听力正常的法布里病患者的耳科问题

Otological aspects of Fabry disease in patients with normal hearing.

作者信息

Wang Fei, Yamamoto Hiroshi, Yoshida Tadao, Sugimoto Satofumi, Teranishi Masaaki, Tsuboi Kazuya, Sone Michihiko

机构信息

Department of Otorhinolaryngology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Department of Otorhinolaryngology, The First Affiliated Hospital of China Medical University, Shenyang, China.

出版信息

Nagoya J Med Sci. 2019 Aug;81(3):469-475. doi: 10.18999/nagjms.81.3.469.

DOI:10.18999/nagjms.81.3.469

PMID:31579337

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6728203/

Abstract

We investigated the otological aspects of Fabry disease (FD) in patients with normal hearing. Forty-one patients (21 men, 20 women) with bilaterally normal hearing were recruited, and their otological symptoms and hearing evaluations, which included pure tone audiometry (PTA) and distortion product otoacoustic emission (DPOAE), were investigated. Ten of the 21 male (47.6%) and eight of the 20 female (40.0%) patients had otological symptoms, of which tinnitus was the most frequent. Cardiac dysfunction was more frequently observed in female patients. The average thresholds on PTA were below 25 dB at all frequencies, but DPOAE amplitudes were significantly lower in female patients at some frequencies. Otological symptoms were frequently observed in patients with FD, despite their normal hearing levels on PTA. DPOAE might provide useful information regarding cochlear disturbances related to the disease..

摘要

我们研究了听力正常的法布里病（FD）患者的耳科情况。招募了41例双侧听力正常的患者（21名男性，20名女性），并对他们的耳科症状以及包括纯音听力测定（PTA）和畸变产物耳声发射（DPOAE）在内的听力评估进行了研究。21名男性患者中有10名（47.6%），20名女性患者中有8名（40.0%）有耳科症状，其中耳鸣最为常见。女性患者中更常观察到心脏功能障碍。PTA在所有频率下的平均阈值均低于25 dB，但在某些频率下女性患者的DPOAE振幅明显更低。尽管FD患者的PTA听力水平正常，但仍经常观察到耳科症状。DPOAE可能为与该疾病相关的耳蜗功能障碍提供有用信息。