Electrical promontory stimulation test using a portable peripheral nerve stimulator with an ear canal electrode.

OBJECTIVE: An intact cochlear nerve is necessary for successful cochlear implantation (CI). Although the promontory stimulation test (PST) using a promontory stimulator (PS) and a transtympanic needle electrode is invasive, it is still commonly used to verify cochlear nerve function. PSs are currently unavailable because they are no longer manufactured; however, considering that PST continues to be beneficial in certain situations, alternative equipment is needed. The PNS-7000® (PNS) was developed as a neurologic instrument for stimulating the peripheral nerves. This study investigated the usefulness of the ear canal stimulation test (ECST) using PNS with a silver ball ear canal electrode, which is a new noninvasive alternative technique to the PST. METHODS: ECST was performed from November 2013 to December 2018 using PS and PNS for patients with severe to profound sensorineural hearing loss. The electrical threshold, most comfortable loudness level, uncomfortable loudness level, dynamic range, and gap detection were measured in the ECST. The results of these measured PNS items were compared with PS. RESULTS: ECST was performed in 61 ears of 35 patients (age, 59.9 ± 20.1 years) using PS and PNS. The sound sensation was elicited in 51 (83.6%) and 52 (85.2%) ears with PS and PNS, respectively. All items excluding GAP were measured in 46 (75%) and 43 (70%) ears at 50 and 100 Hz, respectively. GAP was measured in 33 ears by the ascending and descending methods using PS and PNS. Spearman's rank-order correlation coefficient revealed a significant positive linear correlation between the PS and PNS results in all measurements. No significant difference was found between the PS and PNS thresholds in all measured items. CONCLUSIONS: PNS is a useful instrument for performing ECST as a new alternative to PS. ECST using a silver ball electrode is a less invasive and easier test than PST.

Hearing Outcome and Predictors after Implanting Bone Conduction or Middle Ear Implants in Ears with Refractory Otitis Media.

Bone conduction implants (BCIs) and middle ear implants (MEIs) are promising options for individuals with persistent chronic inflammation of the middle or outer ear. However, the structure of the middle ear is often altered in patients who undergo mastoidectomy or posterior wall removal for refractory otitis media, leaving uncertainty regarding the efficacy of hearing devices. Only a few studies have examined auditory outcomes based on the etiology of hearing impairment. We investigated hearing outcomes, including speech audiometry, in patients who underwent implantation after surgery for refractory otitis media. Our findings indicated that patients who received BCIs or MEIs achieved favorable hearing outcomes. Furthermore, a correlation was observed between the preoperative bone-conduction threshold at 1 kHz in the better ear and the sound-field threshold at 1 kHz with BCIs, whereas no correlation was observed between the preoperative bone-conduction threshold and the sound-field threshold with MEIs. This study highlights the positive impact of BCIs and MEIs in patients who undergo implantation after surgery for refractory otitis media. Additionally, our study identified parameters that predict postoperative efficacy.

Speech Perception of Second Cochlear Implant after 6 Years of Age in Prelingually Deaf Children.

Sequential bilateral cochlear implantation in children becomes less effective as the inter-implant interval increases. However, the cause of this and the age at which speech perception becomes impossible are unclear. We examined the cases of 11 prelingually deaf children who underwent unilateral cochlear implantation at our hospitals before the age of 5 years old, followed by a second implantation procedure on the contralateral side when they were aged ≥ 6 years old (6-12 years old). The subjects' hearing thresholds and speech discrimination scores for the second cochlear implant were evaluated at 3 postoperative months and 1-7 years. All of the subjects demonstrated improvements in their hearing thresholds to a mean of 30 dB HL at 1 year. Regarding speech perception, one patient (a 12-year-old), who had developed bilateral hearing loss at 30 months of age after contracting mumps, demonstrated a 90% improvement in his speech discrimination score at 1 year. However, among the other congenitally deaf children, there were two patients whose speech discrimination scores had improved by ≥ 80% at > 4 postoperative years. The congenitally deaf children exhibited poor speech perception despite showing improved hearing thresholds in the ears that received second cochlear implants. Assuming that the auditory pathway beyond the superior olivary complex remained functional, the reduced speech perception abilities associated with the second cochlear implants may have been attributable to the loss of the spiral ganglion and cochlear nucleus cells due to a lack of auditory input since birth.

Active Middle Ear Implant in a Patient with Neurofibromatosis Type 1 and Multiple Calvarial Defects: A Case Report.

Bony abnormalities, including sphenoid dysplasia and calvarial defects, are well recognized in patients with neurofibromatosis type 1. However, having multiple calvarial defects is rare. We present a case of a 35-year-old Japanese male patient who was referred to our hospital because of hearing loss. He was diagnosed with neurofibromatosis type 1 during early childhood. Otoscopic examination revealed a protrusion from the anterior wall of the external auditory canal that obstructed the external auditory canal. Computed tomography findings revealed multiple defects and an uneven skull surface. Large bony defects of the anterior wall of the external auditory canal were also identified bilaterally. Conductive hearing loss was caused by temporomandibular joint herniation that was obstructing the external auditory canal in both ears. An active middle ear implant was implanted in the right ear. A floating mass transducer was placed into the round window niche using a round window coupler. The active middle ear implant improved postoperative audiometric thresholds to approximately 35 dB across all frequencies. No complications occurred for up to 30 months after the operation. An active middle ear implant is a feasible and valuable option for patients with neurofibromatosis type 1 and conductive hearing loss due to multiple skull defects that result in temporomandibular joint herniation.

Rapid Genetic Diagnosis for Okinawan Patients with Enlarged Vestibular Aqueduct Using Single-Stranded Tag Hybridization Chromatographic Printed-Array Strip.

Both Pendred syndrome (PS) and nonsyndromic hearing loss with an enlarged vestibular aqueduct (EVA) are autosomal recessive disorders caused by SLC26A4 pathogenic variants. The spectrum of SLC26A4 pathogenic variants varies with the ethnic background. Among the patients with EVA in Okinawa, 94% had some combination of NM_000441.2(SLC26A4):c.1707+5G>A and NM_000441.2(SLC26A4):c.2168A>G(p.His723Arg), the two SLC26A4 pathogenic variants that are the most common in this population. We identified these two pathogenic variants using a novel genotyping method that employed an allele-specific polymerase chain reaction (PCR) from a gDNA and single-stranded tag hybridization chromatographic printed-array strip (STH-PAS) in DNA samples obtained from 48 samples in Okinawa, including 34 patients with EVA and 14 carriers of c.1707+5G>A or c.2168A>G. In addition, whole blood and saliva samples were used for analysis in this genotyping method with direct PCR. The results of STH-PAS genotyping were consistent with those obtained using standard Sanger sequencing for all samples. The accuracy of the STH-PAS method is 100% under the optimized conditions. STH-PAS genotyping provided a diagnosis in 30 out of 34 patients (88%) in Okinawan patients with EVA in under 3 h. The turn-around time for STH-PAS genotyping used with direct PCR was 2 h as a result of the omission of the DNA extraction and purification steps. Using information about the ethnic distribution of pathogenic variants in the SLC26A4 gene, STH-PAS genotyping performs a rapid genetic diagnosis that is simple and has a considerably improved efficiency.

Presentation of dizziness in individuals with chronic otitis media: data from the multinational collaborative COMQ-12 study.

PURPOSE: In chronic otitis media (COM), disease chronicity and severity of middle ear inflammation may influence the development of inner ear deficits, increasing the risk of vestibular impairment. This secondary analysis of the multinational collaborative Chronic Otitis Media Questionnaire-12 (COMQ-12) dataset sought to determine the prevalence of vestibular symptoms in patients with COM and identify associated disease-related characteristics. METHODS: Adult patients with a diagnosis of COM in outpatient settings at nine otology referral centers across eight countries were included. We investigated the presence of vestibular symptoms (dizziness and/or disequilibrium) using participant responses to item 6 of a native version of the COMQ-12. Audiometric data and otoscopic assessment were also recorded. RESULTS: This analysis included 477 participants suffering from COM, with 56.2% (n = 268) reporting at least mild inconvenience related to dizziness or disequilibrium. There was a significant association between air conduction thresholds in the worse hearing ear and presence of dizziness [adjusted odds ratio (AOR), 1.01; 95% CI 1.00-1.02; p = 0.0177]. Study participants in European countries (AOR 1.53; 95% CI 1.03-2.28; p = 0.0344) and Colombia (AOR 2.48; 95% CI 1.25-4.92; p = 0.0096) were more likely to report dizziness than participants in Asian countries. However, ear discharge and cholesteatoma showed no association with dizziness in the adjusted analyses. CONCLUSION: Vestibular symptoms contribute to burden of disease in patients with COM and associates with hearing disability in the worse hearing ear. Geographical variation in presentation of dizziness may reflect financial barriers to treatment or cultural differences in how patients reflect on their health state.

Combined Electric acoustic stimulation in a patient with otitis media with antineutrophil cytoplasmic antibody-associated vasculitis.

There are few reports of the treatment for severe hearing loss due to otitis media with antineutrophil cytoplasmic antibody-associated vasculitis (OMAAV) achieved by cochlear implantation (CI). Here, we have reported the case of a patient with severe bilateral sensorineural hearing loss with low-frequency residual hearing by OMAAV. CI was performed in her right ear based on the results of contrast-enhanced magnetic resonance imaging (CE-MRI) and promontory stimulation test (PST). The residual hearing in her right ear was preserved after CI and utilized for combined electric acoustic stimulation (EAS). The combined EAS was used for 3 years until the residual hearing became stabilized. However, the usable hearing in low frequency worsened gradually, and the fitting strategy of cochlear implant was changed from combined EAS to CI alone 4 years after CI. Even when the speech discrimination score with CI no longer exceeds 50 %, the patient continued using CI because of its advantages in maintaining the quality of life of the patient. The combined EAS was found to be a feasible option even in an OMAAV patient with residual hearing. CE-MRI and PST may thus be helpful in deciding the side of CI surgery in a patient with OMAAV.

Comparison of speech perception lists used to evaluate Japanese single syllable hearing.

OBJECTIVE: In Japan, the 57S and 67S single syllable lists have been used to test speech perception in cochlear implant (CI) users. However, due to advances in implants and processors, these lists have become too simple for CI users. In 2019, the Japan Otological Society created a new list, referred to as the iCI2004. The objective of this study was to compare the performance of these lists. METHODS: The Japanese single syllable speech perception tests, iCI2004, 57S, and 67S, were administered to 70 patients with CI users. The effects of single syllable characteristics on the test scores were examined. The type and number of single syllables used in each list were different. Therefore, we compared the scores for normal lists, shared single syllables, and non-shared single syllables. RESULTS: The average test results were 52% for iCI2004, 64% for 57S, and 77% for 67S; 67S performed the best, followed by 57S and iCI2004. The test results were significantly different. In a comparison of shared single syllables, the average scores were 58% for iCI2004 and 63% for 57S (45 pieces). A comparison of iCI2004 and 67S (17 pieces) showed that the average scores were 63% for iCI2004 and 75% for 67S. A comparison of 57S and 67S (20 pieces) showed that the average score for 57S was 70% and the average scored for 67S was 77%. No significant differences were detected under all conditions. Based on these results, the type of single syllable adopted affected the result. CONCLUSION: The data gives no clear indication that the selection of the word table or presentation of sound pressure affects listening to sound for CI users in Japan. Based on the type and number of single syllables used, iCI2004 seems appropriate for evaluation of hearing in patients using CI.

A Case of Amelanotic Malignant Melanoma of the Lingual Base That Was Diagnosed Based on a Biopsy of Late Metastasis to a Lumbar Vertebra after Being Misdiagnosed as HPV-Positive Oropharyngeal Anterior Wall Squamous Cell Carcinoma.

We report a case of amelanotic malignant melanoma (AMM) in a 66-year-old female. AMM of the lingual base was diagnosed based on a biopsy of late metastasis to the bone marrow of the L4 lumbar vertebra. The patient was initially treated with chemoradiotherapy after being misdiagnosed with poorly differentiated human papillomavirus- (HPV-) related squamous cell carcinoma of the oropharyngeal anterior wall. p16 immunostaining is used to diagnose HPV-related oropharyngeal cancer. However, while p16 expression is used as a surrogate marker of HPV infection, it is important to be aware that p16 protein overexpression can also be caused by other factors. Malignant melanoma is known to express the p16 protein. Morphologically differentiating between AMM and poorly differentiated squamous cell carcinoma based on hematoxylin-eosin staining is difficult. Therefore, in cases that are pathologically diagnosed as p16-positive poorly differentiated oropharyngeal squamous cell carcinoma, it is important to rule out AMM.

Usefulness of Virtual Monochromatic Images and Iodine Maps Derived from Dual-Energy Computed Tomography for Diagnosing Deep Neck Abscesses.

OBJECTIVE: We aimed to determine whether dual-energy computed tomography (CT) is useful for evaluating deep neck abscesses. METHODS: This study included 22 consecutive patients who were clinically suspected of having a deep neck abscess and underwent dual-energy CT. Conventional 120-kVp images, 70- and 40-keV virtual monochromatic images (VMIs), and iodine maps were inspected to calculate the contrast ratio of the abscess rim (AR) to the abscess center (AC) or to the adjacent muscle (M). The diagnostic certainty of abscesses was assessed on these images. RESULTS: Twenty (91%) of 22 patients had a definitive diagnosis. The contrast ratio for AR/AC and AR/M was significantly higher on 40-keV VMIs and iodine maps than on 120-kVp images and 70-keV VMIs (P < 0.05). On both 40-keV VMIs and iodine maps, the diagnostic certainty of abscess improved in 3 (15%) cases compared with 120-kVp images and 70-keV VMIs. CONCLUSIONS: Dual-energy CT-based 40-keV VMIs and iodine maps are useful for evaluating deep neck abscesses and may improve diagnostic certainty.

Impact of Hearing Disability and Ear Discharge on Quality-of-Life in Patients with Chronic Otitis Media: Data from the Multinational Collaborative COMQ-12 Study.

OBJECTIVE: This study aimed to assess how two disease-related factors, hearing disability and ear discharge, affect health-related quality-of-life (HRQoL) in patients with chronic otitis media (COM). STUDY DESIGN: Multinational prospective cohort study. SETTING: Nine otology referral centers in eight countries. PATIENTS: Adult patients suffering from COM. MAIN OUTCOME MEASURES: Hearing disability and ear discharge were assessed by audiometry (Department of Health and Social Security formula) and otoscopy, respectively. Participants completed a native version of the Chronic Otitis Media Questionnaire-12 (COMQ-12). We determined how the two disease-related factors affect HRQoL by performing two separate analyses: (1) using a 6-item score combining responses to COMQ-12 items independent of hearing loss and ear discharge and (2) using item 12 alone as a proxy for global HRQoL. RESULTS: This study included 478 participants suffering from COM. There was a significant association between HRQoL and hearing disability in the adjusted analysis. For every unit increase in the Department of Health and Social Security average hearing threshold (1) there was an increase of 0.06 (95% CI [0.007, 0.121], p = 0.0282) in the 6-item score and (2) the adjusted odds of having a higher item 12 score was 1.03 (95% CI [1.01, 1.04], p = 0.0004). There was no association between the presence of ear discharge and HRQoL in both COMQ-12 score analyses. CONCLUSIONS: Knowledge of disease-related factors that influence HRQoL will aid interpretation of patient-reported measures for COM. Patients with a greater degree of hearing impairment appear to have poorer HRQoL, which is not exacerbated by the presence of ear discharge. The magnitude of postoperative hearing improvement rather than the attainment of a dry ear may be a better indicator of surgical success from the patient's perspective.

Is the Conductive Hearing Loss in NOG-Related Symphalangism Spectrum Disorder Congenital?

We describe a dominant Japanese patient with progressive conductive hearing loss who was diagnosed with NOG-related symphalangism spectrum disorder (NOG-SSD), a spectrum of congenital stapes fixation syndromes caused by NOG mutations. Based on the clinical features, including proximal symphalangism, conductive hearing loss, hyper-opia, and short, broad middle, and distal phalanges of the thumbs, his family was diagnosed with stapes ankylosis with broad thumbs and toes syndrome (SABTT). Genetic analysis revealed a heterozygous substitution in the NOG gene, c.645C>A, p.C215* in affected family individuals. He had normal hearing on auditory brainstem response (ABR) testing at ages 9 months and 1 and 2 years. He was followed up to evaluate the hearing level because of his family history of hearing loss caused by SABTT. Follow-up pure tone average testing revealed the development of progressive conductive hearing loss. Stapes surgery was performed, and his post-operative hearing threshold improved to normal in both ears. According to hearing test results, the stapes ankylosis in our SABTT patient seemed to be incomplete at birth and progressive in early childhood. The ABR results in our patient indicated the possibility that newborn hearing screening may not detect conductive hearing loss in patients with NOG-SSD. Hence, children with a family history and/or known congenital joint abnormality should undergo periodic hearing tests due to possible progressive hearing loss. Because of high success rates of stapes surgeries in cases of SABTT, early surgical interventions would help minimise the negative effect of hearing loss during school age. Identification of the nature of conductive hearing loss due to progressive stapes ankylosis allows for better genetic counselling and proper intervention in NOG-SSD patients.

Nationwide survey of middle ear cholesteatoma surgery cases in Japan: Results from the Japan Otological society registry using the JOS staging and classification system.

OBJECTIVE: This study was aimed to determine the characteristics of middle ear cholesteatoma and to investigate short-term outcomes regarding the rates of residual and recurrent cholesteatoma and the postoperative hearing results in Japan, via a nationwide survey using staging and classification criteria for middle ear cholesteatoma, as proposed by the Japan Otological Society (JOS). METHODS: The first-round survey was conducted in 2016. The target was patients with middle ear cholesteatoma who were surgically treated in Japan between January and December 2015. Medical information on the patients was anonymized. The questionnaire entries were age, sex, cholesteatoma classification and stage, preoperative hearing level, mastoid development, status of the stapes, and surgical method. There were a total of 1,787 registered patients from 74 facilities from all over Japan. The second survey was conducted in January 2018 and received 1,456 responses from 49 facilities in Japan. Of the 1,456 cases, 1,060 were conducted in the postoperative hearing survey and 1,084 in the residual recurrence survey. RESULTS: The most common cholesteatoma type was pars flaccida cholesteatoma (63.3%), followed by pars tensa cholesteatoma (13.0%), congenital cholesteatoma (12.9%), and cholesteatoma secondary to chronic tensa perforation (5.6%). Cholesteatoma of uncertain origin accounted for 5.0% (90 cases). Stage II was predominant in pars flaccida and pars tensa cholesteatoma, which frequently involves the mastoid, whereas about half of cases of cholesteatoma secondary to chronic tensa perforation and congenital cholesteatoma were classified as stage I. One hundred fifty-two of 1,084 cases (14.0%) had recurrent cholesteatoma, residual cholesteatoma, or both following first surgeries. The postoperative rates of hearing success rate was 63.3%. CONCLUSION: We were able to clarify not only the current epidemiological status of middle ear cholesteatoma but also the current trends of cholesteatoma surgery in Japan. The development of a staging system by the JOS Committee serving an epidemiological database for international or time-dependent comparison. It is possible to use this staging system with reasonable reliability.

Clinical characteristics, the diagnostic criteria and management recommendation of otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV) proposed by Japan Otological Society.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a form of necrotizing vasculitis with few or no immune deposits. It primarily affects small and medium blood vessels. AAV is classified into three categories, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangitis (EGPA), and two major ANCAs, proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA are involved in their pathogenesis. Intractable otitis media frequently occurs in patients with GPA, MPA or EGPA, although all patients show similar clinical features, regardless of the type of AAV. Furthermore, approximately 15% patients with otitis media caused by AAV do not show ANCA positivity, histopathological evidence, or any other AAV-related lesions at the initial visit; therefore, these patients do not fulfill the ordinary diagnostic criteria for systemic AAV. Thus, we first proposed that this condition could be categorized as "otitis media with AAV (OMAAV)". Subsequently, the Japanese Otological Society (JOS) conducted a nationwide survey between December 2013 and February 2014 and identified 297 patients with OMAAV. The survey revealed that OMAAV is a disease that initially occurs in the middle ear and subsequently spreads to other organs such as the lungs and kidneys, with eventual involvement of all body organs. Severe sequelae such as facial palsy, hypertrophic pachymeningitis, complete deafness, and subarachnoid hemorrhage resulting in death can also occur. In this review, we introduce the clinical features, diagnostic criteria, and treatment strategies recommended by JOS for early diagnosis and treatment of OMAAV.

The Cytokine Expression in Patients with Cardiac Complication after Immune Checkpoint Inhibitor Therapy.

We herein report the cytokine expression at different stages for three patients who developed cardiac complications after immune checkpoint inhibitor (ICI) therapy. Case 1 with biopsy-proven myocarditis showed increased levels of interleukin (IL)-8, monocyte chemotactic and activating factor, and granulocyte macrophage colony-stimulating factor (GM-CSF) when he developed Takotsubo cardiomyopathy. Case 2 with subclinical myocarditis showed predominant activation of IL-8 during the progressive clinical course. Case 3 with cytokine-releasing syndrome showed substantial activations of IL-6, IL-8, GM-CSF, and interferon-γ. Our data suggest the development of unique cytokine activation in individual patients with cardiac complications after ICI therapy.

Multinational Appraisal of the Chronic Otitis Media Questionnaire 12 (COMQ-12).

OBJECTIVES: The Chronic Otitis Media Questionnaire-12 (COMQ-12) assesses patient-reported health-related quality of life. A multinational collaborative project was performed to translate and appraise the psychometric properties of the COMQ-12 across Europe, Asia, and South America. METHODS: Eight otology units from seven countries (China, Colombia, France, Italy, Japan, Korea, Turkey) created native versions of the COMQ-12 by the process of translation and back-translation. Questionnaire reliability was assessed on the basis of internal consistency by calculating Cronbach's coefficient alpha. Exploratory factor analysis was performed to identify underlying correlations between individual questionnaire items. RESULTS: This study included 478 participants from 8 countries. Calculated values for Cronbach's coefficient alpha were between 0.71 and 0.90. Exploratory factor analysis allowed the identification of three dominant factors, the primary factor (related to hearing problems) explaining 42% of the total variance, the secondary factor (related to daily activities) explaining 30% of the variance, and the third factor (related to acute disease activity) explaining 28% of the variance. CONCLUSIONS: This is a large study of patients with chronic otitis media, from centers from within many different countries spanning Europe, Asia, and South America. This study supports the use of the COMQ-12 within the individual countries where it was tested.

Development and validation of an iPad-based Japanese language monosyllable speech perception test (iCI2004 monosyllable).

BACKGROUND: Speech perception tests are commonly used as indices reflecting hearing ability in daily life. In Japan, the CI-2004 test, first developed in 2004, is widely used as standard, but it was not validated against a large number of normal hearing controls and hearing loss patients. AIMS/OBJECTIVES: The primary objective of the present study was to develop and validate iPad-based software for the Japanese monosyllable speech perception test, 'iCI2004'. MATERIAL AND METHODS: Seven universities and two medical centers participated in this study. The hearing threshold and Japanese monosyllable speech perception test results of 77 people with normal hearing and 459 people with hearing loss were collected. RESULTS: All participants with normal hearing achieved almost perfect perception results both in quiet and in noise. For cochlear implant users, the average monosyllable speech perception score was 55.1 ± 19.6% in quiet and 40.3 ± 19.2% in noise (SNR + 10dB). CONCLUSIONS AND SIGNIFICANCE: We developed iPad-based Japanese monosyllable speech perception test software and validated it by testing a large number of controls and hearing loss patients with cochlear implants or hearing aids. The developed monosyllable speech perception test has a sufficiently large dynamic range for assessing improvement in speech perception in Japanese cochlear implant users.

Essential role of submandibular lymph node dendritic cells in protective sublingual immunotherapy against murine allergy.

While sublingual immunotherapy (SLIT) is known as an allergen-specific treatment for type-1 allergies, how it controls allergic pathogenesis remains unclear. Here, we show the prerequisite role of conventional dendritic cells in submandibular lymph nodes (ManLNs) in the effectiveness of SLIT for the treatment of allergic disorders in mice. Deficiency of conventional dendritic cells or CD4+Foxp3+ regulatory T (Treg) cells abrogates the protective effect of SLIT against allergic disorders. Furthermore, sublingual antigenic application primarily induces antigen-specific CD4+Foxp3+ Treg cells in draining ManLNs, in which it is severely impaired in the absence of cDCs. In ManLNs, migratory CD11b+ cDCs are superior to other conventional dendritic cell subsets for the generation of antigen-specific CD4+Foxp3+ Treg cells, which is reflected by their dominancy in the tolerogenic features to favor this program. Thus, ManLNs are privileged sites in triggering mucosal tolerance mediating protect effect of SLIT on allergic disorders that requires a tolerogenesis of migratory CD11b+ conventional dendritic cells.

Comprehensive hearing care network for early identification and intervention in children with congenital and late-onset/acquired hearing loss: 8 years' experience in Miyazaki.

OBJECTIVE: In 2010, we established the Miyazaki Comprehensive Hearing Care Network (MCHCN) for early identification and intervention in children with congenital and late-onset/acquired hearing loss with the cooperation of related administrative bodies in Miyazaki prefecture. The central roles of the MCHCN program are played by the Hearing Care Center (HCC) at the University of Miyazaki Hospital established in 2010 to facilitate audiological diagnoses, hearing aid interventions, and educational efforts, as well as linkage with the Department of Otolaryngology for surgical interventions. Herein, we aimed to present the main outcomes of the MCHCN program organized by the HCC at the University of Miyazaki Hospital. METHODS: The MCHCN consists of two different networks, the Newborn Hearing Screening Network (NHSN) and the Pediatric Hearing Care Network (PHCN). All children suspected of having hearing loss by Newborn Hearing Screening (NHS) are referred to the HCC via the NHSN. In addition, children suspected of late-onset/acquired hearing loss by municipality-led health checkups, pediatricians, public health nurses, and childcare workers are referred to the HCC via the PHCN. Children who were born in Miyazaki prefecture between January 2010 and December 2017 and referred to the HCC for detailed hearing examination were included in this study. RESULTS: Within the study period, 89,390 infants were born in Miyazaki prefecture, and 84,737 (94.9%) of them underwent NHS. A total of 698 infants and 182 children with suspected hearing loss were referred to the HCC via the NHSN and PHCN, respectively. Of the 880 referrals, 169 were diagnosed with hearing loss, which included 80 children with bilateral hearing loss and 89 children with unilateral hearing loss. Of the 80 children with bilateral hearing loss, 76 began wearing hearing aids and 15 had cochlear implants in the follow-up period. In children with bilateral conductive hearing loss, 4 children with bilateral middle ear anomalies underwent ossiculoplasty, following which two of these children no longer required hearing aids. Imaging assessments performed on 71 of the 89 children with unilateral hearing loss revealed that 20 of the 30 (66%) children who underwent CT exhibited ossicular anomalies and 28 out of the 48 (58%) children who underwent MRI were found to have ipsilateral cochlear nerve hypoplasia. Among the 169 children with hearing loss, no follow-up loss was observed during the period of this study. CONCLUSION: The MCHCN that was organized at the initiative of the HCC at the University of Miyazaki Hospital has enabled the provision of comprehensive and continuous support, ranging from diagnosis to intervention, not only for children with suspected hearing loss referred based on their NHS results but also for those who pass the screening. Via this system, children with late-onset/acquired hearing loss can be identified early and can receive medical interventions tailored to the cause of their hearing loss while simultaneously avoiding a loss to follow-up.