聽覺神經病變－病例報告

Auditory Neuropathy – Case Report

許權振(Chuan-Jen Hsu)

《中華民國耳鼻喉科醫學雜誌》 34卷2期 (1999/03) Pp. 169-172

https://doi.org/10.6286/1999.34.2.169

摘要

聽覺神經病變乃指介於耳蝸內毛細胞和聽覺神經元細胞的輸入聽覺系統的病變。本部經驗1名7歲男孩因漸進性聽覺反應不好而前來就診，兩耳聽力檢查結果：純音聽力檢查正常，鼓室圖正常，耳聲傳射檢查正常，無聽反射，腦幹聽性反應閾值檢查（滴答聲，57.7／s,95 Db Nhl），兩耳均無波形出現，腦幹聽性反應的神經耳學檢查（滴答聲，11.1／s,85 Db Nhl），兩耳均呈І波延長而Ш、Ｖ波缺失。病人無中樞神經症狀，電腦斷層攝影結果，耳蝸、聽神經和腦幹無異常。這些特性符合聽覺神經病變的診斷，在國內尚無此類病例報告，特提出報告。

關鍵字

耳聲傳射檢查 ； 腦幹聽性反應 ； 聽反射 ； 聽覺神經病變

並列摘要

The purpose of the present paper is to report a case of a rather exceptional form of auditory dysfunction that is characterized by abnormal auditory brainstem response (ABR) and normal distortion product otoacoustic emissions. This peculiar pattern of auditory dysfunction wad recently termed “aucitory neuropathy”. There is no such case reported before in Taiwan. This case is a 7 year-old boy complained of hearing impairment for 1 year. His diagnostic profile included normal pure-tone hearing, normal tympanogram, normal distortion product otoacoustic emissions, absent acoustic reflexes, and abnormal auditory brainstem response on both sides. Auditory brainstem response showed delayed wave l and absence of waves lll and V bilaterally. Using only otoacoustic emissions as a hearing screening test may result in a false-negative reading. Therefore, ABR testing combined with otoacoustic emissions testing is recommended for children with suspicious hearing loss.

並列關鍵字

otoacoustic emissions ； auditory brainstem response ； acoustic reflex ； auditory neuropathy