Cacace A T, Parnes S M, Lovely T J, Kalathia A
Department of Surgery, Albany Medical College, New York.
Ear Hear. 1994 Aug;15(4):287-98.
To document the existence of a peripheral auditory disconnection syndrome in a 23-yr-old male with a large tumor of the right cerebellopontine angle using contemporary behavioral, electroacoustic, and electrophysiologic auditory tests.
Single subject with repeated measures.
In spite of a profound hearing loss in an ear with a large cerebellopontine angle tumor determined by behavioral audiometry, distortion product otoacoustic emissions, surface recorded brain stem auditory evoked potentials, and transtympanic electrocochleography (click evoked compound action potentials and tone evoked cochlear microphonics) demonstrated that the cochlea was intact and functional over a broad bandwidth and intensity range.
A peripheral auditory disconnection syndrome is demonstrated in a 23-yr-old male with a large tumor of the cerebellopontine angle. In this instance, the tumor effectively deafferentated the peripheral from central auditory nervous system, resulting in profound hearing loss of presumed neural origin. It is suggested that other pathologic conditions/mechanisms could produce similar findings.
运用当代行为学、电声学和电生理学听觉测试，记录一名23岁患有右侧桥小脑角大型肿瘤男性患者外周听觉分离综合征的存在情况。
单受试者重复测量。
尽管通过行为测听法确定患有大型桥小脑角肿瘤的一侧耳朵存在严重听力损失，但畸变产物耳声发射、表面记录的脑干听觉诱发电位以及经鼓膜电耳蜗图（短声诱发复合动作电位和纯音诱发耳蜗微音电位）表明，耳蜗在宽频带和强度范围内完好且功能正常。
在一名患有大型桥小脑角肿瘤的23岁男性患者中证实存在外周听觉分离综合征。在此病例中，肿瘤有效地使外周听觉神经系统与中枢听觉神经系统分离，导致推测为神经源性的严重听力损失。提示其他病理状况/机制可能产生类似结果。
