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Hearing impairment in Engelmann disease

K Higashi1, C Matsuki

  • 1Department of Otolaryngology, Akita University, Japan.

The American Journal of Otology
|January 1, 1996
PubMed
Summary

Engelmann disease, a skull hyperostosis, can cause hearing loss due to bony encroachment. This case highlights the potential underreporting of hearing impairment in patients with this rare condition.

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Area of Science:

  • Medical research
  • Genetics
  • Otolaryngology

Background:

  • Skull hyperostosis diseases can manifest with hearing impairment.
  • Engelmann disease is a rare condition with over 130 reported cases.
  • Hearing loss may be underreported in Engelmann disease literature.

Purpose of the Study:

  • To present a case of Engelmann disease.
  • To discuss the association between Engelmann disease and hearing impairment.
  • To explore the potential underestimation of hearing loss prevalence in this condition.

Main Methods:

  • Case report presentation.
  • Literature review of Engelmann disease cases.
  • Analysis of reported symptoms, focusing on auditory complications.

Main Results:

  • The presented case involves Engelmann disease.
  • Approximately 18% of reported Engelmann disease cases include hearing impairment.
  • Potential for higher prevalence of hearing loss due to reporting bias.

Conclusions:

  • Deafness in Engelmann disease results from narrowing of auditory canals, affecting nerves and vessels.
  • Progressive perceptive hearing loss and serous otitis media are potential outcomes.
  • Further investigation into auditory function is warranted for comprehensive patient assessment.

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