Cochlear Function Research Articles

Considering gene therapy to protect from X-linked deafness DFNX2 and associated neurodevelopmental disorders.

Mutations and deletions in the gene or upstream of the gene encoding the POU3F4 transcription factor cause X-linked progressive deafness DFNX2 and additional neurodevelopmental disorders in humans. Hearing loss can be purely sensorineural or mixed, that is, with both conductive and sensorineural components. Affected males show anatomical abnormalities of the inner ear, which are jointly defined as incomplete partition type III. Current approaches to improve hearing and speech skills of DFNX2 patients do not seem to be fully effective. Owing to inner ear malformations, cochlear implantation is surgically difficult and may predispose towards severe complications. Even in cases where implantation is safely performed, hearing and speech outcomes remain highly variable among patients. Mouse models for DFNX2 deafness revealed that sensorineural loss could arise from a dysfunction of spiral ligament fibrocytes in the lateral wall of the cochlea, which leads to reduced endocochlear potential. Highly positive endocochlear potential is critical for sensory hair cell mechanotransduction and hearing. In this context, here, we propose to develop a therapeutic approach in male Pou3f4 -/y mice based on an adeno-associated viral (AAV) vector-mediated gene transfer in cochlear spiral ligament fibrocytes. Among a broad range of AAV vectors, AAV7 was found to show a strong tropism for the spiral ligament. Thus, we suggest that an AAV7-mediated delivery of Pou3f4 complementary DNA in the spiral ligament of Pou3f4 -/y mice could represent an attractive strategy to prevent fibrocyte degeneration and to restore normal cochlear functions and properties, including a positive endocochlear potential, before hearing loss progresses to profound deafness.

Cochlear Neurotrophin-3 overexpression at mid-life prevents age-related inner hair cell synaptopathy and slows age-related hearing loss.

Age‐related hearing loss (ARHL) is the most prevalent sensory deficit in the elderly. This progressive pathology often has psychological and medical comorbidities, including social isolation, depression, and cognitive decline. Despite ARHL's enormous societal and economic impact, no therapies to prevent or slow its progression exist. Loss of synapses between inner hair cells (IHCs) and spiral ganglion neurons (SGNs), a.k.a. IHC synaptopathy, is an early event in cochlear aging, preceding neuronal and hair cell loss. To determine if age‐related IHC synaptopathy can be prevented, and if this impacts the time‐course of ARHL, we tested the effects of cochlear overexpression of neurotrophin‐3 (Ntf3) starting at middle age. We chose Ntf3 because this neurotrophin regulates the formation of IHC‐SGN synapses in the neonatal period. We now show that triggering Ntf3 overexpression by IHC supporting cells starting in middle age rapidly increases the amplitude of sound‐evoked neural potentials compared with age‐matched controls, indicating that Ntf3 produces a positive effect on cochlear function when the pathology is minimal. Furthermore, near the end of their lifespan, Ntf3‐overexpressing mice have milder ARHL, with larger sound‐evoked potentials along the ascending auditory pathway and reduced IHC synaptopathy compared with age‐matched controls. Our results also provide evidence that an age‐related decrease in cochlear Ntf3 expression contributes to ARHL and that Ntf3 supplementation could serve as a therapeutic for this prevalent disorder. Furthermore, these findings suggest that factors that regulate synaptogenesis during development could prevent age‐related synaptopathy in the brain, a process involved in several central nervous system degenerative disorders.

Endoscopic diving technique for hearing preservation in managing labyrinth-invading cholesteatomas.

Complete removal of the matrix over the existing fistula in the event of an extensive cholesteatoma with labyrinth invasion can result in total deafness. This study aimed to present a novel method of underwater endoscopic labyrinth dissection using continuous steroid irrigation (CSI) and systemic steroid administration for hearing preservation. The endoscopic diving technique (EDT) was used to dissect semicircular canals, revealing the underwater anatomy of membranous labyrinth structures, in two cadaver temporal bones. EDT with CSI was used in three clinical cases with extensive cholesteatoma. On cadaver temporal bones, the anatomy of the lateral (LSC), superior (SSC), and posterior membranous semicircular canals with their respective ampullas and common crus was documented. In the first case managed with transcanal EDT, the LSC was eroded across almost its entire length. The fallopian canal was circumferentially eroded at the second genu and part of the mastoid segment. The cholesteatoma matrix was completely removed, and the membranous LSC was preserved. In the second and third cases, we were able to remove the cholesteatoma matrix along the eroded bony semicircular canals while keeping the membranes intact. No sensorineural hearing loss was detected in the postoperative masked pure-tone audiometry at a mean follow-up time of 12months or cholesteatoma recurrence at the follow-up imaging. EDT with CSI can be safely utilized in the course of temporal bone labyrinth dissections and provides advantages during cholesteatoma removal over the eroded labyrinth on preservation of the membranous structures, and thus may help preserve cochlear function.

Subclinical Hearing Deficits in Noise-Exposed Firefighters.

Noise-induced hearing loss (NIHL) is the most prevalent occupational disease in the world and firefighters are at increased risk of NIHL due to their frequent exposure to hazardous levels of noise during service. Adverse effects of NIHL include acceleration of age-related hearing loss and an increased risk of cognitive decline. A critical challenge in addressing NIHL is the delayed clinical presentation of symptoms and lack of sensitive tools for early detection. To study the early clinical symptoms of NIHL in this high-risk group, we collected hearing function data including behavioral audiometric thresholds and distortion product otoacoustic emissions (DPOAEs) in 176 firefighters during annual physical assessments. Results revealed significant deficits in cochlear outer hair cell function in the presence of normal audiograms. Additionally, 55% of firefighters self-reported changes in hearing, while 20% self-reported concerns about their balance. This study is the first to characterize DPOAEs in firefighters who display decreased DPOAE amplitudes with increasing years in the fire service. These effects were observed even when controlling for hearing loss and age and are suggestive of a link between hearing loss and occupational exposure to hazardous noise.

Changes in vestibular and cochlear function following platinum-based chemotherapy: A preliminary report.

This study investigated the changes in vestibular and cochlear function in patients receiving platinum-based chemotherapy. A longitudinal study of 32 participants (10-70years) receiving chemotherapy participated in the study. Baseline and exit vestibular and hearing assessments that included video head impulse (VHIT) testing, cervical and ocular vestibular evoked myogenic potentials (VEMP), dynamic visual acuity (DVA) and pure-tone audiometry were performed at the patient's treatment venue. Half (50%) of the participants showed cochleotoxicity from baseline to exit testing, with left ears significantly more affected than right ears. There was no consistent relationship between hearing loss and vestibular dysfunction. DVA yielded normal results at baseline and exit testing in all participants. VEMP responses were absent in 28.1% of participants at baseline, reflecting the challenges of using VEMP for monitoring. VEMP and VHIT results showed a statistically significant (p < 0.05) decline in results from baseline to exit testing; however, participants did not report symptoms related to vestibular dysfunction. VHIT also showed left ears significantly (p < 0.05) more affected than right ears. VHIT proved to be a valuable measure of changes in vestibular function secondary to ototoxicity. Future investigations should determine vestibulotoxicity criteria and optimal protocols for vestibulotoxicity monitoring at the patient's treatment venue.

Prevalence of Endolymphatic Hydrops in Cochlear Implant Candidates with Idiopathic Profound Sensorineural Hearing Loss.

To determine the prevalence of endolymphatic hydrops (EH) in cochlear implant (CI) candidates with idiopathic profound sensorineural hearing loss (SNHL) and its influence on the preservation of audiovestibular function after cochlear implantation. Prospective case series. Tertiary referral center. CI candidates with idiopathic progressive SNHL, but without classic EH-associated symptoms. Delayed intravenous gadolinium-enhanced inner ear fluid-attenuated inversion recovery magnetic resonance imaging as well as pure-tone audiograms, video head impulse tests, and vestibular evoked myogenic potentials before and 4 weeks after cochlear implantation. Prevalence of EH before cochlear implantation, audiovestibular function before and after surgery in hydropic and nonhydropic ears. Thirty-two ears in 16 CI candidates were included. Nine ears (28%) with EH were detected. Although preoperative hearing thresholds, utricular function, and semicircular canal function were not different between the two groups, saccular function was reduced in hydropic ears. Ten subjects received a unilateral CI. Of these, 3 (30%) showed EH on the implanted side. There was no difference regarding postoperative hearing loss between the two groups, but the results point toward a higher vulnerability of hydropic ears with respect to loss of otolith function after cochlear implantation. This is the first study showing that EH can be assumed in about one third of CI candidates with idiopathic profound SNHL, but no classic EH-associated symptoms. Preliminary results suggest that EH has no influence on the preservation of cochlear function but could be a risk factor for loss of otolith function after cochlear implantation.

Otoacoustic emission and its application in anesthesia.

An otoacoustic emission (OAE) is a low-level sound emitted by the cochlea. OAEs are able to objectively evaluate the auditory perception and reflect the functional status of the auditory system. With the characteristics of non-invasiveness, high reliability, and easy manipulation, OAEs have gained wide popularity in clinical audiology and anesthesiology. This review aims to summarize the application of OAE in anesthesia. This study collected data from the databases Web of Science-Clarivate Analytics, PubMed, and Google Scholar in English, covering research in the last 40 years. The keywords were defined as anesthesia, cochlea, OAEs, distortion product otoacoustic emissions, transient evoked otoacoustic emissions, bispectral index, auditory evoked potentials, and depth of anesthesia. Documents that matched defined keywords were selected and reviewed. Research showed that the types and doses of anesthetic drugs impacted OAEs. Ketamine-based anesthesia has a greater effect on OAE sensitivity over time compared to isoflurane. A higher dose of ketamine-xylazine significantly reduced the amplitude of OAEs. According to those characteristics, OAEs could be used as an objective evaluation method for the effect of anesthetics and have great potential to be applied for anesthetic drug dose control during surgery. OAEs also have been used to detect the cochlear function during anesthesia, which may cause irreversible damage to the cochlea. Studies reported that OAEs have been used in anesthesia. However, the existing studies have mainly focused on the influence of anesthetic types or dosages on OAEs. Considering the characteristics of OAEs, such as a convenient measurement, less susceptibility to interference, and fast detection speed, the application of OAE has a great potential in the anesthesia field.

Cochlear ribbon synapse maturation requires Nlgn1 and Nlgn3

SummaryHearing depends on precise synaptic transmission between cochlear inner hair cells and spiral ganglion neurons through afferent ribbon synapses.Neuroligins (Nlgns) facilitate synapse maturation in the brain, but they have gone unstudied in the cochlea. We report Nlgn3 and Nlgn1 knockout (KO) cochleae have fewer ribbon synapses and have impaired hearing. Nlgn3 KO is more vulnerable to noise trauma with limited activity at high frequencies one day after noise. Furthermore, Nlgn3 KO cochleae have a 5-fold reduction in synapse number compared to wild type after two weeks of recovery. Double KO cochlear phenotypes are more prominent than the KOs, for example, 5-fold smaller synapses, 25% reduction in synapse density, and 30% less synaptic output. These observations indicate Nlgn3 and Nlgn1 are essential to cochlear ribbon synapse maturation and function.

Promontory Electrocochleography Recordings to Predict Speech-Perception Performance in Cochlear Implant Recipients.

1) To determine the relationship of electrocochleography (ECochG) responses measured on the promontory with responses measured at the round window (RW) and various intracochlear sites. 2) To evaluate if promontory ECochG responses correlate with postoperative speech-perception performance using the cochlear implant (CI). Prospective cohort study. Tertiary referral center. Ninety-six adult CI recipients with no cochlear malformations or previous otologic surgery. Acoustically evoked ECochG responses were measured intraoperatively at both extracochlear and intracochlear locations. ECochG total response (ECochG-TR), a measure of residual cochlear function, was calculated by summing the fast Fourier transformation amplitudes in response to 250-Hz to 2-kHz acoustic stimuli. Speech-perception performance was measured at 3 months. There were strong linear correlations for promontory ECochG-TR with the ECochG-TRs measured at the RW ( r = 0.95), just inside scala tympani ( r = 0.91), and after full insertion ( r = 0.83). For an individual subject, the morphology of the ECochG response was similar in character across all positions; however, the response amplitude increased from promontory to RW (~1.6-fold) to just inside scala tympani (~2.6-fold), with the largest response at full insertion (~13.1-fold). Promontory ECochG-TR independently explained 51.8% of the variability ( r2 ) in consonant-nucleus-consonant at 3 months. Promontory ECochG recordings are strongly correlated with responses previously recorded at extracochlear and intracochlear sites and explain a substantial portion of the variability in CI performance. These findings are a critical step in supporting translation of transtympanic ECochG into the clinic preoperatively to help predict postoperative CI performance.

Is there an unmet medical need for improved hearing restoration?

Hearing impairment, the most prevalent sensory deficit, affects more than 466 million people worldwide (WHO). We presently lack causative treatment for the most common form, sensorineural hearing impairment; hearing aids and cochlear implants (CI) remain the only means of hearing restoration. We engaged with CI users to learn about their expectations and their willingness to collaborate with health care professionals on establishing novel therapies. We summarize upcoming CI innovations, gene therapies, and regenerative approaches and evaluate the chances for clinical translation of these novel strategies. We conclude that there remains an unmet medical need for improving hearing restoration and that we are likely to witness the clinical translation of gene therapy and major CI innovations within this decade.

Neural processing and perception of Schroeder-phase harmonic tone complexes in the gerbil: Relating single-unit neurophysiology to behavior.

Schroeder-phase harmonic tone complexes have been used in physiological and psychophysical studies in several species to gain insight into cochlear function. Each pitch period of the Schroeder stimulus contains a linear frequency sweep; the duty cycle, sweep velocity, and direction are controlled by parameters of the phase spectrum. Here, responses to a range of Schroeder-phase harmonic tone complexes were studied both behaviorally and in neural recordings from the auditory nerve and inferior colliculus of Mongolian gerbils. Gerbils were able to discriminate Schroeder-phase harmonic tone complexes based on sweep direction, duty cycle, and/or velocity for fundamental frequencies up to 200 Hz. Temporal representation in neural responses based on the van Rossum spike-distance metric, with time constants of either 1ms or related to the stimulus' period, was compared with average discharge rates. Neural responses and behavioral performance were both expressed in terms of sensitivity, d', to allow direct comparisons. Our results suggest that in the auditory nerve, stimulus fine structure is represented by spike timing, whereas envelope is represented by rate. In the inferior colliculus, both temporal fine structure and envelope appear to be represented best by rate. However, correlations between neural d' values and behavioral sensitivity for sweep direction were strongest for both temporal metrics, for both auditory nerve and inferior colliculus. Furthermore, the high sensitivity observed in the inferior colliculus neural rate-based discrimination suggests that these neurons integrate across multiple inputs arising from the auditory periphery.

Single-cell RNA-sequencing of zebrafish hair cells reveals novel genes potentially involved in hearing loss.

Hair cells play key roles in hearing and balance, and hair cell loss would result in hearing loss or vestibular dysfunction. Cellular and molecular research in hair cell biology provides us a better understanding of hearing and deafness. Zebrafish, owing to their hair cell-enriched organs, have been widely applied in hair cell-related research worldwide. Similar to mammals, zebrafish have inner ear hair cells. In addition, they also have lateral line neuromast hair cells. These different types of hair cells vary in morphology and function. However, systematic analysis of their molecular characteristics remains lacking. In this study, we analyzed the GFP+ cells isolated from Tg(Brn3c:mGFP) larvae with GFP expression in all hair cells using single-cell RNA-sequencing (scRNA-seq). Three subtypes of hair cells, namely macula hair cell (MHC), crista hair cell (CHC), and neuromast hair cell (NHC), were characterized and validated by whole-mount in situ hybridization analysis of marker genes. The hair cell scRNA-seq data revealed hair cell-specific genes, including hearing loss genes that have been identified in humans and novel genes potentially involved in hair cell formation and function. Two novel genes were discovered to specifically function in NHCs and MHCs, corresponding to their specific expression in NHCs and MHCs. This study allows us to understand the specific genes in hair cell subpopulations of zebrafish, which will shed light on the genetics of both human vestibular and cochlear hair cell function.

Value of Preoperative Imaging Results in Predicting Cochlear Nerve Function in Children Diagnosed With Cochlear Nerve Aplasia Based on Imaging Results.

This study aimed to assess the function of the cochlear nerve using electrically evoked compound action potentials (ECAPs) for children with cochlear implants who were diagnosed with cochlear nerve aplasia and to analyze the correlation between preimplantation imaging results and ECAP responses. Thirty-five children diagnosed with cochlear nerve aplasia based on magnetic resonance imaging (MRI) were included. Preimplantation MRI and high-resolution computed tomography (HRCT) images were reconstructed, and the width of the bone cochlear nerve canal (BCNC), the diameter of the vestibulocochlear nerve (VCN), and the diameter of the facial nerve (FN) were measured. ECAP input/output (I/O) functions were measured at three electrode locations along the electrode array for each participant. The relationship between ECAP responses (including ECAP threshold, ECAP maximum amplitude, and slope of ECAP I/O function) and sizes of the BCNC and VCN was analyzed using Pearson's correlation coefficients. Our analysis revealed that ECAP responses varied greatly among individual participants. Overall, ECAP thresholds gradually increased, while maximum amplitudes and ECAP I/O function slopes gradually decreased, as the electrode location moved from the basal to the apical direction in the cochlea. ECAP responses exhibited no significant correlations with BCNC width or VCN diameter. The ratio of the VCN to FN diameters was significantly correlated with the slope of the ECAP I/O function and the maximum amplitude. BCNC width could not predict the function of the cochlear nerve. Compared with the absolute size of the VCN, the size of the VCN relative to the FN may represent an indicator for predicting the functional status of the cochlear nerve in children diagnosed with cochlear nerve aplasia based on imaging results.

The Induction of Hypothyroidism during Gestation Decreases Outer Hair Cell Motility in Rat Offspring.

Introduction Perinatal hypothyroidism has a negative repercussion on the development and maturation of auditory system function. However, its long-term effect on auditory function remains unsettled. Objective To evaluate the effect of prenatal hypothyroidism on the auditory function of adult offspring in rats. Methods Pregnant Wistar rats were given the antithyroid drug methimazole (0.02% -1-methylimidazole-2-thiol- MMI) in drinking water, ad libitum, from gestational day (GD) 9 to postnatal day 15 (PND15). Anesthetized offspring from MMI-treated dams (OMTD) and control rats were evaluated by tympanometry, distortion product otoacoustic emission (DPOAE), and auditory brainstem response (ABR) at PNDs 30, 60, 90, and 120. Results Our data demonstrated no middle ear dysfunction, with the OMTD compliance lower than that of the control group. The DPOAE revealed the absence of outer hair cells function, and the ABR showed normal integrity of neural auditory pathways up to brainstem level in the central nervous system. Furthermore, in the OMTD group, hearing loss was characterized by a higher electrophysiological threshold. Conclusion Our data suggest that perinatal hypothyroidism leads to irreversible damage to cochlear function in offspring.

Sensorineural Hearing Loss Post-COVID-19 Infection: An Update.

The course of COVID-19 infection may be complicated by a variety of neurological manifestations. Since the inner ear is vulnerable to viruses, sensorineural hearing loss (SNHL) has been reported to occur following the SARS-CoV-2 infection, often resulting in long-term morbidity and worsening the quality of life. The interest in how the virus affects the inner ear has gradually increased since the pandemic’s spread, but little is still known about the SNHL potentially caused by SARS-CoV-2. The aim of this paper is to evaluate the possible association between SNHL and COVID-19 infection, through a systematic literature review. Currently available data suggest that SARS-CoV-2 may hamper cochlear function; however, available reports are still limited. Large cohort and prospective studies are necessary to evaluate the long-term effects of this viral infection in the inner ear.

COVID-19 during pregnancy and its impact on the developing auditory system

BackgroundThis study compared distortion product otoacoustic emissions (DPOAEs) and click-evoked auditory brainstem responses (ABRs) recorded from infants whose mother had Covid-19 during pregnancy (Covid-19 group) to infants whose mother did not have Covid-19 (Control group) during pregnancy. MethodsThis study retrospectively examined records of infants in the Covid-19 group (n = 15) and control group (n = 46) who had distortion product otoacoustic emissions (DPOAEs) and click-evoked auditory brainstem responses (ABRs) recorded as part of their clinical assessment. DPOAE amplitudes, absolute latencies (I, III, and V), and I-V interpeak intervals were examined. ResultsDPOAE amplitudes were similar between the Covid-19 group and the control group. The absolute latency of wave I was similar between groups. But absolute latencies III and V and I-V interpeak intervals of the Covid-19 group were significantly prolonged compared to the control group. ConclusionCovid-19 infection and its complications during pregnancy may not affect the cochlear function but may affect the functioning of the auditory brainstem.

The contribution of fetal-derived tissue resident macrophages to cytomegalovirus-associated sensorineural hearing loss

Abstract Cytomegalovirus (CMV) is the most common congenital viral infection in the developed world and the leading cause of non-genetic sensorineural hearing loss (SNHL) in children. Congenital CMV causes progressive SNHL long after acute infection has resolved, suggesting a lasting effect on the developing host immune response within the cochlea and surrounding temporal bone marrow (TB). Using fate-mapping models, we have investigated the contribution of fetal-derived tissue resident macrophages (TRMs) to normal cochlear development and function, and their role in mediating CMV-associated SNHL. Our analysis defined two distinct myeloid population within the inner ear: 1) classical tissue resident macrophages (F4/80hiCD11blo) that highly express CD64 and are found exclusively in cochlear tissue; and 2) transitional monocytes (F4/80midCD11bhi) that express both CD64 and Ly6C and are found in cochlea and TB. Fate mapping of these populations uncovered dual contribution of fetal-derived cochlear TRMs from yolk sac (YS) and fetal liver (FL) hematopoiesis. Transitional monocytes increase during acute CMV infection, acting as drivers of expanded lymphoid populations after CMV resolves. Importantly, analysis of Flt3-Cre labeling within myeloid populations suggests that TRMs are replaced by BM-derived populations at later postnatal stages following CMV infection, coinciding with SNHL. Ongoing work examines spatial distribution of distinct fetal-derived TRMs along cochlear architecture, defining transcriptional regulation differences between YS-, FL-, and adult-derived TRMs in response to CMV, and examining how replacement of fetal-derived TRMs with adult-derived TRMs affects tissue function and response to infection. Supported by grants from University of Utah Molecular Medicine and Immunology, Inflammation and Infectious Disease (3i) Initiative and NIAID (T32 5T32AI138945)

Electrophysiological study of the cochlea in patients with unilateral acute tinnitus

Objective:To analyze the objective test results of the pure-tone audiogram （PTA）, extended high-frequency audiometry （EHFA）, distortion product otoacoustic emission （DPOAE）, auditory brainstem response（ABR）, and electrocochleogram （ECochG） in patients with unilateral acute tinnitus, summarize their characteristics and explore their clinical application value in hidden hearing loss. Methods:PTA, DPOAE, ABR, and ECochG tests were performed in 33 patients with unilateral acute tinnitus as the chief complaint. The detection rate and response amplitude of each DPOAE frequency, incubation period, and interval of ABR waves and -SP/AP in ECochG were analyzed. Results:①The thresholds of PTA at 0.25-8 kHz in both ears were in the normal range （P>0.05）, and the thresholds of PTA at 9-16 kHz in affected ears were higher than those in healthy ears （P<0.001）; ②There was statistical significance in the detection rate and response amplitude of DPOAE at 3, 4, 6, 8 kHz between ears （P<0.05）; ③The incubation period of ABR Ⅰ wave in affected ears was （1.55±0.17） ms, that in the healthy ear was （1.50±0.14） ms, among them, the incubation period of ABR Ⅰ wave in the affected ear was longer than that in the healthy ear, and the difference was statistically significant （P<0.05）; ④In ECochG, there was no significant difference in -SP amplitude between ears （P>0.05）, but there was a significant difference in AP amplitude and -SP/AP amplitude between ears （P<0.05）. Conclusion:EHFA, DPOAE, ABR, and ECochG have clinical significance in evaluating cochlear function in tinnitus patients.

Hearing Impairment in the Extended High Frequencies in Children Despite Clinically Normal Hearing.

Pediatric hearing impairment, regardless of degree and type, has a detrimental effect on speech perception, cognition, oral language development, academic outcomes, and literacy. Hearing assessment in the clinic is limited to 8 kHz although humans can hear up to 20 kHz. Hearing impairment in the extended high frequencies (EHFs > 8 kHz) can occur despite clinically normal hearing. However, to date, the nature and effects of EHF hearing impairment in children remain unknown. The goals of the present study were to determine the effects of EHF hearing impairment on speech-in-noise recognition in children and to examine whether hearing impairment in the EHFs is associated with altered cochlear functioning in the standard frequencies. A volunteer sample of 542 participants (4 to 19 years) with clinically normal audiograms were tested. Participants identified with EHF impairment were assigned as cases in a subsequent case-control study. EHF loss was defined as hearing thresholds greater than 20 dB in at least one EHFs (10, 12.5, or 16 kHz). Speech recognition thresholds in multi-talker babble were measured using the digit triplet test. Distortion product otoacoustic emissions ( f2 = 2, 3, 4, and 5 kHz) were measured to assess cochlear functioning. Thresholds in the EHFs were as reliable as those in the standard frequency range. Thirty-eight children had EHF hearing impairment regardless of a clinically normal audiogram. A linear mixed-effects model revealed that children with EHF hearing impairment had higher (poorer) mean speech recognition threshold than children with normal EHF sensitivity ( estimate = 2.14 dB, 95% CI: 1.36 to 3.92; effect size = small). The overall magnitude of distortion product otoacoustic emissions was lower for children with EHF impairment ( estimate = -2.47 dB, 95% CI: -4.60 to -0.73; effect size = medium). In addition, the pure-tone average for standard audiometric frequencies was relatively higher for EHF-impaired children ( estimate = 3.68 dB, 95% CI: 2.56 to 4.80; effect size = small). Hearing impairment in the EHFs is common in children despite clinically normal hearing and can occur without a history of otitis media. EHF impairment is associated with poorer speech-in-noise recognition and preclinical cochlear deficits in the lower frequencies where hearing thresholds are normal. This study highlights the clinical need to identify EHF impairments in children.

Multi-Frequency Intraoperative Monitoring of Hearing Preservation during Cochlear Implantation.

(1) Background: Current indications for cochlear implants (CIs) have expanded to include patients with appreciable low-frequency hearing. However, longitudinal results indicate that only one-third of these recipients retain full hearing preservation. In another words, the remaining two-thirds lose this facility either partially or fully. This points to the need to better understand the impact of cochlear implantation on cochlear integrity. Intracochlear electrocochleography (ECochG) involves the recording of electrical potentials generated in the inner ear in response to acoustic stimuli, and previous studies have shown that these potentials give an indication of residual inner ear function. Aim of the research: The aim is to monitor intracochlear ECochG during CI surgery and gain a better understanding of how the implant impacted inner ear function. A newly developed SPL Chirp was used for stimulation. (2) Methods: Intracochlear ECochG signals were measured in a subject with residual preoperative low-frequency hearing, while an electrode array was introduced into the cochlea and was continued until the round window was sealed. Afterwards, surgical events were reviewed with the surgeon; preoperative and postoperative radiological data and hearing thresholds were also evaluated. (3) Conclusions: Real-time intraoperative monitoring, with multifrequency evaluation and video recording, has the potential to allow surgeons and audiologists to continuously assess cochlear function. ECochG monitoring may be a useful tool during cochlear implantation to gain frequency-specific information on the status of the patient’s hearing, assisting surgeons to lower hearing trauma during the operation.