Relationship between sensitivity to temporal fine structure and spoken language abilities in children with mild-to-moderate sensorineural hearing loss.
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ABSTRACT:
SUBMITTER: Cabrera L
PROVIDER: S-EPMC7613189 | biostudies-literature |
REPOSITORIES: biostudies-literature
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Project description:Previous research has shown deficits in vocal emotion recognition in sub-populations of individuals with hearing loss, making this a high priority research topic. However, previous research has only examined vocal emotion recognition using verbal material, in which emotions are expressed through emotional prosody. There is evidence that older individuals with hearing loss suffer from deficits in general prosody recognition, not specific to emotional prosody. No study has examined the recognition of non-verbal vocalization, which constitutes another important source for the vocal communication of emotions. It might be the case that individuals with hearing loss have specific difficulties in recognizing emotions expressed through prosody in speech, but not non-verbal vocalizations. We aim to examine whether vocal emotion recognition difficulties in middle- aged-to older individuals with sensorineural mild-moderate hearing loss are better explained by deficits in vocal emotion recognition specifically, or deficits in prosody recognition generally by including both sentences and non-verbal expressions. Furthermore a, some of the studies which have concluded that individuals with mild-moderate hearing loss have deficits in vocal emotion recognition ability have also found that the use of hearing aids does not improve recognition accuracy in this group. We aim to examine the effects of linear amplification and audibility on the recognition of different emotions expressed both verbally and non-verbally. Besides examining accuracy for different emotions we will also look at patterns of confusion (which specific emotions are mistaken for other specific emotion and at which rates) during both amplified and non-amplified listening, and we will analyze all material acoustically and relate the acoustic content to performance. Together these analyses will provide clues to effects of amplification on the perception of different emotions. For these purposes, a total of 70 middle-aged-older individuals, half with mild-moderate hearing loss and half with normal hearing will perform a computerized forced-choice vocal emotion recognition task with and without amplification.
| S-EPMC8740977 | biostudies-literature
Project description:Brain structure can predict many aspects of human behavior, though the extent of this relationship in healthy adults, particularly for language-related skills, remains largely unknown. The objective of the present study was to explore this relation using magnetic resonance imaging (MRI) on a group of 21 healthy young adults who completed two language tasks: (1) semantic fluency and (2) sentence generation. For each region of interest, cortical thickness, surface area, and volume were calculated. The results show that verbal fluency scores correlated mainly with measures of brain morphology in the left inferior frontal cortex and bilateral insula. Sentence generation scores correlated with structure of the left inferior parietal and right inferior frontal regions. These results reveal that the anatomy of several structures in frontal and parietal lobes is associated with spoken language performance. The presence of both negative and positive correlations highlights the complex relation between brain and language.
| S-EPMC4587378 | biostudies-literature
Project description:BackgroundThe main clinical intervention for mild to moderate hearing loss is the provision of hearing aids. These are routinely offered and fitted to those who seek help for hearing difficulties. By amplifying and improving access to sounds, and speech sounds in particular, the aim of hearing aid use is to reduce the negative consequences of hearing loss and improve participation in everyday life.ObjectivesTo evaluate the effects of hearing aids for mild to moderate hearing loss in adults.Search methodsThe Cochrane ENT Information Specialist searched the ENT Trials Register; the Cochrane Register of Studies Online; MEDLINE; PubMed; EMBASE; CINAHL; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The date of the search was 23 March 2017.Selection criteriaRandomised controlled trials (RCTs) of hearing aids compared to a passive or active control in adults with mild to moderate hearing loss.Data collection and analysisWe used the standard methodological procedures expected by Cochrane. The primary outcomes in this review were hearing-specific health-related quality of life and the adverse effect pain. Secondary outcomes were health-related quality of life, listening ability and the adverse effect noise-induced hearing loss. We used GRADE to assess the quality of the evidence for each outcome; this is indicated in italics.Main resultsWe included five RCTs involving 825 participants. The studies were carried out in the USA and Europe, and were published between 1987 and 2017. Risk of bias across the studies varied. Most had low risk for selection, reporting and attrition bias, and a high risk for performance and detection bias because blinding was inadequate or absent.All participants had mild to moderate hearing loss. The average age across all five studies was between 69 and 83 years. The duration of the studies ranged between six weeks and six months.There was a large beneficial effect of hearing aids on hearing-specific health-related quality of life associated with participation in daily life as measured using the Hearing Handicap Inventory for the Elderly (HHIE, scale range 1 to 100) compared to the unaided/placebo condition (mean difference (MD) -26.47, 95% confidence interval (CI) -42.16 to -10.77; 722 participants; three studies) (moderate-quality evidence).There was a small beneficial effect of hearing aids on general health-related quality of life (standardised mean difference (SMD) -0.38, 95% CI -0.55 to -0.21; 568 participants; two studies) (moderate-quality evidence). There was a large beneficial effect of hearing aids on listening ability (SMD -1.88, 95% CI -3.24 to -0.52; 534 participants; two studies) (moderate-quality evidence).Adverse effects were measured in only one study (48 participants) and none were reported (very low-quality evidence).Authors' conclusionsThe available evidence concurs that hearing aids are effective at improving hearing-specific health-related quality of life, general health-related quality of life and listening ability in adults with mild to moderate hearing loss. The evidence is compatible with the widespread provision of hearing aids as the first-line clinical management in those who seek help for hearing difficulties. Greater consistency is needed in the choice of outcome measures used to assess benefits from hearing aids. Further placebo-controlled studies would increase our confidence in the estimates of these effects and ascertain whether they vary according to age, gender, degree of hearing loss and type of hearing aid.
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Project description:EYA4 is a member of the vertebrate eya gene family of transcriptional activators and plays several roles in both embryonic and inner ear development. The majority of EYA4 gene mutations are associated with autosomal dominant non-syndromic hearing loss (DFNA10). In addition, some mutations in this gene cause autosomal dominant syndromic hearing loss with dilated cardiomyopathy. EYA4 is a rare cause of sensorineural hearing loss, and only a limited number of papers regarding mutations in this gene have been published. Thus, detailed clinical features remain unclear. We conducted next-generation sequencing of a Japanese individual with progressive sensorineural hearing loss and identified an EYA4 pathogenic variant. Pure-tone audiometry revealed bilateral, nearly symmetric, moderate sensorineural hearing loss in the low and middle frequencies. Minor abnormalities were observed on the patient's electrocardiogram and echocardiography without any apparent symptoms. Next-generation sequencing is effective in elucidating the etiology of hearing loss, and the present findings suggested the possible phenotypic expansion of deafness caused by EYA4 gene mutations.
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Project description:Mutations in the human gap junction beta-2 gene (GJB2) that encodes connexin-26 have been shown to cause non-syndromic sensorineural hearing loss (NSSNHL) at the DFNB1 locus on 13q11. Functional and genetic data regarding the disease causing potential of one particular GJB2 sequence variant, 101 T-->C (M34T), have proven contradictory. In this study, we found the prevalence of the M34T allele in a cohort of white sib pairs and sporadic cases with NSSNHL from the United Kingdom and Ireland to be 3.179% of chromosomes screened. Significantly, we identified the first M34T/M34T genotype cosegregating in a single family with mid to high frequency NSSNHL. Screening a control population of 630 subjects we identified 25 M34T heterozygotes; however, no M34T homozygotes were detected. Surprisingly, the majority of M34T alleles (88%) were in cis with a 10 bp deletion in the 5' non-coding sequence. This non-coding deletion was also homozygous in the homozygous M34T subjects. Microsatellite analysis of flanking loci in M34T heterozygotes and controls does not define an extensive ancestral haplotype but preliminary data suggest two common alleles in subjects with the M34T allele. In summary, we provide data that support M34T acting as a recessive GJB2 allele associated with mild-moderate prelingual hearing impairment.
| S-EPMC1734724 | biostudies-other
Project description:Permanent childhood hearing loss affects 1 to 3 per 1000 children and frequently disrupts typical spoken language acquisition. Early identification of hearing loss through universal newborn hearing screening and the use of new hearing technologies including cochlear implants make spoken language an option for most children. However, there is no consensus on what constitutes optimal interventions for children when spoken language is the desired outcome. Intervention and educational approaches ranging from oral language only to oral language combined with various forms of sign language have evolved. Parents are therefore faced with important decisions in the first months of their child's life.This article presents the protocol for a systematic review of the effects of using sign language in combination with oral language intervention on spoken language acquisition. Studies addressing early intervention will be selected in which therapy involving oral language intervention and any form of sign language or sign support is used. Comparison groups will include children in early oral language intervention programs without sign support. The primary outcomes of interest to be examined include all measures of auditory, vocabulary, language, speech production, and speech intelligibility skills. We will include randomized controlled trials, controlled clinical trials, and other quasi-experimental designs that include comparator groups as well as prospective and retrospective cohort studies. Case-control, cross-sectional, case series, and case studies will be excluded. Several electronic databases will be searched (for example, MEDLINE, EMBASE, CINAHL, PsycINFO) as well as grey literature and key websites. We anticipate that a narrative synthesis of the evidence will be required. We will carry out meta-analysis for outcomes if clinical similarity, quantity and quality permit quantitative pooling of data. We will conduct subgroup analyses if possible according to severity/type of hearing disorder, age of identification, and type of hearing technology.This review will provide evidence on the effectiveness of using sign language in combination with oral language therapies for developing spoken language in children with hearing loss who are identified at a young age. The information from this review can provide guidance to parents and intervention specialists, inform policy decisions and provide directions for future research.CRD42013005426.
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Project description:When children start formal education, they are expected to be able to express complex thoughts. However, in order to do so, they need to be able to use both complex grammatical structures and a variety of words. One group that is at risk of having a delay in terms of their expressive language ability is children with cochlear implants (CI). In this study, we evaluated whether children with CI perform comparably to children with typical hearing (TH) on a standard expressive spoken grammar and a standard expressive spoken vocabulary task when the groups were matched on non-verbal intelligence and working memory capacity. It was found that the children with CI in this study performed more poorly on a standard expressive spoken vocabulary task but not on a standard expressive spoken grammar task when compared to the children with TH. Differences in terms of expressive spoken vocabulary do not seem to be explained by differences in cognitive ability. In addition, the variation in terms of expressive spoken language ability was larger in the children with CI compared to the children with TH. This might be explained by additional confounding factors, like the time of language deprivation or by a greater influence of cognitive differences for the acquisition of spoken language for children with CI.
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Project description:Individuals' moral views have been shown to affect their event-related potentials (ERP) response to spoken statements, and people's political ideology has been shown to guide their sentence completion behavior. Using pupillometry, we asked whether political ideology and disgust sensitivity affect online spoken language comprehension. 60 native speakers of English listened to spoken utterances while their pupil size was tracked. Some of those utterances contained grammatical errors, semantic anomalies, or socio-cultural violations, statements incongruent with existing gender stereotypes and perceived speaker identity, such as "I sometimes buy my bras at Hudson's Bay," spoken by a male speaker. An individual's disgust sensitivity is associated with the Behavioral Immune System, and may be correlated with socio-political attitudes, for example regarding out-group stigmatization. We found that more disgust-sensitive individuals showed greater pupil dilation with semantic anomalies and socio-cultural violations. However, political views differently affected the processing of the two types of violations: whereas more conservative listeners showed a greater pupil response to socio-cultural violations, more progressive listeners engaged more with semantic anomalies, but this effect appeared much later in the pupil record.
| S-EPMC8542873 | biostudies-literature
Project description:Hereditary hearing loss is characterized by a high degree of genetic heterogeneity. Here we present OTOGL mutations, a homozygous one base pair deletion (c.1430 delT) causing a frameshift (p.Val477Glufs(?)25) in a large consanguineous family and two compound heterozygous mutations, c.547C>T (p.Arg183(?)) and c.5238+5G>A, in a nonconsanguineous family with moderate nonsyndromic sensorineural hearing loss. OTOGL maps to the DFNB84 locus at 12q21.31 and encodes otogelin-like, which has structural similarities to the epithelial-secreted mucin protein family. We demonstrate that Otogl is expressed in the inner ear of vertebrates with a transcription level that is high in embryonic, lower in neonatal, and much lower in adult stages. Otogelin-like is localized to the acellular membranes of the cochlea and the vestibular system and to a variety of inner ear cells located underneath these membranes. Knocking down of otogl with morpholinos in zebrafish leads to sensorineural hearing loss and anatomical changes in the inner ear, supporting that otogelin-like is essential for normal inner ear function. We propose that OTOGL mutations affect the production and/or function of acellular structures of the inner ear, which ultimately leads to sensorineural hearing loss.
| S-EPMC3487139 | biostudies-literature
Project description:Hearing impairment affects 1 in 650 newborns, making it the most common congenital sensory impairment. Autosomal recessive nonsyndromic sensorineural hearing impairment (ARNSHI) comprises 80% of familial hearing impairment cases. Mutations in GJB2 account for a significant number of ARNSHI (and up to 50% of documented recessive (e.g., more than 1 affected sibling) hearing impairment in some populations). Mutations in the GJB2 gene are amongst the most common causes of hearing impairment in populations of various ethnic backgrounds. Two mutations of this gene, 35delG and 167delT, account for the majority of reported mutations in Caucasian populations, especially those of Mediterranean and Ashkenazi Jewish background. The 235delC mutation is most prevalent in East Asian populations. Some mutations are of less well-characterized significance. The V37I missense mutation, common in Asian populations, was initially described as a polymorphism and later as a potentially pathogenic mutation. We report here on 15 unrelated individuals with ARNSHI and homozygosity for the V37I GJB2 missense mutation. Nine individuals are of Chinese ancestry, two are of unspecified Asian descent, one is of Japanese descent, one individual is of Vietnamese ancestry, one of Philippine background and one of Italian and Cuban/Caucasian background. Homozygosity for the V37I GJB2 mutation may be a more common pathogenic missense mutation in Asian populations, resulting in mild to moderate sensorineural hearing impairment. We report a presumed haplotype block specific to East Asian individuals with the V37I mutation encompassing the GJB2 gene that may account for the high prevalence in East Asian populations.
| S-EPMC3745519 | biostudies-literature