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Hearing loss is one of the most prevalent genetic disorders in humans. Locus and allelic heterogeneity cause fundamental challenges in hearing loss genetic diagnosis and management of patients and their families. This study examined the genetic profile of patients with prelingual hearing loss who were referred to the Genetic Foundation of Khorasan Razavi spanning over a decade. Deleterious variants in GJB2 were evaluated through Sanger sequencing among 745 non‐syndromic hearing loss patients. Furthermore, exome sequencing was applied in 250 patients with negative GJB2 sequencing results and 30 patients with syndromic hearing loss. The findings revealed a relatively low frequency of GJB2 variants among the studied patients. Exome sequencing successfully identified the genetic causes of hearing loss in 70% of the patients. Moreover, variants in 10 genes, namely SLC26A4, MYO15A, TMPRSS3, TMC1, OTOF, CDH23, PJVK, MYO7A, TECTA, and PCDH15, accounted for 66% of the positive exome sequencing findings in this study. At least three prevalent founder alleles in the hearing‐impaired population of eastern Iran were identified. This study emphasizes the efficiency of exome sequencing as a powerful tool for determining the etiology of prelingual hearing loss in the eastern Iranian population. [ABSTRACT FROM AUTHOR]

Objective: Biological, structural, and acoustical constraints faced by cochlear implant (CI) users can alter the perception of music. Bimodal fitting not only provides bilateral hearing but can also improve auditory skills. This study was conducted to assess the impact of this amplification style on the emotional perception of music among children with hearing loss (HL). Methods: Twenty-five children with congenital severe to profound HL and unilateral CIs, 20 children with bimodal fitting, and 30 children with normal hearing participated in this study. Their emotional perceptions of music were measured using a method where children indicated happy or sad feelings induced by music by pointing to pictures of faces showing these emotions. Results: Children with bimodal fitting obtained significantly higher mean scores than children with unilateral CIs for both happy and sad music items and in overall test scores (P < .001). Both groups with HL obtained significantly lower scores than children with normal hearing (P < .001). Conclusions: Bimodal fitting results in a better emotional perception of music compared to unilateral CI. Given the influence of music in neurological and linguistic development and social interactions, it is important to evaluate the possible benefits of bimodal fitting prescriptions for individuals with unilateral CIs. [ABSTRACT FROM AUTHOR]