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OtoRhinoLaryngology by Sfakianakis G.Alexandros Sfakianakis G.Alexandros,Anapafseos 5 Agios Nikolaos 72100 Crete Greece,tel : 00302841026182,00306932607174
To many Minnesotans, Prince Rogers Nelson was a larger-than-life character who transformed Minnesota’s music scene in the early 1980s with his provocative, charismatic, and engaging style. His magnum opus, “Purple Rain,” dominated the album charts for nearly one-half of 1984, and the movie opened at #1 when it opened in July 1984. He was definitely at the top of his game when he came to Washington, DC, in the fall that year, where he performed seven sold-out shows at Landover’s Capital Centre over a two-week time period.
The Voice Track multiband single-channel modified Wiener-filter noise reduction system for cochlear implants: patients' outcomes and subjective appraisal.
Int J Audiol. 2016 Apr 25;:1-8
Authors: Guevara N, Bozorg-Grayeli A, Bebear JP, Ardoint M, Saaï S, Gnansia D, Hoen M, Romanet P, Lavieille JP
Abstract
OBJECTIVE: The aim of this study was to evaluate the potential improvements of speech perception and sound quality provided by a multiband single channel noise-reduction algorithm based on the modified Wiener-filter adapted to cochlear implant sound processing.
DESIGN: This study was a longitudinal trial with a repeated-measures design. Outcome measures were performed on the first day when the noise reduction feature was provided and after a one month habituation period. Objective measures included pure-tone thresholds and vocal audiometry assessments. Speech perception was measured in quiet and in the presence of two types of noise: a stationary speech shaped noise and a two-talker cocktail noise. Subjective sound quality was assessed using a ten item questionnaire.
STUDY SAMPLE: Thirteen post-lingual deaf adults, experienced users of a cochlear implant system, took part in this study.
RESULTS: The noise-reduction algorithm provided a benefit for the perception of speech presented in a stationary speech shaped noise and an overall improvement in subjective sound quality ratings.
CONCLUSIONS: It was shown that a single channel noise reduction system based on a modified Wiener-filter approach can improve speech in noise perception performance and subjective sound quality in cochlear implant patients.
PMID: 27108635 [PubMed - as supplied by publisher]
The Voice Track multiband single-channel modified Wiener-filter noise reduction system for cochlear implants: patients' outcomes and subjective appraisal.
Int J Audiol. 2016 Apr 25;:1-8
Authors: Guevara N, Bozorg-Grayeli A, Bebear JP, Ardoint M, Saaï S, Gnansia D, Hoen M, Romanet P, Lavieille JP
Abstract
OBJECTIVE: The aim of this study was to evaluate the potential improvements of speech perception and sound quality provided by a multiband single channel noise-reduction algorithm based on the modified Wiener-filter adapted to cochlear implant sound processing.
DESIGN: This study was a longitudinal trial with a repeated-measures design. Outcome measures were performed on the first day when the noise reduction feature was provided and after a one month habituation period. Objective measures included pure-tone thresholds and vocal audiometry assessments. Speech perception was measured in quiet and in the presence of two types of noise: a stationary speech shaped noise and a two-talker cocktail noise. Subjective sound quality was assessed using a ten item questionnaire.
STUDY SAMPLE: Thirteen post-lingual deaf adults, experienced users of a cochlear implant system, took part in this study.
RESULTS: The noise-reduction algorithm provided a benefit for the perception of speech presented in a stationary speech shaped noise and an overall improvement in subjective sound quality ratings.
CONCLUSIONS: It was shown that a single channel noise reduction system based on a modified Wiener-filter approach can improve speech in noise perception performance and subjective sound quality in cochlear implant patients.
PMID: 27108635 [PubMed - as supplied by publisher]
Corrigendum.
Int J Audiol. 2016 Apr 25;:1
Authors:
PMID: 27108535 [PubMed - as supplied by publisher]
Corrigendum.
Int J Audiol. 2016 Apr 25;:1
Authors:
PMID: 27108535 [PubMed - as supplied by publisher]
The Voice Track multiband single-channel modified Wiener-filter noise reduction system for cochlear implants: patients' outcomes and subjective appraisal.
Int J Audiol. 2016 Apr 25;:1-8
Authors: Guevara N, Bozorg-Grayeli A, Bebear JP, Ardoint M, Saaï S, Gnansia D, Hoen M, Romanet P, Lavieille JP
Abstract
OBJECTIVE: The aim of this study was to evaluate the potential improvements of speech perception and sound quality provided by a multiband single channel noise-reduction algorithm based on the modified Wiener-filter adapted to cochlear implant sound processing.
DESIGN: This study was a longitudinal trial with a repeated-measures design. Outcome measures were performed on the first day when the noise reduction feature was provided and after a one month habituation period. Objective measures included pure-tone thresholds and vocal audiometry assessments. Speech perception was measured in quiet and in the presence of two types of noise: a stationary speech shaped noise and a two-talker cocktail noise. Subjective sound quality was assessed using a ten item questionnaire.
STUDY SAMPLE: Thirteen post-lingual deaf adults, experienced users of a cochlear implant system, took part in this study.
RESULTS: The noise-reduction algorithm provided a benefit for the perception of speech presented in a stationary speech shaped noise and an overall improvement in subjective sound quality ratings.
CONCLUSIONS: It was shown that a single channel noise reduction system based on a modified Wiener-filter approach can improve speech in noise perception performance and subjective sound quality in cochlear implant patients.
PMID: 27108635 [PubMed - as supplied by publisher]
Corrigendum.
Int J Audiol. 2016 Apr 25;:1
Authors:
PMID: 27108535 [PubMed - as supplied by publisher]
The Voice Track multiband single-channel modified Wiener-filter noise reduction system for cochlear implants: patients' outcomes and subjective appraisal.
Int J Audiol. 2016 Apr 25;:1-8
Authors: Guevara N, Bozorg-Grayeli A, Bebear JP, Ardoint M, Saaï S, Gnansia D, Hoen M, Romanet P, Lavieille JP
Abstract
OBJECTIVE: The aim of this study was to evaluate the potential improvements of speech perception and sound quality provided by a multiband single channel noise-reduction algorithm based on the modified Wiener-filter adapted to cochlear implant sound processing.
DESIGN: This study was a longitudinal trial with a repeated-measures design. Outcome measures were performed on the first day when the noise reduction feature was provided and after a one month habituation period. Objective measures included pure-tone thresholds and vocal audiometry assessments. Speech perception was measured in quiet and in the presence of two types of noise: a stationary speech shaped noise and a two-talker cocktail noise. Subjective sound quality was assessed using a ten item questionnaire.
STUDY SAMPLE: Thirteen post-lingual deaf adults, experienced users of a cochlear implant system, took part in this study.
RESULTS: The noise-reduction algorithm provided a benefit for the perception of speech presented in a stationary speech shaped noise and an overall improvement in subjective sound quality ratings.
CONCLUSIONS: It was shown that a single channel noise reduction system based on a modified Wiener-filter approach can improve speech in noise perception performance and subjective sound quality in cochlear implant patients.
PMID: 27108635 [PubMed - as supplied by publisher]
Corrigendum.
Int J Audiol. 2016 Apr 25;:1
Authors:
PMID: 27108535 [PubMed - as supplied by publisher]
A small molecule mitigates hearing loss in a mouse model of Usher syndrome III.
Nat Chem Biol. 2016 Apr 25;
Authors: Alagramam KN, Gopal SR, Geng R, Chen DH, Nemet I, Lee R, Tian G, Miyagi M, Malagu KF, Lock CJ, Esmieu WR, Owens AP, Lindsay NA, Ouwehand K, Albertus F, Fischer DF, Bürli RW, MacLeod AM, Harte WE, Palczewski K, Imanishi Y
Abstract
Usher syndrome type III (USH3), characterized by progressive deafness, variable balance disorder and blindness, is caused by destabilizing mutations in the gene encoding the clarin-1 (CLRN1) protein. Here we report a new strategy to mitigate hearing loss associated with a common USH3 mutation CLRN1(N48K) that involves cell-based high-throughput screening of small molecules capable of stabilizing CLRN1(N48K), followed by a secondary screening to eliminate general proteasome inhibitors, and finally an iterative process to optimize structure-activity relationships. This resulted in the identification of BioFocus 844 (BF844). To test the efficacy of BF844, we developed a mouse model that mimicked the progressive hearing loss associated with USH3. BF844 effectively attenuated progressive hearing loss and prevented deafness in this model. Because the CLRN1(N48K) mutation causes both hearing and vision loss, BF844 could in principle prevent both sensory deficiencies in patients with USH3. Moreover, the strategy described here could help identify drugs for other protein-destabilizing monogenic disorders.
PMID: 27110679 [PubMed - as supplied by publisher]
A small molecule mitigates hearing loss in a mouse model of Usher syndrome III.
Nat Chem Biol. 2016 Apr 25;
Authors: Alagramam KN, Gopal SR, Geng R, Chen DH, Nemet I, Lee R, Tian G, Miyagi M, Malagu KF, Lock CJ, Esmieu WR, Owens AP, Lindsay NA, Ouwehand K, Albertus F, Fischer DF, Bürli RW, MacLeod AM, Harte WE, Palczewski K, Imanishi Y
Abstract
Usher syndrome type III (USH3), characterized by progressive deafness, variable balance disorder and blindness, is caused by destabilizing mutations in the gene encoding the clarin-1 (CLRN1) protein. Here we report a new strategy to mitigate hearing loss associated with a common USH3 mutation CLRN1(N48K) that involves cell-based high-throughput screening of small molecules capable of stabilizing CLRN1(N48K), followed by a secondary screening to eliminate general proteasome inhibitors, and finally an iterative process to optimize structure-activity relationships. This resulted in the identification of BioFocus 844 (BF844). To test the efficacy of BF844, we developed a mouse model that mimicked the progressive hearing loss associated with USH3. BF844 effectively attenuated progressive hearing loss and prevented deafness in this model. Because the CLRN1(N48K) mutation causes both hearing and vision loss, BF844 could in principle prevent both sensory deficiencies in patients with USH3. Moreover, the strategy described here could help identify drugs for other protein-destabilizing monogenic disorders.
PMID: 27110679 [PubMed - as supplied by publisher]
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Comprehensive molecular diagnosis of 67 Chinese Usher syndrome probands: high rate of ethnicity specific mutations in Chinese USH patients.
Orphanet J Rare Dis. 2015;10:110
Authors: Jiang L, Liang X, Li Y, Wang J, Zaneveld JE, Wang H, Xu S, Wang K, Wang B, Chen R, Sui R
Abstract
BACKGROUND: Usher syndrome (USH) is the most common disease causing combined deafness and blindness. It is predominantly an autosomal recessive genetic disorder with occasionally digenic cases. Molecular diagnosis of USH patients is important for disease management. Few studies have tried to find the genetic cause of USH in Chinese patients. This study was designed to determine the mutation spectrum of Chinese USH patients.
METHODS: We applied next generation sequencing to characterize the mutation spectrum in 67 independent Chinese families with at least one member diagnosed with USH. Blood was collected at Peking Union Medical College Hospital. This cohort is one of the largest USH cohorts reported. We utilized customized panel and whole exome sequencing, variant analysis, Sanger validation and segregation tests to find disease causing mutations in these families.
RESULTS: We identified biallelic disease causing mutations in known USH genes in 70 % (49) of our patients. As has been previously reported, MYO7A is the most frequently mutated gene in our USH type I patients while USH2A is the most mutated gene in our USH type II patients. In addition, we identify mutations in CLRN1, DFNB31, GPR98 and PCDH15 for the first time in Chinese USH patients. Together, mutations in CLRN1, DNFB31, GPR98 and PCDH15 account for 11.4 % of disease in our cohort. Interestingly, although the spectrum of disease genes is quite similar between our Chinese patient cohort and other patient cohorts from different (and primarily Caucasian) ethnic backgrounds, the mutations themselves are dramatically different. In particular, 76 % (52/68) of alleles found in this study have never been previously reported. Interestingly, we observed a strong enrichment for severe protein truncating mutations expected to have severe functional consequence on the protein in USH II patients compared to the reported mutation spectrum in RP patients, who often carry partial protein truncating mutations.
CONCLUSIONS: Our study provides the first comprehensive genetic characterization of a large collection of Chinese USH patients. Up to 90 % of USH patients have disease caused by mutations in known USH disease genes. By combining NGS-based molecular diagnosis and patient clinical information, a more accurate diagnosis, prognosis and personalized treatment of USH patients can be achieved.
PMID: 26338283 [PubMed - indexed for MEDLINE]