Congenital inner ear deformity with spontaneous cerebrospinal fluid leakage: a case series report of 9 children

Junhong CHEN; Ling DING; Ying JIANG; Shuping SU

doi:10.16016/j.2097-0927.202108213

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Congenital inner ear deformity with spontaneous cerebrospinal fluid leakage: a case series report of 9 children

Clinical Medicine | 更新时间：2026-01-09

- Congenital inner ear deformity with spontaneous cerebrospinal fluid leakage: a case series report of 9 children
- Journal of Army Medical University Vol. 44, Issue 5, Pages: 495-498(2022)
- 作者机构：
  
  400014 重庆，重庆医科大学附属儿童医院耳鼻咽喉科，国家儿童健康与疾病临床医学研究中心
- 作者简介：
- 基金信息：
- DOI：10.16016/j.2097-0927.202108213
  CLC： R726.511;R764.73
- Received：30 August 2021，
  
  Revised：2021-09-07，
  
  Published：15 March 2022
- 稿件说明：
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Junhong CHEN, Ling DING, Ying JIANG, et al. Congenital inner ear deformity with spontaneous cerebrospinal fluid leakage: a case series report of 9 children[J]. Journal of Army Medical University, 2022, 44(5): 495-498.
DOI：

Junhong CHEN, Ling DING, Ying JIANG, et al. Congenital inner ear deformity with spontaneous cerebrospinal fluid leakage: a case series report of 9 children[J]. Journal of Army Medical University, 2022, 44(5): 495-498. DOI： 10.16016/j.2097-0927.202108213.

摘要

目的

探讨儿童先天性内耳畸形伴自发性脑脊液耳漏的临床特征、诊断及治疗。

方法

收集我院2012—2021年9例先天性内耳畸形伴自发性脑脊液耳漏患儿的临床资料，回顾性总结本组病例的临床表现、辅助检查、治疗及预后等情况。

结果

9例患儿均为单侧发病，左耳1例，右耳8例，均伴发热。部分患儿伴头痛、呕吐、惊厥发作、耳溢液、耳痛等临床症状。听力检查均提示重度以上感音神经性耳聋，CT提示内听道底均可见骨质缺损。所有患儿并发细菌性脑膜炎，4例共同腔畸形患儿出现不同程度脑积水、惊厥持续状态、继发性癫痫、周围性面瘫等并发症。9例患儿均采用经乳突入路的手术方式行漏口修补。8例患儿一次手术成功，1例患儿因术后2个月复发行二次手术，所有患儿术后随访3月至2年均无复发。

结论

儿童先天性内耳畸形伴自发性脑脊液耳漏常因细菌性脑膜炎相关症状就诊而发现，及时行听力检查和颞骨高分辨CT有助于早期诊断；手术治疗是最有效的治疗方法。

Abstract

Objective

To investigate the clinical features

diagnosis and treatment of congenital inner ear deformity with spontaneous cerebrospinal fluid leakage in children.

Methods

Clinical data of 9 children suffering from inner ear deformity with spontaneous cerebrospinal fluid leakage treated in our hospital from 2012 to 2021 were collected retrospectively. The clinical manifestations

results of auxiliary examinations

surgical management and outcomes were analyzed.

Results

All the 9 children had unilateral disease

including 1 case in the left ear and 8 cases in the right ear

and were accompanied with fever. Some of them had the symptoms like headache

vomiting

convulsion

otorrhea and earache. Hearing examination indicated more severe sensorineural hearing loss

and CT scanning indicated bone defects in the bottom of the inner hear duct. All the children were complicated with bacterial meningitis

and 4 children with common cavity also had different severities of hydrocephalus

status convulsion secondary epilepsy and peripheral facial paralysis. All the 9 cases were repaired through mastoid approach. Eight children were successfully operated at the first time

and one underwent second operation 2 months after first surgery. All the children were followed up from 3 months to 2 years

and no recurrence was found.

Conclusion

Congenital inner ear deformity with spontaneous cerebrospinal fluid otorrhea is often found in children due to symptoms related to bacterial meningitis. Timely hearing examination and high resolution CT scanning of temporal bone are helpful for early diagnosis. Surgery is the most effective treatment.

关键词

Keywords

references

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