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


METHODS
Case report and literature review.


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'.


CONCLUSIONS
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.
Ott
I O
IO
Department of Otorhinolaryngology, Head, Neck and Plastic Facial Surgery, Klinikum Bad Hersfeld, Germany. Ingo.Ott@online.de
Issing
P R
PR
D000015:Abnormalities, Multiple; D000293:Adolescent; D001301:Audiometry, Pure-Tone / Q000379:methods; D004429:Ear Ossicles / Q000002:abnormalities; D006310:Hearing Aids; D006312:Hearing Loss, Bilateral / Q000150:complications* / Q000628:therapy; D006801:Humans; D017880:Limb Deformities, Congenital / Q000150:complications*; D008297:Male; D013577:Syndrome; D016896:Treatment Outcome