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Hearing loss (HL) is the most common sensory disorder worldwide and arises from a heterogeneous set of genetic and environmental etiologies. Currently, therapy for sensorineural HL is non-specific and limited to amplification devices and implanted neuroprosthetics. Recent advances in the burgeoning field of precision medicine focused on individualizing disease diagnosis and tailoring treatment to each patient’s own biology hold great promise to provide tailored care for hearing loss patients. In this review, we discuss the current diagnostic algorithm and treatment options for hearing loss, the advances in using precision medicine tools to identify specific genetic variants that predispose to or result in hearing loss, the role of genetics in determining surgical outcomes following cochlear implantation, recent advances in gene and stem cell therapies for treating hearing loss, and patient-specific disease modeling using induced pluripotent stem cells. All of these precision medicine techniques will result in improved care for patients based on the precise etiology of their hearing loss.