Propose <p>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.</p> Methods <p>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.</p> Results <p>Tympanometry (152 ears) showed 71.7% Type A tympanograms. There was a significant association between tympanogram type and hearing loss (<i>p</i> &lt; 0.01). DPOAEs were predominantly abnormal in ears with sensorineural and mixed hearing loss (<i>p</i> &lt; 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 (<i>p</i> &lt; 0.01). Adults’ group exhibited higher pure-tone averages (11.3 dB vs. 8.8 dB, <i>p</i> &lt; 0.01), average air-bone gap (2.5 dB vs.2.5 dB, <i>p</i> &lt; 0.05), and bone conduction thresholds than children’s group.</p> Conclusions <p>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.</p>