Superior semicircular canal dehiscence simulating otosclerosis

J Laryngol Otol. 2003 Jul;117(7):553-7. doi: 10.1258/002221503322113003.

Abstract

This is a report of a patient with an air-bone gap, thought 10 years ago to be a conductive hearing loss due to otosclerosis and treated with a stapedectomy. It now transpires that the patient actually had a conductive hearing gain due to superior semicircular canal dehiscence. In retrospect for as long as he could remember the patient had experienced cochlear hypersensitivity to bone-conducted sounds so that he could hear his own heart beat and joints move, as well as a tuning fork placed at his ankle. He also had vestibular hypersensitivity to air-conducted sounds with sound-induced eye movements (Tullio phenomenon), pressure-induced nystagmus and low-threshold, high-amplitude vestibular-evoked myogenic potentials. Furthermore some of his acoustic reflexes were preserved even after stapedectomy and two revisions. This case shows that if acoustic reflexes are preserved in a patient with an air-bone gap then the patient needs to be checked for sound- and pressure-induced nystagmus and needs to have vestibular-evoked myogenic potential testing. If there is sound- or pressure-induced nystagmus and if the vestibular-evoked myogenic potentials are also preserved, the problem is most likely in the floor of the middle fossa and not in the middle ear, and the patient needs a high-resolution spiral computed tomography (CT) of the temporal bones to show this.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Bone Conduction / physiology
  • Diagnosis, Differential
  • Evoked Potentials, Auditory
  • Hearing Disorders / etiology*
  • Hearing Disorders / physiopathology
  • Humans
  • Labyrinth Diseases / complications
  • Labyrinth Diseases / diagnosis*
  • Labyrinth Diseases / physiopathology
  • Male
  • Otosclerosis / diagnosis*
  • Otosclerosis / physiopathology
  • Reflex, Vestibulo-Ocular / physiology
  • Semicircular Canals* / physiopathology
  • Sound
  • Tomography, Spiral Computed / methods
  • Vestibular Function Tests
  • Vestibule, Labyrinth / physiopathology