Etiology of single-sided deafness and asymmetrical hearing loss

Abstract

Conclusions: The present study revealed that various etiologies are involved in single-sided deafness (SSD), and that the cause of SSD and asymmetrical hearing loss (AHL) differed greatly between congenital/early-onset cases and adult cases. Clarification of the etiology is the first step toward providing appropriate intervention.

Objectives: The study aimed to clarify the etiology of SSD and AHL patients.

Methods: The etiology of a total of 527 SSD or AHL patients who visited Shinshu University Hospital between 2006 and 2016 were analyzed by imaging as well as serological tests for mumps virus, and CMV DNA testing.

Results: In our cohort of congenital/early-onset SSD (n = 210), the most prevalent cause in children was cochlear nerve deficiency (43.7%; 87 of 199 patients undergoing CT and/or MRI), followed by CMV infection, mumps infection, anomalies of the inner ear, ANSD, and other rare etiologies. In contrast, half of the adult SSD patients presented with idiopathic sensorineural hearing loss, followed by various types of otitis media, cerebellopontine angle tumor and other rare etiologies.

Chinese abstract

结论: 本研究显示, 有各种病因可涉及单侧聋 (SSD) , SSD和不对称听力损失 (AHL) 的原因在先天性/早发性病例和成人病例之间有很大差异。澄清病因是提供适当干预的第一步。

目的: 本研究旨在澄清SSD和AHL患者的病因。

方法: 采用腮腺炎病毒的成像和血清学检测以及CMV DNA检测对在2006年至2016年期间赴Shinshu大学附属医院看病的527例SSD或AHL患者进行了病因学分析。

结果: 在先天性/早发性SSD (n = 210) 病例中, 对于儿童来说, 最常见的原因是耳蜗神经缺乏 (43.7％; 199例中的87例接受了CT和/或MRI) , 其次是CMV感染、内耳异常、ANSD和其它罕见病因。相比之下, 半数成年SSD患者呈现特发性感觉神经性听力损失, 其次是各种类型的中耳炎、小脑蝶碱角瘤和其它罕见的病因。

Keywords:

• Single-sided deafness
• asymmetrical hearing loss
• cochlear nerve deficiency
• cytomegalovirus
• mumps
• inner ear anomaly
• auditory neuropathy spectrum disorder
• idiopathic sensorineural hearing loss
• cochlear implant

Acknowledgements

This study was supported by a Health and Labour Sciences Research Grant for Comprehensive Research on Disability Health and Welfare from the Ministry of Health, Labour and Welfare, Japan (http://www.mhlw.go.jp/english/) (S.U.).

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article. The Shinshu University Conflict of Interest Committee also approved the study.

Additional information

Funding

This study was supported by a Health and Labour Sciences Research Grant for Comprehensive Research on Disability Health and Welfare from the Ministry of Health, Labour and Welfare, Japan (http://www.mhlw.go.jp/english/) (S.U.).