Clinical characteristics and inner ear MR features in children with absent auditory brainstem response
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摘要:
目的 探讨听性脑干诱发反应(ABR)-V波消失的双耳极重度听力损失患儿的临床特征及内耳MR特征。 方法 回顾性收集2018年7月~2021年5月先天性感音神经性听力损失双侧ABR-V波消失患儿，其中男58例，女41例，共99例190耳。所有患者均已行内耳磁共振成像评估，同时收集到了部分患儿的围产期病史：早产、高胆红素血症、出生低体质量、出生窒息史、外耳畸形、先兆流产、耳聋家族史、孕期病毒感染。 结果 在收集到有围产期病史的67例患儿中，出生时高胆红素血症的病史24例(35.8%)；出生低体质量病史12例(17.9%)，早产11例(16.4%)，先兆流产10例(14.9%)，孕期病毒感染10例(14.9%)，出生窒息史5例(7.5%)，耳聋家族史4例(6%)，外耳畸形1例(1.5%)。性别、年龄、围产期高危因素和内耳畸形无相关性(P>0.05)。在190例患耳中，畸形的耳数为43例，总体比例为22.6%，其中单发畸形17例(8.9%)，两种及两种畸形多发的为26耳(13.7%)，在43例畸形耳中，总共出现了畸形次数为81次：蜗神经发育不良有35耳，占所有耳数的18.4%，半规管发育不良20耳(10.5%)，耳蜗异常12耳(6.3%)，大前庭导水管11耳(5.8%)，前庭畸形3耳1.5%。半规管异常与蜗神经发育不良(P < 0.05)和耳蜗畸形成正相关(P < 0.01)。 结论 不同的内耳畸形类型其造成听力损失的原理可能会不同，干预的方式也会存在差别，在需要植入耳蜗的患儿中，全面评估内耳的情况，对于预后有重要指导意义 -
关键词:
- 双耳极重度感音神经性听力损失 /
- 听性脑干诱发电位缺失 /
- 内耳畸形 /
- 磁共振成像
Abstract:Objective To analyze the clinical characteristics and inner ear MR features in children with absent auditory brainstem response (ABR). Methods Children in the otolaryngology department with extremely severe sensorineural deafness between July 2018 to May 2021 were included. Each of these children had shown no recordable ABR to presented at maximum levels (100 dB). There were 58 males and 41 females, a total of 99 cases with 190 ears. All patients had been evaluated with magnetic resonance imaging of the inner ear, and a perinatal history of some of the children were collected: prematurity, hyperbilirubinemia, low birth weight, history of birth asphyxia, external ear malformation, preterm abortion, family history of deafness, and viral infection during pregnancy. Results A total of the 67 children with perinatal history were included. There were 24 children with a history of hyperbilirubinemia (35.8%); 12 children with a history of low birth weight (17.9%), 11 children with a history of preterm birth(16.4%), 10 children with a history of preterm abortion(14.9%), 10 children with a history of viral infection during pregnancy (14.9%), 5 children with a history of birth asphyxia (7.5%), 4 children with a family history of deafness(6%), and 1 child with a history of external ear malformation(1.5%). The association between gender, age, perinatal risk factors, and inner ear malformations were not significant. Inner ear malformations were found in 43 of 192 ears (22.6%), and Only 17 (8.9%) ears had single anomaly; others (13.7%) had multiple anomalies. In the 43 deformed ears, 81 deformities were found: 35 ears with cochlear nerve dysplasia (18.4%), 20 ears with semicircular canal dysplasia(10.5%), 12 ears with cochlear anomalies(6.3%), 11 ears with large vestibular aqueducts(5.8%), and 3 ears with vestibular malformations (1.5%). A significant correlation was foud between semicircular canal anomalies and cochlear dysplasia (P < 0.05) and cochlear malformations (P < 0.01). Conclusion Children with hearing loss may have various etiologies and should be actively sought out for possible causes to select the best treatment option and improve prognosis. -
图 1 内耳畸形的MRI表现
A: 显示扩张的耳蜗和前庭形成共腔; B: 大前庭导水管导致内淋巴囊扩大; C: 蜗神经发育不良(箭头所指); D: 呈囊状畸形的外侧半规管(箭头所指).
Figure 1. MRI scan of inner ear malformation
表 1 患者的临床特征
Table 1. Clinical characteristics of patients
临床特征 n(%) 内耳无畸形 内耳畸形 P 年龄(月)* 12(9.0, 30.2) 11.5(9.0, 23.0) 22.5(10.5, 39.0) 0.06 性别 0.53 男 57(58.2) 57 46 女 41(41.8) 41 30 早产 11(16.4) 9 2 0.19 出生低体质量 12(17.9) 9 3 0.51 出生窒息史 5(7.5) 5 0 0.15 外耳畸形 1(1.5) 0 1 0.37 先兆流产 10(14.9) 6 4 1.00 耳聋家族史 4(6.0) 1 3 0.14 孕期病毒感染 10(14.9) 8 2 0.30 出生黄疸史 24(35.8) 15 9 1.00 *以中位数(四分位间距)表示. 
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表 2 内耳畸形发生率
Table 2. Incidence of inner ear malformation
畸形类型 例数 占比(%) 总体畸形耳 43 22.6 单发畸形耳 17 8.9 多发畸形耳 26 13.7 蜗神经发育不良 35 18.4 大前庭导水管 11 5.8 半规管 20 10.5 迷路异常 12 6.3 前庭异常 3 1.5 畸形合计 81 -
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表 3 内耳畸形之间的相关性
Table 3. Correlations between semicircular canal malformations and cochlear nerve dysplasia, abnormal cochlea, Vestibular malformations and abnormal vestibular aqueduct (n)
畸形类型 半规管正常 半规管异常 P 蜗神经 0.04 正常 12 3 发育不良 16 17 耳蜗 < 0.01 正常 26 10 异常 2 10 前庭 0.38 正常 27 18 异常 1 2 前庭导水管 0.91 正常 22 16 异常 6 4
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