<p>The sensory organs are ideally adapted to detecting environmental signals and are highly sensitive to a diversity of stimuli. Congenital hearing loss associated with dysfunction of hair cells, the receptors that perceive sound, is the most common sensory deficit in humans, and in most cases arises as a result of genetic causes. The significant role of genetics lies in the complex structure of the auditory analyzer and the multitude of highly specialized cells and proteins supporting hair cell function. Deletion or substitution of even a single nucleotide in the genes encoding these proteins can disrupt the functioning of the inner ear’s highly coordinated, high-precision system. The objective of the present article was to introduce readers to the molecular structure of the auditory analyzer, hereditary forms of hearing loss, and modern diagnostic and preventative measures for hereditary forms of hearing loss. The insidiousness of genetic causes lies in the birth of a child with congenital deafness to healthy parents whose pedigree is “not burdened,” but who are carriers of mutations in genes responsible for hearing impairment. These carrier states can now be detected even before the child’s birth thanks to the use of next-generation sequencing (NGS) methods. Prevention of severe hereditary diseases is gradually being introduced into the practice of medical and genetic counseling for healthy couples when planning to have children, while the development of preimplantation diagnosis methods within the framework of in vitro fertilization (IVF) programs allows couples at risk of hereditary diseases to have a healthy child.</p>