以突发听力下降为首发症状的听神经瘤临床特征分析

Clinical Characteristics of Acoustic Neuroma Presenting as Sudden Sensorineural Hearing Loss

目的分析以突发听力下降为首发症状的听神经瘤患者临床特征,为诊疗决策提供参考。方法回顾分析我院2015年8月至2019年8月因突发听力下降按突发性聋收治的408例患者临床资料,先按突发性聋治疗,确诊后调整治疗策略,随访6月~4年。结果确诊以突发听力下降为首发症状的听神经瘤患者12例,其发病率约为2.94%。纯音测听结果:低频下降型2例,平坦下降型6例,全聋型2例,高频下降型2例;听力损失程度:轻度3例,中度5例,重度2例,极重度2例。言语测听结果:言语识别率4例为0~50%,3例为50~70%,5例为70~100%。耳声发射6例正常,5例部分引出,1例均未引出。ABR检查:1例正常,其余均异常。MRI显示瘤体大小(Koos分级):Ⅰ级7例,Ⅱ级4例,Ⅳ级1例;10例位于内听道,2例位于桥小脑角。经药物治疗后听力改善结果:4例痊愈,3例显效,2例有效,3例无效,听力提高总有效率为75%。结论以突发听力下降为首发症状的听神经瘤多发生在内听道,听力损失程度与发生部位有关,与瘤体大小相关性不大。早期确诊依靠MRI检查,及时按突发性聋药物治疗,可明显提高听力,但不能因听力恢复而忽视MRI检查以免漏诊。

Objective To report clinical characteristics of acoustic neuroma presenting as sudden sensorineural hearing loss to improve its diagnosis and treatment. Methods Data from 408 cases of sudden sensorineural hearing loss seen from August 2015 to August 2019 were retrospectively analyzed. Results Acoustic neuroma was eventually diagnosed in 12 of the patients(2.94%), in whom audiograms showed low frequency loos in 2 cases, flat loss pattern in 6 cases, total deafness in 2 cases and high frequency loss in 2 cases. Severity of hearing loss ranged from mild(n=3) to moderate(n=5), severe(n=2) and profound(n=2). Speech discrimination scores were under 50% in 4 cases, 50-70% in 3 cases and 70-100% in 5 cases. Otoacoustic emissions were normal in 6 cases, partially missing in 5 cases and absent in 1 case. Auditory brainstem response(ABR) examination was normal only in 1 case. Magnetic resonance imaging(MRI)revealed Koos grade I tumor in 7 cases of, grade II tumor in 4 cases and grade IV tumor in 1 case. Tumor was located in the inner auditory canal in 10 cases and in the pontine cerebellum in 2 cases. Following pharmacological interventions,hearing fully recovered in 4 cases, partially recovered in 3 cases, improved in 2 cases and showed no improvement in 3 cases. The overall rate of hearing improvement was 75%. Conclusion Acoustic neuromas presenting with sudden sensorineural hearing loss is mostly located in the inner auditory canal, and the degree of hearing loss is related to tumor location but not to tumor size. Early diagnosis relies on MRI, and timely treatment targeting sudden sensorineural hearing loss can significantly improve hearing. However, MRI should be considered when indicated to avoid missed diagnosis.