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Bir Olgu Nedeniyle Van der Hoeve Sendromu

Özeri C., Samim E., Şafak M.A., Aslan N., Eryılmaz A.

Osteogenesis imperfekta (OI) tanısıyla izlenen ve kliniğimiz işitme kaybı şikayetiyle başvuran bir olgu nedeniyle Van der Hoeve Sendromu gözden geçirilerek sunuldu.

Van der Hoeve Syndrome: Presentation of a Case

Özeri C., Samim E., Şafak M.A., Aslan N., Eryılmaz A.

Van der Hoeve’s syndrome is rewieved in relation with a case who applied to our clinic with hearing loss diagnosed as Osteogenesis Imperfecta (OI) previously. OI designates a heterogeneous group of heritable disorders of connective tisue that in addition to bone may affect tendons,ligaments, facia, skin, sclera, vessels, teeth and hearing. The current classification idenitifies at least four major syndrome group or types. Loss of hearing is the least constant of the prominent features of OI. Its incidence varies exween 23% and 58. In the temporal bone the development of the inner ear capsule may be involved severely. Microfractures also can be seen at stapes footplate. OI can be associated with otosclerosis, another bone dysplasia with a different morphology.

Türk Otolarengoloji Arşivi 29(1)/20-22

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