Language development in Japanese children who receive cochlear implant and/or hearing aid

Objectives: This study aimed to investigate a wide variety of factors that influence auditory, speech, and language development following pediatric cochlear implantation (CI). Study design: Prospective collection of language tested data in profound hearing-impaired children. Hypothesis: Pediatric CI can potentially be effective to development of practical communication skills and early implantation is more effective. Methods: We proposed a set of language tests (assessment package of the language development for Japanese hearing-impaired children; ALADJIN) consisting of communication skills testing (test for question-answer interaction development; TQAID), comprehensive (Peabody Picture Vocabulary Test-Revised; PVT-R and Standardized Comprehension Test for Abstract Words; SCTAW) and productive vocabulary (Word Fluency Test; WFT), and comprehensive and productive syntax (Syntactic processing Test for Aphasia; STA). Of 638 hearing-impaired children recruited for this study, 282 (44.2%) with >70 dB hearing impairment had undergone CI. After excluding children with low birth weight (11 points on the Pervasive Developmental Disorder ASJ Rating Scale for the test of autistic tendency, and those <2 SD on Raven's Colored Progressive Matrices for the test of non-verbal intelligence, 190 children were subjected to this set of language tests. Results: Sixty children (31.6%) were unilateral CI-only users, 128 (67.4%) were CI-hearing aid (HA) users, and 2 (1.1%) were bilateral CI users. Hearing loss level of CI users was significantly (p < 0.01) worse than that of HA-only users. However, the threshold level, maximum speech discrimination score, and speech intelligibility rating in CI users were significantly (p < 0.01) better than those in HA-only users. The scores for PVT-R (p < 0.01), SCTAW, and WET in CI users were better than those in HA-only users. STA and TQAID scores in CI-HA users were significantly (p < 0.05) better than those in unilateral CI-only users. The high correlation (r = 0.52) has been found between the age of CI and maximum speech discrimination score. The scores of speech and language tests in the implanted children before 24 months of age have been better than those in the implanted children after 24 months of age. Conclusions: We could indicate that CI was effective for language development in Japanese hearing-impaired children and early CI was more effective for productive vocabulary and syntax.ArticleINTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY. 76(3):433-438 (2012)journal articl

Effectiveness of Domain-Based Intervention for Language Development in Japanese Hearing-Impaired Children: A Multicenter Study

Objective: Decreasing language delay in hearing-impaired children is a key issue in the maintenance of their quality of life. Language training has been presented mainly by experience-based training; effective intervention programs are crucially important for their future. The aim of this study was to confirm the efficacy of 6-month domain-based language training of school-age, severe-to-profound hearing-impaired children. Methods: We conducted a controlled before-after study involving 728 severe-to-profound prelingual hearing-impaired children, including an intervention group (n = 60), control group (n = 30), and baseline study group (n = 638). Language scores of the participants and questionnaires to the caregivers/therapists were compared before and after the intervention. Average monthly increase in each language score of the control group and baseline study group were compared with those of the intervention group. Results: Language scores and the results of the questionnaire of the intervention group showed a significant improvement (P < .05). The average monthly language growth of the intervention group was twice that of the control group and 3 to 4 times that of the baseline study group (P < .05). The effect size was largest in communication (1.914), followed by syntax (0.931). Conclusion: Domain-based language training improved the language development and daily communication of hearing-impaired children without any adverse effects

Transplantation of a human induced pluripotent stem cell-derived airway epithelial cell sheet into the middle ear of rats

[Introduction] Early postoperative regeneration of the middle ear mucosa is essential for the prevention of postoperative refractory otitis media and recurrent cholesteatoma. As a means for intractable otitis media management, we focused on human induced pluripotent stem cell (hiPSC)-derived airway epithelial cells (AECs), which have been used in upper airway mucosal regeneration and transplantation therapy. In this study, we transplanted hiPSC-derived AECs into the middle ear of immunodeficient rats. [Methods] Following the preparation of AEC sheets from hiPSCs, the bilateral middle ear mucosa of X-linked severe combined immunodeficient rats was scraped, and the AEC sheets were transplanted in the ears unilaterally. [Results] Human nuclear antigen (HNA)-positive ciliated cells were observed on the transplanted side of the middle ear cavity surface in three of six rats in the 1-week postoperative group and in three of eight rats in the 2-week postoperative group. No HNA-positive cells were found on the control side. The percentage of HNA-positive ciliated cells in the transplanted areas increased in the 2-week postoperative group compared with the 1-week group, suggesting survival of hiPSC-derived AECs. Additionally, HNA-positive ciliated cells were mainly located at sites where the original ciliated cells were localized. Immunohistochemical analysis showed that the transplanted AECs contained cytokeratin 5- and mucin 5AC-positive cells, indicating that both basal cells and goblet cells had regenerated within the middle ear cavity. [Conclusions] The results of this study are an important first step in the establishment of a novel transplantation therapy for chronic otitis media

Psychiatric-disorder-related behavioral phenotypes and cortical hyperactivity in a mouse model of 3q29 deletion syndrome

3q29 microdeletion, a rare recurrent copy number variant (CNV), greatly confers an increased risk of psychiatric disorders, such as schizophrenia and autism spectrum disorder (ASD), as well as intellectual disability. However, disease-relevant cellular phenotypes of 3q29 deletion syndrome remain to be identified. To reveal the molecular and cellular etiology of 3q29 deletion syndrome, we generated a mouse model of human 3q29 deletion syndrome by chromosome engineering, which achieved construct validity. 3q29 deletion (Df/+) mice showed reduced body weight and brain volume and, more importantly, impaired social interaction and prepulse inhibition. Importantly, the schizophrenia-related impaired prepulse inhibition was reversed by administration of antipsychotics. These findings are reminiscent of the growth defects and neuropsychiatric behavioral phenotypes in patients with 3q29 deletion syndrome and exemplify that the mouse model achieves some part of face validity and predictive validity. Unbiased whole-brain imaging revealed that neuronal hyperactivation after a behavioral task was strikingly exaggerated in a restricted region of the cortex of Df/+ mice. We further elucidated the cellular phenotypes of neuronal hyperactivation and the reduction of parvalbumin expression in the cortex of Df/+ mice. Thus, the 3q29 mouse model provides invaluable insight into the disease-causative molecular and cellular pathology of psychiatric disorders

What Factors Are Associated with Good Performance in Children with Cochlear Implants? From the Outcome of Various Language Development Tests, Research on Sensory and Communicative Disorders Project in Japan: Nagasaki Experience

ObjectivesWe conducted multi-directional language development tests as a part of the Research on Sensory and Communicative Disorders (RSVD) in Japan. This report discusses findings as well as factors that led to better results in children with severe-profound hearing loss.MethodsWe evaluated multiple language development tests in 33 Japanese children with cochlear implants (32 patients) and hearing aid (1 patient), including 1) Test for question and answer interaction development, 2) Word fluency test, 3) Japanese version of the Peabody picture vocabulary test-revised, 4) The standardized comprehension test of abstract words, 5) The screening test of reading and writing for Japanese primary school children, 6) The syntactic processing test of aphasia, 7) Criterion-referenced testing (CRT) for Japanese language and mathematics, 8) Pervasive development disorders ASJ rating scales, and 9) Raven's colored progressive matrices. Furthermore, we investigated the factors believed to account for the better performances in these tests. The first group, group A, consisted of 14 children with higher scores in all tests than the national average for children with hearing difficulty. The second group, group B, included 19 children that scored below the national average in any of the tests.ResultsOverall, the results show that 76.2% of the scores obtained by the children in these tests exceeded the national average scores of children with hearing difficulty. The children who finished above average on all tests had undergone a longer period of regular habilitation in our rehabilitation center, had their implants earlier in life, were exposed to more auditory verbal/oral communication in their education at affiliated institutions, and were more likely to have been integrated in a regular kindergarten before moving on to elementary school.ConclusionIn this study, we suggest that taking the above four factors into consideration will have an affect on the language development of children with severe-profound hearing loss

Idiopathic Normal-Pressure Hydrocephalus: Temporal Changes in ADC during Cardiac Cycle

Normal-pressure hydrocephalus (NPH) is characterized by a clinical triad of ataxia, incontinence, and dementia, as well as dilated ventricles but normal cerebrospinal fluid (CSF) pressures. In patients with NPH, CSF shunt placement is effective for improving these symptoms (1). NPH has attracted attention as one of the few treatable causes of dementia. Diagnosis of idiopathic NPH (INPH) without a known cause of communicating hydrocephalus, including subarachnoid hemorrhage or meningitis, is particularly difficult (2). Moreover, to clarify the cause of NPH, accurately diagnose NPH, and identify appropriate patients for shunt surgery, several tests have been proposed, including cisternography, the CSF tap test, resistance measures, external lumbar drainage, and intracranial pressure recording, in addition to clinical findings and conventional imaging diagnosis with x-ray computed tomography or magnetic resonance (MR) imaging (3–6). It has also been reported (7) that a single standard for the prognostic evaluation of patients with INPH was lacking and that supplemental tests could increase the predictive accuracy of the prognosis. The CSF tap test is a particularly reliable examination, but it is invasive and has low sensitivity. It has been proposed that MR imaging CSF flow studies can be used to noninvasively obtain information about intracranial mechanical properties in INPH (eg, intracranial compliance) (2,8–12). However, the diagnostic utility of this latter method is still not established.Arterial inflow into the cranium induces venous and CSF outflow and displacement of the intracranial spinal cord during the cardiac cycle, resulting in pulsatile brain motion (12–14). Brain pulsation (ie, bulk motion) reportedly can give rise to artifactual phase dispersion and may lead to overestimation of the apparent diffusion coefficient (ADC) (15,16). Brockstedt et al (17) reported that ADC was not changed significantly during the cardiac cycle with the single-shot echo-planar imaging (EPI) sequence widely used in diffusion MR imaging. However, they analyzed only two delay times (100 and 400 msec) between the R peaks in the cardiac cycle. To more completely analyze ADC changes during the cardiac cycle, our own group has previously evaluated the temporal change in ADC during the entire cardiac cycle by using an electrocardiographically (ECG)-triggered single-shot diffusion EPI sequence to minimize bulk motion effect. As a result, we revealed in a previous study that white matter ADC changed significantly over the cardiac cycle and that such changes were synchronized with the arterial inflow (volume loading) at systole (18). We further hypothesize that changes in ADC during the cardiac cycle are related to the biomechanical properties of intracranial tissues; hence, observed temporal changes in ADC in diseases such as INPH that are characterized by low intracranial compliance (12) may well be different than those in other diseases. Therefore, the purpose of our study was to determine whether temporal changes in ADC over the cardiac cycle were different in patients with INPH as compared with patients with ex vacuo ventricular dilatation and healthy control subjects

NBRP databases: databases of biological resources in Japan

The National BioResource Project (NBRP) is a Japanese project that aims to establish a system for collecting, preserving and providing bioresources for use as experimental materials for life science research. It is promoted by 27 core resource facilities, each concerned with a particular group of organisms, and by one information center. The NBRP database is a product of this project. Thirty databases and an integrated database-retrieval system (BioResource World: BRW) have been created and made available through the NBRP home page (http://www.nbrp.jp). The 30 independent databases have individual features which directly reflect the data maintained by each resource facility. The BRW is designed for users who need to search across several resources without moving from one database to another. BRW provides access to a collection of 4.5-million records on bioresources including wild species, inbred lines, mutants, genetically engineered lines, DNA clones and so on. BRW supports summary browsing, keyword searching, and searching by DNA sequences or gene ontology. The results of searches provide links to online requests for distribution of research materials. A circulation system allows users to submit details of papers published on research conducted using NBRP resources

Language development in Japanese children who receive cochlear implant and/or hearing aid

Objectives: This study aimed to investigate a wide variety of factors that influence auditory, speech, and language development following pediatric cochlear implantation (CI). Study design: Prospective collection of language tested data in profound hearing-impaired children. Hypothesis: Pediatric CI can potentially be effective to development of practical communication skills and early implantation is more effective. Methods: We proposed a set of language tests (assessment package of the language development for Japanese hearing-impaired children; ALADJIN) consisting of communication skills testing (test for question-answer interaction development; TQAID), comprehensive (Peabody Picture Vocabulary Test-Revised; PVT-R and Standardized Comprehension Test for Abstract Words; SCTAW) and productive vocabulary (Word Fluency Test; WFT), and comprehensive and productive syntax (Syntactic processing Test for Aphasia; STA). Of 638 hearing-impaired children recruited for this study, 282 (44.2%) with >70 dB hearing impairment had undergone CI. After excluding children with low birth weight (11 points on the Pervasive Developmental Disorder ASJ Rating Scale for the test of autistic tendency, and those <2 SD on Raven's Colored Progressive Matrices for the test of non-verbal intelligence, 190 children were subjected to this set of language tests. Results: Sixty children (31.6%) were unilateral CI-only users, 128 (67.4%) were CI-hearing aid (HA) users, and 2 (1.1%) were bilateral CI users. Hearing loss level of CI users was significantly (p < 0.01) worse than that of HA-only users. However, the threshold level, maximum speech discrimination score, and speech intelligibility rating in CI users were significantly (p < 0.01) better than those in HA-only users. The scores for PVT-R (p < 0.01), SCTAW, and WET in CI users were better than those in HA-only users. STA and TQAID scores in CI-HA users were significantly (p < 0.05) better than those in unilateral CI-only users. The high correlation (r = 0.52) has been found between the age of CI and maximum speech discrimination score. The scores of speech and language tests in the implanted children before 24 months of age have been better than those in the implanted children after 24 months of age. Conclusions: We could indicate that CI was effective for language development in Japanese hearing-impaired children and early CI was more effective for productive vocabulary and syntax.ArticleINTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY. 76(3):433-438 (2012)journal articl