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先证者,女,17岁。自述听力下降近10年,且逐渐加重。患者半年前曾在我院以“卡他性中耳炎”治疗,无效。否认耳部疾病、肾病及高热、药物中毒史。查体:神清,一般情况好,理解语言能力较强,面部发育正常,鼻腔通气好,下鼻甲不大,各鼻道未见脓性分泌物;外耳无畸形,外耳道无充血、盯聍栓塞,鼓膜轻度内陷,无穿孔、白斑及异常分泌物,音叉试验256HZ,518HZ示双耳气导、骨导明显下降,电测听示气导平均下降60～70dB骨导平均下降50～60dB,以高频区为甚,前庭反应正常,诊断为神经性进行性聋。
Proof, female, 17 years old. Self-reported hearing dropped nearly 10 years, and gradually increased. Six months ago in our hospital patients with “catarrhal otitis media” treatment, invalid. Denied ear disease, kidney disease and fever, drug poisoning history. Physical examination: God clear, the general situation is good, to understand the language ability is strong, normal facial development, nasal ventilation is good, the inferior turbinate is not, no purulent secretions of the nasal passages; no deformity of the outer ear, external auditory canal without congestion, stare embolism , Tympanic membrane mild invagination, no perforation, leukoplakia and abnormal secretions, tuning fork test 256HZ, 518HZ showed both ears air conduction, bone conduction decreased significantly, the average airway electrical conductivity decreased 60 ~ 70dB average bone conduction decreased 50 ~ 60dB , To high frequency area is even, normal vestibular response, diagnosis of neurological deafness.