Language：Japanese   Publisher：(有)科学評論社  

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Authorship：Lead author   Language：Japanese   Publisher：(株)東京医学社  

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Language：English   Publishing type：Research paper (scientific journal)  

Mutations in the OTOF gene are a common cause of hereditary hearing loss and the main cause of auditory neuropathy spectrum disorder (ANSD). Although it is reported that most of the patients with OTOF mutations have stable, congenital or prelingual onset severe-to-profound hearing loss, some patients show atypical clinical phenotypes, and the genotype-phenotype correlation in patients with OTOF mutations is not yet fully understood. In this study, we aimed to reveal detailed clinical characteristics of OTOF-related hearing loss patients and the genotype-phenotype correlation. Detailed clinical information was available for 64 patients in our database who were diagnosed with OTOF-related hearing loss. As reported previously, most of the patients (90.6%) showed a "typical" phenotype; prelingual and severe-to-profound hearing loss. Forty-seven patients (73.4%) underwent cochlear implantation surgery and showed successful outcomes; approximately 85-90% of the patients showed a hearing level of 20-39 dB with cochlear implant and a Categories of Auditory Performance (CAP) scale level 6 or better. Although truncating mutations and p.Arg1939Gln were clearly related to severe phenotype, almost half of the patients with one or more non-truncating mutations showed mild-to-moderate hearing loss. Notably, patients with p.His513Arg, p.Ile1573Thr and p.Glu1910Lys showed "true" auditory neuropathy-like clinical characteristics. In this study, we have clarified genotype-phenotype correlation and efficacy of cochlear implantation for OTOF-related hearing loss patients in the biggest cohort studied to date. We believe that the clinical characteristics and genotype-phenotype correlation found in this study will support preoperative counseling and appropriate intervention for OTOF-related hearing loss patients.

DOI： 10.1007/s00439-021-02351-7

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Authorship：Lead author   Language：Japanese   Publisher：(株)医学書院  

＜文献概要＞POINT ◆聴性定常反応(ASSR)は他覚的に聴覚閾値を測定する検査法で,周波数特異性に優れている.◆乳幼児,知的障害や認知機能低下者,機能性難聴者など,純音聴力検査の信頼性が低い者の聴力評価に有用である.

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BACKGROUND: Histological evaluation of lymph node is crucial for the definitive diagnosis of Kikuchi-Fujimoto disease (KFD). However, lymph node biopsy under local anesthesia is often difficult in pediatric patients. OBJECTIVES: We evaluated cytological findings for pediatric patients with prolonged cervical lymphadenitis clinically suggestive of KFD and investigated the clinical characteristics of patients diagnosed with KFD by fine-needle aspiration cytology (FNAC). METHODS: This retrospective clinical study included 58 Japanese pediatric patients with cervical lymphadenitis who underwent FNAC. RESULTS: Cytological diagnosis was KFD for 22 and suspicion of KFD for 11 patients. The remaining 25 patients were diagnosed with non-specific lymphadenitis (NSL). Tenderness was independently associated with a higher frequency of both KFD in narrow and broad senses, compared with NSL (p = .009; p = .038). The percentage of patients who underwent FNAC within 28 days from symptom onset tended to be higher among patients with KFD in a narrow sense than those with NSL (p = .052). CONCLUSION: This study indicated that the period from symptom onset to FNAC (<28 days) and the symptom of tenderness were associated with the cytological diagnosis of KFD.

DOI： 10.1080/00016489.2022.2040744

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：日本耳鼻咽喉科免疫アレルギー感染症学会  

1日2回の生理食塩水洗浄と抗菌薬の保存的加療により、人工内耳植え込み術後の局所感染を制御できた小児の1例(1歳11ヵ月男児)について報告した。本症例で術後創部感染の保存的加療が可能であった要因として、「起炎菌がMRSAなどではなく、MSSAであったこと」「鼓膜所見や起炎菌から、中耳からの感染ではなく、皮膚からの感染であったため」「当院では、人工内耳植え込み術の際、minimum invasive surgeryを行っていること」が考えられた。

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Other Link： https://search-tp.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2021&ichushi_jid=J07545&link_issn=&doc_id=20220120310010&doc_link_id=%2Ffm9areka%2F2021%2F000103%2F010%2F0177-0180%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Ffm9areka%2F2021%2F000103%2F010%2F0177-0180%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Authorship：Lead author   Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：日本小児耳鼻咽喉科学会  

先天性難聴の早期発見,補聴器・人工内耳装用に伴い,難聴児の聴取能や言語発達は向上し,近年インクルーシブ教育を受ける児が増加している。しかしながら難聴児の聴取は補聴器や人工内耳を装用していても正常聴力児と同等ではなく,言語発達や学習面,友人関係,行動面で問題が生じることは多い。また新型コロナウイルス感染症拡大予防策のマスク着用やソーシャルディスタンスにより難聴児の聴取や読話は困難となり,コミュニケーションにおける支障は増大している。しかしながら,特にインクルーシブ教育を受けている児では,聴取の困難さや抱えている問題を担任教師が正確には把握ができていない場合が多い。学校生活において適切な配慮や支援が受けられるよう情報提供が必要であると考え,当院では教師対象パンフレットを作成している。学齢期の難聴児の多岐にわたる問題に対し,医療,療育・教育,行政等多職種で連携して支援する体制の構築が必要である。(著者抄録)

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：耳鼻咽喉科臨床学会  

当科で化学療法を施行した小児、思春期・若年(CAYA)世代がん患者20例を対象に、妊孕性温存に関する説明の実態調査を行った。その結果、妊孕性低下・消失について説明していた患者は2例のみであり、それぞれ17歳と23歳の未婚若年女性であった。一方、その他の女性5例(30歳台)や男性(13〜38歳)には説明が行われていなかった。また、妊孕性温存ガイドラインが出版された2017年の前後で妊孕性低下・消失について説明を実施した割合には著しい変化はみられず、妊孕性に対する配慮は担当医レベル、診療科レベルでも改善できる余地が十分にあると考えられた。

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Other Link： https://search-tp.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2021&ichushi_jid=J00580&link_issn=&doc_id=20211209280011&doc_link_id=10.5631%2Fjibirin.114.957&url=https%3A%2F%2Fdoi.org%2F10.5631%2Fjibirin.114.957&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_2.gif

Authorship：Lead author   Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

難聴児の早期発見・療育,人工内耳装用に伴い,聴取能や言語発達は向上している.加えて,障害児の共生に向けた社会的体制の変化も伴い,近年地域の学校でインクルーシブ教育を受ける児は増加している.しかし,難聴児の聴取は補聴器や人工内耳を装用しても正常聴力児と同等ではなく,大勢でさまざまな方向からの聴取を要する学校生活の環境において多くの場面で支障がある.また学年が上がるとともに学習面の限界や学力低下,友人との関係性の問題などが出現する頻度が高く,留意や配慮を要する.加えて,新型コロナウイルス感染症拡大予防策のマスク着用やソーシャルディスタンスにより,さらにコミュニケーションの支障は増大している.しかしながら,担任教師であってもそれらの問題を正確には把握しにくく,適切な配慮や支援が施されないことが多い.われわれはこれまでに実施した難聴児・者への質問紙による調査結果をもとに,難聴児の直面している問題と支援について教師が実際に活用しやすい形式でまとめたパンフレット「難聴をもつ小・中・高校生の学校生活で大切なこと 先生編」を作成した.難聴児の抱える問題は成長の段階で変化するため,乳幼児期の補聴機器のフィッティングや言語訓練を中心とした指導で完結すべきではなく,学齢に達しても専門的支援を継続する必要がある.本冊子が難聴児の育成の一助となることに期待するとともに,今後学齢期以降の支援を充実させるよう努めたい.(著者抄録)

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1097/MAO.0000000000003220

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WHO has recommended various measures to combat the COVID-19 pandemic, including mask-wearing and physical distancing. However, these changes impair communication for individuals with hearing loss. We investigated the changes in auditory communication associated with COVID-19 measures in 269 patients (male: 45.7%, female: 54.3%, median age: 54 y.o.). Most patients with hearing loss had difficulty engaging in auditory communication with people wearing masks, especially in noisy surroundings or with physical distanc-ing. These difficulties were noticeable in patients with severe hearing loss. Developing communication support strategies for people with hearing loss is an urgent need while COVID-19 measures are in place.

DOI： 10.18926/AMO/62404

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

DOI： 10.1016/j.xocr.2021.100294

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OBJECTIVES: Sialolipoma has been classified as a benign soft tissue lesion in the 2017 World Health Organization classification of head and neck tumors. To our knowledge, only one case of laryngeal sialolipoma has been reported in the English literature. We conducted a retrospective study to identify clinical characteristics of supraglottic sialolipoma-like lesion and differentiate it from other supraglottic subepithelial masses. METHODS: Medical records of 16 patients with supraglottic subepithelial benign mass lesions who underwent histological evaluation between 2003 and 2019 were retrospectively analyzed. Sialolipoma-like lesion was defined as a local finding of a well-circumscribed gross mass with pathological presence of salivary gland-like parenchymal lobules with evenly interspersed adipose tissue. RESULTS: Eight patients showed histological positivity for sialolipoma-like lesion, 3 for amyloidosis, 2 for hemangioma, and 1 each for cyst, lymphoid hyperplasia, and chondrometaplasia. Sialolipoma-like lesion tended to be predominant among men; those affected had a mean age of 52.8 (range, 39-74) years. By contrast, among patients with amyloidosis, the ratio of men to women was 1:2 (100% vs. 33%; p = 0.055). Fiberscopic examination of all patients with sialolipoma-like lesions identified well-circumscribed, yellowish masses, closely resembling local amyloidosis findings. Sialolipoma-like lesion was associated with a significantly higher body-mass index (BMI; 27.4 ± 2.8 kg/m2) than amyloidosis (21.6 ± 1.4 kg/m2; p = 0.014). The transoral approach was used for lesion resection in all patients with sialolipoma-like lesion. No patient experienced postoperative recurrence. CONCLUSION: Laryngeal sialolipoma-like lesion might be more prevalent than was previously reported, and histological examination is important to differentiate it from amyloidosis. Supraglottic sialolipoma-like lesion must be differentially diagnosed in patients with high BMI presenting with well-circumscribed, yellowish supraglottic masses.

DOI： 10.1016/j.anl.2020.07.017

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Language：Japanese   Publishing type：Research paper (scientific journal)  

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OBJECTIVES: The aim of this study was to retrospectively document prevalence rates of delayed-onset hearing loss (DOHL) under 7 years old after passing the newborn hearing screening (NHS) program using its database in Okayama Prefecture, as well as records from Okayama Kanariya Gakuen (OKG, Auditory Center for Hearing Impaired Children, Okayama Prefecture, Japan). We explored the percentage of children with DOHL among all children who underwent the NHS and surveyed risk factors abstracted from their clinical records. METHODS: We collected data of 1171 children, who first visited OKG from April 2006 to March 2018. DOHL children were defined as bilaterally hearing-impaired children who were diagnosed under 7 years old after passing the NHS at birth. Based on the medical records, we investigated age at diagnosis, hearing levels, and risk factors. As population-based data of 168,104 children, the percentage of DOHL subjects was retrospectively calculated among the total number of children who underwent the NHS in Okayama Prefecture from April 2005 to March 2017. RESULTS: During the period, we identified 96 children with bilateral DOHL, of which 34 children had failed the NHS unilaterally and 62 had passed the NHS bilaterally. Among all children who underwent the NHS in Okayama Prefecture, the prevalence rate of DOHL in unilaterally referred infants was 5.2%, and 0.037% in bilaterally passed children. The prevalence of bilateral DOHL was 0.057% overall. Unilaterally referred children with DOHL were diagnosed at an average of 13.9 months, while bilaterally passed children with DOHL were diagnosed at an average of 42.3 months. Approximately 59.4% of children with DOHL had risk factors, among which family history of hearing loss was the most frequent. CONCLUSION: We propose the first English report of DOHL prevalence in the prefecture population in Japann, which is among the largest community-based population ever reported. The NHS is not a perfect strategy to detect all early-childhood hearing loss; therefore, careful assessment of hearing throughout childhood is recommended, especially in children with risk factors of hearing loss. Further interventional strategies must be established, such as regular hearing screening in high-risk children and assessments of hearing and speech/language development in public communities and nursery schools.

DOI： 10.1016/j.ijporl.2020.110298

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Background: Subglottic cancer (SGC) is extremely rare, as most laryngeal cancers are localized to the glottic region. Accordingly, the clinical characteristics of SGC have not been well characterized.Objectives: In the current study, SGCs were clinically evaluated, and the outcomes of radiotherapy (RT) in patients with stage II SGC were assessed.Materials and Methods: Medical data derived from 11 patients with SGC, who were treated at our hospital between 1995 and 2019, were retrospectively reviewed.Results: In our department SGC accounted for 3.9% of the 280 laryngeal cancer patients treated during the study period. At the time of SGC diagnosis, 9 (81.8%) had stage II cancer, 1 had stage III cancer, and 1 had stage IV cancer. Stage II SGC patients treated with concurrent chemoradiotherapy (CCRT) showed a significantly higher local control rate (p = .026) and laryngeal dysfunction free rate (p = .026) than those treated with RT alone. Salvage surgery, performed in 4 patients whose disease was not locally controlled with CCRT/RT, was successful in 3 patients.Conclusion: As a treatment strategy for stage II SGC, CCRT is an acceptable initial treatment for laryngeal function and preservation while salvage surgery is effective for recurrence after CCRT/RT treatment.

DOI： 10.1080/00016489.2020.1767303

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HYPOTHESIS: Macrophage migration inhibitory factor plays an important role in the expression of interleukin (IL)-1β and the nucleotide-binding oligomerization domain-like receptor protein 3 (NLRP3) inflammasome in lipopolysaccharide-induced otitis media. BACKGROUND: NLRP3 inflammasome and macrophage migration inhibitory factor are critical molecules mediating inflammation. However, the interaction between the NLRP3 inflammasome and macrophage migration inhibitory factor has not been fully examined. METHODS: Wild-type mice and macrophage migration inhibitory factor gene-deficient (MIF) mice received a transtympanic injection of either lipopolysaccharide or phosphate-buffered saline. The mice were sacrificed 24 hours after the injection. Concentrations of IL-1β, NLRP3, ASC (apoptosis-associated speck-like protein containing a caspase recruitment domain and a pyrin domain), and caspase-1 in the middle ear effusions were measured by enzyme-linked immunosorbent assay. Temporal bones were processed for histologic examination and immunohistochemistry. RESULTS: In the immunohistochemical study using the wild-type mice, positive staining of macrophage migration inhibitory factor, NLRP3, ASC, and caspase-1 were observed in infiltrating inflammatory cells induced by lipopolysaccharide in the middle ear. The number of inflammatory cells caused by lipopolysaccharide administration decreased remarkably in the MIF mice as compared with the wild-type mice. The concentrations of IL-1β, NLRP3, ASC, and caspase-1 increased in the lipopolysaccharide-treated wild-type mice. The MIF mice with lipopolysaccharide had decreased levels of IL-1β, NLRP3, ASC, and caspase-1 as compared with the wild-type mice. CONCLUSION: Macrophage migration inhibitory factor has an important role in the production of IL-1β and the NLRP3 inflammasome. Controlling the inflammation by modulating macrophage migration inhibitory factor and the NLRP3 inflammasome may be a novel therapeutic strategy for otitis media.

DOI： 10.1097/MAO.0000000000002537

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Background: Demographic data of patients with sensorineural hearing loss (SNHL) in super-aged societies are still limited.Aims/objectives: To report audiometric statistics of SNHL and hearing aid (HA) use in patients in their 60s, 70s, and 80s and older during the super-aged era.Material and methods: Medical charts and audiograms of 2064 older patients with SNHL who visited a Japanese University Hospital in 2007-2018 were retrospectively reviewed. Among 270 patients referred to the HA service unit (HASU), the percentage of final decisions to continue using HAs was calculated.Results: The average pure tone thresholds on initial visit to the clinic were 56.9, 60.6, 69.4, and 82.4 dB HL in patients in their 60s, 70s, 80s, and 90s, respectively. The rates of progression were 0.25, 0.87, 1.19, and 1.37 dB/year in patients in their 50s, 60s, 70s, and 80s, respectively. The percentage of patients in HASU who chose to use HAs did not differ among the 60s (59.3%), 70s (51.2%), and 80s and older (58.2%).Conclusions and significance: The clinical picture of patients with SNHL in their 70s and 80s differs because progression accelerates exponentially through these ages. HAs can be recommended to older adult patients in all the age groups.

DOI： 10.1080/00016489.2019.1667531

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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.

DOI： 10.1016/j.ijporl.2019.109619

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More than 400 syndromes associated with hearing loss and other symptoms have been described, corresponding to 30% of cases of hereditary hearing loss. In this study we aimed to clarify the mutation spectrum of syndromic hearing loss patients in Japan by using next-generation sequencing analysis with a multiple syndromic targeted resequencing panel (36 target genes). We analyzed single nucleotide variants, small insertions, deletions and copy number variations in the target genes. We enrolled 140 patients with any of 14 syndromes (BOR syndrome, Waardenburg syndrome, osteogenesis imperfecta, spondyloepiphyseal dysplasia congenita, Stickler syndrome, CHARGE syndrome, Jervell and Lange-Nielsen syndrome, Pendred syndrome, Klippel-Feil syndrome, Alport syndrome, Norrie disease, Treacher-Collins syndrome, Perrault syndrome and auditory neuropathy with optic atrophy) and identified the causative variants in 56% of the patients. This analysis could identify the causative variants in syndromic hearing loss patients in a short time with a high diagnostic rate. In addition, it was useful for the analysis of the cases who only partially fulfilled the diagnostic criteria.

DOI： 10.1038/s41598-019-47141-4

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The OTOF gene (Locus: DFNB9), encoding otoferlin, is reported to be one of the major causes of non-syndromic recessive sensorineural hearing loss, and is also reported to be the most common cause of non-syndromic recessive auditory neuropathy spectrum disorder (ANSD). In the present study, we performed OTOF mutation analysis using massively parallel DNA sequencing (MPS). The purpose of this study was to reveal the frequency and precise genetic and clinical background of OTOF-related hearing loss in a large hearing loss population. A total of 2,265 Japanese sensorineural hearing loss (SNHL) patients compatible with autosomal recessive inheritance (including sporadic cases) from 53 otorhinolaryngology departments nationwide participated in this study. The mutation analysis of 68 genes, including the OTOF gene, reported to cause non-syndromic hearing loss was performed using MPS. Thirty-nine out of the 2,265 patients (1.72%) carried homozygous or compound heterozygous mutations in the OTOF gene. It is assumed that the frequency of hearing loss associated with OTOF mutations is about 1.72% of autosomal recessive or sporadic SNHL cases. Hearing level information was available for 32 of 39 patients with biallelic OTOF mutations; 24 of them (75.0%) showed profound hearing loss, 7 (21.9%) showed severe hearing loss and 1 (3.1%) showed mild hearing loss. The hearing level of patients with biallelic OTOF mutations in this study was mostly severe to profound, which is consistent with the results of past reports. Eleven of the 39 patients with biallelic OTOF mutations had been diagnosed with ANSD. The genetic diagnosis of OTOF mutations has significant benefits in terms of clinical decision-making. Patients with OTOF mutations would be good candidates for cochlear implantation; therefore, the detection of OTOF mutations is quite beneficial for patients, especially for those with ANSD.

DOI： 10.1371/journal.pone.0215932

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BACKGROUND: The nucleotide-binding oligomerization domain-like receptor protein 3 (NLRP3) inflammasome is a critical molecule mediating interleukin-1β (IL-1β) responses. However, the role of the NLRP3 inflammasome in otitis media has not been fully examined. AIMS/OBJECTIVES: The purpose of this study is to assess the expression of NLRP3, ASC (apoptosis-associated speck-like protein containing a caspase recruitment domain and a pyrin domain), and caspase-1 in lipopolysaccharide-induced otitis media. MATERIALS AND METHODS: BALB/c mice received a transtympanic injection of either lipopolysaccharide or phosphate-buffered saline. The mice were sacrificed 24 h after injection. Concentrations of IL-1β, NLRP3, ASC, and caspase-1 in the middle ear effusions were measured by enzyme-linked immunosorbent assay. Temporal bones were processed for histologic examination and immunohistochemistry. RESULTS: The transtympanic injection of lipopolysaccharide significantly upregulated levels of IL-1β, NLRP3, ASC, and caspase-1 in the middle ear as compared with the control mice. The proteins of NLRP3, ASC, and caspase-1 were observed in infiltrating inflammatory cells induced by lipopolysaccharide in the middle ear cavity. CONCLUSIONS AND SIGNIFICANCE: Lipopolysaccharide induces NLRP3 inflammasome components in the middle ear. The NLRP3 inflammasome may play an important role in the pathogenesis of otitis media. Modulation of inflammasome-mediated inflammation may be a novel therapeutic strategy for otitis media.

DOI： 10.1080/00016489.2018.1515499

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OBJECTIVE: To clarify how the pure-tone threshold (PTT) on the PTA predicts speech perception (SP) in elderly Japanese persons. METHODS: Data on PTT and SP were cross-sectionally analyzed in Japanese persons (656 ears in 353 patients, aged ≥65 years). Correlations of SP and average PTT in all tested frequencies were evaluated by Pearson's correlation coefficient and simple linear regression. After adjusting for sex, laterality of ears, and age, the relationship of average and frequency-specific PTT with impaired SP ≤50% was estimated by logistic regression models. RESULTS: SP correlated well (r = -0.699) with the average PTT of all tested frequencies. On the other hand, the correlation between patient age and SP was weak, especially among ≤85-year-old persons (r = -0.092). Linear regression showed that the average PTT corresponding to SP of 50% was 76.4 dB nHL. Odds ratios for impaired SP were highest for PTT at 2000 Hz. Odds ratios were higher for middle (500, 1000, 2000 Hz) and high frequencies (4000, 8000 Hz) than low frequencies (125, 250 Hz). CONCLUSION: The PTT on the pure-tone audiogram (PTA) is a good predictor of SP by speech audiometry among older persons, which could provide clinically important information for hearing aid fitting and cochlear implantation.

DOI： 10.1080/00016489.2017.1378435

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Language：Japanese   Publishing type：Research paper (scientific journal)  

Since April 2012, genetic testing for congenital hearing loss is covered by the public health insurance in Japan. Recent (since August 2015) developments in next-generation sequencing technology have enabled the detection of 154 mutations in 19 genes. Genetic testing provides valuable information on hearing phenotype, prognosis, and prediction of associated symptoms. We report a hearing-impaired patient in whom multiple genetic mutations were detected. This patient carries two missense mutations in GJB2 (p.G45E, p.Y136X), as well as a mitochondrial mutation (7445A>G). Since the number of genes detectable by genetic testing has increased, the diagnosis of hearing loss can be made with greater accuracy. However, it is often difficult to clinically understand and interpret the genotype information, especially when multiple gene variants are detected in one patient or family. Genetic counseling plays an important part in the intervention for or follow-up of such patients. Genotypic and phenotypic information of other family members is necessary, so that both the patient and the family can understand and accept the results of genetic testing.

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Language：English   Publishing type：Research paper (scientific journal)  

CONCLUSION: The prevalence of low-tone hearing loss (LTHL) is significantly high in spinocerebellar degeneration (SCD) with cerebellar predominance, including multiple-system atrophy C (MSA-C) and cortical cerebellar atrophy (CCA). OBJECTIVE: This study aimed to test the hypothesis that SCD with cerebellar predominance, MSA-C and CCA may cause auditory symptoms. METHODS: The shape and threshold of pure-tone audiograms were evaluated for MSA-C (n = 47; mean (± SD) age, 61.6 ± 8.9 years), CCA (n = 16; 62.8 ± 9.5 years), and age-matched controls (n = 169; 62.5 ± 10.7 years). To differentiate specific hearing loss for MSA-C and CCA from presbycusis, the shape of audiograms was examined based on previously established audiological criteria. RESULTS: When audiogram shape was defined according to audiological criteria, the odds ratio for LTHL in SCD compared to controls was 2.492 (95% confidence interval (CI) = 1.208-5.139; p < 0.05, Pearson's Chi-square test) in MSA-C and 2.194 (95% CI = 0.709-6.795) in CCA. When the selection of audiogram shape according to these criteria was verified by three certified audiologists, odds ratios for LTHL in MSA-C and CCA were 3.243 (95% CI = 1.320-7.969) and 3.692 (95% CI = 1.052-12.957), respectively, significantly higher than in controls.

DOI： 10.3109/00016489.2015.1104722

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Language：English   Publishing type：Research paper (scientific journal)  

CONCLUSIONS: The nucleotide-binding oligomerization domain-like receptor protein 3 (NLRP3) inflammasome plays an important role in the pathogenesis of middle ear diseases. Modulation of inflammasome-mediated inflammation may be a novel therapeutic strategy for cholesteatoma and chronic otitis media. OBJECTIVE: NLRP3 inflammasome is a critical molecule mediating interleukin (IL)-1β responses. However, the expression of NLRP3 in the pathogenesis of cholesteatoma and chronic otitis media has not been fully examined. This study sought to assess the expression of NLRP3, ASC (apoptosis-associated speck-like protein containing a caspase recruitment domain and a pyrin domain), and caspase-1 in middle ear tissues in patients with cholesteatoma or chronic otitis media. METHODS: Middle ear tissue samples were obtained from patients with cholesteatoma or chronic otitis media. Control middle ear samples were collected during cochlear implant surgery of patients without middle ear inflammation. The expression of NLRP3, ASC, and caspase-1 were examined by reverse transcription polymerase chain reaction (RT-PCR) assay and immunohistochemical study. RESULTS: The levels of mRNA of NLRP3, ASC, and caspase-1 were significantly elevated in cholesteatoma and chronic otitis media as compared with that of normal controls. The proteins of NLRP3, ASC, and caspase-1 were observed in infiltrating inflammatory cells in cholesteatoma and chronic otitis media.

DOI： 10.3109/00016489.2015.1093171

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J-GLOBAL

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Language：English   Publishing type：Research paper (scientific journal)  

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.

DOI： 10.1016/j.ijporl.2015.09.036

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Language：Japanese   Publisher：(一社)日本耳科学会  

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Language：Japanese   Publisher：(一社)日本耳科学会  

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Charcot-Marie-Tooth disease (CMT) is the most common form of hereditary sensorimotor neuropathy and sometimes involves disorders of the peripheral auditory system. We present a case of steroid-dependent auditory neuropathy associated with CMT, in which the patient experienced 3 episodes of acute exacerbation of hearing loss and successful rescue of hearing by prednisolone. An 8-year-old boy was referred to the otolaryngology department at the University Hospital. He had been diagnosed with CMT type 1 (demyelinating type) at the Child Neurology Department and was suffering from mild hearing loss due to auditory neuropathy. An audiological diagnosis of auditory neuropathy was confirmed by auditory brainstem response and distortion-product otoacoustic emissions. At 9 years and 0 months old, 9 years and 2 months old, and 10 years and 0 months old, he had experienced acute exacerbations of hearing loss, each of which was successfully rescued by intravenous or oral prednisolone within 2 weeks. Steroid-responsive cases of CMT have been reported, but this is the first case report of steroid-responsive sensorineural hearing loss in CMT. The present case may have implications for the mechanisms of action of glucocorticoids in the treatment of sensorineural hearing loss.

DOI： 10.1016/j.anl.2014.11.002

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Language：Japanese   Publisher：(一社)日本遺伝カウンセリング学会  

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Language：Japanese   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

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Language：Japanese   Publisher：日本小児耳鼻咽喉科学会  

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Language：Japanese   Publisher：日本小児耳鼻咽喉科学会  

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Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Permanent hearing impairment has a life-long impact on children and its early identification is important for language development. A newborn hearing screening (NHS) program has started in Okayama Prefecture, Japan, in 1999 to detect hearing impairment immediately after birth. We aim to examine the effect of this screening program on vocabulary development in pre-school children in a before and after comparative study design. METHODS: A total of 107 5-year-old children who graduated from Okayama Kanariya Gakuen (an auditory center for hearing-impaired children) between 1998 and 2011 were enrolled in this study. The pre-NHS group (n=40) was defined as those who graduated between 1998 and 2003, while the post-NHS group (n=67) was defined as those who graduated between 2004 and 2011. The primary outcome was receptive vocabulary, which was assessed by the Picture Vocabulary Test [score <18 (low) vs. score ≥18 (high)]. The secondary outcome was productive vocabulary, or the number of productive words, which was assessed by an original checklist [<1773 words (low) vs. ≥1773 (high)]. We calculated odds ratios and 95% confidence intervals for vocabulary development and compared both groups. RESULTS: The adjusted Picture Vocabulary Test score and number of productive words were significantly higher (p<0.01) in the post-NHS group than the pre-NHS group. Odds ratios were 2.63 (95% confidence interval: 1.17-5.89) for receptive vocabulary and 4.17 (95% confidence interval: 1.69-10.29) for productive vocabulary. CONCLUSIONS: The introduction of NHS in Okayama Prefecture significantly improved both receptive and productive vocabulary development in hearing-impaired children.

DOI： 10.1016/j.ijporl.2014.12.006

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Language：Japanese   Publisher：日本聴覚医学会  

DOI： 10.4295/audiology.58.335

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Other Link： http://search.jamas.or.jp/link/ui/2016019205

Language：Japanese   Publisher：Japan Society for Pediatric Otorhinolaryngology  

DOI： 10.11374/shonijibi.36.321

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Other Link： http://search.jamas.or.jp/link/ui/2016123054

Language：English   Publishing type：Research paper (scientific journal)  

CONCLUSION: Performance in consonant-vowel (CV) monosyllable speech perception after cochlear implantation (CI) in the elderly (≥ 65 years) is equivalent to that of young adults (18-64 years). Present data in the Japanese language supported the indication for CI in the elderly. Word recognition after CI was significantly lower in the elderly than young adults. OBJECTIVE: This study compared outcomes of monosyllable perception and word recognition after CI between elderly patients and young adults with post-lingual deafness. Age-related differences in CV monosyllable speech perception in Japanese were examined. METHODS: A retrospective chart review of 137 patients with post-lingual deafness who underwent CI at Okayama University Hospital during 1992-2014 [young adults aged 18-64 years (n = 96) and elderly aged ≥ 65 years (n = 41) at implantation] was conducted. CV monosyllable speech perception post-CI was compared among age groups (18-29, 30-39, 40-49, 50-59, 60-69, 70-79, 80-89). CV monosyllable perception and word recognition scores post-CI were compared between the elderly and young adults. RESULTS: There was no significant difference in CV monosyllable speech perception among age groups. CV monosyllable speech perception in the elderly (61.4 ± 25.5%) did not differ from that of young adults (65.9 ± 24.8%). Word recognition scores were significantly lower in the elderly (64.3 ± 28.1%) than young adults (80.4 ± 25.9%) (p < 0.05).

DOI： 10.3109/00016489.2015.1062547

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Language：Japanese   Publishing type：Research paper (scientific journal)  

The spread of neonatal hearing screening has made the identification of hearing loss in early childhood possible, and most of the children with hearing loss are diagnosed and provided with early intervention within several months after their birth. However, they have to wait to receive the Special Child Dependent's Allowance until their hearing levels are evaluated with audiometers. Under the Special Child Dependent's Allowance Act now in force, a delay of several years occurs for those children before they can receive the allowance after diagnosis. We retrospectively reviewed hearing test results of children who had not reacted at 90dBnHL bilaterally in ABR within 12 months after birth, and examined when those results were certain enough to determine their allowance eligibility. We found that the eligibility in children with hearing loss alone could be determined at 1 year of age; as for children who had other disorders, since it was mostly difficult to test them with an audiometer, the diagnosis of hearing loss must always be made carefully but it was also possible to determine the eligibility for the allowance at 1 year of age.

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Language：Japanese   Publisher：(一社)日本耳科学会  

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Language：Japanese   Publisher：日本耳鼻咽喉科感染症・エアロゾル学会  

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Language：Japanese   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

近年新生児聴覚スクリーニングの普及により、難聴児の多くは生後数ヵ月のうちに診断され、療育を開始している。一方、特別児童扶養手当に関してはオージオメーターによる検査が可能となるまで受給されず、診断から受給までの間に年単位の時間差が生じているのが現状である。われわれは、生後12ヵ月までにABRを実施し、両耳90dBnHLで無反応であった児の聴力経過の検討を行った。その結果、聴覚障害単独の児では特別児童扶養手当に該当するか否かは1歳時点で可能であるが、等級の判定は1歳で慎重な判断が必要であること、また重複障害をもつ児は検査を繰り返し慎重に診断する必要があるが特別児童扶養手当の判定は可能であると判断した。(著者抄録)

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Other Link： https://search-tp.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2014&ichushi_jid=J01099&link_issn=&doc_id=20141003450006&doc_link_id=1390282679988087552&url=https%3A%2F%2Fcir.nii.ac.jp%2Fcrid%2F1390282679988087552&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_3.gif

Language：Japanese   Publisher：(一社)日本耳科学会  

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Language：Japanese   Publishing type：Research paper (scientific journal)  

The presence of hearing impairment deteriorates patients' quality of life by affecting their daily-life communication. Several self-report assessments are available to evaluate the subjective outcome of hearing aids, however, the procedures to evaluate the impairment of communication induced by hearing impairment has not been well established. A PC-recorded audio file prepared to evaluate the communication ability of hearing aid users, by extracting fifteen spoken language items from the Communication ADL Test: sp-CADL. A before and after study was performed among the hearing aid users who were attending Okayama University Hospital to evaluate the improvement in each of the 15 sp-CADL scores. Twenty-one adults were included in this study whose age ranged from 22 to 83 years old (mean: 56.7 years old). The total and individual scores of the sp-CADL improved significantly after the use of the hearing aids (p < 0.05). The total sp-CADL score of the patients with hearing aids showed a good correlation with the best scores of the speech discrimination test measured before wearing hearing aids.

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Language：Japanese   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

成人の聴覚障害者の補聴器装用効果の指標としては、日本聴覚医学会の補聴器適合指針をはじめ、各種質問紙調査が用いられている。しかし、聴覚障害に伴うコミュニケーション障害を評価するための指標は定まっていない。われわれは、実用コミュニケーション能力検査(Communication ADL Test:CADL)から音声に関する項目を抜粋し、音声ファイルを作成、このCADL簡易版を用いて、当科補聴器外来にて補聴器装用を介入とした前後比較試験を行ったところ、各15の質問項目および総得点で装用後有意な得点の改善がみられた。また、装用後の総得点は補聴器装用耳の語音明瞭度と良好な相関を認め、本検査がコミュニケーション障害のアウトカム指標に応用できる可能性が示唆された。(著者抄録)

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Other Link： https://search-tp.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2014&ichushi_jid=J01099&link_issn=&doc_id=20140902380011&doc_link_id=1390001205010834816&url=https%3A%2F%2Fcir.nii.ac.jp%2Fcrid%2F1390001205010834816&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_3.gif

Language：Japanese   Publisher：(一社)日本遺伝カウンセリング学会  

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Language：Japanese   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

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Language：Japanese   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

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J-GLOBAL

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J-GLOBAL

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Language：Japanese   Publisher：(一社)日本耳科学会  

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Language：Japanese   Publisher：(株)東京医学社  

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Language：English   Publishing type：Research paper (scientific journal)  

Perilymphatic fistula (PLF) is defined as an abnormal leakage between perilymph from the labyrinth to the middle ear. Symptoms include hearing loss, tinnitus, and vertigo. The standard mode of PLF detection is intraoperative visualization of perilymph leakage and fistula, which ostensibly confirms the existence of PLF. Other possible methods of diagnosis include confirmation of pneumolabyrinth via diagnostic imaging. Recently, a cochlin-tomoprotein (CTP) detection test has been developed that allows definitive diagnosis of PLF-related hearing loss. We report the case of a 45-year-old man who presented with right-sided tinnitus, hearing loss, and dizziness 30 years after stapes surgery. Middle ear lavage was performed after myringotomy. A preoperative diagnosis of PLF was reached using the CTP detection test. Intraoperative observations included a necrotic long process of the incus, displaced wire piston, and fibrous tissue in the oval window. Perilymph leakage was not evident. The oval window was closed with fascia, and vertigo disappeared within 2 weeks postoperatively. When PLF is suspected after stapes surgery, the CTP detection test can be a useful, highly sensitive, and less invasive method for preoperative diagnosis.

DOI： 10.1016/j.anl.2012.08.001

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Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: Otitis media is one of the most common infectious diseases, especially in young children. Multiple factors affect the onset or development of otitis media. Human toll-like receptors recognize associated patterns and play a critical role in innate immune mechanisms. Toll-like receptors are considered to be important factors for clearance of infection and resolution of inflammation in otitis media. The purpose of this study was to evaluate the histological expression of toll-like receptor 2, which recognizes many kinds of pathogen-associated molecular patterns, and toll-like receptor 4, which recognizes lipopolysaccharide on Gram-negative bacteria, in tissue samples from patients with chronic otitis media and middle ear cholesteatoma. METHODS: Human middle ear tissue samples from 12 patients with chronic otitis media (n=7) and acquired middle ear cholesteatoma (n=5) were examined. Normal control middle ear samples without any inflammation were also included (n=7). The expressions of toll-like receptors 2 and 4 in middle ear tissues were examined immunohistochemically. RESULTS: Only one normal control middle ear sample showed weak expression of toll-like receptor 2, and toll-like receptor 4 was not observed in all control samples. On the other hand, both toll-like receptors 2 and 4 were markedly expressed in chronic otitis media and cholesteatoma. There was a significant difference between chronic otitis media and normal controls in the expressions of both toll-like receptors. Significant up-regulation of toll-like receptors 2 and 4 was observed in cholesteatoma as compared with control samples. CONCLUSIONS: Toll-like receptors 2 and 4 were strongly expressed in chronic otitis media and middle ear cholesteatoma. These findings suggest that toll-like receptors may play a principal role in human chronic otitis media and cholesteatoma.

DOI： 10.1016/j.ijporl.2013.01.010

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Language：Japanese   Publisher：(一社)日本遺伝カウンセリング学会  

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Language：Japanese   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

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Language：Japanese   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

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Language：Japanese   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

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Language：Japanese   Publisher：岡山医学会  

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Other Link： https://search-tp.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2012&ichushi_jid=J00175&link_issn=&doc_id=20120802150012&doc_link_id=10.4044%2Fjoma.124.155&url=https%3A%2F%2Fdoi.org%2F10.4044%2Fjoma.124.155&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Language：Japanese   Publisher：耳鼻咽喉科臨床学会  

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Language：Japanese   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

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Language：Japanese   Publisher：Japan Society for Pediatric Otorhinolaryngology  

A case of rapidly progressive ossification of the cochlea after bacterial meningitis is reported. An eleven-month-old boy was referred to our hospital due to his loss of response to sound after suffering from meningitis at 8 months old. He did not respond to sound either by BOA or ABR at 105dB nHL. 3D reconstruction imaging with MRI suggested that the cochlea was completely occluded in his right inner ear. Possible progression to his left inner ear was suspected, and cochlear implant in his left ear was performed immediately. Ossification of the cochlea is frequently observed after meningitis. In cases with early and rapidly progressive ossification of the cochlea, repeated audiological evaluation and MRI imaging is indispensable in the follow-up after bacterial meningitis with very young infants.

DOI： 10.11374/shonijibi.33.23

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Other Link： http://search.jamas.or.jp/link/ui/2012208704

Language：Japanese   Publisher：THE JAPAN OTOLOGICAL SOCIETY  

Ceruminous adenoma is a rare benign neoplasm arising from the ceruminous grand of the external auditory canal. We reported a case of ceruminous adenoma mimicking squamous cell carcinoma in the external auditory canal. A 59-year-old woman presented with ear discharge in her left ear. She had no pain, hearing loss, tinnitus, nor vertigo. Physical examination showed a space-occupying lesion on the superior part of left external auditory canal. Biopsy was performed and squamous cell carcinoma was suspected. A local resection was performed with reconstruction of the external auditory canal using retroauricular flap. Postoperative pathological diagnosis was ceruminous adenoma. There was no evidence of recurrence at 18 months of follow-up.

DOI： 10.11289/otoljpn.22.36

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Other Link： http://search.jamas.or.jp/link/ui/2012167712

Language：Japanese   Publisher：Japan Society for Pediatric Otorhinolaryngology  

Revision/additional surgeries after cochlear implants are sometimes required because of device failure, hematoma, infections, and malinsertion/extrusion of electrodes. Younger implant age may also increase the incidence of additional ear surgery due to the immaturity of the Eustachean tubes. Since 1992, we have conducted 150 pediatric cochlear implants with three revisions (2.0%) or two additional surgeries (1.3%) required so far. All three revision cases were due to device failure, and ipsilateral explantation/reimplantation surgeries were conducted. The two additional cases included a case of ventilation tube malplacement and a case of myringoplasty. A case with implant failure after head trauma is reported here in detail.<br/>  A 7-year-old girl who received cochlear implantation at the age of two visited our hospital because she didn't respond to sound after a head trauma. Her speech perception had been good until her head was struck by a swing in a park, and she came to our hospital four days later. An impedance check or NRT of the cochlear implant was impossible, and explantation and reimplantation of the same side cochlear implant was performed six weeks later. Insertion of the electrodes was extremely difficult because of inner ear malformation, but the right position of the electrode was finally confirmed by X-ray. Her speech perception and language development were good after the operation.

DOI： 10.11374/shonijibi.32.409

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Other Link： http://search.jamas.or.jp/link/ui/2012118058

Language：Japanese   Publisher：(一社)日本耳科学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

MicroRNAs (miRNAs) play functional roles in sound transduction in cochlea. This study focuses on the validity of cochlear culture as an in vitro experimental tool, in view of miRNA expression. E15 cochleae were dissected and maintained in vitro for 48 h before extraction of miRNAs. MiRNA expression was comprehensively screened in explanted cochleae using a miRNA array that covers 380 miRNAs. A strong correlation was observed between expression levels of miRNAs in in vitro and in in vivo cochleae. Levels of 43 miRNAs were altered in vitro and these changes were reproducible over three trials. These findings indicate that in vitro miRNA profiling is a viable method for analysis of gene expression and action of chemical compounds on cochleae. NeuroReport 22:652-654 (C) 2011 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins.

DOI： 10.1097/WNR.0b013e32834a0273

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Language：Japanese   Publisher：(一社)日本聴覚医学会  

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Other Link： https://search-tp.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2011&ichushi_jid=J00125&link_issn=&doc_id=20111013470109&doc_link_id=%2Fdm7audio%2F2011%2F005405%2F109%2F0513-0514%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdm7audio%2F2011%2F005405%2F109%2F0513-0514%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：Japanese   Publisher：(一社)日本聴覚医学会  

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Other Link： https://search-tp.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2011&ichushi_jid=J00125&link_issn=&doc_id=20111013470037&doc_link_id=%2Fdm7audio%2F2011%2F005405%2F037%2F0369-0370%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdm7audio%2F2011%2F005405%2F037%2F0369-0370%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：Japanese   Publishing type：Research paper (scientific journal)  

The dissemination of newborn hearing screening (NHS) has enabled those with mild to moderate hearing loss to be diagnosed in early infancy. The "handicapped independence support law", however, prevents those not determined to be physically handicapped; i.e., those with mild to moderate hearing loss-, from government assistance in purchasing hearing aids. This results either in (i) parents purchasing hearing aids at their own expense or (ii) nonpurchase of hearing aids. To redress this situation, subvention in purchasing hearing aids of mild to moderate hearing loss children has been begun by some local governments. We petitioned for such aid at the Okayama Prefecture Assembly. A society for supporting pediatric hearing difficulty in Okayama Prefecture also submitted a similar petition in September 2009, and subvention began in April 2010 for all such children in Okayama Prefecture. We report procedures and details leading to this subvention.

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Language：Japanese   Publishing type：Research paper (scientific journal)  

The introduction of newborn-hearing screening has enabled early childhood hearing loss to be diagnosed and increased the number of children undergoing early care. Bilateral hearing loss is found in 0.08% of newborns and children whose hearing loss progresses or onset is delayed account for 4 to 30% of all pediatric hearing impairment. Children with perinatal risk factors tend to have deteriorated hearing or delayed-onset hearing loss in early childhood, necessitating audiometric follow-up. We also are aware of some children without risk factors who develop hearing impairment during infancy or early childhood. Hearing deterioration may be difficult to diagnose objectively, especially in young children, the presence of risk factors must be determines as soon as possible, especially given the lack of hearing management and close examination of children without apparent risk factors. We retrospectively studied children born from April 1998 to March 2007 and undergoing cochlear implantation as of April 2008. Among cases, we focused on 10 whose hearing impairment advanced during infancy -4 with risk factors known before hearing deterioration progressed, and 6 cases thought not to have any risk factors. We detected enlarged vestibular acquaduct in 3 of these 6 cases, and 3 more of whom had no risk factors -2 passing newborn-hearing screening and 1 in whom such screening detected hemilateral hearing loss. Our results underscore the need for early temporal computed tomography for detecting enlarged vestibular aquaduct. Even children with mild or hemilateral hearing loss should undergo audiometric and developmental testing at least every 6 months up to going to elementary school. Children suspected of impaired hearing should undergo thorough hearing tests regardless of newborn hearing-screening results to catch any problems early. Appropriate regular hearing and language development check-up tests must also be developed.

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Language：Japanese   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

新生児聴覚スクリーニング検査導入以後、乳児期および幼児期早期に難聴の確定診断がされ、療育が行われる例が増加した。しかし、乳幼児期に難聴が進行する児や、新生児期には難聴が認められなかったにもかかわらず遅発性に難聴を発症した児が存在することが報告され、その割合は難聴児の4-30%に及ぶとされている。進行性および遅発性難聴児の中に、難聴の危険因子を予見できない児が少数ながら存在することも明らかになっている。今回われわれは1998年4月から2007年3月までに出生した岡山県在住の小児で、2008年4月までに当院で人工内耳埋込み術を行った45例の中から乳幼児期に進行性および遅発性難聴が認められた10例(人工内耳装用児中の22.2%)を抽出し、新生児期に危険因子をもたないと考えられていた6例について詳細に検討した。6例中3例では、難聴進行後に画像検査にて前庭水管拡大症が発見されたが、他の3例の原因は不明であった。対象数は少ないが、新生児期に難聴の危険因子をもたない半数に画像検査で前庭水管拡大症が発見されたことにより、可能な限り画像検査を行うことが難聴増悪の早期発見の点から望ましいと考えられた。また、進行性難聴や新生児聴覚スクリーニング検査にパスした場合でも乳幼児期に遅発性難聴となる可能性があることを念頭に置き、就学前までは定期的な聴覚検査や現在自治体で行われている健診における聴覚に関する質問事項や検査の充実なども考慮すべきである。(著者抄録)

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Other Link： https://search-tp.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2011&ichushi_jid=J01099&link_issn=&doc_id=20110708420003&doc_link_id=1390001205012106368&url=https%3A%2F%2Fcir.nii.ac.jp%2Fcrid%2F1390001205012106368&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_3.gif

Language：Japanese   Publisher：日本小児耳鼻咽喉科学会  

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Language：Japanese   Publisher：日本小児耳鼻咽喉科学会  

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Language：Japanese   Publisher：日本小児耳鼻咽喉科学会  

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Language：Japanese   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

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Language：Japanese   Publisher：耳鼻咽喉科臨床学会  

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Language：Japanese   Publisher：耳鼻咽喉科臨床学会  

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Language：Japanese   Publisher：Japan Society for Pediatric Otorhinolaryngology  

Patients with Townes-Brocks syndrome (TBS) exhibit polydactyl, atresia ani and a variety of ear anomalies including congenital external ear atresia and profound hearing impairment. We reported a case of TBS that demonstrated profound hearing impairment due to inner ear malformation. A cochlear implant was chosen as a hearing device for this patient. Three dimensional re-construction imaging was useful for preoperative planning of the cochlear implant.

DOI： 10.11374/shonijibi.30.222

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Other Link： http://search.jamas.or.jp/link/ui/2010091910

Language：English   Publishing type：Research paper (international conference proceedings)   Publisher：SPRINGER  

Among 80 prelingually deafened children who underwent cochlear implant at Okayama University Hospital, two auditory neuropathy/auditory dys-synchrony (AN/AD) cases with cochlear implant were identified from review of medical records. These two cases first demonstrated stable responses with distortion product otoacoustic emission, although later the response disappeared. In spite of the presence of AN/AD, the language development of these cases was quite satisfactory so far. Herein, we report the clinical course and language development of these auditory neuropathy cases with cochlear implant.

DOI： 10.1007/978-4-431-09433-3_8

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：SPRINGER  

The goal of our study was to identify the role of auditory steady-state responses for hearing assessment in patients with functional hearing loss. The study design was to compare auditory steady-state response thresholds and standard pure-tone audiometry thresholds between patients with functional or sensorineural hearing loss. Subjects comprised 16 patients (24 ears) with functional hearing loss and 17 patients (24 ears) with sensorineural hearing loss. Differences and correlations between auditory steady-state response thresholds and standard pure-tone audiometry thresholds at 500, 1,000, 2,000 and 4,000 Hz were evaluated. In children with functional hearing loss, pure-tone audiometry thresholds and auditory steady-state response thresholds were significantly different at all frequencies and were not significantly correlated. In patients with sensorineural hearing loss, pure-tone audiometry thresholds and auditory steady-state response thresholds did not differ significantly at any frequencies and were significantly correlated. Auditory steady-state responses may have principal role in the assessment of auditory brainstem acuity, particularly at low frequencies in patients with functional hearing loss.

DOI： 10.1007/s00405-007-0550-3

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：ELSEVIER SCI LTD  

Learning disability combined with hearing impairment (LDHI) is a poor prognostic factor for the language development of hearing impaired children after educational intervention. A typical example of a child with LDHI and effective interventions provided by cochlear implants are presented in this report. A case of congenital cytomegaloviral infection that showed dysgraphia as well as profound deafness was reported and an underlying visual processing problem diagnosed in the present case caused the patient's dysgraphia. The dysgraphia could be circumvented by the use of auditory memory fairly established by a cochlear implant. (C) 2007 Published by Elsevier Ireland Ltd.

DOI： 10.1016/j.anl.2007.07.013

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：ELSEVIER IRELAND LTD  

Introduction: This study examines the ability and development in the comprehension of abstract words with hearing impaired children. The ability to understand abstract words is quite important for their academic learning and adaptation in their school life. Here, we qualitatively and quantitatively analyzed the development of abstract vocabulary in hearing impaired children using The Standardized Comprehension Test for Abstract Words (SCTAW).
Subjects and methods: We examined 75 hearing impaired children (hearing aid users, 61; cochlear implant users, 14; 1st to 10th grade) and 188 children with normal hearing (1st to 6th grade) using the Picture Vocabulary Test (PVT) and SCTAW.
Results: The PVT and SCTAW results closely correlated (r = 0.87). The SCTAW scores of the hearing impaired group were lower than those of their peers with normal. hearing, but the scores improved as their school. grade advanced. In particular, their abstract ability began to catch up from the fifth grade. The error trends of abstract vocabulary in the two groups did not significantly differ.
Conclusions: The SCTAW was useful as an abstract lexical. evaluation of hearing impaired children. The development of an abstract vocabulary did not qualitatively differ between children with or without a hearing impairment. (c) 2007 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.ijport.2007.06.015

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Language：Japanese   Publisher：Japan Audiological Society  

Language development was evaluated among 7 hearing-impaired, school-aged children who were identified through newborn hearing screening. Six of the 7 cases were enrolled into our intervention program before they were 6 months of age and their hearing levels were finally confirmed by 10 months of age. No additional developmental delay was observed in 5 of the 7 cases and their monosyllable speech perception and speech intelligibility developed fairly well. Among these children, 4 children who used cochlear implants showed a normal range of language development as assessed by WPPSI. A borderline mentally retarded case with a cochlear implant developed her monosyllable speech perception and speech intelligibility in a fair manner, although she still showed mild delay in her language development. A patient who showed poor intra-postoperative NRT preferred to use visually presented information and demonstrated poor speech perception as well as speech intelligibility. His language skills gradually developed after addition of visual modalities. Greater benefit can be expected by combining available methods in accompaniment with newborn hearing screening and cochlear implants. These methods will become one of models for intervention in deaf children.

DOI： 10.4295/audiology.50.254

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Language：Japanese   Publisher：Japan Audiological Society  

DOI： 10.4295/audiology.50.453

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Language：Japanese   Publishing type：Research paper (scientific journal)  

DOI： 10.11334/jibi1954.53.1_8

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Language：Japanese   Publishing type：Research paper (scientific journal)  

DOI： 10.11334/jibi1954.53.4_191

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Language：Japanese   Publisher：Japan Society for Pediatric Otorhinolaryngology  

Various outcomes of language development have been reported among children with prelingual hearing impairment and successful hearing intervention may not always ensure appropriate language development in their future. Learning disability is the symptom that is most frequently combined with hearing impairment, accounting for nearly 10% of these children. The combination of developmental dyslexia, which is the main symptom of learning disabilities, may hamper their language development by affecting their reading/writing ability. However, the detection of developmental dyslexia during their preschool period is highly difficult regardless of the presence of hearing loss. Before evaluating hearing impaired children,37 normal-hearing children in preschool education were examined in terms of the cognitive abilities that are required for reading/writing abilities. The evaluations included Raben's colored progressive matrix test (RCPM), language developmental tests (S-S), Benton's visual memory tests, Phonological awareness tests and the Single Kana-moji writing test. S-S test and RCPM are useful for the exclusion of the cases with apparent developmental delays and reading comprehension did not correlate with their calendar age. However, drastic development of Kana-writing was observed after 60 moths of age.

DOI： 10.11374/shonijibi1980.27.3_262

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Other Link： http://search.jamas.or.jp/link/ui/2007099016

Language：English   Publishing type：Research paper (scientific journal)   Publisher：ELSEVIER IRELAND LTD  

Objective: While investigators have reported that patients with GJB2-associated deafness and cochlear implants have preferable language development, the mechanisms of this phenomenon remains unknown. The goat of the present study was to assess higher brain functions of patients with GJB2-retated and GJB2-unrelated deafness as a method of evaluating language development.
Methods: Eight children with cochlear implants were subjected to genetic testing for GJB2 and underwent the Raven colored progressive matrices test, Rey's auditory verbal learning test, Rey's complex figure test, the standardized Language test for aphasia, the picture vocabulary test, and the standardized comprehension test for abstract words.
Results: Three children were diagnosed with GJB2-related deafness, and five children were diagnosed with GJB2-unrelated deafness. All three GJB2-related cases demonstrated normal range higher brain functions and fair language development. By contrast, one GJB2-unrelated case showed a semantic disorder, another demonstrated a visual cognitive disorder with dyslexia, and another had attention deficit-hyperactivity disorder.
Conclusions: Children with GJB2-unrelated deafness showed a high frequency of heterogeneous disorders that can affect proper language development. This difference between children with GJB2-retated and GJB2-unrelated deafness may account for the improved language development in children with GJB2-related deafness and cochlear implants. Further, genetic diagnosis of the non-syndromic hearing toss represents a useful tool for the preoperative prediction of outcomes following a cochlear implant procedure. (C) 2006 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.ijporl.2006.01.010

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Language：Japanese   Publisher：JIBI TO RINSHO KAI  

EMG syndrome is a relatively frequent congenital syndrome that is typically associated with exomphalos, macroglossia and gigantism. A hearing impairment has also been previously reported in 4 cases of EMG syndrome. A case of EMG syndrome which demonstrated demineralization as well as mixed hearing loss is herein reported. A nine-year-old girl was referred to our hospital because of a mild hearing impairment. Pure tone audiometry revealed a bilateral, mild mixed hearing impairment and temporal bone CT imaging demonstrated demineralization around the bony labyrinth. This is the first report to describe the temporal bone CT findings of EMG syndrome. Along with surgically proven stapes fixation that has been previously reported, these findings also suggested a similarity of this case with the background pathophysiology of otoscrelosis.

DOI： 10.11334/jibi1954.52.6_338

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Other Link： http://search.jamas.or.jp/link/ui/2007108867

Language：Japanese   Publisher：Japan Audiological Society  

Nine hearing-impaired children with cochlear implants were subjected to the confusion matrix analysis by the 57S monosyllable speech discrimination test. The mean monosyllable discrimination score was 85.1% and its confusion pattern with cochlear implants themselves was generally better or equivalent to that with hearing aids, except for poorer discrimination of voiced plosives. Discrimination of voiceless consonants was better than that of voiced consonants, except for that of /j/ and /w/. Fricatives, with the exception of the glottal fricative /h/, could be perceived better by cochlear implantees. The lowest monosyllable discrimination was observed for the palato-alveolar affricate /t∫/. As the source of the articulation moved farther, poorer discrimination of fricatives and affricates was observed. Fine frequency- and time-resolution provided by cochlear implants play an important role in the improvement of monosyllable speech perception.

DOI： 10.4295/audiology.49.798

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Language：Japanese   Publisher：Japan Audiological Society  

Maternal anxiety has been considered as a possible disadvantage of Newborn Hearing Screening (NHS). We reviewed the results of an interview survey conducted by public-health nurses (PHN) during initial home visits performed as part of the Okayama Prefecture NHS Program. The questionnaires assessed maternal anxiety, worry, certainty about the babies' hearing, and maternal attitude toward hearing screening. In total, 47, 346 newborns underwent NHS in Okayama Prefecture, and 248 babies were referred for further evaluation, One hundred seventy-one interviews were available for review in this study. Thirtynine (22.8%) mothers complained of anxiety or worry during the home visit by the PHN. Nineteen cases (11.1%) were diagnosed as having additional handicaps, and the major causes of maternal anxiety in these cases were severer disabilities other than hearing impairment. Sixteen cases (9.4%) were in the process of coping with a confirmed or potential diagnosis of hearing impairment. Only 4 cases (2.3%) demonstrated unexpected anxiety because of a suspicion of hemilateral hearing impairment. Child-neglect was observed in one case with multiple anomalies. Although the incidence of maternal anxiety after NHS was not high, compared with maternal anxiety in the absence of NHS, efforts to minimize maternal anxiety and worry, especially of mothers whose infants are referred for further evaluation, will be indispensable to establishing workable NHS programs in Japan.

DOI： 10.4295/audiology.49.227

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：JIBI TO RINSHO KAI  

The auditory steady-state responses (ASSRs) have been investigated since the 1990s, which can be examined as the frequency-specific auditory responses. Especially recently, with the introduction of newborn hearing screening, the ASSRs are clinically expected to become one of the objective audio grams for newborn infants. However, so far no report has attempted to determine the ASSRs reli ability or validity regarding the frequency-specific hearing threshold, or the effect on the level of consciousness. We regard it as important to examine the reliability in estimating the hearing level objectively. In this study, we recorded the ASSRs from 36 adults (72 ears) with normal hearing and 17 children and adults with severe sensory neural hearing loss (30 ears), and thus determined any cor relations between the ASSRs and standard audiograms using a multivaliate analysis. In addition, we also performed the bispectral index with a bispectral monitor and ASSRs at the same time from 4 nor mal hearing adults (8 ears), and examined the data both when the subjects were awake and asleep.

DOI： 10.11334/jibi1954.52.1_33

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：Japan Society for Pediatric Otorhinolaryngology  

Learning disability is the single most frequent complication of hearing impairment, occurring in up to 15% of all school-aged deaf children. Neuropsychological evaluations of these cases may contribute to further diagnosis of the additional handicaps and the identification of a suitable procedure for better intervention. A nine-year-old boy with mild hearing impairment was referred to our hospital because of his poor language development. His average pure tone audiometry was 35dBnHL in the right ear and 65 dBnHL in the left ear and audiological interventions with a hearing aid had already been started before the referral to our clinic. Although mild mental retardation was suggested by WISC-III, as FIQ65 (VIQ 65, VIQ71, and PIQ65), non-verbal intelligence examined by RCPM corresponded to the normal range. Verbal and non-verbal semantic disorder was considered by the results from ScTaw and SLTA. He was later diagnosed as having Asperger syndrome and his semantic problem concomitant with Asperger syndrome could be another cause of his additional language delay.

DOI： 10.11374/shonijibi1980.26.2_51

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Language：Japanese   Publisher：Japan Society for Pediatric Otorhinolaryngology  

A considerable number of prelingually-deafened children with cochlear implants demonstrated discrepancies between their speech perception ability and language development. The difference may be caused partly by the intrapersonal discrepancies among cognitive functions, including visual and auditory cognitive functions. Different clinical outcomes were observed among 3 prelingual deafness cases with cochlear implants and the profiles of their language development and neuropsychological tests were reported here. One case (MW) demonstrated the proper syntactic strategy corresponding to her hearing peers and neuropsychological evaluations revealed that her cognitive functions were well balanced. Two other cases (SN and YH) demonstrated considerable delay in the syntactic strategy for their communication and visual cues were needed to accomplish the interventional goal. It was assumed that the auditory memories of these two cases were affected. Although their medical and educational backgrounds were highly similar, the first outcome and the consequent procedures for intervention were different. Neuropsychological tests can provide crucial information for the optimum interventional approach for implant users.

DOI： 10.11374/shonijibi1980.25.2_46

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Other Link： http://search.jamas.or.jp/link/ui/2005089882

Publishing type：Research paper (scientific journal)  

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. © 2003 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/S0165-5876(03)00187-3

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：ELSEVIER SCI IRELAND LTD  

We report 3 rare cases of Meniere's disease in children. In Case I and 3, vertigo, hearing loss and tinnitus recovered soon after medical therapy. In Case 2. however, vertigo recurred and the hearing level on the right side markedly deteriorated. The equal-loudness contours on three-dimensional audiogram showed that right-sided aggravated hearing loss fluctuated for 4 years at middle-and low-frequencies despite medication. Finally intratympanic injection of gentamicin sulfate was performed. The patient has had no definitive spell of vertigo after gentamicin therapy. At our department, the incidence of Meniere's disease in pediatric patients with vertigo was 2.9%. (C) 2001 Elsevier Science Ireland Ltd. All rights reserved.

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Language：Japanese   Publisher：The Society of Practical Otolaryngology  

Granulocytic sarcoma was first described by Burns in 1811 and it is known to be an extramedullary tumor composed of immature precursor cells. It sometimes occurs with acute myeloid leukemia or myeloproliferative disorders, but there have been reports of cases occurring without such hematological disorders. A 40-year-old female with a chief complaint of neck swelling was examined in our department in 1997. CT scan showed bilateral neck lymph nodes swelling. Histological examination of open biopsy confirmed myeloblastic-type tumor cells, but peripheral blood and bone marrow examination showed no evidence of leukemia. The final diagnosis of this case was granulocytic sarcoma without hematological disorder.

DOI： 10.5631/jibirin.94.645

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Other Link： http://search.jamas.or.jp/link/ui/2002017688

J-GLOBAL

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Language：Japanese   Publishing type：Research paper, summary (national, other academic conference)   Publisher：(一社)日本聴覚医学会  

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Authorship：Last author   Language：Japanese   Publishing type：Research paper, summary (national, other academic conference)   Publisher：(一社)日本聴覚医学会  

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Authorship：Last author   Language：Japanese   Publishing type：Research paper, summary (national, other academic conference)   Publisher：(一社)日本聴覚医学会  

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Authorship：Lead author   Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (scientific journal)   Publisher：(一社)日本聴覚医学会  

聴覚情報処理障害(APD)/聞き取り困難(LiD)は,聴覚閾値は正常であるが中枢性聴覚情報処理が困難である状態で,騒音下や複数音声下の聞き取り困難を呈する。そのため,学校や社会生活の様々な場面において聴取および理解が難しく,コミュニケーションや学習,業務に支障を来すことが多い。APD/LiDの診断には,聴覚検査にて末梢性聴覚障害を除外し,聴覚情報処理検査(両耳分離聴検査,早口音声聴取検査,雑音下聴取検査等)を用いて行うが,質問紙での自覚的認知検査結果も参考にする。APD/LiDの介入と支援の方法は,音声入力情報の調整,環境調整,機器を用いた情報強化,トレーニングがある。音声入力情報の調整以外は,聴取の困難さに対するアプローチであるボトムアップ式と,内容を推測しやすくするアプローチであるトップダウン式の手段があり,各人の課題を分析した上で,必要な介入・支援方法を選定している。(著者抄録)

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2023&ichushi_jid=J00125&link_issn=&doc_id=20230901310002&doc_link_id=10.4295%2Faudiology.66.230&url=https%3A%2F%2Fdoi.org%2F10.4295%2Faudiology.66.230&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (scientific journal)   Publisher：金原出版(株)  

症例は47歳女性で、左鼠径部に3cm大の皮下腫瘤を自覚し、CTでリンパ節腫脹を指摘されたため前医外科を受診した。その後、原発不明悪性黒色腫の疑いで当院を紹介受診し、左鼠径リンパ節郭清を全身麻酔下で施行した。約6時間後に夕食を摂取し、その30分後に両耳介周囲の腫脹が出現、麻酔科、耳鼻科と併診した。麻酔科では手技や術後経過は麻酔科的に問題がないと考えられ、アナフィラキシーのような全身症状や皮膚症状もなく、限局した腫脹のみで使用した薬剤との関連は明らかではなかった。耳鼻科では臨床症状、採血結果から細菌感染症、ウイルス感染症の可能性は低いと判断された。アレルギー性耳下腺炎を疑われベポタスチンの内服を開始し、発症から3日で症状が軽快し、血清アミラーゼ値も正常化したためベポタスチンの内服は終了とした。以降症状の再燃はなかった。鼠径リンパ節の病理診断は悪性黒色腫の転移であった。

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Authorship：Lead author   Language：Japanese   Publishing type：Rapid communication, short report, research note, etc. (scientific journal)   Publisher：(一社)日本小児神経学会  

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Authorship：Lead author   Language：Japanese   Publishing type：Research paper, summary (national, other academic conference)   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

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Authorship：Lead author   Language：Japanese   Publishing type：Research paper, summary (national, other academic conference)   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

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Authorship：Lead author   Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (scientific journal)   Publisher：(株)東京医学社  

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2023&ichushi_jid=J00621&link_issn=&doc_id=20230511070054&doc_link_id=10.24479%2Fperi.0000000888&url=https%3A%2F%2Fdoi.org%2F10.24479%2Fperi.0000000888&type=%E5%8C%BB%E6%9B%B8.jp_%E3%82%AA%E3%83%BC%E3%83%AB%E3%82%A2%E3%82%AF%E3%82%BB%E3%82%B9&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00024_2.gif

Language：Japanese   Publisher：(一社)日本耳鼻咽喉科頭頸部外科学会  

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Authorship：Lead author   Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (scientific journal)   Publisher：(一社)日本聴覚医学会  

小児難聴のうち身体障害者に該当しない軽度・中等度難聴児は約60%を占める。近年新生児聴覚スクリーニングによる早期診断,療育開始や軽度・中等度難聴児補聴器助成事業導入といった社会的制度が拡充されてきた。軽度・中等度難聴児は一般的に聴取や言語発達が良好で,通常学校・級在籍児が多く,通級も含めて聴覚に関する特別支援教育を受ける児は少数である。しかし一方で,聴取能,言語発達遅滞や学力の低下,社会性の問題などが出現する頻度が高く,年齢が上がるにつれて顕著化,複雑化する傾向がある。インクルーシブ教育においては一見コミュニケーションに支障がなさそうであるが故,必ずしも適切な介入,教育者等による適切な理解と支援が受けられていないことが多い。通学校での合理的配慮や課題の把握を行うとともに,言語聴覚士や聴覚支援学校教師等による専門的検査や指導を併用できるよう,連携した支援体制の構築,情報共有が望まれる。(著者抄録)

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16歳男性。幼少時より右高度感音難聴があった。今回、意識障害で前医に救急搬送され、細菌性髄膜炎の診断にてCeftriaxone(CTRX)投与のもとICU入院加療となった。頭痛・嘔吐は改善したが、CRPは陰転化せず、CTにて中耳陰影、内耳奇形を疑われ、原因精査目的で著者らの施設へ紹介となった。来院時、右鼓膜は膨隆し軽度混濁していたが発赤はなかった。だが、右耳管咽頭口よりは透明な液体の流出がみられた。また、左鼓膜は正常で、純音聴力検査では右側は聾、左側は正常であった。一方、側頭骨CTでは右鼓室内・乳突腔内に貯留液様の軟部陰影がみられ、右蝸牛は低形成で、蝸牛と前庭は癒合し、内耳道底の隔壁の欠損が疑われた。以上より、本症例は内耳奇形(Incomplete partition type I)に伴う髄液漏出による細菌性髄膜炎と診断され、髄液からはStreptcoccus pneumoniaeが検出された。治療として内耳開窓閉鎖術を施行したところ、術後4日目より耳内は乾燥、耳管咽頭口からの髄液漏出は認めずCRPも陰性化した。目下、術後3ヵ月経過で液漏出や髄膜炎の再発は認められていない。

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79歳女性。関節リウマチ、糖尿病、高血圧症、右人工股関節留置ほか、原因不明のS状結腸穿孔後ストーマ留置の既往歴があり、プレドニゾロン+タクロリムス水和物などを内服中であった。今回、左耳の耳漏を主訴に近医を受診、外耳炎と診断され、点耳治療中であったが、耳漏が改善せず、食事摂取時の耳漏流出が止らないため、著者らの施設へ来院となった。初診時、採血で炎症所見が認められ、即日入院となり、Chiari点を中心とした扁桃周囲の切開排膿、更に超音波ガイド下に穿刺排膿およびCEZ+CLDMの投与が行われた。その結果、入院第4病日目には炎症所見はやや改善したがALPが高値のためCLDMの影響と考え中止した。以後、第5病日目には膿瘍および耳漏の培養の結果から、Streptococcus anginosusが原因菌と判明したため抗菌薬をアンピシリンに変更、第8病日目にはCRP値が正常化し、第10病日目に経過良好で退院となった。以上より、深頸部感染症は糖尿病・高血圧・慢性腎不全・慢性肝炎などの基礎疾患を有する症例で多くみられ、本症例も基礎疾患が多岐にわたることから今後の厳重な経過観察が必要と考えられた。

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Presentation type：Symposium, workshop panel (nominated)  

File： ENG_Sept 29 Healthy Aging seminar and panel discussion Invitation.pdf

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Presentation type：Public lecture, seminar, tutorial, course, or other speech  

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Presentation type：Public lecture, seminar, tutorial, course, or other speech  

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Presentation type：Public lecture, seminar, tutorial, course, or other speech  

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Presentation type：Symposium, workshop panel (nominated)  

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Presentation type：Symposium, workshop panel (nominated)  

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Presentation type：Public lecture, seminar, tutorial, course, or other speech  

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Presentation type：Public lecture, seminar, tutorial, course, or other speech  

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Presentation type：Poster presentation  

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Presentation type：Oral presentation (general)  

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Presentation type：Public lecture, seminar, tutorial, course, or other speech  

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Type：Seminar, workshop

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Type：Lecture

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Type：Lecture

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Type：Lecture

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Type：Lecture

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Type：Citizen’s meeting/Assembly

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Type：Lecture

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Type：Lecture

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Type：Lecture

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Type：Lecture

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Type：Lecture

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Type：Lecture

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Type：Lecture

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