Auditory Development Research Articles

Autophagy-Mediated Synaptic Refinement and Auditory Neural Pruning Contribute to Ribbon Synaptic Maturity in the Developing Cochlea.

In rodents, massive initial synapses are formed in the auditory peripheral nervous system at the early postnatal stage, and one of the major phenomena is that the number of afferent synapses in the cochlea is significantly reduced in the duration of development. This raises the hypothesis that the number of cochlear ribbon synapses are dramatically changed with hearing development and maturation. In this study, several tracers identifying activities of autophagy were applied to estimate the level of autophagy activity in the process of ribbon synapse development in mice; further, changes in the synaptic number and spiral ganglion nerve (SGN) fibers were quantitatively measured. We found robust expression of LC3B and lysosomal-associated membrane protein 1 as well as LysoTracker in or near inner hair cells and cochlear ribbon synapses in the early stage of postnatal development. Moreover, we found a significant loss in the intensity of SGN fibers at ribbon synaptic development and hearing onset. Thus, this study demonstrates that ribbon synaptic refinement and SGN fibers pruning are closely associated with the morphological and functional maturation of ribbon synapses and that synaptic refinement and SGN fiber pruning are regulated by the robust activities of autophagy in the earlier stages of auditory development.

American Cochlear Implant Alliance Task Force Guidelines for Determining Cochlear Implant Candidacy in Children.

This article summarizes the available evidence on pediatric cochlear implantation to provide current guidelines for clinical protocols and candidacy recommendations in the United States. Candidacy determination involves specification of audiologic and medical criteria per guidelines of the Food and Drug Administration. However, recommendations for a cochlear implant evaluation also should maintain flexibility and consider a child’s skill progression (i.e., month-for-month progress in speech, language, and auditory development) and quality of life with appropriately fit hearing aids. Moreover, evidence supports medical and clinical decisions based on other factors, including (a) ear-specific performance, which affords inclusion of children with asymmetric hearing loss and single-sided deafness as implant candidates; (b) ear-specific residual hearing, which influences surgical technique and device selection to optimize hearing; and (c) early intervention to minimize negative long-term effects on communication and quality of life related to delayed identification of implant candidacy, later age at implantation, and/or limited commitment to an audiologic rehabilitation program. These evidence-based guidelines for current clinical protocols in determining pediatric cochlear implant candidacy encourage a team-based approach focused on the whole child and the family system.

Early Deletion of Neurod1 Alters Neuronal Lineage Potential and Diminishes Neurogenesis in the Inner Ear.

Neuronal development in the inner ear is initiated by expression of the proneural basic Helix-Loop-Helix (bHLH) transcription factor Neurogenin1 that specifies neuronal precursors in the otocyst. The initial specification of the neuroblasts within the otic epithelium is followed by the expression of an additional bHLH factor, Neurod1. Although NEUROD1 is essential for inner ear neuronal development, the different aspects of the temporal and spatial requirements of NEUROD1 for the inner ear and, mainly, for auditory neuron development are not fully understood. In this study, using Foxg1Cre for the early elimination of Neurod1 in the mouse otocyst, we showed that Neurod1 deletion results in a massive reduction of differentiating neurons in the otic ganglion at E10.5, and in the diminished vestibular and rudimental spiral ganglia at E13.5. Attenuated neuronal development was associated with reduced and disorganized sensory epithelia, formation of ectopic hair cells, and the shortened cochlea in the inner ear. Central projections of inner ear neurons with conditional Neurod1 deletion are reduced, unsegregated, disorganized, and interconnecting the vestibular and auditory systems. In line with decreased afferent input from auditory neurons, the volume of cochlear nuclei was reduced by 60% in Neurod1 mutant mice. Finally, our data demonstrate that early elimination of Neurod1 affects the neuronal lineage potential and alters the generation of inner ear neurons and cochlear afferents with a profound effect on the first auditory nuclei, the cochlear nuclei.

Differential auditory cortical development in left and right cochlear implanted children.

Unilateral aural stimulation has been shown to cause massive cortical reorganization in brain with congenital deafness, particularly during the sensitive period of brain development. However, it is unclear which side of stimulation provides most advantages for auditory development. The left hemisphere dominance of speech and linguistic processing in normal hearing adult brain has led to the assumption of functional and developmental advantages of right over left implantation, but existing evidence is controversial. To test this assumption and provide evidence for clinical choice, we examined 34 prelingually deaf children with unilateral cochlear implants using near-infrared spectroscopy. While controlling for age of implantation, residual hearing, and dominant hand, cortical processing of speech showed neither developmental progress nor influence of implantation side weeks to months after implant activation. In sharp contrast, for nonspeech (music signal vs. noise) processing, left implantation showed functional advantages over right implantation that were not yet discernable using clinical, questionnaire-based outcome measures. These findings support the notion that the right hemisphere develops earlier and is better preserved from adverse environmental influences than its left counterpart. This study thus provides, to our knowledge, the first evidence for differential influences of left and right auditory peripheral stimulation on early cortical development of the human brain.

Assessment of auditory processing in childhood dysphonia

IntroductionClinical experience shows that children with functional dysphonia often present disorders that are associated with abnormal auditory and emotional development. These children also struggle with voice therapy, perhaps because of difficulties with auditory control during speech. It has been hypothesized that difficulties in auditory processing in children may be an important factor in the pathogenesis of childhood dysphonia. ObjectiveThe study aimed to assess selected auditory functions in children with hyperfunctional dysphonia. Materials and methodThe study group consisted of 331 children aged from 7 to 12 years suffering from hyperfunctional dysphonia. The control group consisted of 213 children aged 7–12 years. All patients underwent ENT and phoniatric examination. All children underwent two standardized psychoacoustic tests: the Frequency Pattern Test (FPT) and the Duration Pattern Test (DPT). ResultsIn the examined material, 223 children had edematous vocal fold nodules. The largest statistically significant differences were seen in the acoustic parameters describing relative frequency changes. FPT and DPT showed statistically significant differences in children with hyperfunctional dysphonia compared to the control group. At all ages the percentage of correctly identified tone sequences was significantly lower in children with dysphonia. ConclusionChildren with hyperfunctional dysphonia have difficulties in judging the pitch and duration of auditory stimuli. Difficulties in auditory processing appear to be important in the pathomechanism of functional voice disorders. Impaired hearing processes in children with hyperfunctional dysphonia can make it difficult to obtain positive and lasting effects from voice therapy.

Diagnostic value of IT-MAIS scores by logistic regression and ROC curve in predicting the degree of hearing loss in infants and toddlers aged 0-36 months

Objective:This study is inorder to compare the early prelingual auditory development trajectory of infants and toddlers with normal hearing and different degrees of hearing loss, and to explore the value of the Infant-Toddler Meaningful Auditory Integration Scale （IT-MAIS） scores in the diagnosis of hearing loss severity in infants and toddlers aged 0-36 months. Methods:Eight hundred and forty-two cases of infant-toddler from March 2009 to March 2021 were selected as participants, including 231 cases with normal hearing and 611 cases with hearing loss. The IT-MAIS scores of participants with different degrees of hearing loss were fitted with nonlinear regression with age. By respectively constructing three logistic regression models of IT-MAIS total scores, perception scores, discrimination scores and evaluation age, the ROC curve was drawn to evaluate the diagnostic efficacy. Results:The IT-MAIS scores of children with mild, moderate, severe, and profound hearing loss participants changes with age are similar to the development of infants with normal hearing, and they all increase with age. And the more severe the hearing loss, the slower the growth rate, and the lower the peak value that can be reached. The logistic model constructed by combining IT-MAIS total score and evaluation age is the best to distinguish mild and above, moderate and above, severe and above, and profound hearing loss, whose AUC are 0.827, 0.889, 0.948, 0.946. The diagnostic efficiency is the best for infant-toddlers with profound hearing loss, with a sensitivity of 89.6% and a specificity of 88.4%. The more severe the hearing loss, the higher the discrimination accuracy of IT-MAIS and the better the diagnostic efficiency. Conclusion:The logistic model based on the IT-MAIS scale and the ROC curve method have a good efficiency in the diagnosis of hearing loss severity in infants and toddlers aged 0-36 months. When the child cannot cooperate with behavioral audiometry, the results of behavioral audiometry are unreliable, and there is no electrophysiological condition, the IT-MAIS scale is expected to evaluate the degree of infants' hearing loss as an auxiliary tool. It can understand the children's auditory function state more and provides a basis for the subsequent formulation of rehabilitation intervention strategies with certain clinical application value.

Auditory and verbal skills development post-cochlear implantation in Mandarin children with auditory neuropathy: a follow-up study

Background Cochlear implant (CI) is commonly used as one of the interventions in auditory neuropathy (AN) children. However, there are limited studies regarding the efficiency of CI in AN children. Objective This study aimed to compare the auditory and verbal skills development between the AN and typically developing (TD) children with CI. Methods The follow-up study compared the post-CI scores of questionnaires of AN and TD children about auditory and verbal skills development at 0, 1, 2, 3, 6, 9, 12, and 18 months of CI use. Results The results of auditory perception in AN and TD groups showed a significant improvement after first 3 months. Furthermore, the score was significantly lower in AN group after 18 months of CI use. The results of verbal skills in AN group showed a progressive trend after 9 months of CI use. Besides, the scores were significantly lower in AN group after 12 months of CI use. Conclusion and significance The auditory perception development in AN children with CI was rapidly improved during first 3 months, and verbal skills showed a trend of improvement after 9 months of CI use. However, the differences in auditory and verbal skills between AN and TD groups increased over time.

Prospective cohort study reveals MMP-9, a neuroplasticity regulator, as a prediction marker of cochlear implantation outcome in prelingual deafness treatment

Because of vast variability of cochlear implantation outcomes in prelingual deafness treatment, identification of good and poor performers remains a challenging task. To address this issue, we investigated genetic variants of matrix metalloproteinase 9 (MMP9) and brain-derived neurotrophic factor (BDNF) and plasma levels of MMP-9, BDNF, and pro-BDNF that have all been implicated in neuroplasticity after sensory deprivation in the auditory pathway. We recruited a cohort of prelingually deaf children, all implanted before the age of 2, and carried out a prospective observation (N = 61). Next, we analyzed the association between (i) functional MMP9 (rs20544, rs3918242, rs2234681) and BDNF (rs6265) gene variants (and their respective protein levels) and (ii) the child’s auditory development as measured with the LittlEARS Questionnaire (LEAQ) before cochlear implant (CI) activation and at 8 and 18 months post-CI activation. Statistical analyses revealed that the plasma level of MMP-9 measured at implantation in prelingually deaf children was significantly correlated with the LEAQ score 18 months after CI activation. In the subgroup of DFNB1-related deafness (N = 40), rs3918242 of MMP9 was significantly associated with LEAQ score at 18 months after CI activation; also, according to a multiple regression model, the ratio of plasma levels of pro-BDNF/BDNF measured at implantation was a significant predictor of overall LEAQ score at follow-up. In the subgroup with DFNB1-related deafness, who had CI activation after 1 year old (N = 22), a multiple regression model showed that rs3918242 of MMP9 was a significant predictor of overall LEAQ score at follow-up.

Connexin30-Deficiency Causes Mild Hearing Loss With the Reduction of Endocochlear Potential and ATP Release.

GJB2 and GJB6 are adjacent genes encoding connexin 26 (Cx26) and connexin 30 (Cx30), respectively, with overlapping expressions in the inner ear. Both genes are associated with the commonest monogenic hearing disorder, recessive isolated deafness DFNB1. Cx26 plays an important role in auditory development, while the role of Cx30 in hearing remains controversial. Previous studies found that Cx30 knockout mice had severe hearing loss along with a 90% reduction in Cx26, while another Cx30 knockout mouse model showed normal hearing with nearly half of Cx26 preserved. In this study, we used CRISPR/Cas9 technology to establish a new Cx30 knockout mouse model (Cx30−/−), which preserves approximately 70% of Cx26. We found that the 1, 3, and 6-month-old Cx30−/− mice showed mild hearing loss at full frequency. Immunofluorescence and HE staining suggested no significant differences in microstructure of the cochlea between Cx30−/− mice and wild-type mice. However, transmission electron microscopy showed slight cavity-like damage in the stria vascularis of Cx30−/− mice. And Cx30 deficiency reduced the production of endocochlear potential (EP) and the release of ATP, which may have induced hearing loss. Taken together, this study showed that lack of Cx30 can lead to hearing loss with an approximately 30% reduction of Cx26 in the present Cx30 knockout model. Hence, Cx30 may play an important rather than redundant role in hearing development.

Verbal abuse and auditory development in the fetus

Verbal abuse and auditory development in the fetus

Особенности раннего подключения речевого процессора у пациентов после кохлеарной имплантации

Hearing loss affects the entire course of a child’s development, especially if it occurred at an early age. At the same time, features of auditory, cognitive, and emotional-personal development are noted. Hearing loss limits social activity, communication, and psychoemotional comfort. The widespread spread of the disease presents a serious problem for public health. In the context of the spread of the new coronavirus infection COVID-19, the epidemiological picture in different regions and within them may change; in this regard, there are certain difficulties with hospitalization of patients, which are associated with an increased risk of morbidity and the collection of all the necessary analyses. To solve this problem, an early connection is used without patients being discharged from the hospital after surgery. Cochlear implantation followed by early intensive rehabilitation can allow patients to master speech, language, and social skills as soon as possible, which can be successfully used during a critical period when children master speech. The purpose of this article is to identify and compare the perceived advantages of early connection of the cochlear implantation system processor: early start of settings, allowing the child to have physical hearing, on the basis of which spontaneous oral speech will form naturally.

Effect of Maternal Sensitiveness and Demographic Variables on Auditory and Speech Outcomes in Pediatric Cochlear Implant Users-Prospective Study

Background: Congenitally deaf children receive cochlear implantation at a young age to gain almost ordinary language development. Improvements in children’s functioning after cochlear implantation are noted particularly in their spoken language and ability to communicate. However, the rehabilitation technique following pediatric cochlear implantation rests heavily on parental involvement in some important dimensions. Methods: A prospective study was carried out to determine maternal sensitiveness using parental questionnaire administered on parents of 205 children who have undergone Cochlear implant surgery and aural rehabilitation. The parental questionnaire was administered through telephone monitoring interview and face to face interview, Results: A significant difference between parental questionnaires in terms of auditory and speech outcome measure were observed. On analysis maternal education and age of implantation has a significant effect on parental sensitiveness score and auditory and speech outcome scores. No significant correlation was observed between implant ages, number of siblings with parental sensitiveness, CAP & SIR score. Conclusion: This study shows that maternal sensitiveness or responsiveness towards cochlear implant recipient, higher maternal education, implantation within critical period and demographic variables like family structure had greater effect on auditory and speech development in cochlear implant recipient.

Establishment of Reference Values for Early Auditory Preverbal Skills of Children with Cochlear Implants.

Auditory and verbal abilities of children with early cochlear implant (CI) surgery usually take some time to develop, and a reference index of early development for horizontal comparisons is urgently needed to guide the mapping process and adjust rehabilitation programs. Therefore, the aim of this study was to establish a reference value for early auditory preverbal skills development in children with CI and investigate the developmental curve and influencing factors for these children. The LittlEARS® Auditory Questionnaire (LEAQ) scores of 287 eligible Chinese participants were obtained at 1, 2, 3, 6, 9, 12, 18, 24, 28, and 36 months interval after CI activation. The median and standard deviation of the LEAQ score of each hearing stage for these children with different activation ages were calculated to establish the reference values. Quadratic regression was conducted to fit the expected developmental curve of the LEAQ score ( [0 to 20 months]). With a linear mixed-effects model, we found that the receptive auditory behavior increased rapidly after CI activation, while expressive language skills developed at a steady rate. We also found that the following factors all significantly influenced the LEAQ: the duration of CI use, the development quotient, age of implantation and activation, and the presence of large vestibular aqueduct syndrome or auditory neuropathy spectrum disorder. The reference values and the expected developmental curve for the LEAQ in children with CI established by the present study provides guidance to clinicians and parents as well as realistic expectations regarding language and speech outcomes.

Алгоритм диагностического обследования и хирургического этапа кохлеарной имплантации у пациентов с различной этиологией врожденной глухоты

Over the past few decades, in the world scientific literature devoted to the problems of auditory-speech rehabilitation, there has been an increased interest in studying the etiological features of congenital deafness. A deep understanding of the etiology and mechanisms of deafness development helped optimize the various stages of cochlear implantation and, thereby, increase its effectiveness. Objective. To develop an algorithm for obtaining the earliest possible information about the causative factor of congenital deafness for timely planning of rehabilitation measures, reducing the cost of excessive diagnostic search, and achieving the most effective results in the auditory and speech development of children. Patients and methods. From 2018 to 2021, a study of the etiological spectrum as well as the characteristics of cochlear implantation in patients with various etiologies of congenital deafness was conducted at the Saint Petersburg Research Institute for Ear, Throat, Nose, and Speech. We examined 100 patients under the age of 3 years (mean age 2,15 ± 0,2 years), of which 58 were female, and 42 were male. All patients had congenital bilateral deafness on newborn audiological screening and were candidates for cochlear implantation. Results and discussion. According to the data obtained, 37 patients (16 boys, 21 girls, mean age 2,3 ± 0,3 years) had congenital cytomegalovirus infection as an etiological factor in deafness. The presence of signs of congenital deafness of genetic etiology was detected in 58 patients (24 boys, 34 girls, mean age 1,9 ± 0,2 years): 43 of them with nonsyndromic deafness and 15 patients with syndromic deafness (4 boys, 11 girls, mean age 2,1 ± 0,4 years). In 5 patients, the etiological factor of congenital deafness could not be established even at the present stage of diagnostic search.

Peran Orang Tua Upaya Peningkatan Pembelajaran Anak Usia Dini Dengan Media Digital Interaktif

Interactive digital media is a computer-based learning media that contains images, narration, music, games and animations that can train the visual and auditory development of PAUD students with interactive designs so that they are able to train children's motor skills. Early childhood is a child aged 0-6 years or also called the golden egg or golden child, and is a very important age to receive maximum learning so that this period affects the future of the child. So, parents must be active in supervising their child's learning wisely and purposefully. Interactive digital media can provide variety and innovation in children's learning and as a refresher in the process, it can also make children happier without any element of coercion.

Bilateral EABR stimulating mode testing bilateral CI patients refecting binaural integration:a preliminary study

ObjectiveTo analyze binaural integration, we used a new stimulation mode of the electrically evoked auditory brainstem response (EABR), to reflect bilaterally implanted cochlear function. DesignEABR was tested using the following procedure: First, both ears were evaluated separately, with the contralateral speech processor closed (C), followed by another measurement with both processors open (O). Subsequently, the eV latencies and amplitudes were assessed. The Speech, Spatial, and Qualities of Hearing Scale (SSQ), Categories of auditory performance (CAP) and speech intelligibility rating (SIR) scores were used to assess binaural hearing ability subjectively. Study sampleFifteen subjects with bilateral CI from 1997 to 2018 were recruited, each diagnosed with severe to profound hearing loss. ResultsAll SSQ scores, except for one, were greater than six (the exception scored 1.3/0.8/1.0). All CAP/SIR scores except one were greater than 6/4 (the exception scored 0/1). All patients exhibited good quality EABR measurements. The open contralateral processor significantly reduced the eV latency while enhancing the eV amplitude compared to monaural stimulation. The objective EABR results were consistent with subjective speech perception and auditory ability assessed using the SSQ scale. ConclusionThe EABR accurately reflected auditory pathway maturation and development after CI; thus, reflecting accordance with subjective speech and hearing performances. Furthermore, bilateral CI facilitates binaural integration and auditory brainstem plasticity.

Auditory Behavior and Language Development in Children with Simultaneous Bilateral Cochlear Implants During the First Two Years After Surgery

Auditory Behavior and Language Development in Children with Simultaneous Bilateral Cochlear Implants During the First Two Years After Surgery

GENETIC FOUNDATIONS OF THE INDIVIDUAL’S MUSICAL ABILITIES DEVELOPMENT

The article presents an overview of available foreign publications on the genetics of musicality, which is a new direction of modern science (see list of references). It is emphasized that national psychologists define the following components of musicality structure: emotional response to music as its center; the sense of rhythm; the sense of mode, and musical auditory perceptions. At the same time, foreign scholars consider musical pitch, the sense of rhythm, and the ability to experience the pleasure of music as the criteria of musicality. Foreign researchers have linked 36 genes and 7 chromosome regions with those traits. Thus, the genes AVPR1, GALM, GATA2, PCDH7, SLC6A4, and chromosome regions 4q22, 4q23, and 8q13-21 with still unidentified genes correlate most closely with musicality (Szyfter & Witt, 2020). Among the above-mentioned genes, genes GATA2 and PCDH7 are involved in the auditory system development and functioning. Genes SLC6A4, and AVPR1 are responsible for getting pleasure from music, and GALM conducts carbohydrate metabolism. According to Tan, McPherson, Peretz, Berkovic & Wilson, the standard expression of TRPA1 (8q21.11) provides absolute pitch and a sense of rhythm (2014). However, absolute pitch is typical only for 1-2% of professional musicians, which gives reason to doubt it is necessary to achieve a musical career (Jobling, 2014).
 The authors suggest that further research on the genetics of musicality should be based on the technology of broad genome associations research. Its value is not determined by the genes involved in musicality identification but by distinguishing the minor structural variations in the DNA molecule. These variations can alter the functioning of relevant genes in a wide range and, thus, vary the level of general musicality or special musical abilities. The identified set of genetic variations will serve as a marker for early diagnosis of musically-gifted children to ensure their successful education, upbringing, and development in the process of mandatory and regular musical and practical activities.

Peran Orang Tua Upaya Peningkatan Pembelajaran Anak Usia Dini Dengan Media Digital Interaktif

Interactive digital media is a computer-based learning media that contains images, narration, music, games and animations that can train the visual and auditory development of PAUD students with interactive designs so that they are able to train children's motor skills. Early childhood is a child aged 0-6 years or also called the golden egg or golden child, and is a very important age to receive maximum learning so that this period affects the future of the child. So, parents must be active in supervising their child's learning wisely and purposefully. Interactive digital media can provide variety and innovation in children's learning and as a refresher in the process, it can also make children happier without any element of coercion.

Aberrant Auditory and Visual Memory Development of Children with Upper Limb Motor Disorders.

The current study aimed to compare differences in the cognitive development of children with and without upper limb motor disorders. The study involved 89 children from 3 to 15 years old; 57 children with similar upper limb motor disorders and 32 healthy children. Our results showed that motor disorders could impair cognitive functions, especially memory. In particular, we found that children between 8 and 11 years old with upper limb disorders differed significantly from their healthy peers in both auditory and visual memory scales. These results can be explained by the fact that the development of cognitive functions depends on the normal development of motor skills, and the developmental delay of motor skills affects cognitive functions. Correlation analysis did not reveal any significant relationship between other cognitive functions (attention, thinking, intelligence) and motor function. Altogether, these findings point to the need to adapt general habilitation programs for children with motor disorders, considering the cognitive impairment during their development. The evaluation of children with motor impairment is often limited to their motor dysfunction, leaving their cognitive development neglected. The current study showed the importance of cognitive issues for these children. Moreover, early intervention, particularly focused on memory, can prevent some of the accompanying difficulties in learning and daily life functioning of children with movement disorders.