Leukoencephalitis, Acute Hemorrhagic

Humans have the remarkable ability to integrate information from different senses, which greatly facilitates the detection, localization and identification of events in the environment. About 466 million people worldwide suffer from hearing loss. Yet, the impact of hearing loss on how the senses work together is rarely investigated. Here, we investigate how a common sensory impairment, asymmetric conductive hearing loss (AHL), alters the way our senses interact by examining human orienting behaviour with normal hearing (NH) and acute AHL. This type of hearing loss disrupts auditory localization. We hypothesized that this creates a conflict between auditory and visual spatial estimates and alters how auditory and visual inputs are integrated to facilitate multisensory spatial perception. We analysed the spatial and temporal properties of saccades to auditory, visual and audiovisual stimuli before and after plugging the right ear of participants. Both spatial and temporal aspects of multisensory integration were affected by AHL. Compared with NH, AHL caused participants to make slow, inaccurate and unprecise saccades towards auditory targets. Surprisingly, increased weight on visual input resulted in accurate audiovisual localization with AHL. This came at a cost: saccade latencies for audiovisual targets increased significantly. The larger the auditory localization errors, the less participants were able to benefit from audiovisual integration in terms of saccade latency. Our results indicate that observers immediately change sensory weights to effectively deal with acute AHL and preserve audiovisual accuracy in a way that cannot be fully explained by statistical models of optimal cue integration.

(1) Background: For successful hearing aid (HA) use during daily life, an objective parameter reflecting the subjective satisfaction is required. We explored the aided hearing status, hearing in noise test (HINT) scores, and subjective outcomes to predict performance improvements in everyday living. (2) Methods: A total of 406 patients with hearing loss (HL) who were prescribed HAs were included and were divided into two groups according to the symmetricity of HL. The relationship between audiometric data and subjective questionnaires under unaided and aided (3 months) conditions were investigated. (3) Results: Patients with symmetric HL showed a significant HINT signal-to-noise ratio (SNR) change and significant increase in their subjective satisfaction questionnaire score under the bilateral HA condition. On the other hand, the HINT SNR change and subjective questionnaire score showed various significances according to the side of HA (better or worse hearing) in asymmetric HL HINT SNR and was significantly correlated with the subjective questionnaire score in symmetric HL patients and AHL patients with unilateral HA in their better ear. (4) Conclusions: The HINT SNR improvement after long-term HA use could be an effective tool for predicting the subjective satisfaction of HA use and HA validation.

Although serum amyloid A (AA) amyloid may occasionally show nonspecific staining by immunofluorescence (IF), the correct diagnosis can usually be determined by integrating pathologic features and clinical scenario, and using AA amyloid immunohistochemistry (IHC) and/or mass spectrometry. A recent mass spectrometry-based study described false-positive Ig IF staining in a subset of AA amyloid cases.

We sought to delineate clinicopathologic features of AA amyloid with Ig-dominant staining by using a retrospective review.

AA amyloid with Ig-dominant staining was identified in 10 patients from 5 institutions, representing 1.2% to 4% of AA amyloid kidney biopsies. Evidence of a monoclonal protein was documented in 0% to 2.7% of patients with AA amyloid screened for inclusion, but 30% of those with Ig-dominant staining. The patient population had equal sex distribution and presented at median age of 68.5 years with nephrotic proteinuria and kidney impairment. Etiologies of AA amyloid included injection drug use (30%), autoimmune disease (20%), and chronic infection (10%); 40% had no identified clinical association. On biopsy, heavy chain (co)dominant staining by IF (in 80%), discordant distribution in Ig staining (in 20%), tubulointerstitial nephritis (in 30%), and/or crescents (in 10%) were present. Two of 3 patients with paraproteinemia had concordant heavy and/or light chain dominant staining within the AA amyloid. Two cases were initially misdiagnosed as Ig-associated amyloidosis.

We describe the morphologic spectrum of AA amyloidosis with Ig-dominant staining which may have clinical, laboratory, and pathologic overlap with amyloid light chain (AL), amyloid heavy chain, and heavy and light chain (AHL) amyloidosis.