The role of in bony labyrinth development and otoconial mineralization in mouse models.

Inner ear malformations are predominantly attributed to developmental arrest during the embryonic stage of membranous labyrinth development. Due to the inherent difficulty in clinically assessing the status of the membranous labyrinth, these malformations are diagnosed with radiographic imaging, based on the morphological characteristics of the bony labyrinth. While extensive research has elucidated the intricacies of membranous labyrinth development in mouse models, comprehensive investigations into the developmental trajectory of the bony labyrinth, especially about its calcification process, have been notably lacking.

High fluence in laser stapedotomy aggravates transient subjective dizziness.

Background: Laser fenestration in stapedotomy has thermal effect to the vestibule.

Aims/objectives: To evaluate the role of energy density (fluence) in the severity of postoperative vestibular symptoms.

Materials And Methods: The retrospective chart-review study included 84 patients with otosclerosis that underwent primary laser stapedotomy.

Clinical practice guidelines for the diagnosis and management of acute otitis media in children-2018 update.

Objective: "Clinical Practice Guidelines for the Diagnosis and Management of Acute Otitis Media in Children-2018 update (2018 Guidelines)" aim to provide appropriate recommendations about the diagnosis and management of children with acute otitis media (AOM), including recurrent acute otitis media (recurrent AOM), in children under 15 years of age. These evidence-based recommendations were created with the consensus of the subcommittee members, taking into consideration unique characteristics of bacteriology and antimicrobial susceptibilities of AOM pathogens in Japan, as well as global advances in vaccines.

Methods: The subcommittee re-evaluated key clinical issues based on SCOPE (a master plan of the guidelines) and created clinical questions (CQ) about the diagnosis and management of AOM patients.

Rapid screening of copy number variations in C by droplet digital PCR in patients with mild-to-moderate hearing loss.

Copy number variations (CNVs) are commonly reported in , the causal gene for DFNB16. Various techniques are used clinically for CNV detection, and droplet digital PCR (ddPCR) provides highly precise absolute quantification of DNA copy number. We aimed to validate the feasibility and efficiency of ddPCR in combination with long-range PCR (LR-PCR) in identifying CNVs and mutations in .

Increased expression of pendrin in eosinophilic chronic rhinosinusitis with nasal polyps.

Introduction: Chronic rhinosinusitis with nasal polyps is a heterogeneous disease and appropriate diagnostic algorithms in individual cases are necessary for effective medical treatment.

Objective: The purpose of this study was to clarify the relationship between the pendrin expression of nasal polyps and clinical and pathological characteristic features of eosinophilic chronic rhinosinusitis.

Methods: A total of 68 patients were classified into eosinophilic chronic rhinosinusitis or non-eosinophilic chronic rhinosinusitis groups according to the degree of eosinophilic infiltration into the nasal polyps.

Brain activity in patients with unilateral sensorineural hearing loss during auditory perception in noisy environments.

Patients with unilateral sensorineural hearing loss (UHL) often complain of hearing difficulties in noisy environments. To clarify this, we compared brain activation in patients with UHL with that of healthy participants during speech perception in a noisy environment, using functional magnetic resonance imaging (fMRI). A pure tone of 1 kHz, or 14 monosyllabic speech sounds at 65‒70 dB accompanied by MRI scan noise at 75 dB, were presented to both ears for 1 second each and participants were instructed to press a button when they could hear the pure tone or speech sound.

Relationship between cardiac calcification and left ventricular hypertrophy in patients with chronic kidney disease at hemodialysis initiation.

Coronary artery calcification (CAC), cardiac valve calcification (CVC) and left ventricular hypertrophy (LVH) are frequently observed in chronic kidney disease (CKD) patients. These abnormalities significantly affect morbidity and mortality. The aim of this study was to investigate the relationship between CAC, CVC and LVH in CKD patients.

Randomized controlled trial of juzen-taiho-to in children with recurrent acute otitis media.

Objective: Recurrent acute otitis media (AOM) in young children is rapidly increasing worldwide. Repeated antibiotic use leads to antibiotic-resistant pathogen development. Complementary and alternative medicine approaches have been suggested as a supplemental treatment option to conventional antimicrobial medicine.

[Clinical Characteristics of Pediatric Deep Neck Abscesses].

Pediatric deep neck abscesses are a relatively rare and can lead to critical or life-threatening situations. However, the clinical characteristics of pediatric deep neck abscesses are not fully understood in Japan. We conducted a retrospective study of the clinical characteristics of children presenting with pediatric deep neck abscesses at our hospital.

A nationwide study on enlargement of the vestibular aqueduct in Japan.

Objective: To document the clinical features and associated pure-tone audiometry data in patients with enlargement of the vestibular aqueduct (EVA), and to identify risk factors for fluctuating hearing loss (HL) and vertigo/dizziness in EVA patients.

Methods: In this nationwide survey in Japan, a first survey sheet was mailed to 662 board-certified otolaryngology departments to identify the ones treating EVA patients. A second survey sheet, which contained solicited clinical information and the results of the hearing tests, was mailed to all facilities that reported treating EVA cases.

[Otologic Manifestations in Patients with ANCA Associated Vasculitis-Comparative Analysis among Microscopic Polyangiitis, Granulomatosis with Polyangiitis and Eosinophilic Granulomatosis with Polyangiitis].

Anti-neutrophil cytoplasmic antibody (ANCA) -associated vasculitides (AAVs) include microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA), the incidences of which are reported to be increasing in Japan. We reviewed the clinical records of 20 cases with systemic AAVs (five cases with MPA, nine cases with GPA, and six cases with EGPA), who visited our otolaryngology department with otological symptoms from 2004 to 2014, and compared the otological characteristics among the diseases. Otologic symptoms appeared as an initial symptom(s) in 40% of MPA cases, 56% of GPA cases, and 83% of EGPA cases.

Heterozygous mutation of Ush1g/Sans in mice causes early-onset progressive hearing loss, which is recovered by reconstituting the strain-specific mutation in Cdh23.

Most clinical reports have suggested that patients with congenital profound hearing loss have recessive mutations in deafness genes, whereas dominant alleles are associated with progressive hearing loss (PHL). Jackson shaker (Ush1g) is a mouse model of recessive deafness that exhibits congenital profound deafness caused by the homozygous mutation of Ush1g/Sans on chromosome 11. We found that C57BL/6J-Ush1g heterozygous mice exhibited early-onset PHL (ePHL) accompanied by progressive degeneration of stereocilia in the cochlear outer hair cells.

SOD1 gene polymorphisms in sudden sensorineural hearing loss.

Conclusion: The results suggest that SOD1 rs4998557 could be associated with susceptibility to SSNHL in the Japanese population.

Objectives: To assess the gene association with sudden sensorineural hearing loss (SSNHL).

Methods: A two-stage case control study was conducted to explore the relationship of the candidate genes to SSNHL.

Vertigo by Breast Cancer Metastasis 33 Years after Treatment.

A 76-year-old woman complained of vertigo for two years. She manifested left deafness, loss of caloric response, and right-beaten nystagmus. An imaging study revealed a tumorous lesion located from the clivus to the left temporal bone with inner ear destruction.

Delayed restoration of maximum speech discrimination scores in patients with idiopathic sudden sensorineural hearing loss.

Objective: To assess possible delayed recovery of the maximum speech discrimination score (SDS) when the audiometric threshold ceases to change.

Methods: We retrospectively examined 20 patients with idiopathic sudden sensorineural hearing loss (ISSNHL) (gender: 9 males and 11 females, age: 24-71 years). The findings of pure-tone average (PTA), maximum SDS, auditory brainstem responses (ABRs), and tinnitus handicap inventory (THI) were compared among the three periods of 1-3 months, 6-8 months, and 11-13 months after ISSNHL onset.

Sensorineural hearing loss associated with a factitious disorder.

Factitious disorders are characterized by intentionally abnormal physical and/or psychological behavior, and affected patients often make up their symptoms and clinical histories. The most serious and chronic type of factitious disorder is Munchausen syndrome. We report the case of a 24-year-old woman with a 2-year history of sensorineural hearing loss (SNHL) who later confessed to feigning her hearing loss.

Otological complications associated with hyperbaric oxygen therapy.

The objective of the present study was to clarify the features of otological complications for hyperbaric oxygen therapy (HBOT) and the risk factors for these complications. We enrolled 1115 patients (776 males and 339 females; age 5-89 years) who underwent HBOT. All otological symptoms experienced during HBOT sessions were evaluated, and risk factors were analysed using multivariate logistic regression analysis.

An aberrant carotid artery in the temporal bone with fatal complication.

We report the case of an 84-year-old female presenting with an aberrant ICA with cerebral air embolization caused by Eustachian tube air inflation (ETAI). High pressure of air inflation developed because of an aberrant ICA blocking the tympanic orifice of the Eustachian tube, with release of the high-pressure air into the aberrant ICA. It must be kept in mind that complications may occur not only during transtympanic treatment, but also in any treatment, such as ETAI, in aberrant ICA cases.

Long-term result of meatoplasty using inferiorly based retroauricular island pedicle flap for external auditory canal stenosis.

Objective: To characterize the long-term outcomes of meatoplasty using our new technique, inferiorly based retroauricular island pedicle flap for external auditory canal (EAC) stenosis.

Methods: A long-term clinical analysis of meatoplasty for nine patients (mean age, 33 years; age range, 8-64 years) with stenotic EAC was performed. The follow-up period after meatoplasty ranged from 5 years to 14 years, with a mean duration of 8.

A suspected case of plasma cell-rich acute renal transplant rejection associated with de novo donor-specific antibody.

A kidney transplant case with de novo donor-specific antibody showed monoclonal plasma cell infiltration into the graft with ABO incompatibility. Three years after transplantation, the patient's graft function suddenly deteriorated. Interstitial edema and the predominant infiltration of inflammatory plasma cells with kappa chain monoclonality were observed in biopsy specimens.

Clinical practice guidelines for the diagnosis and management of acute otitis media (AOM) in children in Japan - 2013 update.

Objective: To (1) indicate methods of diagnosis and testing for childhood (<15 years) acute otitis media (AOM) and (2) recommend methods of treatment in accordance with the evidence-based consensus reached by the Subcommittee of Clinical Practice Guideline for Diagnosis and Management of AOM in Children (Subcommittee of Clinical Practice Guideline), in light of the causative bacteria and their drug sensitivity of AOM in Japan.

Methods: We investigated the most recently detected bacteria causing childhood AOM in Japan as well as antibacterial sensitivity and the worldwide distinct progress of vaccination, produced Clinical Questions concerning the diagnosis, testing methods, and treatment of AOM, searched literature published during 2000-2004, and issued the 2006 Guidelines. In the 2009 and 2013 Guidelines, we performed the same investigation with the addition of literature, which were not included in the 2006 Guidelines and published during 2005-2008 and during 2009-2012, respectively.

Clinical characteristics of Japanese oropharyngeal squamous cell carcinoma positive for human papillomavirus infection.

Conclusion: Human papillomavirus (HPV)-related oropharyngeal squamous cell carcinoma (OPSCC) is considered to be a distinct entity in Japan. The combination of both HPV-DNA sequencing analysis and immunohistochemistry (IHC) for p16(INK4A) is useful to discriminate the OPSCC patients with a better prognosis from other cases, especially in the advanced stage. Surgical treatment is recommended for HPV-negative advanced cancer.

Pleomorphic adenoma presenting with conductive hearing loss in the ear canal: a case report and review of the literature.

Introduction: Pleomorphic adenoma accounts for 65 percent of all salivary gland tumors. It has been identified in several anatomical regions, but pleomorphic adenoma arising in the ear canal, first described in 1951, is extremely rare.

Case Presentation: A 40-year-old Japanese man's left ear canal was obstructed by a pleomorphic adenoma that caused mild conductive hearing loss.

Ex vivo visualization of the mouse otoconial layer compared with micro-computed tomography.

Hypothesis: Micro-computed tomography (micro-CT) is useful for assessing the 3-dimensional (3D) morphology and age-related changes of the otoconial layer.

Background: Wriggle mouse Sagami (WMS) is a mutant of the plasma membrane Ca2+ ATPase type2 gene (Atp2b2) with deficits in the saccular otoconia. We examined the effectiveness of micro-CT in evaluating the otoconial layer of WMS and C57BL/6J mice.