Publications by authors named "Jay Rubinstein"

Article Synopsis
  • Loss of function mutations in the STRC gene are a leading cause of inherited hearing loss, impacting outer hair cells and their connection to the tectorial membrane, which affects sound amplification.
  • A study involving nine young participants with these mutations evaluated their ability to perceive temporal and spectral modulations, as well as speech in noisy environments, comparing their performance to normal hearing individuals and cochlear implant users.
  • Results indicated that STRC subjects had poorer spectral ripple discrimination and spondee reception thresholds compared to normal hearing but performed better than cochlear implant users, highlighting differences in hearing capacity linked to STRC mutations.
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Objective: To explore socioeconomic disparities in cochlear implant evaluation (CIE) referrals and cochlear implantation.

Study Design: Retrospective chart review.

Setting: Tertiary referral academic center.

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Objective: Cochlear implantation of prelingually deaf infants provides auditory input sufficient to develop spoken language; however, outcomes remain variable. Inability to participate in speech perception testing limits testing device efficacy in young listeners. In postlingually implanted adults (aCI), speech perception correlates with spectral resolution an ability that relies independently on frequency resolution (FR) and spectral modulation sensitivity (SMS).

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Importance: In the US, most childhood-onset bilateral sensorineural hearing loss is genetic, with more than 120 genes and thousands of different alleles known. Primary treatments are hearing aids and cochlear implants. Genetic diagnosis can inform progression of hearing loss, indicate potential syndromic features, and suggest best timing for individualized treatment.

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Objectives: Spectral resolution correlates with speech understanding in post-lingually deafened adults with cochlear implants (CIs) and is proposed as a non-linguistic measure of device efficacy in implanted infants. However, spectral resolution develops gradually through adolescence regardless of hearing status. Spectral resolution relies on two different factors that mature at markedly different rates: Resolution of ripple peaks (frequency resolution) matures during infancy whereas sensitivity to across-spectrum intensity modulation (spectral modulation sensitivity) matures by age 12.

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Article Synopsis
  • The study aims to differentiate NOG-related symphalangism spectrum disorder (NOG-SSD) from other syndromes caused by deletions on chromosome 17q22 and enhance surgical approaches for stapes surgery in these patients.
  • Two families with NOG-SSD were analyzed, revealing a chromosomal deletion in one and a mutation in the other, both contributing to distinct skeletal and hearing issues.
  • The findings suggest that the clinical features of NOG-SSD are less severe than those associated with other chromosome 17q22 deletions, and surgeons should use longer stapes prostheses during surgeries for patients with NOG-SSD.
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Auditory nerve responses to electrical stimulation exhibit aberrantly synchronous response latencies to low-rate pulse trains, nevertheless, cochlear implant users generally have elevated inter-aural timing difference detection thresholds. These findings present an apparent paradox in which single units are unusually precise but downstream within the auditory pathway access to this precision is lost. Auditory nerves innervating a region of cochlea exhibit natural heterogeneity in their diameter, myelination, and other structural properties; a key question is whether this diversity may contribute to the loss of temporal fidelity.

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This opinion statement proposes a set of candidacy criteria for vestibular implantation of adult patients with bilateral vestibulopathy (BVP) in a research setting. The criteria include disabling chronic symptoms like postural imbalance, unsteadiness of gait and/or head movement-induced oscillopsia, combined with objective signs of reduced or absent vestibular function in both ears. These signs include abnormal test results recorded during head impulses (video head impulse test or scleral coil technique), bithermal caloric testing and rotatory chair testing (sinusoidal stimulation of 0.

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Objective: To determine if Menière's disease is associated with fluctuations in afferent excitability in four human subjects previously implanted with vestibular stimulators.

Study Design: Longitudinal repeated measures.

Setting: Tertiary referral center, human vestibular research laboratory.

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Spectral ripple discrimination tasks are commonly used to probe spectral resolution in cochlear implant (CI), normal-hearing (NH), and hearing-impaired individuals. In addition, these tasks have also been used to examine spectral resolution development in NH and CI children. In this work, stimulus sine-wave carrier density was identified as a critical variable in an example spectral ripple-based task, the Spectro-Temporally Modulated Ripple (SMR) Test, and it was demonstrated that previous uses of it in NH listeners sometimes used values insufficient to represent relevant ripple densities.

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Background: A combined vestibular and cochlear prosthesis may restore hearing and balance to patients who have lost both. To do so, the device should activate each sensory system independently.

Objectives: In this study, we quantify auditory and vestibular interactions during interleaved stimulation with a combined 16-channel cochlear and 6-channel vestibular prosthesis in human subjects with both hearing and vestibular loss.

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While micro-CT systems are instrumental in preclinical research, clinical micro-CT imaging has long been desired with cochlear implantation as a primary application. The structural details of the cochlear implant and the temporal bone require a significantly higher image resolution than that (about 0.2 ) provided by current medical CT scanners.

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Objective: Auditory and vestibular outcomes after placement of a vestibular-cochlear implant in subjects with varying causes of vestibular loss.

Study Design: Prospective case study.

Setting: Tertiary referral center.

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Objective: To report audiovestibular outcomes following endolymphatic shunt surgery (ELS) and intratympanic gentamicin injections (ITG) in patients with Meniere's disease (MD).

Study Design: Retrospective matched cohort study METHODS: Patients with MD refractory to medical management between 2004 and 2017 were reviewed: 44 patients underwent ELS and had outcomes available, while 27 patients underwent ITG and had outcomes available. Mean follow-up durations for the ELS and ITG groups were 39.

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Studies have demonstrated the benefits of low frequency residual hearing in music perception and for psychoacoustic abilities of adult cochlear implant (CI) users, but less is known about these effects in the pediatric group. Understanding the contribution of combined electric and acoustic stimulation in this group can help to gain a better perspective on decisions regarding bilateral implantation. We evaluated the performance of six unilaterally implanted children between 9 and 13 years of age with contralateral residual hearing using the Clinical Assessment of Music Perception (CAMP), spectral ripple discrimination (SRD), and temporal modulation transfer function (TMTF) tests and compared findings with previous research.

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Mandarin-speaking cochlear implant users have difficulty perceiving tonal changes in speech with current signal processing strategies. The purpose of this study was to evaluate whether English-speaking cochlear implant and normal hearing listeners can be trained to recognise closed-set Mandarin tones. The validity of using native-English speakers to evaluate Mandarin tone perception in cochlear implants was tested.

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Objective: To examine whether or not electric-acoustic music perception outcomes, observed in a recent Hybrid L24 clinical trial, were related to the availability of low-frequency acoustic cues not present in the electric domain.

Study Design: Prospective, repeated-measures, within-subject design.

Setting: Academic research hospital.

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Electrical vestibular neurostimulation may be a viable tool for modulating vestibular afferent input to restore vestibular function following injury or disease. To do this, such stimulators must provide afferent input that can be readily interpreted by the central nervous system to accurately represent head motion to drive reflexive behavior. Since vestibular afferents have different galvanic sensitivity, and different natural sensitivities to head rotational velocity and acceleration, and electrical stimulation produces aphysiological synchronous activation of multiple afferents, it is difficult to assign a priori an appropriate transformation between head velocity and acceleration and the properties of the electrical stimulus used to drive vestibular reflex function, i.

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Since cochlear implant function involves direct depolarization of spiral ganglion neurons (SGNs) by applied current, SGN physiological health must be an important factor in cochlear implant (CI) outcomes. This expected relationship has, however, been difficult to confirm in implant recipients. Suggestively, animal studies have demonstrated both acute and progressive SGN ultrastructural changes (notably axon demyelination), even in the absence of soma death, and corresponding altered physiology following sensorineural deafening.

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Background: Children with steeply sloping sensorineural hearing loss (SNHL) lack access to critical high-frequency cues despite the use of advanced hearing aid technology. In addition, their auditory-only aided speech perception abilities often meet Food and Drug Administration criteria for cochlear implantation.

Purpose: The objective of this study was to describe hearing preservation and speech perception outcomes in a group of young children with steeply sloping SNHL who received a cochlear implant (CI).

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Spectral resolution limits speech perception with a cochlear implant (CI) in post-lingually deaf adults. However, the development of spectral resolution in pre-lingually deaf implanted children is not well understood. Acoustic spectral resolution was measured as a function of age (school-age versus adult) in CI and normal-hearing (NH) participants using spectral ripple discrimination (SRD).

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