Frequency of Audiology Testing Among Individuals with Osteogenesis Imperfecta and Suggestions for Improving Audiogram Participation

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Background: Osteogenesis imperfecta (OI), characterized by brittle bones and frequent fractures, often manifests with earlier onset hearing loss compared with the general population. Purpose: This study aims to assess the frequency of audiology testing in OI individuals and evaluate a portable audiometry device to enhance audiogram participation. Research Design: This is a prospective observational study. Study Sample: Ninety-seven participants were prospectively enrolled. Data Collection and Analysis: Participants underwent a one-time audiology test using SHOEBOX Audiometry Pro with RadioEar DD450 circumaural headphones (Clearwater Clinical Limited, Ottawa, ON, Canada). Hearing loss was defined as having a pure tone threshold (PTT) of ≥25 dB at one or more tested frequencies. Results: Most participants (54/97) reported undergoing professional audiology testing less often than once every 2 years. The most common reported reason for infrequent testing was because patients did not perceive issues with their hearing, even if hearing loss was subsequently found during screening. Seventy-one percent (69/97) of participants had hearing loss (PTT ≥ 25 dB) at one or more frequencies. Conclusions: Using a portable audiometry device in OI clinics could facilitate early hearing loss detection and improve follow-up care, enhancing quality of life.

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