// Hearing Aid Benefit in Auditory Neuropathy Due to Friedreich Ataxia: A Case Report
本研究評估一名44歲患有弗里德萊希共濟失調及聽覺神經病變譜系障礙（ANSD）女性使用助聽器的效果｜行為與電生理測試確認ANSD，雙耳助聽提升安靜環境語音識別，並減少患者疲勞感｜雖然吵雜環境助益有限，患者自覺聽力與生活品質均有改善，建議針對此類患者進行助聽器試用與個人化評估。
// This study evaluated hearing aids in a 44-year-old woman with Friedreich ataxia and auditory neuropathy spectrum disorder (ANSD). Behavioral and electrophysiological tests confirmed ANSD, and binaural amplification improved speech detection in quiet and reduced patient fatigue. Although benefits in noisy environments were limited, the patient reported broader hearing improvements beyond speech discrimination. The findings suggest hearing aids can enhance hearing and quality of life in Friedreich ataxia patients with ANSD, recommending trials with amplification devices and patient-centered assessments.
Published 2026年8月26日
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