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Contains fulltext : 231715.pdf (Publisher’s version ) (Open Access) OBJECTIVES: Patients with a vestibular schwannoma (VS) experience a reduced quality of life (QoL). The main objective of this study was to determine the strongest predictors reducing physical and mental QoL from the disease-specific Penn Acoustic Neuroma Quality of Life (PANQOL) questionnaire in patients with VS. DESIGN: Observational study. SETTING: Radboudumc Skull Base Centre, Nijmegen. PARTICIPANTS: Patients newly diagnosed with VS between 2014 and 2017 managed with either observation, stereotactic radiosurgery or microsurgery. MAIN OUTCOME MEASURES: Quality of life was assessed using the disease-specific PANQOL and general Short-Form (36) Health Survey (SF-36). Multiple linear regression models with PANQOL domains as predictors were used to determine the strongest predictors for SF-36 QoL physical and mental health scores. Standardised beta coefficients (β) were used for ranking. RESULTS: A total of 174 patients (50% females, mean age 58.9 years) returned the questionnaires, providing a 69% response rate. Fifteen patients (9%) were treated with microsurgery, 29 (17%) with stereotactic radiosurgery and 130 patients (75%) were observed in a wait and scan strategy. A lack of energy (β = .28; P ≤ .001), lower general health (β = .22; P ≤ .001), headache (β = .16; P ≤ .001), anxiety (β = .15; P ≤ .001) and balance problems (β = .10; P ≤ .001) are the strongest predictors affecting physical health, while mental health is most affected by anxiety (β = .37; P ≤ .001), a lack of energy (β = .34; P ≤ .001), facial nerve dysfunction (β = .07; P ≤ .001), balance problems (β = .04; P ≤ .001) and headaches (β = .04; P ≤ .001). CONCLUSION: A lack of energy, anxiety, headache and balance problems are the strongest predictors of both SF-36 physical and mental QoL in patients with VS. More awareness and supportive care regarding energy, anxiety, headache and balance in informing, evaluating and treating patients with VS could improve QoL.

Contains fulltext : 229559.pdf (Publisher’s version ) (Open Access) OBJECTIVES: To develop a prediction model to predict vestibular schwannoma (VS) growth for patients in a wait and scan (W&S) strategy. DESIGN: Retrospective cohort study. SETTING: Tertiary hospital (Radboud university medical center, Nijmegen, the Netherlands). PARTICIPANTS: Patients with unilateral VS, entering a W&S strategy and at least one follow-up MRI available. Data on demographics, symptoms, audiometry and MRI characteristics at time of diagnosis were collected from medical records. MAIN OUTCOME MEASURES: Following multiple imputation, a multivariable Cox regression model was used to select variables, using VS growth (>/=2 mm) as outcome. Decision curve analyses (DCA) were performed to compare the model to the current strategy. RESULTS: Of 1217 analysed VS patients, 653 (53.7%) showed growth during follow-up. Balance complaints (HR 1.57 (95% CI: 1.31-1.88)) and tinnitus complaints in the affected ear (HR 1.36 (95% CI: 1.15-1.61)), Koos grade (Koos 1 is reference, Koos 2 HR 1.03 (95% CI: 0.80-1.31), Koos 3 HR 1.55 (95% CI: 1.16-2.06), Koos 4 HR 2.18 (95% CI: 1.60-2.96)), time since onset of symptoms (IQR HR 0.83 (95% CI: 0.77-0.88) and intrameatal diameter on MRI (IQR HR 1.67 (95% CI: 1.42-1.96)) were selected as significant predictors. The model's discrimination (Harrell's C) was 0.69 (95% CI: 0.67-0.71), and calibration was good. DCA showed that the model has a higher net benefit than the current strategy for probabilities of VS growth of >12%, 15% and 21% for the first consecutive 3 years, respectively. CONCLUSIONS: Patients with balance and tinnitus complaints, a higher Koos grade, short duration of symptoms and a larger intrameatal diameter at time of diagnosis have a higher probability of future VS growth. After external validation, this model may be used to inform patients about their prognosis, individualise the W&S strategy and improve (cost-)effectiveness.

OBJECTIVEGamma Knife radiosurgery (GKRS) has become an accepted treatment for vestibular schwannoma, with a high rate of tumor control and good clinical outcome. In a small number of cases, additional treatment is needed. This retrospective study examines the clinical outcome, reproducibility of volumetric response patterns, and tumor control rate after administering a second GKRS to treat vestibular schwannomas.METHODSA total of 38 patients were included: 28 patients underwent a radiosurgical procedure as the initial treatment (Group 1), and 10 patients underwent microsurgical resection with adjuvant radiosurgery on the tumor remnant as the initial treatment (Group 2). The indication for a second GKRS treatment was growth observed on follow-up imaging. The median margin dose was 11.0 Gy for the first procedure and 11.5 Gy for the second procedure. Tumor control after retreatment was assessed through volumetric analysis. Clinical outcome was assessed through medical chart review.RESULTSMedian tumor volume at retreatment was 3.6 cm3, with a median treatment interval of 49 months. All patients showed tumor control in a median follow-up period of 75 months after the second radiosurgical procedure. Volumetric tumor response after the second procedure did not correspond to response after the first procedure. After retreatment, persisting House-Brackmann Grade II facial nerve dysfunction was observed in 3 patients (7.9%), facial spasms in 5 patients (13%), and trigeminal nerve hypesthesia in 3 patients (7.9%). Hearing preservation was not evaluated because of the small number of patients with serviceable hearing at the second procedure.CONCLUSIONSRepeat GKRS after a failed first treatment appears to be an effective strategy in terms of tumor control. The volumetric response after a repeat procedure could not be predicted by the volumetric response observed after first treatment. This justifies considering repeat GKRS even for tumors that do not show any volumetric response and show continuous growth after first treatment. An increased risk of mild facial and trigeminal nerve dysfunction was observed after the second treatment compared with the first treatment.

Objective: Stereotactic radiosurgery (SRS) is one of the treatment modalities for vestibular schwannomas (VSs). However, tumor progression can still occur after treatment. Currently, it remains unknown how to predict long-term SRS treatment outcome. This study investigates possible magnetic resonance imaging (MRI)-based predictors of long-term tumor control following SRS.Study Design: Retrospective cohort study.Setting: Tertiary referral center.Patients: Analysis was performed on a database containing 735 patients with unilateral VS, treated with SRS between June 2002 and December 2014. Using strict volumetric criteria for long-term tumor control and tumor progression, a total of 85 patients were included for tumor texture analysis.Intervention(s): All patients underwent SRS and had at least 2 years of follow-up.Main Outcome Measure(s): Quantitative tumor texture features were extracted from conventional MRI scans. These features were supplied to a machine learning stage to train prediction models. Prediction accuracy, sensitivity, specificity, and area under the receiver operating curve (AUC) are evaluated.Results: Gray-level co-occurrence matrices, which capture statistics from specific MRI tumor texture features, obtained the best prediction scores: 0.77 accuracy, 0.71 sensitivity, 0.83 specificity, and 0.93 AUC. These prediction scores further improved to 0.83, 0.83, 0.82, and 0.99, respectively, for tumors larger than 5 cm3.Conclusions: Results of this study show the feasibility of predicting the long-term SRS treatment response of VS tumors on an individual basis, using MRI-based tumor texture features. These results can be exploited for further research into creating a clinical decision support system, facilitating physicians, and patients to select a personalized optimal treatment strategy.

OBJECTIVE: To evaluate short- and long-term hearing results of surgery for acquired atresia of the external auditory canal (EAC) in a large patient cohort and to define preoperative audiometric conditions useful for patient counseling.STUDY DESIGN: Retrospective cohort study.SETTING: Academic tertiary referral center.PATIENTS: Seventy-eight ears from 72 patients with postinflammatory acquired atresia of the EAC who underwent canal- and meatoplasty were included. Patients with involvement of the ossicular chain, (syndromic) external ear malformations, or congenital aural atresia were excluded.INTERVENTION: Canal- and meatoplasty.MAIN OUTCOME MEASURES: Mean pure-tone averages of thresholds at 0.5, 1, 2, and 3 kHz (PTA0.5,1,2,3) for air conduction (AC), bone conduction, and air-bone gap (ABG) were calculated preoperatively and at short-term (≤0.55 yr) and long-term follow-up (>0.55 yr). Additionally, the numbers of ears with a closed ABG ≤10 dB and ≤20 dB, and with Social hearing (defined as: AC PTA0.5,1,2,3 ≤35 dB) were assessed.RESULTS: At short-term follow-up AC PTA0.5,1,2,3 improved by 18 dB. Social hearing was obtained in 81% of the ears. Postoperatively, 35% of the ears had a closed ABG ≤10 dB, 83% was closed ≤20 dB. During follow-up, significant deterioration of 5 to 7 dB occurred for AC thresholds at 0.25, 0.5, and 1 kHz.CONCLUSIONS: Surgery for acquired atresia of the EAC is often beneficial. This study suggests overall advantageous surgery when the preoperative indication criteria ABG PTA0.5,1,2,3 >20 dB and AC PTA0.5,1,2,3 >35 dB are applied.

The objective of this study was to achieve uniform reporting of complications and failures in cochlear implantation, to analyze complications and failures and to identify risk factors for complications in a series of over 1300 cochlear implantations. In a retrospective chart review and observational study, data from all cochlear implantations from 1987 to 2015 were entered in a custom-made database. Complications were classified using the contracted form of the Clavien–Dindo system and risk factors were identified by statistical analysis. A complication rate of 18.4% and a device failure rate of 2.9% were found. There was a higher rate of hematoma in patients with a clotting disorder and when a subtotal petrosectomy was performed, a higher rate of wound infections in patients who were not vaccinated against Streptococcus pneumoniae and a higher rate of meningitis in patients with an inner ear malformation. The use of a strict definition of a medical complication and device failure—in combination with the Clavien–Dindo classification system—enables uniform and objective registration of adverse events and prevents any tendency to downgrade complications. Complication and failure rates in this series are comparable to those reported in the literature. These results stress the need for pneumococcal vaccination, which may prevent general wound infections, but is especially important for patients with inner ear malformation, who have an increased risk of (postoperative) meningitis.

Item does not contain fulltext BACKGROUND: With expanding inclusion criteria for cochlear implantation, the number of prelingually deafened persons who are implanted as adults increases. Compared with postlingually deafened adults, this group shows limited improvement in speech recognition. In this study, the changes in health-related quality of life in late-implanted prelingually deafened adults are evaluated and related to speech recognition. METHODS: Quality of life was measured before implantation and 1 year after implantation in a group of 28 prelingually deafened adults, who had residual hearing and who used primarily oral communication. Patients completed 3 questionnaires (Nijmegen Cochlear Implant Questionnaire, Glasgow Benefit Inventory, and Health Utility Index 3). Postoperative scores were compared with preoperative scores. Additionally, phoneme recognition scores were obtained preimplantation and 1 year postimplantation. RESULTS: Quality of life improved after implantation: scores on the Nijmegen Cochlear Implant Questionnaire improved significantly in all subdomains (basic speech perception, advanced speech perception, speech production, self-esteem, activity, and social interaction), the total Glasgow Benefit Inventory score improved significantly, and the Health Utility Index 3 showed a significant improvement in the utility score and in the subdomains "hearing" and "emotion." Additionally, a significant improvement in speech recognition scores was found. No significant correlations were found between gain in quality of life and speech perception scores. CONCLUSION: The results suggest that quality of life and speech recognition in prelingually deafened adults significantly improved as a result of cochlear implantation. Lack of correlation between quality of life and speech recognition suggests that in evaluating performance after implantation in prelingually deafened adults, measures of both speech recognition and quality of life should be used. 01 februari 2014

Item does not contain fulltext OBJECTIVES/HYPOTHESIS: A prospective long-term follow-up study was conducted to evaluate the results of gamma knife radiosurgery (GKRS) for vestibular schwannoma (VS) patients. Both axial and volumetric measurements are used to determine tumor size during follow-up. STUDY DESIGN: Individual prospective cohort study. METHODS: A total of 110 VS patients were referred for radiosurgery between 2002 and 2007. All patients were treated with a Leksell 4C gamma knife. There were 12.5 to 13 Gy prescribed to the isodose covering 90% of the tumor volume. The resulting marginal dose was on average 11.0 Gy (range, 9.3-12.5 Gy). Tumor size and tumor volume were determined before and after gamma knife treatment at regular intervals. The minimal follow-up period was 2 years. RESULTS: There were 100 patients included in the study. Eight patients needed additional treatment after a mean follow-up period of 38 months. One patient experienced a temporary facial nerve deficit. No growth pattern could be recognized for tumor growth after GKRS. Based on the measurements of the largest extrameatal diameter, the tumor size would have decreased or remained stable in 94%. Based on volumetric measurement, the tumor size was decreased or remained stable in 79%. CONCLUSIONS: High tumor control and low complication rates make GKRS a good therapy for VS. If tumor growth occurs after GKRS, a conservative management can be considered because continued tumor growth is uncertain. The extrameatal diameter on axial magnetic resonance imaging seems to be a reliable parameter of the size of a VS. Volumetry is the preferred method to assess the dimensions of a VS, although the consequences of strong volumetric increase, especially in small tumors, can be different depending on individual differences in tumor size.

Item does not contain fulltext OBJECTIVE: To evaluate the transcanal surgical implantation of the semi-implantable Vibrant Soundbridge (VSB) device in patients with severe external otitis. STUDY DESIGN: Retrospective analysis. METHODS: Long-term postoperative complications and postoperative hearing thresholds were evaluated in 13 adults with bilateral sensorineural hearing loss (average of between 40 and 55 dB HL) and therapy-resistant external otitis after implantation of the VSB by a transcanal surgical method. RESULTS: Postoperative audiometry findings were comparable with those reported after the transmastoidal posterior tympanotomy approach. In 2 patients, the chorda tympani was intentionally sacrificed to maximize the size of the facial recess. Seven postoperative complications occurred in 6 patients (46%) during a mean follow-up period of 51 months: extrusion of the conducting wire into the ear canal (n=5), collapse of the cartilaginous part of the ear canal (n=1), and tympanic membrane perforation (n=1). In the revision operations that added additional layers of fascia for the patients with wire extrusions, repeated extrusion occurred in 3 of 4 cases. CONCLUSION: The transcanal approach for the implantation of the VSB has led to postoperative complications different from those reported after the transmastoidal posterior tympanotomy approach. External otitis should be considered as a contraindication for VSB surgery by the transcanal approach.

Item does not contain fulltext OBJECTIVES: Endolymphatic sac tumors (ELSTs) are rare adenomatous tumors arising in the petrous bone, either spontaneously (isolated) or in association with von Hippel-Lindau (VHL) disease. For 9 patients, this study describes the typical features and management of ELST and the clinical outcome of treatment. Similarities and differences are identified for ELST in isolation and in association with VHL disease. STUDY DESIGN: Case series. SETTING: Tertiary referral center. PATIENTS: The patient database was searched for patients diagnosed with ELST. All other tertiary referral centers in The Netherlands were invited to do likewise. INTERVENTIONS: The patient records were screened for presenting symptoms, hearing, imaging, treatment, and treatment outcome. Histopathologic finding was reviewed by a senior pathologist. RESULTS: Six patients with an isolated ELST and 3 patients with an ELST in association with VHL disease were identified. In the isolated ELST group, 5 patients were treated surgically and 1 patient was administered primary radiotherapy. In the VHL group, 1 patient underwent surgery and 2 patients were treated conservatively after a wait-and-magnetic resonance imaging-scan protocol. All individual treatment outcomes are scrupulously presented in this article. CONCLUSION: This study compiles and describes 9 cases of ELST. In 8 of 9 patients, the ELST could be controlled by surgery, radiotherapy, or a wait-and-scan protocol. The large interpatient differences make it difficult to standardize the management of this disease. The management is strongly influenced by comorbidity and tumor stage.

Contains fulltext : 89525timmer.pdf (Publisher’s version ) (Closed access) This study evaluates the impact of gamma knife radiosurgery (GKRS) on the quality of life (QOL) of patients with a sporadic vestibular schwannoma (VS). This study pertains to 108 VS patients who had GKRS in the years 2003 through 2007. Two different QOL questionnaires were used: medical outcome study short form 36 (SF36) and Glasgow benefit inventory (GBI). Radiosurgery was performed using a Leksell 4C gamma knife. The results of the QOL questionnaires in relation to prospectively and retrospectively gathered data of the VS patients treated by GKRS. Eventually, 97 patients could be included in the study. Their mean tumor size was 17 mm (range 6-39 mm); the mean maximum dose on the tumor was 19.9 Gy (range 16-25.5 Gy) and the mean marginal dose on the tumor was 11.1 (range 9.3-12.5 Gy). SF36 scores showed results comparable to those for a normal Dutch population. GBI showed a marginal decline in QOL. No correlation was found between QOL and gender, age, tumor size, or radiation dose. Increased audiovestibular symptoms after GKRS were correlated with a decreased GBI score, and decreased symptoms were correlated with a higher QOL post-GKRS. In this study shows that GKRS for VS has little impact on the general QOL of the VS patient. However, there is a wide range in individual QOL results. Individual QOL was influenced by the audiovestibular symptoms. No predictive patient, tumor, or treatment factors for QOL outcome after GKRS could be determined. Comparison with microsurgery is difficult because of intra group variability. 01 juni 2010

Contains fulltext : 81894timmer.pdf (Publisher’s version ) (Closed access) Management of a sporadic vestibular schwannoma (VS) is still a subject of controversy, mainly due to distinct and unpredictable growth patterns. To embark on an appropriate therapy it is necessary to dispose of a reliable prediction about tumor progression. This study aims to design a risk profile with predictors for VS growth. A total of 234 VS patients who were managed conservatively were included. Data concerning (duration of) symptoms and localization of VS were analyzed with Cox proportional hazards regression models. Predictors for growth are unsteadiness/vertigo, no sudden onset of hearing loss and short duration of hearing loss. High-risk patients have (1) VS with an extrameatal localization, short duration of hearing loss and at least one of the two other predictors (unsteadiness/vertigo or no sudden sensorineural hearing loss) or (2) VS with an intrameatal localization and all three other predictors. Low-risk patients have (1) VS with an extrameatal component and no other predictor or (2) VS with an intrameatal localization and at most one other predictor. High-risk patients have a risk of growth of 36.9% in the first year and 64.6% in the second year. For patients with a low risk this is 2.5 and 12.7%, respectively. Simple data gathered at the moment of diagnosis may provide useful information since they may lead to a risk profile for growth.

Item does not contain fulltext OBJECTIVE: To evaluate the long-term medical and technical results, implant survival, and complications of the semi-implantable vibrant soundbridge (VSB), otologics middle ear transducer (MET), and the otologics fully implantable ossicular stimulator (FIMOS). STUDY DESIGN: Retrospective cohort study. PATIENTS: Patients with chronic external otitis and either moderate to severe sensorineural or conductive/mixed hearing loss. SETTING: Tertiary referral center. INTERVENTION: Implantation with the VSB, MET, or FIMOS. MAIN OUTCOME MEASURES: Medical complications, number of reimplantations, and explantations. RESULTS: Ninety-four patients were implanted, 12 patients with a round window or stapes application. 28 patients were lost to follow-up. The average follow-up duration was 4.4 years (range, 1 month-15 years). 128 devices were evaluated: (92 VSB, 32 MET, 4 FIMOS). 36 devices (28%) have been explanted or replaced (18 VSB, 14 MET, 4 FIMOS). Device failure was 7% for VSB, 28% for MET, and 100% for FIMOS. In 16 patients (17%) revision surgery (n = 20) was performed. Twenty patients (21%) suffered any medical complication. CONCLUSION: Medical and technical complications and device failures have mostly occurred in the initial period of active middle ear implants (AMEI) implementation and during clinical trials or experimental procedures. All four FIMOS had technical difficulties. An important decrease in the occurrence of both medical and technical complications was observed. Application in more recent years did not show any complications and the recent device failure rates are acceptable. Magnetic resonance imaging (MRI) incompatibility should be taken into account when indicating AMEI.

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Item does not contain fulltext Averaged electrode voltages (AEVs) are of secondary importance for integrity testing of cochlear implant devices featuring back-telemetry. However, AEVs are device-independent and may show intermittent failures and deviant stimulation patterns unnoticed by telemetry. We collected AEVs from 18 users of the Clarion 1.2 system and 6 users of the HiFocus system in order to establish norms for evaluating AEVs in difficult cases. The stimuli were presented with the standard clinical software. Monopolar stimulation at about 16 microA showed large AEVs (mean, 173 microV) suitable for integrity testing. No electrode failures were found. The AEV amplitudes from neighboring electrodes differed by less than 30% (2 SD). The AEVs from subjects with the Clarion HiFocus electrode and/or the Clarion electrode positioner were within the normal range. The AEV amplitudes from bipolar stimulation were much more variable. Inversion of phases between electrodes was found in patients with an altered state of the cochlea (otosclerosis and osteogenesis imperfecta) and in a patient with a curled electrode tip. There was no correlation across subjects between AEVs and electrode impedances. Therefore, impedances are dominated by the electrode-tissue interface, in contrast to AEVs, which are determined by the volume conduction in the body.

Objective:To create a system for the uniform registration and classification of complications and failures in cochlear implant surgery, and apply it to all the patients who underwent implantation in our clinic.Method:The definition of a medical complication was established, and data for all cochlear implantations performed between 1987 and 2012 were entered into a custom-made database system.Results:One or more medical complications were registered in 19.0 per cent of 1003 cochlear implantations. The incidence of ‘hard failure’ was 2.3 per cent. Findings revealed a decrease in device failures over the years; the rate of medical complications remained constant.Conclusion:Our database system, which is available free of charge, enables fast and accurate data entry. There were a relatively high number of (minor) complications in our series, but comparison with the relevant literature is difficult. This emphasises the need for a uniform definition of ‘complication’ as it relates to cochlear implant surgery, and an appropriate classification system for such complications.

Item does not contain fulltext OBJECTIVES: To compare amplification options for patients with mixed hearing loss. Devices tested include percutaneous and transcutaneous bone conductors (BCDs) and middle ear implants with their actuator directly coupled to the cochlea. SETTING: Tertiary academic medical center. METHOD AND PARTICIPANTS: Maximum output was studied with simulators. As simulators are lacking for the middle ear implants (the Vibrant Soundbridge [VSB] and the Cochlear's Direct Acoustic Cochlear Stimulator [Codacs]), the maximum output had to be measured in patients (4 and 5 patients, respectively). MAIN OUTCOME MEASURE: The maximum output averaged at 0.5, 1, and 2 kHz was the main outcome measure, which was expressed in dB HL, using appropriate transformation tables. RESULTS: The maximum output was the highest for the Codacs device and was above the patients' uncomfortable loudness levels. The maximum output of the VSB varied between 65 and 85 dB HL, and that of percutaneous BCD varied between 68 and 80 dB HL depending on the type of device. The transcutaneous BCD, the Sophono device, had the lowest output. CONCLUSION: Only with the Codacs device can the complete dynamic range be used. The maximum output of the VSB is lower and variable owing to the coupling to the cochlea. For patients with a sensorineural hearing loss component up to 50 dB HL, a percutaneous BCD forms a good treatment option that is completely independent of the middle ear status. The transcutaneous Sophono BCD is suitable for patients with a (sub-)normal sensorineural hearing loss component of 20 dB or less. 01 februari 2014

Contains fulltext : 127680.pdf (Publisher’s version ) (Closed access) A patient with neurofibromatosis type 2 (bilateral vestibular schwannomas) was treated with bevacizumab (anti-vascular endothelial growth factor [VEFG] monoclonal antibody). The left-sided tumor showed intense uptake on pretreatment In-bevacizumab scintigraphy, indicating VEGF production in the tumor, and no uptake 4 weeks later, demonstrating effective binding of nonradiolabeled bevacizumab to the VEGF produced in the tumor. The right-sided tumor showed no tracer uptake at any time point. Significant tumor volume reduction (assessed with MRI) and hearing improvement were observed on the left side. In-bevacizumab scintigraphy may be a promising upfront patient selection tool to identify patients who may benefit from expensive bevacizumab treatment. 01 maart 2014

OBJECTIVE: To study long-term subjective benefit of patients with sensorineural hearing loss and chronic external otitis who use active middle ear implants. DESIGN: Single-subject repeated measures in a preintervention and postintervention design with multiple postintervention measurements (questionnaires). SETTING: Tertiary academic center. PATIENTS: Moderate-to-severe sensorineural hearing loss (n = 56) with severe chronic external otitis who use the Vibrant Soundbridge (VSB) or Otologics MET middle ear implant systems. MAIN OUTCOME MEASURE: Changes in hearing disability and handicap as evaluated using the Abbreviated Profile of Hearing Aid Benefit (APHAB), the Nijmegen Cochlear Implant Questionnaire (NCIQ), and the Glasgow Benefit Inventory (GBI). RESULTS: Data of 33 patients (mean postoperative duration of 7.5 yr) were available. No difference in subjective results was found between the VSB and Otologics MET patient groups. Total percentage of nonuse was 13%. Long-term APHAB results show a significant decrease in disability for 43% of the patients compared with 54% at 1-year postoperative. NCIQ results show a significant benefit for all subdomains with a negative trend over time. The GBI results show a significant long-term increase in quality of life with positive scores for 82% of the assessed patients. CONCLUSION: Long-term postoperative patient satisfaction and quality of life results show a significant difference compared with preoperative measurements, with conventional hearing aids. A negative trend over time is found on all questionnaires, which might reflect patient aging (increase of hearing loss) or habituation to a situation with fewer concerns regarding a patient's external otitis.

We describe three cases of a fibroinflammatory pseudotumor (tumefactive fibroinflammatory lesion) of the middle and inner ear. The patients presented with total deafness in the affected ear and no response to caloric stimulation. The computed tomographic pattern showed destruction of inner ear structures and a typical widening of parts of the labyrinth. Magnetic resonance imaging performed in all three patients showed an extension greater than expected based on computed tomographic images of both areas of destruction, as well as areas of radiologic normality. An enhancing mass was seen in the inner ear with a characteristic extension into both the internal auditory canal and the middle ear. A transotic approach or subtotal petrosectomy was used to remove the tumor in all three cases. Although histologically benign, these tumors are locally destructive and, as such, behave like a neoplastic lesion. They are composed of fibrovascular tissue admixed with chronic inflammatory cells. To our knowledge, this is the first report on pseudotumors of the middle ear, inner ear, and internal auditory canal.Inflammatory pseudotumorused to be a somewhat confusing term for a recognized entity of unknown origin. It is likely that infection is an important contributing factor in the development of these lesions. Although surgical removal seems to be the treatment of choice, no clear judgment of its prognosis can be made owing to the rarity of this tumor. (Arch Otolaryngol Head Neck Surg. 1995;121:930-933)

Item does not contain fulltext PURPOSE OF REVIEW: Recently, new information on the natural course and on the results of radiation therapy of vestibular schwannomas has been published. The aim of this study is to summarize the most recent literature on the contemporary insights on the natural course and the results of the latest strategies of radiotherapy for vestibular schwannomas. RECENT FINDINGS: After diagnosis only about one-third of all vestibular schwannomas will progress. Many patients do well with a 'wait and see' policy and, when necessary, radiation treatment has the advantage that tumor control rates are high (95%) and treatment-related side effects are very low. Different approaches to radiotherapy continue to evolve. Up till now stereotactic radiosurgery (SRS) and fractionated stereotactic radiotherapy (FSRT) yield comparable results both in terms of tumor control and cranial nerve preservation. With new data available on hearing preservation after radiotherapy, a watchful waiting policy is a renewed matter of debate. SUMMARY: When a vestibular schwannoma grows, radiotherapy (SRS or FSRT) may be a valuable treatment modality. Future clinical research (properly designed randomized trials) should focus on when and when not to treat, even if a vestibular schwannoma is not growing and on potential differences in long-term effects between SRS and fractionated radiotherapy. 01 oktober 2012

Item does not contain fulltext OBJECTIVE: To determine the long-term benefit of the Vibrant Soundbridge (VSB) middle ear implant in patients with severe mixed hearing loss and to compare it with other hearing devices. DESIGN: A retrospective analysis. SETTING: University-affiliated medical center. PATIENTS: Six patients with severe mixed hearing loss and a mean sensorineural hearing loss component between 40 and 70 dB. INTERVENTIONS: Patients received a VSB with the floating mass transducer (FMT) coupled to the round window or to the oval window via a residual stapes structure. MAIN OUTCOME MEASURES: Functional gain and speech recognition results. Results are compared with 2 control groups matched for mean sensorineural hearing loss: 1) patients with mixed hearing loss and a bone-anchored hearing device, and 2) patients with sensorineural hearing loss and traditional implantation of the VSB. RESULTS: There is large variance in functional gain between the patients suggesting high variability in the effectivity of the FMT coupling. The speech recognition results for the experimental group were not systematically better than in either control group. CONCLUSION: There is large variability in results that might be ascribed to coupling effectivity. On the average, speech recognition results were not better or worse than those found in patients with similar hearing loss fitted with bone-anchored hearing devices. 01 april 2012

Gamma knife radiosurgery (GKRS) has become an important treatment modality for vestibular schwannomas. The primary aim of this study was to investigate whether tumor growth at the moment of GKRS has any correlation with the outcome. The secondary aim was to identify clinical predictors of radioresistance in vestibular schwannoma patients treated with GKRS.One hundred vestibular schwannoma patients, treated with GKRS, were divided into two groups: 1) proven tumor growth preceding GKRS; and 2) previous history of growth unknown. GKRS outcome was defined in two ways. According to the first definition, GKRS was said to have failed when additional treatment had taken place. According to the second one, a volume decrease20% after 2 years marked successful treatment.Correlations between outcome and growth status were determined with SPSS software. Furthermore, the study assessed how different variables (patient data, history, tumor characteristics, imaging, and audiovestibular examinations) correlated with the outcome of GKRS.No significant difference regarding success and failure of GKRS was found between the two patient groups. The mean reduction in tumor volume after GKRS was less pronounced in patients in whom tumor growth was demonstrated before treatment, but this finding was not significant. No significant predictors (P.05) could be identified in this data set.This study found no indication that growth at the moment of GKRS influences therapeutic outcome, nor did it identify any predictors of the outcome after GKRS in vestibular schwannoma patients.

Objectives: The aim of this study was to formulate a predictive rule for vestibular schwannoma growth during the initial observation period after diagnosis. Methods: Logistic regression models were fitted, with tumor growth in the first year as the dependent variable and patient characteristics as the independent variables. Backward selection was used to eliminate superfluous predictors. The area under the receiver operating characteristic curve was taken as a measure of the model's discriminative power. Results: Eventually, the model or rule consisted of 4 significant growth predictors: Localization (if extrameatal, +1; if intrameatal, 0), sudden sensorineural hearing loss (if present, −1; if absent, 0), balance symptoms (if present, +1; if absent, 0), and complaints of hearing loss for less than 2 years (if present, +1; if absent, or present for more than 2 years, 0). A higher score indicates a higher likelihood of tumor growth during the period of observation after diagnosis. If the total score is 0 or less, the likelihood of tumor growth during the first year after diagnosis is less than 10%. If the score is 3, the likelihood of growth during the first year after diagnosis is more than 70%. Conclusions: We were able to create a useful rule to predict vestibular schwannoma growth during the first year after diagnosis.

Contains fulltext : 87946.pdf (Publisher’s version ) (Closed access) OBJECTIVE: To optimize intraoperatively the coupling of the floating mass transducer (FMT) of the Vibrant Soundbridge middle ear implant to the round or oval cochlear window in patients with mixed hearing loss. STUDY DESIGN: Intraoperative measurement of objective hearing thresholds using auditory steady state responses (ASSRs). SETTING: Radboud University Nijmegen Medical Centre, tertiary referral hospital. PATIENTS: Four individuals with mixed hearing loss and, at least no incus, in need for a middle ear implant. INTERVENTION: Surgical placement of the Vibrant Soundbridge. ASSR thresholds were measured intraoperatively, whereas FMT coupling to the cochlea was manipulated to find the most effective coupling of the FMT. MAIN OUTCOME MEASURE: Differences in ASSR thresholds between different FMT coupling options within patients. RESULTS: With ASSR, we assessed placement of the FMT in the round window niche, loosely or tightly packed in the niche; creation of a mobile window in case of a fixed stapes footplate; and FMT position coupled to the stapes that afforded vibration in the natural vibration direction or perpendicular to it. Furthermore, test-retest variations in ASSR thresholds were studied. It was shown that differences in ASSR thresholds could be detected, while manipulating the FMT couplings, which were statistically significant. CONCLUSION: Intraoperative ASSR measurement is a good method to study different positions of the FMT and to determine the best position of the FMT for a patient. 01 december 2010

Contains fulltext : 88719.pdf (Publisher’s version ) (Closed access) Tripolar and other electrode configurations that use simultaneous stimulation inside the cochlea have been tested to reduce channel interactions compared to the monopolar stimulation conventionally used in cochlear implant systems. However, these "focused" configurations require increased current levels to achieve sufficient loudness. In this study, we investigate whether highly accurate recordings of the intracochlear electrical field set up by monopolar and tripolar configurations correlate to their effect on loudness. We related the intra-scalar potential distribution to behavioral loudness, by introducing a free parameter (alpha) which parameterizes the degree to which the potential field peak set up inside the scala tympani is still present at the location of the targeted neural tissue. Loudness balancing was performed on four levels between behavioral threshold and the most comfortable loudness level in a group of 10 experienced Advanced Bionics cochlear implant users. The effect of the amount of focusing on loudness was well explained by alpha per subject location along the basilar membrane. We found that alpha was unaffected by presentation level. Moreover, the ratios between the monopolar and tripolar currents, balanced for equal loudness, were approximately the same for all presentation levels. This suggests a linear loudness growth with increasing current level and that the equal peak hypothesis may predict the loudness of threshold as well as at supra-threshold levels. These results suggest that advanced electrical field imaging, complemented with limited psychophysical testing, more specifically at only one presentation level, enables estimation of the loudness growth of complex electrode configurations.

Objectives: We evaluated 5 patients who were changed over from a Baha Cordelle to a cochlear implant (CI). Moreover, the level of phoneme score was determined at which a Baha Cordelle user was better helped with a CI. Methods: We offer descriptive case reports and a retrospective evaluation of speech recognition in Baha Cordelle users and CI users. Results: In the CI users with noncompromised cochleas, the 10th percentile of the aided phoneme score in quiet at 65 dB sound pressure level (PS65) was 42%. We consider this PS65 as the cutoff level for switching from a Baha Cordelle to a CI. When patients with mixed hearing loss were using the Baha Cordelle, the PS65 of 42% was obtained at a mean sensorineural hearing loss component of about 70 dB hearing level (HL). This 70–dB HL component was used to consider Baha Cordelle users for cochlear implantation. The results of the 5 patients support these transition criteria. Conclusions: A CI is a valuable option in patients with mixed hearing loss when the sensorineural hearing loss component exceeds 70 dB HL or when the PS65 with a Baha Cordelle is less than about 40%. In such patients, the implantation procedure should be individualized on the basis of the clinical findings in the middle ear and mastoid cavity.

This study was designed to evaluate hearing preservation after gamma knife radiosurgery (GKRS) and to determine the relation between hearing preservation and cochlear radiation dose in patients with a sporadic vestibular schwannoma (VS).Prospective study involving patients suffering from VS who received GKRS from June 2003 until November 2007. Pure tone and speech audiometry were conducted before and after GKRS. The thresholds at pure tone audiometry were taken as a measure of hearing. Pure tone average (PTA) was defined as the mean threshold at 0.5 kHz, 1.0 kHz, 2.0 kHz, and 4.0 kHz. Hearing was classified according to the 2003 consensus meeting in Tokyo. Stereotactic surgery was performed using a Leksell 4C Gamma Knife (Elekta, Stockholm, Sweden).A total of 69 patients were included in the study. Mean tumor size was 17 mm. Mean marginal dose at the tumor was 11.0 Gy (range, 9.3 Gy-12.3 Gy), mean maximal dose was 19.7 Gy (range, 16 Gy-25.5 Gy). Mean maximal dose at the cochlea was 10.27 Gy (range, 3.1 Gy-16.1 Gy), and mean minimal dose at the cochlea was 2.6 Gy (range, 0.9 Gy-7.4 Gy). Mean PTA before GKRS was 43 dB (standard deviation [SD] 20 dB), mean PTA after GKRS was 63 dB (SD 30 dB). Mean interval between pre-GKRS audiometry and GKRS was 8.0 months. Between GKRS and post-GKRS audiometry, mean interval was 14.2 months. Hearing was considered to be preserved (max +1 class, Tokyo classification) in 52 (75%) of 69 patients. However, only 32 patients had class A, B, or C (serviceable hearing) before GKRS. Within this group, only 13 patients (41%) had a hearing class A, B, or C after GKRS. A significant relation was found between the maximal cochlear dose and the difference in PTA before and after GKRS.Hearing preservation is correlated to the maximal radiation dose at the cochlea. The purpose of developing GKRS techniques was to avoid collateral damage in healthy tissues. This study emphasizes the need for exact radiation planning to reduce the cochlear radiation dose if the hearing is to be preserved. Laryngoscope, 2009.

Contains fulltext : 80244.pdf (Publisher’s version ) (Closed access) OBJECTIVE: To assess whether free-living and/or biofilm bacteria are present in the putative sterile middle ear cavity before insertion of the electrode array during cochlear implantation. DESIGN: Prospective study. SETTING: Tertiary academic hospital. PATIENTS: The study included 45 healthy children (with or without a history of otitis media) undergoing cochlear implantation. INTERVENTIONS: Transmission electron microscopy or scanning electron microscopy was used to detect the presence of bacteria. MAIN OUTCOME MEASURE: Presence of both free-living bacteria and biofilm bacteria on the epithelial surface of biopsy specimens of middle ear mucosa. RESULTS: A majority of all mucosal specimens from clinically healthy tympanic cavities displayed inflammatory areas as well as dispersed, nonmatrix-enclosed bacteria. Also, rarely, fragments of biofilms were found. CONCLUSIONS: The presence of bacteria in the tympanic cavity, which is generally assumed to be sterile in healthy individuals, may provide an explanation for infectious complications after cochlear implantation. However, the possibility that the electrode array of a cochlear implant will actually become contaminated during insertion is unlikely because of the small amounts and dispersed presence of bacteria, which may account for the relatively low incidence of infectious complications after cochlear implantation.

Contains fulltext : 69164.pdf (Publisher’s version ) (Closed access) OBJECTIVES: To compare the effects of Monopole (Mono), Tripole (Tri), and "Virtual channel" (Vchan) electrode configurations on spectral resolution and speech perception in a crossover design. DESIGN: Nine experienced adults who received an Advanced Bionics CII/90K cochlear implant participated in a crossover design using three experimental strategies for 2 wk each. Three strategies were compared: (1) Mono; (2) Tri with current partly returning to adjacent electrodes and partly (25 or 75%) to the extracochlear reference; and (3) a monopolar "Vchan" strategy creating seven intermediate channels between two contacts. Each strategy was a variant of the standard "HiRes" processing strategy using 14 channels and 1105 pulses/sec/ channel, and a pulse duration of 32 microsec/phase. Spectral resolution was measured using broadband noise with a sinusoidally rippled spectral envelope with peaks evenly spaced on a logarithmic frequency scale. Speech perception was measured for monosyllables in quiet and in steady-state and fluctuating noises. Subjective comments on music experience and preferences in everyday use were assessed through questionnaires. RESULTS: Thresholds and most comfortable levels with Mono and Vchan were both significantly lower than levels with Tri. Spectral resolution was significantly higher with Tri than with Mono; spectral resolution with Vchan did not differ significantly from the other configurations. Moderate but significant correlations between word recognition and spectral resolution were found in speech in quiet and fluctuating noise. For speech in quiet, word recognition was best with Mono and worst with Vchan; Tri did not significantly differ from the other configurations. Pooled across the noise conditions, word recognition was best with Tri and worst with Vchan (Mono did not significantly differ from the other configurations). These differences were small and insufficient to result in a clear increase in performance across subjects if the result from the best configuration per subject was compared with the result from Mono. Across all subjects, music appreciation and satisfaction in everyday use did not clearly differ between configurations. CONCLUSIONS: (1) Although spectral resolution was improved with the tripolar configuration, differences in speech performance were too small in this limited group of subjects to justify clinical introduction. (2) Overall spectral resolution remained extremely poor compared with normal hearing; it remains to be seen whether further manipulations of the electrical field will be more effective.

Item does not contain fulltext Osteogenesis imperfecta (OI) is a heterogeneous disease of the connective tissue caused by a defective gene that is responsible for the production of collagen type I, leading to defective bone matrix and connective tissue. Hearing loss affects 35-60% of the patients and will progress to deafness in 2-11% of OI patients for whom cochlear implantation may become the only remaining treatment option. Three patients with OI were retrieved from the Nijmegen Cochlear Implant Centre's database. Most of the specific observations in ear surgery on patients with OI, such as brittle scutum, sclerotic thickening of the cochlea, hyperplastic mucosa in the middle ear and persistent bleeding, were encountered in these 3 patients. In case 3, with severe deformities on the CT scan, misplacement of the electrode array into the horizontal semicircular canal occurred. In all 3 cases, programming was hindered by nonauditory stimulation. Even after reimplantation, nonauditory sensations lead to case 3 becoming a nonuser. Averaged electrode voltages in case 3 were deviant in accordance with an abnormally conductive otic capsule. Spatial spread of neural excitation responses in cases 1 and 2 suggested intracochlear channel interaction for several electrodes, often in combination with facial nerve stimulation (FNS). In case 1, the estimated pitch of the electrodes that caused FNS varied consistently. Despite the electrophysiological changes, after 1-year follow-up, open set phoneme scores of 81% and 78% were reached in cases 1 and 2, respectively. When aware and prepared for the specific changes of the temporal bone in OI, cochlear implantation can be a safe and feasible procedure. Preoperative imaging is recommended to be fully informed on the morphology of the petrosal bone. In case of severe deformities on the CT scan, during counseling the possibility of misplacement should be mentioned. Rehabilitation is often hindered by FNS requiring frequent refitting.

The subjective benefit of middle ear implantation was studied in a group of 23 hearing-impaired patients who could not use conventional hearing aids owing to severe chronic external otitis. Changes in hearing disability (Abbreviated Profile of Hearing Aid Benefit [APHAB]) and changes in quality of life (Glasgow Benefit Inventory [GBI]) were determined. Mean benefit value on the APHAB for the subscale Ease of Communication was close to the mean reference value for conventional hearing aids. For the subscales Reverberation and Background Noise, a poorer result was found. Individual analysis of the APHAB scores showed significant benefit in 12 out of the 23 patients. According to the GBI, 16 out of 17 patients reported that middle ear implantation had made a positive impact on their quality of life. It is concluded that middle ear implantation has a positive effect on hearing difficulties and quality of life in hearing-impaired subjects who cannot use conventional devices. The APHAB outcomes were not better than those reported for conventional devices. Se estudió el beneficio subjetivo de la implantación del oído medio en un grupo de 23 pacientes hipoacúsicos que no podían utilizar auxiliares auditivos convencionales debido a una otitis externa crónica severa. Se determinaron los cambios en discapacidad auditiva (Perfil Abreviado del Beneficio del Auxiliar Auditivo [APHAB]) y los cambios en la calidad de vida (Inventario Glasgow de Beneficio [GBI]). El valor medio de beneficio en el APHAB para la sub-escala Facilidad de Comunicación fue cercano al valor medio de referencia para los auxiliares auditivos convencionales. Para las sub-escalas de Reverberación y Ruido de Fondo, se encontró un resultado más pobre. El análisis individual de los puntajes APHAB mostró un beneficio significativo en 12 de los 23 pacientes. De acuerdo con el GBI, 16 de los 17 pacientes reportaron que la implantación del oído medio había tenido un impacto positivo en la calidad de sus vidas. Se concluye que la implantación del oído medio tiene un efecto positivo sobre las dificultades de comunicación y sobre la calidad de vida en sujetos hipoacúsicos que no pueden utilizar dispositivos convencionales. Los resultados del APHAB no fueron mejores que aquellos reportados con dispositivos convencionales.

Contains fulltext : 52725.pdf (Publisher’s version ) (Closed access) OBJECTIVE: To investigate long-term quality of life (QoL) in postlingually deaf adults after entering the cochlear implantation (CI) program. STUDY DESIGN AND SETTING: Follow-up study from 1998 onwards in tertiary university medical center. Long-term CI users, patients who have not received a CI, and relatively short-term CI users were re-evaluated six years after initial data collection in 1998 by using three questionnaires (NCIQ, HUI3, and SF36) and speech perception tests. RESULTS AND CONCLUSIONS: In general, the beneficial effect of CI remained stable during long-term follow-up, though scores on the questionnaires decreased slightly. Outcomes before and after cochlear implantation were significantly different. The group without a CI demonstrated slightly decreasing trends in outcomes. Long-term speech perception performance improved in time. SIGNIFICANCE: This is the first study to investigate long-term follow-up of CI patients, in all aspects of QoL combined with speech perception performance, in comparison with postlingually deaf adults without CI.

Contains fulltext : 144464.pdf (Publisher’s version ) (Open Access) PURPOSE: To investigate the clinical features and molecular causes of autosomal dominant rhegmatogenous retinal detachment (RRD) in two large families. METHODS: Clinical examination and linkage analysis of both families using markers flanking the COL2A1 gene associated with Stickler syndrome type 1, the loci for Wagner disease/erosive vitreoretinopathy (5q14.3), high myopia (18p11.31 and 12q21-q23), and nonsyndromic congenital retinal nonattachment (10q21). RESULTS: Fifteen individuals from family A and 12 individuals from family B showed RRD or retinal tears with minimal (family A) or no (family B) systemic characteristics of Stickler syndrome and no ocular features of Wagner disease or erosive vitreoretinopathy. The RRD cosegregated fully with a chromosomal region harboring the COL2A1 gene with maximum lod scores of 6.09 (family A) and 4.97 (family B). In family B, an Arg453Ter mutation was identified in exon 30 of the COL2A1 gene, that was previously described in a patient with classic Stickler syndrome. In family A, DNA sequence analysis revealed no mutation in the coding region and at the splice sites of the COL2A1 gene. CONCLUSIONS: In two large families with RRD, linkage was found at the COL2A1 locus. In one of these families an Arg453Ter mutation was identified, which is surprising, because all predominantly ocular Stickler syndrome cases until now have been associated with protein-truncating mutations in exon 2, an exon subject to alternative splicing. In contrast, the Arg453Ter mutation and other protein-truncating mutations in the helical domain of COL2A1 have been associated until now with classic Stickler syndrome.

Item does not contain fulltext Patients with chronic or recurrent otitis media with effusion who do not respond to treatment with conventional ventilation tubes are often treated with long-term ventilation tubes. The aim of this retrospective study was to determine the extrusion and complication rates of long-term ventilation tubes. The median ventilation time of the tubes was 31.5 months. The most common complication was otorrhoea. One episode of otorrhoea occurred after 24% of all tube insertions, and recurrent otorrhoea occurred in 29%. A persisting perforation developed in 19% after extrusion or removal of the tube. Other, mostly minor, complications occurred in 36% with the tube in situ and in 3% after extrusion or removal of the tube. Long-term ventilation tubes provided prolonged ventilation of the middle ear, but also resulted in a considerable number of complications. For each individual case, one should therefore determine whether the advantages of long-term ventilation tubes outweigh the possible complications in view of the available alternatives for middle ear ventilation.

Item does not contain fulltext In order to establish whether the electrical field created by a cochlear implant electrode is more focussed in a perimodiolar than in a medial position, voltages at non-stimulated electrodes were collected from the Clarion 1.2 implant before and after insertion of the Clarion Electrode Positioning System. The positioner is inserted lateral to the electrode array and is intended to bring the array close to the medial wall of the scala tympani. These intracochlear electrode voltages (IEVs) were collected in 9 surgeries with the HiFocus I electrode array with positioner. Stapedius reflex thresholds were decreased after insertion of the positioner on 20 out of 22 electrodes. Electrode impedances did not increase significantly. IEVs increased slightly for all stimulated electrodes. However, the positioner did not significantly affect the slope of the IEVs for any of the stimulated electrodes. Individual differences between patients in the mean IEV amplitude were considerable; 2 patients deafened by otosclerosis showed particularly low amplitudes. Thus, IEVs provided no evidence of a focussing of the electrical field by introducing the positioner. Possible benefits of a perimodiolar position regarding efficiency and channel separation are discussed in terms of a reduced distance between electrodes and neural elements.

Three cases of infantile subglottic hemangioma are described. Generally conservative management is favored by most authors. These present lesions were removed surgically by midline cricotracheotomy. Follow-up showed that all children were well and without complaints. The authors believe that besides management with the CO2 laser and short-term steroid therapy, operative treatment of infantile subglottic hemangioma is a valuable alternative which should receive more attention. All children were decannulated a few days after surgery.