I O Ott1, P R Issing. 1. Department of Otorhinolaryngology, Head, Neck and Plastic Facial Surgery, Klinikum Bad Hersfeld, Germany. Ingo.Ott@online.de
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.
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 syndromepatient. 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: A M Waryah; M Shahzad; H Shaikh; S A Sheikh; N A Channa; R B Hufnagel; A Makhdoom; S Riazuddin; Z M Ahmed Journal: Clin Genet Date: 2015-12-21 Impact factor: 4.438