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Anomalies

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Morphological Aspects of Inner Ear Disease

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Abstract

This chapter discusses cochlear hyperplasia, hypophosphatasia, Alagille syndrome, Scheibe dysplasia, and Mondini dysplasia. Anomalies of the inner ear usually derive from arrested embryonic organ development. Cochlear hyperplasia results from excessive development of the organ of Corti and cochlear duct. Because the bony cochlea is normal in shape, the overdeveloped cochlear duct with the organ of Corti reverses at its apical end and heads backwards into the scala tympani, producing a mirror image of the organ of Corti. The case of hypophosphatasia described here showed immature ossification of the bony labyrinth with abnormal tissue formation in the lateral wall of the membranous cochlea. The parents of this patient were consanguineous and their sera showed low alkaline phosphatase activity. Alagille syndrome is a systemic malformation, in which the posterior semicircular canal shows a partial deficit. In cases of Scheibe dysplasia the bony labyrinth develops normally, but the cochleosaccular portion of the membranous labyrinth is dysplastic. Mondini dysplasia is relatively common, but no two cases are identical in detail.

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Authors and Affiliations

  1. The Society for Promotion of International Oto-Rhino-Laryngology, Tokyo, Japan

    Yasuya Nomura (President)

  2. The University of Tokyo, Tokyo, Japan

    Yasuya Nomura (Professor Emeritus)

  3. Showa University, Tokyo, Japan

    Yasuya Nomura (Visiting Professor)

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  1. Yasuya Nomura
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Nomura, Y., Nomura, Y., Nomura, Y. (2014). Anomalies. In: Morphological Aspects of Inner Ear Disease. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54204-9_9

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  • DOI: https://doi.org/10.1007/978-4-431-54204-9_9

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  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-54203-2

  • Online ISBN: 978-4-431-54204-9

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