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Autoimmune mechanisms may play crucial roles in the etiology of endolymphatic hydrops (ELH), which was previously regarded as a postmortem finding in the temporal bone. Recently, ELH has been visualized using 3-T MR imaging in living patients. A 47-year-old woman with deafness in the left ear since adolescence developed right-sided steroid-responsive sensorineural hearing loss in the low frequencies. During over 15 years of follow-up at our otolaryngology clinic, acute deteriorations of hearing in the only hearing ear repeatedly recovered with administration of intravenous and oral steroids. Hearing in the only hearing ear at 62 years old was preserved and comparable to that at 47 years old. At 61 years old, cochlear ELH was documented bilaterally on MR imaging, appearing more severe in the deafened ear than in the hearing ear. This case provides new evidence of the potential steroid-responsiveness of hearing loss due to contralateral-type delayed ELH distinctly visualized on MR imaging.

DOI： 10.1016/j.anl.2023.03.009

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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)日本耳科学会  

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

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

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J00125&link_issn=&doc_id=20220912480024&doc_link_id=%2Fdm7audio%2F2022%2F006505%2F025%2F0323-0323%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdm7audio%2F2022%2F006505%2F025%2F0323-0323%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

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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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

The vestibulo-ocular reflex (VOR) in neurotologic patients can be evaluated most quickly by spontaneous nystagmus examinations, and the video Head Impulse Test (vHIT) quantitatively evaluates the VOR of the semicircular canals. We aimed to clarify the concordance and discrepancies between spontaneous nystagmus and vHIT findings, to provide guidance on screening vestibular functions by initial spontaneous nystagmus examination then vHIT. We evaluated 169 outpatients by spontaneous nystagmus examination in the dark using a charge-coupled device camera, then by horizontal vHIT the same day. Vestibular loss on vHIT was defined if both reduced VOR gain (<0.8) and corrective saccade were observed. Adjusted logistic regression modelling revealed that differences in right and left VOR gain positively impacted the presence of nystagmus, which suggests lateralised vestibular dysfunction (P<0.05; odds ratio 1.39 [95% confidence interval, 1.1-1.8] per 0.1 increment). When vHIT is regarded as the standard clinical test, the positive predictive value of nystagmus for vestibular loss on vHIT was 44.4%, and the negative predictive value was 93.5%. The adjusted odds ratio of adults ≥65 years old compared to younger patients associated with a discrepancy of nystagmus and vHIT was significant (2.4 [1.1-5.3]). In conclusion, if spontaneous nystagmus is initially observed in patients, further assessment by vHIT could confirm vestibular dysfunction in 40-50% of cases. If no nystagmus is observed, vHIT might also result in a negative finding in >90% of cases. Older adults appear more likely to show discrepancies between nystagmus and vHIT.

DOI： 10.1016/j.jocn.2022.03.018

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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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In the original article published, there is a correction in Table 1. We would like to remove the variant (c.4023 + 1G > A) from the Table. The correct Table 1 is attached below. The original article has been corrected.

DOI： 10.1007/s00439-021-02392-y

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Understanding how sensorineural hearing loss (SNHL) impacts postural balance in patients is important, as postural balance predicts the risk of falls. AIMS/OBJECTIVES: We aimed to clarify the relationship between characteristics in the configuration of audiograms and static postural balance as measured by posturography. MATERIALS AND METHODS: We evaluated 385 outpatients (mean [± standard deviation] age, 58.4 ± 18.4 years) with SNHL by audiometry and posturography. Data were analysed by multiple regression models with the outcome of postural sway area with eyes closed (PSA) and predictive variables of audiometric data, adjusted for sex, age and the presence of nystagmus. RESULTS: The increased hearing threshold in the better hearing ear was associated with poorer or higher PSA (beta coefficient [β] = 0.39, 95% confidence interval [CI] = 0.03-0.75, per 10-dB increment). No difference in PSA was detected between patients with asymmetric or symmetric SNHL. None of the frequent diagnoses (presbyacusis, Meniere's disease, and idiopathic sudden SNHL) were associated with poorer PSA. Hearing thresholds at middle (β = 0.39, 95%CI = 0.10-0.67) and high frequencies (β = 0.31, 95%CI = 0.07-0.55) were associated with poorer PSA, whereas those at low frequencies was not. CONCLUSIONS AND SIGNIFICANCE: Postural balance in neurotologic patients may be associated with middle-high-frequency hearing levels during ageing.

DOI： 10.1080/00016489.2022.2047222

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

Epidemiological data suggest that inflammation and innate immunity play significant roles in the pathogenesis of age-related hearing loss (ARHL) in humans. In this mouse study, real-time RT-PCR array targeting 84 immune-related genes revealed that the expressions of 40 genes (47.6%) were differentially regulated with greater than a twofold change in 12-month-old cochleae with ARHL relative to young control mice, 33 (39.3%) of which were upregulated. These differentially regulated genes (DEGs) were involved in functional pathways for cytokine-cytokine receptor interaction, chemokine signaling, TNF signaling, and Toll-like receptor signaling. An NF-κB subunit, Nfkb1, was upregulated in aged cochleae, and bioinformatic analyses predicted that NF-κB would interact with the genomic regulatory regions of eight upregulated DEGs, including Tnf and Ptgs2. In aging cochleae, major proinflammatory molecules, IL1B and IL18rap, were upregulated by 6 months of age and thereafter. Remarkable upregulations of seven immune-related genes (Casp1, IL18r1, IL1B, Card9, Clec4e, Ifit1, and Tlr9) occurred at an advanced stage (between 9 and 12 months of age) of ARHL. Immunohistochemistry analysis of cochlear sections from the 12-month-old mice indicated that IL-18r1 and IL-1B were localized to the spiral ligament, spiral limbus, and organ of Corti. The two NF-κB-interacting inflammatory molecules, TNFα and PTGS2, immunolocalized ubiquitously in cochlear structures, including the lateral wall (the stria vascularis and spiral ligament), in the histological sections of aged cochleae. IBA1-positive macrophages were observed in the stria vascularis and spiral ligament in aged mice. Therefore, inflammatory and immune reactions are modulated in aged cochlear tissues with ARHL.

DOI： 10.1371/journal.pone.0258977

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

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

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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OBJECTIVES: To obtain new insights into research questions on how executive function and social interaction would be observed to change after the introduction of hearing aids (HAs) in older people with hearing impairment. DESIGN: Multi-institutional prospective single-arm observational study. SETTING AND PARTICIPANTS: Outpatients with complaints of hearing difficulty who visited HA clinics between October 18, 2017, and June 30, 2019, in 7 different university hospitals in Japan. METHODS: The inclusion criteria of the study named Hearing-Aid Introduction for Hearing-Impaired Seniors to Realize a Productive Aging Society-A Study Focusing on Executive Function and Social Activities Study (HA-ProA study) were age ≥60 years and no history of HA use. A series of multi-institution common evaluations including audiometric measurements, the digit symbol substitution test to assess executive functions, convoy model as an index of social relations, and hearing handicap inventory for the elderly (HHIE) were performed before (pre-HA) and after 6 months of the HA introduction (post-HA). RESULTS: Out of 127 enrollments, 94 participants completed a 6-month follow-up, with a mean age of 76.9 years. The digit symbol substitution test score improved significantly from 44.7 at baseline to 46.1 at 6 months (P = .0106). In the convoy model, the social network size indicated by the number of persons in each and whole circles were not significantly different between pre- and post-HA; however, the total count for kin was significantly increased (P = .0344). In the analyses of HHIE, the items regarding the family and relatives showed significant improvement. CONCLUSIONS AND IMPLICATIONS: HA use could benefit older individuals beginning to use HAs in executive function and social interaction, though the results should be interpreted cautiously given methodological limitations such as a single-arm short 6 months observation. Reduction in daily hearing impairment would have a favorable effect on relationships with the family.

DOI： 10.1016/j.jamda.2021.02.035

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

Pathologic mechanisms in cochleae immediately following the onset of noise-induced hearing loss (NIHL) remain unclear. In this study, mice were exposed to 120 dB of octave band noise for 2 h to induce NIHL. Three hours after noise exposure, expression levels of the whole mouse genome in cochleae were analyzed by RNA-seq and DNA microarray. Differentially expressed genes (DEGs) exhibiting >2-fold upregulation or downregulation in noise-exposed cochleae compared to controls without noise exposure were identified. RNA-seq and microarray analyses identified 273 DEGs regulated at 3 h post-noise (51 upregulated and 222 downregulated). Bioinformatic analysis revealed that these DEGs were associated with the functional gene pathway "neuroactive ligand-receptor interaction" and included 28 genes encoding receptors for neurotransmitters such as gamma-aminobutyric acid and glutamate. Other DEGs included 25 genes encoding transcription factors. Downregulation of 4 neurotransmitter receptors (Gabra3, Gabra5, Gabrb1, Grm1) and upregulations of 5 transcription factors (Atf3, Dbp, Helt, Maff, Nr1d1) were validated by RT-PCR. The differentially regulated transcription factor Atf3 immunolocalized to supporting cells and hair cells in the organ of Corti at 12-h post-noise. The present data serve as a basis for further studies aimed at developing medical treatments for acute sensorineural hearing loss.

DOI： 10.1016/j.neures.2020.05.001

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Congenital hearing loss affects 1 in every 1000 births, with genetic mutations contributing to more than 50% of all cases. X-linked nonsyndromic hereditary hearing loss is associated with six loci (DFNX1-6) and five genes. Recently, the missense mutation (c.1771G>A, p.Gly591Ser) in COL4A6, encoding the basement membrane (BM) collagen α6(IV) chain, was shown to be associated with X-linked congenital nonsyndromic hearing loss with cochlear malformation. However, the mechanism by which the COL4A6 mutation impacts hereditary hearing loss has not yet been elucidated. Herein, we investigated Col4a6 knockout (KO) effects on hearing function and cochlear formation in mice. Immunohistochemistry showed that the collagen α6(IV) chain was distributed throughout the mouse cochlea within subepithelial BMs underlying the interdental cells, inner sulcus cells, basilar membrane, outer sulcus cells, root cells, Reissner's membrane, and perivascular BMs in the spiral limbus, spiral ligament, and stria vascularis. However, the click-evoked auditory brainstem response analysis did not show significant changes in the hearing threshold of Col4a6 KO mice compared with wild-type (WT) mice with the same genetic background. In addition, the cochlear structures of Col4a6 KO mice did not exhibit morphological alterations, according to the results of high-resolution micro-computed tomography and histology. Hence, loss of Col4a6 gene expression in mice showed normal click ABR thresholds and normal cochlear formation, which differs from humans with the COL4A6 missense mutation c.1771G>A, p.Gly591Ser. Therefore, the deleterious effects in the auditory system caused by the missense mutation in COL4A6 are likely due to the dominant-negative effects of the α6(IV) chain and/or α5α6α5(IV) heterotrimer with an aberrant structure that would not occur in cases with loss of gene expression.

DOI： 10.1371/journal.pone.0249909

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Language：Japanese  

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2021&ichushi_jid=J00125&link_issn=&doc_id=20210302480007&doc_link_id=%2Fdm7audio%2F2021%2F006401%2F009%2F0087-0095%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdm7audio%2F2021%2F006401%2F009%2F0087-0095%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

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

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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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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

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

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Other Link： https://search.jamas.or.jp/default/link?pub_year=2019&ichushi_jid=J00125&link_issn=&doc_id=20191015460116&doc_link_id=10.4295%2Faudiology.62.463&url=https%3A%2F%2Fdoi.org%2F10.4295%2Faudiology.62.463&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_3.gif

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

Background: Difficulty in listening comprehension is a major audiological complaint of older adults. Behavioural auditory processing tests (APTs) may evaluate it. Aims/Objectives: The aim was to assess the feasibility of administering Japanese APTs to older adults at otolaryngology clinics. Material and Methods: Using computer programs interfaced with an audiometer, APTs (dichotic listening test; fast speech test, FST; gap detection test, GDT; speech in noise test; rapidly alternating speech perception test) were administered to 20 older adults (65-84 years old; mean 75.3 years) and 20 young adults at the 40 dB sensation level. Monosyllable speech perception (MSP) and the Mini-Mental State Examination (MMSE) were evaluated. Results: APT results except for GDT were significantly correlated with MSP. The performance on each APT was worse in older adults than in young adults (p < .01). The older adults with good MSP ≥ 80% (n = 13) or excellent cognitive function (MMSE ≥ 28; n = 11) also did worse on APTs (p < .05). A ceiling effect was noted in the APT data, with FST showing a minimum ceiling effect and reflecting interindividual variations of data. Conclusions and Significance: It is feasible to administer APTs to older adults who visit otolaryngology clinics. Among our Japanese APTs, FST may be suitable for further large-scale clinical studies.

DOI： 10.1080/00016489.2019.1612532

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Authorship：Lead author,　Last author   Language：English   Publishing type：Research paper (scientific journal)  

AIM: To comprehensively analyze cochlear gene expressions related to innate immunity and glucocorticoid signaling at onset of acute noise-induced hearing loss (NIHL). BACKGROUND: Recent studies suggested innate immunity is involved in the cochlear pathology of NIHL. Glucocorticoids may modulate immune actions in cochleae. METHODS: Mice were exposed to 120 dB-octave band noise for 2 hours. Twelve hours later, a targeted PCR array analyzed cochlear expressions of 84 key genes in inflammation and immune pathways and 84 genes in the glucocorticoid signaling pathway. Real-time RT-PCR was used to analyze expression of two immune-related genes, Ccl12 and Glycam1, in noise-exposed cochleae with or without dexamethasone. RESULT: In inflammatory and immune gene pathways, 31.0% (26/84 genes) were significantly upregulated (>2-fold change) or downregulated (<0.5-fold change) (p < 0.05) in noise-exposed cochleae compared with controls. Sixteen of these differentially expressed genes (DEGs) encoded chemokines. DEGs included Ccl12, Ccl2, Ccl4, Ccl7, Cxcl1, Cxcl10, and Ptgs2 (upregulated genes), and Ccr7, Cxcr2, Kng1, Ltb, and Tnfsf14 (downregulated genes). In the glucocorticoid signaling pathway, 92.9% (78/84 genes) were unchanged in noise-exposed cochleae without dexamethasone administration. Cochlear expressions of Ccl12 and Glycam1 were significantly upregulated by noise and downregulated by dexamethasone. CONCLUSION: The targeted PCR array demonstrated that several dozen genes involved in innate immunity are actively regulated in cochleae with NIHL. The glucocorticoid signaling pathway was not endogenously regulated at 12 hours post-noise trauma. Systemic dexamethasone downregulated Ccl12 and Glycam1, which are upregulated in noise-exposed cochleae. These data may provide a basis for genomic medicine treatment of acute sensorineural hearing loss.

DOI： 10.1097/MAO.0000000000001874

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

Transcanal endoscopic ear surgery (TEES) will become a very useful therapeutic option. A perilymphatic fistula (PLF) is defined as sudden sensorineural hearing loss and/or vertigo caused by leakage of the perilymph through a fistula from the oval window and/or round window. We report a case of PLF after electric acoustic stimulation (EAS), a kind of cochlear implant, successfully treated by TEES. A 38-year-old man presented to our hospital with vertigo and hearing loss (HL). His vertigo was induced by Valsalva maneuvers. Eight months ago, he underwent EAS for his right ear for congenital sensorineural HL. Although he maintained his hearing level after EAS, his pure tone audiogram this time showed deterioration of hearing at low frequencies in his right ear. A diagnosis of right PLF was made. After confirming the non-effectiveness of oral prednisolone treatment, PLF repair surgery to patch the oval and round windows by TEES was performed. His vertigo did not recur after the surgery. To the best of our knowledge, this is the first report of PLF repair surgery by TEES without a microscope. The wide-field view of the middle ear by TEES was useful to prevent electrode damage in a PLF patient with a cochlear implant.

DOI： 10.1016/j.anl.2017.10.004

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Public Library of Science  

Perilymphatic fistula is defined as an abnormal communication between the perilymph-filled space and the middle ear, or cranial spaces. The manifestations include a broad spectrum of neuro-otological symptoms such as hearing loss, vertigo/dizziness, disequilibrium, aural fullness, tinnitus, and cognitive dysfunction. By sealing the fistula, perilymphatic fistula is a surgically correctable disease. Also, appropriate recognition and treatment of perilymphatic fistula can improve a patient’s condition and hence the quality of life. However, the difficulty in making a definitive diagnosis due to the lack of an appropriate biomarker to detect perilymph leakage has caused a long-standing debate regarding its management. We have reported a clinical test for the diagnosis of perilymphatic fistula by detecting a perilymph specific protein, Cochlin-tomoprotein, as a diagnostic marker using a western blot. The aim of this study is to establish an ELISA-based human Cochlin-tomoprotein detection test and to evaluate its diagnostic accuracy in clinical subjects. The results of ELISA showed good dilution reproducibility. The mean concentration was 49.7±9.4 of 10 perilymph samples. The ROC curve in differentiating the perilymph leakage condition from the normal middle ear was significant (P &lt
0.001) with an area under the curve (AUC) of 0.918 (95% CI 0.824–0.100). We defined the diagnostic criteria as follows: CTP&lt
0.4 negative
0.4≦CTP&lt
0.8 intermediate
0.8≦CTP(ng/ml) positive in the clinical usage of the hCTP ELISA, and sensitivity and specificity were 86.4% and 100%, respectively. We further tested the expression specificity of the Cochlin-tomoprotein by testing blood and CSF samples. The concentration was below the detection limit (0.2 ng/ml) in 38 of the 40 blood, and 14 of the 19 CSF samples. We report the accuracy of this test for the diagnosis of perilymphatic fistula. Using ELISA, we can improve the throughput of the test. Furthermore, it is useful for a large-scale study to characterize the clinical picture and delineate the management of this medical condition.

DOI： 10.1371/journal.pone.0191498

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

Aim: To elucidate molecular mechanisms of noise-induced hearing loss (NIHL) and glucocorticoid therapy in the cochlea.
Background: Glucocorticoids are used to treat many forms of acute sensorineural hearing loss, but their molecular action in the cochlea remains poorly understood.
Methods: Dexamethasone was administered intraperitoneally immediately following acoustic overstimulation at 120 dB SPL for 2 hours to mice. The whole cochlear transcriptome was analyzed 12 and 24 hours following noise trauma and dexamethasone administration by both next-generation sequencing (RNA-seq) and DNA microarray. Differentially expressed genes (DEGs) with more than 2-fold changes after noise trauma and dexamethasone administration were identified. The functions of these DEGs were analyzed by David Bioinformatics Resources and a literature search.
Results: Twelve hours after acoustic overstimulation, immune-related gene pathways such as "chemokine signaling activity,'' "cytokine-cytokine receptor interaction,'' and "cell adhesion molecules (CAMs) in the immune system'' were significantly changed compared with the baseline level without noise. These DEGs were involved in immune and defense responses in the cochlea. Dexamethasone was administered to this NIHL model, and it modulated gene pathways of "cytokine-cytokine receptor interaction'' and "cell adhesion molecules (CAMs) in the immune system'' at 12 hours, compared with saline-injected control. Dexamethasone-dependent DEGs were also involved in immune and defense responses. A literature search showed that 10 other genes associated with hearing functions were regulated by dexamethasone both at 12 and 24 hours post-administration.
Conclusion: Dexamethasone modulates the immune reaction in the traumatized cochlea following acoustic overstimulation. Dexamethasone may also regulate cochlear functions other than immunity.

DOI： 10.1097/MAO.0000000000001373

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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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Authorship：Lead author,　Corresponding author   Language：English   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

DOI： 10.1097/MAO.0000000000001295

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：ELSEVIER SCI LTD  

Central auditory processing disorder (CAPD) is a condition in which dysfunction in the central auditory system causes difficulty in listening to conversations, particularly under noisy conditions, despite normal peripheral auditory function. Central auditory testing is generally performed in patients with normal hearing on the pure tone audiogram (PTA). This report shows that diagnosis of CAPD is possible even in the presence of an elevated threshold on the PTA, provided that the normal function of the peripheral auditory pathway was verified by distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), and auditory steady state response (ASSR). Three pediatric cases (9- and 10-year-old girls and an 8-year-old boy) of CAPD with elevated thresholds on PTAs are presented. The chief complaint was difficulty in listening to conversations. PTA showed elevated thresholds, but the responses and thresholds for DPOAE, ABR, and ASSR were normal, showing that peripheral auditory function was normal. Significant findings of central auditory testing such as dichotic speech tests, time compression of speech signals, and binaural interaction tests confirmed the diagnosis of CAPD. These threshold shifts in PTA may provide a new concept of a clinical symptom due to central auditory dysfunction in CAPD. (C) 2016 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.anl.2016.02.005

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：TAYLOR & FRANCIS LTD  

Conclusion: Significant expression of macrophage migration inhibitory factor and its receptor (CD74) was observed in both the middle ear and inner ear in experimental otitis media in mice. Modulation of macrophage migration inhibitory factor and its signaling pathway might be useful in the management of inner ear inflammation due to otitis media.Objectives: Inner ear dysfunction secondary to otitis media has been reported. However, the specific mechanisms involved are not clearly understood. The aim of this study is to investigate the expression of macrophage migration inhibitory factor and CD74 in the middle ear and inner ear in lipopolysaccharide-induced otitis media.Method: BALB/c mice received a transtympanic injection of either lipopolysaccharide or phosphate-buffered saline (PBS). The mice were sacrificed 24h after injection, and temporal bones were processed for polymerase chain reaction (PCR) analysis, histologic examination, and immunohistochemistry.Results: PCR examination revealed that the lipopolysaccharide-injected mice showed a significant up-regulation of macrophage migration inhibitory factor in both the middle ear and inner ear as compared with the PBS-injected control mice. The immunohistochemical study showed positive reactions for macrophage migration inhibitory factor and CD74 in infiltrating inflammatory cells, middle ear mucosa, and inner ear in the lipopolysaccharide-injected mice.

DOI： 10.1080/00016489.2016.1179786

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：TAYLOR & FRANCIS LTD  

Conclusion: Audiological parameters alone do not determine the choice to use hearing aids (HA). Subjective hearing-related QoL is a major factor that determines whether or not an older person will continue to wear HA.Objective: This study aimed to identify which audiological parameters and quality-of-life (QoL) measures determine whether or not older persons will continue wearing HA.Methods: Charts of 157 patients aged 65 years who attended the HA service unit at the Otolaryngology Department were retrospectively reviewed. After HA fitting and a trial, the patients were divided into groups, depending upon whether or not they wanted to continue wearing the HA (users, 58.2%; non-users, 41.8%) and then audiological parameters were compared between them. At least 4 months after the HA fitting, the self-reported QoL questionnaire, Hearing Handicap Inventory for the Elderly (HHIE), was mailed to all 157 patients and HHIE scores were compared between HA users and non-users.Result: Speech discrimination score and dynamic range did not significantly differ between HA users and non-users. A difference in the average hearing threshold was marginally significant. The response rate to the HHIE was 65.2%. Total HHIE and emotional scores were higher (more impaired) among HA users than non-users.

DOI： 10.1080/00016489.2016.1183817

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：CAMBRIDGE UNIV PRESS  

Background: Superficial siderosis is a rare disease that results from chronic bleeding in the subarachnoid space. Haemosiderin deposits throughout the subpial layers of the brain and spinal cord lead to progressive sensorineural hearing loss, which is seen in 95 per cent of patients with superficial siderosis. The impact of cochlear implantation on the quality of life of superficial siderosis patients is under debate.
Case report: A 38-year-old male with superficial siderosis presented with bilateral progressive sensorineural hearing loss. The patient underwent cochlear implantation and his quality of life was improved as evaluated by the Abbreviated Profile of Hearing Aid Benefit inventory.
Conclusion: The remarkable improvement in Abbreviated Profile of Hearing Aid Benefit scores shown in this study indicates that cochlear implantation leads to a better quality of life in superficial siderosis patients.

DOI： 10.1017/S0022215116000335

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：TAYLOR & FRANCIS LTD  

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)-1 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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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：TAYLOR & FRANCIS LTD  

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 &lt; 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)   Publisher：ELSEVIER IRELAND LTD  

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 Clan 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 &lt; 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 &lt; 0.05).
Conclusions: Early use of HAs until the CI operation may result in better language perception and academic achievement among Cl users with prelingual deafness. A long-term follow-up is required to assess the usefulness of NHS for language development. (C) 2015 Elsevier Ireland Ltd. All rights reserved.

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

Aim
Using proteomics, we aimed to identify the proteins differentially regulated by dexamethasone in the mouse cochlea based on mass-spectrometry data.
Background
Glucocorticoid therapy is widely used for many forms of sensorineural hearing loss; however, the molecular mechanism of its action in the cochlea remains poorly understood.
Methods
Dexamethasone or control saline was intratympanically applied to the cochleae of mice. Twelve hours after application, proteins differentially regulated by dexamethasone in the cochlea were analyzed by isobaric Tag for Relative and Absolute Quantitation (iTRAQ)-mass spectrometry. Next, dexamethasone-dependent regulation of these proteins was verified in the cochleae of mice with noise-induced hearing loss (NIHL) and systemic administration of dexamethasone by western blotting. Immunolocalizations of these proteins were examined in cochleae with NIHL.
Results
A total of 247 proteins with a greater than 95% confidence interval of protein identification were found, and 11 differentially expressed proteins by dexamethasone were identified by the iTRAQ-mass spectrometry. One protein, myelin protein zero (Mpz), was upregulated (1.870 +/- 0.201-fold change, p &lt; 0.01) at 6 hours post-systemic dexamethasone and noise exposure in a mouse model of NIHL. Heat shock protein 70 (Hsp70) was downregulated (0.511 +/- 0.274-fold change, p &lt; 0.05) at 12 hours post-systemic dexamethasone. Immunohistochemistry confirmed Mpz localization to the efferent and afferent processes of the spiral neurons, whereas Hsp70 showed a more ubiquitous expression pattern in the cochlea.
Conclusion
Both Mpz and Hsp70 have been reported to be closely associated with sensorineural hearing loss in humans. Dexamethasone significantly modulated the expression levels of these proteins in the cochleae of mice.

DOI： 10.1097/MAO.0000000000000748

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

Hypothesis Macrophage migration inhibitory factor plays an important role in noise-induced hearing loss.
Background Macrophage migration inhibitory factor is an essential factor in axis formation and neural development. Macrophage migration inhibitory factor is expressed in the inner ear, but its function remains to be elucidated.
Methods Macrophage migration inhibitory factor-deficient mice (MIF-/- mice) were used in this study. Wild-type and MIF-/- mice received noise exposure composed of octave band noise. Auditory brainstem response thresholds were examined before (control) and at 0, 12, and 24 hours and 2 weeks after the intense noise exposure. Morphological findings of cochlear hair cells were investigated using scanning electron microscopy. Histopathological examination with hematoxylin and eosin staining and TUNEL assay were also performed.
Results In both the wild-type and MIF-/- mice, acoustic overstimulation induced significant hearing loss compared with the control level. Two weeks after the intense noise exposure, the MIF-/- mice had an increased hearing threshold compared with the wild-type mice. Scanning electron microscopy demonstrated that the outer hair cells in the MIF-/- mice were affected 2 weeks after noise exposure compared with the wild-type mice. TUNEL-positive cells were identified in the organ of Corti of the MIF-/- mice.
Conclusion The MIF-/- mice had prolonged hearing loss and significant loss of cochlear hair cells after intense noise exposure. Macrophage migration inhibitory factor may play an important role in recovery from acoustic trauma. Management of macrophage migration inhibitory factor may be a novel therapeutic option for noise-induced hearing loss.

DOI： 10.1097/MAO.0000000000000755

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

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. (C) 2014 Elsevier Ireland Ltd. All rights reserved.

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

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 &lt;18 (low) vs. score &gt;18 (high)]. The secondary outcome was productive vocabulary, or the number of productive words, which was assessed by an original checklist [&lt;1773 words (low) vs. &gt;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 &lt; 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. (C) 2014 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.ijporl.2014.12.006

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：TAYLOR & FRANCIS LTD  

Conclusion: Performance in consonant-vowel (CV) monosyllable speech perception after cochlear implantation (CI) in the elderly (&gt;= 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 &gt;= 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 &lt; 0.05).

DOI： 10.3109/00016489.2015.1062547

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：PERGAMON-ELSEVIER SCIENCE LTD  

Hearing loss related to aging is the most common sensory disorder among elderly individuals. Macrophage migration inhibitory factor (MIF) is a multi-functional molecule. The aim of this study was to identify the role of MIF in the inner ear. MIF-deficient mice (MIF-/- mice) of BALB/c background and wild-type BALB/c mice were used in this study. Expression of MIF protein in the inner ear was examined by immunohistochemistry in wild-type mice (WT). The hearing function was assessed by the click-evoked auditory brain-stem response in both MIF-/- mice and WT at 1, 3, 6, 9, 12, and 18 months of age. Morphological examination of the cochlea was also performed using scanning electron microscopy and light microscopy. MIF was observed in the spiral ligament, stria vascularis, Reissner's membrane, spiral ganglion cells (SGCs), saccular macula, and membranous labyrinth. The MIF-/- mice had a significant hearing loss as compared with the WT at 9, 12, and 18 months of age. In the MIF-/- mice, scanning electron microscopy showed that the outer cochlear hair cells were affected, but that the inner cochlear hair cells were relatively well preserved. The number of SGCs was lower in the MIF-/- mice. MIF was strongly expressed in the mouse inner ear. Older MIF-/- mice showed accelerated age-related hearing loss and morphological inner ear abnormalities. These findings suggest that MIF plays an important role in the inner ear of mice. (c) 2014 IBRO. Published by Elsevier Ltd. All rights reserved.

DOI： 10.1016/j.neuroscience.2014.08.042

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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   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)日本耳科学会  

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

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

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. (C) 2012 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.anl.2012.08.001

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

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

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. (C) 2013 Elsevier Ireland Ltd. All rights reserved.

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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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：PUBLIC LIBRARY SCIENCE  

Mitogen-activated protein kinases (MAP kinases) are intracellular signaling kinases activated by phosphorylation in response to a variety of extracellular stimuli. Mammalian MAP kinase pathways are composed of three major pathways: MEK1 (mitogen-activated protein kinase kinase 1)/ERK 1/2 (extracellular signal-regulated kinases 1/2)/p90 RSK (p90 ribosomal S6 kinase), JNK (c-Jun amino (N)-terminal kinase)/c-Jun, and p38 MAPK pathways. These pathways coordinately mediate physiological processes such as cell survival, protein synthesis, cell proliferation, growth, migration, and apoptosis. The involvement of MAP kinase in noise-induced hearing loss (NIHL) has been implicated in the cochlea; however, it is unknown how expression levels of MAP kinase change after the onset of NIHL and whether they are regulated by transient phosphorylation or protein synthesis. CBA/J mice were exposed to 120-dB octave band noise for 2 h. Auditory brainstem response confirmed a component of temporary threshold shift within 0-24 h and significant permanent threshold shift at 14 days after noise exposure. Levels and localizations of phospho-and total-MEK1/ERK1/2/p90 RSK, JNK/c-Jun, and p38 MAPK were comprehensively analyzed by the Bio-Plex (R) Suspension Array System and immunohistochemistry at 0, 3, 6, 12, 24 and 48 h after noise exposure. The phospho-MEK1/ERK1/2/p90 RSK signaling pathway was activated in the spiral ligament and the sensory and supporting cells of the organ of Corti, with peaks at 3-6 h and independently of regulations of total-MEK1/ERK1/2/p90 RSK. The expression of phospho-JNK and p38 MAPK showed late upregulation in spiral neurons at 48 h, in addition to early upregulations with peaks at 3 h after noise trauma. Phospho-p38 MAPK activation was dependent on upregulation of total-p38 MAPK. At present, comprehensive data on MAP kinase expression provide significant insight into understanding the molecular mechanism of NIHL, and for developing therapeutic models for acute sensorineural hearing loss.

DOI： 10.1371/journal.pone.0058775

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Authorship：Lead author   Language：English   Publisher：NATURE PUBLISHING GROUP  

DOI： 10.1038/jhg.2012.89

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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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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：INFORMA HEALTHCARE  

Conclusions: Quantitative, real-time RT-PCR demonstrated that intratympanic dexamethasone significantly up-regulates the expression of Fkbp5 in cochleae of mice in vivo. The immunohistochemistry results showed fundamentally ubiquitous expression of Fkbp5 in cochlear structures, with relatively strong expression in type 4 fibrocytes and weak signal in the inner hair cells. These data indicate that dexamethasone regulates gene expression at the level of transcription in vivo and that this process is basically ubiquitous in the cochlea. Objectives: To demonstrate that intratympanically applied dexamethasone upregulates Fkbp5 in the cochlea in vivo. Methods: Dexamethasone or control saline were intratympanically applied to adult C57/BL6 mice and dexamethasone-dependent changes in the levels of Fkbp5 expression in the cochlea were analyzed using quantitative real-time RT-PCR. The expression pattern of Fkbp5 in cochlea was investigated by immunohistochemistry in mice that were administered dexamethasone and in controls. Results: Quantitative real-time RT-PCR demonstrated significant increases of Fkbp5 expression levels in cochleae of dexamethasone-treated mice as compared with controls at 12 h after application (244.8 +/- 155.5, n = 5 vs 100.0 +/- 3.0, n = 6, p &lt; 0.01). Immunohistochemistry showed fundamentally ubiquitous expression of Fkbp5 in cochlear structures, with some strongly positive fibrocytes in the spiral ligaments and weak immunoreactivity in the inner hair cells. Distribution of Fkbp5 signaling was not different between the dexamethasone-treated group and controls.

DOI： 10.3109/00016489.2011.619571

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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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Authorship：Corresponding author   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の普及にしたがい、軽度・中等度難聴児も早期に診断され、早期に補聴器を装用し、療育を開始するケースが増加している。しかしながら、現在の障害者自立支援法では、身体障害者には該当しない軽度・中等度難聴者は、補聴器購入に際して公的援助が受けられないため、全額自費で購入するか、補聴器装用を断念せざるを得ないのが現状である。この是正を求め、「岡山県難聴児を支援する会」は、平成21年9月岡山県議会に「軽度および中等度難聴児に対する補聴器購入費用助成に関する陳情書」を提出し、平成22年4月より「岡山県難聴児補聴器交付事業」が開始された。事業導入に至った経緯、概要を報告する。(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=20110831340005&doc_link_id=1390282679989499904&url=https%3A%2F%2Fcir.nii.ac.jp%2Fcrid%2F1390282679989499904&type=CiNii&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00003_3.gif

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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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：TAYLOR & FRANCIS AS  

Conclusions: The microarray analysis identified 39 genes up- or down-regulated by dexamethasone in the cultured tissue of mice cochlea. Of the eight genes most highly affected, several are suggested to have protective effects in the traumatized inner ear (Fkbp5, Glucocorticoid-induced leucine zipper (Gilz), glutathione peroxidase 3) and for others, a plausible mechanism of action can be offered (claudin 10, glutamate-ammonia ligase). The present data may support the use of dexamethasone to treat acute sensorineural hearing loss. It is warrantable to test these results in the in vivo cochlea. Objectives: To identify genes whose expression is markedly up- or down-regulated by dexamethasone in the cochlear tissue. Methods: Murine cochlear tissue was cultured with or without dexamethasone for 48 h in vitro. The gene expression profiles were compared between the dexamethasone-treated and untreated cochlear tissue using a microarray that covers 33 696 transcripts (24 878 genes) of mice and quantitative real-time RT-PCR. Results: The microarray analysis identified 39 genes that are up- or down-regulated by more than twofold in the presence of dexamethasone in the cochlear culture. Genes up- or down-regulated by at least threefold include Fkbp5, Gilz, glutathione peroxidase 3, claudin 10, glutamate-ammonia ligase, proteoglycan 1, integrin beta-like 1, and alpha subunit of glycoprotein hormone.

DOI： 10.3109/00016489.2010.498836

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：OKAYAMA UNIV MED SCHOOL  

This study examined whether pathological findings were present in cochlear vessels for patients with diabetes mellitus. Twenty-six temporal bones from 13 patients with type 1 diabetes mellitus and 40 temporal bones from 20 patients with type 2 diabetes mellitus were examined. Type 2 diabetic temporal bones were divided into 2 groups according to diabetic management (22 temporal bones with insulin therapy, and 18 with oral hypoglycemic drugs). Age-matched normal control temporal bones were also selected. The vessel wall thickness in the cochlear spiral modiolar artery was measured under a light microscope, and the vessel wall ratio (vessel wall thickness/outer diameter of the vessel x 100) was calculated. The vessel wall thickness and vessel wall ratio in type 1 diabetes mellitus were significantly greater than in normal controls. Type 2 diabetic patients with insulin therapy showed significantly greater vessel wall thickness and vessel wall ratios than controls. In type 2 diabetes mellitus, the vessel wall thickness and vessel wall ratio were greater in patients treated with insulin therapy than in those treated with oral hypoglycemic agents. Type 2 diabetic patients with insulin therapy showed an increased vessel wall thickness and vessel wall ratio compared to patients with type 1 diabetes mellitus. In conclusion, the cochlea in patients with diabetes mellitus shows circulatory disturbance compared to age-matched normal controls.

DOI： 10.18926/AMO/41323

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

Development of a gene-delivery method to the inner ear is an essential step for studies of hearing function and gene therapy. Application of liposomes or adenoviral vectors onto the intact round window membrane (RWM) offers the possibility of atraumatic exogenous gene transfer. GJB2 encodes the gap junction protein Connexin26, which plays a crucial role in potassium recycling in the inner ear. The R75W allele of GJB is a well-characterized mutation that causes deafness at the DFNA3 through a dominant-negative mechanism of action. In this study, a plasmid vector, pGJB2(R75W)-eGFP, was lipocomplexed with N-[1-(2,3-Dioleoloxy)propyl]N,N,N-trimethylammonium methylsulfate: cholesterol and applied onto mouse RWM. At 3 days (3 d) post-treatment, immunohistochemistry demonstrated GJB2(R75W)-eGFP transgene expression in the cochlea in: inner and outer pillar cells, outer hair cells, Claudius cells and, in the spiral limbus and ligament. Significant hearing loss was detected by auditory brainstem response testing after 1, 2 and 3 d post-treatment; hearing levels returned to control levels at 5 d post-treatment. These data confirm that GJB2(R75W) induces functional impairment in the mature cochlea through a dominant negative effect, and importantly, that RWM application of exogenous genes is a feasible method to test their impact on hearing. (C) 2007 Elsevier Ireland Ltd and the Japan Neuroscience Society. All rights reserved.

DOI： 10.1016/j.neures.2007.03.006

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：TAYLOR & FRANCIS AS  

Conclusion. Notch signaling pathway may play an important role in the development of the olfactory epithelium (OE). Objectives. To elucidate whether the Notch signaling pathway mediates the developmental processes in the developing OE. Materials and methods. The expression of Notch1 and Hes5 in the developing OE of mice with ages ranging from embryonic day ( E) 11 to postnatal day (PN) 14 was examined. Results. As detected by in situ hybridization, Notch1 was expressed in scattered cells located in the basal portion of the embryonic OE and later in the cell layer adjacent to the basal lamina from E11 to PN14. Hes5 was expressed in scattered cells located in the basal portion of the embryonic OE from E11. However, at the late embryonic stage, the number of Hes5-positive cells decreased and after birth distinct Hes5-positive cells were not observed in the OE.

DOI： 10.1080/00016480500437419

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：OXFORD UNIV PRESS  

Mutations in GJB2 (gap junction protein, beta-2) are the major cause of autosomal recessive non-syndromic hearing loss. A few allele variants of this gene also cause autosomal dominant non-syndromic hearing loss as a dominant-negative consequence of expression of the mutant protein. Allele-specific gene suppression by RNA interference (RNAi) is a potentially attractive strategy to prevent hearing loss caused by this mechanism. In this proof-of-principle study, we identified a potent GJB2-targeting short interfering RNA (siRNA) to post-transcriptionally silence the expression of the R75W allele variant of GJB2 in cultured mammalian cells. In a mouse model, this siRNA duplex selectively suppressed GJB2(R75W) expression by &gt; 70% of control levels, thereby preventing hearing loss. The level of endogenous murine Gjb2 expression was not affected. Our data show that RNAi can be used with specificity and efficiency in vivo to protect against hearing loss caused as a dominant-negative consequence of mutant gene expression.

DOI： 10.1093/hmg/ddi172

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

Tecta, Coch, Eya4 and Strc are mouse orthologs of four human deafness-associated genes. Their expression is markedly restricted to specific cell types in cochleae. Cochleae were dissected on embryonic day 15 and cultured in vitro. Relative messenger RNA abundance of each gene was quantified by RT-PCR and compared in-vivo cochleae of equivalent embryonic age. After 48 h in culture, in-vivo and explant Strc expression levels were equivalent, Eya4 level reduced in explanted tissues, and expression of Tecta and Coch did not show the expected temporal rise. Expression of these genes was detectable even after 96 h. These results suggest that it is feasible to test the expression of inner ear specific genes in explanted cochleae. (c) 2005 Lippincott Williams T Wilkins.

DOI： 10.1097/00001756-200503150-00011

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

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 &gt; 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. (C) 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  

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. (C) 2003 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/S0165-5876(03)00193-9

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

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 month's 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. (C) 2003 Elsevier Science Ireland Ltd. All rights reserved.

DOI： 10.1016/S0165-5876(03)00016-8

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：CELLULAR & MOLECULAR BIOLOGY  

A quite rare case of nasopharyngeal calculus in a woman in her twenties associated with the nasal discharge of pseudomonas infection was reported. As the substance was irregularly large in size, we extracted it partially by piecemeal resection using forceps and also by cracking technique using the holmium ytriium-aluminum-garnet (YAG) laser, under saline irrigation and stereotactic microscopic navigator (SMN) system under endoscopic observation. The substance was firmly fixed to the pharyngeal tonsil bed. The final extract was a small piece of singly folded bandage, which is probably the focal background for calculus formation. In a cross section of calculus specimen removed during surgery, Fourier transform infrared (FT-IR) analysis revealed that a) signal ratio of methylene group (organic substance) to amide I (protein) was 21.6% at the nasal cavity side, gradually decreased toward nasal mucous membrane showing approximate 50%, b) signal ratio of amide I to PO43- (inorganic substance) ranged between 17.7% and 26.7% at the different sites and inside the calculus, the protein content was approximate 115 of the inorganic substance, and c) signal ratio of the methylene group to amide I at the nasal cavity site showed that their contents were almost equal. The quantity of the organic substance was estimated at approximate 1/2 quantity of the protein at both the central part and the part contacted with the mucous membrane. From these results, it seems that throughout the course of calculus growth, both inorganic substance and protein remain almost constant inside the calculus, while organic substance is released from the internal part of the calculus being probably formed at an early stage.

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

TECTA and DFNA5 are the mouse orthologues of the human deafness-associated genes TECTA and DFNA5. To determine how expression of these genes is regulated during development, relative mRNA abundance was examined in mice by nonradioactive RT-PCR. TECTA mRNA was detected on embryonic day 15 (E15), increased to its highest level on postnatal day 3 (P3) and then dramatically decreased by P15. Low levels persisted (adulthood, P45 to 67) with mean mRNA abundance after P15 less than 25% of P3 levels. DFNA5 mRNA expression was constant throughout these time points. These results imply that TECTA is transcribed at a particularly high level during tectorial membrane morphogenesis. In contrast, DFNA5 is present in both the developing and mature cochlea. NeuroReport 12:3223-3226 (C) 2001 Lippincott Williams & Wilkins.

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

A detailed analysis of the differential expression of a nuclear enzyme, DNA topoisomerase (topo) II beta, was performed in the rat hippocampal pyramidal layer. Three-dimensional (3-D) reconstruction from serial sections immunostained with anti-topo II beta antibody showed that the immunoreactivity was apparently weak in the entire CA3 region. Almost all CA1 pyramidal cells showed similar immunoreactivity to that seen in the dentate granular cells, the subicular neurons, and the cerebral neocortical neurons. In addition, immuoblotting analysis in the adult dorsal hippocampus showed that the expression level of topo II beta in the CA3 was significantly lower than that in the CA1 region. The dissociation in the expression level between CA1 and CA3 occurred in postnatal days 4 (P4) through P6. The present finding suggests that the enzyme is possibly involved in activities of the hippocampal pyramidal neurons. (C) 2000 Elsevier Science Ireland Ltd and the Japan Neuroscience Society. All rights reserved.

DOI： 10.1016/S0168-0102(00)00098-5

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：ACADEMIC PRESS INC JNL-COMP SUBSCRIPTIONS  

Amphiphysin, an SH3-domain containing protein concentrated in nerve terminalis, is believed, to be involved in the synaptic vesicle recycling. We have cloned cDNAs of a novel isoform of amphiphysin (amphiphysin II) by exploiting sequence information for homologous ESTs deposited in databases, At least 9 different subtypes of the isoform with 50-60% amino acid identity to the human amphiphysin were identified by a conventional library screening and PCR amplification of cDNA libraries. Each subtype probably represents a splice variant of a single gene transcript, Analysis of mRNA expression in various tissues by RT-PCR showed that the isoform is ubiquitously distributed. The expression spectrum of the isoform subtypes, however, is significantly different in several tissues examined, suggesting that they are involved in the regulation of endocytic processes that are unique to each cell type. (C) 1997 Academic Press.

DOI： 10.1006/bbrc.1997.6927

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

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

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

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DOI： 10.11374/shonijibi.31.299

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Language：Japanese   Publisher：(一社)日本耳鼻咽喉科学会  

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