Using electrocochleography to detect sensory and neural damages in a gerbil model

Hearing is one of the five sensory organs that allows us to interact with society and our environment. However, one in eight Americans suffers from sensorineural hearing loss that is great enough to adversely impact their daily life. There is an urgent need to identify what part/degree of the auditory pathway (sensory or neural) is compromised so that appropriate treatment/intervention can be implemented. Single- or two-tone evoked potentials, the electrocochleography (eCochG), were measured along the auditory pathway, i.e., at the round window and remotely at the vertex, with simultaneous recordings of ear canal distortion product otoacoustic emissions. Sensory (cochlear) and neural components in the (remote-) eCochG responses showed distinct level- and frequency-dependent features allowing to be differentiated from each other. Specifically, the distortion products in the (remote-)eCochGs can precisely localize the sensory damage showing that they are effective to determine the sensory or neural damage along the auditory pathway.

Acceleration sound design for vehicles using distortion products

When considering the acoustic design of automobiles, low-frequency sounds can increase the excitement levels for users. However, there are several problems accompany an increase in the low-frequency levels of an engine sound. For example, it is difficult to create a balance between silence and excitement when a sound's different order components are changed. It is also difficult to generate heavy bass engine sounds in practical scenarios. Thus, the application of distortion products in the auditory system of the cochlea is considered. Distortion products are perceived when two or more sounds with slightly different frequencies are played simultaneously. This study was conducted to examine the possibility of achieving powerful engine sounds using distortion products. At first, the relationship between different combinations of complex sounds and the pitch perception of distortion products was investigated. As a second step, the application of distortion products to the acceleration sound was also considered. The results suggested the possibility of synthesizing a low-frequency component using distortion products inside a cochlea.

Origins and minimization of intermodulation distortion in a pseudo-differential CMOS beamforming receiver

This paper studies how nonlinear distortion is generated in the combination of an inverter-based low-noise amplifier and a passive mixer. The dominant nonlinearity appears to be the quadratic $$V_{gs}V_{ds}$$ V gs V ds mixing term in the passive mixer that first causes low-frequency IM2 and then upconverts it to IM3. Adding a common-mode feedback (CMFB) cancels the IM2 in a pseudo-differential structure, and hence also reduces the IM3 caused by the cascaded second order nonlinearities significantly. The effect of CMFB gain, bandwidth and linearity were analyzed, and it is concluded that from the linearity point of view, the feedback circuit does not have to be very wideband since the dominant distortion products originate from baseband. Finally, the paper takes a look at the spurious tones rising in the mixing, and how to extend the analysis to include the actual frequency translation effect.

Intracochlear distortion products are broadly generated by outer hair cells but their contributions to otoacoustic emissions are spatially restricted

Detection of low-level sounds by the mammalian cochlea requires electromechanical feedback from outer hair cells (OHCs). This feedback arises due to the electromotile response of OHCs, which is driven by the modulation of their receptor potential caused by the stimulation of mechano-sensitive ion channels. Nonlinearity in these channels distorts impinging sounds, creating distortion-products that are detectable in the ear canal as distortion-product otoacoustic emissions (DPOAEs). Ongoing efforts aim to develop DPOAEs, which reflects the ear’s health, into diagnostic tools for sensory hearing loss. These efforts are hampered by limited knowledge on the cochlear extent contributing to DPOAEs. Here, we report on intracochlear distortion products (IDPs) in OHC electrical responses and intracochlear fluid pressures. Experiments and simulations with a physiologically motivated cochlear model show that widely generated electrical IDPs lead to mechanical vibrations in a frequency-dependent manner. The local cochlear impedance restricts the region from which IDPs contribute to DPOAEs at low to moderate intensity, which suggests that DPOAEs may be used clinically to provide location-specific information about cochlear damage.

Patterns of noise exposure and prevalence of hearing loss amongst Cape Town Minstrel Carnival musicians

Background: Cape Town Minstrel Carnival is one of the oldest and most authentic indigenous New Year’s customs in South Africa. Musicians who perform at this carnival are exposed to excessively loud music and therefore at a risk of acquiring noise-induced hearing loss (NIHL).Objectives: This study aimed to determine patterns of exposure to loud music and prevalence of hearing loss amongst Cape Town Minstrel Carnival musicians.Method: A descriptive, observational exploratory survey design was used and 43 participants (21 males and 22 females; mean age, 21 ± 9 years) took part in this study. Sound level measurements were conducted to assess musicians’ sound exposure during rehearsals and performances. All participants underwent the following audiological test battery at least 2 h before music exposure: Case history, otoscopic examination, tympanometry, pure tone audiometry and distortion products otoacoustic emission (DPOAE).Results: Average noise levels recorded were 86 dBA during rehearsals and 98.7 dBA at performances and average durations of exposure were 240 and 10 min at rehearsals and performances, respectively. One out of 43 (1/43) participants presented with sensorineural hearing loss. Audiometric results of the remaining participants were normal and did not show a pattern suggestive of NIHL. A high proportion of participants (21/43) reported experiencing tinnitus.Conclusion: Despite being exposed to high levels of noise, there was a low prevalence of hearing loss amongst these musicians. However, a high proportion of them reported tinnitus, which could be an indication that they were at a high risk of NIHL from the music that they played.

VGLUT3-p.A211V variant fuses stereocilia bundle and elongates synaptic ribbons in the human deafness DFNA25

DFNA25 is an autosomal-dominant and progressive form of human deafness caused by mutations in the SLC17A8 gene, which encodes the vesicular glutamate transporter type 3 (VGLUT3). To resolve the mechanisms underlying DFNA25, we studied the phenotype of the mouse harboring the p.A221V mutation in human (corresponding to p.A224V in mouse). Using auditory brainstem response and distortion products of otoacoustic emissions, we showed that VGLUT3A224V/A224V mouse replicates the DFNA25 progressive hearing loss with intact cochlear amplification. Scanning electron microscopy examinations demonstrated fused stereocilia bundle of the inner hair cells (IHCs) as the primary cause for DFNA25. In addition, the IHC ribbon synapses undergo structural and functional modifications at later stages. Using super-resolution microscopy, we observed oversized synaptic ribbons associated with an increase in the rate of the sustained releasable pool of exocytosis. These results indicate that the primary defect in DFNA25 stems from a failure in the mechano-transduction followed by a change in synaptic transfer. The VGLUT3A224V/A224V mouse model opens the way to a deeper understanding and to a potential treatment of DFNA25.