Moon In Seok, Bang Mi-Young, Shim Dae Bo, Shin Seung-Ho, Choi Jae Young
Department of Otorhinolaryngology-Head and Neck Surgery, College of Medicine, Chung-Ang University, Seoul, Korea.
Acta Otolaryngol. 2009 Sep;129(9):982-7. doi: 10.1080/00016480802545588.
The concept of hearing loss severity must be redefined, as there is a clear need for more active hearing management in Alport's syndrome patients with severe and profound hearing loss.
Sensorineural hearing loss (SNHL) caused by Alport's syndrome generally does not exceed 60-70 dB, because a cochlear lesion is responsible for this hearing loss. Careful management of renal function improves the prognosis and the longevity of Alport's syndrome patients; it is useful to reassess SNHL caused by Alport's syndrome.
Thirty-two patients with Alport's syndrome were analyzed retrospectively. Pure tone audiograms (PTAs), speech audiograms, and transiently evoked otoacoustic emissions (TEOAEs) were performed. Hearing loss severity was compared to duration of disease and severity of renal dysfunction. We also evaluated the correlation between OAEs and PTAs according to the hypothesis that evoked OAEs would be abnormal even in early stage SNHL in Alport's syndrome.
The level of hearing was positively correlated with disease duration. The hearing of the end-stage renal disease (ESRD) group, whose hearing threshold could exceed 70 dB, was worse than that of the non-ESRD group. OAEs were found in patients with normal hearing and mild hearing loss and had no significant early detection value.
听力损失严重程度的概念必须重新定义，因为对于患有重度和极重度听力损失的奥尔波特综合征患者，显然需要更积极的听力管理。
奥尔波特综合征引起的感音神经性听力损失（SNHL）通常不超过60 - 70分贝，因为这种听力损失是由耳蜗病变引起的。仔细管理肾功能可改善奥尔波特综合征患者的预后和寿命；重新评估由奥尔波特综合征引起的SNHL是有用的。
对32例奥尔波特综合征患者进行回顾性分析。进行了纯音听力图（PTA）、言语听力图和瞬态诱发耳声发射（TEOAEs）检查。将听力损失严重程度与疾病持续时间和肾功能障碍严重程度进行比较。我们还根据在奥尔波特综合征早期SNHL中诱发的耳声发射即使在早期也会异常这一假设，评估了耳声发射与纯音听力图之间的相关性。
听力水平与疾病持续时间呈正相关。终末期肾病（ESRD）组的听力阈值可能超过70分贝，其听力比非ESRD组差。在听力正常和轻度听力损失的患者中发现了耳声发射，且其没有显著的早期检测价值。
