ArticleJournal of otology2026
Progressive Conductive Hearing Loss in Osteogenesis Imperfecta (Lobstein Disease): A Case Report.
Article in Journal of otology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Background: Osteogenesis imperfecta (OI), also known as Lobstein disease, is a rare inherited connective tissue disorder characterized by bone fragility and various extra-skeletal manifestations. Hearing loss is a frequent but often underestimated complication that may significantly impair quality of life. Case presentation: We report the case of a 34-year-old woman with clinically established osteogenesis imperfecta type I who developed progressive bilateral conductive hearing loss over a three-year period. Audiological evaluation revealed symmetrical conductive impairment with preserved speech discrimination. High-resolution temporal bone computed tomography (CT) demonstrated diffuse otic capsule demineralization, bilateral stapes footplate thickening, and atypical crown-shaped hypodense lesions surrounding the cochlea. Conclusion: Early recognition of hearing loss in osteogenesis imperfecta, combined with detailed imaging and multidisciplinary management, is essential to optimize functional outcomes.
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