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Hearing Disorders: HELP
Articles by Shujiro B. Minami
Based on 12 articles published since 2009
(Why 12 articles?)
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Between 2009 and 2019, S. B. Minami wrote the following 12 articles about Hearing Disorders.
 
+ Citations + Abstracts
1 Article A clinical and genetic study of 16 Japanese families with Waardenburg syndrome. 2019

Minami, Shujiro B / Nara, Kiyomitsu / Mutai, Hideki / Morimoto, Noriko / Sakamoto, Hirokazu / Takiguchi, Tetsuya / Kaga, Kimitaka / Matsunaga, Tatsuo. ·Department of Otolaryngology, National Hospital Organization Tokyo Medical Center, Tokyo, Japan; Division of Hearing and Balance Research, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, Tokyo, Japan. · Division of Hearing and Balance Research, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, Tokyo, Japan. · Department of Otorhinolaryngology, National Center for Child Health and Development, Tokyo, Japan. · Department of Otolaryngology, Head and Neck Surgery, Osaka City University, Osaka, Japan. · Department of Otolaryngology, National Hospital Organization Kanazawa Medical Center, Kanazawa, Japan. · Division of Hearing and Balance Research, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, Tokyo, Japan; Center for Speech and Hearing Disorders, International University of Health and Welfare, Tochigi, Japan. · Department of Otolaryngology, National Hospital Organization Tokyo Medical Center, Tokyo, Japan; Division of Hearing and Balance Research, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, Tokyo, Japan. Electronic address: matsunagatatsuo@kankakuki.go.jp. ·Gene · Pubmed #30978479.

ABSTRACT: The purpose of this study is to profile the clinical and genetic features of Japanese Waardenburg syndrome (WS) patients and validate the W index. Sixteen Japanese WS families with congenital sensorineural hearing loss were included in the study. The inner canthal, interpupillary, and outer canthal distances (ICD, IPD, and OCD) were measured for all patients, and patients were screened for presence of PAX3, MITF, SOX10, and EDNRB mutations. The WS patients were clinically classified under the current W index as follows: 13 families with WS1, 2 families with WS2, and 1 family with WS4. In the 13 WS1 families, genetic tests found PAX3 mutations in 5 families, MITF mutations in 4 families, SOX10 mutations in 3 families, and EDNRB mutations in 1 family. 61% of clinically classified WS1 patients under the current W index conflicted with the genetic classification, which implies W index is not appropriate for Japanese population. Resetting the threshold of W index or novel index formulated with ethnicity matched samples is necessary for clinical classification which is consistent with genetic classification for WS patients with distinct ethnicity.

2 Article Elongated EABR wave latencies observed in patients with auditory neuropathy caused by 2018

Hosoya, Makoto / Minami, Shujiro B / Enomoto, Chieko / Matsunaga, Tatsuo / Kaga, Kimitaka. ·National Institute of Sensory Organs Tokyo Japan. · Laboratory of Auditory Disorders and Division of Hearing and Balance Research Tokyo Japan. · Medical Genetics Center; National Institute of Sensory Organs, National Tokyo Medical Center Tokyo Japan. · Center for Speech and Hearing Disorders International University of Health and Welfare Tokyo Japan. ·Laryngoscope Investig Otolaryngol · Pubmed #30410993.

ABSTRACT: Objectives: We sought to determine how the pathology altered electrically evoked auditory brainstem responses (EABRs) in patients with hearing loss by evaluating EABRs in auditory neuropathy patients with Methods: We included 15 patients with congenital hearing loss, grouped according to pathology: Results: The EABR latencies of Wave III and Wave V, and of the interval between them, were significantly longer in the Conclusions: Our results suggest Level of Evidence: Mainly a case report.

3 Article Auditory Related Resting State fMRI Functional Connectivity in Tinnitus Patients: Tinnitus Diagnosis Performance. 2018

Minami, Shujiro B / Oishi, Naoki / Watabe, Takahisa / Uno, Kimiichi / Ogawa, Kaoru. ·National Tokyo Medical Center, National Institute of Sensory Organs. · Department of Otolaryngology, Head and Neck Surgery, School of Medicine, Keio University. · Gaienhigashi Clinic, Tokyo, Japan. ·Otol Neurotol · Pubmed #29210942.

ABSTRACT: OBJECTIVE: The purpose of the present study was to investigate functional connectivity in tinnitus patients with and without hearing loss, and design the tinnitus diagnosis performance by resting state functional magnetic resonance imaging (rs-fMRI). SUBJECTS AND METHODS: Nineteen volunteers with normal hearing without tinnitus, 18 tinnitus patients with hearing loss, and 11 tinnitus patients without hearing loss were enrolled in this study. The subjects were evaluated with rs-fMRI, and region of interests (ROIs) based correlation analyses were performed using the CONN toolbox version 16 and SPM version 8. The correlation coefficients from individual level results were converted into beta values. RESULTS: With a beta threshold of more than 0.2, 91% of all possible connections between auditory-related ROIs (Heschl's gyrus, planum temporale, planum polare, operculum, insular cortex, superior temporal gyrus) in the control group remained intact, whereas 83 and 66% of such connections were present in the hearing loss and the normal-hearing tinnitus group. However, between non-auditory-related ROIs, the rates of intact connections at a beta threshold of more than 0.2 were 17% in the control group, and 16 and 15% in the tinnitus groups. When resting state fMRI positive is defined as less than 9% of all possible connections between auditory-related ROIs with a beta threshold of more than 0.7, the sensitivity and specificity of tinnitus diagnosis is 86 and 74%, respectively. CONCLUSIONS: The associations between auditory-related networks are weakened in tinnitus patients, even if they have normal hearing. It is possible that rs-fMRI can be a tool for objective examination of tinnitus, by focusing the auditory-related areas.

4 Article Microbiomes of the normal middle ear and ears with chronic otitis media. 2017

Minami, Shujiro B / Mutai, Hideki / Suzuki, Tomoko / Horii, Arata / Oishi, Naoki / Wasano, Koichiro / Katsura, Motoyasu / Tanaka, Fujinobu / Takiguchi, Tetsuya / Fujii, Masato / Kaga, Kimitaka. ·National Hospital Organization Tokyo Medical Center, National Institute of Sensory Organs, Tokyo, Japan. · Niigata University, Department of Otolaryngology Head and Neck Surgery, Nigata, Japan. · Keio University, School of Medicine, Department of Otolaryngology, Keio, Japan. · Japanese Red Cross Shizuoka Hospital, Department of Otolaryngology, Shizuoka, Japan. · National Hospital Organization Ureshino Medical Center, Department of Otolaryngology, Ureshino, Japan. · National Hospital Organization Nagasaki Medical Center, Department of Otolaryngology, Nagasaki, Japan. · National Hospital Organization Kanazawa Medical Center, Department of Otolaryngology, Kanazawa, Japan. · International University of Health and Welfare, Center for Speech and Hearing Disorders, Tochigi, Japan. ·Laryngoscope · Pubmed #28397271.

ABSTRACT: OBJECTIVE: The aim of this study was to profile and compare the middle ear microbiomes of human subjects with and without chronic otitis media. STUDY DESIGN: Prospective multicenter cohort study. METHODS: All consecutive patients undergoing tympanoplasty surgery for chronic otitis media or ear surgery for conditions other than otitis media were recruited. Sterile swab samples were collected from the middle ear mucosa during surgery. The variable region 4 of the 16S rRNA gene in each sample were amplified using region-specific primers adapted for the Illumina MiSeq sequencer (Illumina, CA, USA)). The sequences were subjected to local blast and classified using Metagenome@KIN (World Fusion, Tokyo, Japan). RESULTS: In total, 155 participants were recruited from seven medical centers. Of these, 88 and 67 had chronic otitis media and normal middle ears, respectively. The most abundant bacterial phyla on the mucosal surfaces of the normal middle ears were Proteobacteria, followed by Actinobacteria, Firmicutes, and Bacteroidetes. The children and adults with normal middle ears differed significantly in terms of middle ear microbiomes. Subjects with chronic otitis media without active inflammation (dry ear) had similar middle ear microbiomes as the normal middle ears group. Subjects with chronic otitis media with active inflammation (wet ear) had a lower prevalence of Proteobacteria and a higher prevalence of Firmicutes than the normal middle ears. CONCLUSION: The human middle ear is inhabited by more diverse microbial communities than was previously thought. Alteration of the middle ear microbiome may contribute to the pathogenesis of chronic otitis media with active inflammation. LEVEL OF EVIDENCE: 2b. Laryngoscope, 127:E371-E377, 2017.

5 Article Clinical characteristics of a Japanese family with hearing loss accompanied by compound heterozygous mutations in LOXHD1. 2016

Minami, Shujiro B / Mutai, Hideki / Namba, Kazunori / Sakamoto, Hirokazu / Matsunaga, Tatsuo. ·National Hospital Organization Tokyo Medical Center, Department of Otolaryngology, 2-5-1 Higashigaoka, Meguroku, Tokyo 152-8901, Japan; National Hospital Organization Tokyo Medical Center, National Institute of Sensory Organs, 2-5-1 Higashigaoka, Meguroku, Tokyo 152-8901, Japan. · National Hospital Organization Tokyo Medical Center, National Institute of Sensory Organs, 2-5-1 Higashigaoka, Meguroku, Tokyo 152-8901, Japan. · Hyogo Prefectural Kobe Children's Hospital, Department of Otolaryngology, 1-1-1 Takakuradai, Sumaku, Koube, Hyogo 654-0091, Japan. · National Hospital Organization Tokyo Medical Center, Department of Otolaryngology, 2-5-1 Higashigaoka, Meguroku, Tokyo 152-8901, Japan; National Hospital Organization Tokyo Medical Center, National Institute of Sensory Organs, 2-5-1 Higashigaoka, Meguroku, Tokyo 152-8901, Japan. Electronic address: matsunagatatsuo@kankakuki.go.jp. ·Auris Nasus Larynx · Pubmed #26973026.

ABSTRACT: OBJECTIVE: To report two novel LOXHD1 mutations, including missense mutations and the clinical features of the patients. METHODS: We studied a three-generation Japanese family with hearing loss. Targeted next-generation sequencing was used for genetic analysis. Conditional orientation response audiometry and pure tone audiometry were used to assess hearing. SWISS-MODEL was used for molecular modeling of the PLAT domain in LOXHD1 protein. RESULTS: The two sisters, who had either mild or severe high-frequency hearing loss, were compound heterozygous for two novel mutations (c.5674G>T [p.V1892F] and c.4212+1G>A) in LOXHD1, which is responsible for autosomal-recessive nonsyndromic hearing loss DFNB77. These cases showed less severe hearing impairment than the previously reported cases carrying LOXHD1 mutations, but their hearing loss appeared to be progressive. Molecular modeling predicted that distorted structure of the PLAT domain in the p.V1892F mutant could lead to decreased affinity of the protein to lipid membrane resulting in hair cell dysfunction. CONCLUSION: We report a Japanese family carrying compound heterozygotes of truncating and nontruncating mutations in LOXHD1 identified by targeted NGS analysis. The fact of lower degree of hearing impairment in our cases than previously reported and the molecular modeling of the missense mutant provide insight to the genotype-phenotype correlation of DFNB77.

6 Article Auditory resting-state functional connectivity in tinnitus and modulation with transcranial direct current stimulation. 2015

Minami, Shujiro B / Oishi, Naoki / Watabe, Takahisa / Uno, Kimiichi / Kaga, Kimitaka / Ogawa, Kaoru. ·a 1 National Institute of Sensory Organs, National Tokyo Medical Center , Tokyo, Japan. · b 2 Department of Otolaryngology, Head and Neck Surgery, School of Medicine, Keio University , Tokyo, Japan. · c 3 Gaienhigashi Clinic , Tokyo, Japan. · d 4 Center for Speech and Hearing Disorders, International University of Health and Welfare , Tokyo, Japan. ·Acta Otolaryngol · Pubmed #26181225.

ABSTRACT: CONCLUSIONS: The functional connectivity (FC) between the right and left auditory cortex is weak in tinnitus patients. Transcranial direct current stimulation (tDCS) over the auditory cortex has potential as a tool to modulate auditory-based FC. OBJECTIVE: This study investigated the effects of applying tDCS in tinnitus patients, and searched for modulation of brain networks in resting-state functional magnetic resonance imaging (rs-fMRI) through an analysis of FC with the stimulated brain region. SUBJECTS AND METHODS: Nine male patients with chronic tinnitus and 10 male volunteers with normal hearing were enrolled. The subjects were evaluated with rs-fMRI immediately before and after tDCS. The tinnitus patients filled out the self-evaluation questionnaires designed to measure tinnitus conditions before tDCS treatment and 1 week afterwards. RESULTS: The FC between the right and left auditory cortex was significantly weaker in tinnitus patients than in controls. After tDCS treatment, in the tinnitus group, the primary auditory cortex showed a reduction in the amount of statistically significant connectivity with the somatosensory area and motor area, but maintained strong significant connectivity (p < 0.005) with the auditory area and insular cortex. In contrast, in the control group, there remained strong significant connectivity between the primary auditory cortex and the somatosensory area, motor area, insular cortex, and auditory area.

7 Article Usefulness of measuring electrically evoked auditory brainstem responses in children with inner ear malformations during cochlear implantation. 2015

Minami, Shujiro B / Takegoshi, Hideki / Shinjo, Yukiko / Enomoto, Chieko / Kaga, Kimitaka. ·National Institute of Sensory Organs, National Tokyo Medical Center , Tokyo , Japan. ·Acta Otolaryngol · Pubmed #26062093.

ABSTRACT: CONCLUSIONS: EABR is a reliable and effective way of objectively confirming device function and implant-responsiveness of the peripheral auditory neurons up to the level of the brainstem in cases of inner ear malformation. OBJECTIVE: To investigate the usefulness of measuring the intra-operative electrically evoked compound action potential (ECAP) and electrically evoked auditory brainstem response (EABR) in patients with and without congenital inner ear anomalies during cochlear implantation. METHOD: Thirty-eight consecutive children (40 ears) aged 5 or younger with congenital profound hearing loss. Twenty-four (25 ears) lacked congenital inner ear anomalies. The 14 patients (15 ears) with a malformation had common cavities (four ears), incomplete partition type I (three ears), cochlea hypoplasia type III (three ears), enlarged vestibular aqueduct (four ears), and cochlear nerve canal stenosis (one ear). Main outcome measures are ECAP and EABR responses. RESULTS: Of the 25 ears lacking any malformation, 21, three, and one showed 'Good', 'Variable', and 'No' ECAP responses, respectively, and 24 and one showed 'Good' and 'Variable' intra-cochlear responses, respectively. Of the 15 ears with a malformation, two showed 'Good' ECAP responses, nine had 'Variable' ECAP responses, and four showed 'No' ECAP responses. Moreover, five showed 'Good' EABR responses and 10 showed 'Variable' EABR responses.

8 Article GJB2-associated hearing loss undetected by hearing screening of newborns. 2013

Minami, Shujiro B / Mutai, Hideki / Nakano, Atsuko / Arimoto, Yukiko / Taiji, Hidenobu / Morimoto, Noriko / Sakata, Hideaki / Adachi, Nodoka / Masuda, Sawako / Sakamoto, Hirokazu / Yoshida, Haruo / Tanaka, Fujinobu / Morita, Noriko / Sugiuchi, Tomoko / Kaga, Kimitaka / Matsunaga, Tatsuo. ·Department of Otolaryngology, National Hospital Organization Tokyo Medical Center, Tokyo, Japan; Laboratory of Auditory Disorders, National Institute of Sensory Organs, National Hospital Organization Tokyo Medical Center, Tokyo, Japan. ·Gene · Pubmed #24013081.

ABSTRACT: The hearing loss caused by GJB2 mutations is usually congenital in onset, moderate to profound in degree, and non-progressive. The objective of this study was to study genotype/phenotype correlations and to document 14 children with biallelic GJB2 mutations who passed newborn hearing screening (NHS). Genetic testing for GJB2 mutations by direct sequencing was performed on 924 individuals (810 families) with hearing loss, and 204 patients (175 families) were found to carry biallelic GJB2 mutations. NHS results were obtained through medical records. A total of 18 pathological mutations were identified, which were subclassified as eight inactivating and 10 non-inactivating mutations. p.I128M and p.H73Y were identified as novel missense GJB2 mutations. Of the 14 children with biallelic GJB2 mutations who passed NHS, eight were compound heterozygotes and 3 were homozygous for the c.235delC mutation in GJB2, and the other three combinations of non-c.235delC mutations identified were p.Y136X-p.G45E/p.V37I heterozygous, c.512ins4/p.R143W heterozygous, and p.V37I/p.R143W heterozygous. These 14 cases demonstrate that the current NHS does not identify all infants with biallelic GJB2 mutations. They suggest that the frequency of non-penetrance at birth is approximately 6.9% or higher in DFNB1 patients and provide further evidence that GJB2 hearing loss may not always be congenital in onset.

9 Article Secondary, profound, sensorineural hearing loss after recovery from haemolytic uraemic syndrome due to enterohaemorrhagic Escherichia coli, and subsequent cochlear implantation, in two Japanese children. 2013

Minami, S B / Takegoshi, H / Shinjo, Y / Kaga, K. ·National Institute of Sensory Organs, National Tokyo Medical Centre, Tokyo, Japan. shujirominami@me.com ·J Laryngol Otol · Pubmed #23406716.

ABSTRACT: OBJECTIVES: To describe two cases of profound hearing loss secondary to enterohaemorrhagic Escherichia coli infection, and to report the efficacy of subsequent cochlear implantation. RESULTS: The first case was a four-year-old girl admitted to hospital with Escherichia coli O157 infection and haemolytic uraemic syndrome. Mild hearing loss was confirmed five months after discharge, progressing to profound loss three months later. At the age of seven years, she underwent cochlear implantation, with remarkable improvement in speech perception and production. The second case was a three-year-old boy admitted with haemolytic uraemic syndrome caused by Escherichia coli O111 infection. One year after disease onset, profound hearing loss was confirmed. Cochlear implantation at the age of five years produced significant recovery of auditory function. CONCLUSION: This study represents the first published report of secondary hearing loss after recovery from haemolytic uraemic syndrome caused by enterohaemorrhagic Escherichia coli. It indicates that cochlear implantation can restore hearing function in such patients.

10 Article Correlations of inflammatory biomarkers with the onset and prognosis of idiopathic sudden sensorineural hearing loss. 2012

Masuda, Masatsugu / Kanzaki, Sho / Minami, Shujiro / Kikuchi, Jun / Kanzaki, Jin / Sato, Hiroaki / Ogawa, Kaoru. ·Department of Otolaryngology, School of Medicine, Keio University, Tokyo, Japan. masoeur13@mac.com ·Otol Neurotol · Pubmed #22872174.

ABSTRACT: HYPOTHESIS: We investigated whether inflammatory biomarkers and stress are involved in the pathophysiology of idiopathic sensorineural hearing loss (ISHL). STUDY DESIGN: Individual cohort study. SETTING: Two tertiary centers. PATIENTS: Forty-three ISHL and 10 non-ISHL patients seen in our ENT departments from 2004 to 2010 within a week from the onset of new symptoms and without steroid administration before visiting our departments. INTERVENTION: Multiple audiologic evaluations, blood tests including leukocyte counts, natural killer cell activity (NKCA), interleukin 6 (IL-6), tumor necrosis factor, high-sensitivity CRP (hCRP), and the General Health Questionnaire were used to evaluate the systemic stress and inflammatory response. MAIN OUTCOME MEASURES: Correlations between biomarkers and ISHL severity and prognosis were evaluated by statistical analysis. RESULTS: In the ISHL patients, a neutrophil count above the reference range was associated with severe hearing loss and poor prognosis, and was accompanied by low NKCA and high IL-6. In the non-ISHL patients, these associations were not present. The abnormal neutrophil count was independent of preexisting vascular diseases. The abnormal counts responded to treatment and decreased into the reference range. CONCLUSION: Neutrophil counts above the reference range of a facility will be a useful indicator of poor prognosis of ISHL. Synchronism of different types of NF-κB activation pathways could be required to cause severe ISHL. An NKCA decrease, an acute neutrophil count increase, and an IL-6 increase can induce NF-κB activation in the cochlea and cause severe ISHL. Further epidemiologic surveys should be conducted to evaluate whether stressful life events increase the risk of severe ISHL onset.

11 Article Comorbidity of GJB2 and WFS1 mutations in one family. 2012

Minami, Shujiro B / Masuda, Sawako / Usui, Satoko / Mutai, Hideki / Matsunaga, Tatsuo. ·NHO Tokyo Medical Center, Department of Otolaryngology, Japan. shujirominami@me.com ·Gene · Pubmed #22498363.

ABSTRACT: It is rarely reported that two distinct genetic mutations affecting hearing have been found in one family. We report on a family exhibiting comorbid mutation of GJB2 and WFS1. A four-generation Japanese family with autosomal dominant sensorineural hearing loss was studied. In 7 of the 24 family members, audiometric evaluations and genetic analysis were performed. We detected A-to-C nucleotide transversion (c.2576G>C) in exon 8 of WFS1 that was predicted to result in an arginine-to-proline substitution at codon 859 (R859P), G-to-A transition (c.109G>A) in exon 2 of GJB2 that was predicted to result in a valine-to-isoleucine substitution at codon 37 (V37I), and C-to-T transition (c.427C>T) in exon 2 of GJB2 that was predicted to result in an arginine-to-tryptophan substitution at codon 143 (R143W). Two individuals who had heterozygosity of GJB2 mutations and heterozygosity of WFS1 mutations showed low-frequency hearing loss. One individual who had homozygosity of GJB2 mutation without WFS1 mutation had moderate, gradual high tone hearing loss. On the other hand, a moderate flat loss configuration was seen in one individual who had compound heterozygosity of GJB2 and heterozygosity of WFS1 mutations. Our results indicate that the individual who has both GJB2 and WFS1 mutations can show GJB2 phenotype.

12 Article Repetitive transcranial magnetic stimulation (rTMS) for treatment of chronic tinnitus. 2011

Minami, Shujiro B / Shinden, Seiichi / Okamoto, Yasuhide / Watada, Yukiko / Watabe, Takahisa / Oishi, Naoki / Kanzaki, Sho / Saito, Hideyuki / Inoue, Yasuhiro / Ogawa, Kaoru. ·National Institute of Sensory Organs, National Tokyo Medical Center, 2-5-1 Higashigaoka, Tokyo, Japan. shujirominami@mac.com ·Auris Nasus Larynx · Pubmed #20971587.

ABSTRACT: OBJECTIVE: There is compelling evidence that tinnitus is associated with functional alterations in the central nervous system. Repetitive transcranial magnetic stimulation (rTMS) is a potent tool for modifying neural activity at the stimulated area and at a distance along the functional anatomical connections. Depending on the stimulation parameters, cortical networks can be functionally disturbed or modulated in their activities. Low-frequency rTMS has been shown to result in a decrease in cortical excitability. The technique can alleviate tinnitus by modulating the excitability of neurons in the auditory cortex. We aimed to investigate the effects of low-frequency rTMS in patients and determine the factors that predict a beneficial outcome with rTMS treatment. METHODS: Sixteen patients (male 10, female 6) with chronic tinnitus underwent low-frequency (1Hz) rTMS (intensity: 110% motor threshold; number of stimuli: 1200) to the left auditory cortex. The treatment outcome was assessed with a visual analog scale (VAS) of loudness, annoyance and duration, loudness balance test, and tinnitus handicap inventory (THI). Therapeutic success was studied according to the patients' clinical characteristics. RESULTS: A significant reduction in the VAS (loudness and annoyance) occurred immediately after rTMS, with a gradual return to pretreatment levels after 7 days. The tinnitus patients with sudden deafness were significant resistant to rTMS treatment compared with those diagnosed with age-related hearing loss. CONCLUSION: These results support the potential of rTMS as a new therapeutic tool for the treatment of chronic tinnitus. Because this study was performed with a small sample size and showed high interindividual variability in treatment effects, further development of the technique is needed before it can be recommended for clinical applications.