Temporal Bone Study of Trisomy 13 Syndrome

Authors

  • Hisaki Fukushima MD,

    1. Department of Otolaryngology, University of Minnesota, Minneapolis, Minnesota, U.S.A.
    2. International Hearing Foundation, Minneapolis, Minnesota, U.S.A.
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  • Patricia A. Schachern BS,

    1. Department of Otolaryngology, University of Minnesota, Minneapolis, Minnesota, U.S.A.
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  • Sebahattin Cureoglu MD,

    Corresponding author
    1. Department of Otolaryngology, University of Minnesota, Minneapolis, Minnesota, U.S.A.
    2. International Hearing Foundation, Minneapolis, Minnesota, U.S.A.
    • Sebahattin Cureoglu, MD, Room 226 Lions Research Building 2001 6th St. S.E., Minneapolis, MN 55455, U.S.A.
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  • Michael M. Paparella MD

    1. Department of Otolaryngology, University of Minnesota, Minneapolis, Minnesota, U.S.A.
    2. Minnesota Ear Head and Neck Clinic, Minneapolis, Minnesota, U.S.A.
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  • This work was supported in part by National Institutes of Health- National Institute on Deafness and Other Communication Disorders Grant P30 DC4660, the Lions International of Minnesota, and the International Hearing Foundation.

Abstract

The temporal bones of a 23-week-old female who had multiple congenital anomalies and bilateral hearing loss with trisomy 13 syndrome were evaluated under light microscopy.

In the left cochlea, spiral ganglion cells and cochlear nerve fibers are absent. In the right ear, an abnormal branch of the singular nerve passes between the utricle and lateral semicircular canals and separates the two portions, one to the flattened crista of the lateral semicircular canal, the other to the utricular macula. This is a rare report describing a patient with abnormalities in three nerves, including the facial, vestibular, and cochlear nerves.

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