hearing loss

Summary

Summary: A general term for the complete or partial loss of the ability to hear from one or both ears.

Top Publications

  1. pmc Forty-six genes causing nonsyndromic hearing impairment: which ones should be analyzed in DNA diagnostics?
    Nele Hilgert
    Department of Medical Genetics, University of Antwerp UA, Universiteitsplein 1, B 2610 Antwerp, Belgium
    Mutat Res 681:189-96. 2009
  2. pmc Sirt3 mediates reduction of oxidative damage and prevention of age-related hearing loss under caloric restriction
    Shinichi Someya
    Departments of Genetics and Medical Genetics, University of Wisconsin, Madison, 53706, USA
    Cell 143:802-12. 2010
  3. pmc An ENU-induced mutation of miR-96 associated with progressive hearing loss in mice
    Morag A Lewis
    Wellcome Trust Sanger Institute, Hinxton, UK
    Nat Genet 41:614-8. 2009
  4. ncbi Newborn hearing screening--a silent revolution
    Cynthia C Morton
    Department of Obstetrics, Gynecology, and Reproductive Biology, Brigham and Women s Hospital and Harvard Medical School, Boston, USA
    N Engl J Med 354:2151-64. 2006
  5. pmc Comprehensive genetic testing for hereditary hearing loss using massively parallel sequencing
    A Eliot Shearer
    Department of Otolaryngology, Head and Neck Surgery, University of Iowa, Iowa City, IA 52242, USA
    Proc Natl Acad Sci U S A 107:21104-9. 2010
  6. pmc Polymorphisms in Toll-like receptors 2, 4, and 9 are highly associated with hearing loss in survivors of bacterial meningitis
    Gijs Th J van Well
    Department of Pediatric Infectious Diseases, Immunology and Rheumatology, VU University Medical Center, Amsterdam, The Netherlands
    PLoS ONE 7:e35837. 2012
  7. pmc Whole-exome sequencing efficiently detects rare mutations in autosomal recessive nonsyndromic hearing loss
    Oscar Diaz-Horta
    John P Hussman Institute for Human Genomics and the Dr John T Macdonald Department of Human Genetics, University of Miami Miller School of Medicine, Miami, Florida, United States of America
    PLoS ONE 7:e50628. 2012
  8. ncbi Development of tinnitus-related neuronal hyperactivity through homeostatic plasticity after hearing loss: a computational model
    Roland Schaette
    Department of Biology, Institute for Theoretical Biology, Humboldt Universitat zu Berlin, Invalidenstr 43, 10115 Berlin, Germany
    Eur J Neurosci 23:3124-38. 2006
  9. pmc Re-examining the relationship between audiometric profile and tinnitus pitch
    Magdalena Sereda
    MRC Institute of Hearing Research, University Park, Nottingham, UK
    Int J Audiol 50:303-12. 2011
  10. pmc Targeted genomic capture and massively parallel sequencing to identify genes for hereditary hearing loss in Middle Eastern families
    Zippora Brownstein
    Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
    Genome Biol 12:R89. 2011

Detail Information

Publications282 found, 100 shown here

  1. pmc Forty-six genes causing nonsyndromic hearing impairment: which ones should be analyzed in DNA diagnostics?
    Nele Hilgert
    Department of Medical Genetics, University of Antwerp UA, Universiteitsplein 1, B 2610 Antwerp, Belgium
    Mutat Res 681:189-96. 2009
    ..With 46 genes implicated in nonsyndromic hearing loss, it is also an extremely heterogeneous trait...
  2. pmc Sirt3 mediates reduction of oxidative damage and prevention of age-related hearing loss under caloric restriction
    Shinichi Someya
    Departments of Genetics and Medical Genetics, University of Wisconsin, Madison, 53706, USA
    Cell 143:802-12. 2010
    ..CR) extends the life span and health span of a variety of species and slows the progression of age-related hearing loss (AHL), a common age-related disorder associated with oxidative stress...
  3. pmc An ENU-induced mutation of miR-96 associated with progressive hearing loss in mice
    Morag A Lewis
    Wellcome Trust Sanger Institute, Hinxton, UK
    Nat Genet 41:614-8. 2009
    Progressive hearing loss is common in the human population, but little is known about the molecular basis. We report a new N-ethyl-N-nitrosurea (ENU)-induced mouse mutant, diminuendo, with a single base change in the seed region of Mirn96...
  4. ncbi Newborn hearing screening--a silent revolution
    Cynthia C Morton
    Department of Obstetrics, Gynecology, and Reproductive Biology, Brigham and Women s Hospital and Harvard Medical School, Boston, USA
    N Engl J Med 354:2151-64. 2006
  5. pmc Comprehensive genetic testing for hereditary hearing loss using massively parallel sequencing
    A Eliot Shearer
    Department of Otolaryngology, Head and Neck Surgery, University of Iowa, Iowa City, IA 52242, USA
    Proc Natl Acad Sci U S A 107:21104-9. 2010
    The extreme genetic heterogeneity of nonsyndromic hearing loss (NSHL) makes genetic diagnosis expensive and time consuming using available methods...
  6. pmc Polymorphisms in Toll-like receptors 2, 4, and 9 are highly associated with hearing loss in survivors of bacterial meningitis
    Gijs Th J van Well
    Department of Pediatric Infectious Diseases, Immunology and Rheumatology, VU University Medical Center, Amsterdam, The Netherlands
    PLoS ONE 7:e35837. 2012
    ..recognition and immune response genes with clinical features that determine severity and outcome (especially hearing loss) of childhood MM and pneumococcal meningitis (PM)...
  7. pmc Whole-exome sequencing efficiently detects rare mutations in autosomal recessive nonsyndromic hearing loss
    Oscar Diaz-Horta
    John P Hussman Institute for Human Genomics and the Dr John T Macdonald Department of Human Genetics, University of Miami Miller School of Medicine, Miami, Florida, United States of America
    PLoS ONE 7:e50628. 2012
    Identification of the pathogenic mutations underlying autosomal recessive nonsyndromic hearing loss (ARNSHL) is difficult, since causative mutations in 39 different genes have so far been reported...
  8. ncbi Development of tinnitus-related neuronal hyperactivity through homeostatic plasticity after hearing loss: a computational model
    Roland Schaette
    Department of Biology, Institute for Theoretical Biology, Humboldt Universitat zu Berlin, Invalidenstr 43, 10115 Berlin, Germany
    Eur J Neurosci 23:3124-38. 2006
    Tinnitus, the perception of a sound in the absence of acoustic stimulation, is often associated with hearing loss. Animal studies indicate that hearing loss through cochlear damage can lead to behavioral signs of tinnitus that are ..
  9. pmc Re-examining the relationship between audiometric profile and tinnitus pitch
    Magdalena Sereda
    MRC Institute of Hearing Research, University Park, Nottingham, UK
    Int J Audiol 50:303-12. 2011
    ..arising from neurophysiological models of tinnitus: 'Is the dominant tinnitus pitch associated with the edge of hearing loss?' and 'Is such a relationship more robust in people with narrow tinnitus bandwidth or steep sloping hearing ..
  10. pmc Targeted genomic capture and massively parallel sequencing to identify genes for hereditary hearing loss in Middle Eastern families
    Zippora Brownstein
    Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
    Genome Biol 12:R89. 2011
    ..Due to the genetic heterogeneity of hearing loss, targeted DNA capture and massively parallel sequencing are ideal tools to address this challenge...
  11. ncbi Hearing impairment: a panoply of genes and functions
    Amiel A Dror
    Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
    Neuron 68:293-308. 2010
    ..Overall, studies in genetics have provided research scientists and clinicians with insight regarding practical implications for the hearing impaired, while heralding hope for future development of therapeutics...
  12. pmc Neuromagnetic indicators of tinnitus and tinnitus masking in patients with and without hearing loss
    Peyman Adjamian
    MRC Institute of Hearing Research, University Park, Nottingham, UK NG7 2RD
    J Assoc Res Otolaryngol 13:715-31. 2012
    ..can be attributed to the disruption of coherent oscillatory activity between thalamus and cortex following hearing loss. However, the extent to which this disruption specifically contributes to tinnitus or is simply a consequence ..
  13. pmc Targeted massive parallel sequencing: the effective detection of novel causative mutations associated with hearing loss in small families
    Jeong In Baek
    Department of Biology, College of Natural Sciences, Kyungpook National University, Daegu, South Korea
    Orphanet J Rare Dis 7:60. 2012
    Hereditary hearing loss is one of the most common heterogeneous disorders, and genetic variants that can cause hearing loss have been identified in over sixty genes...
  14. pmc Hearing loss and cognitive decline in older adults
    Frank R Lin
    Department of Otolaryngology Head and Neck Surgery, The Johns Hopkins School of Medicine, and Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, USA
    JAMA Intern Med 173:293-9. 2013
    BACKGROUND Whether hearing loss is independently associated with accelerated cognitive decline in older adults is unknown. METHODS We studied 1984 older adults (mean age, 77...
  15. pmc Infant hearing loss: from diagnosis to therapy Official Report of XXI Conference of Italian Society of Pediatric Otorhinolaryngology
    G Paludetti
    Department of Head and Neck Surgery, Institute of Otorhinolaryngology, Catholic University of the Sacred Heart, Rome, Italy
    Acta Otorhinolaryngol Ital 32:347-70. 2012
    b>Hearing loss is one of the most common disabilities and has lifelong consequences for affected children and their families...
  16. pmc Hearing loss and cognition in the Baltimore Longitudinal Study of Aging
    Frank R Lin
    Department of Otolaryngology Head and Neck Surgery, Johns Hopkins School of Medicine, Baltimore, MD 21205, USA
    Neuropsychology 25:763-70. 2011
    To determine the relationship between hearing loss and cognitive function as assessed with a standardized neurocognitive battery...
  17. ncbi Treated hypotension is associated with neonatal morbidity and hearing loss in extremely low birth weight infants
    Jonathan M Fanaroff
    Department of Pediatrics, Rainbow Babies and Children s Hospital, Case Western Reserve University, Cleveland, Ohio, USA
    Pediatrics 117:1131-5. 2006
    ..Neonatal hypotension may be a risk factor for neurologic impairment. Few studies have examined the impact of low blood pressure in extremely low birth weight (ELBW) infants weighing 400 to 999 g on neurodevelopmental outcome...
  18. pmc Supporting sensory transduction: cochlear fluid homeostasis and the endocochlear potential
    Philine Wangemann
    Anatomy and Physiology Department, 205 Coles Hall, Kansas State University, Manhattan, 66506, USA
    J Physiol 576:11-21. 2006
    ..Disturbance of cochlear homeostasis is the cause of many forms of hearing loss including the most frequently occurring syndromic and non-syndromic forms of hereditary hearing loss, Pendred ..
  19. ncbi Hearing loss: mechanisms revealed by genetics and cell biology
    Amiel A Dror
    Department of Human Molecular Genetics and Biochemistry, Sackler School of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
    Annu Rev Genet 43:411-37. 2009
    b>Hearing loss (HL), or deafness in its most severe form, affects an estimated 28 and 22.5 million Americans and Europeans, respectively...
  20. ncbi Mechanisms of cisplatin-induced ototoxicity and prevention
    Leonard P Rybak
    Department of Surgery, Division of Otolaryngology, Southern Illinois University, School of Medicine, P O Box 19653, Springfield, IL 62794 9653, USA
    Hear Res 226:157-67. 2007
    ..and the application of agents that act further downstream in the cell death cascade to prevent apoptosis and hearing loss. This review summarizes recent data that shed new light on the mechanisms of cisplatin ototoxicity and its ..
  21. ncbi Distribution and frequencies of PDS (SLC26A4) mutations in Pendred syndrome and nonsyndromic hearing loss associated with enlarged vestibular aqueduct: a unique spectrum of mutations in Japanese
    Koji Tsukamoto
    Department of Otorhinolaryngology, Shinshu University School of Medicine, 3 1 1 Asahi, Matsumoto 390 8621, Japan
    Eur J Hum Genet 11:916-22. 2003
    ..Mutations in the PDS (SLC26A4) gene are known to be responsible for both Pendred syndrome and nonsyndromic hearing loss associated with enlarged vestibular aqueduct, and the molecular confirmation of the PDS gene has become ..
  22. pmc Mutations in TPRN cause a progressive form of autosomal-recessive nonsyndromic hearing loss
    Yun Li
    Center for Molecular Medicine Cologne CMMC, University of Cologne, 50931 Cologne, Germany
    Am J Hum Genet 86:479-84. 2010
    ..mapping in a large consanguineous family from Morocco and mapped the autosomal-recessive nonsyndromic hearing loss (ARNSHL) in this family to the DFNB79 locus on chromosome 9q34...
  23. pmc Mutations in LARS2, encoding mitochondrial leucyl-tRNA synthetase, lead to premature ovarian failure and hearing loss in Perrault syndrome
    Sarah B Pierce
    Division of Medical Genetics, Department of Medicine, University of Washington, Seattle WA 98195, USA
    Am J Hum Genet 92:614-20. 2013
    ..In two families affected by POF accompanied by hearing loss (together, these symptoms compose Perrault syndrome), exome sequencing revealed mutations in LARS2, encoding ..
  24. pmc Prevalence of age-related hearing loss in Europe: a review
    Thomas Niklaus Roth
    Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital of Zurich, Frauenklinikstr 24, 8091 Zurich, Switzerland
    Eur Arch Otorhinolaryngol 268:1101-7. 2011
    ..In contrast to epidemiological data of other continents, the prevalence of age-related hearing loss (ARHL) in Europe is not well defined, due in part to the use of different classification systems...
  25. ncbi Hereditary hearing loss: from human mutation to mechanism
    Danielle R Lenz
    Department of Human Molecular Genetics and Biochemistry, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
    Hear Res 281:3-10. 2011
    The genetic heterogeneity of hereditary hearing loss is thus far represented by hundreds of genes encoding a large variety of proteins...
  26. pmc Murine malaria is associated with significant hearing impairment
    Joachim Schmutzhard
    Department of Otorhinolaryngology, Innsbruck Medical University, Anichstrasse 35, 6020 Innsbruck Austria
    Malar J 9:159. 2010
    Plasmodium falciparum malaria has been suspected to cause hearing loss. Developmental, cognitive and language disorders have been observed in children, surviving cerebral malaria...
  27. pmc Neuroanatomical changes due to hearing loss and chronic tinnitus: a combined VBM and DTI study
    Fatima T Husain
    Department of Speech and Hearing Science, University of Illinois at Urbana Champaign, Champaign, IL 61820, USA
    Brain Res 1369:74-88. 2011
    ..Tinnitus is often accompanied by hearing loss but not everyone with hearing loss experiences tinnitus...
  28. pmc Selective attention in normal and impaired hearing
    Barbara G Shinn-Cunningham
    Hearing Research Center, Departments of Cognitive and Neural Systems and Biomedical Engineering, Boston University, Boston, MA 02421, USA
    Trends Amplif 12:283-99. 2008
    A common complaint among listeners with hearing loss (HL) is that they have difficulty communicating in common social settings...
  29. ncbi Prevention of cisplatin ototoxicity using transtympanic N-acetylcysteine and lactate
    Won Taek Choe
    Department of Otolaryngology, Head and Neck Surgery, Stanford University School of Medicine, Stanford, California 94305, USA
    Otol Neurotol 25:910-5. 2004
    ..Transtympanic administration of the antioxidant N-acetylcysteine or lactated Ringer's solution onto the round window membrane will prevent cisplatin ototoxicity in the guinea pig model...
  30. pmc Audioprofile-directed screening identifies novel mutations in KCNQ4 causing hearing loss at the DFNA2 locus
    Michael S Hildebrand
    Department of Otolaryngology Head and Neck Surgery, University of Iowa, Iowa City, IA 52242, USA
    Genet Med 10:797-804. 2008
    Gene identification in small families segregating autosomal dominant sensorineural hearing loss presents a significant challenge. To address this challenge, we have developed a machine learning-based software tool, AudioGene v2...
  31. pmc Factors influencing help seeking, hearing aid uptake, hearing aid use and satisfaction with hearing aids: a review of the literature
    Line Vestergaard Knudsen
    Oticon Research Centre, Eriksholm, Snekkersten, Denmark
    Trends Amplif 14:127-54. 2010
    ..the literature provides an overview of the available studies (published between January 1980 and January 2009) on correlates of help-seeking behavior for hearing loss, hearing-aid uptake, hearing-aid use, and satisfaction with the device.
  32. ncbi Cross-modal plasticity in deaf subjects dependent on the extent of hearing loss
    Nicole Lambertz
    Department of Diagnostic and Interventional Radiology, Neuroradiology, University of Duisburg Essen, Hufelandstr 55, D 45127 Essen, Germany
    Brain Res Cogn Brain Res 25:884-90. 2005
    ..We tried to figure out whether the plasticity is dependent on the extent of hearing loss. Three groups of volunteers, comprising twelve individuals each, were investigated...
  33. ncbi Rehabilitative online education versus internet discussion group for hearing aid users: a randomized controlled trial
    Elisabet Thorén
    Department of Clinical and Experimental Medicine, Linkoping University, Sweden
    J Am Acad Audiol 22:274-85. 2011
    ....
  34. ncbi Risk factors for hearing loss in US adults: data from the National Health and Nutrition Examination Survey, 1999 to 2002
    Yuri Agrawal
    Department of Otolaryngology Head and Neck Surgery, Johns Hopkins Hospital, Baltimore, Maryland 21287, USA
    Otol Neurotol 30:139-45. 2009
    ....
  35. ncbi Acoustic stimulation treatments against tinnitus could be most effective when tinnitus pitch is within the stimulated frequency range
    Roland Schaette
    Institute for Theoretical Biology, Humboldt Universitat zu Berlin, Berlin, Germany
    Hear Res 269:95-101. 2010
    ..However, the differences we observed between the low- and the high-pitch group show that tinnitus pitch might influence the outcome of acoustic stimulation treatments when devices with a limited frequency range are used...
  36. ncbi A large cohort study of GJB2 mutations in Japanese hearing loss patients
    K Tsukada
    Department of Otorhinolaryngology, Shinshu University School of Medicine, 3 1 1 Asahi, Matsumoto 390 8621, Japan
    Clin Genet 78:464-70. 2010
    GJB2 is the gene most frequently associated with hereditary hearing loss, and the GJB2 mutation spectrums vary among different ethnic groups...
  37. ncbi Noise-induced stress response and hearing loss in goldfish (Carassius auratus)
    Michael E Smith
    Department of Biology and Center for Comparative and Evolutionary Biology of Hearing, University of Maryland, College Park, MD 20742, USA
    J Exp Biol 207:427-35. 2004
    ..Further noise exposure did not increase threshold shifts, suggesting an asymptote of maximal hearing loss within 24 h...
  38. ncbi Mutations in the seed region of human miR-96 are responsible for nonsyndromic progressive hearing loss
    Angeles Mencia
    Unidad de Genetica Molecular, Hospital Ramon y Cajal, Madrid, Spain
    Nat Genet 41:609-13. 2009
    ..region of miR-96, a miRNA expressed in hair cells of the inner ear, result in autosomal dominant, progressive hearing loss. This is the first study implicating a miRNA in a mendelian disorder...
  39. pmc Mitochondrial 12S rRNA variants in 1642 Han Chinese pediatric subjects with aminoglycoside-induced and nonsyndromic hearing loss
    Jianxin Lu
    Attardi Institute of Mitochondrial Biomedicine and Zhejiang Provincial Key Laboratory of Medical Genetics, School of Life Sciences, Wenzhou Medical College, Wenzhou, Zhejiang, China
    Mitochondrion 10:380-90. 2010
    ..variants in a large cohort of 1642 Han Chinese pediatric subjects with aminoglycoside-induced and nonsyndromic hearing loss. Mutational analysis of 12S rRNA gene in these subjects identified 68 (54 known and 14 novel) variants...
  40. ncbi The impact of hearing loss on quality of life in older adults
    Dayna S Dalton
    Department of Ophthalmology and Visual Sciences, University of Wisconsin, Madison, 53726 2336, USA
    Gerontologist 43:661-8. 2003
    The authors investigate the impact of hearing loss on quality of life in a large population of older adults.
  41. pmc Comprehensive molecular etiology analysis of nonsyndromic hearing impairment from typical areas in China
    Yongyi Yuan
    Department of Otolaryngology, PLA General Hospital, Beijing, PR China
    J Transl Med 7:79. 2009
    ..To provide appropriate genetic testing and counseling to families, we performed a comprehensive investigation of the molecular etiology of nonsyndromic deafness in two typical areas from northern and southern China...
  42. pmc Deficient forward transduction and enhanced reverse transduction in the alpha tectorin C1509G human hearing loss mutation
    Anping Xia
    The Bobby R Alford Department of Otolaryngology Head and Neck Surgery, Baylor College of Medicine, Houston, TX 77030, USA
    Dis Model Mech 3:209-23. 2010
    Most forms of hearing loss are associated with loss of cochlear outer hair cells (OHCs). OHCs require the tectorial membrane (TM) for stereociliary bundle stimulation (forward transduction) and active feedback (reverse transduction)...
  43. pmc Transtympanic administration of short interfering (si)RNA for the NOX3 isoform of NADPH oxidase protects against cisplatin-induced hearing loss in the rat
    Debashree Mukherjea
    Department of Surgery, Southern Illinois University School of Medicine, Springfield, Illinois 62794 9649, USA
    Antioxid Redox Signal 13:589-98. 2010
    Cisplatin produces hearing loss in cancer patients. Reactive oxygen species (ROS) in the cochlea leads to lipid peroxidation, death of outer hair cells (OHCs), and hearing loss...
  44. ncbi A hearing and vestibular phenotyping pipeline to identify mouse mutants with hearing impairment
    Rachel E Hardisty-Hughes
    MRC Harwell, Mammalian Genetics Unit, Harwell Science and Innovation Campus, Harwell, UK
    Nat Protoc 5:177-90. 2010
    ..phenotyping tests, this protocol integrates a set of tests for the comprehensive determination of the causes of hearing loss. It comprises a primary screen of relatively simple auditory and vestibular tests...
  45. ncbi Screening and management of adult hearing loss in primary care: scientific review
    Bevan Yueh
    Veterans Affairs Puget Sound Health Care System, Department of Otolaryngology Head and Neck Surgery, University of Washington, Seattle 98108, USA
    JAMA 289:1976-85. 2003
    b>Hearing loss is the third most prevalent chronic condition in older adults and has important effects on their physical and mental health. Despite these effects, most older patients are not assessed or treated for hearing loss.
  46. pmc Next-generation sequencing identifies mutations of SMPX, which encodes the small muscle protein, X-linked, as a cause of progressive hearing impairment
    Margit Schraders
    Department of Otorhinolaryngology, Head and Neck Surgery, Nijmegen, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    Am J Hum Genet 88:628-34. 2011
    ..We hypothesize that SMPX functions in inner ear development and/or maintenance in the IGF-1 pathway, the integrin pathway through Rac1, or both...
  47. pmc Carcinoembryonic antigen-related cell adhesion molecule 16 interacts with alpha-tectorin and is mutated in autosomal dominant hearing loss (DFNA4)
    Jing Zheng
    Department of Otolaryngology Head and Neck Surgery, Feinberg School of Medicine, Northwestern University, Chicago, IL 60611, USA
    Proc Natl Acad Sci U S A 108:4218-23. 2011
    ..that mutation of CEACAM16 leads to autosomal dominant nonsyndromic deafness (ADNSHL) at the autosomal dominant hearing loss (DFNA4) locus...
  48. ncbi Characteristics of tinnitus and etiology of associated hearing loss: a study of 123 patients
    Cécile Nicolas-Puel
    Service d ORL, CHU Gui de Chauliac, Unité INSERM U 254, Montpellier, France
    Int Tinnitus J 8:37-44. 2002
    ..Analysis of those patients with a stable high-pitched tinnitus associated with a high-frequency hearing loss shows a statistically significant correlation between the elevation of the audiometric thresholds and the ..
  49. pmc Mutations in DNMT1 cause hereditary sensory neuropathy with dementia and hearing loss
    Christopher J Klein
    Mayo Clinic, Department of Neurology, Division of Peripheral Nerve Diseases, Rochester, Minnesota, USA
    Nat Genet 43:595-600. 2011
    ..and peripheral neurodegeneration in one form of hereditary sensory and autonomic neuropathy with dementia and hearing loss. Exome sequencing led to the identification of DNMT1 mutation c.1484A>G (p...
  50. pmc GJB2 mutations and degree of hearing loss: a multicenter study
    Rikkert L Snoeckx
    Department of Medical Genetics, University of Antwerp, Universiteitsplein 1, B 2610 Antwerp, Belgium
    Am J Hum Genet 77:945-57. 2005
    ..is involved in inner ear homeostasis, are found in up to 50% of patients with autosomal recessive nonsyndromic hearing loss. Because of the high frequency of GJB2 mutations, mutation analysis of this gene is widely available as a ..
  51. ncbi Change in prevalence of hearing loss in US adolescents
    Josef Shargorodsky
    Channing Laboratory, Department of Medicine, Brigham and Women s Hospital, Boston, Massachusetts 02115, USA
    JAMA 304:772-8. 2010
    b>Hearing loss is common and, in young persons, can compromise social development, communication skills, and educational achievement.
  52. pmc Atp6v0a4 knockout mouse is a model of distal renal tubular acidosis with hearing loss, with additional extrarenal phenotype
    Elizabeth E Norgett
    Department of Medical Genetics, University of Cambridge, Cambridge, United Kingdom
    Proc Natl Acad Sci U S A 109:13775-80. 2012
    ..This mouse model recapitulates the loss of H(+)-ATPase function seen in human disease and can provide additional insights into dRTA and the physiology of the a4 subunit...
  53. pmc Influence of clinical status on the association between plasma total and unbound bilirubin and death or adverse neurodevelopmental outcomes in extremely low birth weight infants
    W Oh
    Warren Alpert Medical School of Brown University, RI, USA
    Acta Paediatr 99:673-8. 2010
    ..To assess the influence of clinical status on the association between total plasma bilirubin and unbound bilirubin on death or adverse neurodevelopmental outcomes at 18-22 months corrected age in extremely low birth weight infants...
  54. pmc Discrimination task reveals differences in neural bases of tinnitus and hearing impairment
    Fatima T Husain
    Department of Speech and Hearing Science, University of Illinois at Urbana Champaign, Champaign, Illinois, United States of America
    PLoS ONE 6:e26639. 2011
    We investigated auditory perception and cognitive processing in individuals with chronic tinnitus or hearing loss using functional magnetic resonance imaging (fMRI)...
  55. pmc Molecular and clinical characterization of the variable phenotype in Korean families with hearing loss associated with the mitochondrial A1555G mutation
    Jae Woong Bae
    Department of Biology, College of Natural Sciences, Kyungpook National University, Daegu, South Korea
    PLoS ONE 7:e42463. 2012
    b>Hearing loss, which is genetically heterogeneous, can be caused by mutations in the mitochondrial DNA (mtDNA)...
  56. pmc Molecular epidemiology and functional assessment of novel allelic variants of SLC26A4 in non-syndromic hearing loss patients with enlarged vestibular aqueduct in China
    Yongyi Yuan
    Department of Otolaryngology, Chinese PLA General Hospital, Beijing, People s Republic of China
    PLoS ONE 7:e49984. 2012
    ..To investigate the incidence of EVA in Chinese population and to provide appropriate genetic testing and counseling to patients with SLC26A4 variants, we conducted a large-scale molecular epidemiological survey of SLC26A4...
  57. ncbi Effectiveness of computer-based auditory training for adult users of cochlear implants
    Paula C Stacey
    Division of Psychology, Nottingham Trent University, Nottingham, UK
    Int J Audiol 49:347-56. 2010
    ..Self-reported benefits were variable and generally small. Further research is needed to establish whether auditory training is particularly effective for identifiable sub-groups of cochlear-implant users...
  58. ncbi Self-reported hearing problems among older adults: prevalence and comparison to measured hearing impairment
    Samuli Hannula
    Department of Clinical Medicine, Otorhinolaryngology, University of Oulu, Oulu, Finland
    J Am Acad Audiol 22:550-9. 2011
    ....
  59. pmc Carboplatin-associated ototoxicity in children with retinoblastoma
    Ibrahim Qaddoumi
    St Jude Children s Research Hospital, Memphis, TN 38105 3678, USA
    J Clin Oncol 30:1034-41. 2012
    ..We retrospectively assessed the incidence of ototoxicity and its risk factors in children with retinoblastoma who were treated with carboplatin...
  60. pmc Mutations in LOXHD1, an evolutionarily conserved stereociliary protein, disrupt hair cell function in mice and cause progressive hearing loss in humans
    Nicolas Grillet
    Department of Cell Biology, The Scripps Research Institute, La Jolla, CA 92037, USA
    Am J Hum Genet 85:328-37. 2009
    b>Hearing loss is the most common form of sensory impairment in humans and is frequently progressive in nature. Here we link a previously uncharacterized gene to hearing impairment in mice and humans...
  61. ncbi Hearing loss in children with congenital cytomegalovirus infection born to mothers with preexisting immunity
    Shannon A Ross
    Department of Pediatrics, University of Alabama at Birmingham, 35233, USA
    J Pediatr 148:332-6. 2006
    ..To define hearing outcomes in children with congenital cytomegalovirus (CMV) infection born to mothers with non-primary CMV infection...
  62. ncbi Developmental differences in susceptibility to neomycin-induced hair cell death in the lateral line neuromasts of zebrafish (Danio rerio)
    Setsuko L Murakami
    Graduate Program in Audiology, University of Washington, Seattle, WA 98195, USA
    Hear Res 186:47-56. 2003
    ..Thus zebrafish hair cells show developmental differences in sensitivity to aminoglycoside-induced death similar to those reported for inner ear hair cells of birds and mammals...
  63. ncbi Adapting to changed hearing: the potential role of formal training
    Arthur Boothroyd
    Speech and Hearing Sciences, City University of New York, USA
    J Am Acad Audiol 21:601-11. 2010
    ....
  64. pmc The R109H variant of fascin-2, a developmentally regulated actin crosslinker in hair-cell stereocilia, underlies early-onset hearing loss of DBA/2J mice
    Jung Bum Shin
    Oregon Hearing Research Center, Oregon Health and Science University, Portland, Oregon 97239, USA
    J Neurosci 30:9683-94. 2010
    The quantitative trait locus ahl8 is a key contributor to the early-onset, age-related hearing loss of DBA/2J mice...
  65. ncbi The guide to plotting a cochleogram
    Agneta Viberg
    Department of Physiology and Pharmacology, Karolinska Institutet, 171 77 Stockholm, Sweden
    Hear Res 197:1-10. 2004
    ..These procedures are illustrated and discussed...
  66. ncbi Prevalence of hearing loss and differences by demographic characteristics among US adults: data from the National Health and Nutrition Examination Survey, 1999-2004
    Yuri Agrawal
    Department of Otolaryngology Head and Neck Surgery, Johns Hopkins Hospital, 601 N Caroline St, Baltimore, MD 21287, USA
    Arch Intern Med 168:1522-30. 2008
    b>Hearing loss affects health and quality of life. The prevalence of hearing loss may be growing because of an aging population and increasing noise exposure...
  67. ncbi No hearing loss associated with the use of artemether-lumefantrine to treat experimental human malaria
    Matthew B B McCall
    Department of Medical Microbiology, Radboud University Nijmegen Medical Centre, 6500 HB, Nijmegen, The Netherlands
    Trans R Soc Trop Med Hyg 100:1098-104. 2006
    ..No effects on hearing loss that were deemed to be caused by drug treatment were found using tone audiometry...
  68. ncbi The role of intratympanic lactate injection in the prevention of cisplatin-induced ototoxicity
    Marc Elie Nader
    Department of Otolaryngology Head and Neck Surgery, Sainte Justine University Hospital Center, Montreal University, Montreal, Quebec, Canada
    Laryngoscope 120:1208-13. 2010
    ....
  69. ncbi Sensory and cognitive association in older persons: findings from an older Australian population
    Tien Tay
    Centre for Vision Research, Department of Ophthalmology, Westmead Millennium Institute, University of Sydney, Sydney, Australia
    Gerontology 52:386-94. 2006
    ..Explanations for associations found between sensory and cognitive function remain unclear...
  70. ncbi Speech reception thresholds in noise and self-reported hearing disability in a general adult population
    Cas Smits
    Department of Otolaryngology Audiology, EMGO Institute, VU University Medical Center, Amsterdam, The Netherlands
    Ear Hear 27:538-49. 2006
    ..A secondary objective was to investigate to what extent the functional measurements could be predicted on the basis of the self-reported data...
  71. pmc Prevalence of the GJB2 IVS1+1G >A mutation in Chinese hearing loss patients with monoallelic pathogenic mutation in the coding region of GJB2
    Yongyi Yuan
    Department of Otolaryngology, PLA General Hospital, Beijing, People s Republic of China
    J Transl Med 8:127. 2010
    Mutations in the GJB2 gene are the most common cause of nonsyndromic recessive hearing loss in China...
  72. ncbi Gray matter in the brain: differences associated with tinnitus and hearing loss
    Kris Boyen
    Department of Otorhinolaryngology Head and Neck Surgery, University of Groningen, University Medical Center Groningen, P O Box 30 001, 9700 RB Groningen, The Netherlands
    Hear Res 295:67-78. 2013
    Tinnitus, usually associated with hearing loss, is characterized by the perception of sound without an external sound source. The pathophysiology of tinnitus is poorly understood...
  73. ncbi Hearing loss impacts on the use of community and informal supports
    Julie Schneider
    Menzies Centre for Health Policy, University of Sydney, Australia
    Age Ageing 39:458-64. 2010
    the aim of this study is to estimate the cross-sectional and longitudinal impact of hearing loss on use of community support services and reliance on non-spouse family/friends among older people.
  74. pmc Common molecular etiologies are rare in nonsyndromic Tibetan Chinese patients with hearing impairment
    Yongyi Yuan
    Department of Otolaryngology, PLA General Hospital, Beijing, People s Republic of China
    PLoS ONE 7:e30720. 2012
    ..To provide appropriate genetic testing and counseling to Tibetan families, we investigated molecular etiology of nonsyndromic deafness in this population...
  75. pmc High frequency of the p.R34X mutation in the TMC1 gene associated with nonsyndromic hearing loss is due to founder effects
    Mariem Ben Saïd
    Targets for Diagnosis and Therapy Unit, Centre of Biotechnology of Sfax, Sfax, Tunisia
    Genet Test Mol Biomarkers 14:307-11. 2010
    ..in the GJB2 gene, have to a large extent contributed to the high frequency of autosomal recessive nonsyndromic hearing loss (ARNSHL). Mutations in transmembrane channel-like gene 1 (TMC1) cause ARNSHL. The p...
  76. ncbi Vitamin D-deficient diet rescues hearing loss in Klotho mice
    Marina R Carpinelli
    Walter and Eliza Hall Institute of Medical Research, 1G Royal Parade, Parkville, VIC 3050, Australia
    Hear Res 275:105-9. 2011
    Klotho-deficient mice exhibit a premature aging syndrome, a feature of which is mild hearing loss. In the present study, the hearing phenotype of Klotho mice was characterized to better determine how well this phenotype resembles ..
  77. pmc HIF-VEGF pathways are critical for chronic otitis media in Junbo and Jeff mouse mutants
    Michael T Cheeseman
    MRC Mammalian Genetics Unit, MRC Harwell, Harwell, United Kingdom
    PLoS Genet 7:e1002336. 2011
    Otitis media with effusion (OME) is the commonest cause of hearing loss in children, yet the underlying genetic pathways and mechanisms involved are incompletely understood...
  78. ncbi Grhl2 deficiency impairs otic development and hearing ability in a zebrafish model of the progressive dominant hearing loss DFNA28
    Yanchao Han
    Developmental Genetics Laboratory of Tsinghua University, School of Life Sciences, Tsinghua University, Beijing 100084, China
    Hum Mol Genet 20:3213-26. 2011
    Congenital and progressive hearing impairment is a common distressing disease. The progressive dominant hearing loss DFNA28 in human is associated with a frameshift mutation of Grainyhead-like 2 (GRHL2) but its etiology and mechanism ..
  79. pmc Insulin receptor substrate 2 (IRS2)-deficient mice show sensorineural hearing loss that is delayed by concomitant protein tyrosine phosphatase 1B (PTP1B) loss of function
    Silvia Murillo-Cuesta
    Institute of Biomedical Research Alberto Sols IIBM, Spanish National Research Council Autonomous University of Madrid CSIC UAM, Madrid, Spain
    Mol Med 18:260-9. 2012
    ..IGF1 gene mutations cause syndromic sensorineural hearing loss in humans and mice...
  80. ncbi Otitis media, hearing loss, and language learning: controversies and current research
    Joanne Roberts
    FPG Child Development Institute, University of North Carolina, Chapel Hill, North Carolina 27599 8180, USA
    J Dev Behav Pediatr 25:110-22. 2004
    ..About half of children with an episode of OME experience a mild hearing loss while about 5-10% of children have moderate hearing loss...
  81. ncbi Association between peak serum bilirubin and neurodevelopmental outcomes in extremely low birth weight infants
    William Oh
    Women and Infants Hospital of Rhode Island Brown University, Providence, Rhode Island 02905, USA
    Pediatrics 112:773-9. 2003
    ..To assess the association between peak total serum bilirubin (PSB) levels during the first 2 weeks of life and neurodevelopmental outcomes of extremely low birth weight (ELBW) infants at 18 to 22 months' postmenstrual age...
  82. pmc Mutations of GIPC3 cause nonsyndromic hearing loss DFNB72 but not DFNB81 that also maps to chromosome 19p
    Atteeq U Rehman
    Laboratory of Molecular Genetics, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Rockville, MD 20850, USA
    Hum Genet 130:759-65. 2011
    A missense mutation of Gipc3 was previously reported to cause age-related hearing loss in mice. Point mutations of human GIPC3 were found in two small families, but association with hearing loss was not statistically significant...
  83. pmc Seven novel mutations in the long isoform of the USH2A gene in Chinese families with nonsyndromic retinitis pigmentosa and Usher syndrome Type II
    Wenjun Xu
    Beijing Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing Ophthalmology and Visual Sciences Key Laboratory, Beijing, China
    Mol Vis 17:1537-52. 2011
    ..To describe the clinical and genetic findings in one Chinese family with autosomal recessive retinitis pigmentosa (arRP) and in three unrelated Chinese families with Usher syndrome type II (USH2)...
  84. pmc Novel sequence variants in the TMC1 gene in Pakistani families with autosomal recessive hearing impairment
    Regie Lyn P Santos
    Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, Texas 77030, USA
    Hum Mutat 26:396. 2005
    ..4% (95%CI: 1.9, 8.6%). The TMC1 protein might have an important function in K(+) channels of inner hair cells, which would be consistent with the hypothetical structure of protein domains in which sequence variants were identified...
  85. ncbi Clinical features of patients with GJB2 (connexin 26) mutations: severity of hearing loss is correlated with genotypes and protein expression patterns
    Tomohiro Oguchi
    Department of Otorhinolaryngology, Shinshu University School of Medicine, 3 1 1 Asahi, Matsumoto, 390 8621, Japan
    J Hum Genet 50:76-83. 2005
    ..The present in vitro study further confirmed that differences in phenotypes could be explained by the protein expression pattern...
  86. ncbi Pathogenic effects of a novel mutation (c.664_681del) in KCNQ4 channels associated with auditory pathology
    Jeong In Baek
    Department of Biology, College of Natural Sciences, Kyungpook National University, Daegu, 702 701, South Korea
    Biochim Biophys Acta 1812:536-43. 2011
    b>Hearing loss is a common communication disorder caused by various environmental and genetic factors. Hereditary hearing loss is very heterogeneous, and most of such cases involve sensorineural defects in the auditory pathway...
  87. pmc Addressing the global neglect of childhood hearing impairment in developing countries
    Bolajoko O Olusanya
    Unit of Audiological Medicine, Institute of Child Health, University College London, London, United Kingdom
    PLoS Med 4:e74. 2007
  88. ncbi Estimation of the frequency of occult mutations for an autosomal recessive disease in the presence of genetic heterogeneity: application to genetic hearing loss disorders
    William J Kimberling
    Center for the Study and Treatment of Usher Syndrome, Boys Town National Research Hospital and the Department of Biomedical Sciences, Creighton University School of Medicine, Omaha, Nebraska 68131, USA
    Hum Mutat 26:462-70. 2005
    ..The test is applied to GJB2 recessive nonsyndromic deafness, Usher syndrome types Ib and IIa, and Pendred-enlarged vestibular aqueduct syndrome...
  89. ncbi Ageing and hearing loss
    X Z Liu
    Department of Otolaryngology, University of Miami, Miami, FL 33101, USA
    J Pathol 211:188-97. 2007
    Although many adults retain good hearing as they age, hearing loss associated with ageing is common among elderly persons...
  90. ncbi Development of hyperactivity after hearing loss in a computational model of the dorsal cochlear nucleus depends on neuron response type
    Roland Schaette
    Institute for Theoretical Biology, Department of Biology, Humboldt Universitat zu Berlin, Invalidenstr 43, 10115 Berlin, Germany
    Hear Res 240:57-72. 2008
    ..This theoretical framework for the mechanisms and circumstances of the development of hyperactivity in central auditory neurons might also provide new insights into the development of tinnitus...
  91. ncbi A systematic review of telehealth applications in audiology
    De Wet Swanepoel
    Department of Communication Pathology, University of Pretoria, Pretoria, South Africa
    Telemed J E Health 16:181-200. 2010
    Abstract Hearing loss is a pervasive global healthcare concern with an estimated 10% of the global population affected to a mild or greater degree...
  92. ncbi Speech recognition in noise for cochlear implant listeners: benefits of residual acoustic hearing
    Christopher W Turner
    Dept of Speech Pathology and Audiology, Dept of Otolaryngology Head and Neck Surgery, University of Iowa, Iowa City, Iowa 52242, USA
    J Acoust Soc Am 115:1729-35. 2004
    ..The results are consistent with the idea that better perception of voice pitch, which can aid in separating voices in a background of other talkers, was responsible for this advantage...
  93. ncbi Educational attainment, labour force status and injury: a comparison of Canadians with and without deafness and hearing loss
    Kathryn Woodcock
    School of Occupational and Public Health, Ryerson University, Toronto, Ontario, Canada
    Int J Rehabil Res 31:297-304. 2008
    ..52 vs. 3.53%). Respondents classified as having a hearing problem, whether hearing loss or deafness, were more likely to have achieved less education, less likely to be working and experience higher ..
  94. ncbi The need for and development of an adaptive Listening and Communication Enhancement (LACE) Program
    Robert W Sweetow
    Department of Otolaryngology, University of California, San Francisco, USA
    J Am Acad Audiol 17:538-58. 2006
    ..Results showed statistically significant improvements for the trained subjects on all but one of the outcome measures. Barriers facing the widespread implementation of home-based aural rehabilitation are discussed...
  95. pmc Cisplatin ototoxicity and protection: clinical and experimental studies
    Leonard P Rybak
    Department of Surgery, Southern Illinois University School of Medicine, IL, USA
    Tohoku J Exp Med 219:177-86. 2009
    ..The future strategies for limiting cisplatin ototoxicity will need to avoid interference with the therapeutic effect of cisplatin in order to enhance the quality of life of patients receiving this important anti-tumor agent...
  96. pmc Five novel loci for inherited hearing loss mapped by SNP-based homozygosity profiles in Palestinian families
    Hashem Shahin
    Department of Life Sciences, Bethlehem University, Bethlehem, Palestine
    Eur J Hum Genet 18:407-13. 2010
    ..In 20 Palestinian kindreds with prelingual nonsyndromic hearing loss, we generated homozygosity profiles reflecting linkage to the phenotype...
  97. pmc Ablation of whirlin long isoform disrupts the USH2 protein complex and causes vision and hearing loss
    Jun Yang
    The Berman Gund Laboratory for the Study of Retinal Degenerations, Harvard Medical School, Massachusetts Eye and Ear Infirmary, Boston, Massachusetts, United States of America
    PLoS Genet 6:e1000955. 2010
    ..e., disruption of the PMC. Third, whirlin mutations that ablate the N-terminal PDZ domains lead to Usher syndrome, but non-syndromic hearing loss will result if they are spared.
  98. ncbi M34T and V37I mutations in GJB2 associated hearing impairment: evidence for pathogenicity and reduced penetrance
    Agnieszka Pollak
    Institute of Physiology and Pathology of Hearing, Warsaw, Poland
    Am J Med Genet A 143:2534-43. 2007
    ..79, P < 10(-5)). We tentatively suggest that M34T and V37I might cause mild HI characterized by relatively late onset and progression...
  99. pmc Mutations in the DBP-deficiency protein HSD17B4 cause ovarian dysgenesis, hearing loss, and ataxia of Perrault Syndrome
    Sarah B Pierce
    Department of Medicine, University of Washington, Seattle, WA 98195, USA
    Am J Hum Genet 87:282-8. 2010
    ....
  100. pmc c-Ret-mediated hearing loss in mice with Hirschsprung disease
    Nobutaka Ohgami
    Unit of Environmental Health Sciences, Department of Biomedical Sciences, College of Life and Health Sciences, Chubu University, 1200 Matsumoto, Kasugai, Aichi 487 8501, Japan
    Proc Natl Acad Sci U S A 107:13051-6. 2010
    ..Taken together with our results for three patients with congenital deafness with c-RET-mediated severe HSCR, our results indicate that c-Ret and c-RET are a deafness-related molecule in mice and humans...
  101. pmc Role for Toll-like receptor 2 in the immune response to Streptococcus pneumoniae infection in mouse otitis media
    Fengchan Han
    Department of Otolaryngology HNS, Case Western Reserve University, Cleveland, Ohio 44106, USA
    Infect Immun 77:3100-8. 2009
    ..Of those that survived, more severe hearing loss in the TLR2(-/-) mice than in the WT mice was indicated by an elevation in auditory-evoked brain stem response ..

Research Grants80

  1. NIH Director's Pioneer Award
    Junying Yuan; Fiscal Year: 2009
    ..No Abstract provided ..
  2. Targeting Inflammation for the Amelioration of Cisplatin-Hearing Loss
    Vickram Ramkumar; Fiscal Year: 2011
    ..Knockdown of these proteins reduced damage to OHCs and reduced hearing loss induced by cisplatin...
  3. Steroid Responsive Mechanisms in the Ear
    DENNIS ROYAL TRUNE; Fiscal Year: 2012
    ..Glucocorticoids (prednisone, methlyprednisolone, dexamethasone) have been employed for decades for control of hearing loss. However, little is known of the cellular and molecular processes of the ear that are under their control...
  4. Genetic Studies of Optic Atrophy
    Taosheng Huang; Fiscal Year: 2012
    ..The hearing loss is associated with asynchronous cochlear conduction and cochlear implants provide remarkable results to restore ..
  5. Transplatin: A Novel Agent to Mitigate Cisplatin Toxicity
    Vickram Ramkumar; Fiscal Year: 2013
    ..short interfering (si) RNAs into the cochlea reduced damage to OHCs and attenuated cisplatin-induced hearing loss in rat...
  6. Predicting Bilirubin-induced Auditory Toxicity in Infants with Severe Jaundice
    Sanjiv B Amin; Fiscal Year: 2010
    Severe jaundice causes chronic post-kernicteric encephalopathy, sensori-neural hearing loss (SNHL), and auditory neuropathy (AN) in infants...
  7. WIDEBAND CLINICAL DIAGNOSIS AND MONITORING OF MIDDLE-EAR AND COCHLEAR FUNCTION
    Douglas H Keefe; Fiscal Year: 2013
    ..in newborn hearing screening (NHS) and diagnostic programs, and may improve adult programs to identify ototoxic hearing loss and diagnose middle-ear disease in patients receiving middle-ear surgery...
  8. MECHANISMS OF FGF RECEPTOR REGULATION AND SIGNALING
    Moosa Mohammadi; Fiscal Year: 2013
    ..Perturbed FGF signaling leads to numerous human diseases, including skeletal, reproductive syndromes, hearing loss, renal phosphate wasting, neurodegenerative disorders, and cancer...
  9. Amelioration of cisplatin ototoxicity by transplatin
    Debashree Mukherjea; Fiscal Year: 2010
    ..Specific Aim 1 will determine the effectiveness of transplatin against cisplatin-induced hearing loss, using either systemic administration or by direct round window application of transplatin...
  10. Mechanisms of Cochlear Oxidative Stress Injury and Strategies to Protect Hearing
    Michael J Brenner; Fiscal Year: 2013
    ..the treatment of life-threatening infections, but they are also associated with a risk of permanent, bilateral hearing loss, as well as balance disturbance, tinnitus, and nephrotoxicity...
  11. Evolution of the Immune Response to Cytomegalovirus Transmitted via Breast Milk
    Lynn Puddington; Fiscal Year: 2010
    ..HCMV) infection in utero (congenital) can result in growth retardation, microcephaly, sensorineural hearing loss, and even death. HCMV infection also occurs postnatally in newborns via viral transmission in breast milk...
  12. IDENTIFICATION OF ACOUSTICO-LATERALIS TRANSMITTERS
    Dennis G Drescher; Fiscal Year: 2013
    ..and balance, pointing the way to development of biochemical and genetic therapies for transmitter-related hearing loss, vertigo, and tinnitus...
  13. Characterization of an essential mitochondrial rRNA methyltransferase
    Kip E Guja; Fiscal Year: 2013
    ..be related to the effect of aminoglycoside antibiotics on the mitochondrial ribosome and aminoglycoside-induced hearing loss. Consistently, aminoglycoside sensitivity in humans is often maternally inherited, and methylation activity by ..
  14. Understanding thyroid hormone regulation of neurogenesis in the cochlea
    Mirna Mustapha; Fiscal Year: 2013
    ..no available clinical cures for hereditary inner ear disease or reliable means of preventing age related hearing loss. The mouse serves as an ideal mammalian model for understanding genetic risk factors for hearing impairment and ..
  15. Preclinical testing of a novel PDK1 inhibitor for treating vestibular schwannoma
    Abraham Jacob; Fiscal Year: 2013
    ..VS are intracranial tumors that cause sensorineural hearing loss, balance abnormalities, tinnitus, vertigo, facial weakness, hydrocephalus, seizures, blindness, and even death...
  16. Basic and Clinical Studies of Noise-Induced and Age-Related Hearing Loss
    Sharon G Kujawa; Fiscal Year: 2012
    ..It suggests that primary neuronal loss is a more common and important aspect of acquired sensorineural hearing loss than previously thought...
  17. Endothelial-reactive antibodies: a diagnostic test for Susac's syndrome
    WILLIAM JAMES WALDMAN; Fiscal Year: 2011
    ..disorder characterized by a clinical triad of encephalopathy, branch retinal artery occlusion (BRAO), and hearing loss. It appears to result from microangiopathy affecting the precapillary arterioles of the brain, retina, and ..
  18. Basic Mechanisms in Hearing Loss of Cochlear Origin
    Allen F Ryan; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Objectives: Sensorineural hearing loss (SNHL) is strongly associated with many aspects of military service including blast injury...
  19. Functional characterization of the Bronx waltzer deafness gene
    Botond Banfi; Fiscal Year: 2013
    ..One of these strains is Bronx waltzer (bv), which was described in 1979 with respect to the hearing loss and "waltzing" behavior observed in homozygous mutants...
  20. Familial and Birth Cohort Effects on the Aging Senses
    Karen J Cruickshanks; Fiscal Year: 2013
    ..Stored samples from adults, ages 45-79 yrs in the BOSS and the parent cohort seen in the Epidemiology of Hearing Loss Study (AG11099), will be genotyped using a 50,000 SNP panel of candidate genes for cardiovascular disease, ..
  21. COCHLEAR MACROPHAGES AND EPITHELIAL REPAIR
    Keiko Hirose; Fiscal Year: 2013
    ..Research in human subjects demonstrates that hearing loss is successfully treated with corticosteroids in some patients, presumably due to their anti-inflammatory and ..
  22. Matrix Otopathology
    MICHAEL ANNE NMI GRATTON; Fiscal Year: 2011
    ..The relationship between abnormal basement membranes and hearing loss is explored by determining the consequences of basement membrane accumulation on peripheral auditory function;..
  23. INTRACELLULAR CALCIUM IN HAIR CELLS
    Robert Fettiplace; Fiscal Year: 2013
    ..Hearing impairment is the most common disabling sensory defect in humans. Severe to profound hearing loss, largely attributable to injury to the sensory hair cells, affects 1 in 1,000 newborns, and 60% of people older ..
  24. Genetics of Age-Related Hearing Loss
    Kenneth R Johnson; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Presbycusis or age-related hearing loss (AHL) is the most common sensory deficit in aging human populations and a major factor in reducing the quality of life of the elderly...
  25. Effects of nerve demyelination on auditory synaptic functions
    Jun Hee Kim; Fiscal Year: 2012
    ..lay the foundations for the development of novel therapeutic strategies for preventing a permanent childhood hearing loss and an adult hearing impairment due to the consequences of demyelinating diseases like MS or auditory ..
  26. Genomic approaches to inner ear hair cell regeneration
    Michael Lovett; Fiscal Year: 2010
    ..PUBLIC HEALTH RELEVANCE: Damage to inner ear hair cells is a leading cause of irreversible human hearing loss and balance disorders, which affect >10% of the world's population...
  27. Inner Ear Impact of Chronic Middle Ear Inflammation
    DENNIS ROYAL TRUNE; Fiscal Year: 2013
    ..into the inflammatory mechanisms by which otitis media causes permanent cochlear changes, such as sensorineural hearing loss. Our preliminary studies also showed that steroid treatments can reverse these cochlear problems if caught ..
  28. Novel biofilm polymicrobial infection-resistant implants for otologic application
    Paul Stoodley; Fiscal Year: 2010
    ..tube otorrhea (PTTO) is often refractory to treatment and inflicts significant morbidity in the form of hearing loss and long-term middle ear dysfunction...
  29. ENDOGENOUS MODULATION OF COCHLEAR INJURY
    Leonard P Rybak; Fiscal Year: 2012
    ..This enzyme generates superoxide radicals that can damage outer hair cells (OHCs), leading to hearing loss. ROS can activate cochlear genes that may be protective or harmful...
  30. Novel immunotherapeutics for the management of otitis media due to H. influenzae
    Steven D Goodman; Fiscal Year: 2013
    ..e. hearing loss due to OM), a goal of Strategic Plan Priority Area I.
  31. For Hearing Component in the Hispanic Community Health Study (HCHS)
    Lisa Lavange; Fiscal Year: 2009
    ..NIDCD is providing funding to support audiometry exams and questions relating to hearing loss/hearing aid use, tinnitus, noise exposure, and other factors that affect hearing thresholds...
  32. Environment, Novel Aging Outcomes, and Genetics
    Sung Kyun Park; Fiscal Year: 2013
    ..two novel outcomes that have been relatively little studied in environmental health, age-related cataract and hearing loss. In addition, the project aims to identify factors that impact susceptibility to such pollutants in an aging ..
  33. CODING IN AUDITORY NEURONS: EFFECTS OF AMINO ACIDS
    Donald M Caspary; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Age-related hearing loss is a complex disorder affecting 30% of the US population aged 65 to 74 years, and 50% of the population over 75 years of age and older...
  34. Novel vaccine to CMV based on a disc virus
    Alistair McGregor; Fiscal Year: 2010
    ..1% of live births in the US) can lead to serious symptomatic disease including mental retardation and hearing loss. Indeed it is estimated that congenital HCMV related sensorineural hearing loss (SNHL) occurs at a greater ..
  35. A cell-specific inducible model of hearing loss
    Albert S Edge; Fiscal Year: 2011
    ..cell types in the cochlea including hair cells and spiral ganglion neurons, two critical cell types for hearing loss. Such a model would expedite studies on cell function and development as well as inner ear regeneration...
  36. Brain changes in tinnitus
    Josef P Rauschecker; Fiscal Year: 2010
    ..external stimulus, is perceived by about 15% of the adult population, and about 75% of all patients with hearing loss. It can cause severe suffering, and to date, no reliable cure has been found...
  37. Identifying the Atoh1 targetome in hair cells with deep sequencing
    Andrew K Groves; Fiscal Year: 2013
    ..1% of newborns having some form of hereditary hearing loss and roughly half of all adults suffering from some degree of hearing loss by the time they reach retirement age...
  38. Transport ATPases: From Molecules to Maladies
    Rajini Rao; Fiscal Year: 2010
    ..them: cancer and drug resistance, heart disease, immune and infectious diseases, ulcers, diabetes, vision and hearing loss, neurodegeneration and mitochondrial diseases...
  39. Myosin 15:Genetics, Pathology and Therapeutic Potential
    Sally A Camper; Fiscal Year: 2013
    ..Although there is no enhanced risk of age related hearing loss in double heterozygotes, these studies revealed unique functions of each myosin gene, and suggested the ..
  40. DETERMINANTS OF H. INFLUENZAE VIRULENCE IN OTITIS MEDIA
    Lauren O Bakaletz; Fiscal Year: 2013
    ..physician's office and emergency room visits, undergo surgery requiring general anesthesia, and experience hearing loss. The widely held practice of prescribing of broad-spectrum antibiotics to treat OM has been a major driving ..
  41. Auditory Neuroprotection by Small Molecule Agonists of the TrkB Receptor
    Keqiang Ye; Fiscal Year: 2013
    ..to the human cochlea to promote the survival of residual SG neurons in the cochlea of severe sensorineural hearing loss patients...
  42. INTRACOCHLEAR ELECTROCHEMICAL GRADIENTS
    Alexander A Spector; Fiscal Year: 2012
    ..the anion transport properties of its close family members including pendrin, a mutation of which also leads to hearing loss. In addition to potential benefits to the hearing impaired, elucidation of the physical principles underlying ..
  43. Preventing hair cell loss by regulating prestin's function
    Jing Zheng; Fiscal Year: 2010
    ..OHCs are also the most vulnerable cells in the inner ear and are involved in a majority of sensorineural hearing loss. Although OHC regeneration through either stem cell replacement or gene therapy provides an exciting option in ..
  44. Regeneration of stereocilia of cochlear hair cells
    David Z Z He; Fiscal Year: 2010
    ..repairing injured hair cells are essential to the biological remediation and prevention of hair cell-related hearing loss and deafness...
  45. Novel Approaches for Prevention and Treatment of Noise Induced Hearing loss
    Debashree Mukherjea; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Noise induced hearing loss (NIHL) is a significant problem in the United States, affecting ~15 percent of individuals from all age groups...
  46. Light-Alarming Personal Noise Dosimeter System
    ALEXANDER MILOVANOV; Fiscal Year: 2013
    ..Occupational noise is a widespread risk factor, linking it strongly to critical health hazards, such as hearing loss, psychiatric disorders, increased blood pressure, and harmful biochemical, immune system, and birth-weight ..
  47. The Role of Eya4 in Hearing and Disease
    Jonathan G Seidman; Fiscal Year: 2010
    ..Mutations in the transcriptional co-activator EYA4 are responsible for autosomal dominant sensorineural hearing loss at the DFNA10 locus. Mice carrying mutant Eya4 alleles provide a model of human hearing loss...
  48. Genetic hearing screening for newborns
    Xi Lin; Fiscal Year: 2009
    ..More importantly, current methods for UNHS are unable to detect early-onset hearing loss and provide a diagnosis that defines underlying etiology among those real positives...
  49. Cellular therapy for the inner ear
    Albert S Edge; Fiscal Year: 2013
    ..of neural connections to hair cells in the adult ear is important to any therapeutic approach to sensorineural hearing loss. The Specific Aims comprise 3 inter-related experiments to probe key variables likely to influence the success ..
  50. CELLULAR AND MOLECULAR BIOLOGY OF CHOLESTEATOMA
    RICHARD ARTHUR CHOLE; Fiscal Year: 2013
    ..are epithelial cyst-like structures that expand into adjacent bone, resulting in chronic infection and hearing loss. The overall goal of this proposal is to investigate the mechanisms of bacterial-host interactions in the ..
  51. Assessing the Impact of Osteogenesis Imperfecta on Non-Skeletal Systems
    Laura Tosi; Fiscal Year: 2012
    ..Thus, individuals with OI may experience impaired dentition, joint laxity, hearing loss, and a wide range of cardiac problems...
  52. MOLECULAR BASIS OF INHERITED DEAFNESS
    David P Corey; Fiscal Year: 2013
    ..characterized by congenital deafness and progressive blindness, but it is not known how these mutations cause hearing loss. We will investigate the binding between these cadherins, by solving the crystal structures of the distal ends ..
  53. Mitochondrial Thioredoxin, Caloric Restriction, and Age-related Hearing Loss
    Shinichi Someya; Fiscal Year: 2013
    ..of a variety of laboratory animals and delays the progression of age-associated diseases such as age-related hearing loss (AHL) (1-9), a common feature of aging that affects more than 40% of people over 65 years of age in the US (13)...
  54. Middle-ear Mechanics in Normal and Pathological Ears
    John J Rosowski; Fiscal Year: 2013
    ..S., are common causes of significant conductive hearing loss that range in severity up to 60 dB...
  55. Molecular mechanisms of auditory nAChR synapse assembly
    Michele H Jacob; Fiscal Year: 2013
    Abstract: Sensorineural hearing loss affects 30 million Americans. Aging, noise-overexposure, infection and ototoxic antibiotics all lead to sensory hair cell degeneration and permanent hearing loss...
  56. Confirmatory Screening for Congenital Non-Syndromic Genetic Hearing Loss Using Ta
    Edwin W Naylor; Fiscal Year: 2013
    ..provided by applicant): Approximately 8,000 children in the USA are born each year with some form of hearing loss severe enough to require special services...
  57. Mechanisms of Sensory Regeneration
    Mark Warchol; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Hearing loss and vertigo are serious health problems that currently affect millions of Americans...
  58. Repair of HCMV-Induced DNA Damage in Infected Cells
    Elizabeth A Fortunato; Fiscal Year: 2013
    ..Ramifications of HCMV infection are primarily observed in the central nervous system (CNS) and include hearing loss, vision loss, microcephaly and mental retardation...
  59. Stimuli promoting the survival of spiral ganglion neurons
    Steven H Green; Fiscal Year: 2011
    ..Sensorineural hearing loss affects about 20,000,000 Americans and the only current means to replace the function of the lost sensory cells ..
  60. Occupational exposure to manganese and noise potentiate hearing loss
    Richard J Salvi; Fiscal Year: 2013
    ..Moreover, we hypothesize that Mn-induced hearing loss observed in welders is exacerbated by the high levels of noise present in the occupational working environment...
  61. The role of cochlear pericytes in control of cochlear microcirculation
    Xiao Rui Shi; Fiscal Year: 2009
    ..Dysfunctions in the cochlear blood supply can cause serious hearing disorders including sudden sensorineural hearing loss, presbyacusis, noise-induced hearing loss, tinnitus, and certain vestibulopathies...
  62. PROTECTION FROM AMINOGLYCOSIDE OTOTOXICITY
    Jochen Schacht; Fiscal Year: 2013
    ..Aminoglycosides are indispensable antibiotics commonly used worldwide and a primary cause of preventable hearing loss. They have traditional roles against infectious diseases such as tuberculosis and in the prophylactic treatment ..
  63. DEAFNESS AND OSSIFICATION IN LABYRINTHITIS OSSIFICANS
    Hilary Brodie; Fiscal Year: 2004
    ..It is multifactorial in origin and may result in deafness. Profound hearing loss and LO in children are most commonly associated with meningogenic labyrinthitis...
  64. AGE RELATED SYNAPTIC CHANGES IN THE AUDITORY BRAINSTEM
    Robert Helfert; Fiscal Year: 1999
    Age-related hearing loss (presbycusis) is the major communication disorder of industrialized society and is second only to arthritic diseases in its impact on the quality of life for people over 65...
  65. Targeting S1P receptors to prevent hearing loss
    Jerold Chun; Fiscal Year: 2010
    Targeting S1P receptors to prevent hearing loss Cochlear hair cells, the primary functional subunits required for the transduction of vibration into the auditory sensation, are subject to irreversible degeneration and consequent loss of ..
  66. Can L-carnitine prevent drug-induced hearing loss?
    Gilda Kalinec; Fiscal Year: 2004
    b>Hearing loss is a prevalent and chronic health condition that affects between 22 and 28 million Americans...
  67. DEVELOPMENT OF A DRUG THERAPY TO AMELIORATE PERMANENT HEARING LOSS
    Jianxin Bao; Fiscal Year: 2010
    ..Noise-induced hearing loss (NIHL) is the second most common form of sensorineural hearing deficit, after age-related hearing loss (..
  68. Determinants of Word Recognition Speed in Older Listeners
    Frederick J Gallun; Fiscal Year: 2011
    ..This problem is common even among older adults who do not have hearing loss. However, it is aggravated by hearing impairment, and among those so impaired, is not corrected through hearing ..
  69. Hearing Therapies for the Future
    Gabrielle Saunders; Fiscal Year: 2007
    ..The conference objective is to expand understanding and facilitate effective treatment of noise induced hearing loss (NIHL)...
  70. IMPROVED STEROID THERAPY FOR HEARING LOSS
    Dennis Trune; Fiscal Year: 2000
    In spite of years of treating various forms of hearing loss with glucocorticoids (prednisone, dexamethasone), there is virtually no clinical or experimental evidence that inflammation of the ear is the underlying etiology...
  71. The Ear-Brain System: Approaches to the Study and Treatment of Hearing Loss
    Gabrielle Saunders; Fiscal Year: 2009
    ..for the upcoming 4th Biennial Conference titled "The Ear-Brain System: Approaches to the Study and Treatment of Hearing Loss." It will take place in Portland, Oregon on October 7th-9th, 2009...
  72. Tribal Community-Based Prevention of Noise-Induced Hearing Loss and Tinnitus
    William Martin; Fiscal Year: 2007
    unreadable] DESCRIPTION (provided by applicant): Native Americans are 2-4 times likely to have hearing loss compared to white, African American, or Asian Americans...
  73. Ecological Momentary Assessment in Hearing Disorders
    James Henry; Fiscal Year: 2009
    The two most common auditory disorders among middle-aged Americans are hearing loss and tinnitus...
  74. ANTIBODY INDUCED HEARING LOSS--MODEL FOR IMMUNE DEAFNESS
    Thomas Carey; Fiscal Year: 1999
    Sensorineural hearing loss (SNHL) of unexplained etiology is a common entity with an incidence rate of 1:10,000 for which there are few effective treatments...
  75. TOOTH LOSS AND AGE RELATED HEARING LOSS
    Herenia Lawrence; Fiscal Year: 1999
    This study will evaluate a hypothetical association between tooth loss and age-related hearing loss in health male adults...
  76. IDENTIFYING RISK FACTORS FOR NOISE INDUCED HEARING LOSS
    Peter Rabinowitz; Fiscal Year: 2000
    Noise-induced hearing loss is one of the most common occupational diseases. Thirty million US workers are exposed to potentially hazardous workplace noise...
  77. CTL2 ELISA: A Diagnostic Test for Autoimmune Hearing Loss
    Vijay Kumar; Fiscal Year: 2007
    unreadable] DESCRIPTION (Provided by applicant): Autoimmune hearing loss (AHL) is a subset of sensorineural hearing loss (SNHL) in which there is a sudden onset, or rapidly progressing and/or fluctuating hearing loss that often ..
  78. Congenital CMV Infection and Hearing Loss in Rural Indian Population
    Suresh B Boppana; Fiscal Year: 2010
    Congenital CMV infection is a leading cause of hearing loss and brain disease in children and the Institute of Medicine has targeted CMV for vaccine development...
  79. SENSORINEURAL HEARING LOSS--PERILYMPHATIC FISTULAS
    Robert Kohut; Fiscal Year: 1999
    Sudden or rapidly progressive sensorineural hearing loss and/or specific balance disorders appear to be related to perilymphatic fistulas (PLF). This statement is based on both clinical and human temporal hone studies...
  80. Effects of Acoustic Trauma on Age-Related Hearing Loss
    Dianne Durham; Fiscal Year: 2002
    b>Hearing loss is a common consequence of aging, with 70% of older Americans reporting some degree of hearing or balance disorder...