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Eighth cranial nerve dysfunction in hyperostosis cranialis interna.

J J Manni1, P L Huygen, J F Noten

  • 1Department of Otolaryngology, University Hospital Nijmegen, The Netherlands.

Acta Oto-Laryngologica
|January 1, 1992
PubMed
Summary
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Hyperostosis cranialis interna, a skull bone disorder, can cause cranial nerve issues. This study tracked auditory and vestibular functions in affected individuals, revealing progressive hearing loss and nerve involvement.

Area of Science:

  • Genetics and Neurology
  • Ophthalmology and Otolaryngology

Background:

  • Hyperostosis cranialis interna (HCI) is an autosomal dominant bone disorder.
  • It is characterized by skull hyperostosis and osteosclerosis, particularly in the calvarium and skull base.

Observation:

  • Variable involvement of cranial nerves I (olfactory), II (optic), VII (facial), and VIII (vestibulocochlear) was observed in affected family members.
  • Auditory and vestibular functions were monitored in three young individuals over eight years.
  • Initial audiograms were normal, but progressive hearing loss and abnormal brainstem auditory-evoked responses (BAERs) were noted.

Findings:

  • One case developed severe hearing loss and deafness in one ear, with transient issues in the other.
  • Abnormal BAERs, with preserved wave I and later return of wave V with prolonged I-V intervals, indicated nerve dysfunction.

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  • Facial nerve paralysis and diminished caloric responses were also observed, correlating with nerve entrapment.

    • Implications:

    • Nerve entrapment due to cranial bone changes is a likely mechanism for neurological deficits in HCI.
    • Long-term audiological and vestibular monitoring is crucial for early detection and management of cranial nerve involvement.
    • Understanding these progressive neurological complications can guide future therapeutic strategies for HCI patients.