Frequency and natural course of congenital cytomegalovirus-associated hearing loss in children

Abstract

Background

Congenital cytomegalovirus-associated hearing loss (cCMV-associated HL) is a common cause of congenital or early-onset deafness. Although cCMV infection has been reported to lead to various types of HL, the natural course of cCMV-associated HL over a long period is not yet known.

Objectives

To investigate the clinical phenotype of cCMV-associated HL in the largest study to date.

Methods

Thirty-one CMV-positive children, diagnosed by examining CMV DNA extracted from their dried umbilical cords retrospectively, were divided into unilateral and bilateral HL groups, and their hearing ability was evaluated using pure-tone audiometry and auditory steady-state response over time.

Results

Thirteen patients (41.9%) had unilateral HL and 18 (58.1%) had bilateral HL. In most cases of unilateral cCMV-associated HL, the ear with better hearing maintained a normal hearing threshold. Notably, in most cases of both unilateral and bilateral HL, the ear with worse hearing ultimately showed severe to profound HL.

Conclusion

Our findings revealed that the natural course of cCMV-associated HL was different between the cases of unilateral and bilateral HL, as well as between the ears with better or worse hearing in all cases. These findings indicate that accurate diagnosis could enable proper follow-up and management of cCMV-associated HL in children.

Chinese abstract

背景：先天性巨细胞病毒相关听力损失（cCMV 相关 HL）是一种常见的导致先天性或早发性耳聋的原因。尽管据报道, cCMV 感染会导致对于各种类型的 HL, cCMV 相关 HL的长期自然病程尚不清楚。

目的：在最大的研究中调查 cCMV 相关 HL 的临床表型。

方法：31 名 CMV 阳性儿童, 通过回顾性检查从干燥脐带中分离出的CMV染色体, 从而确诊。他们被分为单侧和双侧 HL 组, 并通过纯音测听法和听觉长期稳态响应接受听力能力评估。

结果：13 名患者 (41.9%) 患有单侧 HL, 18 名 (58.1%) 患有双侧 HL。大多数单侧 cCMV 相关的 HL患者, 听力较好的耳朵保持正常的听力阈值。值得注意的是, 大多数单侧和双侧 HL 患者, 听力较差的耳朵表现为重度至深度 HL。

结论：我们的研究结果表明, cCMV 相关 HL 的自然病程在单侧HL和双侧HL病例之间是不同的, 以及在所有病例中好耳朵和坏耳朵之间也是不同的。这些发现表明, 准确的诊断可以实现对 cCMV 相关 HL儿童进行合适的随访和管理。

Keywords:

• Congenital cytomegalovirus infection
• hearing loss
• dried umbilical cord
• newborn hearing screening
• natural course

Acknowledgments

This research was funded by a Health and Labor Sciences Research Grant for Research on Rare and Intractable diseases and Comprehensive Research on Disability Health and Welfare from the Ministry of Health, Labor and Welfare of Japan (S. U.: H26-Nanchitou(Nan)-Ippan-032, H29-Nanchitou(Nan)-Ippan-031, 20FC1048), a Grant-in-Aid from Japan Agency for Medical Research and Development (AMED) (S. U.: 16dk0310067h0001, 18ek0109363h0001, and 19dk0310097h0001), JSPS KAKENHI Grant no. JP 15H02565 (S. U.).

Disclosure statement

The authors declare no conflicts of interest.