Etiology of syndromic and nonsyndromic sensorineural hearing loss

Abstract

The past 10 years have seen an explosive gain in our understanding of molecular mechanisms of hearing and deafness. This has already resulted in improved diagnosis for the population with hereditary hearing loss. For syndromic hearing loss, we will see a shift from the historical terminology to a more precise genetic definition based on specific genetic abnormality. Functional studies of nonsyndromic deafness genes will elucidate the complex functional and hemostatic mechanisms in the inner ear. Ultimately, availability of gene therapy for the affected patients will bring to closure the circle of detection, identification, and correction of the disease.