Hearing loss and auditory processing ability in people with aphasia

BACKGROUND: Hearing loss can add to the linguistic deficits present in aphasia to make comprehension of speech difficult. Although some studies document a relatively high prevalence of hearing loss in adults with aphasia, many people with aphasia do not have their hearing tested. Self-reported disability measures offer a possible alternative to pure- tone audiometry when this service is not readily available. AIMS: This study aims to investigate the prevalence of hearing loss in a group of people with aphasia and to determine the usefulness of self-reported measures to screen for hearing impairment. METHODS AND PROCEDURES: Hearing ability was measured using pure-tone audiometry and five measures of auditory processing, which looked at speech perception in quiet and noise, for 21 individuals with aphasia recruited from a community clinic and 21 age- matched individuals without aphasia. The Speech, Spatial and Qualities of Hearing Scale (SSQ) and a brief questionnaire exploring whether they had experienced hearing difficulties were used to measure self-perception of hearing acuity. Differences in scores between the groups were analysed. Correlations and regressions were used to establish the relationship between self-perception of hearing and measures of hearing ability. OUTCOMES AND RESULTS: Despite minimal impairment and a non-significant difference between performance on pure-tone audiometry for participants with and without apha- sia, participants with aphasia performed significantly worse on measures of speech perception in noise than participants without aphasia. They also had a significantly greater degree of perceived hearing disability. Although SSQ scores were correlated with some behavioural measures for the participants with aphasia, the SSQ only predicted the hearing status and speech in noise performance of control participants. CONCLUSIONS: The results suggest that the prevalence of hearing loss for people with aphasia (at least for this group) is no greater than the general population. However, they are significantly more affected in their recognition of speech in noise and experience greater disability in listening situations than people without aphasia. The latter problems were not predicted by pure-tone audiograms or sound-in-noise performance. The brief questionnaire was not effective in identifying hearing impairment, indicating the need for a regular hearing screen to ensure provision of the most effective rehabilitation. Ideally, the screen should include disability and behavioural measures, as our results suggest they cannot replace each other. These findings should assist clinicians in setting realistic goals and delivering interventions in the most effective way for people with aphasia

Dementia assessment and management in primary care settings: a survey of current provider practices in the United States.

BACKGROUND:Primary care providers (PCPs) are typically the first to screen and evaluate patients for neurocognitive disorders (NCDs), including mild cognitive impairment and dementia. However, data on PCP attitudes and evaluation and management practices are sparse. Our objective was to quantify perspectives and behaviors of PCPs and neurologists with respect to NCD evaluation and management. METHODS:A cross-sectional survey with 150 PCPs and 50 neurologists in the United States who evaluated more than 10 patients over age 55 per month. The 51-item survey assessed clinical practice characteristics, and confidence, perceived barriers, and typical practices when diagnosing and managing patients with NCDs. RESULTS:PCPs and neurologists reported similar confidence and approaches to general medical care and laboratory testing. Though over half of PCPs performed cognitive screening or referred patients for cognitive testing in over 50% of their patients, only 20% reported high confidence in interpreting results of cognitive tests. PCPs were more likely to order CT scans than MRIs, and only 14% of PCPs reported high confidence interpreting brain imaging findings, compared to 70% of specialists. Only 21% of PCPs were highly confident that they correctly recognized when a patient had an NCD, and only 13% were highly confident in making a specific NCD diagnosis (compared to 72 and 44% for neurologists, both p < 0.001). A quarter of all providers identified lack of familiarity with diagnostic criteria for NCD syndromes as a barrier to clinical practice. CONCLUSIONS:This study demonstrates how PCPs approach diagnosis and management of patients with NCDs, and identified areas for improvement in regards to cognitive testing and neuroimaging. This study also identified all providers' lack of familiarity with published diagnostic criteria for NCD syndromes. These findings may inform the development of new policies and interventions to help providers improve the efficacy of their decision processes and deliver better quality care to patients with NCDs

Assessment of active tubulointerstitial nephritis in non-scarred renal cortex improves prediction of renal outcomes in patients with IgA nephropathy

Background: The addition of tubulointerstitial inflammation to the existing pathological classification of IgA nephropathy (IgAN) is appealing but was previously precluded due to reportedly wide inter-observer variability. We report a novel method to score percentage of non-atrophic renal cortex containing active tubulointerstitial inflammation (ATIN) in patients with IgAN and assess its utility to predict clinical outcomes. Methods: All adult patients with a native renal biopsy diagnosis of IgAN between 2010 and 2015 in a unit serving 1.5 million people were identified. Baseline characteristics, biopsy reports and outcome data were collected. ATIN was calculated by subtracting the percentage of atrophic cortex from the percentage of total cortex with tubulointerstitial inflammation, with ≥10% representing significant ATIN. The primary outcome was a composite of requiring renal replacement therapy or doubling of serum creatinine. Results: In total 153 new cases of IgAN were identified, of which 111 were eligible for inclusion. Of these, 76 (68%) were male and 54 (49%) had ATIN on biopsy. During a median follow-up of 2.3 years, 34 (31%) reached the primary outcome. On univariable Cox regression analysis, ATIN was associated with a five-fold increase in the primary outcome [hazard ratio (HR) (95% confidence interval) 4.9 (95% confidence interval (CI) 2.1–11.3)]. On multivariable analysis, mesangial hypercellularity, tubular atrophy and interstitial fibrosis and ATIN independently associated with renal outcome (P = 0.02 for ATIN). Inter-observer reproducibility revealed fair agreement in the diagnosis of ATIN (κ=0.43, P = 0.05). Conclusions: Within our centre, ATIN was significantly associated with renal outcome in patients with IgAN, independently of established histological features and baseline clinical characteristics

Empathic Accuracy Deficits in Patients with Neurodegenerative Disease: Association with Caregiver Depression.

OBJECTIVES:To investigate whether deficits in empathic accuracy (i.e., ability to recognize emotion in others) in patients with neurodegenerative disease are associated with greater depression in their caregivers. DESIGN:Two cross-sectional studies. SETTING:Academic medical center and research university. PARTICIPANTS:Two independent samples (N = 172, N = 63) of patients with a variety of neurodegenerative diseases and their caregivers; comparison group of healthy couples. MEASUREMENT:Patients' empathic accuracy was assessed in the laboratory using a novel dynamic tracking task (rating another person's changing emotions over time) and more traditional measures (recognizing the emotion expressed in photographs of facial expressions and by characters in films). Caregivers completed self-report inventories of depression. RESULTS:Lower empathic accuracy in patients was associated with greater depression in caregivers in both studies. In study 1, this association was found when empathic accuracy was measured using the dynamic tracking measure but not when measured using the more traditional photograph and film measures. In study 2, we found preliminary support for our theoretical model wherein lower empathic accuracy in patients is associated with increased caregiver stress (loneliness, strain, and burden), which in turn is associated with greater caregiver depression. CONCLUSIONS:Caring for a patient with deficits in empathic accuracy is associated with greater loneliness, strain, and burden for caregivers, and increased depression. Caregivers may benefit from interventions designed to compensate for the stress and interpersonal loss associated with patients' declining empathic accuracy

The 5-HTTLPR variant in the serotonin transporter gene modifies degeneration of brain regions important for emotion in behavioral variant frontotemporal dementia

AbstractThe serotonin transporter length polymorphism (5-HTTLPR) short allele (5-HTTLPR-s) has been associated with differential susceptibility for anxiety and depression in multiple psychiatric disorders. 5-HTTLPR-s modifies the serotonergic systems that support emotion and behavioral regulation by reducing gene expression, which slows the reuptake of serotonin, and is associated with distinct morphological and functional effects. Serotonergic systems are also shown to be dysfunctional in behavioral variant frontotemporal dementia (bvFTD), a disease characterized by marked socioemotional dysfunction. However, studies of 5-HTTLPR-s effects in bvFTD have been inconsistent. Our objective was to investigate the patterns of gray matter volume by 5-HTTLPR-s genotype in both healthy older controls and bvFTD patients. We performed voxel-based morphometry of 179 cognitively normal older adults and 24 bvFTD cases to determine brain changes associated with dose (0/1/2) of 5-HTTLPR-s allele. 5-HTTLPR-s frequency did not differ between controls and bvFTD. We found a significant interaction effect whereby carrying more 5-HTTLPR-s alleles in bvFTD was associated with smaller volume in left inferior frontal gyrus (T = 4.86, PFWE = 0.03) and larger volume in right temporal lobe (T = 5.01, PFWE = 0.01). These results suggest that the 5-HTTLPR-s allele differentially influences brain morphology in bvFTD. We propose that patients with bvFTD and 5-HTTLPR-s have altered volumes in regions that support socioemotional behavior, which may be a developmental or disease-related compensation for altered serotonergic activity

State and trait characteristics of anterior insula time-varying functional connectivity.

The human anterior insula (aINS) is a topographically organized brain region, in which ventral portions contribute to socio-emotional function through limbic and autonomic connections, whereas the dorsal aINS contributes to cognitive processes through frontal and parietal connections. Open questions remain, however, regarding how aINS connectivity varies over time. We implemented a novel approach combining seed-to-whole-brain sliding-window functional connectivity MRI and k-means clustering to assess time-varying functional connectivity of aINS subregions. We studied three independent large samples of healthy participants and longitudinal datasets to assess inter- and intra-subject stability, and related aINS time-varying functional connectivity profiles to dispositional empathy. We identified four robust aINS time-varying functional connectivity modes that displayed both "state" and "trait" characteristics: while modes featuring connectivity to sensory regions were modulated by eye closure, modes featuring connectivity to higher cognitive and emotional processing regions were stable over time and related to empathy measures

Emotion recognition in frontotemporal dementia and Alzheimer's disease: A new film-based assessment.

Deficits in recognizing others' emotions are reported in many psychiatric and neurological disorders, including autism, schizophrenia, behavioral variant frontotemporal dementia (bvFTD) and Alzheimer's disease (AD). Most previous emotion recognition studies have required participants to identify emotional expressions in photographs. This type of assessment differs from real-world emotion recognition in important ways: Images are static rather than dynamic, include only 1 modality of emotional information (i.e., visual information), and are presented absent a social context. Additionally, existing emotion recognition batteries typically include multiple negative emotions, but only 1 positive emotion (i.e., happiness) and no self-conscious emotions (e.g., embarrassment). We present initial results using a new task for assessing emotion recognition that was developed to address these limitations. In this task, respondents view a series of short film clips and are asked to identify the main characters' emotions. The task assesses multiple negative, positive, and self-conscious emotions based on information that is multimodal, dynamic, and socially embedded. We evaluate this approach in a sample of patients with bvFTD, AD, and normal controls. Results indicate that patients with bvFTD have emotion recognition deficits in all 3 categories of emotion compared to the other groups. These deficits were especially pronounced for negative and self-conscious emotions. Emotion recognition in this sample of patients with AD was indistinguishable from controls. These findings underscore the utility of this approach to assessing emotion recognition and suggest that previous findings that recognition of positive emotion was preserved in dementia patients may have resulted from the limited sampling of positive emotion in traditional tests

Neuropsychological, Behavioral, and Anatomical Evolution in Right Temporal Variant Frontotemporal Dementia: A Longitudinal Single Case Analysis

We examine longitudinal clinical and anatomical data for a patient with the right temporal variant of frontotemporal dementia. The patient received comprehensive clinical evaluations and structural MRI scans over three years. She presented with early behavioral deficits and ultimately developed semantic impairments consistent with the semantic variant of primary progressive aphasia. Imaging revealed early atrophy of the right temporal lobe, with later involvement of the left, and pathology confirmed bilateral temporal involvement. Findings support the view that right and left temporal variants reflect early asymmetry of atrophy that may become more bilateral over time, resulting in a mixed clinical picture

Anatomical correlates of early mutism in progressive nonfluent aphasia

Patients with progressive nonfluent aphasia (PNFA) can become mute early in the course of the disease. Voxel-based morphometry showed that PNFA is associated with left anterior insula and inferior frontal atrophy. In PNFA with early mutism, volume loss was more prominent in the pars opercularis and extended into the left basal ganglia. Damage to the network of brain regions involved in both coordination and execution of speech causes mutism in PNFA

The anatomy of category-specific object naming in neurodegenerative diseases

Neuropsychological studies suggest that knowledge about living and nonliving objects is processed in separate brain regions. However, lesion and functional neuroimaging studies have implicated different areas. To address this issue, we used voxel-based morphometry to correlate accuracy in naming line drawings of living and nonliving objects with gray matter volumes in 152 patients with various neurodegenerative diseases. The results showed a significant positive correlation between gray matter volumes in bilateral temporal cortices and total naming accuracy regardless of category. Naming scores for living stimuli correlated with gray matter volume in the medial portion of the right anterior temporal pole, whereas naming accuracy for familiarity-matched nonliving items correlated with the volume of the left posterior middle temporal gyrus. A previous behavioral study showed that the living stimuli used here also had in common the characteristic that they were defined by shared sensory semantic features, whereas items in the nonliving group were defined by their action-related semantic features. We propose that the anatomical segregation of living and nonliving categories is the result of their defining semantic features and the distinct neural subsystems used to process them