Single-cell RNA sequencing analysis of vestibular schwannoma reveals functionally distinct macrophage subsets.

BACKGROUND: Vestibular schwannomas (VSs) remain a challenge due to their anatomical location and propensity to growth. Macrophages are present in VS but their roles in VS pathogenesis remains unknown. OBJECTIVES: The objective was to assess phenotypic and functional profile of macrophages in VS with single-cell RNA sequencing (scRNAseq). METHODS: scRNAseq was carried out in three VS samples to examine characteristics of macrophages in the tumour. RT-qPCR was carried out on 10 VS samples for CD14, CD68 and CD163 and a panel of macrophage-associated molecules. RESULTS: scRNAseq revealed macrophages to be a major constituent of VS microenvironment with three distinct subclusters based on gene expression. The subclusters were also defined by expression of CD163, CD68 and IL-1ß. AREG and PLAUR were expressed in the CD68+CD163+IL-1ß+ subcluster, PLCG2 and NCKAP5 were expressed in CD68+CD163+IL-1ß- subcluster and AUTS2 and SPP1 were expressed in the CD68+CD163-IL-1ß+ subcluster. RT-qPCR showed expression of several macrophage markers in VS of which CD14, ALOX15, Interleukin-1ß, INHBA and Colony Stimulating Factor-1R were found to have a high correlation with tumour volume. CONCLUSIONS: Macrophages form an important component of VS stroma. scRNAseq reveals three distinct subsets of macrophages in the VS tissue which may have differing roles in the pathogenesis of VS.

Outcomes of cochlear implantation in Usher syndrome: a systematic review.

PURPOSE: This study is a systematic review of the literature which seeks to evaluate auditory and quality of life (QOL) outcomes of cochlear implantation in patients with Usher syndrome. METHODS: Systematic review of studies indexed in Medline via PubMed, Ovid EMBASE, Web of Science, CENTRAL and clinicaltrials.gov was performed up to March 9th 2022, conducted in accordance with the PRISMA statement. Patient demographics, comorbidity, details of cochlear implantation, auditory, and QOL outcomes were extracted and summarized. RESULTS: 33 studies reported over 217 cochlear implants in 187 patients with Usher syndrome, comprising subtypes 1 (56 patients), 2 (9 patients), 3 (23 patients), and not specified (99 patients). Auditory outcomes included improved sound detection, speech perception, and speech intelligibility. QOL outcomes were reported for 75 patients, with benefit reported in the majority. CONCLUSIONS: Many patients with Usher syndrome develop improved auditory outcomes after cochlear implantation with early implantation being an important factor.

Fibroblasts Derived From Vestibular Schwannoma Express Protumorogenic Markers.

BACKGROUND AND AIM: Vestibular schwannomas (VSs), despite being histologically benign, cause significant morbidity because of their challenging intracranial location and the propensity for growth. The role of the stroma and particularly fibroblasts, in the progression of VS, is not completely understood. This study examines the profile of fibroblasts in VS. METHODS: Seventeen patients undergoing surgical excision of VS were recruited into the study. Reverse transcription with quantitative polymerase chain reaction (RT-qPCR) was performed on VS tissue samples and fibroblast-associated molecules examined. Immunofluorescence and immunohistochemistry in VS tissue were used to study the expression of fibroblast markers CD90 and podoplanin in situ. Fibroblast cultures were established from VS, and RT-qPCR analysis was performed on a panel of fibroblast markers on VS and control tissue fibroblasts. RESULTS: Several fibroblast-associated molecules including members of galectin family and matrix metalloproteinases were found to be expressed in VS tissue on RT-qPCR analysis. In situ, expression of CD90 and podoplanin was observed in VS tissue both on immunohistochemistry and immunofluorescence. RT-qPCR analysis of fibroblasts from VS and control vestibular neuroepithelium (NE) showed a higher expression of several molecules of the galectin and matrix metalloproteinases family on VS fibroblasts compared with NE fibroblasts. CONCLUSION: This work examines fibroblasts from VS and shows qualitative differences from NE fibroblasts on RT-qPCR. Further understanding of the fibroblast function in the progression of VS will potentially unveil new targets to manage VS growth.

Long-term osseointegration of laser-ablated hearing implants in sheep cranial bone.

Osseointegration, the ability for an implant to be anchored in bone tissue with direct bone-implant contact and allowing for continuous adaptive remodelling, is clinically used in different reconstructive fields, such as dentistry, orthopedics and otology. The latter uses a bone conducting sound processor connected to a skin-penetrating abutment that is mounted on a titanium implant placed in the temporal bone, thereby acting as a path for transmission of the vibrations generated by the sound processor. The success of the treatment relies on bone healing and osseointegration, which could be improved by surface modifications. The aim of this study was to evaluate the long-term osseointegration in a sheep skull model and compare a laser-ablated implant surface with a machined implant. Commercially available 4 mm titanium implants, either with a machined (Wide Ponto) or a laser-ablated surface (Ponto BHX, Oticon Medical, Sweden), were used in the current study. The surfaces were evaluated by scanning electron microscopy. The implantation was performed with a full soft tissue flap and the osteotomy was prepared using the MIPS drill kit (Oticon Medical, Sweden) prior to installation of the implants in the frontal bone of eight female sheep. After five months, biopsies including the implant and surrounding bone tissue obtained, processed and analysed using histology, histomorphometry, scanning electron microscopy and Raman spectroscopy. The animals healed well, without signs of adverse events. Histomorphometry showed a large amount of bone tissue around both implant types, with 75% of the threaded area occupied by bone for both implant types. A large amount of bone-implant contact was observed for both implant types, with 67%-71% of the surface covered by bone. Both implant types were surrounded by mature remodelled lamellar bone with high mineral content, corroborating the histological observations. The current results show that the laser-ablated surface induces healing similar to the well-known clinically used machined surface in ovine cranial bone. In conclusion, the present long-term experimental results indicate that a laser-ablated implant performs equally well as a clinically used implant with a machined surface. This, together with previously reported, improved early biomechanical anchorage, suggests future, safe and efficient clinical potential.

Anatomical and audiological considerations in branchiootorenal syndrome: A systematic review.

Objective: Establish anatomical considerations, audiological outcomes, and optimal management in patients with branchiootic/branchiootorenal syndrome (BO/BOR). Methods: Databases reviewed: Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. Clinical or radiological studies of patients with BOR syndrome describing either the audiological profile or anatomical changes were included. Articles in which BOR syndrome was associated with other syndromes, and those that were focused only on general and genetic aspects of BOR syndrome were excluded. Articles were assessed using Oxford Centre for Evidence-Based Medicine (OCEBM) grading system and the Brazzelli risk of bias tool for nonrandomized studies. Results: Searches identified 379 articles. Of these, 64 studies met the inclusion criteria, reporting outcomes in 482 patients from at least 95 families. In 308 patients, hearing loss was categorized as sensorineural (29%), conductive (20%), and mixed (51%). Hearing outcomes were variable in terms of onset, pattern, and severity; ranging from mild to profound deafness. One hundred sixty-nine patients presented with inner ear anomalies, 145 had middle, and 151 had external ear abnormalities. In 44 studies, 58 ear operations were described. Mixed outcomes were reported in patients managed with hearing aids or middle ear surgery; however, successful cochlear implantation was described in all five cases. Conclusion: The anatomical and audiological profiles of patients with BO/BOR are variable. A range of surgical procedures were described, however lacked objective outcome measures. Given the range of anatomical variants, management decisions should be made on an individual basis including full audiological and radiological assessment. Level of evidence: NA.

Congenital Mastoid Cholesteatoma.

BACKGROUND: Congenital cholesteatomas account for just up to 5% of all cholesteatomas and most commonly arise in the petrous apex and middle ear. Congenital cholesteatomas arising in the mastoid are rare and typically present late. METHODS: In this study, we report a case series of 3 cases managed in our department between 2006 and 2021 and present a summary of the current literature. RESULTS: Congenital cholesteatomas arising in the mastoid is a rare finding and even among reported cases, not all are clearly mastoid in origin. Their location allows for considerable growth before symptoms develop. Pain and localized swelling in the temporal area are the most common presenting symptoms which can lead to diagnostic challenges. Our cases show that although surgery is often appropriate, conservative manage- ment may be suitable in certain situations. CONCLUSION: Congenital cholesteatoma of mastoid origin is rare and can present a diagnostic challenge. Greater awareness is important to facilitate early detection. A high index of suspicion is needed in those presenting with retro-auricular pain and swelling in the context of a normal ontological examination.

Development and validation of an improved classification and risk stratification system for carotid body tumors: Multinational collaborative cohort study.

BACKGROUND: This study aims to develop and validate a new classification system that better predicts combined risk of neurological and neurovascular complications following CBT surgery, crucial for treatment decision-making. METHODS: Multinational retrospective cohort study with 199 consecutive cases. A cohort of 132 CBT cases was used to develop the new classification. To undertake external validation, assessment was made between the actual complication rate and predicted risk by the model on an independent cohort (n = 67). RESULTS: Univariate analyses showed statistically significant associations between developing a complication and the following factors: craniocaudal dimension, volume, Shamblin classification, and Mehanna types. In the multivariate prognostic model, only Mehanna type remained as a significant risk predictor. The risk of developing complications increases with increasing Mehanna type. CONCLUSIONS: We have developed and then validated a new classification and risk stratification system for CBTs, which demonstrated better prognostic power for the risk of developing neurovascular complications after surgery.

Hearing outcome measures for conductive and mixed hearing loss treatment in adults: a scoping review.

OBJECTIVE: Rehabilitation options for conductive and mixed hearing loss are continually expanding, but without standard outcome measures comparison between different treatments is difficult. To meaningfully inform clinicians and patients core outcome sets (COS), determined via a recognised methodology, are needed. Following our previous work that identified hearing, physical, economic and psychosocial as core areas of a future COS, the AURONET group reviewed hearing outcome measures used in existing literature and assigned them into different domains within the hearing core area. DESIGN: Scoping review. STUDY SAMPLE: Literature including hearing outcome measurements for the treatment of conductive and/or mixed hearing loss. RESULTS: The literature search identified 1434 studies, with 278 subsequently selected for inclusion. A total of 837 hearing outcome measures were reported and grouped into nine domains. The largest domain constituted pure-tone threshold measurements accounting for 65% of the total outcome measures extracted, followed by the domains of speech testing (20%) and questionnaires (9%). Studies of hearing implants more commonly included speech tests or hearing questionnaires compared with studies of middle ear surgery. CONCLUSIONS: A wide range of outcome measures are currently used, highlighting the importance of developing a COS to inform individual practice and reporting in trials/research.

Skin flap thickness and magnet strength in Cochlear implants.

Objectives: The new CI600 series cochlear implants (Nucleus® Profile™ Plus Series; CI) are more MRI (magnetic resonance imaging) compatible. The magnet's attraction force is lower, possibly posing a problem in patients needing a higher strength magnet due to increased skin thickness. This study aims to investigate the relationship between skin thickness and magnet strength in patients undergoing cochlear implantation. Methods: This cross-sectional study used data from 46 patients with cochlear implants. Data collected included age, body mass index, magnet strength used, pre-operative and post-operative imaging and skin thickness. Results: There was a weak, positive correlation between magnet strength and skin thickness, which was not statistically significant (r(46) = 0.149, p = 0.324). There was a weak, positive correlation between magnet strength and body mass index, which was not statistically significant (r(46) = 0.113, p = 0.456). There was a moderate, positive correlation between body mass index and skin thickness, which was statistically significant (r(46) = 0.362, p = 0.012). Discussion: The increased skin thickness in patients with higher body mass indexes, might mean that these patients will require a stronger magnet. Conclusion: There are concerns that the lower attraction force in the CI600 (Nucleus® Profile™ Plus Series) may pose a retention problem for these patients. This might mean that discussions about skin flap reduction surgery are needed.

Cochlear Implantation Outcomes in Post Synaptic Auditory Neuropathies: A Systematic Review and Narrative Synthesis.

To establish outcomes following cochlear implantation (CI) in patients with postsynaptic auditory neuropathy (AN). Systematic review and narrative synthesis. Databases searched: MEDLINE, PubMed, EMBASE, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Review conducted in accordance with the PRISMA statement. Searches identified 98 studies in total, of which 14 met the inclusion criteria reporting outcomes in 25 patients with at least 28 CIs. Of these, 4 studies focused on Charcot-Marie-Tooth disease (CMT), 3 on Brown-Vialetto-Van-Laere syndrome (BVVL), 2 on Friedreich Ataxia (FRDA), 2 on Syndromic dominant optic atrophy (DOA+), 2 on Cerebellar ataxia - areflexia - pes cavus - optic atrophy - sensorineural hearing loss (CAPOS) syndrome, and 1 on Deafness-dystonia-optic neuronopathy (DDON) syndrome. All studies were Oxford Centre for Evidence Based Medicine (OCEBM) grade IV. Overall trend was towards good post-CI outcomes with 22 of the total 25 patients displaying modest to significant benefit. Hearing outcomes following CI in postsynaptic ANs are variable but generally good with patients showing improvements in hearing thresholds and speech perception. In the future, development of a clearer stratification system into pre, post, and central AN would have clinical and academic benefits. Further research is required to understand AN pathophysiology and develop better diagnostic tools for more accurate identification of lesion sites. Multicenter longitudinal studies with standardized comprehensive outcome measures including health-related quality of life data will be key in establishing a better understanding of short and long-term post-CI outcomes.

Outcomes of Cochlear Implantation in Patients with Jervell and Lange-Nielsen Syndrome: A Systematic Review and Narrative Synthesis.

Establish outcomes following cochlear implantation (CI) in patients with Jervell and Lange-Nielsen Syndrome (JLNS). Methods Systematic review and narrative synthesis. Databases searched on Medline, Pubmed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Review conducted in accordance with the PRISMA statement. Searches identified 63 abstracts and 19 full texts. Of these, 9 studies met inclusion criteria reporting outcomes in 66 patients with at least 72 implants. Hearing outcomes were generally good. Mortality secondary to cardiac complications within the follow up period occurred in at least five cases (7.6%), though three of these were thought to be unrelated to surgery. Potentially dangerous arrhythmias without associated morbidity were also noted in at least five patients. The methodological quality of included studies was modest, predominantly consisting of case reports and non-controlled case series with small numbers of patients. All studies were OCEBM grade IV. One study contributed 41/66 patients (62%). Hearing outcomes following CI in JLNS are generally good with the majority of patients experiencing useful hearing improvement. Significant peri-operative cardiac risks exist and should be discussed with the patient and family during pre-operative counselling and prompt thorough investigation, pre-operative optimisation and peri-operative monitoring.

Results of a 2-Year Prospective Multicenter Study Evaluating Long-term Audiological and Clinical Outcomes of a Transcutaneous Implant for Bone Conduction Hearing.

OBJECTIVE(S): To evaluate 2-year audiological and clinical outcomes of a transcutaneous implant for bone conduction hearing of a previously published 6-month evaluation. DESIGN: Fifty-four unilaterally implanted adult patients with conductive or mild mixed hearing loss or single-sided sensorineural deafness were included in this prospective multicenter study. Follow-up visits were scheduled post-surgery at 10 days; 4, 6, and 12 weeks; 6, 12, and 24 months. Main outcomes were audiological benefit, patient-reported outcomes (PROs), soft tissue status, pain, numbness, implant survival, and daily usage. RESULTS: In the study population, the transcutaneous implant resulted in statistically significant improvement in objective hearing test and PROs compared with the unaided situation. Soft tissue complications were observed in 4.6% of the patients per visit. Pain/discomfort and numbness were initially reported in the majority of the patients, but declined over time; approximately 9% of patients reported some degree of numbness and 15% (slight) pain/discomfort after 2 years. During the 24-month period, two implant magnets were removed (3.7%), while two other implants were converted to the percutaneous counterpart (3.7%). At the final visit, 89.6% (n = 42 out of 47) of the patients used their sound processor, with a median daily usage of 6 h/d (range, 0-18 h/d). CONCLUSIONS: After 24 months, the transcutaneous implant provided statistically significant mean improvement in objective and subjective hearing performance as well as PROs compared with the preoperative unaided condition and had a low soft tissue complication rate. The test device could be considered as an alternative treatment option for appropriately selected and counseled patients.

Comparing Ocular Responses to Caloric Irrigation and Electrical Vestibular Stimulation in Vestibular Schwannoma.

Electrical Vestibular Stimulation (EVS) is a non-invasive technique for activating the vestibular-ocular reflex, evoking mainly a torsional eye movement response. We have previously demonstrated that this response can be used to detect vestibular asymmetry in patients with vestibular schwannoma (VS). Here we perform a direct comparison of EVS with caloric irrigation in this patient group. We studied 30 patients with unilateral VS, alongside an equal number of aged-matched healthy control subjects. EVS current was delivered to the mastoid process in a monaural configuration using a sinusoidal stimulus (2 Hz; ± 2 mA; 10 s), with an electrode placed over the spinous C7 process. Evoked eye movements were recorded from the right eye in darkness using an infra-red sensitive camera while the subject sat relaxed with their head on a chinrest. Ocular torsion was subsequently tracked off-line using iris striations. Each subject separately underwent water caloric irrigation, in accordance with the British Society of Audiology guidelines. For the caloric test, eye movement was recorded in the yaw axis using electro-oculography. For both EVS and calorics, inter-aural response asymmetry was calculated to determine the extent of canal paresis. Both tests revealed impaired vestibular function in the ipsilesional ear of VS patients, with a mean asymmetry ratio of 15 ± 17% and 18 ± 16% for EVS and calorics, respectively. Overall, the caloric test results discriminated controls from patients slightly more effectively than EVS (Cohen's D effect size = 1.44 vs. 1.19). Importantly, there was a significant moderate correlation between the AR values produced by EVS and calorics (r = 0.53, p < 0.01), and no significant difference between mean AR estimates. When questioned, ≥85% of participants subjectively preferred the EVS experience, in terms of comfort. Moreover, it took ~15 min to complete, vs. ~1 h for caloric. These results confirm that the results of the EVS test broadly agree with those of caloric irrigation, in terms of detecting vestibular asymmetry. Furthermore, they suggest a higher degree of convenience and patient comfort.

Novel Minimal Access Bone Anchored Hearing Implant Surgery and a New Surface Modified Titanium Implant, the Birmingham Experience.

BACKGROUND: Bone anchored hearing implants (BAHI) are widely used and highly successful, accompanied with a high level of patient satisfaction across most techniques. A large UK teaching hospital switched from the previously used wide diameter titanium fixture and drilling system to the novel minimally invasive technique and laser ablated titanium implant. Before this change the rates of fixture failure and skin problems necessitating abutment change were 1% each. METHODS: Retrospective case note review of consecutive BAHI patients drawn from an electronic database between January 2015 and October 2016. RESULTS: Data from a total of 118 procedures were reviewed, with different combinations of surgical techniques and implant types. Sixty procedures were performed via the novel minimally invasive technique with 21 failures (35%). Fourty-eight modified minimally invasive technique procedures were performed with seven failures. In 64 of the procedures, laser ablated titanium fixtures were placed with 21 failures (32.8%). In 54 procedures wide diameter titanium fixtures were placed with eight failures (5%). CONCLUSION: Initial experience with the novel minimally invasive technique and laser ablated titanium fixture showed significantly higher failure rates than expected. This prompted a change to an open technique and subsequent abandonment of the laser ablated titanium fixture and custom drilling solution in our institution.

Inter-rater Reliability and Validity of Holgers Scores for the Assessment of Bone-anchored Hearing Implant Images.

OBJECTIVES: This study aims to review the utility and interassessor reliability of Holgers classification by simultaneously testing various professionals of the bone-anchored implant team for their impression of a series of randomized images. STUDY DESIGN: Retrospective review of a randomized series of bone-anchored implant fixture clinical photographs from the database at a tertiary referral university hospital. Raters were blinded to the contemporaneous Holgers grading assigned by the Clinical Nurse Specialist at initial assessment. Multivariate analysis was performed for correlation between scores for assessors and between grades of assessor. SETTING: Queen Elizabeth Hospital, Birmingham, UK a tertiary center for BAHIs. PATIENTS: Patients implanted from May 2012 until November 2014. MAIN OUTCOME MEASURE: Photographs of fixture sites of adult patients were taken following bone-anchored hearing implant surgery using either a tissue reduction (a split skin graft or linear incision technique was used) or tissue preservation approach, at 1 week, 6 months, and 12 months postoperatively. On a single occasion 263 images were reviewed by 10 assessors (2 consultants, 2 higher surgical trainees, 3 junior doctors, and 3 audiologists). Images were displayed at 10-second intervals and were scored by each assessor. Assessors were blinded to patient identity, time points and to each other's scores. Results were then compared against the real-time scoring of Holgers grades done by the BAHI specialist nurse to compare scores. RESULTS: Overall 227 (86.2%) images were with tissue reduction technique of which 110 (41.8%) were with linear incision and 117 (44.4%) were with a split skin graft (SSG); and 36 (13.6%) were with tissue preservation technique. Of these 263 images, 104 were at 1 week (39.5%), 70 were at 6 months (26.6%), and 89 were at 12 months (33.9%). The cumulative scores for each grade scored by blinding the time points were: 0 = 1132 (43.04%), total 1 = 995 (37.83%), total 2 = 346 (13.15%), total 3 = 141 (5.36%), total 4 = 16 (0.6%). 2630 data points had a variance of only 0.6415 for each nominal. Multivariate correlation between all assessors was r =0.7230 (Pearson's R). Correlations between consultants r=0.6317, higher surgical trainees r=0.7351, junior doctors r=0.7599, and audiologists r=0.7981.There is a good correlation (r=0.89) with no statistically significant differences between the SSG and linear incision groups (p>0.05), possibly suggesting Holgers score is comparable within both these tissue reduction techniques.There is a moderate correlation (r=0.58) with statistically significant differences between tissue preservation versus tissue reduction groups (p<0.05), possibly suggesting tissue preservation gives better results with lower Holgers scores than tissue reduction. CONCLUSION: Holgers scoring system is a reliable tool with respect to inter-rater variability across all levels of experience. Correlation was closer with audiologists and lesser experienced assessors.

Audiological and clinical outcomes of a transcutaneous bone conduction hearing implant: Six-month results from a multicentre study.

OBJECTIVES: To compare the hearing performance of patients with conductive and mild mixed hearing loss and single-sided sensorineural deafness provided with a new transcutaneous bone conduction hearing implant (the Baha Attract System) with unaided hearing as well as aided with a sound processor on a softband. Furthermore, to evaluate safety and subjective benefit before and after implantation of the test device. PARTICIPANTS: Fifty-four adult patients in five participating centres were enrolled in this prospective study. Baseline data were collected during a pre-operative visit, and after a softband trial, all patients were implanted unilaterally. Follow-up visits were scheduled at 10 days, 4, 6, 12 weeks and 6 months. MAIN OUTCOME MEASURES: Free-field hearing thresholds pure-tone average (PTA4 in dB HL; mean threshold at 500, 1000, 2000, 4000 Hz; primary outcome measure). Individual free-field hearing thresholds, speech recognition in quiet and in noise, soft tissue status during follow-up and subjective benefit as measured with the Abbreviated Profile of Hearing Aid Benefit (APHAB), Speech, Spatial and Qualities of Hearing Scale (SSQ) and Health Utilities Index (HUI) questionnaires. RESULTS: Implantation of the Baha Attract System resulted in favourable audiological outcomes compared to unaided conditions. On the primary outcome parameter, a statistically significant improvement was observed compared to unaided hearing for the patients with conductive/mixed hearing loss (mean PTA4 difference -20.8 dB HL, SD 9.8; P < 0.0001) and for the patients with single-sided sensorineural deafness (SSD) (mean PTA4 difference -21.6 dB HL, SD 12.2; P < 0.0001). During all audiology tests, the non-test ear was blocked. Statistically significant improvements were also recorded in speech tests in quiet and noise compared to unaided hearing for the conductive/mixed hearing loss group and for speech in quiet in the SSD group. Compared to the pre-operative measurement with softband, no significant differences were recorded in the PTA4 free-field hearing threshold or the other audiological outcomes in either of the groups (P > 0.05). Soft tissue-related issues observed during follow-up included numbness, pain/discomfort at the implant site and to a lesser extent pressure-related skin complications. A declining trend was noted in the rate of these complications during follow-up. Approximately 20% of patients reported some degree of numbness and 38% (slight) pain/discomfort at final follow-up of 6 months. Good results on the subjective benefit questionnaires were observed, with statistically significant improvements on APHAB and SSQ questionnaires, and on the hearing attribute of HUI3. CONCLUSIONS: The Baha Attract System provided a significant improvement in hearing performance and subjective benefit compared to the pre-operative unaided condition (with the non-test ear blocked). Hearing performance of the Baha Attract was similar to a test situation with the same sound processor on a softband. A proportion of the patients reported numbness and pain/discomfort at the implant site during follow-up, especially during the first post-operative weeks. Based on the results of the current multicentre study, the Baha Attract can be considered as a treatment option for patients with the aforementioned hearing losses. Especially in the SSD patients, a careful selection procedure is warranted. Therefore, a pre-operative trial should be part of the decision-making process before fitting a patient with the Baha Attract System.

Ocular torsion responses to electrical vestibular stimulation in vestibular schwannoma.

OBJECTIVES: We determined if eye movements evoked by Electrical Vestibular Stimulation (EVS) can be used to detect vestibular dysfunction in patients with unilateral vestibular schwannoma (VS). METHODS: Ocular torsion responses to monaural sinusoidal EVS currents (±2 mA, 2 Hz) were measured in 25 patients with tumours ranging in size from Koos grade 1-3. For comparative purposes we also measured postural sway response to EVS, and additionally assessed vestibular function with the lateral Head Impulse Test (HIT). Patient responses were compared to age-matched healthy control subjects. RESULTS: Patients exhibited smaller ocular responses to ipsilesional versus contralesional EVS, and showed a larger asymmetry ratio (AR) than control subjects (19.4 vs. 3.3%, p < 0.05). EVS-evoked sway responses were also smaller in ipsilesional ear, but exhibited slightly more variability than the eye movement response, along with marginally lower discriminatory power (patients vs. controls: AR = 16.6 vs 2.6%, p < 0.05). The HIT test exhibited no significant difference between groups. CONCLUSIONS: These results demonstrate significant deficits in the ocular torsion response to EVS in VS patients. SIGNIFICANCE: The fast, convenient and non-invasive nature of the test are well suited to clinical use.

An Assessment of the Clinical Acceptability of Direct Acoustic Cochlear Implantation for Adults With Advanced Otosclerosis in the United Kingdom.

HYPOTHESIS: Assess the clinical acceptability of direct acoustic cochlear implantation for patients with advanced otosclerosis and the support for conducting a controlled trial of its effectiveness in the United Kingdom. BACKGROUND: Emerging evidence supports the efficacy of direct acoustic cochlear implantation in patients with advanced otosclerosis whose needs cannot be managed using the combination of stapes surgery and hearing aids. A controlled trial would provide evidence for its effectiveness and cost-effectiveness to healthcare commissioners. METHODS: An online survey of clinical professionals was constructed to characterize current standard of care for patients with advanced otosclerosis and to assess whether clinicians would be willing to refer patients into a trial to evaluate direct acoustic cochlear implantation. A consensus process was conducted to define inclusion criteria for the future trial. RESULTS: No survey respondent considered direct acoustic cochlear implantation to be inappropriate with a majority indicating that they would refer patients into a future trial. The consensus was that there is a lack of available treatment options for those patients with bone conduction thresholds worse than 55 dB HL and who did not meet current criteria for cochlear implantation. CONCLUSION: The present study confirms that a controlled trial to evaluate the effectiveness of direct acoustic cochlear implantation would have the support of clinicians in the United Kingdom. A feasibility study would be required to determine whether patients who meet the inclusion criteria could be recruited in a timely manner and in sufficient numbers to conduct a formal evaluation of effectiveness.