进行性桥延麻痹伴耳聋又称Van Laere氏病,Brawn-Vialetto Van Laere综合征由Brown于1894年首次报告。1984年Oppenhermer统计世界上临床病例18例(其中尸检4例),除1例来自美国外,多数为西欧报告。该病属常染色体隐性遗传。现将我们临床遇到的1例报告如下: 病例报告沈××,女,28岁,病案号244104,安徽淮南人。因两耳听力减退5年,四肢无力、变细半年,声音嘶哑,饮水呛咳5个月于1988年5月11日住院。5年前丈夫偶然发现需高声与其谈话,随之到当地医院就诊,作电测听为两侧神经性耳聋伴嗡嗡声,服用多种维生素无效。近半年来一直自感四肢无力,右侧为甚,疲劳时明显,时伴前额跳痛,无恶心、呕吐,听力障碍及四肢无力进行性加重,听不见别人大声喊叫,四肢渐细,体重减轻约10kg,不能参加工作及家务劳动,仅能自理个人生活。住院前5个月出现声音嘶哑,饭水呛咳,吞咽困难,时感 Progressive paralysis with paralysis, deafness also known as Van Laere’s disease, Brawn-Vialetto Van Laere syndrome was first reported by Brown in 1894. 1984 Oppenhermer statistics 18 cases of clinical cases in the world (including autopsy in 4 cases), except one case from the United States, most of the Western European report. The disease is autosomal recessive. Now we encounter a clinical report as follows: Case Report Shen × ×, female, 28 years old, case number 244104, Huainan, Anhui. Due to hearing loss of both ears for 5 years, limb weakness, thinning for half a year, hoarseness, cough drinking water for 5 months in May 11, 1988 hospitalization. Five years ago, her husband accidentally discovered that he needed to talk loudly to the local hospital. He then went to the local hospital for electric audiometry with deafness on both sides and a buzzing fever. He took multiple vitamins and was ineffective. Over the past six months has always felt weakness on their own, the right is even more obvious when fatigue, accompanied by forehead jump pain, no nausea, vomiting, hearing loss and weakness, exacerbations, can not hear others shouting, thinning limbs, weight loss About 10kg, can not participate in work and housework, can only take care of personal life. 5 months before hospitalization hoarse voice, rice cough, difficulty swallowing, sense of time