Faculty Profiles - SUGAYA AKIKO

Papers - SUGAYA AKIKO

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Three Cases of BONEBRIDGE Implantation at Okayama University Hospital

Akiko Sugaya, Shin Kariya

The Japan Journal of Logopedics and Phoniatrics 66 ( 2 ) 99 - 106 2025.4

Potential for Radiation Dose Reduction in Temporal Bone CT Imaging Using Photon-Counting Detector CT.

Fumiyo Higaki, Yusuke Morimitsu, Toshihiro Iguchi, Sung Il Hwang, Takahiro Kitayama, Yuka Takahashi, Mayu Uka, Noriaki Akagi, Akiko Sugaya, Toshiharu Mitsuhashi, Yusuke Matsui, Takao Hiraki

Acta medica Okayama 79 ( 2 ) 75 - 80 2025.4

Language：English Publishing type：Research paper (scientific journal)

Temporal bone computed tomography (CT) is frequently performed for pediatric patients with ear diseases. Advances in CT technology have improved diagnostic imaging quality, but reduction of radiation exposure remains a goal. We evaluated the potential for radiation dose reduction in temporal bone CT examinations using porcine ear ossicles and a photon-counting detector CT system. Three scans of the bilateral temporal bone were performed on each of three pig cadaver heads. In each of seven successive imaging sessions, the radiation dose was reduced by an additional one-seventh of the recommended dose (RD). Two board-certified radiologists independently scored the resulting images on a scale of 1 to 5 points, where 5 represented the image quality at the RD. Images scoring ≥4.5 points were considered acceptable. Noise was assessed in a 2-cm-diameter region near the ear ossicles, and standard deviation was measured for each of the seven decrements from the RD. As the radiation dose decreased, the noise progressively increased, and visual assessment scores progressively decreased. Acceptable image scores were obtained at six-sevenths (4.9), five-sevenths (4.8), four-sevenths (4.7), and three-sevenths (4.6) of the RD. Thus, acceptable porcine temporal bone CT images were obtained with a radiation dose reduction of approximately 50%.

DOI： 10.18926/AMO/68645

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Short-process incudo-stapedioplasty in congenital ear malformation. International journal

Ryotaro Omichi, Shin Kariya, Akiko Sugaya, Mizuo Ando

Laryngoscope investigative otolaryngology 10 ( 1 ) e70055 2025.2

Language：English Publishing type：Research paper (scientific journal)

OBJECTIVES: Although various stapedotomy and stapedectomy techniques exist, anchoring the piston can be challenging. We present a novel surgical approach for treating congenital stapes malformations with an atypical facial nerve trajectory. METHODS: This is a case of a 7-year-old boy presenting with bilateral conductive hearing loss. Prior attempts at tympanoplasty had proven unsuccessful in improving his hearing. Presurgical imaging studies revealed an unusual anatomical configuration, with the facial nerve positioned inferior to the oval window. This anatomical variation precluded the use of conventional prosthesis-anchoring techniques typically employed in stapedotomies. Thus, we devised an innovative approach, opting to anchor the prosthesis to the short process of the incus. RESULTS: This novel technique circumvented the atypical course of the facial nerve, allowing for successful reconstruction of the ossicular chain. The patient demonstrated an acceptable improvement (30 dB gain) in hearing 1-year post-surgery, with no reported complications. CONCLUSION: This case underscores the critical importance of adapting surgical techniques to address the unique anatomical challenges that may arise in the context of congenital ear malformations. It also highlights the potential of the short process of the incus as a viable alternative anchoring site for stapes prostheses, thereby improving the outcomes of such complex cases. This technique not only restored the patient's hearing but also contributed valuable insights into the management of similar cases, potentially improving the quality of life for individuals with rare and challenging anatomical variations. LEVEL OF EVIDENCE: 5.

DOI： 10.1002/lio2.70055

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Middle-Ear Salivary Gland Choristoma with Congenital, Single-Sided Hearing Loss. Reviewed

Yuichiro Tominaga, Akiko Sugaya, Shin Kariya, Aiko Shimizu, Yuko Kataoka, Mizuo Ando

Acta medica Okayama 78 ( 4 ) 349 - 355 2024.8

Authorship：Corresponding author Language：English Publishing type：Research paper (scientific journal)

Middle-ear salivary gland choristoma (SGCh) is a rare, benign tumor that causes conductive hearing loss owing to middle-ear morphological abnormalities. Early diagnosis is challenging, and surgical resection is indispensable for a definitive diagnosis. We report the case of a 3-year-old boy diagnosed with middle-ear SGCh during the follow-up period for left-sided hearing loss discovered at newborn hearing screening (NHS). Long-term follow-up after the NHS result, subsequent computed tomography/magnetic resonance imaging, and surgical resection led to its relatively early diagnosis and treatment.

DOI： 10.18926/AMO/67554

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『今から始めるこどもから大人への移行支援』難聴児のスキル獲得のための多職種連携支援について考える Invited Reviewed

菅谷明子

小児耳鼻咽喉科 45 ( 1 ) 10 - 14 2024.6

The prevalence and clinical features of MYO7A-related hearing loss including DFNA11, DFNB2 and USH1B. International journal

Kizuki Watanabe, Shin-Ya Nishio, Shin-Ichi Usami

Scientific reports 14 ( 1 ) 8326 - 8326 2024.4

Language：English Publishing type：Research paper (scientific journal)

The MYO7A gene is known to be responsible for both syndromic hearing loss (Usher syndrome type1B:USH1B) and non-syndromic hearing loss including autosomal dominant and autosomal recessive inheritance (DFNA11, DFNB2). However, the prevalence and detailed clinical features of MYO7A-associated hearing loss across a large population remain unclear. In this study, we conducted next-generation sequencing analysis for a large cohort of 10,042 Japanese hearing loss patients. As a result, 137 patients were identified with MYO7A-associated hearing loss so that the prevalence among Japanese hearing loss patients was 1.36%. We identified 70 disease-causing candidate variants in this study, with 36 of them being novel variants. All variants identified in autosomal dominant cases were missense or in-frame deletion variants. Among the autosomal recessive cases, all patients had at least one missense variant. On the other hand, in patients with Usher syndrome, almost half of the patients carried biallelic null variants (nonsense, splicing, and frameshift variants). Most of the autosomal dominant cases showed late-onset progressive hearing loss. On the other hand, cases with autosomal recessive inheritance or Usher syndrome showed congenital or early-onset hearing loss. The visual symptoms in the Usher syndrome cases developed between age 5-15, and the condition was diagnosed at about 6-15 years of age.

DOI： 10.1038/s41598-024-57415-1

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中耳炎様所見で発症し内耳窓閉鎖術を施行した外リンパ瘻2例の検討

菅谷明子, 假谷伸, 大道亮太郎, 片岡祐子, 前田幸英, 安藤瑞生

日本耳鼻咽喉科頭頸部外科学会会報 127 ( 4 ) 600 - 600 2024.4

市民公開講座参加者における聴力像の実際

浜本真一, 假谷伸, 菅谷明子, 片岡祐子, 前田幸英, 大道亮太郎, 安藤瑞生, 原浩貴

日本耳鼻咽喉科頭頸部外科学会会報 127 ( 4 ) 549 - 549 2024.4

Cochlear Implantation in the Poorer-Hearing Ear Is a Reasonable Choice. Reviewed

Ryotaro Omichi, Shin Kariya, Yukihide Maeda, Kunihiro Fukushima, Yuko Kataoka, Akiko Sugaya, Kazunori Nishizaki, Mizuo Ando

Acta medica Okayama 77 ( 6 ) 589 - 593 2023.12

Language：English Publishing type：Research paper (scientific journal)

Choosing the optimal side for cochlear implantation (CI) remains a major challenge because of the lack of evidence. We investigated the choice of the surgery side for CI (i.e., the better- or poorer-hearing ear) in patients with asymmetric hearing. Audiological records of 74 adults with a unilateral hearing aid who had undergone surgery at Okayama University Hospital were reviewed. The definition of 'better-hearing ear' was the aided ear, and the unaided ear was considered the poorer-hearing ear. We performed a multiple regression analysis to identify potential predictors of speech recognition performance after unilateral CI in the patients. Fifty-two patients underwent CI in the poorer-hearing ear. The post-Ci bimodal hearing rate was far higher in the poorer-ear group (77.8% vs. 22.2%). A multivariate analysis revealed that prelingual hearing loss and the patient's age at CI significantly affected the speech recognition outcome (beta coefficients: 24.6 and -0.33, 95% confidence intervals [11.75-37.45] and [-0.58 to -0.09], respectively), but the CI surgery side did not (-6.76, [-14.92-1.39]). Unilateral CI in the poorer-hearing ear may therefore be a reasonable choice for adult patients with postlingual severe hearing loss, providing a greater opportunity for postoperative bimodal hearing.

DOI： 10.18926/AMO/66150

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突発性難聴治療に対する新型コロナウイルス感染症の影響

大道亮太郎, 佐藤明日香, 菅谷明子, 前田幸英, 片岡祐子, 安藤瑞生

日本耳科学会総会・学術講演会抄録集 33回 260 - 260 2023.11

切除不能中耳粘膜悪性黒色腫に対して陽子線治療及びニボルマブ投与を実施した一例

佐藤明日香, 安藤瑞生, 假谷伸, 片岡祐子, 前田幸英, 菅谷明子, 大道亮太郎

日本耳科学会総会・学術講演会抄録集 33回 314 - 314 2023.11

一般地域住民における難聴の実地調査難聴に関する公開講座参加者を対象に

浜本真一, 假谷伸, 菅谷明子, 片岡祐子, 前田幸英, 大道亮太郎, 安藤瑞生, 原浩貴

日本耳科学会総会・学術講演会抄録集 33回 283 - 283 2023.11

Revision cochlear implant surgery in adults and children is a safe and effective technique: a retrospective single faculty study in Okayama University Hospital, Japan. Reviewed International journal

Akiko Sugaya, Shin Kariya, Kunihiro Fukushima, Ryotaro Omichi, Mizuo Ando

Acta oto-laryngologica 1 - 5 2023.10

Authorship：Lead author,　Corresponding author Language：English Publishing type：Research paper (scientific journal)

BACKGROUND: Cochlear implant (CI) surgery is a safe surgical technique, although some patients require revision CI surgery. AIMS/OBJECTIVES: This study investigated the cause and underlying reason of revision CI surgery as well as hearing outcomes in a single institution. PATIENTS AND METHODS: This retrospective study evaluated patients who underwent CI surgery between April 2006 to March 2022 (n = 351). Sex, aetiology of hearing loss (HL), age and period from initial CI surgery to reimplantation, cause of revision, and related factors were examined. RESULTS: Twelve patients (8 males, 4 females) received CI reimplantation. The revision surgery rate was 2.59% (3.15% children, 1.69% adults); the period from initial surgery to reoperation was 8.60 ± 6.56 years for 9 children with congenital HL and 15.27 ± 5.72 years for 3 adults with progressive HL. Device failure was the most common cause (n = 8), followed by infections (n = 2), advanced facial irritation symptoms (n = 1), and electrode slip-out (n = 1). Mean preoperative and postoperative CI thresholds were 44.0 ± 9.46 dBnHL and 39.19 ± 8.89 dBnHL (p < .068), respectively. CONCLUSION AND SIGNIFICANCE: Caregiver education, surgical technique advances, flap design, and extensive antibiotic use may decrease the revision surgery rate. The lack of post-revision deterioration of the hearing threshold contributed to well-being in patients with CI.

DOI： 10.1080/00016489.2023.2270701

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Evaluation of the ear ossicles with photon-counting detector CT. Reviewed

Yuka Takahashi, Fumiyo Higaki, Akiko Sugaya, Yudai Asano, Katsuhide Kojima, Yusuke Morimitsu, Noriaki Akagi, Toshihide Itoh, Yusuke Matsui, Takao Hiraki

Japanese journal of radiology 2023.8

Language：English Publishing type：Research paper (scientific journal)

Recently, computed tomography with photon-counting detector (PCD-CT) has been developed to enable high-resolution imaging at a lower radiation dose. PCD-CT employs a photon-counting detector that can measure the number of incident X-ray photons and their energy. The newly released PCD-CT (NAEOTOM Alpha, Siemens Healthineers, Forchheim, Germany) has been in clinical use at our institution since December 2022. The PCD-CT offers several advantages over current state-of-the-art energy-integrating detector CT (EID-CT). The PCD-CT does not require septa to create a detector channel, while EID-CT does. Therefore, downsizing the anode to achieve higher resolution does not affect the dose efficiency of the PCD-CT. CT is an indispensable modality for evaluating ear ossicles. The ear ossicles and joints are clearly depicted by PCD-CT. In particular, the anterior and posterior legs of the stapes, which are sometimes unclear on conventional CT scans, can be clearly visualized. We present cases of congenital anomalies of the ossicular chain, ossicular chain dislocation, tympanosclerosis, and cholesteatoma in which PCD-CT was useful. This short article reports the usefulness of PCD-CT in the 3D visualization of the ear ossicles.

DOI： 10.1007/s11604-023-01485-0

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人工内耳装用児の術後コミュニケーション状態における術前CTによる前庭水管拡大症の影響の検討

菅谷明子, 假谷伸, 福島邦博, 片岡祐子, 前田幸英, 大道亮太郎, 安藤瑞生

日本耳科学会総会・学術講演会抄録集 32回 342 - 342 2022.10

Cogan症候群に対して人工内耳植込術を施行した一例

佐藤明日香, 安藤瑞生, 假谷伸, 片岡祐子, 前田幸英, 菅谷明子, 大道亮太郎

日本耳科学会総会・学術講演会抄録集 32回 271 - 271 2022.10

小児難聴と人工聴覚器

假谷伸, 菅谷明子, 片岡祐子

耳鼻咽喉科 2 ( 2 ) 263 - 269 2022.8

Correction to: Detailed clinical features and genotype-phenotype correlation in an OTOF-related hearing loss cohort in Japan. Reviewed International journal

Yoh-Ichiro Iwasa, Shin-Ya Nishio, Hidekane Yoshimura, Akiko Sugaya, Yuko Kataoka, Yukihide Maeda, Yukihiko Kanda, Kyoko Nagai, Yasushi Naito, Hiroshi Yamazaki, Tetsuo Ikezono, Han Matsuda, Masako Nakai, Risa Tona, Yuika Sakurai, Remi Motegi, Hidehiko Takeda, Marina Kobayashi, Chiharu Kihara, Takashi Ishino, Shin-Ya Morita, Satoshi Iwasaki, Masahiro Takahashi, Sakiko Furutate, Shin-Ichiro Oka, Toshinori Kubota, Yasuhiro Arai, Yumiko Kobayashi, Daisuke Kikuchi, Tomoko Shintani, Noriko Ogasawara, Yohei Honkura, Shuji Izumi, Misako Hyogo, Yuzuru Ninoyu, Mayumi Suematsu, Jun Nakayama, Nana Tsuchihashi, Mayuri Okami, Hideaki Sakata, Hiroshi Yoshihashi, Taisuke Kobayashi, Kozo Kumakawa, Tadao Yoshida, Tomoko Esaki, Shin-Ichi Usami

Human genetics 141 ( 3-4 ) 993 - 995 2022.4

Etiology of hearing loss affects auditory skill development and vocabulary development in pediatric cochlear implantation cases. Reviewed International journal

Shin-Ya Nishio, Hideaki Moteki, Maiko Miyagawa, Tatsuya Yamasoba, Akinori Kashio, Satoshi Iwasaki, Masahiro Takahashi, Yasushi Naito, Keizo Fujiwara, Akiko Sugaya, Haruo Takahashi, Kyoko Kitaoka, Shin-Ichi Usami

Acta oto-laryngologica 142 ( 3-4 ) 308 - 315 2022

Language：English Publishing type：Research paper (scientific journal)

BACKGROUND: Cochlear implantation (CI) is an effective treatment for severe-to-profound hearing loss patients and is currently used as the standard therapeutic option worldwide. However, the outcomes of CI vary among patients. AIMS/OBJECTIVES: This study aimed to clarify the clinical features for each etiological group as well as the effects of etiology on CI outcomes. MATERIALS AND METHODS: We collected clinical information for 308 pediatric cochlear implant cases, including the etiology, hearing thresholds, age at CI, early auditory skill development, total development, monosyllable perception, speech intelligibility and vocabulary development in school age, and compared them for each etiology group. RESULTS: Among the 308 CI children registered for this survey, the most common etiology of hearing loss was genetic causes. The genetic etiology group showed the most favorable development after CI followed by the unknown etiology group, syndromic hearing loss group, congenital CMV infection group, inner ear malformation group, and cochlear nerve deficiency group. CONCLUSIONS AND SIGNIFICANCE: Our results clearly indicated that the etiology of HL affects not only early auditory skill development, but also vocabulary development in school age. The results of the present study will aid in more appropriate CI outcome assessment and in more appropriate intervention or habilitation programs.

DOI： 10.1080/00016489.2022.2065027

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Detailed clinical features and genotype-phenotype correlation in an OTOF-related hearing loss cohort in Japan. Reviewed International journal

Yoh-Ichiro Iwasa, Shin-Ya Nishio, Hidekane Yoishimura, Akiko Sugaya, Yuko Kataoka, Yukihide Maeda, Yukihiko Kanda, Kyoko Nagai, Yasushi Naito, Hiroshi Yamazaki, Tetsuo Ikezono, Han Matsuda, Masako Nakai, Risa Tona, Yuika Sakurai, Remi Motegi, Hidehiko Takeda, Marina Kobayashi, Chiharu Kihara, Takashi Ishino, Shin-Ya Morita, Satoshi Iwasaki, Masahiro Takahashi, Sakiko Furutate, Shin-Ichiro Oka, Toshinori Kubota, Yasuhiro Arai, Yumiko Kobayashi, Daisuke Kikuchi, Tomoko Shintani, Noriko Ogasawara, Yohei Honkura, Shuji Izumi, Misako Hyogo, Yuzuru Ninoyu, Mayumi Suematsu, Jun Nakayama, Nana Tsuchihashi, Mayuri Okami, Hideaki Sakata, Hiroshi Yoshihashi, Taisuke Kobayashi, Kozo Kumakawa, Tadao Yoshida, Tomoko Esaki, Shin-Ichi Usami

Human genetics 141 ( 3-4 ) 865 - 875 2021.9

Language：English Publishing type：Research paper (scientific journal)

Mutations in the OTOF gene are a common cause of hereditary hearing loss and the main cause of auditory neuropathy spectrum disorder (ANSD). Although it is reported that most of the patients with OTOF mutations have stable, congenital or prelingual onset severe-to-profound hearing loss, some patients show atypical clinical phenotypes, and the genotype-phenotype correlation in patients with OTOF mutations is not yet fully understood. In this study, we aimed to reveal detailed clinical characteristics of OTOF-related hearing loss patients and the genotype-phenotype correlation. Detailed clinical information was available for 64 patients in our database who were diagnosed with OTOF-related hearing loss. As reported previously, most of the patients (90.6%) showed a "typical" phenotype; prelingual and severe-to-profound hearing loss. Forty-seven patients (73.4%) underwent cochlear implantation surgery and showed successful outcomes; approximately 85-90% of the patients showed a hearing level of 20-39 dB with cochlear implant and a Categories of Auditory Performance (CAP) scale level 6 or better. Although truncating mutations and p.Arg1939Gln were clearly related to severe phenotype, almost half of the patients with one or more non-truncating mutations showed mild-to-moderate hearing loss. Notably, patients with p.His513Arg, p.Ile1573Thr and p.Glu1910Lys showed "true" auditory neuropathy-like clinical characteristics. In this study, we have clarified genotype-phenotype correlation and efficacy of cochlear implantation for OTOF-related hearing loss patients in the biggest cohort studied to date. We believe that the clinical characteristics and genotype-phenotype correlation found in this study will support preoperative counseling and appropriate intervention for OTOF-related hearing loss patients.

DOI： 10.1007/s00439-021-02351-7

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思春期から青年期の難聴者が持つ代償・補償スキルと知識に関する一考察

中川敦子, 片岡祐子, 田中里実, 細川夏実, 菅谷明子, 假谷伸

Audiology Japan 64 ( 5 ) 389 - 389 2021.9

Effects of Protective Measures against COVID-19 on Auditory Communication for People with Hearing Loss. Reviewed

Yuko Kataoka, Yukihide Maeda, Akiko Sugaya, Ryotaro Omichi, Shin Kariya

Acta medica Okayama 75 ( 4 ) 511 - 516 2021.8

Language：English Publishing type：Research paper (scientific journal)

WHO has recommended various measures to combat the COVID-19 pandemic, including mask-wearing and physical distancing. However, these changes impair communication for individuals with hearing loss. We investigated the changes in auditory communication associated with COVID-19 measures in 269 patients (male: 45.7%, female: 54.3%, median age: 54 y.o.). Most patients with hearing loss had difficulty engaging in auditory communication with people wearing masks, especially in noisy surroundings or with physical distanc-ing. These difficulties were noticeable in patients with severe hearing loss. Developing communication support strategies for people with hearing loss is an urgent need while COVID-19 measures are in place.

DOI： 10.18926/AMO/62404

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Immediate changes in transcription factors and synaptic transmission in the cochlea following acoustic trauma: A gene transcriptome study. Reviewed International journal

Yukihide Maeda, Shin Kariya, Kensuke Uraguchi, Junko Takahara, Shohei Fujimoto, Akiko Sugaya, Kazunori Nishizaki

Neuroscience research 165 6 - 13 2021.4

Language：English Publishing type：Research paper (scientific journal)

Pathologic mechanisms in cochleae immediately following the onset of noise-induced hearing loss (NIHL) remain unclear. In this study, mice were exposed to 120 dB of octave band noise for 2 h to induce NIHL. Three hours after noise exposure, expression levels of the whole mouse genome in cochleae were analyzed by RNA-seq and DNA microarray. Differentially expressed genes (DEGs) exhibiting >2-fold upregulation or downregulation in noise-exposed cochleae compared to controls without noise exposure were identified. RNA-seq and microarray analyses identified 273 DEGs regulated at 3 h post-noise (51 upregulated and 222 downregulated). Bioinformatic analysis revealed that these DEGs were associated with the functional gene pathway "neuroactive ligand-receptor interaction" and included 28 genes encoding receptors for neurotransmitters such as gamma-aminobutyric acid and glutamate. Other DEGs included 25 genes encoding transcription factors. Downregulation of 4 neurotransmitter receptors (Gabra3, Gabra5, Gabrb1, Grm1) and upregulations of 5 transcription factors (Atf3, Dbp, Helt, Maff, Nr1d1) were validated by RT-PCR. The differentially regulated transcription factor Atf3 immunolocalized to supporting cells and hair cells in the organ of Corti at 12-h post-noise. The present data serve as a basis for further studies aimed at developing medical treatments for acute sensorineural hearing loss.

DOI： 10.1016/j.neures.2020.05.001

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Fertility Preservation Therapy Provided for Childhood, Adolescent, and Young Adult Cancer Patients —Current Status and Efforts to Improvement—,小児，思春期・若年がん患者に対する妊孕性温存療法―現状と改善の取り組み―

Akisada, N., Makino, T., Sugaya, A., Kataoka, Y., Ando, M., Monden, N., Nakatsuka, M.

Practica Oto-Rhino-Laryngologica 114 ( 12 ) 2021

Prevalence and risk factors for delayed-onset hearing loss in early childhood: A population-based observational study in Okayama Prefecture, Japan. Reviewed International journal

Yuko Kataoka, Yukihide Maeda, Kunihiro Fukushima, Akiko Sugaya, Akiko Shigehara, Shin Kariya, Kazunori Nishizaki

International journal of pediatric otorhinolaryngology 138 110298 - 110298 2020.11

Language：English Publishing type：Research paper (scientific journal)

OBJECTIVES: The aim of this study was to retrospectively document prevalence rates of delayed-onset hearing loss (DOHL) under 7 years old after passing the newborn hearing screening (NHS) program using its database in Okayama Prefecture, as well as records from Okayama Kanariya Gakuen (OKG, Auditory Center for Hearing Impaired Children, Okayama Prefecture, Japan). We explored the percentage of children with DOHL among all children who underwent the NHS and surveyed risk factors abstracted from their clinical records. METHODS: We collected data of 1171 children, who first visited OKG from April 2006 to March 2018. DOHL children were defined as bilaterally hearing-impaired children who were diagnosed under 7 years old after passing the NHS at birth. Based on the medical records, we investigated age at diagnosis, hearing levels, and risk factors. As population-based data of 168,104 children, the percentage of DOHL subjects was retrospectively calculated among the total number of children who underwent the NHS in Okayama Prefecture from April 2005 to March 2017. RESULTS: During the period, we identified 96 children with bilateral DOHL, of which 34 children had failed the NHS unilaterally and 62 had passed the NHS bilaterally. Among all children who underwent the NHS in Okayama Prefecture, the prevalence rate of DOHL in unilaterally referred infants was 5.2%, and 0.037% in bilaterally passed children. The prevalence of bilateral DOHL was 0.057% overall. Unilaterally referred children with DOHL were diagnosed at an average of 13.9 months, while bilaterally passed children with DOHL were diagnosed at an average of 42.3 months. Approximately 59.4% of children with DOHL had risk factors, among which family history of hearing loss was the most frequent. CONCLUSION: We propose the first English report of DOHL prevalence in the prefecture population in Japann, which is among the largest community-based population ever reported. The NHS is not a perfect strategy to detect all early-childhood hearing loss; therefore, careful assessment of hearing throughout childhood is recommended, especially in children with risk factors of hearing loss. Further interventional strategies must be established, such as regular hearing screening in high-risk children and assessments of hearing and speech/language development in public communities and nursery schools.

DOI： 10.1016/j.ijporl.2020.110298

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Progression of hearing loss and choice of hearing aids by patients in their 60s, 70s, and 80s and older: experience in the Japanese super-aged era. Reviewed

Maeda Y, Kariya S, Fujimoto S, Sugaya A, Kataoka Y, Nakagawa A, Tanaka S, Hosokawa N, Nishizaki K.

Acta Otolaryngol 139 1077 - 1082 2019.9

Impact of reading and writing skills on academic achievement among school-aged hearing-impaired children. Reviewed

Sugaya A, Fukushima K, Takao S, Kasai N, Maeda Y, Fujiyoshi A, Kataoka Y, Kariya S, Nishizaki K.

Int J Pediatr Otorhinolaryngol 126 109619 - 109619 2019.8

Targeted PCR Array Analysis of Genes in Innate Immunity and Glucocorticoid Signaling Pathways in Mice Cochleae Following Acoustic Trauma. Reviewed International journal

Yukihide Maeda, Shin Kariya, Ryotaro Omichi, Yohei Noda, Akiko Sugaya, Shohei Fujimoto, Kazunori Nishizaki

Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 39 ( 7 ) e593 - e600 2018

Language：English Publishing type：Research paper (scientific journal)

AIM: To comprehensively analyze cochlear gene expressions related to innate immunity and glucocorticoid signaling at onset of acute noise-induced hearing loss (NIHL). BACKGROUND: Recent studies suggested innate immunity is involved in the cochlear pathology of NIHL. Glucocorticoids may modulate immune actions in cochleae. METHODS: Mice were exposed to 120 dB-octave band noise for 2 hours. Twelve hours later, a targeted PCR array analyzed cochlear expressions of 84 key genes in inflammation and immune pathways and 84 genes in the glucocorticoid signaling pathway. Real-time RT-PCR was used to analyze expression of two immune-related genes, Ccl12 and Glycam1, in noise-exposed cochleae with or without dexamethasone. RESULT: In inflammatory and immune gene pathways, 31.0% (26/84 genes) were significantly upregulated (>2-fold change) or downregulated (<0.5-fold change) (p < 0.05) in noise-exposed cochleae compared with controls. Sixteen of these differentially expressed genes (DEGs) encoded chemokines. DEGs included Ccl12, Ccl2, Ccl4, Ccl7, Cxcl1, Cxcl10, and Ptgs2 (upregulated genes), and Ccr7, Cxcr2, Kng1, Ltb, and Tnfsf14 (downregulated genes). In the glucocorticoid signaling pathway, 92.9% (78/84 genes) were unchanged in noise-exposed cochleae without dexamethasone administration. Cochlear expressions of Ccl12 and Glycam1 were significantly upregulated by noise and downregulated by dexamethasone. CONCLUSION: The targeted PCR array demonstrated that several dozen genes involved in innate immunity are actively regulated in cochleae with NIHL. The glucocorticoid signaling pathway was not endogenously regulated at 12 hours post-noise trauma. Systemic dexamethasone downregulated Ccl12 and Glycam1, which are upregulated in noise-exposed cochleae. These data may provide a basis for genomic medicine treatment of acute sensorineural hearing loss.

DOI： 10.1097/MAO.0000000000001874

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WFS1 mutation screening in a large series of Japanese hearing loss patients: Massively parallel DNA sequencing-based analysis. Reviewed International journal

Masafumi Kobayashi, Maiko Miyagawa, Shin-Ya Nishio, Hideaki Moteki, Taro Fujikawa, Kenji Ohyama, Hirofumi Sakaguchi, Ikuyo Miyanohara, Akiko Sugaya, Yasushi Naito, Shin-Ya Morita, Yukihiko Kanda, Masahiro Takahashi, Kotaro Ishikawa, Yuki Nagano, Tetsuya Tono, Chie Oshikawa, Chiharu Kihara, Haruo Takahashi, Yoshihiro Noguchi, Shin-Ichi Usami

PloS one 13 ( 3 ) e0193359 2018

Language：English Publishing type：Research paper (scientific journal)

A heterozygous mutation in the Wolfram syndrome type 1 gene (WFS1) causes autosomal dominant nonsyndromic hereditary hearing loss, DFNA6/14/38, or Wolfram-like syndrome. To date, more than 40 different mutations have been reported to be responsible for DFNA6/14/38. In the present study, WFS1 variants were screened in a large series of Japanese hearing loss (HL) patients to clarify the prevalence and clinical characteristics of DFNA6/14/38 and Wolfram-like syndrome. Massively parallel DNA sequencing of 68 target genes was performed in 2,549 unrelated Japanese HL patients to identify genomic variations responsible for HL. The detailed clinical features in patients with WFS1 variants were collected from medical charts and analyzed. We successfully identified 13 WFS1 variants in 19 probands: eight of the 13 variants were previously reported mutations, including three mutations (p.A684V, p.K836N, and p.E864K) known to cause Wolfram-like syndrome, and five were novel mutations. Variants were detected in 15 probands (2.5%) in 602 families with presumably autosomal dominant or mitochondrial HL, and in four probands (0.7%) in 559 sporadic cases; however, no variants were detected in the other 1,388 probands with autosomal recessive or unknown family history. Among the 30 individuals possessing variants, marked variations were observed in the onset of HL as well as in the presence of progressive HL and tinnitus. Vestibular symptoms, which had been rarely reported, were present in 7 out of 30 (23%) of the affected individuals. The most prevalent audiometric configuration was low-frequency type; however, some individuals had high-frequency HL. Haplotype analysis in three mutations (p.A716T, p.K836T, and p.E864K) suggested that the mutations occurred at these mutation hot spots. The present study provided new insights into the audiovestibular phenotypes in patients with WFS1 mutations.

DOI： 10.1371/journal.pone.0193359

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Relationship between pure-tone audiogram findings and speech perception among older Japanese persons. Reviewed International journal

Yukihide Maeda, Soshi Takao, Akiko Sugaya, Yuko Kataoka, Shin Kariya, Satomi Tanaka, Rie Nagayasu, Atsuko Nakagawa, Kazunori Nishizaki

Acta oto-laryngologica 138 ( 2 ) 140 - 144 2018

Language：English Publishing type：Research paper (scientific journal)

OBJECTIVE: To clarify how the pure-tone threshold (PTT) on the PTA predicts speech perception (SP) in elderly Japanese persons. METHODS: Data on PTT and SP were cross-sectionally analyzed in Japanese persons (656 ears in 353 patients, aged ≥65 years). Correlations of SP and average PTT in all tested frequencies were evaluated by Pearson's correlation coefficient and simple linear regression. After adjusting for sex, laterality of ears, and age, the relationship of average and frequency-specific PTT with impaired SP ≤50% was estimated by logistic regression models. RESULTS: SP correlated well (r = -0.699) with the average PTT of all tested frequencies. On the other hand, the correlation between patient age and SP was weak, especially among ≤85-year-old persons (r = -0.092). Linear regression showed that the average PTT corresponding to SP of 50% was 76.4 dB nHL. Odds ratios for impaired SP were highest for PTT at 2000 Hz. Odds ratios were higher for middle (500, 1000, 2000 Hz) and high frequencies (4000, 8000 Hz) than low frequencies (125, 250 Hz). CONCLUSION: The PTT on the pure-tone audiogram (PTA) is a good predictor of SP by speech audiometry among older persons, which could provide clinically important information for hearing aid fitting and cochlear implantation.

DOI： 10.1080/00016489.2017.1378435

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次世代シークエンサー(NGS)を併用した難聴遺伝子検査が有用であった3症例

菅谷明子, 片岡祐子, 前田幸英, 假谷伸, 大道亮太郎, 峠和美, 佐藤吏江, 西崎和則

日本遺伝カウンセリング学会誌 38 ( 2 ) 120 - 120 2017.5

中耳炎により外科的処置を要した小児人工内耳装用例の検討

片岡祐子, 假谷伸, 菅谷明子, 大道亮太郎, 福島邦博, 西崎和則

小児耳鼻咽喉科 38 ( 2 ) 153 - 153 2017.5

新生児聴覚スクリーニング実施における費用対効果の検討

片岡祐子, 菅谷明子, 西崎和則

日本耳鼻咽喉科学会会報 120 ( 4 ) 588 - 588 2017.4

Cochlear Transcriptome Following Acoustic Trauma and Dexamethasone Administration Identified by a Combination of RNA-seq and DNA Microarray. Reviewed International journal

Yukihide Maeda, Ryotaro Omichi, Akiko Sugaya, Shin Kariya, Kazunori Nishizaki

Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 38 ( 7 ) 1032 - 1042 2017

Language：English Publishing type：Research paper (scientific journal)

AIM: To elucidate molecular mechanisms of noise-induced hearing loss (NIHL) and glucocorticoid therapy in the cochlea. BACKGROUND: Glucocorticoids are used to treat many forms of acute sensorineural hearing loss, but their molecular action in the cochlea remains poorly understood. METHODS: Dexamethasone was administered intraperitoneally immediately following acoustic overstimulation at 120 dB SPL for 2 hours to mice. The whole cochlear transcriptome was analyzed 12 and 24 hours following noise trauma and dexamethasone administration by both next-generation sequencing (RNA-seq) and DNA microarray. Differentially expressed genes (DEGs) with more than 2-fold changes after noise trauma and dexamethasone administration were identified. The functions of these DEGs were analyzed by David Bioinformatics Resources and a literature search. RESULTS: Twelve hours after acoustic overstimulation, immune-related gene pathways such as "chemokine signaling activity," "cytokine-cytokine receptor interaction," and "cell adhesion molecules (CAMs) in the immune system" were significantly changed compared with the baseline level without noise. These DEGs were involved in immune and defense responses in the cochlea. Dexamethasone was administered to this NIHL model, and it modulated gene pathways of "cytokine-cytokine receptor interaction" and "cell adhesion molecules (CAMs) in the immune system" at 12 hours, compared with saline-injected control. Dexamethasone-dependent DEGs were also involved in immune and defense responses. A literature search showed that 10 other genes associated with hearing functions were regulated by dexamethasone both at 12 and 24 hours post-administration. CONCLUSION: Dexamethasone modulates the immune reaction in the traumatized cochlea following acoustic overstimulation. Dexamethasone may also regulate cochlear functions other than immunity.

DOI： 10.1097/MAO.0000000000001373

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Genetic counseling of a hearing-impaired patient with multiple genetic mutations Reviewed

Yuko Kataoka, Akiko Sugaya, Yukihide Maeda, Shin Kariya, Ryotaro Omichi, Kunihiro Fukushima, Kazunori Nishizaki

Journal of Otolaryngology of Japan 120 ( 2 ) 131 - 136 2017

Reversal of the inner magnet of a cochlear implant after magnet resonance imaging Reviewed

Yuko Kataoka, Tomoyuki Naito, Shin Kariya, Akiko Sugaya, Yukihide Maeda, Kunihiro Fukushima, Kazunori Nishizaki

Journal of Otolaryngology of Japan 120 ( 5 ) 727 - 732 2017

Pediatric central auditory processing disorder showing elevated threshold on pure tone audiogram Reviewed

Yukihide Maeda, Atsuko Nakagawa, Rie Nagayasu, Akiko Sugaya, Ryotaro Omichi, Shin Kariya, Kunihiro Fukushima, Kazunori Nishizaki

AURIS NASUS LARYNX 43 ( 5 ) 570 - 574 2016.10

Subjective hearing-related quality-of-life is a major factor in the decision to continue using hearing aids among older persons Reviewed

Yukihide Maeda, Akiko Sugaya, Rie Nagayasu, Atsuko Nakagawa, Kazunori Nishizaki

ACTA OTO-LARYNGOLOGICA 136 ( 9 ) 919 - 922 2016.9

MRI検査後に人工内耳インプラントのマグネットの反転を来たした2例

内藤智之, 片岡祐子, 假谷伸, 菅谷明子, 大道亮太郎, 前田幸英, 西崎和則

Otology Japan 26 ( 4 ) 570 - 570 2016.9

急性感音難聴モデルマウスへのステロイド投与後の内耳遺伝子発現 RNA-seqとDNAマイクロアレイを用いた解析

前田幸英, 大道亮太郎, 菅谷明子, 片岡祐子, 假谷伸, 西崎和則

Otology Japan 26 ( 4 ) 489 - 489 2016.9

人工内耳装用児の英語学習における実態調査

菅谷明子, 福島邦博, 谷伸, 長安吏江, 藤吉昭江, 片岡祐子, 前田幸英, 西崎和則

Otology Japan 26 ( 4 ) 405 - 405 2016.9

乳幼児鼓膜インピーダンス測定における1000Hz tympanometryの有用性の検討

片岡祐子, 菅谷明子, 假谷伸, 大道亮太郎, 前田幸英, 福島邦博, 西崎和則

Otology Japan 26 ( 4 ) 449 - 449 2016.9

頸部リンパ管腫に合併した滲出性中耳炎の1例

大道亮太郎, 菅谷明子, 前田幸英, 片岡祐子, 假谷伸, 西崎和則

耳鼻咽喉科臨床補冊 ( 補冊146 ) 99 - 99 2016.6

岡山県における小児難聴例の診断時期の検討

片岡祐子, 鳥越暁子, 菅谷明子, 大道亮太郎, 前田幸英, 福島邦博

小児耳鼻咽喉科 37 ( 2 ) 129 - 129 2016.5

人工内耳装用前の早期補聴が言語発達に与える効果の検証

菅谷明子, 福島邦博, 笠井紀夫, 藤吉昭江, 長安吏江, 片岡祐子, 前田幸英, 西崎和則

小児耳鼻咽喉科 37 ( 2 ) 126 - 126 2016.5

初回手術後に長期間経過して対側人工内耳埋込術を施行された高度感音難聴児6例の検討

菅谷明子, 福島邦博, 假谷伸, 長安吏江, 片岡祐子, 前田幸英, 大道亮太郎, 西崎和則

日本耳鼻咽喉科学会会報 119 ( 4 ) 619 - 619 2016.4

純音聴力閾値上昇を呈した聴覚情報処理障害の小児例

前田幸英, 菅谷明子, 片岡祐子, 大道亮太郎, 假谷伸, 福島邦博, 西崎和則

日本耳鼻咽喉科学会会報 119 ( 4 ) 618 - 618 2016.4

Characteristics of audiogram configuration in multiple-system atrophy C and cortical cerebellar atrophy Reviewed

Ryotaro Omichi, Yukihide Maeda, Akiko Sugaya, Yuko Kataoka, Shin Kariya, Rie Nagayasu, Atsuko Nakagawa, Toru Yamashita, Koji Abe, Kazunori Nishizaki

ACTA OTO-LARYNGOLOGICA 136 ( 3 ) 266 - 270 2016

Impact of early intervention on comprehensive language and academic achievement in Japanese hearing-impaired children with cochlear implants Reviewed

Akiko Sugaya, Kunihiro Fukushima, Norio Kasai, Yuko Kataoka, Yukihide Maeda, Rie Nagayasu, Naomi Toida, Shyuhei Ohmori, Akie Fujiyoshi, Tomoko Taguchi, Ryotaro Omichi, Kazunori Nishizaki

INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY 79 ( 12 ) 2142 - 2146 2015.12

Steroid-dependent sensorineural hearing loss in a patient with Charcot-Marie-Tooth disease showing auditory neuropathy Reviewed

Yukihide Maeda, Yuko Kataoka, Akiko Sugaya, Shin Kariya, Katsuhiro Kobayashi, Kazunori Nishizaki

AURIS NASUS LARYNX 42 ( 3 ) 249 - 253 2015.6

Novel ABHD12 Mutations in PHARC Patients: The Differential Diagnosis of Deaf-Blindness Reviewed

Hidekane Yoshimura, Takao Hashimoto, Toshinori Murata, Kunihiro Fukushima, Akiko Sugaya, Shin-ya Nishio, Shin-ichi Usami

ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY 124 ( 1 ) 77S - 83S 2015.5

岡山県内胃瘻造設術施行施設へのアンケートからみる嚥下機能評価における耳鼻咽喉科医の役割

西崎和則, 春名威範, 牧野琢丸, 三木健太郎, 野田洋平, 檜垣貴哉, 菅谷明子, 小野田友男

日本耳鼻咽喉科学会会報 118 ( 4 ) 501 - 501 2015.4

Does the introduction of newborn hearing screening improve vocabulary development in hearing-impaired children? A population-based study in Japan Reviewed

Shuhei Ohmori, Akiko Sugaya, Naomi Toida, Etsuji Suzuki, Masato Izutsu, Tomoko Tsutsui, Yuko Kataoka, Yukihide Maeda, Kunihiro Fukushima, Kazunori Nishizaki

INTERNATIONAL JOURNAL OF PEDIATRIC OTORHINOLARYNGOLOGY 79 ( 2 ) 196 - 201 2015.2

Outcomes of consonant-vowel monosyllable perception and word recognition after cochlear implantation in elderly Japanese patients Reviewed

Ryotaro Omichi, Yukihide Maeda, Akiko Sugaya, Yuko Kataoka, Shin Kariya, Kunihiro Fukushima, Kazunori Nishizaki

ACTA OTO-LARYNGOLOGICA 135 ( 11 ) 1108 - 1112 2015

2年以上聴力経過を観察した小児心因性難聴の7例

前田幸英, 片岡祐子, 菅谷明子, 長安吏江, 中川敦子, 假谷伸, 西﨑和則

AUDIOLOGY JAPAN 58 ( 5 ) 335 - 336 2015

当科で経験したBOR症候群の2家族例

浦口健介, 菅谷明子, 片岡祐子, 前田幸英, 假谷伸, 福島邦博, 西崎和則

Otology Japan 24 ( 4 ) 548 - 548 2014.9

Assessment of hearing aid outcome using CADL Reviewed

Rie Nagayasu, Yukihide Maeda, Akiko Sugaya, Yuko Kataoka, Kunihiro Fukushima, Kazunori Nishizaki

Journal of Otolaryngology of Japan 117 ( 8 ) 1126 - 1131 2014.8

Effectiveness of Domain-Based Intervention for Language Development in Japanese Hearing-Impaired Children: A Multicenter Study Reviewed

Akiko Sugaya, Kunihiro Fukushima, Norio Kasai, Toshiyuki Ojima, Goro Takahashi, Takashi Nakagawa, Seiko Murai, Yasoichi Nakajima, Kazunori Nishizaki

ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY 123 ( 7 ) 500 - 508 2014.7

[The age at which the grade of Special Child Dependent's Allowance Act can be judged in the case of deaf children]. Reviewed

Yuko Kataoka, Kunihiro Fukushima, Akiko Sugaya, Yukihide Maeda, Kazunori Nishizaki

Nihon Jibiinkoka Gakkai kaiho 117 ( 9 ) 1200 - 4 2014

Language：Japanese Publishing type：Research paper (scientific journal)

The spread of neonatal hearing screening has made the identification of hearing loss in early childhood possible, and most of the children with hearing loss are diagnosed and provided with early intervention within several months after their birth. However, they have to wait to receive the Special Child Dependent's Allowance until their hearing levels are evaluated with audiometers. Under the Special Child Dependent's Allowance Act now in force, a delay of several years occurs for those children before they can receive the allowance after diagnosis. We retrospectively reviewed hearing test results of children who had not reacted at 90dBnHL bilaterally in ABR within 12 months after birth, and examined when those results were certain enough to determine their allowance eligibility. We found that the eligibility in children with hearing loss alone could be determined at 1 year of age; as for children who had other disorders, since it was mostly difficult to test them with an audiometer, the diagnosis of hearing loss must always be made carefully but it was also possible to determine the eligibility for the allowance at 1 year of age.

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Cochlin-tomoprotein (CTP) detection test identified perilymph leakage preoperatively in revision stapes surgery Reviewed

Yuko Kataoka, Tetsuo Ikezono, Kunihiro Fukushima, Koji Yuen, Yukihide Maeda, Akiko Sugaya, Kazunori Nishizaki

AURIS NASUS LARYNX 40 ( 4 ) 422 - 424 2013.8

Language Ability in the Intermediate-Scoring Group of Hearing-Impaired Children Reviewed

Akiko Sugaya, Kunihiro Fukushima, Norio Kasai, Akie Fujiyoshi, Tomoko Taguchi, Kana Omori, Toshiyuki Ojima, Kazunori Nishizaki

ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY 121 ( 4 ) 21 - 27 2012.4

Effects of Early Identification and Intervention on Language Development in Japanese Children With Prelingual Severe to Profound Hearing Impairment Reviewed

Norio Kasai, Kunihiro Fukushima, Kana Omori, Akiko Sugaya, Toshiyuki Ojima

ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY 121 ( 4 ) 16 - 20 2012.4

Assessment Package for Language Development in Japanese Hearing-Impaired Children (ALADJIN) as a Test Battery for the Development of Practical Communication Reviewed

Kunihiro Fukushima, Norio Kasai, Kana Omori, Akiko Sugaya, Akie Fujiyoshi, Tomoko Taguchi, Takayuki Konishi, Syuuhei Sugishita, Wataru Takei, Hiroshi Fujino, Toshiyuki Ojima, Kazunori Nishizaki

ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY 121 ( 4 ) 3 - 15 2012.4

Syntactic Development in Japanese Hearing-Impaired Children Reviewed

Akie Fujiyoshi, Kunihiro Fukushima, Tomoko Taguchi, Kana Omori, Norio Kasai, Shinya Nishio, Akiko Sugaya, Rie Nagayasu, Takayuki Konishi, Syuuhei Sugishita, Jyunpei Fujita, Kazunori Nishizaki, Masae Shiroma

ANNALS OF OTOLOGY RHINOLOGY AND LARYNGOLOGY 121 ( 4 ) 28 - 34 2012.4

Cochlear implant at 11 months old for deafness after meningitis Reviewed

HAMADA Koji, SUGAYA Akiko, KATAOKA Yuko, MAEDA Yukihide, FUKUSHIMA Kunihiro, NISHIZAKI Kazunori

Ped ORL J 33 ( 1 ) 23 - 28 2012.3

Language：Japanese Publisher：Japan Society for Pediatric Otorhinolaryngology

A case of rapidly progressive ossification of the cochlea after bacterial meningitis is reported. An eleven-month-old boy was referred to our hospital due to his loss of response to sound after suffering from meningitis at 8 months old. He did not respond to sound either by BOA or ABR at 105dB nHL. 3D reconstruction imaging with MRI suggested that the cochlea was completely occluded in his right inner ear. Possible progression to his left inner ear was suspected, and cochlear implant in his left ear was performed immediately. Ossification of the cochlea is frequently observed after meningitis. In cases with early and rapidly progressive ossification of the cochlea, repeated audiological evaluation and MRI imaging is indispensable in the follow-up after bacterial meningitis with very young infants.

DOI： 10.11374/shonijibi.33.23

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Other Link： http://search.jamas.or.jp/link/ui/2012208704

Five cases requiring revision or middle ear surgery after cochlear implantation Reviewed

SUGAYA Akiko, FUKUSHIMA Kunihiro, KATAOKA Yuko, MAEDA Yukihide, KASAI Norio, HIRAI Misato, NAGAYASU Rie, NISHIZAKI Kazunori

Ped ORL J 32 ( 3 ) 409 - 414 2011.12

Authorship：Lead author Language：Japanese Publisher：Japan Society for Pediatric Otorhinolaryngology

Revision/additional surgeries after cochlear implants are sometimes required because of device failure, hematoma, infections, and malinsertion/extrusion of electrodes. Younger implant age may also increase the incidence of additional ear surgery due to the immaturity of the Eustachean tubes. Since 1992, we have conducted 150 pediatric cochlear implants with three revisions (2.0%) or two additional surgeries (1.3%) required so far. All three revision cases were due to device failure, and ipsilateral explantation/reimplantation surgeries were conducted. The two additional cases included a case of ventilation tube malplacement and a case of myringoplasty. A case with implant failure after head trauma is reported here in detail.<br/> A 7-year-old girl who received cochlear implantation at the age of two visited our hospital because she didn't respond to sound after a head trauma. Her speech perception had been good until her head was struck by a swing in a park, and she came to our hospital four days later. An impedance check or NRT of the cochlear implant was impossible, and explantation and reimplantation of the same side cochlear implant was performed six weeks later. Insertion of the electrodes was extremely difficult because of inner ear malformation, but the right position of the electrode was finally confirmed by X-ray. Her speech perception and language development were good after the operation.

DOI： 10.11374/shonijibi.32.409

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Other Link： http://search.jamas.or.jp/link/ui/2012118058

Expression analysis of microRNAs in murine cochlear explants Reviewed

Misato Hirai, Yukihide Maeda, Kunihiro Fukushima, Akiko Sugaya, Yuko Kataoka, Kazunori Nishizaki

NEUROREPORT 22 ( 13 ) 652 - 654 2011.9

Orbital Emphysema Causing Syncope Reviewed

Kentaroh Miki, Yorihisa Orita, Fumiaki Kumase, Akiko Sugaya, Misato Hirai, Yasuyuki Noyama, Kiyoko Nakai, Akiko Narita, Jiroh Seguchi

ACTA MEDICA OKAYAMA 65 ( 1 ) 55 - 57 2011.2

Subsidizing hearing aid purchase in mild to moderate pediatric hearing - Pediatric status following prefectural assembly subvention Reviewed

Yuko Kataoka, Kunihiro Fukushima, Akiko Sugaya, Yukihide Maeda, Yu Masuda, Kazunori Nishizaki

Journal of Otolaryngology of Japan 114 ( 8 ) 731 - 736 2011

Progressive or delayed early-onset pediatric sensorineural hearing loss Reviewed

Yuko Kataoka, Kunihiro Fukushima, Yukihide Maeda, Akiko Sugaya, Rie Nagayasu, Yu Masuda, Kazunori Nishizaki

Journal of Otolaryngology of Japan 114 ( 6 ) 557 - 561 2011

A case of oncocytoma of the deep lobe of the parotid gland

MIKI Kentaro, ORITA Yorihisa, SUGAYA Akiko, HIRAI Misato, NAKAI Kiyoko, NOSE Soichiro

Stomato-pharyngology 23 ( 2 ) 237 - 241 2010.6

Language：Japanese Publisher：Japan Society of Stomato-pharyngology

We report a case of oncocytoma of the deep parotid gland lobe. A 64-year-old man admitted for an infra auricular mass was confirmed to have a firm mass deep in the left parotid gland lobe, suggesting Warthin's tumor. The resected tumor was histologically diagnosed as oncocytoma of the parotid gland. Despite its relative rarity, this tumor should be completly resected with sufficient margin, as with other benign parotid gland tumors.

DOI： 10.14821/stomatopharyngology.23.237

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Satellite tumors surrounding primary pleomorphic adenomas of the parotid gland Reviewed

Yorihisa Orita, Kazuo Hamaya, Kentaroh Miki, Akiko Sugaya, Misato Hirai, Kiyoko Nakai, Sohichiroh Nose, Tadashi Yoshino

EUROPEAN ARCHIVES OF OTO-RHINO-LARYNGOLOGY 267 ( 5 ) 801 - 806 2010.5

Large cerebellar arteriovenous malformation with dizziness Reviewed

Misato Hirai, Yorihisa Orita, Akiko Sugaya, Kazunori Seno, Koji Yuen

Practica Oto-Rhino-Laryngologica 101 ( 10 ) 759 - 764 2008

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