[Clinical review of inner ear malformation].
[Clinical review of inner ear malformation].
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内耳畸形的临床回顾[J].
DOI：
10.3950/jibiinkoka.106.1038
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发表时间：
2003
期刊：
影响因子：
--
通讯作者：
Y. Terayama
中科院分区：
文献类型：
--
作者：
H. Kokai;Masami Oohashi;K. Ishikawa;Kouji Harada;H. Hiratsuka;M. Ogasawara;Souji Miyashita;Y. Terayama
1996年至2002年间，我们在麻布三科医院有126名内耳畸形患者通过颞骨计算机断层扫描（CT）诊断。我们根据Jackler等人的分类方法对内耳畸形进行分类。鼻唇沟畸形61%（孤立性外半规管发育不良56%，复合性半规管发育不良4%，半规管发育不全1%），2。皮质骨异常24%，3.前庭导水管闭塞12%，4。内耳道狭窄2%，5。完全性甲状腺发育不全1%，6。耳蜗导水管封闭0%。最常见的异常是孤立性外半规管发育不良。我们没有在该异常中检测到任何显著的临床特征。有2例耳蜗异常患者既往有脑膜炎病史。部分前庭导水管扩大患者常出现听力波动。在临床上，重要的是要检测患者的内耳畸形，如耳蜗异常和前庭水管扩大，通常伴有先天性感音神经性听力损失。对于先天性感音神经性耳聋的患者，我们建议进行颞骨CT扫描。
We had 126 patients with inner ear malformation diagnosed with temporal bone computed tomography (CT) scans at Azabu Triology Hospital between 1996 and 2002. We classified cases of inner ear malformation according to Jackler et al. The incidence of inner ear malformation in our series was as follows; 1. Labyrynthine anomalies 61% (isolated lateral semicircular canal dysplasia 56%, compound semicircular canal dysplasia 4%, semicircular canal aplasia 1%), 2. Cochlear anomalies 24%, 3. Enlargement of the vestibular aqueduct 12%, 4. Narrow internal auditory canal 2%, 5. Complete labyrinthine aplasia 1%, 6. Enlargement of the cochlear aqueduct 0%. The most frequent anomaly was isolated lateral semicircular canal dysplasia. We did not detect any significant clinical features in this anomaly. There were 2 patients with cochlear anomalies who had past histories of meningitis. Some patients with enlargement of the vestibular aqueduct had frequent attacks of fluctuating hearing. Clinically it is important to detect patients with inner ear malformation such as cochlear anomalies and enlargement of the vestibular aqueduct usually accompanied by congenital sensorineural hearing loss. For patients with congenital sensorineural hearing loss, we recommend temporal bone CT scan.