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Auditory and temporal bone abnormalities in CHARGE association.

C G Wright, O E Brown, W L Meyerhoff

    The Annals of Otology, Rhinology, and Laryngology
    |September 1, 1986
    PubMed
    Summary
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    CHARGE association, a condition causing multiple birth defects, is linked to severe inner ear malformations. This study reveals significant temporal bone abnormalities, including middle ear defects and cochlear malformations, contributing to hearing loss in affected individuals.

    Area of Science:

    • Otolaryngology
    • Genetics
    • Developmental Biology

    Background:

    • CHARGE association is a complex congenital disorder with diverse anomalies.
    • Congenital hearing loss is recognized in CHARGE association, but underlying temporal bone pathology remains poorly understood.

    Observation:

    • Four patients with CHARGE syndrome were evaluated, including two survivors with severe bilateral hearing loss.
    • Autopsy examination of temporal bones from two non-surviving patients was performed.

    Findings:

    • All four patients exhibited severe middle ear defects: ossicular deformities, absent stapedius muscle, absent oval window, aberrant facial nerve course, and facial nerve canal dehiscence.
    • One patient presented with a Mondini-type cochlear malformation and vestibular anomalies.
    • The other patient showed vestibular defects but normally developed cochleae.

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    Implications:

    • This study provides the first detailed histologic description of temporal bone anomalies in CHARGE syndrome.
    • Findings highlight the structural basis for hearing loss in CHARGE association, emphasizing middle and inner ear malformations.
    • Understanding these defects is crucial for diagnosing and managing hearing impairment in CHARGE syndrome patients.