Unilateral Multifocal Inner Ear and Internal Auditory Canal or Cerebellopontine Angle Cochleovestibular Schwannomas-Genetic Analysis and Management by Surgical Resection and Cochlear Implantation.

OBJECTIVE: To describe the genetic characteristics and the management of two very rare cases of unilateral multifocal inner ear and internal auditory canal or cerebellopontine angle cochleovestibular schwannomas not being associated to full neurofibromatosis type 2-related schwannomatosis. PATIENTS: In a 29-year-old man and a 55-year-old woman with single-sided deafness multifocal unilateral cochleovestibular schwannomas were surgically resected, and hearing was rehabilitated with a cochlear implant (CI). Unaffected tissue was analyzed using next generation sequencing of the NF2 gene. Tumor tissue was analyzed using a 340-parallel sequencing gene panel. MAIN OUTCOME MEASURES: Mutations in the NF2 gene, word recognition score for monosyllables at 65 dB SPL (WRS65) with CI. RESULTS: No disease-causing mutation was detected in the examined sequences in blood leucokytes. All tumor samples revealed, among others, somatic pathogenic NF2 mutations. While the anatomically separate tumors in case 1 were likely molecular identical, the tumors in case 2 showed different genetic patterns. WRS65 was 55% at 6 years of follow-up and 60% at 4.5 years of follow-up, respectively. CONCLUSIONS: The occurrence of multifocal unilateral cochleovestibular schwannomas without pathogenic variants in NF2 in non-affected blood leucocytes can be associated with mosaic NF2-related schwannomatosis (case 1), or with likely sporadic mutations (case 2) and may be overlooked due to their extreme rarity. Although challenging, successful hearing rehabilitation could be achieved through surgical resection of the tumors and cochlear implantation.

Does Location of Intralabyrinthine Vestibular Schwannoma Determine Objective and Subjective Vestibular Function?

BACKGROUND AND OBJECTIVE: The incidence of intralabyrinthine schwannomas is increasing, and a growing attention is given to the detrimental effects on hearing function. On the contrary, the vestibular profile of intralabyrinthine vestibular schwannomas (VSs) is still not well understood. We aimed to investigate and report the observed relationships between the intralabyrinthine location of the schwannomas and objective and subjective vestibular profile of the patients. METHODS: Retrospective cohort study of 20 consecutive individuals with sporadic intralabyrinthine schwannomas and grouped according to the intralabyrinthine location of the schwannomas. Vestibular testing consisted of the video head impulse test of all three semicircular canals, the caloric test, cervical and ocular vestibular evoked myogenic potentials, and the dizziness handicap inventory. A nonparametric unpaired t test was performed to compare groups, and Fisher's exact test was used for categorical data. RESULTS: The median video head impulse test gains (lateral, anterior, posterior) were 0.40, 0.50, and 0.75 for intravestibular schwannomas and 0.93, 1.52, and 0.91 for intracochlear schwannomas ( p = 0.0001, p = 0.009, p = 0.33), respectively. Caloric unilateral weakness had a median of 100% for intravestibular schwannomas and 14% for intracochlear schwannomas ( p = 0.0001). The mean dizziness handicap inventory was 21 for intravestibular schwannomas and 1 for cochlear schwannomas ( p = 0.02). There were no significant differences in vestibular evoked myogenic potentials according to intralabyrinthine location. CONCLUSION: By both objective and subjective measures, intralabyrinthine schwannomas with an intravestibular component has significantly worse vestibular function than schwannomas with purely cochlear involvement.

Cochlear Implantation in Sporadic Intralabyrinthine Schwannomas with Single-Sided Deafness: Implications for Binaural Hearing.

BACKGROUND AND OBJECTIVE: Intralabyrinthine schwannomas (ILSs) may have detrimental effects on ipsilateral hearing, commonly leading to single-sided deafness (SSD). Cochlear implantation in patients with ILSs is an option to partly restore ipsilateral hearing; however, the available literature fails to account for the binaural hearing benefits of a cochlear implant (CI) for ILSs. METHODS: We prospectively evaluated SSD patients with sporadic ILS undergoing cochlear implantation with simultaneous tumor resection (n = 10) or with tumor observation (n = 1). Patients completed the Speech, Spatial and Qualities Questionnaire (SSQ12) and the Nijmegen Cochlear Implant Questionnaire (NCIQ) pre- and postoperatively, as well as the Bern Benefit in Single-Sided Deafness (BBSSD) questionnaire postoperatively. Patients were also tested postoperatively with and without their CI to measure the effect on localization ability and binaural summation, squelch, and head shadow. RESULTS: Evaluation was completed for nine and six patients (subjective and objective data, respectively). The CI significantly improved the speech reception threshold (SRT) in the head shadow condition where the target signal was presented to the CI side and the noise to the front (SCIN0). On the other hand, the SRTs in the colocated condition (S0N0) and the condition where the target signal was presented to the front and the noise to the CI side (S0NCI) were unaffected by the CI. The mean localization error decreased significantly from 102° to 61° (p = 0.0031) with the addition of a CI. The scores from SSQ12 demonstrated nonsignificant changes. For NCIQ, the self-esteem and the social interaction domains increased significantly but insignificantly for the remaining domains. The BBSSD responses ranged from +0.5 to +3.5 points. CONCLUSION: After implantation, patients achieved significantly better scores across some of the patient-reported and objective parameters. In addition to reporting on a number of ILS cases where implantation was performed, the study is the first of its kind to document patient-reported and objective binaural hearing improvement after cochlear implantation in patients with ILS and, thereby, lends support to the active management of ILS.

Transmastoid Surgery for Superior Canal Dehiscence: Prospective Longitudinal Objective and Patient-Reported Audiovestibular Outcomes.

OBJECTIVE: We assess symptom control, reversal and stability of objective third-window indicators, and hearing and balance function after transmastoid superior canal dehiscence plugging. To do this, we combine patient self-assessment questionnaires with auditory and vestibular test data collected at a preoperative and multiple postoperative visits. STUDY DESIGN: Prospective cohort study. SETTING: Tertiary referral center. PATIENTS: Patients who underwent transmastoid plugging for superior canal dehiscence from 2012 to 2020. INTERVENTIONS: Plugging of the superior semicircular canal using the transmastoid approach. MAIN OUTCOME MEASURES: Patients were evaluated preoperatively, 3-month postoperative, and at yearly follow-ups (up to 8 yr postoperative). Self-assessment questionnaires included Autophony Index, Dizziness Handicap Inventory, Tinnitus Handicap Inventory, and Hearing Handicap Inventory. Objective tests included audiometry, cervical vestibular evoked myogenic potential, ocular vestibular evoked myogenic potential, and video head impulse test. RESULTS: There was a statistically significant improvement in Autophony Index and Hearing Handicap Inventory total scores from the preoperative evaluation to 1 year postoperative. Dizziness Handicap Inventory and Tinnitus Handicap Inventory total scores also improved but statistically insignificant. Cervical and ocular vestibular evoked myogenic potential amplitudes and low-frequency air-bone gap showed statically significant reversal of the third-window effect preoperative versus 1 year postoperative. Video head impulse test of the superior semicircular canal revealed cases of pathological function preoperative with postoperative recovery. Results were stable over time. CONCLUSIONS: Superior canal dehiscence repair resolves symptoms of autophony and improves hearing disability. Objective third-window indicators reverse and show long-term stability. Auditory and vestibular function is preserved postoperative, even superior semicircular canal function may be normal. Data suggest that surgical outcomes should not be evaluated until 1 year postoperative.

Life Expectancy After Diagnosis of a Vestibular Schwannoma in Patients 70 Years and Older.

Importance: Over the past decades, the number of patients, especially in the older adult patient group, diagnosed with vestibular schwannoma (VS) has increased. Assuming that older adult patients have more comorbidities, a longer recovery period after surgery, a higher rate of surgical complications, and a higher mortality rate after VS surgery, a treatment strategy for this group of patients is warranted, based on clinical evidence on postsurgical survival. Objective: To evaluate the survival after diagnosis of a VS in patients 70 years and older, treated with either observation or surgery, and to compare these findings with the life span of an age-matched background population in Denmark. Design, Setting, and Participants: This was a retrospective cohort study of 624 patients 70 years and older diagnosed with VS in Denmark from 1976 to 2016. Since 1976, all patients with a VS have been registered in a national database, which contains 3637 patients. Of the included patients in this study, 477 were treated conservatively with the "wait-and-scan" strategy, and 147 were treated surgically with removal of the tumor. The survival of the patients was compared with a matched background population in Denmark. Data analysis was performed from January 1976 to January 2017. Exposures: Surgery, radiotherapy, or none. Main Outcomes and Measures: The main outcome was survival among the patients and compared with the matched background population. Results: A total of 624 patients were included (317 female patients [50.8%] and 307 male patients [49.2%]). The mean (SD) survival in the observed patients was 9.2 (4.7) years after diagnosis, whereas for the background population, the expected survival was 11 years from the mean age at diagnosis. For the surgically treated patients, the mean (SD) survival was 11.8 (6.6) years, and expected survival was 11 years for the matched background population. The mean (SD) survival was 10.7 (5.5) years in female patients and 8.9 (5.0) years in male patients. There was no significant difference in survival between treatment modalities, irrespective of tumor size. Conclusions and Relevance: In this cohort study, survival after diagnosis of a VS in patients 70 years and older was similar in the surgical group compared with the age-matched background population. In the wait-and-scan group, the survival after diagnosis was marginally shorter, which may be associated with increased comorbidity.

Cochlear implantation for children.

The introduction of cochlear implants (CI) for pediatric populations with deafness has changed life conditions for deaf children markedly. A new generation of children with CI has emerged, and this review investigates how it has been documented that early intervention with CI and enrolment in family-centered auditory-verbal intervention allow children to close the language gap and develop age-equivalent language before entering school. At the school level, children keep up the language level. Most importantly children assess themselves to have levels of social well-being comparable to their peers with normal hearing.

Enlarged Vestibular Aqueduct and Associated Inner Ear Malformations: Hearing Loss Prognostic Factors and Data Modeling from an International Cohort.

ACKGROUND: There is a need to operationalize existing clinical data to support precision medicine in progressive hearing loss (HL). By utilizing enlarged vestibular aqueduct (EVA) and its associated inner ear abnormalities as an exemplar, we model data from a large international cohort, confirm prognostic factors for HL, and explore the potential to generate a prediction model to optimize current management paradigms. METHODS: An international retrospective cohort study. Regression analyses were utilized to model frequency-specific HL and identify prognostic factors for baseline average HL severity and progression. Elastic-net regression and machine learning (ML) techniques were utilized to predict future average HL progression based upon routinely measurable clinical, genetic, and radiological data. RESULTS: Higher frequencies of hearing were lost more severely. Prognostic factors for HL were the presence of incomplete partition type 2 (coefficient 12.95 dB, P=.011, 95% CI 3.0-22 dB) and presence of sac signal heterogeneity (P=.009, 95% CI 0.062-0.429) on magnetic resonance imaging. Elastic-net regression outperformed the ML algorithms (R2 0.32, mean absolute error 11.05 dB) with coefficients for baseline average hearing level and the presence of sac heterogeneity contributing the most to prediction outcomes. CONCLUSION: Incomplete partition type 2 and endolymphatic sac signal heterogeneity phenotypes should be monitored closely for hearing deterioration and need for early audiological rehabilitation/cochlear implant. Preliminary prediction models have been generated using routinely collected health data in EVA. This study showcases how international collaborative research can use exemplar techniques to improve precision medicine in relatively rare disease entities.

Rationale for the Development of a Novel Clinical Grading Scale for Postoperative Facial Nerve Function: Results of a Multidisciplinary International Working Group.

OBJECTIVE: The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. STUDY DESIGN: Survey. SETTING: A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. RESULTS: House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. CONCLUSIONS: Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.

Tinnitus suppression in a prospective cohort of 45 cochlear implant recipients: occurrence, degree and correlates.

OBJECTIVE: To determine tinnitus prevalence and severity in a cohort of unselected first-time cochlear implant (CI) recipients whose primary motive for CI was sensorineural hearing loss (SNHL), and to evaluate the effect of CI on tinnitus after cochlear implantation. METHODS: Prospective longitudinal study of 45 adult CI recipients with moderate to profound SNHL. Patients completed the Danish version of the Tinnitus Handicap Inventory (THI) and a visual analogue scale (VAS) for tinnitus burden before implantation, 4 months after implantation and 14 months after implantation. RESULTS: The study included 45 patients, of which 29 (64%) had pre-implant tinnitus. Median THI score (IQR) significantly decreased from 20 (34) to 12 (24) at first follow-up (p < 0.05) and to 6 points (17) at second follow-up (p < 0.001). Median VAS (IQR) for tinnitus burden decreased from 33 (62) to 17 (40; p = 0.228) and 12 (27, p < 0.05) at the first and second follow-ups, respectively. Tinnitus was totally suppressed in 19% of patients, improved in 48%, remained unchanged in 19% and worsened in 6%. 2 patients reported new tinnitus. At the second follow-up, 74% of patients had slight or no tinnitus handicap, 16% had mild handicaps, 6% had moderate handicaps, and 3% had severe handicaps. High pre-implant THI and VAS scores correlated with greater decrease in THI scores over time. CONCLUSION: 64% of the patients with SNHL had pre-implant tinnitus, which was decreased 4 and 14 months after implantation. Overall, 68% of patients with tinnitus improved their tinnitus handicap after CI. Patients with higher THI and VAS scores had a larger decline and the highest benefits in terms of tinnitus handicap improvement. The study findings demonstrate that the majority of patients with moderate to profound SNHL eligible for cochlear implantation benefit from complete or partial tinnitus suppression and improved quality of life after implantation.

Hearing Loss in Bacterial Meningitis Revisited-Evolution and Recovery.

Background: Hearing loss and deafness are well-known sequelae from bacterial meningitis (ABM) and may result in social dysfunction and learning difficulties. Yet, the timely development of hearing loss and restitution is poorly studied, especially among adults. Hearing loss was revisited using otoacoustic emissions (OAEs) to determine the occurrence, magnitude, and development of hearing loss among adults with ABM. Methods: Distortion product OAEs were measured in patients with ABM the day of admission and days 2, 3, 5-7, and 10-14 and at follow-up 30-60 days after discharge. Frequencies were categorized as low (1, 1.5, 2 kHz), mid (3, 4, 5 kHz), mid-high (6, 7, 8 kHz), and high (9, 10 kHz). Audiometry was performed on discharge and 60 days after. Results were compared with 158 healthy controls. Results: OAE was obtained in 32 patients. ABM was due to S. pneumoniae in 12 patients (38%). All patients were treated with dexamethasone. OAE emission threshold levels (ETLs) were significantly decreased upon admission and at follow-up in all frequencies compared with healthy controls. A substantial and significant decrease in ETLs was found in S. pneumoniae meningitis. Sensorineural hearing loss (SNHL) >20 dB was present in 13 of 23 (57%) at discharge and in 11 of 18 patients (61%) 60 days after discharge. Hearing recovery decreased from day 3. Conclusions: Hearing loss in ABM still affects >60% of patients despite treatment with dexamethasone. In S. pneumoniae meningitis, SNHL is profound and permanent. A window of opportunity for systemic or local treatments aiming to preserve cochlear function is proposed.

The reliability and agreement of scores in a novel balance measure for older adults: Specific Training According to BaLance Evaluation (STABLE).

PURPOSE: Falls are the leading cause of injury and premature death among community dwelling elderly but can be prevented through comprehensive balance rehabilitation which ideally targets the patient's specific needs. In this study, we evaluate the reliability and agreement of six novel clinical measures of different balance domains which applied in a patient-specific balance profile guides exercise prescription in balance rehabilitation. MATERIALS AND METHODS: The intra-rater reliability and agreement of the six measures were evaluated on the same day in six different cohorts of elderly with balance disability (n = 65-100). Further, the inter-day intra-rater and inter-rater and test-retest reliability and agreement of the measures and the balance profile were evaluated (n = 100). RESULTS: The intra-day intra-rater reliability and agreement was moderate to excellent (ICC2.1 = 0.525-0.968, with SDC% = 6.5-284.9%) but poor to good for the inter-day conditions (ICC2.1 = 0.123-0.832, with SDC% = 6.6-229.2%). The reliability of classifying the lower domain in the balance profile was fair, with kappa = 0.56 (95%CI 0.36-0.76). CONCLUSIONS: Five of the six measures may reliably be applied to measure balance disability and to guide rehabilitation.Implications for rehabilitationBalance disability is the primary reason for accidental falls among elderly but can be prevented through comprehensive individualized balance rehabilitation.Specific Training According to BaLance Evaluation (STABLE) is a novel approach for designing effective balance exercises based on clinical measurements.Five of the six measures are reliable when applied in a patient-specific balance profile to guide rehabilitation following the STABLE approach.

Cochlear Implantation in Children with Enlarged Vestibular Aqueduct: A Systematic Review of Surgical Implications and Outcomes.

OBJECTIVES: This study investigated age at implantation, improvement in hearing and speech perception outcomes, as well as surgical complications in pediatric cochlear implant recipients with Pendred Syndrome (PS) or non-syndromic enlarged vestibular aqueduct (NSEVA). DESIGN: A systematic review of the literature between 1984 and 2021 was performed. Two independent reviewers performed abstract and full-text screening using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. The inclusion criteria were: English language, cochlear implant, age at implantation available, age <18 years, PS, Mondini malformation, and enlarged vestibular aqueduct. Full-text analysis was completed using the National Institute of Health assessment tool for case series and case-control studies. Studies were also graded according to the Oxford Centre for Evidence-Based Medicine grading system. RESULTS: In total, 198 studies were identified and screened, and 55 studies were included for analysis. Audiological outcomes were available in 46 studies, and the four-frequency pure-tone audiogram average improved by 60 to 78 dB HL due to cochlear implantation. Auditory performance and speech intelligibility scores increased by 44%. The overall average implantation age was 60 months. The implantation age was 21 months lower in the studies where individuals were implanted after the year 2000 compared with those implanted before the year 2000. Perilymph gusher/oozing was the most common surgical incident reported, occurring in 187 of 1572 implantations. CONCLUSIONS: In children with PS/NSEVA, cochlear implantation improves pure-tone average by 60 to 78 dB HL and capacity of auditory performance/speech intelligibility by 44%. The implantation age for these children has decreased during the last two decades but is still somewhat higher than reported for unselected pediatric cochlear implantation. Perilymph gusher/oozing is the most common surgical complication.

Cochlear Implantation for Patients with a Vestibular Schwannoma: Effect on Tinnitus Handicap.

BACKGROUND: Tinnitus is a common symptom among individuals with a vestibular schwannoma. In recent years, cochlear implantation, often combined with tumor resection, is an increasingly used option in the management of these patients. The existing literature does not account well for the effect of treatment on tinnitus burden. Thus, this paper reports the effect of cochlear implantation on tinnitus in a cohort of vestibular schwannoma patients. METHODS: Individuals with vestibular schwannoma undergoing cochlear implantation were retrospectively reviewed for tinnitus burden, as evaluated by the Tinnitus Handicap Inventory, administered before and after implantation. The outcome measures were total Tinnitus Handicap Inventory score and scores from each of the Tinnitus Handicap Inventory subdomains (functional, emotional, and catastrophic). In addition, the existing literature on tinnitus in cochlear implanted vestibular schwannoma patients was reviewed. RESULTS: Tumor management consisted of simultaneous resection (77%), previous resection (9%), observation (9%) and radiation (5%). Complete Tinnitus Handicap Inventory evaluation was available for 17 patients. After implantation, the median THItotal changed from 18 to 10 (P = .0006), the subdomain THIfunctional from 10 to 3 (P = .006), the THIemotional from 3 to 0 (P = .023) and the THIcatastrophic from 6 to 1 (P = .004). In the scarcely reported tinnitus outcomes in the literature, most but not all cases experienced a decrease in tinnitus. CONCLUSIONS: The tinnitus burden is significantly reduced by cochlear implantation in individuals with a vestibular schwannoma. This agrees with findings for other etiologies indicating cochlear implantation and supports the eligibility for hearing rehabilitation with a cochlear implant for this specific group of patients.

Cochlear implantation and simultaneous posterior semicircular canal plugging.

BACKGROUND: Intractable benign paroxysmal positional vertigo (BPPV) may be treated by plugging the affected semicircular canal (SCC). A cochlear implant (CI) can diminish subjective symptoms of tinnitus. We present a case with intractable BPPV and incapacitating tinnitus who underwent surgery, simultaneously plugging his posterior SCC (PSCC) and implanting an ipsilateral CI. CASE: A 50-year-old male experienced single-sided deafness (SSD) with severe ipsilateral tinnitus, and intractable BPPV related to the ipsilateral PSCC. Two years earlier, he had been treated with a bone anchored hearing system (BAHS) for his single-sided hearing loss, but his tinnitus and BPPV persisted. The patient was elected for surgical plugging of the affected SCC and was offered a simultaneous ipsilateral CI to treat his hearing loss and reduce his disabling tinnitus. The procedure was initially clinically and subjectively successful, but the tinnitus worsened, following an MRI despite regular precautions. CONCLUSION: This is the first case presentation of cochlear implantation performed concurrent to plugging of the ipsilateral PSCC. The outcome of the procedure was overall successful.

Impact of an Endolymphatic Sac Tumor on Subjective and Objective Audiovestibular Function.

OBJECTIVE: This study reports subjective and objective audiovestibular function in patients diagnosed with an endolymphatic sac tumor (ELST), to evaluate the clinical implication of vestibular testing in ELST patients. STUDY DESIGN: Retrospective cohort study. SETTING: Tertiary referral center/university hospital. PATIENTS: Five patients diagnosed with unilateral ELST. INTERVENTION: Subjective and objective audiovestibular tests; pure-tone average; speech discrimination score; the Video Head Impulse Test; the cervical vestibular evoked myogenic potential test; the caloric irrigation test; the Speech, Spatial and Qualities of Hearing Scale; the Tinnitus Handicap Inventory; and the Dizziness Handicap Inventory. MAIN OUTCOME MEASURE: Findings and characteristics of both subjective and objective audiovestibular functions. RESULTS: The mean pure-tone average and the mean speech discrimination score were 49 dB HL and 68% on the tumor ear and 13 dB HL and 99% contralaterally. The mean Speech, Spatial and Qualities of Hearing Scale score was 84, and the mean Tinnitus Handicap Inventory score was 64. Caloric testing showed a mean unilateral weakness of 80%. The Video Head Impulse Test of the lateral semicircular canal revealed refixation saccades on all tumor ears and an average gain value of 0.75 on the tumor ear versus 0.94 contralaterally. No cervical vestibular evoked myogenic potentials were elicited on the tumor side, whereas all but one was elicited contralaterally. The average Dizziness Handicap Inventory score was 47. CONCLUSION: A characteristic pattern of abnormal caloric findings, refixation saccades, and normal vestibulo-ocular reflex gain alongside sensorineural hearing loss on the tumor ear was identified. Asymmetric sensorineural hearing loss in any patient should lead to objective vestibular evaluation to strengthen not only a targeted tumor surveillance strategy and the indication for imaging, but also the design of vestibular rehabilitation. In addition, vestibular findings may guide the choice of surgical approach.

Intratympanic corticosteroids for sudden sensorineural hearing loss.

BACKGROUND: Idiopathic sudden sensorineural hearing loss (ISSNHL) is common, and defined as a sudden decrease in sensorineural hearing sensitivity of unknown aetiology. Systemic corticosteroids are widely used, however their value remains unclear. Intratympanic injections of corticosteroids have become increasingly common in the treatment of ISSNHL. OBJECTIVES: To assess the effects of intratympanic corticosteroids in people with ISSNHL. SEARCH METHODS: The Cochrane ENT Information Specialist searched the Cochrane ENT Trials Register; CENTRAL (2021, Issue 9); PubMed; Ovid Embase; CINAHL; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials (search date 23 September 2021). SELECTION CRITERIA: We included randomised controlled trials (RCTs) involving people with ISSNHL and follow-up of over a week. Intratympanic corticosteroids were given as primary or secondary treatment (after failure of systemic therapy). DATA COLLECTION AND ANALYSIS: We used standard Cochrane methods, including GRADE to assess the certainty of the evidence. Our primary outcome was change in hearing threshold with pure tone audiometry. Secondary outcomes included the proportion of people whose hearing improved, final hearing threshold, speech audiometry, frequency-specific hearing changes and adverse effects. MAIN RESULTS: We included 30 studies, comprising 2133 analysed participants. Some studies had more than two treatment arms and were therefore relevant to several comparisons. Studies investigated intratympanic corticosteroids as either primary (initial) therapy or secondary (rescue) therapy after failure of initial treatment. 1. Intratympanic corticosteroids versus systemic corticosteroids as primary therapy We identified 16 studies (1108 participants). Intratympanic therapy may result in little to no improvement in the change in hearing threshold (mean difference (MD) -5.93 dB better, 95% confidence interval (CI) -7.61 to -4.26; 10 studies; 701 participants; low-certainty). We found little to no difference in the proportion of participants whose hearing was improved (risk ratio (RR) 1.04, 95% CI 0.97 to 1.12; 14 studies; 972 participants; moderate-certainty). Intratympanic therapy may result in little to no difference in the final hearing threshold (MD -3.31 dB, 95% CI -6.16 to -0.47; 7 studies; 516 participants; low-certainty). Intratympanic therapy may increase the number of people who experience vertigo or dizziness (RR 2.53, 95% CI 1.41 to 4.54; 1 study; 250 participants; low-certainty) and probably increases the number of people with ear pain (RR 15.68, 95% CI 6.22 to 39.49; 2 studies; 289 participants; moderate-certainty). It also resulted in persistent tympanic membrane perforation (range 0% to 3.9%; 3 studies; 359 participants; very low-certainty), vertigo/dizziness at the time of injection (1% to 21%, 3 studies; 197 participants; very low-certainty) and ear pain at the time of injection (10.5% to 27.1%; 2 studies; 289 participants; low-certainty). 2. Intratympanic plus systemic corticosteroids (combined therapy) versus systemic corticosteroids alone as primary therapy We identified 10 studies (788 participants). Combined therapy may have a small effect on the change in hearing threshold (MD -8.55 dB better, 95% CI -12.48 to -4.61; 6 studies; 435 participants; low-certainty). The evidence is very uncertain as to whether combined therapy changes the proportion of participants whose hearing is improved (RR 1.27, 95% CI 1.15 to 1.41; 10 studies; 788 participants; very low-certainty). Combined therapy may result in slightly lower (more favourable) final hearing thresholds but the evidence is very uncertain, and it is not clear whether the change would be important to patients (MD -9.11 dB, 95% CI -16.56 to -1.67; 3 studies; 194 participants; very low-certainty). Some adverse effects only occurred in those who received combined therapy. These included persistent tympanic membrane perforation (range 0% to 5.5%; 5 studies; 474 participants; very low-certainty), vertigo or dizziness at the time of injection (range 0% to 8.1%; 4 studies; 341 participants; very low-certainty) and ear pain at the time of injection (13.5%; 1 study; 73 participants; very low-certainty).  3. Intratympanic corticosteroids versus no treatment or placebo as secondary therapy We identified seven studies (279 participants). Intratympanic therapy may have a small effect on the change in hearing threshold (MD -9.07 dB better, 95% CI -11.47 to -6.66; 7 studies; 280 participants; low-certainty). Intratympanic therapy may result in a much higher proportion of participants whose hearing is improved (RR 5.55, 95% CI 2.89 to 10.68; 6 studies; 232 participants; low-certainty). Intratympanic therapy may result in lower (more favourable) final hearing thresholds (MD -11.09 dB, 95% CI -17.46 to -4.72; 5 studies; 203 participants; low-certainty). Some adverse effects only occurred in those who received intratympanic injection. These included persistent tympanic membrane perforation (range 0% to 4.2%; 5 studies; 185 participants; very low-certainty), vertigo or dizziness at the time of injection (range 6.7% to 33%; 3 studies; 128 participants; very low-certainty) and ear pain at the time of injection (0%; 1 study; 44 participants; very low-certainty).  4. Intratympanic plus systemic corticosteroids (combined therapy) versus systemic corticosteroids alone as secondary therapy We identified one study with 76 participants. Change in hearing threshold was not reported. Combined therapy may result in a higher proportion with hearing improvement, but the evidence is very uncertain (RR 2.24, 95% CI 1.10 to 4.55; very low-certainty). Adverse effects were poorly reported with only data for persistent tympanic membrane perforation (rate 8.1%, very low-certainty). AUTHORS' CONCLUSIONS: Most of the evidence in this review is low- or very low-certainty, therefore it is likely that further studies may change our conclusions.   For primary therapy, intratympanic corticosteroids may have little or no effect compared with systemic corticosteroids. There may be a slight benefit from combined treatment when compared with systemic treatment alone, but the evidence is uncertain. For secondary therapy, there is low-certainty evidence that intratympanic corticosteroids, when compared to no treatment or placebo, may result in a much higher proportion of participants whose hearing is improved, but may only have a small effect on the change in hearing threshold. It is very uncertain whether there is additional benefit from combined treatment over systemic steroids alone. Although adverse effects were poorly reported, the different risk profiles of intratympanic treatment (including tympanic membrane perforation, pain and dizziness/vertigo) and systemic treatment (for example, blood glucose problems) should be considered when selecting appropriate treatment.

Cochlear Implant Surgery: Virtual Reality Simulation Training and Transfer of Skills to Cadaver Dissection-A Randomized, Controlled Trial.

BACKGROUND: Cochlear implantation requires excellent surgical skills; virtual reality simulation training is an effective method for acquiring basic competency in temporal bone surgery before progression to cadaver dissection. However, cochlear implantation virtual reality simulation training remains largely unexplored and only one simulator currently supports the training of the cochlear implantation electrode insertion. Here, we aim to evaluate the effect of cochlear implantation virtual reality simulation training on subsequent cadaver dissection performance and self-directedness. METHODS: This was a randomized, controlled trial. Eighteen otolaryngology residents were randomized to either mastoidectomy including cochlear implantation virtual reality simulation training (intervention) or mastoidectomy virtual reality simulation training alone (controls) before cadaver cochlear implantation surgery. Surgical performance was evaluated by two blinded expert raters using a validated, structured assess- ment tool. The need for supervision (reflecting self-directedness) was assessed via post-dissection questionnaires. RESULTS: The intervention group achieved a mean score of 22.9 points of a maximum of 44 points, which was 5.4% higher than the control group's 21.8 points (P = .51). On average, the intervention group required assistance 1.3 times during cadaver drilling; this was 41% more frequent in the control group who received assistance 1.9 times (P = .21). CONCLUSION: Cochlear implantation virtual reality simulation training is feasible in the context of a cadaver dissection course. The addition of cochlear implantation virtual reality training to basic mastoidectomy virtual reality simulation training did not lead to a significant improvement of performance or self-directedness in this study. Our findings suggest that learning an advanced temporal bone procedure such as cochlear implantation surgery requires much more training than learning mastoidectomy.

Cochlear Implantation Improves Both Speech Perception and Patient-Reported Outcomes: A Prospective Follow-Up Study of Treatment Benefits among Adult Cochlear Implant Recipients.

Cochlear implantation is considered the best treatment option for patients with severe-to-profound sensorineural hearing loss for whom conventional hearing aids are insufficient. We used a repeated measures longitudinal approach to evaluate speech recognition and patient-reported outcomes after cochlear implantation in an unbiased cohort of Danish adult patients in a prospective cohort study. We assessed 39 recipients before and two times after implantation using a battery of tests that included Dantale I, the Danish Hearing in Noise Test, the Nijmegen Cochlear Implant Questionnaire, and the Speech, Spatial, and Qualities of Hearing Scale. The study group improved significantly on all outcome measures following implantation. On average, Dantale I scores improved by 29 percentage points and Hearing in Noise Test scores improved by 22 percentage points. Most notably, the average Dantale score improved from 26 to 70% in the CI in quiet condition and from 12 to 42% in the cochlear implantation in noise condition when tested monaurally. Dantale demonstrated a significant positive correlation with Nijmegen Cochlear Implant Questionnaire and Speech, Spatial, and Qualities of Hearing Scale scores, while Hearing in Noise Test had no significant correlation with the patient-reported outcome measures. Patients improved significantly at 4 months and marginally improved further at 14 months, indicating that they were approaching a plateau. Our study's use of audiometric and patient-reported outcome measures provides evidence of the treatment benefits of cochlear implantation in adults, which may help physicians advise patients on treatment decisions and align treatment benefit expectations, as well as serve as a foundation for the development of new cochlear implantation selection criteria.

Neurotransmitter and neurotransmitter receptor expression in the saccule of the human vestibular system.

The saccule is one of the vestibular sensory organs of the inner ear. It detects head movements and provides information to maintain balance and orient in space. Despite its critical role, very little is known about its neurotransmission and regulation. Multiple disease entities and medications affect balance, which is why information on neurotransmission in the vestibular end organs including the saccule could have important pharmacological implications. To the best of our knowledge, this is the first paper to describe immunohistochemical expression of a large panel of neurotransmitters and receptors in the human saccule. Saccular tissue was sampled freshly during surgery. Based partly on previous findings in non-humans and partly on potential biological relevance, the neurotransmitters cholecystokinin, dopamine, GABA, glutamate, histamine and serotonin as well as receptors for these were selected for the tested panel. The neuroepithelium expressed glutamate receptor 1 (GluR1), metabotropic glutamate receptor (mGluR), GABA A receptor α (GABAARα), GABA B receptor 2 and cholecystokinin receptor B (CCKBR), whereas l-glutamate, GluR1, CCKBR, GABAARα, dopamine and serotonin receptor 1D were expressed in the subepithelial stroma. The non-sensory epithelium expressed GluR1, mGluR, histamine receptor 3, CCKAR and dopamine transporter. These findings provide a basis for pharmacological research and potential drug development.

Assessing competence in cochlear implant surgery using the newly developed Cochlear Implant Surgery Assessment Tool.

PURPOSE: To develop and gather validity evidence for a novel tool for assessment of cochlear implant (CI) surgery, including virtual reality CI surgery training. METHODS: Prospective study gathering validity evidence according to Messick's framework. Four experts developed the CI Surgery Assessment Tool (CISAT). A total of 35 true novices (medical students), trained novices (residents) and CI surgeons performed two CI-procedures each in the Visible Ear Simulator, which were rated by three blinded experts. Classical test theory and generalizability theory were used for reliability analysis. RESULTS: The CISAT significantly discriminated between the three groups (p < 0.001). The generalizability coefficient was 0.76 and most of the score variance (53.3%) was attributable to the participant and only 6.8% to the raters. When exploring a standard setting for CI surgery, the contrasting groups method suggested a pass/fail score of 36.0 points (out of 55), but since the trained novices performed above this, we propose using the mean CI surgeon performance score (45.3 points). CONCLUSION: Validity evidence for simulation-based assessment of CI performance supports the CISAT. Together with the standard setting, the CISAT might be used to monitor progress in competency-based training of CI surgery and to determine when the trainee can advance to further training.