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OtoRhinoLaryngology by Sfakianakis G.Alexandros Sfakianakis G.Alexandros,Anapafseos 5 Agios Nikolaos 72100 Crete Greece,tel : 00302841026182,00306932607174
Related Articles |
Local gene therapy durably restores vestibular function in a mouse model of Usher syndrome type 1G.
Proc Natl Acad Sci U S A. 2017 Aug 23;:
Authors: Emptoz A, Michel V, Lelli A, Akil O, Boutet de Monvel J, Lahlou G, Meyer A, Dupont T, Nouaille S, Ey E, Franca de Barros F, Beraneck M, Dulon D, Hardelin JP, Lustig L, Avan P, Petit C, Safieddine S
Abstract
Our understanding of the mechanisms underlying inherited forms of inner ear deficits has considerably improved during the past 20 y, but we are still far from curative treatments. We investigated gene replacement as a strategy for restoring inner ear functions in a mouse model of Usher syndrome type 1G, characterized by congenital profound deafness and balance disorders. These mice lack the scaffold protein sans, which is involved both in the morphogenesis of the stereociliary bundle, the sensory antenna of inner ear hair cells, and in the mechanoelectrical transduction process. We show that a single delivery of the sans cDNA by the adenoassociated virus 8 to the inner ear of newborn mutant mice reestablishes the expression and targeting of the protein to the tips of stereocilia. The therapeutic gene restores the architecture and mechanosensitivity of stereociliary bundles, improves hearing thresholds, and durably rescues these mice from the balance defects. Our results open up new perspectives for efficient gene therapy of cochlear and vestibular disorders by showing that even severe dysmorphogenesis of stereociliary bundles can be corrected.
PMID: 28835534 [PubMed - as supplied by publisher]
Related Articles |
Relational quality, illness interference, and partner support in Ménière's disease.
Int J Audiol. 2017 Aug 24;:1-7
Authors: Pyykkö I, Manchaiah V, Zou J, Levo H, Kentala E
Abstract
OBJECTIVE: The current study aimed to better understand how patients and their significant others (SOs) cope with Menière's disease (MD).
DESIGN: The study used a cross-sectional design and the data were collected using questionnaires.
STUDY SAMPLE: Seventy-five dyads in which one person had MD.
RESULTS: SOs of patients with MD not only experienced activity and participation restrictions but also had positive experiences. In relational quality, the SOs reported uncertainty of their future, limited visits in noisy places, limited activities as walking, watching TV, and participating in social life. The illness interference correlated with the patients' complaints, and most significant was the problem of imbalance. The quality of life was significantly reduced in patients with MD, and the illness interference in terms of quality of life was correlated with the SOs in items related to mood and anxiety. The stress related conditions of the SOs were correlated with two positive items (e.g. alleviating the stress factor). The SOs could also identify one positive item (i.e., improved relationship).
CONCLUSIONS: Perceptions of MD as interfering in couples' lives influence dyadic coping in unique ways. The current study identified that dyadic coping has both positive aspects and limitations as a consequence of their partner's MD.
PMID: 28835146 [PubMed - as supplied by publisher]
Related Articles |
Local gene therapy durably restores vestibular function in a mouse model of Usher syndrome type 1G.
Proc Natl Acad Sci U S A. 2017 Aug 23;:
Authors: Emptoz A, Michel V, Lelli A, Akil O, Boutet de Monvel J, Lahlou G, Meyer A, Dupont T, Nouaille S, Ey E, Franca de Barros F, Beraneck M, Dulon D, Hardelin JP, Lustig L, Avan P, Petit C, Safieddine S
Abstract
Our understanding of the mechanisms underlying inherited forms of inner ear deficits has considerably improved during the past 20 y, but we are still far from curative treatments. We investigated gene replacement as a strategy for restoring inner ear functions in a mouse model of Usher syndrome type 1G, characterized by congenital profound deafness and balance disorders. These mice lack the scaffold protein sans, which is involved both in the morphogenesis of the stereociliary bundle, the sensory antenna of inner ear hair cells, and in the mechanoelectrical transduction process. We show that a single delivery of the sans cDNA by the adenoassociated virus 8 to the inner ear of newborn mutant mice reestablishes the expression and targeting of the protein to the tips of stereocilia. The therapeutic gene restores the architecture and mechanosensitivity of stereociliary bundles, improves hearing thresholds, and durably rescues these mice from the balance defects. Our results open up new perspectives for efficient gene therapy of cochlear and vestibular disorders by showing that even severe dysmorphogenesis of stereociliary bundles can be corrected.
PMID: 28835534 [PubMed - as supplied by publisher]
Related Articles |
Relational quality, illness interference, and partner support in Ménière's disease.
Int J Audiol. 2017 Aug 24;:1-7
Authors: Pyykkö I, Manchaiah V, Zou J, Levo H, Kentala E
Abstract
OBJECTIVE: The current study aimed to better understand how patients and their significant others (SOs) cope with Menière's disease (MD).
DESIGN: The study used a cross-sectional design and the data were collected using questionnaires.
STUDY SAMPLE: Seventy-five dyads in which one person had MD.
RESULTS: SOs of patients with MD not only experienced activity and participation restrictions but also had positive experiences. In relational quality, the SOs reported uncertainty of their future, limited visits in noisy places, limited activities as walking, watching TV, and participating in social life. The illness interference correlated with the patients' complaints, and most significant was the problem of imbalance. The quality of life was significantly reduced in patients with MD, and the illness interference in terms of quality of life was correlated with the SOs in items related to mood and anxiety. The stress related conditions of the SOs were correlated with two positive items (e.g. alleviating the stress factor). The SOs could also identify one positive item (i.e., improved relationship).
CONCLUSIONS: Perceptions of MD as interfering in couples' lives influence dyadic coping in unique ways. The current study identified that dyadic coping has both positive aspects and limitations as a consequence of their partner's MD.
PMID: 28835146 [PubMed - as supplied by publisher]
Related Articles |
Book review.
Int J Audiol. 2017 Aug 24;:1
Authors: Thunder T
PMID: 28835149 [PubMed - as supplied by publisher]
Related Articles |
Relational quality, illness interference, and partner support in Ménière's disease.
Int J Audiol. 2017 Aug 24;:1-7
Authors: Pyykkö I, Manchaiah V, Zou J, Levo H, Kentala E
Abstract
OBJECTIVE: The current study aimed to better understand how patients and their significant others (SOs) cope with Menière's disease (MD).
DESIGN: The study used a cross-sectional design and the data were collected using questionnaires.
STUDY SAMPLE: Seventy-five dyads in which one person had MD.
RESULTS: SOs of patients with MD not only experienced activity and participation restrictions but also had positive experiences. In relational quality, the SOs reported uncertainty of their future, limited visits in noisy places, limited activities as walking, watching TV, and participating in social life. The illness interference correlated with the patients' complaints, and most significant was the problem of imbalance. The quality of life was significantly reduced in patients with MD, and the illness interference in terms of quality of life was correlated with the SOs in items related to mood and anxiety. The stress related conditions of the SOs were correlated with two positive items (e.g. alleviating the stress factor). The SOs could also identify one positive item (i.e., improved relationship).
CONCLUSIONS: Perceptions of MD as interfering in couples' lives influence dyadic coping in unique ways. The current study identified that dyadic coping has both positive aspects and limitations as a consequence of their partner's MD.
PMID: 28835146 [PubMed - as supplied by publisher]
Related Articles |
Normative data for diagnosing auditory processing disorder in Norwegian children aged 7-12 years.
Int J Audiol. 2017 Aug 24;:1-11
Authors: Mattsson TS, Follestad T, Andersson S, Lind O, Øygarden J, Nordgård S
Abstract
OBJECTIVE: The main purpose of this research was to obtain normative data for auditory processing tests for Norwegian speaking children.
DESIGN: Participants were administered routine audiological tests and an auditory processing test-battery consisting of Filtered Words, Competing Words, Dichotic Digits, Gaps In Noise, Duration- and Frequency Pattern, Binaural Masking Level Difference and HIST Speech in Noise test. A group of 10-year-old children were retested after two weeks. The effects ear, age and gender and the test-retest reliability were investigated.
STUDY SAMPLE: There were 268 normal hearing children aged 7-12 years who participated in the study.
RESULTS: Results revealed no differences between genders. The children showed improving performance by age on all tests, except from the Gaps In Noise and Binaural Masking Level Difference. As expected, the children showed a right ear advantage on dichotic speech tests that decreased with age. The test-retest reliability for the tests was good, with a small learning effect on the Filtered Words test.
CONCLUSION: Normative data were established and the preferred tests for diagnosing Auditory Processing Disorder were suggested for Norwegian children aged 7-12 years.
PMID: 28835140 [PubMed - as supplied by publisher]
Related Articles |
Book review.
Int J Audiol. 2017 Aug 24;:1
Authors: Thunder T
PMID: 28835149 [PubMed - as supplied by publisher]
Related Articles |
Relational quality, illness interference, and partner support in Ménière's disease.
Int J Audiol. 2017 Aug 24;:1-7
Authors: Pyykkö I, Manchaiah V, Zou J, Levo H, Kentala E
Abstract
OBJECTIVE: The current study aimed to better understand how patients and their significant others (SOs) cope with Menière's disease (MD).
DESIGN: The study used a cross-sectional design and the data were collected using questionnaires.
STUDY SAMPLE: Seventy-five dyads in which one person had MD.
RESULTS: SOs of patients with MD not only experienced activity and participation restrictions but also had positive experiences. In relational quality, the SOs reported uncertainty of their future, limited visits in noisy places, limited activities as walking, watching TV, and participating in social life. The illness interference correlated with the patients' complaints, and most significant was the problem of imbalance. The quality of life was significantly reduced in patients with MD, and the illness interference in terms of quality of life was correlated with the SOs in items related to mood and anxiety. The stress related conditions of the SOs were correlated with two positive items (e.g. alleviating the stress factor). The SOs could also identify one positive item (i.e., improved relationship).
CONCLUSIONS: Perceptions of MD as interfering in couples' lives influence dyadic coping in unique ways. The current study identified that dyadic coping has both positive aspects and limitations as a consequence of their partner's MD.
PMID: 28835146 [PubMed - as supplied by publisher]
Related Articles |
Normative data for diagnosing auditory processing disorder in Norwegian children aged 7-12 years.
Int J Audiol. 2017 Aug 24;:1-11
Authors: Mattsson TS, Follestad T, Andersson S, Lind O, Øygarden J, Nordgård S
Abstract
OBJECTIVE: The main purpose of this research was to obtain normative data for auditory processing tests for Norwegian speaking children.
DESIGN: Participants were administered routine audiological tests and an auditory processing test-battery consisting of Filtered Words, Competing Words, Dichotic Digits, Gaps In Noise, Duration- and Frequency Pattern, Binaural Masking Level Difference and HIST Speech in Noise test. A group of 10-year-old children were retested after two weeks. The effects ear, age and gender and the test-retest reliability were investigated.
STUDY SAMPLE: There were 268 normal hearing children aged 7-12 years who participated in the study.
RESULTS: Results revealed no differences between genders. The children showed improving performance by age on all tests, except from the Gaps In Noise and Binaural Masking Level Difference. As expected, the children showed a right ear advantage on dichotic speech tests that decreased with age. The test-retest reliability for the tests was good, with a small learning effect on the Filtered Words test.
CONCLUSION: Normative data were established and the preferred tests for diagnosing Auditory Processing Disorder were suggested for Norwegian children aged 7-12 years.
PMID: 28835140 [PubMed - as supplied by publisher]
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Related Articles |
Hearing-related quality of life outcomes for Singaporean children using hearing aids or cochlear implants.
Eur Ann Otorhinolaryngol Head Neck Dis. 2016 Jun;133 Suppl 1:S25-30
Authors: Looi V, Lee ZZ, Loo JH
Abstract
OBJECTIVES: The Children Using Hearing Devices Quality of Life Questionnaire (CuHDQOL) is a new parent-administered hearing-specific questionnaire for children fitted with hearing devices. The aim of this study was to assess outcomes for hearing-impaired children in Singapore using this measure, as well as to examine its applicability for use in a clinical setting.
MATERIALS AND METHODS: The CuHDQOL has 26 items, uses a recall period of 1 month, and is divided into three sections: parental perspectives and expectations (eight items), impact on the family (eight items) and hearing-related quality of life (QOL) of the child (10 items). Responses are made on a 5-point Likert scale, and transformed to a score from 0-100. Twenty-two parents of children with hearing aids and 14 parents of children with cochlear implants completed the CuHDQOL.
RESULTS: The mean total CuHDQOL scores was 62/100 for the children using hearing aids and 53/100 for children with cochlear implants. Scores for the children using hearing aids were higher across all subscales, with a linear regression showing this to be significant for the parental perspectives and expectations subscale (B=-10.58, P=0.041). Analyses of Variance showed that both the 'Parent Perspective and Expectations' and the 'Hearing-related QOL' subscales were significantly higher than the 'Impact on Family' subscale for both groups (P≤0.003).
CONCLUSIONS: The CuHDQOL was found to be a simple, efficient questionnaire that could easily be incorporated into clinical practice to provide a more holistic evaluation of a child's outcomes post intervention, and/or to monitor their progress over time.
PMID: 27267231 [PubMed - indexed for MEDLINE]
Related Articles |
Book review.
Int J Audiol. 2017 Aug 24;:1
Authors: Thunder T
PMID: 28835149 [PubMed - as supplied by publisher]
Related Articles |
Relational quality, illness interference, and partner support in Ménière's disease.
Int J Audiol. 2017 Aug 24;:1-7
Authors: Pyykkö I, Manchaiah V, Zou J, Levo H, Kentala E
Abstract
OBJECTIVE: The current study aimed to better understand how patients and their significant others (SOs) cope with Menière's disease (MD).
DESIGN: The study used a cross-sectional design and the data were collected using questionnaires.
STUDY SAMPLE: Seventy-five dyads in which one person had MD.
RESULTS: SOs of patients with MD not only experienced activity and participation restrictions but also had positive experiences. In relational quality, the SOs reported uncertainty of their future, limited visits in noisy places, limited activities as walking, watching TV, and participating in social life. The illness interference correlated with the patients' complaints, and most significant was the problem of imbalance. The quality of life was significantly reduced in patients with MD, and the illness interference in terms of quality of life was correlated with the SOs in items related to mood and anxiety. The stress related conditions of the SOs were correlated with two positive items (e.g. alleviating the stress factor). The SOs could also identify one positive item (i.e., improved relationship).
CONCLUSIONS: Perceptions of MD as interfering in couples' lives influence dyadic coping in unique ways. The current study identified that dyadic coping has both positive aspects and limitations as a consequence of their partner's MD.
PMID: 28835146 [PubMed - as supplied by publisher]
Related Articles |
Normative data for diagnosing auditory processing disorder in Norwegian children aged 7-12 years.
Int J Audiol. 2017 Aug 24;:1-11
Authors: Mattsson TS, Follestad T, Andersson S, Lind O, Øygarden J, Nordgård S
Abstract
OBJECTIVE: The main purpose of this research was to obtain normative data for auditory processing tests for Norwegian speaking children.
DESIGN: Participants were administered routine audiological tests and an auditory processing test-battery consisting of Filtered Words, Competing Words, Dichotic Digits, Gaps In Noise, Duration- and Frequency Pattern, Binaural Masking Level Difference and HIST Speech in Noise test. A group of 10-year-old children were retested after two weeks. The effects ear, age and gender and the test-retest reliability were investigated.
STUDY SAMPLE: There were 268 normal hearing children aged 7-12 years who participated in the study.
RESULTS: Results revealed no differences between genders. The children showed improving performance by age on all tests, except from the Gaps In Noise and Binaural Masking Level Difference. As expected, the children showed a right ear advantage on dichotic speech tests that decreased with age. The test-retest reliability for the tests was good, with a small learning effect on the Filtered Words test.
CONCLUSION: Normative data were established and the preferred tests for diagnosing Auditory Processing Disorder were suggested for Norwegian children aged 7-12 years.
PMID: 28835140 [PubMed - as supplied by publisher]
Related Articles |
Book review.
Int J Audiol. 2017 Aug 24;:1
Authors: Thunder T
PMID: 28835149 [PubMed - as supplied by publisher]
Related Articles |
Relational quality, illness interference, and partner support in Ménière's disease.
Int J Audiol. 2017 Aug 24;:1-7
Authors: Pyykkö I, Manchaiah V, Zou J, Levo H, Kentala E
Abstract
OBJECTIVE: The current study aimed to better understand how patients and their significant others (SOs) cope with Menière's disease (MD).
DESIGN: The study used a cross-sectional design and the data were collected using questionnaires.
STUDY SAMPLE: Seventy-five dyads in which one person had MD.
RESULTS: SOs of patients with MD not only experienced activity and participation restrictions but also had positive experiences. In relational quality, the SOs reported uncertainty of their future, limited visits in noisy places, limited activities as walking, watching TV, and participating in social life. The illness interference correlated with the patients' complaints, and most significant was the problem of imbalance. The quality of life was significantly reduced in patients with MD, and the illness interference in terms of quality of life was correlated with the SOs in items related to mood and anxiety. The stress related conditions of the SOs were correlated with two positive items (e.g. alleviating the stress factor). The SOs could also identify one positive item (i.e., improved relationship).
CONCLUSIONS: Perceptions of MD as interfering in couples' lives influence dyadic coping in unique ways. The current study identified that dyadic coping has both positive aspects and limitations as a consequence of their partner's MD.
PMID: 28835146 [PubMed - as supplied by publisher]
Related Articles |
Normative data for diagnosing auditory processing disorder in Norwegian children aged 7-12 years.
Int J Audiol. 2017 Aug 24;:1-11
Authors: Mattsson TS, Follestad T, Andersson S, Lind O, Øygarden J, Nordgård S
Abstract
OBJECTIVE: The main purpose of this research was to obtain normative data for auditory processing tests for Norwegian speaking children.
DESIGN: Participants were administered routine audiological tests and an auditory processing test-battery consisting of Filtered Words, Competing Words, Dichotic Digits, Gaps In Noise, Duration- and Frequency Pattern, Binaural Masking Level Difference and HIST Speech in Noise test. A group of 10-year-old children were retested after two weeks. The effects ear, age and gender and the test-retest reliability were investigated.
STUDY SAMPLE: There were 268 normal hearing children aged 7-12 years who participated in the study.
RESULTS: Results revealed no differences between genders. The children showed improving performance by age on all tests, except from the Gaps In Noise and Binaural Masking Level Difference. As expected, the children showed a right ear advantage on dichotic speech tests that decreased with age. The test-retest reliability for the tests was good, with a small learning effect on the Filtered Words test.
CONCLUSION: Normative data were established and the preferred tests for diagnosing Auditory Processing Disorder were suggested for Norwegian children aged 7-12 years.
PMID: 28835140 [PubMed - as supplied by publisher]