Impact of reading and writing skills on academic achievement among school-aged hearing-impaired children.

OBJECTIVES: Reading and writing skills are important for hearing-impaired children since these skills help them to develop their language skills, but the prevalence of reading/writing difficulties and its effects on language development aspects among them are unclear. In this study, we identified language development features and demographic factors of Japanese hearing-impaired children diagnosed as having reading/writing difficulties. METHODS: We analyzed data from a total of 546 sever-to-profound pre-school and elementary school hearing-impaired children for this study. Children with reading/writing difficulties (Group A) were defined as children obtaining low scores (-1.5 SD compared to others in the same grade) in the Screening Test of Reading and Writing for Japanese Primary School Children (STRAW), and we compared other language development features (communication ability, vocabulary, syntax and academic achievement) and demographic factors to those of hearing-impaired children with normal reading and writing skills (Group B). We assessed language development domains as outcomes using the Assessment of Language Development for Japanese Children (ALADJIN) package, and analyzed the results stratified by age groups (5-6, 7-8, 9-10, and 11-12 years) using multiple regression analyses. RESULTS: The prevalence of reading/writing difficulties was 20.1% among the participants. Almost all point estimates in each language development domain showed better odds ratios (OR) except Criterion Referenced Test -II (CRT-II) mathematics in 11- to 12-year-olds in fully-adjusted models. Among 9- to 10-year-olds, the ORs (95% confidence interval) for fair academic achievement measured by CRT-II were 2.60 (1.09-6.20) for Japanese and 3.02 (1.29-7.11) for mathematics in Group B, even after adjusting for possible confounding factors. CONCLUSIONS: Reading and writing are important for language development of hearing-impaired children, especially for academic achievement during the middle phase of elementary school. Screening for reading/writing difficulties is important for appropriate intervention and to prevent language and academic delays among hearing-impaired children.

Quantitative evaluation of maxillary bone deformation by computed tomography in patients with leprosy.

BACKGROUND: Facial deformation as a sequela of leprosy is caused not only by a saddle nose but also by regression of the maxilla, as well documented in paleopathological observations of excavated skeletal remains of patients with leprosy. However, maxillary changes in living patients have been evaluated only by the subjective visual grading. Here, we attempted to evaluate maxillary bone deformation in patients with leprosy using three-dimensional computed tomography (3D-CT). METHODS: Three-dimensional images centered on the maxilla were reconstructed using multiplanar reconstruction methods in former patients with leprosy (n = 10) and control subjects (n = 5); the anterior-posterior length of the maxilla (MA-P) was then measured. The difference between the MA-P of the patients and those of controls was evaluated after compensating for individual skull size. These findings were also compared with those from previous paleopathological studies. FINDINGS: Three former patients with lepromatous leprosy showed marked atrophy of the maxilla at the prosthion (-8.6, -11.1 and -17.9 mm) which corresponded with the visual appearance of the maxillary deformity, and these results were consistent with paleopathological findings of excavated skeletal remains. Additionally, the precise bone defects of the maxilla could be individually calculated for accurate reconstructive surgery. INTERPRETATION: We have successfully illustrated maxillary bone deformities in living patients with leprosy. This study also confirmed the maxillary regression described in paleopathological studies.

[Hearing loss in leprosarium: Current status].

Due to aging and aftereffects of leprosy, many of them are now struggling with hearing loss and visual impairment. The purpose of this study was to investigate and analyze the present situation of hearing level and hearing aid among residents in leprosy sanatorium. We have been carrying out hearing screening for the all ex-patients in three national sanatoriums, Nagashima-Aisein (since 2011), Oku-Komyo-en (since 2012), and Matsuoka-Hoyo-en (since 2014). The results of audiometry and questionnaire in 2014 were analyzed. Eligible persons who took pure tone audiometry were 198, and their age ranged between 59 and 99, mean 82.95. Their average hearing level of better hearing ear was 39.3 ± 17.2 dB. Since this result is not different from previous reports, which mentioned about average hearing level in elder persons, history of leprosy may not affect with hearing. According to the questionnaire, not only hearing level but also the awareness of hearing disability may lead to need for hearing aid. Also, it was considered that each communicative condition in sanatorium could have effect on self-assessment of hearing disability.

Impact of early intervention on comprehensive language and academic achievement in Japanese hearing-impaired children with cochlear implants.

OBJECTIVES: Early hearing detection and intervention (EHDI) is critical for achievement of age-appropriate speech perception and language development in hearing-impaired children. It has been 15 years since newborn hearing screening (NHS) was introduced in Japan, and its effectiveness for language development in hearing-impaired children has been extensively studied. Moreover, after over 20 years of cochlear implantation in Japan, many of the prelingual cochlear implant (CI) users have reached school age, and the effect of CI on language development have also been assessed. To identify prognostic factors for language development, audiological/language test scores and demographic factors were compared among prelingual severe-to-profound hearing-impaired children with CI divided into subgroups according to age at first hearing aid (HA) use and whether they received NHS. METHODS: Prelingual severe-to-profound deafened children from the Research on Sensory and Communicative Disorders (RSCD) project who met the inclusion criteria were divided into groups according to the age (in months) of HA commencement (before 6 months: group A, after 7 months: group B), and the presence or absence of NHS (groups C and D). Language development and socio-economic data were obtained from audiological/language tests and a questionnaire completed by caregivers, respectively. RESULTS: In total, 210 children from the RSCD project participated in this study. Group A (n=49) showed significantly higher scores on comprehensive vocabulary and academic achievement (p<0.05) than group B (n=161), with no difference in demographics except for significantly older age in group B. No differences in language scores were observed between group C (n=71) and group D (n=129), although participants of group D was significantly older and had used CIs longer (p<0.05). CONCLUSIONS: Early use of HAs until the CI operation may result in better language perception and academic achievement among CI users with prelingual deafness. A long-term follow-up is required to assess the usefulness of NHS for language development.

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.

Cervical lymph node extirpation for the diagnosis of malignant lymphoma.

PURPOSE: Lymph node enlargement in the neck is a common presentation of malignant lymphoma (ML) and requires tissue sampling for accurate diagnosis. Although delayed diagnosis may be critical for some patients, unnecessary biopsy should be avoided wherever possible. This study examined the process for determining the necessity to perform a biopsy and evaluated the value of an open biopsy as a diagnostic tool to enable definite subclassification of the disease. METHODS: The subjects included 20 patients with suspected ML who underwent cervical lymph node extirpation at Okayama Saiseikai general hospital between 2007 and 2010. The decision to perform a biopsy was made based on the results of sonographic evaluation, fine needle aspiration cytology (FNAC), and serum levels of lactate dehydrase (LDH) and soluble interleukin-2 receptor (sIL-2r). RESULTS: The diagnosis was ML in 15 patients (75%), Castleman's disease in 1 (5%), and benign lymphadenopathy in 4 (20%). CONCLUSIONS: A lymph node biopsy remains the gold standard for the diagnostic evaluation of ML. Sonographic evaluation combined with serum levels of LDH and sIL-2r is useful in determining the need for biopsy. Many of the cases of ML where it was difficult to determine whether a biopsy should be performed were relatively low grade and critical conditions could be avoided by close observation of the patient.

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.

OBJECTIVES: We 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. METHODS: We 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. RESULTS: Overall, 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. CONCLUSION: In 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.

Assessment package for language development in Japanese hearing-impaired children (ALADJIN) as a test battery for the development of practical communication.

OBJECTIVES: The measurement of language development in hearing-impaired children is an important step in assessing the appropriateness of an intervention. We proposed a set of language tests (the Assessment Package for Language Development in Japanese Hearing-Impaired Children [ALADJIN]) to evaluate the development of practical communication skills. This package consisted of communication skills (TQAID), comprehensive (PVT-R and SCTAW) and productive vocabulary (WFT), comprehensive and productive syntax (STA), and the STRAW. METHODS: A total of 638 children with greater than 70-dB hearing impairment were subjected to this set of language tests. Additional tests, including the PARS, the RCPM, and parental questionnaires, were administered to assess the backgrounds of the children. RESULTS: A trimodal distribution was observed among hearing-impaired children by the histogram-based analysis of each test. CONCLUSIONS: The ALADJIN is a useful Japanese-language evaluation kit for hearing-impaired children.

Language ability in the intermediate-scoring group of hearing-impaired children.

OBJECTIVES: Language development is a key issue in hearing-impaired children. However, interpersonal differences complicate our understanding of the situation. The bimodal or trimodal distribution of language scores in our other reports in this publication imply the presence of fundamental differences among these groups. The characteristic aspects of each group were profiled according to language data. METHODS: We divided 268 children with prelingual severe to profound hearing impairment into 3 groups according to their trimodal distribution observed on histogram-based analysis of their responses to the Test of Question-Answer Interaction Development. Test results in several language domains, including productive and comprehensive vocabulary, productive and comprehensive syntax, and academic achievement, were profiled and compared among these 3 groups. RESULTS: Significant differences were observed in the results of the Word Fluency Test, the Picture Vocabulary Test-Revised, and the Syntax Test of Aphasia among the 3 groups. No significant difference was observed between groups who were lower-scoring and intermediate-scoring on the academic achievement tests referred to as Criterion Referenced Test-II and the Standardized Comprehension Test for Abstract Words. Only the higher-scoring group showed excellent results. The demographic factors were not significantly different among the 3 groups. CONCLUSIONS: Relatively poor academic achievement despite fair language production was the dominant feature of the intermediate-scoring group. This profile might correlate with academic failure in school.

Effects of early identification and intervention on language development in Japanese children with prelingual severe to profound hearing impairment.

OBJECTIVES: Early identification and intervention for prelingual bilateral severe to profound hearing loss is supposed to reduce the delay in language development. Many countries have implemented early detection and hearing intervention and conducted regional universal newborn hearing screening (UNHS). However, the benefits of UNHS in later childhood have not yet been confirmed, although language development at school age has a lifelong impact on children's future. Our Research on Sensory and Communicative Disorders project attempted to reveal the effects of UNHS and those of early intervention on the development of verbal communication in Japanese children. METHODS: In this study, 319 children with prelingual bilateral severe to profound hearing loss, 4 to 10 years of age, were evaluated with the Test of Question-Answer Interaction Development used as an objective variable. Participation in UNHS and early intervention were used as explanatory variables. The adjusted odds ratio (AOR) was calculated after adjusting several confounding factors with use of logistic regression analysis. In addition, caregivers' answers were obtained by a questionnaire, and the process of diagnosis with and without UNHS was analyzed retrospectively. RESULTS: Early intervention was significantly associated with better language development (AOR, 3.23; p < 0.01). Participation in UNHS may contribute to better language development to some extent (AOR, 1.32), but not one that was statistically significant (p = 0.37). However, UNHS was significantly associated with early intervention (AOR, 20.21; p < 0.001). The questionnaire results indicated a lag in treatment after UNHS in more than 40% of screened cases. CONCLUSIONS: Early intervention strongly influenced language development. It is necessary to ensure that early identification leads directly to early intervention.

Language development, interpersonal communication, and academic achievement among Japanese children as assessed by the ALADJIN.

OBJECTIVES: Japanese-speaking children in a standard sample were subjected to a test battery (ALADJIN: Assessment Package for Language Development in Japanese Hearing-Impaired Children) to evaluate the effect of language development on both interpersonal communication skills and academic achievement. METHODS: A total of 414 preschool and school-age children without hearing impairment were included in this study. The following tests make up the ALADJIN: the Test of Question-Answer Interaction Development (TQAID), the Japanese Language by Criterion Referenced Test-II (CRT-II) for measuring academic achievement, the Picture Vocabulary Test-Revised (PVT-R), the Standardized Comprehension Test of Abstract Words (SCTAW), both parts of the Syntactic Processing Test for Aphasia (STA), and the Word Fluency Test (WFT). Means and standard deviations at each academic grade level were calculated, and a multiple regression analysis was performed. RESULTS: A ceiling effect was observed for the TQAID and the STA in children in grade 3 of elementary school, and the scores for the PVT-R, SCTAW, and WFT increased incrementally according to grade level. Multiple regression analysis revealed that the PVT-R, WFT, and STA (production) have predictive power for the results of the TQAID (R = 0.59; R2 = 0.58; p <0.0001), whereas the SCTAW and STA (comprehension) have predictive power for the results of the CRT-II. CONCLUSIONS: Both vocabulary and syntax are important in communication development among children. The results of our multiple regression analysis suggest that different language domains may play different roles in the development of interpersonal communication skills and in academic achievement. The development of interpersonal communication skills is largely based on productive vocabulary and syntax abilities, whereas academic achievement is largely based on comprehensive vocabulary and syntax abilities. Children who have difficulties in either area should be evaluated with detailed language assessment tools such as the ALADJIN in an effort to aid in the selection of appropriate intervention.

Syntactic development in Japanese hearing-impaired children.

OBJECTIVES: This study examined syntactic development of auditory comprehension of sentences in Japanese-speaking school-age children with and without hearing impairment. METHODS: In total, 592 preschool and school-age children (421 normal-hearing and 171 hearing-impaired) were included in this cross-sectional observation study conducted using the Syntactic Processing Test for Aphasia for Japanese language users. Linear regression analysis was used to determine the estimated age at which each syntactic structure was acquired. RESULTS: Acquisition of syntactic structures was observed in hearing-impaired and normal-hearing children. Basic word order sentences of agent-object-verb and the goal benefactive construction were acquired at preschool age (earlier group), whereas reverse word order sentences of object-agent-verb, source benefactive construction, passive voice, and relative clauses were acquired at school age (later group). The results showed that many hearing-impaired children may not acquire Japanese grammatical structures until the age of 12 years. CONCLUSIONS: Adequate screening for language development for school-age hearing-impaired children is required for an effective intervention.

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 (<1800 g), those with >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 WFT 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.

Role of macrophage migration inhibitory factor in paranasal sinus mucocele.

BACKGROUND: Little is known about the immunologic aspects and the pathogenesis of the paranasal sinus mucocele. METHODS: The fluids of paranasal sinus mucoceles were obtained from 12 subjects. The concentration of macrophage migration inhibitory factor (MIF), interleukin 1beta, tumor necrosis factor alpha, and regulated on activation normal T cell expressed and secreted (RANTES) were determined by enzyme-linked immunosorbent assay, and the levels of endotoxin were detected with kinetic Turbidimetric Assay. RESULTS: MIF and endotoxin were detected in the fluid of all samples, whereas interleukin-1beta and RANTES were detected in 1 and 3 subjects out of 12 samples. Tumor necrosis factor alpha was not detected in any of the samples. A significant positive correlation between the levels of MIF and the period with symptoms such as pain, swelling of face, and visual disturbance was observed. CONCLUSION: These findings suggest that MIF and endotoxin may play an important role in the pathogenesis of paranasal sinus mucocele. MIF may be an important factor causing the development and exacerbation of the disease.

Monosyllable speech perception of Japanese hearing aid users with prelingual hearing loss: implications for surgical indication of cochlear implant.

OBJECTIVE: The monosyllable speech perception ability after years of educational intervention was compared between prelingually deafened pediatric hearing aid users and their cochlear implant counterparts. DESIGN: An open-set monosyllabic speech perception test was conducted on all subjects. The test required subjects to indicate a corresponding Japanese character to that spoken by the examiner. Fifty-two subjects with prelingual hearing impairment (47 hearing aid users and 5 cochlear implant users) were examined. RESULTS: Hearing aid users with average pure-tone thresholds less than 90 dB HL demonstrated generally better monosyllable perception than 70%, which was equivalent or better performance than that of the cochlear implant group. Widely dispersed speech perception was observed within the 90-99 dB HL hearing-aid user group with most subjects demonstrating less than 50% speech perception. In the cluster of >100 dB HL, few cases demonstrated more than 50% in speech perception. The perception ability of the vowel part of each mora within the cochlear implant group was 100% and corresponding to that of hearing aid users with moderate and severe hearing loss. CONCLUSION: Hearing ability among cochlear implant users can be comparable with that of hearing aid users with average unaided pure-tone thresholds of 90 dB HL, after monosyllabic speech perception testing was performed.

Deletion mapping of split hand/split foot malformation with hearing impairment: a case report.

Split hand/split foot malformation (SHFM), which typically appears as lobster-like limb malformation, is a rare clinical condition caused by a partial deletion of chromosome 7q. Hearing impairment sometimes accompanies syndromic SHFM cases; a case of inner and middle ear malformation with SHFM is described in this report. We conducted a genetic evaluation of this patient and found a deleted region that overlaps a previously reported locus of SHFM as well as a DFNB14 locus that can cause nonsyndromic hearing impairment by autosomal recessive inheritance.

Language development of a multiply handicapped child after cochlear implantation.

The presence of additional handicaps in hearing-impaired children makes the prediction of language ability after cochlear implantation unreliable. Only limited follow-up data on developmental improvement after implantation among multiply handicapped children is available. The present study reports the course of development (audiological and linguistic) after cochlear implantation in one subject with moderate mental retardation. Preoperatively, his language development showed 34 months delay when compared to chronological age. The difference had shortened to 23 months by 2 years post-surgery. The subject's cognitive delay had not changed upon 2-year follow-up. The cochlear implant can be credited to his improvement in language development.

High-throughput screening for GJB2 mutations--its clinical application to genetic testing in prelingual deafness screening for GJB2 mutations.

OBJECTIVES: Mutations in connexin26 (GJB2) are one of the most frequent causes of prelingual hearing impairment. Several different types of one-base deletions in exon2 were the most common type of GJB2 mutation regardless of ethnicity, including 35delG in American-European populations, 235delC in Japanese population and 167delT in Ashkenazi Jewish population. Various types of one-base substitutions were also considered to be causative mutations of GJB2 associated hearing impairment. This article describes a rapid and high-throughput screening procedure for the detection of one-base deletion/substitution in GJB2 with less invasive sampling procedure in the implication for the clinical application. METHODS: 53 hearing-impaired children and 50 healthy controls were admitted to take part in this study program. DNA samples obtained from buccal swab were used to amplify the exon2 of GJB2, and single run with an automated sequencer was used to identify the one-base deletion. Single-base substitutions were also screened by primer-extension procedure with dye terminators. The presence of both types of mutations was confirmed by direct sequence of the GJB2 exon2. RESULTS: Two of 50 controls (4%) included one-base deletion in GJB2 as heterozygote. 14 of 53 hearing impaired cases (26.4%) contained deletion in GJB2 either as homozygote (five cases) or heterozygote (nine cases) form. Sequencing analysis of whole exon2 of GJB2 identified all these deletions as 235delC. Primer-extension analysis revealed additional mutations with single base substitutions in three cases with compound heterozygote with 235delC. CONCLUSIONS: Rapid screening procedure of GJB2 can be potentially useful for the identification of prelingual deafness.

Better speech performance in cochlear implant patients with GJB2-related deafness.

OBJECTIVE: We applied mutation screening in seven cochlear implant users to identify those persons with GJB2-related deafness to determine whether etiology of deafness was predictive of speech performance after implantation. METHODS: Direct sequence of GJB2 was conducted over seven cochlear implant users with prelingual hearing impairment and their speech, language and cognitive performance was examined. RESULTS: The three persons with GJB2-related deafness had a mean vocabulary of 1243 words compared to a mean vocabulary of 195 words in the four children with GJB2-unrelated deafness, although the number of patients examined here was limited. The developmental quotient (DQ) of cognitive ability also was higher in those children with GJB2-related deafness. CONCLUSIONS: These preliminary results suggest that better speech performance after cochlear implantation may be observed in persons with GJB2-related deafness. In the future, detailed phenotypic studies and mutation screening for non-syndromic hearing loss may play an important role in the preoperative assessment of prelingually-deafened children.