
宇佐美 真一 (ウサミ シンイチ)
医学部 医学科 人工聴覚器学(寄附講座)
特任教授(寄附講座)
研究者情報
研究活動情報
論文
- The Prevalence and Clinical Characteristics of MYO3A-Associated Hearing Loss in 15,684 Hearing Loss Patients.
Maekawa K, Nishio SY, Miyazaki H, Ohta Y, Oishi N, Kasai M, Yamamoto A, Okami M, Wasano K, Sakai A, Usami SI.
Genes (Basel). , 16(1), 92, 2025年 - Novel OTOG Variants and Clinical Features of Hearing Loss in a Large Japanese Cohort.
Arai Y, Nishio SY, Goto S, Kobayashi Y, Honkura Y, Ganaha A, Ishikawa K, Oka SI, Futagawa H, Okami M, Takada F, Nagai K, Esaki T, Okano T, Ohta Y, Masuda S, Egusa K, Teraoka M, Sugahara K, Usami SI.
Genes (Basel). , 16(1), 60, 2025年 - The Heterozygous p.A684V Variant in the WFS1 Gene Is a Mutational Hotspot Causing a Severe Hearing Loss Phenotype.
Otsuka S, Morimoto C, Nishio SY, Morita S, Kikuchi D, Takahashi M, Kumakawa K, Arai Y, Sano H, Yoshimura H, Yamamoto N, Kondo S, Hasegawa M, Nishi T, Kitahara T, Usami SI.
Genes (Basel). , 16(1), 57, 2025年 - Auditory Neuropathy Caused by a Structural Variation in the OTOF Gene, Identified Using Oxford Nanopore Adaptive Sampling.
Kumai T, Nishio SY, Moteki H, Katada A, Usami SI.
Genes (Basel). , 16(2), 116, 2025年 - Prevalence and Clinical Characteristics of OTOGL-Associated Hearing Loss Identified in a Cohort of 7065 Japanese Patients with Hearing Loss.
Maekawa K, Nishio SY, Ishikawa K, Takahashi M, Kumakawa K, Okami M, Yoshimura H, Nakayama J, Teraoka M, Usami SI.
Genes (Basel). , 16(2), 123, 2025年 - ClinGen recuration of hearing loss associated-genes demonstrates significant changes in gene-disease validity over time.
Tshering KC, DiStefano MT, Oza AM, Ajuyah P, Webb R, Edoh E, Broeren E, Ratliff J, Gitau V, Paris K, Aburyyan A, Alexander J, Albano V, Bai D, Booth KT, Buonfiglio PI, Charfeddine C, Dalamón V, Del Castillo I, Moreno-Pelayo MA, Duzkale H, Dorshorst B, Faridi R, Kenna M, Lewis MA, Luo M, Lu Y, Mkaouar R, Matsunaga T, Nara K, Pandya A, Redfield S, Roux I, Schimmenti LA, Schrauwen I, Shaaban S, Shen J, Vona B, Smith RJ, Rehm HL, Azaiez H, Abou Tayoun AN, Amr SS; ClinGen Hearing Loss Clinical Domain Working Group(Usami SI, Nishio SY).
Genet Med., 27(5), 101392, 2025年 - 難聴の遺伝学的検査と社会実装
宇佐美真一
Audiology Japan, 67, 43-53, 2024年 - Validation of RNA Extraction Methods and Suitable Reference Genes for Gene Expression Studies in Developing Fetal Human Inner Ear Tissue.
Steinacher C, Rieder D, Turner JE, Solanky N, Nishio SY, Usami SI, Hausott B, Schrott-Fischer A, Dudas J.
Int J Mol Sci., 25(5), 2907, 2024年 - The prevalence and clinical features of MYO7A-related hearing loss including DFNA11, DFNB2 and USH1B.
Watanabe K, Nishio SY, Usami SI; Deafness Gene Study Consortium.
Sci Rep. , 14(1), 8326, 2024年 - Epidemiology, aetiology and diagnosis of congenital hearing loss via hearing screening of 153 913 newborns.
Yoshimura H, Okubo T, Shinagawa J, Nishio SY, Takumi Y, Usami SI.
Int J Epidemiol., 53(3), dyae052, 2024年 - Detailed Clinical Features of PTPRQ-Associated Hearing Loss Identified in a Large Japanese Hearing Loss Cohort.
Sakuma N, Nishio SY, Goto SI, Honkura Y, Oda K, Takeda H, Kobayashi M, Kumakawa K, Iwasaki S, Takahashi M, Ito T, Arai Y, Isono Y, Obara N, Matsunobu T, Okubo K, Usami SI.
Genes., 15(4), 489, 2024年 - Comprehensive Genetic Evaluation in Patients with Special Reference to Late-Onset Sensorineural Hearing Loss.
Miyanohara I, Ohori J, Tabuchi M, Nishio SY, Yamashita M, Usami SI.
Genes., 15(5), 571, 2024年 - Comparison of vestibular function in hereditary hearing loss patients with GJB2, CDH23, and SLC26A4 variants.
Tsukada K, Nishio SY, Takumi Y, Usami SI.
Sci Rep. , 14(1), 10596, 2024年 - The natural history and genotype-phenotype correlations of TMPRSS3 hearing loss: an international, multi-center, cohort analysis.
Colbert BM, Lanting C, Smeal M, Blanton S, Dykxhoorn DM, Tang PC, Getchell RL, Velde H, Fehrmann M, Thorpe R, Chapagain P, Elkhaligy H, Kremer H, Yntema H, Haer-Wigman L, Redfield S, Sun T, Bruijn S, Plomp A, Goderie T, van de Kamp J, Free RH, Wassink-Ruiter JK, Widdershoven J, Vanhoutte E, Rotteveel L, Kriek M, van Dooren M, Hoefsloot L, de Gier HHW; DOOFNL Consortium; Schaefer A, Kolbe D, Azaiez H, Rabie G, Aburayyan A, Kawas M, Kanaan M, Holder J, Usami SI, Chen Z, Dai P, Holt J, Nelson R, Choi BY, Shearer E, Smith RJH, Pennings R, Liu XZ.
Hum Genet., 143(5), 721-734, 2024年 - Clinical practice guidelines for the diagnosis and management of acute sensorineural hearing loss.
Kitoh R, Nishio SY, Sato H, Ikezono T, Morita SY,Wada T, Usami SI on behalf of Research Group on Intractable Hearing
Auris Nasus Larynx, 51, 811-821, 2024年 - Generation of an induced pluripotent stem cell line from a late-onset, progressive high frequency hearing loss patient due to mutation in CDH23
Arai D, Takahashi-Shibata M, Ukaji T, Tsutsumi H, Tajima S, Nishio SY, Ishikawa KI, Akamatsu W, Matsumoto F, Ikeda K, Usami SI, Kamiya K.
Stem Cell Res., 79, 103471, 2024年 - HEARRING group genetic marker study: genetic background of CI patients.
Usami SI, Nishio SY, Gavilán J, Van de Heyning P, Mertens G, Karltorp E, Skarżyński H, Hagr A, Manoj M, Staecker H, Zernotti ME, Rajan GP, Müller J, Simon F, Anderson I.
Acta Otolaryngol., 144(10), 542-551, 2024年 - Testing for genetic and viral etiologies in congenital hearing loss based on a survey of cochlear implant centers: proposed HEARRING group consensus and future directions.
Usami SI, Nishio SY, Gavilán J, Acharya A, Hagr A, Lassaletta L, Li Y, Maheshwari SS, Kameswaran M, Parzefall T, Raine C, Rak K, Selleck AM, Staecker H, Stöver T, Sugarova S, Topsakal V, Van Rompaey V, Mertens G, Volkenstein S, Völter C, Shehata-Dieler W, Zernotti ME, Van de Heyning P.
Acta Otolaryngol., 144(11-12), 602-609, 2024年 - Phylogeography of the Hungarian Glider( Neptis rivularis, Lepidoptera Nymphalidae): Notes on Morphological Form in China.
Nakatani T, Fukuda H, Minotani N, Nishio SY, Usami SI.
Butterfly Science, 30, 92-103, 2024年 - Geographical Variations of the Hungarian Glider( Neptis rivularis, Lepidoptera Nymphalidae): Notes on Two Morphological Types of Hungarian Glider in China.
Fukuda H, Minotani N, Nakatani T, Nishio SY, Usami SI.
Butterfly Science, 30, 83-91, 2024年 - Enhanced Cochlear Coverage and Hearing Preservation in High-Frequency Hearing Loss via Electric Acoustic Stimulation with Longer Electrode.
Yoshimura H, Takumi Y, Nishio SY, Usami SI.
J Vis Exp., 212, e66565, 2024年 - Expression of Neurotrophins and Its Receptors During Fetal Development in the Human Cochlea.
Steinacher C, Nishio SY, Usami SI, Dudas J, Rieder D, Rask-Andersen H, Crespo B, Moreno N, Konschake M, Seifarth C, Glueckert R.
Int J Mol Sci., 25(23), 13007, 2024年 - Genetic structure of the endangered butterfly Shijimiaeoides divinus (Lepidoptera: Lycaenidae) in Japan.
Isaka Y, Nishio SY, Usami SI.
New Entomol., 73(1-4), 35-46, 2024年 - 遺伝性難聴 遺伝性難聴はごくありふれた疾患である
宇佐美真一
耳鼻咽喉科・頭頸部外科, 96, 834-837, 2024年 - 2023 年夏の北海道渡島半島におけるウラギンヒョウモン類 2 種と1 系統
藤田創斗、西尾信哉、対馬誠、鈴木修一、前田俊信、朝日純一、伊藤建夫、宇佐美真一
Butterfly Science, 30, 76-78, 2024年 - 高山蝶タカネヒカゲの分子系統:八ヶ岳亜種の保全に関連して
宇佐美真一、西尾信哉
昆虫と自然, 59(14), 13-17, 2024年 - 難聴の全ゲノム解析
西尾信哉、宇佐美真一
医学のあゆみ, 285(1), 59-66, 2023年 - 難聴の遺伝学的検査
宇佐美真一
遺伝子医学 , 13(2), 69-78, 2023年 - 個別診療分野における遺伝学的診断の進歩 耳鼻科領域-難聴
宇佐美真一
日本医師会雑誌, 152, s160-s164, 2023年 - 残存聴力活用型人工内耳(EAS)のマッピングにおける低音域の刺激方法の検討
權田綾子、吉村豪兼、西尾信哉、宇佐美真一、工 穣
Audiology Japan, 66, 123-128, 2023年 - 小耳症耳介形成前の人工中耳植え込み術-皮膚切開のデザインおよび側頭筋弁作成法について-
高橋優宏、岩崎聡、吉村豪兼、古舘左起子、岡晋一郎、宇佐美真一
耳鼻咽喉科臨床, 116(11), 1077-1082, 2023年 - 人工内耳患者における遺伝的背景に関する検討
岡晋一郎、高橋優宏、古舘佐起子、小山田匠吾、西尾信哉、宇佐美真一、岩崎聡
耳鼻咽喉科臨床, 116(11), 1065-1070, 2023年 - A plea for systematic literature analysis and conclusive study design, comment on: "Systematic review of magnetic resonance imaging for diagnosis of Meniere disease"
Gürkov R, Barath K, de Foer B, Fukushima M, Gluth M, Hornibrook J, Perez-Fernandez N, Pyykkö I, Sone M, Usami SI, Wang W, Zou J, Naganawa S.
J Vestib Res., 33(2), 151-157, 2023年 - The relationship between preoperative factors and the pattern of longitudinal improvement in speech perception following cochlear implantation.
Shinagawa J, Yoshimura H, Nishio SY, Takumi Y, Usami SI.
Acta Otolaryngol., 143(5), 402-407, 2023年 - Generation and characterization of a human iPSC line (JUFMDOi007-A) from a patient with Usher syndrome due to mutation in USH2A.
Ukaji T, Takahashi-Shibata M, Arai D, Tsutsumi H, Tajima S, Akamatsu W, Matsumoto F, Ikeda K, Usami SI, Kamiya K.
Stem Cell Res., 69, 103100, 2023年 - Otological Features of Patients with Musculocontractural Ehlers–Danlos Syndrome Caused by Pathogenic Variants in CHST14 (mcEDS-CHST14).
Kawakita M, Iwasaki S, Moteki H, Nishio SY, Kosho T, Usami SI.
Genes., 14(7), 1350, 2023年 - Determining optimal cochlear implant electrode array with OTOPLAN.
Yoshimura H,Watanabe K, Nishio SY, Takumi Y, Usami SI.
Acta Otolaryngol., 143(9), 748-752, 2023年 - Identification of source populations for reintroduction in extinct populations based on genome‑wide SNPs and mtDNA sequence:a case study of the endangered subalpine grassland butterfly Aporia hippia (Lepidoptera; Pieridae) in Japan.
Nakahama N, Hanaoka T, Itoh T, Kishimoto T, Ohwaki A, Matsuo A, Kitahara M, Usami SI, Suyama Y, Suka T.
Journal of Insect Conservation, 26, 121-130, 2022年 - Frequency of the STRC ‑CATSPER2 deletion in STRC ‑associated hearing loss patients.
Nishio SY, Usami SI.
Sci Rep., 12(1), 634, 2022年 - Variants in CDH23 cause a broad spectrum of hearing loss:from non‑syndromic to syndromic hearing loss as well as from congenital to age‑related hearing loss.
Usami SI, Isaka Y, Miyagawa M, Nishio SY.
Hum Genet., 41(3_4), 903-914, 2022年 - Successful cochlear implantation in a patient with Epstein syndrome during long-term follow-up.
Mori A, Takeda H, Kobayashi M, Misawa T, Watanabe R, Abe S, Kumakawa K, Nishio SY, Usami SI, Yamasoba T.
AURIS NASUS LARYNX, 49, 308-312, 2022年 - Effects of salicylate derivatives on localization of p.H723R allele product of SLC26A4 .
Murakoshi M, Koike Y, Koyama S, Usami SI, Kaimiya K, Ikeda K, Haga Y, Tsumoto K, Nakamura H, Hirasawa N, Ishihara K, Wada H.
AURIS NASUS LARYNX, 49(6), 928-937, 2022年 - Suitable Electrode Choice for Robotic-Assisted Cochlear Implant Surgery: A Systematic Literature Review of Manual Electrode Insertion Adverse Events.
Van de Heyning P, Roland P, Lassaletta L, Agrawal S, Atlas M, Baumgartner WD, Brown K, Caversaccio M, Dazert S, Gstöttner W, Hagen R, Hagr A, Jablonski G.E., Kameswaran M, Kuzovkov V, Leinung M, Yongxin Li, Loth A, Magel A, Mlynski R, Mueller J, Parnes L, Radeloff A, Raine C, Rajan G, Schmutzhard J, Skarzynski H, Skarzynski PH, Sprinzl G, Staecker H, Stover T, Tavora-Viera D, Topsakal V, Usami SI, Van Rompaey V, Weiss N.M., Wimmer W, Zernotti M, Gavilan J.
Front Surg., 9, 823219, 2022年 - Genetic background in late-onset sensorineural hearing loss patients
Uehara N, Fujita T, Yamashita D, Yokoi J, Katsunuma S, Kakigi A, Nishio SY, Nibu KI, Usami SI.
J Hum Genet., 67(4), 223-230, 2022年 - Speech perception in noise in patients with idiopathic sudden hearing loss.
Kitoh R, Nishio SY, Usami SI.
Acta Otolaryngol., 142(3_4), 302-307, 2022年 - Prevalence and clinical features of autosomal dominant and recessive TMC1-associated hearing loss.
Nishio SY, Usami SI.
Hum Genet., 141(3_4), 929-937, 2022年 - The genetic etiology of hearing loss in Japan revealed by the social health insurance‑based genetic testing of 10K patients.
Usami SI, Nishio SY.
Hum Genet., 141(3_4), 665-681, 2022年 - Detailed clinical features and genotype-phenotype correlation in an OTOF-related hearing loss cohort in Japan.
Iwasa YI, Nishio SY, Yoshimura H, Sugaya A, Kataoka Y, Maeda Y, Kanda Y, Nagai K, Naito Y, Yamazaki H, Ikezono T, Matsuda H, Nakai M, Tona R, Sakurai Y, Motegi R, Takeda H, Kobayashi M, Kihara C, Ishino T, Morita SY, Iwasaki S, Takahashi M, Furutate S, Oka SI, Kubota T, Arai Y, Kobayashi Y, Kikuchi D, Shintani T, Ogasawara N, Honkura Y, Izumi S, Hyogo M, Ninoyu Y, SuematsuM, Nakayama J, Tsuchihashi N, Okami M, Sakata H, Yoshihashi H, Kobayashi T, kumakawa K, Yoshida T, Esaki T, Usami SI.
Hum Genet., 141(3_4), 865-875, 2022年 - Geographical Variation of Japanese Alpine Butterfly Oeneis melissa daisetsuzana( Lepidoptera, Nymphalidae, Satyrinae)from Hokkaido, Northern Japan.
Nakatani T, Yagimoto S, Nishio SY, Usami SI.
Butterfly Science, 23, 5-13, 2022年 - Human deafness-associated variants alter the dynamics of key molecules in hair cell stereocilia F-actin cores.
Miyoshi T, Belyantseva IA, Kitajiri SI, Miyajima H, Nishio SY, Usami SI, Kim BJ, Choi BY, Omori K, Shroff H, Friedman TB.
Hum Genet., 141(3_4), 363-382, 2022年 - Etiology of hearing loss affects auditory skill development and vocabulary development in pediatric cochlear implantation cases.
Nishio SY, Moteki H, Miyagawa M, Yamasoba T, Kashio A, Iwasaki S, Takahashi M, Naito Y, Fujiwara K, Sugaya A, Takahashi H,Kitaoka K,Usami SI.
Acta Otolaryngol., 142(3_4), 308-315, 2022年 - A 14-year nationwide epidemiological analysis of delayed endolymphatic hydrops in Japan.
Ito S, Takakura H, Akaogi K, Shojaku H, Takeda N, Suzuki M, Watanabe Y, Aoki M, Doi K, Ikezono T, Kakigi A, Kitahara T, Koizuka I, Murofushi T, Naganuma H, Omori K, Takahashi K, Takumida M, Usami SI. Yamashita H.
Acta Otolaryngol., 142(7_8), 568-574, 2022年 - Systematic Literature Review of Hearing Preservation Rates in Cochlear Implantation Associated With Medium- and Longer-Length Flexible Lateral Wall Electrode Arrays.
Van de Heyning PH, Dazert S, Gavilan J, Lassaletta L, Lorens A, Rajan GP, Skarzynski H, Skarzynski PH, Tavora-Vieira D, Topsakal V, Usami SI, Van Rompaey V, Weiss NM, Polak M.
Front Surg., 9, 893839, 2022年 - Minimally Traumatic Cochlear Implant Surgery: Expert Opinion in 2010 and 2020.
Topsakal V, Agrawal S, Atlas M, Baumgartner WD, Brown K, Bruce IA, Dazert S, Hagen R, Lassaletta L, Mlynski R, Raine CH, Rajan GP, Schmutzhard J, Sprinzl GM, Staecker H, Usami SI, Van Rompaey V, Zernotti M, Heyning PV.
J Pers Med., 120(10), 1551, 2022年 - Estimated number and prevalence of patients with delayed endolymphatic hydrops in Japan: a nationwide survey.
Ito S, Takakura H, Akaogi K, Shojaku H, Kitahara T, Nishio SY, Usami SI. Intractable Hearing Disorder Consortium.
Acta Otolaryngol., 142(9_12), 675-678, 2022年 - 人工内耳:適応基準改定と最適なデバイス選択
宇佐美真一
日本耳鼻咽喉科頭頸部外科学会会報, 125, 12-17, 2022年 - 難聴遺伝子の各タイプ別人工内耳術後の聴覚・言語発達の比較
宇佐美真一
JOHNS, 38(2), 187-191, 2022年 - 遺伝性難聴 解析技術の進歩と臨床応用
宇佐美真一
耳鼻咽喉科・頭頸部外科, 94(4), 370-382, 2022年 - Zona pellucida ドメインのTECTA遺伝子変異により発症した非症候群性常染色体優性遺伝性難聴家系における聴力像の解析:特徴的な皿型オージオグラムの加齢性変化
中西啓、喜夛淳哉、西尾信哉、宇佐美真一、三澤清
AUDIOLOGY JAPAN, 65, 145-151, 2022年 - 遺伝学的検査の用語を理解する
西尾信哉、宇佐美真一
日本耳鼻咽喉科頭頸部外科学会会報, 94, 1002-1008, 2022年 - Vestibular Preservation After Cochlear Implantation Using the Round Window Approach
Tsukada K, Usami SI.
Front Neurol, 12, 656592, 2021年 - Two-phase survey on the frequency of use and safety of MRI for hearing implant recipients
van de Heyning P, Mertens G, Topsakal V, de Brito R, Wimmer W, Caversaccio MD, Dazert S, Volkenstein S, Zernotti M, Parnes LS, Staecker H, Bruce IA, Rajan G, Atlas M, Friedland P, Skarzynski PH, Sugarova S, Kuzovkov V, Hagr A, Mlynski R, Schmutzhard J, Usami SI, Lassaletta L, Gavilán J, Godey B, Raine CH, Hagen R, Sprinzl GM, Brown K, Baumgartner WD, Karltorp E.
Eur Arch Otorhinolaryngol, 2021年 - Development and validation of an iPad-based Japanese language monosyllable speech perception test (iCI2004 monosyllable)
Nishio SY, Tono T, Iwaki T, Moteki H, Suzuki K, Tsushima Y, Kashio A, Akamatsu Y, Sato H, Yaegashi K, Takeda H, Kumagai F, Nakashima T, Matsuda Y, Hato N, Dairoku T, Shiroma M, Kawai R, Usami SI.
Acta Otolaryngol, 141(3), 267-272, 2021年 - Is the Conductive Hearing Loss in NOG-Related Symphalangism Spectrum Disorder Congenital?
Nakashima T, Ganaha A, Tsumagari S, Nakamura T, Yamada Y, Nakamura E, Usami SI, Tono T.
ORL J Otorhinolaryngol Relat Spec, 83(3), 196-202, 2021年 - Improvement of a Rapid and Highly Sensitive Method for the Diagnosis of the Mitochondrial m.1555A>G Mutation Based on a Single-Stranded Tag Hybridization Chromatographic Printed-Array Strip
Isaka Y, Nishio SY, Hishinuma E, Hiratsuka M, Usami SI.
Genet Test Mol Biomarkers, 25(1), 79-83, 2021年 - Cochlear implantation in a patient with a POU4F3 mutation.
Miyake K, Shirai K, Nishiyama N, Kawaguchi S, Ohta Y, Kawano A, Usami SI, Kitano T, Tsukahara K.
Clin Case Rep., 9(1), 298-303, 2021年 - Phylogeny and biogeography of arctic-alpine butterflies of the genus Oeneis (Nymphalidae: Satyrinae)
Usami SI, Isaka Y, Nisho SY, Nakatani T, Itoh T.
Entomological Science, 1-13, 2021年 - Identification of a Novel Copy Number Variation of EYA4 Causing Autosomal Dominant Non-Syndromic Hearing Loss.
Ishino T, Ogawa Y, Sonoyama T, Taruya T, Kono T, Hamamoto T, Ueda T, Ishino T, Ogawa Y, Sonoyama T, Taruya T, Kono T, Hamamoto T, Ueda T, Takeno S, Moteki H, Nishio SY, Usami SI, Nagano Y, Yoshimura A, Yoshikawa K, Kato M, Ichimoto M, Watanabe
Otol Neurotol., 42(7), e866-e874, 2021年 - A novel case of concurrent occurrence of demyelinating-polyneuropathy-causing PMP22 duplication and SOX10 gene mutation producing severe hypertrophic neuropathy.
Matsuda N, Ootsuki K, Kobayashi S, Nemoto A, Kubo H, Usami SI, Kanai K.
BMC Neurol., 21(1), 243, 2021年 - Human deafness-associated variants alter the dynamics of key molecules in hair cell stereocilia F-actin cores.
Miyoshi T, Belyantseva IA, Kitajiri SI, Miyajima H, Nishio SY, Usami SI, Kim BJ, Choi BY, Omori K, Shroff H, Friedman TB.
Hum Genet., 141((3-4)), 363-382, 2021年 - Vibrant soundbridge implantation prior to auricular reconstruction with unilateral microtia-atresia.
Yoshimura H, Takahashi M, Iwasaki S, Shinagawa J, Takashimizu I, Nagai F, Yotsuyanagi T, Usami SI.
Clin Case Rep., 9(8), e04408, 2021年 - A nationwide epidemiologic, clinical, genetic study of Usher syndrome in Japan.
Yoshimura H, Nishio SY, Isaka Y, Kurokawa T, Usami SI, Interactable Hearing Disorder Consortium.
Acta Otolaryngol., 141(9), 841-846, 2021年 - Milestones toward cochlear gene therapy for patients with hereditary hearing loss.
Yoshimura H, Nishio SY, Usami SI.
LARYNGOSCOPE INVESTIGATIVE OTOLARYNGOLOGY, 6(5), 958-967, 2021年 - Successful cochlear implantation in a patient with Epstein syndrome during long-term follow-up.
Mori A, Takeda H, Kobayashi M, Misawa T, Watanabe R, Abe S, Kumakawa K, Nishio SY, Usami SI, Yamasoba T.
AURIS NASUS LARYNX, 47, 905-908, 2021年 - Vestibular nerve deficiency and vestibular function in children with unilateral hearing loss caused by cochlear nerve deficiency.
Tsukada K, Usami SI.
Acta Otolaryngol., 141(9), 835-840, 2021年 - Prevalence and Clinical Characteristics of Hearing Loss Caused by MYH14 Variants.
Hiramatsu K, Nishio SY, Kitajiri SI, Kitano T, Moteki H, Usami SI, On Behalf Of The Deafness Gene Study Consortium.
Genes(Basel)., 12(10), 1623, 2021年 - Frequency and natural course of congenital cytomegalovirus-associated hearing loss in children.
Kasuga M, Yoshimura H, Shinagawa J, Nishio SY, Takumi Y, Usami SI.
Acta Otolaryngol., 141(12), 1038-1043, 2021年 - Cochlear implantation in a patient with a POU4F3 mutation
Miyake K, Shirai K, Nishiyama N, Kawaguchi S, Ohta Y, Kawano A, Usami SI, Kitano T, Tsukahara K.
Clin Case Rep, 9(1), 298-303, 2020年 - Detailed MR imaging assessment of endolymphatic hydrops in patients with SLC26A4 mutations
Tsukada K, Usami SI.
AURIS NASUS LARYNX, 47(6), 958-964, 2020年 - Congenital Membranous Stapes Footplate Producing Episodic Pressure-Induced Perilymphatic Fistula Symptoms
Matsuda H, Tanzawa Y, Sekine T, Matsumura T, Saito S, Shindo S, Usami SI, Kase Y, Itoh A, Ikezono T.
Front Neurol, 11, 585747, 2020年 - The use of a MITO-Porter to deliver exogenous therapeutic RNA to a mitochondrial disease's cell with a A1555G mutation in the mitochondrial 12S rRNA gene results in an increase in mitochondrial respiratory activity
Yamada Y, Maruyama M, Kita T, Usami SI, Kitajiri SI, Harashima H.
Mitochondrion, 55, 134-144, 2020年 - Correction to: Prevalence of the mitochondrial 1555 A>G and 1494 C>T mutations in a community-dwelling population in Japan
Maeda Y, Sasaki A, Kasai S, Goto S, Nishio SY, Sawada K, Tokuda I, Itoh K, Usami SI, Matsubara A.
Hum Genome Var, 7, 36, 2020年 - A comparative analysis of genetic hearing loss phenotypes in European/American and Japanese populations
Walls WD, Moteki H, Thomas TR, Nishio SY, Yoshimura H, Iwasa Y, Frees KL, Nishimura CJ, Azaiez H, Booth KT, Marini RJ, Kolbe DL, Weaver AM, Schaefer AM, Wang K, Braun TA, Usami SI, Barr-Gillespie PG, Richardson GP, Smith RJ, Casavant TL.
Hum Genet, 139(10), 1315-1323, 2020年 - Prevalence of the mitochondrial 1555 A>G and 1494 C>T mutations in a community-dwelling population in Japan
Maeda Y, Sasaki A, Kasai S, Goto S, Nishio SY, Sawada K, Tokuda I, Itoh K, Usami SI, Matsubara A.
Hum Genome Var, 7, 27, 2020年 - Electric-acoustic stimulation with longer electrodes for potential deterioration in low-frequency hearing
Yoshimura H, Moteki H, Nishio SY, Usami SI.
Acta Otolaryngol, 140(8), 632-638, 2020年 - The reliability of hearing implants: report on the type and incidence of cochlear implant failures
Van de Heyning P, Atlas M, Baumgartner WD, Caversaccio M, Gavilan J, Godey B, Gstöttner W, Hagen R, Yongxin L, Karltorp E, Kameswaran M, Kuzovkov V, Lassaletta L, Manoj M, Parnes L, Pillsbury H, Raine C, Rajan G, Schmutzhard J, Skarzynski H, Staecker H, Usami SI, Zernotti M.
Cochlear Implants Int, 21(4), 228-237, 2020年 - Genetic testing has the potential to impact hearing preservation following cochlear implantation
Yoshimura H, Moteki H, Nishio SY, Miyajima H, Miyagawa M, Usami SI.
Acta Otolaryngol, 140(6), 438-444, 2020年 - A phase I/IIa double blind single institute trial of low dose sirolimus for Pendred syndrome/DFNB4
Fujioka M, Akiyama T, Hosoya M, Kikuchi K, Fujiki Y, Saito Y, Yoshihama K, Ozawa H, Tsukada K, Nishio SY, Usami SI, Matsunaga T, Hasegawa T, Sato Y, Ogawa K.
Medicine (Baltimore), 99(19), e19763, 2020年 - Novel ACTG1 mutations in patients identified by massively parallel DNA sequencing cause progressive hearing loss
Miyajima H, Moteki H, Day T, Nishio SY, Murata T, Ikezono T, Takeda H, Abe S, Iwasaki S, Takahashi M, Naito Y, Yamazaki H, Kanda Y, Kitajiri SI, Usami SI.
Sci Rep, 10(1), 7056, 2020年 - The availability of an adhesive bone conduction hearing device: a preliminary report of a single-center experience
Moteki H, Kitoh R, Usami SI.
Acta Otolaryngol, 140(4), 319-326, 2020年 - Digenic inheritance of mutations in EPHA2 and SLC26A4 in Pendred syndrome
Li M, Nishio SY, Naruse C, Riddell M, Sapski S, Katsuno T, Hikita T, Mizapourshafiyi F, Smith FM, Cooper LT, Lee MG, Asano M, Boettger T, Krueger M, Wietelmann A, Graumann J, Day BW, Boyd AW, Offermanns S, Kitajiri SI, Usami SI, Nakayama M.
Nat Commun, 11(1), 1343, 2020年 - Clinical Characteristics and In Vitro Analysis of MYO6 Variants Causing Late-Onset Progressive Hearing Loss
Oka SI, Day TF, Nishio SY, Moteki H, Miyagawa M, Morita S, Izumi S, Ikezono T, Abe S, Nakayama J, Hyogo M, Okamoto N, Uehara N, Oshikawa C, Kitajiri SI, Usami SI.
Genes (Basel), 11(3), 273, 2020年 - Cochlear Implantation From the Perspective of Genetic Background
Usami SI, Nishio SY, Moteki H, Miyagawa M, Yoshimura H.
Anat Rec (Hoboken), 303(3), 563-593, 2020年 - Haplotype Analysis of GJB2 Mutations: Founder Effect or Mutational Hot Spot?
Shinagawa J, Moteki H, Nishio SY, Noguchi Y, Usami SI.
Genes (Basel), 11(3), 250, 2020年 - Prevalence and clinical features of hearing loss caused by EYA4 variants
Shinagawa J, Moteki H, Nishio SY, Ohyama K, Otsuki K, Iwasaki S, Masuda S, Oshikawa C, Ohta Y, Arai Y, Takahashi M, Sakuma N, Abe S, Sakurai Y, Sakaguchi H, Ishino T, Uehara N, Usami SI.
Sci Rep, 10(1), 3662, 2020年 - Treatment algorithm for idiopathic sudden sensorineural hearing loss based on epidemiologic surveys of a large Japanese cohort
Kitoh R, Nishio SY, Usami SI.
Acta Otolaryngol, 140(1), 32-39, 2020年 - Mid-Frequency Hearing Loss Is Characteristic Clinical Feature of OTOA-Associated Hearing Loss.
Sugiyama K, Moteki H, Kitajiri SI, Kitano T, Nishio SY, Yamaguchi T, Wakui K, Abe S, Ozaki A, Motegi R, Matsui H, Teraoka M, Kobayashi Y, Kosho T, Usami SI.
Genes (Basel), 10(9), E715, 2019年 - Detailed Clinical Features of Deafness Caused by a Claudin-14 Variant.
Kitano T, Kitajiri SI, Nishio SY, Usami SI.
Int J Mol Sci, 20(18), E4579, 2019年 - Comprehensive analysis of syndromic hearing loss patients in Japan.
Ideura M, Nishio SY, Moteki H, Takumi Y, Miyagawa M, Sato T, Kobayashi Y, Ohyama K, Oda K, Matsui T, Ito T, Suzumura H, Nagai K, Izumi S, Nishiyama N, Komori M, Kumakawa K, Takeda H, Kishimoto Y, Iwasaki S, Furutate S, Ishikawa K, et al.
Sci Rep, 9(1), 11976, 2019年 - OTOF mutation analysis with massively parallel DNA sequencing in 2,265 Japanese sensorineural hearing loss patients.
Iwasa YI, Nishio SY, Sugaya A, Kataoka Y, Kanda Y, Taniguchi M, Nagai K, Naito Y, Ikezono T, Horie R, Sakurai Y, Matsuoka R, Takeda H, Abe S, Kihara C, Ishino T, Morita SY, Iwasaki S, Takahashi M, Ito T, Arai Y, Usami SI.
PLOS ONE, 14(5), e0215932, 2019年 - Frequency and clinical features of hearing loss caused by STRC deletions.
Yokota Y, Moteki H, Nishio SY, Yamaguchi T, Wakui K, Kobayashi Y, Ohyama K, Miyazaki H, Matsuoka R, Abe S, Kumakawa K, Takahashi M, Sakaguchi H, Uehara N, Ishino T, Kosho T, Fukushima Y, Usami SI.
Sci Rep, 9(1), 4408, 2019年 - Diagnostic pitfalls for GJB2-related hearing loss: A novel deletion detected by Array-CGH analysis in a Japanese patient with congenital profound hearing loss.
Abe S, Nishio SY, Yokota Y, Moteki H, Kumakawa K, Usami SI.
Clin Case Rep, 6(11), 2111-2116, 2019年 - A rational approach to identifying newborns with hearing loss caused by congenital cytomegalovirus infection by dried blood spot screening.
Moteki H, Isaka Y, Inaba Y, Motobayashi M, Nishio SY, Ohira S, Yano T, Iwasaki S, Shiozawa T, Koike K, Usami SI.
Acta Otolaryngol, 138(8), 708-712, 2019年 - Targeted Allele Suppression Prevents Progressive Hearing Loss in the Mature Murine Model of Human TMC1 Deafness.
Yoshimura H, Shibata SB, Ranum PT, Moteki H, Smith RJH.
Mol Ther., 27(3), 681-690, 2019年 - Investigation of Recurrent Cases of Head and Neck Cancer after Initial Treatment.
Kitoh R, Mori K, Usami SI.
Int J Pract Otolaryngol, 2, e11-e17, 2019年 - The Prevalence and Clinical Characteristics of TECTA-Associated Autosomal Dominant Hearing Loss.
Yasukawa R, Moteki H, Nishio SY, Ishikawa K, Abe S, Honkura Y, Hyogo M, Mihashi R, Ikezono T, Shintani T, Ogasawara N, Shirai K, Yoshihashi H, Ishino T, Otsuki K, ItoT, Sugahara K, Usami SI.
Genes., 10, 744, 2019年 - Mutational Spectrum and Clinical Features of Patients with LOXHD1 Variants Identified in an 8074 Hearing Loss Patient Cohort.
Maekawa K, Nishio SY, Abe S, Goto SI, Honkura Y, Iwasaki S, Kanda Y, Kobayashi Y, Oka SI, Okami M, Oshikawa C, Sakuma N, Sano H, Shirakura M, Uehara N, Usami SI.
Genes., 10, 735, 2019年 - Feasibility of hearing preservation for residual hearing with longer cochlear implant electrodes.
Moteki H, Nishio SY, Miyagawa M, Tsukada K, Noguchi Y, Usami SI.
Acta Otolaryngol, 138(12), 1080-1085, 2018年 - Bilateral delayed endolymphatic hydrops evaluated by bilateral intratympanic injection of gadodiamide with 3T-MRI.
Iwasa YI, Tsukada K, Kobayashi M, Kitano T, Mori K, Yoshimura H, Fukuoka H, Usami SI.
PLOS ONE, 13(12), e0206891, 2018年 - Sensorineural hearing loss and mild cardiac phenotype caused by an EYA4 mutation.
Abe S, Takeda H, Nishio SY, Usami SI.
Hum Genome Var, 5, 23, 2018年 - Pneumolabyrinth, intracochlear and vestibular fluid loss after cochlear implantation.
Moteki H, Fujinaga Y, Goto T, Usami SI.
AURIS NASUS LARYNX, 45(5), 1116-1120, 2018年 - Simple and efficient germline copy number variant visualization method for the Ion AmpliSeq™ custom panel.
Nishio SY, Moteki H, Usami SI.
Mol Genet Genomic Med, 2018年 - WFS1 mutation screening in a large series of Japanese hearing loss patients: Massively parallel DNA sequencing-based analysis.
Kobayashi M, Miyagawa M, Nishio SY, Moteki H, Fujikawa T, Ohyama K, Sakaguchi H, Miyanohara I, Sugaya A, Naito Y, Morita SY, Kanda Y, Takahashi M, Ishikawa K, Nagano Y, Tono T, Oshikawa C, Kihara C, Takahashi H, Noguchi Y, Usami SI.
PLOS ONE, 13(3), e0193359, 2018年 - The diagnostic performance of a novel ELISA for human CTP (Cochlin-tomoprotein) to detect perilymph leakage.
Ikezono T, Matsumura T, Matsuda H, Shikaze S, Saitoh S, Shindo S, Hasegawa S, Oh SH, Hagiwara Y, Ogawa Y, Ogawa H, Sato H, Tono T, Araki R, Maeda Y, Usami SI, Kase Y.
PLOS ONE, 13(1), e0191498, 2018年 - Cochlear volume as a predictive factor for residual-hearing preservation after conventional cochlear implantation.
Takahashi M, Arai Y, Sakuma N, Yabuki K, Sano D, Nishimura G, Oridate N, Usami SI.
Acta Otolaryngol, 138(4), 345-350, 2018年 - Hearing preservation cochlear implantation in children: The HEARRING Group consensus and practice guide.
Rajan G, Tavora-Vieira D, Baumgartner WD, Godey B, Müller J, O'Driscoll M, Skarzynski H, Skarzynski P, Usami SI, Adunka O, Agrawal S, Bruce I, De Bodt M, Caversaccio M, Pilsbury H, Gavilán J, Hagen R, Hagr A, Kameswaran M, Karltorp E, Kompis M, Kuzovkov V, et al.
Cochlear Implants Int, 19(1), 1-13, 2018年 - A rational approach to identifying newborns with hearing loss caused by congenital cytomegalovirus infection by dried blood spot screening.
Moteki H, Isaka Y, Inaba Y, Motobayashi M, Nishio SY, Ohira S, Yano T, Iwasaki S, Shiozawa T, Koike K, Usami SI.
Acta Otolaryngol., 138(8), 708-712, 2018年 - Introgressive Hybridization of the Erebia ligea Complex(Lepidoptera, Nymphalidae) in the Far Eastern Region.
Nakatani T, Ueda S, Nishio SI, Usami SI.
Butterfly Science, 11, 26-37, 2018年 - Molecular Phylogeny of the Genus Erebia(Lepidoptera,Nymphalidae,Satyrinae) : Species Groups Inferred from Mitochondrial and Nucleus Gene Sequences.
Nakatani T, Ueda S, Nishio SY, Usami SI.
Butterfly Science, 12, 27-48, 2018年 - Diagnostic pitfalls for GJB2-related hearing loss: A novel deletion detected by Array-CGH analysis in a Japanese patient with congenital profound hearing loss.
Abe S, Nishio SY,Yokota Y, Moteki H, Kumakawa K, Usami SI.
Cin Case Rep., 6, 2111-2116, 2018年 - Long-term results of hearing preservation cochlear implant surgery in patients with residual low frequency hearing.
Moteki H, Nishio S, Miyagawa M, Tsukada K, Iwasaki S, Usami S.
Acta Otolaryngol, 137(5), 516-521, 2017年 - Congenital hearing loss.
Korver AM, Smith RJ, Van Camp G, Schleiss MR, Bitner-Glindzicz MA, Lustig LR, Usami SI, Boudewyns AN.
Nat Rev Dis Primers. , 3, 16094, 2017年 - The Clinical Next-Generation Sequencing Database: A Tool for the Unified Management of Clinical Information and Genetic Variants to Accelerate Variant Pathogenicity Classification.
Nishio SY, Usami SI.
Hum Mutat., 38(3), 252-259, 2017年 - Towards a Unified Testing Framework for Single-Sided Deafness Studies: A Consensus Paper.
Van de Heyning P1, Távora-Vieira D, Mertens G, Van Rompaey V, Rajan GP, Müller J, Hempel JM, Leander D, Polterauer D, Marx M, Usami SI, Kitoh R, Miyagawa M, Moteki H, Smilsky K, Baumgartner WD, Keintzel TG, Sprinzl GM, Wolf-Magele A, Arndt S, Wesarg T, Zirn S, Baumann U, Weissgerber T, Rader T, Hagen R, Kurz A, Rak K, Stokroos R, George E, Polo R, Medina M, Henkin Y, Hilly O, Ulanovski D, Rajeswaran R, Kameswaran M, Di Gregorio MF, Zernotti ME.
Audiol Neurootol. , 21(6), 391-398, 2017年 - The clinical features and prognosis of mumps-associated hearing loss: a retrospective, multi-institutional investigation in Japan.
Morita S, Fujiwara K, Fukuda A, Fukuda S, Nishio SY, Kitoh R, Hato N, Ikezono T, Ishikawa K, Kaga K, Matsubara A, Matsunaga T, Murata T, Naito Y, Nishizaki K, Ogawa K, Sano H, Sato H, Sone M, Suzuki M, Takahashi H, Tono T, Yamashita H, Yamasoba T, Usami SI.
Acta Otolaryngol., 137, S44-S47, 2017年 - Laser-capture micro dissection combined with next-generation sequencing analysis of cell type-specific deafness gene expression in the mouse cochlea.
Nishio SY, Takumi Y, Usami SI
Hear Res., 348, 87-97, 2017年 - A nationwide study on enlargement of the vestibular aqueduct in Japan.
Noguchi Y, Fukuda S, Fukushima K, Gyo K, Hara A, Nakashima T, Ogawa K, Okamoto M, SatoH, Usami SI, Yamasoba T, Yokoyama T, Kitamura K.
Auris Nasus Larynx., 44(1), 33-39, 2017年 - The effect of initial treatment on hearing prognosis in idiopathic sudden sensorineural hearing loss: a nationwide survey in Japan.
Okada M, Hato N, Nishio SY, Kitoh R, Ogawa K, Kanzaki S, Sone M, Fukuda S, Hara A, Ikezono T, Ishikawa K, Iwasaki S, Kaga K, Kakehata S, Matsubara A, Matsunaga T, Murata T, Naito Y, Nakagawa T, Nishizaki K, Noguchi Y, Sano H, Sato H, Suzuki M, Shojaku H, Takahashi H, Takeda H, Tono T, Yamashita H, Yamasoba T, Usami SI.
Acta Otolaryngol., 137, S30-S33, 2017年 - Etiology of single-sided deafness and asymmetrical hearing loss.
Usami SI, Kitoh R, Moteki H, Nishio SY, Kitano T, Kobayashi M, Shinagawa J, Yokota Y, Sugiyama K, Watanabe K.
Acta Otolaryngol., 137, S2-S7, 2017年 - Nationwide epidemiological survey of idiop.athic sudden sensorineural hearing loss in Japan.
Kitoh R, Nishio SY, Ogawa K, Kanzaki S, Hato N, Sone M, Fukuda S, Hara A6, Ikezono T, Ishikawa K, Iwasaki S, Kaga K, Kakehata S, Matsubara A, Matsunaga T, Murata T, Naito Y, Nakagawa T, Nishizaki K, Noguchi Y, Sano H, Sato H, Suzuki M, Shojaku H, Takahashi H, Takeda H, Tono T, Yamashita H, Yamasoba T, Usami SI.
Acta Otolaryngol., 137, S8-S16, 2017年 - Differences between acoustic trauma and other types of acute noise-induced hearing loss in terms of treatment and hearing prognosis.
Wada T, Sano H2, Nishio SY, Kitoh R, Ikezono T, Iwasaki S, Kaga K, Matsubara A, Matsunaga T, Murata T, Naito Y, Suzuki M, Takahashi H, Tono T, Yamashita H, Hara A, Usami SI.
Acta Otolaryngol., 137, S48-S52, 2017年 - Prognostic impact of gene polymorphisms in patients with idiopathic sudden sensorineural hearing loss.
Kitoh R, Nishio SY, Usami SI.
Acta Otolaryngol., 137, S24-S29, 2017年 - Relationships among drinking and smoking habits, history of diseases, body mass index and idiopathic sudden sensorineural hearing loss in Japanese patients.
Umesawa M, Kobashi G, Kitoh R, Nishio SY, Ogawa K, Hato N, Sone M, Fukuda S, Hara A, Ikezono T, Ishikawa K, Iwasaki S, Kaga K, Kakehata S, Matsubara A, Matsunaga T, Murata T, Naito Y, Nakagawa T, Nishizaki K, Noguchi Y, Sano H, Sato H, Suzuki M, Shojaku H, Takahashi H, Takeda H, Tono T, Yamashita H, Yamasoba T, Usami SI.
Acta Otolaryngol., 137, S17-S23, 2017年 - Acute sensorineural hearing loss.
Usami SI
Acta Otolaryngol., 137, S1, 2017年 - A nationwide multicenter study of the Cochlin tomo-protein detection test: clinical characteristics of perilymphatic fistula cases.
Matsuda H, Sakamoto K, Matsumura T, Saito S, Shindo S, Fukushima K, Nishio SY, Kitoh R, Shibasaki O, Ito A, Araki R, Usami SI, Suzuki M, Ogawa K, Hasegawa T, Hagiwara Y, Kase Y, Ikezono T,
Acta Otolaryngol., 137, S53-S59, 2017年 - Epidemiological survey of acute low-tone sensorineural hearing loss.
Sato H, Kuwashima S, Nishio SY, Kitoh R, Fukuda S, Hara A, Hato N, Ikezono T, Ishikawa K, Iwasaki S, Kaga K, Matsubara A, Matsunaga T, Murata T, Naito Y, Nakagawa T, Nishizaki K, Noguchi Y, Ogawa K, Sano H, Sone M, Shojaku H, Takahashi H, Tono T, Yamashita H, Yamasoba T, Usami SI.
Acta Otolaryngol., 137, S34-S37, 2017年 - Outcomes of cochlear implantation for the patients with specific genetic etiologies: a systematic literature review.
Nishio SY, Usami SI
Acta Otolaryngol., 137, 730-742, 2017年 - Idiopathic sudden sensorineural hearing loss and acute low-tone sensorineural hearing loss: a comparison of the results of a nationwide epidemiological survey in Japan.
Yoshida T, Sone M, Kitoh R, Nishio SY, Ogawa K, Kanzaki S, Hato N, Fukuda S, Hara A, Ikezono T, Ishikawa K, Iwasaki S, Kaga K, Kakehata S, Matsubara A, Matsunaga T, Murata T, Naito Y, Nakagawa T, Nishizaki K, Noguchi Y, Sano H, Sato H, Suzuki M, Shojaku H, Takahashi H, Takeda H, Tono T, Yamashita H, Yamasoba T, Usami SI.
Acta Otolaryngol., 137, S38-S59, 2017年 - POU4F3 mutation screening in Japanese hearing loss patients: Massively parallel DNA sequencing-based analysis identified novel variants associated with autosomal dominant hearing loss.
Kitano T, Miyagawa M, Nishio SY, Moteki H, Oda K, Ohyama K, Miyazaki H, Hidaka H, Nakamura KI, Murata T, Matsuoka R, Ohta Y, Nishiyama N, Kumakawa K, Furutate S, Iwasaki S, Yamada T, Ohta Y, Uehara N, Noguchi Y, Usami SI.
PLOS ONE, 12(5), e0177636, 2017年 - Round Window Application of an Active Middle Ear Implant: A Comparison With Hearing Aid Usage in Japan.
Iwasaki S, Usami SI, Takahashi H, Kanda Y, Tono T, Doi K, Kumakawa K, Gyo K, Naito Y, Kanzaki S, Yamanaka N, Kaga K.
Otol Neurotol. , 38(6), e145-E151, 2017年 - Compound heterozygous dominant and recessive GJB2 mutations cause deafness with palmoplantar karatoderma.
Arai Y, Takahashi M, Sakuma N, Nishio S, Oridate N, Usami SI.
Acta Otolaryngol., 2(1), 137-140, 2017年 - Frequency of Usher syndrome type 1 in deaf children by massively parallel DNA sequencing.
Yoshimura H, Miyagawa M, Kumakawa K, Nishio S, Usami S.
J Hum Genet, 61, 419-422, 2016年 - Correlation between White Matter Lesions and Intelligence Quptient in patients with Congenital Cytomegalovirus Infection.
Inaba Y, Motobayashi M, Nishioka M, Kaneko T, Yamauchi S, Kawasaki Y, Shiba N, Nishio S, Moteki H, Miyagawa M, Takumi Y, Usami S, Koike K.
Pediatr Neurol, 55, 52-57, 2016年 - The effects of cochlear implantation in Japanese single-sided deafness patients: five case reports.
Kitoh R, Moteki H, Nishio S, Shinden S, Kanzaki S, Iwasaki S, Ogawa K, Usami S.
Acta Otolaryngol, 136(460-464), 2016年 - SOD1 gene polymorphisms in sudden sensorineural hearing loss.
Kitoh R, Nishio S, Ogawa K, Okamura M Kitamura K, Gyo K, Sato H, Nakashima T, Fukuda S, Fukushima K, Hara A, Yamasoba T, Usami S.
Acta Otolaryngol, 136, 465-469, 2016年 - A Comprehensive Study on the Etiology of Patients Receiving Cochlear Implantation With Special Emphasis on Genetic Epidemiology.
Miyagawa M, Nishio S, Usami S.
Otol Neurotol, 37(2), e126-e134, 2016年 - An effective screening strategy for deafness in combination with a next-generation sequencing platform: a consecutive analysis.
Sakuma N, Moteki H, Takahashi M, Nishio S, Arai Y, Yamashita Y, Oridate N, Usami S.
J Hum Genet, 61(3), 253-261, 2016年 - Comprehensive genetic testing with ethnic-specific filtering by allele frequency in a Japanese hearing-loss population.
Moteki H, Azaiez H, Booth K.T, Shearer A.E, Sloan C.M, Kolbe D.L, Nishio S, Hattori M, Usami S, Smith R.J.H.
Clin Genet, 89, 466-472, 2016年 - Discrimination of Japanese monosyllables in patients with high-frequency hearing loss.
Karino S, Usami S, Kumakawa K, Takahashi H, Tono T, Naito Y, Doi K, Ito K, Suzuki M, Sakata H, Takumi Y, Iwasaki S, Kakigi A, Yamasoba T.
AURIS NASUS LARYNX, 43(269-280), 2016年 - Detection and Confirmation of Deafness-Causing Copy Number Variations in the STRC Gene by Massively Parallel Sequencing and Comparative Genomic Hybridization.
Moteki H, Azaiez H, Sloan-Heggen CM, Booth K, Nishio S, Wakui K, Yamaguchi T, Kolbe DL, Iwasa Y, Shearer AE, Fukushima Y, Smith RJ, Usami S.
Ann Otol Rhinol Laryngol, 125(918-923), 2016年 - MRIと内リンパ水腫
宇佐美真一、福岡久邦、吉村豪兼
JOHNS, 32(1), 37-40, 2016年 - 若年発症型両側性感音難聴
西尾信哉、宇佐美真一
耳喉頭頸, 88(3), 224-232, 2016年 - シスプラチンを用いた化学放射線療法の聴力変化の検討
鬼頭良輔、森健太郎、北野友裕、宇佐美真一
耳鼻臨床, 109(4), 233-238, 2016年 - 舌扁桃肥大に対しコブレーション手術が有効であった1例
岩佐陽一郎、工 穣、宇佐美真一
口喉科, 29(1), 115-120, 2016年 - 残存聴力活用型人工内耳(EAS: Electric Acoustic Stimulation)における内耳機能温存
茂木英明、宇佐美真一
Otol Jpn, 26(3), 149-153, 2016年 - 難聴の遺伝子診断とその臨床応用
西尾信哉、宇佐美真一
耳鼻臨床, 109, 828-829, 2016年 - 聴覚障害と遺伝子
宇佐美真一
Medical Science Digest, 42(4), 166-169, 2016年 - 人工聴覚器手術 人工内耳手術ー残存聴力型人工内耳ー
宇佐美真一
JOHNS, 32(9), 1282-1284, 2016年 - Social Health Insurance-Based Simultaneous Screening for 154 Mutations in 19 Deafness Genes Efficiently Identified Causative Mutations in Japanese Hearing Loss Patients.
Mori K, Moteki H, Miyagawa M, Nishio SY, Usami S.
PLOS ONE, 11(9), e0162230, 2016年 - Constitutive activation of DIA1 (DIAPH1) via C-terminal truncation causes human sensorineural hearing loss.
Ueyama T, Ninoyu Y, Nishio S, Miyoshi T, Torii H, Nishimura K, Sugahara K, Sakata H, Thumkeo D, Sakaguchi H, Watanabe N, Usami S, Saito N, Kitajiri S.
EMBO Molecular Medicine, 8(11), 1310-1324, 2016年 - Comprehensive Genetic Analysis of Japanese Autosomal Dominant Sensorineural Hearing Loss Patients.
Iwasa Y, Nishio S, Usami S.
PLOS ONE, 11(12), e0166781, 2016年 - Carrier frequency of the GJB2 mutations that cause hereditary hearing loss in the Japanese population
Taniguchi, Mirei; Matsuo, Hirotaka; Shimizu, Seiko; Nakayama, Akiyoshi; Suzuki, Koji; Hamajima, Nobuyuki; Shinomiya, Nariyoshi; Nishio, Shinya; Kosugi, Shinji; Usami, Shin-ichi; Ito, Juichi; Kitajiri, Shin-ichiro;
JOURNAL OF HUMAN GENETICS, 60(10), 613-617, 2015年10月 - Mutations in the MYO15A Gene Are a Significant Cause of Nonsyndromic Hearing Loss: Massively Parallel DNA Sequencing-Based Analysis
Miyagawa, Maiko; Nishio, Shin-ya; Hattori, Mitsuru; Moteki, Hideaki; Kobayashi, Yumiko; Sato, Hiroaki; Watanabe, Tomoo; Naito, Yasushi; Oshikawa, Chie; Usami, Shin-ichi;
ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY, 124, 158S-168S, 2015年05月 - Novel PTPRQ Mutations Identified in Three Congenital Hearing Loss Patients With Various Types of Hearing Loss
Sakuma, Naoko; Moteki, Hideaki; Azaiez, Hela; Booth, Kevin T.; Takahashi, Masahiro; Arai, Yasuhiro; Shearer, A. Eliot; Sloan, Christina M.; Nishio, Shin-ya; Kolbe, Diana L.; Iwasaki, Satoshi; Oridate, Nobuhiko; Smith, Richard J. H.; Usami, Shin-ichi;
ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY, 124, 184S-192S, 2015年05月 - Massively Parallel DNA Sequencing Successfully Identified Seven Families With Deafness-Associated MYO6 Mutations: The Mutational Spectrum and Clinical Characteristics
Miyagawa, Maiko; Nishio, Shin-ya; Kumakawa, Kozo; Usami, Shin-ichi;
ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY, 124, 148S-157S, 2015年05月 - USH2 Caused by GPR98 Mutation Diagnosed by Massively Parallel Sequencing in Advance of the Occurrence of Visual Symptoms
Moteki, Hideaki; Yoshimura, Hidekane; Azaiez, Hela; Booth, Kevin T.; Shearer, A. Eliot; Sloan, Christina M.; Kolbe, Diana L.; Murata, Toshinori; Smith, Richard J. H.; Usami, Shin-ichi;
ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY, 124, 123S-128S, 2015年05月 - Non-ocular Stickler Syndrome With a Novel Mutation in COL11A2 Diagnosed by Massively Parallel Sequencing in Japanese Hearing Loss Patients
Iwasa, Yoh-ichiro; Moteki, Hideaki; Hattori, Mitsuru; Sato, Ririko; Nishio, Shin-ya; Takumi, Yutaka; Usami, Shin-ichi;
ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY, 124, 111s-117S, 2015年05月 - The Patients Associated With TMPRSS3 Mutations Are Good Candidates for Electric Acoustic Stimulation
Miyagawa, Maiko; Nishio, Shin-ya; Sakurai, Yuika; Hattori, Mitsuru; Tsukada, Keita; Moteki, Hideaki; Kojima, Hiromi; Usami, Shin-ichi;
ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY, 124, 193S-204S, 2015年05月 - De Novo Mutation in X-Linked Hearing Loss-Associated POU3F4 in a Sporadic Case of Congenital Hearing Loss
Moteki, Hideaki; Shearer, A. Eliot; Izumi, Shuji; Kubota, Yamato; Azaiez, Hela; Booth, Kevin T.; Sloan, Christina M.; Kolbe, Diana L.; Smith, Richard J. H.; Usami, Shin-ichi;
ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY, 124, 169S-176S, 2015年05月 - Mutational spectrum and clinical features of patients with ACTG1 mutations identified by massively parallel DNA sequencing
Miyagawa, Maiko; Nishio, Shin-ya; Ichinose, Aya; Iwasaki, Satoshi; Murata, Takaaki; Kitajiri, Shin-ichiro; Usami, Shin-ichi;
ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY, 124, 84S-93S, 2015年05月 - Hearing Loss Caused by a P2RX2 Mutation Identified in a MELAS Family With a Coexisting Mitochondrial 3243AG Mutation
Moteki, Hideaki; Azaiez, Hela; Booth, Kevin T.; Hattori, Mitsuru; Sato, Ai; Sato, Yoshihiko; Motobayashi, Mitsuo; Sloan, Christina M.; Kolbe, Diana L.; Shearer, A. Eliot; Smith, Richard J. H.; Usami, Shin-ichi;
ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY, 124, 177S-183S, 2015年05月 - Gene Expression Profiles of the Cochlea and Vestibular Endorgans: Localization and Function of Genes Causing Deafness
Nishio, Shin-ya; Hattori, Mitsuru; Moteki, Hideaki; Tsukada, Keita; Miyagawa, Maiko; Naito, Takehiko; Yoshimura, Hidekane; Iwasa, Yoh-ichiro; Mori, Kentaro; Shima, Yutaka; Sakuma, Naoko; Usami, Shin-ichi;
ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY, 124, 6S-48S, 2015年05月 - Guanine nucleotide-binding protein 1 is one of the key molecules contributing to cancer cell radioresistance
Fukumoto, Motoi; Amanuma, Tatsuya; Kuwahara, Yoshikazu; Shimura, Tsutomu; Suzuki, Masatoshi; Mori, Shiro; Kumamoto, Hiroyuki; Saito, Yohei; Ohkubo, Yasuhito; Duan, Zhenfeng; Sano, Kenji; Oguchi, Tomohiro; Kainuma, Kazuyuki; Usami, Shinichi; Kinoshita, Kengo; Lee, Inchul; Fukumoto, Manabu;
CANCER SCIENCE, 105(10), 1351-1359, 2014年10月 - A clinical experience of 'STAMP' plate-guided Bonebridge implantation
Takumi, Yutaka; Matsumoto, Nozomu; Cho, Byunghyun; Ono, Hidenori; Mori, Kentaro; Tsukada, Keita; Ichinose, Aya; Yoshimura, Hidekane; Iwasaki, Satoshi; Komune, Shizuo; Usami, Shin-ichi;
ACTA OTO-LARYNGOLOGICA, 134(10), 1042-1046, 2014年10月 - Hearing preservation and clinical outcome of 32 consecutive electric acoustic stimulation (EAS) surgeries
Usami, Shin-Ichi; Moteki, Hideaki; Tsukada, Keita; Miyagawa, Maiko; Nishio, Shin-Ya; Takumi, Yutaka; Iwasaki, Satoshi; Kumakawa, Kozo; Naito, Yasushi; Takahashi, Haruo; Kanda, Yukihiko; Tono, Tetsuya;
ACTA OTO-LARYNGOLOGICA, 134(7), 717-727, 2014年07月 - Evaluation of cortical processing of language by use of positron emission tomography in hearing loss children with congenital cytomegalovirus infection
Moteki, Hideaki; Suzuki, Mika; Naito, Yasushi; Fujiwara, Keizo; Oguchi, Kazuhiro; Nishio, Shin-ya; Iwasaki, Satoshi; Usami, Shin-ichi;
INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY, 78(2), 285-289, 2014年02月 - A randomized controlled clinical trial of topical insulin-like growth factor-1 therapy for sudden deafness refractory to systemic corticosteroid treatment
Nakagawa, Takayuki; Kumakawa, Kozo; Usami, Shin-ichi; Hato, Naohito; Tabuchi, Keiji; Takahashi, Mariko; Fujiwara, Keizo; Sasaki, Akira; Komune, Shizuo; Sakamoto, Tatsunori; Hiraumi, Harukazu; Yamamoto, Norio; Tanaka, Shiro; Tada, Harue; Yamamoto, Michio; Yonezawa, Atsushi; Ito-Ihara, Toshiko; Ikeda, Takafumi; Shimizu, Akira; Tabata, Yasuhiko; Ito, Juichi;
BMC MEDICINE, 12, 2014年 - Utilizing Ethnic-Specific Differences in Minor Allele Frequency to Recategorize Reported Pathogenic Deafness Variants
Shearer, A. Eliot; Eppsteiner, Robert W.; Booth, Kevin T.; Ephraim, Sean S.; Gurrola, Jose, II; Simpson, Allen; Black-Ziegelbein, E. Ann; Joshi, Swati; Ravi, Harini; Giuffre, Angelica C.; Happe, Scott; Hildebrand, Michael S.; Azaiez, Hela; Bayazit, Yildirim A.; Erdal, Mehmet Emin; Lopez-Escamez, Jose A.; Gazquez, Irene; Tamayo, Marta L.; Gelvez, Nancy Y.; Leal, Greizy Lopez; Jalas, Chaim; Ekstein, Josef; Yang, Tao; Usami, Shin-ichi; Kahrizi, Kimia; Bazazzadegan, Niloofar; Najmabadi, Hossein; Scheetz, Todd E.; Braun, Terry A.; Casavant, Thomas L.; LeProust, Emily M.; Smith, Richard J. H.;
AMERICAN JOURNAL OF HUMAN GENETICS, 95(4), 445-453, 2014年 - Ubiquinol-10 supplementation activates mitochondria functions to decelerate senescence in senescence-accelerated mice
Tian, Geng; Sawashita, Jinko; Kubo, Hiroshi; Nishio, Shin-ya; Hashimoto, Shigenari; Suzuki, Nobuyoshi; Yoshimura, Hidekane; Tsuruoka, Mineko; Wang, Yaoyong; Liu, Yingye; Luo, Hongming; Xu, Zhe; Mori, Masayuki; Kitano, Mitsuaki; Hosoe, Kazunori; Takeda, Toshio; Usami, Shin-ichi; Higuchi, Keiichi;
ANTIOXIDANTS & REDOX SIGNALING, 20(16), 2606-2620, 2014年 - Massively Parallel DNA Sequencing Facilitates Diagnosis of Patients with Usher Syndrome Type 1
Yoshimura, Hidekane; Iwasaki, Satoshi; Nishio, Shin-ya; Kumakawa, Kozo; Tono, Tetsuya; Kobayashi, Yumiko; Sato, Hiroaki; Nagai, Kyoko; Ishikawa, Kotaro; Ikezono, Tetsuo; Naito, Yasushi; Fukushima, Kunihiro; Oshikawa, Chie; Kimitsuki, Takashi; Nakanishi, Hiroshi; Usami, Shin-ichi;
PLOS ONE, 9(3), 2014年 - An Usher syndrome type 1 patient diagnosed before the appearance of visual symptoms by MYO7A mutation analysis
Yoshimura, Hidekane; Iwasaki, Satoshi; Kanda, Yukihiko; Nakanishi, Hiroshi; Murata, Toshinori; Iwasa, Yoh-ichiro; Nishio, Shin-ya; Takumi, Yutaka; Usami, Shin-ichi;
INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY, 77(2), 298-302, 2013年02月 - Comprehensive Genetic Screening of KCNQ4 in a Large Autosomal Dominant Nonsyndromic Hearing Loss Cohort: Genotype-Phenotype Correlations and a Founder Mutation
Naito, Takehiko; Nishio, Shin-ya; Iwasa, Yoh-ichiro; Yano, Takuya; Kumakawa, Kozo; Abe, Satoko; Ishikawa, Kotaro; Kojima, Hiromi; Namba, Atsushi; Oshikawa, Chie; Usami, Shin-ichi;
PLOS ONE, 8(5), 2013年 - TECTA mutations in Japanese with mid-frequency hearing loss affected by zona pellucida domain protein secretion
Moteki, Hideaki; Nishio, Shin-ya; Hashimoto, Shigenari; Takumi, Yutaka; Iwasaki, Satoshi; Takeichi, Norihito; Fukuda, Satoshi; Usami, Shin-ichi;
JOURNAL OF HUMAN GENETICS, 57(9), 587-592, 2012年09月 - Patients with CDH23 mutations and the 1555A > G mitochondrial mutation are good candidates for electric acoustic stimulation (EAS)
Usami, Shin-ichi; Miyagawa, Maiko; Nishio, Shin-Ya; Moteki, Hideaki; Takumi, Yutaka; Suzuki, Mika; Kitano, Yoko; Iwasaki, Satoshi;
ACTA OTO-LARYNGOLOGICA, 132(4), 377-384, 2012年04月 - Comparison of the diagnostic value of 3 T MRI after intratympanic injection of GBCA, electrocochleography, and the glycerol test in patients with Meniere's disease
Fukuoka, Hisakuni; Takumi, Yutaka; Tsukada, Keita; Miyagawa, Maiko; Oguchi, Tomohiro; Ueda, Hitoshi; Kadoya, Masumi; Usami, Shin-ichi;
ACTA OTO-LARYNGOLOGICA, 132(2), 141-145, 2012年02月 - Different cortical metabolic activation by visual stimuli possibly due to different time courses of hearing loss in patients with GJB2 and SLC26A4 mutations
Moteki, Hideaki; Naito, Yasushi; Fujiwara, Keizo; Kitoh, Ryosuke; Nishio, Shin-ya; Oguchi, Kazuhiro; Takumi, Yutaka; Usami, Shin-ichi;
ACTA OTO-LARYNGOLOGICA, 131(11), 1232-1236, 2011年11月 - Novel USH2A mutations in Japanese Usher syndrome type 2 patients: marked differences in the mutation spectrum between the Japanese and other populations
Nakanishi, Hiroshi; Ohtsubo, Masafumi; Iwasaki, Satoshi; Hotta, Yoshihiro; Usami, Shin-ichi; Mizuta, Kunihiro; Mineta, Hiroyuki; Minoshima, Shinsei;
JOURNAL OF HUMAN GENETICS, 56(7), 484-490, 2011年07月 - Achievement of hearing preservation in the presence of an electrode covering the residual hearing region
Usami, Shin-Ichi; Moteki, Hideaki; Suzuki, Nobuyoshi; Fukuoka, Hisakuni; Miyagawa, Maiko; Nishio, Shin-Ya; Takumi, Yutaka; Iwasaki, Satoshi; Jolly, Claude;
ACTA OTO-LARYNGOLOGICA, 131(4), 405-412, 2011年04月 - Systematic analysis of mitochondrial genes associated with hearing loss in the Japanese population: dHPLC reveals a new candidate mutation
Mutai, Hideki; Kouike, Hiroko; Teruya, Eiko; Takahashi-Kodomari, Ikuko; Kakishima, Hiroki; Taiji, Hidenobu; Usami, Shin-ichi; Okuyama, Torayuki; Matsunaga, Tatsuo;
BMC MEDICAL GENETICS, 12, 2011年 - Clinical characteristics of delayed endolymphatic hydrops in Japan: A nationwide survey by the Peripheral Vestibular Disorder Research Committee of Japan
Shojaku, Hideo; Watanabe, Yukio; Takeda, Noriaki; Ikezono, Tetsuo; Takahashi, Masahiro; Kakigi, Akinobu; Ito, Juichi; Doi, Katsumi; Suzuki, Mamoru; Takumida, Masaya; Takahashi, Katsumasa; Yamashita, Hiroshi; Koizuka, Izumi; Usami, Shinichi; Aoki, Mitsuhiro; Naganuma, Hideaki;
ACTA OTO-LARYNGOLOGICA, 130(10), 1135-1140, 2010年10月 - Semi-quantitative evaluation of endolymphatic hydrops by bilateral intratympanic gadolinium-based contrast agent (GBCA) administration with MRI for Meniere's disease
Fukuoka, Hisakuni; Tsukada, Keita; Miyagawa, Maiko; Oguchi, Tomohiro; Takumi, Yutaka; Sugiura, Makoto; Ueda, Hitoshi; Kadoya, Masumi; Usami, Shin-Ichi;
ACTA OTO-LARYNGOLOGICA, 130(1), 10-16, 2010年01月 - Salicylate restores transport function and anion exchanger activity of missense pendrin mutations
Ishihara, Kenji; Okuyama, Shuhei; Kumano, Shun; Iida, Koji; Hamana, Hiroshi; Murakoshi, Michio; Kobayashi, Toshimitsu; Usami, Shinichi; Ikeda, Katsuhisa; Haga, Yoichi; Tsumoto, Kohei; Nakamura, Hiroyuki; Hirasawa, Noriyasu; Wada, Hiroshi;
HEARING RESEARCH, 270(1-2), 110-118, 2010年 - Pivotal Advance: Eosinophilia in the MES rat strain is caused by a loss-of-function mutation in the gene for cytochrome b(-245), alpha polypeptide (Cyba)
Mori, Masayuki; Li, Guixin; Hashimoto, Maiko; Nishio, Ayako; Tomozawa, Hiroshi; Suzuki, Nobuyoshi; Usami, Shin-ichi; Higuchi, Keiichi; Matsumoto, Kiyoshi;
JOURNAL OF LEUKOCYTE BIOLOGY, 86(3), 473-478, 2009年09月 - Clinical characteristics and genotype-phenotype correlation of hearing loss patients with SLC26A4 mutations
Suzuki, Hiroaki; Oshima, Aki; Tsukamoto, Koji; Abe, Satoko; Kumakawa, Kozo; Nagai, Kyoko; Satoh, Hitoshi; Kanda, Yukihiko; Iwasaki, Satoshi; Usami, Shin-ichi;
ACTA OTO-LARYNGOLOGICA, 127(12), 1292-1297, 2007年 - A new autosomal recessive form of Stickler syndrome is caused by a mutation in the COL9A1 gene
Van Camp, Guy; Snoeckx, Rikkert L.; Hilgert, Nele; van den Ende, Jenneke; Fukuoka, Hisakumi; Wagatsuma, Michio; Suzuki, Hiroaki; Smets, R. M. Erica; Vanhoenacker, Filip; Declau, Frank; Van De Heyning, Paul; Usami, Shin-ichi;
AMERICAN JOURNAL OF HUMAN GENETICS, 79(3), 449-457, 2006年09月 - Differences in the expression of genes between normal tissue and squamous cell carcinomas of head and neck using cancer-related gene cDNA microarray
Kainuma, Kazuyuki; Katsuno, Satoshi; Hashimoto, Shigenari; Oguchi, Tomohiro; Suzuki, Nobuyoshi; Asamura, Kenji; Usami, Shin-Ichi;
ACTA OTO-LARYNGOLOGICA, 126(9), 967-974, 2006年09月 An Immunoelectron Microscopic Analysis of the Glutamatergic Neurotransmission in the Organ of Corti of the Rat : The Afferent Transmission of Outer Hair Cell Synapses is Glutamatergic? ( ラットコルチ器におけるグルタミン酸神経伝達に関する免疫電子顕微鏡学的検討 : 外有毛細胞はグルタミン酸作動性か
新川 秀一; 松原 篤; 工 穣; 川畑 裕子; 宇佐美 真一;
弘前醫學, 51, S125-S130, 1999年
書籍等出版物
講演・口頭発表等
- Hearing And Structure Preservation CI Surgery : Tips And Tricks
Shin-ichi Usami
The 39th SORL International Conference, 2025年01月30日 - Cochlear Implantaion From The Perspective of Genetic Background
Shin-ichi Usami
The 39th SORL International Conference, 2025年01月30日 - 難聴の遺伝子治療の最前線
宇佐美真一
難聴と遺伝子治療についての勉強会, 2024年12月14日 - わかりやすい難聴遺伝子診断のはなし
宇佐美真一
市民公開講座 わかりやすく解説する難聴の診断と治療の進歩, 2024年10月26日 - FLEX28を用いた残存聴力活用型人工内耳(EAS)装用の症例
佐藤綾華、宇佐美真一、佐野眞一、佐野典子、小泉博美、久保田江里
第69回日本聴覚医学会, 2024年10月25日 - 当科における難聴遺伝学的検査の現況
末松真弓、中村高志、木戸脇尚子、兵庫美沙子、西尾信哉、宇佐美真一、瀧正勝
第69回日本聴覚医学会, 2024年10月25日 - 新生児聴覚スクリーニングをパスしたCDH23遺伝性難聴例
中村高志、末松真弓、木戸脇尚子、兵庫美沙子、西尾信哉、宇佐美真一、瀧正勝
第69回日本聴覚医学会, 2024年10月25日 - 長期間にわたって収集された聴力変化から考える遺伝性難聴の進行速度の計算法に関する考察
山本愛、和佐野浩一郎、大上麻由里、佐野肇、西尾信哉、宇佐美真一、大上研二
第69回日本聴覚医学会, 2024年10月25日 - Clinical Practice Guidelines for the Diagnosis and Management of Acute Sensorineural Hearing Loss
鬼頭良輔、西尾信哉、佐藤宏昭、池園哲郎、森田真也、和田哲郎、宇佐美真一
第69回日本聴覚医学会, 2024年10月25日 - 後期高齢者の補聴器適合と装用効果の検討
中島明人、長井今日子、廣田栄子、中島恭子、相馬眞祈、菅野桃花、内田紘子、長島彩、篠原怜珠、古屋信彦、宇佐美真一
第69回日本聴覚医学会, 2024年10月24日 - 人工内耳装用児の初期聴性行動の変化と親への指導
滝澤佳余子、宮川麻衣子、松岡藍、藤井絵理、北野庸子、西尾信哉、宇佐美真一
第69回日本聴覚医学会, 2024年10月24日 - 長い電極を用いたEAS手術における骨導閾値変化と術中蝸電図モニタリング
吉村豪兼、宇佐美真一、工 穣
第69回日本聴覚医学会, 2024年10月24日 - Next HEARING Event
Shin-ichi Usami
HEARRING The Earlier, The Better, 2024年10月22日 - 新生児153,913人における先天性難聴児の疫学調査と原因頻度
大久保卓哉、吉村豪兼、品川潤、西尾信哉、宇佐美真一、工 穣
第34回日本耳科学会, 2024年10月05日 - The Use of Flex28 Dex-Eluting Electrode in Relation to Genetics and EAS
Shin-ichi Usami
XXI Hearing and Structure Preservation Workshop, 2024年10月04日 - Round Table: DEEP ELECTRODE INSERTION IN EAS
Shin-ichi Usami(Panelist)
XXI Hearing and Structure Preservation Workshop, 2024年10月04日 - 一側性難聴に対する人工内耳植え込み術ー先進医療の結果ー
高橋優宏、岩崎聡、古舘佐起子、岡晋一郎、小山田匠吾、久保田江里、植草智子、桜井梓、西尾信哉、宇佐美真一
第34回日本耳科学会, 2024年10月04日 - 一側性高度感音難聴に対する人工内耳におけるAnatomy-Based Fittingの試み
鬼頭良輔、吉村豪兼、宇佐美真一、工 穣
第34回日本耳科学会, 2024年10月04日 - PRPS1遺伝子バリアントが同定されたCharcot-Marie-Tooth病5型に伴う症候群性難聴例(ポスター)
土橋奈々、橋本希、小宗徳孝、野田哲平、中川尚志、西尾信哉、宇佐美真一
第34回日本耳科学会, 2024年10月04日 - 聴力改善手術が患者QOLに及ぼす影響:邦訳版QOL質問表を用いた検討(第3報)
細谷誠、我那覇章、吉村豪兼、他25名、宇佐美真一、他5名
第34回日本耳科学会, 2024年10月03日 - 中等度難聴を示したGJB2遺伝子バリアントによる難聴症例
西山信宏、白井杏湖、塚原清彰、西尾信哉、宇佐美真一
第34回日本耳科学会, 2024年10月03日 - 両側性感音難聴838例における難聴の進行度別にみた原因解析
春日麻里子、吉村豪兼、鬼頭良輔、西尾信哉、宇佐美真一、工 穣
第34回日本耳科学会, 2024年10月03日 - 日本人難聴患者より認められた新規OTOGバリアントおよび臨床的特徴
荒井康裕、和田昴、高田顕太郎、相澤圭洋、逆井清、森下大樹、折舘伸彦、西尾信哉、宇佐美真一
第34回日本耳科学会, 2024年10月03日 - STRC遺伝子CopyNumberVariation(CNV)による感音難聴の4症例
竹川葉奈、森田真也、干野季美子、福田篤、西尾信哉、宇佐美真一
第34回日本耳科学会, 2024年10月03日 - 当院におけるアッシャー症候群の検討
上原奈津美、横井純、藤田岳、西尾新哉、柿木章伸、松永達雄、宇佐美真一
第34回日本耳科学会, 2024年10月03日 - Is there a different footprint between the hearing loss for EN
Shin-ichi Usami
8th World Congress on CI in Emerging Nation, 2024年09月17日 - Advantages of the RW approach in CI.
Shin-ichi Usami
8th World Congress on CI in Emerging Nation, 2024年09月16日 - 乗鞍岳における高山蝶調査2022,2023,2024
宇佐美真一、西尾信哉、伊藤建夫、中谷貴壽
2024 信州昆虫学会・信越昆虫研究会, 2024年09月08日 - A Seamless Approach to EAS and Conventional Cochler Implantation.
Shin-ichi Usami
CORLAS 2024 VIENNA(Collegium), 2024年08月26日 - Electroacoustic stimulation: indications, surgical techniques, and outcomes.
Shin-ichi Usami
3rd International Otolaryngology, Head&Neck Surgery Conferences, 2024年06月29日 - 遺伝学的検査によりミトコンドリア難聴が確定した4症例
西山信宏、白井杏湖、塚原清彰、宇佐美真一
第86回耳鼻咽喉科臨床学会, 2024年06月28日 - How the genetic origin of deafness can affect the indication and results of cochlear implantaion.
Shin-ichi Usami
3rd International Otolaryngology, Head&Neck Surgery Conferences, 2024年06月28日 - Rational for stimulation of the apex with deep insertion and full cochlear coverge.
Shin-ichi Usami
3rd International Otolaryngology, Head&Neck Surgery Conferences, 2024年06月28日 - 北アルプス・八ヶ岳での高山蝶の調査と保全活動
宇佐美真一、西尾信哉
2024年度上高地研究連絡会, 2024年06月19日 - 当院を受診した軽中度難聴患者における遺伝子の関与
小泉博美、佐野真一、佐野典子、宇佐美真一
第131回 日耳鼻静岡県地方部会学術講演会, 2024年04月07日 - 難聴診断の遺伝子パネル検査
宇佐美真一
第44回 日本臨床薬理学会(12/14-16)), 2023年12月15日 - 人工内耳が変えてくれたもの
宇佐美真一
きこえとことばの勉強会・音楽会・交流会, 2023年11月23日 - Genetic landscape of HL in Japan
Shin-ichi Usami
APSCI, 2023年11月09日 - EAS/人工内耳のシームレス化について
宇佐美真一
低侵襲人工内耳手術講習会, 2023年11月04日 - PJVK遺伝子バリアントが同定されたAuditory Neuropathy Spectrum Disorderの1例
土橋奈々、松本希、小宗徳孝、野田哲平、中川尚志、西尾信哉、宇佐美真一
第33回 日本耳科学会, 2023年11月03日 - 同胞症例における遺伝子難聴の検討
荒井康裕、高田顕太郎、和田昴、森下大樹、相澤圭洋、逆井清、西尾信哉、宇佐美真一、折舘伸彦
第33回 日本耳科学会, 2023年11月03日 - 本邦におけるBO/BOR症候群の臨床的特徴
後藤真一、佐々木亮、松原篤、西尾信哉、宇佐美真一
第33回 日本耳科学会, 2023年11月03日 - 当院における側頭骨骨折の検討ー外リンパ瘻を疑う症例の探索
茂木英明、西尾信哉、宇佐美真一
第33回 日本耳科学会, 2023年11月03日 - 当院におけるミトコンドリア遺伝子バリアント例における聴力像の検討
中西啓、喜夛淳哉、新村大地、西尾信哉、宇佐美真一、三澤清
第33回 日本耳科学会, 2023年11月03日 - Genetics
Shin-ichi Usami
Fall Cochlear Implant meeting, 2023年10月25日 - 難聴の診断と治療の進歩
宇佐美真一
市民公開講座 難聴の診断と治療の進歩, 2023年10月14日 - CDH23遺伝子変異により早期聴力喪失し両側同時人工内耳埋め込み術を施工した小児4症例
織田潔、香取幸夫、宇佐美真一、西尾信哉
第68回 日本聴覚医学会, 2023年10月12日 - Complete cochlear coverageを目指した人工内耳電極選択
吉村豪兼、宇佐美真一、工 穣
第68回 日本聴覚医学会, 2023年10月12日 - 当科で難聴の遺伝学的検査を施工した症例の検討
石谷えみ、近藤英司、東貴弘、坂本幸、島田亜紀、西尾信哉、宇佐美真一、北村嘉章
第68回 日本聴覚医学会, 2023年10月12日 - 進行性難聴を示したMYH9関連疾患の1家系
菅原一真、小林由貴、山本陽平、中津愛子、福田彩、山下裕司、西尾信哉、宇佐美真一
第68回 日本聴覚医学会, 2023年10月12日 - Germline mosaicismを有することが疑われたMYO15A遺伝子変異
後藤真一、佐々木亮、木村恵、松原篤、西尾信哉、宇佐美真一
第68回 日本聴覚医学会, 2023年10月12日 - LENAシステムを用いた難聴児への語りかけの定量化と聴性行動発達の検討
滝澤佳余子、宮川麻衣子、出籠藍、北野庸子、西尾信哉、宇佐美真一
第68回 日本聴覚医学会, 2023年10月12日 - EAS With Long Electrodes
Shin-ichi Usami
XX Hearing and Strucure Preservation Workshop, 2023年10月09日 - The Origin of the Japanese from the Perspective of Defness Genes.
Shin-ichi Usami
CORLAS 2023(コレギウム), 2023年08月28日 - 保険診療での遺伝学的検査:難聴遺伝子解析研究の社会実装 パネルデイスカッション
宇佐美真一
第30回 遺伝子診療学会, 2023年07月28日 - MYO15A変異症例の長期聴力経過
小林有美子、金城伸祐、志賀清人、宇佐美真一、西尾信哉(ポスター発表)
第85回 耳鼻咽喉科臨床学会, 2023年06月25日 - Could the Genetic Origin of Sensorineural Hearing Loss Affect the Outcome of Cochlear Implantation?
Shin-Ichi Usami, Shin-Ya Nishio
3rd INTERNATIONAL SYMPOSIUM ON INNER EAR THERAPEUTICS, 2023年04月29日 - The Challenges in Preserving Residual Hearing in Cochler Implantation.
Shin-Ichi Usami, Hidekane Yoshimura, Shin-Ya Nishio
3rd INTERNATIONAL SYMPOSIUM ON INNER EAR THERAPEUTICS, 2023年04月28日
所属学協会
共同研究・競争的資金等の研究課題
- 遺伝子解析に基づいた難聴発症メカニズムの解明および個別化医療システムの構築
科学研究費補助金, 基盤研究(A)
2015年 - 2017年 - 科学的エビデンスに基づいた遺伝性難聴の治療法確立に関する調査研究
科学研究費補助金, 日本医療研究開発機構, 難治性疾患実用化研究事業
2015年 - 2017年 - 難治性聴覚障害に関する調査研究
科学研究費補助金, 厚生労働省, 難治性疾患政策研究事業
2014年 - 2016年 - 新しい難聴遺伝子診断システムの開発および臨床応用に関する研究
科学研究費補助金, 障害者対策総合研究事業
2013年 - 2015年 - 高度医療残存聴力活用型人工内耳挿入術の適応症および有効性、安全性に関する
科学研究費補助金, 厚生労働省, 医療技術実用化総合研究事業
2012年 - 2013年 - 遺伝性難聴および外耳、中耳、内耳奇形に関する調査研究
科学研究費補助金, 厚生労働省, 難治性疾患等克服研究事業
2012年 - 2013年 - 次世代シークエンサーを用いた難聴遺伝子の網羅的解析
科学研究費補助金, 基盤研究(A)
2010年 - 2012年 - Usher症候群に関する調査研究
科学研究費補助金, 厚生労働省, 難治性疾患克服研究事業
2010年 - 2012年 - 難治性内耳疾患の遺伝子バンク構築
科学研究費補助金, 厚生労働省, 難治性疾患克服研究事業
2009年 - 2009年 - サブタイプ分類に基づく小児難聴診断、療育システムの構築
科学研究費補助金, 厚生労働省, 障害者対策総合研究事業
2008年 - 2010年 - 聴覚・平衡覚・嗅覚刺激が脳機能に及ぼす影響に関する研究
科学研究費補助金, 萌芽研究
2007年 - 2009年 - 老人性難聴に対する分子遺伝学的アプローチ
科学研究費補助金, 基盤研究(B)
2005年 - 2008年 - 難聴遺伝子データベース構築と遺伝カウンセリングに関する研究
科学研究費補助金, 厚生労働省, 感覚器障害研究事業
2002年 - 2004年 - 難聴の遺伝子解析と臨床応用に関する研究
科学研究費補助金, 基盤研究(A)
2000年 - 2002年 - 難聴の遺伝子解析
- 宇宙医学(内耳の神経伝達機構)
- 人工内耳
- 耳鼻咽喉科疾患の分子生物学