Predictors and Pathophysiology of Axial Postural Abnormalities in Parkinsonism: A Scoping Review.

Background: Postural abnormalities involving the trunk are referred to as axial postural abnormalities and can be observed in over 20% of patients with Parkinson's disease (PD) and in atypical parkinsonism. These symptoms are highly disabling and frequently associated with back pain and a worse quality of life in PD. Despite their frequency, little is known about the pathophysiology of these symptoms and scant data are reported about their clinical predictors, making it difficult to prompt prevention strategies. Objectives: We conducted a scoping literature review of clinical predictors and pathophysiology of axial postural abnormalities in patients with parkinsonism to identify key concepts, theories and evidence on this topic. Methods: We applied a systematic approach to identify studies, appraise quality of evidence, summarize main findings, and highlight knowledge gaps. Results: Ninety-two articles were reviewed: 25% reported on clinical predictors and 75% on pathophysiology. Most studies identified advanced disease stage and greater motor symptoms severity as independent clinical predictors in both PD and multiple system atrophy. Discrepant pathophysiology data suggested different potential central and peripheral pathogenic mechanisms. Conclusions: The recognition of clinical predictors and pathophysiology of axial postural abnormalities in parkinsonism is far from being elucidated due to literature bias, encompassing different inclusion criteria and measurement tools and heterogeneity of patient samples. Most studies identified advanced disease stage and higher burden of motor symptoms as possible clinical predictors. Pathophysiology data point toward many different (possibly non-mutually exclusive) mechanisms, including dystonia, rigidity, proprioceptive and vestibular impairment, and higher cognitive deficits.

Task Force Consensus on Nosology and Cut-Off Values for Axial Postural Abnormalities in Parkinsonism.

Background: There is no consensus with regard to the nosology and cut-off values for postural abnormalities in parkinsonism. Objective: To reach a consensus regarding the nosology and cut-off values. Methods: Using a modified Delphi panel method, multiple rounds of questionnaires were conducted by movement disorder experts to define nosology and cut-offs of postural abnormalities. Results: After separating axial from appendicular postural deformities, a full agreement was found for the following terms and cut-offs: camptocormia, with thoracic fulcrum (>45°) or lumbar fulcrum (>30°), Pisa syndrome (>10°), and antecollis (>45°). "Anterior trunk flexion," with thoracic (≥25° to ≤45°) or lumbar fulcrum (>15° to ≤30°), "lateral trunk flexion" (≥5° to ≤10°), and "anterior neck flexion" (>35° to ≤45°) were chosen for milder postural abnormalities. Conclusions: For axial postural abnormalities, we recommend the use of proposed cut-offs and six unique terms, namely camptocormia, Pisa syndrome, antecollis, anterior trunk flexion, lateral trunk flexion, anterior neck flexion, to harmonize clinical practice and future research.

Targeted Re-Instruction for Hearing Aid Use and Care Skills.

Purpose The purpose of this study was to determine if re-instructing a hearing aid (HA) user on the specific skill(s) they could not successfully perform on the Practical Hearing Aid Skills Test-Revised (PHAST-R) resulted in better HA use and care skills than a traditional fitting method. This is referred to as "targeted re-instruction." Also, factors that might affect HA use and care skills, including memory function, hearing handicap, and hearing aid-related attitudes, were assessed. Method This study was a randomized control trial. Twenty-six new HA users (13 experimental; 13 control) were fit with HAs and provided a standard HA orientation. HA use and care skills were measured immediately following the HA fitting and then again at 4 weeks post-HA fitting. The experimental group was administered the PHAST-R twice during the trial period to identify the HA use and care skills that participants needed re-instruction on. Results Participants in the experimental group maintained their HA use and care skills over the 4-week HA trial period, whereas participants in the control group showed a significant decline. Working memory, hearing handicap, and HA-related attitudes were not found to be correlated with HA use and care skills. Conclusions Participants who did not receive targeted re-instruction showed a decline in their HA use and care skills after only 4 weeks of HA use, whereas targeted re-instruction prevented a decline in HA use and care skills. It took an average of less than 10 min to administer the PHAST-R and provide targeted re-instruction, indicating that it would be appropriate to use in a clinical setting.

Use of a Mild-Gain Hearing Aid by Middle-Age Normal-Hearing Adults Who Do and Do Not Self-Report Trouble Hearing in Background Noise.

Purpose The aim of the study was to assess how the use of a mild-gain hearing aid can affect hearing handicap, motivation, and attitudes toward hearing aids for middle-age, normal-hearing adults who do and do not self-report trouble hearing in background noise. Method A total of 20 participants (45-60 years of age) with clinically normal-hearing thresholds (< 25 dB HL) were enrolled in this study. Ten self-reported difficulty hearing in background noise, and 10 did not self-report difficulty hearing in background noise. All participants were fit with mild-gain hearing aids, bilaterally, and were asked to wear them for 2 weeks. Hearing handicap, attitudes toward hearing aids and hearing loss, and motivation to address hearing problems were evaluated before and after participants wore the hearing aids. Participants were also asked if they would consider purchasing a hearing aid before and after 2 weeks of hearing aid use. Results After wearing the hearing aids for 2 weeks, hearing handicap scores decreased for the participants who self-reported difficulty hearing in background noise. No changes in hearing handicap scores were observed for the participants who did not self-report trouble hearing in background noise. The participants who self-reported difficulty hearing in background noise also reported greater personal distress from their hearing problems, were more motivated to address their hearing problems, and had higher levels of hearing handicap compared to the participants who did not self-report trouble hearing in background noise. Only 20% (2/10) of the participants who self-reported trouble hearing in background noise reported that they would consider purchasing a hearing aid after 2 weeks of hearing aid use. Conclusions The use of mild-gain hearing aids has the potential to reduce hearing handicap for normal-hearing, middle-age adults who self-report difficulty hearing in background noise. However, this may not be the most appropriate treatment option for their current hearing problems given that only 20% of these participants would consider purchasing a hearing aid after wearing hearing aids for 2 weeks.

The Effect of Memory Span and Manual Dexterity on Hearing Aid Handling Skills in New and Experienced Hearing Aid Users.

Purpose The aim of the study was to determine the effect of memory function and manual dexterity on new and experienced hearing aid users' abilities to use and care for their hearing aids. Method New and experienced hearing aid users were administered the Practical Hearing Aid Skills Test-Revised (PHAST-R; Doherty & Desjardins, 2012 ), a measure of a hearing aid user's ability to use and care for their hearing aids. The test was administered during their 30-day hearing aid check or yearly hearing evaluation appointment. Participants were also administered the Digit Span Test of memory function ( Wechsler, 1997 ) and the Nine-Hole Peg Test of manual dexterity ( Mathiowetz, Weber, Kashman, & Volland,1985 ). Results Participants with poorer memory span function performed significantly poorer on the PHAST-R than participants with better memory span. However, no significant relationship between manual dexterity and PHAST-R performance was observed. Experienced hearing aid users who were recently reoriented on how to use and care for their hearing aids performed significantly better on the PHAST-R compared to new hearing aid users and experienced hearing aid users who had not received a hearing aid orientation within the last year. Cleaning the hearing aid and telephone use were the 2 PHAST-R tasks that all hearing aid clients needed the most recounseling on. Conclusion Memory span is significantly related to an individual's ability to correctly use and care for their hearing aids regardless of whether they are new or experienced hearing aid users.

Motivation to Address Self-Reported Hearing Problems in Adults With Normal Hearing Thresholds.

Purpose: The purpose of this study was to compare the motivation to change in relation to hearing problems in adults with normal hearing thresholds but who report hearing problems and that of adults with a mild-to-moderate sensorineural hearing loss. Factors related to their motivation were also assessed. Method: The motivation to change in relation to self-reported hearing problems was measured using the University of Rhode Island Change Assessment (McConnaughy, Prochaska, & Velicer, 1983). The relationship between objective and subjective measures and an adult's motivation was examined. Results: The level of hearing handicap did not differ significantly between adults with normal hearing who reported problems hearing in background noise and adults who had a mild-to-moderate sensorineural hearing loss. Hearing handicap, personal distress, and minimization of hearing loss were factors significantly related to motivation. Age, degree of hearing loss, speech-in-noise scores, working memory, and extended high-frequency average thresholds were not significantly related to their motivation. Conclusions: Adults with normal hearing thresholds but self-reported hearing problems had the same level of hearing handicap and were equally motivated to take action for their hearing problems as age-matched adults with a mild-to-moderate sensorineural hearing loss. Hearing handicap, personal distress, and minimization of hearing loss were most strongly correlated with an individual's motivation to change.

Changes in Psychosocial Measures After a 6-Week Field Trial.

PURPOSE: The purpose of this study was to assess the extent to which intervention with hearing aids, namely, a 6-week hearing aid field trial, can minimize the psychosocial consequences of hearing loss in adults who have previously not sought treatment for their hearing loss. METHOD: Twenty-four adults with mild to moderate bilateral sensorineural hearing loss, who had never worn hearing aids or sought help for their hearing loss, participated in this study. Participants were fitted with receiver-in-canal hearing aids, bilaterally, and wore them for 6 weeks. Participants completed subjective measures of hearing handicap and attitudes about hearing loss and hearing aids before, during, and after the hearing aid trial. A control group of age-matched participants followed the same experimental protocol, except they were not fitted with hearing aids. RESULTS: Using hearing aids for 6 weeks significantly reduced participants' perceived stigma of hearing aids, personal distress and inadequacy due to hearing difficulties, and hearing handicap. CONCLUSIONS: A hearing aid trial can have a positive effect on a person's attitudes toward wearing hearing aids and decrease hearing handicap.

A Rare Cause of Myoclonus: A Cupric Conundrum.

A woman aged 22 years presented with a 3-year history of jerks when brushing her teeth and a tremor when carrying drinks. Examination revealed a bilateral jerky tremor, stimulus-sensitive myoclonus, and difficulty with tandem gait. Thyroid and liver function test results were normal, but she had rapidly progressive renal failure. Serum copper, ceruloplasmin, and manganese levels were normal, but her urinary copper level was elevated on 2 occasions. Pathological findings on organ biopsy prompted genetic testing to confirm the diagnosis. The differential diagnosis, tissue biopsy findings, and final genetic diagnosis are discussed.

Deletions at 22q11.2 in idiopathic Parkinson's disease: a combined analysis of genome-wide association data.

BACKGROUND: Parkinson's disease has been reported in a small number of patients with chromosome 22q11.2 deletion syndrome. In this study, we screened a series of large, independent Parkinson's disease case-control studies for deletions at 22q11.2. METHODS: We used data on deletions spanning the 22q11.2 locus from four independent case-control Parkinson's disease studies (UK Wellcome Trust Case Control Consortium 2, Dutch Parkinson's Disease Genetics Consortium, US National Institute on Aging, and International Parkinson's Disease Genomics Consortium studies), which were independent of the original reports of chromosome 22q11.2 deletion syndrome. We did case-control association analysis to compare the proportion of 22q11.2 deletions found, using the Fisher's exact test for the independent case-control studies and the Mantel-Haenszel test for the meta-analyses. We retrieved clinical details of patients with Parkinson's disease who had 22q11.2 deletions from the medical records of these patients. FINDINGS: We included array-based copy number variation data from 9387 patients with Parkinson's disease and 13 863 controls. Eight patients with Parkinson's disease and none of the controls had 22q11.2 deletions (p=0·00082). In the 8451 patients for whom age at onset data were available, deletions at 22q11.2 were associated with Parkinson's disease age at onset (Mann-Whitney U test p=0·001). Age at onset of Parkinson's disease was lower in patients carrying a 22q11.2 deletion (median 37 years, 95% CI 32·0-55·5; mean 42·1 years [SD 11·9]) than in those who did not carry a deletion (median 61 years, 95% CI 60·5-61·0; mean 60·3 years [SD 12·8]). A 22q11.2 deletion was present in more patients with early-onset (p<0·0001) and late-onset Parkinson's disease (p=0·016) than in controls, and in more patients with early-onset than late-onset Parkinson's disease (p=0·005). INTERPRETATION: Clinicians should be alert to the possibility of 22q11.2 deletions in patients with Parkinson's disease who have early presentation or features associated with the chromosome 22q11.2 deletion syndrome, or both. FUNDING: UK Medical Research Council, UK Wellcome Trust, Parkinson's UK, Patrick Berthoud Trust, National Institutes of Health, "Investissements d'Avenir" ANR-10-IAIHU-06, Dutch Parkinson Foundation (Parkinson Vereniging), Neuroscience Campus Amsterdam, National Institute for Health Research, National Institute on Aging, National Institutes of Health.

The effect of presentation level on spectral weights for sentences.

Psychophysical data indicate that spectral weights tend to increase with increasing presentation level at high frequencies. The present study examined whether spectral weights for speech perception are similarly affected by presentation level. Stimuli were sentences filtered into five contiguous frequency bands and presented at each of two levels (75 and 95 dB sound pressure level [SPL]). For the highest band (2807-10,000 Hz), normal-hearing listeners' weights were higher for the higher presentation level. Weights for the 95-dB-SPL level resembled those previously estimated for hearing-impaired listeners tested at comparably high levels, suggesting that hearing loss itself may not play a large role in spectral weighting for a sentence recognition task.

The benefit of amplification on auditory working memory function in middle-aged and young-older hearing impaired adults.

Untreated hearing loss can interfere with an individual's cognitive abilities and intellectual function. Specifically, hearing loss has been shown to negatively impact working memory function, which is important for speech understanding, especially in difficult or noisy listening conditions. The purpose of the present study was to assess the effect of hearing aid use on auditory working memory function in middle-aged and young-older adults with mild to moderate sensorineural hearing loss. Participants completed two objective measures of auditory working memory in aided and unaided listening conditions. An aged matched control group followed the same experimental protocol except they were not fit with hearing aids. All participants' aided scores on the auditory working memory tests were significantly improved while wearing hearing aids. Thus, hearing aids worn during the early stages of an age-related hearing loss can improve a person's performance on auditory working memory tests.

Cues for Diotic and Dichotic Detection of a 500-Hz Tone in Noise Vary with Hearing Loss.

Hearing in noise is a challenge for all listeners, especially for those with hearing loss. This study compares cues used for detection of a low-frequency tone in noise by older listeners with and without hearing loss to those of younger listeners with normal hearing. Performance varies significantly across different reproducible, or "frozen," masker waveforms. Analysis of these waveforms allows identification of the cues that are used for detection. This study included diotic (N0S0) and dichotic (N0Sπ) detection of a 500-Hz tone, with either narrowband or wideband masker waveforms. Both diotic and dichotic detection patterns (hit and false alarm rates) across the ensembles of noise maskers were predicted by envelope-slope cues, and diotic results were also predicted by energy cues. The relative importance of energy and envelope cues for diotic detection was explored with a roving-level paradigm that made energy cues unreliable. Most older listeners with normal hearing or mild hearing loss depended on envelope-related temporal cues, even for this low-frequency target. As hearing threshold at 500 Hz increased, the cues for diotic detection transitioned from envelope to energy cues. Diotic detection patterns for young listeners with normal hearing are best predicted by a model that combines temporal- and energy-related cues; in contrast, combining cues did not improve predictions for older listeners with or without hearing loss. Dichotic detection results for all groups of listeners were best predicted by interaural envelope cues, which significantly outperformed the classic cues based on interaural time and level differences or their optimal combination.

The NTID speech recognition test: NSRT(®).

OBJECTIVE: The purpose of this study was to collect and analyse data necessary for expansion of the NSRT item pool and to evaluate the NSRT adaptive testing software. DESIGN: Participants were administered pure-tone and speech recognition tests including W-22 and QuickSIN, as well as a set of 323 new NSRT items and NSRT adaptive tests in quiet and background noise. Performance on the adaptive tests was compared to pure-tone thresholds and performance on other speech recognition measures. The 323 new items were subjected to Rasch scaling analysis. STUDY SAMPLE: Seventy adults with mild to moderately severe hearing loss participated in this study. Their mean age was 62.4 years (sd = 20.8). RESULTS: The 323 new NSRT items fit very well with the original item bank, enabling the item pool to be more than doubled in size. Data indicate high reliability coefficients for the NSRT and moderate correlations with pure-tone thresholds (PTA and HFPTA) and other speech recognition measures (W-22, QuickSIN, and SRT). CONCLUSION: The adaptive NSRT is an efficient and effective measure of speech recognition, providing valid and reliable information concerning respondents' speech perception abilities.

Bone health in Parkinson's disease: a systematic review and meta-analysis.

OBJECTIVE: Parkinson's disease (PD) and osteoporosis are chronic diseases associated with increasing age. Single studies have reported associations between them and the major consequence, namely, increased risk of fractures. The aim of this systematic review and meta-analysis was to evaluate the relationship of PD with osteoporosis, bone mineral density (BMD) and fracture risk. METHODS: A literature search was undertaken on 4 September 2012 using multiple indexing databases and relevant search terms. Articles were screened for suitability and data extracted where studies met inclusion criteria and were of sufficient quality. Data were combined using standard meta-analysis methods. RESULTS: 23 studies were used in the final analysis. PD patients were at higher risk of osteoporosis (OR 2.61; 95% CI 1.69 to 4.03) compared with healthy controls. Male patients had a lower risk for osteoporosis and osteopenia than female patients (OR 0.45; 95% CI 0.29 to 0.68). PD patients had lower hip, lumbar spine and femoral neck BMD levels compared with healthy controls; mean difference, -0.08, 95% CI -0.13 to -0.02 for femoral neck; -0.09, 95% CI -0.15 to -0.03 for lumbar spine; and -0.05, 95% CI -0.07 to -0.03 for total hip. PD patients were also at increased risk of fractures (OR 2.28; 95% CI 1.83 to 2.83). CONCLUSIONS: This systematic review and meta-analysis demonstrate that PD patients are at higher risk for both osteoporosis and osteopenia compared with healthy controls, and that female patients are at greater risk than male patients. Patients with PD also have lower BMD and are at increased risk of fractures.