The Syndrome of Familial Hypoparathyroidism, Sensorineural Deafness and Renal Dysplasia.

The Syndrome of Familial Hypoparathyroidism, Sensorineural Deafness and Renal Dysplasia.

J Assoc Physicians India. 2015 Jun;63(6):73-5

Authors: Meena RL, Maloo SK, Samar N, Ruhela A, Saini S

Abstract
The syndrome of familial hypoparathyroidism, sensorineural deafness, and renal dysplasia (HDR syndrome) is inherited as an autosomal dominant trait, caused by haploinsufficiency of the GATA3 gene in chromosome 10p. Although first described years ago, but the disease is considered to be very rare. Patients usually present with hypocalcemia, tetany, or afebrile convulsions at any age. Hearing loss is usually bilateral, range from mild to profound impairment. Renal disease includes dysplasia, hypoplasia or aplasia.

PMID: 26710407 [PubMed - in process]

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Preschool television programmes: analysis using SmartSound IQ data logging.

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Preschool television programmes: analysis using SmartSound IQ data logging.

Cochlear Implants Int. 2015 Jan;16 Suppl 1:S26-9

Authors: Hanvey K, DeBold L

PMID: 25614262 [PubMed - indexed for MEDLINE]

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Achievement of early clinical milestones and long-term functional outcomes for children and young adults with bilateral cochlear implants.

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Achievement of early clinical milestones and long-term functional outcomes for children and young adults with bilateral cochlear implants.

Cochlear Implants Int. 2015 Jan;16 Suppl 1:S16-8

Authors: Galvin KL

PMID: 25614259 [PubMed - indexed for MEDLINE]

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Unilateral hearing loss is associated with a negative effect on language scores in adolescents.

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Unilateral hearing loss is associated with a negative effect on language scores in adolescents.

Int J Pediatr Otorhinolaryngol. 2014 Oct;78(10):1611-7

Authors: Fischer C, Lieu J

Abstract
OBJECTIVE: To determine if adolescents with unilateral hearing loss (UHL) demonstrate worse language skills than their siblings with normal hearing (NH).
DESIGN: Case-control study of 12-17-year-old adolescents with UHL (20 cases) compared with sibling controls with NH (13 controls).
METHODS: Scores on the oral portion of the Oral and Written Language Scales (OWLS) and the Clinical Evaluation of Language Fundamentals (CELF) were the primary outcome measure. Wechsler's Abbreviated Scales of Intelligence (WASI) scores were also used as an outcome measure.
RESULTS: Adolescents with UHL demonstrated worse overall and expressive language scores than controls, (98 vs. 114, P=0.001; 100 vs. 114, P=0.006) and had significantly lower full scale (98 vs. 112, P=0.017), verbal (101 vs. 113, P=0.032), and performance IQ (95 vs. 107, P=0.037).
CONCLUSIONS: These findings suggest that UHL in adolescents is associated with a negative effect on standardized language scores and IQ. They also demonstrate that the developmental gap between children with UHL and children with NH does not resolve as the children progress into adolescence and may even widen as the children grow older. Therefore, these results strongly encourage implementation of early intervention for children with UHL to prevent speech-language delays. More studies in adolescents are warranted to evaluate educational outcomes.

PMID: 25081604 [PubMed - indexed for MEDLINE]

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Effectiveness of rehabilitation interventions in improving emotional and functional status in hearing or visually impaired older adults: a systematic review with meta-analyses.

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Effectiveness of rehabilitation interventions in improving emotional and functional status in hearing or visually impaired older adults: a systematic review with meta-analyses.

Clin Rehabil. 2015 Feb;29(2):107-19

Authors: Roets-Merken LM, Draskovic I, Zuidema SU, van Erp WS, Graff MJ, Kempen GI, Vernooij-Dassen MJ

Abstract
OBJECTIVE: To assess the effectiveness of non-equipment based rehabilitation interventions for older adults with an age-related hearing or visual impairment.
DATA SOURCES: We searched PubMed, EMBASE, PsychInfo, CINAHL, and the Cochrane Central Register of Controlled Trials.
REVIEW METHODS: Two authors independently assessed trial eligibility, extracted data and assessed methodological quality. Controlled studies with more than 80% of participants aged ≥ 55 years and with rehabilitation interventions either separately or in combination with technical device provision were included. Meta-analyses were undertaken for the primary outcomes: emotional status, functional status, self-efficacy and social participation. All studies were categorized into 3 subgroups of intervention approaches (cognitive restructuring, education, and problem-solving), and subgroup analysis was performed.
RESULTS: Fourteen studies were identified: six on hearing impairment and eight on visual impairment, involving 1622 sensory impaired participants (mean age 70). Methodological quality of the studies was modest. Eight studies offered data for meta-analysis. No significant effects in favour of interventions on either emotional or functional status, self-efficacy, or social participation were found. In the subgroup analysis, only the problem-solving approach showed a positive effect on emotional status.
CONCLUSIONS: This review found no effects of non-equipment based rehabilitation interventions on emotional and functional status, self-efficacy, and social participation. However, subgroup analysis showed problem-solving as a potential effective approach for positively affecting emotional status.

PMID: 25027446 [PubMed - indexed for MEDLINE]

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Exergames for unsupervised balance training at home: A pilot study in healthy older adults

Publication date: February 2016
Source:Gait & Posture, Volume 44
Author(s): M. van Diest, J. Stegenga, H.J. Wörtche, G.J. Verkerke, K. Postema, C.J.C. Lamoth
Exercise videogames (exergames) are gaining popularity as tools for improving balance ability in older adults, yet few exergames are suitable for home-based use. The purpose of the current pilot study was to examine the effects of a 6-week unsupervised home-based exergaming training program on balance performance. Ten community dwelling healthy older adults (age: 75.9±7.2 years) played a newly developed ice skating exergame for six weeks at home. In the game, the speed and direction of a virtual ice skater on a frozen canal were controlled using lateral weight shifts, which were captured using Kinect. Sway characteristics during quiet standing in eyes open (EO), eyes closed (EC) and dual task (DT) conditions were assessed in time and frequency domain before, and after two, four and six weeks of training. Balance was also evaluated using the narrow ridge balance test (NRBT). Multilevel modeling was applied to examine changes in balance ability. Participants played 631 (±124)min over the intervention period and no subjects dropped out. Balance in terms of sway characteristics improved on average by 17.4% (EO) and 23.3% (EC) after six weeks of training (p<0.05). Differences in rate of improvement (p<0.05) were observed between participants. No intervention effects were found for quiet standing in DT conditions and on the NRBT. In conclusion, the pilot study showed that unsupervised home-based exergaming is feasible in community dwelling older adults, but also that participants do not benefit equally from the program, thereby emphasizing the need for more personalized exergame training programs.



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The correlation between symptomatic fatigue to definite measures of gait in people with multiple sclerosis

Publication date: February 2016
Source:Gait & Posture, Volume 44
Author(s): Alon Kalron
There is a general consensus relating to the multidimensional aspects of fatigue in people with multiple sclerosis (PwMS), however, the exact impact of this symptom on gait is not fully understood. Our primary aim was to examine the relationship between definite parameters of gait with self-reported symptomatic fatigue in PwMS according to their level of neurological impairment. Spatio-temporal parameters of gait were studied using an electronic walkway. The Multiple Sclerosis Walking Scale (MSWS-12) questionnaire, a patient-rated measure of walking ability was collected. The Modified Fatigue Impact Scale (MFIS) questionnaire was used to determine the level of symptomatic fatigue. One hundred and one PwMS (61 women) were included in the study analysis. Subjects were divided into mild and moderate neurological impaired groups. Fatigue was correlated with 5 (out of 14) spatiotemporal parameters. However, correlation scores were all <0.35, thus considered as weak correlations. In the mild group, the double support period was the only variable positively correlated to fatigue (Spearman's rho=0.28, P=0.05). In the moderate group, step and stride length were solely negatively correlated to fatigue (Spearman's rho=0.32, P=0.03). In contrast to the definite gait parameters, the MSWS-12 self-questionnaire was moderately positively correlated to the level of fatigue. Scores for the total, mild and moderate groups were 0.54, 0.57 and 0.51; P<0.01, respectively. The present results indicate that modifications in spatio-temporal parameters of gait are not closely related to symptomatic fatigue in PwMS. On the contrary, the self-reported MSWS-12 questionnaire is predisposed to level of fatigue in PwMS.



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The impact of body fat on three dimensional motion of the paediatric foot during walking

Publication date: February 2016
Source:Gait & Posture, Volume 44
Author(s): Ryan Mahaffey, Stewart C. Morrison, Paul Bassett, Wendy I. Drechsler, Mary C. Cramp
Childhood obesity is commonly associated with a pes planus foot type and altered lower limb joint function during walking. However, limited information has been reported on dynamic intersegment foot motion with the level of obesity in children. The aim of this study was to explore the relationships between intersegment foot motion during gait and body fat in boys age 7–11 years.Fat mass was measured in fifty-five boys using air displacement plethysmography. Three-dimensional gait analysis was conducted on the right foot of each participant using the 3DFoot model to capture angular motion of the shank, calcaneus, midfoot and metatarsals. Two multivariate statistical techniques were employed; principle component analysis reduced the multidimensional nature of gait analysis, and multiple linear regression analysis accounted for potential confounding factors.Higher fat mass predicted greater plantarflexion of the calcaneus during the first half and end of stance phase and at the end of swing phase. Greater abduction of the calcaneus throughout stance and swing was predicted by greater fat mass. At the midfoot, higher fat mass predicted greater dorsiflexion and eversion throughout the gait cycle.The findings present novel information on the relationships between intersegment angular motion of the foot and body fat in young boys. The data indicates a more pronated foot type in boys with greater body fat. These findings have clinical implications for pes planus and a predisposition for pain and discomfort during weight bearing activities potentially reducing motivation in obese children to be physically active.



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Using a goal attainment scale in the evaluation of outcomes in patients with diplegic cerebral palsy

Publication date: February 2016
Source:Gait & Posture, Volume 44
Author(s): D. McMorran, L.W. Robinson, G. Henderson, J. Herman, J.E. Robb, M.S. Gaston
A goal attainment scale (GAS) was used to evaluate outcomes of surgical and non-surgical interventions to improve gait in 45 children with diplegic cerebral palsy. Personal goals were recorded during pre-intervention gait analysis in two groups. Twenty children underwent orthopaedic surgery (Group 1) and 25 children received a non-operative intervention (Group 2). Children and/or their carers were contacted post-intervention by telephone to complete a GAS questionnaire, rating the achievement of goals on a 5-point scale. The goals were similar in both groups. The composite GAS was transformed into a standardised measure (T-score) for each patient. Both groups on average achieved their goals (mean T-score for Group 2 was 56.3, versus 47.1 for Group 1). The difference between these two means was significant (p=0.010). Additionally, 16 children had undergone a follow-up gait analysis during the study period, but the relationship between their Gait Profile Score and GAS was not statistically significant.Both surgical and non-surgical interventions enabled children to achieve their goals, although Group 1 reported higher achievements. The GAS reflects patient's/parent's/carer's aspirations and may be as relevant as post-intervention kinematic or kinetic outcomes.



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Changes in proprioceptive weighting during quiet standing in women with early and established knee osteoarthritis compared to healthy controls

Publication date: February 2016
Source:Gait & Posture, Volume 44
Author(s): Armaghan Mahmoudian, Jaap H. van Dieen, Isabel A.C. Baert, Ilse Jonkers, Sjoerd M. Bruijn, Frank P. Luyten, Gert S. Faber, Sabine M.P. Verschueren
ObjectivesKnee osteoarthritis (OA) is highly prevalent in people above the age of 60, and is typically associated with pain, stiffness, muscle weakness and proprioceptive deficits. Muscle-tendon vibration has been used to assess the spatial reweighting of proprioceptive input during standing. The current study aimed to investigate whether weighting of proprioceptive input is altered in patients with early and established knee OA compared to asymptomatic controls.MethodsThe upright posture of 27 participants with early OA, 26 with established OA, and 27 asymptomatic controls was perturbed by vibrating (frequency: 70Hz and amplitude: approximately 0.5mm) ankle muscles (i.e. tibialis anterior and triceps surae) and knee muscles (vastus medialis). Center of pressure displacements of the participants were recorded using a force plate.ResultsBoth patients with early and established OA were more sensitive to triceps surae vibration compared to their healthy peers (P<0.01 for both). No such difference was found for the vibration of tibialis anterior or vastus medialis muscles between patients with knee OA and healthy controls.ConclusionsThese results suggest that the early stages of knee OA may already lead to reweighting of proprioceptive information, suggesting more reliance on ankle proprioceptive input for postural control.



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Standardized Patients: Potential to Improve Student Training in Aural Rehabilitation

Hearing loss is a growing public health concern. The multifaceted consequences of hearing loss are far reaching and include reduced audibility, well-being, and quality-of-life. Audiologists require specific training to address the wide range of hearing loss (re)habilitation needs, including some of the psychosocial needs of patients. One instructional method that might provide valuable training for the next generation of clinicians is the use of simulated or standardized patients (SPs). Standardized patients (SPs) are actors who are recruited, trained, and typically paid to portray patients, family members, or healthcare team members in a standardized manner for purposes of training healthcare professionals. Advantages to using SPs as an instructional technique were identified through a review of the literature and through discussion with two focus groups of experienced educators in the field of audiology. Advantages included those inherent to SP scenarios (e.g., standardization, authenticity, and safety), as well as those inherent to the experience (e.g., student reflection, comprehensive feedback, etc.). Benefits to students can include increased student confidence and improved interpersonal skills. Although time and resource investments necessary for SP case implementation can be prohibitive for some programs, creative instructors can harness many of the benefits using alternative implementations of SP cases.

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Vocal Performance Teachers and Hearing Loss

Hearing thresholds and distortion product otoacoustic emissions were measured for teachers of vocal performance who were gathered for a national conference. Results showed mean audiometric thresholds to be consistent with noise induced hearing loss, more than what would be expected with normal aging. Years of instruction and age were considered as factors in the hearing loss observed. It was concluded that hearing conservation should be initiated with this group to help raise awareness and protect them from hearing loss due to occupational noise exposure.

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Editor's Column

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SIG 8 Perspectives Vol. 16, No. 1, November 2015: Earn 0.10 CEUs on This Issue

Download the CE Questions PDF from the toolbar, above. Use the questions to guide your Perspectives reading. When you're ready, purchase the activity from the ASHA Store and follow the instructions to take the exam in ASHA's Learning Center. Available until November 21, 2018.

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Tinnitus Suicide

Tinnitus can be a difficult condition to live with because it can impact you on a daily basis and prevent you from leading a normal life. The most basic definition of this condition is a ringing sound in the ears, but it can also be heard as static, buzzing, screeching, roaring, hissing or other sounds. For some, the sound may be quiet and intermittent. For others, it may be constant and loud. This can interfere with your ability to sleep, work and participate in other normal activities. Because it can have a dramatic impact on your quality of life, you may wonder if there is a tinnitus suicide link.

Depression and Tinnitus
Depression is common in those with tinnitus. Because depression is one of the leading causes of suicide, there is in indirect tinnitus suicide link. Depression commonly develops with those who have this condition because it can make sleep difficult, and insomnia can contribute to the development of depression. Tinnitus can also make you feel isolated and alone, and it can make it difficult for you to participate in activities that you once enjoyed before you developed this condition. If you have tinnitus, you should be aware of the potential for depression to develop and then you can take steps to minimize this risk.

How to Cope With Tinnitus
There are several steps that you can take to reduce the severity of tinnitus and to improve your quality of life. For example, some people who are concerned about the tinnitus suicide link or the tinnitus depression link may ease their symptoms significantly by meditating. This can retrain the brain to focus on other things besides the sound. Another effective solution that many benefit from is tonal therapy. This can also train the brain to focus on other sounds and can make the sound appear to be quieter.

Whether you have a minor or severe case of tinnitus, this can be a challenging condition to live with. It can impact all aspects of your life on a daily basis, and you may struggle to complete even regular activities or to have conversations without it interfering. More than that, it can make it difficult to relax and unwind or even to sleep. If you are struggling with this condition, take the time to learn about the treatments available that can improve your symptoms. It’s recommended that you speak with an auditory specialist to learn more about treatments that m

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Effects of Steady-State Noise on Verbal Working Memory in Young Adults

Purpose

We set out to examine the impact of perceptual, linguistic, and capacity demands on performance of verbal working-memory tasks. The Ease of Language Understanding model (Rönnberg et al., 2013) provides a framework for testing the dynamics of these interactions within the auditory-cognitive system.

Methods

Adult native speakers of English (n = 45) participated in verbal working-memory tasks requiring processing and storage of words involving different linguistic demands (closed/open set). Capacity demand ranged from 2 to 7 words per trial. Participants performed the tasks in quiet and in speech-spectrum-shaped noise. Separate groups of participants were tested at different signal-to-noise ratios. Word-recognition measures were obtained to determine effects of noise on intelligibility.

Results

Contrary to predictions, steady-state noise did not have an adverse effect on working-memory performance in every situation. Noise negatively influenced performance for the task with high linguistic demand. Of particular importance is the finding that the adverse effects of background noise were not confined to conditions involving declines in recognition.

Conclusions

Perceptual, linguistic, and cognitive demands can dynamically affect verbal working-memory performance even in a population of healthy young adults. Results suggest that researchers and clinicians need to carefully analyze task demands to understand the independent and combined auditory-cognitive factors governing performance in everyday listening situations.

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From the Editor: An Introduction to the JSLHR Supplement on Implementation Science

Purpose

The JSLHR Supplement on Implementation Science is aimed at providing discussion and examples of research in implementation science, the study of methods designed to promote the incorporation of research findings into clinical practice.

Methods

Practitioners in the language science area were invited to submit articles that address their experience with various aspects of implementation science.

Results

Six articles from several research groups comprise this supplement.

Conclusions

Implementation science is an aspect of intervention research that merits consideration by communication disorders scientists. More extensive practice of implementation science will improve uptake of evidence-based practice in the clinical community.

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Perception of 'mothers of beneficiaries' regarding a rural community based hearing screening service.

Related Articles

Perception of 'mothers of beneficiaries' regarding a rural community based hearing screening service.

Int J Pediatr Otorhinolaryngol. 2014 Dec;78(12):2083-8

Authors: Akilan R, Vidya R, Roopa N

Abstract
UNLABELLED: A rural community-based hearing screening project was established in villages in a rural district of Tamil Nadu in South India. The goal of this project was to address early detection of hearing loss among infants and young children. Village health workers (VHW) were trained to conduct hearing screenings using an Oto Acoustic Emissions (OAE) equipment. They were also trained to provide information about ear and hearing health, and to facilitate follow up visits for diagnostic testing when required.
OBJECTIVES: The purpose of this study was to review the project by examining the caregiver perception from the mothers of children who have undergone hearing screening regarding the service provided.
METHODS: Focus group discussions (FGDs), were conducted in nine villages of the district to obtain information and perceptions from mothers. In all, 70 mothers with children less than 2 years of age, and 13 mothers with children greater than 2 years of age, participated in the FGDs.
RESULTS: Responses obtained from mothers indicated that door to door health services are rare and are primarily related to sensitizing the community regarding health camps and preventive measures for widespread diseases (like dengue fever). Door to door screening for hearing among children is unique in these villages. Mothers were familiar with the NGO which coordinated the hearing screening program. Local pre-school (Balwadi) teachers were informed about the hearing screening program and its significance. From the responses of the participants it was clear that the sensitization carried out through them in all villages was successful. It was noteworthy that mothers mentioned the result of screening as "pass/refer" as instead of "pass/fail". This outcome suggests that health workers have used appropriate terminology to convey screening results. Mothers reported test conditions to be present and therefore confirmed that valid testing was conducted by VHWs.
CONCLUSIONS: Mothers in the community accepted hearing screening services delivered by health workers. The health workers were effective in delivering the services. Pre-school teachers seemed to have played a pivotal role in communicating about the hearing screening program to the mothers. Ultimately, collaborating with local NGO facilitated acceptance and compliance due to the NGOs strong presence in the community.

PMID: 25288387 [PubMed - indexed for MEDLINE]

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Delayed low frequency hearing loss caused by cochlear implantation interventions via the round window but not cochleostomy

Publication date: Available online 29 December 2015
Source:Hearing Research
Author(s): David Rowe, Scott Chambers, Amy Hampson, Hayden Eastwood, Luke Campbell, Stephen O’Leary
Cochlear implant recipients show improved speech perception and music appreciation when residual acoustic hearing is combined with the cochlear implant. However, up to one third of patients lose their pre-operative residual hearing weeks to months after implantation, for reasons that are not well understood. This study tested whether this “delayed” hearing loss was influenced by the route of electrode array insertion and/or position of the electrode array within scala tympani in a guinea pig model of cochlear implantation. Five treatment groups were monitored over 12 weeks: (1) round window implant; (2) round window incised with no implant; (3) cochleostomy with medially-oriented implant; (4) cochleostomy with laterally-oriented implant; and (5) cochleostomy with no implant. Hearing was measured at selected time points by the auditory brainstem response. Cochlear condition was assessed histologically, with cochleae three-dimensionally reconstructed to plot electrode paths and estimate tissue response. Electrode array trajectories matched their intended paths. Arrays inserted via the round window were situated nearer to the basilar membrane and organ of Corti over the majority of their intrascalar path compared with arrays inserted via cochleostomy. Round window interventions exhibited delayed, low frequency hearing loss that was not seen after cochleostomy. This hearing loss appeared unrelated to the extent of tissue reaction or injury within scala tympani, although round window insertion was histologically the most traumatic mode of implantation. We speculate that delayed hearing loss was related not to the electrode position as postulated, but rather to the muscle graft used to seal the round window post-intervention, by altering cochlear mechanics via round window fibrosis.



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Research Supported by the Tinnitus Research Consortium

Publication date: Available online 29 December 2015
Source:Hearing Research
Author(s): Allen F. Ryan, Robert A. Dobie, Judy R. Dubno, Brenda L. Lonsbury-Martin




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