<p class="abstract"><strong><span lang="EN-US">Background: </span></strong>Knowledge about the association of family history and consanguinity with congenital hearing loss is vital in order to prevent occurrence of such morbidity in future generations. The aim of the study was to find the strength of association between family history and consanguinity and congenital hearing loss.</p><p class="abstract"><strong><span lang="EN-US">Methods: </span></strong>A case control study was done using 50 congenitally deaf children and 50 children with normal hearing. A detailed history was taken from the parents with regard to family history of hearing loss and consanguinity.</p><p class="abstract"><strong><span lang="EN-US">Results: </span></strong>In the case group, 28% children had a family history of hearing loss and in the control group; none had a family history of hearing loss. In the case group, 48% of the children had parents with consanguinity. In the control group, 28% of the children had parents with consanguinity.</p><p class="abstract"><strong><span lang="EN-US">Conclusions: </span></strong>Family history and consanguinity play significant role in children having congenital hearing loss. Hence, avoidance of consanguineous marriages and pre-marital genetic counselling can prevent congenital hearing loss.</p>