教室案内

論文実績

2020

  • 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. Prevalence and clinical features of hearing loss caused by EYA4 variants. Sci Rep. 2020 Feb 27;10(1):3662.
  • Usami SI, Nishio SY, Moteki H, Miyagawa M, Yoshimura H. Cochlear Implantation From the Perspective of Genetic Background. Anat Rec (Hoboken). 2020 Mar;303(3):563-593.
  • Kitoh R, Nishio SY, Usami SI. Treatment algorithm for idiopathic sudden sensorineural hearing loss based on epidemiologic surveys of a large Japanese cohort. Acta Otolaryngol. 2020 Jan;140(1):32-39.
  • Shinagawa J, Moteki H, Nishio SY, Noguchi Y, Usami SI. Haplotype Analysis of GJB2Mutations: Founder Effect or Mutational Hot Spot? Genes (Basel). 2020 Feb 27;11(3):250.
  • Moteki H, Kitoh R, Usami SI. The availability of an adhesive bone conduction hearing device: a preliminary report of a single-center experience. Acta Otolaryngol. 2020 Apr;140(4):319-326.
  • 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. Clinical Characteristics and In Vitro Analysis of MYO6Variants Causing Late-Onset Progressive Hearing Loss. Genes (Basel). 2020 Mar 4;11(3):273.
  • Yoshimura H, Moteki H, Nishio SY, Miyajima H, Miyagawa M, Usami SI. Genetic testing has the potential to impact hearing preservation following cochlear implantation. Acta Otolaryngol. 2020 Jun;140(6):438-444.
  • 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. A phase I/IIa double blind single institute trial of low dose sirolimus for Pendred syndrome/DFNB4. Medicine (Baltimore). 2020 May;99(19):e19763.
  • 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. A comparative analysis of genetic hearing loss phenotypes in European/American and Japanese populations. Hum Genet. 2020 Oct;139(10):1315-1323.
  • 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. Novel ACTG1 mutations in patients identified by massively parallel DNA sequencing cause progressive hearing loss. Sci Rep. 2020 Apr 27;10(1):7056.
  • Yoshimura H, Moteki H, Nishio SY, Usami SI. Electric-acoustic stimulation with longer electrodes for potential deterioration in low-frequency hearing. Acta Otolaryngol. 2020 Aug;140(8):632-638.
  • Tsukada K, Usami SI. Detailed MR imaging assessment of endolymphatic hydrops in patients with SLC26A4 mutations. Auris Nasus Larynx. 2020 Dec;47(6):958-964.
  • Omichi R, Yoshimura H, Shibata SB, Vandenberghe LH, Smith RJH. Hair Cell Transduction Efficiency of Single- and Dual-AAV Serotypes in Adult Murine Cochleae. Mol Ther Methods Clin Dev. 2020 May 13;17:1167-1177.
  • Shibata SB, West MB, Du X, Iwasa Y, Raphael Y, Kopke RD. Gene therapy for hair cell regeneration: Review and new data. Hear Res. 2020 Sep 1;394:107981.

2019

  • G¨urkov R, Barath K, Foer B, Fukushima M, Gluth M, Hornibrook J, Perez-Fernandez N, Pyykk¨o I, Sone M, Usami SI, Wamg W, Zou J, Naganawa S.
    A plea for systematic literature analysisand conclusive study design, comment on: “Systematic review of magnetic resonanceimaging for diagnosis of Meniere disease” J Vestib Res. 2019
  • 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.
    Mid-Frequency Hearing Loss Is Characteristic Clinical Feature of OTOA-Associated Hearing Loss. Genes. 10(9)E715 2019
  • Kitano T, Kitajiri SI, Nishio SY, Usami SI.
    Detailed Clinical Features of Deafness Caused by a Claudin-14 Variant. Int J Mol Sci. 20:4579 2019
  • 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, Nisiyama N, Komori M, Kumakawa K, Takeda H, Kishimoto Y, Iwasaki S, Furutate S, Ishikawa K, Fujioka M, Nakanishi H, Nakayama J, Horie R, Ohta Y, Naito Y, Kakudo M, Sakaguchi H, Kataoka Y, Sugawara K, Hato N, Nakagawa T, Tsuchihashi N, Kanda Y, Kihara CH, Tono T, Miyanohara I, Ganaha A, Usami SI.
    Comprehensive analysis of syndromic hearing loss patients in Japan. Sci Rep. 9 11976 2019
  • Yoshimura H, Shibata SB, Ranum PT, Moteki H, Smith RJH.
    Targeted Allele Suppression Prevents Progressive Hearing Loss in the Mature Murine Model of Human TMC1 Deafness. Mol Ther. 27(3) 681-690 2019
  • Kitoh R, Mori K, Usami SI.
    Investigation of Recurrent Cases of Head and Neck Cancer after Initial Treatment. Int J Pract Otolaryngol 2 e11-e17 2019
  • Iwasa Y, 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 S, Iwasaki S, Takahashi M, Ito T, Arai Y,Usami SI.
    OTOF mutation analysis with massively parallel DNA sequencing in 2,265 Japanese sensorineural hearing loss patients. PLoS ONE 14(5) e0215932 2019
  • 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.
    Frequency and clinical featuresof hearing loss caused by STRC deletions. Sci Rep. 9 4408 2019
  • Moteki H, Nishio SY, Miyagawa M, Tsukada K, Noguchi Y, Usami SI.
    Feasibility of hearing preservation for residual hearing with longer cochlear implant electrodes. Acta Otolaryngol 31: 1084-1089 2019
  • DiStefano MT, Hemphill SE, Oza AM, Siegert RK, Grant AR, Hughes MY, Cushman BJ, Azaiez H, Booth KT, Chapin A, Duzkale H, Matsunaga T, Shen J, Zhang W, Kenna M, Schimmenti LA, Tekin M, Rehm HL, Tayoun ANA, Amr SS; ClinGen Hearing Loss Clinical Domain Working Group. ClinGen expert clinical validity curation of 164 hearing loss gene-disease pairs. Genet Med. 2019 Oct;21(10):2239-2247.
  • Katsuno T, Belyantseva IA, Cartagena-Rivera AX, Ohta K, Crump SM, Petralia RS, Ono K, Tona R, Imtiaz A, Rehman A, Kiyonari H, Kaneko M, Wang YX, Abe T, Ikeya M, Fenollar-Ferrer C, Riordan GP, Wilson EA, Fitzgerald TS, Segawa K, Omori K, Ito J, Frolenkov GI, Friedman TB, Kitajiri SI. TRIOBP-5 sculpts stereocilia rootlets and stiffens supporting cells enabling hearing. JCI Insight. 2019 Jun 20;4(12):e128561.
  • 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, Ito T, Sugahara K, Usami SI. The Prevalence and Clinical Characteristics of TECTA-Associated Autosomal Dominant Hearing Loss. Genes (Basel). 2019 Sep 24;10(10):744.
  • 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. Mutational Spectrum and Clinical Features of Patients with LOXHD1 Variants Identified in an 8074 Hearing Loss Patient Cohort. Genes (Basel). 2019 Sep 23;10(10):735.
  • Kitoh R, Nishio SY, Usami SI. Treatment algorithm for idiopathic sudden sensorineural hearing loss based on epidemiologic surveys of a large Japanese cohort. Acta Otolaryngol. 2020 Jan;140(1):32-39.