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Temporal Bone Study of Down's Syndrome
Haluk Bilgin, MD;
Lalida Kasemsuwan, MD;
Patricia A. Schachern;
Michael M. Paparella, MD;
Chap T. Le, PhD
Arch Otolaryngol Head Neck Surg. 1996;122(3):271-275.
Abstract
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Objective To study temporal bone histopathologic characteristics of the inner ear and middle ear cleft of patients with Down's syndrome.
Design Sixteen temporal bones from eight patients with Down's syndrome were studied. Ten temporal bones from subjects without pathologic ear lesions but with congenital heart disease served as controls. The two-dimensional graphic reconstruction method proposed by Guild and modified by Schuknecht was used to study the cochleas; measurement of the vestibules was based on Igarashi's method.
Setting The temporal bone collection of the Otitis Media Research Center, Department of Otolaryngology, University of Minnesota School of Medicine, Minneapolis.
Results Six bones showed short cochlea, and four of six had Mondini's cochlea. The overall cochlear lengths in this study group were notably shorter than those of the controls. The spiral ganglion cell population and two of three vestibular dimensions also were notably less than the controls. Middle ear findings included residual mesenchyme, stapes abnormality, otitis media, and large facial canal dehiscence.
Conclusions The difficulties encountered in rehabilitation of patients with Down's syndrome caused by mental retardation can be compounded by the hearing loss caused by middle and inner ear abnormalities. A complete evaluation of hearing loss and therapy before starting the rehabilitation gives the most favorable outcome.
(Arch Otolaryngol Head Neck Surg. 1996;122:271-275)
Author Affiliations
From the Otitis Media Research Center, Department of Otolaryngology (Drs Bilgin, Kasemsuwan, and Paparella and Ms Schachern) and the School of Medicine, Department of Biostatistics (Dr Le), University of Minnesota School of Public Health, and the Minnesota Ear, Head, and Neck Clinic (Dr Paparella), Minneapolis.
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ABSTRACT
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