Risk factors and etiology of childhood hearing loss: a cohort review of 296 subjects

Abstract

Background: The association between the Joint Committee on Infant Hearing (JCIH) risk factors and etiology of hearing loss (HL) is not studied well in children.

Objectives: To clarify the etiologic causes and evaluate the JCIH risk characteristics of children with HL.

Methods: A retrospective study of 296 children with HL born between 2009.01 and 2013.12 in Stokholm. Demographic data, family and medical histories, audiologic results, imaging findings, and genetic results were ascertained and analyzed.

Results: In 221 with bilateral hearing loss (BHL), family history and neonatal risk indicators were the most common risks (59 each), followed by syndrome related risks. In 75 with unilateral hearing loss (UHL), craniofacial anomaly was the most common risk, followed by family history. Etiology was established in 93 with BHL, in which syndromic HL accounted for 37.2%, chromosomal aberrations for 21.3%, and environmental causes for 19.1%. Etiology was established in 35 with UHL, in which ear malformation accounted for the most (74.3%), followed by environmental causes (14.3%).

Conclusions and significance: Childhood HL can be attributed to a variety of causes with an etiology identifiable in 42.5% of BHL and 46.7% of UHL. BHL and UHL have different patterns of JCIH risk exposure and etiology.

Chinese abstract

背景：婴幼儿听力联合委员会（JCIH）制定的危险因素与听力损失（HL）病因之间的关联尚未对儿童进行很好的研究。

目的：弄清HL儿童的病因并评估其JCIH风险特征。

方法：回顾性研究斯托克霍尔姆市2009年1月至2013年12月之间出生的296例HL儿童。确定并分析了人口统计学数据、家庭病史、听力学结果、影像学发现和遗传结果。

结果：在221名双侧听力损失（BHL）患者中, 家族史和新生儿风险标示是最常见的风险（每项59个）, 其次是与综合征相关的风险。在75例单侧听力损失（UHL）患者中, 颅面畸形是最常见的风险, 其次是家族史。在93例BHL中已建立病因, 其中综合征性HL占37.2％, 染色体畸变占21.3％, 环境原因占19.1％。 35例UHL以建立病因, 其中耳畸形最多（74.3％）, 其次是环境原因（14.3％）。

结论和意义：儿童期HL可归因于多种原因, 42.5％的BHL和46.7％的UHL已确定其病因。 BHL和UHL有不同的JCIH风险和病因模式。

Keywords:

• Hearing loss
• screening
• prevalence
• etiology

Author contributions

Dr Niu and Prof Duan conceptualized and designed the study, drafted the initial manuscript, and reviewed and revised the manuscript. Drs Brandström and Skenbäck designed the data collection instruments, collected data, carried out the initial analyses, and reviewed and revised the manuscript.

Dr Uhlén conceptualized and designed the study, coordinated and supervised data collection, and critically reviewed the manuscript for important intellectual content. All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.

Disclosure statement

The authors have no conflicts of interest relevant to this article to disclose.