Hearing Loss Identified in 11% of Children With Osteogenesis Imperfecta

Five of 45 children with osteogenesis imperfecta (OI) who underwent audiological testing had mild or moderate conductive hearing loss in at least 1 ear, according to an abstract scheduled for the American Society for Bone and Mineral Research (ASBMR) 2026 meeting, October 9–12. The children with hearing loss were significantly older, on average, than those without hearing loss.

The retrospective, institutional review board–approved study included 45 children aged 6 to 17 years with a clinical or genetic diagnosis of OI. All attended a pediatric metabolic bone clinic and had been referred for audiological testing. Researchers reviewed pure-tone audiometry, tympanometry at 226 Hz, and distortion-product otoacoustic emission (DPOAE) results. They excluded type C tympanograms from analysis because, according to the abstract, these findings do not indicate pathologic middle ear changes in OI.

Five children (11.1%) had mild or moderate conductive hearing loss. The age difference between children with and without hearing loss was statistically significant (P < .05). Six of 44 available tympanograms (13.6%) were consistent with middle ear dysfunction. Among those 6 children, 3 had type 1 OI, 1 had type 4, and 2 had rarer forms: types 5 and 18.

The researchers found no association between prior bisphosphonate use and hearing loss (R = 0.130; P = .635) or middle ear dysfunction (R = 0.179; P = .380). Hearing loss also did not differ significantly by OI type (P = .110).

Fifteen children (33.3%) had at least one absent DPOAE between 1,500 and 8,000 Hz in at least 1 ear. Fourteen (31.1%) had a history of otitis media, although 5 of the 6 children with abnormal tympanometry had no such history.

The authors described the observed prevalence of mild to moderate conductive hearing loss as slightly increased, particularly among older children in the cohort. They suggested that type AD and AS tympanograms without a history of otitis media may indicate early otosclerotic changes. That interpretation remains preliminary. They also called for further study of whether bisphosphonates could help prevent future hearing loss in children with specific OI types; this review did not establish a protective effect.

Reference

Derocher C, Steuerwald W, Wallace M. Evaluation of hearing loss in children with osteogenesis imperfecta. Abstract scheduled for the American Society for Bone and Mineral Research (ASBMR) 2026 meeting; October 9–12, 2026. https://eppro01.ativ.me/web/index.php?page=IntHtml&project=ASBMR26&id=p7702