Histopathologic features of the temporal bone in usher syndrome type I

Mariette Wagenaar, Harold Schuknecht, Joseph Nadol, Meeke Benraad-Van Rens, Sandra Pieke-Dahl, William Kimberling, Cor Cremers

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22 Scopus citations


Temporal bones of 2 patients with Usher syndrome type I were examined using light microscopy. In both patients, findings from histopathologic examination of the cochlea were characterized by degeneration of the organ of Corti, which was most marked in the basal turn, atrophy of the stria vascularis, and a decrease in the number of spiral ganglion cells. The cochlear nerve appeared to be diminished. The sensory epithelium of the saccular and utricular maculae of patient 1 was normal for age. The left temporal bone of patient 2, classified as Usher syndrome genetic subtype USH1D or USH1F, demonstrated the typical signs of severe cochleosaccular degeneration. Present cases and cases from the literature were reviewed in search of an explanation for the above-described differences in histologic findings.

Original languageEnglish (US)
Pages (from-to)1018-1023
Number of pages6
JournalArchives of Otolaryngology - Head and Neck Surgery
Issue number8
StatePublished - 2000

ASJC Scopus subject areas

  • Surgery
  • Otorhinolaryngology

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    Wagenaar, M., Schuknecht, H., Nadol, J., Benraad-Van Rens, M., Pieke-Dahl, S., Kimberling, W., & Cremers, C. (2000). Histopathologic features of the temporal bone in usher syndrome type I. Archives of Otolaryngology - Head and Neck Surgery, 126(8), 1018-1023. https://doi.org/10.1001/archotol.126.8.1018