■与没有前庭损害的儿童相比,严重听力损失(HL)和前庭损害的儿童的人工耳蜗植入结果更差。然而,人工耳蜗植入的决定很少基于前庭功能评估作为听力学测试的补充.
■根据HL起源确定前庭损伤的患病率,并评估患有严重HL的儿童前庭损伤与尿后运动发育延迟之间的关系。
■这项队列研究是在巴黎一家人工耳蜗的儿科转诊中心进行的,法国,使用HL来源的医疗记录数据,前庭评估,和发展里程碑成就的时代。该队列包括患有严重HL(HL损失>90dB)的儿童,他们在2009年1月1日至2019年12月31日期间在人工耳蜗植入之前完成了前庭评估。数据分析在2023年1月至6月之间进行。
■主要结果是根据HL起源的前庭损害的患病率。根据对前庭测试的反应将儿童分为3组:正常前庭功能(NVF),前庭功能部分受损(PVF),双侧前庭完全丢失(CBVL)。进行了广义logit模型来评估前庭损伤与HL的原因以及后运动发育延迟之间的关联。
■共包括592名儿童(308名男性[52.0%];平均[SD]年龄,38[34]个月)。在有记录的HL起源的儿童中(n=266),45.1%(120)患有遗传起源的HL,其中50.0%为综合征(主要是Usher和Waardenburg综合征),50.0%为非综合征(主要与连接蛋白26有关)。在具有传染性HL起源的患者中(n=74),70.3%(52例)患有巨细胞病毒(CMV)感染。在44.4%(592例中的263)的儿童中发现前庭损伤;在88.9%(526)的病例中大部分是对称的,在5.7%(34)的病例中为CBVL。78.3%(47)的遗传综合征HL患儿存在前庭损害(56.7%[34]伴PVF;21.7%[13]伴CBVL),69.2%(36)的CMV感染患儿存在前庭损害(57.7%[30]伴PVF;11.5%[6]伴CBVL)。与其他HL原因相比,发现遗传综合征HL起源与PVF和CBVL更常见。4个发展里程碑延迟的可能性(持头,坐着,站在支持下,和独立行走)在PVF和CBVL中均较高(例如,头部保持赔率比:2.55和4.79)与NVF相比,CBVL中实现这些里程碑的年龄高于PVF(例如,头部持有:7.33年vs4.03年;P<.001)。所有4个发育里程碑均与前庭损伤程度相关。
■这项队列研究发现,在患有严重HL的儿童中,前庭损害很普遍,根据HL起源而变化,并与运动后发育相关;而所有发育里程碑均与前庭功能损害严重程度相关,并非所有HL原因均与前庭功能损害严重程度相关.患有严重HL的儿童可能会在人工耳蜗植入前从完整的前庭评估中受益,这将支持早期和适应性管理,如CBVL的物理治疗和人工耳蜗植入策略。
UNASSIGNED: Children with profound hearing loss (HL) and vestibular impairment have worse cochlear implant outcomes compared with those without vestibular impairment. However, the decision for cochlear implantation is rarely based on vestibular function assessment as a complement to audiologic testing.
UNASSIGNED: To identify the prevalence of vestibular impairment according to HL origin and to assess the association between vestibular impairment and delayed posturomotor development in children with profound HL.
UNASSIGNED: This cohort study was conducted in a pediatric referral center for cochlear implantation in Paris, France, using medical records data on HL origin, vestibular assessment, and ages of developmental milestone achievement. The cohort included children with profound HL (loss >90 dB HL) who completed vestibular assessment prior to cochlear implantation between January 1, 2009, and December 31, 2019. Data analyses were conducted between January and June 2023.
UNASSIGNED: The primary outcome was prevalence of vestibular impairment according to HL origin. Children were classified into 3 groups according to their responses to vestibular testing: normal vestibular function (NVF), partially impaired vestibular function (PVF), and complete bilateral vestibular loss (CBVL). Generalized logit models were performed to evaluate the association between vestibular impairment and causes of HL as well as posturomotor development delay.
UNASSIGNED: A total of 592 children were included (308 males [52.0%]; mean [SD] age, 38 [34] months). In children with documented HL origin (n = 266), 45.1% (120) had HL with genetic origin, 50.0% of which were syndromic (mainly Usher and Waardenburg syndromes) and 50.0% were nonsyndromic (mainly associated with connexin 26). Among patients with infectious HL origin (n = 74), 70.3% (52) had cytomegalovirus (CMV) infection. Vestibular impairment was found in 44.4% (263 of 592) of the children; it was mostly symmetrical in 88.9% (526) and was CBVL in 5.7% (34) of the cases. Vestibular impairment was present in 78.3% (47) of children with genetic syndromic HL (56.7% [34] with PVF; 21.7% [13] with CBVL) and in 69.2% (36) of children with CMV infection (57.7% [30] with PVF; 11.5% [6] with CBVL). Genetic syndromic HL origin was found to be more often associated with both PVF and CBVL than other HL causes. The odds of having delays in 4 developmental milestones (head holding, sitting, standing with support, and independent walking) were higher in both PVF and CBVL (eg, head-holding odds ratios: 2.55 and 4.79) compared with NVF, and the age of achieving these milestones was higher in CBVL than PVF (eg, head holding: 7.33 vs 4.03 years; P < .001). All 4 developmental milestones were associated with the degree of vestibular impairment.
UNASSIGNED: This cohort study found that among children with profound HL, vestibular impairment was prevalent, varied according to HL origin, and associated with posturomotor development; while all developmental milestones were associated with vestibular impairment severity, not all HL causes were associated with vestibular impairment severity. Children with profound HL may benefit from complete vestibular assessment before cochlear implantation, which would support early and adapted management, such as physical therapy for CBVL and cochlear implantation strategy.