Section 5

30-year-old female presents with reduced hearing in her ears following recent pregnancy.


Describe the above pure tone audiogram

Left sided conductive hearing loss of 30dB with Cahart’s notch at 2kHz

What is the diagnosis?

Otosclerosis

What may you see on otoscopy?

Schwartz sign – pink tinge of cochlear promontory due to increased vascularity

What other audiometric investigation may help your diagnosis?

Stapedial reflexes

Describe 2 possible treatment options.

  • Hearing aids
  • Stapedotomy
  • Bone anchored hearing aid (BAHA)

Supporting Information

Otosclerosis – Replacement of mature bone of the otic capsule with woven bone.

  • Autosomal dominant 85% bilateral- metabolic bone disease. 
  • Foci most common anterior to oval window
  • M:F = 2:1
  • Symptoms occur when the stapes footplate is fixed. Prevalence –  0.5-2% of population
    • Deafness – conductive due to reduced stapes footplate mobility
    • Tinnitus
    • Vertigo
    • Paracusis willisii – hearing better in noisy surroundings
    • Schwartz’s sign – pink tinge on tympanic membrane – dilated vessels of promontory
    • Carhart notch – dip at 2000 Hz conductive loss
    • Good speech discrimination
  • Investigation – tympanogram (effusion), PTA (Carhart notch), CT (surgical planning), stapedial reflexes (absent in otosclerosis) and speech audiogram to see if cochlear is involved (poor speech discrimiation = cochlear involvement). 
  • Management:
    • Hearing aid + observe
    • Medical – sodium fluoride + bisphosphonates
    • Stapedectomy if loss is over 15dB conductive and 60% speech discrimination- teflon piston
      • Risks – bleeding, dead ear, facial nerve palsy, perilymph fistula, vertigo, tinnitus, prosthetic displacement, necrosis of long process of incus. 
    • Women who haven’t completed family should avoid it as it may progress in pregnancy

Stapedotomy

  • Involves – creation of fenestra to remove fixed portion of the stapes footplate which may be:
  • Small – formed with micro-instruments or laser
  • Large – removal of a large part of the footplate

Oval window sealed with vein graft. A prosthesis is inserted to fill air/bone gap. 

  • Risks – dead ear, vertigo, alteration/loss of taste, facial nerve damage. 
  • Complications – tympanosclerosis, floating or depressed footplate, saccular injury, perilymph fistula, prosthetic displacement, necrosis of incus. 
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