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摘要: 目的 探讨先天性咽鼓管骨部异常扩大畸形的临床特征。方法 回顾性分析6例(9耳)确诊为先天性咽鼓管骨部异常扩大患者的病史、体格检查、听力检查、颞骨高分辨CT(high-resolution computed tomography，HRCT)检查等结果。结果 6例先天性咽鼓管骨部异常扩大患者中3例双侧咽鼓管扩大，3例左侧咽鼓管扩大。7耳听力结果提示，3耳为传导性聋，平均骨导13.7 dB，平均气导71.3 dB；3耳为混合性聋，平均骨导27.7 dB，平均气导83.7 dB；1耳为极重度感音神经性聋。颞骨HRCT上骨性咽鼓管异常扩大典型表现为咽鼓管骨部增长、增宽，水平位上测量最大长度和最大宽度分别为(22.61±2.94) mm和(6.50±2.33) mm。此外，合并外耳道畸形6耳，鼓室狭小6耳，鼓窦畸形5耳，听骨链畸形7耳，耳蜗畸形6耳，蜗孔畸形3耳，前庭增宽2耳，半规管畸形3耳，前庭窗畸形6耳，面神经走形异常5耳，内听道畸形2耳，颅中窝低位8耳，颈内动脉畸形1耳。结论 咽鼓管骨部异常扩大属于罕见的先天性中耳畸形，可以合并外耳、其他中耳结构以及内耳畸形。患者可以无咽鼓管异常开放症状而仅表现为不同程度听力下降，通常在影像学检查时被无意发现，因此颞骨HRCT具有重要提示意义。Abstract: Objective To investigate the clinical features of patients with congenitally enlarged bony portions of the Eustachian tube(ET).Methods The medical history, physical examination, hearing test, temporal bone high resolution computed tomography(HRCT) of six patients(nine ears) with congenitally enlarged bony portion of the ET were retrospectively analyzed.Results Four patients were men and two were women. The minimum, maximum, and average ages were 5, 21, and(14.7±6.4) years, respectively. Three malformations were bilateral and three were left-sided. Three ears had conductive hearing loss(average bone and air conduction thresholds were 13.7 dB and 71.3 dB), three had mixed hearing loss(average bone and air conduction thresholds were 27.7 dB and 83.7 dB), and one had extremely severe sensorineural hearing loss. The average maximum length and width of the enlarged bony ET on temporal bone HRCT were(22.61±2.94) mm and(6.50±2.33) mm, respectively. The enlargement was combined with an external auditory canal malformation in six ears, narrow tympanic cavity in six, tympanic antrum malformation in five, ossicular chain malformation in seven, cochlear malformation in six, helicotrema malformation in three, vestibule widening in two, semicircular canal malformation in three, vestibular window malformation in six, facial nerve abnormality in five, internal auditory meatus malformation in two, low middle cranial fossa in eight, and severe internal carotid artery malformation in one.Conclusion Bony ET enlargement is a rare congenital middle ear malformation which could combined with other ear malformations. Patients can have no ET dysfunction but different patterns of hearing loss. The defect is usually found unintentionally during imaging, and the HRCT of temporal bone is significant.
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Key words:
- congenital /
- eustachian tube /
- malformation /
- middle ear /
- inner ear
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表 1 咽鼓管异常扩大患者的一般资料
例序 性别 年龄/岁 主诉 体征 外耳道 耳廓畸形 耳前瘘管 颈部瘘管 1 女 5 发现右耳听力差1年 - - - - 2 男 21 双耳听力差21年，右耳前及双颈前瘘口反复肿胀流脓10余年 右侧脓性分泌物 - 右侧 双侧 3 女 18 听力差18年 - - - - 4 男 11 听力差11年 双侧狭窄 - - - 5 男 21 听力差21年 双侧狭窄 - - - 6 男 12 听力差12年 左垂直且宽敞；右狭窄 右侧条索状 双侧 - 
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表 2 咽鼓管骨部异常扩大在颞骨HRCT水平位的特征
例序 咽鼓管扩大侧别 最大宽度/mm 最大长度/mm 1 双侧 左：5.9右：6.1 左：24.6右：21.6 2 双侧 左：11.9右：5.8 左：20.6右：22.8 3 左侧 5.8 19.5 4 左侧 4.1 27.0 5 双侧 左：5.5右：5.0 左：19.4右：21.1 6 左侧 8.4 26.9
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表 3 咽鼓管骨部异常扩大伴外中耳畸形的颞骨HRCT特征
例序 侧别 外耳道畸形 鼓室腔 鼓窦 乳突 听骨链畸形 1 左 - 狭小 未发育 气化型 + 右 - 狭小 未发育 气化型 + 2 左 走行异常 - - 气化型 + 右 走行异常，上壁骨质缺损 狭小，上鼓室软组织影 软组织影 硬化型，部分骨质破坏可见软组织影 + 3 左 - 狭小 - 板障型 + 4 左 狭窄 狭小，上鼓室软组织影 未发育 硬化型 + 5 左 狭窄 - - 气化型 - 右 狭窄 - - 板障型 - 6 左 上壁骨质缺损 狭小，鼓室内可见软组织影 软组织影 硬化型，部分骨质破坏可见软组织影 +
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表 4 咽鼓管骨部异常扩大伴内耳畸形颞骨HRCT特征
例序 侧别 耳蜗 蜗孔 前庭 半规管 前庭窗 蜗窗 1 左 周数不足 狭窄 - - - - 右 小，周数不足 狭窄 - 后半规管短小 - - 2 左 中、顶周小 - - - - - 右 中、顶周小 - - - 闭锁 - 3 左 中、顶周融合 - 增宽 上半规管畸形 狭窄 - 4 左 - - - - 闭锁 - 5 左 - - - - 闭锁 - 右 - - - - 闭锁 - 6 左 中、顶周部分融合 闭锁 增宽 外半规管短粗 闭锁 -
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表 5 咽鼓管骨部异常扩大伴其他重要结构畸形颞骨HRCT特征
例序 侧别 面神经 内听道 颅中窝 颈内动脉畸形 1 左 迷路段起源及走形异常 - 低位 - 右 迷路段起源及走形异常 - 低位 2 左 - - - - 右 乳突段前移 - 低位 3 左 - - 低位 - 4 左 鼓室段移，乳突段前移 - 低位 - 5 左 - 略宽 低位 + 右 - - 低位 6 左 乳突段前移 狭窄 低位 -
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