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  • Title: Bilateral, mixed hearing loss with a predominant sensorineural component in Larsen's syndrome.
    Author: Ott IO, Issing PR.
    Journal: J Laryngol Otol; 2008 Mar; 122(3):e6. PubMed ID: 18279569.
    Abstract:
    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.
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