Experience with the Vibrant Soundbridge RW-Coupler for round window Vibroplasty with tympanosclerosis

Usage of the Vibrant Soundbridge (VSB) with round window (RW)-Coupler placement at the RW has been shown to successfully treat mixed hearing loss. Coupling between the VSB's floating mass transducer (FMT) and the RW membrane is difficult in the case of sclerosis in the RW and drilling down the bony lip until the RW membrane can be seen completely can possibly induce a perilymphatic fistula. A 68-year-old woman who had bilateral mixed hearing loss with sclerosis in the RW due to tympanosclerosis underwent a RW-Vibroplasty with a RW-Coupler. Speech discrimination scores in quiet and noise and functional gain with the VSB with RW-Coupler were better than those using a conventional hearing aid. The results of the present case have shown the feasibility of implanting a VSB with RW-Coupler in patients with mixed hearing loss due to tympanosclerosis.ArticleACTA OTO-LARYNGOLOGICA. 132(6):676-682 (2012)journal articl

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

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

ヒガシ ナンキョク ソウヤ カイガン ノ リクジョウ エンガン ロガンイキ ニ テンザイスル ミコケツ サイリュウ タイシキブツ ノ リュウド ソセイ ノ トクチョウ ト セイイン

宗谷海岸露岩域に点在する未固結細粒堆積物は,従来の研究で氷成堆積物(ティル)や氷河底ティルと記載されてきたが,その成因や堆積環境について検討されたことはなかった.本研究では,未固結細粒堆積物95試料の粒度を測定し,氷河底成岩(底面氷中の岩とロッジメントティル)の粒度組成との比較によって,それらの成因を検討した.その結果,氷河底成岩は粘土から砂までの幅広いサイズの粒子で構成され,砂分と粘土分にモードが現れるバイモーダルな度分布で特徴づけられることが明らかとなった.一方,未固結細粒堆積物の大部分は主に粘土とシルトから成り,多様な粒径度分布を持つ.ただし,礫や隆起海浜堆積物に覆われる未固結細粒堆積物には氷河底成岩と同じ粒度組成を持つものがある.これらのことから,宗谷海岸露岩域の未固結細粒堆積物は,その地域にかつて広く堆積していた氷河底ティル由来の堆積物が再移動した物質を大量に含むと考えられた.Unconsolidated fine sediments (UFS), sporadically deposited in Soya Coast ice-free areas, have often been described as glacial deposits (till) or subglacial till in previous studies, but their genesis and depositional environments have never been discussed so far. In this study, 95 samples of UFS were analyzed for grain-size distribution, and their properties and origins were examined by contrast with the grain-size distribution of primary subglacial-debris (PSD): debris in basal ice and lodgement tills. Primary subglacial-debris is composed of a very broad range of grain size from clay to sand, and exhibit a typically bimodal frequency distribution with peaks in sand and clay parts. In contrast, some of the UFS covered by gravel or raised beach deposits have the same grain-size distribution with PSD, although almost all UFS consist mainly of clay and/or silt and exhibit various frequency distributions. Therefore, it seems reasonable to conclude that the UFS contains abundant reworked materials from the PSD, which were widely deposited on the Soya Coast formerly ice-free areas

TECTA mutations in Japanese with mid-frequency hearing loss affected by zona pellucida domain protein secretion

信州大学博士（医学）・学位論文・平成24年7月3日授与（乙第1146号）・茂木英明TECTA gene encodes alpha-tectorin, the major component of noncollagenous glycoprotein of the tectorial membrane, and has a role in intracochlear sound transmission. The TECTA mutations are one of the most frequent causes of autosomal dominant (AD) hearing loss and genotype-phenotype correlations are associated with mutations of TECTA in exons according to alpha-tectorin domains. In this study, we investigated the prevalence of hearing loss caused by TECTA mutations in Japanese AD hearing loss families, and confirmed genotype-phenotype correlation, as well as the intracellular localization of missense mutations in the alpha-tectorin domain. TECTA mutations were detected in 2.9% (4/139) of our Japanese AD hearing loss families, with the prevalence in moderate hearing loss being 7.7% (4/52), and all patients showed typical genotype-phenotype correlations as previously described. The present in vitro study showed differences of localization patterns between wild type and mutants, and suggested that each missense mutation may lead to a lack of assembly of secretion, and may reduce the incorporation of alpha-tectorin into the tectorial membrane. Journal of Human Genetics (2012) 57, 587-592; doi:10.1038/jhg.2012.73; published online 21 June 2012ArticleJOURNAL OF HUMAN GENETICS. 57(9):587-592 (2012)journal articl

Patients with CDH23 mutations and the 1555A > G mitochondrial mutation are good candidates for electric acoustic stimulation (EAS)

Conclusions: CDH23 mutations and the 1555A>G mitochondrial mutation were identified among our series of electric acoustic stimulation (EAS) patients, confirming that these genes were important in hearing loss with involvement of high frequency. Successful hearing preservation as well as good outcomes from EAS indicated that patients with this combination of mutations are good candidates for EAS. Objectives: Screening for gene mutations that possibly cause hearing loss involving high frequency was performed to identify the responsible genes in patients with EAS. In addition to a review of the genetic background of the patients with residual hearing loss, the benefit of EAS for patients with particular gene mutations was evaluated. Methods: Eighteen patients (15 late-onset, 3 early-onset) with residual hearing who had received EAS were included in this study. Genetic analysis was performed to identify GJB2, CDH23, SLC26A4, and the 1555 mitochondrial mutations. Results: Three early-onset patients had CDH23 mutations. One late-onset patient had the 1555 A>G mitochondrial mutation.ArticleACTA OTO-LARYNGOLOGICA. 132(4):377-384 (2012)journal articl

Achievement of hearing preservation in the presence of an electrode covering the residual hearing region

Conclusions: With full insertion with a long electrode, hearing preservation can be achieved even in the presence of a long electrode covering the residual hearing region. Objectives: Advances in developing new atraumatic concepts of electrode design as well as surgical technique have enabled hearing preservation after cochlear implantation surgery, and EAS (electric acoustic stimulation) accompanied with hearing preservation is a new trend for patients with residual hearing at the lower frequencies. However, full insertion with a long/medium electrode and hearing preservation is still a challenging field that calls for discussion. Method: In this study, round window insertion, an atraumatic electrode, and dexamethasone administration were used and atraumaticity (hearing preservation and conservation of vestibular function) was evaluated with full insertion of the electrode. Results: Postoperative evaluation after full insertion of the electrodes showed that hearing at low frequencies was well preserved in all five cases. Combined postoperative imaging with the referential tonotopic map confirmed achievement of full insertion and indicated the corresponding frequencies and the depth of the electrode. Achievement of atraumaticity of round window insertion in the present cases was confirmed from the viewpoint of the minimal drilling time as well as the preserved vestibular function.ArticleACTA OTO-LARYNGOLOGICA. 131(4):405-412 (2011)journal articl

Anti-viral actions and viral dynamics in the early phase of three different regimens of interferon treatment for chronic hepatitis C: differences between the twice-daily administration of interferon-beta treatment and the combination therapy with interferon-alpha plus ribavirin.

To improve the efficacy of interferon (IFN) treatment for chronic hepatitis C, we have proposed the twice-daily administration of IFN-beta as a promising induction therapy. In this study, we demonstrated differences between the clearance of circulating HCV-RNA and the induction of anti-viral actions during the first 2 weeks of treatment. Nine patients with a high viral load and genotype 1b were randomly assigned to 3 groups: group A received 3MU of IFN-beta twice a day at intervals of 5 and 19 h; group B received 3MU of IFN-beta twice a day at intervals of 10 and 14 h; group C received 6MU of IFN-alpha once a day with ribavirin. The expression of OAS2, PKR, and MxA in peripheral blood mononuclear cells (PBMCs) were quantified by real-time polymerase chain reaction method. The viral clearance showed a bi-phasic pattern, and those in the second phase of groups A and B were significantly steeper than that of group C. The peak level of OAS2 during the first phase was correlated with the first phase decay. The MxA expression tended to be higher in group A and B than in group C. The expression of these 3 proteins tended to decrease at day 6 in group C, but increase in groups A and B. These might make differences in the viral decay during the second phase</p