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 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.

The effect of hearing aid noise reduction on listening effort in hearing-impaired adults.

OBJECTIVES: The purpose of the present study was to evaluate the effect of a noise-reduction (NR) algorithm on the listening effort hearing-impaired participants expend on a speech in noise task. DESIGN: Twelve hearing-impaired listeners fitted with behind-the-ear hearing aids with a fast-acting modulation-based NR algorithm participated in this study. A dual-task paradigm was used to measure listening effort with and without the NR enabled in the hearing aid. The primary task was a sentence-in-noise task presented at fixed overall speech performance levels of 76% (moderate listening condition) and 50% (difficult listening condition) correct performance, and the secondary task was a visual-tracking test. Participants also completed measures of working memory (Reading Span test), and processing speed (Digit Symbol Substitution Test) ability. RESULTS: Participants' speech recognition in noise scores did not significantly change with the NR algorithm activated in the hearing aid in either listening condition. The NR algorithm significantly decreased listening effort, but only in the more difficult listening condition. Last, there was a tendency for participants with faster processing speeds to expend less listening effort with the NR algorithm when listening to speech in background noise in the difficult listening condition. CONCLUSIONS: The NR algorithm reduced the listening effort adults with hearing loss must expend to understand speech in noise.

Pisa syndrome in Parkinson's disease: a mobile or fixed deformity?

BACKGROUND: Although Pisa syndrome and scoliosis are sometimes used interchangeably to describe a laterally flexed postural deviation in Parkinson's disease (PD), the imaging findings of Pisa syndrome in PD have not been previously studied in detail. METHODS: Patients with PD and Pisa syndrome (lateral flexion >10° in the standing position) were examined clinically and underwent radiological assessment using standing radiograph and supine CT scan of the whole spine. RESULTS: Fifteen patients were included in this observational study. All patients had scoliosis on standing radiographs, and 12 had scoliosis persisting in the supine position. Scoliotic curves improved by a mean of 44% when patients moved from standing to supine. Only a quarter of patients with structural scoliosis had evidence of bony fusion on the side of their lateral deviation rendering their deformity fixed. CONCLUSIONS: Pisa syndrome describes a patient who lists to the side whereas scoliosis is defined by spinal curvature and rotation and may not be associated with lateral flexion. The finding of 'structural scoliosis' in Pisa syndrome should not preclude intervening to improve posture as most patients had little or no evidence of structural bony changes even when the deformity had been present for a number of years.

Primary progressive aphasia with parkinsonism.

A 65-year-old man presented with word-finding difficulty and gait disturbance. His speech was nonfluent with word retrieval impairment and difficulties with sentence repetition. Other cognitive domains were intact initially. He developed asymmetrical bradykinesia, rigidity and a rest tremor. Over the following 8 years, his speech production impairment slowly deteriorated with the development of a motor speech disorder, anomia, impaired repetition of single words as well as sentences, and impaired comprehension of initially sentences then single words. His parkinsonian syndrome also deteriorated with limited response to levodopa. Serial brain MRI revealed progressive asymmetric perisylvian atrophy. He died after a disease duration of 12 years. The clinical syndrome is discussed by an expert, the pathology is described, and important clinical points from the case are highlighted.

Age-related changes in listening effort for various types of masker noises.

OBJECTIVE: The purpose of the present study was to evaluate the relationship between cognitive function, listening effort, and speech recognition for a group of younger and older adults with normal hearing and a group of older adults with hearing impairment in various types of background maskers. The authors hypothesize that, as the masker condition becomes more difficult listening effort will increase, but the increase will be greater for older participants than for younger participants. DESIGN: A dual-task paradigm was used to objectively evaluate listening effort. The primary task required participants to repeat sentences presented in three different background-masker conditions: (1) two-talker (TT), (2) six-talker, and (3) speech-shaped noise (SSN). The secondary task was a Digital Visual Pursuit Rotor Tracking test, for which participants were instructed to use a computer mouse to track a moving target around an ellipse that was displayed on a computer screen. Each of the two tasks was separately and concurrently presented at a fixed overall speech-recognition performance level of 76% correct. In addition, participants subjectively rated how easy it was to listen to the sentences in each masker condition on a scale from 0 (i.e., very difficult) to 100 (i.e., very easy). Last, participants completed a battery of cognitive tests that measured working memory (Reading Span Test), processing speed (Digit Symbol Substitution Test), and selective attention (Stroop Test) ability. RESULTS: Results revealed that participants' working memory and processing speed abilities were significantly related to their speech-recognition performance in noise in all three background-masker conditions. Participants rated the TT condition to be the most difficult listening condition and the SSN condition to be the easiest listening condition. Both groups of older participants expended significantly more listening effort than younger participants did in the SSN and TT masker conditions. For each group of participants, there were no significant differences in listening effort measured across the masker conditions, with the exception of the younger participants, who expended more effort listening in the six-talker masker condition compared with the SSN condition. Participants' listening effort expended on the TT and SSN masker conditions was significantly correlated with their working memory and processing speed performance. CONCLUSIONS: Findings from the present study indicate that older adults require more cognitive resources than younger adults to understand speech in background noise.

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.

Decision making, impulsivity, and addictions: do Parkinson's disease patients jump to conclusions?

Links between impulsive-compulsive behaviors (ICBs) in treated Parkinson's disease (PD), behavioral addictions, and substance abuse have been postulated, but no direct comparisons have been carried out so far. We directly compared patients with PD with and without ICBs with illicit drug abusers, pathological gamblers, and age-matched healthy controls using the beads task, a test of reflection impulsivity, and a working memory task. We found that all patients with PD made more impulsive and irrational choices than the control group. PD patients who had an ICB showed similar behavior to illicit substance abusers, whereas patients without ICBs more closely resembled pathological gamblers. In contrast, we found no difference in working memory performance within the PD groups. However, PD patients without ICBs remembered distractors significantly less than all other patients during working memory tests. We were able to correctly classify 96% of the PD patients with respect to whether or not they had an ICB by analyzing three trials of the 80/20 loss condition of the beads task with a negative prediction value of 92.3%, and we propose that this task may prove to be a powerful screening tool to detect an ICB in PD. Our results also suggest that intact cortical processing and less distractibility in PD patients without ICBs may protect them from developing behavioral addictions.

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.

Globular glial tauopathies (GGT) presenting with motor neuron disease or frontotemporal dementia: an emerging group of 4-repeat tauopathies.

A number of recent studies have described cases with tau-positive globular oligodendroglial inclusions (GOIs) and such cases have overlapping pathological features with progressive supranuclear palsy (PSP), but present with clinical features of motor neuron disease (MND) and/or frontotemporal dementia (FTD). These two clinical phenotypes have been published independently and as a result, have come to be considered as distinct disease entities. We describe the clinicopathological and biochemical features of two cases with GOIs: one with clinical symptoms suggestive of MND and the other with FTD. Histological changes in our two cases were consistent with their clinical symptoms; the MND case had severe neurodegeneration in the primary motor cortex and corticospinal tract, whereas the FTD case had severe involvement of the frontotemporal cortices and associated white matter. Immunohistochemistry in both cases revealed significant 4-repeat (4R) tau pathology primarily in the form of GOIs, but also in astrocytes and neurons. Astrocytic tau pathology was morphologically similar to that seen in PSP, but in contrast was consistently negative for Gallyas silver staining. Tau-specific western blotting revealed 68, 64 and 35 kDa bands, showing further overlap with PSP. The underlying neuropathological features of these two cases were similar, with the major difference relating to the regional distribution of pathology and resulting clinical symptoms and signs. The globular nature of glial inclusions and the non-fibrillar properties of tau in astrocytes are characteristic features that allow them to be distinguished from PSP and other tauopathies. We, therefore, propose the term globular glial tauopathy as an encompassing term to classify this emerging class of 4R tauopathy.

Decades of delayed diagnosis in 4 levodopa-responsive young-onset monogenetic parkinsonism patients.

BACKGROUND: We report 4 patients with young-onset monogenetic parkinsonism, each of whom was misdiagnosed with either a psychogenic movement disorder or chronic fatigue syndrome for 10 to 23 years after the onset of their first symptoms. RESULTS: Once the diagnosis was eventually made, they all had a rapid and excellent response to levodopa, albeit with the early appearance of interdose dyskinesias in 3. CONCLUSIONS: We discuss possible reasons for the missed diagnosis despite the relentless progression of their motor handicap. DAT scanning supported the revised clinical diagnosis of parkinsonism. © 2011 Movement Disorder Society.

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.