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Updated: May 14, 2025

Systematic Hearing Performance Evaluation Process for Adolescents with Cochlear Implantation at Early Ages
Published on: March 24, 2023
Hearing loss in Chinese osteogenesis imperfecta patients
Yuan Tian1, Yanxuan Shao1, Yazhao Mei1
1Department of Osteoporosis and Bone Disease, Shanghai Clinical Research Center of Bone Disease, Shanghai Sixth People's Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.
Propose:
Osteogenesis imperfecta (OI) is a genetic connective tissue disorder primarily characterized by bone fragility. Hearing loss is an extra-skeletal manifestation of OI. This study aims to characterize the audiological findings of a sample of patients with OI in China.
Methods:
A total of 76 patients (aged 4-77 years) were recruited and evaluated using audiometric evaluations, including pure tone audiometry (PTA), acoustic admittance measurements, and distortion-product otoacoustic emissions (DPOAEs). Patients were categorized into 2 age groups: adults (more than 18 years) and children (under 18 years), and classified by hearing type: normal hearing, conductive, sensorineural, or mixed hearing loss.
Results:
Tympanometry (152 ears) showed 71.7% Type A tympanograms. There was a significant association between tympanogram type and hearing loss (p < 0.01). DPOAEs were predominantly abnormal in ears with sensorineural and mixed hearing loss (p < 0.01). PTA (146 ears) revealed hearing loss in 39.0%: 16.4% conductive, 15.1% sensorineural, and 7.5% mixed. Age correlated significantly with hearing loss type (p < 0.01). Adults' group exhibited higher pure-tone averages (11.3 dB vs. 8.8 dB, p < 0.01), average air-bone gap (2.5 dB vs.2.5 dB, p < 0.05), and bone conduction thresholds than children's group.
Conclusions:
Hearing loss is common and progressive in Chinese OI patients, with a significant age-related increase in both the prevalence and severity of hearing loss. Establishing a hearing baseline and regular follow-up is essential for early intervention.
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