Types of Inheritance and Genes Associated with Familial Meniere Disease
AbstractMeniere disease (MD) is a rare disorder of the inner ear defined by sensorineural hearing loss (SNHL) associated with episodes of vertigo and tinnitus. The phenotype is variable, and it may be associated with other comorbidities such as migraine, respiratory allergies, and several autoimmune disorders. The condition has a significant heritability according to epidemiological and familial segregation studies. Familial MD is found in 10% of cases, the most frequently found genes beingOTOG,MYO7A, andTECTA, previously associated with autosomal dominant and recessive non-syndromic SNHL. These findings suggest a new hypo...
Source: JARO - Journal of the Association for Research in Otolaryngology - April 6, 2023 Category: ENT & OMF Source Type: research

The Remarkable Outer Hair Cell: Proceedings of a Symposium in Honour of W. E. Brownell
AbstractIn 1985, Bill Brownell and colleagues published the remarkable observation that cochlear outer hair cells (OHCs) express voltage-driven mechanical motion: electromotility. They proposed OHC electromotility as the mechanism for the elusive “cochlear amplifier” required to explain the sensitivity of mammalian hearing. The finding and hypothesis stimulated an explosion of experiments that have transformed our understanding of cochlear mechanics and physiology, the evolution of hair cell structure and function, and audiology. Here, w e bring together examples of current research that illustrate the continuing impac...
Source: JARO - Journal of the Association for Research in Otolaryngology - April 1, 2023 Category: ENT & OMF Source Type: research

High Spectral and Temporal Acuity in Primary Auditory Cortex of Awake Cats
AbstractMost accounts of single- and multi-unit responses in auditory cortex under anesthetized conditions have emphasized V-shaped frequency tuning curves and low-pass sensitivity to rates of repeated sounds. In contrast, single-unit recordings in awake marmosets also show I-shaped and O-shaped response areas having restricted tuning to frequency and (for O units) sound level. That preparation also demonstrates synchrony to moderate click rates and representation of higher click rates by spike rates of non-synchronized tonic responses, neither of which are commonly seen in anesthetized conditions. The spectral and tempora...
Source: JARO - Journal of the Association for Research in Otolaryngology - April 1, 2023 Category: ENT & OMF Source Type: research

Minimum Detectable Differences in Electrocochleography Measurements: Bayesian-Based Predictions
AbstractPhysiology of the cochlea and auditory nerve can be assessed with electrocochleography (ECochG), a technique that involves measuring auditory evoked potentials from an electrode placed near or within the cochlea. Research, clinical, and operating room applications of ECochG have in part centered on measuring the auditory nerve compound action potential (AP) amplitude, the summating potential (SP) amplitude, and the ratio of the two (SP/AP). Despite the common use of ECochG, the variability of repeated amplitude measurements for individuals and groups is not well understood. We analyzed ECochG measurements made with...
Source: JARO - Journal of the Association for Research in Otolaryngology - April 1, 2023 Category: ENT & OMF Source Type: research

The Long Outer-Hair-Cell RC Time Constant: A Feature, Not a Bug, of the Mammalian Cochlea
AbstractThe cochlea of the mammalian inner ear includes an active, hydromechanical amplifier thought to arise via the piezoelectric action of the outer hair cells (OHCs). A classic problem of cochlear biophysics is that theRC (resistance-capacitance) time constant of the hair-cell membrane appears inconveniently long, producing an effective cut-off frequency much lower than that of most audible sounds. The longRC time constant implies that the OHC receptor potential —and hence its electromotile response—decreases by roughly two orders of magnitude over the frequency range of mammalian hearing, casting doubt on the hypo...
Source: JARO - Journal of the Association for Research in Otolaryngology - April 1, 2023 Category: ENT & OMF Source Type: research

Untargeted Metabolomic Analysis in Endolymphatic Sac Luminal Fluid from Patients with Meniere ’s Disease
AbstractDysfunction of the endolymphatic sac (ES) is one of the etiologies of Meniere ’s disease (MD), the mechanism of which remains unclear. The aim of the present study was to explore the molecular pathological characteristics of ES during the development of MD. Metabolomic profiling of ES luminal fluid from patients with MD and patients with acoustic neuroma (AN) was performed. Diluted ES luminal fluid (ELF) samples were obtained from 10 patients who underwent endolymphatic duct blockage for the treatment of intractable MD and from 6 patients who underwent translabyrinthine surgery for AN. ELF analysis was performed ...
Source: JARO - Journal of the Association for Research in Otolaryngology - April 1, 2023 Category: ENT & OMF Source Type: research

Enhanced Expression of Plasminogen Activators and Inhibitor in the Healing of Tympanic Membrane Perforation in Rats
AbstractThe significance of plasminogen activation during the tympanic membrane (TM) healing is known mainly from studies performed on knock-out mice. In the previous study, we reported activation of genes coding proteins of plasminogen activation and inhibition system in rat ’s TM perforation healing. The aim of the present study was the evaluation of protein products expressed by these genes and their tissue distribution using Western blotting and immunofluorescent method, respectively, during 10-day observation period after injury. Otomicroscopical and histological evaluation were employed to assess the healing proces...
Source: JARO - Journal of the Association for Research in Otolaryngology - April 1, 2023 Category: ENT & OMF Source Type: research

Visualizing Collagen Fibrils in the Cochlea ’s Tectorial and Basilar Membranes Using a Fluorescently Labeled Collagen-Binding Protein Fragment
ConclusionThe CNA35 probe provides a unique and useful view of collagenous structures in the cochlea. (Source: JARO - Journal of the Association for Research in Otolaryngology)
Source: JARO - Journal of the Association for Research in Otolaryngology - April 1, 2023 Category: ENT & OMF Source Type: research

Surfactant Protein A Can Affect Macrophage Phagocytosis: An Important Pathogenic Mechanism of Otitis Media with Effusion
AbstractOtitis media with effusion (OME), also known as secretory otitis media, is a common condition in otorhinolaryngology. The main manifestations include middle ear effusion and conductive hearing loss. Recently, increasing attention has been paid to the etiology of OME, wherein immune dysfunction is one important pathogenic mechanism. However, it is unknown whether changes in surfactant protein A (SPA) secretion affect the phagocytic activity of macrophages in the Eustachian tube, thereby altering pathogen clearance, during the pathogenesis of OME. In our study, an OME animal model was established and evaluated. Diffe...
Source: JARO - Journal of the Association for Research in Otolaryngology - April 1, 2023 Category: ENT & OMF Source Type: research

Nuclear Translocation Triggered at the Onset of Hearing in Cochlear Inner Hair Cells of Rats and Mice
ConclusionNuclear migration at the onset of hearing is a key step in the morphological maturation of IHCs. Whether this plays a role in functional maturation remains to be explored. (Source: JARO - Journal of the Association for Research in Otolaryngology)
Source: JARO - Journal of the Association for Research in Otolaryngology - March 17, 2023 Category: ENT & OMF Source Type: research

An Inverse Relationship Between Gray Matter Volume and Speech-in-Noise Performance in Tinnitus Patients with Normal Hearing Sensitivity
In this study, pure-tone audiometry and Quick Speech-in-Noise test were conducted on individuals with tinnitus and normal hearing and hearing-matched controls. T1-weighted structural MRI images were obtained from all participants. After preprocessing, GM volumes were compared between tinnitus and control groups using whole-brain and region-of-interest analyses. Further, regression analyses were performed to examine the correlation between regional GM volume and SiN scores in each group. The results showed decreased GM volume in the right inferior frontal gyrus in the tinnitus group relative to the control group. In the tin...
Source: JARO - Journal of the Association for Research in Otolaryngology - March 3, 2023 Category: ENT & OMF Source Type: research

Surfactant Protein A Can Affect Macrophage Phagocytosis: An Important Pathogenic Mechanism of Otitis Media with Effusion
AbstractOtitis media with effusion (OME), also known as secretory otitis media, is a common condition in otorhinolaryngology. The main manifestations include middle ear effusion and conductive hearing loss. Recently, increasing attention has been paid to the etiology of OME, wherein immune dysfunction is one important pathogenic mechanism. However, it is unknown whether changes in surfactant protein A (SPA) secretion affect the phagocytic activity of macrophages in the Eustachian tube, thereby altering pathogen clearance, during the pathogenesis of OME. In our study, an OME animal model was established and evaluated. Diffe...
Source: JARO - Journal of the Association for Research in Otolaryngology - February 23, 2023 Category: ENT & OMF Source Type: research

Enhanced Expression of Plasminogen Activators and Inhibitor in the Healing of Tympanic Membrane Perforation in Rats
AbstractThe significance of plasminogen activation during the tympanic membrane (TM) healing is known mainly from studies performed on knock-out mice. In the previous study, we reported activation of genes coding proteins of plasminogen activation and inhibition system in rat ’s TM perforation healing. The aim of the present study was the evaluation of protein products expressed by these genes and their tissue distribution using Western blotting and immunofluorescent method, respectively, during 10-day observation period after injury. Otomicroscopical and histological evaluation were employed to assess the healing proces...
Source: JARO - Journal of the Association for Research in Otolaryngology - February 21, 2023 Category: ENT & OMF Source Type: research

High Spectral and Temporal Acuity in Primary Auditory Cortex of Awake Cats
AbstractMost accounts of single- and multi-unit responses in auditory cortex under anesthetized conditions have emphasized V-shaped frequency tuning curves and low-pass sensitivity to rates of repeated sounds. In contrast, single-unit recordings in awake marmosets also show I-shaped and O-shaped response areas having restricted tuning to frequency and (for O units) sound level. That preparation also demonstrates synchrony to moderate click rates and representation of higher click rates by spike rates of non-synchronized tonic responses, neither of which are commonly seen in anesthetized conditions. The spectral and tempora...
Source: JARO - Journal of the Association for Research in Otolaryngology - February 16, 2023 Category: ENT & OMF Source Type: research

Minimum Detectable Differences in Electrocochleography Measurements: Bayesian-Based Predictions
AbstractPhysiology of the cochlea and auditory nerve can be assessed with electrocochleography (ECochG), a technique that involves measuring auditory evoked potentials from an electrode placed near or within the cochlea. Research, clinical, and operating room applications of ECochG have in part centered on measuring the auditory nerve compound action potential (AP) amplitude, the summating potential (SP) amplitude, and the ratio of the two (SP/AP). Despite the common use of ECochG, the variability of repeated amplitude measurements for individuals and groups is not well understood. We analyzed ECochG measurements made with...
Source: JARO - Journal of the Association for Research in Otolaryngology - February 16, 2023 Category: ENT & OMF Source Type: research