1. 承德医学院附属医院耳鼻咽喉头颈外科
2. 解放军总医院耳鼻咽喉头颈外科医学部,北京,100853
3. 国家耳鼻咽喉疾病临床医学研究中心,北京,100853
4. 聋病教育部重点实验室,聋病防治北京市重点实验室
纸质出版:2022
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刘慧慧, 张宇丽, 刘军. 伴阵发性睡眠性血红蛋白尿症的突聋病例报告[J]. 中国听力语言康复科学杂志, 2022,20(1):40-42+58.
刘慧慧, 张宇丽, 刘军. A Case Report: Sudden Sensorineural Hearing Loss with Paroxysmal Nocturnal Hemoglobinuria[J]. 2022, 20(1): 40-42+58.
突聋(sudden sensorineural hearing loss
SSHL)是耳鼻咽喉科常见病、多发病
突聋的病因及发病机制目前并未完全阐明
局部因素及全身因素都可能引起突聋
常见的可能病因包括血管性疾病、病毒感染、微循环障碍、血液粘稠度改变、膜迷路积水、自身免疫性疾病、传染性疾病、肿瘤、生活习惯、睡眠障碍、心理因素等。本文报道1例伴阵发性睡眠性血红蛋白尿症(paroxysmal nocturnal hemoglobinuria
PNH)病史的突聋病例
PNH是一种后天获得性造血干细胞基因突变所致的红细胞缺陷性溶血病
并发症之一为血栓形成。推测突聋是由PNH继发血栓形成引起
此病例罕见
经溶栓、纠正贫血及激素治疗
患者听力恢复良好。通过此病例分析讨论
希望可以为临床的诊疗提供参考。
Sudden sensorineural hearing loss(SSHL) is a common and frequently occurring disease in otolaryngology department. The etiology and pathogenesis of SSHL have not been fully elucidated at present
and both local and systemic factors may cause SSHL.Common possible causes include vascular disease
virus infection
microcirculation disturbance
changes in blood viscosity
labyrinthine hydrops
autoimmune disease
infectious disease
tumour
living habit
sleep disorder and psychological factors
etc.This paper reportes a case of idiopathic sudden deafness with paroxysmal nocturnal hemoglobinuria(PNH). PNH is a red cell defect hemolytic disease caused by acquired hematopoietic stem cell gene mutation.One of the complications of PNH is thrombosis.It is speculated that sudden sensorineural hearing loss is caused by thrombosis which is one of the complications of PNH.This case of SSHL with PNH is rare. After thrombolytic therapy
correct anemia and hormone therapy
the patient’s hearing recovered significantly.The analysis and discussion hope to provide partial reference for clinical diagnosis and treatment.
任翔,郑以州.阵发性睡眠性血红蛋白尿症患者血栓形成的病理生理机制研究进展[J].国际输血及血液学杂志,2018(04).
李丽燕,付蓉.阵发性睡眠性血红蛋白尿症发病机制[J].中华血液学杂志,2018(06).
王秋菊,冰丹.突发性聋的分型诊治与临床研究新进展[J].临床耳鼻咽喉头颈外科杂志,2016(14).
王洪阳,熊文萍,关静,王秋菊.伴发单侧突发性聋的遗传性聋1例:GJB2 235delC纯合突变相关的迟发型中度听力损失[J].临床耳鼻咽喉头颈外科杂志,2016(14).
宋攀攀,张琼敏,王延藏,陈晓云,朱翌.遗传性耳聋相关基因在突发性耳聋发病中的作用[J].温州医学院学报,2013(04).
赵晖,傅窈窈,张天宇,迟放鲁,景江华.突发性极重度聋与全聋的预后特点及差异[J].中华耳鼻咽喉头颈外科杂志,2010(07).
杨剑,刘博,韩德民.突发性耳聋的循环病因机制[J].国际耳鼻咽喉头颈外科杂志,2006(03).
陈协群,孙秉中,郭文怡,朱华锋.尿激酶联合低分子肝素钙治疗阵发性睡眠性血红蛋白尿症合并突发性耳聋一例[J].中华血液学杂志,2000(10).
Devalet Bérangère,Mullier François,Chatelain Bernard,Dogné Jean-Michel,Chatelain Christian.Pathophysiology, diagnosis, and treatment of paroxysmal nocturnal hemoglobinuria: a review.[J].European journal of haematology,2015.
Kiliç Rahmi,Safak Mustafa Asim,Oğuz Haldun,Kargin Selda,Demirci Münir,Samim Erdal,Ozlüoğlu Levent Naci.Intratympanic methylprednisolone for sudden sensorineural hearing loss.[J].Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology,2007.
Choung Yun-Hoon,Park Keehyun,Shin You Ree,Cho Min Jung.Intratympanic dexamethasone injection for refractory sudden sensorineural hearing loss.[J].The Laryngoscope,2006.
Park H M,Jung S W,Rhee C K.Vestibular diagnosis as prognostic indicator in sudden hearing loss with vertigo.[J].Acta oto-laryngologica. Supplementum,2001.
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