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Using Automatic Speech Recognition to Optimize Hearing-Aid Time Constants
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In: ISSN: 1662-4548 ; EISSN: 1662-453X ; Frontiers in Neuroscience ; https://hal.archives-ouvertes.fr/hal-03627441 ; Frontiers in Neuroscience, Frontiers, 2022, 16 (779062), ⟨10.3389/fnins.2022.779062⟩ ; https://www.frontiersin.org/articles/10.3389/fnins.2022.779062/full (2022)
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USE OF SIGN LANGUAGES BY HEALTHCARE PROFESSIONALS TO HELP DEAF PEOPLE IN HEALTH SERVICES: INTEGRATIVE REVIEW ...
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Detection and Recognition of Asynchronous Auditory/Visual Speech: Effects of Age, Hearing Loss, and Talker Accent ...
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An Audibility Model of the Bone Conduction Device during Headband Trial in Single-sided Deaf Subjects. ...
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An Audibility Model of the Bone Conduction Device during Headband Trial in Single-sided Deaf Subjects. ...
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An Audibility Model of the Bone Conduction Device during Headband Trial in Single-sided Deaf Subjects. ...
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Assessing Self-Efficacy in Families of Children with Hearing Concerns through an Audiological Early Intervention Training
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In: Appalachian Student Research Forum & Jay S. Boland Undergraduate Research Symposium (2022)
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Cochlear Implantation Improves Both Speech Perception and Patient-Reported Outcomes: A Prospective Follow-Up Study of Treatment Benefits among Adult Cochlear Implant Recipients
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In: Journal of Clinical Medicine; Volume 11; Issue 8; Pages: 2257 (2022)
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Functional Reorganization of the Central Auditory System in Children with Single-Sided Deafness: A Protocol Using fNIRS
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In: Brain Sciences; Volume 12; Issue 4; Pages: 423 (2022)
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Perceived Anger in Clear and Conversational Speech: Contributions of Age and Hearing Loss
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In: Brain Sciences; Volume 12; Issue 2; Pages: 210 (2022)
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Clinical and Genetic Characteristics of Finnish Patients with Autosomal Recessive and Dominant Non-Syndromic Hearing Loss Due to Pathogenic TMC1 Variants
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In: Journal of Clinical Medicine; Volume 11; Issue 7; Pages: 1837 (2022)
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Abstract:
Sensorineural hearing loss (SNHL) is one of the most common sensory deficits worldwide, and genetic factors contribute to at least 50–60% of the congenital hearing loss cases. The transmembrane channel-like protein 1 (TMC1) gene has been linked to autosomal recessive (DFNB7/11) and autosomal dominant (DFNA36) non-syndromic hearing loss, and it is a relatively common genetic cause of SNHL. Here, we report eight Finnish families with 11 affected family members with either recessively inherited homozygous or compound heterozygous TMC1 variants associated with congenital moderate-to-profound hearing loss, or a dominantly inherited heterozygous TMC1 variant associated with postlingual progressive hearing loss. We show that the TMC1 c.1534C>T, p.(Arg512*) variant is likely a founder variant that is enriched in the Finnish population. We describe a novel recessive disease-causing TMC1 c.968A>G, p.(Tyr323Cys) variant. We also show that individuals in this cohort who were diagnosed early and received timely hearing rehabilitation with hearing aids and cochlear implants (CI) have reached good speech perception in noise. Comparison of the genetic data with the outcome of CI rehabilitation increases our understanding of the extent to which underlying pathogenic gene variants explain the differences in CI rehabilitation outcomes.
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Keyword:
cochlear implant; congenital; hearing loss; hearing rehabilitation; TMC1
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URL: https://doi.org/10.3390/jcm11071837
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Candidacy for Cochlear Implantation in Prelingual Profoundly Deaf Adult Patients
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In: Journal of Clinical Medicine; Volume 11; Issue 7; Pages: 1874 (2022)
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Diversity of Child and Family Characteristics of Children with Hearing Loss in Family-Centered Early Intervention in The Netherlands
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In: Journal of Clinical Medicine; Volume 11; Issue 8; Pages: 2074 (2022)
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Neural Correlates of Visual Stimulus Encoding and Verbal Working Memory Differ between Cochlear Implant users and Normal-hearing Controls
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Detection and Recognition of Asynchronous Auditory/Visual Speech: Effects of Age, Hearing Loss, and Talker Accent
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Moderators and Predictors in a Parent Hearing Aid Management eHealth Program
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In: Psychology Student Research (2022)
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Hearing disorders and biotinidase deficiency: an integrative literature review
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In: Revista CEFAC, Vol 24, Iss 1 (2022) (2022)
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Contributions of Age-Related and Audibility-Related Deficits to Aided Consonant Identification in Presbycusis: A Causal-Inference Analysis
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In: ISSN: 1663-4365 ; Frontiers in Aging Neuroscience ; https://hal.archives-ouvertes.fr/hal-03154679 ; Frontiers in Aging Neuroscience, Frontiers, 2021, 13, pp.640522. ⟨10.3389/fnagi.2021.640522⟩ ; https://www.frontiersin.org/articles/10.3389/fnagi.2021.640522/full (2021)
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Contributions of Age-Related and Audibility-Related Deficits to Aided Consonant Identification in Presbycusis: A Causal-Inference Analysis
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In: ISSN: 1663-4365 ; Frontiers in Aging Neuroscience ; https://hal-pasteur.archives-ouvertes.fr/pasteur-03219614 ; Frontiers in Aging Neuroscience, Frontiers, 2021, 13, pp.640522. ⟨10.3389/fnagi.2021.640522⟩ (2021)
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Rapid Assessment of Non-Verbal Auditory Perception in Normal-Hearing Participants and Cochlear Implant Users
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In: ISSN: 2077-0383 ; Journal of Clinical Medicine ; https://hal.archives-ouvertes.fr/hal-03413817 ; Journal of Clinical Medicine, MDPI, 2021, 10 (10), pp.2093. ⟨10.3390/jcm10102093⟩ (2021)
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