Details of a Researcher - Hosoya, Makoto

Papers - Hosoya, Makoto

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Low-dose rapamycin-induced autophagy in cochlear outer sulcus cells

Saegusa Chika, Hosoya Makoto, Nishiyama Takanori, Saeki Tsubasa, Fujimoto Chisato, Okano Hideyuki, Fujioka Masato, Ogawa Kaoru

LARYNGOSCOPE INVESTIGATIVE OTOLARYNGOLOGY 5 ( 3 ) 520 - 528 2020.06

ISSN 2378-8038

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A phase I/IIa double blind single institute trial of low dose sirolimus for Pendred syndrome/DFNB4

Fujioka M., Akiyama T., Hosoya M., Kikuchi K., Fujiki Y., Saito Y., Yoshihama K., Ozawa H., Tsukada K., Nishio S.Y., Usami S.I., Matsunaga T., Hasegawa T., Sato Y., Ogawa K.

Medicine (Medicine) 99 ( 19 ) e19763 2020.05

ISSN 0025-7974

INTRODUCTION: Pendred syndrome (PDS)/DFNB 4 is a disorder with fluctuating and progressive hearing loss, vertigo, and thyroid goiter. We identified pathophysiology of a neurodegenerative disorder in PDS patient derived cochlear cells that were induced via induced pluripotent stem cells and found sirolimus, an mTOR inhibitor, as an inhibitor of cell death with the minimum effective concentration less than 1/10 of the approved dose for other diseases. Given that there is no rational standard therapy for PDS, we planned a study to examine effects of low dose oral administration of sirolimus for the fluctuating and progressive hearing loss, and the balance disorder of PDS by daily monitor of their audio-vestibular symptoms. METHODS AND ANALYSIS: This is a phase I/IIa double blind parallel-group single institute trial in patient with PDS/DFNB4. Sixteen of outpatients with fluctuating hearing diagnosed as PDS in SLC26A4 genetic testing aged in between 7 and 50 years old at the time of consent are given either placebo or sirolimus tablet (NPC-12T). In NPC-12T placebo arm, placebo will be given for 36 weeks; in active substance arm, placebo will be given for 12 weeks and the NPC-12T for 24 weeks. Primary endpoints are safety and tolerability. The number of occurrences and types of adverse events and of side effects will be sorted by clinical symptoms and by abnormal change of clinical test results. A 2-sided 95% confidence interval of the incidence rate by respective dosing arms will be calculated using the Clopper-Pearson method. Clinical effects on audio-vestibular tests performed daily and precise physiological test at each visit will also be examined as secondary and expiratory endpoints. TRIAL REGISTRATION NUMBER: JMA-IIA00361; Pre-results.

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A case report of Gorham-Stout disease diagnosed during the course of recurrent meningitis and cholesteatoma

Hosoya M., Oishi N., Nishiyama J., Ogawa K.

Journal of Otolaryngology - Head and Neck Surgery (Journal of Otolaryngology - Head and Neck Surgery) 49 ( 1 ) 18 2020.04

ISSN 1916-0216

© 2020 The Author(s). Background: Gorham-Stout disease is a rare bone disorder. Here, we present a case of Gorham-Stout disease diagnosed during follow-up of a patient with cholesteatoma; the disease affected the temporal bone and other sites of the skull. To the best of our knowledge, this is the first report of Gorham-Stout disease diagnosed with recurrent cerebrospinal leakage after surgery to treat cholesteatoma. Case presentation: A 25-year-old male patient re-presented to our department for the first time in 7 years with otorrhea in the right ear and recurrent meningitis. The patient had a history of multiple surgeries for cholesteatoma and suffered from recurrent cerebrospinal fluid leakage, which initially was thought to be caused by recurrence of cholesteatoma. Therefore, skull base reconstruction was planned. However, the underlying cause was identified eventually as defects in the temporal bone caused by massive osteolysis due to Gorham-Stout disease. Skull base reconstruction was abandoned because the osteolysis was considered to be progressive. Conservative treatment with infectious control was implemented as an alternative. Conclusion: This case describes unusual temporal bone osteolysis after cholesteatoma surgery and the importance of considering the possibility of multiple concurrent diseases in such individuals. The distinguishing features of this case are the fact that the temporal bone had disappeared, and deconstruction was complicated by infection and inflammation caused by cholesteatoma, surgical invasion, and Gorham-Stout disease. Appropriate diagnosis saved the patient from ineffective multiple surgeries for cerebrospinal fluid leakage or cholesteatoma, and improved his quality of life.

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A rare case of bifurcated chorda tympani

Kasahara K., Hosoya M., Oishi N., Ogawa K.

Journal of International Advanced Otology (Journal of International Advanced Otology) 16 ( 1 ) 141 - 144 2020.04

ISSN 13087649

© 2020, AVES. All rights reserved. Preservation of the chorda tympani is important in middle ear surgery to prevent dysgeusia postoperatively. However, determining the exact course of the chorda tympani before surgery is not always possible, especially in cases with accompanying malformations. In this report, we pre-sented an extremely rare case of bifurcation of the chorda tympani in a 15-year-old male patient. We performed tympanoplasty for a middle ear malformation with conductive hearing loss. During the operation, we noticed and carefully preserved the bifurcated chorda tympani. The patient did not develop dysgeusia postoperatively. Appropriate handling and understanding of the anomalous chorda tympani preserved the patient’s sense of taste and hence quality of life.

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A new training method for velopharyngeal dysfunction: Self-inhalation for hypernasality

Kobayashi R., Tsunoda K., Takazawa M., Ueha R., Hosoya M., Fujimaki Y., Nito T., Yamasoba T.

Auris Nasus Larynx (Auris Nasus Larynx) 47 ( 2 ) 250 - 253 2020.04

ISSN 03858146

© 2019 Elsevier B.V. Objective: There are various methods to treat velopharyngeal dysfunction including surgery and rehabilitation therapy. Even if a rehabilitation program is effective, the evaluation of its efficacy remains subjective. In this paper, we propose a new method of rehabilitation training for velopharyngeal dysfunction focusing on the objective peak inspiratory flow (PIF) rate. Methods: Four patients, who were diagnosed with velopharyngeal dysfunction without cleft palate at ENT clinic of the National Hospital Organization, Tokyo Medical Center, participated in this study. All patients underwent our original rehabilitation program for velopharyngeal dysfunction, a method using the In-Check Dial, Turbohaler model. As a self-training rehabilitation program, we asked them to inhale forcefully 10 times daily at home using the In-Check Dial to increase the value of PIF rate for 3 months. We measured the patients’ PIF rates with the In-Check Dial at the ENT clinic at the initial visit and after the 3-month training. Results: The PIF rates of the four patients without nasal clips were higher than the rates with nasal clips at the initial visit. After the training, PIF rate without a nasal clip of all patients increased than the rate at the initial visit, which represented significant difference (P < 0.05). Also, after 3 months, PIF rate without a nasal clip was higher or equal than the rates with a nasal clip at the initial visit except one case. Naso-pharyngo-laryngeal fiberscopy did not detect salivary pooling around larynx and mirror fogging test did not show nasal escape in the three of four patients after 3 months of training. All reported improvement in dysphagia and dysarthria. Conclusion: This new method can be used not only to evaluate velopharyngeal function but also as an effective self-training treatment.

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Distribution of tight junctions in the primate cochlear lateral wall

Saeki T., Hosoya M., Shibata S., Okano H., Fujioka M., Ogawa K.

Neuroscience Letters (Neuroscience Letters) 717 134686 2020.01

ISSN 03043940

© 2019 Elsevier B.V. The blood-labyrinth barrier (BLB) is a major structure that separates the inner ear from the systemic blood circulation. Many drugs cannot access the inner ear because of their inability to cross the BLB. In the cochlea, the BLB is mainly distributed in the lateral wall. However, the ultrastructure of the cochlear lateral wall, including the distribution of tight junctions (TJs), which are its main component, has not been thoroughly examined in primates. This study investigated the distribution of TJs in the cochlear lateral wall of the common marmoset by performing immunohistochemistry for TJ markers and transmission electron microscopy. As previously reported in rodents, TJs were distributed at the lumenal side of stria marginal cells and basal cells. In outer sulcus cells, which are more developed in primates than in rodents, TJs were distributed at the side with the endolymph but not at the side with the spiral ligament, where many capillaries were located. These findings indicate that drugs and small compounds circulating systemically in the blood can easily access outer sulcus cells, but have a limited ability to enter endolymph. No structural differences were detected between species, indicating that the in vivo distribution of drugs in cochlear lateral wall cells, including outer sulcus cells, in primates can be predicted by performing rodent experiments.

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The common marmoset as suitable nonhuman alternative for the analysis of primate cochlear development

Hosoya M., Fujioka M., Murayama A.Y., Okano H., Ogawa K.

FEBS Journal (FEBS Journal) 2020

ISSN 1742464X

© 2020 The Authors. The FEBS Journal published by John Wiley & Sons Ltd on behalf of Federation of European Biochemical Societies Cochlear development is a complex process with precise spatiotemporal patterns. A detailed understanding of this process is important for studies of congenital hearing loss and regenerative medicine. However, much of our understanding of cochlear development is based on rodent models. Animal models that bridge the gap between humans and rodents are needed. In this study, we investigated the development of hearing organs in a small New World monkey species, the common marmoset (Callithrix jacchus). We describe the general stages of cochlear development in comparison with those of humans and mice. Moreover, we examined more than 25 proteins involved in cochlear development and found that expression patterns were generally conserved between rodents and primates. However, several proteins involved in supporting cell processes and neuronal development exhibited interspecific expression differences. Human fetal samples for studies of primate-specific cochlear development are extremely rare, especially for late developmental stages. Our results support the use of the common marmoset as an effective alternative for analyses of primate cochlear development.

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Validation and multidimensional analysis of the japanese penn acoustic neuroma quality-of-life scale

Nishiyama T., Oishi N., Kojima T., Kasuya K., Noguchi M., Ishikawa T., Hosoya M., Ogawa K.

Laryngoscope (Laryngoscope) 2020

ISSN 0023852X

© 2020 The American Laryngological, Rhinological and Otological Society, Inc. Objectives: In the management of vestibular schwannoma (VS), patient-perceived outcomes should be given as much attention as physician-reported objective outcomes. Concomitant symptoms can be annoying for sufferers, lowering quality of life (QoL). However, it is difficult to determine the degree to which these symptoms lower QoL using only objective measures. The Penn Acoustic Neuroma Quality of Life Scale (PANQOL) is a validated disease-specific questionnaire commonly used to assess QoL in VS patients. It was developed and published in English. In this study, we translated the PANQOL questionnaire into Japanese and validated its reliability and utility. We also evaluated patient factors that could influence PANQOL scores. Study Design: Cross-sectional study. Methods: Seventy-two VS patients monitored conservatively were included in this study. They were assessed using the Japanese versions of PANQOL (PANQOL-J) and questionnaires about specific symptoms related to VS. The PANQOL questionnaire was translated into Japanese according to the accepted standards. The reliability and utility of the PANQOL-J questionnaire, as well as the relationship with VS-specific symptoms, were statistically analyzed. Results: The mean total score was 78.7. Almost all of its domains had good internal consistency. Most PANQOL-J domains were related to those of the Short Form-36, with the exception of facial dysfunction domain. Scores of all PANQOL-J domains were comparable to scores of other questionnaires related to the VS-specific symptoms. Our analyses indicated tinnitus or facial palsy might affect the PANQOL-J total score. Conclusions: We confirmed the validity of the Japanese version of PANQOL questionnaire and its reliability and utility in the management of VS symptoms concerning facial function. Level of Evidence: 4 Laryngoscope, 2020.

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Differences in hearing levels between siblings with hearing loss caused by GJB2 mutations

Fujioka M., Hosoya M., Nara K., Morimoto N., Sakamoto H., Otsu M., Nakano A., Arimoto Y., Masuda S., Sugiuchi T., Masuda S., Morita N., Ogawa K., Kaga K., Matsunaga T.

Auris Nasus Larynx (Auris Nasus Larynx) 2020

ISSN 03858146

© 2020 Objective: Hearing loss caused by GJB2 mutations is inherited in an autosomal recessive manner (DFNB1); thus siblings of an affected child have a 25% chance of also being affected. Hearing loss among subsequent siblings carrying the same GJB2 mutation is a concern for parents and a frequent topic of enquiry during genetic counseling. Evidence exists for genotype-phenotype correlations of GJB2 mutations; however, no analysis of differences in hearing among siblings, in whom the common genetic background may decrease variation, has been reported. The purpose of the present study was to investigate hearing differences between siblings with identical GJB2 mutations. Methods: We examined the hearing levels of 12 pairs of siblings; each pair had the same pathogenic GJB2 mutations. Differences in hearing acuity between sibling pairs detected by auditory evaluation. Results: No significant correlation was detected between the average hearing levels of first and second affected siblings. Average differences in acoustic threshold >30 dB were observed between four pairs of siblings, whereas the remaining eight pairs had average threshold values within 20 dB of one another. Conclusion: Our results indicate that auditory acuity would be expected to approximate that found in the first child in approximately 70% of subsequent children with GJB2-mediated hearing loss, whereas 30% of subsequent siblings would have average differences of >30 dB.

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Generation of a human iPS cell line (CGMH.SLC26A4919-2) from a Pendred syndrome patient carrying SLC26A4 c.919-2A>G splice-site mutation

Cheng Y.F., Chan Y.H., Hu C.J., Lu Y.C., Saeki T., Hosoya M., Saegusa C., Fujioka M., Okano H., Weng S.M., Hsu C.J., Chang K.H., Wu C.C.

Stem Cell Research (Stem Cell Research) 40 101524 2019.10

ISSN 18735061

© 2019 SLC26A4 is the second most frequent gene implicated in congenital hearing loss after GJB2 mutations. Here, we report the generation of induced pluripotent stem cells (iPSCs), from a patient who was carrying a homozygous c.919-2A>G variant in the SLC26A4 gene. This is the most common variant of SLC26A4 gene in the Chinese population and the second most prevalent one in other Asian countries. The established patient-derived iPSC displayed all the features of pluripotent stem cell markers and had the ability to differentiate into all of the three germ layers and possessed a normal karyotype.

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症例をどうみるか　けいれん発作後に両側混合性難聴を呈した両側耳硬化症術後の1例

細谷誠, 大石直樹, 石川雄惟, 小川郁

JOHNS ((株)東京医学社) 35 ( 8 ) 1021 - 1024 2019.08

ISSN 0910-6820

症例は50代女性で、両側耳硬化症に対して両側アブミ骨手術を行い、当院で定期フォローされていた。1週間前に職場でけいれん発作を起こし、他院救急外来に救急搬送された。けいれん発作後、自覚的に両側難聴が続いたため、ステロイド点滴加療を強く希望し、当院耳鼻咽喉科外来を受診した。けいれん発作前の当院最終受診時の検査結果と比較すると、気骨導差の増悪を認めるとともに、骨導聴力、気導聴力ともに閾値の上昇を認めた。入院後も自覚症状の増悪に伴い、両側の混合性難聴が進行した。入院翌日に緊急で行ったABRでは、両側とも40dBでV波を認め、両側の機能性難聴と診断された。5日間の入院後、退院となった。退院後、自覚症状、検査所見とも改善傾向にあり、今回のエピソードの発端となったけいれん発作の原因は不明であった。

Estimating the concentration of therapeutic range using disease-specific iPS cells: Low-dose rapamycin therapy for Pendred syndrome

Hosoya M., Saeki T., Saegusa C., Matsunaga T., Okano H., Fujioka M., Ogawa K.

Regenerative Therapy (Regenerative Therapy) 10 54 - 63 2019.06

ISSN 2352-3204

© 2018 The Japanese Society for Regenerative Medicine Introduction: Pendred syndrome is an autosomal-recessive disease characterized by congenital hearing loss and thyroid goiter. Previously, cell stress susceptibilities were shown to increase in patient-derived cells with intracellular aggregation using an in vitro acute cochlear cell model derived from patient-specific pluripotent stem (iPS) cells. Moreover, we showed that rapamycin can relieve cell death. However, studies regarding long-term cell survival without cell stressors that mimic the natural course of disease or the rational minimum concentration of rapamycin that prevents cell death are missing. Methods: In this report, we first investigated the rational minimum concentration of rapamycin using patient-specific iPS cells derived-cochlear cells with three different conditions of acute stress. We next confirmed the effects of rapamycin in long-term cell survival and phenotypes by using cochlear cells derived from three different patient-derived iPS cells. Results: We found that inner ear cells derived from Pendred syndrome patients are more vulnerable than those from healthy individuals during long-term culturing; however, this susceptibility was relieved via treatment with low-dose rapamycin. The slow progression of hearing loss in patients may be explained, in part, by the vulnerability observed in patient cells during long-term culturing. We successfully evaluated the rational minimum concentration of rapamycin for treatment of Pendred syndrome. Conclusion: Our results suggest that low-dose rapamycin not only decreases acute symptoms but may prevent progression of hearing loss in Pendred syndrome patients.

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高齢者における人工内耳植込術前後のうつおよび不安傾向の変化

新井雄裕, 細谷誠, 前川明日彩, 片岡ちなつ, 神崎晶, 小川郁

Audiology Japan ((一社)日本聴覚医学会) 62 ( 3 ) 205 - 210 2019.06

ISSN 0303-8106

人工内耳の装用者数が年々増加する中で、付随して生じる心理的諸問題についても注目されるようになってきた。その中で、人工内耳の装用が患者のうつ状態に及ぼす影響はすでに複数の調査が行われているが、本研究においては当科で人工内耳植込術を行った高齢者11名を対象とし、うつ性自己評価尺度(Self-rating Depression Scale:以下SDS)及び状態-特性不安尺度(State-Trait Anxiety Inventory:以下STAI)を術前後に1回ずつ実施し、うつおよび不安状態についての検討を行った。その結果、人工内耳装用により患者の抑うつ傾向は全体としては有意な変化を認めなかったが、一部の症例においては聴能の再獲得などにより抑うつ傾向が高まる可能性が示唆された。人工内耳植込術をした患者の精神的な面を把握して、リハビリテーションに生かすために、術前術後のSTAI、SDSによる抑うつ傾向の評価は臨床的に有用であると考えられた。(著者抄録)

成人人工内耳手術前後におけるエゴグラムの変化

前川明日彩, 細谷誠, 片岡ちなつ, 新井雄裕, 浅野和海, 森隆範, 野口勝, 神崎晶, 小川郁

Audiology Japan ((一社)日本聴覚医学会) 62 ( 2 ) 150 - 156 2019.04

ISSN 0303-8106

当院で人工内耳手術を施行し、定期的な診療を続けている成人人工内耳装用者17名を65歳以上の高齢群と65歳未満の若年群に分け、手術前後に実施した東大式エゴグラム(Tokyo University Egogram、以下TEG)の結果について検討を行った。さらに、失聴期間2年未満群と2年以上群での検討も行った。全症例及び高齢群で、術後「他者本位・自己抑制的・自己否定的」な傾向が低下した。若年群では「自由・積極的・自己肯定的」な傾向に上昇する様子がみられた。人工内耳を装用して音を聴取することで、高齢者はより自律的に、若年者はより能動的になる傾向があると考えた。失聴期間別の検討では、失聴期間2年以上群で、術後の「他者本位・自己抑制的・自己否定的」な傾向が低下した。音が聴取可能になり活気が出たため、抑制的な自我状態が軽減したと推察した。高齢者や失聴期間の長い症例においても、人工内耳導入により自己否定傾向を軽減させる効果があると考えられた。(著者抄録)

ClinGen expert clinical validity curation of 164 hearing loss gene–disease pairs

DiStefano M., Hemphill S., Oza A., Siegert R., Grant A., Hughes M., Cushman B., Azaiez H., Booth K., Chapin A., Duzkale H., Matsunaga T., Shen J., Zhang W., Kenna M., Schimmenti L., Tekin M., Rehm H., Tayoun A., Amr S., Abdelhak S., Alexander J., Avraham K., Bhatia N., Bai D., Boczek N., Brownstein Z., Burt R., Bylstra Y., del Castillo I., Choi B., Downie L., Friedman T., Giersch A., Goh J., Greinwald J., Griffith A., Hernandez A., Holt J., Hosoya M., Ying L., Jain K., Kim U., Kremer H., Krantz I., Leal S., Lewis M., Liu X., Low W., Lu Y., Luo M., Masmoudi S., Ming T., Moreno-Pelayo M., Morín M., Morton C., Murray J., Mutai H., Nara K., Pandya A., Pei-Rong S., Smith R., Jamuar S., Suer F., Usami S., Van Camp G., Yamazawa K., Yuan H., Black-Zeigelbein E., Zhang K.

Genetics in Medicine (Genetics in Medicine) 21 ( 10 ) 2239 - 2247 2019

ISSN 10983600

© 2019, American College of Medical Genetics and Genomics. Purpose: Proper interpretation of genomic variants is critical to successful medical decision making based on genetic testing results. A fundamental prerequisite to accurate variant interpretation is the clear understanding of the clinical validity of gene–disease relationships. The Clinical Genome Resource (ClinGen) has developed a semiquantitative framework to assign clinical validity to gene–disease relationships. Methods: The ClinGen Hearing Loss Gene Curation Expert Panel (HL GCEP) uses this framework to perform evidence-based curations of genes present on testing panels from 17 clinical laboratories in the Genetic Testing Registry. The HL GCEP curated and reviewed 142 genes and 164 gene–disease pairs, including 105 nonsyndromic and 59 syndromic forms of hearing loss. Results: The final outcome included 82 Definitive (50%), 12 Strong (7%), 25 Moderate (15%), 32 Limited (20%), 10 Disputed (6%), and 3 Refuted (2%) classifications. The summary of each curation is date stamped with the HL GCEP approval, is live, and will be kept up-to-date on the ClinGen website (https://search.clinicalgenome.org/kb/gene-validity). Conclusion: This gene curation approach serves to optimize the clinical sensitivity of genetic testing while reducing the rate of uncertain or ambiguous test results caused by the interrogation of genes with insufficient evidence of a disease link.

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Intraoperative facial nerve monitoring revealed the origin of rapidly progressing schwannoma in the cerebellopontine angle: A case of large intermediate nerve schwannoma

Hosoya M., Oishi N., Noguchi M., Kasuya K., Nishiyama T., Ishikawa T., Kasahara K., Miyazaki H., Ogawa K.

Journal of International Advanced Otology (Journal of International Advanced Otology) 14 ( 3 ) 488 - 492 2018.12

ISSN 13087649

© 2018 by The European Academy of Otology and Neurotology and The Politzer Society Schwannoma arising from the intermediate nerve is very rare, with only a few cases reported in the literature. Here, we report a rare case of a rapidly growing schwannoma that originated from the intermediate nerve in a 17-year-old woman. We performed tumor resection using the translabyrinthine approach with continuous intraoperative monitoring, in which the facial nerve root was used to evoke muscle action potential. The monitoring revealed the tumor origin and enabled complete surgical resection with the preservation of facial nerve motor function. The facial nerve root evoked muscle action potential-based continuous monitoring revealed the origin of the rare intermediate nerve schwannoma. Appropriate surgery using this monitoring system could spare facial nerve function even in this rare case and retain the patient’s quality of life.

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Vocal Hygiene Education Program Reduces Surgical Interventions for Benign Vocal Fold Lesions: A Randomized Controlled Trial

Hosoya M., Kobayashi R., Ishii T., Senarita M., Kuroda H., Misawa H., Tanaka F., Takiguchi T., Tashiro M., Masuda S., Hashimoto S., Goto F., Minami S., Yamamoto N., Nagai R., Sayama A., Wakabayashi T., Toshikuni K., Ueha R., Fujimaki Y., Takazawa M., Sekimoto S., Itoh K., Nito T., Kada A., Tsunoda K.

Laryngoscope (Laryngoscope) 128 ( 11 ) 2593 - 2599 2018.11

ISSN 0023852X

© 2018 The Authors. The Laryngoscope published by Wiley Periodicals, Inc. on behalf of The American Laryngological, Rhinological and Otological Society, Inc. Objectives/Hypothesis: Vocal fold polyps and nodules are common benign laryngeal lesions. Currently, the Japanese health insurance system covers surgical interventions. However, the establishment of more cost-effective conservative methods is required, because healthcare costs are viewed as a major concern, and the government and taxpayers are demanding more economical, effective treatments. In this situation, more suitable vocal hygiene education may be important for the success of cost-effective conservative treatment. In this study, we developed a novel reinforced vocal hygiene education program and compared the results of this program with those of previous methods of teaching vocal hygiene. Study Design: Multicenter randomized controlled trial. Methods: Patients who visited a National Hospital Organization (NHO) hospital for the surgical indication of hoarseness were included in the study. Before undergoing surgery, 200 patients with benign vocal fold lesions (vocal fold polyps/nodules) were enrolled and randomly allocated to the NHO-style vocal hygiene educational program (intervention group) or control education program (control group). Two months after enrollment, the patients in both groups underwent laryngeal fiberscopic examinations to determine whether the benign lesions had resolved or whether surgery was indicated for the vocal fold polyps/nodules. Results: After 2 months, in the intervention group, the proportion of lesion resolution (61.3%) was significantly greater than that in the control group (26.3%) (P <.001, Fisher exact test). Conclusions: Our results clearly indicate that the quality and features of the education program could affect the outcome of the intervention. We found that a reinforced vocal hygiene education program increased the rate of the resolution of benign vocal fold polyps and nodules in a multicenter randomized clinical trial. Level of Evidence: 1b Laryngoscope, 2593–2599, 2018.

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Elongated EABR wave latencies observed in patients with auditory neuropathy caused by OTOF mutation

Hosoya M., Minami S., Enomoto C., Matsunaga T., Kaga K.

Laryngoscope investigative otolaryngology (Laryngoscope investigative otolaryngology) 3 ( 5 ) 388 - 393 2018.10

ISSN 0023852X

Objectives: We sought to determine how the pathology altered electrically evoked auditory brainstem responses (EABRs) in patients with hearing loss by evaluating EABRs in auditory neuropathy patients with OTOF mutations comparing with various types of congenital deafness. Methods: We included 15 patients with congenital hearing loss, grouped according to pathology: OTOF mutations (n = 4), GJB2 mutations (n = 4), SLC26A4 mutations (n = 4), or cytomegalovirus infections (n = 3). EABRs were recorded when patients underwent cochlear implantation surgery. We evaluated the latencies and amplitudes of the recorded EABRs and compared them statistically between four groups. Results: The EABR latencies of Wave III and Wave V, and of the interval between them, were significantly longer in the OTOF mutation group than in the GJB2 and SLC26A4 mutation groups (Wave III) and in all three other groups (Wave V and Wave III-V latency); amplitudes were not significantly different between groups. Conclusions: Our results suggest OTOF mutations cause delayed (or slowed) postsynaptic neurotransmission, although the presumed mechanism involved reduced presynaptic transmission between hair cells and spiral ganglion neurons. Level of Evidence: Mainly a case report.

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A rare case of bilateral vagus nerve schwanomatosis

Hosoya, Makoto, Yamamoto, Nobuko, Wakabayashi, Takeshi, Toshikuni, Keitaro, Yabe, Haruna, Minami, Shujiro B.

AURIS NASUS LARYNX (Auris Nasus Larynx) 45 ( 4 ) 871 - 874 2018.08

Research paper (scientific journal), ISSN 0385-8146

© 2017 Elsevier B.V. Schwanomatosis is the third most common form of neurofibromatosis. Schwanomatosis affecting the vagus nerve is particularly rare. In this report, we describe an extremely rare case bilateral vagus nerve schwanomatosis in a 45-year-old male patient. The patient initially presented with bilateral neck tumors and hoarseness arising after thoracic surgery. We performed left neck surgery in order to diagnose and resect the remaining tumors followed by laryngeal framework surgery to improve vocal cord closure and symptoms of hoarseness. Voice recovery was successfully achieved after surgery. An appropriate diagnosis and surgical tumor resection followed by phonosurgery improved patient quality of life in this rare case.

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Expression pattern of EYA4 in the common marmoset (Callithrix jacchus) cochlea

Matsuzaki, Saeko, Hosoya, Makoto, Okano, Hideyuki, Fujioka, Masato, Ogawa, Kaoru

NEUROSCIENCE LETTERS (Neuroscience Letters) 662 185 - 188 2018.01

Research paper (scientific journal), ISSN 0304-3940

© 2017 The eyes absent (EYA)-like genes are essential for the formation of sensory organs among fly (Drosophila melanogaster) and mammals. EYA4, one of the vertebrate genes of Eya family, is reported to be causative for late-onset mid-frequency sensorineural hearing loss in humans, while Eya4-deficient mice exhibited congenital profound deafness and otitis media with effusion due to the eustachian tube dysmorphology. Because of the species difference in the phenotype, the pathophysiology of EYA4 in the human cochlea has yet to be elucidated. Here, we examine the expression pattern of EYA4 in the cochlea of common marmoset (Callithrix jacchus), a non-human primate. The results indicated a distinct expression pattern of EYA4 in the adult marmoset cochleae, especially strong in all supporting cells, while in mouse their expressions were diminished. Interestingly, EYA4 expression in the hair cells, supporting cells and neurons was co-localized with sine oculis homeobox–SIX1, a transcription factor essential for the transcriptional activity of EYA4. The results revealed inter-species differences in the expression pattern of EYA4 gene in supporting cells between primates and rodents. The results also indicated a fundamental role of EYA4 in the primate auditory cells. Experiments with primate models such as marmosets or with human cochlear cells may provide cues about the unknown pathogenesis of EYA4-related hearing loss.

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