Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
  1. Home
  2. Search

Refine search

Refine search

Access:
Content type:
Publication date:
Apply   From and To filters
Subject: Show more
Journals Show more
Publishers: Show more
Societies Show more
Series: Show more
Collections: Show more

Actions for selected content:

Select all | Deselect all
  • View selected items
  • Export citations
  • Download PDF (zip)
  • Save to Kindle
  • Save to Dropbox
  • Save to Google Drive

Save Search

You can save your searches here and later view and run them again in "My saved searches".

Please provide a title, maximum of 40 characters.
Cancel
×

1 results

  • Two siblings with progressive, fluctuating hearing loss after head trauma, treated with cochlear implantation

  • M J F de Wolf, J Honings, F B M Joosten, L Hoefsloot, E A M Mylanus, C W R J Cremers
  • Journal:
    The Journal of Laryngology & Otology / Volume 124 / Issue 1 / January 2010
    Published online by Cambridge University Press:
    23 June 2009, pp. 86-89
    Print publication:
    January 2010
  • Objective:

    Description of two siblings with unexplained, progressive, perceptive hearing loss after head trauma.

    Design:

    Case report.

    Subjects:

    Two siblings aged six and eight years old with bilateral, intermittent but progressive hearing loss.

    Results:

    These patients had a c.1172G>A (p.Ser391Asn) mutation in the SLC26A4 gene, which has not previously been reported and which caused Pendred or enlarged vestibular aqueduct syndrome. The diagnosis was based on the perceptive hearing loss, computed tomography findings and mutation analysis. The patients were each fitted with a cochlear implant because of their severe, progressive, perceptive hearing loss with deep fluctuations. The results were good.

    Conclusion:

    Further testing for the presence of an enlarged vestibular aqueduct is recommended when children present with sudden progression in perceptive hearing loss, whether or not in combination with head trauma. Cochlear implantation is indicated in patients with persistent, progressive hearing loss that leads to deafness. Implantation can be undertaken successfully despite cochlear hypoplasia.