Etiology of hearing loss affects auditory skill development and vocabulary development in pediatric cochlear implantation cases

Abstract

Background

Cochlear implantation (CI) is an effective treatment for severe-to-profound hearing loss patients and is currently used as the standard therapeutic option worldwide. However, the outcomes of CI vary among patients.

Aims/Objectives

This study aimed to clarify the clinical features for each etiological group as well as the effects of etiology on CI outcomes.

Materials and methods

We collected clinical information for 308 pediatric cochlear implant cases, including the etiology, hearing thresholds, age at CI, early auditory skill development, total development, monosyllable perception, speech intelligibility and vocabulary development in school age, and compared them for each etiology group.

Results

Among the 308 CI children registered for this survey, the most common etiology of hearing loss was genetic causes. The genetic etiology group showed the most favorable development after CI followed by the unknown etiology group, syndromic hearing loss group, congenital CMV infection group, inner ear malformation group, and cochlear nerve deficiency group.

Conclusions and significance

Our results clearly indicated that the etiology of HL affects not only early auditory skill development, but also vocabulary development in school age. The results of the present study will aid in more appropriate CI outcome assessment and in more appropriate intervention or habilitation programs.

Chinese Abstract

背景：人工耳蜗植入（CI）是治疗严重至重度听力损失患者的有效方法, 目前被用作世界范围内的标准治疗选择。然而, CI结果因患者而异。

目的：本研究旨在阐明每个病因组的临床特征以及病因对 CI 结果的影响。

材料与方法：我们收集了 308 例儿童人工耳蜗病例的临床资料, 包括病因、听力阈值、CI 年龄、早期听觉技能发展、全面发展、单音节感知、语言清晰度和学龄词汇发展, 并且对每个病因组进行比较。

结果：在登记参加本次调查的 308 名 CI 儿童中, 最常见的听力损失病因是遗传因素。遗传病因组在CI后表现最佳, 往后依次是病因不明组、综合征性听力损失组、先天性巨细胞病毒感染组、内耳畸形组、耳蜗神经缺损组。

结论和意义：我们的结果清楚地表明, 听力损失的病因不仅影响早期听觉技能的发展, 还影响学龄期词汇的发展。本研究的结果将有助于更合适的 CI 结果评估和更合适的干预或康复计划。

Keywords:

• Cochlear implantation
• hearing loss
• etiology
• genetic testing
• congenital cytomegalovirus infection
• inner ear malformation
• auditory behavior development
• vocabulary development

Disclosure statement

No potential conflict of interest was reported by the author(s).

Correction Statement

This article has been republished with minor changes. These changes do not impact the academic content of the article.

Additional information

Funding

This study was supported by a Grant-in-Aid from the Japan Agency for Medical Research and Development (AMED) [S.U. 16kk0205010h001, 15ek0109114h001].