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Progressive Conductive Hearing Loss in Osteogenesis Imperfecta (Lobstein Disease): A Case Report
Karim Messaoudi1, Nassim Ait Mesbah2, Nadia Yahi2
1Department of Medicine, Faculty of Medicine, University of Kasdi Merbah, Ouargla 30000, Algeria.
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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