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1 results

  • Bilateral, mixed hearing loss with a predominant sensorineural component in Larsen's syndrome

  • I O Ott, P R Issing
  • Journal:
    The Journal of Laryngology & Otology / Volume 122 / Issue 3 / March 2008
    Published online by Cambridge University Press:
    18 February 2008, e6
    Print publication:
    March 2008
  • Background:

    Larsen's syndrome is primarily known as an orthopaedic disease. However, it has been found to be associated with conductive hearing loss caused by ossicular malformation. The possibility of a sensorineural hearing impairment as an additional part of this syndrome has been rather neglected in the past.

    Method:

    Case report and literature review.

    Patient and results:

    We present a teenage boy suffering from typical Larsen's syndrome. Despite no history of recurrent middle-ear infections, he showed a bilaterally symmetrical, mixed hearing loss dominated by an extensive sensorineural component. In order to review the corresponding literature, a Medline search was performed using the criteria ‘Larsen's syndrome’ and ‘hearing loss/deficit’.

    Conclusion:

    The literature review showed the presented case to be the first sufficiently documented report describing a predominant sensorineural hearing loss in a Larsen's syndrome patient. Although a specific association of Larsen's syndrome with sensorineural hearing loss is still speculative, this case might give grounds for further examinations of this rare finding.