聽神經病變（Auditory neuropathy）是一種內耳機能正常，可是聽覺神經傳導系統呈現異常之病變。此類患者臨床症狀以因為不同程度的聽力損失與語音知覺辨識不佳，所造成的語言發展遲緩以及學習障礙為主。其在臨床聽力學的表現如下：耳聲傳射（OAE）及耳蝸麥克風（CM）正常，鐙骨肌反射（AR）消失，純音聽力檢查（PTA）結果從正常到極重度聽力損失皆有可能，語音辨識能力（SRT、SDS）異常，聽性腦幹反應（ABR）異常。歷年文獻指出，此類患者使用調頻系統（FM system）、或調頻助聽器（FM+HA）、或人工電子耳（CI），並輔以聽能訓練，可獲得較佳言語聽知覺能力。本院於民國88年5月間，就經歷一主述言語發展遲緩的九歲學童，其臨床聽力學檢查結果：耳聲傳射正常，純音聽力檢查為兩側輕度感音神經性聽力損失，但聽性腦幹反應卻指出85dBnHL才有反應；另外並無伴隨其他運動發展異常、心智異常，乃被診斷為聽神經病變。由於聽神經病變易被忽略，特別提出報告。
In auditory neuropathy, the auditory neural pathway is abnormal and inner ear function is normal. Language development delay and learning disabilities are the main clinical symptoms. Audiological findings include variable pure tone thresholds ranging from normal sensitivity to severe or profound hearing loss. Speech recognition capability is much poorer than expected based on pure tone thresholds. In electrophysiological assessments, normal otoacoustic emissions and abnormal auditory brainstem responses are typical. Management of patients with auditory neuropathy requires special consideration because conventional approaches may not work. Patients may benefit from a frequency modulation (FM) amplification system and speech-language therapy. A case study of auditory neuropathy is reported in this paper.
