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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Papers - SUGAYA AKIKO