The prevalence and clinical features of spasmodic dysphonia: A review of epidemiological surveys conducted in Japan.

Objectives: Spasmodic dysphonia (SD) is a rare disease and its epidemiological status is unclear. This review aimed to explore the current prevalence and clinical features of SD in Japan.

Methods: We reviewed Japanese surveys of SD and compared them to surveys reported from other countries.

The integrity of cochlear hair cells is established and maintained through the localization of Dia1 at apical junctional complexes and stereocilia.

Dia1, which belongs to the diaphanous-related formin family, influences a variety of cellular processes through straight actin elongation activity. Recently, novel DIA1 mutants such as p.R1213X (p.

Hearing vulnerability after noise exposure in a mouse model of reactive oxygen species overproduction.

Previous studies have convincingly argued that reactive oxygen species (ROS) contribute to the development of several major types of sensorineural hearing loss, such as noise-induced hearing loss (NIHL), drug-induced hearing loss, and age-related hearing loss. However, the underlying molecular mechanisms induced by ROS in these pathologies remain unclear. To resolve this issue, we established an in vivo model of ROS overproduction by generating a transgenic (TG) mouse line expressing the human NADPH oxidase 4 (NOX4, NOX4-TG mice), which is a constitutively active ROS-producing enzyme that does not require stimulation or an activator.

An oral pharyngeal scope for objective oropharyngeal examination: a new device for oropharyngeal study.

Objective: There has been little progress in examination of the oropharynx with a light source such as electric light, a penlight, or a forehead mirror over the past 100 years. It is therefore necessary to develop methods to display and record oropharyngeal observations.

Method: Since the aim of this study was to assess the safeness to use from the perspective of physicians, medical staffs, patients, and patients' families and usefulness of pharyngeal scope, the number of devices was limited, the number of patients was not set based on hypothetical statistical tests.

Novel role of Rac-Mid1 signaling in medial cerebellar development.

Rac signaling impacts a relatively large number of downstream targets; however, few studies have established an association between Rac pathways and pathological conditions. In the present study, we generated mice with double knockout of and ( ) in cerebellar granule neurons (CGNs). We observed impaired tangential migration at E16.

A case of recurrent malignant triton tumor successfully treated with radiotherapy.

A 15-year-old female was previously admitted to another hospital because of painless swelling of the lateral right nasal ala for 2 months. Magnetic resonance imaging revealed an expansive enhancing lesion in close proximity to the anterior surface of the right maxillary sinus and lateral wall of the right nasal cavity. Tumor extirpation was performed via the supragingival transantral approach under general anesthesia.

Expression of Sex Steroid Hormone Receptors in Vagal Motor Neurons Innervating the Trachea and Esophagus in Mouse.

The medullary vagal motor nuclei, the nucleus ambiguus (NA) and dorsal motor nucleus of the vagus (DMV), innervate the respiratory and gastrointestinal tracts. We conducted immunohistochemical analysis of expression of the androgen receptor (AR) and estrogen receptor α (ERα), in relation to innervation of the trachea and esophagus via vagal motor nuclei in mice. AR and ERα were expressed in the rostral NA and in part of the DMV.

Deletion of Tricellulin Causes Progressive Hearing Loss Associated with Degeneration of Cochlear Hair Cells.

Tricellulin (also known as MARVELD2) is considered as a central component of tricellular tight junctions and is distributed among various epithelial tissues. Although mutations in the gene encoding tricellulin are known to cause deafness in humans (DFNB49) and mice, the influence of its systemic deletion in vivo remains unknown. When we generated tricellulin-knockout mice (Tric(-/-)), we found an early-onset rapidly progressive hearing loss associated with the degeneration of hair cells (HCs); however, their body size and overall appearance were normal.

Role of the retrotrapezoid nucleus/parafacial respiratory group in coughing and swallowing in guinea pigs.

The retrotrapezoid/parafacial respiratory group (RTN/pFRG) located ventral to the facial nucleus plays a key role in regulating breathing, especially enhanced expiratory activity during hypercapnic conditions. To clarify the roles of the RTN/pFRG region in evoking coughing, during which reflexive enhanced expiration is produced, and in swallowing, during which the expiratory activity is consistently halted, we recorded extracellular activity from RTN/pFRG neurons during these fictive behaviors in decerebrate, paralyzed, and artificially ventilated guinea pigs. The activity of the majority of recorded respiratory neurons was changed in synchrony with coughing and swallowing.

Differential responses to steroid hormones in fibroblasts from the vocal fold, trachea, and esophagus.

There is accumulating evidence that fibroblasts are target cells for steroids such as sex hormones and corticoids. The characteristics of fibroblasts vary among tissues and organs. Our aim in this study is to examine differences in responses to steroid hormones among fibroblasts from different cervicothoracic regions.

Aberrant Myosin 1b Expression Promotes Cell Migration and Lymph Node Metastasis of HNSCC.

Unlabelled: Lymph node metastasis is the major clinicopathologic feature associated with poor prognosis in patients with head and neck squamous cell carcinoma (HNSCC). Here, web-based bioinformatics meta-analysis was performed to elucidate the molecular mechanism of lymph node metastasis of human HNSCC. Preferential upregulation of Myosin 1b (MYO1B) transcript in HNSCC datasets was identified.

Ocular surface reconstruction with a tissue-engineered nasal mucosal epithelial cell sheet for the treatment of severe ocular surface diseases.

Severe ocular surface diseases (OSDs) with severe dry eye can be devastating and are currently some of the most challenging eye disorders to treat. To investigate the feasibility of using an autologous tissue-engineered cultivated nasal mucosal epithelial cell sheet (CNMES) for ocular surface reconstruction, we developed a novel technique for the culture of nasal mucosal epithelial cells expanded ex vivo from biopsy-derived human nasal mucosal tissues. After the protocol, the CNMESs had 4-5 layers of stratified, well-differentiated cells, and we successfully generated cultured epithelial sheets, including numerous goblet cells.

Maintenance of stereocilia and apical junctional complexes by Cdc42 in cochlear hair cells.

Cdc42 is a key regulator of dynamic actin organization. However, little is known about how Cdc42-dependent actin regulation influences steady-state actin structures in differentiated epithelia. We employed inner ear hair-cell-specific conditional knockout to analyze the role of Cdc42 in hair cells possessing highly elaborate stable actin protrusions (stereocilia).

Japanese Board Certification System for head and neck surgeons.

The Japan Society for Head and Neck Surgery (JSHNS) started a board certification system for head and neck surgeons in 2010. To become certified, the following qualification and experiences are required: (1) board certification as otorhinolaryngologist, (2) 2 years of clinical experience in a board-certified training facility, (3) clinical care of 100 patients with head and neck cancer under the supervision of board-certified faculty and (4) surgical experience in 50 major head and neck surgical procedures, including 20 neck dissections, under the supervision of board-certified faculty. The following scientific activities are also required during the preceding 5 years: (1) two clinical papers on head and neck cancers presented at major scientific meetings, (2) one clinical paper on head and neck cancer published in a major journal, (3) attendance at two annual meetings of JSHNS and (4) enrolment in three educational programs approved by JSHNS.

Activity of respiratory neurons in the rostral medulla during vocalization, swallowing, and coughing in guinea pigs.

To examine the relationship between the neuronal networks underlying respiration and non-respiratory behaviors such as vocalization and airway defensive reflexes, we compared the activity of respiratory neurons in the ventrolateral medulla during breathing with that during non-respiratory behaviors including vocalization, swallowing, and coughing in guinea pigs. During fictive vocalization the activity of augmenting expiratory neurons ceased, whereas the other types of expiratory neurons did not show a consistent tendency of increasing or decreasing activity. All inspiratory neurons discharged in synchrony with the phrenic nerve activity.

Middle ear malformations in identical twins.

The majority of the congenital anomalies of middle ear are solitary and a non-hereditary. We report cases of identical twins with congenital incudo-stapedial disconnection. Case 1 was an 8-year-old girl.

Periodic alternating nystagmus during caloric stimulation.

Periodic alternating nystagmus (PAN) is a form of horizontal jerk nystagmus characterized by periodic reversals in direction. We report a case who exhibited transient PAN induced by caloric stimulation. The patient was a 75-year-old male.

The mechanism underlying maintenance of the endocochlear potential by the K+ transport system in fibrocytes of the inner ear.

  The endocochlear potential (EP) of +80 mV in the scala media, which is indispensable for audition, is controlled by K+ transport across the lateral cochlear wall. This wall includes two epithelial barriers, the syncytium and the marginal cells. The former contains multiple cell types, such as fibrocytes, which are exposed to perilymph on their basolateral surfaces.

NMII forms a contractile transcellular sarcomeric network to regulate apical cell junctions and tissue geometry.

Nonmuscle myosin II (NMII) is thought to be the master integrator of force within epithelial apical junctions, mediating epithelial tissue morphogenesis and tensional homeostasis. Mutations in NMII are associated with a number of diseases due to failures in cell-cell adhesion. However, the organization and the precise mechanism by which NMII generates and responds to tension along the intercellular junctional line are still not known.

Does a vascular supercharge improve the clinical outcome for free jejunal transfer?

To clarify whether a supercharged free jejunal transfer would have a different clinical outcome from the usual transfer method, we examined clinical data from cases of esophago-pharyngeal reconstruction. Fifty-three patients in whom the hypopharynx and cervical esophagus was reconstructed with a free jejunal transfer were divided into two groups: 19 normal procedures and 34 supercharged. Clinical outcomes including intraoperative and postoperative events, complications and deglutition were compared statistically.

K(+)-Cl(-) cotransporter 1 (KCC1) negatively regulates NGF-induced neurite outgrowth in PC12 cells.

Potassium chloride cotransporters (KCCs) mediate electroneutrally-coupled transport of K(+) and Cl(-), and play crucial roles in various cell functions including regulation of cell volume and homeostasis of cellular Cl(-)content. Four isoforms of KCCs (KCC1, 2, 3, and 4) have been identified. KCC1 is ubiquitously expressed, whereas KCC2 is mainly expressed in neuronal cells of central nervous system.

Laryngeal stenosis in a patient with severe congenital neutropenia.

Objectives/hypothesis: We report the first case of laryngeal stenosis with granuloma in a patient with severe congenital neutropenia (SCN).

Study Design: Case report and retrospective review.

Methods: Review of medical records.