Publications by authors named "Yutaka Takumi"

40 Publications

A Multi-Institutional Study of Older Hearing Aids Beginners-A Prospective Single-Arm Observation on Executive Function and Social Interaction.

J Am Med Dir Assoc 2021 06 1;22(6):1168-1174. Epub 2021 Apr 1.

Department of Otolaryngology-Head and Neck Surgery, Nagoya City University, Graduate School of Medical Sciences and Medical School; East Medical Center, Nagoya, Aichi, Japan.

Objectives: To obtain new insights into research questions on how executive function and social interaction would be observed to change after the introduction of hearing aids (HAs) in older people with hearing impairment.

Design: Multi-institutional prospective single-arm observational study.

Setting And Participants: Outpatients with complaints of hearing difficulty who visited HA clinics between October 18, 2017, and June 30, 2019, in 7 different university hospitals in Japan.

Methods: The inclusion criteria of the study named Hearing-Aid Introduction for Hearing-Impaired Seniors to Realize a Productive Aging Society-A Study Focusing on Executive Function and Social Activities Study (HA-ProA study) were age ≥60 years and no history of HA use. A series of multi-institution common evaluations including audiometric measurements, the digit symbol substitution test to assess executive functions, convoy model as an index of social relations, and hearing handicap inventory for the elderly (HHIE) were performed before (pre-HA) and after 6 months of the HA introduction (post-HA).

Results: Out of 127 enrollments, 94 participants completed a 6-month follow-up, with a mean age of 76.9 years. The digit symbol substitution test score improved significantly from 44.7 at baseline to 46.1 at 6 months (P = .0106). In the convoy model, the social network size indicated by the number of persons in each and whole circles were not significantly different between pre- and post-HA; however, the total count for kin was significantly increased (P = .0344). In the analyses of HHIE, the items regarding the family and relatives showed significant improvement.

Conclusions And Implications: HA use could benefit older individuals beginning to use HAs in executive function and social interaction, though the results should be interpreted cautiously given methodological limitations such as a single-arm short 6 months observation. Reduction in daily hearing impairment would have a favorable effect on relationships with the family.
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http://dx.doi.org/10.1016/j.jamda.2021.02.035DOI Listing
June 2021

The iArmS Robotic Armrest Prolongs Endoscope Lens-Wiping Intervals in Endoscopic Sinus Surgery.

Surg Innov 2020 Oct 30;27(5):515-522. Epub 2020 Jun 30.

Institute of Advanced Biomedical Engineering and Science, 13131Tokyo Women's Medical University, Japan.

. Fouling of the endoscope lens is a major problem in endoscopic sinus surgery (ESS). We examined whether the use of the intelligent arm support system (iArmS), a robotic armrest, could prolong endoscope lens-wiping intervals in ESS and thus allow for continuously clear endoscopic images. . This study is a prospective, nonrandomized crossover study. . Three surgeons who performed ESS at 2 centers each conducted 3 operations with the iArmS and 3 operations without the iArmS; thus, 18 operations were assessed. To blind the assessments, we performed them prospectively without informing subjects of the endpoints. We recorded the operations and observed the recordings at a later date; endoscope lens-wiping times were noted in seconds to determine the endoscope lens-wiping intervals. Our examination was based on the null hypothesis that endoscope lens-wiping intervals would not differ according to the use or nonuse of the iArmS. . The median endoscope lens-wiping intervals with and without using the iArmS were 361 seconds and 135 seconds, respectively. Based on the Wilcoxon rank-sum test, this difference was significant ( = 0.001); thus, the null hypothesis was rejected. This result indicated that endoscope lens-wiping intervals are greatly prolonged by the use of the iArmS. . The iArmS robotic armrest is suitable for ESS, prolongs endoscope lens-wiping intervals, and facilitates obtaining continuous clear endoscopic images.
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http://dx.doi.org/10.1177/1553350620929864DOI Listing
October 2020

Comprehensive analysis of syndromic hearing loss patients in Japan.

Sci Rep 2019 08 19;9(1):11976. Epub 2019 Aug 19.

Department of Otorhinolaryngology - Head and Neck Surgery, Kyoto Prefectural University of Medicine, Kyoto, Japan.

More than 400 syndromes associated with hearing loss and other symptoms have been described, corresponding to 30% of cases of hereditary hearing loss. In this study we aimed to clarify the mutation spectrum of syndromic hearing loss patients in Japan by using next-generation sequencing analysis with a multiple syndromic targeted resequencing panel (36 target genes). We analyzed single nucleotide variants, small insertions, deletions and copy number variations in the target genes. We enrolled 140 patients with any of 14 syndromes (BOR syndrome, Waardenburg syndrome, osteogenesis imperfecta, spondyloepiphyseal dysplasia congenita, Stickler syndrome, CHARGE syndrome, Jervell and Lange-Nielsen syndrome, Pendred syndrome, Klippel-Feil syndrome, Alport syndrome, Norrie disease, Treacher-Collins syndrome, Perrault syndrome and auditory neuropathy with optic atrophy) and identified the causative variants in 56% of the patients. This analysis could identify the causative variants in syndromic hearing loss patients in a short time with a high diagnostic rate. In addition, it was useful for the analysis of the cases who only partially fulfilled the diagnostic criteria.
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http://dx.doi.org/10.1038/s41598-019-47141-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6700179PMC
August 2019

Laser-capture micro dissection combined with next-generation sequencing analysis of cell type-specific deafness gene expression in the mouse cochlea.

Hear Res 2017 05 3;348:87-97. Epub 2017 Mar 3.

Department of Otorhinolaryngology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto 390-8621, Japan; Department of Hearing Implant Sciences, Shinshu University School of Medicine 3-1-1 Asahi, Matsumoto 390-8621, Japan. Electronic address:

Cochlear implantation (CI), which directly stimulates the cochlear nerves, is the most effective and widely used medical intervention for patients with severe to profound sensorineural hearing loss. The etiology of the hearing loss is speculated to have a major influence of CI outcomes, particularly in cases resulting from mutations in genes preferentially expressed in the spiral ganglion region. To elucidate precise gene expression levels in each part of the cochlea, we performed laser-capture micro dissection in combination with next-generation sequencing analysis and determined the expression levels of all known deafness-associated genes in the organ of Corti, spiral ganglion, lateral wall, and spiral limbs. The results were generally consistent with previous reports based on immunocytochemistry or in situ hybridization. As a notable result, the genes associated with many kinds of syndromic hearing loss (such as Clpp, Hars2, Hsd17b4, Lars2 for Perrault syndrome, Polr1c and Polr1d for Treacher Collins syndrome, Ndp for Norrie Disease, Kal for Kallmann syndrome, Edn3 and Snai2 for Waardenburg Syndrome, Col4a3 for Alport syndrome, Sema3e for CHARGE syndrome, Col9a1 for Sticker syndrome, Cdh23, Cib2, Clrn1, Pcdh15, Ush1c, Ush2a, Whrn for Usher syndrome and Wfs1 for Wolfram syndrome) showed higher levels of expression in the spiral ganglion than in other parts of the cochlea. This dataset will provide a base for more detailed analysis in order to clarify gene functions in the cochlea as well as predict CI outcomes based on gene expression data.
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http://dx.doi.org/10.1016/j.heares.2017.02.017DOI Listing
May 2017

Correlation Between White Matter Lesions and Intelligence Quotient in Patients With Congenital Cytomegalovirus Infection.

Pediatr Neurol 2016 Feb 2;55:52-7. Epub 2015 Dec 2.

Department of Pediatrics, Shinshu University School of Medicine, Matsumoto, Japan.

Background: It is well known that congenital cytomegalovirus infection exhibits white matter and other types of lesions in magnetic resonance imaging (MRI), but little is known on the clinical significance of white matter lesions because they are also present in asymptomatic congenital cytomegalovirus infection. We investigated for relationships among white matter lesions, intelligence quotient, and other neurodevelopmental features.

Methods: Nine children (five boys and four girls; mean age: 87.4 months, range: 63-127 months) with sensorineural hearing loss (five bilateral and four unilateral) had been diagnosed as having congenital cytomegalovirus infection by positive polymerase chain reaction findings of dried umbilical cords. They were evaluated for the presence of autistic features, tested using Wechsler Intelligence Scale for Children-Fourth Edition for intelligence quotient, and underwent brain MRI to measure white matter lesion localization and volume.

Results: At the time of MRI examination (mean age: 69.4 months, range: 19-92 months), white matter lesions were detected in eight of nine patients. Five subjects were diagnosed as having autism spectrum disorders. We observed increased white matter lesion volume was associated with lower intelligence quotient scores (R(2) = 0.533, P = 0.026) but not with autism spectrum disorders.

Conclusions: In individuals with congenital cytomegalovirus, an increased white matter lesion volume is associated with lower intelligence quotient scores but not with an increased likelihood of autistic behavior.
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http://dx.doi.org/10.1016/j.pediatrneurol.2015.11.014DOI Listing
February 2016

Discrimination of Japanese monosyllables in patients with high-frequency hearing loss.

Auris Nasus Larynx 2016 Jun 19;43(3):269-80. Epub 2015 Nov 19.

Department of Otolaryngology, University of Tokyo, Japan.

Objective: To analyze the difficulty of discriminating Japanese nonsense monosyllables in each of several grades of high-frequency hearing loss and to evaluate the limitations of amplification.

Methods: We collected retrospective data on the discrimination of Japanese nonsense monosyllables by patients with three grades of high-frequency hearing loss who fulfilled or nearly fulfilled the Japanese criteria for EAS. Discrimination of the twenty monosyllables included in the 67-S speech audiometric test, which is approved by the Japan Audiological Society, was evaluated under quiet conditions.

Results: One hundred and five ears of ninety-one adults with high-frequency hearing loss were tested. We classified the ears according to hearing threshold at 1000 Hz; Group 1: <45 dB; Group 2: ≥45 dB and <70 dB; Group 3: ≥70 dB. Under the best conditions, the best speech discrimination scores were 72.3 ± 18.6% (mean ± SD, N=11), 56.9 ± 19.9% (N=57) and 38.1 ± 22.6% (N=37) in Group 1, Group 2 and Group 3, respectively. For most of the monosyllables, discrimination score declined gradually as high-frequency hearing loss became more severe. The high incidence in the Japanese language of [k], an easy consonant to distinguish, may be an advantage for patients with high-frequency hearing loss who use hearing aids. By employing a new confusion matrix that displays consonants and the following vowels separately, we were able to reveal the interactions of those two components. We observed that discrimination of preceding nasal consonants and that of the following vowels were not independent in patients with high-frequency hearing loss.

Conclusion: Our classification based on threshold at 1000 Hz was useful to predict the effectiveness and limitations of amplification in high-frequency hearing loss. Threshold at 1000 Hz can be an index enabling us to refine the indications of EAS for native Japanese speakers to maximize its effectiveness against high-frequency hearing loss.
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http://dx.doi.org/10.1016/j.anl.2015.10.003DOI Listing
June 2016

Silicone impression material foreign body in the middle ear: Two case reports and literature review.

Auris Nasus Larynx 2015 Oct 5;42(5):419-23. Epub 2015 May 5.

Department of Otorhinolaryngology, Shinshu University School of Medicine, 3-1-1, Asahi, Matsumoto 390-8621, Japan. Electronic address:

We report two cases of impression material foreign body in the middle ear. The first case had been affected with chronic otitis media. The silicone flowed into the middle ear through a tympanic membrane perforation during the process of making an ear mold. About 4 years and 8 months after, the patient had severe vertigo and deafness. We found bone erosion of the prominence of the lateral semicircular canal and diagnosed labyrinthitis caused by silicone impression material. In the second case silicone flowed into the canal wall down mastoid cavity. Both cases required surgery to remove the foreign body. The clinical courses in such cases are variable and timing of surgery is sometimes difficult. In addition to reporting these two cases, we present here a review of the literature regarding impression material foreign bodies.
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http://dx.doi.org/10.1016/j.anl.2015.03.010DOI Listing
October 2015

[Hearing disorders of infants and young children].

Authors:
Yutaka Takumi

Nihon Jibiinkoka Gakkai Kaiho 2014 Oct;117(10):1288-91

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October 2014

Non-ocular Stickler syndrome with a novel mutation in COL11A2 diagnosed by massively parallel sequencing in Japanese hearing loss patients.

Ann Otol Rhinol Laryngol 2015 May 16;124 Suppl 1:111S-7S. Epub 2015 Mar 16.

Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Japan Department of Hearing Implant Sciences, Shinshu University School of Medicine, Matsumoto, Japan

Objectives: This study aims to document the clinical features of patients with COL11A2 mutations and to describe the usefulness of massively parallel sequencing.

Methods: One thousand one hundred twenty (1120) Japanese hearing loss patients from 53 ENT departments nationwide participated in this study. Massively parallel sequencing of 63 genes implicated in hearing loss was performed to identify the genetic causes in the Japanese hearing loss patients.

Results: A novel mutation in COL11A2 (c.3937_3948delCCCCCAGGGCCA) was detected in an affected family, and it was segregated in all hearing loss individuals. The clinical findings of this family were compatible with non-ocular Stickler syndrome. Orofacial features of mid-facial hypoplasia and slowly progressive mild to moderate hearing loss were also presented. Audiological examinations showed favorable auditory performance with hearing aid(s).

Conclusion: This is the first case report of the genetic diagnosis of a non-ocular Stickler syndrome family in the Japanese population. We suggest that it is important to take both genetic analysis data and clinical symptoms into consideration to make an accurate diagnosis.
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http://dx.doi.org/10.1177/0003489415575044DOI Listing
May 2015

Germinal mosaicism in a family with BO syndrome.

Ann Otol Rhinol Laryngol 2015 May 16;124 Suppl 1:118S-22S. Epub 2015 Mar 16.

Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Japan Department of Hearing Implant Sciences, Shinshu University School of Medicine, Matsumoto, Japan

Objectives: To clarify the existence of germinal mosaicism, we performed a genetic analysis of 2 siblings identified with an EYA1 mutation associated with branchiooto (BO) syndrome but who were born from normal parents.

Methods: Detailed data from the 2 affected siblings were collected for clinical diagnosis, with haplotype analysis also performed to prove germinal mosaicism.

Results: The 2 sisters showed characteristic clinical features of BO syndrome (middle and inner ear anomalies, microtia, and auditory canal stenosis/atresia). Haplotype analysis confirmed the genetic relationship between the affected sisters and their parents. The younger sister with auditory canal atresia received a bone-anchored hearing aid (Baha), a transcutaneous bone conduction hearing device, resulting in a good hearing outcome.

Conclusions: Based on the results of haplotype analysis, we proved that the BO syndrome in these cases was caused by germinal mosaicism of the EYA1 gene in either the mother or father. We also demonstrated that the bone-conduction hearing implant is a good option for BO patients with complex outer, middle, and inner ear anomalies.
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http://dx.doi.org/10.1177/0003489415575062DOI Listing
May 2015

Template-guided implantation of the Bonebridge: clinical experience.

Eur Arch Otorhinolaryngol 2015 Dec 19;272(12):3669-75. Epub 2014 Dec 19.

Department of Otorhinolaryngology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi Higashi-ku, Fukuoka, 812-8582, Japan.

The surgical procedure for Bonebridge implantation cannot be done in some cases without exposing the dura mater or sigmoid sinus. Surgical simulation technology can help to identify such difficulties prior to surgery and be used to clarify the optimal location and orientation of the device to be implanted. However, there has not been a simple strategy to drill the temporal bone at exactly the same location as that simulated on the computer. Based on our previous development of the surface template-assisted marker positioning (STAMP) method for performing image-guided otologic surgery, we recently developed a noninvasive guiding method, the BB-STAMP method, for performing image-guided Bonebridge implantation. Three patients underwent Bonebridge implantation at our surgical center during the years of 2013-2014. The authors in the simulation center supported the surgery using the BB-STAMP method. The time and effort required to prepare for the surgery were evaluated. In addition, a postoperative analysis was performed to assess the accuracy of placing the device in the planned location. The BB-STAMP method enabled the surgeon to precisely replicate the computer simulation in the real patient with submillimetric accuracy without complexity. Thus, the use of experienced and elaborative simulation coupled with the creation of a tailor-made three-dimensional template (BB-STAMP) enables surgeons to perform quick, precise and safe surgical procedures at distant institutions.
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http://dx.doi.org/10.1007/s00405-014-3452-1DOI Listing
December 2015

Gene expression pattern after insertion of dexamethasone-eluting electrode into the guinea pig cochlea.

PLoS One 2014 20;9(10):e110238. Epub 2014 Oct 20.

Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Japan.

A cochlear implant is an indispensable apparatus for a profound hearing loss patient. But insertion of the electrode entails a great deal of stress to the cochlea, and may cause irreversible damage to hair cells and related nerve structure. Although damage prevention effects of dexamethasone have been reported, long-term administration is difficult. In this study, we used a dexamethasone-eluting electrode in the guinea pig cochlea, and compared the gene expression after 7 days insertion with that of a normal electrode and non-surgically treated control by microarray. 40 genes were up-regulated 2-fold or more in the normal electrode group compared to the non-surgically treated group. Most of the up-regulated genes were associated with immune response and inflammation. In the dexamethasone-eluting group, compared to the normal electrode group, 7 of the 40 genes were further up-regulated, while 12 of them were down-regulated and there was a tendency to return to the non-surgical condition. 9 genes were down-regulated 2-fold or less with normal electrode insertion, and 4 of the 9 tended to return to the non-surgical condition in the dexamethasone-eluting group. These genes are certainly involved in the maintenance of the physiological functions of the cochlea. Our results indicate that the dexamethasone-eluting electrode will have an effect on the normalization of homeostasis in the cochlea.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0110238PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4203767PMC
July 2015

A clinical experience of 'STAMP' plate-guided Bonebridge implantation.

Acta Otolaryngol 2014 Oct 13;134(10):1042-6. Epub 2014 Aug 13.

Department of Otorhinolaryngology.

Conclusion: The surface template-assisted marker positioning (STAMP) method is useful for successful Bonebridge™ (BB) implantation on a planned site while avoiding dangerous positions.

Objectives: To confirm the usefulness of the STAMP method for the safe operation of BB.

Methods: From a patient's temporal bone CT data, a guide plate and confirmation plate were generated by the STAMP method. The guide plate is used to mark the correct place for implantation, while the confirmation plate lets us know the correct angle and depth of the hole.

Results: With the guide plate, the correct place for BB implantation was easily found. The hole was made to be an appropriate size with the confirmation plate while exposing only a small part of sigmoid sinus as simulated. Finally, the BB implant was successfully placed exactly at the planned site.
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http://dx.doi.org/10.3109/00016489.2014.900703DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4196550PMC
October 2014

Hearing preservation and clinical outcome of 32 consecutive electric acoustic stimulation (EAS) surgeries.

Acta Otolaryngol 2014 Jul 19;134(7):717-27. Epub 2014 May 19.

Department of Otorhinolaryngology and.

Conclusions: Our results indicated that electric acoustic stimulation (EAS) is beneficial for Japanese-speaking patients, including those with less residual hearing at lower frequencies. Comparable outcomes for the patients with less residual hearing indicated that current audiological criteria for EAS could be expanded. Successful hearing preservation results, together with the progressive nature of loss of residual hearing in these patients, mean that minimally invasive full insertion of medium/long electrodes in cochlear implantation (CI) surgery is a desirable solution. The minimally invasive concepts that have been obtained through EAS surgery are, in fact, crucial for all CI patients.

Objectives: This study was conducted to evaluate hearing preservation results and speech discrimination outcomes of hearing preservation surgeries using medium/long electrodes.

Methods: A total of 32 consecutive minimally invasive hearing preservation CIs (using a round window approach with deep insertion of a flexible electrode) were performed in 30 Japanese patients (two were bilateral cases), including patients with less residual hearing. Hearing preservation rates as well as speech discrimination/perception scores were investigated on a multicenter basis.

Results: Postoperative evaluation after full insertion of the flexible electrodes (24 mm, 31.5 mm) showed that residual hearing was well preserved in all 32 ears. In all patients, speech discrimination and perception scores were improved postoperatively.
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http://dx.doi.org/10.3109/00016489.2014.894254DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4086239PMC
July 2014

Deafness gene expression patterns in the mouse cochlea found by microarray analysis.

PLoS One 2014 27;9(3):e92547. Epub 2014 Mar 27.

Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Nagano, Japan.

Background: Tonotopy is one of the most fundamental principles of auditory function. While gradients in various morphological and physiological characteristics of the cochlea have been reported, little information is available on gradient patterns of gene expression. In addition, the audiograms in autosomal dominant non syndromic hearing loss can be distinctive, however, the mechanism that accounts for that has not been clarified. We thought that it is possible that tonotopic gradients of gene expression within the cochlea account for the distinct audiograms.

Methodology/principal Findings: We compared expression profiles of genes in the cochlea between the apical, middle, and basal turns of the mouse cochlea by microarray technology and quantitative RT-PCR. Of 24,547 genes, 783 annotated genes expressed more than 2-fold. The most remarkable finding was a gradient of gene expression changes in four genes (Pou4f3, Slc17a8, Tmc1, and Crym) whose mutations cause autosomal dominant deafness. Expression of these genes was greater in the apex than in the base. Interestingly, expression of the Emilin-2 and Tectb genes, which may have crucial roles in the cochlea, was also greater in the apex than in the base.

Conclusions/significance: This study provides baseline data of gradient gene expression in the cochlea. Especially for genes whose mutations cause autosomal dominant non syndromic hearing loss (Pou4f3, Slc17a8, Tmc1, and Crym) as well as genes important for cochlear function (Emilin-2 and Tectb), gradual expression changes may help to explain the various pathological conditions.
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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0092547PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3967995PMC
December 2015

Hearing handicap in adults with unilateral deafness and bilateral hearing loss.

Otol Neurotol 2013 Jun;34(4):644-9

Department of Hearing Implant Science, Shinshu University School of Medicine, Matsumoto City, Nagano, Japan.

Objective: To assess the perception of hearing handicap in adult patients with unilateral sudden sensorineural hearing loss (SNHL) compared with those with bilateral SNHL or unilateral congenital SNHL.

Study Design: Retrospective chart review.

Setting: Multicenter department of otolaryngology referrals.

Patients: Seventy-one subjects in the unilateral severe-profound (>70 dB) sudden SNHL group (Group 1), 17 subjects in the unilateral prelingual or congenital SNHL group (Group 2), and 121 subjects in the bilateral SNHL group (Group 3).

Interventions: Questionnaire.

Main Outcome Measures: Hearing Handicap Inventory for Adults (HHIA) and visual analogue scale (VAS) measurements of hearing handicap.

Results: Average levels of hearing loss were 92 dB in Group 1, 109 dB in Group 2, and 67 dB in Group 3. The relative percentage scores of HHIA and VAS compared with Group 3 were 72.6% and 81.0% in Group 1 and 25.4% and 50.3% in Group 2, respectively. A mild correlation between the HHIA subscale or VAS scores and degree of hearing loss could be found in Group 3. No significant correlation was found between the HHIA subscale or VAS scores and duration of hearing loss in Group 1 or Group 3. Higher scores were obtained in male subjects than in female subjects. Patients in Group 1 who were troubled by tinnitus scored significantly higher in the HHIA. In multiple logistic regression analysis, presence of tinnitus, older age, higher average hearing loss level, and group (bilateral SNHL>unilateral sudden SNHL>unilateral precongenital SNHL) revealed a significant positive association with high score (>42) of HHIA (odds ratio, 3.171, 1.021, 1.031, and 6.690, respectively).

Conclusion: The results of HHIA and VAS suggest that not only patients with bilateral SNHL but also those with unilateral sudden SNHL, particularly those who have tinnitus, experience a hearing handicap.
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http://dx.doi.org/10.1097/MAO.0b013e318287f1feDOI Listing
June 2013

[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].

Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2012 Oct;26(19):893-6

Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Japan.

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October 2012

An Usher syndrome type 1 patient diagnosed before the appearance of visual symptoms by MYO7A mutation analysis.

Int J Pediatr Otorhinolaryngol 2013 Feb 11;77(2):298-302. Epub 2012 Dec 11.

Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Japan.

Usher syndrome type 1 (USH1) appears to have only profound non-syndromic hearing loss in childhood and retinitis pigmentosa develops in later years. This study examined the frequency of USH1 before the appearance of visual symptoms in Japanese deaf children by MYO7A mutation analysis. We report the case of 6-year-old male with profound hearing loss, who did not have visual symptoms. The frequency of MYO7A mutations in profound hearing loss children is also discussed. We sequenced all exons of the MYO7A gene in 80 Japanese children with severe to profound non-syndromic HL not due to mutations of the GJB2 gene (ages 0-14 years). A total of nine DNA variants were found and six of them were presumed to be non-pathogenic variants. In addition, three variants of them were found in two patients (2.5%) with deafness and were classified as possible pathogenic variants. Among them, at least one nonsense mutation and one missense mutation from the patient were confirmed to be responsible for deafness. After MYO7A mutation analysis, the patient was diagnosed with RP, and therefore, also diagnosed with USH1. This is the first case report to show the advantage of MYO7A mutation analysis to diagnose USH1 before the appearance of visual symptoms. We believed that MYO7A mutation analysis is valid for the early diagnosis of USH1.
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http://dx.doi.org/10.1016/j.ijporl.2012.11.007DOI Listing
February 2013

[Problem and assignment for distinguishing the Usher syndrome type].

Nihon Jibiinkoka Gakkai Kaiho 2012 Oct;115(10):894-901

Department of Hearing Implant Sciences, Shinshu University, Matsumoto.

Usher syndrome is an autosomal-recessive disorder that causes bilateral sensorineural hearing loss, retinitis pigmentosa (RP), and occasionally vestibular dysfunction. Usher syndrome types 1, 2, and 3 can be distinguished by differences in audiovestibular features. The objectives of this retrospective study were to evaluate 26 patients with Usher syndrome clinically. The 26 patients (male: 12 cases, female: 14 cases) with Usher syndrome, with a clinical diagnosis based on symptoms of bilateral sensorineural hearing loss and RP, had been registered from 13 hospitals as a multicenter study. We assessed the clinical history and performed audiovestibular and ophthalmologic examinations, and genetic testing. Eleven of the patients were classified as having Usher type 1 (38.5%), 6 with Usher type 2 (23.1%), and 9 with Usher type 3 (38.5%). However, many patients with atypical Usher type 1 (70%) and type 2 (83.3%) were found compared with Usher type 3 (10%). The conductive rate of vestibular examinations including the caloric test (50%) was low. There were many variations in the clinical symptoms in Usher syndrome patients, therefore the classification of Usher types 1, 2, and 3 has been complicated. We have proposed a flowchart for the diagnosis of Usher types 1, 2, and 3.
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http://dx.doi.org/10.3950/jibiinkoka.115.894DOI Listing
October 2012

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

J Hum Genet 2012 Sep 21;57(9):587-92. Epub 2012 Jun 21.

Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Japan.

TECTA gene encodes α-tectorin, the major component of noncollagenous glycoprotein of the tectorial membrane, and has a role in intracochlear sound transmission. The TECTA mutations are one of the most frequent causes of autosomal dominant (AD) hearing loss and genotype-phenotype correlations are associated with mutations of TECTA in exons according to α-tectorin domains. In this study, we investigated the prevalence of hearing loss caused by TECTA mutations in Japanese AD hearing loss families, and confirmed genotype-phenotype correlation, as well as the intracellular localization of missense mutations in the α-tectorin domain. TECTA mutations were detected in 2.9% (4/139) of our Japanese AD hearing loss families, with the prevalence in moderate hearing loss being 7.7% (4/52), and all patients showed typical genotype-phenotype correlations as previously described. The present in vitro study showed differences of localization patterns between wild type and mutants, and suggested that each missense mutation may lead to a lack of assembly of secretion, and may reduce the incorporation of α-tectorin into the tectorial membrane.
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http://dx.doi.org/10.1038/jhg.2012.73DOI Listing
September 2012

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

Acta Otolaryngol 2012 Apr;132(4):377-84

Department of Otorhinolaryngology, Shinshu University School of Medicine, Asahi, Matsumoto, Japan.

Conclusions: CDH23 mutations and the 1555A>G mitochondrial mutation were identified among our series of electric acoustic stimulation (EAS) patients, confirming that these genes were important in hearing loss with involvement of high frequency. Successful hearing preservation as well as good outcomes from EAS indicated that patients with this combination of mutations are good candidates for EAS.

Objectives: Screening for gene mutations that possibly cause hearing loss involving high frequency was performed to identify the responsible genes in patients with EAS. In addition to a review of the genetic background of the patients with residual hearing loss, the benefit of EAS for patients with particular gene mutations was evaluated.

Methods: Eighteen patients (15 late-onset, 3 early-onset) with residual hearing who had received EAS were included in this study. Genetic analysis was performed to identify GJB2, CDH23, SLC26A4, and the 1555 mitochondrial mutations.

Results: Three early-onset patients had CDH23 mutations. One late-onset patient had the 1555 A>G mitochondrial mutation.
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http://dx.doi.org/10.3109/00016489.2011.649493DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3335139PMC
April 2012

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

Acta Otolaryngol 2012 Jun 4;132(6):676-82. Epub 2012 Mar 4.

Department of Hearing Implant Sciences, Shinshu University School of Medicine, Matsumoto city, Japan.

Abstract Usage of the Vibrant Soundbridge (VSB) with round window (RW)-Coupler placement at the RW has been shown to successfully treat mixed hearing loss. Coupling between the VSB's floating mass transducer (FMT) and the RW membrane is difficult in the case of sclerosis in the RW and drilling down the bony lip until the RW membrane can be seen completely can possibly induce a perilymphatic fistula. A 68-year-old woman who had bilateral mixed hearing loss with sclerosis in the RW due to tympanosclerosis underwent a RW-Vibroplasty with a RW-Coupler. Speech discrimination scores in quiet and noise and functional gain with the VSB with RW-Coupler were better than those using a conventional hearing aid. The results of the present case have shown the feasibility of implanting a VSB with RW-Coupler in patients with mixed hearing loss due to tympanosclerosis.
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http://dx.doi.org/10.3109/00016489.2011.649492DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3490479PMC
June 2012

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

Int J Pediatr Otorhinolaryngol 2012 Mar 26;76(3):433-8. Epub 2012 Jan 26.

Department of Hearing Implant Sciences, Shinshu University School of Medicine, Japan.

Objectives: This study aimed to investigate a wide variety of factors that influence auditory, speech, and language development following pediatric cochlear implantation (CI).

Study Design: Prospective collection of language tested data in profound hearing-impaired children.

Hypothesis: Pediatric CI can potentially be effective to development of practical communication skills and early implantation is more effective.

Methods: We proposed a set of language tests (assessment package of the language development for Japanese hearing-impaired children; ALADJIN) consisting of communication skills testing (test for question-answer interaction development; TQAID), comprehensive (Peabody Picture Vocabulary Test-Revised; PVT-R and Standardized Comprehension Test for Abstract Words; SCTAW) and productive vocabulary (Word Fluency Test; WFT), and comprehensive and productive syntax (Syntactic processing Test for Aphasia; STA). Of 638 hearing-impaired children recruited for this study, 282 (44.2%) with >70 dB hearing impairment had undergone CI. After excluding children with low birth weight (<1800 g), those with >11 points on the Pervasive Developmental Disorder ASJ Rating Scale for the test of autistic tendency, and those <2 SD on Raven's Colored Progressive Matrices for the test of non-verbal intelligence, 190 children were subjected to this set of language tests.

Results: Sixty children (31.6%) were unilateral CI-only users, 128 (67.4%) were CI-hearing aid (HA) users, and 2 (1.1%) were bilateral CI users. Hearing loss level of CI users was significantly (p<0.01) worse than that of HA-only users. However, the threshold level, maximum speech discrimination score, and speech intelligibility rating in CI users were significantly (p<0.01) better than those in HA-only users. The scores for PVT-R (p<0.01), SCTAW, and WFT in CI users were better than those in HA-only users. STA and TQAID scores in CI-HA users were significantly (p<0.05) better than those in unilateral CI-only users. The high correlation (r=0.52) has been found between the age of CI and maximum speech discrimination score. The scores of speech and language tests in the implanted children before 24 months of age have been better than those in the implanted children after 24 months of age.

Conclusions: We could indicate that CI was effective for language development in Japanese hearing-impaired children and early CI was more effective for productive vocabulary and syntax.
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http://dx.doi.org/10.1016/j.ijporl.2011.12.027DOI Listing
March 2012

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.

Acta Otolaryngol 2012 Feb 27;132(2):141-5. Epub 2011 Dec 27.

Department of Otorhinolaryngology, Shinshu University School of Medicine, 3-1-1 Asahi, Matsumoto, Japan.

Conclusion: 3 T MRI after intratympanic injection of gadolinium-based contrast agent (GBCA) is more useful for the diagnosis of endolymphatic hydrops compared with the glycerol test and electrocochleography (ECoG).

Objective: To investigate the relationship between 3 T MRI after intratympanic injection of GBCA, the glycerol test, and ECoG in patients with Meniere's disease (MD).

Methods: A total of 20 patients with MD were evaluated. Diluted gadodiamide (a gadolinium-based contrast agent) was administered to the bilateral tympanic cavity by injection through the tympanic membrane. After 24 h, the endolymphatic hydrops was evaluated by a 3.0 T MR scanner. To investigate cochlear hydrops, the glycerol test and ECoG were carried out in all patients.

Results: A positive result was observed in 11 patients (55%) in the glycerol test and in 12 patients (60%) by ECoG. The incidence of positive findings when evaluating the same patients with both the glycerol test and ECoG increased to 75%. Nineteen of 20 (95%) patients showed positive results for 3 T MRI.
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http://dx.doi.org/10.3109/00016489.2011.635383DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3490481PMC
February 2012

Different cortical metabolic activation by visual stimuli possibly due to different time courses of hearing loss in patients with GJB2 and SLC26A4 mutations.

Acta Otolaryngol 2011 Nov 5;131(11):1232-6. Epub 2011 Jul 5.

Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Japan.

Conclusion: We have demonstrated differences in cortical activation with language-related visual stimuli in patients who were profoundly deafened due to genetic mutations in GJB2 and SLC26A4. The differences in cortical processing patterns between these two cases may have been influenced by the differing clinical courses and pathogenesis of hearing loss due to genetic mutations. Our results suggest the importance of hearing during early childhood for the development of a normal cortical language network.

Objectives: To investigate the cortical activation with language-related visual stimuli in patients who were profoundly deafened due to genetic mutations in GJB2 and SLC26A4.

Methods: The cortical activity of two adult patients with known genetic mutations (GJB2, SLC26A4) was evaluated with fluorodeoxyglucose-positron emission tomography (FDG-PET) with a visual language task and compared with that of normal-hearing controls.

Results: A patient with a GJB2 mutation showed activation in the right auditory association area [BA21, BA22], and the left auditory association area [BA42] even with visual language task; in contrast, a patient with an SLC26A4 mutation showed no significant activation in the corresponding area.
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http://dx.doi.org/10.3109/00016489.2011.593719DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3490483PMC
November 2011

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

Acta Otolaryngol 2011 Apr 5;131(4):405-12. Epub 2011 Jan 5.

Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Japan.

Conclusions: With full insertion with a long electrode, hearing preservation can be achieved even in the presence of a long electrode covering the residual hearing region.

Objectives: Advances in developing new atraumatic concepts of electrode design as well as surgical technique have enabled hearing preservation after cochlear implantation surgery, and EAS (electric acoustic stimulation) accompanied with hearing preservation is a new trend for patients with residual hearing at the lower frequencies. However, full insertion with a long/medium electrode and hearing preservation is still a challenging field that calls for discussion.

Method: In this study, round window insertion, an atraumatic electrode, and dexamethasone administration were used and atraumaticity (hearing preservation and conservation of vestibular function) was evaluated with full insertion of the electrode.

Results: Postoperative evaluation after full insertion of the electrodes showed that hearing at low frequencies was well preserved in all five cases. Combined postoperative imaging with the referential tonotopic map confirmed achievement of full insertion and indicated the corresponding frequencies and the depth of the electrode. Achievement of atraumaticity of round window insertion in the present cases was confirmed from the viewpoint of the minimal drilling time as well as the preserved vestibular function.
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http://dx.doi.org/10.3109/00016489.2010.539266DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3490476PMC
April 2011

Endolymphatic hydrops and therapeutic effects are visualized in 'atypical' Meniere's disease.

Acta Otolaryngol 2009 Nov;129(11):1326-9

Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Japan.

A 53-year-old male with fluctuating low frequency sensorineural hearing loss and tinnitus, but without vertigo, was evaluated by MRI obtained by intratympanic injection of a gadolinium-based contrast agent (GBCA) before and after the administration of isosorbide. The endolymphatic hydrops was semi-quantitatively evaluated by a 3.0-T MR scanner. For quantification, the affected side/contralateral side ratios were calculated. A gadodiamide (a kind of GBCA)-enhanced space surrounding the endolymph in the affected side with a 0.50 ratio (which may have represented endolymphatic hydrops) improved after isosorbide therapy to a 0.98 ratio. Thus, endolymphatic hydrops was demonstrated in a patient with 'atypical' Meniere's disease (MD), suggesting that at least some atypical MD may share similar etiology with, and therefore be a continuum of, MD. Also, therapeutic effects could be visualized by using MRI. Therefore, MRI-based diagnosis of MD-related disease will be a powerful tool not only because of its precision but also its usefulness for therapeutic evaluation.
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http://dx.doi.org/10.3109/00016480802593513DOI Listing
November 2009

Semi-quantitative evaluation of endolymphatic hydrops by bilateral intratympanic gadolinium-based contrast agent (GBCA) administration with MRI for Meniere's disease.

Acta Otolaryngol 2010 ;130(1):10-6

Department of Otorhinolaryngology, Shinshu University School of Medicine, Matsumoto, Japan.

Conclusion: Bilateral intratympanic administration of a gadolinium-based contrast agent (GBCA) in MRI was successfully performed and proved to be beneficial in the semi-quantitative evaluation of endolymphatic hydrops. Such image-based diagnosis will lead to re-revaluation and reclassification of the diagnostic criteria for Meniere's disease (MD).

Objective: To visualize endolymphatic hydrops semi-quantitatively in patients with MD, by using bilateral intratympanic GBCA administration with MRI.

Patients And Methods: A total of 13 patients were evaluated, including 12 with MD and one with acute low-tone sensorineural hearing loss. Diluted gadodiamide (a kind of GBCA) was administered to the bilateral tympanic cavity by injection through the tympanic membrane. After 24 h, the endolymphatic hydrops was evaluated with a 3.0 T MR scanner. The areas enhanced by gadodiamide were measured semi-quantitatively.

Results: Three-dimensional, fluid-attenuated inversion recovery (3D-FLAIR) MRI showed that the gadodiamide successfully penetrated the round window membrane, entering the perilymphatic space and delineating the gadodiamide-enhanced perilymphatic and gadodiamide-negative endolymphatic spaces of the inner ear. All the patients with MD showed a reduced gadodiamide-enhanced area representing the perilymphatic space, and the quantitative ratio was 0.15 to 0.85. Furthermore, endolymphatic hydrops was also demonstrated in the patient with atypical MD who had fluctuating low frequency sensorineural hearing loss without vertigo.
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http://dx.doi.org/10.3109/00016480902858881DOI Listing
October 2010
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