Background: Various representations exist in the literature to visualize electrocochleography (ECochG) recordings along the basilar membrane (BM). This lack of generalization complicates comparisons within and between cochlear implant (CI) users, as well as between publications. This study synthesized the visual representations available in the literature via a systematic review and provides a novel approach to visualize ECochG data in CI users. Methods: A systematic review was conducted within PubMed and EMBASE to evaluate studies investigating ECochG and CI. Figures that visualized ECochG responses were selected and analyzed. A novel visualization of individual ECochG data, the ZH-ECochG Bode plot (ZH = Zurich), was devised, and the recordings from three CI recipients were used to demonstrate and assess the new framework. Results: Within the database search, 74 articles with a total of 115 figures met the inclusion criteria. Analysis revealed various types of representations using different axes; their advantages were incorporated into the novel visualization framework. The ZH-ECochG Bode plot visualizes the amplitude and phase of the ECochG recordings along the different tonotopic regions and angular insertion depths of the recording sites. The graph includes the pre- and postoperative audiograms to enable a comparison of ECochG responses with the audiometric profile, and allows different measurements to be shown in the same graph. Conclusions: The ZH-ECochG Bode plot provides a generalized visual representation of ECochG data, using well-defined axes. This will facilitate the investigation of the complex ECochG potentials generated along the BM and allows for better comparisons of ECochG recordings within and among CI users and publications. The scripts used to construct the ZH-ECochG Bode plot are provided by the authors.

Understanding speech in noise is particularly difficult for individuals occupationally exposed to noise due to a mix of noise-induced auditory lesions and the energetic masking of speech signals. For years, the monitoring of conventional audiometric thresholds has been the usual method to check and preserve auditory function. Recently, suprathreshold deficits, notably, difficulties in understanding speech in noise, has pointed out the need for new monitoring tools. The present study aims to identify the most important variables that predict speech in noise understanding in order to suggest a new method of hearing status monitoring. Physiological (distortion products of otoacoustic emissions, electrocochleography) and behavioral (amplitude and frequency modulation detection thresholds, conventional and extended high-frequency audiometric thresholds) variables were collected in a population of individuals presenting a relatively homogeneous occupational noise exposure. Those variables were used as predictors in a statistical model (random forest) to predict the scores of three different speech-in-noise tests and a self-report of speech-in-noise ability. The extended high-frequency threshold appears to be the best predictor and therefore an interesting candidate for a new way of monitoring noise-exposed professionals.

Electrocochleography (ECochG) represents a promising approach for monitoring cochlear function during cochlear implantation and for investigating the causes of residual cochlear function loss after implantation. This paper provides an overview of the current research and application status of ECochG, both during and after cochlear implantation. Intraoperative ECochG can be conducted either via the implant itself or an extracochlear measuring electrode. Postoperative ECochG recordings are also feasible via the implant. Various studies have demonstrated that a significant decrease in ECochG amplitude during electrode insertion correlates with an increased risk of losing residual cochlear function, with critical cochlear events occurring primarily towards the end of the insertion. Postoperative data suggest that the loss of cochlear function mainly occurs in the early postoperative phase. Future research directions include the automation and objectification of signal analysis, as well as a more in-depth investigation into the underlying mechanisms of these signal changes.
UNASSIGNED: Die Elektrocochleographie (ECochG) bietet eine aussichtsreiche Möglichkeit zur Überwachung der cochleären Funktion während der Cochleaimplantation und zur Erforschung der Ursachen des Verlusts cochleärer Restfunktion nach der Implantation. Die vorliegende Arbeit gibt einen Überblick über den aktuellen Forschungs- und Anwendungsstand der ECochG, sowohl während als auch nach der Cochleaimplantation. Die intraoperative ECochG kann entweder durch das Implantat selbst oder mittels einer extracochleären Messelektrode durchgeführt werden. Postoperative ECochG-Aufnahmen sind über das Implantat möglich. Verschiedene Studien haben gezeigt, dass ein signifikanter Abfall der ECochG-Amplitude während der Elektrodeninsertion mit einem erhöhten Risiko für den Verlust der cochleären Restfunktion korreliert, wobei bedeutsame cochleäre Ereignisse vornehmlich gegen Ende der Insertion auftreten. Postoperative Daten deuten darauf hin, dass der Verlust der cochleären Funktion hauptsächlich in der frühen postoperativen Phase erfolgt. Zukünftige Forschungsansätze umfassen die Automatisierung und Objektivierung der Signalauswertung sowie eine vertiefte Untersuchung der den Signaländerungen zugrunde liegenden Mechanismen.

To preserve residual hearing during cochlear implant (CI) surgery it is desirable to use intraoperative monitoring of inner ear function (cochlear monitoring). A promising method is electrocochleography (ECochG). Within this project the relations between intracochlear ECochG recordings, position of the recording contact in the cochlea with respect to anatomy and frequency and preservation of residual hearing were investigated. The aim was to better understand the changes in ECochG signals and whether these are due to the electrode position in the cochlea or to trauma generated during insertion. During and after insertion of hearing preservation electrodes, intraoperative ECochG recordings were performed using the CI electrode (MED-EL). During insertion, the recordings were performed at discrete insertion steps on electrode contact 1. After insertion as well as postoperatively the recordings were performed at different electrode contacts. The electrode location in the cochlea during insertion was estimated by mathematical models using preoperative clinical imaging, the postoperative location was measured using postoperative clinical imaging. The recordings were analyzed from six adult CI recipients. In the four patients with good residual hearing in the low frequencies the signal amplitude rose with largest amplitudes being recorded closest to the generators of the stimulation frequency, while in both cases with severe pantonal hearing losses the amplitude initially rose and then dropped. This might be due to various reasons as discussed in the following. Our results indicate that this approach can provide valuable information for the interpretation of intracochlearly recorded ECochG signals.

In recent years, tools for early detection of irreversible trauma to the basilar membrane during hearing preservation cochlear implant (CI) surgery were established in several clinics. A link with the degree of postoperative hearing preservation in patients was investigated, but patient populations were usually small. Therefore, this study\'s aim was to analyze data from intraoperative extracochlear electrocochleography (ECochG) recordings for a larger group.During hearing preservation CI surgery, extracochlear recordings were made before, during, and after CI electrode insertion using a cotton wick electrode placed at the promontory. Before and after insertion, amplitudes and stimulus response thresholds were recorded at 250, 500, and 1000 Hz. During insertion, response amplitudes were recorded at one frequency and one stimulus level. Data from 121 patient ears were analyzed.The key benefit of extracochlear recordings is that they can be performed before, during, and after CI electrode insertion. However, extracochlear ECochG threshold changes before and after CI insertion were relatively small and did not independently correlate well with hearing preservation, although at 250 Hz they added some significant information. Some tendencies-although no significant relationships-were detected between amplitude behavior and hearing preservation. Rising amplitudes seem favorable and falling amplitudes disadvantageous, but constant amplitudes do not appear to allow stringent predictions.Extracochlear ECochG measurements seem to only partially realize expected benefits. The questions now are: do gains justify the effort, and do other procedures or possible combinations lead to greater benefits for patients?

BACKGROUND: The diagnosis of third window syndromes often poses a challenge in clinical practice.
OBJECTIVE: This paper provides an up-to-date overview of diagnostic procedures in third window syndromes, with special emphasis on superior canal dehiscence syndrome (SCDS), large vestibular aqueduct syndrome (LVAS), and X-chromosomal malformation of the cochlea.
METHODS: A literature search was performed in PubMed up to December 2023. Furthermore, a selection of the authors\' own cases is presented.
RESULTS: Audiovestibular tests for the diagnosis of third window syndromes are most often reported for patients with SCDS in the literature. In this context, cut-off values with different sensitivities and specificities have been defined for different outcome parameters of vestibular evoked myogenic potentials. Current developments include the application of electrocochleography, broadband tympanometry, video head impulse testing, and vibration-induced nystagmus. Genetic analyses are increasingly applied in LVAS.
CONCLUSIONS: The diagnosis of third window syndromes is always based on the synthesis of patients\' symptoms, clinical signs, audiovestibular test results, and imaging.
UNASSIGNED: HINTERGRUND: Die Diagnostik von Drittfenstersyndromen stellt in der klinischen Praxis häufig eine Herausforderung dar.
UNASSIGNED: Die vorliegende Arbeit gibt einen aktuellen Überblick über diagnostische Optionen bei diesen Krankheitsbildern, mit besonderem Fokus auf das Syndrom der oberen Bogengangsdehiszenz (SCDS), das Syndrom des erweiterten vestibulären Aquädukts (LVAS) und die X‑chromosomale Malformation der Cochlea.
METHODS: Dazu erfolgte eine Literaturrecherche in der Datenbank PubMed bis Dezember 2023 und die Aufarbeitung eigener Fälle.
UNASSIGNED: Audiovestibuläre Testverfahren zur Diagnose eines Drittfenstersyndroms werden in der Literatur am häufigsten im Rahmen des SCDS beschrieben. Für vestibulär evozierte myogene Potenziale wurden hier Grenzwerte mit unterschiedlichen Sensitivitäten/Spezifitäten für verschiedene Messparameter definiert. Neuere Entwicklungen umfassen die Anwendung der Elektrocochleographie, der Breitbandtympanometrie, des Video-Kopfimpulstests und des vibrationsinduzierten Nystagmus. Beim LVAS kommen zunehmend genetische Analysen zum Einsatz.
UNASSIGNED: Die Diagnose eines Drittfenstersyndroms ergibt sich immer aus der Synthese von Symptomen, klinischen Zeichen, apparativen Untersuchungsbefunden und der Bildgebung.

We herein review the use of electrocochleography (ECoG) to assess peripheral auditory system responsiveness in a growing population of cochlear implant (CI) users with preserved hearing in ears with implants. Twenty-eight recently published intracochlear ECoG articles were thoroughly reviewed to investigate the prognostic utility of intraoperative ECoG monitoring to assess hearing preservation, and the clinical applicability of postoperative ECoG for estimating audiometric thresholds and monitoring longitudinal changes in residual acoustic hearing in patients with EAS. Intraoperative ECoG studies have focused on monitoring the changes in the cochlear microphonics (CM) amplitudes during and after electrode insertion. Mixed results have been reported regarding the relationship between changes in CM amplitude in the operating room and changes in hearing thresholds after surgery. Postoperative ECoG studies have shown that CM and auditory nerve neurophonics thresholds correlate significantly with behavioral thresholds. ECoG thresholds sensitively detect changes as residual acoustic hearing decreases over time in some CI users. This indicates its potential clinical value for monitoring the post-implantation status of the peripheral auditory system. Intracochlear ECoG can provide real-time intraoperative feedback and monitor postoperative hearing preservation in a growing population of CI users.

Auditory nerve (AN) fibers that innervate inner hair cells in the cochlea degenerate with advancing age. It has been proposed that age-related reductions in brainstem frequency-following responses (FFR) to the carrier of low-frequency, high-intensity pure tones may partially reflect this neural loss in the cochlea (Märcher-Rørsted et al., 2022). If the loss of AN fibers is the primary factor contributing to age-related changes in the brainstem FFR, then the FFR could serve as an indicator of cochlear neural degeneration. In this study, we employed electrocochleography (ECochG) to investigate the effects of age on frequency-following neurophonic potentials, i.e., neural responses phase-locked to the carrier frequency of the tone stimulus. We compared these findings to the brainstem-generated FFRs obtained simultaneously using the same stimulation. We conducted recordings in young and older individuals with normal hearing. Responses to pure tones (250 ms, 516 and 1086 Hz, 85 dB SPL) and clicks were recorded using both ECochG at the tympanic membrane and traditional scalp electroencephalographic (EEG) recordings of the FFR. Distortion product otoacoustic emissions (DPOAE) were also collected. In the ECochG recordings, sustained AN neurophonic (ANN) responses to tonal stimulation, as well as the click-evoked compound action potential (CAP) of the AN, were significantly reduced in the older listeners compared to young controls, despite normal audiometric thresholds. In the EEG recordings, brainstem FFRs to the same tone stimulation were also diminished in the older participants. Unlike the reduced AN CAP response, the transient-evoked wave-V remained unaffected. These findings could indicate that a decreased number of AN fibers contributes to the response in the older participants. The results suggest that the scalp-recorded FFR, as opposed to the clinical standard wave-V of the auditory brainstem response, may serve as a more reliable indicator of age-related cochlear neural degeneration.

UNASSIGNED: Superior semicircular canal dehiscence (SSCD) is the best-known and most common presentation of so-called \"third window conditions.\" There are a variety of diagnostic measures and tests for this condition in the current literature, including air-bone gap, vestibular-evoked myogenic potentials, and electrocochleography (EcochG). The purpose of this study was to investigate the diagnostic utility of EcochG and its relationship to air-bone gap in a cohort of patients with confirmed SSCD.
UNASSIGNED: We reviewed data from 20 patients (11 female and 9 male subjects, age ranging 21-78 years), with confirmed unilateral or bilateral superior canal dehiscence. In total, 11 patients had unilateral SSCD and 9 patients had bilateral SSCD as determined by high-resolution CT scan. This resulted in the inclusion of twenty-nine ears with superior canal dehiscence and 11 normal ears.
UNASSIGNED: Our results indicated that all confirmed SSCD ears presented with an abnormal EcochG SP/AP value and that there was a statistically significant difference between normal and dehiscent ears. There was no statistically significant relationship between air-bone gap and SP/AP ratio in the ears diagnosed with SSCD nor was there a significant difference between dehiscent and normal ears in terms of air-bone gap at three frequencies.
UNASSIGNED: These results are consistent with previous studies showing the diagnostic utility of EcochG for this condition and the variability of air-bone gap. While an unexpected air-bone gap continues to be a red flag for SSCD, its absence along with the presence of subjective symptoms is a reasonable indicator for further clinical investigation to include EcochG.

Cochlear synaptic loss (termed cochlear synaptopathy) has been suggested to contribute to suprathreshold hearing difficulties. However, its existence and putative effects in humans remain inconclusive, largely due to the heterogeneous methods used across studies to indirectly evaluate the health of cochlear synapses. There is a need to standardize proxies of cochlear synaptopathy to appropriately compare and interpret findings across studies. Early auditory evoked potentials (AEPs), including the compound action potential (AP)/Wave I of the auditory brainstem response are a popular proxy, yet remain variable based on technical considerations. This study evaluated one such consideration-electrode array (i.e., montage)-to optimize the use of early AEP waveforms. In 35 young adults, electrocochleography (ECochG) responses were collected using vertical and horizontal montages. Standard ECochG measures and AP/Wave I and Wave II peak-to-trough amplitudes and latencies were compared between montages. Vertical montage recordings consistently produced significantly larger AP/Wave I peak-to-trough amplitudes compared to horizontal recordings. These findings support the use of a vertical electrode montage for optimal recordings of peripheral cochlear nerve activity. As cochlear synaptopathy continues to be explored in humans, the methods highlighted here should be considered in the development of a standardized assessment.