title: Apoptosis in acquired and genetic hearing impairment: the programmed death of the hair cell.
abstract: Apoptosis is an important physiological process. Normally, a healthy cell maintains a delicate balance between pro- and anti-apoptotic factors, allowing it to live and proliferate. It is thus not surprising that disturbance of this delicate balance may result in disease. It is a well known fact that apoptosis also contributes to several acquired forms of hearing impairment. Noise-induced hearing loss is the result of prolonged exposure to excessive noise, triggering apoptosis in terminally differentiated sensory hair cells. Moreover, hearing loss caused by the use of therapeutic drugs such as aminoglycoside antibiotics and cisplatin potentially may result in the activation of apoptosis in sensory hair cells leading to hearing loss due to the "ototoxicity" of the drugs. Finally, apoptosis is a key contributor to the development of presbycusis, age-related hearing loss. Recently, several mutations in apoptosis genes were identified as the cause of monogenic hearing impairment. These genes are TJP2, DFNA5 and MSRB3. This implies that apoptosis not only contributes to the pathology of acquired forms of hearing impairment, but also to genetic hearing impairment as well. We believe that these genes constitute a new functional class within the hearing loss field. Here, the contribution of apoptosis in the pathology of both acquired and genetic hearing impairment is reviewed.
authors.affiliation: Center of Medical Genetics, Department of Biomedical Sciences, University of Antwerp, Antwerp, Belgium.
authors.forename: Ken
authors.initials: K
authors.lastname: Op de Beeck
authors.forename: Jochen
authors.initials: J
authors.lastname: Schacht
authors.forename: Guy
authors.initials: G
authors.lastname: Van Camp
mesh_terms: D000818:Animals; D017209:Apoptosis / Q000235:genetics*; D020022:Genetic Predisposition to Disease; D006198:Hair Cells, Auditory / Q000473:pathology*; D034381:Hearing Loss / Q000139:chemically induced / Q000235:genetics* / Q000473:pathology*; D006317:Hearing Loss, Noise-Induced / Q000235:genetics / Q000473:pathology; D006801:Humans; D008565:Membrane Proteins / Q000235:genetics; D051150:Methionine Sulfoxide Reductases / Q000235:genetics; D009154:Mutation; D010641:Phenotype; D011304:Presbycusis / Q000235:genetics / Q000473:pathology; D011960:Receptors, Estrogen / Q000235:genetics; D062827:Zonula Occludens-2 Protein; D000094524:Gasdermins
chemical_list: D008565:Membrane Proteins; D051150:Methionine Sulfoxide Reductases; D011960:Receptors, Estrogen; D062827:Zonula Occludens-2 Protein; C111432:GSDME protein, human; D000094524:Gasdermins; C569046:TJP2 protein, human; C524352:MSRB3 protein, human