A single dose of AC102 restores hearing in a guinea pig model of noise-induced hearing loss to almost prenoise levels.

Although sudden sensorineural hearing loss (SSNHL) is a serious condition, there are currently no approved drugs for its treatment. Nevertheless, there is a growing understanding that the cochlear pathologies that underlie SSNHL include apoptotic death of sensory outer hair cells (OHCs) as well as loss of ribbon synapses connecting sensory inner hair cells (IHCs) and neurites of the auditory nerve, designated synaptopathy. Noise-induced hearing loss (NIHL) is a common subtype of SSNHL and is widely used to model hearing loss preclinically. Here, we demonstrate that a single interventive application of a small pyridoindole molecule (AC102) into the middle ear restored auditory function almost to prenoise levels in a guinea pig model of NIHL. AC102 prevented noise-triggered loss of OHCs and reduced IHC synaptopathy suggesting a role of AC102 in reconnecting auditory neurons to their sensory target cells. Notably, AC102 exerted its therapeutic properties over a wide frequency range. Such strong improvements in hearing have not previously been demonstrated for other therapeutic agents. In vitro experiments of a neuronal damage model revealed that AC102 protected cells from apoptosis and promoted neurite growth. These effects may be explained by increased production of adenosine triphosphate, indicating improved mitochondrial function, and reduced levels of reactive-oxygen species which prevents the apoptotic processes responsible for OHC death. This action profile of AC102 might be causal for the observed hearing recovery in in vivo models.

150 years ago: Schwartze's 1873 mastoidectomy and its implementation over the following 2 years.

BACKGROUND: In 1873, Hermann Schwartze and Adolf Eysell described a new surgical technique for treating mastoid disease using a mallet, chisels, and gouges of various sizes instead of trephines or drill instruments also called "modern mastoidectomy." On the 150th jubilee of this landmark article, we pay tribute by studying the reception and implementation of mastoidectomy in the 2 years following its publication. METHODS: The commentaries published in the otological and medical literature between the second part of 1873 to the end of 1875 were studied with an emphasis on the three specialized otological journals and the otological textbooks that existed during this period. RESULTS AND CONCLUSION: The princeps paper Ueber die künstliche Eröffnung des Warzenfortsatzes ("On the artificial opening of the mastoid process") by Hermann Schwartze and Adolf Eysell published in 1873 was rapidly disseminated in the medical literature for nearly 1 year, and then entered a phase of evaluation followed by a phase of extension and implementation, before finding its definitive place in the history of mastoid process surgery.

Bilateral intracochlear schwannomas: histopathological confirmation and outcomes following tumour removal and cochlear implantation with lateral wall electrodes.

Intracochlear schwannomas (ICS) are very rare benign tumours of the inner ear. We present histopathological proof of the extremely rare bilateral occurrence of intracochlear schwannomas with negative blood genetic testing for neurofibromatosis type 2 (NF2). Bilateral schwannomas are typically associated with the condition NF2 and this case is presumed to have either mosaicism for NF2 or sporadic development of bilateral tumours. For progressive bilateral tumour growth and associated profound hearing loss, surgical intervention via partial cochleoectomy, tumour removal, preservation of the modiolus, and simultaneous cochlear implantation with lateral wall electrode carrier with basal double electrode contacts was performed. The right side was operated on first with a 14-month gap between each side. The hearing in aided speech recognition for consonant-nucleus-consonant (CNC) phonemes in quiet improved from 57% to 83% 12 months after bilateral cochlear implantation (CI). Bilateral intracochlear schwannomas in non-NF2 patients are extremely rare but should be considered in cases of progressive bilateral hearing loss. Successful tumour removal and cochlear implantation utilizing a lateral wall electrode is possible and can achieve good hearing outcomes.

Individual computer-assisted 3D planning for placement of auricular prosthesis anchors in combination with an implantable transcutaneous bone conduction hearing device in patients with aural atresia.

BACKGROUND: The simultaneous implantation of the Bonebridge (MED-EL, Innsbruck, Austria), a semi-implantable active transcutaneous bone conduction hearing device and anchors for auricular prostheses can be challenging as both implants contain magnets and compete for the narrow space in the designated implantation area. MATERIAL AND METHODS: A preoperative planning tool (virtual surgery) was used with individual 3D computer models of the skull and implants for finding optimal implant positions for both the floating mass transducer (FMT) and the anchors for the auricular prosthesis. The interaction between the magnetic prosthesis anchors and the FMT was measured by means of static magnetic forces. A retrospective data analysis was conducted to evaluate the surgical and audiological outcome. RESULTS: Between 2014 and 2021, a 3D planning of a simultaneous implantation of the Bonebridge with auricular prosthesis anchors was conducted on 6 ears of 5 patients (3 males, 2 females; age range 17-56 years). The individual preoperative planning was considered very useful for the optimal placement of bone anchors in combination with the Bonebridge. Audiological data showed a clear benefit for hearing 3 months and > 11 months after implantation. No adverse interactions between the magnetic prosthesis anchors and the FMT were observed. In two patients, revision surgery was carried out due to skin inflammation or wound healing problems. No long-term complications were observed 3-5 years after surgery. CONCLUSION: Preoperative 3D planning represents a clear benefit for the simultaneous audiological and esthetic rehabilitation using the Bonebridge and anchors for auricular prostheses.

[Individual computer-assisted 3D planning for placement of auricular prosthesis anchors in combination with an implantable transcutaneous bone conduction hearing device in patients with aural atresia. German version]. / Individuelle computergestützte 3D-Planung zur Platzierung von Epithesenankern in Kombination mit einem implantierbaren transkutanen Knochenleitungshörgerät bei Patienten mit Ohrfehlbildungen.

BACKGROUND: The simultaneous implantation of the Bonebridge (MED-EL, Innsbruck, Austria), a semi-implantable active transcutaneous bone conduction hearing device and anchors for auricular prostheses can be challenging as both implants contain magnets and compete for the narrow space in the designated implantation area. MATERIAL AND METHODS: A preoperative planning tool (virtual surgery) was used with individual 3D computer models of the skull and implants for finding optimal implant positions for both the floating mass transducer (FMT) and the anchors for the auricular prosthesis. The interaction between the magnetic prosthesis anchors and the FMT was measured by means of static magnetic forces. A retrospective data analysis was conducted to evaluate the surgical and audiological outcome. RESULTS: Between 2014 and 2021 a 3D planning of a simultaneous implantation of the Bonebridge with auricular prosthesis anchors was conducted on 6 ears of 5 patients (3 males, 2 females; age range 17-56 years). The individual preoperative planning was considered very useful for the optimal placement of bone anchors in combination with the Bonebridge. Audiological data showed a clear benefit for hearing 3 months and > 11 months after implantation. No adverse interactions between the magnetic prosthesis anchors and the FMT were observed. In two patients, revision surgery was carried out due to skin inflammation or wound healing problems. No long-term complications were observed 3-5 years after surgery. CONCLUSION: Preoperative 3D planning represents a clear benefit for the simultaneous audiological and esthetic rehabilitation using the Bonebridge and anchors for auricular prostheses.

Intratympanic corticosteroids for sudden sensorineural hearing loss.

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

Clinical and functional results after implantation of the bonebridge, a semi-implantable, active transcutaneous bone conduction device, in children and adults.

PURPOSE: Aim of the study was to evaluate the surgical, clinical and audiological outcome of 32 implantations of the Bonebridge, a semi-implantable transcutaneous active bone conduction implant. METHODS: In a retrospective cohort study, we analyzed data for 32 implantations in 31 patients (one bilateral case; seven age < 16 years) with conductive or mixed hearing loss, malformations, after multiple ear surgery, or with single-sided deafness as contralateral routing of signal (CROS). RESULTS: Four implantations were done as CROS. Five cases were simultaneously planned with ear prosthesis anchors, and 23 implantations (72%) were planned through three-dimensional (3D) "virtual surgery." In all 3D-planned cases, the implant could be placed as expected. For implant-related complications, rates were 12.5% for minor and 3.1% for major complications. Implantation significantly improved mean sound field thresholds from a preoperative 60 dB HL (SD 12) to 33 dB HL (SD 6) at 3 postoperative months and 34 dB HL (SD 6) at > 11 postoperative months (p < 0.0001). Word recognition score in quiet at 65 dB SPL improved from 11% (SD 20) preoperatively to 74% (SD 19) at 3 months and 83% (SD 15) at > 11 months (p < 0.0001). The speech reception threshold in noise improved from - 1.01 dB unaided to - 2.69 dB best-aided (p = 0.0018). CONCLUSION: We found a clinically relevant audiological benefit with Bonebridge. To overcome anatomical challenges, we recommend preoperative 3D planning in small and hypoplastic mastoids, children, ear malformation, and simultaneous implantation of ear prosthesis anchors and after multiple ear surgery.

Course of social support and associations with distress after partial laryngectomy.

PURPOSE: Social support has been shown to be positively associated with quality of life and adjustment after a cancer diagnosis. The present study investigates the course of social support up to one year after partial laryngectomy and its association with distress. DESIGN: Longitudinal questionnaire study. SAMPLE: A total of 428 patients after partial laryngectomy (mean age: 64, SD = 11, 91% male). METHODS: Patients completed questionnaires before treatment (t1), one week after a partial laryngectomy (t2), 3 months (t3), and one year (t4) thereafter. Social support was evaluated at t2, t3, and t4 using a brief version of the Social Support Questionnaire. Distress was measured at t2, t3, and t4 using the HADS. Descriptive statistics for social support were computed across the three measurement points. Changes were analyzed by Wilcoxon signed-rank tests. Associations with distress were identified using linear regression analyses. FINDINGS: Social support increased between t2 and t3 and decreased to baseline level between t3 and t4. Distress at t2 was associated with social support at t2 (B = -0.15, p < 0.01) and distress at t3 with social support at t3 (B = -0.19, p < 0.01). Distress at t4 was related to social support at t2 (B = -0.10, p = 0.05). CONCLUSIONS: Although perceived social support increases after partial laryngectomy, it decreases again during the course of aftercare. IMPLICATIONS FOR PSYCHOSOCIAL PROVIDERS OR POLICY: Social support resources should be assessed to identify patients at risk for worse psychological well-being.

[Hyperbaric oxygen therapy : Selected indications in the discipline of otorhinolaryngology]. / Hyperbare Sauerstofftherapie : Ausgewählte Indikationen im Fachgebiet HNO-Heilkunde.

Hyperbaric oxygenation (HBO) represents the controlled exposure to positive pressure with simultaneous inhalation of pure oxygen. It is considered to be an effective treatment option for diseases with restricted blood flow as oxygen not only binds chemically to hemoglobin but also physically dissolves in blood plasma. With the help of a hyperbaric chamber the ambient pressure of a patient can be modified and the physiological characteristics in positive pressure can be medically used. The indications relevant to otorhinolaryngology are necrotizing otitis externa and skull base osteomyelitis, other forms of osteomyelitis, gangrene, wound healing disorders and sudden loss of hearing. When choosing a treatment, not only the availability and costs of HBO treatment play a role but also the patient's tolerance of overpressure. The risks of treatment, e.g., due to oxygen toxicity or barotrauma of the middle ear or the paranasal sinuses, must be weighed up against the potential benefits.

Efficacy and safety of systemic, high-dose glucocorticoid therapy for idiopathic sudden sensorineural hearing loss : Study protocol for a three-armed, randomized, triple-blind, multicenter trial (HODOKORT).

BACKGROUND: Systemic glucocorticosteroids ("steroids") are widely used worldwide as a standard of care for primary therapy of idiopathic sudden sensorineural hearing loss (ISSHL). The German ISSHL guideline recommends high-dose steroids without evidence from randomized controlled trials (RCTs) and refers solely to retrospective cohort studies. This RCT aims to assess the efficacy (improvement in hearing) and safety (especially systemic side effects) of high-dose steroids versus standard of care (standard dose systemic steroids) for the treatment of unilateral ISSHL, when given as a primary therapy. METHODS: The study is designed as a multicenter (approximately 40 centers), randomized, triple-blind, three-armed, parallel group, clinical trial with 312 adult patients. The interventions consist of 5 days of 250 mg/day intravenous prednisolone (intervention 1) + oral placebo, or 5 days of 40 mg/day oral dexamethasone (intervention 2) + intravenous placebo. The control intervention consists of 60 mg oral prednisolone for 5 days followed by five tapering doses + intravenous placebo. The primary efficacy endpoint is the change in hearing threshold in the three most affected contiguous frequencies between 0.25 and 8 kHz 1 month after ISSHL. Secondary endpoints include further measures of hearing improvement including speech audiometry, tinnitus, quality of life, blood pressure, and altered glucose tolerance. DISCUSSION: There is an unmet medical need for an effective medical therapy of ISSHL. Although sensorineural hearing impairment can be partially compensated by hearing aids or cochlear implants (CI), generic hearing is better than using hearing aids or CIs. Since adverse effects of a short course of high-dose systemic corticosteroids have not been documented with good evidence, the trial will improve knowledge on possible side effects in the different treatment arms with a focus on hyperglycemia and hypertension. TRIAL REGISTRATION: EudraCT (European Union Drug Regulating Authorities Clinical Trials Database) Nr. 2015-002602-36; Sponsor code: KKSH-127.

[Case report on traumatic complete laryngotracheal separation with a positive outcome]. / Fallbericht zum traumatischen Totalabriss der Trachea vom Kehlkopf mit positivem Ausgang.

HISTORY: A 21-year-old female was injured by accidental strangulation. Dyspnea and stridor occurred with delay, and led to emergency intubation. FINDINGS: Physical examination showed strangulation marks and neck emphysema. Computed tomography confirmed laryngotracheal separation and revealed misplacement of the ventilation tube. DIAGNOSIS: Further surgical exploration revealed complete laryngotracheal (cricotracheal) separation. TREATMENT: After initial emergency tracheotomy, cricotracheal reanastomosis was achieved by a two-stage surgical approach. CONCLUSION: Laryngotracheal separation is associated with high mortality. In the case presented herein, the patient survived and was discharged from hospital without a tracheostomy tube despite bilateral recurrent laryngeal nerve palsy.

[Objective Audiological Test Procedures: Indications and Differential Diagnostics]. / Objektive audiologische Messverfahren: Indikationen und Differenzialdiagnostik.

Diagnostics of hearing loss comprises subjective and objective methods and is successfully applied since many decades. This article introduces into the methods of impedance audiometry, otoacoustic emissions, auditory evoked potentials and electrically evoked potentials and describes the respective methodologies. Using an exemplary case, in this article we guide through all stages of objective audiological diagnostics and introduce the scientific and technical background, the application and evaluation of the findings of the objective test procedures. An application pathway for objective audiometric diagnostic tools is also described, including newborn hearing screening, differential diagnostics of hearing loss, auditory neuropathy, sudden sensorineural hearing loss, vestibular schwannoma and pediatrics patients. Finally, the application to patients with active middle-ear implants and cochlear implants is described.

A retrospective European multicenter analysis of the functional outcomes after active middle ear implant surgery using the third generation vibroplasty couplers.

PURPOSE: To evaluate the safety and performance of three novel vibroplasty couplers that allow attachment of the floating mass transducer of a transcutaneous active middle ear implant (AMEI) to the round window (RW) membrane, the long process (LP), or the incus body and the short process (SP) of the incus. METHODS: Retrospective multicenter cohort study of 25 AMEI users with sensorineural or mixed hearing loss that were among the first implanted with an AMEI vibrating ossicular prosthesis in combination with the third generation of vibroplasty couplers between 2014 and 2016. Main Outcome Measures were bone-conduction pure-tone and vibroplasty thresholds, postoperative aided sound field thresholds and postoperative aided word recognition score (WRS). RESULTS: Bone conduction threshold changes of more than 10 dB in 4PTABC were observed in two subjects. A mean improvement of 57.8% in speech recognition was observed with a mean WRS at 65 dB SPL improving from 14.8% (SD 21.9%) preoperatively to a mean aided score of 72.6% (SD 18.6%). Sound field thresholds improved from an average 4PTASF of 64.1 dB HL (SD 9.8 dB HL) to 37.0 dB HL (SD 8.9 dB HL), resulting in a mean functional gain of 27.1 dB. There was no significant difference in WRS or functional gain between the coupler types. CONCLUSION: Initial experience shows that all three third generation vibroplasty couplers represent safe and efficient attachment options for the FMT allowing the surgeon to choose the coupling type based on the present pathology.

Hearing rehabilitation after subtotal cochleoectomy using a new, perimodiolar malleable cochlear implant electrode array: a preliminary report.

PURPOSE: We here report about the first surgical experience and audiological outcome using a new, perimodiolar malleable cochlear implant electrode array for hearing rehabilitation after subtotal cochleoectomy for intralabyrinthine schwannoma (ILS). METHOD: Based on a cochlear implant with MRI compatibility of the magnet in the receiver coil up to 3 T, a cochlear implant electrode array was developed that is malleable and can be placed perimodiolar after tumor removal from the cochlea via subtotal cochleoectomy. Malleability was reached by incorporating a nitinol wire into the silicone of the electrode array lateral to the electrode contacts. The custom-made device was implanted in four patients with intracochlear, intravestibulocochlear or transmodiolar schwannomas. Outcome was assessed by evaluating the feasibility of the surgical procedure and by measuring sound field thresholds and word recognition scores. RESULTS: After complete or partial tumor removal via subtotal cochleoectomy with or without labyrinthectomy, the new, perimodiolar malleable electrode array could successfully be implanted in all four patients. Six months after surgery, the averaged sound field thresholds to pulsed narrowband noise in the four patients were 36, 28, 41, and 35 dB HL, and the word recognitions scores for monosyllables at 65 dB SPL were 65, 80, 70, and 25% (one patient non-German speaking). CONCLUSION: The surgical evaluation demonstrated the feasibility of cochlear implantation with the new, perimodiolar malleable electrode array after subtotal cochleoectomy. The audiological results were comparable to those achieved with another commercially available type of perimodiolar electrode array from a different manufacturer applied in patients with ILS.

Intraoperative quantification of floating mass transducer coupling quality in active middle ear implants: a multicenter study.

PURPOSE: Evaluating the effectiveness of intraoperative auditory brainstem responses (ABRs) to stimulation by the Vibrant Soundbridge (VSB) active middle ear implant for quantifying the implant's floating mass transducer (FMT) coupling quality. METHODS: In a diagnostic multicentric study, patients (> 18 years) who received a VSB with different coupling modalities were included. Pre- and postoperative bone conduction thresholds, intraoperative VSB-evoked ABR thresholds (VSB-ABR) using a modified audio processor programmed to preoperative bone conduction thresholds, postoperative vibrogram thresholds, and postoperative VSB-ABR thresholds were measured. Coupling quality was calculated from the difference between the pure tone average at 1000, 2000, and 4000 Hz (3PTA) vibrogram and postoperative 3PTA bone conduction thresholds. RESULTS: Twenty-three patients (13 males, 10 females, mean age 56.6 (± 12.5) years) were included in the study. Intraoperative VSB-ABR response thresholds could be obtained in all except one patient where the threshold was > 30 dB nHL. Postoperatively, an insufficient coupling of 36.7 dB was confirmed in this patient. In a Bland-Altman analysis of the intraoperative VSB-ABRs and coupling quality, the limits of agreement exceeded ± 10 dB, i.e., the maximum allowed difference considered as not clinically important but the variation was within the general precision of auditory brainstem responses to predict behavioral thresholds. Five outliers were identified. In two patients, the postoperative VSB-ABR thresholds were in agreement with the coupling quality, indicating a change of coupling before the postoperative testing. CONCLUSION: The response thresholds recorded in this set-up have the potential to predict the VSB coupling quality and optimize postoperative audiological results.

Evaluating the didactic value of 3D visualization in otosurgery.

INTRODUCTION: Improvements of surgical visualization add value to the quality of clinical routine and offer the opportunity to improve surgical education of medical staff. The aim of this study was to determine whether otorhinolaryngology trainees gain additional comprehension of the anatomical structures and the surgical site when 3D visualization is used. METHODS: Data were collected from ENT trainees of microsurgical courses of the middle ear, inner ear and lateral skull base at four university ENT departments (Charité (Berlin), Martin Luther University Halle-Wittenberg (Halle/Saale), Ludwig Maximilian University (Munich) and Rostock University Medical Center). Participants were asked to complete a questionnaire assessing the subjective value of identical surgical field visualization in 3D for surgeon and observer. RESULTS: A total of 112 participants completed the questionnaire. The majority of participants stated a high additional value of 3D visualization compared to 2D visualization, with 75% fully agreeing to the statement that 3D visualization of the surgical field is superior to perceive the anatomical topography and structures compared to 2D representation. Participants encouraged the storage of data in online learning platforms. CONCLUSION: The results show that 3D visualization with identical imaging for surgeon and observer is a useful tool in teaching of microsurgery. It addresses perception of anatomical topography and structures as well as conception of the surgical workflow.

[Multimodal training concept for temporal bone surgery]. / Multimodales Weiterbildungskonzept Schläfenbeinchirurgie.

BACKGROUND: Surgical training is increasingly supported by the use of simulators. For temporal bone surgery, shown here by means of mastoidectomy, there are other training models besides cadaver specimens, such as artificial temporal bones or computer-based simulators. OBJECTIVES: A structured training concept was created which integrates different training methods of mastoidectomy with regard to effectiveness and current learning theory in education. METHOD: A selective literature research was conducted to compare learning-theoretical findings and the availability and effectiveness of currently existing training models. RESULTS: To acquire surgical skills, a stepwise approach is suggested. Depending on the progress with computer-based simulation, plastic or native temporal bones should be used. To achieve a plateau of the learning curve, approximately 25 semi-autonomous preparations are recommended. Different 'Objective Structured Assessments of Technical Skills' (OSATS) are implemented to assess the learning progress at different levels. DISCUSSION: Simulation-based training is recommended until an adequate learning curve plateau is achieved. This is reasonable for patient safety, based on limited accessibility of human cadaveric temporal bones but also by findings of the learning theory. CONCLUSION: The curriculum integrates different training models of mastoidectomy and OSATS into an overall concept. The training plan has to be continuously adapted to new findings and technical developments.

Editorial. / Editorial.

Rare diseases represent a major challenge for affected patients, their relatives, physicians, nursing staff, and therapists. For medical and economic reasons, disease rarity complicates the research and medical care of affected patients. The Hollywood movie, "Lorenzo's Oil", touchingly illustrates the complex problems associated with orphan disease diagnostics, research, and therapy. Directed by George Miller, this film shows the true story of a boy named Lorenzo Michael Murphy Odone, who was diagnosed in 1984 at the age of 6 with the rare neural disease adrenoleukodystrophy (ALD). The movie highlights the manifold problems associated with rare diseases - a large number of which still exist today. However, especially in recent years, orphan diseases have been placed in the focus of public attention.

Rare Diseases and Otorhinolaryngology, Head and Neck Surgery. / Seltene Erkrankungen und Hals-Nasen-Ohren-Heilkunde, Kopf und Halschirurgie.

Rare diseases pose multiple challenges for patients, relatives, physicians, nursing staff, and therapists. Their rarity impedes research and treatments due to medical and economical reasons. Many diseases in the field otorhinolaryngology, head and neck surgery are rare diseases due to their low prevalence. The initiation of the right management processes requires knowledge about diagnostics, resources like centers, networks and registries, about specifics of the physician-patient relationship, follow-up care including communication with family doctors and the role of self-help groups. Of special interest for university hospitals and our scientific society are the specific aspects of research including European networks and research funding, information management, public relations, education, training, financing, and regulations like orphan drugs and clinical trials in small populations.

[Outpatient Surgery in German ENT]. / Ambulantes Operieren in der HNO-Heilkunde in Deutschland.

Currently there is an intense discussion ongoing to enhance and expand outpatient surgery in the ENT in Germany, which is the intention by several politicians. The goal is to achieve a frequency of outpatient surgery comparable to an international level.To achieve this goal, acceptance of outpatient interventions by both, surgeons and patients is required, particularly in regard of equal quality standards and patient safety requirements.In the following review the organization, outcome, quality management and strategies for different ENT outpatient surgery worldwide is analyzed. Basically, outpatient surgery is organized in different ways: office-based-procedures in local anesthesia, procedures in ambulatory surgery center settings as standalone facilities or in connection with and adjacent to a hospital with possible inpatient treatment. Contact and resident times of the patients in the outpatient surgery centers differ between some hours through 23 hours. A deliberated and careful selection of patients which are suitable for outpatient procedures is required and should address comorbidities, medications, social circumstances, health literacy of the patient and its relatives, and distance from home to the hospital. A careful and strict quality management is mandatory which comprises the entire process from patient selection through patient entry, surgery, discharge and postoperative care in a multidisciplinary setting.Zur besseren Lesbarkeit des Textes wird bei geschlechterbezogenen Bezeichnungen die männliche Form benutzt. Es sind jedoch in gleicher Rangfolge auch das jeweilige weibliche Geschlecht oder andere Geschlechtsausprägungen gemeint.