Cerebrospinal fluid otorrhea and pseudomonal meningitis in a child with Mondini dysplasia: case report
Cerebrospinal fluid otorrhea and pseudomonal meningitis in a child with Mondini dysplasia: case report
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DOI：
10.1007/s00381-015-2836-x
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发表时间：
2015-07
期刊：
影响因子：
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通讯作者：
R. N. Hernandez;Abhinav R. Changa;L. Bassani;R. Jyung;J. Liu
中科院分区：
文献类型：
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作者：
R. N. Hernandez;Abhinav R. Changa;L. Bassani;R. Jyung;J. Liu
目的蒙迪尼发育不良是一种罕见的先天性内耳畸形，表现为耳蜗发育异常，伴有前庭扩张和前庭导水管扩大。这种功能失调的解剖结构可能导致感音神经性听力缺陷、脑脊液漏和严重的复发性脑膜炎病例。方法我们介绍了一名患有蒙迪尼发育不良的儿童的病例，该儿童表现为单侧听力损失和脑脊液（CSF）耳漏，通过中耳窝/经耳道中耳联合入路进行手术修复，以减轻感染和脑脊液的复发结果术后，患者神经功能保持稳定，没有任何进一步的脑脊液漏出。脑脊液培养显示铜绿假单胞菌感染，这在蒙迪尼发育不良的情况下罕见发生。理论上，细菌从外耳道逆行扩散到脑脊液间隙是导致脑膜炎的可能发病机制。该患者经静脉注射抗生素成功治疗，没有出现任何神经系统并发症。结论虽然蒙迪尼发育不良是一种罕见的畸形，但由于解剖结构功能失调可能导致危及生命的脑膜炎后遗症，因此需要提高临床警惕性，特别是考虑到与复发性脑膜炎、耳漏或鼻漏相关的听力损失儿童。
PurposeMondini dysplasia is a rare congenital inner ear malformation that presents with abnormal cochlear development with accompanied vestibular dilation and vestibular aqueduct enlargement. This dysfunctional anatomy provides the potential for sensorineural hearing deficits, cerebrospinal fluid leaks, and severe cases of recurrent meningitis.MethodsWe present the case of a child with Mondini dysplasia who presented with unilateral hearing loss and cerebrospinal fluid (CSF) otorrhea that was surgically repaired through a combined middle fossa/transmeatal middle ear approach to alleviate any recurrence of infection and cerebrospinal fluid otorrhea.ResultsPostoperatively, the patient remained neurologically stable without any further CSF leakage. CSF cultures revealed aPseudomonas aeruginosainfection, a rare occurrence within the context of Mondini dysplasia. Retrograde bacterial spread from the external ear canal into the CSF space has been theorized as the possible pathogenesis of the resulting meningitis. The patient was successfully treated with intravenous antibiotics without any neurologic complications.ConclusionsAlthough Mondini dysplasia is a rare malformation, the life-threatening sequelae of meningitis that can result from the dysfunctional anatomy makes it a condition that requires elevated clinical vigilance, especially when considering children with hearing loss associated with recurrent meningitis, otorrhea, or rhinorrhea.