Cochlear Implantation in the Poorer-Hearing Ear Is a Reasonable Choice.

Choosing the optimal side for cochlear implantation (CI) remains a major challenge because of the lack of evidence. We investigated the choice of the surgery side for CI (i.e.

Revision cochlear implant surgery in adults and children is a safe and effective technique: a retrospective single faculty study in Okayama University Hospital, Japan.

Background: Cochlear implant (CI) surgery is a safe surgical technique, although some patients require revision CI surgery.

Aims/objectives: This study investigated the cause and underlying reason of revision CI surgery as well as hearing outcomes in a single institution.

Patients And Methods: This retrospective study evaluated patients who underwent CI surgery between April 2006 to March 2022 ( = 351).

Endolymphatic Hydrops on Magnetic Resonance Imaging May Be an Independent Finding on Aging in Neurotologic Patients.

Objective: To clarify whether age impacts the development of endolymphatic hydrops (ELH) in neurotologic patients. Documentation of ELH in living patients on magnetic resonance imaging (MRI) allows analysis of patient age and formation of ELH, which is impossible by means of postmortem temporal bone pathology.

Study Design: Retrospective case review.

15-year follow-up for steroid-responsive, fluctuating hearing loss in the ear with endolymphatic hydrops confirmed by magnetic resonance imaging.

Autoimmune mechanisms may play crucial roles in the etiology of endolymphatic hydrops (ELH), which was previously regarded as a postmortem finding in the temporal bone. Recently, ELH has been visualized using 3-T MR imaging in living patients. A 47-year-old woman with deafness in the left ear since adolescence developed right-sided steroid-responsive sensorineural hearing loss in the low frequencies.

Mutation-agnostic RNA interference with engineered replacement rescues -related hearing loss.

Hearing loss is the most common sensory deficit, of which genetic etiologies are a frequent cause. Dominant and recessive mutations in , a gene encoding the pore-forming subunit of the hair cell mechanotransduction channel, cause DFNA36 and DFNB7/11, respectively, accounting for ∼2% of genetic hearing loss. Previous work has established the efficacy of mutation-targeted RNAi in treatment of murine models of autosomal dominant non-syndromic deafness.

The voltage-gated Ca2+ channel subunit α2δ-4 regulates locomotor behavior and sensorimotor gating in mice.

Voltage-gated Ca2+ channels are critical for the development and mature function of the nervous system. Variants in the CACNA2D4 gene encoding the α2δ-4 auxiliary subunit of these channels are associated with neuropsychiatric and neurodevelopmental disorders. α2δ-4 is prominently expressed in the retina and is crucial for vision, but extra-retinal functions of α2δ-4 have not been investigated.

Upregulation of a nuclear factor-kappa B-interacting immune gene network in mice cochleae with age-related hearing loss.

Epidemiological data suggest that inflammation and innate immunity play significant roles in the pathogenesis of age-related hearing loss (ARHL) in humans. In this mouse study, real-time RT-PCR array targeting 84 immune-related genes revealed that the expressions of 40 genes (47.6%) were differentially regulated with greater than a twofold change in 12-month-old cochleae with ARHL relative to young control mice, 33 (39.

Effects of Protective Measures against COVID-19 on Auditory Communication for People with Hearing Loss.

WHO has recommended various measures to combat the COVID-19 pandemic, including mask-wearing and physical distancing. However, these changes impair communication for individuals with hearing loss. We investigated the changes in auditory communication associated with COVID-19 measures in 269 patients (male: 45.

Effect of Prostaglandin D on mRNA Expression of Three Isoforms of Hyaluronic Acid Synthase in Nasal Polyp Fibroblasts.

Background: Hyaluronan is one of the major extracellular matrixes in chronic rhinosinusitis (CRS) associated with tissue remodeling. Prostaglandin D (PGD) is also associated with the pathogenesis of CRS. However, little is known about whether PGD regulates hyaluronan production by human airway fibroblasts.

Hair Cell Transduction Efficiency of Single- and Dual-AAV Serotypes in Adult Murine Cochleae.

Gene delivery is a key component for the treatment of genetic hearing loss. To date, a myriad of adeno-associated virus (AAV) serotypes and surgical approaches have been employed to deliver transgenes to cochlear hair cells, but the efficacy of dual transduction remains unclear. Herein, we investigated cellular tropism of single injections of AAV serotype 1 (AAV1), AAV2, AAV8, AAV9, and Anc80L65, and quantitated dual-vector co-transduction rates following co-injection of AAV2 and AAV9 vectors in adult murine cochlea.

Gene therapy for hearing loss.

Sensorineural hearing loss (SNHL) is the most common sensory disorder. Its underlying etiologies include a broad spectrum of genetic and environmental factors that can lead to hearing loss that is congenital or late onset, stable or progressive, drug related, noise induced, age related, traumatic or post-infectious. Habilitation options typically focus on amplification using wearable or implantable devices; however exciting new gene-therapy-based strategies to restore and prevent SNHL are actively under investigation.

Targeted PCR Array Analysis of Genes in Innate Immunity and Glucocorticoid Signaling Pathways in Mice Cochleae Following Acoustic Trauma.

Aim: To comprehensively analyze cochlear gene expressions related to innate immunity and glucocorticoid signaling at onset of acute noise-induced hearing loss (NIHL).

Background: Recent studies suggested innate immunity is involved in the cochlear pathology of NIHL. Glucocorticoids may modulate immune actions in cochleae.

Transcanal endoscopic ear surgery for perilymphatic fistula after electric acoustic stimulation.

Transcanal endoscopic ear surgery (TEES) will become a very useful therapeutic option. A perilymphatic fistula (PLF) is defined as sudden sensorineural hearing loss and/or vertigo caused by leakage of the perilymph through a fistula from the oval window and/or round window. We report a case of PLF after electric acoustic stimulation (EAS), a kind of cochlear implant, successfully treated by TEES.

Evaluation of a new and simple classification for endoscopic sinus surgery.

Objective: In 2013, the Japanese Rhinologic Society proposed a simple classification for endoscopic sinus surgery (ESS). This classification consists of five procedures (type I, fenestration of the ostiomeatal complex, with uncinectomy and widening of the natural ostium; type II, single-sinus procedure, with manipulating the inside of the sinus; type III, polysinus procedure; type IV, pansinus procedure; type V, extended procedure beyond the sinus wall). The clinical relevance of this classification in chronic rhinosinusitis (CRS) and paranasal sinus cyst was evaluated.

Cochlear Transcriptome Following Acoustic Trauma and Dexamethasone Administration Identified by a Combination of RNA-seq and DNA Microarray.

Aim: To elucidate molecular mechanisms of noise-induced hearing loss (NIHL) and glucocorticoid therapy in the cochlea.

Background: Glucocorticoids are used to treat many forms of acute sensorineural hearing loss, but their molecular action in the cochlea remains poorly understood.

Methods: Dexamethasone was administered intraperitoneally immediately following acoustic overstimulation at 120 dB SPL for 2 hours to mice.

[Genetic Counseling of a Hearing-impaired Patient with Multiple Genetic Mutations].

Since April 2012, genetic testing for congenital hearing loss is covered by the public health insurance in Japan. Recent (since August 2015) developments in next-generation sequencing technology have enabled the detection of 154 mutations in 19 genes. Genetic testing provides valuable information on hearing phenotype, prognosis, and prediction of associated symptoms.

Effect of prostaglandin D2 on VEGF release by nasal polyp fibroblasts.

Background: Vascular endothelial growth factor (VEGF) is known to be associated with the pathogenesis of chronic rhinosinusitis with nasal polyps (CRSwNP). VEGF is produced by a variety of cells including fibroblasts. It was recently reported that prostaglandin (PG) E induces VEGF release by nasal polyp fibroblasts.

Pediatric central auditory processing disorder showing elevated threshold on pure tone audiogram.

Central auditory processing disorder (CAPD) is a condition in which dysfunction in the central auditory system causes difficulty in listening to conversations, particularly under noisy conditions, despite normal peripheral auditory function. Central auditory testing is generally performed in patients with normal hearing on the pure tone audiogram (PTA). This report shows that diagnosis of CAPD is possible even in the presence of an elevated threshold on the PTA, provided that the normal function of the peripheral auditory pathway was verified by distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), and auditory steady state response (ASSR).

Regulatory effect of TLR3 signaling on staphylococcal enterotoxin-induced IL-5, IL-13, IL-17A and IFN-γ production in chronic rhinosinusitis with nasal polyps.

Background: Toll-like receptor 3 (TLR3) is expressed in upper airways, however, little is known regarding whether Toll-like receptor 3 (TLR3) signals exert a regulatory effect on the pathogenesis of chronic rhinosinusitis with nasal polyps (CRSwNP), especially on eosinophilic inflammation. We sought to investigate the effect of Poly(IC), the ligand for TLR3, on cytokine production by dispersed nasal polyp cells (DNPCs).

Methods: DNPCs were pretreated with or without Poly(IC), and were then cultured in the presence or absence of staphylococcal enterotoxin B (SEB), following which the levels of IL-5, IL-10, IL-13, IL-17A and interferon (IFN)-γ in the supernatant were measured.

Characteristics of audiogram configuration in multiple-system atrophy C and cortical cerebellar atrophy.

Conclusion: The prevalence of low-tone hearing loss (LTHL) is significantly high in spinocerebellar degeneration (SCD) with cerebellar predominance, including multiple-system atrophy C (MSA-C) and cortical cerebellar atrophy (CCA).

Objective: This study aimed to test the hypothesis that SCD with cerebellar predominance, MSA-C and CCA may cause auditory symptoms.

Methods: The shape and threshold of pure-tone audiograms were evaluated for MSA-C (n = 47; mean (± SD) age, 61.

Impact of early intervention on comprehensive language and academic achievement in Japanese hearing-impaired children with cochlear implants.

Objectives: Early hearing detection and intervention (EHDI) is critical for achievement of age-appropriate speech perception and language development in hearing-impaired children. It has been 15 years since newborn hearing screening (NHS) was introduced in Japan, and its effectiveness for language development in hearing-impaired children has been extensively studied. Moreover, after over 20 years of cochlear implantation in Japan, many of the prelingual cochlear implant (CI) users have reached school age, and the effect of CI on language development have also been assessed.

Outcomes of consonant-vowel monosyllable perception and word recognition after cochlear implantation in elderly Japanese patients.

Conclusion: Performance in consonant-vowel (CV) monosyllable speech perception after cochlear implantation (CI) in the elderly (≥ 65 years) is equivalent to that of young adults (18-64 years). Present data in the Japanese language supported the indication for CI in the elderly. Word recognition after CI was significantly lower in the elderly than young adults.