X-linked progressive mixed deafness with perilymphatic gusher during stapes surgery

Arch Otolaryngol. 1985 Apr;111(4):249-54. doi: 10.1001/archotol.1985.00800060073010.


Stapes gusher is a rare and usually unexpected complication of stapes surgery. This complication will inevitably be encountered during stapes surgery in all affected males with the X-linked, progressive mixed deafness syndrome. The opportunity of studying eight affected males in a large Dutch family with audiometry, vestibulometry, and polytomography was used to identify specific features. Awareness of these features will assist the otologist in recognizing new cases preoperatively.

MeSH terms

  • Child
  • Ear / diagnostic imaging
  • Female
  • Genetic Linkage
  • Hearing Loss / genetics*
  • Hearing Loss, Bilateral / diagnostic imaging
  • Hearing Loss, Bilateral / genetics*
  • Hearing Loss, Bilateral / physiopathology
  • Hearing Loss, Bilateral / surgery
  • Humans
  • Labyrinthine Fluids / physiology*
  • Male
  • Pedigree
  • Perilymph / physiology*
  • Stapes / physiopathology
  • Stapes Surgery / adverse effects*
  • Tomography, X-Ray Computed
  • Vision Disorders / genetics
  • X Chromosome*