title: Bilateral, mixed hearing loss with a predominant sensorineural component in Larsen's syndrome.
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.
  
  
  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.
authors.affiliation: Department of Otorhinolaryngology, Head, Neck and Plastic Facial Surgery, Klinikum Bad Hersfeld, Germany. Ingo.Ott@online.de
authors.forename: I O
authors.initials: IO
authors.lastname: Ott
authors.forename: P R
authors.initials: PR
authors.lastname: Issing
mesh_terms: 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