Fischer C, Joyeux O, Haguenauer J P, Mauguière F, Schott B
Rev Neurol (Paris). 1984;140(2):117-24.
Deafness in multiple sclerosis is rare, being reported in less than 1 p. 100 of the cases. Ten cases of deafness associated with tinnitus during acute episodes of multiple sclerosis are presented, the diagnosis being definite in 9 patients and probable, according to McAlpine's criteria, in the tenth case. These were not cases where routine examinations demonstrated a latent hearing defect, but were all patients with sudden, often unilateral, incapacitating deafness during an acute episode of the disease, with regression usually after less than 3 months. Deafness recurred in 3 patients and was the initial symptom in 5 cases. Serial audiometric examinations were performed in most cases together with recordings of various evoked potentials, including early auditory evoked potentials during or after the onset of deafness in all 10 patients. BAEPs abnormalities were noted in 8 and appeared to be correlated more with brain stem demyelinization, as it has been established for a few years, than with the deafness itself. Severe anomalies of auditory evoked potentials regressed after the acute episode in only 1 patient. Lesions of the auditory pathways within the brain stem appear to be the cause of the deafness, as shown by results of auditory tests, which pointed to a central origin. The onset of deafness did not indicate a particular progression of multiple sclerosis.
多发性硬化症导致的耳聋很罕见,报道显示其在每100例病例中出现不到1例。本文介绍了10例在多发性硬化症急性发作期间伴有耳鸣的耳聋病例,根据麦卡尔平标准,9例诊断明确,第10例为疑似诊断。这些病例并非通过常规检查发现潜在听力缺陷,而是所有患者在疾病急性发作期间突然出现,通常为单侧、导致失能的耳聋,通常在不到3个月后听力减退。3例患者耳聋复发,5例患者耳聋为首发症状。大多数病例均进行了系列听力测验,并记录了各种诱发电位,包括所有10例患者在耳聋发作期间或之后的早期听觉诱发电位。8例患者出现脑干听觉诱发电位异常,且正如多年来所证实的那样,这些异常似乎与脑干脱髓鞘的相关性更大,而非与耳聋本身的相关性更大。仅1例患者在急性发作后严重的听觉诱发电位异常减退。听力测试结果表明耳聋的病因似乎是脑干内听觉通路的病变,提示为中枢性病因。耳聋的发作并不表明多发性硬化症有特定的进展情况。