Audiologic features of the X-linked progressive mixed deafness syndrome with perilymphatic gusher during stapes gusher

Am J Otol. 1985 May;6(3):243-6.


Stapes gusher sometimes occurs at the moment the vestibule is opened. A flood of perilymph can be expected as a complication of stapes surgery in all males affected with the X-linked progressive mixed deafness syndrome. Audiologic features, such as a mixed hearing loss and elicitability of stapedial reflexes, are reported for eight affected males in a large Dutch family.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Aged
  • Child
  • Female
  • Hearing Loss / genetics*
  • Hearing Loss, Bilateral / diagnosis
  • Hearing Loss, Bilateral / genetics*
  • Humans
  • Male
  • Middle Aged
  • Pedigree
  • Perilymph / physiology
  • Sex Chromosome Aberrations / genetics*
  • Stapes Surgery / adverse effects
  • Syndrome
  • X Chromosome*