Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Japan.
Department of Hearing Implant Sciences, Shinshu University School of Medicine, Matsumoto, Japan.
Acta Otolaryngol. 2021 Dec;141(12):1038-1043. doi: 10.1080/00016489.2021.2000638. Epub 2021 Nov 26.
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.
To investigate the clinical phenotype of cCMV-associated HL in the largest study to date.
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.
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.
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.
先天性巨细胞病毒相关性听力损失（cCMV 相关性 HL）是先天性或早发性耳聋的常见原因。虽然已经报道 cCMV 感染可导致各种类型的 HL，但 cCMV 相关性 HL 在很长一段时间内的自然病程尚不清楚。
在迄今为止最大的研究中，调查 cCMV 相关性 HL 的临床表型。
回顾性检查从干燥脐带中提取的 CMV DNA，诊断出 31 例 CMV 阳性儿童，将其分为单侧和双侧 HL 组，并使用纯音测听和听觉稳态反应随时间评估他们的听力能力。
13 名患者（41.9%）有单侧 HL，18 名患者（58.1%）有双侧 HL。在大多数单侧 cCMV 相关性 HL 病例中，听力较好的耳朵保持正常听力阈值。值得注意的是，在单侧和双侧 HL 的大多数病例中，听力较差的耳朵最终表现为重度至极重度 HL。
我们的研究结果表明，单侧和双侧 HL 以及所有病例中听力较好或较差的耳朵之间的 cCMV 相关性 HL 的自然病程不同。这些发现表明，准确的诊断可以使儿童的 cCMV 相关性 HL 得到适当的随访和管理。
