Hearing Loss in Osteogenesis Imperfecta: Characteristics and Treatment Considerations

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Hearing Loss in Osteogenesis Imperfecta: Characteristics and Treatment Considerations

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Title: Hearing Loss in Osteogenesis Imperfecta: Characteristics and Treatment Considerations
Author: Pillion, Joseph P.; Shapiro, Jay; Vernick, David M.

Note: Order does not necessarily reflect citation order of authors.

Citation: Pillion, Joseph P., David Vernick, and Jay Shapiro. 2011. Hearing loss in osteogenesis imperfecta: Characteristics and treatment considerations. Genetics Research International 2011:983942.
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Abstract: Osteogenesis imperfecta (OI) is the most common heritable disorder of connective tissue. It is associated with fractures following relatively minor injury, blue sclerae, dentinogenesis imperfecta, increased joint mobility, short stature, and hearing loss. Structures in the otic capsule and inner ear share in the histologic features common to other skeletal tissues. OI is due to mutations involving several genes, the most commonly involved are the COL1A1 or COL1A2 genes which are responsible for the synthesis of the proalpha-1 and proalpha-2 polypeptide chains that form the type I collagen triple helix. A genotype/phenotype relationship to hearing loss has not been established in OI. Hearing loss is commonly found in OI with prevalence rates ranging from 50 to 92% in some studies. Hearing loss in OI may be conductive, mixed, or sensorineural and is more common by the second or third decade. Treatment options such as hearing aids, stapes surgery, and cochlear implants are discussed.
Published Version: doi:10.4061/2011/983942
Other Sources: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3335494/pdf/
Terms of Use: This article is made available under the terms and conditions applicable to Other Posted Material, as set forth at http://nrs.harvard.edu/urn-3:HUL.InstRepos:dash.current.terms-of-use#LAA
Citable link to this page: http://nrs.harvard.edu/urn-3:HUL.InstRepos:10361987
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