background it seems that there is a relationship between consanguinity and profound hearing loss but there is little data about the association of consanguinity and hearing loss in iran. objectives the aim of this study is to demonstrate the causes of profound bilateral sensorineural hearing loss among iranian samples who are candidates for cochlear implantation. methods this study was retrospe...

Different strains of mice provide a valuable research tool for studying both hereditary and acquired forms of deafness. The cd/1 strain has been found to demonstrate hereditary cochlear pathology. The characteristics of hearing loss in cd/1 mice have not previously been reported. In this investigation auditory thresholds were obtained by measuring evoked brain stem responses in subjects of thre...

1 Recent advances in basic science, technology and treatments have increased our abilities to manage hearing and balance diseases more effectively. Advances in molecular genetics have allowed better understanding of congenital and hereditary hearing loss. The advent of regenerative medicine led to auditory hair cell regeneration in laboratory animals. Advances in hearing aids, cochlear implants...

Molecular diagnostic testing of individuals with congenital sensorineural hearing loss typically begins with DNA sequencing of the GJB2 gene. If the cause of the hearing loss is not identified in GJB2, additional testing can be ordered. However, the step-wise analysis of several genes often results in a protracted diagnostic process. The more comprehensive Hereditary Hearing Loss Arrayed Primer...

Mutations in the Connexin 26 (Cx26) gene are a common cause of hereditary hearing loss in different populations. In the present study, an Iranian patient with bilateral hearing loss underwent molecular analysis for the causative mutation. DNA studies were performed for the Cx26 gene by PCR and sequencing methods. We describe a novel compound heterozygous mutation (35delG, 363delC) in the Cx26 g...

Understanding the genetic basis of hearing loss is important because almost 50% of profound hearing loss are caused by genetic factors and more than 120 independent genes have been identified. In this review, after a brief explanation of some genetic terms (allele, heterozygosis, homozygosis, polymorphism, genotype and phenotype), classification of genetic hearing loss (syndromic versus nonsynd...