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先天性内耳畸形患者脑脊液耳漏和鼻漏的罕见病例

Rare Cases of Cerebrospinal Fluid Oto-Rhinorrhoea in Patients with Congenital Inner Ear Malformations.

作者信息

Agarwal Priyanshi, Ramalingam W V B S, Ramesh A V, Sood Neha, Manzoor Jameel, Sekhri Kanika

机构信息

Department of ENT and Head and Neck Surgery, BLK Super Speciality Hospital, New Delhi, India.

Department of Radiology, BLK Super Speciality Hospital, New Delhi, India.

出版信息

Indian J Otolaryngol Head Neck Surg. 2023 Dec;75(4):4101-4105. doi: 10.1007/s12070-023-04098-1. Epub 2023 Jul 21.

Abstract

Spontaneous cerebrospinal fluid (CSF) oto-rhinorrhoea is rare and may develop secondary to inner ear malformation. Any child discharging watery fluid through nose or ear spontaneously in a head dependent position should be a high index of suspicion of CSF leak. If watery rhinorrhoea is present then apart from biochemical analysis of fluid discharge to confirm it to be CSF, computed tomography of paranasal sinuses and temporal bone should be carried out to differentiate between CSF oto-rhinorrhoea and CSF rhinorrhoea. Congenital deformities of the inner ear can be associated with meningitis and varying degrees of hearing loss. Here we describe two cases, one of CSF oto-rhinorrhea in a 1 year old child who presented with spontaneous watery rhinorrhoea following violent projectile vomiting of 1 month duration. On evaluation, child was found to have CSF oto-rhinorrhoea with right Mondini deformity and profound hearing loss on right side. 2nd case was of 12 years old male with profound hearing loss right ear and recurrent episodes of meningitis diagnosed as common cavity malformation of inner ear. Both children underwent closure of CSF leak from oval window successfully. There was no recurrence after more than 1 year of follow up. Congenital inner ear malformations are an important cause of recurrent meningitis in children and require a high index of suspicion for diagnosis. Thorough clinical evaluation and radiological study is suggested in all cases of CSF oto-rhinorrhoea for the identification inner ear malformation and management of CSF oto-rhinorrhoea. Patients who receive an accurate and early diagnosis can avoid severe complications and have a good prognosis.

摘要

自发性脑脊液耳鼻漏较为罕见,可能继发于内耳畸形。任何在头低位时自发经鼻或耳排出水样液体的儿童,都应高度怀疑存在脑脊液漏。如果出现水样鼻漏,除了对流出液体进行生化分析以确认其为脑脊液外,还应进行鼻窦和颞骨的计算机断层扫描,以区分脑脊液耳鼻漏和脑脊液鼻漏。内耳先天性畸形可伴有脑膜炎和不同程度的听力损失。在此,我们描述两例病例,一例为1岁儿童的脑脊液耳鼻漏,该患儿在持续1个月的剧烈喷射性呕吐后出现自发水样鼻漏。经评估,患儿被诊断为脑脊液耳鼻漏,伴有右侧Mondini畸形和右侧重度听力损失。第二例为一名12岁男性,右耳重度听力损失,反复发作脑膜炎,被诊断为内耳共同腔畸形。两名患儿均成功进行了椭圆窗脑脊液漏修补术。随访1年以上均无复发。先天性内耳畸形是儿童反复发生脑膜炎的重要原因,诊断时需要高度怀疑。对于所有脑脊液耳鼻漏病例,建议进行全面的临床评估和影像学检查,以识别内耳畸形并处理脑脊液耳鼻漏。获得准确早期诊断的患者可避免严重并发症,预后良好。

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本文引用的文献

1
Recurrent Meningitis in Congenital Inner Ear Malformation.先天性内耳畸形的复发性脑膜炎。
Ear Nose Throat J. 2021 Feb;100(1_suppl):38S-41S. doi: 10.1177/0145561320920399. Epub 2020 Apr 22.
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Acta Otorrinolaringol Esp. 2005 Jun-Jul;56(6):273-9. doi: 10.1016/s0001-6519(05)78613-1.
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Classification of spontaneous cerebrospinal fluid middle ear effusion: review of forty-nine cases.
Otolaryngol Head Neck Surg. 1985 Oct;93(5):625-34. doi: 10.1177/019459988509300511.

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