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HYPOTHESIS Automated processing of postoperative clinical cone-beam CT (CBCT) of cochlear implant (CI) patients can be used to accurately determine electrode contacts and integrated with an atlas-based mapping of cochlear microstructures to calculate modiolar distance, angular insertion distance, and scalar location of electrode contacts. BACKGROUND Hearing outcomes after CI surgery are dependent on electrode placement. CBCT is increasingly used for in-office temporal bone imaging and might be routinely used for pre- and post-surgical evaluation. METHODS Thirty-six matched pairs of pre- and postimplant CBCT scans were obtained. These were registered with an atlas to model cochlear microstructures in each dataset. Electrode contact center points were automatically determined using thresholding and electrode insertion parameters were calculated. Automated localization and calculation were compared with manual segmentation of contact center points as well as manufacturer specifications. RESULTS Automated electrode contact detection aligned with manufacturer specifications of spacing and our algorithms worked for both distantly- and closely spaced arrays. The average difference between the manual and the automated selection was 0.15 mm, corresponding to a 1.875 voxel difference in each plane at the scan resolution. For each case, we determined modiolar distance, angular insertion depth, and scalar location. These calculations also resulted in similar insertion values using manual and automated contact points as well as aligning with electrode properties. CONCLUSION Automated processing of implanted high-resolution CBCT images can provide the clinician with key information on electrode placement. This is one step toward routine use of clinical CBCT after CI surgery to inform and guide postoperative treatment.

OBJECTIVES. To compare intraoperative intracochlear electrocochleography (ECochG) with hearing preservation outcomes in cochlear implant (CI) subjects. DESIGN. Intraoperative electrocochleography was performed in adult CI subjects who were recipients of Advanced Bionics’ Bionics LLC precurved HiFocus(™) MidScala or straight HiFocus(™) SlimJ electrode arrays. ECochG responses were recorded from the most apical electrode contact during insertion. No changes to the insertions were made due to ECochG monitoring. No information about insertion resistance was collected. ECochG drops were estimated as the change in amplitude from peak (defined as maximum amplitude response) to drop (largest drop) point after the peak during insertion was measured following the peak response. .Audiometric thresholds from each subject were obtained before and approximately one month after CI surgery. The change in pure tone average (PTA) for frequencies between 125 Hz and 500 Hz was measured after surgery. No post-operative CT scans were collected as part of this study. RESULTS. A total of 68 subjects from 5 surgical centers participated in the study. The study sample included 30 MidScala and 38 SlimJ electrodes implanted by approximately 20 surgeons who contributed to the study. Although a wide range of results were observed, there was a moderate positive correlation (Pearson Correlation coefficient r = 0.56, p < 0.01) between the size of the ECochG drop and the magnitude of PTA change. This trend was present for both the MidScala and SlimJ arrays. The SlimJ and MidScala arrays produced significantly different hearing loss after surgery. CONCLUSION. Large ECochG amplitude drops observed during electrode insertion indicated poorer hearing preservation. Although the outcomes were variable, this information may be helpful to guide surgical decision-making when contemplating full electrode insertion and the likelihood of hearing preservation.

Objectives Histologic reports of temporal bones of ears with vestibular schwannomas (VSs) have indicated findings of endolymphatic hydrops (ELH) in some cases. The main goal of this investigation was to test ears with VSs to determine if they exhibit electrophysiological characteristics similar to those of ears expected to experience ELH. Design Fifty-three subjects with surgically confirmed VS aged ≥18 and with normal middle ear status were included in this study. In addition, a second group of adult subjects (n = 24) undergoing labyrinthectomy (n = 6) or endolymphatic sac decompression and shunt (ELS) placement (n = 18) for poorly controlled vestibular symptoms associated with Meniere's disease (MD) participated in this research. Intraoperative electrocochleography (ECochG) from the round window was performed using tone burst stimuli. Audiometric testing and word recognition scores (WRS) were performed preoperatively. ECochG amplitudes, cochlear microphonic/auditory nerve neurophonic (ANN) in the form of the "ongoing" response and summation potential, were analyzed and compared between the two groups of subjects. In addition, to evaluate any effect of auditory nerve function, the auditory nerve score was calculated for each subject. Pure-tone averages were obtained using the average air conduction thresholds at 0.5, 1, and 2 kHz while WRS was assessed using Northwestern University Auditory Test No. 6 word lists. Results In the VS group the average pure-tone averages and WRS were 59.6 dB HL and 44.8%, respectively, while in the MD group they were 52.3 dB HL and 73.8%. ECochG findings in both groups revealed a reduced trend in amplitude of the ongoing response with increased stimulus frequency. The summation potential amplitudes of subjects with VS were found to be less negative than the MD subjects for nearly all test frequencies. Finally, the VS group exhibited poorer amounts of auditory nerve function compared to the MD group. Conclusions The current findings suggest cochlear pathology (e.g., hair cell loss) in both groups but do not support the hypothesis that VSs cause ELH.

OBJECTIVE: To investigate the influence of the COVID-19 pandemic on operative practices of otology and neurotology providers internationally. STUDY DESIGN: Cross-sectional survey. METHODS: A 78-question survey was distributed to otologists and neurotologists between May 12, 2020 and June 8, 2020 to assess the impact of the pandemic on surgical practices. Sections within the survey delineated time periods: prior to the crisis, onset of the crisis, during the crisis, postcrisis transition. RESULTS: Of 396 survey respondents, 284 participants from 38 countries met inclusion criteria.Respondents were 16.9% female and 82.4% male, with a most common age range of 40 to 49 years (36.3%). 69.8% of participants had been in practice for over 10 years and most respondents worked in an academic medical center (79.2%). The average operative weekly caseload was 5.3 (SD 3.9) per surgeon prior to the crisis, 0.7 (SD 1.2) during the COVID-19 crisis, and 3.5 (SD 3.3) for those who had begun a postcrisis transition at the time of survey administration (p 

To evaluate the utility of intracochlear electrocochleography (ECochG) monitoring during cochlear implant (CI) surgery on postoperative hearing preservation.Prospective, randomized clinical trial.Ten high-volume, tertiary care CI centers.Adult patients with sensorineural hearing loss meeting the CI criteria who selected an Advanced Bionics CI.Patients were randomized to CI surgery either with audible ECochG monitoring available to the surgeon during electrode insertion or without ECochG monitoring. Hearing preservation was determined by comparing preoperative unaided low-frequency (125-, 250-, and 500-Hz) pure-tone average (LF-PTA) to postoperative LF-PTA at CI activation. Pre- and post-CI computed tomography was used to determine electrode scalar location and electrode translocation.Eighty-five adult CI candidates were enrolled. The mean (standard deviation [SD]) unaided preoperative LF-PTA across the sample was 54 (17) dB HL. For the whole sample, hearing preservation was "good" (i.e., LF-PTA change 0-15 dB) in 34.5%, "fair" (i.e., LF-PTA change15-29 dB) in 22.5%, and "poor" (i.e., LF-PTA change ≥30 dB) in 43%. For patients randomized to ECochG "on," mean (SD) LF-PTA change was 27 (20) dB compared with 27 (23) dB for patients randomized to ECochG "off" ( p = 0.89). Seven percent of patients, all of whom were randomized to ECochG off, showed electrode translocation from the scala tympani into the scala vestibuli.Although intracochlear ECochG during CI surgery has important prognostic utility, our data did not show significantly better hearing preservation in patients randomized to ECochG "on" compared with ECochG "off."

Background Congenital hearing loss is remarkably heterogeneous, with over 130 deafness genes and thousands of variants, making for innumerable genotype/phenotype combinations. Understanding both the pathophysiology of hearing loss and molecular site of lesion along the auditory pathway permits for significantly individualized counseling. Electrophysiologic techniques such as electrocochleography (ECochG) and electrically-evoked compound action potentials (eCAP) are being studied to localize pathology and estimate residual cochlear vs. neural health. This review describes the expanding roles of genetic and electrophysiologic evaluation in the precision medicine of congenital hearing loss. The basics of genetic mutations in hearing loss and electrophysiologic testing (ECochG and eCAP) are reviewed, and how they complement each other in the diagnostics and prognostication of hearing outcomes. Used together, these measures improve the understanding of insults to the auditory system, allowing for individualized counseling for CI candidacy/outcomes or other habilitation strategies. Conclusion Despite tremendous discovery in deafness genes, the effects of individual genes on neural function remain poorly understood. Bridging the understanding between molecular genotype and neural and functional phenotype is paramount to interpreting genetic results in clinical practice. The future hearing healthcare provider must consolidate an ever-increasing amount of genetic and phenotypic information in the precision medicine of hearing loss.

OBJECTIVES Electrocochleography (ECochG), obtained before the insertion of a cochlear implant (CI) array, provides a measure of residual cochlear function that accounts for a substantial portion of variability in postoperative speech perception outcomes in adults. It is postulated that subsequent surgical factors represent independent sources of variance in outcomes. Prior work has demonstrated a positive correlation between angular insertion depth (AID) of straight arrays and speech perception under the CI-alone condition, with an inverse relationship observed for precurved arrays. The purpose of the present study was to determine the combined effects of ECochG, AID, and array design on speech perception outcomes. DESIGN Participants were 50 postlingually deafened adult CI recipients who received one of three straight arrays (MED-EL Flex24, MED-EL Flex28, and MED-EL Standard) and two precurved arrays (Cochlear Contour Advance and Advanced Bionics HiFocus Mid-Scala). Residual cochlear function was determined by the intraoperative ECochG total response (TR) measured before array insertion, which is the sum of magnitudes of spectral components in response to tones of different stimulus frequencies across the speech spectrum. The AID was then determined with postoperative imaging. Multiple linear regression was used to predict consonant-nucleus-consonant (CNC) word recognition in the CI-alone condition at 6 months postactivation based on AID, TR, and array design. RESULTS Forty-one participants received a straight array and nine received a precurved array. The AID of the most apical electrode contact ranged from 341° to 696°. The TR measured by ECochG accounted for 43% of variance in speech perception outcomes (p < 0.001). A regression model predicting CNC word scores with the TR tended to underestimate the performance for precurved arrays and deeply inserted straight arrays, and to overestimate the performance for straight arrays with shallower insertions. When combined in a multivariate linear regression, the TR, AID, and array design accounted for 72% of variability in speech perception outcomes (p < 0.001). CONCLUSIONS A model of speech perception outcomes that incorporates TR, AID, and array design represents an improvement over a model based on TR alone. The success of this model shows that peripheral factors including cochlear health and electrode placement may play a predominant role in speech perception with CIs.

To determine when infants in the neonatal intensive care unit (NICU) have the first hearing screen performed, and thus inform targeted testing for cytomegalovirus (CMV)-related hearing loss. Retrospective review of electronic health records of infants admitted to a Level 4 outborn NICU and had a first hearing screen performed from 8/2016–8/2018. Among 1498 infants, 546 (36%) had a first hearing screen performed at age >21 days when a positive CMV PCR test cannot distinguish congenital from postnatal CMV acquisition. While most infants tested at >21 days of age were

This combined American Neurotology Society, American Otological Society, and American Academy of Otolaryngology - Head and Neck Surgery Foundation document aims to provide guidance during the coronavirus disease of 2019 (COVID-19) on 1) "priority" of care for otologic and neurotologic patients in the office and operating room, and 2) optimal utilization of personal protective equipment. Given the paucity of evidence to inform otologic and neurotologic best practices during COVID-19, the recommendations herein are based on relevant peer-reviewed articles, the Centers for Disease Control and Prevention COVID-19 guidelines, United States and international hospital policies, and expert opinion. The suggestions presented here are not meant to be definitive, and best practices will undoubtedly change with increasing knowledge and high-quality data related to COVID-19. Interpretation of this guidance document is dependent on local factors including prevalence of COVID-19 in the surgeons' local community. This is not intended to set a standard of care, and should not supersede the clinician's best judgement when managing specific clinical concerns and/or regional conditions.Access to otologic and neurotologic care during and after the COVID-19 pandemic is dependent upon adequate protection of physicians, audiologists, and ancillary support staff. Otolaryngologists and associated staff are at high risk for COVID-19 disease transmission based on close contact with mucosal surfaces of the upper aerodigestive tract during diagnostic evaluation and therapeutic procedures. While many otologic and neurotologic conditions are not imminently life threatening, they have a major impact on communication, daily functioning, and quality of life. In addition, progression of disease and delay in treatment can result in cranial nerve deficits, intracranial and life-threatening complications, and/or irreversible consequences. In this regard, many otologic and neurotologic conditions should rightfully be considered "urgent," and almost all require timely attention to permit optimal outcomes. It is reasonable to proceed with otologic and neurotologic clinic visits and operative cases based on input from expert opinion of otologic care providers, clinic/hospital administration, infection prevention and control specialists, and local and state public health leaders. Significant regional variations in COVID-19 prevalence exist; therefore, physicians working with local municipalities are best suited to make determinations on the appropriateness and timing of otologic and neurotologic care.

The indications for cochlear implantation have gradually expanded as advancements in technology have evolved, resulting in improved audiologic outcomes for both adult and children. There remains a significant underutilization of cochlear implant technology in the United States, and recognition of the potential benefits of cochlear implantation for non-traditional indications is critical for encouraging the evolution of candidacy criteria. Adult cochlear implantation candidacy has progressed from patients with bilateral profound sensorineural hearing loss (SNHL) to include patients with greater degrees of residual hearing, single-sided deafness and asymmetric hearing, and atypical etiologies of hearing loss (eg, vestibular schwannoma, Ménière’s disease, and otosclerosis). Indications for pediatric cochlear implantation have similarly evolved from children with bilateral severe to profound SNHL to implanting children at a younger age, including those with residual hearing, asymmetric hearing loss, inner ear malformations, as well as cochlear nerve deficiency. In this editorial, the literature investigating cochlear implantation for nontraditional indications is reviewed with an aim to use the best available evidence to encourage the evolution of candidacy criteria.

OBJECTIVES: Intraoperative electrocochleography (ECochG) has provided insight regarding inner ear pathophysiology during neurotologic procedures. In this study, intraoperative ECochG findings are reported in patients who presented with episodic aural and vestibular symptoms during resection of posterior fossa neoplasms. PATIENTS: Three patients with episodic vertigo who underwent resection of posterior fossa tumors. INTERVENTION: Intraoperative ECochG was performed before and after tumor resection with the active electrode at the round window. Acoustic stimuli consisted of click and tone bursts presented in alternating polarity. MAIN OUTCOME MEASURE: ECochG responses including summation potential (SP), action potential (AP), and SP:AP ratio values to evaluate for endolymphatic hydrops. RESULTS: All subjects presented with asymmetric sensorineural hearing loss (SNHL), episodic vertigo, and tinnitus. Subject 1 was a 63-year-old woman who underwent left translabyrinthine excision of an endolymphatic sac (ELS) tumor and demonstrated no measurable responses until fenestration of the lateral semicircular canal, suggesting severe hydrops relieved by labyrinthotomy. Subject 2 was a 44-year-old woman who underwent right ELS tumor resection and exhibited an elevated SP:AP ratio. Subject 3 was a 55-year-old woman who underwent right retrolabyrinthine resection of a meningioma and exhibited robust responses without hydrops. CONCLUSIONS: Endolymphatic hydrops secondary to mechanical obstruction by a posterior fossa neoplasm may be demonstrated using intraoperative ECochG. Immediate improvement of hydrops may not be demonstrated after tumor resection.

OBJECTIVES/HYPOTHESIS Results from a prospective trial demonstrated better speech recognition for cochlear implant (CI) recipients implanted with a long lateral wall electrode array compared to subjects with a short array after 1 year of listening experience. As short array recipients may require an extended adaptation period, this study investigated whether differences in speech recognition continued through 4 years of CI use. STUDY DESIGN Long-term follow-up of a prospective randomized trial. METHODS Subjects were randomized to receive a MED-EL medium (24 mm) or standard (31.5 mm) array. Linear mixed models compared speech recognition between cohorts with word recognition in quiet and sentence recognition in noise at 1, 3, 6, 12, 24, and 48 months postactivation. Postoperative imaging and electric frequency filters were reviewed to assess the influence of frequency-to-place mismatch and angular separation between neighboring contacts, a metric associated with peripheral spectral selectivity. RESULTS Long (31.5 mm) array recipients demonstrated superior speech recognition out to 4 years postactivation. There was a significant effect of angular separation between contacts, with more closely spaced contacts associated with poorer speech recognition. There was no significant effect of mismatch, yet this may have been obscured by changes in frequency filters over time. CONCLUSIONS Conventional MED-EL CI recipients implanted with 31.5-mm arrays experience better speech recognition than 24-mm array recipients, initially and with long-term listening experience. The benefit conferred by longer arrays in the present cohort can be partially attributed to more widely spaced electrode contacts, presumably a result of reduced channel interaction. LEVEL OF EVIDENCE 2 Laryngoscope, 131:892-897, 2021.

Objective Given the heterogeneity of papers about electrocochleography (ECochG) and cochlear implantation (CI) and the absence of a systematic review in the current literature, the aim of this work was to analyze the uses of ECochG in the different stages of CI. Data sources A search of PubMed from inception to December 8, 2019, with cross-references, was executed. Keywords were: "Cochlear Implant" OR "Cochlear Implantation" AND "Electrocochleography" OR "ECochG." The main eligibility criteria were English-language articles, investigating the use of ECochG in the different phases of CI. Study selection Literature reviews, editorials, case reports, conference papers were excluded, as were papers in which ECochG was just sporadically executed. Data extraction The quality of the included studies was assessed using "The Strengthening the Reporting of Observational Studies in Epidemiology" (STROBE) Statement. Data synthesis A total of 95 articles were identified and 60 papers were included. The included articles covered a timeframe from 2003 to 2019. Of the 60 papers, 46 were human studies, 12 animal studies, and two involved more data sets. Eleven related to the diagnostic phase, 43 described intraoperative monitoring, and 10 were regarding follow-up testing. Hearing preservation was the most discussed topic with 25 included articles. Conclusions and relevance ECochG measurements appeared to be useful in many aspects of CI, such as hearing preservation. Our review is the first that shows the evolution of the technique and how much has been achieved from the earliest experiments to the most recent signal process refinements and device implementation in CI.

To examine opioid prescribing patterns and consumption among patients undergoing common otologic surgeries.Retrospective cohort study with chart review and telephone survey.Tertiary academic medical center.Retrospective chart review and telephone survey of those undergoing tympanoplasty, tympanomastoidectomy, stapedectomy, and cochlear implantation in 2018. The survey consisted of questions regarding the details of the number of pills taken, duration of opioid use, subjective pain control, the use of over-the-counter pain medications, opioid disposal, and their history of substance abuse.Sixty-one patients were able to be contacted and agreed to participate in the study. Fifty-nine (96.7%) stated that their pain was controlled, and 10 (16.4%) did not take any opioids postoperatively despite their prescription. The mean morphine milligram equivalent (MME) prescribed was 99.9 (44.3) and MME taken was 45.2 (SD 46.3) (p 0.001). Similarly, the mean number of pills prescribed was 17.8 (SD 8.6) and mean taken was 7.9 (SD 8.3) (p 0.001). Comparison between males and females regarding MME and pills prescribed and taken were not statistically significantly different (p 0.05). Analysis of the MME and pills prescribed and taken among the different surgeries (tympanoplasty, stapes surgery, tympanomastoidectomy, and cochlear implantation) revealed no statistically significant interactions (p 0.05). Pain control was achieved for 50% of patients with 5 pills (MME = 25 mg), for 75% with 12 pills (MME = 60 mg), and for 90% with 24 pills (MME = 135 mg).The opioid epidemic continues to be an ongoing issue in the United States, and prescription opioid abuse is a large contributor. There is increasing literature to suggest a practice of overprescribing in multiple surgical specialties. This same finding appears to be present in common otologic surgeries, where on average patients took less than half of the prescribed MME/pills, and 75% of patients had their pain controlled with 12 pills or fewer. Otolaryngologists performing otologic surgery should strongly consider adjusting their postoperative regimens to reflect these findings.2b.

Current cochlear implants (CIs) are semi-implantable devices with an externally worn sound processor that hosts the microphone and sound processor. A fully implantable device, however, would ultimately be desirable as it would be of great benefit to recipients. While some prototypes have been designed and used in a few select cases, one main stumbling block is the sound input. Specifically, subdermal implantable microphone technology has been poised with physiologic issues such as sound distortion and signal attenuation under the skin. Here we propose an alternative method that utilizes a physiologic response composed of an electrical field generated by the sensory cells of the inner ear to serve as a sound source microphone for fully implantable hearing technology such as CIs. Electrophysiological results obtained from 14 participants (adult and pediatric) document the feasibility of capturing speech properties within the electrocochleography (ECochG) response. Degradation of formant properties of the stimuli /da/ and /ba/ are evaluated across various degrees of hearing loss. Preliminary results suggest proof-of-concept of using the ECochG response as a microphone is feasible to capture vital properties of speech. However, further signal processing refinement is needed in addition to utilization of an intracochlear recording location to likely improve signal fidelity.

This study aimed to characterize time and spectral domain characteristics of the phoneme-evoked electrocochleography (ECochG) response and explore potential associations between the ECochG spectral content, word recognition scores (WRSs), residual hearing, and aging in normal and hearing-impaired listeners.This was a prospective study with 25 adult participants. All participants underwent intraoperative ECochG testing and responses were recorded from the round window niche. Participants were divided into two groups based on their preoperative pure tone average: normal/mild sensorineural hearing loss and moderate/moderately-severe sensorineural hearing loss. Target stimuli were a 40 ms /da/ and an 80 ms /ba/ presented in alternating polarity (rarefaction/condensation). Waveform response patterns were analyzed including amplitude, latency, and spectra. Structural similarity index measure (SSIM) was used to determine similarity between the stimulus spectrum and that of the ECochG differential waveform. Correlation analyses were carried out among pure tone average, SSIM, age, and WRS.ECochG alternating waveform morphology evoked by the /da/ stimulus consisted of five prominent peaks labeled N 1 -N 5 . Its spectrum was dominated by the fundamental (F 0 ) frequency. The ECochG alternating response evoked by /ba/ consisted of nine prominent peaks labeled N 1 -N 9 and was also dominated by F 0 . Amplitudes and latencies were not statistically different between groups for both stimuli. Significant positive correlations were found between SSIM and WRS for responses evoked by /da/ (r = 0.56) and responses evoked by /ba/ (r = 0.67). High frequency pure tone average and the /ba/ SSIM were found to have a significant negative correlation (r = -0.58).Speech-like stimuli have become increasingly utilized in the assessment of auditory function. Here, we provided the groundwork for understanding how commonly employed syllable stimuli are encoded by the peripheral auditory system in regard to temporal and spectral characteristics. Expanding this work to include measurements of central auditory processing in conjunction with cochlear physiology is warranted to further understand the relationship between peripheral and central encoding of speech-like stimuli.

A 9-year-old male with history of mixed hearing loss presented with petrous apex lesion following episode of meningitis. Serial imaging revealed persistence of the lesion necessitating biopsy to rule out malignancy. Biopsy revealed inflammatory changes. The management of petrous apex lesions following meningitis can be conservative but repeat imaging is necessary to rule out progression and to rule out neoplastic process.

HYPOTHESIS Objective physiologic changes measured using electrocochleography at the round window (ECOG) are observable during endolymphatic sac decompression and shunt surgery (ELS). BACKGROUND Limited effective treatment options are available to patients with Meniere's disease (MD) who have failed conservative management, experience persistent vertigo symptoms, and have substantial residual hearing. ELS is a feasible therapeutic option for these patients. However, the efficacy of this procedure has been questioned, and objective measures assessing inner ear physiologic alterations are lacking. METHODS ECOG was measured in patients with MD undergoing ELS. Stimuli consisted of tone bursts (250, 500, 1000, 2000, 4000 Hz) and 100 μs broadband clicks at various intensities (60-90 dB nHL). Cochlear microphonic (CM), summation potential (SP), compound action potential (AP), SP:AP ratio, and CM harmonic distortions were measured. RESULTS ECOG was completed in 18 patients. The mean SP magnitude at 500 Hz changed significantly from -7.1 μV before to -5.1 μV after ELS (p

Among the various cochlear implant systems approved by the Food and Drug Administration, current labeling for pediatric usage encompasses (1) bilateral profound bilateral sensorineural hearing loss in children aged 9 to 24 months and bilateral severe to profound sensorineural hearing loss in children older than 2 years; (2) use of appropriately fitted hearing aids for 3 months (this can be waived if there is evidence of ossification); and (3) demonstration of limited progress with auditory, speech, and language development. Pediatric guidelines require children to have significantly worse speech understanding before qualifying for cochlear implantation. The early years of life have been shown to be critical for speech and language development, and auditory deprivation is especially detrimental during this crucial time.Level of evidence: 2.

International audience; Importance Cochlear implants are a treatment option for individuals with severe, profound, or moderate sloping to profound bilateral sensorineural hearing loss (SNHL) who receive little or no benefit from hearing aids; however, cochlear implantation in adults is still not routine.Objective To develop consensus statements regarding the use of unilateral cochlear implants in adults with severe, profound, or moderate sloping to profound bilateral SNHL.Design, Setting, and Participants This study was a modified Delphi consensus process that was informed by a systematic review of the literature and clinical expertise. Searches were conducted in the following databases: (1) MEDLINE In-Process & Other Non-Indexed Citations and Ovid MEDLINE, (2) Embase, and (3) the Cochrane Library. Consensus statements on cochlear implantation were developed using the evidence identified. This consensus process was relevant for the use of unilateral cochlear implantation in adults with severe, profound, or moderate sloping to profound bilateral SNHL. The literature searches were conducted on July 18, 2018, and the 3-step Delphi consensus method took place over the subsequent 9-month period up to March 30, 2019.Main Outcomes and Measures A Delphi consensus panel of 30 international specialists voted on consensus statements about cochlear implantation, informed by an SR of the literature and clinical expertise. This vote resulted in 20 evidence-based consensus statements that are in line with clinical experience. A modified 3-step Delphi consensus method was used to vote on and refine the consensus statements. This method consisted of 2 rounds of email questionnaires and a face-to-face meeting of panel members at the final round. All consensus statements were reviewed, discussed, and finalized at the face-to-face meeting.Results In total, 6492 articles were identified in the searches of the electronic databases. After removal of duplicate articles, 74 articles fulfilled all of the inclusion criteria and were used to create the 20 evidence-based consensus statements. These 20 consensus statements on the use of unilateral cochlear implantation in adults with SNHL were relevant to the following 7 key areas of interest: level of awareness of cochlear implantation (1 consensus statement); best practice clinical pathway from diagnosis to surgery (3 consensus statements); best practice guidelines for surgery (2 consensus statements); clinical effectiveness of cochlear implantation (4 consensus statements); factors associated with postimplantation outcomes (4 consensus statements); association between hearing loss and depression, cognition, and dementia (5 consensus statements); and cost implications of cochlear implantation (1 consensus statement).Conclusions and Relevance These consensus statements represent the first step toward the development of international guidelines on best practices for cochlear implantation in adults with SNHL. Further research to develop consensus statements for unilateral cochlear implantation in children, bilateral cochlear implantation, combined electric-acoustic stimulation, unilateral cochlear implantation for single-sided deafness, and asymmetrical hearing loss in children and adults may be beneficial for optimizing hearing and quality of life for these patients.

Intraoperative electrocochleography (ECochG) has provided insight regarding inner ear pathophysiology during neurotologic procedures. In this study, intraoperative ECochG findings are reported in patients who presented with episodic aural and vestibular symptoms during resection of posterior fossa neoplasms.Three patients with episodic vertigo who underwent resection of posterior fossa tumors.Intraoperative ECochG was performed before and after tumor resection with the active electrode at the round window. Acoustic stimuli consisted of click and tone bursts presented in alternating polarity.ECochG responses including summation potential (SP), action potential (AP), and SP:AP ratio values to evaluate for endolymphatic hydrops.All subjects presented with asymmetric sensorineural hearing loss (SNHL), episodic vertigo, and tinnitus. Subject 1 was a 63-year-old woman who underwent left translabyrinthine excision of an endolymphatic sac (ELS) tumor and demonstrated no measurable responses until fenestration of the lateral semicircular canal, suggesting severe hydrops relieved by labyrinthotomy. Subject 2 was a 44-year-old woman who underwent right ELS tumor resection and exhibited an elevated SP:AP ratio. Subject 3 was a 55-year-old woman who underwent right retrolabyrinthine resection of a meningioma and exhibited robust responses without hydrops.Endolymphatic hydrops secondary to mechanical obstruction by a posterior fossa neoplasm may be demonstrated using intraoperative ECochG. Immediate improvement of hydrops may not be demonstrated after tumor resection.

Introduction Family-centered healthcare demands that families provide input regarding the care of their children. Very little is known, however, about how families perceive their experience in different types of multidisciplinary team models, and specifically, in the multidisciplinary setting currently utilized in many pediatric hearing clinics. Methods Quantitative and qualitative parent survey responses were collected and analyzed in a tertiary care pediatric medical center after a one-day multidisciplinary assessment clinical appointment. Questions pertained to information across five domains, including overall experience, diagnosis, treatment plan formulation, additional testing, and resources. Quantitative responses were analyzed descriptively while qualitative responses were evaluated using content analysis to derive themes. Quantitative and qualitative data were evaluated separately and then compared to delineate themes for strengths and weaknesses. Results Overall, high satisfaction was evident in both quantitative and qualitative responses. Results suggested that a one-day multidisciplinary assessment appointment may contribute to parents feeling overwhelmed by information shared and not fully understanding which disciplines are providing care. Analysis revealed a specific area of weakness in our particular setting was inadequate provision of information about functional hearing (e.g., listening socially and academically). Results contributed to a change from a multidisciplinary team model to an interdisciplinary care coordination approach to pediatric hearing healthcare. Conclusions Understanding parent perspectives and expectations is the corner stone of family-centered care and may ultimately influence a child's developmental outcome. A systematic way of evaluating parent perspectives on the clinical process can influence service delivery and help children with hearing loss meet their potential.

Background Surgical resection of vestibular schwannomas (VS) located within the internal auditory canal (IAC) is challenging, especially those located very laterally in the IAC. Various transcranial approaches have been described for resection of intracanalicular VS including retrosigmoid, translabyrinthine, and middle fossa approaches. Each approach has its indications, advantages, and limitations. The middle fossa approach (MFA) is considered by many authors as the gold standard approach for resection of small intracanalicular VS in young patients with serviceable hearing; however, there is often a limitation in complete visualization of the tumor. Methods The authors present an illustrative case to highlight the technical nuances of complementary use of endoscopy in MFA for complete resection of intracanalicular VS located at the IAC fundus, preserving preoperative hearing status and mainting intact facial nerve function. Results In our experience, the combined use of the endoscope and the microspe as described here and illustrated in our case, improves visualization of the IAC and its contents, improving ressection and outcomes. Conclusions MFA allows for a direct yet safe surgical corridor to small intracanalicular VS. The implementation of endoscopy with MFA, especially for VS located laterally in the IAC, allows for better opportunity for complete resection of the tumor with improved preservation of hearing and facial nerve function.

OBJECTIVES: Electrocochleography (ECochG) is increasingly being utilized as an intraoperative monitor of cochlear function during cochlear implantation (CI). Intracochlear recordings from the advancing electrode can be obtained through the device by on-board capabilities. However, such recordings may not be ideal as a monitor because the recording electrode moves in relation to the neural and hair cell generators producing the responses. The purposes of this study were to compare two extracochlear recording locations in terms of signal strength and feasibility as intraoperative monitoring sites, and to characterize changes in cochlear physiology during CI insertion. DESIGN: In 83 human subjects, responses to 90 dB nHL tone bursts were recorded both at the round window (RW) and then at an extracochlear position – either adjacent to the stapes or on the promontory just superior to the RW. Recording from the fixed, extracochlear position continued during insertion of the CI in 63 cases. RESULTS: Prior to CI insertion, responses to low-frequency tones at the RW were roughly 6 dB larger than when recording at either extracochlear site, but the two extracochlear sites did not differ from one another. During CI insertion, response losses from the promontory or adjacent to the stapes stayed within 5 dB in ~61% (38/63) of cases, presumably indicating atraumatic insertions. Among responses which dropped more than 5 dB at any time during CI insertion, 12 subjects showed no response recovery while in 13 the drop was followed by partial or complete response recovery by the end of CI insertion. In cases with recovery the drop in response occurred relatively early (

Hypothesis Electrocochleography (ECochG) patterns observed during cochlear implant (CI) electrode insertion may provide information about scalar location of the electrode array. Background Conventional CI surgery is performed without actively monitoring auditory function and potential damage to intracochlear structures. The central hypothesis of this study was that ECochG obtained directly through the CI may be used to estimate intracochlear electrode position and, ultimately, residual hearing preservation. Methods Intracochlear ECochG was performed on 32 patients across 3 different implant centers. During electrode insertion, a 50-ms tone burst stimulus (500 Hz) was delivered at 110 dB SPL. The ECochG response was monitored from the apical-most electrode. The amplitude and phase changes of the first harmonic were imported into an algorithm in an attempt to predict the intracochlear electrode location (scala tympani [ST], translocation from ST to scala vestibuli [SV], or interaction with basilar membrane). Anatomic electrode position was verified using postoperative computed tomography (CT) with image processing. Results CT analysis confirmed 25 electrodes with ST position and 7 electrode arrays translocating from ST into SV. The ECochG algorithm correctly estimated electrode position in 26 (82%) of 32 subjects while 6 (18%) electrodes were wrongly identified as translocated (sensitivity = 100%, specificity = 77%, positive predictive value = 54%, and a negative predictive value = 100%). Greater hearing loss was observed postoperatively in participants with translocated electrode arrays (36 ± 15 dB) when compared with isolated ST insertions (28 ± 20 dB HL). This result, however, was not significant (p = 0.789). Conclusion Intracochlear ECochG may provide information about CI electrode location and hearing preservation.

Objectives Increasing evidence suggests that hearing loss may be linked to cognitive decline, and that cochlear implantation may lead to improvements in cognition. The goal of this study was to examine the effects of severe-to-profound hearing loss and cochlear implantation in post-lingually deafened adults, compared with age-matched normal-hearing (NH) peers. Participants were tested on several non-auditory measures of cognition: working memory (WM) (digit span, object span, symbol span), non-verbal reasoning (Raven's progressive matrices), information-processing speed and inhibitory control (Stroop test), speed of phonological and lexical access (Test of Word Reading Efficiency), and verbal learning and memory (California Verbal Learning Test). Demographic measures were also collected. Methods Cohort study at tertiary neurotology center. Forty-three post-lingually deafened experienced CI users, 19 post-lingually deafened CI candidates, and 40 age-matched NH controls with no cognitive impairment were enrolled. Comparisons among the groups on the cognitive measures were performed. Results Adult CI users and CI candidates demonstrated worse (or a trend towards worse) performance as compared with NH peers on non-verbal reasoning, information-processing speed, speed of lexical access, and verbal learning and memory. However, after controlling for gender, socioeconomic status (SES), and vocabulary knowledge among groups, some of these differences were no longer significant. Similarly, large differences were not found in most cognitive abilities between experienced CI users and CI candidates. Conclusions Adult CI users, CI candidates, and NH peers generally demonstrated equivalent non-auditory cognitive abilities, after controlling for gender, SES, and vocabulary knowledge. These findings provide support for a link between cognitive decline and hearing loss, but this association may be partly attributable to group differences in SES and vocabulary knowledge. Level of evidence 2b.

Objective: To demonstrate the safety and effectiveness of the MED-EL Electric-Acoustic Stimulation (EAS) System, for adults with residual low-frequency hearing and severe-to-profound hearing loss in the mid to high frequencies. Study Design: Prospective, repeated measures. Setting: Multicenter, hospital. Patients: Seventy-three subjects implanted with PULSAR or SONATA cochlear implants with FLEX24 electrode arrays. Intervention: Subjects were fit postoperatively with an audio processor, combining electric stimulation and acoustic amplification. Main Outcome Measures: Unaided thresholds were measured preoperatively and at 3, 6, and 12 months postactivation. Speech perception was assessed at these intervals using City University of New York sentences in noise and consonant–nucleus–consonant words in quiet. Subjective benefit was assessed at these intervals via the Abbreviated Profile of Hearing Aid Benefit and Hearing Device Satisfaction Scale questionnaires. Results: Sixty-seven of 73 subjects (92%) completed outcome measures for all study intervals. Of those 67 subjects, 79% experienced less than a 30 dB HL low-frequency pure-tone average (250–1000 Hz) shift, and 97% were able to use the acoustic unit at 12 months postactivation. In the EAS condition, 94% of subjects performed similarly to or better than their preoperative performance on City University of New York sentences in noise at 12 months postactivation, with 85% demonstrating improvement. Ninety-seven percent of subjects performed similarly or better on consonant–nucleus–consonant words in quiet, with 84% demonstrating improvement. Conclusion: The MED-EL EAS System is a safe and effective treatment option for adults with normal hearing to moderate sensorineural hearing loss in the low frequencies and severe-to-profound sensorineural hearing loss in the high frequencies who do not benefit from traditional amplification.

Hypothesis Intraoperative, intracochlear electrocochleography (ECochG) will provide a means to monitor cochlear hair cell and neural response during cochlear implant (CI) electrode insertion. Distinct patterns in the insertion track can be characterized. Background Conventional CI surgery is performed without a means of actively monitoring cochlear hair cell and neural responses. Intracochlear ECochG obtained directly through the CI may be a source of such feedback. Understanding the patterns observed in the "insertion track" is an essential step toward refining intracochlear ECochG as a tool that can be used to assist in intraoperative decision making and prognostication of hearing preservation. Methods Intracochlear ECochG was performed in 17 patients. During electrode insertion, a 50-ms tone burst acoustic stimulus was delivered with a frequency of 500 Hz at 110 dB SPL. The ECochG response was monitored from the apical-most electrode. The amplitude of the first harmonic was plotted and monitored in near real time by the audiologist-surgeon team during CI electrode insertion. Results Three distinct patterns in first harmonic amplitude change were observed across subjects during insertion: Type A (52%), overall increase in amplitude from the beginning of insertion until completion; Type B (11%), a maximum amplitude at the beginning of insertion, with a decrease in amplitude as insertion progressed to completion; and Type C (35%), comparable amplitudes at the beginning and completion of the insertion with the maximum amplitude mid-insertion. Conclusion Three ECochG patterns were observed during electrode advancement into the cochlea. Ongoing and future work will broaden our scope of knowledge regarding the relationship among these patterns, the presence of cochlear trauma, and functional outcomes related to hearing preservation.

Objectives Neurocognitive functions contribute to speech recognition in postlingual adults with cochlear implants (CIs). In particular, better verbal working memory (WM) on modality-specific (auditory) WM tasks predicts better speech recognition. It remains unclear, however, whether this association can be attributed to basic underlying modality-general neurocognitive functions, or whether it is solely a result of the degraded nature of auditory signals delivered by the CI. Three hypotheses were tested: 1) Both modality-specific and modality-general tasks of verbal WM would predict scores of sentence recognition in speech-shaped noise; 2) Basic modality-general neurocognitive functions of controlled fluency and inhibition-concentration would predict both modality-specific and modality-general verbal WM; and 3) Scores on both tasks of verbal WM would mediate the effects of more basic neurocognitive functions on sentence recognition. Study Design Cross-sectional study of 30 postlingual adults with CIs and thirty age-matched normal-hearing (NH) controls. Materials and Methods Participants were tested for sentence recognition in speech-shaped noise, along with verbal WM using a modality-general task (Reading Span) and an auditory modality-specific task (Listening Span). Participants were also assessed for controlled fluency and inhibition-concentration abilities. Results For CI users only, Listening Span scores predicted sentence recognition, and Listening Span scores mediated the effects of inhibition-concentration on speech recognition. Scores on Reading Span were not related to sentence recognition for either group. Conclusion Inhibition-concentration skills play an important role in CI users' sentence recognition skills, with effects mediated by modality-specific verbal WM. Further studies will examine inhibition-concentration and WM skills as novel targets for clinical intervention. Level of Evidence 4.

Utilizing the cochlear implant to record electrophysiologic responses during device placement is a feasible and efficacious technique for monitoring near real-time cochlear physiology during and following electrode insertion.Minimizing intracochlear trauma during cochlear implantation has emerged as a highly researched area to help improve patient performance. Currently, conventional cochlear implant technology allows for the recording of electrically evoked compound action potentials (eCAPs). Acoustically evoked potentials may be more sensitive in detecting physiologic changes occurring as a result of electrode insertion. Electrocochleography obtained from within the cochlea allows hair cell and neural response monitoring along the cochlear spiral at locations where changes most likely would occur.Intracochlear electrocochleography (ECochG) was recorded from the cochlear implant during surgery in 14 subjects. A long acquisition time (54.5 ms), capable of measuring potentials from the low frequency-serving apical region of the cochlea (125 and 500 Hz) was employed. Two distinct intracochlear processing methods were used and compared in obtaining electrophysiologic data.Measureable intracochlear ECochG responses were obtained from all 14 participants. The 1st harmonic distortions (cochlear microphonic and auditory nerve neurophonic) generally increased steadily with electrode insertion. Electrode and frequency scan following insertion revealed that response amplitude varied based on location of recording electrode and frequency of stimulation. Exquisite sensitivity to manipulation during round window muscle packing was demonstrated.Intracochlear ECochG recorded from the electrode array of the cochlear implant is a highly feasible technique that sheds light on cochlear micromechanics during cochlear implant electrode placement.

Hypothesis Intraoperative electrocochleography (ECochG) can differentiate hair cell and neural dysfunction caused by a vestibular schwannoma (VS) and help define the site of lesion as peripheral or central to the spiral ganglion. Background Hearing loss in patients with a VS can be caused by both sensory (cochlear) and neural dysfunction. Methods Round-window ECochG using low and high-frequency tone bursts was performed in 49 subjects with a VS. Responses were analyzed for magnitude, spectrum, and shape of the time waveform. Components examined included the cochlear microphonic, auditory nerve neurophonic, compound action potential (CAP), and summating potential (SP). Results Variability in the summed response magnitudes across frequency, or "total response" (ECochG-TR), varied from 0.1 to 100 μV. Responses were larger for lower frequencies. Subjective estimates revealed a wide range of neural contributions from the auditory nerve neurophonic to low frequencies and the CAP to high frequencies. Cases with larger CAPs had smaller SPs. The correlation of ECochG-TR, with word recognition score (WRS), was moderate (r = 0.67), as well as the correlation between pure-tone averages and ECochG (r = 0.63). The cochlea remained functional in two cases of sudden sensorineural hearing loss with 0% WRS. Conclusion Reduced ECochG-TR and neural activity in many cases indicates both sensory and neural deficits. Smaller SPs when CAPs are present indicate a neural contribution to the SP. Good cochlear function with 0% WRS, and cases of sudden sensorineural hearing loss with retained cochlear function, indicate retrocochlear effects, typically proximal to the spiral ganglion cells.

We present a unique case of a patient with a jugular foramen tumor with serviceable hearing. This study discusses the audiometric results and intraoperative electrocochleographic (ECochG) findings recorded during tumor removal to illustrate the potential utility of this technique in skull base surgery.A 22-year-old female patient presented with a jugular foramen schwannoma and associated symptoms of right-sided otalgia, mild hearing loss, and blurry vision.Intraoperative ECochG responses during an infratemporal fossa approach: click and tone burst (1, 2, 4 kHz) stimuli were used and presented at 90 dB nHL.Intraoperative ECochG testing using frequency-specific tone bursts and clicks before and after tumor resection.The compound action potential magnitudes, cochlear microphonic, and summation potential were recorded pre- and post-tumor removal. For statistical analysis, a paired t test with significance set at p 0.05 was used. The compound action potential magnitudes increased at all test frequencies (p 0.01) while the summation potential and cochlear microphonic remained relatively stable (p 0.05). Audiometric testing demonstrated an improvement of the preoperative mild right-sided hearing loss after tumor resection (pure-tone average for 0.5, 1, 2, and 4 kHz of 30 dB HL preoperation and 7.5 dB HL after tumor resection).Intraoperative ECochG may allow for real-time monitoring during complex skull base surgery.

A 15-year-old female presented for evaluation of progressive hearing loss over a year. Computed tomographic imaging revealed a 11 x 6 × 6 mm osseous lesion with 'groundglass' appearance within the left posterior petrous bone lateral to vestibular aqueduct suspicious for an endolymphatic sac tumor. Surgical excision revealed fibrous dysplasia on histological analysis. Fibrous dysplasia of the temporal bone is not uncommon, but few cases of extension causing sensorineural hearing loss exist in the literature. We describe the first reported case of fibrous dysplasia mimicking an endolymphatic sac tumor; interestingly, the patient also showed hearing improvement immediately following removal.

HYPOTHESIS: Electrocochleography (ECochG) recorded during cochlear implant (CI) insertion from the apical electrode in conjunction with post-insertion ECochG can identify electrophysiologic differences that exist between groups with and without a translocation of the array from the scala tympani (ST) into the scala vestibuli (SV). BACKGROUND: Translocation of the CI electrode from ST into SV, can limit performance post-operatively. ECochG markers of trauma may be able to aid in the ability to detect electrode array-induced trauma/scalar translocation intraoperatively. METHODS: Twenty-one adult CI patients were included. Subjects were post-operatively parsed into two groups based on analysis of post-operative imaging: (1) ST (n=14) insertion; (2) SV (n=7) insertion, indicating translocation of the electrode. The ECochG response elicited from a 500 Hz acoustic stimulus was recorded from the lead electrode during insertion when the distal electrode marker was at the round window, and was compared to the response recorded from a basal electrode (e13) after complete insertion. RESULTS: No statistically significant change in mean ECochG magnitude was found in either group between recording intervals. There was a mean loss of pre-operative pure tone average of 52% for the non-translocation group and 94% for the translocation group. CONCLUSIONS: Intraoperative intracochlear ECochG through the CI array provides a unique opportunity to explore the impact of the CI electrode on the inner ear. Specifically, a translocation of the array from ST to SV does not appear to change the biomechanics of the cochlear region that lies basal to the area of translocation in the acute period.

Objectives The goal of this review was to review our series of isolated malleus fixation in pediatric patients, a rare entity causing conductive hearing loss. Malleolar fixation is poorly described in this patient population. Methods A retrospective review of pediatric tympanoplasties by the senior author over a four-year period was performed. Only cases with isolated fixation of the malleus were reviewed. Primary outcome of interest was post-operative hearing. Paired t-tests were used to calculate pre- and post-operative hearing outcomes. Results Five cases were analyzed. Mean age at time of surgery was 9.1 years (range 4.4–16.0 years). Average follow-up after surgery was 13.9 months (range 4.4–31.2 months). Patients were otherwise healthy and typically presented after a failed school hearing test despite previously good hearing. Three out of five cases showed radiographic evidence of bony fixation (60%) on computerized tomography (CT). Otoscopy was unremarkable in all cases. Average procedure time was 41.2 min and consisted of transcanal tympanoplasty with excision of fixed bony segment. A significant improvement in both pre- and post-operative air bone gaps was observed (p = 0.005)., with average ABG of 14.75 dB. Conclusions Isolated pediatric malleolar fixation is an uncommon cause of pediatric conductive hearing loss. CT scan is useful for identifying this abnormality, and surgical correction results in improved post-operative hearing outcomes, potentially obviating the need for hearing amplification.

To determine the influence of posterior fossa dimensions on surgical outcomes after vestibular schwannoma management.Retrospective chart review.Tertiary care academic medical center.Patients with sporadic vestibular schwannomas who underwent surgical resection via retrosigmoid or translabyrinthine techniques.Diagnostic.One and two-way analysis of variance (ANOVAs) of the cohort based on volumetric and clinical outcome groupings.A total of 95 patients were identified. A one-way ANOVA looking at the entire cohort of patients showed that outcomes like surgical time (p 0.001) and whether a Good Outcome (House-Brackmann score of 1 or 2, no complications, and a complete resection) was achieved (p = 0.009) correlated very well with preoperative tumor volume, but not with posterior fossa volume (p = 0.412 and p = 0.345, respectively). However, in medium sized tumors, House-Brackmann group was correlated with posterior fossa volume (p = 0.032).The present data suggest a small effect of posterior fossa size on some clinical outcomes in medium sized vestibular schwannomas.

Objectives/Hypothesis To determine if prolongation of the interdevice interval in children receiving bilateral cochlear implants adversely affects speech perception outcomes. Study Design Retrospective chart review. Methods Retrospective review of our pediatric cochlear implant database was performed. Children who had undergone revision surgery or had less than 12 months listening experience with either the first or second implant were excluded. The interdevice interval, best Phonetically Balanced Kindergarten word lists (PBK) score from each ear, and demographic data about each patient were collected. A ratio of PBK was generated (PBK second side/PBK first side) to minimize potential confounding from other individual patient factors that affect speech outcomes. Results Two hundred forty children met the study criteria. Mean age at first cochlear implantation (CI) was 3.2 years (0.6–17.9), and the second was 6.6 years (0.8–22.4). Mean best PBK score from the first CI side was 83.8% (0–100), and the second was 67.5% (0–100) (P < .001). When the PBK ratio was plotted against interdevice interval, R2 was 0.47 (P < .001). When analyzed for hearing stability, those with a progressive loss history demonstrated less influence of prolonged interdevice interval on performance. Multivariate analysis did not identify other factors influencing the ratio. A line of best fit for those with stable hearing loss suggested best outcomes were with an interdevice interval less than 3 to 4 years. Beyond 7 to 8 years, very few achieved useful speech recognition from the second CI. Conclusions Where possible, the second implant should be received within 3 to 4 years of the first to maximize outcome in those with stable, severe to profound sensorineural hearing loss. Level of Evidence 4. Laryngoscope, 2016

Background Congenital cytomegalovirus (CMV) is the leading non-genetic cause of sensorineural hearing loss (SNHL) in children. While SNHL is often present at birth, as many as 25% of congenital CMV-infected infants may develop late-onset hearing loss. Antiviral therapy improves hearing outcomes, but its effect on the occurrence of late-onset SNHL is not fully known. Thus, our objective was to describe the prevalence of late-onset SNHL among congenital CMV-infected children treated with antiviral therapy in the first month of age. Methods From 2013 to present, infants with congenital CMV infection referred to Nationwide Children’s Hospital’s (NCH) NEO-ID Clinic, Columbus, OH underwent complete evaluation including hearing testing. Pertinent demographic, clinical, laboratory, and radiographic data were obtained and managed using REDCap electronic data capture tools. Infants who passed the newborn hearing screen and subsequently developed late-onset SNHL were identified and compared with respect to receipt of antiviral therapy in the neonatal period. Statistical analyses were performed using GraphPad Prism for macOS version 8.3.0. Results During the 6-year study period, 99 infants had congenital CMV infection and 69 (70%) of them passed the newborn hearing screen. 46 (46%) neonates received antiviral therapy (1, ganciclovir; 38, valganciclovir; 7, both) for clinically apparent congenital CMV infection. One (2%) child developed late-onset SNHL.This infant was born at 37 weeks’ gestation (birth weight, 2525 g) with microcephaly (head circumference, 31 cm) and cerebral calcifications and was diagnosed with congenital CMV infection at 8 days of age. Treatment with valganciclovir was initiated at 9 days of age, and he developed mild unilateral SNHL at 1 month of age while on treatment and subsequently right severe-profound SNHL and left mild-moderate SNHL. In comparison, among 23 infants with clinically inapparent disease who passed the newborn hearing screen and did not receive antiviral therapy, 5 (22%) subsequently developed SNHL (p=0.014). Conclusion Infants who received antiviral therapy for clinically apparent congenital CMV infection had significantly less late-onset SNHL than untreated infants, thus supporting a hearing protective effect of antiviral treatment. Disclosures All Authors: No reported disclosures