吉林大学学报(医学版)  2019, Vol. 45 Issue (06): 1427-1431     DOI: 10.13481/j.1671-587x.20190639

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刘涛, 姚静, 徐静, 姚孟薇, 冯亚茹, 张伟, 李晓瑜
LIU Tao, YAO Jing, XU Jing, YAO Mengwei, FENG Yaru, ZHANG Wei, LI Xiaoyu
单侧内耳Mondini畸形并发脑脊液耳漏1例报告及文献复习
Unilateral inner ear Mondini malformation complicated with cerebrospinal fluid ear leakage: A case report and literature review
吉林大学学报(医学版), 2019, 45(06): 1427-1431
Journal of Jilin University (Medicine Edition), 2019, 45(06): 1427-1431
10.13481/j.1671-587x.20190639

文章历史

收稿日期: 2018-11-18
单侧内耳Mondini畸形并发脑脊液耳漏1例报告及文献复习
刘涛1 , 姚静2 , 徐静3 , 姚孟薇1 , 冯亚茹1 , 张伟4 , 李晓瑜1     
1. 济宁医学院附属医院耳鼻喉科, 山东 济宁 272067;
2. 济宁医学院基础医学院, 山东 济宁 272067;
3. 济宁医学院附属医院妇产科, 山东 济宁 272067;
4. 河北省衡水市人民医院骨关节运动科, 河北 衡水 053000
[摘要]: 目的 探讨单侧内耳Mondini畸形并发脑脊液耳漏患者的发病机制、临床表现、诊断和手术方法,提高对其临床特征的认识。方法 收集1例单侧内耳Mondini畸形并发脑脊液耳漏患者的临床资料,结合国内外相关文献,分析其临床特征、影像学表现、诊断和手术方法。结果 患者因"左侧鼻腔流水、头痛20年,加重1个月"就诊。查体见左耳鼓膜内陷、完整,左耳听力下降。鼻内镜下,颅内压增加时左侧咽鼓管咽口有脑脊液流出。结合患者相关检查结果和临床表现初步考虑左耳Mondini畸形并脑脊液耳漏。行左脑脊液耳漏修补术,患者术后听力良好,无鼻腔流液和头痛不适。随访1年,患者未复发脑膜炎。结论 反复发作不明原因的脑膜炎患者应考虑先天性内耳畸形的可能。颞骨薄层CT与MRI检查是确诊的主要手段,鼓室探查修补术是填补漏口的有效方法,手术是主要的治疗方法。
关键词: 单侧内耳Mondini畸形    脑脊液耳漏    脑膜炎    鼓膜探查修补术    
Unilateral inner ear Mondini malformation complicated with cerebrospinal fluid ear leakage: A case report and literature review
LIU Tao1 , YAO Jing2 , XU Jing3 , YAO Mengwei1 , FENG Yaru1 , ZHANG Wei4 , LI Xiaoyu1     
1. Department of Otorhinolaryngology, Affiliated Hospital, Jining Medical University, Jining 272067, China;
2. School of Basic Medical Sciences, Jining Medical University, Jining 272067, China;
3. Department of Gynecology and Obstetrics, Affiliated Hospital, Jining Medical University, Jining 272067, China;
4. Department of Bone and Joint Movement, People's Hospital, Hengshui City, Hebei Province, Hengshui 053000, China
[ABSTRACT]: Objective To discuss the pathogenesis, clinical manifestation, diagnosis and operation methods of the patients with unilateral inner ear Mondini malformation complicated with cerebrospinal fluid ear leakage, and to improve the understanding of the clinical features. Methods The clinical materials of one patient diagnosed as unilateral inner ear Mondini malformation complicated with cerebrospinal fluid ear leakage were collected, and the clinical features, imageological performance and diagnosis and treatment methods were analyzed combined with the relevant literatures. Results Because of left nasal discharge, headache for 20 years, aggravation for 1 month, the patient went to our hospital. The physical examination results showed that the left tympanic membrane was invaginated and intact, and there was hearing loss in the left ear.The endoscopic examination results showed that when the intracranial pressure was increased, the cerebrospinal fluid flowed out from the left eustachian tube orifice. The Mondini malformation and cerebrospinal fluid otorrhea in the left ear were initially considered in combination with the clinical manifestations, and relative examination results of the patient. The left cerebrospinal fluid otorrhea repair was performed, the postoperative hearing was good, and there was no nasal flow, headache and discomfort. The patient was followed up for 1 year and there was no recurrence of meningitis. Conclusion The patient with recurrently unclear meningitis would be firstly considered as congenital malformation of inner ear. The diagnosis of the disease is mainly based on the examinations of temporal bone CT and MRI; the tymanic exploration and repair surgery might be an effective method, and operation is the main treatment method.
KEYWORDS: unilateral inner ear Mondini malformation    cerebrospinal fluid ear leakage    tymanic exploration and repair surgery    

脑脊液耳漏的原因是由于脑脊液循环系统的蛛网膜下腔与中耳相通,引起脑脊液流入中耳,耳鼓膜有破裂时溢液经外耳道流出,主要临床表现为耳内出现无色、无味、无黏性的清水样物[1-5]。一般情况下,自发性脑脊液耳漏非常罕见,可能是由先天性中耳内耳畸形、感染和肿瘤这些疾病引起,临床症状复杂且缺乏特异性,容易漏诊和误诊。常见的脑脊液耳漏多为继发性,主要可能由慢性中耳炎、耳及颅脑创伤或手术等引起[4, 6-7]。其中以Mondini畸形最为多见,该病多数情况下会影响耳蜗,主要表现为内耳骨迷路和膜迷路发育不全等症状,经常会出现前庭水管、半规管和耳蜗部位的病变[1, 3, 8-9]。患者主要表现为反复高热头痛,一般会就诊于儿科或内科,多不进行耳鼻喉专科检查以及后续检查,容易被忽视而漏诊。如果鼓膜完整,脑脊液可经耳咽管流向咽部,由鼻后孔返流到鼻腔再自鼻孔溢出,因此常将脑脊液耳鼻漏相互混淆[1-2]。临床上内耳Mondini畸形并发脑脊液耳漏容易误诊漏诊而延误治疗。虽然在以往的报道中已有相关的描述,但并不详尽,缺少对于相关诊断检查结果的回顾和分析。本文作者对1例Mondini畸形伴有脑脊液耳漏患者就诊和治疗的全部临床资料进行回顾和分析,包括患者的一般资料、辅助检查扫描成像结果图、诊断依据以及手术治疗经过,并结合国内外相关文献,总结该病的临床表现、影像学特点和治疗策略,重点分析如何防止该病的误诊和漏诊,以期为此类疾病的早期诊断和及时治疗提供依据。

1 临床资料 1.1 一般资料

患者,女性,49岁,主因“左侧鼻腔流水、头痛20年,加重1个月”就诊于济宁医学院附属医院耳鼻喉科。患者曾多次就诊于多家医院,每次考虑“鼻窦炎”“脑膜炎”“脑脊液鼻漏”等,给予抗感染治疗后症状缓解,但反复发作。入院后耳鼻喉专科检查结果显示:左耳鼓膜内陷、完整,标志欠清,左耳听力下降。鼻腔黏膜充血,鼻中隔略偏曲,未见异常新生物。尤为注意的是嘱患者低头用力时可见左侧鼻腔流出无色、透明、清亮的液体,同时按压颈部可见液体增加。鼻内镜下检查鼻腔各处,同时嘱咐患者屏气,并按压颈内静脉增加颅内压,见左侧咽鼓管咽口有脑脊液流出,其他部位未见脑脊液流出。行快速血糖试纸检查,结果提示左侧鼻腔流出的清亮液体即“脑脊液”。

1.2 辅助检查

鼻窦CT扫描(图 1)显示:右侧上颌窦内低密度,筛窦、额窦和蝶窦气化良好,鼻中隔稍偏曲,双下鼻甲黏膜稍肥厚。鼻咽部核磁共振(MRI)(图 2)显示:左颅底中线旁骨质信号欠连续、局部脑脊液间隙增宽,脑脊液鼻漏不能排除,建议进一步检查、左侧中耳乳突炎。纯音测听:右耳正常,左耳全聋。鼓室导抗图:左侧B型,右侧A型。中耳乳突CT检查结果显示:左侧内听道扩大、左侧耳发育不良(不完全分隔Ⅰ型)并前庭扩大及外侧半规管发育不良、左侧中耳乳突内低密度影(图 3)。中耳MRI+内耳水成像显示:左侧前庭和耳蜗形态异常,耳蜗及前庭明显扩大,半规管形态未见异常,右侧乳突区见T2WI高信号。右侧半规管、前庭及耳蜗形态、信号未见异常。结合中耳乳突CT+中耳MRI检查结果判断患者左耳听力下降的原因为左耳蜗骨迷路发育畸形(Mondini畸形)(图 4,见插页七)。入院诊断:左耳Mondini畸形并脑脊液耳漏。

A: CT axial view; B: CT coronal view. 图 1 单侧内耳Mondini畸形并发脑脊液耳漏患者术前鼻窦CT图像 Fig. 1 CT images of sinuses of patient with unilateral inner ear Mondini malformation complicated with cerebrospinal fluid ear leakage before operation
A: MRI axial view; B: MRI coronal view. 图 2 单侧内耳Mondini畸形并发脑脊液耳漏患者术前鼻咽部MRI图像 Fig. 2 MRI photoes of nasopharynx of patient with unilateral inner ear Mondini malformation complicated with cerebrospinal fluid ear leakage before operation
A: CT axial view (aures unitas); B: CT axial view (left ear); C: CT coronal view. 图 3 单侧内耳Mondini畸形并发脑脊液耳漏患者术前中耳乳突CT图像 Fig. 3 CT images of middle ear mastoid of patient with unilateral inner ear M ondini malformation complicated with cerebrospinal fluid ear leakage before operation
图 4 单侧内耳Mondini畸形并发脑脊液耳漏患者术前中耳MRI和内耳水成像图 Fig. 4 Middle ear MRI and inner ear water imaging of patient with unilateral inner ear Mondini malformation complicated with cerebrospinal fluid car leakage before operation
1.3 治疗方法

全麻下行“左脑脊液耳漏修补术”,术中探查见鼓室天盖骨质缺损,硬脑膜暴露,但未见脑脊液耳漏。脑脊液由鼓室和鼓窦流出,沿脑脊液流出方向继续追踪,暴露锤砧关节,见脑脊液由听骨下流出,因听骨遮挡术野,去除砧骨及锤骨头,见前庭窗脑脊液流出(图 5,见插页七);先以明胶海绵填充,后用肌瓣填塞漏口,见脑脊液漏停止(图 6,见插页七);颞肌肌瓣填塞咽鼓管和乳突腔,对位缝合耳后切口。手术结束。术后患者恢复良好,术后第1天偶有眩晕,给予对症处理后眩晕明显好转;术后第2天眩晕好转,恢复良好,无头痛不适;术后第7天拆除耳部缝线,愈合良好,无鼻腔流液和头痛不适。病例随访1年,未再出现脑膜炎复发。

Yellow arrow referred to site of CSF leakage. 图 5 单侧内耳Mondini畸形并发脑脊液耳漏患者手术治疗录像截图 Fig. 5 Video image of patient with surgical treatment for unilateral inner ear Mondini malformation complicated with cerebrospinal fluid ear leakage
White object referred to gelatin sponge. 图 6 单侧内耳Mondini畸形并发脑脊液耳漏患者手术治疗录像截图 Fig. 6 Video image of patient with surgical treatment for unilateral inner ear Monnidi malformation complicated with cerebrospinal fluid ear leakage
2 讨论

先天性内耳畸形是内耳胚胎阶段发育不良引发的内耳结构发生异常。Mondini畸形是一种常见的先天性内耳发育畸形,绝大多数情况下会影响耳蜗[10]。Mondini畸形临床常见的症状是听力严重损伤致重度听力下降或者直接引发耳聋等表现。有些患者出生时即丧失听力,有些患者则在成年期仍有残余听力。单侧的畸形会因为对侧听力正常而被忽略。一般当患者出现反复脑膜炎时才引起注意而就诊。本例患者即出现反复脑膜炎而未进一步明确病因,导致反复发病。Mondini畸形脑脊液耳漏通常情况下会首先发生脑膜炎,同时也会出现耳闷塞感、耳溢液等症状,脑脊液经漏口沿着咽鼓管流入鼻腔则表现为水样涕。Mondini畸形在影像学观察中发现耳蜗底周已经发育完全,但第2周和顶周表现为发育不全,同时有耳蜗偏小和(或)空囊状耳蜗,在发生脑脊液耳漏时乳突腔内可见积液[11]。由此可知,如果该病能得到正确的诊断可直接提高治疗的效果。多次反复发作的脑膜炎患者在诊断时需要详细询问病史,在排除其他可疑疾病同时,应考虑内耳发育畸形伴脑脊液耳漏的情况[1, 3-4]。另外还应对漏出液体进行葡萄糖水平测定,检测其是否为脑脊液;同时进行颞骨高分辨CT、MRI检查等影像学检查,观察中耳、内耳的形态,确定脑脊液漏口位置,这是确诊先天性内耳发育畸形并发脑脊液耳漏的主要手段[1, 3-4, 9]

该患者行中耳乳突CT和中耳MRI加内耳水成像检查,检查结果显示为Mondini畸形。Mondini畸形以耳蜗畸形为特征。轻度的自发性脑脊液耳漏表现为脑膜炎发作,只针对脑膜炎进行治疗会出现漏诊误诊的现象。当出现这种情况时,不应忽略耳科专科检查,应彻底排除耳源性病症,观察患者是否有先天性内耳畸形,并对其听力受损情况进行检测,并仔细询问患者是否存在耳闷、耳鼻流水、夜间呛咳及咳嗽不适等临床表现。如存在上述异常情况,应对患者行影像学检查,确定内耳畸形的种类并排除中耳畸形,明确找出漏口位置。常规行颞骨CT检查平扫时需要对内听道、耳蜗水管和乳突气房等部位进行仔细观察[3, 8, 12-15],并在该基础上,进一步行MRI和水成像技术及三维立体成像检查,根据观察情况准确定位漏口的位置,明确漏口的形态、大小和周围软组织及硬化的骨质边缘[16]。该患者的颞骨薄层CT显示为内听道、耳蜗和前庭的扩大畸形,为确诊Mondini畸形伴有脑脊液耳漏提供了可靠的依据。

对脑脊液鼻漏就诊患者,应注意辨别其是否为脑脊液耳鼻漏。当“脑脊液鼻漏”患者就诊时,应先行鼻内镜检查,定位脑脊液鼻漏的具体部位。确认检查需要压迫双侧颈内静脉促使颅压升高,这样可以更好地观察漏口的位置和形状[6, 17-18];如脑脊液经外耳道流出或经咽鼓管流向咽部则可诊断为脑脊液耳漏[19-20]。该患者经鼻内镜检查发现脑脊液自左侧咽鼓管咽口流出,其他部位未见脑脊液流出,故考虑流出的脑脊液是由耳漏进入左侧鼻腔的,诊断为脑脊液耳漏。临床上一些脑脊液耳漏患者也易被误诊为中耳炎[7]。一般认为内耳畸形导致脑脊液漏的部位大多是镫骨底板和(或)足弓发育不良,有骨质缺失,中耳炎症易经缺损处到达蛛网膜下腔,引起脑膜炎;该患者即属于此种类型,摘除听小骨后可见脑脊液从前庭窗流出。因此及时修补瘘口、防止颅内感染是治疗脑脊液耳漏的重要环节[21]。发生Mondini畸形多伴有镫骨畸形,手术只需封闭前庭窗即可。一般还需根据患者身体素质和缺损部位等情况选择最佳途径进行手术。在选择填充材料时首先应考虑自体组织,还有一些适宜材料,如明胶海绵和纤维蛋白胶等[1-2, 21]。该患者采用经乳突入路,应用自体组织修补取得了成功,术后未再复发。

由于脑脊液耳漏患者缺乏临床表现的特异性,很容易出现漏诊和误诊的情况。经常多次发作的脑膜炎伴有耳聋的患者,一般是由内耳畸形所致脑脊液耳漏引起的。由此可见,详细询问患者的病史,对患者进行系统全面的体格检查,及时准确对漏出液进行定性联合相应的辅助检查对脑脊液耳漏的确诊非常重要。确诊为脑脊液耳漏后,及时进行手术修补是非常有效的治疗手段[21]

参考文献
[1]
刘贝贝, 徐百成, 陈迟, 等. 先天性内耳畸形致自发性脑脊液耳漏1例报告[J]. 中华耳科学杂志, 2018, 16(2): 258-260.
[2]
GALBARRIATU L, AURRECOECHEA J, RUIZ DE G, OPEGUI E, et al. Adult spontaneous cerebrospinal fluid otorrhea. Report of two cases and review of the literature[J]. Neurocirugia (Astur), 2011, 22(2): 150-156. DOI:10.1016/S1130-1473(11)70013-6
[3]
袁群芳, 王为, 周明. Mondini畸形伴脑脊液耳漏2例临床分析[J]. 武汉大学学报:医学版, 2014, 35(1): 152-154.
[4]
刘日渊, 侯琨, 侯昭晖, 等. 脑脊液耳漏的临床诊治分析[J]. 临床耳鼻咽喉头颈外科杂志, 2016, 30(8): 627-629.
[5]
SÖNMEZ S, ŞAHIN B, POLAT B, et al. Repair of tegmen tympani defect presenting with spontaneous cerebrospinal fluid otorrhea using the middle cranial fossa approach[J]. J Int Adv Otol, 2017, 13(3): 430-433. DOI:10.5152/iao.2017.3386
[6]
BROWN S M, TABAEE A, SINGH A, et al. Three-dimensional endoscopic sinus surgery:feasibility and technical aspects[J]. Otolaryngol Head Neck Surg, 2008, 138(3): 400-402.
[7]
ZAKARYAN A, POULSGAARD L, HOLLANDER C, et al. Spontaneous cerebrospinal fluid otorrhea from a persistent tympanomeningeal fissure presenting as recurrent serous otitis media[J]. J Neurol Surg Rep, 2015, 76(1): e117-e119. DOI:10.1055/s-0035-1549220
[8]
王红立. 脑脊液耳漏诊断与治疗分析[J]. 医药论坛杂志, 2010(21): 150.
[9]
管业箫, 宗世民, 肖红俊. 自发性脑脊液耳漏的诊断与治疗[J]. 临床耳鼻咽喉头颈外科杂志, 2017(20): 1617-1621.
[10]
LI W, HAN D, YANG W. Surgical management of Mondini dysplasia with cerebrospinal fluid leakage[J]. JClin Otorhinolaryngol Head Neck Surg, 2006, 20(16): 730-731, 734.
[11]
IŞERI M, UÇAR S, DERIN S, et al. Cerebrospinal fluid otorrhea and recurrent bacterial meningitis in a pediatric case with Mondini dysplasia[J]. Kulak Burun Bogaz Ihtis Derg, 2013, 23(1): 57-59. DOI:10.5606/kbbihtisas.2013.35762
[12]
REDDY M, BAUGNON K. Imaging of cerebrospinal fluid rhinorrhea and otorrhea[J]. Radiol Clin North Am, 2017, 55(1): 167-187. DOI:10.1016/j.rcl.2016.08.005
[13]
YE Y F, PAN B G, ZHANG S Q, et al. Reconstruction processing of multi-slice spiral CT in the diagnosis of complex bilateral inner ear malformation with cerebrospinal fluid otorrhea:one case report[J]. Chin Otorhinolaryngol Head Neck Surg, 2017, 52(4): 297-299.
[14]
王肖敏, 刘根久. 复发性耳漏的临床手术治疗效果观察[J]. 吉林医学, 2012, 33(29): 6388-6388. DOI:10.3969/j.issn.1004-0412.2012.29.103
[15]
叶玉芳, 潘宝根, 张淑倩, 等. 多层螺旋CT重建技术诊断双侧内耳复杂畸形并脑脊液耳漏一例[J]. 中华耳鼻咽喉头颈外科杂志, 2017, 52(4): 297-299. DOI:10.3760/cma.j.issn.1673-0860.2017.04.014
[16]
樊建华. 显微单纯鼓室成形术与乳突根治术在中耳乳突手术中的应用[J]. 航空航天医学杂志, 2012, 23(7): 830-831. DOI:10.3969/j.issn.2095-1434.2012.07.030
[17]
KLJAJIC V, VULEKOVIC P, VLAŠKI L, et al. Endoscopic repair of cerebrospinal fluid rhinorrhea[J]. Braz J Otorhinolaryngol, 2017, 83(4): 388-393. DOI:10.1016/j.bjorl.2016.04.024
[18]
YANG Q T, LI P, HUANG J C, et al. Transnasal endoscopic and combined intra-extranasal approach for the surgical treatment of frontal sinus cerebrospinal fluid rhinorrhea[J]. Ther Clin Risk Manag, 2017, 13: 709-715. DOI:10.2147/TCRM.S134537
[19]
开学, 马培如, 张宇皓, 等. 经鼻内镜复杂脑脊液鼻漏修补术三例[J]. 中华耳鼻咽喉头颈外科杂志, 2017, 52(1): 62-64. DOI:10.3760/cma.j.issn.1673-0860.2017.01.013
[20]
张立强, 李学忠, 史丽, 等. 鼻内镜下脑脊液鼻漏修补术[J]. 中华耳鼻咽喉头颈外科杂志, 2012, 47(1): 34-38. DOI:10.3760/cma.j.issn.1673-0860.2012.01.010
[21]
GIOACCHINI F M, CASSANDRO E, ALICANDRI-CIUCANDRI M, et al. Surgical outcomes in the treatment of temporal bone cerebrospinal fluid leak:A systematic review[J]. Auris Nasus Larynx, 2018, 45(5): 903-910. DOI:10.1016/j.anl.2018.03.006