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題名 | 具正常耳聲傳射之聽障兒=Hearing-impaired Infants with Normal Otoacoustic Emissions |
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作 者 | 吳俊良; 趙文元; 高純珠; 李恆惠; | 書刊名 | 中華民國耳鼻喉科醫學雜誌 |
卷期 | 34:3 民88.05-06 |
頁次 | 頁200-206 |
分類號 | 417.6841 |
關鍵詞 | 聽覺神經病變; 先天性聽障; 耳聲傳射; Auditory neuropathy; Congenital deafness; Otoacoustic emissions; |
語文 | 中文(Chinese) |
中文摘要 | 背景:先天性聽障大多源自於耳蝸病變,然而我們於新生兒聽力篩檢時發現了一些 具有正常耳聲傳射(OAEs)的聽障兒,顯然他們的聽障原因不同於一般耳蝸病變所引起之聽 障。因此,我們針對這一些聽障兒作深入的探討。 方法:自1997年1月至1998年7月止,本院共計有102名嬰兒以聽性腦幹反應(ABR)判 定有聽障。但其中有4名之變頻耳聲傳射(DPOAE)呈現正常反應。我們針對這4名嬰兒進行後 續追蹤,包括行為聽力檢查、聽反射檢查、評估整體發展、磁振攝影、體感覺神經誘發電位 (SSEP)與視神經誘發電位(VEP)、配戴助聽器並觀察其效果。 結果:4名嬰兒的聽性腦幹反應均呈現無反應;但兩耳均有正常的變頻耳聲傳射,聽反射 皆無。聽力狀況則分別為(1)兩耳極重度聽障、(2)右耳重度聽障與左耳極重度聽障、(3)原先 音場測試為重度聽障,目前則變成兩耳中度聽障、(4)兩耳重度聽障。磁振攝影顯示4名都無 構造上的異常。SSEP及VEP檢查,其中1名同時有視神經徑路與體感覺神經徑路的異常,另 外兩人則個別有視神經與體感覺神經的異常。有3名已使用助聽器,使用效果均不理想。目 前我們將這4名嬰兒歸類為因聽覺神經病變(auditory neuropathy)所引起之聽力障礙。 結論:聽覺神經病變的特徵是內耳擁有正常的外毛細胞但其耳蝸神經與聽覺徑路至腦幹 部位的神經功能卻是異常的。他們的聽閾則差異很大,從中度至極重度聽障皆有,且聽力狀 況也可能會產生變化。基本上這些聽障兒的智商是正常的。除了聽覺神經徑路的神經功能受 到影響外,也常常合併有其他的神經病變。助聽器使用效果往往不佳,復健方式應該以全方 位溝通模式為之。 |
英文摘要 | Background:Most congenital deafness is caused by cochlear lesions. Auditory brainstem response and otoacoustic emissions were expected to be abnormal in these patients. However, we present four hearing-impaired infants who had normal otoacoustic emissions. Methods: Over a period of 18 months, we identified four hearing-impaired infants with normal otoacoustic emissions and an absent auditory brainstem response. A series of examinations, including a behavioral audiometric test, acoustic reflex, evaluation of mental development, magnetic resonance imaging (MRI), evoked potential test of peripheral sensation and vision, and a hearing aid evaluation, were done. Results: Behavioral audiometric testing showed profound hearing loss in one patient, severe loss in two, and moderate loss in one. The pure tone threshold of the patient with moderate impairment appeared to have changed over the past 6 months. All four patients showed no acoustic reflex up to the level of 110 dB HL pure tone stimulation. No patients had mental retardation. MRI showed no abnormalities. One patient had a deficit of the visual pathway, one had a deficit of the somatosensory pathway, and one had both of these deficits. Amplification with hearing aids was applied without an improvement in hearing in three patients. Thus, auditory neuropathy might account for these patients' hearing impairment. Conclusion:Using auditory brainstem response and otoacoustic emissions, we are able to identify patients with disorders of retrocochlear function, in which the function of the cochlear outer hair cells is preserved. In patients with auditory neuropathy, the severity of hearing loss varies, from moderate to profound, or fluctuated. Their intelligence is normal essentially. Some patients are found to have accompanying peripheral neuropathy. Amplification with hearing aids is shown to be with little benefit. We suggest that total communication, instead of auditory/verbal training, is the method of choice for their rehabilitation. |
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