One in every 1,000 newborn suffers from congenital hearing impairment. More than 60% of the congenital cases are caused by genetic factors. In most cases, hearing loss is a multifactorial disorder caused by both genetic and environmental factors. Molecular genetics of deafness has experienced remarkable progress in the last decade. Genes responsible for hereditary hearing impairment are being mapped and cloned progressively. This review focuses on non-syndromic hearing loss, since the gene involved in this type of hearing loss have only recently begun to be identified.
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http://dx.doi.org/10.1016/s1808-8694(15)31313-6 | DOI Listing |
JAMA Otolaryngol Head Neck Surg
January 2025
Cochlear Center for Hearing and Public Health, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland.
Importance: Investigating rural-urban and regional differences in the association between dual sensory loss (concurrent hearing and vision loss) and depression may highlight gaps in sensory loss research and health care services, and by socioeconomic status. Whether urbanicity and region may modify associations between sensory loss and depression is unknown.
Objective: To describe the rural-urban and regional differences in the association of dual sensory loss with depression among older adults.
JAMA Otolaryngol Head Neck Surg
January 2025
Department of Otolaryngology-Head and Neck Surgery, School of Medicine, Virginia Commonwealth University, Richmond.
Front Mol Neurosci
December 2024
School of Basic Medical Science, Jining Key Laboratory of Pharmacology, Jining Medical University, Jining, Shandong, China.
Sensorineural hearing loss (SNHL) is characterized by a compromised cochlear perception of sound waves. Major risk factors for SNHL include genetic mutations, exposure to noise, ototoxic medications, and the aging process. Previous research has demonstrated that inflammation, oxidative stress, apoptosis, and autophagy, which are detrimental to inner ear cells, contribute to the pathogenesis of SNHL; however, the precise mechanisms remain inadequately understood.
View Article and Find Full Text PDFFront Public Health
January 2025
Department of Otorhinolaryngology, Air Force Hospital Medical Service Department in Western Theatre, Chengdu, China.
Background: Hearing loss (HL) is a common sensory disorder in humans. Studies on the relationship between arsenic, which is a highly toxic and widely distributed heavy metal with a health risk to humans, and hearing status in humans are contradictory and mostly focused on people living in arsenic-contaminated areas. This study investigated the association between urinary arsenic levels and hearing threshold shifts in the general population in the United States.
View Article and Find Full Text PDFInt J Med Sci
January 2025
Department of Rheumatism and Immunology, Tianjin First Central hospital, Tianjin, China.
Autoimmune inner ear disease (AIED) is a rare condition characterized by immune-mediated damage to the inner ear, leading to progressive sensorineural hearing loss (SNHL) and vestibular symptoms such as vertigo and tinnitus. This study investigates the pathogenesis and therapeutic strategies for AIED through the analysis of three cases with different underlying autoimmune disorders: rheumatoid arthritis, relapsing polychondritis, and IgG4-related disease. The etiology of AIED involves complex immunopathological mechanisms, including molecular mimicry and the "bystander effect," with specific autoantibodies, such as those against heat shock protein 70 (HSP70), playing a potential role in cochlear damage.
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