Temporal Bone Histopathology of Atypical Cogan Syndrome.

Cogan's syndrome is a rare disorder first clinically defined in 1945, characterized by nonsyphilitic interstitial keratitis and progressive audiovestibular symptoms. Later, patients with audiovestibular dysfunction and various types of inflammatory eye disease were classified as having atypical Cogan's syndrome. The etiology and pathogenesis of Cogan's syndrome remain largely unknown.

Utility and optimal management of planned drug holidays during lenvatinib treatment in patients with unresectable differentiated thyroid cancer: a real-world multi-center study.

Purpose: Lenvatinib achieves favorable therapeutic outcomes for patients with radioactive iodine therapy refractory differentiated thyroid cancer (DTC); however, its use is associated with a high incidence of adverse events. To avoid severe adverse events, planned drug holidays (PDH) have been proposed. This study aimed to evaluate treatment effects, identify prognostic factors, and investigate the usefulness of PDH in patients with unresectable DTC who received lenvatinib across the multi-institutions.

Fast abdominal magnetic resonance elastography with simultaneous encoding of three-dimensional displacements.

Three-dimensional (3D) magnetic resonance elastography (MRE) is more accurate than two-dimensional (2D) MRE; however, it requires long-term acquisition. This study aimed to reduce the acquisition time of abdominal 3D MRE using a new sample interval modulation (short-SLIM) approach that can acquire all three motions faster while reducing the prolongation of echo time and flow compensation. To this end, two types of phantom studies and an in vivo test of the liver in three healthy volunteers were performed to compare the performances of conventional spin-echo echo-planar (SE-EPI) MRE, conventional SLIM and short-SLIM.

Evoked Potentials by Tone Burst on the Auditory Cortices in Cats -Comparison of Off Responses in Awake and Anesthetized Conditions.

Background: Auditory Brainstem Response (ABR) recording in awake is essential to detect off-responses. This study clarified whether after-termination responses on ABR were offset responses, off-responses or a mixture of the two.

Methods: Evoked potentials in the auditory cortex of cats in response to tone burst stimuli were recorded, and off responses were examined with chronically implanted electrodes.

Liposarcoma of the pleural cavity.

Liposarcoma rarely occurs in the pleura or thoracic cavity, and few reports appear in the literature. We hypothesized that combining clinicopathologic, immunohistochemical, and fluorescence in situ hybridization methods would allow definite diagnoses. Using formalin-fixed, paraffin-embedded blocks, we examined 6 atypical lipomatous tumor/well-differentiated liposarcomas (ALT/WDLPS), 5 dedifferentiated liposarcomas (DDLPSs), 2 pleomorphic liposarcomas, and 1 myxoid liposarcoma (MLPS).

Serum cytokines and bone metabolic markers in patients with rheumatoid arthritis treated with biological disease modifying anti-rheumatic drugs.

Introduction: /objectives Several biological disease-modifying anti-rheumatic drugs (bDMARDs) have been widely used for the management of rheumatoid arthritis (RA). These drugs target different molecules important for the pathophysiology of RA; however, only a few studies have compared the effects of these biological drugs on cytokines and bone metabolic markers. The main aim of this study is to clarify the effects of bDMARDs with different modes of action on the cytokine and bone metabolic marker levels in patients with RA.

Comparison of vestibular ocular reflex and gross motor development in children with semicircular canal aplasia and hypoplasia.

Objective: To examine the effect of vestibular ocular reflex on gross motor development in children with semicircular canal aplasia and hypoplasia.

Study Design: Case series.

Materials And Methods: Children with congenital hearing loss underwent temporal bone computed tomography to assess their inner ear morphology.

Efficacy of transtympanic infusion of dexamethasone into the tympanic cavity in mice with acute sensorineural hearing loss associated with cytomegalovirus infection.

Background: There is no report on acute sensorineural hearing loss with congenital cytomegalovirus (cCMV) infection in basic experiments.

Aims/objectives: The aim of this study was to evaluate the effect of dexamethasone, an anti-inflammatory steroid, on acute sensorineural hearing loss in the mouse cytomegalovirus (MCMV) infection model mice.

Material And Methods: Sensorineural hearing loss model mice were divided into two groups, one with and one without intratympanic dexamethasone.

Association of work environment with stress and depression among Japanese workers.

Background: There is a lack of studies that investigated the effect of a wide range of work environmental factors on stress and depression in Japan.

Objectives: To examine the association of work environment factors with stress and depression among workers in Japan.

Methods: We conducted questionnaire surveys of workers that mainly engage in desk work in Japan.

Dietary Patterns and Their Associations with Intermediate Age-Related Macular Degeneration in a Japanese Population.

This population-based cross-sectional study investigated the influence of dietary patterns on age-related macular degeneration (AMD) in a Japanese population. The Tsuruoka Metabolomics Cohort Study enrolled a general population aged 35-74 years from among participants in annual health check-up programs in Tsuruoka City, Japan. Eating habits were assessed using a food frequency questionnaire.

Subcortical deafness as a subtype of auditory agnosia after injury of bilateral auditory radiations caused by two cerebrovascular accidents - normal auditory brainstem responses with I-VII waves and abolished consciousness of hearing.

Background: In central auditory disorders caused by damage of the cerebral hemispheres, there are cortical deafness and auditory agnosia. Although clinical cases of cortical deafness have been reported, little is known about the hearing problems and localized lesions associated with cortical deafness.

Aims/objectives: The aims of our research are to elucidate lesion sites associated with cortical deafness and to clarify why patients with cerebral lesions are not aware of any sound at all.

Should We Aim to Create a Perfect Healthy Utopia? Discussions of Ethical Issues Surrounding the World of Project Itoh's Harmony.

To consider whether or not we should aim to create a perfect healthy utopia on Earth, we focus on the SF novel Harmony (2008), written by Japanese writer Project Ito, and analyze various issues in the world established in the novel from a bioethical standpoint. In the world depicted in Harmony, preserving health and life is a top priority. Super-medicine is realized through highly advanced medical technologies.

Cohort Profile: The Network (GEN), a Network of Japanese Ophthalmological Epidemiology Studies.

Purpose: Japan has been known as a super-aged society, and ageing is a well-known risk factor for blinding eye diseases. However, epidemiological studies in ophthalmology are still scarce in Japan, and the sizes of the cohorts are relatively small. "Ganka-Ekigaku Network" (GEN, an acronym for the epidemiological network in ophthalmology in Japanese) is established to develop a capacity to boost each epidemiological study and enrich a potential inter-study collaboration to identify risk factors of visual impairment in aged society.

Vestibular Aging Process from 3D Physiological Imaging of the Membranous Labyrinth.

There is no three-dimensional (3D) technique to study the microanatomical structures of the in vivo 3D vestibular membranous labyrinth. Recent two MRI methods using a contrast agent can only depict the low-resolution imaging of endolymphatic hydrops. Therefore, we provide the new precise volume rendering algorithms to create the in vivo 3D vestibular membranous labyrinth images from high-resolution temporal bone low-dose CT data.

An unperceived acoustic stimulus decreases reaction time to visual information in a patient with cortical deafness.

Responding to multiple stimuli of different modalities has been shown to reduce reaction time (RT), yet many different processes can potentially contribute to multisensory response enhancement. To investigate the neural circuits involved in voluntary response initiation, an acoustic stimulus of varying intensities (80, 105, or 120 dB) was presented during a visual RT task to a patient with profound bilateral cortical deafness and an intact auditory brainstem response. Despite being unable to consciously perceive sound, RT was reliably shortened (~100 ms) on trials where the unperceived acoustic stimulus was presented, confirming the presence of multisensory response enhancement.

Electrically evoked ABR during cochlear implantation and postoperative development of speech and hearing abilities in infants with common cavity deformity as a type of inner ear malformation.

The electrically evoked auditory brainstem response (eABR) during cochlear implantation in common cavity (CC) deformity has not been clinically well studied. To investigate the eABR wave configuration during cochlear implantation, the postoperative development of hearing, and educational settings in infants with CC deformity. Nine infants who were congenitally deaf and found to have CC deformity and suspected cochlear nerve deficiency by temporal bone CT and MRI were studied.

Characterization of GUCA1A-associated dominant cone/cone-rod dystrophy: low prevalence among Japanese patients with inherited retinal dystrophies.

GUCA1A gene variants are associated with autosomal dominant (AD) cone dystrophy (COD) and cone-rod dystrophy (CORD). GUCA1A-associated AD-COD/CORD has never been reported in the Japanese population. The purpose of this study was to investigate clinical and genetic features of GUCA1A-associated AD-COD/CORD from a large Japanese cohort.

Radiogenomics Consortium Genome-Wide Association Study Meta-Analysis of Late Toxicity After Prostate Cancer Radiotherapy.

Background: A total of 10%-20% of patients develop long-term toxicity following radiotherapy for prostate cancer. Identification of common genetic variants associated with susceptibility to radiotoxicity might improve risk prediction and inform functional mechanistic studies.

Methods: We conducted an individual patient data meta-analysis of six genome-wide association studies (n = 3871) in men of European ancestry who underwent radiotherapy for prostate cancer.

Prediction of Causative Genes in Inherited Retinal Disorders from Spectral-Domain Optical Coherence Tomography Utilizing Deep Learning Techniques.

Purpose: To illustrate a data-driven deep learning approach to predicting the gene responsible for the inherited retinal disorder (IRD) in macular dystrophy caused by and gene aberration in comparison with retinitis pigmentosa caused by gene aberration and normal subjects.

Methods: Seventy-five subjects with IRD or no ocular diseases have been ascertained from the database of Japan Eye Genetics Consortium; 10 retinopathy, 20 retinopathy, 28 retinopathy, and 17 normal patients/subjects. Horizontal/vertical cross-sectional scans of optical coherence tomography (SD-OCT) at the central fovea were cropped/adjusted to a resolution of 400 pixels/inch with a size of 750 × 500 pix for learning.

Development of vestibular ocular reflex and gross motor function in infants with common cavity deformity as a type of inner ear malformation.

Unlabelled: Background： The function of common cavity deformity demonstrated by temporal bone CT and MRI has been unknown.

Aim/objective: To investigate the developmental changes of vestibular ocular reflex and acquisition of postural control in infants with common cavity deformity.

Material And Methods: Eight infants who were congenitally deaf complicated by common cavity deformity were studied.

Novel mutations in the gene in Japanese patients with X-linked congenital retinoschisis.

X-linked congenital retinoschisis (XLRS) is an inherited retinal disorder characterized by reduced central vision and schisis of the macula and peripheral retina. XLRS is caused by mutations in the gene. We have identified 37 different mutations in the gene, including 12 novel mutations, in 67 Japanese patients from 56 XLRS families.

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

Introduction: Pendred syndrome is an autosomal-recessive disease characterized by congenital hearing loss and thyroid goiter. Previously, cell stress susceptibilities were shown to increase in patient-derived cells with intracellular aggregation using an acute cochlear cell model derived from patient-specific pluripotent stem (iPS) cells. Moreover, we showed that rapamycin can relieve cell death.