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Cochlear implants (CI) restore hearing of severely hearing-impaired patients. Although this auditory prosthesis is widely considered to be very successful, structural cochlear trauma during cochlear implantation is an important problem, reductions of which could help to improve hearing outcomes and to broaden selection criteria. The surgical approach in cochlear implantation, i.e. round window (RW) or cochleostomy (CO), and type of electrode-array, perimodiolar (PM) or lateral wall (LW), are variables that might influence the probability of severe trauma. We investigated the effect of these two variables on scalar translocation (STL), a specific type of severe trauma. Thirty-two fresh frozen human cadaveric ears were evenly distributed over four groups receiving either RW or CO approach, and either LW or PM array. Conventional radiological multiplanar reconstruction (MPR) was compared with a reconstruction method that uncoils the spiral shape of the cochlea (UCR). Histological analysis showed that RW with PM array had STL rate of 87% (7/8), CO approach with LW array 75% (6/8), RW approach with LW array 50% (4/8) and CO approach with PM array 29% (2/7). STL assessment using UCR showed a higher inter-observer and histological agreement (91 and 94% respectively), than that using MPR (69 and 74% respectively). In particular, LW array positions were difficult to assess with MPR. In conclusion, the interaction between surgical approach and type of array should be preoperatively considered in cochlear implant surgery. UCR technique is advised for radiological assessment of CI positions, and in general it might be useful for pathologies involving the inner ear or other complex shaped bony tubular structures.

OBJECTIVE To report our results on hearing preservation after linear accelerator (LINAC)-based stereotactic radiotherapy for vestibular schwannomas (VS) in a tertiary referral center. METHODS All patients who presented with VS in our center between 2010 and 2018 and who were treated with LINAC-based radiotherapy were retrospectively analyzed. Pure tone average and speech discrimination score represented hearing outcome, pre- and postradiotherapy. A Gardner-Robertson grade I or II hearing represented functional hearing. RESULTS In total, 35 patients were treated with LINAC-based radiotherapy. Median age was 55 years (range 18-86 years), 22 (63%) were female. Sixteen patients had a Koos grade III or IV tumor. Twenty-four patients were treated with radiosurgery (1 or 5 fractions; stereotactic radiosurgery), and eleven patients were treated with fractionated stereotactic radiotherapy. Mean follow-up was 4.8 years (range 1.8-8.4 years). In 34 patients, hearing was assessed pre- and post-radiotherapy. In seventeen patients, hearing remained stable. In eleven patients, a decrease in GR scale was observed, of which seven patients showed a decrease from a functional to a non-functional level (4 GR III, 2 GR IV, and 1 GR V). Tumor control was 95% (34/35), and except for hearing loss, all post-radiation complications and morbidity were transient. CONCLUSION These data emphasize that although the rate of tumor control (the primary goal of radiotherapeutic treatment) is high, it is important to adequately manage patients' expectations regarding the outcomes of the secondary possibly positive outcome; hearing preservation.

Hypothesis To develop and validate the optimal design and evaluate accuracy of individualized 3D- printed surgical guides for cochlear implantation. Background Positioning and fixation of the cochlear implant (CI) are commonly performed free hand. Applications of 3-dimensional (3D) technology now allow us to make patient specific, bone supported surgical guides, to aid CI surgeons with precise placement and drilling out the bony well which accommodates the receiver/stimulator device of the CI. Methods Cone beam CT (CBCT) scans were acquired from temporal bones in 9 cadaveric heads (18 ears), followed by virtual planning of the CI position. Surgical, bone-supported drilling guides were designed to conduct a minimally invasive procedure and were 3D-printed. Fixation screws were used to keep the guide in place in predetermined bone areas. Specimens were implanted with 3 different CI models. After implantation, CBCT scans of the implanted specimens were performed. Accuracy of CI placement was assessed by comparing the 3D models of the planned and implanted CI’s by calculating the translational and rotational deviations. Results Median translational deviations of placement in the X- and Y-axis were within the predetermined clinically relevant deviation range (< 3 mm per axis); median translational deviation in the Z-axis was 3.41 mm. Median rotational deviations of placement for X-, Y- and Z-rotation were 5.50°, 4.58° and 3.71°, respectively. Conclusion This study resulted in the first 3D-printed, patient- and CI- model specific surgical guide for positioning during cochlear implantation. The next step for the development and evaluation of this surgical guide will be to evaluate the method in clinical practice.

Objectives: In this study, we aim to analyze audiometric outcomes of middle ear surgery in patients with congenital middle ear anomalies. Methods: In this single center retrospective cohort study, audiological outcomes were extracted from patient files. Patients with a congenital middle ear anomaly treated surgically in a tertiary referral center between June 2015 and December 2020 were included. Pre- and postoperative short- and long-term audiometric data (at ≥3 and ≥10 months respectively) were compared to analyze hearing outcomes. Results: Eighteen ears (15 patients) were treated surgically with an exploratory tympanotomy. At short term follow up statistically significant improvements in air conduction thresholds and air-bone gaps were found. Hearing improved in 94.4% (17/18) of operated ears. Successful outcome, defined as an air-bone gap closure to within 20 dB after surgery, was reached in 44.4% (8/18). Serviceable hearing (air conduction ≤30 dB) was reached in 55.6% (10/18). Negative outcome (any significant deterioration in hearing) occurred in 1 patient: in this ear otitis media occurred during the postoperative course. At long term follow up, available for 50% of the cohort, hearing remained stable in 5 ears, improved in 1 ear and deteriorated in 3, all of which underwent revision surgery. Sensorineural hearing loss due to surgery, or other complications, were not encountered. Conclusion: middle ear surgery was found to be an effective treatment option to improve hearing in this cohort of patients with congenital middle ear anomalies. Surgical goals of obtained gain in air conduction thresholds and serviceable hearing levels were met by most patients without the occurrence of any iatrogenic sensorineural hearing loss.

OBJECTIVE This study aims to assess and compare the reliability of the Holgers, the IPS, and the Tullamore scales for skin tolerability assessment of postoperative bone-anchored hearing implant images. STUDY DESIGN A survey study and retrospective review of percutaneous osseointegrated auditory implant images for scoring using three skin classification scales. SETTING McGill University Health Center, Montreal, Quebec, Canada. PARTICIPANTS Healthcare workers experienced and inexperienced with osseointegrated auditory implant skin classification scales. MAIN OUTCOME MEASURES Participation involved completing: 1) survey questionnaires assessing experience with osseointegrated auditory implants and related skin reactions and 2) scoring postoperative osseointegrated auditory implant with surrounding skin images using the Holgers Classification, the IS (of the IPS) scale, and the Tullamore Classification. Participants were asked to rate 12 images of postoperative osseointegrated auditory implant and surrounding soft tissue. This process was repeated until participants scored all images using the three scales; each rater graded 36 images in total. The order in which scales were presented occurred at random. Intraclass correlation coefficients were calculated to assess reliability. RESULTS Thirty-one participants were recruited to the study. Fourteen (45.2%) had experience with at least 1 osseointegrated auditory implant skin classification scale, while 17 (54.8%) did not have experience. The wide and overlapping 95% confidence intervals of the intraclass correlation coefficients results do not provide us with enough evidence to define a well-established degree and hierarchy of reliability when comparing the scales. Among experienced raters, all scales presented moderate to good reliability. CONCLUSIONS The Holgers Classification, the IPS scale, and the Tullamore Classification all present moderate to good reliability when used by experienced raters to assess skin reactions following surgical implantation of an osseointegrated hearing device. As a result, clinicians should use these scales with a degree of caution. The findings of this study do not provide us with enough evidence to single out one of the scales as a standard to follow, but more extensive studies are required to assess the reliability of the scales.

Importance Tympanic membrane retraction (TMR) is a relatively common otological finding. However, no consensus on its management exists. We are looking especially for a treatment strategy in the military population who are unable to attend frequent follow-up visits, and who experience relatively more barotrauma at great heights and depths and easily suffer from otitis externa from less hygienic circumstances. Objective To assess and summarize the available evidence for the effectiveness of surgical interventions and watchful waiting policy in patients with a tympanic membrane retraction. Evidence review The protocol for this systematic review was published at Prospero (207859). PubMed, Embase, and the Cochrane Database of Systematic Reviews were systematically searched from inception up to September 2020 for published and unpublished studies. We included randomized trials and observational studies that investigated surgical interventions (tympanoplasty, ventilation tube insertion) and wait-and-see policy. The primary outcomes of this study were clinical remission of the tympanic membrane retraction, tympanic membrane perforations and cholesteatoma development. Findings In total, 27 studies were included, consisting of 1566 patients with TMRs. We included data from 2 randomized controlled trials (76 patients) and 25 observational studies (1490 patients). Seven studies (329 patients) investigated excision of the TMR with and without ventilation tube placement, 3 studies (207 patients) investigated the wait-and-see policy and 17 studies (1030 patients) investigated tympanoplasty for the treatment of TMRs. Conclusions and relevance This study provides all the studies that have been published on the surgical management and wait-and-policy for tympanic membrane retractions. No high level of evidence comparative studies has been performed. The evidence for the management of tympanic membrane retractions is heterogenous and depends on many factors such as the patient population, location and severity of the TMR and presence of other ear pathologies (e.g., perforation, risk of cholesteatoma and serous otitis media).

Purpose Postoperative headache (POH) is a complication that occurs after surgical resection of cerebellopontine angle (CPA) tumors. The two most common surgical approaches are the translabyrinthine (TL), and retrosigmoid (RS) approach. The objective of this systematic review was to investigate whether POH occurs more frequently after RS compared to TL approaches. Methods A systematic search was conducted in Cochrane, Pubmed and Embase. Studies were included if POH after CPA tumor removal was reported and both surgical approaches were compared. The methodological quality of the studies was assessed using the Risk Of Bias In Non-randomized Studies of Interventions (ROBINS-I) tool. Results In total, 3,942 unique articles were screened by title and abstract. After the initial screening process 63 articles were screened for relevance to the inquiry, of which seven studies were included. Three studies found no significant difference between both surgical approaches (p = 0.871, p = 0.120, p = 0.592). Three other studies found a lower rate of POH in the TL group compared to the RS group (p = 0.019, p p p = 0.006), but not after 1 year (p = 0.6). Conclusion The results of this systematic review show some evidence of a lower rate of POH in favor of the TL approach versus the RS approach for CPA tumor resection. Prospective research studies are needed to further investigate this finding.

Purpose The round window approach has become the most preferred option for cochlear implant (CI) insertion, however, sometimes it may not be possible due to the (in)visibility of the round window membrane (RWM). We addressed the prevalence, consequences and indicators of difficult detection of the RWM in cochlear implant surgery. Methods This study retrospectively analysed the operative reports and preoperative high resolution axial-computed tomography (CT) scans of a consecutive cohort of patients who underwent a CI insertion. The main outcomes were surgical outcomes of the RW approach, and assessment of radiological markers. Results The operative reports showed that RWM insertion was feasible in 151 out of 153 patients. In 18% of the patients the RWM was difficult to visualize. All these patients had at least one intraoperative event. The chorda tympani nerve (CTN) or posterior canal wall was affected in 8% of the 153 patients and the fallopian canal in 6%. These patients had a facial-chorda tympani nerve distance on the CT scan that was considerably smaller than normal patients (1.5 mm vs 2.3 mm). In addition, a prediction line towards the anterolateral side of the RWM was found to be more prevalent in these patients’ CT scans (sensitivity 81%, specificity 63%). Conclusion The RW approach is feasible in almost all patients undergoing CI surgery. Difficult visualisation of the RWM seems to lead to at least one intraoperative event. Radiological measures showed that these patients had a smaller facial recess and a more anteriorly placed facial nerve, which can be used to better plan a safe insertion approach.

OBJECTIVES/HYPOTHESIS Two types of electrode arrays for cochlear implants (CIs) are distinguished: lateral wall and perimodiolar. Scalar translocation of the array can lead to intracochlear trauma by penetrating from the scala tympani into the scala vestibuli or scala media, potentially negatively affecting hearing performance of CI users. This systematic review compares the lateral wall and perimodiolar arrays with respect to scalar translocation. STUDY DESIGN Systematic review. METHODS PubMed, Embase, and Cochrane databases were reviewed for studies published within the last 11 years. No other limitations were set. All studies with original data that evaluated the occurrence of scalar translocation or tip fold-over (TF) with postoperative computed tomography (CT) following primary cochlear implantation in bilateral sensorineuronal hearing loss patients were considered to be eligible. Data were extracted independently by two reviewers. RESULTS We included 33 studies, of which none were randomized controlled trials. Meta-analysis of five cohort studies comparing scalar translocation between lateral wall and perimodiolar arrays showed that lateral wall arrays have significantly lower translocation rates (7% vs. 43%; pooled odds ratio = 0.12). Translocation was negatively associated with speech perception scores (weighted mean 41% vs. 55%). Tip fold-over of the array was more frequent with perimodiolar arrays (X2 = 6.8, P

Painful neuromas are a devastating condition that is notoriously difficult to treat. The large number of techniques that have been attempted suggest that no one technique is superior. Neuromas often occur in the extremities, but iatrogenically caused pain in the head and neck area has also been described. This article describes 3 consecutive patients diagnosed with traumatic neuroma who underwent transection of the causative nerve, followed by capping of the nerve stump with a Neurocap. With a follow-up of 7 to 24 months, our results show a marked reduction in the pain scores of all 3 patients. The preliminary results indicate that this technique might be a viable treatment option for patients with a suspected neuroma in the head and neck area.

Persistent otorrhoea is a common issue for both children and adults, which can be caused by leakage of cerebrospinal fluid from the lateral skull base. Bacterial superinfection of the chronically humid middle ear, arising from continuous cerebrospinal fluid leakage, may contribute to an atypical clinical presentation. That is, otogenous cerebrospinal fluid leakage may mimic serious otitis media with concomitant conductive hearing loss, leading to a serious diagnostic delay in some patients. On the basis of three cases with cerebrospinal fluid leakage, resulting in persistent otorrhoea, we underline the importance of its timely diagnosis and treatment.

To assess whether day-case major otologic ear surgery is a safe and feasible alternative to inpatient surgery, while maintaining equal complication rates, and to identify causes of admission after day-case surgery.PubMed, Embase, and Cochrane.A systematic search was conducted. Studies reporting original data on the effect of day-case ear surgery on admission rate, patient satisfaction, and/or postoperative complications were included. Risk of bias of the selected articles was assessed using criteria based on the Cochrane Collaboration's tool for assessing risk of bias.A total of 1,734 unique studies were retrieved of which 35 articles discussing 34 studies were eligible for data extraction. The admission rates ranged from 0% to 88% following day-case endaural surgery, 0% to 13% following day-case stapes surgery, 0% to 82% following day-case mastoid surgery, and 0% to 15% following day-case cochlear implant surgery. Patient and parent satisfaction regarding day-case surgery ranged from 67% to 99%. Five studies comparing day-case and inpatient otologic surgery showed no difference in hearing outcome, postoperative complications, or patient satisfaction.The highest pooled admission rate was seen following day-case mastoid surgery. Studies comparing day-case and inpatient care suggest hearing results and postoperative complication rates in day-case otologic surgery are similar to inpatient otologic surgery in both children and adults. Therefore, day-case major otologic surgery seems to be a safe and feasible alternative to inpatient surgery for both children and adults.

Objective Given the lack of consensus on fixation techniques of the cochlear implant, this review aims to create an up-to-date overview of intra- and postoperative complications, focusing on migration of the internal receiver/stimulator (R/S) device and the electrode array. Data sources On June 29, 2020 we conducted a search in PubMed, Embase, Cochrane, Web of Science, and CINAHL. Keywords were "Cochlear implant," "complication," "migration," and synonyms. Study selection Studies were considered if: 1) the adult study population consisted of ≥ 10 patients, 2) the R/S device was fixated using the bony well or tight subperiostal pocket technique without bone-anchoring sutures or screws on the implant, and 3) migration of the R/S device or displacement of the electrode array were described as outcomes. Data extraction Study characteristics, interventions, follow-up, and outcomes were extracted. For critical appraisal, an adapted version of the Newcastle-Ottawa quality assessment scale for cohort studies was used. Data synthesis Seven studies were included (n = 430 patients). Migration of the R/S device was reported by three studies. Two studies applying the tight pocket technique reported migration rates ranging from 9.0 to 69.2%. One study using the bony bed technique reported migration of 100%, with an average of 2.5 mm. All studies lacked the required standard for comparability, assessment of outcome, and follow-up. Conclusions and relevance There is currently no evidence of a difference between the bony bed- and tight pocket fixation technique, regarding migration of the R/S device or the electrode array, in adult patients.

Background The peak of the global COVID-19 pandemic has not yet been reached, and many countries face the prospect of a second wave of infections before effective vaccinations will be available. After an initial phase of viral replication, some patients develop a second illness phase in which the host thrombotic and inflammatory responses seem to drive complications. Severe COVID-19 disease is linked to high mortality, hyperinflammation, and a remarkably high incidence of thrombotic events. We hypothesize a crucial pathophysiological role for the contact pathway of coagulation and the kallikrein-bradykinin pathway. Therefore, drugs that modulate this excessive thromboinflammatory response should be investigated in severe COVID-19. Methods In this adaptive, open-label multicenter randomized clinical trial, we compare low molecular weight heparins at 50 IU anti-Xa/kg twice daily—or 75 IU anti-Xa twice daily for intensive care (ICU) patients—in combination with aprotinin to standard thromboprophylaxis in hospitalized COVID-19 patients. In the case of hyperinflammation, the interleukin-1 receptor antagonist anakinra will be added on top of the drugs in the interventional arm. In a pilot phase, the effect of the intervention on thrombotic markers (D-dimer) will be assessed. In the full trial, the primary outcome is defined as the effect of the interventional drugs on clinical status as defined by the WHO ordinal scale for clinical improvement. Discussion In this trial, we target the thromboinflammatory response at multiple levels. We intensify the dose of low molecular weight heparins to reduce thrombotic complications. Aprotinin is a potent kallikrein pathway inhibitor that reduces fibrinolysis, activation of the contact pathway of coagulation, and local inflammatory response. Additionally, aprotinin has shown in vitro inhibitory effects on SARS-CoV-2 cellular entry. Because the excessive thromboinflammatory response is one of the most adverse prognostic factors in COVID-19, we will add anakinra, a recombinant interleukin-1 receptor antagonist, to the regimen in case of severely increased inflammatory parameters. This way, we hope to modulate the systemic response to SARS-CoV-2 and avoid disease progressions with a potentially fatal outcome. Trial registration The EU Clinical Trials Register 2020-001739-28. Registered on April 10, 2020.

Background In order to preserve residual hearing in patients with sensorineural hearing loss (SNHL) who receive a cochlear implant (CI), insertion trauma to the delicate structures of the cochlea needs to be minimized. The surgical approach comprises the conventional mastoidectomy-posterior tympanotomy (MPT) to arrive at the middle ear, followed by either a cochleostomy (CO) or the round window (RW) approach. Both techniques have their benefits and disadvantages. Another important aspect in structure preservation is the design of the electrode array. Two different designs are used: a “straight” lateral wall lying electrode array (LW) or a “pre-curved” perimodiolar lying electrode array (PM). Interestingly, until now, the best surgical approach and design of the implant is uncertain. Our hypothesis is that there is a difference in hearing preservation outcomes between the four possible treatment options. Methods We designed a monocenter, multi-arm, randomized controlled trial to compare insertion trauma between four groups of patients, with each group having a unique combination of an electrode array type (LW or PM) and surgical approach (RW or CO). In total, 48 patients will be randomized into one of these four intervention groups. Our primary objective is the comparison of postoperative hearing preservation between these four groups. Secondly, we aim to assess structure preservation (i.e., scalar translocation, with basilar membrane disruption or tip fold-over of array) for each group. Thirdly, we will compare objective outcomes of hearing and structure preservation by way of electrocochleography (ECochG). Discussion Cochlear implantation by way of a cochleostomy or round window approach, using different electrode array types, is the standard medical care for patients with severe to profound bilateral sensorineural hearing loss, as it is a relatively simple and low-risk procedure that greatly benefits patients. However, loss of residual hearing remains a problem. This trial is the first randomized controlled trial that evaluates the effect of cochlear insertion trauma of several CI treatment options on hearing preservation. Trial registration Netherlands Trial Register (NTR) NL8586. Registered on 4 May 2020. Retrospectively registered; 3/48 participants were included before registration.

Contains fulltext : 221007.pdf (Publisher’s version ) (Open Access) OBJECTIVE: To investigate the association between patients' preferred treatment and eventual treatment. Second, to compare patients with surgical treatment to watchful waiting in order to identify predictive factors for surgery. METHODS: A single-centre retrospective study was performed between December 2015 and August 2018. Patients (≥18 years) who used a patient decision aid (PDA) for gallstones or inguinal hernia were included. After their first surgical consultation, patients received access to an online PDA. The patients' preferred treatment after the PDA was compared with their choice of eventual treatment. Multivariable regression analyses were performed for predictive factors for surgery. RESULTS: In total, 567 patients with gallstones and 585 patients with an inguinal hernia were included. Of the patients with gallstones, 121 (21%) preferred watchful waiting, 367 (65%) preferred surgery, and 79 (14%) were not sure. The patients' preferred treatment was performed in 85.9%. Frequent pain attacks (OR 2.1, 95% CI 1.1-3.9, P = .020) and preference for surgery (OR 4.4, 95% CI 1.9-10.1, P = .001) independently predicted surgery. Of the patients with an inguinal hernia, 77 (13.2%) preferred watchful waiting, 452 (78.8%) preferred surgery, and 56 (9.6%) were not sure. The patients' preferred treatment was performed in 86.0%. The preference for surgery (OR 5.2, 95% CI 2.5-10.6, P

Objective The objective of our systematic review is to investigate the postoperative gustatory function of the chorda tympani nerve following noninflammatory ear surgery for which the chorda tympani is at risk for iatrogenic injury (stretching, handling, or sacrificing). Data Sources PubMed and EMBASE. Review Methods A PubMed and EMBASE databases search was conducted on November 15, 2016. Study inclusion criteria included: 1) ear surgery performed for noninflammatory ear diseases, and 2) gustatory function of the chorda tympani reported as an outcome. The quality of eligible studies was assessed using the risk of bias assessment tool for nonrandomized studies. Study characteristics and outcome data of the included studies were extracted. Results In total 1,094 articles were retrieved. Fourteen studies encompassing 1,062 operated ears were included after quality assessment. Stapedectomy was the most frequent surgical procedure performed in 398 ears. The follow-up time varied between 6 weeks and 99 months. Patients with a preserved chorda tympani were less symptomatic (24% was symptomatic) compared to patients with a stretched (53% was symptomatic) or sacrificed chorda tympani (47% was symptomatic). The recovery rate varied from 61% to 79%. The results of the electrogustometry and strip test showed a discrepancy with the subjective complaints of the patients. Conclusion Patients with a stretched chorda tympani were slightly more symptomatic compared to patients with a sacrificed chorda tympani. Therefore, in cases for which the chorda tympani greatly hinders a proper view of the surgical field, sacrificing the nerve could be considered to maximize surgical performance and have a satisfactory postoperative result. Laryngoscope, 2017

Background: Hearing loss and otitis media are frequently reported in patients with 22q11.2 deletion syndrome. Objective of review: Our objective was to review the current literature on the prevalence of hearing loss and otologic manifestations in patients with 22q11.2 deletion syndrome. Type of review: Systematic review. Search strategy: We conducted a systematic search in PubMed and Embase combining the term “22q11.2 deletion syndrome” and synonyms with “hearing loss” and “otologic manifestations” and synonyms. Evaluation method: We screened title/abstract and full text of all retrieved articles on pre-defined in- and exclusion criteria. The remaining articles were assessed on risk of bias. Outcome measures included the prevalence of hearing loss and otologic manifestations such as otitis media. Results: Our search yielded 558 unique studies of which a total of 25 articles were included for critical appraisal and data extraction. Twenty-one studies reported on hearing loss, and 21 studies on otologic manifestations. The prevalence of hearing loss varied from 6.0% to 60.3%, where in most studies conductive hearing loss was most prevalent. Rates of recurrent or chronic otitis media varied from 2.2% to 89.8%. Conclusion: Although a very broad range in prevalences is reported in different studies, hearing loss and recurrent or chronic otitis media are frequently present in patients with 22q11.2 deletion syndrome. Regular check-ups and audiometric testing are advised in these patients.

IntroductionOtosclerosis is a common cause of acquired conductive hearing loss and can be treated using hearing aids or surgically. Surgical success rates or surgical results are usually reported using pure-tone audiometric thresholds and/or speech discrimination scores. Audiometric results and patient-reported quality of life after stapes surgery do not seem to correlate well. It is therefore our opinion that health-related quality of life measurements should be implemented as an additional outcome measure after stapes surgery. So far, there is a lack of a valid, reliable and clinically feasible measuring tool for determining health-related quality of life in Dutch patients with otosclerosis who undergo stapes surgery.Methods and analysisA prospective validation study was designed to translate and validate the disease-specific Stapesplasty Outcome Test 25 (SPOT-25) in a population of Dutch patients with otosclerosis who undergo primary stapes surgery. A total of 125 patients with otosclerosis who will be undergoing primary stapes surgery and 50 healthy controls will be included. The patients with otosclerosis will fulfil several questionnaires preoperatively, 6 to 8 weeks postoperatively and 8 to 10 weeks postoperatively with a 2-week interval between the postoperative administrations. The patients’ audiometric results, which are measured routinely before and after undergoing primary stapes surgery, will also be used. The healthy controls will fulfil the translated SPOT-25 once. First, the original SPOT-25 will be translated from German to Dutch in a six-step process. Second, the translated SPOT-25 will be pilot-tested in a subset of patients. Lastly, validity, reliability and responsiveness of the translated SPOT-25 will be analysed.Ethics and disseminationThe research protocol was approved by the Institutional Review Board of the University Medical Center Utrecht (protocol 18–768/C; V.1, November 2018). Initially, we planned to include 50 patients. At a later stage we decided to increase the sample size to 100 patients. We notified the Institutional Review Board of this change to the protocol. The trial results will be disseminated through peer-reviewed medical journals and presented at scientific conferences.Trial registration numberNL7586.

A 35-year-old woman developed acute swallowing problems caused by a big oral blood blister after eating nuts. The blister ruptured 32 hours later and healed without scarring. 'Angina bullosa haemorrhagica' was diagnosed after ruling out bleeding disorders.

Objectives: To evaluate pure-tone audiometric results in otosclerosis patients undergoing revision stapes surgery following previous middle ear interventions. Methods: A retrospective cohort study was performed in a tertiary referral center. Consecutive otosclerosis patients who underwent revision stapes surgeries, following previous middle ear interventions, for persistent conductive hearing loss, recurrent conductive hearing loss, or vertigo and had available postoperative pure-tone audiometry were included. Mean pre- and postoperative air conduction, bone conduction, and air-bone gap averaged over 0.5, 1, 2, and 3 kHz were obtained. Audiometric outcomes were obtained at 7 weeks postoperatively. Results: In total, 63 consecutive otosclerosis patients who underwent 66 revision stapes surgeries were included. Air-bone gap improved significantly with a mean gain of 19 dB (95% CI, 15-22). Air conduction improved significantly with a mean gain of 18 dB (95% CI, 14-23). Bone conduction did not change significantly, with a mean deterioration of 0 dB (95% CI, –2 to 1). Air-bone gap closure to 10 dB or less was achieved in 38% of cases and to 20 dB or less in 80% of cases. Indication for surgery, previous type of procedure, primary cause of failure, and current surgical technique were not significantly associated with air-bone gap closure to 10 dB or less. Indication for surgery and primary cause of failure were associated with one another. Conclusions: Compared to the available literature, a slightly larger gain in air conduction and air-bone gap was achieved in our study. Air-bone gap closure to 10 dB or less was achieved less often in our study.

For both gallbladder removal and inguinal hernia repair, it is important to include patients’ perspective in the decision-making process, as watchful waiting is an accepted alternative in selected patients. The aim of this study was to evaluate operation rates before and after implementation of decision aids (DAs) and to assess patient compliance with the use of DAs. A single-centered retrospective study was performed, including all patients ≥18 years referred to the surgical outpatient clinic with symptomatic gallstones or an inguinal hernia between January 2014 and December 2017. Operation rates before and after implementation of DAs (December 2015) were compared. In addition, patient compliance with the use of DAs and satisfaction with final treatment were assessed. Overall, 1625 patients with gallstones and 1798 patients with an inguinal hernia were included. After implementation, DAs were provided to 512 patients (63.1%) with gallstones of whom 80.7% (413/512) used the DA and to 528 patients (58.8%) with an inguinal hernia, which was used by 80.7% (426/528). Before implementation, the operation rate in patients with gallstones was 72.0% (586/814) and after implementation 56.7% (460/811) (− 15.3%, p

OBJECTIVE: To evaluate the effectiveness of hyperbaric oxygen in the treatment of patients with idiopathic sudden sensorineural hearing loss (ISSHL). DATA SOURCES: An Embase, MEDLINE and Cochrane search were utilised to identify various clinical trials on the treatment of ISSHL. Studies that were published between 2002 and 2018 and written in the English, Dutch or German language were included. Search terms included synonyms for idiopathic sudden hearing loss. DATA SYNTHESIS: A total of 16 articles were identified regarding hyperbaric oxygen therapy. All patients were evaluated with pure-tone audiometry. A major part of the cases presented with unilateral hearing loss(bilateral hearing loss less than 5%). In several studies, the average of the mean hearing gain at five contiguous frequencies was significantly higher in the hyperbaric oxygen (HBO) therapy and systemic steroid (SS) group in patients with severe or profound hearing impairment. They recorded a significant treatment effect (p = 0.005) of HBO + SS therapy on patients with an initial hearing loss of ≥ 81 dB. CONCLUSIONS: On the whole group of ISSHL patients, no significant difference was demonstrated between the intervention and control group. However, in severe or profound hearing-impaired ISSHL patients, significant benefit was observed in the intervention group. These results likely indicate that adding HBO to steroid therapies might be of benefit in cases of severe and profound hearing impairment.

Introduction: Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disease affecting the cytotoxic pathway. Due to the recent advances in molecular diagnosis, immuno-chemo therapy, and hematopoietic stem cell transplantation treatment, FHL survival rates have drastically increased. Case Presentation: Herein, we describe a case of FHL type 5 presenting with low-frequency sensorineural hearing loss. Alongside our reported case, 6 additional patients were identified in the literature. Management and Outcome: The progressive nature of FHL disorder may cause bilateral, low-frequency, irreversible sensorineural hearing loss. This type of hearing loss should be considered among the long-term sequelea presenting with FHL5. Discussion: We recommend audiological evaluation at initial FHL5 diagnosis to assess for hearing functions. Follow-up in audiology should be part of the long-term monitoring of patients with FHL5 as hearing loss could develop long after diagnosis.

The objective of this study is to report the surgical outcome after middle fossa approach (MFA) plugging in patients suffering from a superior semi-circular canal dehiscence (SCD) syndrome. This is a retrospective case review. Tertiary referral center. Sixteen ears in 13 patients with a SCD syndrome suffering from severe and disabling vestibular symptoms with a bony dehiscence on CT scan >3 mm and decreased threshold of cervical vestibular evoked potentials (cVEMPs). We assessed preoperatively: clinical symptoms, hearing, cVEMPs threshold, size of dehiscence and videonystagmography (VNG) with caloric and 100 Hz vibratory tests. Postoperatively, we noted occurrences of neurosurgical complication, evolution of audiological and vestibular symptoms, and evaluation of cVEMP data. Tullio’s phenomenon was observed in 13 cases (81.3 %) and subjectively reported hearing loss in seven (43.7 %). All patients were so disabled that they had to stop working. No neurosurgical complications were observed in the postoperative course. In three cases (16.6 %), an ipsilateral and transitory immediate postoperative vestibular deficit associated with a sensorineural hearing loss (SNHL) was noted, which totally resolved with steroids and bed rest. All patients were relieved of audiological and vestibular symptoms and could return to normal activity with a mean follow-up of 31.1 months (range 3–95). No patient had residual SNHL. cVEMPs were performed in 14 ears postoperatively and were normalized in 12 (85.7 %). Two of the three patients operated on both sides kept some degree of unsteadiness and oscillopsia. MFA plugging of the superior semi-circular canal is an efficient and non-hearing deteriorating procedure.

To compare the effect of vasodilators with that of corticosteroids in patients with idiopathic sudden sensorineural hearing loss (ISSHL). A search in PubMed, Cochrane, and Embase was conducted. Two reviewers screened the data sources to identify articles that comply with predefined inclusion criteria. Studies that compared the therapeutic effect of vasodilators with prednisone or placebo in patients with ISSHL were identified. Five articles were selected, involving a total of 611 patients. The odds ratios for perceptive hearing levels (pure tone average) post treatment varied between 0.58 and 2.18. One study demonstrated a cumulative effect (optimal hearing recovery) when vasodilators and glucocorticoids were combined (odds ratio, 1.82). Vasodilators have no beneficial effect on the treatment of ISSHL. Some evidence suggests that a combination of vasodilators with steroid treatment results in better hearing outcome than the use of corticosteroids alone.

Item does not contain fulltext OBJECTIVES/HYPOTHESIS: To investigate hearing capabilities and self-reported benefits of simultaneous bilateral cochlear implantation (BiCI) compared with unilateral cochlear implantation (UCI) after a 2-year follow-up and to evaluate the learning effect of cochlear implantees over time. STUDY DESIGN: Multicenter randomized controlled trial. METHODS: Thirty-eight postlingually deafened adults were included in this study and randomly allocated to either UCI or simultaneous BiCI. Our primary outcome was speech intelligibility in noise, with speech and noise coming from straight ahead (Utrecht-Sentence Test with Adaptive Randomized Roving levels). Secondary outcomes were speech intelligibility in noise with spatially separated sources, speech intelligibility in silence (Dutch phoneme test), localization capabilities and self-reported benefits assessed with different quality of hearing and quality of life (QoL) questionnaires. This article describes the results after 2 years of follow-up. RESULTS: We found comparable results for the UCI and simultaneous BiCI group, when speech and noise were both presented from straight ahead. Patients in the BiCI group performed significantly better than patients in the UCI group, when speech and noise came from different directions (P = .01). Furthermore, their localization capabilities were significantly better. These results were consistent with patients' self-reported hearing capabilities, but not with the questionnaires regarding QoL. We found no significant differences on any of the subjective and objective reported outcomes between the 1-year and 2-year follow-up. CONCLUSIONS: This study demonstrates important benefits of simultaneous BiCI compared with UCI that remain stable over time. Bilaterally implanted patients benefit significantly in difficult everyday listening situations such as when speech and noise come from different directions. Furthermore, bilaterally implanted patients are able to localize sounds, which is impossible for unilaterally implanted patients. LEVEL OF EVIDENCE: 1b Laryngoscope, 127:1161-1168, 2017.

Here, we provide a literature overview of cases with protruding molding material for earplugs or hearing aids and subsequent required treatment, including our own cases. Patients at risk are those with impaired tympanic membranes or who previously underwent otologic surgery. Symptoms such as otalgia, tinnitus, and vertigo are alarming but do not always arise. In case of doubt, a CT scan is of additive value to prepare for adequate surgical removal and to limit potential damage. A stepwise approach for the clinician on how to address these challenging cases is presented, based upon the literature and our own experience.

Animal studies have evidenced protection of the auditory nerve by exogenous neurotrophic factors. In order to assess clinical applicability of neurotrophic treatment of the auditory nerve, the safety and efficacy of neurotrophic therapies in various human disorders were systematically reviewed. Outcomes of our literature search included disorder, neurotrophic factor, administration route, therapeutic outcome, and adverse event. From 2103 articles retrieved, 20 randomized controlled trials including 3974 patients were selected. Amyotrophic lateral sclerosis (53%) was the most frequently reported indication for neurotrophic therapy followed by diabetic polyneuropathy (28%). Ciliary neurotrophic factor (50%), nerve growth factor (24%) and insulin-like growth factor (21%) were most often used. Injection site reaction was a frequently occurring adverse event (61%) followed by asthenia (24%) and gastrointestinal disturbances (20%). Eighteen out of 20 trials deemed neurotrophic therapy to be safe, and six out of 17 studies concluded the neurotrophic therapy to be effective. Positive outcomes were generally small or contradicted by other studies. Most non-neurodegenerative diseases treated by targeted deliveries of neurotrophic factors were considered safe and effective. Hence, since local delivery to the cochlea is feasible, translation from animal studies to human trials in treating auditory nerve degeneration seems promising.

Introduction Cochlear implantation is an increasingly common procedure in the treatment of severe to profound sensorineural hearing loss (SNHL) in children and adults. It is often performed as a day-case procedure. The major drive towards day-case surgery has been from a logistical, economical and societal perspective, but we also speculate that the patient9s quality of life (QoL) is at least equal to inpatient surgery if not increased as a result of rapid discharge and rehabilitation. Even though cochlear implantation seems well suited to a day-case approach and this even seems to be common practice in some countries, evidence is scarce and of low quality to guide us towards the preferred treatment option. Methods and analysis A single-centre, non-blinded, randomised, controlled trial was designed to (primarily) investigate the effect on general QoL of day-case cochlear implantation compared to inpatient cochlear implantation and (secondarily) the effect of both methods on (subjective) hearing improvement, disease-specific QoL, tinnitus, vertigo and cost-effectiveness. 30 adult patients with severe to profound bilateral postlingual SNHL who are eligible for unilateral cochlear implantation will be randomly assigned to either the day-case or inpatient treatment group. The outcome measures will be assessed using auditory evaluations, questionnaires (preoperatively, at 1-week, 3-week, 3-month and 1-year follow-up) and costs diaries (weekly during the first month postoperatively, after which once in a month until 1-year follow-up). Preoperative and postoperative outcomes will be compared. The difference in costs and benefit will be represented using the incremental cost utility/effectiveness ratio. The analyses will be carried out on an intention-to-treat basis. Ethics and dissemination This research protocol was approved by the Institutional Review Board of the UMC Utrecht (NL45590.041.13; V.5, November 2015). The trial results will be disseminated through peer-reviewed medical journals and presented at scientific conferences. Trial registration number NTR4464; Pre-results.

OBJECTIVE:: To investigate skin-related postoperative complications from tissue preservation approaches in percutaneous bone conduction device (BCD) implantations. DATA SOURCES:: PubMed, Embase, and Cochrane Library. STUDY SELECTION:: We identified studies on BCDs including the opted surgical technique and derived complications. Retrieved articles were screened using predefined inclusion criteria. Critical appraisal included directness of evidence and risk of bias. Studies that successfully passed critical appraisal were included. DATA EXTRACTION:: Outcome measures included patient demographics, surgery time, follow-up time, and complications reported by Holgers classification. DATA SYNTHESIS:: We selected 18 articles for data extraction; encompassing 381 BCDs implanted using non-skin thinning approaches. Four studies reported an implantation technique using the punch method (81 implants), 13 studies applied the linear incision technique without soft tissue reduction (288 implants) and one study used the Weber technique (12 implants). Holgers 3 was described in 2.5% following the punch technique, in 5.9% following the linear incision technique, and in no implants following the Weber technique. One patient was mentioned having Holgers 4, skin overgrowth was reported in 6 patients, and 10 studies compared their non-skin thinning technique with a skin-thinning technique. Overall, the soft tissue preservation technique had a similar or superior complication rate, shorter surgical time, and better and faster healing, compared with the soft tissue reduction technique. CONCLUSION:: Tissue preservation surgical techniques for percutaneous BCDs have limited postoperative skin complication rates. Moreover, these techniques are suggested to have at least similar complications rates compared with skin-thinning techniques.

Item does not contain fulltext OBJECTIVES: To describe the audiometric results in a consecutive series of patients with congenital ossicular aplasia (Class 4a) or dysplasia of the oval and/or round window (Class 4b), which might include a possible anomalous course of the facial nerve. STUDY DESIGN: Retrospective chart study. SETTING: Tertiary referral center. PATIENTS: A tertiary referral center study with a total of 14 patients with congenital minor ear anomalies as part of a consecutive series (n = 89) who underwent exploratory tympanotomies (15 ears). MAIN OUTCOME MEASURES: Audiometric results. RESULTS: In 8 of 15 ears, ossicular reconstruction was attempted. In the short term (1 mo), there was a serviceable hearing outcome (air-bone gap closure to within 25 dB) in 4 ears. However, the long-term results showed deterioration because of an increased air-bone gap in all but 1 ear. No facial nerve lesion was observed postoperatively. CONCLUSION: Congenital dysplasia or aplasia of the oval and/or round window is an uncommon congenital minor ear anomaly. Classical microsurgical opportunities are rare in this group of anomalies. Newer options for hearing rehabilitation, such as the osseointegrated passive bone conduction devices, have become viable alternatives for conventional air conduction hearing devices. In the near future, upcoming active bone conduction devices might become the most preferred surgical option. In cases in which the facial nerve is only partially overlying the oval window, a type of malleostapedotomy procedure might result in a serviceable postoperative hearing level. 01 juli 2012

Item does not contain fulltext The aim of the study was to report otologic and audiologic characteristics in a group of children with Turner syndrome (TS) and correlate these findings to karyotype. Additionally, we give recommendations for the otologic care of these children. Sixty children (age 1.7-21.2 years) were included in this retrospective study. Medical history and karyotypes were recorded and otologic and audiologic evaluation was performed. A history of recurrent otitis media was reported in 41/60 (68%) children and 3/60 (5%) had suffered from cholesteatoma. Audiometric data in 56 children revealed that normal hearing was only present in 33/112 (29%) ears. All other ears 79/112 (71%) were classified in five different audiometric categories for hearing loss. Hearing thresholds in general appeared to be about 10-11 dB worse in children with a monosomy 45,X or isochromosome (both have a total deletion of the short (p) arm of the X-chromosome) compared to those having a mosaicism or structural anomaly (partial deletion, or total deletion in only a few cells). Our findings support the hypothesis that hearing can be affected by loss of the p-arm of the X-chromosome. It is for the first time that a relation between hearing problems and karyotype is statistically confirmed in a large group of children with TS.

Objective To develop and internally validate a prediction model for tinnitus recovery following unilateral cochlear implantation. Design A cross-sectional retrospective study. Setting A questionnaire concerning tinnitus was sent to patients with bilateral severe to profound hearing loss, who underwent unilateral cochlear implantation at the University Medical Center Utrecht, the Netherlands, between 1 January 2006 and 31 December 2015. Participants Of 137 included patients, 87 patients experienced tinnitus preoperatively. Data of these 87 patients were used to develop the prediction model. Primary and secondary outcome measures The outcome of the prediction model was tinnitus recovery. Investigated predictors were: age, gender, duration of deafness, preoperative hearing performance, tinnitus duration, severity and localisation, follow-up duration, localisation of cochlear implant (CI) compared with tinnitus side, surgical approach, insertion depth of the electrode, CI brand and difference in hearing threshold following cochlear implantation. Multivariable backward logistic regression was performed. Missing data were handled using multiple imputation. The performance of the model was assessed by the calibrative and discriminative ability of the model. The prediction model was internally validated using bootstrapping techniques. Results The tinnitus recovery rate was 40%. A lower preoperative Consonant-Vowel-Consonant (CVC) score, unilateral localisation of tinnitus and larger deterioration of residual hearing at 250 Hz revealed to be relevant predictors for tinnitus recovery. The area under the receiver operating characteristics curve (AUC) of the initial model was 0.722 (IQR: 0.703–0.729). After internal validation of this prediction model, the AUC decreased to 0.696 (IQR: 0.667–0.700). Conclusion and relevance Lower preoperative CVC score, unilateral localisation of tinnitus and larger deterioration of residual hearing at 250 Hz were significant predictors for tinnitus recovery following unilateral cochlear implantation. The performance of the model developed in this retrospective study is promising. However, before clinical use of the model, the conduction of a larger prospective study is recommended.

Attachment to and proliferation on the substrate are deemed important considerations when Schwann cells (SCs) are to be seeded in synthetic nerve grafts. Attachment is a prerequisite for the SCs to survive and fast proliferation will yield large numbers of SCs in a short time, which appears promising for stimulation of peripheral nerve regeneration. The aim of the present study was to compare the adhesion and proliferation of human Schwann cells (HSCs) on different substrates. The following were selected for their suitability as an internal coating of synthetic nerve grafts; the extracellular matrix proteins fibronectin, laminin and collagen type I and the poly-electrolytes poly( -lysine) (PDL) and poly(ethylene-imine) (PEI). On all coatings, attachment of HSCs was satisfactory and comparable, indicating that this factor is not a major consideration in choosing a suitable coating. Proliferation was best on fibronectin, laminin and PDL, and worst on collagen type I and PEI. Since nerve regeneration is enhanced by laminin and/or fibronectin, these are preferred as coatings for synthetic nerve grafts seeded with SCs.

Object. Recent progress in the understanding of cerebral plastic changes that occur after an intercostal nerve (ICN)—musculocutaneous nerve (MCN) transfer motivated a study with functional magnetic resonance (fMR) imaging to map reorganization in the primary motor cortex. Methods. Eleven patients with traumatic root avulsions of the brachial plexus were studied. Nine patients underwent ICN—MCN transfer to restore biceps function and two patients were studied prior to surgery. The biceps muscle recovered well in seven patients who had undergone surgery and remained paralytic in the other two patients. Maps of neural activity within the motor cortex were generated for both arms in each patient by using fMR imaging, and the active pixels were counted. The motor task consisted of biceps muscle contraction. Patients with a paralytic biceps were asked to contract this muscle virtually. The location and intensity of motor activation of the seven surgically treated arms that required good biceps muscle function were compared with those of the four arms with a paralytic biceps and with activity obtained in the contralateral hemisphere regulating the control arms. Activity could be induced in the seven surgically treated patients whose biceps muscles had regained function and was localized within the primary motor area. In contrast, activity could not be induced in the four patients whose biceps muscles were paralytic. Neither the number of active pixels nor the mean value of their activations differed between the seven arms with good biceps function and control arms. The weighted center of gravity of the distribution of activity also did not appear to differ. Conclusions. Reactivation of the neural input activity for volitional biceps control after ICN—MCN transfer, as reflected on fMR images, is induced by successful biceps muscle reinnervation. In addition, the restored input activity does not differ from the normal activity regulating biceps contraction and, therefore, has MCN acceptor qualities. After ICN—MCN transfer, cerebral activity cannot reach the biceps muscle following the normal nervous system pathway. The presence of a common input response between corticospinal neurons of the ICN donor and the MCN acceptor seems crucial to obtain a functional result after transfer. It may even be the case that a common input response between donor and acceptor needs to be present in all types of nerve transfer to become functionally effective.

Traumatic root avulsions of the brachial plexus constitute a devastating lesion resulting in loss of function of the upper limb and carry a large emotional and socioeconomic impact. In this literature survey, the different factors involved in root avulsion are discussed in combination with various surgical techniques for repair of experimental ventral root avulsion. Until now repair of root avulsions did not generate unequivocal proof of recovery of limb function, particularly of the hand. More experimental studies are needed to assess the efficacy of several repair techniques, the optimal timing for surgery, and the complications associated with spinal cord manipulation.

Object. The authors conducted a study to evaluate the results of a unique surgical procedure for treating primary lumbar stenosis in patients with achondroplasia, based on its distorted anatomical dimensions. Methods. A consecutive single-center series of 36 achondroplastic dwarfs with symptomatic lumbar stenosis underwent dynamic lumbar myelography to determine the most stenotic lumbar segment. This level was surgically decompressed by selective widening of the lumbar interapophyseolaminar diameter; laminectomy was not performed. The L2–3 level was decompressed in 97% of the cases and the L1–2 and L3–4 levels in 58 and 61%, respectively. Lower lumbar levels were rarely affected by stenosis. Stenosis typically did not occur at the level of the lamina, where the pseudoscalloping of the vertebral body compensates for the spinal narrowing, obviating the need for laminectomy. Complete relief of symptoms was demonstrated in 25 (71.4%) of 35 patients. Tolerable disease remained in eight patients (22.9%) postoperatively. In two cases (5.7%) surgery resulted in no benefit. One case was lost to follow up. The presence of a lumbar epidural hematoma complicated one procedure. Conclusions. In this series the interapophyseolaminar decompression was demonstrated to be an effective and safe procedure for the treatment of symptomatic stenosis in achondroplasia. The upper lumbar segments, particularly the L2–3 level, were most frequently involved. Dynamic lumbar myelography proved mandatory in demonstrating the symptomatic level.

Object. Patients harboring colloid cysts of the third ventricle can present with acute neurological deterioration, or the first indication of the lesion may appear when the patient suddenly dies. The risk of such an occurrence in a patient already identified as harboring a colloid cyst is unknown. The goal of this study was to estimate the risk of acute deterioration in patients with colloid cysts. Methods. A retrospective study was made of a cohort of patients with newly diagnosed colloid cysts who were recruited in The Netherlands between January 1, 1993, and December 31, 1997. Seventy-eight patients were identified, all of whom displayed symptoms. Twenty-five patients (32%) presented with symptoms of acute deterioration; four patients died suddenly and the cysts were discovered at autopsy. The overall mortality rate was 12%. Results of a multivariate logistic regression analysis demonstrated that no subgroup of patients presenting without acute deterioration could be identified on the basis of patient age, duration of symptoms, cyst size, or the presence of hydrocephalus. The national incidence of colloid cysts in The Netherlands is 1/106 person-years; the prevalence was estimated to be 1800 asymptomatic colloid cysts. Conclusions. Acute deterioration was a frequent presentation among a national cohort of Dutch patients harboring symptomatic colloid cysts. The risk of acute deterioration in a symptomatic patient with a colloid cyst in The Netherlands is estimated to be 34%. The estimated risk for an asymptomatic patient with an incidental colloid cyst is significantly lower. These results strongly advocate the selection of surgical treatment for patients with symptomatic colloid cysts.

INTRODUCTION Hydrops and abnormalities of inner fluid pressure are involved in some otologic diseases such as Meniere's disease (MD). However, demonstrating abnormal perilymphatic or endolymphatic pressure is challenging. Multifrequency tympanometry studies in MD patients demonstrated an increase of the width of conductance tympanograms (outside an attack) compared with controls. To confirm that the increase in conductance width is caused by hyperpressure and not hypopressure in these patients tested outside an attack, we assessed the effect of changes in inner ear fluid pressure caused by body tilt on the results of multifrequency admittancemetry tympanograms. MATERIALS AND METHODS A multifrequency tympanometry including conductance (G) tympanogram at 2 kHz and resonance frequency measurements were performed in 20 volunteers (40 ears) free of otologic or neurologic disease. The measures were collected in three different positions: vertical, supine, and Trendelenburg positions. RESULTS Changes in inner ear fluid pressure caused by body tilt induced an increase in the width of G tympanograms. In the vertical position, the mean value was 141.7 ± 56.5 daPa; in the supine position, it increased to 158 ± 58.3 daPa; and increased even more in the Trendelenburg position (20 degrees), with a mean of 184 ± 69.6 daPa (p < 0.01). Resonance frequency also increased in the Trendelenburg position.We conclude that the increased width of G tympanograms in MD patients outside an attack may be caused by an increase in inner ear fluid pressure.

Object. Proximal spinal nerve stumps were used as donor sites for grafts to repair brachial plexus traction lesions. The quality of the stumps was assessed histologically, and its correlation with the strength attained in the target muscle was studied.Methods. Four histopathological parameters in frozen tissue sections of 31 C-5 or C-6 nerve stumps were examined by a neuropathologist. The total quantity of myelin was compared with normal values. Also, thick myelinated fibers, fibrosis, and misdirected axons were assessed. Stumps embedded in plastic were used in a morphometric study of myelinated fiber profiles. The fiber density, mean size, and size distribution in five donor stumps were determined; three normal C-5 spinal nerves obtained at autopsy served as controls. Finally, the relative area occupied by fiber profiles and interspace was computed. Linear regression was used as a multivariate analysis, adjusting the outcome of surgical repair for effects of age, interval between trauma and surgery, and graft length.Histopathological examination showed that the total quantity of myelin in donor stumps used for biceps muscle reinnervation was considerably reduced. On morphometric examination the fiber density did not differ significantly between stumps obtained in patients and control stumps obtained at autopsy. However, a significant reduction of the area occupied by myelinated fibers was measured: from 46% in controls to 13% in patients (p < 0.0001). Likewise, a significant reduction was found in the mean fiber size: from 7.4 µm in controls to 3.7 µm in patients (p < 0.0001).The relationship between the myelin quantity in the proximal stump and the grade of biceps muscle recovery was statistically significant (p = 0.02). From the 95% confidence interval it was concluded that the estimated effect of a mean increase of myelinated fibers by 25% almost equals an increase in recovery of one point on the Medical Research Council scale (grade range 0–5).Conclusions. Both histopathological and morphometric examination showed a reduction of the quantity of myelin in C-5 or C-6 stumps used as donor sites for grafts. The amount of myelin is significantly correlated with biceps muscle function after nerve grafting. Because it is possible to assess the quantity of myelin by intraoperative examination of frozen sections, this correlation is potentially useful in the decision-making process of whether to use stumps for grafting or to use nerve transfer procedures to restore biceps muscle function.

Object. Hypoglossal nerve (12th cranial nerve) transfer was performed to treat the sequelae of brachial plexus root avulsion in 12 adults and two infants, and the patients were followed to assess the effectiveness of the surgery.Methods. The 12th cranial nerve was transected at the base of the tongue, and a sural nerve graft was used to bridge the gap between the donor (12th) and recipient nerves: C-5 spinal, axillary, suprascapular, or musculocutaneous nerve. The mean graft length in adult patients was 15.75 ± 5.5 cm (± standard deviation, median 14.5 cm) and in the two infants the graft lengths were 7 and 8 cm, respectively. After a mean postoperative interval of 1138 ± 254 days, electromyographic examination of the target muscles showed tongue movement—related activity in all patients. Muscle force strength measured according to the Medical Research Council's guidelines, was Grade 3 or higher in 21% of patients. Contraction, however, could only be attained by tongue movements, and volitional control was not achieved.Conclusions. Although recovery of muscle strength was obtained by 12th cranial nerve transfer, the functional gain remained virtually nonexistent because central control was missing.

Many surgical techniques are employed for access to midline lesions of the anterior and middle fossa. A convenient one is the midfrontobasal approach. For various indications this surgical procedure was performed in 105 patients. Its benefits are outlined and a method for dealing with the two main drawbacks, postoperative loss of the sense of smell and the presumed risk of infection related to the inevitable opening of the frontal sinus, are described. In 83% of the cases the authors were able to anatomically retain the olfactory nerves, with preservation of the sense of smell in at least 65% of them. The frontal sinus was opened in 76% of the cases. The incidence of infection in the simple and quick integral reconstruction appeared 1%, equal to the rate of infection in the classical reduction of the frontal sinus by a periosteal flap.

Objectives To report the hearing impairment in a new autosomal recessive metabolic disorder due to a mutation in the ANKH gene and to report the outcomes of exploratory tympanotomy. Study design Retrospective chart study. Setting Tertiary referral center. Patients One large consanguineous family was examined. Three patients underwent exploratory tympanotomy. Intervention Exploratory tympanotomies in three patients. Main outcome measures Medical and otological histories; postoperative hearing outcomes. Results In the patients who received tympanotomies, a postoperative hearing gain of between 5 and 20 dB was noted, with a residual air-bone gap of between 6 and 35 dB (follow-up between 4 and 67 months). The sensorineural component of the hearing impairment varies greatly, between 4 and 23 dB, and this factor might also affect the final hearing outcome. Conclusions Exploratory tympanotomy might improve the hearing outcome in patients with this syndrome and therefore surgery has a limited audiometric benefit in general. Based on anatomical findings, a congenital origin for the ossicular chain anomaly seems likely. It remains unclear whether the sensorineural component of the hearing impairment is progressive and this should be investigated further.

Sarcoidosis is a multisystem disorder of unknown cause with a highly variable course. Corticosteroids are considered the standard agent for treatment, however there is no consensus about when and in whom therapy should be initiated, what dose should be given and for how long. There seems to be a limited benefit on chest radiographic findings, forced vital capacity and diffusing capacity. The evidence supporting the disease-modulating effect is limited. Cytotoxic agents are often used as steroid-sparing in patients requiring chronic therapy, however there are only little randomized controlled trials to support their use and side effects are common. Tumour necrosis factor-a is thought to be crucial in the development of the typical granulomas in sarcoidosis. Many case reports and case series suggest that specific therapy targeted against this cytokine is very effective. Despite these promising results, only limited evidence is found in multicenter randomized controlled trials.

Objectives: We describe the audiometric results following surgery in a consecutive series of patients with a congenital ossicular middle ear disorder that was associated with a mobile stapes footplate. Methods: We performed a retrospective analysis of patient charts from a tertiary referral center. A total of 23 patients (23 ears) underwent exploratory tympanotomy and ossicular reconstruction between 1986 and 2001. The main outcome measure was the audiometric results. Results: Overall, we observed a mean gain in air conduction pure tone average of 17 dB (from 47 dB to 30 dB), a sensorineural deterioration of 3 dB, and a mean postoperative air-bone gap of 19 dB (mean preoperative air-bone gap of 38 dB). The air-bone gap closure was 20 dB or less in 15 of the 23 cases (65%), in agreement with the few results reported in the literature. Moreover, the audiometric results remained stable. In the syndromic group, the mean gain in air conduction was only 13 dB, which was worse than that observed for the nonsyndromic ears. Conclusions: Surgery for congenital ossicular chain anomalies with a concomitant mobile stapes footplate provides positive audiometric outcomes. Most ears had some sensorineural impairment (10 to 20 dB), which influenced the final hearing level attained after surgery. Preoperative assessment is mandatory to search for syndromal diagnoses, which might be important for patient counseling and prognosis.

To describe the audiometric results after stapes surgery in a consecutive series of patients with stapes footplate ankylosis combined with another ossicular middle ear anomaly.A retrospective analysis of charts collected between 1986 and 2001.A tertiary referral center.A total of 25 patients (30 ears) underwent exploratory tympanotomies and ossicular reconstruction.Audiometric results.Overall, a mean gain in air conduction of 18 dB (from 49 dB to 31 dB) and a mean postoperative air-bone gap (ABG) of 20 dB (mean preoperative ABG, 40 dB) were observed. The ABG closure was 20 dB or less in 70% of cases, which is in agreement with the few results reported in the literature. Moreover, the audiometric results remained stable. In the group of ears with a syndrome, the mean gain of air conduction was only 19 dB, which was comparable to that observed among nonsyndromic ears.Surgery for congenital stapes footplate ankylosis with a concomitant ossicular chain anomaly can provide worthwhile hearing improvement. The ABG closure was 20 dB or less in 21 of 30 ears (70%). Most ears had some sensorineural impairment (10-20 dB), which influenced the final hearing level after surgery. Over recent decades, the technique of the malleostapedotomy procedure has been improved. Preoperative assessment is mandatory for syndromal diagnoses, which might be important for patient counseling and prognosis.