Temporal bone histopathological study of Noonan syndrome

Makoto Miura, Isamu Sando, Yorihisa Orita, Barry E. Hirsch

Research output: Contribution to journalArticle

10 Citations (Scopus)

Abstract

Four temporal bone specimens, obtained from three individuals 1-6 years of age with Noonan syndrome (NS), were studied histopathologically. All four specimens were accompanied by similar inner ear abnormalities including the reduced number of spiral ganglion cells, enlarged lateral semicircular canal, and dislocated endolymphatic sac and vestibular aqueduct. The mean population of spiral ganglion cells (15 699 cells) was approximately half of those (32 978 cells) in four age-matched control cases. In addition, they had several middle ear abnormalities including the remaining mesenchyme and dehiscence of the facial canal. To our knowledge, this is the first report to describe the histopathological temporal bone findings in patients with NS. We discuss the implications of the observed abnormalities with regard to clinical issues.

Original languageEnglish
Pages (from-to)73-82
Number of pages10
JournalInternational Journal of Pediatric Otorhinolaryngology
Volume60
Issue number1
DOIs
Publication statusPublished - Jul 30 2001

Keywords

  • Anomaly
  • Histopathology
  • Noonan syndrome
  • Spiral ganglion cell
  • Temporal bone

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Otorhinolaryngology

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