Dataset Information

Alteration of Cortical and Subcortical Structures in Children With Profound Sensorineural Hearing Loss.

ABSTRACT: Profound sensorineural hearing loss (SNHL) is an auditory disability associated with auditory and cognitive dysfunction. Due to distinct pathogenesis, some associated structural and functional changes within the brain have been investigated in previous studies, but whole-brain structural alterations are incompletely understood. We extended the exploration of neuroanatomic differences in whole-brain structure in children with profound SNHL who are primarily users of Chinese sign language (CSL). We employed surface-based morphometry (SBM) and subcortical analyses. T1-weighted magnetic resonance images of 26 children with profound SNHL and 27 age- and sex-matched children with normal hearing were analyzed. Compared with the normal control (NC) group, children with profound SNHL showed diverse structural changes in surface-based and subcortical analyses, including decreased cortical thickness in the left postcentral gyrus, superior parietal lobule, paracentral lobule, precuneus, the right transverse temporal gyri, and the middle temporal gyrus; a noticeable increase in the Local Gyrification Index (LGI) in the left precuneus and superior parietal lobule; and diverse changes in gray-matter volume (GMV) in different brain regions. Surface-based vertex analyses revealed regional contractions in the right thalamus, putamen, pallidum, and the brainstem of children with profound SNHL when compared with those in the NC group. Volumetric analyses showed decreased volumes of the right thalamus and pallidum in children with profound SNHL. Our data suggest that children with profound SNHL are associated with diffuse cerebral dysfunction to cortical and subcortical nuclei, and revealed neuroplastic reorganization in the precuneus, superior parietal lobule, and temporal gyrus. Our study provides robust evidence for changes in connectivity and structure in the brain associated with hearing loss.

SUBMITTER: Qu H

PROVIDER: S-EPMC7756106 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature

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Alteration of Cortical and Subcortical Structures in Children With Profound Sensorineural Hearing Loss.

Qu Hang H Tang Hui H Pan Jiahao J Zhao Yi Y Wang Wei W

Frontiers in human neuroscience 20201209

Profound sensorineural hearing loss (SNHL) is an auditory disability associated with auditory and cognitive dysfunction. Due to distinct pathogenesis, some associated structural and functional changes within the brain have been investigated in previous studies, but whole-brain structural alterations are incompletely understood. We extended the exploration of neuroanatomic differences in whole-brain structure in children with profound SNHL who are primarily users of Chinese sign language (CSL). W ...[more]

PMID: 33362488

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Project description:OBJECTIVES:We, herein, report two novel USH2A variants from two unrelated Korean families and their clinical phenotypes, with attention to severe or more than severe sensorineural hearing loss (SNHL). METHODS:Two postlingually deafened subjects (SB237-461, M/46 and SB354-692, F/34) with more than severe SNHL and also with suspicion of Usher syndrome type II (USH2) were enrolled. A comprehensive audiological and ophthalmological assessments were evaluated. We conducted the whole exome sequencing and subsequent pathogenicity prediction analysis. RESULTS:We identified the following variants of USH2A from the two probands manifesting more than severe SNHL and retinitis pigmentosa (RP): compound heterozygosity for a nonsense (c.8176C>T: p.R2723X) and a missense variant (c.1823G>A: p.C608Y) in SB237, and compound heterozygosity for two frameshift variants (c.14835delT: p.S4945fs & c.13112_13115delAAAT: p.G4371fs) in SB354. Based on the American College of Medical Genetics and Genomics/Association for Molecular Pathology guidelines, two novel variants, c.1823G>A: p.C608Y and c.14835delT: p.Ser4945fs, can be classified as "uncertain significance" and "pathogenic," respectively. The audiogram exhibited more than severe SNHL and a down-sloping configuration, necessitating cochlear implantation. The ophthalmic examinations revealed typical features of RP. Interestingly, one proband (SB 354-692) carrying two truncating compound heterozygous variants exhibited more severe hearing loss than the other proband (SB 237-461), carrying one truncation with one missense variant. CONCLUSION:Our results provide insight on the expansion of audiological spectrum encompassing more than severe SNHL in Korean subjects harboring USH2A variants, suggesting that USH2A should also be included in the candidate gene of cochlear implantation. A specific combination of USH2A variants causing truncating proteins in both alleles could demonstrate more severe audiological phenotype than that of USH2A variants carrying one truncating mutation and one missense mutation, suggesting a possible genotype-phenotype correlation. The understanding of audiological complexity associated with USH2A will be helpful for genetic counseling and treatment starategy.

Project description:BackgroundThe stria vascularis (SV), located in the lateral wall of the cochlea, maintains cochlear fluid homeostasis and mechanoelectrical transduction (MET) activity required for sound wave conduction. The pathogenesis of a number of human inheritable deafness syndromes, age related hearing loss, drug-induced ototoxicity and noise-induced hearing loss results from the morphological changes and functional impairments in the development of the SV. In this study, we investigate the implications of intercellular communication within the SV in the pathogenesis of sensorineural hearing loss (SNHL). We aim to identify commonly regulated signaling pathways using publicly available single-cell transcriptomic sequencing (scRNA-seq) datasets.MethodsWe analyzed scRNA-seq data, which was derived from studying the cochlear SV in mice with SNHL compared to normal adult mice. After quality control and filtering, we obtained the major cellular components of the mouse cochlear SV and integrated the data. Using Seurat's FindAllMarkers and FindMarkers packages, we searched for novel conservative genes and differential genes. We employed KEGG and GSEA to identify molecular pathways that are commonly altered among different types of SNHL. We utilized pySCENIC to discover new specific regulatory factors in SV subpopulation cells. With the help of CellChat, we identified changes in subpopulation cells showing similar trends across different SNHL types and their alterations in intercellular communication pathways.ResultsThrough the analysis of the integrated data, we discovered new conserved genes to SV specific cells and identified common downregulated pathways in three types of SNHL. The enriched genes for these pathways showing similar trends are primarily associated with the Electron Transport Chain, related to mitochondrial energy metabolism. Using the CellChat package, we further found that there are shared pathways in the incoming signaling of specific intermediate cells in SNHL, and these pathways have common upstream regulatory transcription factor of Nfe2l2. Combining the results from pySCENIC and CellChat, we predicted the transcription factor Nfe2l2 as an upstream regulatory factor for multiple shared cellular pathways in IC. Additionally, it serves as an upstream factor for several genes within the Electron Transport Chain.ConclusionOur bioinformatics analysis has revealed that downregulation of the mitochondrial electron transport chain have been observed in various conditions of SNHL. E2f1, Esrrb, Runx1, Yy1, and Gata2 could serve as novel important common TFs regulating the electron transport chain. Adm has emerged as a potential new marker gene for intermediate cells, while Itgb5 and Tesc show promise as potential new marker genes for marginal cells in the SV. These findings offer a new perspective on SV lesions in SNHL and provide additional theoretical evidence for the same drug treatment and prevention of different pathologies of SNHL.

Project description:When selectively attending to a speech stream in multi-talker scenarios, low-frequency cortical activity is known to synchronize selectively to fluctuations in the attended speech signal. Older listeners with age-related sensorineural hearing loss (presbycusis) often struggle to understand speech in such situations, even when wearing a hearing aid. Yet, it is unclear whether a peripheral hearing loss degrades the attentional modulation of cortical speech tracking. Here, we used psychoacoustics and electroencephalography (EEG) in male and female human listeners to examine potential effects of hearing loss on EEG correlates of speech envelope synchronization in cortex. Behaviorally, older hearing-impaired (HI) listeners showed degraded speech-in-noise recognition and reduced temporal acuity compared with age-matched normal-hearing (NH) controls. During EEG recordings, we used a selective attention task with two spatially separated simultaneous speech streams where NH and HI listeners both showed high speech recognition performance. Low-frequency (<10 Hz) envelope-entrained EEG responses were enhanced in the HI listeners, both for the attended speech, but also for tone sequences modulated at slow rates (4 Hz) during passive listening. Compared with the attended speech, responses to the ignored stream were found to be reduced in both HI and NH listeners, allowing for the attended target to be classified from single-trial EEG data with similar high accuracy in the two groups. However, despite robust attention-modulated speech entrainment, the HI listeners rated the competing speech task to be more difficult. These results suggest that speech-in-noise problems experienced by older HI listeners are not necessarily associated with degraded attentional selection.SIGNIFICANCE STATEMENT People with age-related sensorineural hearing loss often struggle to follow speech in the presence of competing talkers. It is currently unclear whether hearing impairment may impair the ability to use selective attention to suppress distracting speech in situations when the distractor is well segregated from the target. Here, we report amplified envelope-entrained cortical EEG responses to attended speech and to simple tones modulated at speech rates (4 Hz) in listeners with age-related hearing loss. Critically, despite increased self-reported listening difficulties, cortical synchronization to speech mixtures was robustly modulated by selective attention in listeners with hearing loss. This allowed the attended talker to be classified from single-trial EEG responses with high accuracy in both older hearing-impaired listeners and age-matched normal-hearing controls.

Project description:IntroductionTo compare inpatient treated patients with idiopathic (ISSNHL) and non-idiopathic sudden sensorineural hearing loss (NISSNHL) regarding frequency, hearing loss, treatment and outcome.MethodsAll 574 inpatient patients (51% male, median age: 60 years) with ISSNHL and NISSNHL, who were treated in federal state Thuringia in 2011 and 2012, were included retrospectively. Univariate and multivariate statistical analyses were performed.ResultsISSNHL was diagnosed in 490 patients (85%), NISSNHL in 84 patients (15%). 49% of these cases had hearing loss due to acute otitis media, 37% through varicella-zoster infection or Lyme disease, 10% through Menière disease and 7% due to other reasons. Patients with ISSNHL and NISSNHL showed no difference between age, gender, side of hearing loss, presence of tinnitus or vertigo and their comorbidities. 45% of patients with ISSNHL and 62% with NISSNHL had an outpatient treatment prior to inpatient treatment (p < 0.001). The mean interval between onset of hearing loss to inpatient treatment was shorter in ISSNHL (7.7 days) than in NISSNHL (8.9 days; p = 0.02). The initial hearing loss of the three most affected frequencies in pure-tone average (3PTAmax) scaled 72.9 dBHL ± 31.3 dBHL in ISSNHL and 67.4 dBHL ± 30.5 dBHL in NISSNHL. In the case of acute otitis media, 3PTAmax (59.7 dBHL ± 24.6 dBHL) was lower than in the case of varicella-zoster infection or Lyme disease (80.11 dBHL ± 34.19 dBHL; p = 0.015). Mean absolute hearing gain (Δ3PTAmaxabs) was 8.1 dB ± 18.8 dB in patients with ISSNHL, and not different in NISSNHL patients with 10.2 dB ± 17.6 dB. A Δ3PTAmaxabs ≥ 10 dB was reached in 34.3% of the patients with ISSNHL and to a significantly higher rate of 48.8% in NISSNHL patients (p = 0.011).ConclusionsISSNHL and NISSNHL show no relevant baseline differences. ISSNHL tends to have a higher initial hearing loss. NISSHNL shows a better outcome than ISSNHL.

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Alteration of Cortical and Subcortical Structures in Children With Profound Sensorineural Hearing Loss.

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Alteration of Cortical and Subcortical Structures in Children With Profound Sensorineural Hearing Loss.

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