The analysis of the characteristics of hearing loss in patients with cochlear nerve aphasia identified by MRI
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摘要: 目的 探讨蜗神经未发育(CNA)患者听力损失的特征, 为临床此类患者的诊断和治疗提供依据。方法 回顾性分析51例CNA患者的听力学结果、颞骨HRCT和内听道MRI检查结果, 分析患者听力损失的特征。结果 77.19%的患耳呈极重度听力损失, 7.02%的患耳呈中度听力损失; CNA患耳的残余听力集中分布于低中频; 伴蜗神经孔闭锁的CNA的患耳可表现为中度听力损失。结论 CNA患者仍有残余听力的可能性。中度听力损失的患者不排除CNA的可能。内听道MRI、颞骨薄层HRCT扫描和听力学评估的"三维一体"的综合评估体系更有助于对CNA疾病的诊断。Abstract: Objective To explore the characteristics of hearing loss in patients with cochlear nerve aphasia(CNA) and to provide evidences for diagnosis and treatment of cochlear nerve aphasia.Methods A retrospective study was performed. A total of 51 cases were included in the study. The data of hearing test, inner auditory canal MRI and temporal HRCT were analyzed.Results 77.19% of the affected ears had extremely severe hearing loss, and 7.02% of the affected ears had moderate hearing loss. The residual hearing was concentrated in low-medium frequency. A CNA ear with bone cochlear nerve canal atresia can exhibited moderate hearing loss.Conclusion The patient with CNA may still present residual hearing function. CNA could not be excluded in patients with moderate hearing loss. The "three-dimensional integration" comprehensive evaluation system, which includesinternal auditory canal MRI, temporal thin-layer CT scan and audiology evaluation, could be helpful to the diagnosis of cochlear nerveaphasia.
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Key words:
- cochlear nerve /
- dysplasia /
- hearing loss
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表 1 CNA患耳的PTA分布
耳(%) PTA/dB HL 单耳 双耳 合计 41~60 3(6.38) 1(10.00) 4(7.02) 61~70 3(6.38) 0(0.00) 3(5.26) 71~80 5(10.64) 1(10.00) 6(10.53) >80 36(76.60) 8(80.00) 44(77.19) 
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表 2 CNA患耳在不同听阈的分布
耳(%) PTA/dB HL 0.5 kHz 1.0 kHz 2.0 kHz 4.0 kHz < 26 0(0.00) 1(1.75) 0(0.00) 0(0.00) 26~40 1(1.75) 0(0.00) 1(1.75) 1(1.75) 41~60 7(12.28) 5(8.77) 2(3.51) 2(3.51) 61~70 7(12.28) 5(8.77) 3(5.26) 5(8.77) 71~80 3(5.26) 7(12.28) 6(10.53) 2(3.51) >80 39(68.43) 39(68.43) 45(78.95) 47(82.46)
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表 3 伴内听道狭窄、BCNC狭窄及闭锁的CNA患耳的PTA分布
耳 PTA/ dB HL 内听道狭窄 BCNC狭窄 BCNC闭锁 BCNC狭窄+IAC狭窄 BCNC闭锁+IAC狭窄 41~60 0 3 0 0 0 61~70 0 2 0 0 0 71~80 0 2 1 0 0 >80 9 5 1 1 3 合计 9 12 2 1 3
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