Lack of Association between Audiogram and Hearing Disability Measures in Mild Cognitive Impairment and Dementia: What Audiogram Does Not Tell You

(1) Introduction: The validity of self-reported hearing disability measures has been assessed using their correlation with the pure-tone average (PTA) hearing loss for non-cognitively impaired adults. However, for people with cognitive impairment, factors in addition to the PTA can play a role in their self-reported difficulties. Patients with cognitive impairment may experience more hearing difficulties due to their brain processing sounds abnormally, irrespective of PTA. (2) Methods: Three groups of hearing aid users who had normal cognition, mild cognitive impairment and dementia were recruited. Self-reported hearing abilities were assessed with the modified Amsterdam inventory for auditory disability (mAIAD) and the speech, spatial and qualities of hearing scale (SSQ). (3) Results: The SSQ and mAIAD scores were highly correlated with each other for all three groups. However, a correlation with objective PTA was found in the normal cognition but not the cognitively impaired groups. Self-reported hearing scores were associated with cognitive scores for the dementia group (4) Discussion: In people with combined cognitive and hearing impairment, PTA alone may be a poor predictor of hearing abilities. Subjective hearing questionnaires together with hearing tests may provide a better understanding of their hearing difficulties

Adaption and Preliminary Validation of the Addenbrooke’s Cognitive Examination‐III as a Screening Test for Mild Cognitive Impairment and Dementia in Hearing‐Impaired Individuals

Background: A large proportion of older adults assessed for cognitive impairment likely have hearing loss, potentially affecting accuracy of cognitive performance estimations. This study aimed to develop a hearing‐impaired version of the Addenbrooke’s Cognitive Examination‐III (HI‐ACE‐III) and to assess whether the HI‐ACE‐III can accurately distinguish people with Mild Cognitive Impairment (MCI) and dementia from cognitively intact controls. / Methods: The HI‐ACE‐III was developed by converting verbal instructions into a visual, timed PowerPoint presentation. 74 participants over the age of 60 were classified into three groups: 29 had MCI, 15 had mild to moderate dementia and 30 cognitively intact controls. Receiver Operating Characteristic (ROC) curves were graphed to test screening accuracy. Concurrent validity was examined through correlations between HI‐ACE‐III domain scores and relevant, visually presented standardised neuropsychological measures. / Results: ROC analysis for dementia revealed an Area Under the Curve (AUC) of 0.99, achieving excellent sensitivity (100%) and good specificity (93.3%) at an optimum cut‐off of <87. The AUC for MCI was 0.86, achieving reasonable sensitivity (75.9%) and good specificity (86.7%) at an optimum cut‐off of <92. HI‐ACE‐III subtests shared anticipated and statistically significant correlations with established measures of cognitive functioning. Internal consistency of the HI‐ACE‐III was excellent as verified with Cronbach’s alpha (α = .904). / Conclusion: Preliminarily, the HI‐ACE‐III showed good reliability, validity and screening utility for MCI and dementia in older adults in a hearing‐impairment context. The adapted HI‐ACE‐III may offer accurate and reliable indication of cognitive performance, supporting timely diagnosis and research examining links between hearing loss and cognitive decline

The Screening Accuracy of a Visually Based Montreal Cognitive Assessment Tool for Older Adult Hearing Aid Users

OBJECTIVES: This research aims to validate a modified visually based Montreal Cognitive Assessment for hearing-aid users (MoCA-HA). This population should be the target of cognitive screening due to high risk of developing dementia. DESIGN: Case-control study. SETTING: The participants were recruited from referral hearing-aid center and memory clinic in central London, United Kingdom. PARTICIPANT: 75 hearing-aid users were recruited. Of these, thirty were cognitively intact controls with hearing impairment (NC-HI); thirty had mild cognitive impairment with hearing impairment (MCI-HI); fifteen had dementia with hearing impairment (D-HI). MEASUREMENTS: The baseline characteristics and analysis of the MoCA-HA for the NC-HI were recorded. The MoCA-HA performance of the MCI-HI cohort and D-HI cohort were also studied. RESULTS: The cutpoint of <26 yields 93.3% sensitivity with 80% specificity in distinguishing MCI-HI from NC-HI. The specificity increased to 95.6% in screening for all cognitive impairment (MCI-HI and D-HI) from NC-HI. CONCLUSION: The MoCA-HA has been validated with a cutpoint which is comparable to the traditional MoCA. This tool may help clinicians to early identify older adult hearing-aid users for appropriate cognitive evaluation

A Hearing Screening Protocol for Stroke Patients: An Exploratory Study

Background: Auditory impairment post stroke is common and may be due to both peripheral hearing loss and or central auditory processing disorder. When auditory impairment remains untreated, it may impact on patient communication and rehabilitation after stroke. Offering a comprehensive audiological assessment to all stroke patients would be both costly and time-consuming. A brief hearing screening is thus required. Objective: The aim of this study was to determine whether a two-tiered hearing screening approach, with use of a handheld hearing screener and two validated hearing questionnaires could be used as a hearing screening for peripheral hearing loss and central auditory processing disorder in stroke survivors. The sensitivity and specificity of the screening method was analysed. Methods: This was a prospective study conducted in a tertiary neurology hospital. Forty-two consecutive stroke patients were recruited and tested within three to twelve months post-onset of their stroke. Three screening tools for the identification of hearing impairment were evaluated in this study: A handheld hearing screener for determination of peripheral audiometric hearing loss and two validated questionnaires (The Amsterdam Inventory Auditory for Disability and the Hearing Handicap Inventory for Elderly questionnaires) for determination of peripheral hearing loss and/or central auditory processing disorder. Results: The hearing screener had a sensitivity of 92. 59% detecting a hearing loss and specificity of 100%. The greatest test accuracy in identifying a central auditory processing type hearing impairment in stroke patients was found when the handheld hearing screener and the Amsterdam Inventory Auditory for Disability questionnaire were combined. Conclusion: This study is a first step towards addressing the complex auditory needs of stroke survivors in a systematic manner, with the ultimate aim to support their communication needs and long-term recovery and wellbeing

Serum miRNA125a-5p, miR-125b-5p, and miR-433-5p as biomarkers to differentiate between posterior circulation stroke and peripheral vertigo

BACKGROUND: Acute vertigo is a common presentation of inner ear disease. However, it can also be caused by more serious conditions, especially posterior circulation stroke. Differentiating between these two conditions by clinical presentations and imaging studies during the acute phase can be challenging. This study aimed to identify serum microRNA (miRNA) candidates that could differentiate between posterior circulation stroke and peripheral vertigo, among patients presenting with acute vertigo. METHODS: Serum levels of six miRNAs including miR-125a-5p, miR-125b-5p, miR-143-3p, miR-342-3p, miR-376a-3p, and miR-433-5p were evaluated. Using quantitative reverse-transcription polymerase chain reaction (RT-qPCR), the serum miRNAs were assessed in the acute phase and at a 90 day follow-up visit. RESULTS: A total of 58 patients with posterior circulation stroke (n = 23) and peripheral vertigo (n = 35) were included in the study. Serum miR-125a-5p (P = 0.001), miR-125b-5p (P <  0.001), miR-143-3p (P = 0.014) and miR-433-5p (P = 0.0056) were present at significantly higher levels in the acute phase, in the patients with posterior circulation infarction. Based on the area under the receiver operating characteristic curve (AUROC) only miR-125a-5p (0.75), miR-125b-5p(0.77), and miR-433-5p (0.71) had an acceptable discriminative ability to differentiate between the central and peripheral vertigo. A combination of miRNAs revealed no significant improvement of AUROC when compared to single miRNAs. CONCLUSION: This study demonstrated the potential of serum miR-125a-5p, miR-125b-5p, and miR-433-5p as biomarkers to assist in the diagnosis of posterior circulation infarction among patients presenting with acute vertigo

Clinical and preclinical therapeutic outcome metrics for USH2A-related disease

USH2A variants are the most common cause of Usher syndrome type 2, characterized by congenital sensorineural hearing loss and retinitis pigmentosa (RP), and also contribute to autosomal recessive non-syndromic RP. Several treatment strategies are under development; however, sensitive clinical trial endpoint metrics to determine therapeutic efficacy have not been identified. In the present study, we have performed longitudinal retrospective examination of the retinal and auditory symptoms in (i) 56 biallelic molecularly confirmed USH2A patients and (ii) ush2a mutant zebrafish to identify metrics for the evaluation of future clinical trials and rapid preclinical screening studies. The patient cohort showed a statistically significant correlation between age and both rate of constriction for the ellipsoid zone length and hyperautofluorescent outer retinal ring area. Visual acuity and pure tone audiograms are not suitable outcome measures. Retinal examination of the novel ush2au507 zebrafish mutant revealed a slowly progressive degeneration of predominantly rods, accompanied by rhodopsin and blue cone opsin mislocalization from 6 to 12 months of age with lysosome-like structures observed in the photoreceptors. This was further evaluated in the ush2armc zebrafish model, which revealed similar changes in photopigment mislocalization with elevated autophagy levels at 6 days post fertilization, indicating a more severe genotype-phenotype correlation and providing evidence of new insights into the pathophysiology underlying USH2A-retinal disease

Hearing‐impaired population performance and the effect of hearing interventions on Montreal Cognitive Assessment (MoCA): systematic review and meta‐analysis

BACKGROUND: Older adults are at high risk of developing age-related hearing loss (HL) and/or cognitive impairment. However, cognitive screening tools rely on oral administration of instructions and stimuli that may be impacted by HL. This systematic review aims to investigate (1) whether people with HL perform worse than those without HL on the Montreal Cognitive Assessment (MoCA), a widely used screening tool for cognitive impairment, and what the effect size of that difference is (2) whether HL treatment mitigates the impact of HL. METHOD: We conducted a systematic review and meta-analysis including studies that reported mean MoCA scores and standard deviations for individuals with HL. RESULTS: People with HL performed significantly worse on the MoCA (4 studies, N = 533) with a pooled mean difference of -1.66 points (95% confidence interval CI -2.74 to -0.58). There was no significant difference in MoCA score between the pre- vs post-hearing intervention (3 studies, N = 75). However, sensitivity analysis in the cochlear implant studies (2 studies, N = 33) showed improvement of the MoCA score by 1.73 (95% CI 0.18 to 3.28). CONCLUSION: People with HL score significantly lower than individuals with normal hearing on the standard orally administered MoCA. Clinicians should consider listening conditions when administering the MoCA and report the hearing status of the tested individuals, if known, taking this into account in interpretation or make note of any hearing difficulty during consultations which may warrant onward referral. Cochlear implants may improve the MoCA score of individuals with HL, and more evidence is required on other treatments

"The Dichotic Digit Test" as an Index Indicator for Hearing Problem in Dementia: Systematic Review and Meta-Analysis

BACKGROUND:  Patients with dementia commonly have problems processing speech in the presence of competing background speech or noise. This difficulty can be present from the very early stages of dementia, and may be a preclinical feature of Alzheimer's disease. PURPOSE:  This study investigates whether people with dementia perform worse on the dichotic digit test (DDT), an experimental probe of speech processing in the presence of competing speech, and whether test performance may predict dementia onset. RESEARCH DESIGN:  Systematic review and meta-analysis. DATA COLLECTION AND ANALYSIS:  A literature search was conducted in Medline, Embase, Scopus, and Psycinfo. We included (1) studies that included people with a diagnosis of dementia and a healthy control group with no cognitive impairment; (2) studies that reported results from a DDT in a free-recall response task; and (3) studies that had the dichotic digit mean correct percentage score or right-ear advantage, as outcome measurements. RESULTS:  People with dementia had a lower DDT total score, with a pooled mean difference of 18.6% (95% confidence interval [CI]: 21.2-15.9). Patients with dementia had an increased right-ear advantage relative to controls with a pooled difference of 24.4% (95% CI: 21.8-27.0). CONCLUSION:  The DDT total scores are lower and the right-ear advantage increased in cognitively impaired versus normal control participants. The findings also suggest that the reduction of dichotic digit total score and increase of right-ear advantage progress as cognitive impairment increases. Whether abnormalities in dichotic digit scores could predict subsequent dementia onset should be examined in further longitudinal studies

Simultaneous auditory agnosia: Systematic description of a new type of auditory segregation deficit following a right hemisphere lesion

We investigated auditory processing in a young patient who experienced a single embolus causing an infarct in the right middle cerebral artery territory. This led to damage to auditory cortex including planum temporale that spared medial Heschl’s gyrus, and included damage to the posterior insula and inferior parietal lobule. She reported chronic difficulties with segregating speech from noise and segregating elements of music. Clinical tests showed no evidence for abnormal cochlear function. Follow-up tests confirmed difficulties with auditory segregation in her left ear that spanned multiple domains, including words-in-noise and music streaming. Testing with a stochastic figure-ground task—a way of estimating generic acoustic foreground and background segregation—demonstrated that this was also abnormal. This is the first demonstration of an acquired deficit in the segregation of complex acoustic patterns due to cortical damage, which we argue is a causal explanation for the symptomatic deficits in the segregation of speech and music. These symptoms are analogous to the visual symptom of simultaneous agnosia. Consistent with functional imaging studies on normal listeners, the work implicates non-primary auditory cortex. Further, the work demonstrates a (partial) lateralisation of the necessary anatomical substrate for segregation that has not been previously highlighted