記述言語：英語   掲載種別：研究論文（学術雑誌）  

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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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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担当区分：筆頭著者,　責任著者   記述言語：英語   掲載種別：研究論文（学術雑誌）  

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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記述言語：英語   掲載種別：研究論文（学術雑誌）  

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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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(有)科学評論社  

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記述言語：英語  

DOI： 10.1007/s00439-021-02392-y

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

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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記述言語：英語   掲載種別：研究論文（学術雑誌）  

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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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

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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記述言語：英語   掲載種別：研究論文（学術雑誌）  

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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掲載種別：研究論文（学術雑誌）  

DOI： 10.5631/jibirin.114.957

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

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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担当区分：筆頭著者,　責任著者  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1097/MAO.0000000000001874

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

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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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1080/00016489.2017.1378435

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記述言語：日本語   出版者・発行元：(一社)日本遺伝カウンセリング学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

DOI： 10.1097/MAO.0000000000001373

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記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Oto-Rhino-Laryngological Society of Japan Inc.  

Since April 2012, genetic testing for congenital hearing loss is covered by the public health insurance in Japan. Recent(since August 2015)developments in next-generation sequencing technology have enabled the detection of 154 mutations in 19 genes. Genetic testing provides valuable information on hearing phenotype, prognosis, and prediction of associated symptoms. We report a hearing-impaired patient in whom multiple genetic mutations were detected. This patient carries two missense mutations in GJB2(p.G45E, p.Y136X), as well as a mitochondrial mutation(7445A&gt
G). Since the number of genes detectable by genetic testing has increased, the diagnosis of hearing loss can be made with greater accuracy. However, it is often difficult to clinically understand and interpret the genotype information, especially when multiple gene variants are detected in one patient or family. Genetic counseling plays an important part in the intervention for or follow-up of such patients. Genotypic and phenotypic information of other family members is necessary, so that both the patient and the family can understand and accept the results of genetic testing.

DOI： 10.3950/jibiinkoka.120.131

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記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Oto-Rhino-Laryngological Society of Japan Inc.  

In general, patients with recent versions of cochlear implants(CIs)can safely undergo Magnetic Resonance Imaging (MRI) within 1.5 T scanners, without the need of inner magnet removal surgery. However, even if they wear tight head bandages on their inner coil, complications can occur in CI users during MRI, such as pain, skin reaction, demagnetization of the internal magnet, and dislocation of the magnet out of its silicon flange. We encountered two cases of reversal of the inner magnet of a CI during MRI examination. In both cases, the inner magnets were reversed by 180 degrees, although they remained in the silicon flange. We ordered a polar-changed extra magnet for replacing the reversed inner magnet in one case, and performed a re-implant surgery of the inner magnet in the other. In recent years, the number of hearing-impaired patients with CIs has been increasing along with expansion of the medical indications for CI in the rapidly aging population of the developed countries. On the other hand, MRI is an indispensable clinical diagnostic tool in modern medical practice. Physicians and paramedics must be aware of possible complications that can occur during MRI in patients with CIs. Prompt assessment and appropriate treatment are important in patients with CIs developing complications during MRI examination.

DOI： 10.3950/jibiinkoka.120.727

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER SCI LTD  

Central auditory processing disorder (CAPD) is a condition in which dysfunction in the central auditory system causes difficulty in listening to conversations, particularly under noisy conditions, despite normal peripheral auditory function. Central auditory testing is generally performed in patients with normal hearing on the pure tone audiogram (PTA). This report shows that diagnosis of CAPD is possible even in the presence of an elevated threshold on the PTA, provided that the normal function of the peripheral auditory pathway was verified by distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), and auditory steady state response (ASSR). Three pediatric cases (9- and 10-year-old girls and an 8-year-old boy) of CAPD with elevated thresholds on PTAs are presented. The chief complaint was difficulty in listening to conversations. PTA showed elevated thresholds, but the responses and thresholds for DPOAE, ABR, and ASSR were normal, showing that peripheral auditory function was normal. Significant findings of central auditory testing such as dichotic speech tests, time compression of speech signals, and binaural interaction tests confirmed the diagnosis of CAPD. These threshold shifts in PTA may provide a new concept of a clinical symptom due to central auditory dysfunction in CAPD. (C) 2016 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.anl.2016.02.005

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：TAYLOR & FRANCIS LTD  

Conclusion: Audiological parameters alone do not determine the choice to use hearing aids (HA). Subjective hearing-related QoL is a major factor that determines whether or not an older person will continue to wear HA.Objective: This study aimed to identify which audiological parameters and quality-of-life (QoL) measures determine whether or not older persons will continue wearing HA.Methods: Charts of 157 patients aged 65 years who attended the HA service unit at the Otolaryngology Department were retrospectively reviewed. After HA fitting and a trial, the patients were divided into groups, depending upon whether or not they wanted to continue wearing the HA (users, 58.2%; non-users, 41.8%) and then audiological parameters were compared between them. At least 4 months after the HA fitting, the self-reported QoL questionnaire, Hearing Handicap Inventory for the Elderly (HHIE), was mailed to all 157 patients and HHIE scores were compared between HA users and non-users.Result: Speech discrimination score and dynamic range did not significantly differ between HA users and non-users. A difference in the average hearing threshold was marginally significant. The response rate to the HHIE was 65.2%. Total HHIE and emotional scores were higher (more impaired) among HA users than non-users.

DOI： 10.1080/00016489.2016.1183817

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：TAYLOR & FRANCIS LTD  

Conclusion: The prevalence of low-tone hearing loss (LTHL) is significantly high in spinocerebellar degeneration (SCD) with cerebellar predominance, including multiple-system atrophy C (MSA-C) and cortical cerebellar atrophy (CCA). Objective: This study aimed to test the hypothesis that SCD with cerebellar predominance, MSA-C and CCA may cause auditory symptoms. Methods: The shape and threshold of pure-tone audiograms were evaluated for MSA-C (n = 47; mean (+/- SD) age, 61.6 +/- 8.9 years), CCA (n = 16; 62.8 +/- 9.5 years), and age-matched controls (n = 169; 62.5 +/- 10.7 years). To differentiate specific hearing loss for MSA-C and CCA from presbycusis, the shape of audiograms was examined based on previously established audiological criteria. Results: When audiogram shape was defined according to audiological criteria, the odds ratio for LTHL in SCD compared to controls was 2.492 (95% confidence interval (CI) = 1.208-5.139; p &lt; 0.05, Pearson's Chi-square test) in MSA-C and 2.194 (95% CI = 0.709-6.795) in CCA. When the selection of audiogram shape according to these criteria was verified by three certified audiologists, odds ratios for LTHL in MSA-C and CCA were 3.243 (95% CI = 1.320-7.969) and 3.692 (95% CI = 1.052-12.957), respectively, significantly higher than in controls.

DOI： 10.3109/00016489.2015.1104722

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担当区分：筆頭著者,　責任著者   記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER IRELAND LTD  

Objectives: Early hearing detection and intervention (EHDI) is critical for achievement of age-appropriate speech perception and language development in hearing-impaired children. It has been 15 years since newborn hearing screening (NHS) was introduced in Japan, and its effectiveness for language development in hearing-impaired children has been extensively studied. Moreover, after over 20 years of cochlear implantation in Japan, many of the prelingual cochlear implant (CI) users have reached school age, and the effect of Clan language development have also been assessed. To identify prognostic factors for language development, audiological/language test scores and demographic factors were compared among prelingual severe-to-profound hearing-impaired children with CI divided into subgroups according to age at first hearing aid (HA) use and whether they received NHS.
Methods: Prelingual severe-to-profound deafened children from the Research on Sensory and Communicative Disorders (RSCD) project who met the inclusion criteria were divided into groups according to the age (in months) of HA commencement (before 6 months: group A, after 7 months: group B), and the presence or absence of NHS (groups C and D). Language development and socio-economic data were obtained from audiological/language tests and a questionnaire completed by caregivers, respectively.
Results: In total, 210 children from the RSCD project participated in this study. Group A (n = 49) showed significantly higher scores on comprehensive vocabulary and academic achievement (p &lt; 0.05) than group B (n = 161), with no difference in demographics except for significantly older age in group B. No differences in language scores were observed between group C (n = 71) and group D (n = 129), although participants of group D was significantly older and had used CIs longer (p &lt; 0.05).
Conclusions: Early use of HAs until the CI operation may result in better language perception and academic achievement among Cl users with prelingual deafness. A long-term follow-up is required to assess the usefulness of NHS for language development. (C) 2015 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.ijporl.2015.09.036

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER SCI LTD  

Charcot-Marie-Tooth disease (CMT) is the most common form of hereditary sensorimotor neuropathy and sometimes involves disorders of the peripheral auditory system. We present a case of steroid-dependent auditory neuropathy associated with CMT, in which the patient experienced 3 episodes of acute exacerbation of hearing loss and successful rescue of hearing by prednisolone. An 8-year-old boy was referred to the otolaryngology department at the University Hospital. He had been diagnosed with CMT type 1 (demyelinating type) at the Child Neurology Department and was suffering from mild hearing loss due to auditory neuropathy. An audiological diagnosis of auditory neuropathy was confirmed by auditory brainstem response and distortion-product otoacoustic emissions. At 9 years and 0 months old, 9 years and 2 months old, and 10 years and 0 months old, he had experienced acute exacerbations of hearing loss, each of which was successfully rescued by intravenous or oral prednisolone within 2 weeks. Steroid-responsive cases of CMT have been reported, but this is the first case report of steroid-responsive sensorineural hearing loss in CMT. The present case may have implications for the mechanisms of action of glucocorticoids in the treatment of sensorineural hearing loss. (C) 2014 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.anl.2014.11.002

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SAGE PUBLICATIONS INC  

Objective: This study examines ABHD12 mutation analysis in 2 PHARC patients, originally thought to be Usher syndrome.
Methods: The ABHD12 gene of 2 patients, who suffered from deaf-blindness and dysfunctional central and peripheral nervous systems, were sequenced.
Results: We identified that both cases carried the same novel splice site mutation in the ABHD12 gene. However, 1 had epilepsy and the other had peripheral neuropathy. Based on haplotype analysis, the mutation is likely not a hot spot, but rather could be attributable to a common ancestor.
Conclusion: This study, shows that PHARC has phenotypic variability, even within a family, which is consistent with previous reports. Differential diagnosis of "deaf-blindness" diseases is crucial. Confirming the presence of associated symptoms is necessary for differentiating some deaf-blindness syndromes. In addition, mutation analysis is a useful tool for confirming the diagnosis.

DOI： 10.1177/0003489415574513

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科学会  

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担当区分：責任著者   記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER IRELAND LTD  

Objective: Permanent hearing impairment has a life-long impact on children and its early identification is important for language development. A newborn hearing screening (NHS) program has started in Okayama Prefecture, Japan, in 1999 to detect hearing impairment immediately after birth. We aim to examine the effect of this screening program on vocabulary development in pre-school children in a before and after comparative study design.
Methods: A total of 107 5-year-old children who graduated from Okayama Kanariya Gakuen (an auditory center for hearing-impaired children) between 1998 and 2011 were enrolled in this study. The pre-NHS group (n = 40) was defined as those who graduated between 1998 and 2003, while the post-NHS group (n = 67) was defined as those who graduated between 2004 and 2011. The primary outcome was receptive vocabulary, which was assessed by the Picture Vocabulary Test [score &lt;18 (low) vs. score &gt;18 (high)]. The secondary outcome was productive vocabulary, or the number of productive words, which was assessed by an original checklist [&lt;1773 words (low) vs. &gt;1773 (high)]. We calculated odds ratios and 95% confidence intervals for vocabulary development and compared both groups.
Results: The adjusted Picture Vocabulary Test score and number of productive words were significantly higher (p &lt; 0.01) in the post-NHS group than the pre-NHS group. Odds ratios were 2.63 (95% confidence interval: 1.17-5.89) for receptive vocabulary and 4.17 (95% confidence interval: 1.69-10.29) for productive vocabulary.
Conclusions: The introduction of NHS in Okayama Prefecture significantly improved both receptive and productive vocabulary development in hearing-impaired children. (C) 2014 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.ijporl.2014.12.006

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：TAYLOR & FRANCIS LTD  

Conclusion: Performance in consonant-vowel (CV) monosyllable speech perception after cochlear implantation (CI) in the elderly (&gt;= 65 years) is equivalent to that of young adults (18-64 years). Present data in the Japanese language supported the indication for CI in the elderly. Word recognition after CI was significantly lower in the elderly than young adults. Objective: This study compared outcomes of monosyllable perception and word recognition after CI between elderly patients and young adults with post-lingual deafness. Age-related differences in CV monosyllable speech perception in Japanese were examined. Methods: A retrospective chart review of 137 patients with post-lingual deafness who underwent CI at Okayama University Hospital during 1992-2014 [young adults aged 18-64 years (n = 96) and elderly aged &gt;= 65 years (n = 41) at implantation] was conducted. CV monosyllable speech perception post-CI was compared among age groups (18-29, 30-39, 40-49, 50-59, 60-69, 70-79, 80-89). CV monosyllable perception and word recognition scores post-CI were compared between the elderly and young adults. Results: There was no significant difference in CV monosyllable speech perception among age groups. CV monosyllable speech perception in the elderly (61.4 +/- 25.5%) did not differ from that of young adults (65.9 +/- 24.8%). Word recognition scores were significantly lower in the elderly (64.3 +/- 28.1%) than young adults (80.4 +/- 25.9%) (p &lt; 0.05).

DOI： 10.3109/00016489.2015.1062547

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記述言語：日本語   出版者・発行元：日本聴覚医学会  

DOI： 10.4295/audiology.58.335

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その他リンク： http://search.jamas.or.jp/link/ui/2016019205

記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Oto-Rhino-Laryngological Society of Japan Inc.  

The presence of hearing impairment deteriorates patients' quality of life by affecting their daily-life communication. Several self-report assessments are available to evaluate the subjective outcome of hearing aids, however, the procedures to evaluate the impairment of communication induced by hearing impairment has not been well established. A PC-recorded audio file prepared to evaluate the communication ability of hearing aid users, by extracting fifteen spoken language items from the Communication ADL Test: sp-CADL. A before and after study was performed among the hearing aid users who were attending Okayama University Hospital to evaluate the improvement in each of the 15 sp-CADL scores. Twenty-one adults were included in this study whose age ranged from 22 to 83 years old (mean: 56.7 years old). The total and individual scores of the sp-CADL improved significantly after the use of the hearing aids (p &gt
0.05). The total sp-CADL score of the patients with hearing aids showed a good correlation with the best scores of the speech discrimination test measured before wearing hearing aids. CADL may be used for measuring communicative outcomes in patients using hearing aids.

DOI： 10.3950/jibiinkoka.117.1126

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担当区分：筆頭著者   記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SAGE PUBLICATIONS INC  

Objective: Decreasing language delay in hearing-impaired children is a key issue in the maintenance of their quality of life. Language training has been presented mainly by experience-based training; effective intervention programs are crucially important for their future. The aim of this study was to confirm the efficacy of 6-month domain-based language training of school-age, severe-to-profound hearing-impaired children.
Methods: We conducted a controlled before-after study involving 728 severe-to-profound prelingual hearing-impaired children, including an intervention group (n = 60), control group (n = 30), and baseline study group (n = 638). Language scores of the participants and questionnaires to the caregivers/therapists were compared before and after the intervention. Average monthly increase in each language score of the control group and baseline study group were compared with those of the intervention group.
Results: Language scores and the results of the questionnaire of the intervention group showed a significant improvement (P &lt; .05). The average monthly language growth of the intervention group was twice that of the control group and 3 to 4 times that of the baseline study group (P &lt; .05). The effect size was largest in communication (1.914), followed by syntax (0.931).
Conclusion: Domain-based language training improved the language development and daily communication of hearing-impaired children without any adverse effects.

DOI： 10.1177/0003489414525023

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記述言語：日本語   掲載種別：研究論文（学術雑誌）  

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ELSEVIER SCI LTD  

Perilymphatic fistula (PLF) is defined as an abnormal leakage between perilymph from the labyrinth to the middle ear. Symptoms include hearing loss, tinnitus, and vertigo. The standard mode of PLF detection is intraoperative visualization of perilymph leakage and fistula, which ostensibly confirms the existence of PLF. Other possible methods of diagnosis include confirmation of pneumolabyrinth via diagnostic imaging. Recently, a cochlin-tomoprotein (CTP) detection test has been developed that allows definitive diagnosis of PLF-related hearing loss.
We report the case of a 45-year-old man who presented with right-sided tinnitus, hearing loss, and dizziness 30 years after stapes surgery. Middle ear lavage was performed after myringotomy. A preoperative diagnosis of PLF was reached using the CTP detection test. Intraoperative observations included a necrotic long process of the incus, displaced wire piston, and fibrous tissue in the oval window. Perilymph leakage was not evident. The oval window was closed with fascia, and vertigo disappeared within 2 weeks postoperatively. When PLF is suspected after stapes surgery, the CTP detection test can be a useful, highly sensitive, and less invasive method for preoperative diagnosis. (C) 2012 Elsevier Ireland Ltd. All rights reserved.

DOI： 10.1016/j.anl.2012.08.001

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ANNALS PUBL CO  

Objectives: Language development is a key issue in hearing-impaired children. However, interpersonal differences complicate our understanding of the situation. The bimodal or trimodal distribution of language scores in our other reports in this publication imply the presence of fundamental differences among these groups. The characteristic aspects of each group were profiled according to language data.
Methods: We divided 268 children with prelingual severe to profound hearing impairment into 3 groups according to their trimodal distribution observed on histogram-based analysis of their responses to the Test of Question-Answer Interaction Development. Test results in several language domains, including productive and comprehensive vocabulary, productive and comprehensive syntax, and academic achievement, were profiled and compared among these 3 groups.
Results: Significant differences were observed in the results of the Word Fluency Test, the Picture Vocabulary Test Revised, and the Syntax Test of Aphasia among the 3 groups. No significant difference was observed between groups who were lower-scoring and intermediate-scoring on the academic achievement tests referred to as Criterion Referenced Test II and the Standardized Comprehension Test for Abstract Words. Only the higher-scoring group showed excellent results. The demographic factors were not significantly different among the 3 groups.
Conclusions: Relatively poor academic achievement despite fair language production was the dominant feature of the intermediate-scoring group. This profile might correlate with academic failure in school.

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ANNALS PUBL CO  

Objectives: Early identification and intervention for prelingual bilateral severe to profound hearing loss is supposed to reduce the delay in language development. Many countries have implemented early detection and hearing intervention and conducted regional universal newborn hearing screening (UNHS). However, the benefits of UNHS in later childhood have not yet been confirmed, although language development at school age has a lifelong impact on children's future. Our Research on Sensory and Communicative Disorders project attempted to reveal the effects of UNHS and those of early intervention on the development of verbal communication in Japanese children.
Methods: In this study, 319 children with prelingual bilateral severe to profound hearing loss, 4 to 10 years of age, were evaluated with the Test of Question-Answer Interaction Development used as an objective variable. Participation in UNHS and early intervention were used as explanatory variables. The adjusted odds ratio (AOR) was calculated after adjusting several confounding factors with use of logistic regression analysis. In addition, caregivers' answers were obtained by a questionnaire, and the process of diagnosis with and without UNHS was analyzed retrospectively.
Results: Early intervention was significantly associated with better language development (AOR, 3.23; p &lt; 0.01). Participation in UN HS may contribute to better language development to some extent (AOR, 1.32), but not one that was statistically significant (p = 0.37). However, UNHS was significantly associated with early intervention (AOR, 20.21; p &lt; 0.001). The questionnaire results indicated a lag in treatment after UNHS in more than 40% of screened cases.
Conclusions: Early intervention strongly influenced language development. It is necessary to ensure that early identification leads directly to early intervention.

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ANNALS PUBL CO  

Objectives: The measurement of language development in hearing-impaired children is an important step in assessing the appropriateness of an intervention. We proposed a set of language tests (the Assessment Package for Language Development in Japanese Hearing-Tmpaired Children [ALADJIN]) to evaluate the development of practical communication skills. This package consisted of communication skills (TQAID), comprehensive (PVT-R and SCTAW) and productive vocabulary (WFT), comprehensive and productive syntax (STA), and the STRAW.
Methods: A total of 638 children with greater than 70-dB hearing impairment were subjected to this set of language tests. Additional tests, including the PARS, the RCPM, and parental questionnaires, were administered to assess the backgrounds of the children.
Results: A trimodal distribution was observed among hearing-impaired children by the histogram-based analysis of each test.
Conclusions: The ALADJIN is a useful Japanese-language evaluation kit for hearing-impaired children.

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：ANNALS PUBL CO  

Objectives: This study examined syntactic development of auditory comprehension of sentences in Japanese-speaking school-age children with and without hearing impairment.
Methods: In total, 592 preschool and school-age children (421 normal-hearing and 171 hearing-impaired) were included in this cross-sectional observation study conducted using the Syntactic Processing Test for Aphasia for Japanese language users. Linear regression analysis was used to determine the estimated age at which each syntactic structure was acquired.
Results: Acquisition of syntactic structures was observed in hearing-impaired and normal-hearing children. Basic word order sentences of agent-object-verb and the goal benefactive construction were acquired at preschool age (earlier group), whereas reverse word order sentences of object-agent-verb, source benefactive construction, passive voice, and relative clauses were acquired at school age (later group). The results showed that many hearing-impaired children may not acquire Japanese grammatical structures until the age of 12 years.
Conclusions: Adequate screening for language development for school-age hearing-impaired children is required for an effective intervention.

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記述言語：日本語   出版者・発行元：Japan Society for Pediatric Otorhinolaryngology  

生後11ヶ月で人工内耳埋込術を施行した髄膜炎後難聴の 1 例を経験したので報告する。症例は生後 8 ヶ月で，細菌性髄膜炎に罹患し，治癒後の聴性脳幹反応検査では両側 105 dBnHL で反応がみられなかった。内耳 MRI の 3D 再構築画像で右内耳は既に閉塞し，内耳内腔の骨化が急速に進行していると考えられた。左内耳は今後閉塞が高度となる可能性が考えられ，直ちに左人工内耳埋込術を施行した。髄膜炎後の難聴にはしばしば蝸牛内骨化を伴うが，中には髄膜炎罹患後約 2 週間から内耳骨化が進行するような，急速な骨化例もある。髄膜炎直後の乳児では難聴の早期発見のための ABR 検査や，難聴の存在を疑われた場合の迅速な MRI 検査は不可欠である。

DOI： 10.11374/shonijibi.33.23

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その他リンク： http://search.jamas.or.jp/link/ui/2012208704

担当区分：筆頭著者   記述言語：日本語   出版者・発行元：Japan Society for Pediatric Otorhinolaryngology  

一般的には人工内耳術後再手術の適応には，機器の故障，血腫の形成，創部感染，および電極の位置不良などが挙げられる。また最近の手術時年齢の低下からは，耳管機能の未熟さに伴い，経過中に中耳疾患を合併することも少なくない。<br/>  今回当院で実施した小児の人工内耳埋め込み術150例のうち再手術を要した 3 例および追加手術を要した 2 例について検討した。再手術の 3 例は全員機器不良によるものであり，同側の人工内耳 explant/implant 術を施行した。また，追加手術の 2 例では鼓膜換気チューブ留置術および鼓膜形成術を施行した。このうち外傷後に再手術を行った内耳奇形の 1 例の詳細を報告した。<br/>  再手術を要する症例では失聴期間をできるだけ短くするために迅速な対応を行い，術側の決定および術中の電極の位置確認を慎重に行うことが重要であると考える。

DOI： 10.11374/shonijibi.32.409

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その他リンク： http://search.jamas.or.jp/link/ui/2012118058

記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：LIPPINCOTT WILLIAMS & WILKINS  

MicroRNAs (miRNAs) play functional roles in sound transduction in cochlea. This study focuses on the validity of cochlear culture as an in vitro experimental tool, in view of miRNA expression. E15 cochleae were dissected and maintained in vitro for 48 h before extraction of miRNAs. MiRNA expression was comprehensively screened in explanted cochleae using a miRNA array that covers 380 miRNAs. A strong correlation was observed between expression levels of miRNAs in in vitro and in in vivo cochleae. Levels of 43 miRNAs were altered in vitro and these changes were reproducible over three trials. These findings indicate that in vitro miRNA profiling is a viable method for analysis of gene expression and action of chemical compounds on cochleae. NeuroReport 22:652-654 (C) 2011 Wolters Kluwer Health vertical bar Lippincott Williams & Wilkins.

DOI： 10.1097/WNR.0b013e32834a0273

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：OKAYAMA UNIV MED SCHOOL  

Orbital emphysema is an abnormal condition in which air is present within the orbit. We report a rare case of a 19-year-old man who suffered syncopic attacks caused by sniffles following orbital emphysema as a result of trauma. Treating rhinitis is important in patients with orbital emphysema, and patients with cardiac disorders in addition to those with this condition must be warned about the risks of sniffles, sneezing, or nose blowing.

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記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Oto-Rhino-Laryngological Society of Japan Inc.  

The dissemination of newborn hearing screening (NHS) has enabled those with mild to moderate hearing loss to be diagnosed in early infancy. The "handicapped independence support law", however, prevents those not determined to be physically handicapped,-i.e., those with mild to moderate hearing loss-, from government assistance in purchasing hearing aids. This results either in (i) parents purchasing hearing aids at their own expense or (ii) nonpurchase of hearing aids. To redress this situation, subvention in purchasing hearing aids of mild to moderate hearing loss children has been begun by some local governments. We petitioned for such aid at the Okayama Prefecture Assembly. A society for supporting pediatric hearing difficulty in Okayama Prefecture also submitted a similar petition in September 2009, and subvention began in April 2010 for all such children in Okayama Prefecture. We report procedures and details leading to this subvention.

DOI： 10.3950/jibiinkoka.114.731

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記述言語：日本語   掲載種別：研究論文（学術雑誌）   出版者・発行元：Oto-Rhino-Laryngological Society of Japan Inc.  

The introduction of newborn-hearing screening has enabled early childhood hearing loss to be diagnosed and increased the number of children undergoing early care. Bilateral hearing loss is found in 0.08% of newborns and children whose hearing loss progresses or onset is delayed account for 4 to 30% of all pediatric hearing impairment. Children with perinatal risk factors tend to have deteriorated hearing or delayed-onset hearing loss in early childhood, necessitating audiometric follow-up. We also are aware of some children without risk factors who develop hearing impairment during infancy or early childhood. Hearing deterioration may be difficult to diagnose objectively, especially in young children, the presence of risk factors must be determines as soon as possible, especially given the lack of hearing management and close examination of children without apparent risk factors. We retrospectively studied children born from April 1998 to March 2007 and undergoing cochlear implantation as of April 2008. Among cases, we focused on 10 whose hearing impairment advanced during infancy-4 with risk factors known before hearing deterioration progressed, and 6 cases thought not to have any risk factors. We detected enlarged vestibular acquaducl in 3 of these 6 cases, and 3 more of whom had no risk factors-2 passing newborn-hearing screening and 1 in whom such screening detected hemilateral hearing loss. Our results underscore the need for early temporal computed tomography for detecting enlarged vestibular aquaduct. Even children with mild or hemilateral hearing loss should undergo audiometric and developmental testing at least every 6 months up to going to elementary school. Children suspected of impaired hearing should undergo thorough hearing tests regardless of newborn hearing-screening results to catch any problems early. Appropriate regular hearing and language development check-up tests must also be developed.

DOI： 10.3950/jibiinkoka.114.557

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記述言語：日本語   出版者・発行元：日本口腔・咽頭科学会  

耳下腺深葉に発生したオンコサイトーマの症例を報告する. 症例は64歳の男性. 左耳下部の腫脹にて受診し, 画像検査などにて耳下腺深葉腫瘍が疑われ, 腫瘍摘出術が施行された. 病理検査にてオンコサイトーマと診断され, 非常に稀な腫瘍型であるため, 若干の文献的考察を加え報告した.

DOI： 10.14821/stomatopharyngology.23.237

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記述言語：英語   掲載種別：研究論文（学術雑誌）   出版者・発行元：SPRINGER  

The occasional local recurrence of benign pleomorphic adenoma (PA) has generally been attributed to the vulnerability of the tumor capsule. Although some reports have also noted the presence of satellite tumors associated with PA recurrence, only few reports have focused on this issue. We paid special attention to the satellite lesions apart from the main tumors and discussed their frequency, origin, nature and the ways of treating them. A total of 108 specimens of primary parotid gland PA resected at the Okayama Saiseikai General Hospital from 1988 to 2008 were microscopically reviewed. Four (3.7%) patients displayed a main mass with satellite tumors in a single parotid gland. The immunohistochemical analysis of p53 and Ki-67 index showed no distinct difference between PAs with satellite tumors and those without. Satellite tumors surrounding the main mass of parotid PA is relatively rare. In most cases, such satellite tumors will arise from capsular perforation of the primary tumor cells. Preoperative evaluation to recognize the existence of satellite tumors would be important and capsular dissection should be discouraged. We could not find any evidence suggesting that primary PA with satellite tumors could be more biologically aggressive than those without.

DOI： 10.1007/s00405-009-1149-7

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掲載種別：研究論文（学術雑誌）  

We report a 21-year-old female who complained of dizziness with cerebellar arteriovenous malformation (AVM). At her first visit, she complained only of dizziness and did not show any other cranial nerve symptoms. She was therefore diagnosed with peripheral vertigo and started treatments. Two weeks after the first examination, she was examined by electronystagmograph (ENG) because her dizziness had not improved. The ENG result suggested that the dizziness was caused by a central lesion disorder and a MRI scan revealed a large AVM in the cerebellum and brainstem. Although AVM commonly shows cerebral hemorrhage or convulsion as the first symptom, we experienced a case of AVM with only mild symptoms of dizziness and headache.

DOI： 10.5631/jibirin.101.759

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：日本音声言語医学会  

中等度難聴があり,音韻障害の合併が疑われた7歳左利き男児についての症例報告である.ことばの遅れからさまざまな施設を受診していたが,難聴も含め確定診断にいたっていなかった.発話の特徴は,文章では子音が省略され母音のみで,単語では子音も表出されるが誤りが浮動的で一貫性が見られなかった.このため,聴覚に関する精密検査に加え,音韻検査を含む言語認知検査,脳SPECT等の画像診断を実施した.その結果中等度難聴と診断され,同時にSLI(specific language impairment:特異的言語発達障害)の特徴が見られたためDLI(disproportionate language impairment)と判断した.本児には難聴に対する介入と同時に音韻的治療を含めた言語指導を行い,その後言語発達は同年齢の平均範囲内に追いついた.難聴の診断も重要であるが,児の症状から言語認知検査を詳細に行い適切な支援をする必要がある症例であったと考えられた.(著者抄録)

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2023&ichushi_jid=J00189&link_issn=&doc_id=20230801440006&doc_link_id=10.5112%2Fjjlp.64.182&url=https%3A%2F%2Fdoi.org%2F10.5112%2Fjjlp.64.182&type=J-STAGE&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00007_2.gif

記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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その他リンク： https://search.jamas.or.jp/default/link?pub_year=2022&ichushi_jid=J00125&link_issn=&doc_id=20220912480024&doc_link_id=%2Fdm7audio%2F2022%2F006505%2F025%2F0323-0323%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fdm7audio%2F2022%2F006505%2F025%2F0323-0323%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

記述言語：日本語   出版者・発行元：(有)科学評論社  

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

当科で化学療法を施行した小児、思春期・若年(CAYA)世代がん患者20例を対象に、妊孕性温存に関する説明の実態調査を行った。その結果、妊孕性低下・消失について説明していた患者は2例のみであり、それぞれ17歳と23歳の未婚若年女性であった。一方、その他の女性5例(30歳台)や男性(13〜38歳)には説明が行われていなかった。また、妊孕性温存ガイドラインが出版された2017年の前後で妊孕性低下・消失について説明を実施した割合には著しい変化はみられず、妊孕性に対する配慮は担当医レベル、診療科レベルでも改善できる余地が十分にあると考えられた。

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記述言語：日本語   出版者・発行元：日本耳鼻咽喉科免疫アレルギー感染症学会  

1日2回の生理食塩水洗浄と抗菌薬の保存的加療により、人工内耳植え込み術後の局所感染を制御できた小児の1例(1歳11ヵ月男児)について報告した。本症例で術後創部感染の保存的加療が可能であった要因として、「起炎菌がMRSAなどではなく、MSSAであったこと」「鼓膜所見や起炎菌から、中耳からの感染ではなく、皮膚からの感染であったため」「当院では、人工内耳植え込み術の際、minimum invasive surgeryを行っていること」が考えられた。

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：耳鼻と臨床会  

人工内耳装用を行った言語習得期前難聴児で、知的な障害がなく、音に対する反応も良好であるにもかかわらず言語発達の遅れを呈した児(disproportionate language impairment:DLI)に対して、音韻処理過程を考慮した言語指導を実施した2例について報告した。いずれも知的な遅れなく、また人工内耳装用下での聴取閾値も20-30dBSPLで良好な反応を示していたが、言語発達では3歳以上の遅れを呈していた。両症例とも非語の復唱で著しい困難さを呈しており、音韻認識の障害の合併を推定した。種村らの既報をベースにした音韻認識をターゲットにした言語指導を行い、それぞれ指導後の1年間で順調な言語発達ののびがみられた。音韻障害に起因するDLI児に対しては、病態に応じた言語指導が有効である可能性が示された。(著者抄録)

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

先天性難聴の早期発見、早期療育、人工内耳手術の低年齢化などに伴い、難聴児の聴取能、言語発達は向上し、近年地域の学校でインクルーシブ教育を受ける者が増加しているが、それに伴う問題も挙げられている。我々は、小学校5年生以上25歳未満のインクルーシブ教育を受けた経験のある両側難聴者89名に、学校生活で抱える問題に関して質問紙での実態把握調査を実施した。対象者の多くは、授業中の支障に加え、グループ学習や雑音下、距離が離れた場所からの聞き取りの支障を抱えており、また英語、音楽、体育をはじめとする教科学習での課題や、友人関係での問題も挙げていた。難聴の程度が重いほど頻度が高い傾向がみられた。個々の学校生活における状況と問題を正確に把握した上で、視覚情報を用いたコミュニケーション、支援員の配属、学習面でのサポート、専門家による心理的負担へのアプローチといった個々に対応した介入の必要性が示唆される。(著者抄録)

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

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記述言語：日本語   出版者・発行元：日本耳鼻咽喉科感染症・エアロゾル学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：日本耳鼻咽喉科感染症・エアロゾル学会  

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記述言語：日本語   出版者・発行元：日本耳鼻咽喉科感染症・エアロゾル学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

近年の人工内耳は、1.5Tまでの磁場であればインプラント磁石を取り出すことなくMRI検査を行うことができる。ただ疼痛や皮膚発赤、減磁や脱磁、磁石の変位などの合併症が起こり得る。今回われわれはMRI後に人工内耳インプラントの磁石の反転を来した2症例を経験した。2例とも磁石は180度反転してシリコンフランジ内に格納されており、1例は極性を逆にした体外磁石を特注し、もう1例はインプラント磁石の入れ替え手術を行った。人工内耳は適応の拡大、高齢化などに伴い、今後も装用者は増加すると見込まれる。医療者として、人工内耳患者がMRIを受ける上での留意点、合併症が生じた場合の検査、対応などを認識する必要がある。(著者抄録)

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：(株)東京医学社  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

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記述言語：日本語   出版者・発行元：(一社)日本遺伝カウンセリング学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：日本耳鼻咽喉科感染症・エアロゾル学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

近年新生児聴覚スクリーニングの普及により、難聴児の多くは生後数ヵ月のうちに診断され、療育を開始している。一方、特別児童扶養手当に関してはオージオメーターによる検査が可能となるまで受給されず、診断から受給までの間に年単位の時間差が生じているのが現状である。われわれは、生後12ヵ月までにABRを実施し、両耳90dBnHLで無反応であった児の聴力経過の検討を行った。その結果、聴覚障害単独の児では特別児童扶養手当に該当するか否かは1歳時点で可能であるが、等級の判定は1歳で慎重な判断が必要であること、また重複障害をもつ児は検査を繰り返し慎重に診断する必要があるが特別児童扶養手当の判定は可能であると判断した。(著者抄録)

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

成人の聴覚障害者の補聴器装用効果の指標としては、日本聴覚医学会の補聴器適合指針をはじめ、各種質問紙調査が用いられている。しかし、聴覚障害に伴うコミュニケーション障害を評価するための指標は定まっていない。われわれは、実用コミュニケーション能力検査(Communication ADL Test:CADL)から音声に関する項目を抜粋し、音声ファイルを作成、このCADL簡易版を用いて、当科補聴器外来にて補聴器装用を介入とした前後比較試験を行ったところ、各15の質問項目および総得点で装用後有意な得点の改善がみられた。また、装用後の総得点は補聴器装用耳の語音明瞭度と良好な相関を認め、本検査がコミュニケーション障害のアウトカム指標に応用できる可能性が示唆された。(著者抄録)

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記述言語：日本語   出版者・発行元：(株)診断と治療社  

難聴の早期発見および早期介入の浸透により、難聴児の療育環境は近年飛躍的に向上している。しかし、難聴児が補聴器や人工内耳の装用を行ったとしても、依然としてその言語発達には多くの課題が残されていることが、筆者らの調査で明らかとなった。言語発達の遅れは難聴児の学校生活や社会生活に多くの支障をきたし、結果的にQOLの低下をもたらす。これを予防するために、言語発達を経時的にフォローし、適切な介入を行う必要がある。(著者抄録)

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記述言語：日本語   出版者・発行元：(一社)日本遺伝カウンセリング学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(株)医学書院  

＜POINT＞先天的な難聴の約半数は遺伝子が関与するため,難聴の原因として念頭に置く必要がある。幼小児の難聴の原因を明らかにすることの意義は,児の難聴の今後の経過や予後などの将来を予見し,その児に適した療育・治療方針を決定することにある。難聴の診断には詳細な家族歴の聴取,家系図の作成,聴力像の把握が必須である。また,遺伝学的検査も重要な役割を果たすが,実施にあたっては十分な遺伝カウンセリングが不可欠である。こうした難聴の診療には遺伝診療部門との連携が必要となる。(著者抄録)

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(株)東京医学社  

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

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記述言語：日本語   出版者・発行元：(一社)日本遺伝カウンセリング学会  

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記述言語：日本語   出版者・発行元：(一社)日本遺伝カウンセリング学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語  

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記述言語：日本語   出版者・発行元：岡山医学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：(株)東京医学社  

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記述言語：日本語  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語   出版者・発行元：(株)東京医学社  

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記述言語：日本語   出版者・発行元：東京医学社  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳科学会  

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：(株)全日本病院出版会  

点耳抗菌薬は耳鼻咽喉科診療の中で頻用される薬剤である。点耳薬中の抗菌薬は、局所で用いるために全身投与された場合に比較して病巣では高濃度となる。このため、細菌が薬剤に耐性を獲得する前に殺菌すること、全身への影響が少なく病巣以外の細菌叢に影響を及ぼさないことなどにより、全身投与よりも細菌の耐性化を引き起こす可能性は低いと考えられる。このような理由から、幅広い菌種に適応があるキノロン系点耳抗菌薬は耳漏を伴う中耳・外耳疾患に対して第一選択になる治療法と考えられるが、キノロン系点耳抗菌薬が臨床効果を示さない小児の難治性中耳炎の報告が散見されるようになっている。点耳抗菌薬の安易な使用は耐性化を引き起こす危険性があるため、抗菌薬の適正な使用、抗菌薬の使用を抑制するためのワクチン接種など国をあげての薬剤耐性化防止対策が必要だと考えられる。(著者抄録)

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記述言語：日本語   出版者・発行元：(一社)日本聴覚医学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

新生児聴覚スクリーニング検査の普及にしたがい、軽度・中等度難聴児も早期に診断され、早期に補聴器を装用し、療育を開始するケースが増加している。しかしながら、現在の障害者自立支援法では、身体障害者には該当しない軽度・中等度難聴者は、補聴器購入に際して公的援助が受けられないため、全額自費で購入するか、補聴器装用を断念せざるを得ないのが現状である。この是正を求め、「岡山県難聴児を支援する会」は、平成21年9月岡山県議会に「軽度および中等度難聴児に対する補聴器購入費用助成に関する陳情書」を提出し、平成22年4月より「岡山県難聴児補聴器交付事業」が開始された。事業導入に至った経緯、概要を報告する。(著者抄録)

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記述言語：日本語   出版者・発行元：(一社)日本外来小児科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

新生児聴覚スクリーニング検査導入以後、乳児期および幼児期早期に難聴の確定診断がされ、療育が行われる例が増加した。しかし、乳幼児期に難聴が進行する児や、新生児期には難聴が認められなかったにもかかわらず遅発性に難聴を発症した児が存在することが報告され、その割合は難聴児の4-30%に及ぶとされている。進行性および遅発性難聴児の中に、難聴の危険因子を予見できない児が少数ながら存在することも明らかになっている。今回われわれは1998年4月から2007年3月までに出生した岡山県在住の小児で、2008年4月までに当院で人工内耳埋込み術を行った45例の中から乳幼児期に進行性および遅発性難聴が認められた10例(人工内耳装用児中の22.2%)を抽出し、新生児期に危険因子をもたないと考えられていた6例について詳細に検討した。6例中3例では、難聴進行後に画像検査にて前庭水管拡大症が発見されたが、他の3例の原因は不明であった。対象数は少ないが、新生児期に難聴の危険因子をもたない半数に画像検査で前庭水管拡大症が発見されたことにより、可能な限り画像検査を行うことが難聴増悪の早期発見の点から望ましいと考えられた。また、進行性難聴や新生児聴覚スクリーニング検査にパスした場合でも乳幼児期に遅発性難聴となる可能性があることを念頭に置き、就学前までは定期的な聴覚検査や現在自治体で行われている健診における聴覚に関する質問事項や検査の充実なども考慮すべきである。(著者抄録)

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：日本小児耳鼻咽喉科学会  

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記述言語：日本語   出版者・発行元：(一社)日本耳鼻咽喉科頭頸部外科学会  

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記述言語：日本語  

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

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DOI： 10.11374/shonijibi.31.299

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記述言語：日本語   出版者・発行元：(株)医学書院  

交感神経由来が疑われる頸部神経鞘腫は術前の画像所見から由来神経を確定するのは困難であるが、術後にホルネル徴候が出現すると患者のQOLが低下するため、手術に際しては慎重な手術操作が求められる。今回、術前の画像所見から迷走神経由来もしくは交感神経由来の神経鞘腫が考えられた3例(27歳女、36歳男、63歳女)を経験した。手術は3例とも神経機能を温存しつつ腫瘍を確実に摘出する目的で被膜間摘出術を施行したが、術後に交感神経由来と思われるホルネル徴候が全例に出現し、2例ではその後改善したが、1例は現在も残存している。このことから、交感神経由来が疑われる神経鞘腫では被膜間摘出術によっても術後に神経脱落症状を呈する場合があることに留意し、術前に十分なインフォームド・コンセントを行う必要があると考えられた。

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記述言語：日本語   出版者・発行元：耳鼻咽喉科臨床学会  

21歳女。浮動性めまいで当院内科にて投薬を受けるが改善せず、頭痛、嘔気を訴えて当科を紹介受診した。頭位眼振検査で方向交代性上向性眼振、頭位変換眼振検査で水平性眼振を認めたが、脳神経症状は認めず、末梢性めまい症として治療を開始した。症状は改善せず、めまい感は増悪した。追跡眼球運動検査(ETT)でsaccadicを認め、視運動性眼振検査(OKP)で解発が低下し、左向き刺激では緩徐相と急速相の区別がつかない異常パターンを認めた。以上より中枢病変を疑い、頭部MRIにて右小脳半球のほぼ全域と中部から一部左小脳半球にかけて未破裂動静脈奇形(AVM)を認めた。AVMの一部は右前庭神経核とその近傍にも及んでいた。頭部MRAにより右の複数の小脳動脈から血管が流入し、背側の静脈洞に流出する所見を認めたが、CTでは出血は認めなかった。病巣が脳幹部に及んでいるため外科的治療、血管塞栓術は困難と判断して経過観察とした。

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記述言語：日本語  

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記述言語：日本語   出版者・発行元：岡山済生会総合病院  

70歳代女性。患者は舌根部の腫瘤を主訴とした。受診時、左舌根部に2cm大の白色の潰瘍を伴う腫瘍が認められた。また、頸部MRIでは左舌根部筋層内に境界明瞭な腫瘍がみられ、T1にて低信号、T2にて等信号を呈していた。頸部リンパ節には転移はみられなかったが、生検の結果、左舌根部に発生した乳頭状嚢胞腺癌と診断され、正中下口唇下顎骨舌切断法による腫瘤摘出術が行なわれた。その結果、術後は機能障害は特にみられず、術後6ヵ月時点で再発・転移を認めず、経過は良好である。

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