Investigating the Knowledge, Skills, and Tasks Required for Hearing Aid Management: Perspectives of Clinicians and Hearing Aid Owners

Purpose

The purpose of this study is to identify hearing aid owners' and clinicians' opinions of the knowledge, skills, and tasks required for hearing aid management and the importance of each of these to overall success with hearing aids.

Method

Concept mapping techniques were used to identify key themes, wherein participants generated, sorted, and rated the importance of statements in response to the question “What must hearing aid owners do in order to use, handle, manage, maintain, and care for their hearing aids?” Twenty-four hearing aid owners (56 to 91 years of age; 54.2% men, 45.8% women) and 22 clinicians (32 to 69 years of age; 9.1% men, 90.9% women) participated.

Result

Participants identified 111 unique items describing hearing aid management within 6 concepts: (a) “Daily Hearing Aid Use,” (b) “Hearing Aid Maintenance and Repairs,” (c) “Learning to Come to Terms with Hearing Aids,” (d) “Communication Strategies,” (e) “Working With Your Clinician,” and (f) “Advanced Hearing Aid Knowledge.” Clinicians' opinions of the importance of each statement varied only slightly from the opinions of the hearing aid owner group. Hearing aid owners indicated that all 6 concepts were of similar importance, whereas clinicians indicated that the concept “Advanced Hearing Aid Knowledge” was significantly less important than the other 5 concepts.

Conclusion

The results highlight the magnitude of information and skill required to optimally manage hearing aids. Clinical recommendations are made to improve hearing aid handling education and skill acquisition.

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Grammar Clinical Marker Yields Substantial Heritability for Language Impairments in 16-Year-Old Twins

Purpose

There is a need for well-defined language phenotypes suitable for adolescents in twin studies and other large-scale research projects. Rice, Hoffman, and Wexler (2009) have developed a grammatical judgment measure as a clinical marker of language impairment, which has an extended developmental range to adolescence.

Method

We conducted the first twin analysis, along with associated phenotypic analyses of validity, of an abridged, 20-item version of this grammatical judgment measure (GJ-20), based on telephone administration using prerecorded stimuli to 405 pairs of 16-year-olds (148 monozygotic and 257 dizygotic) drawn from the Twins Early Development Study (Haworth, Davis, & Plomin, 2012).

Results

The distribution of scores is markedly skewed negatively, as expected for a potential clinical marker. Low performance on GJ-20 is associated with lower maternal education, reported learning disability (age 7 years), and low scores on language tests administered via the Twins Early Development Study (age 16 years) as well as General Certificate of Secondary Education English and Math examination performance (age 16 years). Liability threshold estimates for the genetic influence on low performance on GJ-20 are substantial, ranging from 36% with a lowest 10% criterion to 74% for a lowest 5% criterion.

Conclusions

The heritability of GJ-20 scores, especially at more extreme cutoffs, along with the score distribution and association with other indicators of language impairments, provides additional evidence for the potential value of this measure as a clinical marker of specific language impairment.

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Author's Rebuttal to Smits et al. (2017), “Comment on ‘Sensitivity of the Speech Intelligibility Index to the Assumed Dynamic Range’ by Jin et al. (2017)”

Purpose

The purpose of this letter is to refute the comments written by Smits, Goverts, and Versfeld (2017).

Method

Refutations to each issue including the fixed mathematical relationship between dynamic range (DR) and a fitting constant (Q value), deviating results for small DRs, and determination of Speech Intelligibility Index (SII) model parameters are described.

Results

Although Smits et al. (2017) correctly identified several issues, those comments do not diminish the results of the original article (Jin, Kates, & Arehart, 2017) in providing new insights for the SII.

Conclusions

Jin et al. (2017) clearly provided the impact of languages and DR on the SII, which was the main result of the study.

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Comment on “Sensitivity of the Speech Intelligibility Index to the Assumed Dynamic Range,” by Jin et al. (2017)

Purpose

This letter to the editor is in response to a research note by Jin, Kates, and Arehart (2017), “Sensitivity of the Speech Intelligibility Index to the Assumed Dynamic Range,” published in June 2017 by the Journal of Speech, Language, and Hearing Research.

Conclusion

The authors argue that the approach and line of reasoning in the Jin et al. (2017) research note suggest new findings but do not lead to essentially new insights.

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Investigating the Knowledge, Skills, and Tasks Required for Hearing Aid Management: Perspectives of Clinicians and Hearing Aid Owners

Purpose

The purpose of this study is to identify hearing aid owners' and clinicians' opinions of the knowledge, skills, and tasks required for hearing aid management and the importance of each of these to overall success with hearing aids.

Method

Concept mapping techniques were used to identify key themes, wherein participants generated, sorted, and rated the importance of statements in response to the question “What must hearing aid owners do in order to use, handle, manage, maintain, and care for their hearing aids?” Twenty-four hearing aid owners (56 to 91 years of age; 54.2% men, 45.8% women) and 22 clinicians (32 to 69 years of age; 9.1% men, 90.9% women) participated.

Result

Participants identified 111 unique items describing hearing aid management within 6 concepts: (a) “Daily Hearing Aid Use,” (b) “Hearing Aid Maintenance and Repairs,” (c) “Learning to Come to Terms with Hearing Aids,” (d) “Communication Strategies,” (e) “Working With Your Clinician,” and (f) “Advanced Hearing Aid Knowledge.” Clinicians' opinions of the importance of each statement varied only slightly from the opinions of the hearing aid owner group. Hearing aid owners indicated that all 6 concepts were of similar importance, whereas clinicians indicated that the concept “Advanced Hearing Aid Knowledge” was significantly less important than the other 5 concepts.

Conclusion

The results highlight the magnitude of information and skill required to optimally manage hearing aids. Clinical recommendations are made to improve hearing aid handling education and skill acquisition.

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Grammar Clinical Marker Yields Substantial Heritability for Language Impairments in 16-Year-Old Twins

Purpose

There is a need for well-defined language phenotypes suitable for adolescents in twin studies and other large-scale research projects. Rice, Hoffman, and Wexler (2009) have developed a grammatical judgment measure as a clinical marker of language impairment, which has an extended developmental range to adolescence.

Method

We conducted the first twin analysis, along with associated phenotypic analyses of validity, of an abridged, 20-item version of this grammatical judgment measure (GJ-20), based on telephone administration using prerecorded stimuli to 405 pairs of 16-year-olds (148 monozygotic and 257 dizygotic) drawn from the Twins Early Development Study (Haworth, Davis, & Plomin, 2012).

Results

The distribution of scores is markedly skewed negatively, as expected for a potential clinical marker. Low performance on GJ-20 is associated with lower maternal education, reported learning disability (age 7 years), and low scores on language tests administered via the Twins Early Development Study (age 16 years) as well as General Certificate of Secondary Education English and Math examination performance (age 16 years). Liability threshold estimates for the genetic influence on low performance on GJ-20 are substantial, ranging from 36% with a lowest 10% criterion to 74% for a lowest 5% criterion.

Conclusions

The heritability of GJ-20 scores, especially at more extreme cutoffs, along with the score distribution and association with other indicators of language impairments, provides additional evidence for the potential value of this measure as a clinical marker of specific language impairment.

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Author's Rebuttal to Smits et al. (2017), “Comment on ‘Sensitivity of the Speech Intelligibility Index to the Assumed Dynamic Range’ by Jin et al. (2017)”

Purpose

The purpose of this letter is to refute the comments written by Smits, Goverts, and Versfeld (2017).

Method

Refutations to each issue including the fixed mathematical relationship between dynamic range (DR) and a fitting constant (Q value), deviating results for small DRs, and determination of Speech Intelligibility Index (SII) model parameters are described.

Results

Although Smits et al. (2017) correctly identified several issues, those comments do not diminish the results of the original article (Jin, Kates, & Arehart, 2017) in providing new insights for the SII.

Conclusions

Jin et al. (2017) clearly provided the impact of languages and DR on the SII, which was the main result of the study.

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Comment on “Sensitivity of the Speech Intelligibility Index to the Assumed Dynamic Range,” by Jin et al. (2017)

Purpose

This letter to the editor is in response to a research note by Jin, Kates, and Arehart (2017), “Sensitivity of the Speech Intelligibility Index to the Assumed Dynamic Range,” published in June 2017 by the Journal of Speech, Language, and Hearing Research.

Conclusion

The authors argue that the approach and line of reasoning in the Jin et al. (2017) research note suggest new findings but do not lead to essentially new insights.

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Investigating the Knowledge, Skills, and Tasks Required for Hearing Aid Management: Perspectives of Clinicians and Hearing Aid Owners

Purpose

The purpose of this study is to identify hearing aid owners' and clinicians' opinions of the knowledge, skills, and tasks required for hearing aid management and the importance of each of these to overall success with hearing aids.

Method

Concept mapping techniques were used to identify key themes, wherein participants generated, sorted, and rated the importance of statements in response to the question “What must hearing aid owners do in order to use, handle, manage, maintain, and care for their hearing aids?” Twenty-four hearing aid owners (56 to 91 years of age; 54.2% men, 45.8% women) and 22 clinicians (32 to 69 years of age; 9.1% men, 90.9% women) participated.

Result

Participants identified 111 unique items describing hearing aid management within 6 concepts: (a) “Daily Hearing Aid Use,” (b) “Hearing Aid Maintenance and Repairs,” (c) “Learning to Come to Terms with Hearing Aids,” (d) “Communication Strategies,” (e) “Working With Your Clinician,” and (f) “Advanced Hearing Aid Knowledge.” Clinicians' opinions of the importance of each statement varied only slightly from the opinions of the hearing aid owner group. Hearing aid owners indicated that all 6 concepts were of similar importance, whereas clinicians indicated that the concept “Advanced Hearing Aid Knowledge” was significantly less important than the other 5 concepts.

Conclusion

The results highlight the magnitude of information and skill required to optimally manage hearing aids. Clinical recommendations are made to improve hearing aid handling education and skill acquisition.

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Grammar Clinical Marker Yields Substantial Heritability for Language Impairments in 16-Year-Old Twins

Purpose

There is a need for well-defined language phenotypes suitable for adolescents in twin studies and other large-scale research projects. Rice, Hoffman, and Wexler (2009) have developed a grammatical judgment measure as a clinical marker of language impairment, which has an extended developmental range to adolescence.

Method

We conducted the first twin analysis, along with associated phenotypic analyses of validity, of an abridged, 20-item version of this grammatical judgment measure (GJ-20), based on telephone administration using prerecorded stimuli to 405 pairs of 16-year-olds (148 monozygotic and 257 dizygotic) drawn from the Twins Early Development Study (Haworth, Davis, & Plomin, 2012).

Results

The distribution of scores is markedly skewed negatively, as expected for a potential clinical marker. Low performance on GJ-20 is associated with lower maternal education, reported learning disability (age 7 years), and low scores on language tests administered via the Twins Early Development Study (age 16 years) as well as General Certificate of Secondary Education English and Math examination performance (age 16 years). Liability threshold estimates for the genetic influence on low performance on GJ-20 are substantial, ranging from 36% with a lowest 10% criterion to 74% for a lowest 5% criterion.

Conclusions

The heritability of GJ-20 scores, especially at more extreme cutoffs, along with the score distribution and association with other indicators of language impairments, provides additional evidence for the potential value of this measure as a clinical marker of specific language impairment.

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Author's Rebuttal to Smits et al. (2017), “Comment on ‘Sensitivity of the Speech Intelligibility Index to the Assumed Dynamic Range’ by Jin et al. (2017)”

Purpose

The purpose of this letter is to refute the comments written by Smits, Goverts, and Versfeld (2017).

Method

Refutations to each issue including the fixed mathematical relationship between dynamic range (DR) and a fitting constant (Q value), deviating results for small DRs, and determination of Speech Intelligibility Index (SII) model parameters are described.

Results

Although Smits et al. (2017) correctly identified several issues, those comments do not diminish the results of the original article (Jin, Kates, & Arehart, 2017) in providing new insights for the SII.

Conclusions

Jin et al. (2017) clearly provided the impact of languages and DR on the SII, which was the main result of the study.

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Comment on “Sensitivity of the Speech Intelligibility Index to the Assumed Dynamic Range,” by Jin et al. (2017)

Purpose

This letter to the editor is in response to a research note by Jin, Kates, and Arehart (2017), “Sensitivity of the Speech Intelligibility Index to the Assumed Dynamic Range,” published in June 2017 by the Journal of Speech, Language, and Hearing Research.

Conclusion

The authors argue that the approach and line of reasoning in the Jin et al. (2017) research note suggest new findings but do not lead to essentially new insights.

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InnerScope to Distribute Universal Remote Hearing Aid Programming Software

​InnerScope Hearing Technologies (http://www.innd.com/) has entered into a letter of intent (LOI) for an exclusive worldwide licensing and distribution for the FlexiHub Software as it relates to remote programming of hearing aids. The FlexiHub Software, created by Eltima Software GmbH, is a USB-over-Ethernet technology that allows total secure access and connection remotely via the internet for sharing of USB port devices to any remote computers around the world or using an Android smartphone. Hearing care providers can use the FlexiHub to remotely program and adjust their patients' hearing aids remotely, reducing the number of clinic visits for fine-tuning adjustments of hearing aids. InnerScope plans to make the FlexiHub available through a monthly or yearly subscription for hearing professionals and hearing aid users. Under the LOI, the company will also be able to white label the software and sublicense the use for other teleaudiology software platforms to global retailers and manufactures of hearing aid devices and other hearing augmented technologies.​

Published: 1/5/2018 10:45:00 AM

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InnerScope to Distribute Universal Remote Hearing Aid Programming Software

​InnerScope Hearing Technologies (http://www.innd.com/) has entered into a letter of intent (LOI) for an exclusive worldwide licensing and distribution for the FlexiHub Software as it relates to remote programming of hearing aids. The FlexiHub Software, created by Eltima Software GmbH, is a USB-over-Ethernet technology that allows total secure access and connection remotely via the internet for sharing of USB port devices to any remote computers around the world or using an Android smartphone. Hearing care providers can use the FlexiHub to remotely program and adjust their patients' hearing aids remotely, reducing the number of clinic visits for fine-tuning adjustments of hearing aids. InnerScope plans to make the FlexiHub available through a monthly or yearly subscription for hearing professionals and hearing aid users. Under the LOI, the company will also be able to white label the software and sublicense the use for other teleaudiology software platforms to global retailers and manufactures of hearing aid devices and other hearing augmented technologies.​

Published: 1/5/2018 10:45:00 AM

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InnerScope to Distribute Universal Remote Hearing Aid Programming Software

​InnerScope Hearing Technologies (http://www.innd.com/) has entered into a letter of intent (LOI) for an exclusive worldwide licensing and distribution for the FlexiHub Software as it relates to remote programming of hearing aids. The FlexiHub Software, created by Eltima Software GmbH, is a USB-over-Ethernet technology that allows total secure access and connection remotely via the internet for sharing of USB port devices to any remote computers around the world or using an Android smartphone. Hearing care providers can use the FlexiHub to remotely program and adjust their patients' hearing aids remotely, reducing the number of clinic visits for fine-tuning adjustments of hearing aids. InnerScope plans to make the FlexiHub available through a monthly or yearly subscription for hearing professionals and hearing aid users. Under the LOI, the company will also be able to white label the software and sublicense the use for other teleaudiology software platforms to global retailers and manufactures of hearing aid devices and other hearing augmented technologies.​

Published: 1/5/2018 10:45:00 AM

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Depression and posture in patients with Parkinson’s disease

Publication date: March 2018
Source:Gait & Posture, Volume 61
Author(s): Youkyung Kim, Sang-Myung Cheon, Changhong Youm, Minji Son, Jae Woo Kim
BackgroundDepression is an important non-motor symptom of Parkinson’s disease (PD) that significantly impacts the daily activities of affected patients. Furthermore, the stooped posture that characterizes patients with PD has also been associated with depression. The purpose of this study was to investigate the relationship between the presence of depressive symptoms and body posture in patients with PD.MethodsForty-six patients with mild-to-moderate PD were recruited. The patients were divided into depression and no depression groups based on Beck Depression Inventory scores. All patients underwent kinematic analysis conducted in the upright standing posture with a motion capture system.ResultsThere were no differences in clinical characteristics between the depression (n = 22) and no depression groups (n = 24). In the standing position, patients with depression showed anterior tilting of the head from the pelvis and an increased distance between head and pelvis. The severity of depression was correlated with the degree of flexion at the lower trunk level and the degree of anterior tilting of the head, neck, and trunk from the pelvis and base of support.ConclusionsPatients with PD and depression showed increased flexion at pelvis level, which caused the trunk to tilt anteriorly. In addition, the severity of depression was correlated with the degree of anterior tilting of the head and trunk. These findings suggest that stooped posture, especially from the pelvis level, could be a marker of depression in patients with PD.



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Repeatability of the Oxford Foot Model in children with foot deformity

Publication date: March 2018
Source:Gait & Posture, Volume 61
Author(s): Jennifer McCahill, Julie Stebbins, Bart Koning, Jaap Harlaar, Tim Theologis
IntroductionThe Oxford Foot Model (OFM) is a multi-segment, kinematic model developed to assess foot motion. It has previously been assessed for repeatability in healthy populations. To determine the OFM’s reliability for detecting foot deformity, it is important to know repeatability in pathological conditions. The aim of the study was to assess the repeatability of the OFM in children with foot deformity.MethodsIntra-tester repeatability was assessed for 45 children (15 typically developing, 15 hemiplegic, 15 clubfoot). Inter-tester repeatability was assessed in the clubfoot population. The mean absolute differences between testers (clubfoot) and sessions (clubfoot and hemiplegic) were calculated for each of 15 clinically relevant, kinematic variables and compared to typically developing children.ResultsChildren with clubfoot showed a mean difference between visits of 2.9° and a mean difference between raters of 3.6° Mean absolute differences were within one degree for the intra and inter-rater reliability in 12/15 variables. Hindfoot rotation, forefoot/tibia abduction and forefoot supination were the most variable between testers. Overall the clubfoot data were less variable than the typically developing population.Children with hemiplegia demonstrated slightly higher differences between sessions (mean 4.1°), with the most reliable data in the sagittal plane, and largest differences in the transverse plane.ConclusionsThe OFM was designed to measure different types of foot deformity. The results of this study show that it provides repeatable results in children with foot deformity. To be distinguished from measurement artifact, changes in foot kinematics as a result of intervention or natural progression over time must be greater than the repeatability reported here.



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Depression and posture in patients with Parkinson’s disease

Publication date: March 2018
Source:Gait & Posture, Volume 61
Author(s): Youkyung Kim, Sang-Myung Cheon, Changhong Youm, Minji Son, Jae Woo Kim
BackgroundDepression is an important non-motor symptom of Parkinson’s disease (PD) that significantly impacts the daily activities of affected patients. Furthermore, the stooped posture that characterizes patients with PD has also been associated with depression. The purpose of this study was to investigate the relationship between the presence of depressive symptoms and body posture in patients with PD.MethodsForty-six patients with mild-to-moderate PD were recruited. The patients were divided into depression and no depression groups based on Beck Depression Inventory scores. All patients underwent kinematic analysis conducted in the upright standing posture with a motion capture system.ResultsThere were no differences in clinical characteristics between the depression (n = 22) and no depression groups (n = 24). In the standing position, patients with depression showed anterior tilting of the head from the pelvis and an increased distance between head and pelvis. The severity of depression was correlated with the degree of flexion at the lower trunk level and the degree of anterior tilting of the head, neck, and trunk from the pelvis and base of support.ConclusionsPatients with PD and depression showed increased flexion at pelvis level, which caused the trunk to tilt anteriorly. In addition, the severity of depression was correlated with the degree of anterior tilting of the head and trunk. These findings suggest that stooped posture, especially from the pelvis level, could be a marker of depression in patients with PD.



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Repeatability of the Oxford Foot Model in children with foot deformity

Publication date: March 2018
Source:Gait & Posture, Volume 61
Author(s): Jennifer McCahill, Julie Stebbins, Bart Koning, Jaap Harlaar, Tim Theologis
IntroductionThe Oxford Foot Model (OFM) is a multi-segment, kinematic model developed to assess foot motion. It has previously been assessed for repeatability in healthy populations. To determine the OFM’s reliability for detecting foot deformity, it is important to know repeatability in pathological conditions. The aim of the study was to assess the repeatability of the OFM in children with foot deformity.MethodsIntra-tester repeatability was assessed for 45 children (15 typically developing, 15 hemiplegic, 15 clubfoot). Inter-tester repeatability was assessed in the clubfoot population. The mean absolute differences between testers (clubfoot) and sessions (clubfoot and hemiplegic) were calculated for each of 15 clinically relevant, kinematic variables and compared to typically developing children.ResultsChildren with clubfoot showed a mean difference between visits of 2.9° and a mean difference between raters of 3.6° Mean absolute differences were within one degree for the intra and inter-rater reliability in 12/15 variables. Hindfoot rotation, forefoot/tibia abduction and forefoot supination were the most variable between testers. Overall the clubfoot data were less variable than the typically developing population.Children with hemiplegia demonstrated slightly higher differences between sessions (mean 4.1°), with the most reliable data in the sagittal plane, and largest differences in the transverse plane.ConclusionsThe OFM was designed to measure different types of foot deformity. The results of this study show that it provides repeatable results in children with foot deformity. To be distinguished from measurement artifact, changes in foot kinematics as a result of intervention or natural progression over time must be greater than the repeatability reported here.



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Depression and posture in patients with Parkinson’s disease

Publication date: March 2018
Source:Gait & Posture, Volume 61
Author(s): Youkyung Kim, Sang-Myung Cheon, Changhong Youm, Minji Son, Jae Woo Kim
BackgroundDepression is an important non-motor symptom of Parkinson’s disease (PD) that significantly impacts the daily activities of affected patients. Furthermore, the stooped posture that characterizes patients with PD has also been associated with depression. The purpose of this study was to investigate the relationship between the presence of depressive symptoms and body posture in patients with PD.MethodsForty-six patients with mild-to-moderate PD were recruited. The patients were divided into depression and no depression groups based on Beck Depression Inventory scores. All patients underwent kinematic analysis conducted in the upright standing posture with a motion capture system.ResultsThere were no differences in clinical characteristics between the depression (n = 22) and no depression groups (n = 24). In the standing position, patients with depression showed anterior tilting of the head from the pelvis and an increased distance between head and pelvis. The severity of depression was correlated with the degree of flexion at the lower trunk level and the degree of anterior tilting of the head, neck, and trunk from the pelvis and base of support.ConclusionsPatients with PD and depression showed increased flexion at pelvis level, which caused the trunk to tilt anteriorly. In addition, the severity of depression was correlated with the degree of anterior tilting of the head and trunk. These findings suggest that stooped posture, especially from the pelvis level, could be a marker of depression in patients with PD.



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Repeatability of the Oxford Foot Model in children with foot deformity

Publication date: March 2018
Source:Gait & Posture, Volume 61
Author(s): Jennifer McCahill, Julie Stebbins, Bart Koning, Jaap Harlaar, Tim Theologis
IntroductionThe Oxford Foot Model (OFM) is a multi-segment, kinematic model developed to assess foot motion. It has previously been assessed for repeatability in healthy populations. To determine the OFM’s reliability for detecting foot deformity, it is important to know repeatability in pathological conditions. The aim of the study was to assess the repeatability of the OFM in children with foot deformity.MethodsIntra-tester repeatability was assessed for 45 children (15 typically developing, 15 hemiplegic, 15 clubfoot). Inter-tester repeatability was assessed in the clubfoot population. The mean absolute differences between testers (clubfoot) and sessions (clubfoot and hemiplegic) were calculated for each of 15 clinically relevant, kinematic variables and compared to typically developing children.ResultsChildren with clubfoot showed a mean difference between visits of 2.9° and a mean difference between raters of 3.6° Mean absolute differences were within one degree for the intra and inter-rater reliability in 12/15 variables. Hindfoot rotation, forefoot/tibia abduction and forefoot supination were the most variable between testers. Overall the clubfoot data were less variable than the typically developing population.Children with hemiplegia demonstrated slightly higher differences between sessions (mean 4.1°), with the most reliable data in the sagittal plane, and largest differences in the transverse plane.ConclusionsThe OFM was designed to measure different types of foot deformity. The results of this study show that it provides repeatable results in children with foot deformity. To be distinguished from measurement artifact, changes in foot kinematics as a result of intervention or natural progression over time must be greater than the repeatability reported here.



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Dystonia-deafness syndrome caused by a β-actin gene mutation and response to deep brain stimulation.

Related Articles

Dystonia-deafness syndrome caused by a β-actin gene mutation and response to deep brain stimulation.

Mov Disord. 2017 Jan;32(1):162-165

Authors: Eggink H, van Egmond ME, Verschuuren-Bemelmans CC, Schönherr MC, de Koning TJ, Oterdoom DL, van Dijk JM, Tijssen MA

Abstract
INTRODUCTION: Dystonia-deafness syndrome is a distinct clinical presentation within the dystonia-spectrum. Although several genetic and acquired causes have been reported, etiology remains unknown in the majority of patients.
OBJECTIVES: To describe two patients with dystonia-deafness syndrome due to a beta-actin gene mutation.
METHODS: We report on disease course, genetic testing, and management of 2 patients, mother and daughter, presenting with dystonia-deafness syndrome.
RESULTS: After exclusion of known dystonia-deafness syndrome causes, whole-exome sequencing revealed a beta-actin gene mutation (p.Arg183Trp) in both patients. Although beta-actin gene mutations are generally associated with developmental Baraitser-Winter syndrome, dystonia-deafness syndrome has been reported once in identical twin brothers. Bilateral GPi-DBS led to a significant decrease of dystonia and regain of independency in our patients.
CONCLUSION: The p.Arg183Trp mutation in the beta-actin gene is associated with the clinical presentation of dystonia-deafness syndrome, even with only minimal or no developmental abnormalities of Baraitser-Winter syndrome. GPi-DBS should be considered to ameliorate the invalidating dystonia in these patients. © 2016 International Parkinson and Movement Disorder Society.

PMID: 27862284 [PubMed - indexed for MEDLINE]

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The reduction of gunshot noise and auditory risk through the use of firearm suppressors and low-velocity ammunition.

Related Articles

The reduction of gunshot noise and auditory risk through the use of firearm suppressors and low-velocity ammunition.

Int J Audiol. 2018 Jan 04;:1-14

Authors: Murphy WJ, Flamme GA, Campbell AR, Zechmann EL, Tasko SM, Lankford JE, Meinke DK, Finan DS, Stewart M

Abstract
OBJECTIVE: This research assessed the reduction of peak levels, equivalent energy and sound power of firearm suppressors.
DESIGN: The first study evaluated the effect of three suppressors at four microphone positions around four firearms. The second study assessed the suppressor-related reduction of sound power with a 3 m hemispherical microphone array for two firearms.
RESULTS: The suppressors reduced exposures at the ear between 17 and 24 dB peak sound pressure level and reduced the 8 h equivalent A-weighted energy between 9 and 21 dB depending upon the firearm and ammunition. Noise reductions observed for the instructor's position about a metre behind the shooter were between 20 and 28 dB peak sound pressure level and between 11 and 26 dB LAeq,8h. Firearm suppressors reduced the measured sound power levels between 2 and 23 dB. Sound power reductions were greater for the low-velocity ammunition than for the same firearms fired with high-velocity ammunition due to the effect of N-waves produced by a supersonic bullet.
CONCLUSIONS: Firearm suppressors may reduce noise exposure, and the cumulative exposures of suppressed firearms can still present a significant hearing risk. Therefore, firearm users should always wear hearing protection whenever target shooting or hunting.

PMID: 29299940 [PubMed - as supplied by publisher]

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Is non-linear frequency compression amplification beneficial to adults and children with hearing loss? A systematic review.

Related Articles

Is non-linear frequency compression amplification beneficial to adults and children with hearing loss? A systematic review.

Int J Audiol. 2018 Jan 03;:1-12

Authors: Akinseye GA, Dickinson AM, Munro KJ

Abstract
OBJECTIVE: To conduct a systematic review of the benefits of non-linear frequency compression (NLFC) in adults and children.
DESIGN: Ten databases were searched for studies comparing the effects of NLFC and conventional processing (CP) for the period January 2008 to September 2017.
STUDY SAMPLE: Twelve articles were included in this review: four adults and school-aged only, one pre-school only and three with both adults and school-aged children.
RESULTS: A two-stage process was implemented to grade the evidence. The individual studies were graded based on their study type (from 1 = highest quality of evidence to 5 = the lowest quality) and then sub-graded based on their quality ("a" for "good quality" or "b" for "lesser quality"). All studies were awarded 4a, except the single pre-school study, which was awarded 2a. The overall evidence for each population was graded based on the quality, quantity and consistency of the studies. The body of evidence was rated as very low for both adults and school-aged children, but high for pre-school children.
CONCLUSION: The low number (and quality) of studies means that evidence supporting the benefit from NLFC is inconclusive. Further high-quality RCTs are required to provide a conclusive answer to this question.

PMID: 29298522 [PubMed - as supplied by publisher]

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The reduction of gunshot noise and auditory risk through the use of firearm suppressors and low-velocity ammunition.

Related Articles

The reduction of gunshot noise and auditory risk through the use of firearm suppressors and low-velocity ammunition.

Int J Audiol. 2018 Jan 04;:1-14

Authors: Murphy WJ, Flamme GA, Campbell AR, Zechmann EL, Tasko SM, Lankford JE, Meinke DK, Finan DS, Stewart M

Abstract
OBJECTIVE: This research assessed the reduction of peak levels, equivalent energy and sound power of firearm suppressors.
DESIGN: The first study evaluated the effect of three suppressors at four microphone positions around four firearms. The second study assessed the suppressor-related reduction of sound power with a 3 m hemispherical microphone array for two firearms.
RESULTS: The suppressors reduced exposures at the ear between 17 and 24 dB peak sound pressure level and reduced the 8 h equivalent A-weighted energy between 9 and 21 dB depending upon the firearm and ammunition. Noise reductions observed for the instructor's position about a metre behind the shooter were between 20 and 28 dB peak sound pressure level and between 11 and 26 dB LAeq,8h. Firearm suppressors reduced the measured sound power levels between 2 and 23 dB. Sound power reductions were greater for the low-velocity ammunition than for the same firearms fired with high-velocity ammunition due to the effect of N-waves produced by a supersonic bullet.
CONCLUSIONS: Firearm suppressors may reduce noise exposure, and the cumulative exposures of suppressed firearms can still present a significant hearing risk. Therefore, firearm users should always wear hearing protection whenever target shooting or hunting.

PMID: 29299940 [PubMed - as supplied by publisher]

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via IFTTT

Is non-linear frequency compression amplification beneficial to adults and children with hearing loss? A systematic review.

Related Articles

Is non-linear frequency compression amplification beneficial to adults and children with hearing loss? A systematic review.

Int J Audiol. 2018 Jan 03;:1-12

Authors: Akinseye GA, Dickinson AM, Munro KJ

Abstract
OBJECTIVE: To conduct a systematic review of the benefits of non-linear frequency compression (NLFC) in adults and children.
DESIGN: Ten databases were searched for studies comparing the effects of NLFC and conventional processing (CP) for the period January 2008 to September 2017.
STUDY SAMPLE: Twelve articles were included in this review: four adults and school-aged only, one pre-school only and three with both adults and school-aged children.
RESULTS: A two-stage process was implemented to grade the evidence. The individual studies were graded based on their study type (from 1 = highest quality of evidence to 5 = the lowest quality) and then sub-graded based on their quality ("a" for "good quality" or "b" for "lesser quality"). All studies were awarded 4a, except the single pre-school study, which was awarded 2a. The overall evidence for each population was graded based on the quality, quantity and consistency of the studies. The body of evidence was rated as very low for both adults and school-aged children, but high for pre-school children.
CONCLUSION: The low number (and quality) of studies means that evidence supporting the benefit from NLFC is inconclusive. Further high-quality RCTs are required to provide a conclusive answer to this question.

PMID: 29298522 [PubMed - as supplied by publisher]

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via IFTTT

The reduction of gunshot noise and auditory risk through the use of firearm suppressors and low-velocity ammunition

.


from #Audiology via ola Kala on Inoreader http://ift.tt/2qofNiy
via IFTTT

The reduction of gunshot noise and auditory risk through the use of firearm suppressors and low-velocity ammunition

.


from #Audiology via xlomafota13 on Inoreader http://ift.tt/2qofNiy
via IFTTT

The reduction of gunshot noise and auditory risk through the use of firearm suppressors and low-velocity ammunition

.


from #Audiology via ola Kala on Inoreader http://ift.tt/2qofNiy
via IFTTT

The reduction of gunshot noise and auditory risk through the use of firearm suppressors and low-velocity ammunition.

The reduction of gunshot noise and auditory risk through the use of firearm suppressors and low-velocity ammunition.

Int J Audiol. 2018 Jan 04;:1-14

Authors: Murphy WJ, Flamme GA, Campbell AR, Zechmann EL, Tasko SM, Lankford JE, Meinke DK, Finan DS, Stewart M

Abstract
OBJECTIVE: This research assessed the reduction of peak levels, equivalent energy and sound power of firearm suppressors.
DESIGN: The first study evaluated the effect of three suppressors at four microphone positions around four firearms. The second study assessed the suppressor-related reduction of sound power with a 3 m hemispherical microphone array for two firearms.
RESULTS: The suppressors reduced exposures at the ear between 17 and 24 dB peak sound pressure level and reduced the 8 h equivalent A-weighted energy between 9 and 21 dB depending upon the firearm and ammunition. Noise reductions observed for the instructor's position about a metre behind the shooter were between 20 and 28 dB peak sound pressure level and between 11 and 26 dB LAeq,8h. Firearm suppressors reduced the measured sound power levels between 2 and 23 dB. Sound power reductions were greater for the low-velocity ammunition than for the same firearms fired with high-velocity ammunition due to the effect of N-waves produced by a supersonic bullet.
CONCLUSIONS: Firearm suppressors may reduce noise exposure, and the cumulative exposures of suppressed firearms can still present a significant hearing risk. Therefore, firearm users should always wear hearing protection whenever target shooting or hunting.

PMID: 29299940 [PubMed - as supplied by publisher]

from #Audiology via ola Kala on Inoreader http://ift.tt/2E90wnO
via IFTTT

Is non-linear frequency compression amplification beneficial to adults and children with hearing loss? A systematic review.

Related Articles

Is non-linear frequency compression amplification beneficial to adults and children with hearing loss? A systematic review.

Int J Audiol. 2018 Jan 03;:1-12

Authors: Akinseye GA, Dickinson AM, Munro KJ

Abstract
OBJECTIVE: To conduct a systematic review of the benefits of non-linear frequency compression (NLFC) in adults and children.
DESIGN: Ten databases were searched for studies comparing the effects of NLFC and conventional processing (CP) for the period January 2008 to September 2017.
STUDY SAMPLE: Twelve articles were included in this review: four adults and school-aged only, one pre-school only and three with both adults and school-aged children.
RESULTS: A two-stage process was implemented to grade the evidence. The individual studies were graded based on their study type (from 1 = highest quality of evidence to 5 = the lowest quality) and then sub-graded based on their quality ("a" for "good quality" or "b" for "lesser quality"). All studies were awarded 4a, except the single pre-school study, which was awarded 2a. The overall evidence for each population was graded based on the quality, quantity and consistency of the studies. The body of evidence was rated as very low for both adults and school-aged children, but high for pre-school children.
CONCLUSION: The low number (and quality) of studies means that evidence supporting the benefit from NLFC is inconclusive. Further high-quality RCTs are required to provide a conclusive answer to this question.

PMID: 29298522 [PubMed - as supplied by publisher]

from #Audiology via ola Kala on Inoreader http://ift.tt/2F3kG43
via IFTTT

The reduction of gunshot noise and auditory risk through the use of firearm suppressors and low-velocity ammunition.

The reduction of gunshot noise and auditory risk through the use of firearm suppressors and low-velocity ammunition.

Int J Audiol. 2018 Jan 04;:1-14

Authors: Murphy WJ, Flamme GA, Campbell AR, Zechmann EL, Tasko SM, Lankford JE, Meinke DK, Finan DS, Stewart M

Abstract
OBJECTIVE: This research assessed the reduction of peak levels, equivalent energy and sound power of firearm suppressors.
DESIGN: The first study evaluated the effect of three suppressors at four microphone positions around four firearms. The second study assessed the suppressor-related reduction of sound power with a 3 m hemispherical microphone array for two firearms.
RESULTS: The suppressors reduced exposures at the ear between 17 and 24 dB peak sound pressure level and reduced the 8 h equivalent A-weighted energy between 9 and 21 dB depending upon the firearm and ammunition. Noise reductions observed for the instructor's position about a metre behind the shooter were between 20 and 28 dB peak sound pressure level and between 11 and 26 dB LAeq,8h. Firearm suppressors reduced the measured sound power levels between 2 and 23 dB. Sound power reductions were greater for the low-velocity ammunition than for the same firearms fired with high-velocity ammunition due to the effect of N-waves produced by a supersonic bullet.
CONCLUSIONS: Firearm suppressors may reduce noise exposure, and the cumulative exposures of suppressed firearms can still present a significant hearing risk. Therefore, firearm users should always wear hearing protection whenever target shooting or hunting.

PMID: 29299940 [PubMed - as supplied by publisher]

from #Audiology via ola Kala on Inoreader http://ift.tt/2E90wnO
via IFTTT

Is non-linear frequency compression amplification beneficial to adults and children with hearing loss? A systematic review.

Related Articles

Is non-linear frequency compression amplification beneficial to adults and children with hearing loss? A systematic review.

Int J Audiol. 2018 Jan 03;:1-12

Authors: Akinseye GA, Dickinson AM, Munro KJ

Abstract
OBJECTIVE: To conduct a systematic review of the benefits of non-linear frequency compression (NLFC) in adults and children.
DESIGN: Ten databases were searched for studies comparing the effects of NLFC and conventional processing (CP) for the period January 2008 to September 2017.
STUDY SAMPLE: Twelve articles were included in this review: four adults and school-aged only, one pre-school only and three with both adults and school-aged children.
RESULTS: A two-stage process was implemented to grade the evidence. The individual studies were graded based on their study type (from 1 = highest quality of evidence to 5 = the lowest quality) and then sub-graded based on their quality ("a" for "good quality" or "b" for "lesser quality"). All studies were awarded 4a, except the single pre-school study, which was awarded 2a. The overall evidence for each population was graded based on the quality, quantity and consistency of the studies. The body of evidence was rated as very low for both adults and school-aged children, but high for pre-school children.
CONCLUSION: The low number (and quality) of studies means that evidence supporting the benefit from NLFC is inconclusive. Further high-quality RCTs are required to provide a conclusive answer to this question.

PMID: 29298522 [PubMed - as supplied by publisher]

from #Audiology via ola Kala on Inoreader http://ift.tt/2F3kG43
via IFTTT