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一例由双侧初级听觉皮层、听辐射和中央后回脑血管病变引起的皮质性耳聋及前庭和躯体感觉丧失——尽管畸变产物耳声发射(DPOAE)和听性脑干反应(ABR)正常,但听力完全丧失。

A case of cortical deafness and loss of vestibular and somatosensory sensations caused by cerebrovascular lesions in bilateral primary auditory cortices, auditory radiations, and postcentral gyruses - complete loss of hearing despite normal DPOAE and ABR.

作者信息

Kaga Kimitaka, Shinjo Yukiko, Enomoto Chieko, Shindo Mitsuko

机构信息

National Institute of Sensory Organs, National Tokyo Medical Center , Tokyo , Japan.

出版信息

Acta Otolaryngol. 2015 Apr;135(4):389-94. doi: 10.3109/00016489.2014.980914. Epub 2015 Jan 28.

Abstract

CONCLUSION

A right-handed 38-year-old man's complete loss of hearing could be diagnosed as cortical deafness caused by cerebral vascular lesions in bilateral auditory cortices.

OBJECTIVE

The aim of this case report was to study the pathophysiology of a particular patient who manifested deafness without residual hearing and lost somatosensory sensation, vestibular sensation, and articulation ability after a right internal carotid-posterior communicating artery (IC-PC) aneurysm and subarachnoidal hemorrhage.

METHODS

MRI, aphasia and neurological tests, subjective and objective audiometry, and vestibular function tests were performed.

RESULTS

The neurological test revealed system loss of somatosensory sensation with normal motor function and articulation ability. Brain imaging revealed extensive infarction in the bilateral primary auditory cortices, postcentral gyruses, and the bilateral partial third frontal gyruses. Pure-tone audiometry of both ears revealed off-the-scale results and speech audiometry demonstrated 0% maximum speech discrimination. However, objective audiometry showed normal distortion product otoacoustic emissions (DPOAE) and normal auditory brainstem response (ABR). The patient showed 0% perception of environmental, speech, and music sounds in both ears. He was unable to feel vestibular sensation despite normal caloric nystagmus. He showed no damage to his larynx or articulation organs.

摘要

结论

一名38岁右利手男性的完全听力丧失可被诊断为双侧听觉皮层脑血管病变所致的皮质聋。

目的

本病例报告旨在研究一名特殊患者的病理生理学,该患者在右侧颈内动脉-后交通动脉(IC-PC)动脉瘤及蛛网膜下腔出血后出现无残余听力且伴有体感、前庭感觉及发音能力丧失的耳聋症状。

方法

进行了MRI、失语症及神经学检查、主观和客观听力测定以及前庭功能测试。

结果

神经学检查显示体感系统丧失,运动功能及发音能力正常。脑部影像学检查显示双侧初级听觉皮层、中央后回及双侧部分额下回广泛梗死。双耳纯音听力测定结果超出范围,言语听力测定显示最大言语辨别率为0%。然而,客观听力测定显示畸变产物耳声发射(DPOAE)正常,听觉脑干反应(ABR)正常。患者双耳对环境声、言语声及音乐声的感知均为0%。尽管冷热试验眼震正常,但他无法感知前庭感觉。他的喉部及发音器官未受损。

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