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J Laryngol Otol. 2008 Mar;122(3):e6. doi: 10.1017/S0022215107001466. Epub 2008 Feb 18.

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

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  • 1Department of Otorhinolaryngology, Head, Neck and Plastic Facial Surgery, Klinikum Bad Hersfeld, Germany. Ingo.Ott@online.de



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.


Case report and literature review.


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'.


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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