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  • Title: Spondyloepiphyseal dysplasia congenita associated with conductive hearing loss.
    Author: Dahiya R, Cleveland S, Megerian CA.
    Journal: Ear Nose Throat J; 2000 Mar; 79(3):178-82. PubMed ID: 10743764.
    Abstract:
    Spondyloepiphyseal dysplasia is a disorder characterized by abnormalities of growth. Previous studies of this disorder have identified a significant incidence of associated hearing loss. Hearing loss has been reported to occur in 25 to 30% of affected patients. To date, all reports of associated hearing loss have indicated the presence of a sensorineural component. In this article, we report the case of a child who was diagnosed with spondyloepiphyseal dysplasia congenita and who was found to have a significant conductive hearing loss with a Carhart's notch, indicating the likely presence of stapes footplate fixation. We also review the diagnosis of this condition and the literature associated with hearing loss as it occurs with this disorder.
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