Background

Hearing loss is a common sensory defect, affecting ap-proximately one in 500 to 1000 newborns [1]. Approxi-mately 50 % of congenital hearing loss cases and 70 % of childhood hearing loss cases are attributed to genetic mutations [1]. The remaining 50 % of congenital cases are attributable to other factors such as prenatal exposure to measles, cytomegalovirus, premature birth, and new-born meningitis. Genetic tests for hereditary hearing loss assist in the clinical management of patients and can pro-vide the first step in the development of therapeutics [2]. For example, early diagnosis of Usher syndrome, which comprises congenital hearing loss and late-onset retinitis pigmentosa, provides important information to choose communication modalities. However, causes of hereditary hearing loss are highly heterogeneous; more than 60 gene