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Temporal bone histopathological findings in campomelic dysplasia

Published online by Cambridge University Press:  29 June 2007

H. Takahashi ,
I. Sando  and
H. Masutani
H. Takahashi
Affiliation:
Elizabeth McCullough Knowles Otopathology Laboratory, Division of Otopathology, Department of Otolaryngology, University of Pittsburgh School of Medicine, Eye and Ear Institute of Pittsburgh.
I. Sando*
Affiliation:
Elizabeth McCullough Knowles Otopathology Laboratory, Division of Otopathology, Department of Otolaryngology, University of Pittsburgh School of Medicine, Eye and Ear Institute of Pittsburgh.
H. Masutani
Affiliation:
Elizabeth McCullough Knowles Otopathology Laboratory, Division of Otopathology, Department of Otolaryngology, University of Pittsburgh School of Medicine, Eye and Ear Institute of Pittsburgh.
*
Dr I. Sando, Division of Otopathology, Eye & Ear Institute of Pittsburgh, 203 Lothrop Street, Pittsburgh, PA 15213.
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Abstract

Both temporal bones of a newborn (35 gestational weeks old) with campomelic syndrome were studied histopathologically. This is to our knowledge the second temporal bone report (third case) of this syndrome. The findings included: abnormal cartilagenous and osseous tissues and abnormality in the globuli interossei in the otic capsule; deformities of the vestibule and semicircular canals, probably due to compression by the abnormal cartilaginous tissue; hypoplastic cochleaand semicircular canals; aberrant course of the facial nerve; wide dehiscence of the facial canal in the tympanic portion; slight hypoplasia of the malleus and anomalies in the incus and stapes; and large epitympanic space. These findings closely resembled those of the first report, and suggest that: 1) campomelic dysplasia is a definite disease entity with consistent pathogenesis, and 2) similar otologic manifestations may be expected in the majority of patients with this syndrome.

Keywords

Temporal boneHistologyDysplasia, compomelic
Type
Clinical Records
Information
The Journal of Laryngology & Otology , Volume 106 , Issue 4 , April 1992 , pp. 361 - 365
Copyright
Copyright © JLO (1984) Limited 1992

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