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Galvanic disruption of vestibulospinal postural control by cochlear implant devices.

All subjects with implanted cochlear stimulators demonstrated evidence of abnormal postural stability without their stimulators activated. Instability increased when they were tested with cochlear stimulation units turned on, and additional instability was demonstrated in four of these subjects when tested in noise. These findings suggest that the electrical stimulation delivered by the cochlear prosthesis is not limited to the auditory system. The precise characteristics of electrical stimulation devices designed for stimulations limited to the cochlea and their spurious effects upon motor performance should be investigated. The design of future intralabyrinthine auditory electrical prostheses must include hardware designs and stimulus paradigms that avoid undesirable vestibular system stimulation.

Adult

The Soundtrack of Everyday Life: Real-world Music Listening Habits of Adult Cochlear Implant Users.

OBJECTIVE: Characterize real-world patterns of music listening and reward sensitivity among adult cochlear implant (CI) users compared with normal-hearing (NH) listeners. STUDY DESIGN: Cross-sectional observational study. SETTING: Online. PATIENTS: Adults (&#x2265;18&#xa0;y) with a CI or NH who used a music-streaming platform as their primary listening method. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Objective measures included platform-derived audio features (acousticness, danceability, energy, tempo, and valence), listening volume, unique-song ratio, and decade preferences. Self-reported measures included listening habits and the Barcelona Music Reward Questionnaire (BMRQ). Group comparisons used ANCOVAs and mixed-effect models adjusting for age and gender; within-CI analyses compared prelingual versus postlingual deafness. RESULTS: Among 16 CI users (69% male, 39.0&#xb1;15.6&#xa0;y) and 29 NH listeners (38% male, 32.2&#xb1;11.3&#xa0;y), CI users demonstrated a higher unique-song ratio (&#x3b2;=-0.157, CI as reference; 95%CI [-0.281, -0.034]; P =0.014) and stronger preference for older music (Pillai trace=0.537; F8,35 =5.07; P <0.001), adjusting for age. No significant group differences were observed in weekly listening time, listening volume, audio features, or BMRQ scores (total and subscores). Equivalence was confirmed for Emotion Evocation and Sensory-Motor subscales. There were no statistically significant differences in listening environments after Holm correction. No statistically detectable differences were observed between pre- and postlingually deafened CI users. CONCLUSIONS: Musically active CI users showed no significant differences in listening volume or overall music-reward sensitivity compared with NH peers, but demonstrated higher unique-song ratios and a bias toward older music. Findings highlight the value of ecologically valid data in understanding real-world music experiences among CI users.

Adult

Cochlear Implantation in Sickle Cell Disease: A Systematic Review of Feasibility and Outcomes.

INTRODUCTION: Sickle cell disease (SCD) is associated with systemic complications, including sensorineural hearing loss (SNHL) from microvascular occlusion and chronic inflammation. Although reports link SCD to higher rates of SNHL, current evidence is limited by small sample size, varied audiologic methods, and lack of standardized screening. This systematic review summarizes available literature on SNHL and cochlear implantation (CI) in SCD. METHODS: A literature search of PubMed MEDLINE, Embase, Scopus, Web of Science, and CINAHL identified 79 citations. After removal of duplicates, 35 records were screened in Rayyan. Studies published between January 1, 2000, and June 30, 2025, were eligible if they reported patients with SCD who developed hearing loss and underwent CI. Nine full texts were reviewed, and 4 met the inclusion criteria. Screening and review were performed independently by 2 authors per PRISMA guidelines. RESULTS: Across 4 case reports, a total of 5 patients with SCD underwent CI, ranging in age from 2 to 42 years. Four presented with bilateral severe-to-profound SNHL and one with unilateral loss. Implantation was technically feasible in all cases, including patients with cochlear fibrosis or ossification requiring modified insertion techniques. Postoperative outcomes were favorable: all patients demonstrated reliable device function and low impedances. Only 60% showed meaningful auditory benefit, characterized by improved functional speech perception in 2 patients (40%) and access to the speech frequency range with hearing testing going from moderate/profound hearing loss to mild hearing loss in 3 patients (60%). Complications occurred in 2 patients (40%): one developed unilateral middle ear infection leading to meningitis, and another experienced a postoperative pulmonary embolism requiring anticoagulation. The remaining 3 patients (60%) had uncomplicated recoveries with reported improved hearing from moderate/profound hearing loss to mild hearing loss post implantation. CONCLUSION: While CI appears feasible in SCD, our findings suggest that additional data are needed to assess its effectiveness in this patient population. However, evidence is limited to case reports, and complications such as thromboembolism and rapid cochlear fibrosis highlight the need for close perioperative management. More comprehensive studies with larger sample size are required to define surgical risk, optimize management, and establish best practices for timely implantation in this population.

Humans

The technical aspects of cochlear implantation.

The historical development of attempts to provide some hearing ability, in totally deaf patients, by electrical stimulation of the cochlear nerve is reviewed to the point at which single channel intracochlear electrodes were tried in patients. The results obtained with this type of prosthesis are described and discussed in terms of the possible physiological mechanisms involved. Results obtained from promontory stimulation are also presented as a means of patient selection and are compared with the results from intracochlear electrodes. Based on these findings, the approaches for the development of multichannel intracochlear electrodes and for a single channel electrode with speech processing are described. The problems involved with materials, coding, electrode isolation and transmission are considered and the current results in these areas are detailed. Such results imply that the long-term prospects of developing a prosthesis that could provide adequate speech intelligibility are good.

Animals

Assessment of Surgical Salvage Outcomes for Exposed Cranial Neuromodulating Devices.

OBJECTIVES: Implanted neuromodulating devices (NMDs) such as cochlear implants (CIs) and deep brain stimulators (DBSs) are commonly used in modern medicine. Rarely, complications arise post-operatively, including hardware exposure. Traditional teaching suggests that these devices require removal if exposed; however, surgical salvage is a high risk, high reward alternative. We review our single institution experience managing NMD exposure with surgical salvage. METHODS: Retrospective chart review was performed on individuals who had a NMD implanted and underwent an attempt at surgical salvage for exposure during the study period (January 01, 2021 through December 31, 2023). Study outcome success was defined as maintaining a functional NMD 1&#x2009;year after salvage was attempted. Surgical techniques associated with successful salvage were compared. RESULTS: Nine of 729 patients (1.2%) implanted with NMDs experienced hardware exposure during this 2-year study period. Nine subjects were referred for NMD salvage; however, only 6 of 9 subjects (66.7%, CI&#x2009;=&#x2009;3; DBS&#x2009;=&#x2009;3) underwent NMD salvage attempts. Four of the subjects had successful salvage demonstrated successful salvage with a functioning NMD and without wound healing concerns 1&#x2009;year after their salvage procedure. CONCLUSIONS: Classic teaching states that exposed NMDs require explantation. However, this approach necessarily imposes time without benefit from the NMD between explantation and reimplantation. Our experience demonstrates that surgical salvage can be a successful alternative for the majority (66.7%) of individuals.

Humans

Can ChatGPT Replace Human Clinical Coders? A Comparative Study in Otology Billing.

OBJECTIVE: Evaluate the utility of the large language model (LLM), ChatGPT, for the analysis of operative notes and the generation of Current Procedural Terminology (CPT) codes in comparison to human clinical coders. STUDY DESIGN: CPT billing codes assigned by ChatGPT were compared to existing billing data. Otology practice within a tertiary academic center. METHODS: About 191 operative notes from a single surgeon (9/2022-10/2023) were analyzed. ChatGPT-3.5 and 4 models were prompted for CPT codes based on operative notes. Assessment included determining exact and partial match rates, sensitivity and specificity for targeted procedures, and work Relative Value Units (wRVU) differences between ChatGPT-generated and human-assigned codes. RESULTS: ChatGPT-3.5 achieved exact matches in 22% of cases and partial matches in 32%, while ChatGPT-4 achieved 14% exact and 33% partial matches. When cochlear implantation (CI) was excluded, performance dropped significantly. For CI, ChatGPT-3.5 demonstrated a sensitivity of 94% and specificity of 90%, while ChatGPT-4 showed a sensitivity of 96% and specificity of 92%. In contrast, performance on cartilage grafting was poor, with sensitivities of 4.2% for ChatGPT-3.5 and 0% for ChatGPT-4. ChatGPT-3.5 and 4 showed moderate CPT code matching accuracy among themselves, with slight agreement to human coders. Both models tended to underbill for wRVUs compared to human coders, with significant differences in the values generated. CONCLUSION: This study assessed ChatGPT's effectiveness in automating CPT code assignment for otologic surgeries. While the models achieved high sensitivity values for assigning codes related to cochlear implantation, both models struggled with complex cases, failed to apply modifiers, and often assigned fewer wRVUs. The findings highlight ChatGPT's potential in medical billing but indicate a need for further refinement.

Humans

External electrical stimulation of the cochlea: clinical, psychophysical, speech-perceptual and histological findings.

Our progress towards the development of a particular form of cochlear implant for the totally deaf is described. A single channel stimulation at the round window or promontory is used. This involves a minimum of surgical intervention and infective risk, preserves the possibility of remission and allows the application of later developments. The signal used for stimulation is designed to be matched both to the deaf lip-reader's needs and to his new, restricted, auditory ability. This is done by concentrating on the acoustic pattern components of speech which carry intonation and voiced-voiceless information. Surgical electrophysical, psychoacoustic and speech perceptual aspects of our work with twelve patients are described. The tests involve responses, for example, relating to: threshold for sinusoids; frequency difference limens; periodic -aperiodic discrimination; stress placement; and consonant labelling using combined visual and electrical inputs. Relatively extensive measurements were made with six patients. Significant individual differences were found and the sets of responses provide an essential basis for an appraisal of the potential usefulness of our work to the individual patient. Possible reasons for the individual differences are discussed. A brief indication is given of the techniques which we have developed for the future speech training and speech production evaluation of patients with electro-cochlear voice monitoring. The final section of our paper mentions our histological investigation of the effects of this type of stimulation in the guinea pig.

Acoustic Impedance Tests

[Clinical observations in electric stimulation of the ear (author's transl)].

For 20 years direct electrical stimulation has been used in cases of severe bilateral hearing loss or complete deafness to mediate acoustic percepts. The relevant literature is reviewed. While these attempts were initially thought to be unphysiological and unsuited for transferring speech, acoustic sensations were successfully conveyed even with the very simple unichannel electrodes through percutaneous signal transfer. Patients fitted with such simple systems were able to hear and distinguish environmental noise and speech, their lip reading as well as their speech improved. Speech discrimination was, however, impossible with such simple implants both on theoretical grounds and in practical terms, because frequency analysis is exclusively based on periodicity (up to 400 Hz). Designing bipolar multichannel electrodes which, when introduced into the scala tympani or the modiolus, produce discrete stimulation of several circumscribed groups of nerve fibers, was the logical consequence of earlier attempts along these lines, Implantation of these systems can be done along the transmeatal, meato-mastoidal or mastoidal approach. The electrodes can be implanted in bundles through the round window or into the modiolus; they can, however, also be introduced individually through several drill holes in the promontory for placement in the scala tympani and vestibuli. This produces a far more differentiated stimulation simulating a tonotopic pattern of stimuli. In addition to periodicity, the place principle can thus be utilized for frequency coding. While their dynamic range is rather poor (15 to 30 dB at best), multichannel systems, in theory, offer substantially more favorable conditions for speech intelligibility. Since current knowledge of speech coding is, however, inadequate, the degree of intelligibility obtainable is still insufficient for everyday life. Inspite of this flaw, such implants as are available today substantially benefit the patients, who are able to establish acoustic communications with their environment by distinguishing environmental noise from speech, to discriminate between male and female voices, to recognize musical rhythms and even to understand a few words. Indications for the implantation of prostheses, the requisite conditions and postoperative training programs are discussed. While cochlear implants are still experimental, they appear to be reasonably justified in selected cases, since they have been well tolerated by all patients treated sofar without causing any complications and since many of the data obtainable can only be collected in humans. It is, however, essential that experimental implantation be exclusively dealt with by specialized teams, which should evaluate such data as are available and translate them into practice as soon as possible. A routine impantation of hearing prostheses is currently unwarranted.

Cochlea

A translational framework for early-phase inner-ear gene therapy: clinical trial design, regulatory strategy, and ethical considerations.

PURPOSE OF REVIEW: Hereditary hearing loss has historically been approached as a diagnostic category rather than a therapeutically modifiable disease. Recent advances in molecular genetics, cochlear gene delivery, and first-in-human clinical trials are changing that. This review summarizes contemporary progress in the genetics of hearing loss, with emphasis on emerging gene-based therapies, clinical trial design, regulatory and ethical considerations, and practical implications for otolaryngologists as biologic treatment enters clinical practice. RECENT FINDINGS: Early clinical trials targeting OTOF -related DFNB9 deafness have demonstrated satisfactory safety profiles and meaningful auditory recovery, establishing the first proof-of-concept for cochlear gene therapy in humans, culminating in the April 2026 FDA approval of Otarmeni. Genetic diagnoses are increasingly informing prognosis, cochlear implant counseling, and therapeutic candidacy. Preclinical research continues to expand toward recessive, dominant, and syndromic hearing loss using gene replacement, antisense, RNA interference, and genome-editing strategies. Substantial challenges remain, including heterogeneous outcome measures, uncertain long-term efficacy, regulatory complexity, and inequitable global access. SUMMARY: The genetics of hearing loss is transitioning from a diagnostic modality to an interventional one. Widespread clinical impact will require advances in vector engineering, equitable implementation, multidisciplinary counseling, and integration with established rehabilitation pathways. For otolaryngologists, genetic literacy is becoming essential to contemporary hearing care.

Humans

Vestibular effects of electrical stimulation of the cochlea. Monitored in the awake primate.

The effect of electrical stimulation of the cochlea on vestibular response was monitored in six rhesus monkeys. Eye movements and single-unit activity from the vestibular portion of the eighth nerve, vestibular nuclei, reticular formation, and abducens nucleus were observed while electrical stimulation was delivered through an implanted cochlear prosthesis. In one animal of this series, neural activity from the inferior colliculus and cochlear nuclear complex was also recorded. Electrical stimulation elicited eye-movement responses in only one animal. In the animals from which single-unit activity was recorded, no positive vestibular effects were noted. In one animal of this study, responses were elicited from auditory structures by relatively low intensities of electrical stimulation.

Acoustic Stimulation

Utility of hearing aid use on neurocognitive functions and auditory processing: A systematic review.

PURPOSE: Hearing loss not only affects the peripheral auditory system but also leads to changes in central auditory pathways and neurocognitive skills. The use of hearing aids has shown potential benefits in mitigating the adverse effects of hearing loss. Earlier literature showed various studies assessing the impact of hearing aid use on cognitive functions and auditory processing, a comprehensive review of the existing literature is warranted. Existing literature showed heterogeneous outcomes among studies investigated the effect of Hearing aid use in combating the adverse effects of hearing loss on neurocognitive functions and auditory processing. METHOD: Databases including PubMed, SciELO, Cochrane Library, PsycINFO, Web of Science, and Index Copernicus were searched (2005-2025) using English keywords on hearing aid effects on neurocognition and auditory processing in individuals with hearing loss. Studies on effect of hearing aid use on neurocognition and auditory processing were included, while those involving cochlear implant users or other neurological disorders unrelated to hearing loss were excluded. Titles, authors and publication years were examined, and duplicates were removed. RESULTS: Total of 61 research papers were found on specific area and were included in the present review. Findings of these 61 articles have been analysed and are reported in this article. The outcome of the present study showed usefulness of hearing aid use on neurocognitive functions and auditory processing. CONCLUSION: The present literature review emphasized the importance of early rehabilitation through hearing aid use to alleviate the adverse effects of hearing loss on neurocognitive function and auditory processing.

Humans

Newborn screening for common genetic variants associated with permanent hearing loss: Implementation in Ontario and review of the first 3&#xa0;years.

PURPOSE: Early hearing detection and intervention (EHDI) programs using audiometric screening techniques alone have a limited ability to detect noncongenital childhood permanent hearing loss (PHL). In 2019, Ontario launched universal newborn screening (NBS) for PHL risk factors, including congenital cytomegalovirus and 22 common variants in GJB2 and SLC26A4. Here, we describe our experience in screening for genetic risk factors. METHODS: Ontario newborns who participated in universal newborn hearing screening (UNHS) were offered risk factor screening using dried blood spots (DBS) collected for conventional newborn screening. The screening was conducted using a custom MassArray assay, and positive results were confirmed by Sanger sequencing or polymerase chain reaction. Diagnostic audiological assessments were performed for all screen-positive infants. RESULTS: Of the 412,424 infants screened, 93 had 2 variants in GJB2 or SLC26A4. Of these, 72 had confirmed PHL, 20 had normal hearing, and 1 declined follow-up. Thirteen infants with PHL (1 in 31,724; 11.8% of screen positives) were not identified through audiometric testing as they passed (3) or missed (10) the screening. Importantly, among infants who ultimately received cochlear implants, the detection of genetic etiology through NBS led to an accelerated time to diagnosis, assessment, and intervention. CONCLUSION: Genetic screening has strengthened UNHS and care for infants with or at risk of PHL in Ontario. This study is a step toward the broader inclusion of genomic testing in NBS.

Humans

Psychophysical evaluation of cochlear prostheses in a monkey model.

Functional aspects of cochlear prostheses implanted in the scala tympani were tested in monkeys trained to perform a simple reaction-time task. Thresholds for detection of electrical stimulation and dynamic ranges were tested for a wide range of frequencies of sinusoidal stimulation and for biphasic rectangular pulses of various durations and repetition rates. The results are comparable with available data from implanted human patients and extend these findings, exploring various aspects of electrical stimulation in greater detail.

Acoustic Stimulation