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https://www.readbyqxmd.com/read/28222437/assessment-and-outcome-in-non-traditional-cochlear-implant-candidates
#1
Rebecca L Heywood, Deborah A Vickers, Francesca Pinto, George Fereos, Azhar Shaida
The objectives of this study were to assess: (i) patient expectations met as a measure of outcome in early-deafened, late-implanted (non-traditional) cochlear implant recipients and (ii) pre-implantation predictive factors for postoperative speech perception. The notes of 13 recipients were retrospectively reviewed. The mean age at onset of profound deafness was 1.5 years (range 0-6). The mean age at implantation was 37 years (range 22-51 years). Patient expectations were assessed pre-operatively and 1 year after implantation...
February 22, 2017: Audiology & Neuro-otology
https://www.readbyqxmd.com/read/28221712/discovery-of-myh14-as-an-important-and-unique-deafness-gene-causing-prelingually-severe-autosomal-dominant-non-syndromic-hearing-loss
#2
Bong Jik Kim, Ah Reum Kim, Jin Hee Han, Chung Lee, Doo Yi Oh, Byung Yoon Choi
BACKGROUND: Pathogenic variants of MYH14 have been known to be associated-in either a syndromic or non-syndromic manner-with hearing loss. Interestingly, all reported cases to date of MYH14-related non-syndromic hearing loss with detailed phenotypes have demonstrated mild-to-moderate progressive hearing loss with postlingual onset. METHODS: In this study, targeted resequencing (TRS) of known deafness genes was performed to identify the causative variant in two multiplex families segregating AD inherited hearing loss...
February 21, 2017: Journal of Gene Medicine
https://www.readbyqxmd.com/read/28220562/systemic-allergic-contact-dermatitis-caused-by-methyl-aminolaevulinate-in-a-patient-with-keratosis-ichthyosis-deafness-syndrome
#3
Aysha Al Malki, Marie-Claude Marguery, Françoise Giordano-Labadie, Maria P Konstantinou, Lorraine Mokeddem, Laurence Lamant, Carle Paul, Aude Maza, Juliette Mazereeuw-Hautier
No abstract text is available yet for this article.
March 2017: Contact Dermatitis
https://www.readbyqxmd.com/read/28220085/peripheral-visual-reaction-time-is-faster-in-deaf-adults-and-british-sign-language-interpreters-than-in-hearing-adults
#4
Charlotte J Codina, Olivier Pascalis, Heidi A Baseler, Alexandra T Levine, David Buckley
Following auditory deprivation, the remaining sense of vision has shown selective enhancement in visual cognition, especially in the area of near peripheral vision. Visual acuity is poor in the far periphery and may be an area where sound confers the greatest advantage in hearing persons. Experience with a visuospatial language such as British Sign Language (BSL) makes additional demands on the visual system. To test the different and separable effects of deafness and use of a visuo-spatial language on far peripheral visual processing, we investigated visual reaction times (RTs) and response accuracy to visual stimuli, between 30° and 85° along the four cardinal and four inter-cardinal meridians...
2017: Frontiers in Psychology
https://www.readbyqxmd.com/read/28220063/vibrotactile-discrimination-training-affects-brain-connectivity-in-profoundly-deaf-individuals
#5
Andrés A González-Garrido, Vanessa D Ruiz-Stovel, Fabiola R Gómez-Velázquez, Hugo Vélez-Pérez, Rebeca Romo-Vázquez, Ricardo A Salido-Ruiz, Aurora Espinoza-Valdez, Luis R Campos
Early auditory deprivation has serious neurodevelopmental and cognitive repercussions largely derived from impoverished and delayed language acquisition. These conditions may be associated with early changes in brain connectivity. Vibrotactile stimulation is a sensory substitution method that allows perception and discrimination of sound, and even speech. To clarify the efficacy of this approach, a vibrotactile oddball task with 700 and 900 Hz pure-tones as stimuli [counterbalanced as target (T: 20% of the total) and non-target (NT: 80%)] with simultaneous EEG recording was performed by 14 profoundly deaf and 14 normal-hearing (NH) subjects, before and after a short training period (five 1-h sessions; in 2...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28219845/gait-ground-reaction-force-characteristics-in-deaf-and-hearing-children
#6
Amir Ali Jafarnezhadgero, Mahdi Majlesi, Elaheh Azadian
The link between gait parameters and hearing loss is not well understood. The objective of this study was to investigate the effects of the gait ground reaction forces, their time to peak, vertical loading rate, impulses and free moment during gait in deaf and hearing children. Thirty male children were equally divided into a healthy group and a group with hearing loss problems (Deaf group). Ground reaction forces were analyzed during barefoot walking. MANOVA test was used for between group comparisons. The significance level was set at p<0...
February 14, 2017: Gait & Posture
https://www.readbyqxmd.com/read/28219180/-advances-in-effect-of-unilateral-auditory-deprivation-on-sound-localization
#7
J F Liu, J S Dai, N Y Wang
In patients with single-sided deafness (SSD), the interaural time difference and the interaural level difference signals are insufficient or missing, which result in the lack of sound localization ability and the decrease of speech comprehension in the noise environments. SSD can also cause the morphological and functional changes of the central auditory system, resulting in auditory deprivation. In early stage of the development, the auditory center is more susceptible to ambient environment and auditory inputs...
February 7, 2017: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/28219036/-i-use-it-when-i-see-it-the-role-of-development-and-experience-in-deaf-and-hearing-children-s-understanding-of-iconic-gesture
#8
Rachel W Magid, Jennie E Pyers
Iconicity is prevalent in gesture and in sign languages, yet the degree to which children recognize and leverage iconicity for early language learning is unclear. In Experiment 1 of the current study, we presented sign-naïve 3-, 4- and 5-year-olds (n=87) with iconic shape gestures and no additional scaffolding to ask whether children can spontaneously map iconic gestures to their referents. Four- and five-year-olds, but not three-year-olds, recognized the referents of iconic shape gestures above chance. Experiment 2 asked whether preschoolers (n=93) show an advantage in fast-mapping iconic gestures compared to arbitrary ones...
February 16, 2017: Cognition
https://www.readbyqxmd.com/read/28218016/auditory-performance-and-subjective-benefits-in-adults-with-congenital-or-prelinguistic-deafness-who-receive-cochlear-implants-during-adulthood
#9
Louise Duchesne, Isabelle Millette, Maurice Bhérer, Suzie Gobeil
OBJECTIVES: (1) To describe auditory performance and subjective benefits in adults with congenital or prelingual deafness who received a cochlear implant (CI) during adolescence or adulthood, and (2) to examine the benefits as experienced by these CI users. METHODS: Twenty-one adults aged 23-65 years participated in the study. All had a congenital or prelingual deafness (onset before age 3). They received a CI between the age of 16 and 61 years (mean age: 31). Speech recognition scores before and after implantation were computed and a questionnaire on subjective benefits (French adaptation of the Adult Cochlear Implant Questionnaire, designed by Zwolan and collaborators (1996, Self-report of CI use and satisfaction by prelingually deafened adults...
February 20, 2017: Cochlear Implants International
https://www.readbyqxmd.com/read/28215374/collaboration-with-deaf-communities-to-conduct-accessible-health-surveillance
#10
Steven L Barnett, Kelly A Matthews, Erika J Sutter, Lori A DeWindt, Jacqueline A Pransky, Amanda M O'Hearn, Tamala M David, Robert Q Pollard, Vincent J Samar, Thomas A Pearson
INTRODUCTION: Populations of deaf sign language users experience health disparities unmeasured by current public health surveillance. Population-specific health data are necessary to collaboratively identify health priorities and evaluate interventions. Standardized, reproducible, and language-concordant data collection in sign language is impossible via written or telephone surveys. METHODS: Deaf and hearing researchers, community members, and other stakeholders developed a broad computer-based health survey based on the telephone-administered Behavioral Risk Factor Surveillance System...
March 2017: American Journal of Preventive Medicine
https://www.readbyqxmd.com/read/28213671/exome-analysis-of-smith-magenis-like-syndrome-cohort-identifies-de-novo-likely-pathogenic-variants
#11
Seth I Berger, Carla Ciccone, Karen L Simon, May Christine Malicdan, Thierry Vilboux, Charles Billington, Roxanne Fischer, Wendy J Introne, Andrea Gropman, Jan K Blancato, James C Mullikin, William A Gahl, Marjan Huizing, Ann C M Smith
Smith-Magenis syndrome (SMS), a neurodevelopmental disorder characterized by dysmorphic features, intellectual disability (ID), and sleep disturbances, results from a 17p11.2 microdeletion or a mutation in the RAI1 gene. We performed exome sequencing on 6 patients with SMS-like phenotypes but without chromosomal abnormalities or RAI1 variants. We identified pathogenic de novo variants in two cases, a nonsense variant in IQSEC2 and a missense variant in the SAND domain of DEAF1, and candidate de novo missense variants in an additional two cases...
February 17, 2017: Human Genetics
https://www.readbyqxmd.com/read/28213135/genetic-variants-in-the-peripheral-auditory-system-significantly-affect-adult-cochlear-implant-performance
#12
A Eliot Shearer, Robert W Eppsteiner, Kathy Frees, Viral Tejani, Christina M Sloan-Heggen, Carolyn Brown, Paul Abbas, Camille Dunn, Marlan R Hansen, Bruce J Gantz, Richard J H Smith
BACKGROUND: Cochlear implantation is an effective habilitation modality for adults with significant hearing loss. However, post-implant performance is variable. A portion of this variance in outcome can be attributed to clinical factors. Recent physiological studies suggest that the health of the spiral ganglion also impacts post-operative cochlear implant outcomes. The goal of this study was to determine whether genetic factors affecting spiral ganglion neurons may be associated with cochlear implant performance...
February 14, 2017: Hearing Research
https://www.readbyqxmd.com/read/28209736/pejvakin-a-candidate-stereociliary-rootlet-protein-regulates-hair-cell-function-in-a-cell-autonomous-manner
#13
Marcin Kazmierczak, Piotr Kazmierczak, Anthony W Peng, Suzan L Harris, Prahar Shah, Jean-Luc Puel, Marc Lenoir, Santos J Franco, Martin Schwander
Mutations in the Pejvakin (PJVK) gene are thought to cause auditory neuropathy and hearing loss of cochlear origin by affecting noise-induced peroxisome proliferation in auditory hair cells and neurons. Here we demonstrate that loss of pejvakin in hair cells, but not in neurons, causes profound hearing loss and outer hair cell degeneration in mice. Pejvakin binds to and co-localizes with the rootlet component TRIOBP at the base of stereocilia in injectoporated hair cells, a pattern that is disrupted by deafness-associated PJVK mutations...
February 16, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28207578/the-impact-of-a-cochlear-implant-electrode-array-on-the-middle-ear-transfer-function
#14
David Pazen, Andreas Anagiotos, Maike Nünning, Antoniu-Oreste Gostian, Magdalene Ortmann, Dirk Beutner
OBJECTIVES: As a treatment for partial deafness with residual hearing in the lower frequency range, the combined acoustic and electric stimulation of the cochlea has become widespread. Acoustic stimulation is provided by a hearing aid's airborne sound and the electric stimulation by a cochlear implant electrode array, which may be inserted through the round window or a cochleostomy. To take advantage of that concept, it is essential to preserve residual hearing after surgery. Therefore, the intracochlear electrode array should not compromise the middle ear vibration transmission...
February 15, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28207576/relationship-between-peripheral-and-psychophysical-measures-of-amplitude-modulation-detection-in-cochlear-implant-users
#15
Viral D Tejani, Paul J Abbas, Carolyn J Brown
OBJECTIVE: This study investigates the relationship between electrophysiological and psychophysical measures of amplitude modulation (AM) detection. Prior studies have reported both measures of AM detection recorded separately from cochlear implant (CI) users and acutely deafened animals, but no study has made both measures in the same CI users. Animal studies suggest a progressive loss of high-frequency encoding as one ascends the auditory pathway from the auditory nerve to the cortex...
February 15, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28207575/health-related-quality-of-life-among-young-children-with-cochlear-implants-and-developmental-disabilities
#16
Anat Zaidman-Zait, Deirdre Curle, Janet R Jamieson, Ruth Chia, Frederick K Kozak
OBJECTIVE: The present study examined differences in health-related quality of life (HRQoL) between deaf children with cochlear implants (CI) with and without developmental disabilities (DD) and differences across HRQoL domains within both groups of children. METHODS: Ninety-two parents of children with CI aged 3-7 years participated in this cross-sectional study. Of these children, 43 had DD (i.e., CI-DD group) and 49 had no DD or chronic illness, demonstrating overall typical development (i...
February 15, 2017: Ear and Hearing
https://www.readbyqxmd.com/read/28204923/language-deprivation-syndrome-a-possible-neurodevelopmental-disorder-with-sociocultural-origins
#17
Wyatte C Hall, Leonard L Levin, Melissa L Anderson
PURPOSE: There is a need to better understand the epidemiological relationship between language development and psychiatric symptomatology. Language development can be particularly impacted by social factors-as seen in the developmental choices made for deaf children, which can create language deprivation. A possible mental health syndrome may be present in deaf patients with severe language deprivation. METHODS: Electronic databases were searched to identify publications focusing on language development and mental health in the deaf population...
February 16, 2017: Social Psychiatry and Psychiatric Epidemiology
https://www.readbyqxmd.com/read/28203210/auditory-and-visual-electrophysiology-of-deaf-children-with-cochlear-implants-implications-for-cross-modal-plasticity
#18
David P Corina, Shane Blau, Todd LaMarr, Laurel A Lawyer, Sharon Coffey-Corina
Deaf children who receive a cochlear implant early in life and engage in intensive oral/aural therapy often make great strides in spoken language acquisition. However, despite clinicians' best efforts, there is a great deal of variability in language outcomes. One concern is that cortical regions which normally support auditory processing may become reorganized for visual function, leaving fewer available resources for auditory language acquisition. The conditions under which these changes occur are not well understood, but we may begin investigating this phenomenon by looking for interactions between auditory and visual evoked cortical potentials in deaf children...
2017: Frontiers in Psychology
https://www.readbyqxmd.com/read/28198501/effect-of-gjb2-235delc-and-30-35delg-genetic-polymorphisms-on-risk-of-congenital-deafness-in-a-chinese-population
#19
Y Xiong, M Zhong, J Chen, Y L Yan, X F Lin, X Li
Congenital deafness is a serious and irreversible condition in humans. The GJB2 gene is implicated in the pathogenesis of autosomal recessive nonsyndromic hearing loss. Its 235delC and 30-35delG polymorphisms are reported to be associated with risk of hereditary deafness. However, the effect of the interaction between GJB2 235delC and 30-35delG and environmental factors on congenital deafness has not been described. Therefore, we performed a case-control study to investigate the influence of these polymorphisms on congenital deafness risk, and their interaction with maternal and other environmental factors in the development of this disease...
February 8, 2017: Genetics and Molecular Research: GMR
https://www.readbyqxmd.com/read/28196001/efficacy-of-bone-anchored-hearing-aids-in-single-sided-deafness-a-systematic-review
#20
Gaeun Kim, Hyun Mi Ju, Sun Hee Lee, Hee-Soon Kim, Jeong A Kwon, Young Joon Seo
OBJECTIVES: Bone-anchored hearing aids (BAHAs) have been known to partially restore some of the functions lost in subjects with single-sided deafness (SSD). Our aims in this systemic review were to analyze the present capabilities of BAHAs in the context of SSD, and to evaluate the efficacy of BAHAs in improving speech recognition in noisy conditions, sound localization, and subjective outcomes. DESIGN: A systematic search was undertaken until August 2015 by two independent reviewers, with disagreements resolved by consensus...
February 10, 2017: Otology & Neurotology
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