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Although hearing loss is a well-known symptom of mitochondria-related disorders, it is not clear how often it is a congenital and cochlear impairment. The Newborn Hearing Screening Program (NHSP) enables to distinguish congenital cochlear deafness from an acquired hearing deficit. The initial aim of the study was to research the frequency of the congenital cochlear hearing loss among patients with various gene defects resulting in mitochondrial disorders. The research process brought on an additional gain: basing on our preliminary study group of 80 patients, in 12 patients altogether we identified two defected genes responsible for mitochondrial disorders, whose carriers did not pass the NHSP. Finally, these patients were diagnosed with the congenital cochlear deafness.
This article was published in the following journal.
Name: International journal of pediatric otorhinolaryngology
Rehabilitation of patients with sensorineural hearing loss is an urgent task of otorhinolaryngology. One of the diseases leading to a pronounced hearing loss is the cochlear form of otosclerosis. The ...
Modulation of miR-34a/SIRT1 signaling protects cochlear hair cells against oxidative stress and delays age-related hearing loss through coordinated regulation of mitophagy and mitochondrial biogenesis.
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Due to the constant development of technology and medicine, the eligibility criteria for cochlear implantation (CI) are undergoing gradual expansion.
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Mutations in the GJB2 gene (which encodes Connexin26 (Cx26)) are the most common cause of non-syndromic deafness. Previous studies showed that an extensive knockout of the Gjb2 gene in cochlear epithe...
Congenital cytomegalovirus (CMV) infection is the leading cause of non-genetic neurosensory deafness and affects 0.5 to 1% of births. Twenty to thirty per cent of children will develop dea...
This is a research study to determine whether a cochlear implantation (CI) device can improve hearing in people who are deaf in one ear (known as single-sided deafness).
Mitochondrial Diseases are rare, progressive, multi-system, often-early fatal disorders affecting both children and adults. KH176 is a novel chemical entity currently under development for...
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The cochlear part of the 8th cranial nerve (VESTIBULOCOCHLEAR NERVE). The cochlear nerve fibers originate from neurons of the SPIRAL GANGLION and project peripherally to cochlear hair cells and centrally to the cochlear nuclei (COCHLEAR NUCLEUS) of the BRAIN STEM. They mediate the sense of hearing.
Diseases caused by abnormal function of the MITOCHONDRIA. They may be caused by mutations, acquired or inherited, in mitochondrial DNA or in nuclear genes that code for mitochondrial components. They may also be the result of acquired mitochondria dysfunction due to adverse effects of drugs, infections, or other environmental causes.
Diseases existing at birth and often before birth, or that develop during the first month of life (INFANT, NEWBORN, DISEASES), regardless of causation. Of these diseases, those characterized by structural deformities are termed CONGENITAL ABNORMALITIES.
Surgical insertion of an electronic hearing device (COCHLEAR IMPLANTS) with electrodes to the COCHLEAR NERVE in the inner ear to create sound sensation in patients with residual nerve fibers.
Multi-channel hearing devices typically used for patients who have tumors on the COCHLEAR NERVE and are unable to benefit from COCHLEAR IMPLANTS after tumor surgery that severs the cochlear nerve. The device electrically stimulates the nerves of cochlea nucleus in the BRAIN STEM rather than the inner ear as in cochlear implants.
Congenital conditions are those which are present from birth. They include structural deformities or loss of function in organs such as the <!--LGfEGNT2Lhm-->heart, gut or skeletal system. They can be corrected by <!--LGfEGNT2Lhm-->surgery, m...
Bioinformatics is the application of computer software and hardware to the management of biological data to create useful information. Computers are used to gather, store, analyze and integrate biological and genetic information which can then be applied...
Hearing, auditory perception, or audition is the ability to perceive sound by detecting vibrations, changes in the pressure of the surrounding medium through time, through an organ such as the ear. Sound may be heard through solid, liquid, or gaseous mat...