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 J Kimberling, Cor Cremers

Research output: Contribution to journalArticle

22 Citations (Scopus)

Abstract

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
Volume126
Issue number8
DOIs
StatePublished - Jan 1 2000

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Usher Syndromes
Temporal Bone
Stria Vascularis
Spiral Ganglion
Organ of Corti
Cochlear Nerve
Cochlea
Atrophy
Microscopy
Epithelium
Light

ASJC Scopus subject areas

  • Surgery
  • Otorhinolaryngology

Cite this

Wagenaar, M., Schuknecht, H., Nadol, J., Benraad-Van Rens, M., Pieke-Dahl, S., Kimberling, W. J., & 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

Histopathologic features of the temporal bone in usher syndrome type I. / Wagenaar, Mariette; Schuknecht, Harold; Nadol, Joseph; Benraad-Van Rens, Meeke; Pieke-Dahl, Sandra; Kimberling, William J; Cremers, Cor.

In: Archives of Otolaryngology - Head and Neck Surgery, Vol. 126, No. 8, 01.01.2000, p. 1018-1023.

Research output: Contribution to journalArticle

Wagenaar, M, Schuknecht, H, Nadol, J, Benraad-Van Rens, M, Pieke-Dahl, S, Kimberling, WJ & Cremers, C 2000, 'Histopathologic features of the temporal bone in usher syndrome type I', Archives of Otolaryngology - Head and Neck Surgery, vol. 126, no. 8, pp. 1018-1023. https://doi.org/10.1001/archotol.126.8.1018
Wagenaar M, Schuknecht H, Nadol J, Benraad-Van Rens M, Pieke-Dahl S, Kimberling WJ et al. Histopathologic features of the temporal bone in usher syndrome type I. Archives of Otolaryngology - Head and Neck Surgery. 2000 Jan 1;126(8):1018-1023. https://doi.org/10.1001/archotol.126.8.1018
Wagenaar, Mariette ; Schuknecht, Harold ; Nadol, Joseph ; Benraad-Van Rens, Meeke ; Pieke-Dahl, Sandra ; Kimberling, William J ; Cremers, Cor. / Histopathologic features of the temporal bone in usher syndrome type I. In: Archives of Otolaryngology - Head and Neck Surgery. 2000 ; Vol. 126, No. 8. pp. 1018-1023.
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