estimate for progression, but this difference did not reach significance, HR 1.9, CI (0.8, 4.3), p = 0.1. No children with BCNC stenosis developed hearing loss in their contralateral year by 12 months of follow-up.
Children with bony cochlear nerve canal stenosis may be at increased risk for progression in their ipsilateral ear. Audiometric and medical follow-up for these children should be considered.
探讨患侧骨性耳蜗神经管（BCNC）狭窄相关的单侧听力损失儿童各耳听力损失进展的风险。
三级儿科转诊中心。
从一个前瞻性听力学数据库中识别出经颞骨计算机断层扫描成像且进行了至少6个月随访听力测试的单侧听力损失患儿。
两名对患耳情况不知情的儿科放射科医生独立评估颞骨异常的影像学表现并测量骨性耳蜗管宽度。回顾所有可用的听力图，并记录气导阈值。
听力损失进展定义为气导纯音平均听阈升高10dB。
128名儿童符合纳入标准。其中，54名（42%）有颞骨异常，22名（17%）有患侧BCNC狭窄。在12个月时，患侧耳的进展率如下：无颞骨异常者为12%，有颞骨异常者为13%，有BCNC狭窄者为17%。与无狭窄的儿童相比，有BCNC狭窄的儿童患侧耳进展风险显著更高：风险比（HR）2.17，95%置信区间（CI）（1.01，4.66），P值0.046。当我们将有BCNC狭窄的儿童与颞骨成像正常的儿童进行比较时，发现有狭窄的儿童进展风险估计值几乎高出两倍，但这种差异未达到显著水平，HR 1.9，CI（0.8，4.3），P = 0.1。在12个月的随访中，没有BCNC狭窄的儿童对侧耳出现听力损失。
骨性耳蜗神经管狭窄的儿童患侧耳听力损失进展风险可能增加。应考虑对这些儿童进行听力测定和医学随访。
