Minimum intraoperative testing battery for cochlear implantation: the international practice trend.

PURPOSE: In cochlear implantation (CI) surgery, there are a wide variety of intraoperative tests available. However, no clear guide exists on which tests must be performed as the minimum intraoperative testing battery. Toward this end, we studied the usage patterns, recommendations, and attitudes of practitioners toward intraoperative testing. METHODS: This study is a multicentric international survey of tertiary referral CI centers. A survey was developed and administered to a group of CI practitioners (n = 34) including otologists, audiologists and biomedical engineers. Thirty six participants were invited to participate in this study based on a their scientific outputs to the literature on the intraoperative testing in CI field and based on their high load of CI surgeries. Thirty four, from 15 countries have accepted the invitation to participate. The participants were asked to indicate the usage trends, perceived value, influence on decision making and duration of each intraoperative test. They were also asked to indicate which tests they believe should be included in a minimum test battery for routine cases. RESULTS: Thirty-two (94%) experts provided responses. The most frequently recommended tests for a minimum battery were facial nerve monitoring, electrode impedance measurements, and measurements of electrically evoked compound action potentials (ECAPs). The perceived value and influence on surgical decision-making also varied, with high-resolution CT being rated the highest on both measures. CONCLUSION: Facial nerve monitoring, electrode impedance measurements, and ECAP measurements are currently the core tests of the intraoperative test battery for CI surgery.

Intralabyrinthine Schwannoma Mimicking Posterior Canalithiasis.

Intralabyrinthine schwannomas are a rare subgroup of vestibular schwannomas located within the membranous labyrinth of the inner ear and are known for their variable clinical presentations and symptoms. In the present study, we report on a patient with a persistent history of dizziness and positional vertigo, who was misdiagnosed with posterior canalithiasis. As hearing loss was not developed until late in the disease course, the patient was not properly diagnosed until magnetic resonance imaging revealed an intralabyrinthine schwannoma, which was not discovered on earlier imaging. In addition to the unusual clinical presentation, we describe the audio-vestibular profile of our patient. We suggest that a thorough vestibular evaluation, including caloric testing and a careful examination of the inner ear on imaging, is warranted in cases of treatment of refractory vertigo, even in patients where a diagnosis seems certain.

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 (WRS 65 ) 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. WRS 65 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.

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.

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.

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.

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.

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.

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.

Global Incidence of Sporadic Vestibular Schwannoma: A Systematic Review.

OBJECTIVE: Ubiquitous throughout the literature and during patient counseling, vestibular schwannoma is often quoted to affect about 1 per 100,000 people. Yet, reports from distinct international populations suggest that the incidence is likely much higher. The objective of the current work was to systematically characterize the global incidence of sporadic vestibular schwannoma. DATA SOURCES: Scopus, Embase, and PubMed. REVIEW METHODS: Population-based studies reporting incidence rates of sporadic vestibular schwannoma between January 2010 and August 2020 were searched with language restrictions requiring reports to be published in Chinese, English, German, Italian, or Spanish. The protocol was registered with PROSPERO (CRD42021228208) prior to commencement of data collection. PRISMA guidelines for transparent reporting of systematic reviews were followed. RESULTS: Among 424 citations, 6 publications covering 4 distinct populations from Denmark, the Netherlands, Taiwan, and the United States met inclusion criteria. Most recent incidence rates of among all ages ranged between 3.0 and 5.2 per 100,000 person-years. Highest incidence rates were reported among patients aged ≥70 years, peaking at 20.6 per 100,000 person-years. One study from the United States reported the incidence of asymptomatic, incidentally diagnosed tumors at a rate of 1.3 per 100,000 person-years from 2012 to 2016. CONCLUSIONS: Recent international incidence rates of sporadic vestibular schwannoma exceed the commonly quoted "1 per 100,000" figure by up to 5-fold among all ages and by up to 20-fold among age groups at highest risk. Based on modern incidence rates, the lifetime prevalence of developing sporadic vestibular schwannoma likely exceeds 1 per 500 persons.

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.

Cochlear implantation in Branchiootorenal syndrome - case report and review of the literature.

INTRODUCTION: Branchiootorenal syndrome (BOR) manifests with branchial fistulae, otological anomalies and renal dysplasia. Management remains largely symptomatic. The most common consequence of BOR is mild-to-profound sensorineural, conductive or mixed hearing loss, where conventional hearing aids (HA) provide limited benefit. Cochlear implantation for BOR-associated hearing loss is an emerging treatment. Existing literature remains limited, centring around paediatric cases. OBJECTIVES: This paper is the first to present a case of an adult/non-paediatric BOR patient receiving bilateral cochlear implants (CI), including pre- and postoperative objective and subjective hearing outcomes. Additionally, an overview of reported cases of cochlear implantation in BOR/BO patients is given. METHODS: A review of the case patient's medical charts from initial visit at our tertiary center to latest follow-up was conducted. A search of the Medline database resulted in four papers on cochlear implantation in BOR/BO patients, forming the basis of a literature review on this topic. RESULTS: Excellent hearing outcomes were achieved after bilateral CI despite the patient's inner ear malformation. DISCUSSION: The case patient's outcomes were comparable to results reported for unselected adult, non-syndromic CI-recipients with normal inner ear anatomy. CONCLUSION: BOR patients experiencing insufficient benefit from conventional HA should be considered for cochlear implantation.

Cochlear Implantation After Radiotherapy of Vestibular Schwannomas.

BACKGROUND AND OBJECTIVES: Vestibular schwannomas (VS) frequently lead to ipsilateral sensorineural hearing loss (HL) as part of its natural history or as a result of treatment. Cochlear implantation represents a well-documented treatment of profound HL that cannot be treated adequately with a conventional hearing aid, thus being offered to selected VS patients. A functional cochlea and cochlear nerve are prerequisites for sound perception with a cochlear implant (CI). The potential impact of radiotherapy on these structures is thus an important issue for subsequent CI hearing outcomes. The objective of this article is to present a case and to review the existing literature on the outcomes of cochlear implantation in irradiated VS patients systematically. METHODS: A systematic literature review using preferred reporting items for systematic reviews and meta-analyses was conducted. Medline was searched systematically. Papers reporting ipsilateral CI outcomes after radiotherapy of VS were included. Additionally, results of CI after stereotactic radiotherapy in a 54-year-old male with neurofibromatosis type 2 are presented. RESULTS: A total of 14 papers (33 patients) fulfilled inclusion criteria. Moderate preoperative HL was found in 11 patients. Six had moderate to severe HL, whereas 16 had severe HL or total deafness. Postoperative hearing outcomes varied from poor in 27% of patients to excellent in 19%, with remaining cases lying in between (mean follow-up of 19 months). Most patients achieved improvement in hearing and quality of life. CONCLUSION: Despite variation in the degree of hearing outcome, CI after radiotherapy of VS appears to be effective in the majority of cases, as more than 70% of patients have good or excellent outcomes within 1-2 years post-implantation. Subjective benefits are considerable, even in cases with relatively poor objective outcome.

Natural History of Growing Sporadic Vestibular Schwannomas During Observation: An International Multi-Institutional Study.

OBJECTIVE: Active treatment of small- or medium-sized vestibular schwannoma during wait-and-scan management is currently recommended at most centers globally once growth is detected. The primary aim of the current study was to characterize the natural history of growing sporadic vestibular schwannoma during observation. STUDY DESIGN: Cohort study. SETTING: Four tertiary referral centers across the United States and Denmark. PATIENTS: Patients with two prior MRI scans demonstrating ≥2 mm of linear growth who continued observational management. INTERVENTION: Observation with serial imaging. MAIN OUTCOME MEASURE: Subsequent linear growth-free survival (i.e., an additional ≥2 mm of growth) following initial growth of ≥2 mm from tumor size at diagnosis. RESULTS: Among 3,402 patients undergoing observation, 592 met inclusion criteria. Median age at initial growth was 66 years (IQR 59-73) for intracanalicular tumors (N = 65) and 62 years (IQR 54-70) for tumors with cerebellopontine angle extension (N = 527). The median duration of MRI surveillance following initial detection of tumor growth was 5.2 years (IQR 2.4-6.9) for intracanalicular tumors and 1.0 year (IQR 1.0-3.3) for cerebellopontine angle tumors. For intracanalicular tumors, subsequent growth-free survival rates (95% CI; number still at risk) at 1, 2, 3, 4, and 5 years following the initial MRI that demonstrated growth were 77% (67-88; 49), 53% (42-67; 31), 46% (35-60; 23), 34% (24-49; 17), and 32% (22-47; 13), respectively. For cerebellopontine angle tumors, subsequent growth-free survival rates were 72% (68-76; 450), 47% (42-52; 258), 32% (28-38; 139), 26% (21-31; 82), and 22% (18-28; 57), respectively. For every 1 mm increase in magnitude of growth from diagnosis to tumor size at detection of initial growth, the HRs associated with subsequent growth were 1.64 (95% CI 1.25-2.15; p < 0.001) for intracanalicular tumors and 1.08 (95% CI 1.01-1.15; p = 0.02) for cerebellopontine angle tumors. CONCLUSIONS: Growth detected during observation does not necessarily portend future growth, especially for slowly growing tumors. Because early treatment does not confer improved long-term quality of life outcomes, toleration of some growth during observation is justifiable in appropriately selected cases.

Intracochlear Vestibular Schwannoma Presenting with Mixed Hearing Loss.

As for other vestibular schwannomas, intralabyrinthine schwannomas commonly cause a sensorineural hearing loss, contrary to more lateral ear pathology that can cause conductive or mixed hearing loss. This case report features a patient that presented with a mixed and thus partly pseudo-conductive hearing loss due to an intracochlear schwannoma, a finding that is very rare. As a result, the patient was initially misdiagnosed as having otosclerosis and a stapedotomy was performed, without hearing improvement. We discuss the clinical implications of this atypical presentation, which illustrates the importance of performing supplementary audiological testing (e.g., the Gellé test), and the importance of considering vestibular system testing when otosclerosis is suspected. In addition, the importance of imaging and considering differential diagnoses in cases of conductive hearing loss is stressed.