Martens S, Dhooge I J M, Swinnen F K R
Department of Speech, Language and Hearing Sciences,Faculty of Medicine and Health Sciences,Ghent University,Belgium.
Department of Otorhinolaryngology,Ghent University Hospital,Belgium.
J Laryngol Otol. 2018 Aug;132(8):703-710. doi: 10.1017/S0022215118000956. Epub 2018 Jun 18.
This prospective study involved a longitudinal analysis of the progression of hearing thresholds in patients with osteogenesis imperfecta.
Audiometric results from 36 osteogenesis imperfecta patients (age range, 6-79 years) were compared between two test times with an average interval of 4 years. Audiometric evaluation included acoustic admittance measurements, acoustic stapedial reflex measurements, pure tone audiometry and otoacoustic emissions testing.
Air conduction pure tone average, corrected for sex and age, and bone conduction pure tone average increased significantly in the study population (p < 0.05 and p < 0.001, respectively). In 14.3 per cent of the evaluated ears, an alteration in type and/or severity of hearing loss was observed.
After an average time interval of four years, significant changes in hearing status occurred in a population of osteogenesis imperfecta patients. These findings highlight the importance of regular audiological follow up in osteogenesis imperfecta patients, including audiometry, and measurements of acoustic admittance, acoustic stapedial reflexes and otoacoustic emissions.
本前瞻性研究对成骨不全患者的听力阈值进展进行了纵向分析。
比较了36例成骨不全患者（年龄范围6至79岁）在两个测试时间点的听力测定结果，平均间隔为4年。听力测定评估包括声导纳测量、镫骨肌声反射测量、纯音听力测定和耳声发射测试。
在研究人群中，经性别和年龄校正后的气导纯音平均值和骨导纯音平均值显著增加（分别为p < 0.05和p < 0.001）。在14.3%的评估耳中，观察到听力损失类型和/或严重程度的改变。
平均四年的时间间隔后，成骨不全患者群体的听力状况发生了显著变化。这些发现凸显了对成骨不全患者进行定期听力随访的重要性，包括听力测定以及声导纳、镫骨肌声反射和耳声发射测量。
