I型成骨不全症中（未手术耳的）听力损失与年龄的关系

Hearing loss (in nonoperated ears) in relation to age in osteogenesis imperfecta type I.

作者信息

Garretsen A J, Cremers C W, Huygén P L

机构信息

Department of Otolaryngology, University Hospital Nijmegen, The Netherlands.

出版信息

Ann Otol Rhinol Laryngol. 1997 Jul;106(7 Pt 1):575-82. doi: 10.1177/000348949710600709.

DOI:10.1177/000348949710600709

PMID:9228859

Abstract

Hearing loss was studied in relation to age in nonoperated ears in a group of 142 subjects with autosomal dominant osteogenesis imperfecta type I, which was compared to that in a random subsample of 70 subjects. In the n = 142 group, particularly below the age of 30 years, considerable selection (ie, for ear surgery) had occurred on hearing loss. The hearing threshold increased gradually with age. A hearing loss of greater than 30 dB (Fletcher index) was observed for 51% of the subjects older than 20 years and younger than 60 years. The median hearing loss progressed from the 10th to the 45th years of life with an average annual threshold increase (ATI) of 1 dB/y (0.5 to 4 kHz) up to 1.7 dB/y (8 kHz). Sensorineural loss accounted for 0.6 dB/y ATI at 0.5 to 4 kHz and 1.3 dB/y ATI at 8 kHz; conductive loss accounted for 0.4 dB/y ATI at all frequencies.

摘要

在一组142例I型常染色体显性遗传性成骨不全患者中，对未手术耳的听力损失与年龄的关系进行了研究，并与70例随机抽取的受试者子样本进行了比较。在n = 142的组中，特别是在30岁以下，因听力损失而进行（耳部手术）选择的情况相当多。听力阈值随年龄逐渐升高。在年龄大于20岁且小于60岁的受试者中，51%观察到听力损失大于30 dB（弗莱彻指数）。听力损失中位数从生命的第10年到第45年有所进展，平均每年阈值升高（ATI）在0.5至4 kHz时为1 dB/年，在8 kHz时为1.7 dB/年。感音神经性损失在0.5至4 kHz时ATI为0.6 dB/年，在8 kHz时为1.3 dB/年；传导性损失在所有频率下ATI均为0.4 dB/年。