CAJ | 학술논문

目的探讨急性双耳感音神经性聋的病因及其相应的临床特点,为临床诊疗提供借鉴.方法回顾性分析2000年11月至2013年12月期间共19例以急性双耳感音神经性聋为主要首发症状患者的临床资料,分别对其临床特征、全身系统检查、实验室检查、听力学及影像学特征进行整理分析,归纳病因,并追踪临床诊疗及预后.结果 19例患者中非耳科系统疾病15例,占78.9％.大部分病例呈现多系统、多器官受累:3例为中枢神经系统疾病,包括真菌性脑膜炎、结核性脑膜炎以及病毒性脑炎各1例,耳聋为双侧进行性加重,伴有发热、头痛、头晕,恶心、呕吐及精神状态的改变等,言语识别率下降,言语识别阈不成比例地明显差于纯音听阈;5例为免疫系统疾病,包括抗中性粒细胞胞质抗体相关小血管炎、复发性多软骨炎、类风湿性关节炎和系统性红斑狼疮,耳聋累及双侧、进展快,同时存在自身免疫性疾病的特点;5例为血液内分泌系统疾病,包括糖尿病、白血病和甲状腺功能低下,听力下降多为双侧对称性、进展性;4例为耳科系统疾病,包括大前庭水管综合征和突发性聋;另有2例为药物性聋.在经过针对病因治疗后,19例患者中,痊愈1例,显效3例,有效7例,无效(亦包括死亡及放弃治疗的病例)8例,总有效率为57.9％.结论急性双耳感音神经性聋多与全身系统性疾病相关,临床诊治上应详细分析病史、临床表现,并完善实验室检查、听力学及影像学等检查以明确诊断,针对病因积极治疗.
목적 탐토급성쌍이감음신경성롱적병인급기상응적림상특점,위림상진료제공차감.방법 회고성분석2000년11월지2013년12월기간공19례이급성쌍이감음신경성롱위주요수발증상환자적림상자료,분별대기림상특정、전신계통검사、실험실검사、은역학급영상학특정진행정리분석,귀납병인,병추종림상진료급예후.결과 19례환자중비이과계통질병15례,점78.9％.대부분병례정현다계통、다기관수루:3례위중추신경계통질병,포괄진균성뇌막염、결핵성뇌막염이급병독성뇌염각1례,이롱위쌍측진행성가중,반유발열、두통、두훈,악심、구토급정신상태적개변등,언어식별솔하강,언어식별역불성비례지명현차우순음은역;5례위면역계통질병,포괄항중성립세포포질항체상관소혈관염、복발성다연골염、류풍습성관절염화계통성홍반랑창,이롱루급쌍측、진전쾌,동시존재자신면역성질병적특점;5례위혈액내분비계통질병,포괄당뇨병、백혈병화갑상선공능저하,은력하강다위쌍측대칭성、진전성;4례위이과계통질병,포괄대전정수관종합정화돌발성롱;령유2례위약물성롱.재경과침대병인치료후,19례환자중,전유1례,현효3례,유효7례,무효(역포괄사망급방기치료적병례)8례,총유효솔위57.9％.결론 급성쌍이감음신경성롱다여전신계통성질병상관,림상진치상응상세분석병사、림상표현,병완선실험실검사、은역학급영상학등검사이명학진단,침대병인적겁치료.
Objective To investigate the etiologies and clinical features for bilateral acute sensorineural hearing loss (bi-ASNHL).Methods The clinical data of 19 cases presenting with bi-ASNHL were retrospectively analyzed,including the clinical features,systemic examinations,laboratory examinations,audiology and radiology results,as well as the prognosis.Results There were 15 nonotologic diseases in 19 patients,accounting for 78.9％ of the total cases,most of which were disorders with multisystem and multi-organ disorder.The central nervous system diseases including fungal meningitis,tuberculous meningitis,and viral encephalitis in 3 patients.The clinical features of deafness were bilateral,progressive,accompanied with fever,headache,dizziness,nausea,vomiting and change of mental status.There was a decrease in speech recognition score (SRS),and speech recognition threshold (SRT) was obviously inferior to pure tone average (PTA) disproportionally.Diseases of immune system including antineural cytoplasmic antibody (ANCA)-associated systemic vasculitis (AASV),relapsing polychondritis (RP),rheumatoid arthritis(RA),and systemic lupus erythematosus (SLE) in 5 patients.They showed the characteristics of bilateral,progressive and simultaneous autoimmune disease.Hematological and endocrine system diseases including diabetes mellitus,leukemia,and thyroid hypofunction in 5 patients.The deafness had the characteristics of symmetry and progressivity.Otologic diseases including large vestibular aqueduct syndrome (LVAS) and sudden sensorineural hearing loss (SSNHL) in 4 patients; Drug-induced sensorineural deafness happened in 2 patients.After the treatment aimed at the causes,1 case was cured,3 patients were markedly effective,7 patients were effective,and 8 patients were ineffective(including dead and refusal cases),with a total effective rate of 57.9％.Conclusions The most of bi-ASNHL cases are often associated with systemic diseases.Clinicians should analyze the history and clinical characteristics in detail,and complete specific laboratory examinations,audiology and imaging examinations in order to reveal the causative diseases.It should be treated aimed at the etiology.