Genomes and Genes
sensorineural hearing loss
Summary: Hearing loss resulting from damage to the COCHLEA and the sensorineural elements which lie internally beyond the oval and round windows. These elements include the AUDITORY NERVE and its connections in the BRAINSTEM.
Articles from Journal RESEARCH
Articles from Journal RESEARCH1
Publications322 found, 100 shown here
- Sensorineural hearing loss in childrenRichard J H Smith
Molecular Otolaryngology Research Laboratories, Department of Otolaryngology, University of Iowa, Iowa City, IA, USA
Lancet 365:879-90. 2005During the past three to four decades, the incidence of acquired sensorineural hearing loss (SNHL) in children living in more developed countries has fallen, as a result of improved neonatal care and the widespread implementation of ..
- Treatment of sudden sensorineural hearing loss: II. A Meta-analysisAnne Elizabeth Conlin
Department of Otolaryngology, University of Ottawa, Ottawa, Ontario, Canada
Arch Otolaryngol Head Neck Surg 133:582-6. 2007To pool and meta-analyze the results of all randomized controlled trials (RCTs) on treatment of sudden sensorineural hearing loss (SSHL).
- Clinical practice. Idiopathic sudden sensorineural hearing lossSteven D Rauch
Department of Otology and Laryngology, Harvard Medical School, Boston, USA
N Engl J Med 359:833-40. 2008
- Effects of sensorineural hearing loss and personal hearing AIDS on cortical event-related potential and behavioral measures of speech-sound processingPeggy A Korczak
Department of Audiology, Speech Language Pathology and Deaf Studies, Towson University, Towson, Maryland 21252 0001, USA
Ear Hear 26:165-85. 2005To systematically investigate the combined effects of sensorineural hearing loss and prescribed personal hearing aid(s) on cortical event-related potentials (ERPs) (waves N1, MMN, N2b, and P3b) and their related behavioral measures of ..
- A spiking neuron model of cortical correlates of sensorineural hearing loss: Spontaneous firing, synchrony, and tinnitusMelissa Dominguez
Neural Comput 18:2942-58. 2006..These changes allow the model to capture some of the cortical correlates of sensorineural hearing loss, including changes in spontaneous firing and synchrony; these phenomena may explain central tinnitus...
- A novel gene for Usher syndrome type 2: mutations in the long isoform of whirlin are associated with retinitis pigmentosa and sensorineural hearing lossInga Ebermann
Institute of Human Genetics, University Hospital of Cologne, Kerpener Str 34, 50931 Cologne, Germany
Hum Genet 121:203-11. 2007Usher syndrome is an autosomal recessive condition characterized by sensorineural hearing loss, variable vestibular dysfunction, and visual impairment due to retinitis pigmentosa (RP)...
- Mutations in TMC1 contribute significantly to nonsyndromic autosomal recessive sensorineural hearing loss: a report of five novel mutationsAsli Sirmaci
Division of Clinical Molecular Pathology and Genetics, Department of Pediatrics, Ankara University School of Medicine, Ankara, Turkey
Int J Pediatr Otorhinolaryngol 73:699-705. 2009..Turkish families with autosomal recessive nonsyndromic severe to profound congenital or prelingual-onset sensorineural hearing loss (SNHL)...
- Biochemical characterization of the mitochondrial tRNASer(UCN) T7511C mutation associated with nonsyndromic deafnessXiaoming Li
Division and Program in Human Genetics and Center for Hearing and Deafness Research, Cincinnati Children s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45229, USA
Nucleic Acids Res 32:867-77. 2004..These observations imply that a combination of the T7511C mutation with two mtDNA mutations accounts for the high penetrance of deafness in this family...
- Sudden sensorineural hearing lossBenjamin E Schreiber
Royal Free Hampstead NHS Trust, London, UK
Lancet 375:1203-11. 2010Sudden sensorineural hearing loss is usually unilateral and can be associated with tinnitus and vertigo. In most cases the cause is not identified, although various infective, vascular, and immune causes have been proposed...
- Diffusion tensor imaging of the auditory pathway in sensorineural hearing loss: changes in radial diffusivity and diffusion anisotropyYuchun Lin
Department of Diagnostic Radiology, Chang Gung Memorial Hospital, and Department of Medical Imaging and Radiological Science, Chang Gung University, Taipei, Taiwan
J Magn Reson Imaging 28:598-603. 2008..of axial diffusivity (lambda parallel), radial diffusivity (lambda perpendicular), mean diffusivity (MD), and fractional anisotropy (FA) along the auditory pathway of patients with sensorineural hearing loss (SNHL) and normal controls.
- Prothrombotic gene mutations in patients with sudden sensorineural hearing loss and cardiovascular thrombotic diseasePasquale Capaccio
Department of Otorhinolaryngological and Ophthalmological Sciences, University of Milan, Fondazione IRCCS Policlinico, Mangiagalli e Regina Elena, Milan, Italy
Ann Otol Rhinol Laryngol 118:205-10. 2009Impaired cochlear perfusion seems to be an important event in sudden sensorineural hearing loss. Prothrombotic gene mutations have been related to vascular disorders and sudden hearing loss...
- KCNQ4 mutations associated with nonsyndromic progressive sensorineural hearing lossLiping Nie
Center for Neuroscience and Department of Otolaryngology Head and Neck Surgery, University of California Davis, Davis, California 95618, USA
Curr Opin Otolaryngol Head Neck Surg 16:441-4. 2008..This article provides an update on the current progress in identification of KCNQ4 mutations responsible for DFNA2, a subtype of autosomal dominant nonsyndromic progressive hearing loss...
- Clinical and genetic features of nonsyndromic autosomal dominant sensorineural hearing loss: KCNQ4 is a gene responsible in JapaneseJ Akita
Department of Otorhinolaryngology, Hirosaki University School of Medicine, Japan
J Hum Genet 46:355-61. 2001Sixteen Japanese nonsyndromic autosomal dominant sensorineural hearing loss (ADSNHL) families were investigated clinically as well as genetically. Most families showed postlingual hearing loss...
- Treatment of sudden sensorineural hearing loss: I. A systematic reviewAnne Elizabeth Conlin
Department of Otolaryngology, University of Ottawa, Ottawa, Ontario, Canada
Arch Otolaryngol Head Neck Surg 133:573-81. 2007To identify, evaluate, and review randomized controlled trials (RCTs) on the treatment of sudden sensorineural hearing loss (SSHL).
- Borate transporter SLC4A11 mutations cause both Harboyan syndrome and non-syndromic corneal endothelial dystrophyJulie Desir
Laboratory for Medical Genetics, ULB, Brussels, Belgium
J Med Genet 44:322-6. 2007..Why some mutations cause hearing loss in addition to corneal dystrophy is presently unclear. These findings extend the implication of the SLC4A11 borate transporter beyond corneal dystrophy to perceptive deafness...
- The tectorial membrane: one slice of a complex cochlear sandwichGuy P Richardson
School of Life Sciences, University of Sussex, Falmer, Brighton, UK
Curr Opin Otolaryngol Head Neck Surg 16:458-64. 2008....
- Mutation in the transcriptional coactivator EYA4 causes dilated cardiomyopathy and sensorineural hearing lossJost Schönberger
Harvard Medical School, Department of Genetics, 77 Avenue Louis Pasteur, Boston, Massachusetts 02115, USA
Nat Genet 37:418-22. 2005We identified a human mutation that causes dilated cardiomyopathy and heart failure preceded by sensorineural hearing loss (SNHL)...
- Mutations in the WFS1 gene that cause low-frequency sensorineural hearing loss are small non-inactivating mutationsKim Cryns
Department of Medical Genetics, University of Antwerp, Universiteitsplein 1, B 2610 Antwerp, Belgium
Hum Genet 110:389-94. 2002..In contrast, 64% of the Wolfram syndrome mutations are inactivating. Our results indicate that only non-inactivating mutations in WFS1 are responsible for non-syndromic low-frequency hearing impairment...
- Progressive hearing loss, and recurrent sudden sensorineural hearing loss associated with GJB2 mutations--phenotypic spectrum and frequencies of GJB2 mutations in AustriaAndreas R Janecke
Institute of Medical Biology and Human Genetics, University of Innsbruck, Schöpfstr 41, A 6020 Innsbruck, Austria
Hum Genet 111:145-53. 2002..We screened 204 consecutive patients with non-syndromic sensorineural hearing loss for GJB2 mutations. Causative GJB2mutations were identified in 31 (15...
- Sensorineural hearing loss in infants with neonatal jaundice in Lagos: a community-based studyB O Olusanya
Maternal and Child Health Unit, Department of Community Health and Primary Care, College of Medicine, University of Lagos, Nigeria
Ann Trop Paediatr 29:119-28. 2009To establish the incidence and predictors of sensorineural hearing loss (SNHL) in infants with neonatal jaundice (NNJ) in an inner-city setting with predominantly non-hospital births.
- Connexin 26 studies in patients with sensorineural hearing lossM A Kenna
Department of Otology and Laryngology, Harvard Medical School, Boston, MA, USA
Arch Otolaryngol Head Neck Surg 127:1037-42. 2001To determine the spectrum of connexin 26 (Cx26) mutations and their phenotypes in children with sensorineural hearing loss (SNHL) or mixed hearing loss (MHL).
- Profound bilateral sensorineural hearing loss in nigerian children: any shift in etiology?A D Dunmade
University of Ilorin, P O Box 6641, Ilorin 240001, Nigeria
J Deaf Stud Deaf Educ 12:112-8. 2007..This study seeks to identify the present causes of profound sensorineural hearing loss in Nigeria, which in our environment is almost synonymous to a life sentence of silence and isolation...
- Non-syndromic progressive hearing loss DFNA38 is caused by heterozygous missense mutation in the Wolfram syndrome gene WFS1T L Young
Department of Genomic Sciences, University of Washington, Seattle, WA 98195 7720, USA
Hum Mol Genet 10:2509-14. 2001..The possibility that 'mild' mutations in WFS1 might be a cause of non-syndromic deafness in the general population should be explored...
- Short-term outcome and prognosis of acute low-tone sensorineural hearing loss by administration of steroidT Fuse
Department of Otolaryngology, Yamagata University School of Medicine, Yamagata, Japan
ORL J Otorhinolaryngol Relat Spec 64:6-10. 2002..High-dose steroid cured some patients who failed to recover with low-dose steroid therapy. Our results showed that steroid is one of the effective therapies for ALHL and supported that etiology of ALHL involves an immune response...
- Measurements of normal inner ear on computed tomography in children with congenital sensorineural hearing lossMing Ying Lan
Department of Otolaryngology, Taichung Veterans General Hospital, 160, Section 3, Chung Kang Rd, Taichung, 40705, Taiwan, ROC
Eur Arch Otorhinolaryngol 266:1361-4. 2009..of the inner ear to see whether there are subtle bony malformations in children with congenital sensorineural hearing loss (SNHL) whose temporal bone computed tomography (CT) are grossly normal...
- Familial Axenfeld-Rieger anomaly, cardiac malformations, and sensorineural hearing loss: a provisionally unique genetic syndrome?S Grosso
Department of Pediatrics, University of Siena, Siena, Italy
Am J Med Genet 111:182-6. 2002..Here we describe a new family in which ARA is associated with cardiac malformations and sensorineural hearing loss. No abnormalities of the teeth, facial bone, or periumbilical skin, which are considered of paramount ..
- Risk factors for sensorineural hearing loss in NICU infants compared to normal hearing NICU controlsS Coenraad
Department of Otorhinolaryngology, Sophia Children s Hospital Erasmus Medical Center, Rotterdam, Netherlands
Int J Pediatr Otorhinolaryngol 74:999-1002. 2010To evaluate independent etiologic factors associated with sensorineural hearing loss in infants who have been admitted to the neonatal intensive care unit compared to normal hearing controls.
- Two temporal bone computed tomography measurements increase recognition of malformations and predict sensorineural hearing lossDerk D Purcell
Department of Radiology, University of California at San Francisco, San Francisco, CA, USA
Laryngoscope 116:1439-46. 2006The objectives of this prospective study were to assess the reproducibility of the measurements of the cochlea and lateral semicircular canal (LSCC) and to determine if abnormal measurements predict sensorineural hearing loss (SNHL).
- Retinitis pigmentosa and progressive sensorineural hearing loss caused by a C12258A mutation in the mitochondrial MTTS2 geneF C Mansergh
Wellcome Ocular Genetics Unit, Genetics Department, Trinity College Dublin, Dublin 2, Ireland
Am J Hum Genet 64:971-85. 1999Family ZMK is a large Irish kindred that segregates progressive sensorineural hearing loss and retinitis pigmentosa. The symptoms in the family are almost identical to those observed in Usher syndrome type III...
- Auditory neural pathway evaluation on sensorineural hearing loss using diffusion tensor imagingYongmin Chang
Department of Diagnostic Radiology, College of Medicine, Kyungpook National University, 2 50 Samduk dong, Jung ku, Daegu 700 412, Republic of Korea
Neuroreport 15:1699-703. 2004..In this study, we investigated the integrity of auditory pathway in patients of sensorineural hearing loss by means of fractional anisotropy of water diffusion to see any subtle changes of auditory pathways ..
- Connexin 26 R143W mutation associated with recessive nonsyndromic sensorineural deafness in AfricaG W Brobby
N Engl J Med 338:548-50. 1998
- The treatment of idiopathic sudden sensorineural hearing loss using pulse therapy: a prospective, randomized, double-blind clinical trialBoris O Westerlaken
Department of Otorhinolaryngology, University Medical Center Groningen, The Netherlands
Laryngoscope 117:684-90. 2007The etiology and treatment of idiopathic sudden sensorineural hearing loss (ISSHL) is still unclear. The anti-inflammatory effect of corticosteroids is thought to play an important part in the recovery from ISSHL...
- Apolipoprotein E alleles and sensorineural hearing lossGwen O'Grady
Department of Surgery Division of Speech Pathology and Audiology, Duke University Medical Center, Durham, NC 27710, USA
Int J Audiol 46:183-6. 2007The purpose of this paper is to determine if a relationship exists between APOE alleles and nonsyndromic, sensorineural hearing loss (SNHL) in adults...
- Usher syndrome 1D and nonsyndromic autosomal recessive deafness DFNB12 are caused by allelic mutations of the novel cadherin-like gene CDH23J M Bork
Laboratory of Molecular Genetics, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, Rockville, MD 20850, USA
Am J Hum Genet 68:26-37. 2001..A northern blot analysis of CDH23 showed a 9.5-kb transcript expressed primarily in the retina. CDH23 is also expressed in the cochlea, as is demonstrated by polymerase chain reaction amplification from cochlear cDNA...
- Perception of dissonance by people with normal hearing and sensorineural hearing lossJennifer B Tufts
Army Audiology and Speech Center, Walter Reed Army Medical Center, 6900 Georgia Avenue NW, Washington, DC 20307, USA
J Acoust Soc Am 118:955-67. 2005..of pairs of pure tones (PT dyads) or pairs of harmonic complex tones (HC dyads) is altered due to sensorineural hearing loss. Four normal-hearing (NH) and four hearing-impaired (HI) listeners judged the sensory dissonance of PT ..
- Reversible sensorineural hearing lossC Lee
Department of Otorhinolaryngology, William Harvey Hospital, Kennington Rd, Ashford, Kent, TN2 40LZ, UK
Int J Pediatr Otorhinolaryngol 66:297-301. 2002We present an unusual case of temporary sensorineural hearing loss in a 6-year-old child due to carbon monoxide. This was shown on both the audiograms and confirmed with objective testing using otoacoustic emissions...
- Major and minor temporal bone abnormalities in children with and without congenital sensorineural hearing lossJohn E McClay
Department of Otolaryngology Head and Neck Surgery, The University of Texas at Southwestern, 5323 Harry Hines Blvd, Dallas, TX 73235 9035, USA
Arch Otolaryngol Head Neck Surg 128:664-71. 2002To determine the extent of correlation between sensorineural hearing loss (SNHL) and abnormal temporal bone anatomy in children.
- Analysis of 60 patients after tympanotomy and sealing of the round window membrane after acute unilateral sensorineural hearing lossClaudia Gedlicka
Department of Otorhinolaryngology, Head and Neck Surgery, University of Vienna, Vienna, Austria
Am J Otolaryngol 30:157-61. 2009..This retrospective study was performed to evaluate the effectiveness of tympanotomy and sealing of the round window membrane after unilateral acute hearing loss...
- Treatment of idiopathic sudden sensorineural hearing loss with antiviral therapy: a prospective, randomized, double-blind clinical trialBoris Olivier Westerlaken
Department of Otorhinolaryngology, University Hospital Groningen, Groningen, The Netherlands
Ann Otol Rhinol Laryngol 112:993-1000. 2003A subclinical viral labyrinthitis has been postulated in the literature to elicit idiopathic sudden sensorineural hearing loss (ISSHL). An etiologic role for the herpes family is assumed...
- Sensorineural hearing loss and neural correlates of temporal acuity in the inferior colliculus of the C57BL/6 mouseJoseph P Walton
Otolaryngology Division of Department of Surgery, University of Rochester School of Medicine and Dentistry, Box 629, Rochester, NY 14642, USA
J Assoc Res Otolaryngol 9:90-101. 2008..We investigated the effects of sensorineural hearing loss (SNHL) on neural correlates of temporal resolution by recording the responses of inferior colliculus ..
- Hearing evaluation of intratympanic methylprednisolone perfusion for refractory sudden sensorineural hearing lossWandong She
Department of Otolaryngology Head and Neck Surgery, Nanjing Drum Tower Hospital, Nanjing University Medical School, 321 Zhongshan Road, Nanjing 210008, China
Otolaryngol Head Neck Surg 142:266-71. 2010To investigate the effectiveness and safety of intratympanic methylprednisolone perfusion (IMP) through a microcatheter in patients with sudden sensorineural hearing loss (SSNHL) who failed a conventional treatment.
- Vestibular-evoked myogenic potential in the prediction of recovery from acute low-tone sensorineural hearing lossChi Te Wang
Department of Otolaryngology, Far Eastern Memorial Hospital, Taipei, Taiwan
Ear Hear 31:289-95. 2010..applied to measure the interaural difference (IAD) of saccular responses in patients with acute low-tone sensorineural hearing loss (ALHL)...
- Randomized, double blind, placebo controlled trial on the safety and efficacy of continuous intratympanic dexamethasone delivered via a round window catheter for severe to profound sudden idiopathic sensorineural hearing loss after failure of systemic theStefan K Plontke
Department of Otorhinolaryngology, Head and Neck Surgery, University of Tubingen, Tubingen, Germany
Laryngoscope 119:359-69. 2009..To study the safety and efficacy of continuous intratympanic dexamethasone-phosphate (Dex-P) for severe to profound sudden idiopathic sensorineural hearing (ISSHL) or sudden idiopathic anacusis after failure of systemic therapy...
- Perilymphatic fistula in children with preexisting sensorineural hearing lossD G Pappas
Division of Otology, University of Alabama, School of Medicine, Birmingham
Laryngoscope 98:507-10. 1988Progression of preexisting sensorineural hearing loss (SNHL) in infants and children has been considered an indication for fistula exploration: is this approach warranted? On exploring 36 middle ears in 26 such children, we found a ..
- Edaravone, a free radical scavenger, in the treatment of idiopathic sudden sensorineural hearing loss with profound hearing lossHajime Sano
Department of Otolaryngology, Kitasato University, School of Medicine, 1 15 1 Kitasato Sagamihara, Kanagawa 228 8555, Japan
Auris Nasus Larynx 37:42-6. 2010..The aim of this study was to investigate whether edaravone could improve treatment result in idiopathic sudden sensorineural hearing loss (ISSHL) patients with severe hearing loss.
- Phonemic restoration by hearing-impaired listeners with mild to moderate sensorineural hearing lossDeniz Baskent
Starkey Hearing Research Center, Berkeley, CA 94704, USA
Hear Res 260:54-62. 2010..The overall findings suggest that, in addition to insufficient audibility, degradations in the bottom-up and/or top-down mechanisms as a result of hearing loss may limit or entirely prevent phonemic restoration...
- Idiopathic sudden sensorineural hearing loss: etiopathogenic aspectsPaulo Roberto Lazarini
Department of Otorhinolaryngology, School of Medical Sciences, Santa Casa de Sao Paulo, Brazil
Braz J Otorhinolaryngol 72:554-61. 2006Several factors have been postulated to elicit the etiology of idiopathic sudden sensorineural hearing loss. Through a bibliographic review, we made a critical analysis of the different etiopathogenic aspects of its clinical manifestation...
- Sensorineural hearing loss in patients with chronic otitis mediaSady Selaimen da Costa
Department of Otolaryngology, Head and Neck Surgery, Universidade Federal do Rio Grande do Sul, Hospital de Clinicas, Porto Alegre, Brazil
Eur Arch Otorhinolaryngol 266:221-4. 2009..An issue that has recently gained attention is additional sensorineural hearing loss due to chronic otitis media...
- Impact of methionine synthase gene and methylenetetrahydrofolate reductase gene polymorphisms on the risk of sudden sensorineural hearing lossMenachem Gross
Department of Otolaryngology Head and Neck Surgery, Hadassah Hebrew University Hospital, Jerusalem, Israel
Audiol Neurootol 11:287-93. 2006Idiopathic sudden sensorineural hearing loss (SSNHL) represents a frequently encountered otological disease of unknown etiology. In recent years, several inherited risk factors have been found in the pathogenesis of vascular diseases...
- Systemic hypotension and the development of acute sensorineural hearing loss in young healthy subjectsA Pirodda
Ear, Nose and Throat Section, Department of Surgical and Anaesthesiological Sciences, S Orsola Hospital, University of Bologna, Bologna, Italy
Arch Otolaryngol Head Neck Surg 127:1049-52. 2001Sudden sensorineural hearing loss (SSHL) is an acute disorder whose origin is often unclear. A vascular disorder may be a causative factor.
- Slight-mild sensorineural hearing loss in children: audiometric, clinical, and risk factor profilesBarbara K Cone
Department of Speech, Language and Hearing Sciences, University of Arizona, Tucson, Arizona, USA
Ear Hear 31:202-12. 2010..factor affecting speech, language, learning, and academic outcomes, but the risk factors for slight-mild sensorineural hearing loss (SNHL) have not been ascertained...
- Novel mutations in LHX3 are associated with hypopituitarism and sensorineural hearing lossAnna Rajab
Genetics Unit, DGHA, Ministry of Health, Muscat, Sultanate of Oman
Hum Mol Genet 17:2150-9. 2008..Clinical evaluation revealed that all four patients exhibit varying degrees of bilateral sensorineural hearing loss, which has not been previously reported in association with LHX3 mutations, in addition to ..
- Molecular analysis of the mitochondrial 12S rRNA and tRNASer(UCN) genes in paediatric subjects with non-syndromic hearing lossR Li
J Med Genet 41:615-20. 2004
- Neurotrophins and electrical stimulation for protection and repair of spiral ganglion neurons following sensorineural hearing lossRobert K Shepherd
The Bionic Ear Institute, 384 388 Albert Street, East Melbourne, Victoria 3002, Australia
Hear Res 242:100-9. 2008..NTs) have been shown to rescue spiral ganglion neurons (SGNs) from degeneration following a sensorineural hearing loss (SNHL)...
- Mutational spectrum of the WFS1 gene in Wolfram syndrome, nonsyndromic hearing impairment, diabetes mellitus, and psychiatric diseaseKim Cryns
Department of Medical Genetics, University of Antwerp, Antwerp, Belgium
Hum Mutat 22:275-87. 2003..In this paper, we provide an overview of the currently known disease-causing and benign allele variants of WFS1 and propose a potential genotype-phenotype correlation for Wolfram syndrome and LFSNHI...
- A common founder for the 35delG GJB2 gene mutation in connexin 26 hearing impairmentL Van Laer
Department of Medical Genetics, University of Antwerp, Universiteitsplein 1, B 2610 Antwerp, Belgium
J Med Genet 38:515-8. 2001..For the most remote SNP, we could not detect any association. We conclude that the 35delG mutation is derived from a common, albeit ancient founder...
- Cytomegalovirus shedding and delayed sensorineural hearing loss: results from longitudinal follow-up of children with congenital infectionLauren Stancik Rosenthal
Centers for Disease Control and Prevention, Atlanta, GA, USA
Pediatr Infect Dis J 28:515-20. 2009..The pathogenesis of cytomegalovirus (CMV)-related hearing loss is not well understood...
- Plastic changes in the central auditory system after hearing loss, restoration of function, and during learningJosef Syka
Institute of Experimental Medicine, Academy of Sciences of the Czech Republic, Prague, Czech Republic
Physiol Rev 82:601-36. 2002....
- Heteroplasmy for the 1555A>G mutation in the mitochondrial 12S rRNA gene in six Spanish families with non-syndromic hearing lossF J del Castillo
J Med Genet 40:632-6. 2003
- The BALB/c mouse as an animal model for progressive sensorineural hearing lossJ F Willott
Department of Psychology, Northern Illinois University, DeKalb 60115, USA
Hear Res 115:162-74. 1998To develop the BALB/c mouse strain as an animal model for the study of progressive sensorineural hearing loss, mice ranging in age from young adult through middle age were studied...
- Mutations in the Wolfram syndrome 1 gene (WFS1) are a common cause of low frequency sensorineural hearing lossI N Bespalova
Mental Health Research Institute, University of Michigan, Ann Arbor, MI 48109, USA
Hum Mol Genet 10:2501-8. 2001Non-syndromic low frequency sensorineural hearing loss (LFSNHL) affecting only 2000 Hz and below is an unusual type of hearing loss that worsens over time without progressing to profound deafness...
- Risk factors of sensorineural hearing loss in patients with ischemic strokeTomasz Przewozny
Department of ENT, Medical University of Gdansk, Gdansk, Poland
Otol Neurotol 29:745-50. 2008We investigated risk factors of sensorineural hearing loss (SHL) in patients with early stage of ischemic stroke (IS).
- Mapping quantitative trait loci for hearing loss in Black Swiss miceMeghan Drayton
Section on Neurogenetics, Laboratory of Molecular Biology, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, 5 Research Court, Rockville, MD 20850, USA
Hear Res 212:128-39. 2006..Haplotype analyses across the Cdh23 locus demonstrated the phylogenetic relationship between Black Swiss and common inbred strains...
- Treatment of corticosteroid-responsive autoimmune inner ear disease with methotrexate: a randomized controlled trialJeffrey P Harris
Division of Otolaryngology Head and Neck Surgery, University of California, San Diego, CA 92103 8895, USA
JAMA 290:1875-83. 2003..been proposed for the long-term management of corticosteroid-responsive, rapidly progressive, bilateral sensorineural hearing loss (autoimmune inner ear disease [AIED])...
- Carbon monoxide poisoning and sensorineural hearing lossM Shahbaz Hassan
Department of ENT, Queen Elizabeth Hospital, Birmingham, UK
J Laryngol Otol 117:134-7. 2003..We report two cases of carbon monoxide poisoning with the associated phenomenon of sensorineural hearing loss. Although hearing loss as a result of acute carbon monoxide exposure has previously been described, here ..
- Autoimmune sensorineural hearing lossJ Mathews
Department of Otolaryngology, Royal Liverpool University Hospital, Wigan, UK
Clin Otolaryngol Allied Sci 28:479-88. 2003Autoimmune sensorineural hearing loss has been increasingly recognized as a clinical entity since its description by McCabe in 1979...
- Diagnostic yield of high-resolution computed tomography for pediatric sensorineural hearing lossP J Antonelli
Department of Otolaryngology, University of Florida, Gainesville 32610 0264, USA
Laryngoscope 109:1642-7. 1999..The purpose of this study was to determine the diagnostic yield of high-resolution CT for pediatric sensorineural hearing loss (HL) (SNHL).
- Dilated cardiomyopathy and sensorineural hearing loss: a heritable syndrome that maps to 6q23-24J Schonberger
Department of Genetics and Howard Hughes Medical Institute, Harvard Medical School, Boston, MA 02115, USA
Circulation 101:1812-8. 2000Dilated cardiomyopathy (DCM) and sensorineural hearing loss (SNHL) are prevalent disorders that occur alone or as components of complex multisystem syndromes...
- Sensorineural hearing loss: potential therapies and gene targets for drug developmentLouisa S Tang
Huffington Center on Aging, Bobby R Alford Department of Otolaryngology Head and Neck Surgery, Baylor College of Medicine, Houston, Texas 77030, USA
IUBMB Life 58:525-30. 2006..This article highlights prospects to manipulate the mammalian hearing organ with gene and stem cell delivery to the inner ear to protect, repair or regenerate the hair cells, supporting cells and associated nerves...
- Classification and hearing evolution of patients with sudden sensorineural hearing lossN O Penido
Departamento de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço, Escola Paulista de Medicina, Universidade Federal de Sao Paulo, Sao Paulo, SP, Brasil
Braz J Med Biol Res 42:712-6. 2009..analyze clinical aspects, hearing evolution and efficacy of clinical treatment of patients with sudden sensorineural hearing loss (SSNHL)...
- Tinnitus suppression by electrical promontory stimulation (EPS) in patients with sensorineural hearing lossW Konopka
Department of Otolaryngology, Military Medical University Lodz, Rzgow Sucharskiego 2, 95-030, Lodz, Poland
Auris Nasus Larynx 28:35-40. 2001..In our opinion EPS could be a method of treatment for persistent tinnitus in cases which fail to respond to other methods...
- Confirmation of genetic homogeneity of nonsyndromic low-frequency sensorineural hearing loss by linkage analysis and a DFNA6/14 mutation in a Japanese familyKazuki Komatsu
Department of Human Genetics, Nagasaki University School of Medicine, Sakamoto, Nagasaki, Japan
J Hum Genet 47:395-9. 2002Nonsyndromic low-frequency sensorineural hearing loss (LFSNHL) comprises a group (DFNA1, DFNA6, DFNA14, and DFNA38) of hearing disorders affecting only frequencies below 2000 Hz, and is often associated with tinnitus...
- Totally implantable middle ear device for rehabilitation of sensorineural hearing loss: preliminary experience with the Esteem, EnvoyMaurizio Barbara
Department of Sensory Organs, ENT Clinic, II Medical School Sapienza University, Rome, Italy
Acta Otolaryngol 129:429-32. 2009According to the first series of implanted patients, Esteem 2 technology can be regarded as a safe and reliable system for hearing restoration in case of moderate to severe sensorineural hearing loss.
- The effects of weather on the incidence of sudden sensorineural hearing loss: a 5-year population-based studyHerng Ching Lin
School of Health Care Administration, Taipei Medical University, and Department of Psychiatry, Taipei Medical University Hospital, Taiwan
Audiol Neurootol 11:165-71. 2006..5-year population data to examine the association between weather conditions and the incidence of sudden sensorineural hearing loss (SSNHL) in Taiwan with a specific focus on ambient temperature, relative humidity, atmospheric pressure, ..
- Risk factors associated with sensorineural hearing loss in infants at the neonatal intensive care unit: 15-year experience at the National Institute of Perinatology (Mexico City)Carlos Fabián Martínez-Cruz
Departamento de Seguimiento Pediátrico, Instituto Nacional de Perinatologia, Dr Isidro Espinosa de los Reyes, Mexico, D F, Mexico
Arch Med Res 39:686-94. 2008Infants from neonatal intensive care units (NICU) are at high risk for sensorineural hearing loss (SNHL); however, risk factors may change from NICU to NICU and from country to country...
- Effects of nitric oxide on morphology of isolated cochlear outer hair cells: possible involvement in sensorineural hearing lossTimothy T K Jung
Division of Otolaryngology Head and Neck Surgery, Loma Linda University School of Medicine, and Jerry L Pettis Memorial Veterans Medical Center, Loma Linda, California, USA
Otol Neurotol 24:682-5. 2003..One of the inflammatory mediators of otitis media, nitric oxide, can damage cochlear outer hair cells...
- Genetic predisposition and sensorineural hearing loss following bacterial meningitis: is there a link?V Jayarajan
J Infect 44:210. 2002
- Phonological processing, language, and literacy: a comparison of children with mild-to-moderate sensorineural hearing loss and those with specific language impairmentJ Briscoe
Oxford Study of Children s Communication Impairments, University of Oxford, UK
J Child Psychol Psychiatry 42:329-40. 2001..language ability, and literacy scores were compared for four groups: 19 children with mild-to-moderate sensorineural hearing loss (SNH), 20 children with specific language impairment (SLI), 20 controls matched on chronological age to ..
- Pontobulbar palsy and neurosensory deafness (Brown-Vialetto-van Laere syndrome) with hyperintense brainstem nuclei on magnetic resonance imaging: new finding in three siblingsRoshan Koul
Division of Paediatric Neurology, Department of Child Health, Sultan Qaboos University Hospital, PO Box 38, Al Khod 123, Muscat, Sultanate of Oman
J Child Neurol 21:523-5. 2006..Optic nerve involvement and hyperintensity of the brainstem nuclei on magnetic resonance imaging (MRI) are two new features described in this report...
- Modifier controls severity of a novel dominant low-frequency MyosinVIIA (MYO7A) auditory mutationV A Street
J Med Genet 41:e62. 2004
- [Treatment of sensorineural hearing loss in acute viral otitis media with intratympanic dexamethasone and hyaluronic acid in comparison with intravenous therapy]M Stenner
Klinik und Poliklinik Fur Hals, Nasen und Ohrenheilkunde der Johannes Gutenberg Universität Mainz
Laryngorhinootologie 85:32-7. 2006..It is not known yet if the same applies to the toxic inner ear damage in acute viral otitis media...
- Non-syndromal autosomal dominant hearing impairment: ongoing phenotypical characterization of genotypesS J Bom
Department of Otorhinolaryngology, University Hospital Nijmegen, Netherlands
Br J Audiol 33:335-48. 1999..It is recommended that sufficient attention is given to a detailed description of the phenotype in each (newly) described hereditary hearing impairment disorder...
- Kv7-type channel currents in spiral ganglion neurons: involvement in sensorineural hearing lossPing Lv
Department of Anesthesiology and Pain Medicine, School of Medicine, University of California, Davis, California 95618, USA
J Biol Chem 285:34699-707. 2010..Thus, this study provides evidence of the cellular etiology and mechanisms of SGN degeneration in DFNA2...
- Ototoxic drugs and sensorineural hearing loss following severe neonatal respiratory failureCharlene M T Robertson
Neonatal and Infant Follow up Clinic, Glenrose Rehabilitation Hospital, Capital Health, Edmonton, Alberta T5G 0B7, Canada
Acta Paediatr 95:214-23. 2006To determine relationships between ototoxic drugs and 4-y sensorineural hearing loss (SNHL) in near-term and term survivors of severe neonatal respiratory failure.
- Lack of association between inherited thrombophilic risk factors and idiopathic sudden sensorineural hearing loss in Italian patientsGabriella Cadoni
Department of Otorhinolaryngology, Catholic University of the Sacred Heart, Rome, Italy
Ann Otol Rhinol Laryngol 115:195-200. 2006We investigated the presence of congenital thrombophilic risk factors in a population of consecutive Italian patients affected by idiopathic sudden sensorineural hearing loss (SSNHL).
- A truncating mutation in SERPINB6 is associated with autosomal-recessive nonsyndromic sensorineural hearing lossAsli Sirmaci
Dr John T Macdonald Department of Human Genetics, University of Miami, Miller School of Medicine, Miami, FL 33136, USA
Am J Hum Genet 86:797-804. 2010..In this study, we mapped a previously unknown autosomal-recessive nonsyndromic sensorineural hearing loss locus (DFNB91) to chromosome 6p25 in a consanguineous Turkish family...
- A recovery from enhancement of activation in auditory cortex of patients with idiopathic sudden sensorineural hearing lossT Morita
Department of Otolaryngology, Shizuoka General Hospital, 420 8527 Shizuoka, Japan
Neurosci Res 58:6-11. 2007..whether this enhancement can exhibit a short-term alteration, we measured auditory evoked magnetic fields (AEFs) in patients with idiopathic sudden sensorineural hearing loss (ISSHL) in the acute phase (AP) and recovery phases (RPs).
- A case of relapsing polychondritis with bilateral sensorineural hearing loss and perforation of the nasal septum at the onsetTokutaro Tsuda
Institute of Rheumatology, Aoyama Hospital, Tokyo Women s Medical University, 2 7 13 Kita Aoyama, Minato ku, Tokyo 107 0061, Japan
Mod Rheumatol 17:148-52. 2007..Audiometry revealed bilateral sensorineural hearing loss. A second biopsy of the nasal septum showed an inflammatory change in the cartilage...
- Preserved otoacoustic emission in a child with a profound unilateral sensorineural hearing lossR Ohwatari
Department of Otolaryngology, Hokkaido University School of Medicine, Sapporo, Japan
Auris Nasus Larynx 28:S117-20. 2001We report a case of profound unilateral sensorineural hearing loss with good response of otoacoustic emission. The patient was a 5-year-old boy...
- [Propionic acidemia and sensorineural hearing loss: is there a connection at the molecular genetics level?]S Brosch
Sektion für Phoniatrie und Pädaudiologie der Universitäts Hals, Nasen, Ohrenklinik, Schillerstr 15, 89077, Ulm, Deutschland
HNO 56:37-42. 2008..Many mutations have been found at the molecular genetic level over the past 20 years, and their implications for the limitation of enzyme activity of PCC in propionic acidemia are discussed...
- [Progressive sensorineural hearing loss: metabolic, hormonal and vascular etiology]F Ravecca
Dipartimento di Neuroscienze,
Acta Otorhinolaryngol Ital 18:42-50. 1998..Metabolic, hormonal and vascular disorders are considered to cause hearing dysfunction such as progressive sensorineural hearing loss (PSNHL)...
- Ear-canal acoustic admittance and reflectance measurements in human neonates. II. Predictions of middle-ear in dysfunction and sensorineural hearing lossDouglas H Keefe
Boys Town National Research Hospital, 555 North 30th Street, Omaha, Nebaska 68131, USA
J Acoust Soc Am 113:407-22. 2003..84 for DPOAE/ABR, and A = 0.81 for TEOAE/ABR tests. The middle-ear dysfunction test adequately generalized to a new sample population (A = 0.82)...
- Distal renal tubular acidosis associated with large vestibular aqueduct and sensorineural hearing lossYukiko Shinjo
Department of Otolaryngology, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
Acta Otolaryngol 125:667-70. 2005..We suggest that all children with distal renal tubular acidosis (dRTA) should be subjected to an equilibrium study and audiological evaluation, as well as to a CT or MRI scan...
- The occurrence of sensorineural hearing loss in a patient with myeloperoxidase-anti-neutrophil cytoplasmic antibody-related microscopic polyangiitisToshiro Sugimoto
Rheumatol Int 27:503-5. 2007
- [Sequencing of SLC26A4 exons 7 and 8 and hot spot mutation analysis in 1552 moderate to profound sensorineural hearing loss patients in China]Pu Dai
Department of Otolaryngology Head and Neck Surgery, PLA General Hospital, Beijing 100853, China
Zhonghua Yi Xue Za Zhi 87:2521-5. 2007To investigate the hot spot mutation of SLC26A4 gene and its incidence among patients with moderate to profound sensorineural hearing loss (SNHL) and to analyze the epidemiology of enlarged vestibular aqueduct syndrome in China.
- Chikungunya virus induced sudden sensorineural hearing lossKranti Bhavana
Neuro Otology Unit, Department of Neurosurgery, SGPGIMS, Rai Bareilly Road, Lucknow 226014, India
Int J Pediatr Otorhinolaryngol 72:257-9. 2008The aim of this study is to demonstrate the association of Chikungunya virus and sudden sensorineural hearing loss. In the case report described we had a case which developed sudden unilateral sensorineural hearing loss following ..
- Challenges in management of childhood sensorineural hearing loss in sub-Saharan Africa, NigeriaO A Lasisi
Department of Otorhinolaryngology, College of Medicine, P O Box 22040, University of Ibadan University College Hospital, Ibadan, Nigeria
Int J Pediatr Otorhinolaryngol 70:625-9. 2006..presenting at the otolaryngology outpatient in the University College Hospital, Ibadan, Nigeria, sensorineural hearing loss was found in 103, giving a hospital prevalence rate of 14%, we still perceived this figure to be ..
- Sound lateralization and speech discrimination in patients with sensorineural hearing lossT Kubo
Department of Otolaryngology, Osaka City University Medical School, Japan
Acta Otolaryngol Suppl 538:63-9. 1998..we investigated the relationship between sound lateralization and speech discrimination in patients with sensorineural hearing loss (SNHL) of unknown aetiology or presbycusis...
- Assessment of saccular function in children with sensorineural hearing lossGuangwei Zhou
Department of Otolaryngology and Communication Enhancement, Children s Hospital Boston, Boston, Massachusetts 02115, USA
Arch Otolaryngol Head Neck Surg 135:40-4. 2009To investigate saccular function using vestibular evoked myogenic potentials in children with congenital or early acquired sensorineural hearing loss.
- Etiology of syndromic and nonsyndromic sensorineural hearing lossNicolas Gürtler
Laboratory of Molecular Otology, Epstein Laboratories, Department of Otolaryngology Head and Neck Surgery, University of California San Francisco, 533 Parnassus Avenue Room U490A, San Francisco, CA 94143 0526, USA
Otolaryngol Clin North Am 35:891-908. 2002..Ultimately, availability of gene therapy for the affected patients will bring to closure the circle of detection, identification, and correction of the disease...
- The role of factor V Leiden and prothrombin G20210A mutations in sudden sensorineural hearing lossKemal Gorur
Department of Otorhinolaryngology, University of Mersin School of Medicine, Mersin, Turkey
Otol Neurotol 26:599-601. 2005To investigate the incidence of factor V Leiden and prothrombin G20210A in sudden sensorineural hearing loss patients.
- NIH Director's Pioneer AwardJunying Yuan; Fiscal Year: 2009..No Abstract provided ..
- WIDEBAND CLINICAL DIAGNOSIS AND MONITORING OF MIDDLE-EAR AND COCHLEAR FUNCTIONDouglas H Keefe; Fiscal Year: 2013..an infant's threshold and may improve the accuracy of newborn hearing screen programs to identify sensorineural hearing loss (SNHL) and conductive hearing loss (CHL)...
- Evolution of the Immune Response to Cytomegalovirus Transmitted via Breast MilkLynn Puddington; Fiscal Year: 2010..cytomegalovirus (HCMV) infection in utero (congenital) can result in growth retardation, microcephaly, sensorineural hearing loss, and even death...
- Preclinical testing of a novel PDK1 inhibitor for treating vestibular schwannomaAbraham Jacob; Fiscal Year: 2013..VS are intracranial tumors that cause sensorineural hearing loss, balance abnormalities, tinnitus, vertigo, facial weakness, hydrocephalus, seizures, blindness, and even ..
- Basic and Clinical Studies of Noise-Induced and Age-Related Hearing LossSharon 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...
- Basic Mechanisms in Hearing Loss of Cochlear OriginAllen F Ryan; Fiscal Year: 2013DESCRIPTION (provided by applicant): Objectives: Sensorineural hearing loss (SNHL) is strongly associated with many aspects of military service including blast injury...
- Inner Ear Impact of Chronic Middle Ear InflammationDENNIS 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 ..
- Novel vaccine to CMV based on a disc virusAlistair McGregor; Fiscal Year: 2010..Indeed it is estimated that congenital HCMV related sensorineural hearing loss (SNHL) occurs at a greater frequency than SNHL related to Hemophilus influenza infection in the pre-HIB ..
- A cell-specific inducible model of hearing lossAlbert S Edge; Fiscal Year: 2011..Because of the clear correlation between loss of cochlear cells and sensorineural hearing loss, improvements in our ability to regenerate these cells will lead directly to new treatments for loss of ..
- Auditory Neuroprotection by Small Molecule Agonists of the TrkB ReceptorKeqiang 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...
- Preventing hair cell loss by regulating prestin's functionJing 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 ..
- The Role of Eya4 in Hearing and DiseaseJonathan 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...
- Cellular therapy for the inner earAlbert 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 ..
- Molecular mechanisms of auditory nAChR synapse assemblyMichele H Jacob; Fiscal Year: 2013Abstract: 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...
- Stimuli promoting the survival of spiral ganglion neuronsSteven H Green; Fiscal Year: 2011..b>Sensorineural hearing loss affects about 20,000,000 Americans and the only current means to replace the function of the lost ..
- The role of cochlear pericytes in control of cochlear microcirculationXiao 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...
- Trophic interactions in developing and adult inner earGabriel Corfas; Fiscal Year: 2013..These experiments will provide important insights into the pathogenesis and potential treatment of sensorineural hearing loss and peripheral balance disorders. Progressive dysfunction of the inner ear is an important health issue...
- Effectiveness of Therapy via Telemedicine following Cochlear ImplantsAnu Sharma; Fiscal Year: 2013DESCRIPTION (provided by applicant): In the U.S., approximately 200 children per 100,000 are born with sensorineural hearing loss. These children have varying degrees of deafness, and are likely to have impaired speech and language...
- Screening for Jervell and Lange-Nielsen SyndromeRuey Kang R Chang; Fiscal Year: 2010..and Lange-Nielsen syndrome (JLNS) is the most severe form of LQTS, with a hallmark feature of congenital sensorineural hearing loss (SNHL)...
- Drug-eluting Stapes Prosthesis for the Prevention of Sensorineural Hearing LossMichael J McKenna; Fiscal Year: 2013..If successful, these studies will lead quickly to clinical trials in humans with progressive sensorineural hearing loss from cochlear otosclerosis using a bisphosphonate-eluting stapes prosthesis, an intratympanic ..
- Role of CMV kinase in regulating infection of ESC-derived neuroprogenitor cellsTarin M Bigley; Fiscal Year: 2013..provided by applicant): Human cytomegalovirus (CMV) is the most common cause of non-hereditary sensorineural hearing loss in the U.S. Current therapies to treat infection during pregnancy or upon birth are unproven and risky...
- Auditory Nerve Degeneration and RepairHainan Lang; Fiscal Year: 2013DESCRIPTION (provided by applicant): Degeneration of spiral ganglion neurons (SGNs) results in permanent sensorineural hearing loss (SNHL) and is irreversible...
- MECHANISMS OF GATING AND PERMEATION IN GAP JUNCTIONSVYTAUTAS K VERSELIS; Fiscal Year: 2013..A subset of these mutations leads to syndromic forms of deafness in which sensorineural hearing loss is accompanied by severe, inflammatory skin disorders, such as keratitis-ichthiosis-deafness (KID) ..
- Effects of Sensorineural Hearing Loss on Robust Speech CodingMichael G Heinz; Fiscal Year: 2013..Recent psychophysical studies have suggested that listeners with sensorineural hearing loss have a reduced ability to use temporal fine-structure cues in speech perception...
- Autosomal dominant hearing lossKaren Friderici; Fiscal Year: 2009..Mutations in the gamma-actin gene cause progressive sensorineural hearing loss that resembles presbycusis except for an earlier age of onset...
- Auditory Processing of Stochastic FM in Relationship to Speech PerceptionSTANLEY E SHEFT; Fiscal Year: 2011..The long-term goal of this work is to develop a clinically feasible psychoacoustic measure of FM processing ability to assess, diagnosis, and aid in the remediation of difficulties with speech perception. ..
- Cholesterol Homeostasis in Auditory FunctionMICHELLE LYNN SEYMOUR; Fiscal Year: 2013..most common cause of hearing loss, involves age-related degeneration of the inner ear, and results in a sensorineural hearing loss beginning at the high frequencies and progressing to the middle and low frequencies...
- Activation of inner ear stem cells by B-cateninFuxin Shi; Fiscal Year: 2012..goal of this project is to find a noninvasive approach that could become a therapy for the treatment of sensorineural hearing loss for which there is currently no effective intervention other than hearing aids or cochlear implants.
- Understanding Noise-Induced Delayed Primary Degeneration of the Auditory NerveKonstantina M Stankovic; Fiscal Year: 2013..the candidate to achieve her long-term career goal of developing new diagnostics and therapeutics for sensorineural hearing loss - a problem of major clinical significance for which curative treatments do not yet exist...
- Delivery of Channel Rhodopsin for Optogenetic Control of Neurons in the CochleaDaniel J Lee; Fiscal Year: 2013..CI) provides meaningful sound and speech perception to the majority of patients with severe to profound sensorineural hearing loss. The CI electrode bypasses the defective or absent hair cells of the deaf inner ear to electrically ..
- Volumetric imaging of blood perfusion and tissue morphology in the cochleaRuikang Wang; Fiscal Year: 2013..are of paramount importance for the improved understanding, diagnosis, and treatment of sudden sensorineural hearing loss (SSHL) and Meniere's disease...
- FUNCTIONAL ORGANIZATION OF PRIMARY AUDITORY CORTEXChristoph E Schreiner; Fiscal Year: 2013..Once the normal functions are better understood, the detrimental consequences of hearing loss on these central mechanisms can be examined and may lead to the development of new therapeutic approaches to communicative disorders. ..
- Micromechanical Device for Intracochlear Drug DeliveryJeffrey Borenstein; Fiscal Year: 2009..and molecular biology have paved the way for new and innovative ways of treating and preventing sensorineural hearing loss. These advances will ultimately benefit millions of individuals...
- Congenital CMV Infection and Hearing Loss in Rural Indian PopulationSuresh B Boppana; Fiscal Year: 2010..These studies have also suggested that CMV-related sensorineural hearing loss (SNHL) occurs in populations with high CMV seroprevalence at a rate similar to that observed in the U.S...
- Genetic Studies of Inner Ear AnomaliesMustafa Tekin; Fiscal Year: 2013..Despite recent progress in identifying genes that determine many forms of syndromic and non-syndromic sensorineural hearing loss, the genetic pathogenesis of inner ear anomalies has remained largely unknown...
- Dynamic Effects in Peripheral Auditory ProcessingMagdalena Wojtczak; Fiscal Year: 2013..These measures will be undertaken in both normal-hearing listeners and listeners with sensorineural hearing loss. Aim 2 extends the investigation from within-channel to across-channel temporal processing to study the ..
- Micromechanical Device for Intracochlear Drug DeliveryJeffrey T Borenstein; Fiscal Year: 2013..and molecular biology have paved the way for new and innovative ways of treating and preventing sensorineural hearing loss. These advances will ultimately benefit millions of individuals...
- Complex Pitch Perception in Complex EnvironmentsAndrew J Oxenham; Fiscal Year: 2013....
- The relationship of hearing loss to maternal CMV immune status in infants with coGail J Demmler; Fiscal Year: 2011..by CT scan, hearing and vision;relate newborn findings with long term growth, neurodevelopment, and sensorineural hearing loss (severity, frequency, range, age at onset, unilateral or bilateral, fluctuations, and progressions);..
- CMV-Induced Embryonic Cochlear PathogenesisTINA contact JASKOLL; Fiscal Year: 2011..by applicant): Congenital cytomegalovirus (CMV) infection is the most common non-genetic cause of sensorineural hearing loss (SNHL) in children, accounting for between 20 and 60% of all SNHL...
- Genetic Control of Otic InductionBYRON HANEY HARTMAN; Fiscal Year: 2013DESCRIPTION (provided by applicant): Genetic Control of Otic Induction Sensorineural hearing loss due to auditory hair cell damage affects millions of Americans...
- Regulation of Inner Ear Stem CellsALAN GI LUN CHENG; Fiscal Year: 2013..a physician, I have noticed a major deficiency in our current treatment approach where we cannot reverse sensorineural hearing loss. As a scientist, I hope to establish a research program that will shed light on therapies that restore ..
- Genetic causes of hearing loss from the population to the molecular levelAiden Eliot Shearer; Fiscal Year: 2013DESCRIPTION (provided by applicant): Sensorineural hearing loss (SNHL) is the most prevalent congenital sensory deficit. In over 50% of cases, the cause is genetic...
- Genetic Regulation of Cochlear DevelopmentAndrew K Groves; Fiscal Year: 2013..cells of the cochlea in individuals where these cells have been destroyed - a common condition known as sensorineural hearing loss. The organ of Corti derives from a prosensory domain of cells running the length of the embryonic ..
- Cochlear Implants in Reinnervated EarsYehoash Raphael; Fiscal Year: 2013..These experiments will set the groundwork for clinical methods to induce nerve regeneration that could enhance the outcome of cochlear implant procedures in patients with severe or profound sensorineural hearing loss.
- The effects of aging and hearing loss on human auditory cortexDavid L Woods; Fiscal Year: 2013..of human ACFs and characterize changes in their anatomy and functional properties caused by aging and sensorineural hearing loss (SNHL)...
- Cellular regulation of cochlear blood flowA PHILINE WANGEMANN; Fiscal Year: 2012..to understand the mechanisms that regulate cochlear blood flow, since many hearing disorders, such as sensorineural hearing loss, acute acoustic trauma, noise-induced hearing loss, tinnitus, Meniere's disease and presbyacusis ..
- DEVELOPMENT EFFECT ON LOOP DIURETIC OTOTOXICITYLeonard Rybak; Fiscal Year: 1999..Recent reports have suggested an increased risk of permanent sensorineural hearing loss among infants receiving such treatments...
- The Impact of Frequency Compression on Cortical Evoked Potentials and PerceptionBenjamin J Kirby; Fiscal Year: 2013..Finally, in the third experiment, a heterogeneous group of participants with high frequency sensorineural hearing loss will be tested...
- A Collaborative search for new genes for non-syndromic deafnessMustafa Tekin; Fiscal Year: 2013..identified in one of 31 different genes in a subset of patients with non-syndromic autosomal recessive sensorineural hearing loss, at least 40% of families do not have an identifiable mutation nor do they demonstrate linkage to any ..
- GROWTH AND REGENERATION IN THE INNER EARJeffrey T Corwin; Fiscal Year: 2012..The goals of this research directly pertain to possible recovery from sensorineural hearing loss and balance dysfunctions that contribute to prevalent communication disorders and to falls by elderly ..
- TMC gene function in sensory hair cellsJeffrey R Holt; Fiscal Year: 2013..gene 1 (TMC1) underlie dominant, progressive hearing loss (DFNA36) and recessive nonsyndromic sensorineural hearing loss (DFNB7/B11) in humans (Kurima et al., 2002)...
- Development and Adaptive Behavior of Young Children with Hearing LossCarren Stika; Fiscal Year: 2012..these aims, we propose a longitudinal multicenter study of 210 children diagnosed with mild to severe sensorineural hearing loss, and a group of 100 typical hearing children for comparison purposes...
- Functional development of hair cellsGWENAELLE S GELEOC; Fiscal Year: 2013..expressed gene 1 (TMC1) underlies dominant progressive hearing loss (DFNA36) and recessive nonsyndromic sensorineural hearing loss deafness (DFNB7/B11) (Kurima et al., 2002)...
- Defining Antigenic Targets of Autoimmune Sensorineural Hearing LossThomas E Carey; Fiscal Year: 2013..In Aim 3 we will study inner ear development, hearing, and balance in transgenic mice with defective CTL2. ..
- A Decoy Receptor as a Novel Biomarker for Autoimmune Inner Ear DiseaseAndrea Vambutas; Fiscal Year: 2010..This leads to immune mediated inner ear inflammation resulting in progressive sensorineural hearing loss. We further hypothesize that this decoy receptor is a novel marker of steroid responsive hearing loss in ..
- Diagnosing outer hair cell health along the cochlear partitionMark E Chertoff; Fiscal Year: 2013b>Sensorineural hearing loss results from damage to a variety of structures within the cochlea and auditory nerve...
- Viral determinants of Cytomegalovirus related hearing lossShannon A Ross; Fiscal Year: 2011ongenital Cytomegalovirus (CMV) infection is a leading cause of sensorineural hearing loss (SNHL) in children. Among the approximately 40,000 infants born with congenital CMV each year, only ~10% develop SNHL...
- Assembly of type IV collagen in health and diseaseRaghu Kalluri; Fiscal Year: 2013..of these chains in Alport syndrome (a condition associated with progressive kidney disease, occasional sensorineural hearing loss and anterior lenticonus) leads to an absence or diminished expression of all three chains in the GBM, ..
- Precision Particle Fabrication-enabled Betamethasone-loaded Microspheres for TranMilind Singh; Fiscal Year: 2012DESCRIPTION (provided by applicant): Current approaches to treat sudden sensorineural hearing loss (SSNHL) do not maintain inner ear drug concentrations within an appropriate therapeutic window for sufficient lengths of time to achieve ..
- Optopathology by Light Microscopy and Molecular TechniquesMichael J McKenna; Fiscal Year: 2013..2. Investigate radiologic-histologic correlations in otosclerosis, and the pathogenetic mechanism of sensorineural hearing loss when otosclerosis involves the cochlea. 3...
- THE LOUDNESS OF BROADBAND SOUNDS IN LISTENERS WITH SENSORINEURAL HEARING LOSSWalt Jesteadt; Fiscal Year: 2013..impossible to provide an adequate specification of what restoration of normal loudness in listeners with sensorineural hearing loss (SNHL) would entail...
- Evaluation of Approaches to Auditory Rehabilitation for Mild TBIGabrielle Saunders; Fiscal Year: 2009..been shown to improve speech understanding and to decrease reported hearing abilities among adults with sensorineural hearing loss and functional hearing complaints...
- Can L-carnitine prevent drug-induced hearing loss?Gilda Kalinec; Fiscal Year: 2004..Among children, premature neonates have the highest prevalence rate of sensorineural hearing loss, which is strongly associated with perinatal exposure to ototoxic agents...
- IMPROVED STEROID THERAPY FOR HEARING LOSSDennis Trune; Fiscal Year: 2000..a variety of laboratories have shown that stria vascularis fluid accumulation occurs with autoimmune sensorineural hearing loss, drug ototoxicity, steroid deficiencies, etc...
- Coupling Strategies for Middle Ear ProthesesANN GRONDA; Fiscal Year: 2003..It is intended for people with moderate to severe sensorineural hearing loss of cochlear origin...
- Cochlear Therapy through Magnetic Targeted Drug DeliveryRichard Kopke; Fiscal Year: 2009b>Sensorineural hearing loss (SNHL), the most common type of deafness affecting more than 30 million Americans, is largely untreatable . When hair cells die, spiral ganglion neurons also may lose their peripheral axons and degenerate...
- Role of serotonin in auditory cortex regulation following early-onset sensorineurGREGORY BASURA; Fiscal Year: 2009..5-HT) and serotonergic neurotransmission in auditory (A1) cortex excitability following early onset sensorineural hearing loss (SNHL)...
- Effects of sensorineural loss on robust speech codingMichael Heinz; Fiscal Year: 2007..by listeners with normal hearing is robust across a wide variety of conditions, whereas people with sensorineural hearing loss (SNHL) typically show much less robust speech perception...
- CTL2 ELISA: A Diagnostic Test for Autoimmune Hearing LossVijay Kumar; Fiscal Year: 2007unreadable] 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 ..
- SENSORINEURAL HEARING LOSS--PERILYMPHATIC FISTULASRobert Kohut; Fiscal Year: 1999Sudden 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...
- Hair Cell Regeneration in the Mature Mammalian CochleaYehoash Raphael; Fiscal Year: 2009b>Sensorineural hearing loss is most commonly a result of hair cell degeneration. Hair cells in the organ of Corti cannot be replaced once lost, and therefore, sensorineural hearing loss is permanent...
- Otitis Media Impact on the Inner EarDennis Trune; Fiscal Year: 2007..In spite of decades of study, it is still not clear how otitis media impacts the inner ear to cause sensorineural hearing loss. Thus, it is impossible to predict who is at risk or what markers can be used to determine if inner ear ..
- Role of Spiral Ligament Fibrocytes in Inner Ear InflammationSung K Moon; Fiscal Year: 2010..It is believed that otitis media (OM)-associated inner ear inflammation results in high frequency sensorineural hearing loss (SNHL) in as high as 3% of OM patients...
- MODIOLAR-HUGGING ELECTRODE SYSTEM FOR COCHLEAR IMPLANTSDorcas Kessler; Fiscal Year: 1999Cochlear implants are designed to restore auditory perception to patients with severe to profound sensorineural hearing loss. CLARION, the only cochlear implant system manufactured in the United States, has eight independent output ..
- ANTIBODY INDUCED HEARING LOSS--MODEL FOR IMMUNE DEAFNESSThomas Carey; Fiscal Year: 1999b>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...