HUWE1 mutations in Juberg-Marsidi and Brooks syndromes: the results of an X-chromosome exome sequencing study.

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HUWE1 mutations in Juberg-Marsidi and Brooks syndromes: the results of an X-chromosome exome sequencing study.

BMJ Open. 2016 Apr 29;6(4):e009537

Authors: Friez MJ, Brooks SS, Stevenson RE, Field M, Basehore MJ, Adès LC, Sebold C, McGee S, Saxon S, Skinner C, Craig ME, Murray L, Simensen RJ, Yap YY, Shaw MA, Gardner A, Corbett M, Kumar R, Bosshard M, van Loon B, Tarpey PS, Abidi F, Gecz J, Schwartz CE

Abstract
BACKGROUND: X linked intellectual disability (XLID) syndromes account for a substantial number of males with ID. Much progress has been made in identifying the genetic cause in many of the syndromes described 20-40 years ago. Next generation sequencing (NGS) has contributed to the rapid discovery of XLID genes and identifying novel mutations in known XLID genes for many of these syndromes.
METHODS: 2 NGS approaches were employed to identify mutations in X linked genes in families with XLID disorders. 1 involved exome sequencing of genes on the X chromosome using the Agilent SureSelect Human X Chromosome Kit. The second approach was to conduct targeted NGS sequencing of 90 known XLID genes.
RESULTS: We identified the same mutation, a c.12928 G>C transversion in the HUWE1 gene, which gives rise to a p.G4310R missense mutation in 2 XLID disorders: Juberg-Marsidi syndrome (JMS) and Brooks syndrome. Although the original families with these disorders were considered separate entities, they indeed overlap clinically. A third family was also found to have a novel HUWE1 mutation.
CONCLUSIONS: As we identified a HUWE1 mutation in an affected male from the original family reported by Juberg and Marsidi, it is evident the syndrome does not result from a mutation in ATRX as reported in the literature. Additionally, our data indicate that JMS and Brooks syndromes are allelic having the same HUWE1 mutation.

PMID: 27130160 [PubMed - indexed for MEDLINE]

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A narrative review of texting as a visually-dependent cognitive-motor secondary task during locomotion

Publication date: February 2017
Source:Gait & Posture, Volume 52
Author(s): T. Krasovsky, P.L. Weiss, R. Kizony
Typing while walking is an example of people’s ability to interact with technology while engaged in real life activities. Indeed, an increasing number of studies have investigated the typing of text messages (texting) as a dual task during locomotion. The objective of this review is to (1) describe the task requirements of texting-while-walking, (2) evaluate the measurement and psychometric properties of texting as a dual task, and (3) formulate methodological recommendations for researchers who use and report on texting-while-walking. Twenty studies which used texting as a dual task during gait were identified via a literature search. The majority of these studies examined texting among young healthy adults and showed that, like other dual tasks, texting-while-walking caused decrements in both gait and texting performance. The cause of these decrements was most likely related to increased visual task requirements, task-dependent cognitive requirements and fine motor skills. Texting-while-walking gait measures were repeatable, but texting performance showed poor reliability which further depended on skill. Preliminary results show that texting-while-walking performance may discriminate between populations (e.g., young vs. older adults) but no studies have yet examined its predictive validity (e.g., for fall risk). In conclusion, texting-while-walking is an ecologically-valid dual task for locomotion which has become much more commonly used in recent years. As opposed to other secondary tasks such as subtraction by 7 or generating words, texting may challenge various cognitive, visual and sensorimotor domains depending on its content. This imposes task-specific methodological challenges on future research, which are discussed.



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Dynamic plantar pressure distribution, strength capacity and postural control after Lisfranc fracture-dislocation

Publication date: February 2017
Source:Gait & Posture, Volume 52
Author(s): Alexander T. Mehlhorn, Markus Walther, Tayfun Yilmaz, Lennart Gunst, Anja Hirschmüller, Norbert P. Südkamp, Hagen Schmal
Substantial progress has been made in the operative treatment of Lisfranc fractures, however, the prognosis remains poor. We hypothesized that Lisfranc injuries change the postural control and muscle strength of the lower limb. Both are suggested to correlate with the clinical outcome and quality of life.17 consecutive patients suffering from a Lisfranc fracture dislocation were registered, underwent open reduction and internal fixation and were followed-up for 50.5±25.7months (Mean±SDM). Biomechanical analysis of muscle strength capacities, postural control and plantar pressure distribution was assessed >6 month postoperatively. Results were correlated to clinical outcome (AOFAS, FFI, Pain, SF-36).The isokinetic assessment revealed a significant reduction in plantar flexor and dorsal extensor peak torque of the injured limb compared to the uninjured limb. The dorsal extensor peak torque thereby correlated well with clinical outcome. Altered postural control was evident by a significant reduction in unilateral stance time, from which we calculated a strong correlation between stance time and the isokinetic strength measurement. Plantar pressure measurements revealed a significant reduction in peak pressure under the midfoot and of Force-Time Integral beneath the second metatarsal.Sufficient rehabilitation is crucial to the clinical outcome following anatomical open reduction of Lisfranc fracture-dislocation. The present study supports a rehabilitation approach focussing on restoring proprioception and calf muscular strength including isometric exercises of the dorsal extensors.



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Hip mechanics underlie lower extremity power training-induced increase in old adults’ fast gait velocity: The Potsdam Gait Study (POGS)

Publication date: February 2017
Source:Gait & Posture, Volume 52
Author(s): Chantal M.I. Beijersbergen, Urs Granacher, Martijn Gäbler, Paul DeVita, Tibor Hortobágyi
BackgroundAging is associated with slowed gait and old compared with young adults generally walk with greater positive hip work (H1) and reduced positive ankle work (A2). The role of exercise interventions on old adults’ gait mechanics that underlie training-induced improvements in gait velocity is unclear. We examined the effects of lower extremity power training and detraining on old adults’ gait kinetics.MethodsAs part of the Potsdam Gait Study (POGS), healthy old adults completed a no-intervention control period (69.1±4.4yrs, n=14) or a power training program followed by detraining (72.9±5.4yrs, n=15). We measured isokinetic knee extensor and plantarflexor power and measured hip, knee and ankle kinetics at habitual, fast and standardized walking speeds.ResultsPower training significantly increased isokinetic knee extensor power (25%), plantarflexor power (43%), and fast gait velocity (5.9%). Gait mechanics underlying the improved fast gait velocity included increases in hip angular impulse (29%) and H1 work (37%) and no changes in positive knee (K2) and A2 work. Detraining further improved fast gait velocity (4.7%) with reductions in H1 (−35%), and increases in K2 (36%) and A2 (7%).ConclusionPower training increased fast gait velocity in healthy old adults by increasing the reliance on hip muscle function and thus further strengthened the age-related distal-to-proximal shift in muscle function.



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A narrative review of texting as a visually-dependent cognitive-motor secondary task during locomotion

Publication date: February 2017
Source:Gait & Posture, Volume 52
Author(s): T. Krasovsky, P.L. Weiss, R. Kizony
Typing while walking is an example of people’s ability to interact with technology while engaged in real life activities. Indeed, an increasing number of studies have investigated the typing of text messages (texting) as a dual task during locomotion. The objective of this review is to (1) describe the task requirements of texting-while-walking, (2) evaluate the measurement and psychometric properties of texting as a dual task, and (3) formulate methodological recommendations for researchers who use and report on texting-while-walking. Twenty studies which used texting as a dual task during gait were identified via a literature search. The majority of these studies examined texting among young healthy adults and showed that, like other dual tasks, texting-while-walking caused decrements in both gait and texting performance. The cause of these decrements was most likely related to increased visual task requirements, task-dependent cognitive requirements and fine motor skills. Texting-while-walking gait measures were repeatable, but texting performance showed poor reliability which further depended on skill. Preliminary results show that texting-while-walking performance may discriminate between populations (e.g., young vs. older adults) but no studies have yet examined its predictive validity (e.g., for fall risk). In conclusion, texting-while-walking is an ecologically-valid dual task for locomotion which has become much more commonly used in recent years. As opposed to other secondary tasks such as subtraction by 7 or generating words, texting may challenge various cognitive, visual and sensorimotor domains depending on its content. This imposes task-specific methodological challenges on future research, which are discussed.



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Dynamic plantar pressure distribution, strength capacity and postural control after Lisfranc fracture-dislocation

Publication date: February 2017
Source:Gait & Posture, Volume 52
Author(s): Alexander T. Mehlhorn, Markus Walther, Tayfun Yilmaz, Lennart Gunst, Anja Hirschmüller, Norbert P. Südkamp, Hagen Schmal
Substantial progress has been made in the operative treatment of Lisfranc fractures, however, the prognosis remains poor. We hypothesized that Lisfranc injuries change the postural control and muscle strength of the lower limb. Both are suggested to correlate with the clinical outcome and quality of life.17 consecutive patients suffering from a Lisfranc fracture dislocation were registered, underwent open reduction and internal fixation and were followed-up for 50.5±25.7months (Mean±SDM). Biomechanical analysis of muscle strength capacities, postural control and plantar pressure distribution was assessed >6 month postoperatively. Results were correlated to clinical outcome (AOFAS, FFI, Pain, SF-36).The isokinetic assessment revealed a significant reduction in plantar flexor and dorsal extensor peak torque of the injured limb compared to the uninjured limb. The dorsal extensor peak torque thereby correlated well with clinical outcome. Altered postural control was evident by a significant reduction in unilateral stance time, from which we calculated a strong correlation between stance time and the isokinetic strength measurement. Plantar pressure measurements revealed a significant reduction in peak pressure under the midfoot and of Force-Time Integral beneath the second metatarsal.Sufficient rehabilitation is crucial to the clinical outcome following anatomical open reduction of Lisfranc fracture-dislocation. The present study supports a rehabilitation approach focussing on restoring proprioception and calf muscular strength including isometric exercises of the dorsal extensors.



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Hip mechanics underlie lower extremity power training-induced increase in old adults’ fast gait velocity: The Potsdam Gait Study (POGS)

Publication date: February 2017
Source:Gait & Posture, Volume 52
Author(s): Chantal M.I. Beijersbergen, Urs Granacher, Martijn Gäbler, Paul DeVita, Tibor Hortobágyi
BackgroundAging is associated with slowed gait and old compared with young adults generally walk with greater positive hip work (H1) and reduced positive ankle work (A2). The role of exercise interventions on old adults’ gait mechanics that underlie training-induced improvements in gait velocity is unclear. We examined the effects of lower extremity power training and detraining on old adults’ gait kinetics.MethodsAs part of the Potsdam Gait Study (POGS), healthy old adults completed a no-intervention control period (69.1±4.4yrs, n=14) or a power training program followed by detraining (72.9±5.4yrs, n=15). We measured isokinetic knee extensor and plantarflexor power and measured hip, knee and ankle kinetics at habitual, fast and standardized walking speeds.ResultsPower training significantly increased isokinetic knee extensor power (25%), plantarflexor power (43%), and fast gait velocity (5.9%). Gait mechanics underlying the improved fast gait velocity included increases in hip angular impulse (29%) and H1 work (37%) and no changes in positive knee (K2) and A2 work. Detraining further improved fast gait velocity (4.7%) with reductions in H1 (−35%), and increases in K2 (36%) and A2 (7%).ConclusionPower training increased fast gait velocity in healthy old adults by increasing the reliance on hip muscle function and thus further strengthened the age-related distal-to-proximal shift in muscle function.



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Balance Performance of Deaf Children With and Without Cochlear Implants.

Related Articles

Balance Performance of Deaf Children With and Without Cochlear Implants.

Acta Med Iran. 2016 Nov;54(11):737-742

Authors: Ebrahimi AA, Movallali G, Jamshidi AA, Haghgoo HA, Rahgozar M

Abstract
 The aim of this study was to compare the static and dynamic balance performance of deaf children with and without cochlear implants. This is a cross-sectional study of 145 school children, aged between 7 and 12 years comprising 85 children with congenital or early acquired bilateral profound sensorineural hearing loss (the hearing loss group) and 60 normal hearing aged-matched control counterparts were assessed using the balance subtest of Bruininks-Oseretsky test of Motor Proficiency (BOTMP). The hearing loss group, 50 without cochlear implants (the non-implant group) and 35 of them with unilateral cochlear implants (the implant group) were recruited from schools for the deaf and normal hearing children (the control group) randomly selected from two randomly selected elementary schools of Tehran city. The scores were analyzed using one-way ANOVA. The total score of deaf children especially the implant group were significantly lower than the control group )P<0.001). The balance performance of the control group was better than the implant group in all of the items as well as the non-implant group except the fourth tested item (walking forward on a line) (P<0.05). The balance score of the implant group was significantly lower than the non-implant group except for the third tested item (standing on the preferred leg on a balance beam with eyes closed). The findings suggested that deaf children, specifically those with cochlear implants are at risk for motor and balance deficits. Thus, vestibular and motor evaluations, as well as interventions to improve balance and motor skills, should be prioritized for this population.

PMID: 28033698 [PubMed - in process]

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A narrative review of texting as a visually-dependent cognitive-motor secondary task during locomotion

Publication date: February 2017
Source:Gait & Posture, Volume 52
Author(s): T. Krasovsky, P.L. Weiss, R. Kizony
Typing while walking is an example of people’s ability to interact with technology while engaged in real life activities. Indeed, an increasing number of studies have investigated the typing of text messages (texting) as a dual task during locomotion. The objective of this review is to (1) describe the task requirements of texting-while-walking, (2) evaluate the measurement and psychometric properties of texting as a dual task, and (3) formulate methodological recommendations for researchers who use and report on texting-while-walking. Twenty studies which used texting as a dual task during gait were identified via a literature search. The majority of these studies examined texting among young healthy adults and showed that, like other dual tasks, texting-while-walking caused decrements in both gait and texting performance. The cause of these decrements was most likely related to increased visual task requirements, task-dependent cognitive requirements and fine motor skills. Texting-while-walking gait measures were repeatable, but texting performance showed poor reliability which further depended on skill. Preliminary results show that texting-while-walking performance may discriminate between populations (e.g., young vs. older adults) but no studies have yet examined its predictive validity (e.g., for fall risk). In conclusion, texting-while-walking is an ecologically-valid dual task for locomotion which has become much more commonly used in recent years. As opposed to other secondary tasks such as subtraction by 7 or generating words, texting may challenge various cognitive, visual and sensorimotor domains depending on its content. This imposes task-specific methodological challenges on future research, which are discussed.



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Dynamic plantar pressure distribution, strength capacity and postural control after Lisfranc fracture-dislocation

Publication date: February 2017
Source:Gait & Posture, Volume 52
Author(s): Alexander T. Mehlhorn, Markus Walther, Tayfun Yilmaz, Lennart Gunst, Anja Hirschmüller, Norbert P. Südkamp, Hagen Schmal
Substantial progress has been made in the operative treatment of Lisfranc fractures, however, the prognosis remains poor. We hypothesized that Lisfranc injuries change the postural control and muscle strength of the lower limb. Both are suggested to correlate with the clinical outcome and quality of life.17 consecutive patients suffering from a Lisfranc fracture dislocation were registered, underwent open reduction and internal fixation and were followed-up for 50.5±25.7months (Mean±SDM). Biomechanical analysis of muscle strength capacities, postural control and plantar pressure distribution was assessed >6 month postoperatively. Results were correlated to clinical outcome (AOFAS, FFI, Pain, SF-36).The isokinetic assessment revealed a significant reduction in plantar flexor and dorsal extensor peak torque of the injured limb compared to the uninjured limb. The dorsal extensor peak torque thereby correlated well with clinical outcome. Altered postural control was evident by a significant reduction in unilateral stance time, from which we calculated a strong correlation between stance time and the isokinetic strength measurement. Plantar pressure measurements revealed a significant reduction in peak pressure under the midfoot and of Force-Time Integral beneath the second metatarsal.Sufficient rehabilitation is crucial to the clinical outcome following anatomical open reduction of Lisfranc fracture-dislocation. The present study supports a rehabilitation approach focussing on restoring proprioception and calf muscular strength including isometric exercises of the dorsal extensors.



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Hip mechanics underlie lower extremity power training-induced increase in old adults’ fast gait velocity: The Potsdam Gait Study (POGS)

Publication date: February 2017
Source:Gait & Posture, Volume 52
Author(s): Chantal M.I. Beijersbergen, Urs Granacher, Martijn Gäbler, Paul DeVita, Tibor Hortobágyi
BackgroundAging is associated with slowed gait and old compared with young adults generally walk with greater positive hip work (H1) and reduced positive ankle work (A2). The role of exercise interventions on old adults’ gait mechanics that underlie training-induced improvements in gait velocity is unclear. We examined the effects of lower extremity power training and detraining on old adults’ gait kinetics.MethodsAs part of the Potsdam Gait Study (POGS), healthy old adults completed a no-intervention control period (69.1±4.4yrs, n=14) or a power training program followed by detraining (72.9±5.4yrs, n=15). We measured isokinetic knee extensor and plantarflexor power and measured hip, knee and ankle kinetics at habitual, fast and standardized walking speeds.ResultsPower training significantly increased isokinetic knee extensor power (25%), plantarflexor power (43%), and fast gait velocity (5.9%). Gait mechanics underlying the improved fast gait velocity included increases in hip angular impulse (29%) and H1 work (37%) and no changes in positive knee (K2) and A2 work. Detraining further improved fast gait velocity (4.7%) with reductions in H1 (−35%), and increases in K2 (36%) and A2 (7%).ConclusionPower training increased fast gait velocity in healthy old adults by increasing the reliance on hip muscle function and thus further strengthened the age-related distal-to-proximal shift in muscle function.



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Balance Performance of Deaf Children With and Without Cochlear Implants.

Related Articles

Balance Performance of Deaf Children With and Without Cochlear Implants.

Acta Med Iran. 2016 Nov;54(11):737-742

Authors: Ebrahimi AA, Movallali G, Jamshidi AA, Haghgoo HA, Rahgozar M

Abstract
 The aim of this study was to compare the static and dynamic balance performance of deaf children with and without cochlear implants. This is a cross-sectional study of 145 school children, aged between 7 and 12 years comprising 85 children with congenital or early acquired bilateral profound sensorineural hearing loss (the hearing loss group) and 60 normal hearing aged-matched control counterparts were assessed using the balance subtest of Bruininks-Oseretsky test of Motor Proficiency (BOTMP). The hearing loss group, 50 without cochlear implants (the non-implant group) and 35 of them with unilateral cochlear implants (the implant group) were recruited from schools for the deaf and normal hearing children (the control group) randomly selected from two randomly selected elementary schools of Tehran city. The scores were analyzed using one-way ANOVA. The total score of deaf children especially the implant group were significantly lower than the control group )P<0.001). The balance performance of the control group was better than the implant group in all of the items as well as the non-implant group except the fourth tested item (walking forward on a line) (P<0.05). The balance score of the implant group was significantly lower than the non-implant group except for the third tested item (standing on the preferred leg on a balance beam with eyes closed). The findings suggested that deaf children, specifically those with cochlear implants are at risk for motor and balance deficits. Thus, vestibular and motor evaluations, as well as interventions to improve balance and motor skills, should be prioritized for this population.

PMID: 28033698 [PubMed - in process]

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Cold Thermal Irrigation Decreases the Ipsilateral Gain of the Vestibulo-Ocular Reflex.

Objectives: During head rotations, neuronal firing rates increase in ipsilateral and decrease in contralateral vestibular afferents. At low accelerations, this "push-pull mechanism" is linear. At high accelerations, however, the change of firing rates is nonlinear in that the ipsilateral increase of firing rate is larger than the contralateral decrease. This mechanism of stronger ipsilateral excitation than contralateral inhibition during high-acceleration head rotation, known as Ewald's second law, is implemented within the nonlinear pathways. The authors asked whether caloric stimulation could provide an acceleration signal high enough to influence the contribution of the nonlinear pathway to the rotational vestibulo-ocular reflex gain (rVOR gain) during head impulses. Design: Caloric warm (44[degrees]C) and cold (24, 27, and 30[degrees]C) water irrigations of the left ear were performed in 7 healthy human subjects with the lateral semicircular canals oriented approximately earth-vertical (head inclined 30[degrees] from supine) and earth-horizontal (head inclined 30[degrees] from upright). Results: With the lateral semicircular canal oriented earth-vertical, the strongest cold caloric stimulus (24[degrees]C) significantly decreased the rVOR gain during ipsilateral head impulses, while all other irrigations, irrespective of head position, had no significant effect on rVOR gains during head impulses to either side. Conclusions: Strong caloric irrigation, which can only be achieved with cold water, reduces the rVOR gain during ipsilateral head impulses and thus demonstrates Ewald's second law in healthy subjects. This unilateral gain reduction suggests that cold-water caloric irritation shifts the set point of the nonlinear relation between head acceleration and the vestibular firing rate toward a less acceleration-sensitive zone. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Three-Dimensional Force Profile During Cochlear Implantation Depends on Individual Geometry and Insertion Trauma.

Objectives: To preserve the acoustic hearing, cochlear implantation has to be as atraumatic as possible. Therefore, understanding the impact of the cochlear geometry on insertion forces and intracochlear trauma might help to adapt and improve the electrode insertion and reduce the probability of intracochlear trauma. Design: The study was conducted on 10 fresh-frozen human temporal bones. The inner ear was removed from the temporal bone. The bony capsule covering the scala vestibuli was removed and the dissected inner ear was mounted on the three-dimensional (3D) force measurement system (Agilent technologies, Nano UTM, Santa Clare, CA). A lateral wall electrode array was inserted, and the forces were recorded in three dimensions with a sensitivity of 2 [mu]N. Afterwards, the bones were scanned using a Skyscan 1173 micro-computed tomography (micro-CT). The obtained 3D force profiles were correlated with the videos of the insertions recorded through the microscope, and the micro-CT images. Results: A correlation was found between intracochlear force profiles measured in three different directions with intracochlear trauma detected with micro-CT imaging. The angle of insertion and the cochlear geometry had a significant impact on the electrode array insertion forces and possible insertion trauma. Intracochlear trauma occurred frequently within the first 180[degrees] from the round window, where buckling of the proximal part of the electrode carrier inside the cochlea, and rupturing of the spiral ligament was observed. Conclusions: The combination of the 3D force measurement system and micro-CT can be used to characterize the mechanical behavior of a CI electrode array and some forms of insertion trauma. Intracochlear trauma does not always correlate with higher force amplitudes, but rather with an abrupt change of force directions. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Some Neurocognitive Correlates of Noise-Vocoded Speech Perception in Children With Normal Hearing: A Replication and Extension of Eisenberg et al. (2002)

Objectives: Noise-vocoded speech is a valuable research tool for testing experimental hypotheses about the effects of spectral degradation on speech recognition in adults with normal hearing (NH). However, very little research has utilized noise-vocoded speech with children with NH. Earlier studies with children with NH focused primarily on the amount of spectral information needed for speech recognition without assessing the contribution of neurocognitive processes to speech perception and spoken word recognition. In this study, we first replicated the seminal findings reported by Eisenberg et al. (2002) who investigated effects of lexical density and word frequency on noise-vocoded speech perception in a small group of children with NH. We then extended the research to investigate relations between noise-vocoded speech recognition abilities and five neurocognitive measures: auditory attention (AA) and response set, talker discrimination, and verbal and nonverbal short-term working memory. Design: Thirty-one children with NH between 5 and 13 years of age were assessed on their ability to perceive lexically controlled words in isolation and in sentences that were noise-vocoded to four spectral channels. Children were also administered vocabulary assessments (Peabody Picture Vocabulary test-4th Edition and Expressive Vocabulary test-2nd Edition) and measures of AA (NEPSY AA and response set and a talker discrimination task) and short-term memory (visual digit and symbol spans). Results: Consistent with the findings reported in the original Eisenberg et al. (2002) study, we found that children perceived noise-vocoded lexically easy words better than lexically hard words. Words in sentences were also recognized better than the same words presented in isolation. No significant correlations were observed between noise-vocoded speech recognition scores and the Peabody Picture Vocabulary test-4th Edition using language quotients to control for age effects. However, children who scored higher on the Expressive Vocabulary test-2nd Edition recognized lexically easy words better than lexically hard words in sentences. Older children perceived noise-vocoded speech better than younger children. Finally, we found that measures of AA and short-term memory capacity were significantly correlated with a child's ability to perceive noise-vocoded isolated words and sentences. Conclusions: First, we successfully replicated the major findings from the Eisenberg et al. (2002) study. Because familiarity, phonological distinctiveness and lexical competition affect word recognition, these findings provide additional support for the proposal that several foundational elementary neurocognitive processes underlie the perception of spectrally degraded speech. Second, we found strong and significant correlations between performance on neurocognitive measures and children's ability to recognize words and sentences noise-vocoded to four spectral channels. These findings extend earlier research suggesting that perception of spectrally degraded speech reflects early peripheral auditory processes, as well as additional contributions of executive function, specifically, selective attention and short-term memory processes in spoken word recognition. The present findings suggest that AA and short-term memory support robust spoken word recognition in children with NH even under compromised and challenging listening conditions. These results are relevant to research carried out with listeners who have hearing loss, because they are routinely required to encode, process, and understand spectrally degraded acoustic signals. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Cold Thermal Irrigation Decreases the Ipsilateral Gain of the Vestibulo-Ocular Reflex.

Objectives: During head rotations, neuronal firing rates increase in ipsilateral and decrease in contralateral vestibular afferents. At low accelerations, this "push-pull mechanism" is linear. At high accelerations, however, the change of firing rates is nonlinear in that the ipsilateral increase of firing rate is larger than the contralateral decrease. This mechanism of stronger ipsilateral excitation than contralateral inhibition during high-acceleration head rotation, known as Ewald's second law, is implemented within the nonlinear pathways. The authors asked whether caloric stimulation could provide an acceleration signal high enough to influence the contribution of the nonlinear pathway to the rotational vestibulo-ocular reflex gain (rVOR gain) during head impulses. Design: Caloric warm (44[degrees]C) and cold (24, 27, and 30[degrees]C) water irrigations of the left ear were performed in 7 healthy human subjects with the lateral semicircular canals oriented approximately earth-vertical (head inclined 30[degrees] from supine) and earth-horizontal (head inclined 30[degrees] from upright). Results: With the lateral semicircular canal oriented earth-vertical, the strongest cold caloric stimulus (24[degrees]C) significantly decreased the rVOR gain during ipsilateral head impulses, while all other irrigations, irrespective of head position, had no significant effect on rVOR gains during head impulses to either side. Conclusions: Strong caloric irrigation, which can only be achieved with cold water, reduces the rVOR gain during ipsilateral head impulses and thus demonstrates Ewald's second law in healthy subjects. This unilateral gain reduction suggests that cold-water caloric irritation shifts the set point of the nonlinear relation between head acceleration and the vestibular firing rate toward a less acceleration-sensitive zone. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Three-Dimensional Force Profile During Cochlear Implantation Depends on Individual Geometry and Insertion Trauma.

Objectives: To preserve the acoustic hearing, cochlear implantation has to be as atraumatic as possible. Therefore, understanding the impact of the cochlear geometry on insertion forces and intracochlear trauma might help to adapt and improve the electrode insertion and reduce the probability of intracochlear trauma. Design: The study was conducted on 10 fresh-frozen human temporal bones. The inner ear was removed from the temporal bone. The bony capsule covering the scala vestibuli was removed and the dissected inner ear was mounted on the three-dimensional (3D) force measurement system (Agilent technologies, Nano UTM, Santa Clare, CA). A lateral wall electrode array was inserted, and the forces were recorded in three dimensions with a sensitivity of 2 [mu]N. Afterwards, the bones were scanned using a Skyscan 1173 micro-computed tomography (micro-CT). The obtained 3D force profiles were correlated with the videos of the insertions recorded through the microscope, and the micro-CT images. Results: A correlation was found between intracochlear force profiles measured in three different directions with intracochlear trauma detected with micro-CT imaging. The angle of insertion and the cochlear geometry had a significant impact on the electrode array insertion forces and possible insertion trauma. Intracochlear trauma occurred frequently within the first 180[degrees] from the round window, where buckling of the proximal part of the electrode carrier inside the cochlea, and rupturing of the spiral ligament was observed. Conclusions: The combination of the 3D force measurement system and micro-CT can be used to characterize the mechanical behavior of a CI electrode array and some forms of insertion trauma. Intracochlear trauma does not always correlate with higher force amplitudes, but rather with an abrupt change of force directions. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Some Neurocognitive Correlates of Noise-Vocoded Speech Perception in Children With Normal Hearing: A Replication and Extension of Eisenberg et al. (2002)

Objectives: Noise-vocoded speech is a valuable research tool for testing experimental hypotheses about the effects of spectral degradation on speech recognition in adults with normal hearing (NH). However, very little research has utilized noise-vocoded speech with children with NH. Earlier studies with children with NH focused primarily on the amount of spectral information needed for speech recognition without assessing the contribution of neurocognitive processes to speech perception and spoken word recognition. In this study, we first replicated the seminal findings reported by Eisenberg et al. (2002) who investigated effects of lexical density and word frequency on noise-vocoded speech perception in a small group of children with NH. We then extended the research to investigate relations between noise-vocoded speech recognition abilities and five neurocognitive measures: auditory attention (AA) and response set, talker discrimination, and verbal and nonverbal short-term working memory. Design: Thirty-one children with NH between 5 and 13 years of age were assessed on their ability to perceive lexically controlled words in isolation and in sentences that were noise-vocoded to four spectral channels. Children were also administered vocabulary assessments (Peabody Picture Vocabulary test-4th Edition and Expressive Vocabulary test-2nd Edition) and measures of AA (NEPSY AA and response set and a talker discrimination task) and short-term memory (visual digit and symbol spans). Results: Consistent with the findings reported in the original Eisenberg et al. (2002) study, we found that children perceived noise-vocoded lexically easy words better than lexically hard words. Words in sentences were also recognized better than the same words presented in isolation. No significant correlations were observed between noise-vocoded speech recognition scores and the Peabody Picture Vocabulary test-4th Edition using language quotients to control for age effects. However, children who scored higher on the Expressive Vocabulary test-2nd Edition recognized lexically easy words better than lexically hard words in sentences. Older children perceived noise-vocoded speech better than younger children. Finally, we found that measures of AA and short-term memory capacity were significantly correlated with a child's ability to perceive noise-vocoded isolated words and sentences. Conclusions: First, we successfully replicated the major findings from the Eisenberg et al. (2002) study. Because familiarity, phonological distinctiveness and lexical competition affect word recognition, these findings provide additional support for the proposal that several foundational elementary neurocognitive processes underlie the perception of spectrally degraded speech. Second, we found strong and significant correlations between performance on neurocognitive measures and children's ability to recognize words and sentences noise-vocoded to four spectral channels. These findings extend earlier research suggesting that perception of spectrally degraded speech reflects early peripheral auditory processes, as well as additional contributions of executive function, specifically, selective attention and short-term memory processes in spoken word recognition. The present findings suggest that AA and short-term memory support robust spoken word recognition in children with NH even under compromised and challenging listening conditions. These results are relevant to research carried out with listeners who have hearing loss, because they are routinely required to encode, process, and understand spectrally degraded acoustic signals. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Cold Thermal Irrigation Decreases the Ipsilateral Gain of the Vestibulo-Ocular Reflex.

Objectives: During head rotations, neuronal firing rates increase in ipsilateral and decrease in contralateral vestibular afferents. At low accelerations, this "push-pull mechanism" is linear. At high accelerations, however, the change of firing rates is nonlinear in that the ipsilateral increase of firing rate is larger than the contralateral decrease. This mechanism of stronger ipsilateral excitation than contralateral inhibition during high-acceleration head rotation, known as Ewald's second law, is implemented within the nonlinear pathways. The authors asked whether caloric stimulation could provide an acceleration signal high enough to influence the contribution of the nonlinear pathway to the rotational vestibulo-ocular reflex gain (rVOR gain) during head impulses. Design: Caloric warm (44[degrees]C) and cold (24, 27, and 30[degrees]C) water irrigations of the left ear were performed in 7 healthy human subjects with the lateral semicircular canals oriented approximately earth-vertical (head inclined 30[degrees] from supine) and earth-horizontal (head inclined 30[degrees] from upright). Results: With the lateral semicircular canal oriented earth-vertical, the strongest cold caloric stimulus (24[degrees]C) significantly decreased the rVOR gain during ipsilateral head impulses, while all other irrigations, irrespective of head position, had no significant effect on rVOR gains during head impulses to either side. Conclusions: Strong caloric irrigation, which can only be achieved with cold water, reduces the rVOR gain during ipsilateral head impulses and thus demonstrates Ewald's second law in healthy subjects. This unilateral gain reduction suggests that cold-water caloric irritation shifts the set point of the nonlinear relation between head acceleration and the vestibular firing rate toward a less acceleration-sensitive zone. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Three-Dimensional Force Profile During Cochlear Implantation Depends on Individual Geometry and Insertion Trauma.

Objectives: To preserve the acoustic hearing, cochlear implantation has to be as atraumatic as possible. Therefore, understanding the impact of the cochlear geometry on insertion forces and intracochlear trauma might help to adapt and improve the electrode insertion and reduce the probability of intracochlear trauma. Design: The study was conducted on 10 fresh-frozen human temporal bones. The inner ear was removed from the temporal bone. The bony capsule covering the scala vestibuli was removed and the dissected inner ear was mounted on the three-dimensional (3D) force measurement system (Agilent technologies, Nano UTM, Santa Clare, CA). A lateral wall electrode array was inserted, and the forces were recorded in three dimensions with a sensitivity of 2 [mu]N. Afterwards, the bones were scanned using a Skyscan 1173 micro-computed tomography (micro-CT). The obtained 3D force profiles were correlated with the videos of the insertions recorded through the microscope, and the micro-CT images. Results: A correlation was found between intracochlear force profiles measured in three different directions with intracochlear trauma detected with micro-CT imaging. The angle of insertion and the cochlear geometry had a significant impact on the electrode array insertion forces and possible insertion trauma. Intracochlear trauma occurred frequently within the first 180[degrees] from the round window, where buckling of the proximal part of the electrode carrier inside the cochlea, and rupturing of the spiral ligament was observed. Conclusions: The combination of the 3D force measurement system and micro-CT can be used to characterize the mechanical behavior of a CI electrode array and some forms of insertion trauma. Intracochlear trauma does not always correlate with higher force amplitudes, but rather with an abrupt change of force directions. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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Some Neurocognitive Correlates of Noise-Vocoded Speech Perception in Children With Normal Hearing: A Replication and Extension of Eisenberg et al. (2002)

Objectives: Noise-vocoded speech is a valuable research tool for testing experimental hypotheses about the effects of spectral degradation on speech recognition in adults with normal hearing (NH). However, very little research has utilized noise-vocoded speech with children with NH. Earlier studies with children with NH focused primarily on the amount of spectral information needed for speech recognition without assessing the contribution of neurocognitive processes to speech perception and spoken word recognition. In this study, we first replicated the seminal findings reported by Eisenberg et al. (2002) who investigated effects of lexical density and word frequency on noise-vocoded speech perception in a small group of children with NH. We then extended the research to investigate relations between noise-vocoded speech recognition abilities and five neurocognitive measures: auditory attention (AA) and response set, talker discrimination, and verbal and nonverbal short-term working memory. Design: Thirty-one children with NH between 5 and 13 years of age were assessed on their ability to perceive lexically controlled words in isolation and in sentences that were noise-vocoded to four spectral channels. Children were also administered vocabulary assessments (Peabody Picture Vocabulary test-4th Edition and Expressive Vocabulary test-2nd Edition) and measures of AA (NEPSY AA and response set and a talker discrimination task) and short-term memory (visual digit and symbol spans). Results: Consistent with the findings reported in the original Eisenberg et al. (2002) study, we found that children perceived noise-vocoded lexically easy words better than lexically hard words. Words in sentences were also recognized better than the same words presented in isolation. No significant correlations were observed between noise-vocoded speech recognition scores and the Peabody Picture Vocabulary test-4th Edition using language quotients to control for age effects. However, children who scored higher on the Expressive Vocabulary test-2nd Edition recognized lexically easy words better than lexically hard words in sentences. Older children perceived noise-vocoded speech better than younger children. Finally, we found that measures of AA and short-term memory capacity were significantly correlated with a child's ability to perceive noise-vocoded isolated words and sentences. Conclusions: First, we successfully replicated the major findings from the Eisenberg et al. (2002) study. Because familiarity, phonological distinctiveness and lexical competition affect word recognition, these findings provide additional support for the proposal that several foundational elementary neurocognitive processes underlie the perception of spectrally degraded speech. Second, we found strong and significant correlations between performance on neurocognitive measures and children's ability to recognize words and sentences noise-vocoded to four spectral channels. These findings extend earlier research suggesting that perception of spectrally degraded speech reflects early peripheral auditory processes, as well as additional contributions of executive function, specifically, selective attention and short-term memory processes in spoken word recognition. The present findings suggest that AA and short-term memory support robust spoken word recognition in children with NH even under compromised and challenging listening conditions. These results are relevant to research carried out with listeners who have hearing loss, because they are routinely required to encode, process, and understand spectrally degraded acoustic signals. Copyright (C) 2016 Wolters Kluwer Health, Inc. All rights reserved.

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