International Journal of Pediatric Otorhinolaryngology Extra
Volume 6, Issue 1 , Pages 23-29 , January 2011

Auditory testing profiles of Pelizaeus-Merzbacher disease

  • James M. Coticchia

      Affiliations

    • Department of Otolaryngology-Head and Neck Surgery, Wayne State University School of Medicine, 540 E. Canfield, 5E-UHC, Detroit, MI 48201, United States
    • Corresponding Author InformationCorresponding author. Tel.: +1 248 357 4151.
    • ,
  • M.A. Diane Roeder

      Affiliations

    • Department of Otolaryngology-Head and Neck Surgery, Wayne State University School of Medicine, 540 E. Canfield, 5E-UHC, Detroit, MI 48201, United States
    • ,
  • Giancarlo F. Zuliani

      Affiliations

    • Department of Otolaryngology-Head and Neck Surgery, Wayne State University School of Medicine, 540 E. Canfield, 5E-UHC, Detroit, MI 48201, United States
    • ,
  • Alexander Gow

      Affiliations

    • Department of Molecular Medicine and Genetics, Wayne State University School of Medicine, United States
    • ,
  • James Y. Garbern

      Affiliations

    • Department of Neurology, Wayne State University School of Medicine, United States

Received 1 September 2009 ,Revised 24 December 2009 ,Accepted 5 January 2010.

  • Image Result

    ABR results from subject A, a 9-year-old male with mild symptoms of the disease. Waves I and II were present in both ears and the absolute latencies of Wave I were within normal limits. Questionable w

    ABR results from subject A, a 9-year-old male with mild symptoms of the disease. Waves I and II were present in both ears and the absolute latencies of Wave I were within normal limits. Questionable wave Vs were present in both ears. Red is right ear; blue is left ear.

  • Image Result
    ABR results from subject C, a 5-year-old male, who had severe developmental delay. Only Wave I was present in both ears with normal absolute latencies. No other repeatable waveforms were present.

    ABR results from subject C, a 5-year-old male, who had severe developmental delay. Only Wave I was present in both ears with normal absolute latencies. No other repeatable waveforms were present.

  • Image Result
    ABR results are from subject F, a 42-year-old male with moderate symptom severity. Waves I, III, and V were present, but all absolute and interpeak latencies were delayed.

    ABR results are from subject F, a 42-year-old male with moderate symptom severity. Waves I, III, and V were present, but all absolute and interpeak latencies were delayed.

  • Image Result
    A T2-weighted MRI of subject F, a 42-year-old male who has moderate symptom severity. Note the increased white matter signal as well as the diffuse atrophy.

    A T2-weighted MRI of subject F, a 42-year-old male who has moderate symptom severity. Note the increased white matter signal as well as the diffuse atrophy.

☆ Poster presentation at the American Academy of Otolaryngology-Head and Neck Surgery; September 16–20, 2006; Toronto, Canada.

PII: S1871-4048(10)00003-1

doi: 10.1016/j.pedex.2010.01.001

International Journal of Pediatric Otorhinolaryngology Extra
Volume 6, Issue 1 , Pages 23-29 , January 2011

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