Contralateral hearing loss in children with a unilateral enlarged vestibular aqueduct,International Journal of Pediatric Otorhinolaryngology

当前位置： X-MOL 学术 › Int. J. Pediatr. Otorhinolaryngol. › 论文详情

Our official English website, www.x-mol.net, welcomes your feedback! (Note: you will need to create a separate account there.)

Contralateral hearing loss in children with a unilateral enlarged vestibular aqueduct
International Journal of Pediatric Otorhinolaryngology ( IF 1.5 ) Pub Date : 2021-08-19 , DOI: 10.1016/j.ijporl.2021.110891
E A van Beeck Calkoen ₁ , R J E Pennings ₂ , J Smits ₂ , S Pegge ₃ , L J C Rotteveel ₄ , P Merkus ₁ , B M Verbist ₅ , E Sanchez ₆ , E F Hensen ₇

Affiliation

Department of Otolaryngology-Head and Neck Surgery Ear & Hearing, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, the Netherlands; Amsterdam Public Health Research Institute, Amsterdam, the Netherlands; Center for Diagnostics in Sensorineural Hearing Loss (CDS), VU University Medical Center, Amsterdam, the Netherlands.
Department of Otolaryngology-Head and Neck Surgery, Section Hearing and Genes, Radboud University Medical Center, Nijmegen, the Netherlands; Donders Institute for Brain Cognition and Behavior, Radboud University Medical Center, Nijmegen, the Netherlands.
Department of Radiology, Radboud University Center, Nijmegen, the Netherlands.
Department of Otolaryngology-Head and Neck Surgery, Leiden University Medical Center, Leiden, the Netherlands.
Department of Radiology, Leiden University Medical Center, Leiden, the Netherlands.
Department of Radiology, VU University Medical Center, Amsterdam, the Netherlands.
Amsterdam Public Health Research Institute, Amsterdam, the Netherlands; Department of Otolaryngology-Head and Neck Surgery, Leiden University Medical Center, Leiden, the Netherlands.

Objective

To evaluate the long-term ipsi- and contralateral hearing of patients with a unilateral enlarged vestibular aqueduct (EVA).

Study design

Multicenter retrospective cohort study.

Setting

Three tertiary otology and audiology referral centers.

Patients and diagnostic interventions

A total of 34 children with a unilateral enlarged vestibular aqueduct as identified on CT and/or MR imaging were evaluated with pure tone and speech perception audiometry.

Mean outcome measures

Radiologic measurements of the vestibular aqueduct, ipsi- and contralateral hearing loss, ipsi- and contralateral hearing loss progression over time and DNA test results.

Results

All patients in this cohort with unilateral EVA presented with hearing loss. Hearing loss was progressive in 38% of the ipsilateral ears. In 29% of the children, hearing loss was also found in the contralateral ear without EVA. In 90%, the contralateral hearing was stable, with a mean follow up of 4.2 years. We found a significant correlation between the severity of the hearing loss and the size of the EVA. A genetic diagnosis associated with EVA and/or SNHL was found in only 7%.

Conclusion

About a third of the children with unilateral EVA are at risk of developing hearing loss in the contralateral ear. This indicates that at least in some patients with a unilateral EVA, a bilateral pathogenic process underlies the hearing loss, in contrary to what the imaging results suggest. These findings are important for counseling of EVA patients and their parents and have implications for follow up.

中文翻译：

单侧前庭导水管扩大的儿童对侧听力损失

客观的

评估单侧扩大前庭导水管 (EVA) 患者的长期同侧和对侧听力。

学习规划

多中心回顾性队列研究。

环境

三个三级耳科和听力学转诊中心。

患者和诊断干预

共有 34 名在 CT 和/或 MR 成像中发现的单侧前庭导水管扩大的儿童接受了纯音和言语感知听力测定法的评估。

平均结果测量

前庭导水管的放射学测量、同侧和对侧听力损失、同侧和对侧听力损失随时间的进展以及 DNA 测试结果。

结果

该队列中所有单侧 EVA 患者均出现听力损失。38% 的同侧耳朵听力丧失是进行性的。在 29% 的儿童中，在没有 EVA 的情况下，对侧耳朵也发现了听力损失。90% 的对侧听力稳定，平均随访 4.2 年。我们发现听力损失的严重程度与 EVA 的大小之间存在显着相关性。仅 7% 的人发现与 EVA 和/或 SNHL 相关的基因诊断。