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"Deafness Essays."
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Whole-Genome Sequencing Improves the Diagnosis of DFNB1 Monoallelic Patients
Hearing loss is the most common sensory defect, due in most cases to a genetic origin. Variants in the GJB2 gene are responsible for up to 30% of non-syndromic hearing loss. Today, several deafness genotypes remain incomplete, confronting us with a diagnostic deadlock. In this study, whole-genome sequencing (WGS) was performed on 10 DFNB1 patients with incomplete genotypes. New variations on GJB2 were identified for four patients. Functional assays were realized to explore the function of one of them in the GJB2 promoter and confirm its impact on GJB2 expression. Thus, in this study WGS resolved patient genotypes, thus unlocking diagnosis. WGS afforded progress and bridged some gaps in our research.
Journal Article
Sonic Agency
In a world dominated by the visual, could contemporary resistances be auditory? This timely and important book highlights sound's invisible, disruptive and affective qualities and asks whether the unseen nature of sound can support a political transformation.
eBook
The Clear-Speech Benefit for School-Age Children: Speech-in-Noise and Speech-in-Speech Recognition
Purpose: Talkers often modify their speech when communicating with individuals who struggle to understand speech, such as listeners with hearing loss. This study evaluated the benefit of clear speech in school-age children and adults with normal hearing for speech-in-noise and speech-in-speech recognition. Method: Masked sentence recognition thresholds were estimated for school-age children and adults using an adaptive procedure. In Experiment 1, the target and masker were summed and presented over a loudspeaker located directly in front of the listener. The masker was either speech-shaped noise or two-talker speech, and target sentences were produced using a clear or conversational speaking style. In Experiment 2, stimuli were presented over headphones. The two-talker speech masker was diotic (M[subscript 0]). Clear and conversational target sentences were presented either in-phase (T[subscript 0]) or out-of-phase (T[subscript [pi]]) between the two ears. The M[subscript 0]T[subscript [pi]] condition introduces a segregation cue that was expected to improve performance. Results: For speech presented over a single loudspeaker (Experiment 1), the clear-speech benefit was independent of age for the noise masker, but it increased with age for the two-talker masker. Similar age effects for the two-talker speech masker were seen under headphones with diotic presentation (M[subscript 0]T[subscript 0]), but comparable clear-speech benefit as a function of age was observed with a binaural cue to facilitate segregation (M[subscript 0]T[subscript [pi]]). Conclusions: Consistent with prior research, children showed a robust clear-speech benefit for speech-in-noise recognition. Immaturity in the ability to segregate target from masker speech may limit young children's ability to benefit from clear-speech modifications for speech-in-speech recognition under some conditions. When provided with a cue that facilitates segregation, children as young as 4-7 years of age derived a clear-speech benefit in a two-talker masker that was similar to the benefit experienced by adults.
Journal Article
Relationship Between Serum ET-1, HDL-C, and sVCAM-1 and Hearing Loss in Patients with Sudden Deafness
Vascular causes are most commonly associated with sudden sensorineural hearing loss (SSHL). This study was performed to determine the relationship between serum endothelin-1 (ET-1), high-density lipoprotein cholesterol (HDL-C), soluble vascular cell adhesion molecule-1 (sVCAM-1) levels, and the degree of hearing loss in patients with SSHL. Firstly, 60 SSHL patients were admitted to The First Hospital of Shanxi Medical University. In the same period, 60 healthy subjects matching the age and gender of SSHL patients were selected as the control group. Then, serum levels of ET-1, HDL-C, and sVCAM-1 were measured by enzyme-linked immunosorbent assay (ELISA). Next, the relationship between serum levels of ET-1, HDL-C, and sVCAM-1 with clinicopathological factors and their diagnostic and prognostic values were analyzed and evaluated. Serum ET-1 and sVCAM-1 were increased, and HDL-C was decreased in patients with SSHL. Serum ET-1 and sVCAM-1 were higher and HDL-C was lower in patients aged ≥ 45 years, or severe hearing loss patients (
P
< 0.05). ROC analysis determined that ET-1 (AUC = 0.839), HDL-C (AUC = 0.830), and sVCAM-1 (AUC = 0.865) had excellent diagnostic values. In addition, patients with low levels of ET-1 and sVCAM-1 and high levels of HDL-C had better hearing prognosis (
P
< 0.05). Abnormal serum ET-1, HDL-C, and sVCAM-1 in patients with SSHL are closely related to age, and degree of hearing loss, and perform diagnostic and prognostic values.
Journal Article
Inclusive citizenship through mathematics education: a conceptual investigation
Citizenship is a contested concept that can be interpreted from different – and also conflicting – perspectives. Inspired by inclusive mathematics education, we try to illustrate what it could mean to work toward inclusive citizenship. We illustrate the idea of inclusive mathematics education, showing how deaf and hearing students can learn together. We interpret inclusive mathematics education as ‘meetings amongst differences’. This interpretation inspires us to see meetings amongst differences in a broader perspective. Such meetings can be considered fundamental to the process of establishing inclusive citizenship. We elaborate on this idea by exemplifying how meetings across national, economic, cultural and existential differences can be established in mathematics education. We conclude that, to work toward inclusive citizenship, it is important to establish dialogues across distances; to make students interested in and able to see situations and problems from perspectives different from their own; to construct tolerance as an expression of shared concerns; and finally, to challenge any form of We–Other dichotomy.
Journal Article
American Folklore Studies and Disability: An Introduction
As an introduction to the special issue on folklore and disability, this essay offers a brief overview of how concerns about disability have historically been included in folklore studies in the United States. It discusses the field of disability studies and how folklore has been an important element of disability theory and writing. In making an argument for how a more serious engagement with disability studies theory and writing can enhance US folkloristics, the essay further suggests the development of a subfield of folklore devoted to disability and folklore.
Journal Article
(Re)Educating the Senses: Multimodal Listening, Bodily Learning, and the Composition of Sonic Experiences
This essay reimagines the way that listening is taught in the multimodal composition classroom. In contrast to listening to sonic content for meaning, the listening pedagogy I introduce is based on my concept of multimodal listening—a practice that involves attending to the sensory, material, and contextual aspects that comprise and shape a sonic event. I argue that cultivating multimodal listening practices will enable students to become more savvy consumers and producers of sound in the composition classroom and in their everyday lives.
Journal Article
T cell inducing vaccine against cytomegalovirus immediate early 1 (IE1) protein provides high level cross strain protection against congenital CMV
Human cytomegalovirus (HCMV) is a leading cause of congenital disease resulting in cognitive impairment and deafness in newborns. Multiple strains of HCMV enable re-infection and convalescent immunity does not protect against risk of congenital CMV (cCMV). Consequently, a cross strain protective CMV vaccine is a high priority. The guinea pig is the only small animal model for cCMV and species specific guinea pig cytomegalovirus (GPCMV) encodes homolog HCMV viral proteins making it suitable for vaccine studies. Neutralizing antibodies against viral entry glycoprotein complexes and cell free virus are insufficient for complete protection because highly cell associated virus enables evasion. CMV T-cell antigens are important in HCMV convalescent immunity and potentially in reducing the risk of cCMV. Immediate early protein IE1 is essential to HCMV and a T-cell target in humans. In this study, a recombinant defective adenovirus encoding GPCMV IE1 (AdIE1) was evaluated in a preclinical vaccine study. AdIE1 vaccinated animals evoked a T-cell response in a guinea pig IFNγ ELISPOT assay to IE1 (GP123). Vaccinated animals exhibited protection against subcutaneous challenge by GPCMV prototype strain (22122) with viral load substantially reduced compared to the unvaccinated control group and previous Ad based vaccine study against viral pp65 tegument protein. In a vaccine study against cCMV, dams were challenged mid-pregnancy with dual wild type virus strains (22122 and clinical strain TAMYC). At birth, pups were evaluated for viral load in target organs. AdIE1 vaccine had high efficacy against cCMV with GPCMV pup transmission reduced from 92% in the litters of the unvaccinated control group of dams to 23% in the vaccine group resulting in an absence of virus or statistically significant reduction in viral load in pup organs. Overall, IE1 is a more protective T-cell antigen than previously studied pp65 providing cross strain immunity against cCMV in this preclinical model.
•GPCMV immediate-early protein 1 (IE1) vaccine induces interferon-γ T cell response•GPCMV IE1 rAd-based vaccine is partially protective against CMV dissemination•GPCMV IE1 rAd-based vaccine reduces congenital CMV (cCMV) infection by 75 %•GPCMV IE1-based vaccine is cross-strain protective against cCMV•IE1 has higher protective efficacy against cCMV than previous single antigen vaccines
Journal Article