蜗神经管发育异常及伴发畸形的HRCT表现

HRCT Findings of Abnormal Development of Cochlear Nerve Canal and Associated Malformations

目的 探讨蜗神经管(CNC)发育异常与内听道(IAC)狭窄及耳蜗发育不良(CD)之间的关系。方法 搜集246例CNC发育异常的感音神经性耳聋(SNHL)患儿的颞骨高分辨率CT(HRCT)资料,测量IAC内径宽度,评估耳蜗螺旋管发育情况及CD的程度。结果 246例CNC发育异常病例中,96.3%(237/246)为CNC发育不良(狭窄和闭锁),3.7%(9/246)为CNC扩大并蜗轴发育不良。237例CNC发育不良病例中,左侧CNC发育不良占38.8%(92/237),右侧CNC发育不良占32.9%(78/237),单侧CNC发育不良共占71.7%,双侧CNC发育不良占28.3%(67/237),单纯CNC发育不良在不同侧别(左侧、右侧、双侧)占比为52.1%(48/92)、46.2%(36/78)、44.8%(30/67),伴CD分别占比22.9%(21/92)、25.6%(20/78)、31.3%(21/67),伴IAC狭窄分别占比18.5%(17/92)、28.2%(22/78)、16.4%(11/67),同时伴CD和IAC狭窄分别占比6.5%(6/92)、0%(0/78)、7.5%(5/67)。左侧、右侧及双侧不同侧别内单纯性CNC发育不良、伴CD、伴IAC狭窄及同时伴CD和IAC狭窄的构成比无显著性差异(Pearson卡方值=8.854,自由度6,P值=0.182>0.05)。9例CNC扩大病例均伴中重度CD及蜗轴发育不良,伴脑脊液耳蜗瘘,其中双侧6例,单侧3例,均为右侧。结论 CNC狭窄或闭锁以单侧多见,48%(114/237)为单纯性发育不良,52%(123/237)合并CD和(或)IAC狭窄,CNC扩大病例均合并中重度CD及蜗轴发育不良,且双侧多见。

Objective To investigate the relationship between abnormal development of cochlear nerve canal(CNC) and stenosis of internal auditory canal(IAC) and cochlear dysplasia(CD).Methods 246 cases of children with sensorineural hearing loss(SNHL) performed by temporal bone HRCT which showed abnormal CNC were collected.The width of IAC diameter was measured,and the development of cochlear spiral duct and the degree of CD were evaluated.Results Among 246 cases of abnormal CNC,there was 96.3%(237/246)with CNC dysplasia(stenosis and atresia),3.7%(9/246) with CNC enlargement and cochlear axis dysplasia.Among the 237 cases of CNC dysplasia,the left case accounted for 38.8%(92/237),right 32.9%(78/237),unilateral CNC dysplasia totally accounted for 71.7%,bilateral CNC dysplasia accounted for 28.3%(67/237);simple CNC dysplasiarespectively accounted for 52.1%(48/92),46.2%(36/78) and 44.8%(30/67) in different sides(left,right and both sides),The proportion of CNC dysplasia with CD was 22.9%(21/92)、25.6%(20/78)、31.3%(21/67),with IAC stenosis 18.5%(17/92)、28.2%(22/78)、16.4%(11/67),with CD and IAC stenosis 6.5%(6/92)、0%(0/78)、7.5%(5/67).There was no significant difference in the constituent ratio of simple CNC dysplasia,with CD,with IAC stenosis and with CD and IAC stenosis in left,right and bilateral sides(Pearson chi square=8.854,degree of freedom=6,P=0.182>0.05).Among the 9 cases with enlarged CNC,there were moderate to severe CD,cochlear axis dysplasia and cerebrospinal fluid cochlear leakage,including 6 cases on both sides,3 cases all on the right side.Conclusion Most of CNC stenosis or atresia was unilateral,48%(114/237) was simple dysplasia,52%(123/237) was complicated with CD and/or IAC stenosis,and the cases of enlarged CNC were complicated with moderate and severe CD and cochlear axis dysplasia,and most of them were bilateral.