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Temporal bone histopathological findings in campomelic dysplasia

  • H. Takahashi (a1), I. Sando (a1) and H. Masutani (a1)

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.

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Corresponding author

Dr I. Sando, Division of Otopathology, Eye & Ear Institute of Pittsburgh, 203 Lothrop Street, Pittsburgh, PA 15213.

References

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Keywords

Temporal bone histopathological findings in campomelic dysplasia

  • H. Takahashi (a1), I. Sando (a1) and H. Masutani (a1)

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