Departments of Otolaryngology–Head and Neck Surgery, Virginia Commonwealth University Medical Center, Richmond, VA 23298, USA.
JAMA Otolaryngol Head Neck Surg. 2013 Jan;139(1):59-63. doi: 10.1001/jamaoto.2013.1097.
To analyze the presence of Joint Committee on Infant Hearing (JCIH) risk factors and co-occurring birth defects (CBDs) in children with unilateral hearing loss (UHL).
Retrospective review.
Statewide registry of universal newborn hearing screen data for all children born in Virginia from 2002 through 2008.
The study population comprised 371 children with confirmed UHL.
Universal newborn hearing screen status, presence or absence of JCIH risk factors, and CBDs
Of the 371 children with confirmed unilateral hearing loss, 362 (97.5%) were identified through a failed universal newborn hearing screen. Of these 362 children, 252 (69.6%) had no JCIH risk factors and 110 (30.3%) had 1 or more risk factor reported. Nine children (2.5%) with 1 or more risk factors passed the universal newborn hearing screen but had later-onset UHL. Craniofacial anomaly was the most commonly reported JCIH risk factor in 48 children (43.6%). A family history of permanent childhood hearing loss was present in 24 children (21.8%). Twenty children (18.2%) had stigmata associated with a syndrome including hearing loss. Of the 110 children with UHL and a JCIH risk factor, additional CBDs were identified in 83 (75.5%). An ear-specific anomaly was most prevalent in 37 infants (44.6%), followed by cardiovascular anomalies in 34 infants (41.0%).
Thirty percent of children with confirmed UHL had a JCIH risk factor, most commonly craniofacial anomalies, family history of hearing loss, and stigmata of syndromes associated with hearing loss. However, the absence of JCIH risk factors does not preclude the development of UHL. Further studies assessing the etiology of UHL and risk factor associations are warranted.
分析单侧听力损失（UHL）儿童中联合委员会婴儿听力（JCIH）风险因素和同时发生的出生缺陷（CBDs）的存在情况。
回顾性研究。
弗吉尼亚州所有 2002 年至 2008 年出生儿童的普遍新生儿听力筛查数据的全州注册处。
研究人群包括 371 例确诊单侧听力损失的儿童。
普遍新生儿听力筛查状态、JCIH 风险因素的存在或不存在以及 CBDs。
在 371 例确诊单侧听力损失的儿童中，362 例（97.5%）通过普遍新生儿听力筛查未通过而确定。在这 362 名儿童中，252 名（69.6%）无 JCIH 风险因素，110 名（30.3%）报告有 1 个或多个风险因素。9 名（2.5%）有 1 个或多个风险因素的儿童通过了普遍新生儿听力筛查，但后来出现了单侧听力损失。颅面畸形是 48 名儿童（43.6%）中最常报告的 JCIH 风险因素。24 名儿童（21.8%）有永久性儿童听力损失的家族史。20 名儿童（18.2%）有与综合征相关的听力损失体征。在 110 名有 JCIH 风险因素的单侧听力损失儿童中，有 83 名（75.5%）发现了其他 CBDs。耳部特异性异常在 37 名婴儿中最为常见（44.6%），其次是心血管异常在 34 名婴儿中（41.0%）。
30%确诊单侧听力损失的儿童存在 JCIH 风险因素，最常见的是颅面畸形、听力损失家族史和与听力损失相关综合征的体征。然而，不存在 JCIH 风险因素并不能排除单侧听力损失的发生。进一步研究评估单侧听力损失的病因和风险因素相关性是必要的。
