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Stapes surgery in osteogenesis imperfecta patients

R Dieler1, J Müller, J Helms

  • 1Department of Otolaryngology-Head and Neck Surgery, University of Würzburg, Germany.

European Archives of Oto-Rhino-Laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
|January 1, 1997
PubMed
Summary

Osteogenesis imperfecta (OI) patients with mixed hearing loss can achieve significant hearing improvement through stapes surgery. This procedure offers predictable functional outcomes, comparable to those seen in otosclerosis patients.

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Area of Science:

  • Otolaryngology
  • Genetics
  • Orthopedics

Background:

  • Osteogenesis imperfecta (OI), also known as Van der Hoeve-de Kleyn syndrome, is a group of connective tissue disorders characterized by bone fragility.
  • Key OI symptoms include hearing impairment, spontaneous bone fractures, and blue sclerae.
  • Middle ear pathologies in OI patients often involve stapes fixation and fractures.

Purpose of the Study:

  • To evaluate the efficacy and functional predictability of stapes surgery in patients with Osteogenesis imperfecta (OI).
  • To compare surgical outcomes in OI patients with those of otosclerosis patients.

Main Methods:

  • Retrospective analysis of eight ears from six OI patients undergoing stapes surgery between 1988 and 1994.
  • Surgical procedures included partial stapedectomy or neo-window creation.

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  • Pre- and postoperative hearing thresholds, particularly air-bone gap, were analyzed.

    • Main Results:

    • No significant difference was observed in bone conduction thresholds before and after surgery.
    • Postoperative air-bone gap values in the speech frequency range averaged below 10 dB.
    • Functional results were comparable to those of 857 otosclerosis patients operated on during the same period.

    Conclusions:

    • Stapes surgery is a viable option for improving hearing in Osteogenesis imperfecta patients.
    • The functional predictability of stapes surgery in OI is similar to that in otosclerosis.
    • Surgical intervention can effectively address mixed hearing loss in OI, despite differing underlying etiologies.