Department of Head and Skin, Ghent University, Ghent, Belgium.
Department of Rehabilitation Sciences, Ghent University, Ghent, Belgium.
Ear Hear. 2021 Jan/Feb;42(1):76-86. doi: 10.1097/AUD.0000000000000904.
Congenital cytomegalovirus (cCMV) infection is the most common nongenetic cause of sensorineural hearing loss in children. Due to the close anatomical relationship between the auditory and the vestibular sensory organs, cCMV can also be an important cause of vestibular loss. However, the prevalence and nature of cCMV-induced vestibular impairment is still underexplored. The aim of this study was to investigate the occurrence and characteristics of vestibular loss in a large group of cCMV-infected children, representative of the overall cCMV-population.
Ninety-three children (41 boys, 52 girls) with a confirmed diagnosis of cCMV were enrolled in this prospective longitudinal study. They were born at the Ghent University Hospital or referred from another hospital for multidisciplinary follow-up in the context of cCMV. The test protocol consisted of regular vestibular follow-up around the ages of 6 months, 1 year, 2 years, and 3 years with the video Head Impulse Test, the rotatory test, and the cervical Vestibular Evoked Myogenic Potential test.
On average, the 93 patients (52 asymptomatic, 41 symptomatic) were followed for 10.2 months (SD: 10.1 mo) and had 2.2 examinations (SD: 1.1). Seventeen (18%) patients had sensorineural hearing loss (7 unilateral, 10 bilateral). Vestibular loss was detected in 13 (14%) patients (7 unilateral, 6 bilateral). There was a significant association between the occurrence of hearing loss and the presence of vestibular loss (p < 0.001), with 59% (10/17) vestibular losses in the group of hearing-impaired children compared to 4% (3/76) in the group of normal-hearing subjects. In the majority of the cases with a vestibular dysfunction (85%, 11/13), both the semicircular canal system and the otolith system were affected. The remaining subjects (15%, 2/13) had an isolated semicircular canal dysfunction. Sixty-one patients already had at least one follow-up examination. Deterioration of the vestibular function was detected in 6 of them (10%, 6/61).
cCMV can impair not only the auditory but also the vestibular function. Similar to the hearing loss, vestibular loss in cCMV can be highly variable. It can be unilateral or bilateral, limited or extensive, stable or progressive, and early or delayed in onset. As the vestibular function can deteriorate over time and even normal-hearing subjects can be affected, vestibular evaluation should be part of the standard otolaryngology follow-up in all children with cCMV.
先天性巨细胞病毒（cCMV）感染是儿童感音神经性听力损失的最常见非遗传原因。由于听觉和前庭感觉器官的解剖关系密切，cCMV 也可能是前庭功能丧失的重要原因。然而，cCMV 引起的前庭损伤的患病率和性质仍未得到充分探索。本研究的目的是调查在一大组代表 cCMV 人群的 cCMV 感染儿童中发生和特征性的前庭功能丧失。
93 名（41 名男孩，52 名女孩）确诊为 cCMV 的儿童参加了这项前瞻性纵向研究。他们出生在根特大学医院，或因 cCMV 而从另一家医院转来进行多学科随访。测试方案包括在 6 个月、1 岁、2 岁和 3 岁左右定期进行前庭随访，使用视频头脉冲试验、旋转试验和颈性前庭诱发肌源性电位试验。
93 名患者（52 名无症状，41 名有症状）平均随访 10.2 个月（标准差：10.1 mo），接受了 2.2 次检查（标准差：1.1）。17 名（18%）患者有感觉神经性听力损失（7 名单侧，10 名双侧）。13 名（14%）患者检测到前庭功能丧失（7 名单侧，6 名双侧）。听力损失的发生与前庭功能丧失有显著相关性（p<0.001），在听力受损儿童组中，有 59%（10/17）出现前庭功能丧失，而在听力正常组中，这一比例为 4%（3/76）。在大多数（85%，11/13）有前庭功能障碍的病例中，半规管系统和耳石系统均受到影响。其余（15%，2/13）患者为单纯半规管功能障碍。61 名患者已经至少进行了一次随访检查。其中 6 名（10%，6/61）检测到前庭功能恶化。
cCMV 不仅会损害听觉，还会损害前庭功能。与听力损失类似，cCMV 引起的前庭功能丧失也可能高度变化。它可以是单侧或双侧，局限性或广泛性，稳定性或进行性，以及早期或延迟发作。由于前庭功能可能随时间恶化，甚至听力正常的患者也可能受到影响，因此，所有 cCMV 患儿的标准耳鼻喉科随访都应包括前庭评估。
