73例波动性感音神经性聋病因及临床特点分析

摘要

Objective To report common etiologies,clinical characteristics and causes of erroneous and missed diagnoses in a series of cases of fluctuating sensorineural hearing loss (FSNHL). Methods The authors retrospectively -analyzed clinical data, audiology and radiology test results of 73 cases with FSNHL. Results ① Etiologies in the 73 cases included: low-tone sensorineural hearing loss (LSNHL, 21/73, 29%), Meniere disease (MD, 17/73, 23%), inner ear malforma-tion(12/73, 16%), delayed endolymphatic hydrops (DEH, 8/73,11%), autoimmune sensorineural hearing loss (ASNHL, 5/ 73, 7%), early stage acoustic neurinoma(4/73, 6%), noise-induced hearing loss(3/73,4%)and unkoown etiologies(3/73, 4%). No auditory neuropathy, perilymph fistula or vascular abnormalities was found in this group of patients. ② Hearing fluctuated only in the low frequency range in LSNHL and early stage MD eases, and only in the high frequency range in early stage acoustic neurinoma and noise-induced hearing loss cases.ASNHL and bilateral inner ear anomalies often showed symmetrical sensorineural hearing loss. ③ Causes of erroneous and missed diagnosis: Symmetrical enlarged vestibule abnormalities could show symmetrical and progressive sensorineural hearing loss attacks in adulthood. Most such cases could be identified by the precipitating factors of attacks, including sudden rises of intracranial or abdominal pressures. Radiology was helpful to differentiate these cases from those of ASNHL; Unilateral high-frequency hearing fluctuation often represented early stage acoustic neurinoma, which could be picked up by enhanced MRI as a routine test to avoid missed diagnosis. Conclusion FSNHLinvolves numerous etiologies. Correct diagnosis depends on not only history, symptomatology and audiology features, but also knowing the precipitating and occupational environment factors.%目的 探讨波动性感音神经性聋(FSNHL)的病因及其相应的临床特点,分析引起误诊及漏诊的原因,为临床诊疗提供借鉴.方法 回顾性分析73例波动性感音神经性聋的临床表现、听力学及影像学特征.结果 ①病因:按照发病率高低依次为低频感音神经性聋(LSNHL)29％(21/73),梅尼埃病(MD)23％(17/73),内耳畸形16％(12/73),迟发性膜迷路积水11％(8/73),自身免疫性感音神经性聋(ASNHL)7％(5/73),早期听神经瘤6％(4/73),噪声性聋4％(3/73)及不明原因者4％(3/73).本组病例未见听神经病、外淋巴漏及耳周血管变异等病因.②LSNHL、MD早期多表现为低频听力波动;早期听神经瘤及噪声性聋表现为高频听力波动;ASNHL及双侧内耳畸形多为双侧对称性感音神经性聋;③容易误诊及漏诊的疾病:双侧对称性前庭扩大畸形可于成年后出现对称性、波动性、进行性感音神经性聋,发病常有导致颅压及腹压骤变的诱因,影像检查可与ASNHL鉴别;单耳高频波动性感音神经性聋需警惕早期听神经瘤,增强MRI应作为常规检查以避免漏诊.结论 波动性感音神经性聋病因众多,表现各异;诊断除需关注病史、症状及听力学特征外,发病诱因及职业环境可提供重要诊断线索.