Tinnitus Talk

 

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If you have ever experienced a constant ringing or hissing sound in the ears, you probably already know how annoying and often debilitating it can be. Although you may not realize it, you are certainly not alone. In fact, approximately one out of every ten adults is affected by tinnitus in some way. The good news is that there is an online forum those who suffer with this condition. Tinnitus Talk Support Forum is more than simply a place to learn about the condition; it is a place to form friendships and get the support you deserve.

Virtually Endless Knowledge at Your Fingertips

One of the best things about Tinnitus Talk is that the website includes a knowledge base that is chock-full of helpful information. Whether you want to learn about how you can get involved in raising awareness and starting a tinnitus fundraiser or simply wish to read about the success stories of others, the range of useful information is virtually endless. Also included in the knowledge base are free downloads of sorts and information about clinical trials.

Get Your Questions Answered by a Real-Live Doctor

Another exceptional component of the online support forum, which makes it a favorite among both tinnitus sufferers and their concerned loved ones, is the vast selection of research articles and information about how to maintain a high quality of life. There is also a place called “Doctors’ Corner”, which allows forum members to receive answers to their questions from doctors who specialize in tinnitus and other ear-related medical conditions.

Discuss Your Tinnitus Experiences With Friends

Although there are a number of different types of tinnitus, Tinnitus Talk is an excellent resource for learning about and discussing them all. Whether you suffer from general tinnitus, pulsatile tinnitus or Hyperacusis and associated ear pain, you are sure to find a plethora of useful information as well as the support you may need to help you get through this time in your life.

Whether you currently have some form of tinnitus or hope to learn more about the condition so you can help one of your family members, Tinnitus Talk is an excellent place to turn. From making friends in the support group to communicating with highly knowledgeable doctors, there is certainly no shortage of ways to arm yourself with the tools and resources you need to move forward with your life. With Tinnitus Talk on your side, there is no need to suffer in silence.

 

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Bilateral cochlear implants in children: Effects of auditory experience and deprivation on auditory perception

Publication date: Available online 30 January 2016
Source:Hearing Research
Author(s): Ruth Y. Litovsky, Karen Gordon
Spatial hearing skills are essential for children as they grow, learn and play. They provide critical cues for determining the locations of sources in the environment, and enable segregation of important sources, such as speech, from background maskers or interferers. Spatial hearing depends on availability of monaural cues and binaural cues. The latter result from integration of inputs arriving at the two ears from sounds that vary in location. The binaural system has exquisite mechanisms for capturing differences between the ears in both time of arrival and intensity. The major cues that are thus referred to as being vital for binaural hearing are: interaural differences in time (ITDs) and interaural differences in levels (ILDs). In children with normal hearing (NH), spatial hearing abilities are fairly well developed by age 4-5 years. In contrast, children who are deaf and hear through cochlear implants (CIs) do not have an opportunity to experience normal, binaural acoustic hearing early in life. These children may function by having to utilize auditory cues that are degraded with regard to numerous stimulus features. In recent years there has been a notable increase in the number of children receiving bilateral CIs, and evidence suggests that while having two CIs helps them function better than when listening through a single CI, they generally perform worse than their NH peers. This paper reviews some of the recent work on bilaterally implanted children. The focus is on measures of spatial hearing, including sound localization, release from masking for speech understanding in noise and binaural sensitivity using research processors. Data from behavioral and electrophysiological studies are included, with a focus on the recent work of the authors and their collaborators. The effects of auditory plasticity and deprivation on the emergence of binaural and spatial hearing are discussed along with evidence for reorganized processing from both behavioral and electrophysiological studies. The consequences of both unilateral and bilateral auditory deprivation during development suggest that the relevant set of issues is highly complex with regard to successes and the limitations experienced by children receiving bilateral cochlear implants.



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Bilateral cochlear implants in children: Effects of auditory experience and deprivation on auditory perception

Publication date: Available online 30 January 2016
Source:Hearing Research
Author(s): Ruth Y. Litovsky, Karen Gordon
Spatial hearing skills are essential for children as they grow, learn and play. They provide critical cues for determining the locations of sources in the environment, and enable segregation of important sources, such as speech, from background maskers or interferers. Spatial hearing depends on availability of monaural cues and binaural cues. The latter result from integration of inputs arriving at the two ears from sounds that vary in location. The binaural system has exquisite mechanisms for capturing differences between the ears in both time of arrival and intensity. The major cues that are thus referred to as being vital for binaural hearing are: interaural differences in time (ITDs) and interaural differences in levels (ILDs). In children with normal hearing (NH), spatial hearing abilities are fairly well developed by age 4-5 years. In contrast, children who are deaf and hear through cochlear implants (CIs) do not have an opportunity to experience normal, binaural acoustic hearing early in life. These children may function by having to utilize auditory cues that are degraded with regard to numerous stimulus features. In recent years there has been a notable increase in the number of children receiving bilateral CIs, and evidence suggests that while having two CIs helps them function better than when listening through a single CI, they generally perform worse than their NH peers. This paper reviews some of the recent work on bilaterally implanted children. The focus is on measures of spatial hearing, including sound localization, release from masking for speech understanding in noise and binaural sensitivity using research processors. Data from behavioral and electrophysiological studies are included, with a focus on the recent work of the authors and their collaborators. The effects of auditory plasticity and deprivation on the emergence of binaural and spatial hearing are discussed along with evidence for reorganized processing from both behavioral and electrophysiological studies. The consequences of both unilateral and bilateral auditory deprivation during development suggest that the relevant set of issues is highly complex with regard to successes and the limitations experienced by children receiving bilateral cochlear implants.



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Bilateral cochlear implants in children: Effects of auditory experience and deprivation on auditory perception

Publication date: Available online 30 January 2016
Source:Hearing Research
Author(s): Ruth Y. Litovsky, Karen Gordon
Spatial hearing skills are essential for children as they grow, learn and play. They provide critical cues for determining the locations of sources in the environment, and enable segregation of important sources, such as speech, from background maskers or interferers. Spatial hearing depends on availability of monaural cues and binaural cues. The latter result from integration of inputs arriving at the two ears from sounds that vary in location. The binaural system has exquisite mechanisms for capturing differences between the ears in both time of arrival and intensity. The major cues that are thus referred to as being vital for binaural hearing are: interaural differences in time (ITDs) and interaural differences in levels (ILDs). In children with normal hearing (NH), spatial hearing abilities are fairly well developed by age 4-5 years. In contrast, children who are deaf and hear through cochlear implants (CIs) do not have an opportunity to experience normal, binaural acoustic hearing early in life. These children may function by having to utilize auditory cues that are degraded with regard to numerous stimulus features. In recent years there has been a notable increase in the number of children receiving bilateral CIs, and evidence suggests that while having two CIs helps them function better than when listening through a single CI, they generally perform worse than their NH peers. This paper reviews some of the recent work on bilaterally implanted children. The focus is on measures of spatial hearing, including sound localization, release from masking for speech understanding in noise and binaural sensitivity using research processors. Data from behavioral and electrophysiological studies are included, with a focus on the recent work of the authors and their collaborators. The effects of auditory plasticity and deprivation on the emergence of binaural and spatial hearing are discussed along with evidence for reorganized processing from both behavioral and electrophysiological studies. The consequences of both unilateral and bilateral auditory deprivation during development suggest that the relevant set of issues is highly complex with regard to successes and the limitations experienced by children receiving bilateral cochlear implants.



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Bilateral cochlear implants in children: Effects of auditory experience and deprivation on auditory perception

Publication date: Available online 30 January 2016
Source:Hearing Research
Author(s): Ruth Y. Litovsky, Karen Gordon
Spatial hearing skills are essential for children as they grow, learn and play. They provide critical cues for determining the locations of sources in the environment, and enable segregation of important sources, such as speech, from background maskers or interferers. Spatial hearing depends on availability of monaural cues and binaural cues. The latter result from integration of inputs arriving at the two ears from sounds that vary in location. The binaural system has exquisite mechanisms for capturing differences between the ears in both time of arrival and intensity. The major cues that are thus referred to as being vital for binaural hearing are: interaural differences in time (ITDs) and interaural differences in levels (ILDs). In children with normal hearing (NH), spatial hearing abilities are fairly well developed by age 4-5 years. In contrast, children who are deaf and hear through cochlear implants (CIs) do not have an opportunity to experience normal, binaural acoustic hearing early in life. These children may function by having to utilize auditory cues that are degraded with regard to numerous stimulus features. In recent years there has been a notable increase in the number of children receiving bilateral CIs, and evidence suggests that while having two CIs helps them function better than when listening through a single CI, they generally perform worse than their NH peers. This paper reviews some of the recent work on bilaterally implanted children. The focus is on measures of spatial hearing, including sound localization, release from masking for speech understanding in noise and binaural sensitivity using research processors. Data from behavioral and electrophysiological studies are included, with a focus on the recent work of the authors and their collaborators. The effects of auditory plasticity and deprivation on the emergence of binaural and spatial hearing are discussed along with evidence for reorganized processing from both behavioral and electrophysiological studies. The consequences of both unilateral and bilateral auditory deprivation during development suggest that the relevant set of issues is highly complex with regard to successes and the limitations experienced by children receiving bilateral cochlear implants.



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Bilateral cochlear implants in children: Effects of auditory experience and deprivation on auditory perception

Publication date: Available online 30 January 2016
Source:Hearing Research
Author(s): Ruth Y. Litovsky, Karen Gordon
Spatial hearing skills are essential for children as they grow, learn and play. They provide critical cues for determining the locations of sources in the environment, and enable segregation of important sources, such as speech, from background maskers or interferers. Spatial hearing depends on availability of monaural cues and binaural cues. The latter result from integration of inputs arriving at the two ears from sounds that vary in location. The binaural system has exquisite mechanisms for capturing differences between the ears in both time of arrival and intensity. The major cues that are thus referred to as being vital for binaural hearing are: interaural differences in time (ITDs) and interaural differences in levels (ILDs). In children with normal hearing (NH), spatial hearing abilities are fairly well developed by age 4-5 years. In contrast, children who are deaf and hear through cochlear implants (CIs) do not have an opportunity to experience normal, binaural acoustic hearing early in life. These children may function by having to utilize auditory cues that are degraded with regard to numerous stimulus features. In recent years there has been a notable increase in the number of children receiving bilateral CIs, and evidence suggests that while having two CIs helps them function better than when listening through a single CI, they generally perform worse than their NH peers. This paper reviews some of the recent work on bilaterally implanted children. The focus is on measures of spatial hearing, including sound localization, release from masking for speech understanding in noise and binaural sensitivity using research processors. Data from behavioral and electrophysiological studies are included, with a focus on the recent work of the authors and their collaborators. The effects of auditory plasticity and deprivation on the emergence of binaural and spatial hearing are discussed along with evidence for reorganized processing from both behavioral and electrophysiological studies. The consequences of both unilateral and bilateral auditory deprivation during development suggest that the relevant set of issues is highly complex with regard to successes and the limitations experienced by children receiving bilateral cochlear implants.



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Differential effects of suppressors on hazardous sound pressure levels generated by AR-15 rifles: Considerations for recreational shooters, law enforcement, and the military.

Differential effects of suppressors on hazardous sound pressure levels generated by AR-15 rifles: Considerations for recreational shooters, law enforcement, and the military.

Int J Audiol. 2016 Jan 28;:1-13

Authors: Lobarinas E, Scott R, Spankovich C, Le Prell CG

Abstract
OBJECTIVE: Firearm discharges produce hazardous levels of impulse noise that can lead to permanent hearing loss. In the present study, we evaluated the effects of suppression, ammunition, and barrel length on AR-15 rifles.
DESIGN: Sound levels were measured left/right of a user's head, and 1-m left of the muzzle, per MIL-STD-1474-D, under both unsuppressed and suppressed conditions.
STUDY SAMPLE: Nine commercially available AR-15 rifles and 14 suppressors were used.
RESULTS: Suppressors significantly decreased peak dB SPL at the 1-m location and the left ear location. However, under most rifle/ammunition conditions, levels remained above 140 dB peak SPL near a user's right ear. In a subset of conditions, subsonic ammunition produced values near or below 140 dB peak SPL. Overall suppression ranged from 7-32 dB across conditions.
CONCLUSIONS: These data indicate that (1) suppressors reduce discharge levels to 140 dB peak SPL or below in only a subset of AR-15 conditions, (2) shorter barrel length and use of muzzle brake devices can substantially increase exposure level for the user, and (3) there are significant left/right ear sound pressure differences under suppressed conditions as a function of the AR-15 direct impingement design that must be considered during sound measurements to fully evaluate overall efficacy.

PMID: 26821935 [PubMed - as supplied by publisher]

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Effects of low speed wind on the recognition/identification and pass-through communication tasks of auditory situation awareness afforded by military hearing protection/enhancement devices and tactical communication and protective systems.

Effects of low speed wind on the recognition/identification and pass-through communication tasks of auditory situation awareness afforded by military hearing protection/enhancement devices and tactical communication and protective systems.

Int J Audiol. 2016 Jan 28;:1-9

Authors: Lee K, Casali JG

Abstract
OBJECTIVE: To investigate the effect of controlled low-speed wind-noise on the auditory situation awareness performance afforded by military hearing protection/enhancement devices (HPED) and tactical communication and protective systems (TCAPS).
DESIGN: Recognition/identification and pass-through communications tasks were separately conducted under three wind conditions (0, 5, and 10 mph). Subjects wore two in-ear-type TCAPS, one earmuff-type TCAPS, a Combat Arms Earplug in its 'open' or pass-through setting, and an EB-15LE™ electronic earplug. Devices with electronic gain systems were tested under two gain settings: 'unity' and 'max'. Testing without any device (open ear) was conducted as a control.
STUDY SAMPLE: Ten subjects were recruited from the student population at Virginia Tech. Audiometric requirements were 25 dBHL or better at 500, 1000, 2000, 4000, and 8000 Hz in both ears.
RESULTS: Performance on the interaction of communication task-by-device was significantly different only in 0 mph wind speed. The between-device performance differences varied with azimuthal speaker locations.
CONCLUSIONS: It is evident from this study that stable (non-gusting) wind speeds up to 10 mph did not significantly degrade recognition/identification task performance and pass-through communication performance of the group of HPEDs and TCAPS tested. However, the various devices performed differently as the test sound signal speaker location was varied and it appears that physical as well as electronic features may have contributed to this directional result.

PMID: 26821823 [PubMed - as supplied by publisher]

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Differential effects of suppressors on hazardous sound pressure levels generated by AR-15 rifles: Considerations for recreational shooters, law enforcement, and the military.

Differential effects of suppressors on hazardous sound pressure levels generated by AR-15 rifles: Considerations for recreational shooters, law enforcement, and the military.

Int J Audiol. 2016 Jan 28;:1-13

Authors: Lobarinas E, Scott R, Spankovich C, Le Prell CG

Abstract
OBJECTIVE: Firearm discharges produce hazardous levels of impulse noise that can lead to permanent hearing loss. In the present study, we evaluated the effects of suppression, ammunition, and barrel length on AR-15 rifles.
DESIGN: Sound levels were measured left/right of a user's head, and 1-m left of the muzzle, per MIL-STD-1474-D, under both unsuppressed and suppressed conditions.
STUDY SAMPLE: Nine commercially available AR-15 rifles and 14 suppressors were used.
RESULTS: Suppressors significantly decreased peak dB SPL at the 1-m location and the left ear location. However, under most rifle/ammunition conditions, levels remained above 140 dB peak SPL near a user's right ear. In a subset of conditions, subsonic ammunition produced values near or below 140 dB peak SPL. Overall suppression ranged from 7-32 dB across conditions.
CONCLUSIONS: These data indicate that (1) suppressors reduce discharge levels to 140 dB peak SPL or below in only a subset of AR-15 conditions, (2) shorter barrel length and use of muzzle brake devices can substantially increase exposure level for the user, and (3) there are significant left/right ear sound pressure differences under suppressed conditions as a function of the AR-15 direct impingement design that must be considered during sound measurements to fully evaluate overall efficacy.

PMID: 26821935 [PubMed - as supplied by publisher]

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Effects of low speed wind on the recognition/identification and pass-through communication tasks of auditory situation awareness afforded by military hearing protection/enhancement devices and tactical communication and protective systems.

Effects of low speed wind on the recognition/identification and pass-through communication tasks of auditory situation awareness afforded by military hearing protection/enhancement devices and tactical communication and protective systems.

Int J Audiol. 2016 Jan 28;:1-9

Authors: Lee K, Casali JG

Abstract
OBJECTIVE: To investigate the effect of controlled low-speed wind-noise on the auditory situation awareness performance afforded by military hearing protection/enhancement devices (HPED) and tactical communication and protective systems (TCAPS).
DESIGN: Recognition/identification and pass-through communications tasks were separately conducted under three wind conditions (0, 5, and 10 mph). Subjects wore two in-ear-type TCAPS, one earmuff-type TCAPS, a Combat Arms Earplug in its 'open' or pass-through setting, and an EB-15LE™ electronic earplug. Devices with electronic gain systems were tested under two gain settings: 'unity' and 'max'. Testing without any device (open ear) was conducted as a control.
STUDY SAMPLE: Ten subjects were recruited from the student population at Virginia Tech. Audiometric requirements were 25 dBHL or better at 500, 1000, 2000, 4000, and 8000 Hz in both ears.
RESULTS: Performance on the interaction of communication task-by-device was significantly different only in 0 mph wind speed. The between-device performance differences varied with azimuthal speaker locations.
CONCLUSIONS: It is evident from this study that stable (non-gusting) wind speeds up to 10 mph did not significantly degrade recognition/identification task performance and pass-through communication performance of the group of HPEDs and TCAPS tested. However, the various devices performed differently as the test sound signal speaker location was varied and it appears that physical as well as electronic features may have contributed to this directional result.

PMID: 26821823 [PubMed - as supplied by publisher]

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Differential effects of suppressors on hazardous sound pressure levels generated by AR-15 rifles: Considerations for recreational shooters, law enforcement, and the military.

Differential effects of suppressors on hazardous sound pressure levels generated by AR-15 rifles: Considerations for recreational shooters, law enforcement, and the military.

Int J Audiol. 2016 Jan 28;:1-13

Authors: Lobarinas E, Scott R, Spankovich C, Le Prell CG

Abstract
OBJECTIVE: Firearm discharges produce hazardous levels of impulse noise that can lead to permanent hearing loss. In the present study, we evaluated the effects of suppression, ammunition, and barrel length on AR-15 rifles.
DESIGN: Sound levels were measured left/right of a user's head, and 1-m left of the muzzle, per MIL-STD-1474-D, under both unsuppressed and suppressed conditions.
STUDY SAMPLE: Nine commercially available AR-15 rifles and 14 suppressors were used.
RESULTS: Suppressors significantly decreased peak dB SPL at the 1-m location and the left ear location. However, under most rifle/ammunition conditions, levels remained above 140 dB peak SPL near a user's right ear. In a subset of conditions, subsonic ammunition produced values near or below 140 dB peak SPL. Overall suppression ranged from 7-32 dB across conditions.
CONCLUSIONS: These data indicate that (1) suppressors reduce discharge levels to 140 dB peak SPL or below in only a subset of AR-15 conditions, (2) shorter barrel length and use of muzzle brake devices can substantially increase exposure level for the user, and (3) there are significant left/right ear sound pressure differences under suppressed conditions as a function of the AR-15 direct impingement design that must be considered during sound measurements to fully evaluate overall efficacy.

PMID: 26821935 [PubMed - as supplied by publisher]

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Effects of low speed wind on the recognition/identification and pass-through communication tasks of auditory situation awareness afforded by military hearing protection/enhancement devices and tactical communication and protective systems.

Effects of low speed wind on the recognition/identification and pass-through communication tasks of auditory situation awareness afforded by military hearing protection/enhancement devices and tactical communication and protective systems.

Int J Audiol. 2016 Jan 28;:1-9

Authors: Lee K, Casali JG

Abstract
OBJECTIVE: To investigate the effect of controlled low-speed wind-noise on the auditory situation awareness performance afforded by military hearing protection/enhancement devices (HPED) and tactical communication and protective systems (TCAPS).
DESIGN: Recognition/identification and pass-through communications tasks were separately conducted under three wind conditions (0, 5, and 10 mph). Subjects wore two in-ear-type TCAPS, one earmuff-type TCAPS, a Combat Arms Earplug in its 'open' or pass-through setting, and an EB-15LE™ electronic earplug. Devices with electronic gain systems were tested under two gain settings: 'unity' and 'max'. Testing without any device (open ear) was conducted as a control.
STUDY SAMPLE: Ten subjects were recruited from the student population at Virginia Tech. Audiometric requirements were 25 dBHL or better at 500, 1000, 2000, 4000, and 8000 Hz in both ears.
RESULTS: Performance on the interaction of communication task-by-device was significantly different only in 0 mph wind speed. The between-device performance differences varied with azimuthal speaker locations.
CONCLUSIONS: It is evident from this study that stable (non-gusting) wind speeds up to 10 mph did not significantly degrade recognition/identification task performance and pass-through communication performance of the group of HPEDs and TCAPS tested. However, the various devices performed differently as the test sound signal speaker location was varied and it appears that physical as well as electronic features may have contributed to this directional result.

PMID: 26821823 [PubMed - as supplied by publisher]

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Differential effects of suppressors on hazardous sound pressure levels generated by AR-15 rifles: Considerations for recreational shooters, law enforcement, and the military.

Differential effects of suppressors on hazardous sound pressure levels generated by AR-15 rifles: Considerations for recreational shooters, law enforcement, and the military.

Int J Audiol. 2016 Jan 28;:1-13

Authors: Lobarinas E, Scott R, Spankovich C, Le Prell CG

Abstract
OBJECTIVE: Firearm discharges produce hazardous levels of impulse noise that can lead to permanent hearing loss. In the present study, we evaluated the effects of suppression, ammunition, and barrel length on AR-15 rifles.
DESIGN: Sound levels were measured left/right of a user's head, and 1-m left of the muzzle, per MIL-STD-1474-D, under both unsuppressed and suppressed conditions.
STUDY SAMPLE: Nine commercially available AR-15 rifles and 14 suppressors were used.
RESULTS: Suppressors significantly decreased peak dB SPL at the 1-m location and the left ear location. However, under most rifle/ammunition conditions, levels remained above 140 dB peak SPL near a user's right ear. In a subset of conditions, subsonic ammunition produced values near or below 140 dB peak SPL. Overall suppression ranged from 7-32 dB across conditions.
CONCLUSIONS: These data indicate that (1) suppressors reduce discharge levels to 140 dB peak SPL or below in only a subset of AR-15 conditions, (2) shorter barrel length and use of muzzle brake devices can substantially increase exposure level for the user, and (3) there are significant left/right ear sound pressure differences under suppressed conditions as a function of the AR-15 direct impingement design that must be considered during sound measurements to fully evaluate overall efficacy.

PMID: 26821935 [PubMed - as supplied by publisher]

from #Audiology via ola Kala on Inoreader http://ift.tt/1Vx4IjH
via IFTTT

Effects of low speed wind on the recognition/identification and pass-through communication tasks of auditory situation awareness afforded by military hearing protection/enhancement devices and tactical communication and protective systems.

Effects of low speed wind on the recognition/identification and pass-through communication tasks of auditory situation awareness afforded by military hearing protection/enhancement devices and tactical communication and protective systems.

Int J Audiol. 2016 Jan 28;:1-9

Authors: Lee K, Casali JG

Abstract
OBJECTIVE: To investigate the effect of controlled low-speed wind-noise on the auditory situation awareness performance afforded by military hearing protection/enhancement devices (HPED) and tactical communication and protective systems (TCAPS).
DESIGN: Recognition/identification and pass-through communications tasks were separately conducted under three wind conditions (0, 5, and 10 mph). Subjects wore two in-ear-type TCAPS, one earmuff-type TCAPS, a Combat Arms Earplug in its 'open' or pass-through setting, and an EB-15LE™ electronic earplug. Devices with electronic gain systems were tested under two gain settings: 'unity' and 'max'. Testing without any device (open ear) was conducted as a control.
STUDY SAMPLE: Ten subjects were recruited from the student population at Virginia Tech. Audiometric requirements were 25 dBHL or better at 500, 1000, 2000, 4000, and 8000 Hz in both ears.
RESULTS: Performance on the interaction of communication task-by-device was significantly different only in 0 mph wind speed. The between-device performance differences varied with azimuthal speaker locations.
CONCLUSIONS: It is evident from this study that stable (non-gusting) wind speeds up to 10 mph did not significantly degrade recognition/identification task performance and pass-through communication performance of the group of HPEDs and TCAPS tested. However, the various devices performed differently as the test sound signal speaker location was varied and it appears that physical as well as electronic features may have contributed to this directional result.

PMID: 26821823 [PubMed - as supplied by publisher]

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The auditory phenotype of children harboring mutations in the prestin gene.

The auditory phenotype of children harboring mutations in the prestin gene.

Acta Otolaryngol. 2016 Jan 29;:1-5

Authors: Matsunaga T, Morimoto N

Abstract
Conclusion Auditory phenotypes of two children harboring prestin gene mutations were congenital or pre-lingual onset, moderate to profound, slowly progressive or non-progressive, and audiograms with either flat configuration or prominently elevated thresholds at middle and high frequencies. Objectives Despite the essential role of the prestin gene in hearing, only one mutation in two families and a missense variant in a family had been reported previously before our study reporting another family. The purpose of this study was to characterize auditory phenotypes in children recently found to harbor novel mutations in the prestin gene. Methods The subjects were two sisters with bilateral sensorineural hearing loss who were compound heterozygotes for c.209G > A (p.W70X) and c.390A > C (p.R130S) mutations in the prestin gene. Clinical history and auditory test results were collected and analyzed. Results Hearing loss was present from birth in the younger sister and occurred before 6 years of age in the elder sister. The degree of hearing loss was profound in the elder sister with little progression, and moderate in the younger sister with no progression. The audiogram of the elder sister showed prominently elevated thresholds at middle and high frequencies, while that of the younger sister demonstrated a flat configuration.

PMID: 26824437 [PubMed - as supplied by publisher]

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Exome sequencing identifies a mutation in TMC1 as a novel cause of autosomal recessive nonsyndromic hearing loss.

Exome sequencing identifies a mutation in TMC1 as a novel cause of autosomal recessive nonsyndromic hearing loss.

J Transl Med. 2016;14(1):29

Authors: Hu J, Liu F, Xia W, Hao L, Lan J, Zhu Z, Ye J, Ma D, Ma Z

Abstract
BACKGROUND: Autosomal recessive non-syndromic hearing loss (ARNSHL) is highly heterogeneous, and mutations in the gene encoding transmembrane channel-like 1 (TMC1) have been implicated in its development. To date, 35 homozygous mutations in TMC1, identified in over 60 families worldwide, have been shown to be associated with ARNSHL. However, few of these mutations were detected in the Chinese population. In this study, we describe a pathogenic missense mutation located in the T5-T6 domain of TMC1 in a three-generation Chinese family with 14 members.
METHODS: Whole exome sequencing was performed using samples from one unaffected individual and two affected individuals to systematically search for deafness susceptibility genes. Candidate mutations and cosegregation of the phenotype were verified by polymerase chain reaction and Sanger sequencing in all of the family members.
RESULTS: We identified a novel TMC1 mutation in exon 20, c.1979C>T, p.P660L, which segregated with prelingual autosomal recessive sensorineural hearing loss.
CONCLUSIONS: We found a new missense mutation in the T5-T6 domain of TMC1, which is highly conserved in many species. These data support the potential conserved role of p.P660L in human TMC1 function.

PMID: 26822030 [PubMed - in process]

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Cochlear Implantation in a Patient With Pfeiffer Syndrome and Temporal Bone Vascular Anomalies.

Objective: Pfeiffer syndrome is a rare craniosynostotic disorder resulting in premature bony fusion of the skull, which can result in abnormal temporal bone and vascular anatomy and hearing loss. Cochlear implantation in these patients requires thoughtful surgical planning given the potential for limited access to the cochlea because of subcutaneous or intratemporal vasculature. Herein, we present a patient with Pfeiffer syndrome who underwent successful cochlear implantation using preoperative Doppler ultrasound to identify large extracranial venous anatomy followed by a modified transcanal surgical approach. Patients: An adult female patient with Pfeiffer syndrome. Intervention(s): Preoperative axial computed tomography, catheter angiography, Doppler ultrasound, and right side cochlear implant. Main Outcome Measure(s): Surgical feasibility and audiometric outcomes including aided thresholds and word recognition score. Results: Successful cochlear implantation was performed via a modified transcanal approach with blind sac closure of the external auditory meatus. Full electrode insertion was obtained through a round window approach. No extracranial or intratemporal vessels were encountered during surgery. Conclusion: Patients with significant craniosynostoses and vascular malformations of the temporal bone can undergo successful cochlear implantation. Careful preoperative planning with high-resolution CT, MRA, and MRV, and/or traditional catheter angiography can assist in determining surgical feasibility and minimizing risk. We recommend preoperative Doppler ultrasound of abnormal extracranial vessels to assist in safe placement of incisions and hardware. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Hearing Preservation Outcomes With a Mid-Scala Electrode in Cochlear Implantation.

Objective: To evaluate hearing preservation (HP) outcomes in adult cochlear implant recipients with a mid-scala electrode. Setting: Tertiary academic center. Patients: Adult patients implanted with a mid-scala electrode between May 2013 and July 2015. Interventions: Cochlear implantation. Main Outcome Measure(s): Age, sex, surgical approach, residual hearing changes post cochlear implantation, HP rates using different published classifications, and speech perception scores. Results: Fifty ears for 47 patients (mean age, 58.2 yr; range, 23-86) were implanted with the electrode. Recognizing that not all patients were true HP candidates and/or underwent generally accepted HP surgical techniques, 39 ears had preoperative low-frequency hearing (audiometric threshold 0.05). Conclusion: The mid-scala electrode evaluated allows preservation of low-frequency hearing in patients undergoing cochlear implantation at rates and degrees of preservation close to other reports in the cochlear implant literature. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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A Comparative Review of Osseointegration Failure Between Osseointegrated Bone Conduction Device Models in Pediatric Patients.

Objective: Compare rates of osseointegration failure (OIF) between system 2 (200 series) and system 3 (BI300) osseointegrated bone conduction device (OCBD) models. Study Design: Case series with chart review. Setting: Tertiary referral center. Patients: Pediatric patients who were implanted with either system 2 or system 3 OCBD between May 2009 and July 2014 at Arkansas Children's Hospital. Interventions: Implantation with either system 2 or system 3 OCBD using 3- and 4-mm implants with 5.5- and 8.5-mm abutments (system 2) or 6- and 9-mm abutments (system 3). Main Outcome Measures: OIF with resulting loss of flange-fixture. Results: OIF occurred in 28.6% (14/49) of implanted fixtures in system 2 compared with 8.0% (2/25) of implanted fixtures in system 3 (p = 0.042). The average time to complication for system 2 was 13 months compared with 3 months for system 3. The causes of OIF in system 2 were trauma (n = 4), chronic soft tissue disease (n = 5), or idiopathic in nature (n = 5). System 3 failures were both because of trauma. Using the combined cohorts, the average age of patients who experienced OIF was 8.7 years, compared with 11.4 years in patients without OIF (p = 0.047). There was no difference in OIF among 3-mm and 4-mm screws (p = 0.876), linear and flap technique (p = 0.375), or surgeons (p = 0.211). Conclusion: System 3 OCBD showed a significantly reduced rate of OIF compared with system 2 in our study. Modification to the system 2 implant resulted in improved rate of OIF. Younger age was independently associated with a higher rate of OIF when evaluating both systems. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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Impaired Perception of Sensory Consonance and Dissonance in Cochlear Implant Users.

Hypothesis: In light of previous research demonstrating poor pitch perception in cochlear implant (CI) users, we hypothesized that the presence of consonant versus dissonant chord accompaniment in real-world musical stimuli would not impact subjective assessment of degree of pleasantness in CI users. Background: Consonance/dissonance are perceptual features of harmony resulting from pitch relationships between simultaneously presented musical notes. Generally, consonant sounds are perceived as pleasant and dissonant ones as unpleasant. CI users exhibit impairments in pitch perception, making music listening difficult and often unenjoyable. To our knowledge, consonance/dissonance perception has not been studied in the CI population. Methods: Twelve novel melodies were created for this study. By altering the harmonic structures of the accompanying chords, we created three permutations of varying dissonance for each melody (36 stimuli in all). Ten CI users and 12 NH listeners provided Likert scale ratings from -5 (very unpleasant) to +5 (very pleasant) for each of the stimuli. Results: A two-way ANOVA showed main effects for Dissonance Level and Subject Type as well as a two-way interaction between the two. Pairwise comparisons indicated that NH stimuli pleasantness ratings decreased with increasing dissonance, whereas CI ratings did not. NH pleasantness ratings were consistently lower than CI ratings. Conclusion: For CI users, consonant versus dissonant chord accompaniment had no significant impact on whether a melody was considered pleasant or unpleasant. This finding may be partially responsible for the decreased enjoyment of many CI users during music perception and is another manifestation of impaired pitch perception in CI users. Copyright (C) 2016 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company

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