Evaluation of Imaging in Children with Congenital Sensorineural Hearing Loss
Evaluation of Imaging in Children with Congenital Sensorineural Hearing Loss
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发表时间：
2009
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通讯作者：
Li Yu
中科院分区：
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作者：
Li Yu
目的：探讨先天性感音神经性聋（SNHL）患儿的颞骨CT和MRI表现，分析其内耳异常的发生率和分型，探讨内耳异常与耳蜗神经的关系，并探讨部分SNHL患儿是否可以单纯用CT检查。方法：对101例SNHL患儿行HRCT和MRI检查，其中双侧重度或极重度听力损失95例，单侧听力损失6例。28例前庭导水管粗大，19例耳蜗神经发育不良，10例内耳道狭窄，均伴有耳蜗神经发育不良，6例单侧SNHL患儿均伴有耳蜗神经发育不良，130例HRCT及MR检查均正常。双侧重度或极重度SNHL儿童的内耳异常比单侧SNHL儿童少（31.6%对100%）内耳异常与耳蜗神经畸形有一定关系，HRCT上有严重内耳异常的患儿，应结合临床进行检查，以耳蜗异常和前庭导水管粗大为多见，内耳异常与耳蜗神经畸形有一定关系。MR检查可排除耳蜗神经畸形，CT检查耳蜗正常，无IAC狭窄的患儿可不做MR检查，单侧SNHL患儿应同时行HRCT和MR检查。
Objective:To investigate CT and MRI findings of temporal bone and analyze the prevalence and classification of inner ear abnormalities in children with congenital sensorineural hearing loss(SNHL).To find out the relationship between inner ear abnormality and cochlear nerve,and discuss whether a portion of patients can be examined only with CT.Methods:101 children with SNHL were examined with HRCT and MR scan,95 of them had bilateral severe or profound hearing loss,6 had unilateral hearing loss.Results:Of the 196 ears,abnormalites of the inner ear were found in 66 ears.3 were found to be cochlear aplasia;3 cochlear dysplasia;7 IP-I malformation;10 IP-II malformation;28 large vestibular aqueduct;19 cochlear nerve aplasia or dysplasia;10 internal auditory canal narrowing all accompanied with cochlear nerve dysplasia.All of the 6 children having ears with unilateral SNHL had cochlear nerve aplasia or dysplasia.130 had normal inner ear both on HRCT and MR.Conclusion:Children having ears with bilateral severe or profound SNHL had less inner ear abnormalities than children with unilateral SNHL(31.6% vs 100%).The most common abnormalities were abnormal cochlea and large vestibular aqueduct.The abnormalities of inner ear have a definite relation to the malformation of cochlear nerve.Children with severe abnormalities of inner ear on HRCT must be examined with MR to exclude malformations of cochlear nerve.Children with normal cochlear on CT having no IAC stenosis can dispense with MR scan.Children having unilateral SNHL must be examined with both HRCT and MR scan.