Τρίτη 18 Οκτωβρίου 2016

Hearing testing in the U.S. Department of Defense: Potential impact on Veterans Affairs hearing loss disability awards

S03785955.gif

Publication date: Available online 18 October 2016
Source:Hearing Research
Author(s): J.T. Nelson, A.A. Swan, B. Swiger, M. Packer, M.J. Pugh
Hearing loss is the second most common disability awarded by the U.S. Department of Veterans Affairs (VA) to former members of the U.S. uniformed services. Hearing readiness and conservation practices differ among the four largest uniformed military services (Air Force, Army, Marine Corps, and Navy). Utilizing a data set consisting of all hearing loss claims submitted to the VA from fiscal years 2003 to 2013, we examined characteristics of veterans submitting claims within one year of separation from military service. Our results indicate that having a hearing loss disability claim granted was significantly more likely for men, individuals over the age of 26 years at the time of the claim, individuals most recently serving in the U.S. Army, and those with at least one hearing loss diagnosis. Importantly, individuals with at least one test record in the Defense Occupational and Environmental Health Readiness System-Hearing Conservation (DOEHRS-HC) system were significantly less likely to have a hearing loss disability claim granted by the VA. Within the DOEHRS-HC cohort, those with at least one threshold shift or clinical hearing loss diagnosis while on active duty were more than two and three times more likely to have a hearing loss disability claim granted, respectively. These findings indicate that an established history of reduced hearing ability while on active duty was associated with a significantly increased likelihood of an approved hearing loss disability claim relative to VA claims without such a history. Further, our results show a persistent decreased rate of hearing loss disability awards overall. These findings support increased inclusion of personnel in DoD hearing readiness and conservation programs to reduce VA hearing loss disability awards.



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Hearing testing in the U.S. Department of Defense: Potential impact on Veterans Affairs hearing loss disability awards

S03785955.gif

Publication date: Available online 18 October 2016
Source:Hearing Research
Author(s): J.T. Nelson, A.A. Swan, B. Swiger, M. Packer, M.J. Pugh
Hearing loss is the second most common disability awarded by the U.S. Department of Veterans Affairs (VA) to former members of the U.S. uniformed services. Hearing readiness and conservation practices differ among the four largest uniformed military services (Air Force, Army, Marine Corps, and Navy). Utilizing a data set consisting of all hearing loss claims submitted to the VA from fiscal years 2003 to 2013, we examined characteristics of veterans submitting claims within one year of separation from military service. Our results indicate that having a hearing loss disability claim granted was significantly more likely for men, individuals over the age of 26 years at the time of the claim, individuals most recently serving in the U.S. Army, and those with at least one hearing loss diagnosis. Importantly, individuals with at least one test record in the Defense Occupational and Environmental Health Readiness System-Hearing Conservation (DOEHRS-HC) system were significantly less likely to have a hearing loss disability claim granted by the VA. Within the DOEHRS-HC cohort, those with at least one threshold shift or clinical hearing loss diagnosis while on active duty were more than two and three times more likely to have a hearing loss disability claim granted, respectively. These findings indicate that an established history of reduced hearing ability while on active duty was associated with a significantly increased likelihood of an approved hearing loss disability claim relative to VA claims without such a history. Further, our results show a persistent decreased rate of hearing loss disability awards overall. These findings support increased inclusion of personnel in DoD hearing readiness and conservation programs to reduce VA hearing loss disability awards.



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Hearing testing in the U.S. Department of Defense: Potential impact on Veterans Affairs hearing loss disability awards

S03785955.gif

Publication date: Available online 18 October 2016
Source:Hearing Research
Author(s): J.T. Nelson, A.A. Swan, B. Swiger, M. Packer, M.J. Pugh
Hearing loss is the second most common disability awarded by the U.S. Department of Veterans Affairs (VA) to former members of the U.S. uniformed services. Hearing readiness and conservation practices differ among the four largest uniformed military services (Air Force, Army, Marine Corps, and Navy). Utilizing a data set consisting of all hearing loss claims submitted to the VA from fiscal years 2003 to 2013, we examined characteristics of veterans submitting claims within one year of separation from military service. Our results indicate that having a hearing loss disability claim granted was significantly more likely for men, individuals over the age of 26 years at the time of the claim, individuals most recently serving in the U.S. Army, and those with at least one hearing loss diagnosis. Importantly, individuals with at least one test record in the Defense Occupational and Environmental Health Readiness System-Hearing Conservation (DOEHRS-HC) system were significantly less likely to have a hearing loss disability claim granted by the VA. Within the DOEHRS-HC cohort, those with at least one threshold shift or clinical hearing loss diagnosis while on active duty were more than two and three times more likely to have a hearing loss disability claim granted, respectively. These findings indicate that an established history of reduced hearing ability while on active duty was associated with a significantly increased likelihood of an approved hearing loss disability claim relative to VA claims without such a history. Further, our results show a persistent decreased rate of hearing loss disability awards overall. These findings support increased inclusion of personnel in DoD hearing readiness and conservation programs to reduce VA hearing loss disability awards.



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Hearing testing in the U.S. Department of Defense: Potential impact on Veterans Affairs hearing loss disability awards

Publication date: Available online 18 October 2016
Source:Hearing Research
Author(s): J.T. Nelson, A.A. Swan, B. Swiger, M. Packer, M.J. Pugh
Hearing loss is the second most common disability awarded by the U.S. Department of Veterans Affairs (VA) to former members of the U.S. uniformed services. Hearing readiness and conservation practices differ among the four largest uniformed military services (Air Force, Army, Marine Corps, and Navy). Utilizing a data set consisting of all hearing loss claims submitted to the VA from fiscal years 2003 to 2013, we examined characteristics of veterans submitting claims within one year of separation from military service. Our results indicate that having a hearing loss disability claim granted was significantly more likely for men, individuals over the age of 26 years at the time of the claim, individuals most recently serving in the U.S. Army, and those with at least one hearing loss diagnosis. Importantly, individuals with at least one test record in the Defense Occupational and Environmental Health Readiness System-Hearing Conservation (DOEHRS-HC) system were significantly less likely to have a hearing loss disability claim granted by the VA. Within the DOEHRS-HC cohort, those with at least one threshold shift or clinical hearing loss diagnosis while on active duty were more than two and three times more likely to have a hearing loss disability claim granted, respectively. These findings indicate that an established history of reduced hearing ability while on active duty was associated with a significantly increased likelihood of an approved hearing loss disability claim relative to VA claims without such a history. Further, our results show a persistent decreased rate of hearing loss disability awards overall. These findings support increased inclusion of personnel in DoD hearing readiness and conservation programs to reduce VA hearing loss disability awards.



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Hearing testing in the U.S. Department of Defense: Potential impact on Veterans Affairs hearing loss disability awards

Publication date: Available online 18 October 2016
Source:Hearing Research
Author(s): J.T. Nelson, A.A. Swan, B. Swiger, M. Packer, M.J. Pugh
Hearing loss is the second most common disability awarded by the U.S. Department of Veterans Affairs (VA) to former members of the U.S. uniformed services. Hearing readiness and conservation practices differ among the four largest uniformed military services (Air Force, Army, Marine Corps, and Navy). Utilizing a data set consisting of all hearing loss claims submitted to the VA from fiscal years 2003 to 2013, we examined characteristics of veterans submitting claims within one year of separation from military service. Our results indicate that having a hearing loss disability claim granted was significantly more likely for men, individuals over the age of 26 years at the time of the claim, individuals most recently serving in the U.S. Army, and those with at least one hearing loss diagnosis. Importantly, individuals with at least one test record in the Defense Occupational and Environmental Health Readiness System-Hearing Conservation (DOEHRS-HC) system were significantly less likely to have a hearing loss disability claim granted by the VA. Within the DOEHRS-HC cohort, those with at least one threshold shift or clinical hearing loss diagnosis while on active duty were more than two and three times more likely to have a hearing loss disability claim granted, respectively. These findings indicate that an established history of reduced hearing ability while on active duty was associated with a significantly increased likelihood of an approved hearing loss disability claim relative to VA claims without such a history. Further, our results show a persistent decreased rate of hearing loss disability awards overall. These findings support increased inclusion of personnel in DoD hearing readiness and conservation programs to reduce VA hearing loss disability awards.



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Disorders induced by direct occupational exposure to noise: Systematic review

Andrea Domingo-Pueyo, Javier Sanz-Valero, Carmina Wanden-Berghe

Noise and Health 2016 18(84):229-239

Background: To review the available scientific literature about the effects on health by occupational exposure to noise. Materials and Methods: A systematic review of the retrieved scientific literature from the databases MEDLINE (via PubMed), ISI-Web of Knowledge (Institute for Scientific Information), Cochrane Library Plus, SCOPUS, and SciELO (collection of scientific journals) was conducted. The following terms were used as descriptors and were searched in free text: “Noise, Occupational,” “Occupational Exposure,” and “Occupational Disease.” The following limits were considered: “Humans,” “Adult (more than 18 years),” and “Comparative Studies.” Results: A total of 281 references were retrieved, and after applying inclusion/exclusion criteria, 25 articles were selected. Of these selected articles, 19 studies provided information about hearing disturbance, four on cardiovascular disorders, one regarding respiratory alteration, and one on other disorders. Conclusions: It can be interpreted that the exposure to noise causes alterations in humans with different relevant outcomes, and therefore appropriate security measures in the work environment must be employed to minimize such an exposure and thereby to reduce the number of associated disorders.

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The effect of room acoustics on the sleep quality of healthy sleepers

Ingo Fietze, Charlotte Barthe, Matthias Hölzl, Martin Glos, Sandra Zimmermann, Ralf Bauer-Diefenbach, Thomas Penzel

Noise and Health 2016 18(84):240-246

Introduction: Noise is one of the factors that can seriously disturb sleep, and sound volume is an important factor in this context. One strategy involves avoiding exposure to sounds in the night, while entail the minimization of background noise in a bedroom. The goal of this study was to investigate the effect of systematic sound attenuation on nocturnal sleep by influencing sound volume and reverberation within the context of room acoustics. Materials and Methods: On this basis, we designed a randomized, controlled crossover trial investigating 24 healthy sleepers (15 men and 9 women, aged 24.9 ± 4.1 years) with a body mass index (BMI) of 21.9 ± 1.6 kg/m2. Each participant slept for three consecutive nights at three different locations: (a) at our sleep lab, (b) at the participant’s home, and (c) at an acoustically isolated room. In addition to conduct of polysomnography (PSG), subjective sleep quality and nocturnal noise level were measured at each location. We likewise measured room temperature and relative humidity. Results: Under conditions of equal sleep efficiency, a significant increase in deep sleep, by 16–34 min, was determined in an acoustically isolated room in comparison to the two other sleep locations. Fewer arousal events and an increase in rapid eye movement (REM) latency became evident in an acoustically isolated environment. Sleep in a domestic environment was subjectively better than sleep under the two test conditions. Discussion: For healthy sleepers, room acoustics influence the microstructure of sleep, without subjective morning benefit. Reduction of noise level and of reverberation leads to an increase in the amount of deep sleep and to reduction of nocturnal arousal events, which is especially important for poor sleepers.

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The Protective effect of the endoplasmic reticulum stress-related factors BiP/GRP78 and CHOP/Gadd153 on noise-induced hearing loss in guinea pigs

Qiuhong Xue, Caihong Li, Jia Chen, Hongmei Guo, Dongqing Li, Xianglei Wu

Noise and Health 2016 18(84):247-255

Context: The audiological features and cochlear morphology of individuals with noise-induced hearing loss (NIHL) are well characterized. However, the molecular processes in the cochlea are not well understood. Aims: To explore the role of the endoplasmic reticulum stress (ERS) response in the guinea pig model of cochlear damage induced by exposure to intense noise. Settings and Design: A pilot case–control study. Subjects and Methods: Forty-eight guinea pigs were divided into four equal groups. At 1, 4, or 14 days (d) post-exposure, the auditory brainstem responses (ABRs) were tested before sacrificing the subjects. The expression levels of the binding immunoglobulin protein/glucose-regulated protein 78 (BiP/GRP78) and C/EBP-homologous protein/growth arrest and DNA damage-inducible gene 153 (CHOP/Gadd153) proteins were evaluated using immunohistochemistry and Western blotting. The number of cochlear hair cells with altered nuclei was counted using confocal fluorescence microscopy. Statistical analysis used: One-way analysis of variance (ANOVA) and the least squares difference (LSD) test. Results: The outer hair cells (OHCs) showed changes of apoptosis, necrosis, and loss after noise exposure. In the 1- and 4-d groups, more apoptotic cells were found than necrotic cells (P < 0.01). The level of BiP/GRP78 was significantly higher in all three experimental groups compared to the control group (P < 0.01). The level of CHOP/Gadd153 was increased at 1 d post-exposure, achieving a peak that was maintained until 4 d, after which it returned to baseline levels by 14 d post-exposure. Conclusions: ERS response was activated by inducing the expression of BiP/GRP78 to lessen the extent of the resulting cellular damage and activating the CHOP/Gadd153 pathway to eliminate the most severely damaged cells.

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Determination of the level of noise in nurseries and pre-schools and the teachers' level of annoyance

Ozan Gokdogan, Cagil Gokdogan

Noise and Health 2016 18(84):256-259

Objective: The aim of this article is to determine the level of noise in nurseries and pre-schools and also to compare measured levels with standard levels and evaluate the teachers’ level of annoyance. Materials and Methods: The level of noise was measured in three different schools. A total of 162 students, whose ages were between 3 and 6 years, and 12 teachers were included the study. Every age groups’ level of noise was measured during sleeping, gaming, and eating activity. In addition, teachers’ annoyance was assessed in different age groups. Results: The 4- to 6-year-old groups were found to have higher level of sounds than 3-year-old group. Eating period was found to be the highest level of sound whereas sleeping was found the lowest. Furthermore, teachers’ annoyance was found higher as the age decreased. Conclusion: Nurseries and pre-schools have noisy environment both for the students and the teachers. High level of noise, which has bad effects on health, is a public health problem. Both the students’ families and teachers must be aware of this annoying situation.

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Auditory brainstem responses for click and CE-chirp stimuli in individuals with and without occupational noise exposure

Zeena Venkatacheluvaiah Pushpalatha, Sreeraj Konadath

Noise and Health 2016 18(84):260-265

Introduction: Encoding of CE-chirp and click stimuli in auditory system was studied using auditory brainstem responses (ABRs) among individuals with and without noise exposure. Materials and Methods: The study consisted of two groups. Group 1 (experimental group) consisted of 20 (40 ears) individuals exposed to occupational noise with hearing thresholds within 25 dB HL. They were further divided into three subgroups based on duration of noise exposure (0–5 years of exposure-T1, 5–10 years of exposure-T2, and >10 years of exposure-T3). Group 2 (control group) consisted of 20 individuals (40 ears). Absolute latency and amplitude of waves I, III, and V were compared between the two groups for both click and CE-chirp stimuli. T1, T2, and T3 groups were compared for the same parameters to see the effect of noise exposure duration on CE-chirp and click ABR. Result: In Click ABR, while both the parameters for wave III were significantly poorer for the experimental group, wave V showed a significant decline in terms of amplitude only. There was no significant difference obtained for any of the parameters for wave I. In CE-Chirp ABR, the latencies for all three waves were significantly prolonged in the experimental group. However, there was a significant decrease in terms of amplitude in only wave V for the same group. Discussion: Compared to click evoked ABR, CE-Chirp ABR was found to be more sensitive in comparison of latency parameters in individuals with occupational noise exposure. Monitoring of early pathological changes at the brainstem level can be studied effectively by using CE-Chirp stimulus in comparison to click stimulus. Conclusion: This study indicates that ABR’s obtained with CE-chirp stimuli serves as an effective tool to identify the early pathological changes due to occupational noise exposure when compared to click evoked ABR.

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Occupational noise exposure on a Royal Navy warship during weapon fire

Gurmail Singh Paddan

Noise and Health 2016 18(84):266-273

Introduction: Measurements were made of the sound pressure levels on a military ship HMS Grimsby during firing of a Heavy Machine Gun (HMG) mounted on the starboard bridge wing. The measurement positions comprised three locations on the ship’s bridge (the wheelhouse) and one location on the starboard bridge wing. Equipment and Procedure: The three locations on the bridge were the starboard door, centre and port door. A total of 255 burst firings were measured during the survey comprising 850 rounds with each burst encompassing from 1 to 10 rounds. Analysis: The data have been assessed and interpreted in accordance with the Control of Noise at Work Regulations 2005. Results: The highest peak sound pressure levels measured on the bridge wing and on the bridge were 160.7 dB(C) (2170 Pa) and 122.7 dB(C) (27.3 Pa), respectively. The highest sound exposure levels measured on the bridge wing and on the bridge corresponding to one round being fired were 127.8 dB(A) and 88.9 dB(A), respectively. The ship’s structure provided about 40 dB attenuation in the transmitted noise. Discussion: The operator of the weapon would be required to wear some form of hearing protection. On the basis of the measured peak noise levels, there would be no requirement for bridge crew to wear any hearing protection during firing of a HMG. However, crew exposure to noise on the bridge is likely to exceed the upper exposure action value corresponding to 85 dB(A) after about 11,750 rounds. Conclusions: Measurements made on the bridge wings are likely to be affected by reflections from the ship’s structure.

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Soft tissue conduction as a possible contributor to the limited attenuation provided by hearing protection devices

Shai Chordekar, Cahtia Adelman, Haim Sohmer, Liat Kishon-Rabin

Noise and Health 2016 18(84):274-279

Context: Damage to the auditory system by loud sounds can be avoided by hearing protection devices (HPDs) such as earmuffs, earplugs, or both for maximum attenuation. However, the attenuation can be limited by air conduction (AC) leakage around the earplugs and earmuffs by the occlusion effect (OE) and by skull vibrations initiating bone conduction (BC). Aims: To assess maximum attenuation by HPDs and possible flanking pathways to the inner ear. Subjects and Methods: AC attenuation and resulting thresholds were assessed using the real ear attenuation at threshold (REAT) procedure on 15 normal-hearing participants in four free-field conditions: (a) unprotected ears, (b) ears covered with earmuffs, (c) ears blocked with deeply inserted customized earplugs, and (d) ears blocked with both earplugs and earmuffs. BC thresholds were assessed with and without earplugs to assess the OE. Results: Addition of earmuffs to earplugs did not cause significantly greater attenuation than earplugs alone, confirming minimal AC leakage through the external meatus and the absence of the OE. Maximum REATs ranged between 40 and 46 dB, leading to thresholds of 46–54 dB HL. Furthermore, calculation of the acoustic impedance mismatch between air and bone predicted at least 60 dB attenuation of BC. Conclusion: Results do not support the notion that skull vibrations (BC) contributed to the limited attenuation provided by traditional HPDs. An alternative explanation, supported by experimental evidence, suggests transmission of sound to inner ear via non-osseous pathways such as skin, soft tissues, and fluid. Because the acoustic impedance mismatch between air and soft tissues is smaller than that between air and bone, air-borne sounds would be transmitted to soft tissues more effectively than to bone, and therefore less attenuation is expected through soft tissue sound conduction. This can contribute to the limited attenuation provided by traditional HPDs. The present study has practical implications for hearing conservation protocols.

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Supporting the global initiative of preventing childhood hearing loss: Act now, here's how!

Saurabh RamBihariLal Shrivastava, Prateek Saurabh Shrivastava, Jegadeesh Ramasamy

Noise and Health 2016 18(84):280-281



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An evaluation of the perceptual quality of phase-aware single-channel speech enhancement

For the enhancement of single-channel speech corrupted by acoustic noise, recently short-time Fourier transform domain clean speech estimators were proposed that incorporate prior information about the clean speech spectral phase. Instrumental measures predict quality improvements for the phase-aware estimators over their conventional phase-blind counterparts. In this letter, these predictions are verified by means of listening experiments. The phase-aware amplitude estimator on average achieves a stronger noise reduction and is significantly preferred over its phase-blind counterpart in a pairwise comparison even if the clean spectral phase is estimated blindly on the noisy signal.



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Relative contribution of envelope and fine structure to the subcortical encoding of noise-degraded speech

Brainstem frequency-following responses (FFR) were elicited to the speech token /ama/ in noise containing only envelope (ENV) or fine structure (TFS) cues to assess the relative contribution of these temporal features to the neural encoding of degraded speech. Successive cue removal weakened FFRs with noise having the most deleterious effect on TFS coding. Neuro-acoustic and response-to-response correlations revealed speech-FFRs are dominated by stimulus ENV for clean speech, with TFS making a stronger contribution in moderate noise levels. Results suggest that the relative weighting of temporal ENV and TFS cues to the neural transcription of speech depends critically on the degree of noise in the soundscape.



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Just noticeable differences of spatial cues in echoic and anechoic acoustical environments

The perceptual limits for detecting changes in binaural cues also define the boundaries for the perception of differences in spatial impression. This study reports just noticeable differences for interaural time delays (ITDs) and interaural level differences (ILDs) of the early part and for the interaural cross-correlation (IACC) of the early and diffuse part of binaural room impulse responses. The results show that ITDs only allow a high accuracy in localization in anechoic environments, whereas ILDs show a higher robustness against reverberation. Subjects are rather insensitive to changes in IACC, only changes that bring the IACC close to one are detectable.



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A case report of severe neuro-Behcet's disease treated with a combination of immunosuppressives and a TNF-inhibitor: A difficult therapeutic choice.

A case report of severe neuro-Behcet's disease treated with a combination of immunosuppressives and a TNF-inhibitor: A difficult therapeutic choice.

Acta Reumatol Port. 2016 Sep 28;

Authors: Korkmaz FN, Ozen G, Ünal AU, Kahraman Koytak P, Tuncer N, Direskeneli H

Abstract
Abstract/ Resumo Behcet's disease (BD) is a multisystem inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions and uveitis. The nervous system involvement of BD, neuro-Behcet's disease (NBD), is one of the important causes of mortality of the disease. Herein, we present a 29-year-old male with parenchymal NBD who has progressed rapidly and was managed with an uncommon aggressive immunosuppresive combination therapy. The patient first presented six years ago with vertigo and difficulty in talking and walking. On examination, he had oral ulcers, acneiform lesions on the torso, genital ulcer scar, dysartria, and ataxia. Along with the magnetic resonance imaging (MRI) findings, the patient was diagnosed as NBD. After pulse methylprednisolone (1g/day, 3 days) and 8 courses of 1g/month iv cylophosphamide therapy, he was put on azathioprine and oral methlyprednisolone. On the 4th year of the maintenance therapy, he was admitted with NBD relapse which was treated with 3 days of iv 1g pulse methlyprednisolone. One year after the last relapse, the patient voluntarily stopped medications and presented with global aphasia, right hemihypoesthesia and quadriparesis. MRI findings were suggestive of NBD relapse. After exclusion of infection, pulse methylprednisolone was started but no improvement was observed. Considering the severity of the NBD, the patient was put on methylprednisolone (1mg/kg/day), iv cylophosphamide (1g) and adalimumab 40 mg/14 days subcutaneously with appropriate tuberculosis prophylaxis. Neurological examination and MRI findings after 4 weeks showed dramatic improvement however patient developed pulmonary tuberculosis. Methylprednisolone dose was decreased (0.5mg/kg/day) and quadruple antituberculosis therapy was started. Patient was discharged with 5/5 muscle strength in extremities without any respiratory symptoms 2 months after first presentation. Prompt introduction of immunosuppressive therapy is crucial in NBD. Although combination of TNF inhibitors and cyclophoshamide is a rare therapeutic approach, it may be life-saving. However a higher awareness is required for opportunistic infections.

PMID: 27750273 [PubMed - as supplied by publisher]



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Clinical characteristics and the identification of novel mutations of COL1A1 and COL1A2 in 61 Chinese patients with osteogenesis imperfecta.

Clinical characteristics and the identification of novel mutations of COL1A1 and COL1A2 in 61 Chinese patients with osteogenesis imperfecta.

Mol Med Rep. 2016 Oct 12;:

Authors: Zhang H, Yue H, Wang C, Hu W, Gu J, He J, Fu W, Hu Y, Li M, Zhang Z

Abstract
Osteogenesis imperfecta (OI) is an inherited connective tissue disorder characterized by brittle bone fractures. The aim of the present study was to investigate the pathogenic gene mutation spectrum and clinical manifestations of mutations in collagen type I, alpha 1 (COL1A1) and collagen type I, alpha 2 (COL1A2) genes in Chinese patients with OI. A total of 61 unrelated Chinese OI patients with COL1A1 and COL1A2 mutations were recruited. All the exons and the exon-intron boundaries of the COL1A1 and COL1A2 genes were amplified and directly sequenced and lumbar spine bone mineral density was measured by dual‑energy X‑ray absorptiometry. The mutations of the 61 probands included 33 missense mutations, 8 nonsense mutations, 7 splicing variants and 13 frameshift mutations in COL1A1 and COL1A2 genes. A total of 25 novel mutations were identified, including 18 in COL1A1 and 7 in COL1A2. The mutations p.Gly257Arg, p.Gly767Ser and p.Gly821Ser in COL1A1 and p.Gly337Ser in COL1A2 may be located at a mutation hotspot for human OI due to the high repetition rate in OI patients. Family history was positive for OI in 33 probands (54%). All probands had suffered fractures and the most common fracture site was the femur. A total of 49 probands presented with blue sclerae (80.3%), 20 probands suffered from dentinogenesis imperfecta (32.8%) and 1 patient had hearing loss (1.6%). These findings may improve understanding of the pathogenic gene mutation spectrum and the clinical manifestations of mutations of COL1A1 and COL1A2 genes in Chinese patients with OI.

PMID: 27748872 [PubMed - as supplied by publisher]



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Unilateral Hearing Loss is Associated With Impaired Balance in Children: A Pilot Study.

Unilateral Hearing Loss is Associated With Impaired Balance in Children: A Pilot Study.

Otol Neurotol. 2016 Sep 30;

Authors: Wolter NE, Cushing SL, Madrigal LD, James AL, Campos J, Papsin BC, Gordon KA

Abstract
OBJECTIVE: To determine if children with unilateral sensorineural hearing loss (UHL) demonstrate impaired balance compared with their normal hearing (NH) peers.
STUDY DESIGN: Prospective, case-control study.
METHODS: Balance was assessed in14 UHL and 14 NH children using the Bruininks-Oseretsky Test-2 (BOT-2) and time to fall (TTF) in an immersive, virtual-reality laboratory. Postural control was quantified by center of pressure (COP) using force plates. The effect of vision on balance was assessed by comparing scores and COP characteristics on BOT-2 tasks performed with eyes open and closed.
RESULTS: Balance ability as measured by the BOT-2 score was significantly worse in children with UHL compared with NH children (p = 0.004). TTF was shorter in children with UHL compared with NH children in the most difficult tasks when visual and somatosensory inputs were limited (p < 0.01). Visual input improved postural control (reduced COP variability) in both groups in all tasks (p < 0.05) but postural control as measured by COP variability was more affected in children with UHL when visual input was removed while performing moderately difficult tasks (i.e., standing on one foot) (p = 0.02).
CONCLUSION: In this pilot study, children with UHL show poorer balance skills than NH children. Significant differences in TTF between the two groups were only seen in the most difficult tasks and therefore may be missed on routine clinical assessment. Children with UHL appear to rely more on vision for maintaining postural control than their NH peers. These findings may point to deficits not only in the hearing but also the vestibular portion of the inner ear.

PMID: 27749751 [PubMed - as supplied by publisher]



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Unilateral Hearing Loss is Associated With Impaired Balance in Children: A Pilot Study.

Unilateral Hearing Loss is Associated With Impaired Balance in Children: A Pilot Study.

Otol Neurotol. 2016 Sep 30;

Authors: Wolter NE, Cushing SL, Madrigal LD, James AL, Campos J, Papsin BC, Gordon KA

Abstract
OBJECTIVE: To determine if children with unilateral sensorineural hearing loss (UHL) demonstrate impaired balance compared with their normal hearing (NH) peers.
STUDY DESIGN: Prospective, case-control study.
METHODS: Balance was assessed in14 UHL and 14 NH children using the Bruininks-Oseretsky Test-2 (BOT-2) and time to fall (TTF) in an immersive, virtual-reality laboratory. Postural control was quantified by center of pressure (COP) using force plates. The effect of vision on balance was assessed by comparing scores and COP characteristics on BOT-2 tasks performed with eyes open and closed.
RESULTS: Balance ability as measured by the BOT-2 score was significantly worse in children with UHL compared with NH children (p = 0.004). TTF was shorter in children with UHL compared with NH children in the most difficult tasks when visual and somatosensory inputs were limited (p < 0.01). Visual input improved postural control (reduced COP variability) in both groups in all tasks (p < 0.05) but postural control as measured by COP variability was more affected in children with UHL when visual input was removed while performing moderately difficult tasks (i.e., standing on one foot) (p = 0.02).
CONCLUSION: In this pilot study, children with UHL show poorer balance skills than NH children. Significant differences in TTF between the two groups were only seen in the most difficult tasks and therefore may be missed on routine clinical assessment. Children with UHL appear to rely more on vision for maintaining postural control than their NH peers. These findings may point to deficits not only in the hearing but also the vestibular portion of the inner ear.

PMID: 27749751 [PubMed - as supplied by publisher]



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