Association Between the Development of Sensorineural Hearing Loss and Blood NAD Levels.

Background: Hearing loss prevalence increases with age, affecting over 25% of the global population aged 60 years or older. The aim of the study was to investigate the association between the development of sensorineural hearing loss (SNHL) and the blood levels of nicotinamide adenine dinucleotide (NAD).

Methods: A single-center, observational study was conducted at Kawagoe Otology Institute in Japan.

Visualization of Alternating Triangular Domains of Charge Density Waves in 2H-NbSe_{2} by Scanning Tunneling Microscopy.

The charge density wave (CDW) state of 2H-NbSe_{2} features commensurate domains separated by domain boundaries accompanied by phase slips known as discommensurations. We have unambiguously visualized the structure of CDW domains using a displacement-field measurement algorithm on a scanning tunneling microscopy image. Each CDW domain is delimited by three vertices and three edges of discommensurations and is designated by a triplet of integers whose sum identifies the types of commensurate structure.

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.

Case Reports on Effect of Intratympanic Steroid Injection Therapy on Late-Onset Hearing Loss in Congenital Cytomegalovirus Infection.

Background: One of the conditions or symptoms caused by congenital cytomegalovirus (cCMV) infection is late-onset hearing loss. This report examines the cases of two children exhibiting late-onset hearing loss after cCMV infection who showed improvement in hearing after undergoing intratympanic steroid injection therapy (IST).

Cases: Case1 is girl aged 8 years and 10 months and case2 is girl aged 5 years and 1 month.

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.

Detailed clinical features and genotype-phenotype correlation in an OTOF-related hearing loss cohort in Japan.

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.

Efficacy, safety, and pharmacokinetics of oral valganciclovir in patients with congenital cytomegalovirus infection.

Objectives: Valganciclovir (VGCV) has been shown to improve sensorineural hearing loss (SNHL) and neurological outcomes in patients with neonatal symptomatic congenital cytomegalovirus (cCMV) infection. However, reports on the pharmacokinetics, efficacy and safety of oral VGCV are limited. The aim of this study is to evaluate the pharmacokinetics of VGCV for use in the treatment of cCMV.

10-year follow-up of congenital cytomegalovirus infection complicated with severe neurological findings in infancy: a case report.

Background: Congenital cytomegalovirus (cCMV) infection leads to sensorineural hearing loss (SNHL) and neurodevelopmental delays. However, the long-term outcomes of cCMV infection with severe neurological manifestations in infancy remain unclear.

Case Presentation: The patient was a one-month-old girl visited owing to abnormalities in neonatal hearing screening.

Proton-Driven Intercalation and Ion Substitution Utilizing Solid-State Electrochemical Reaction.

The development of an unconventional synthesis method has a large potential to drastically advance materials science. In this research, a new synthesis method based on a solid-state electrochemical reaction was demonstrated, which can be made available for intercalation and ion substitution. It was referred to as proton-driven ion introduction (PDII).

A child with severe inner ear malformations with favorable hearing utilization and balance functions after wearing hearing aids.

Infants with congenital deafness caused by severe bilateral inner ear malformations frequently suffer from severe hearing loss and poor balance. Unfortunately, the use of hearing aids is usually ineffective in recovering hearing, necessitating cochlear implants. We report a case of a 6-year-old boy with congenital deafness and bilateral inner ear malformations (right side, incomplete partition type I [IP-I]; left side, common cavity deformity).

Constitutive activation of DIA1 (DIAPH1) via C-terminal truncation causes human sensorineural hearing loss.

DIAPH1 encodes human DIA1, a formin protein that elongates unbranched actin. The c.3634+1G>T DIAPH1 mutation causes autosomal dominant nonsyndromic sensorineural hearing loss, DFNA1, characterized by progressive deafness starting in childhood.

Discrimination of Japanese monosyllables in patients with high-frequency hearing loss.

Objective: To analyze the difficulty of discriminating Japanese nonsense monosyllables in each of several grades of high-frequency hearing loss and to evaluate the limitations of amplification.

Methods: We collected retrospective data on the discrimination of Japanese nonsense monosyllables by patients with three grades of high-frequency hearing loss who fulfilled or nearly fulfilled the Japanese criteria for EAS. Discrimination of the twenty monosyllables included in the 67-S speech audiometric test, which is approved by the Japan Audiological Society, was evaluated under quiet conditions.

Cisplatin-induced hearing loss: the need for a long-term evaluating system.

Cisplatin is an effective chemotherapeutic agent against pediatric cancers; however, ototoxicity is a concern. This study describes the frequency, severity, and clinical course of hearing loss in Japanese pediatric patients treated with cisplatin-based multimodal therapy. A total of 55 children who received cisplatin-based therapy from 1983 to 2012 underwent audiologic evaluations.

GJB2-associated hearing loss undetected by hearing screening of newborns.

The hearing loss caused by GJB2 mutations is usually congenital in onset, moderate to profound in degree, and non-progressive. The objective of this study was to study genotype/phenotype correlations and to document 14 children with biallelic GJB2 mutations who passed newborn hearing screening (NHS). Genetic testing for GJB2 mutations by direct sequencing was performed on 924 individuals (810 families) with hearing loss, and 204 patients (175 families) were found to carry biallelic GJB2 mutations.

Etiology and one-year follow-up results of hearing loss identified by screening of newborn hearing in Japan.

Objective: To evaluate the incidence of newborn hearing loss in a Japanese population and to elucidate etiological factors and one-year prognosis.

Study Design: Screening of newborn hearing.

Setting: Children's tertiary referral center.

Risk factors for hearing loss after pediatric meningitis in Japan.

Objectives: We sought to identify predictors for hearing loss in Japanese children with meningitis.

Methods: We analyzed 155 cases of pediatric meningitis without other entities causing hearing loss in children admitted to Saitama Children's Medical Center between 1990 and 2005 for potential risk factors for hearing loss, using multiple logistic regression. Auditory brain stem response tests were performed to evaluate hearing loss.

Effects of oral care in Down syndrome children with obstructive sleep apnea.

Down syndrome (DS) children with sleep apnea often present with oral breathing associated with nasal obstruction. This causes the oral cavity and pharynx to become dry. We describe the treatment of three DS children with sleep apnea who were treated using products for oral dryness.

Spontaneous otoacoustic emissions in two infants.

Conclusion: We regard spontaneous otoacoustic emission (SOAE) as a unique phenomenon due to spontaneous outer hair cell hyperactivity during the infantile period in these two infants.

Objectives: We report the cases of SOAEs in two infants, which were audible to their parents. We were able to hear continuous sounds from these two infants' ears.

Superconducting Pb island nanostructures studied by scanning tunneling microscopy and spectroscopy.

Superconductivity of nanosized Pb-island structures whose radius is 0.8 to 2.5 times their coherence length was studied under magnetic fields using low-temperature scanning tunneling microscopy and spectroscopy.

Discrepancy between auditory brainstem responses, auditory steady-state responses, and auditory behavior in two patients with Pelizaeus-Merzbacher disease.

We reported two cases of Pelizaeus-Merzbacher disease. Both cases visited our hospital manifesting horizontal nystagmoid movements present from birth, and delayed motor development. Magnetic resonance imaging of the brain showed diffuse dysmyelination of the cerebral white matter, and auditory brainstem response showed waves I and II but absence of all subsequent components.