Factors associated with the anxiety score of deaf and hearing mothers. / Fatores associados ao escore de ansiedade de mães surdas e ouvintes.

PURPOSE: To associate maternal anxiety with sociodemographic factors, breastfeeding practices, oral habits, and the child's entry into daycare among deaf and hearing (non-deaf) mothers. METHODS: This retrospective comparative cross-sectional study included 116 mothers (29 deaf and 87 hearing) of children aged between two and five years. Deaf mothers belonged to a reference center in the city, while hearing mothers were contacted in public daycares where their children were enrolled. Mothers underwent interviews covering socio-economic factors and child development-related aspects. Additionally, they completed the Brazilian Beck Anxiety Inventory, adapted for both deaf and hearing individuals, serving as instruments to assess anxiety. The Kolmogorov-Smirnov normality test, Kruskal Wallis test, Mann-Whitney test, and Poisson Regression were employed for statistical analyses (p<0.05). RESULTS: Deaf mothers exhibited anxiety scores one and a half times higher than hearing mothers. Moreover, mothers of children with thumb-sucking habits showed higher anxiety scores, while mothers whose children started attending daycare as infants demonstrated lower anxiety scores compared to mothers of children without such habits and who did not attend daycare. CONCLUSION: Deaf mothers displayed higher anxiety levels when compared to hearing mothers. Children's behaviors, such as thumb-sucking habits, and early enrollment in daycare during the first year of life influenced maternal anxiety.

OBJETIVO: Associar a ansiedade materna aos fatores sociodemográficos, pratica de aleitamento, hábitos bucais e ingresso da criança em creche entre mulheres surdas e ouvintes. MÉTODO: Participaram deste estudo transversal retrospectivo comparativo, 116 mães (29 surdas e 87 ouvintes) de crianças na faixa etária entre dois e cinco anos. As mães surdas pertenciam a um centro de referência da cidade e as mães ouvintes foram contatadas em creches públicas, onde seus filhos estavam matriculados. As mães foram submetidas a entrevista sobre fatores socioeconômicos e relacionados ao desenvolvimento dos filhos, além de realizarem o preenchimento do Inventário Brasileiro de Ansiedade de Beck, nas versões para surdos e ouvintes, que foram instrumentos usados para avaliar a ansiedade. O teste de normalidade de Kolmogorov-Smirnov, os testes de Kruskal Wallis, Mann-Whitney e Regressão de Poisson foram utilizados para análises estatísticas (p <0,05). RESULTADOS: Mães surdas apresentaram escore de ansiedade uma vez e meia maior que mães ouvintes. Além disso, mães de crianças com hábito de sucção de dedo apresentaram maior escore de ansiedade e mães cujos filhos começaram a frequentar a creche ainda bebês apresentaram menor escore de ansiedade, quando comparados a crianças sem o hábito e que não frequentavam a creche. CONCLUSÃO: Mães surdas apresentaram maior ansiedade quando comparadas às ouvintes. Comportamento dos filhos com hábitos de sucção de dedo e o ingresso em creches no primeiro ano de vida influenciaram a ansiedade materna.

[Late-onset hereditary hearing loss caused by TMPRSS3 compound heterozygous mutations].

Objective:This study aims to identify the genetic etiology underlying late-onset hearing loss in two unrelated Chinese families. Methods:Detailed clinical data of recruited participants of two families were collected and analyzed using next-generation sequencing, combined with Sanger sequencing and bioinformatics tools. Results:Patients in both families manifested as down-sloping audiograms, mainly with severe mid-to-high frequency hearing loss as well as decreased speech recognition rate, both of which occurred during the second decade. Next-generation sequencing panels succeeded in identifying mutations in gene TMPRSS3, and three heterozygous mutations were screened out, among which c. 383T>C was the first reported mutation. In silico functional analysis and molecular modeling defined the five mutations as "pathogenic" or "likely pathogenic" according to official guideline. Conclusion:The novel mutation combinations in TMPRSS3 gene segregated with an exclusive auditory phenotype in the two pedigrees. Our results provided new data regarding the characteristic deafness caused by TMPRSS3 mutations during adolescent period when hearing should be closely monitored.

Teletherapy to address language disparities in deaf and hard-of-hearing children: study protocol for an inclusive multicentre clinical trial.

INTRODUCTION: Children who are deaf or hard-of-hearing (DHH) are at risk for speech and language delay. Language outcomes are worse in DHH children from lower socioeconomic backgrounds, due in part to disparities in access to specialised speech-language therapy. Teletherapy may help improve access to this specialised care and close this language gap. Inclusion of diverse DHH children in prospective randomised clinical trials has been challenging but is necessary to address disparities and pursue hearing health equity. Stakeholder input regarding decisions on study design elements, including comparator groups, masking, assessments and compensation, is necessary to design inclusive studies. We have designed an inclusive, equitable comparativeness effectiveness trial to address disparities in paediatric hearing health. The specific aims of the study are to determine the effect of access to and utilisation of speech-language teletherapy in addressing language disparities in low-income children who are DHH. METHODS AND ANALYSIS: After stakeholder input and pilot data collection, we designed a randomised clinical trial and concurrent longitudinal cohort trial to be conducted at four tertiary children's hospitals in the USA. Participants will include 210 DHH children aged 0-27 months. 140 of these children will be from lower income households, who will be randomised 1:1 to receive usual care versus usual care plus access to supplemental speech-language teletherapy. 70 children from higher income households will be simultaneously recruited as a comparison cohort. Primary outcome measure will be the Preschool Language Scales Auditory Comprehension subscale standard score, with additional speech, language, hearing and quality of life validated measures as secondary outcomes. ETHICS AND DISSEMINATION: This study was approved by the Institutional Review Boards of the participating sites: the University of California, San Francisco (19-28356), Rady Children's Hospital (804651) and Seattle Children's Hospital (STUDY00003750). Parents of enrolled children will provide written informed consent for their child's participation. Professional and parent stakeholder groups that have been involved throughout the study design will facilitate dissemination and implementation of study findings via publication and through national and regional organisations. TRIAL REGISTRATION NUMBER: NCT04928209.

Heterozygous variants in transmembrane channel-like 1 gene cause autosomal recessive nonsyndromic hearing loss.

Autosomal recessive non-syndromic hearing loss (ARNSHL) can cause severe or very severe pre-speech hearing loss. Transmembrane channel-like 1 (TMC1) gene is the sixth deafness gene discovered, but the precise extent of its protein structure and function is unknown. First, history collection, audiology examination and imaging examination were performed on the proband and his family members. Peripheral blood of proband and family members was collected, genomic DNA was extracted, exon high-throughput sequencing technology was used to detect the deafness gene mutation of the proband, and Sanger sequencing was performed to verify the TMC1 gene of the proband's parents. The proband was born with hearing impairment, normal tympanic function, inability to induce acoustic reflex in both ears (acoustic reflex threshold is 100 dBHL), and severe sensorineural deafness. One of his sisters has severe sensorineural hearing loss, and neither his parents nor his other sister is hearing impaired. High-throughput sequencing of the proband identified mutations at c.741+3_741+6delAAGT (splicing) and c.884C>T (p.A295V) of the TMC1 gene, two of which were heterozygous mutations. Sanger sequencing confirmed that the c.884C > T mutation was inherited from the mother, while the c.741+3_741+6delAAGT mutation was derived from the father. Prediction of amino acid function suggested that both mutations were pathogenic mutations. In conclusion, we found a new pathogenic complex heterozygous mutation of the TMC1 gene, which enriched the mutation spectrum of the TMC1 gene and provided a basis for genetic counseling and prenatal diagnosis of ARNSHL.

A cost-utility analysis of cochlear implants for single sided deafness in adults and children in the Netherlands.

BACKGROUND: Cochlear Implant (CI) has been shown to improve speech comprehension, sound localization and tinnitus in adults with Single-Sided-Deafness (SSD) compared to standard treatment currently available in the Dutch setting such as a CROS (Contralateral Routing of Signals) hearing device or a BCD (Bone Conduction Device). Also, for the pediatric population with SSD, CI has shown to be clinically meaningful. Because currently no information is available on the health economic effects of CI in adults and children with SSD in the Netherlands, a cost-utility analysis was conducted. METHODS: We developed a Markov cohort model, for both the adult and pediatric SSD population, with three states: implant, no implant, and dead. CI was compared with the Bone Conduction Device (BCD) treatment, requiring surgery and no specific treatment. The time horizon of the model was lifelong, costs were discounted with 3% and effects with 1.5%. A societal perspective was taken, including productivity costs in the analysis, with costing data based on publicly available prices for the Netherlands. Values for clinical outcome parameters, i.e. hearing gain, and event probabilities were based on existing literature. Deterministic and probabilistic sensitivity analyses as well as scenario analyses were performed to outline uncertainty of individual and combined parameters. RESULTS: Mean per patient costs for CI in the adult population were €194,051 (95%-CrI €177,274 to €211,108) compared to the total costs of €185,310 (95%-CrI €182,367 to €194,142) for BCD resulting in a cost difference of €8,826 (95%-CrI -€5,020 to €18,252). Compared to no treatment, the cost difference was -€25,089 (95%-CrI -€31,678 to -€6,003). Adults who were treated with CI gained 18.41 (95%-CrI 18.07 to 18.75) quality adjusted life years (QALY) whereas BCD patients gained 15.81 QALYs (95%-CrI 15.53 to 16.10), a difference of 2.60 QALYs (95%-CrI 2.15 to 3.05). The Incremental Cost Effectiveness Ratio (ICER) for adults with CI was determined to be €3,494/QALY gained. Patient without treatment gained 13.46 QALY (95%-CrI 13.20 to 13.73), a difference of 4.95 (95%-CrI 4.87 to 5.01) resulting in CI dominating no treatment. The ICER remained below the Dutch threshold of €20,000/QALY. The probabilistic sensitivity analyses confirmed the results. For children, CI dominated when compared to BCD and when compared to no treatment. Compared to BCD, CI led to a cost saving of €29,611 (95%-CrI -€126,800 to €54,375) and compared to no treatment, CI resulted in a cost saving of €57,658 (95%-CrI -€146,687 to €5,919). The incremental QALY gain compared to BCD was 7.22 (95%-CrI 4.19 to 8.55) and 26.03 (95%-CrI 20.82 to 31.06) compared to no treatment. CONCLUSIONS: Based on the results of this health economic evaluation with a Markov cohort model, it is very likely that CI is cost-effective compared to BCD and to no treatment in the Dutch adult and pediatric population with SSD. In both populations the ICER was below the Dutch cost-effectiveness threshold of €20,000/QALY.

[Early auditory processing of speech in prelingually-deafened children with cochlear implants based on event-related potentials].

Objective: To investigate the early auditory discrimination of vowels, consonants and lexical tones in prelingually-deafened children with cochlear implants (CI) using auditory event-related potentials. Methods: Nineteen prelingually-deafened CI children and 19 normal hearing (NH) children were recruited in this study. A multi-deviant oddball paradigm was constructed using the monosyllable/ta1/as the standard stimulus and monosyllables/tu1/,/te1/, /da1/,/ra1/,/ta4/and/ta2/as the deviant stimuli. The event-related potentials evoked by vowel, consonant and lexical tone contrasts were recorded and analyzed in the two groups. Results: NH children showed robust mismatch negativities (MMNs) to vowel, consonant and lexical tone contrasts (P<0.05), whereas CI children only showed positive mismatch responses (pMMRs) and P3a responses to the vowel (P<0.05) and consonant contrasts (P<0.05) and no significant event-related potential to the lexical tone contrasts (P>0.05). The longer pMMR and P3a peak latencies (P<0.01) but similar amplitudes (P>0.05) were found in CI children than in NH children. CI children showed weaker phase synchronization of θ oscillations than NH children (P<0.05). The duration of CI use was positively correlated with the scores of Categories of Auditory Performance (CAP) (P=0.004), Speech Intelligibility Rate (SIR) (P=0.044) and Meaningful Auditory Integration Scale (MAIS) (P=0.001) in CI children. Conclusions: Prelingually-deafened CI children can process vowels and consonants at an early stage. However, their ability of processing speech, especially lexical tones, is still more immature compared with their NH peers. The event-related potentials could be objective electrophysiological indicators reflecting the maturity of CI children's auditory speech functions. Long-term CI use is beneficial for prelingually-deafened children to improve auditory and speech performance.

An approach designed to fail deaf children and their parents and how to change it.

The matter of raising and educating deaf children has been caught up in percepts of development that are persistently inaccurate and at odds with scientific research. These percepts have negatively impacted the health and quality of life of deaf children and deaf people in general. The all too prevalent advice is to raise the child strictly orally and wait to see what happens. Only when the child is seriously behind is a completely accessible language - a sign language - introduced, and that is far too late for protecting cognitive health. The medical profession, along with others, needs to offer parents better advice and better supports so that neither the children nor their parents wait and watch as the oral-only method fails. All must take responsible action to assure an approach that succeeds.

Novel compound heterozygous variants in MARVELD2 causing autosomal recessive hearing loss in two Chinese families.

BACKGROUND: Hereditary hearing loss is an important component of congenital hearing loss. MARVELD2 (OMIM ID:610572), located in the DFNB49 locus, which encodes a tight junction protein tricellulin playing an important role in the sensory epithelial barrier of the inner ear, may contribute to nonsyndromic autosomal recessive hereditary hearing loss. METHODS: Two Han Chinese pedigrees with hearing loss underwent clinical and genetic analyses. Variants were detected by targeted next-generation sequencing and sequencing data were compared with the Human Genome Reference (GRCh 37/hg 19) to identify mutant genes and loci. Furthermore, online tools such as RDDC, SpliceAI, and REVEL were used to predict risks from different variants. RESULTS: Both two probands failed neonatal hearing screening and were diagnosed with sensorineural hearing loss. A total of 3 mutations were detected in the two families, c.1331+1G>A, c.1325A>G, and c.782G>A. According to ACMG/AMP guidelines, they were judged to be pathogenic, uncertain significance, and uncertain significance, respectively. CONCLUSIONS: These findings contribute to a better understanding of the relationship between different variants of MARVELD2 and hearing. This could further expand the spectrum of deafness gene mutations and contribute to deafness patient management and genetic counseling.

Deafness DFNB128 Associated with a Recessive Variant of Human MAP3K1 Recapitulates Hearing Loss of Map3k1-Deficient Mice.

Deafness in vertebrates is associated with variants of hundreds of genes. Yet, many mutant genes causing rare forms of deafness remain to be discovered. A consanguineous Pakistani family segregating nonsyndromic deafness in two sibships were studied using microarrays and exome sequencing. A 1.2 Mb locus (DFNB128) on chromosome 5q11.2 encompassing six genes was identified. In one of the two sibships of this family, a novel homozygous recessive variant NM_005921.2:c.4460G>A p.(Arg1487His) in the kinase domain of MAP3K1 co-segregated with nonsyndromic deafness. There are two previously reported Map3k1-kinase-deficient mouse models that are associated with recessively inherited syndromic deafness. MAP3K1 phosphorylates serine and threonine and functions in a signaling pathway where pathogenic variants of HGF, MET, and GAB1 were previously reported to be associated with human deafness DFNB39, DFNB97, and DFNB26, respectively. Our single-cell transcriptome data of mouse cochlea mRNA show expression of Map3k1 and its signaling partners in several inner ear cell types suggesting a requirement of wild-type MAP3K1 for normal hearing. In contrast to dominant variants of MAP3K1 associated with Disorders of Sex Development 46,XY sex-reversal, our computational modeling of the recessive substitution p.(Arg1487His) predicts a subtle structural alteration in MAP3K1, consistent with the limited phenotype of nonsyndromic deafness.

Investigating the crowding effect on letters and symbols in deaf adults.

Reading requires the transformation of a complex array of visual features into sounds and meaning. For deaf signers who experience changes in visual attention and have little or no access to the sounds of the language they read, understanding the visual constraints underlying reading is crucial. This study aims to explore a fundamental aspect of visual perception intertwined with reading: the crowding effect. This effect manifests as the struggle to distinguish a target letter when surrounded by flanker letters. Through a two-alternative forced choice task, we assessed the recognition of letters and symbols presented in isolation or flanked by two or four characters, positioned either to the left or right of fixation. Our findings reveal that while deaf individuals exhibit higher accuracy in processing letters compared to symbols, their performance falls short of that of their hearing counterparts. Interestingly, despite their proficiency with letters, deaf individuals didn't demonstrate quicker letter identification, particularly in the most challenging scenario where letters were flanked by four characters. These outcomes imply the development of a specialized letter processing system among deaf individuals, albeit one that may subtly diverge from that of their hearing counterparts.

A patient with 18p11.32-p11.21 deletion have monaural deafness caused by an inadequate haplodose of THOC1: A case report.

BACKGROUND: THOC1 mutation causes Deafness, autosomal dominant 86 [OMIM: 620280]. However, it has not been reported whether deletion of the THOC1 gene causes deafness. METHODS: Here, we report a 1-year-old girl with clinical features including Hypotonia, unilateral deafness in the right ear, and widening of lateral ventricles in 6 months. Gene mutations were identified by whole-exome sequencing. RESULTS: Through whole-exome sequencing, a deletion of 18p11.32-p11.21 contains the deletion of all THOC1 genes found in the patient but not in her parents' genomic DNA. The ClinGen Database Haplodose Insufficiency (HI) prediction tool determined that HI, THOC1 HI may cause unilateral deafness. Moreover, after 6 months of rehabilitation training, muscle tone returned to normal. However, at the age of 1 year, the patient developed symptoms of a large liver and hamartoma of both kidneys. CONCLUSION: From the above results, we propose that in our patient, THOC1 HI may cause unilateral deafness. Therefore, this study provides a new THOC1 deletion associated with unilateral deafness.

Duration of cochlear implant use in children with prelingual single-sided deafness is a predictor of word perception in the CI ear.

As part of a longitudinal study regarding the benefit of early cochlear implantation for children with single-sided deafness, the current work explored the children's daily device use, potential barriers to full-time device use, and the children's ability to understand speech with the cochlear implant (CI). Data were collected from 20 children with prelingual SSD who received a CI before the age of 2.5 years, from the initial activation of the sound processor until the children were 4.8 to 11.0 years old. Daily device use was extracted from the CI's data logging, while word perception in quiet was assessed using direct audio input to the children's sound processor. The children's caregivers completed a questionnaire about habits, motivations, and barriers to device use. The children with SSD and a CI used their device on average 8.3 h per day, corresponding to 63 % of their time spent awake. All children except one could understand speech through the CI, with an average score of 59 % on a closed-set test and 73 % on an open-set test. More device use was associated with higher speech perception scores. Parents were happy with their decision to pursue a CI for their child. Certain habits, like taking off the sound processor during illness, were associated with lower device use. Providing timely counselling to the children's parents, focused on SSD-specific challenges, may be helpful to improve daily device use in these children.

Novel Cases of Non-Syndromic Hearing Impairment Caused by Pathogenic Variants in Genes Encoding Mitochondrial Aminoacyl-tRNA Synthetases.

Dysfunction of some mitochondrial aminoacyl-tRNA synthetases (encoded by the KARS1, HARS2, LARS2 and NARS2 genes) results in a great variety of phenotypes ranging from non-syndromic hearing impairment (NSHI) to very complex syndromes, with a predominance of neurological signs. The diversity of roles that are played by these moonlighting enzymes and the fact that most pathogenic variants are missense and affect different domains of these proteins in diverse compound heterozygous combinations make it difficult to establish genotype-phenotype correlations. We used a targeted gene-sequencing panel to investigate the presence of pathogenic variants in those four genes in cohorts of 175 Spanish and 18 Colombian familial cases with non-DFNB1 autosomal recessive NSHI. Disease-associated variants were found in five cases. Five mutations were novel as follows: c.766C>T in KARS1, c.475C>T, c.728A>C and c.1012G>A in HARS2, and c.795A>G in LARS2. We provide audiograms from patients at different ages to document the evolution of the hearing loss, which is mostly prelingual and progresses from moderate/severe to profound, the middle frequencies being more severely affected. No additional clinical sign was observed in any affected subject. Our results confirm the involvement of KARS1 in DFNB89 NSHI, for which until now there was limited evidence.

Auditory and Visual Hallucinations in a Congenitally Deaf Patient with Schizophrenia: A Case Report and Brief Literature Review.

This report discusses the case of a 54-year-old woman with a complex psychiatric history including schizophrenia, tardive dyskinesia, borderline intellectual function, and congenital deafness that reported auditory and visual hallucinations during an acute exacerbation of schizophrenia. After resuming a previous lithium regimen and introducing olanzapine, the patient improved and was discharged without hallucinations. In our report we explore some of the challenges we faced, discuss similar cases, and examine the unresolved debate about whether congenitally deaf patients can experience auditory hallucinations.

Lexical tone recognition in multi-talker babbles and steady-state noise by Mandarin-speaking children with unilateral cochlear implants or bimodal hearing.

BACKGROUND AND OBJECTIVES: Lexical tone presents challenges to cochlear implant (CI) users especially in noise conditions. Bimodal hearing utilizes residual acoustic hearing in the contralateral side and may offer benefits for tone recognition in noise. The purpose of the present study was to evaluate tone recognition in both steady-state noise and multi-talker babbles by the prelingually-deafened, Mandarin-speaking children with unilateral CIs or bimodal hearing. METHODS: Fifty-three prelingually-deafened, Mandarin-speaking children who received CIs participated in this study. Twenty-two of them were unilateral CI users and 31 wore a hearing aid (HA) in the contralateral ear (i.e., bimodal hearing). All subjects were tested for Mandarin tone recognition in quiet and in two types of maskers: speech-spectrum-shaped noise (SSN) and two-talker babbles (TTB) at four signal-to-noise ratios (-6, 0, +6, and +12 dB). RESULTS: While no differences existed in tone recognition in quiet between the two groups, the Bimodal group outperformed the Unilateral CI group under noise conditions. The differences between the two groups were significant at SNRs of 0, +6, and +12 dB in the SSN conditions (all p < 0.05), and at SNRs of +6 and +12 dB of TTB conditions (both p < 0.01), but not significant at other conditions (p > 0.05). The TTB exerted a greater masking effect than the SSN for tone recognition in the Unilateral CI group as well as in the Bimodal group at all SNRs tested (all p < 0.05). Among demographic or audiometric variables, only age at implantation showed a weak but significant correlation with the mean tone recognition performance under the SSN conditions (r = -0.276, p = 0.045). However, when Bonferroni correction was applied to the correlation analysis results, the weak correlation became not significant. CONCLUSION: Prelingually-deafened children with CIs face challenges in tone perception in noisy environments, especially when the noise is fluctuating in amplitude such as the multi-talker babbles. Wearing a HA on the contralateral side when residual hearing permits is beneficial for tone recognition in noise.

Targeted Next-Generation Sequencing Analysis Reveals a Novel Genetic Variant in MYO6 Gene in an Indian Family with Postlingual Nonsyndromic Hearing Loss.

Background: Hereditary nonsyndromic hearing loss (NSHL) is an extremely heterogeneous disorder, both genetically and clinically. Myosin VI (MYO6) pathogenic variations have been reported to cause both prelingual and postlingual forms of NSHL. Postlingual autosomal dominant cases are often overlooked for genetic etiology in clinical setups. In this study, we used next-generation sequencing (NGS)-based targeted deafness gene panel assay to identify the cause of postlingual hearing loss in an Indian family. Methods: The proband and his father from a multigenerational Indian family affected by postlingual hearing loss were examined via targeted capture of 129 deafness genes, after excluding gap junction protein beta 2 (GJB2) pathogenic variants by Sanger sequencing. NGS data analysis and co-segregation of the candidate variants in the family were carried out. The variant effect was predicted by in silico tools and interpreted following American College of Medical Genetics and Genomics-Association for Molecular Pathology guidelines. Results: A novel heterozygous transversion c.3225T>G, p.(Tyr1075*) in MYO6 gene was identified as the disease-causing variant in this family. This stop-gained variant is predicted to form a truncated myosin VI protein, which is devoid of crucial cargo-binding domain. PCR-RFLP screening in 200 NSHL cases and 200 normal-hearing controls showed the absence of this variant indicating its de novo nature in the population. Furthermore, we reviewed MYO6 variants reported from various populations to date. Conclusions: To the best of our knowledge, this is the first family with MYO6-associated hearing loss from an Indian population. The study also highlights the importance of deafness gene panels in molecular diagnosis of GJB2-negative pedigrees, contributing to genetic counseling in the affected families.

The use of a computer-based program focused on the syllabic method to support early literacy in children with cochlear implants.

BACKGROUND: Children with cochlear implants (CIs) often lag behind children with normal hearing (NH) in early literacy skills. Furthermore, the development of language skills associated with their emergent literacy skills seems to depend on good auditory access. Supporting language acquisition and early literacy in children with CIs may prevent difficulties in primary school. The use of technology may facilitate auditory and speech recovery in children with CIs, but evidence on computer-based early literacy programs is limited. OBJECTIVE: This study investigates (a) the effects of a computer-based program focusing on the syllabic method on the literacy skills of children with CIs (CIs group), comparing them with the literacy skills of a group of age-matched NH (normal hearing) peers (NHs group); (b) the associations between language and early literacy skills in the NHs group and between language, auditory and early literacy skills in the CIs group. METHOD: Nine prelingually deaf children with CIs (M = 61.11, SD = 6.90) with severe to profound sensorineural hearing loss and nine age-matched NH children participated in the program. Categories of Auditory Performance (CAP) as measures of children's auditory skills were collected. All participants were tested on phonological, morphosyntax (grammatical comprehension and repetition), and early literacy skills (syllable blending and segmentation, syllable and word reading) (T1). Next, all children participated in the computer-based program for 12 weeks. After the program was completed (T2), only early literacy tests were administered to the children. RESULTS: Although, on average, both groups obtained higher scores in all literacy tasks at T2, the CIs group scored lower than the NHs group. In the CIs group, at T2 we found significant improvements in syllable segmentation (p = 0.042) and word reading (p = 0.035). In the NHs group, at T2 we found significant improvements in syllable segmentation (p = 0.034), syllable blending (p = 0.022), syllable reading (p = 0.008), and word reading (p = 0.009). We also found significant associations in both groups between measures of morphosyntax at T1 and measures of early literacy at T2. In addition, for the CIs group, we found significant associations between children's auditory performance at T1 and measures of morphosyntax at T1 and early literacy at T2. CONCLUSION: a computer-based program focused on the syllabic method could support children with CIs in acquiring emergent literacy abilities. The auditory performance of children with CIs seems to influence their morphosyntax and later early literacy skills.

Cortical volumetric changes after cochlear implantation in postlingually deaf adults: correlation with speech perception abilities.

This study aims to analyse the volumetric changes in brain MRI after cochlear implantation (CI), focusing on the speech perception in postlingually deaf adults. We conducted a prospective cohort study with 16 patients who had bilateral hearing loss and received unilateral CI. Based on the surgical side, patients were categorized into left and right CI groups. Volumetric T1-weighted brain MRI were obtained before and one year after the surgery. To overcome the artifact caused by the internal device in post-CI scan, image reconstruction method was newly devised and applied using the contralateral hemisphere of the pre-CI MRI data, to run FreeSurfer. We conducted within-subject template estimation for unbiased longitudinal image analysis, based on the linear mixed effect models. When analyzing the contralateral cerebral hemisphere before and after CI, a substantial increase in superior frontal gyrus and superior temporal gyrus (STG) volumes was observed in the left CI group. A positive correlation was observed in the STG and post-CI word recognition score in both groups. As far as we know, this is the first study attempting longitudinal brain volumetry based on post-CI MRI scans. We demonstrate that better auditory performance after CI is associated with structural restoration in central auditory structures.

Neural underpinnings of sentence reading in deaf, native sign language users.

The goal of this study was to investigate sentence-level reading circuits in deaf native signers, a unique group of deaf people who are immersed in a fully accessible linguistic environment from birth, and hearing readers. Task-based fMRI, functional connectivity and lateralization analyses were conducted. Both groups exhibited overlapping brain activity in the left-hemispheric perisylvian regions in response to a semantic sentence task. We found increased activity in left occipitotemporal and right frontal and temporal regions in deaf readers. Lateralization analyses did not confirm more rightward asymmetry in deaf individuals. Deaf readers exhibited weaker functional connectivity between inferior frontal and middle temporal gyri and enhanced coupling between temporal and insular cortex. In conclusion, despite the shared functional activity within the semantic reading network across both groups, our results suggest greater reliance on cognitive control processes for deaf readers, possibly resulting in greater effort required to perform the task in this group.