OBJECTIVE: This study aimed to explore the electrophysiological characteristics of patients with chronic tinnitus through electrocochleography (ECochG) findings and determine if these findings correlate with specific audiological patterns that could differentiate tinnitus patients from those without this condition.
METHODS: A retrospective analysis of medical records from patients who visited a tinnitus clinic at a tertiary university hospital between March 2020 and December 2023 was conducted. Inclusion criteria were non-pulsatile subjective tinnitus lasting over three months, and ECochG performed at initial evaluation. Audiological assessments and ECochG results were analyzed, with the SP/AP ratio being a focal point.
RESULTS: Among 256 patients, an elevated SP/AP ratio was observed in 37.5 % of patients. No significant difference in ECochG outcomes was noted based on tinnitus laterality. Patients with an elevated SP/AP ratio reported more sleep disturbances, higher depression scores, attention problems, and aural fullness. These patients also exhibited lower loudness discomfort levels and low-frequency hearing losses. Significant correlations were found between elevated SP/AP ratios and DPOAE responses.
CONCLUSIONS: The findings highlight the SP/AP ratio in ECochG as a valuable biomarker for assessing clinical and psychological aspects of tinnitus, indicating its potential utility in tailoring treatment strategies. Elevated SP/AP ratios were associated with sleep disturbances, depression, attention problems, aural fullness, hyperacusis, and low-frequency hearing loss, suggesting a complex interplay between cochlear pathology and tinnitus perception. This study underscores the need for a nuanced understanding of ECochG results in the clinical evaluation of tinnitus, potentially guiding more personalized management approaches.

OBJECTIVE: Cochlear nerve deficiency (CND) is a common radiologic finding among unilateral sensorineural hearing loss (USNHL) patients. It is generally detected with magnetic resonance imaging (MRI), which is associated with higher cost, less availability, and possible need for sedation. Therefore, identifying computed tomography (CT) findings, such as cochlear aperture stenosis (CAS), that can reliably predict CND is valuable. Our study aimed to determine the prevalence of CND in pediatric patients with CT-diagnosed CAS.
METHODS: Retrospective study.
METHODS: Tertiary care center.
METHODS: We included pediatric patients diagnosed with CAS on temporal bone CT and with available temporal bone MRI. For each patient, an otolaryngologist and a pediatric neuroradiologist measured the cochlear aperture width on CT to confirm CAS (cochlear aperture < 1.4 mm) and assessed the status of the cochlear nerve on MRI.
RESULTS: Fifty-five patients, representing 65 ears, had CAS on CT measurement. Median cochlear aperture width in CAS ears was 0.70 mm (interquartile range [IQR]: 0.40-1.05 mm) versus 2.00 mm in non-CAS ears (IQR: 1.80-2.30 mm, P < .001). CND was found in 98.5% (n = 64/65) of CAS ears, while a normal cochlear nerve was found in 1.5% (n = 1/65) of CAS ears.
CONCLUSIONS: CND is highly prevalent among pediatric patients with CAS. This suggests that MRI may not be needed to assess for CND in USNHL patients with CAS, as initial CT may provide sufficient information to determine cochlear implant candidacy. We recommend thoughtful shared decision-making with parents of USNHL patients when determining whether to pursue MRI in the setting of a CAS diagnosis.

UNASSIGNED: Systemic sclerosis (SSc) is a disease of a very heterogeneous clinical picture and immunological profile with progression rate that varies between individuals. Although hearing deterioration is not a complaint that comes to the fore in SSc patients, as it is not life-threatening compared to many other more severe symptoms of this disease, it can significantly impair the quality of life. Medical literature concerning this problem is rather scarce.
UNASSIGNED: In this article we systematically reviewed the medical publications concerning hearing impairment in patients with systemic sclerosis to evaluate current understanding of this complex problem. Following PRISMA guidelines a total of 19 papers were found and analysed including 11 original studies and 8 case reports.
UNASSIGNED: Although it seems that hearing impairment in SSc patients is relatively more common than in the general population, based on the analysis of available literature, no firm conclusions regarding its frequency and pathomechanism can be drawn yet. Microangiopathy leading to damage to the sensory cells of the inner ear is suspected to be the main mechanism of hearing loss, although damage to the higher levels of the auditory pathway appears to be underestimated due to incomplete audiological diagnosis.
UNASSIGNED: Undoubtedly, the reason for the difficulty in such an evaluation are the complex and still not fully elucidated pathomechanism of SSc, the individually variable dynamics of the disease and the unique heterogeneity of symptoms. Nevertheless, further studies in larger and appropriately selected groups of patients, focused more on the dynamics of microangiopathy and not solely on clinical symptoms could provide answers to many key questions in this regard.

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UNASSIGNED: To describe audiological symptoms, audiometric profile, and distortion product otoacoustic emission in symptomatic patients recovering from SARS-CoV-2 infection (positive RT-PCR test) and asymptomatic patients (negative RT-PCR test).
UNASSIGNED: An analytical cross-sectional study was conducted using data obtained from clinical charts, physical examination, audiometry, and distortion product otoacoustic emission on 40 patients [case patients (CP)] recovering from SARS-CoV-2 infection diagnosed by a positive RT-PCR test and 22 asymptomatic participants with a negative RT-PCR test [non-case (NC)].
UNASSIGNED: Sixty-two patients (mean age: 31.1 and 28.2 years in the CP and NC groups, respectively) were included. All participants were young without significant comorbidities, risk factors for hearing loss or otological history. Vertigo (5%), tinnitus (17.5%) and aural fullness/hearing loss (35%) were found in the CP group. A statistically significant difference was found in specific frequencies (1000, 4000, and 8000 Hz) and pure tone average (low and high conversational frequencies with increased threshold in the PC group compared with the NC group), which was not found in distortion product otoacoustic emission.
UNASSIGNED: Audiovestibular symptoms are frequent in symptomatic patients recovering from SARS-CoV-2 infection. SARS-CoV-2 infection was consistently associated with an increased audiometric hearing threshold at specific frequencies and low tone average.

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Cochlear synaptopathy is the noise-induced or age-related loss of ribbon synapses between inner hair cells (IHCs) and auditory-nerve fibers (ANFs), first reported in CBA/CaJ mice. Recordings from single ANFs in anesthetized, noise-exposed guinea pigs suggested that neurons with low spontaneous rates (SRs) and high thresholds are more vulnerable than low-threshold, high-SR fibers. However, there is extensive postexposure regeneration of ANFs in guinea pigs but not in mice. Here, we exposed CBA/CaJ mice to octave-band noise and recorded sound-evoked and spontaneous activity from single ANFs at least 2 wk later. Confocal analysis of cochleae immunostained for pre- and postsynaptic markers confirmed the expected loss of 40%-50% of ANF synapses in the basal half of the cochlea; however, our data were not consistent with a selective loss of low-SR fibers. Rather they suggested a loss of both SR groups in synaptopathic regions. Single-fiber thresholds and frequency tuning recovered to pre-exposure levels; however, response to tone bursts showed increased peak and steady-state firing rates, as well as decreased jitter in first-spike latencies. This apparent gain-of-function increased the robustness of tone-burst responses in the presence of continuous masking noise. This study suggests that the nature of noise-induced synaptic damage varies between different species and that, in mouse, the noise-induced hyperexcitability seen in central auditory circuits is also observed at the level of the auditory nerve.NEW & NOTEWORTHY Noise-induced damage to synapses between inner hair cells and auditory-nerve fibers (ANFs) can occur without permanent hair cell damage, resulting in pathophysiology that \"hides\" behind normal thresholds. Prior single-fiber neurophysiology in guinea pig suggested that noise selectively targets high-threshold ANFs. Here, we show that the lingering pathophysiology differs in mouse, with both ANF groups affected and a paradoxical gain-of-function in surviving low-threshold fibers, including increased onset rate, decreased onset jitter, and reduced maskability.

Despite the low overall prevalence of individual rare diseases, cochlear dysfunction leading to hearing loss represents a symptom in a large proportion. The aim of this work was to provide a clear overview of rare cochlear diseases, taking into account the embryonic development of the cochlea and the systematic presentation of the different disorders. Although rapid biotechnological and bioinformatic advances may facilitate the diagnosis of a rare disease, an interdisciplinary exchange is often required to raise the suspicion of a rare disease. It is important to recognize that the phenotype of rare inner ear diseases can vary greatly not only in non-syndromic but also in syndromic hearing disorders. Finally, it becomes clear that the phenotype of the individual rare diseases cannot be determined exclusively by classical genetics even in monogenetic disorders.
Auch wenn die einzelnen Krankheitsbilder selten sind, stellen seltene Erkrankungen der Cochlea in ihrer Gänze eine doch gehäufte Entität dar, die zu Hörstörungen führt. Ein/Das Ziel des vorliegenden Referates war es, unter Berücksichtigung der Embryonalentwicklung der Hörschnecke und einer systematischen Zusammenfassung eine übersichtliche Darstellung der seltenen cochleären Erkrankungen zu ermöglichen. Auch wenn rapide biotechnologische und bioinformatische Fortschritte die Diagnose einer seltenen Erkrankung erleichtern, so kann oft nur im interdisziplinären Austausch der Verdacht einer seltenen Erkrankung erhoben werden. Trotz gleicher zugrunde liegender Mutationen kann der Phänotyp nicht nur bei den genetisch bedingten Hörstörungen sondern auch bei den syndromalen Erkrankungen stark variieren. Schließlich wird deutlich, dass der Phänotyp der einzelnen seltenen Erkrankungen nicht ausschließlich durch die klassische Genetik bestimmt werden kann.

Umbilical cord-derived mesenchymal stromal cells (UCMSCs) have potential applications in regenerative medicine. UCMSCs have been demonstrated to repair tissue damage in many inflammatory and degenerative diseases. We have previously shown that UCMSC exosomes reduce nerve injury-induced pain in rats. In this study, we characterized UCMSC exosomes using RNA sequencing and proteomic analyses and investigated their protective effects on cisplatin-induced hearing loss in mice. Two independent experiments were designed to investigate the protective effects on cisplatin-induced hearing loss in mice: (i) chronic intraperitoneal cisplatin administration (4 mg/kg) once per day for 5 consecutive days and intraperitoneal UCMSC exosome (1.2 μg/μL) injection at the same time point; and (ii) UCMSC exosome (1.2 μg/μL) injection through a round window niche 3 days after chronic cisplatin administration. Our data suggest that UCMSC exosomes exert protective effects in vivo. The post-traumatic administration of UCMSC exosomes significantly improved hearing loss and rescued the loss of cochlear hair cells in mice receiving chronic cisplatin injection. Neuropathological gene panel analyses further revealed the UCMSC exosomes treatment led to beneficial changes in the expression levels of many genes in the cochlear tissues of cisplatin-injected mice. In conclusion, UCMSC exosomes exerted protective effects in treating ototoxicity-induced hearing loss by promoting tissue remodeling and repair.