XXXX -prevalence, distribution and hearing management protocol in Indian scenario.

@entity1 @entity2319 @entity1274 @entity393 @entity13526 @entity15499
BACKGROUND: The estimated prevalence of @entity1274 ( @entity1274 ) in @entity1 less than 18 years of age is 6 per 1000. Roughly 50% of cases of congenital @entity1274 can be linked to a genetic cause, with approximately 30% being syndromic and the remaining 70% being non-syndromic. The term "syndromic" implies the presence of other distinctive clinical features in addition to @entity393 . The aim of our study was to find the distribution of various Syndromic associations in @entity1 with profound @entity2319 , presented at Madras ENT Research foundation, Chennai and to formulate a management protocol for these @entity1 and to discuss in detail about the clinical features of commonly encountered @entity2319 . MATERIALS AND METHODS: Our retrospective study was aimed at describing the various Syndromic associations seen in @entity1 with congenital profound @entity2319 . Information was collected from the medical records. At our centre all @entity1 undergo a comprehensive evaluation. The distribution, etiological factors and management protocol for various syndromes are here presented. RESULTS: Out of 700 @entity1 with congenital profound @entity2319 all @entity1 with Syndromic associations (n  =  35) were studied. 5% of profoundly deaf candidates were found to be syndromic. Most common syndrome in our series was found to be @entity13526 followed by Jervell and @entity15499 . CONCLUSION: @entity2319 is an associated feature of many syndromes. Detailed history taking with comprehensive evaluation is mandatory to rule out the associated syndromes. Diagnosis must be confirm by a genetic study. Multidisciplinary approach is essential for appropriate diagnosis and management.
