Intraoperative ultrasonography in microsurgical resection of vestibular schwannomas via retrosigmoid approach: surgical technique and proof-of-concept illustrative case series.

PURPOSE: Intraoperative ultrasonography (ioUS) is an established tool for the real-time intraoperative orientation and resection control in intra-axial oncological neurosurgery. Conversely, reports about its implementation in the resection of vestibular schwannomas (VS) are scarce. The aim of this study is to describe the role of ioUS in microsurgical resection of VS. METHODS: ioUS (Craniotomy Transducer N13C5, BK5000, B Freq 8 MHz, BK Medical, Burlington, MA, USA) is integrated into the surgical workflow according to a 4-step protocol (transdural preresection, intradural debulking control, intradural resection control, transdural postclosure). Illustrative cases of patients undergoing VS resection through a retrosigmoid approach with the use of ioUS are showed to illustrate advantages and pitfalls of the technique. RESULTS: ioUS allows clear transdural identification of the VS and its relationships with surgically relevant structures of the posterior fossa and of the cerebellopontine cistern prior to dural opening. Intradural ioUS reliably estimates the extent of tumor debulking, thereby helping in the choice of the right moment to start peripheral preparation and in the optimization of the extent of resection in those cases where subtotal resection is the ultimate goal of surgery. Transdural postclosure ioUS accurately depicts surgical situs. CONCLUSION: ioUS is a cost-effective, safe, and easy-to-use intraoperative adjunctive tool that can provide a significant assistance during VS surgery. It can potentially improve patient safety and reduce complication rates. Its efficacy on clinical outcomes, operative time, and complication rate should be validated in further studies.

Classification of Acoustic Hearing Preservation After Cochlear Implantation Using Electrocochleography.

The objective to preserve residual hearing during cochlear implantation has recently led to the use of intracochlear electrocochleography (ECochG) as an intraoperative monitoring tool. Currently, a decrease in the amplitude of the difference between responses to alternating-polarity stimuli (DIF response), predominantly reflecting the hair cell response, is used for providing feedback. Including other ECochG response components, such as phase changes and harmonic distortions, could improve the accuracy of surgical feedback. The objectives of the present study were (1) to compare simultaneously recorded stepwise intracochlear and extracochlear ECochG responses to 500 Hz tone bursts, (2) to explore patterns in features extracted from the intracochlear ECochG recordings relating to hearing preservation or hearing loss, and (3) to design support vector machine (SVM) and random forest (RF) classifiers of acoustic hearing preservation that treat each subject as a sample and use all intracochlear ECochG recordings made during electrode array insertion for classification. Forty subjects undergoing cochlear implant (CI) surgery at the Oslo University Hospital, St. Thomas' Hearing Implant Centre, or the University Hospital of Zurich were prospectively enrolled. In this cohort, DIF response amplitude decreases did not relate to postoperative acoustic hearing preservation. Exploratory analysis of the feature set extracted from the ECochG responses and preoperative audiogram showed that the features were not discriminative between outcome classes. The SVM and RF classifiers that were trained on these features could not distinguish cases with hearing loss and hearing preservation. These findings suggest that hearing loss following CI surgery is not always reflected in intraoperative ECochG recordings.

[Health-related quality of life in chronic otitis media-measurement methods and their application in surgical therapy]. / Gesundheitsbezogene Lebensqualität bei der chronischen Otitis media ­ Messmethoden und deren Anwendung bei chirurgischen Therapien.

BACKGROUND: Chronic otitis media (COM) can lead to significant impairment of health-related quality of life (HRQoL) due to symptoms such as otorrhea, pain, hearing loss, tinnitus, or dizziness. A systematic assessment of HRQoL in COM is becoming increasingly important as it complements (semi-)objective outcome parameters in clinical practice and research. HRQoL is measured by means of patient-reported outcome measures (PROMs). There are two disease-specific validated PROMs available for COM in German-the Chronic Otitis Media Outcome Test (COMOT-15) and the Zurich Chronic Middle Ear Inventory (ZCMEI-21)-which have become increasingly popular in recent years. OBJECTIVE: The purpose of this narrative review is to present the current state of research on measuring HRQoL in COM before and after surgical procedures. RESULTS AND CONCLUSION: Hearing is the most important factor influencing HRQoL in COM. Surgical procedures usually result in a clinically relevant improvement in HRQoL in COM with or without cholesteatoma. However, if cholesteatoma is present, its extent does not correlate with HRQoL. While HRQoL plays a secondary role in establishment of the indication for surgical therapy in COM with cholesteatoma, it plays an important role in terms of relative surgical indications, e.g., a symptomatic open mastoid cavity after resection of the posterior canal wall. We encourage the regular use of disease-specific PROMs preoperatively as well as during follow-up to assess HRQoL in COM in individual patients, in research, and in the context of quality monitoring.

Gustatory Function of Patients With and Without Cholesteatoma Undergoing Middle Ear Surgery.

OBJECTIVE: To compare measured and perceived taste function before and after surgery of patients with chronic otitis media with cholesteatoma (OMCC) to patients without cholesteatoma (patients with chronic suppurative otitis media [CSOM] and patients with lateral skull base lesions [LSB]). METHODS: This prospective cohort study included 29 patients undergoing surgery for unilateral OMCC. The chorda tympani nerve (CTN) was resected in 8 of these patients. Fourteen patients undergoing surgery for unilateral CSOM and 5 patients undergoing surgery for unilateral LSB (with CTN resection) served as the comparison group. Taste function was measured using taste strips on both sides of the tongue before surgery, 2 weeks postoperatively and 3 months postoperatively. The affected side of the tongue was compared to the unaffected side. A questionnaire on taste perception was completed at each visit. RESULTS: Preoperatively, cholesteatoma patients showed higher taste strip scores than non-cholesteatoma patients, indicating a larger difference between the healthy and affected sides of the tongue. Despite this difference in measured taste function few cholesteatoma patients reported taste alteration before surgery (3/29 [10.3%]). Postoperatively, patients with CTN resection (OMCC patients with CTN resection and LSB patients) showed a decreased measured taste function. Subjectively, only approximately 20% of these patients reported taste alteration 3 months postoperatively. CONCLUSIONS: Before surgery, cholesteatoma patients displayed an impaired measured taste function compared to patients without cholesteatoma (CSOM, LSB). Subjectively this was often unnoticed. After surgery, despite removal of the CTN and consequent reduction of measured taste function, few patients reported taste alteration and subjective taste perception was seen to be improving. In regards to middle ear surgery, perceived taste function does not seem to reflect measured gustatory function.

Prevalence of Endolymphatic Hydrops in Cochlear Implant Candidates with Idiopathic Profound Sensorineural Hearing Loss.

OBJECTIVE: To determine the prevalence of endolymphatic hydrops (EH) in cochlear implant (CI) candidates with idiopathic profound sensorineural hearing loss (SNHL) and its influence on the preservation of audiovestibular function after cochlear implantation. STUDY DESIGN: Prospective case series. SETTING: Tertiary referral center. PATIENTS: CI candidates with idiopathic progressive SNHL, but without classic EH-associated symptoms. INTERVENTIONS: Delayed intravenous gadolinium-enhanced inner ear fluid-attenuated inversion recovery magnetic resonance imaging as well as pure-tone audiograms, video head impulse tests, and vestibular evoked myogenic potentials before and 4 weeks after cochlear implantation. MAIN OUTCOME MEASURES: Prevalence of EH before cochlear implantation, audiovestibular function before and after surgery in hydropic and nonhydropic ears. RESULTS: Thirty-two ears in 16 CI candidates were included. Nine ears (28%) with EH were detected. Although preoperative hearing thresholds, utricular function, and semicircular canal function were not different between the two groups, saccular function was reduced in hydropic ears. Ten subjects received a unilateral CI. Of these, 3 (30%) showed EH on the implanted side. There was no difference regarding postoperative hearing loss between the two groups, but the results point toward a higher vulnerability of hydropic ears with respect to loss of otolith function after cochlear implantation. CONCLUSIONS: This is the first study showing that EH can be assumed in about one third of CI candidates with idiopathic profound SNHL, but no classic EH-associated symptoms. Preliminary results suggest that EH has no influence on the preservation of cochlear function but could be a risk factor for loss of otolith function after cochlear implantation.

Postural stability and handicap of dizziness after preoperative vestibular ablation and vestibular prehabilitation in patients undergoing vestibular schwannoma resection.

BACKGROUND: Surgical treatment of vestibular schwannoma (VS) leads to acute ipsilateral vestibular loss if there is residual vestibular function before surgery. To overcome the sequelae of acute ipsilateral vestibular loss and to decrease postoperative recovery time, the concept of preemptive vestibular ablation with gentamicin and vestibular prehabilitation before surgery has been developed ("vestibular prehab"). OBJECTIVE: Studying postural stability during walking and handicap of dizziness over a 1-year follow-up period in VS patients undergoing vestibular prehab before surgical treatment of VS. METHODS: A retrospective review of consecutive patients with a diagnosis of a VS undergoing surgical therapy from June 2012 to March 2018 was performed. All patients were included with documentation of the length of hospital duration and the Dizziness Handicap Inventory (DHI) and the Functional Gait Assessment (FGA) assessed preoperatively as well as 6 weeks and 1 year postoperatively. RESULTS: A total 68 VS patients were included, of which 29 patients received preoperative vestibular ablation by intratympanic injection of gentamicin. Mean VS diameter was 20.2 mm (SD 9.4 mm) and mean age at surgery was 49.6 years (SD 11.5 years). Vestibular prehab had no effect on DHI and FGA at any time point studied. CONCLUSIONS: We found no effect of vestibular prehab on postural stability during walking and on the handicap of dizziness. These findings add to the body of knowledge consisting of conflicting results of vestibular prehab. Therefore, vestibular prehab should be applied only in selected cases in an experimental setting.

Retrospective Investigation of Contralateral Hearing Thresholds of Patients With Sporadic Vestibular Schwannoma.

OBJECTIVE: The aim of this study was to investigate the contralateral hearing of patients with sporadic vestibular schwannoma (VS). STUDY DESIGN: Retrospective cohort study. SETTING: Pure-tone audiograms of the contralateral ear from patients with a wait-and-scan strategy were compared to the ones who received therapy. Due to a possible bias caused by the therapy, hearing thresholds before and after radiotherapy or surgery were compared separately with the wait-and-scan group. METHODS: From 1979 to 2017, 508 patients with sporadic VS could be included in the study. Of these, 240 received regular controls in the sense of wait-and-scan, 72 underwent radiotherapy (63 audiograms before and 43 after radiotherapy), and 196 had a surgery (186 audiograms before and 146 after surgery). Age-normalized hearing thresholds of the contralateral ear from patients with a wait-and-scan strategy were compared to ones who received therapy. In addition, hearing thresholds were compared to norm values. RESULTS: There was no evidence for a difference in the contralateral hearing of patients with sporadic VS between the wait-and-scan and therapy groups. The mean difference of hearing thresholds in our sample to norm values was found to be larger for the high frequencies and more pronounced in male patients. CONCLUSION: There was no evidence for a difference in the contralateral hearing loss of patients with sporadic VS between the wait-and-scan and therapy groups. However, there was some indirect indication of poorer contralateral hearing in all patients with sporadic VS compared to normative values.

Implications of Phase Changes in Extracochlear Electrocochleographic Recordings During Cochlear Implantation.

OBJECTIVE: To assess the prevalence and implications of phase changes in extracochlear electrocochleography (ECochG) recordings during cochlear implantation. MATERIALS AND METHODS: Extracochlear ECochG recordings were performed before and after insertion of the cochlear implant (CI) electrode by a recording electrode placed on the promontory. Acoustic stimuli were tone bursts at 250, 500, 750, and 1,000 Hz. The pure tone average (PTA) was determined before and approximately 4 weeks after surgery. RESULTS: Extracochlear ECochG recordings in 69 ears of 68 subjects were included. At 250 Hz, the mean phase change was 43° (n = 50, standard deviation (SD) 44°), at 500 Hz 36° (n = 64, SD 36°), at 750 Hz 33° (n = 42, SD 39°), and at 1,000 Hz 22° (n = 54, SD 27°). Overall, in 48 out of 210 ECochG recordings a phase change of ≥45° (23%) was detectable. Ears with an amplitude drop >3 dB and a phase change ≥45° (n = 3) had a complete or near complete loss of residual cochlear function in all cases. A phase change of ≥90° in one recording was not associated with a larger amplitude change of the ECochG signal (1.9 dB vs. -0.9 dB, p = 0.1052, n = 69), but with a significantly larger postoperative hearing loss (17 dB vs. 26 dB, p = 0.0156, n = 69). CONCLUSIONS: Phase changes occur regularly in extracochlear ECochG recordings during cochlear implantation. Phase changes of ≥90° with or without amplitude changes in the ECochG signal are associated with a larger postoperative hearing loss and could therefore represent an independent marker for cochlear trauma or changes of inner ear mechanics relevant for the postoperative hearing outcome.

Assessment of Surgical Complications With Respect to the Surgical Indication: Proposal for a Novel Index.

Introduction: The Clavien-Dindo classification is a broadly accepted surgical complications classification system, grading complications by the extent of therapy necessary to resolve them. A drawback of the method is that it does not consider why the patient was operated on primarily. Methods: We designed a novel index based on Clavien-Dindo but with respect to the surgical indication. We surveyed an international panel of otolaryngologists who filled out a questionnaire with 32 real case-inspired scenarios. Each case was graded for the surgical complication, surgical indication, and a subjective rating whether the complication was acceptable or not. Results: Seventy-seven otolaryngologists responded to the survey. Mean subjective rating and surgical complication grading for each scenario showed an inverse correlation (r 2 = 0.147, p = 0.044). When grading the surgical complication with respect to the surgical indication, the correlation with the subjective rating increased dramatically (r 2 = 0.307, p = 0.0022). Conclusion: We describe a novel index grading surgical complications with respect to the surgical indication. In our survey, most respondents judged a complication as acceptable or not according to its grade but kept in mind the surgical indication. This subjective judgment could be quantified with our novel index.

Histopathologic Evaluation of Intralabyrinthine Schwannoma.

OBJECTIVES: The aim of this study is to perform a histopathologic analysis of temporal bones with an intralabyrinthine schwannoma (ILS) in order to characterize its extension. METHODS: Archival temporal bones with a diagnosis of sporadic schwannoma were identified. Both symptomatic and occult nonoperated ILS were included for further analysis. RESULTS: A total of 6 ILS were identified, with 4 intracochlear and 2 intravestibular schwannomas. All intracochlear schwannomas involved the osseous spiral lamina, with 2 extending into the modiolus. The intravestibular schwannomas were limited to the vestibule, but growth into the bone next to the crista of the lateral semicircular canal was observed in 1 patient. CONCLUSIONS: Complete removal of an ILS may require partial removal of the modiolus or bone surrounding the crista ampullaris as an ILS may extend into these structures, risking damage of the neuronal structures. Due to the slow growth of the ILS, it remains unclear if a complete resection is required with the risk of destroying neural structures hindering hearing rehabilitation with a cochlear implant.

Preliminary experience and feasibility test using a novel 3D virtual-reality microscope for otologic surgical procedures.

BACKGROUND: Current surgical microscopes suffer from inherent constraints in achievable viewing angles and require manual interaction to control settings. Overcoming these limitations may provide benefits for otologic surgical procedures. OBJECTIVES: We present a first feasibility and usability assessment of a novel commercial surgical microscope featuring a virtual-reality interface and hands-free control of a robot-mounted 3D-camera. MATERIALS AND METHODS: Ex vivo feasibility tests were conducted in a human cadaveric head sample based on common otologic procedures. Usability metrics were obtained from feedback forms. In a first clinical case, the device was used to perform a tympanoplasty in a 68-year-old patient with a subtotal tympanic membrane perforation. RESULTS: Overall practicability of using the device for otologic procedures could be confirmed in the ex vivo tests. Beneficial aspects of the novel system which were subjectively encountered in the ex vivo tests and the first clinical case included hands-free control, visualization quality, and teaching potential. CONCLUSIONS AND SIGNIFICANCE: The novel virtual reality 3D-microscope bears potential to provide benefits for various otologic procedures, which will be evaluated more quantitatively in clinical follow-up studies.

Influence of Semicircular Canal Dehiscence on Cochlear Implant Outcome.

INTRODUCTION: Semicircular canal dehiscence (SCD) is defined as a defect of the bone overlying the semicircular canal. It has a relatively high prevalence of 3% in the general population, which makes it likely that a certain number of patients receiving a cochlear implant (CI) would have it. However, little is known about the influence of SCD on the CI outcome. Therefore, the aim of this study was to determine the influence of SCD on CI outcome with regard to short- and long-term word perception and hearing preservation. METHODS: This study was a retrospective analysis of postoperative word perception ability in the electric-only condition after 6, 12, and ≥18 months and of hearing preservation 4 weeks after surgery in CI recipients with and without SCD. All patients received a preoperative 1.5- or 3-tesla magnetic resonance imaging. RESULTS: Fifty-five patients were included. Forty-eight patients (87%) had no SCD, and 7 patients (13%) had SCD. Mean postoperative word perception scores were 66% in the non-SCD group versus 50% in the SCD group (p = 0.17) after 6 months, 74 versus 64% (p = 0.28) after 12 months, and 77 versus 73% (p = 0.62) after 18 or more months. The mean postoperative hearing loss in patients with functional residual hearing before surgery (n = 34) was 22 dB in the non-SCD group versus 31 dB in the SCD group (p = 0.15). CONCLUSIONS: CI outcome is comparable between recipients without and with SCD. Specifically, hearing preservation rate and word perception ability in the electric-only condition seem not affected by SCD. The rate of progress of word perception ability in the first 12 months after cochlear implantation is not influenced by SCD.

Evaluating hearing outcome, recidivism and complications in cholesteatoma surgery using the ChOLE classification system.

PURPOSE: To establish a standardized reporting system of cholesteatoma, the ChOLE classification has recently been introduced. We here aimed to systematically investigate the association between the ChOLE classification and (i) hearing, (ii) recidivism rate, and (iii) postoperative complications. These data may increase the utility of the ChOLE classification in clinical practice and research by stratifying patients according to expected outcomes or risks for complications. METHODS: In this prospective multicentric study, we included adult patients undergoing tympanomastoid surgery due to cholesteatoma. Main outcome measures included the association of the ChOLE classification system with (i) audiometric data including air conduction (AC) and bone conduction (BC) pure-tone average (PTA), and the air-bone gap (ABG), (ii) recidivism and complication. RESULTS: A total of 160 patients suffering from cholesteatoma were included. ChOLE stage distribution was stage I in 23 (14%), stage II in 128 (80%), and stage III in 9 (6%) patients. The ChOLE stage was associated with the postoperative AC PTA (p = 0.05) and the postoperative BC PTA (p = 0.02). Further, the status of the ossicular chain after surgery (ChOLE subdivision "O") was associated with both the postoperative ABG (p = 0.0001) and the postoperative AC PTA (p = 0.003). Moreover, we found an association between complications (ChOLE subdivision "L) and both the postoperative BC PTA (p = 0.04) and the postoperative ABG (p = 0.04). No association between the ChOLE stage was found to both cholesteatoma recidivism and surgical complications. CONCLUSION: The ChOLE classification is a new system to classify cholesteatomas. We provide evidence that hearing outcomes vary among different ChOLE stages. In particular, hearing outcomes are associated with the ChOLE subdivision "O" and "L". Thus, the ChOLE classification system has a predictive value regarding hearing outcomes.

Endolymphatic hydrops mimicking obstructive Eustachian tube dysfunction: preliminary experience and literature review.

PURPOSE: Aural fullness is a common symptom of middle ear diseases, most importantly Eustachian tube dysfunction (ETD). Yet, aural fullness may also be caused by inner ear disorders, such as hydropic ear diseases. Here, we report our experience with endolymphatic hydrops (EH) mimicking ETD. Furthermore, we review the literature related to (i) EH as a differential diagnosis of symptoms suggesting ETD and (ii) the pathophysiology and treatment of aural fullness due to inner ear disorders. METHODS: We retrospectively included adult patients with aural fullness as chief complaint and radiographically diagnosed EH. Hearing and Eustachian tube function were assessed using audiometry, tympanometry, and tubomanometry. Primarily suspected ETD was treated by balloon dilatation of the Eustachian tube (BDET). The endolymphatic space of the inner ear was imaged using gadolinium-enhanced MRI (Gd-MRI) including a 3D-real inversion-recovery sequence after intravenous gadolinium administration. RESULTS: We report three affected ears of two patients (two females, age 42 and age 51) with aural fullness as chief complaint. Audiometry of main speech frequencies was normal in all affected ears. In one ear, there was a type A tympanogram and in two ears, there was a type B tympanogram. In both patients, medical treatment for ETD and BDET were unsuccessful. Gd-MRI of the inner ears revealed cochlear EH in 3/3 ears affected by aural fullness, but not in the unaffected ear. CONCLUSION: EH may underlay cases with aural fullness and could in these cases explain unsuccessful treatment for ETD. As ETD is often treated by invasive procedures, distinguishing ETD from EH as the underlying cause of aural fullness is important. Our findings raise the question whether Gd-MRI to rule out EH is indicated in patients with unexplained aural fullness, in particular after unsuccessful interventional treatment for ETD.

Measuring health-related quality of life in chronic otitis media in a Chinese population: cultural adaption and validation of the Zurich Chronic Middle Ear Inventory (ZCMEI-21-Chn).

BACKGROUND: The demand for assessing health-related quality of life (HRQoL) in chronic otitis media (COM) is increasing globally. The currently available Chinese-language patient-reported outcome measurement (PROM) specific for COM includes merely a limited range of related symptoms and dimensions. Hence, in this study, we aim to translate, culturally adapt, and validate the Zurich Chronic Middle Ear Inventory (ZCMEI-21) in Chinese, to enable a comprehensive evaluation of the patients' subjective health outcome in COM. METHODS: We sampled and surveyed 223 COM patients at three tertiary referral centers in China, using the Chinese translation of ZCMEI-21 (ZCMEI-21-Chn) and the EQ-5D questionnaire, a generic measure of HRQoL. Confirmatory factor analysis (CFA) was performed to investigate the structural model fit to the dataset. Cronbach's α and test-retest reliability coefficient were calculated to establish reliability, and correlation was tested between ZCMEI-Chn scores and EQ-5D scores for convergent validity. RESULTS: A total of 208 adult patients with COM were included, with a mean age of 46 years (SD 14 years) and a male proportion of 41% (85/208). A modified bifactor model with ωH of 0.65 and ECV of 0.47 was found to fit the scale scores, indicating fair general factor saturation and multidimensionality of the instrument. ZCMEI-21-Chn demonstrated good reliability (Cronbach's α = 0.88, test-retest reliability = 0.88). The total scores of ZCMEI-21-Chn had a moderate correlation with a question directly addressing HRQoL (r = 0.40, p < 0.001), EQ-5D descriptive system score (r = 0.57, p < 0.001), and EQ-5D visual analogous scale (r = 0.30, p < 0.001). CONCLUSIONS: The ZCMEI-21-Chn is valid, reliable and culturally adapted to Chinese adult patients with COM. This study offers clinicians an efficient and comprehensive instrument to quantify COM patients' self-reported health outcomes, which could facilitate the standardization of HRQoL data aggregation in COM on a global scale.

Mapping the ChOLE classification to hearing outcomes and disease-specific health-related quality of life.

OBJECTIVES: To investigate the association between the "ChOLE" classification, hearing outcomes and disease-specific health-related quality of life (HRQoL). METHODS: In two tertiary referral centers, patients requiring primary or revision surgery for cholesteatoma were assessed for eligibility. Audiometric assessment was performed pre- and postoperatively. The ChOLE classification was determined intraoperatively and via the preoperative CT scan. HRQoL was assessed pre- and postoperatively using the Zurich Chronic Middle Ear Inventory (ZCMEI-21). RESULTS: A total of 87 patients (mean age 45.2 years, SD 16.2) were included in this study. ChOLE stage I cholesteatoma was found in 8 (9%), stage II cholesteatoma was found in 65 (75%), and stage III cholesteatoma was found in 14 (16%) patients. Postoperatively, the mean air-bone gap (0.5, 1, 2, 3 kHz) was significantly smaller than before surgery (14.3 dB vs. 23.0 dB; p = 0.0007). The mean ZCMEI-21 total score significantly decreased after surgery (26.8 vs. 20.7, p = 0.004). No correlation between the ZCMEI-21 total score and both the ChOLE stage and the extent of the cholesteatoma (ChOLE subdivision "Ch") was found. A trend towards worse HRQoL associated with a poorer status of the ossicular chain (ChOLE subdivision "O") was observed. The audiometric outcomes were not associated with the extent of the cholesteatoma. The ChOLE subdivision describing the ossicular status showed a strong association with the pre- and postoperative air conduction (AC) thresholds. Further, the ZCMEI-21 total score and its hearing subscore correlated with the AC thresholds. CONCLUSION: The ChOLE classification does not show a clear association with HRQoL measured by the ZCMEI-21. The HRQoL neither seems to be associated with the extent of the disease nor with the ossicular chain status. Yet, surgical therapy significantly improved HRQoL by means of reduced ZCMEI-21 total scores, which were strongly associated with the AC thresholds. Intraoperative assessment of a cholesteatoma using the ChOLE classification and HRQoL complement each other and provide useful information.

Tinnitus With Unexpected Spanish Roots: Head and Neck Paragangliomas Caused by SDHAF2 Mutation.

It is estimated that up to 40% of all head and neck paragangliomas (HNPGL) have a hereditary background with the most common mutations being found in the succinate dehydrogenase (SDH) genes. SDHAF2 mutation leads to the rare paraganglioma syndrome 2. The authors present the case of a 15-year-old male patient with 2, non-secretory HNPGLs, presenting with left-sided, pulsatile tinnitus, and hearing loss. Imaging led to the suspicion of a jugulotympanic paraganglioma on the left, as well as a carotid body tumor on the right. After resection of the jugulotympanic tumor, histology confirmed the presence of a paraganglioma; immunohistochemistry furthermore suggested a loss of SDHB expression. Genetic testing revealed a rare germline, loss-of-function mutation in the SDHAF2 gene, previously described to cause hereditary paraganglioma syndrome 2. Twenty months after the first operation, the patient underwent a resection of the right carotid body paraganglioma. Plasma-free metanephrines/catecholamines always remained within the reference range; the patient is under regular follow-up, and his relatives will be screened. Our findings emphasize the relevance of genetic testing in patients with HNPGL, also with negative family history, especially when the patients present at a young age and with multiple lesions.

Correlation Between Electrocochleographic Changes During Surgery and Hearing Outcome in Cochlear Implant Recipients: A Case Report and Systematic Review of the Literature.

OBJECTIVE: To determine the correlation between intraoperative changes of electrocochleography (ECochG) responses and traumatic cochlear implant insertions as well as postoperative hearing loss. METHODS: ECochG, radiological, and audiological data were collected prospectively in a cochlear implant recipient with otosclerosis and assumed cochlear trauma during electrode insertion. A systematic review was conducted within PubMed-NCBI, EMBASE, and the Cochrane Library using the terms "Cochlear implant" and "Electrocochleography." Original studies that evaluated intraoperative ECochG responses and postoperative hearing loss were selected and analyzed. RESULTS: The case report revealed a drop of intra- and extracochlear ECochG signals during electrode insertion. The postoperative computed tomography scan suggested a scalar dislocation. There was no measurable hearing 4 weeks after surgery. Within the database search, nine articles met the inclusion criteria. All were case series reports (range from 2 to 36 subjects) with a total of 173 subjects. Due to the heterogeneous data, a meta-analysis was unfeasible. CONCLUSIONS: In concordance with some findings in the literature, the presented case report suggests that a drop of intra- and extracochlear ECochG signals during the insertion of the electrode array is associated with cochlear trauma and postoperative hearing loss in some cases. However, the literature is inconclusive regarding the correlation between intraoperative changes of the ECochG signals and postoperative hearing preservation. More studies investigating the correlation are needed to provide sufficient data.

Distorted sound perception and subjective benefit after stapedotomy - a prospective single-centre study.

OBJECTIVE: To evaluate the quality of perceived sound after stapedotomy over a 1-year follow-up period focussing on incidence of dysacusis, particularly distorted sound perception (DSP). DSP was assessed by (i) determination of the frequencies and hearing level that such perceptions are elicited by pure tones (pure-tone-evoked distorted sound perception, PTE-DSP), a novel psychoacoustic measurement introduced in this paper, and (ii) assessment of patient-reported occurrence of DSP using the Amsterdam Post Operative Sound Evaluation (APOSE) questionnaire (APOSE-DSP). DESIGN: Prospective study. STUDY SAMPLE: Patients (n = 23) with otosclerosis undergoing stapedotomy. RESULTS: An air-bone gap of <20 dB was achieved in 100% of the patients. Three weeks postoperatively, 48% of the patients reported measured PTE-DSP and 39% of the patients experienced APOSE-DSP. The PTE-DSP significantly decreased during the 1-year follow-up period (p = 0.03). Postoperatively, APOSE-DSP was associated with a smaller benefit (improvement in air conduction; p = 0.03), yet, a lower bone conduction pure-tone average was associated with PTE-DSP (p = 0.006). CONCLUSIONS: DSP after stapedotomy is associated with a smaller benefit 3 months after stapedotomy. DSP commonly occur after stapedotomy, but decrease over time. This is important information to be included in patient counselling before stapedotomy.

Japanese translation, cross-cultural adaption and multicentre validation of the Zurich chronic middle ear inventory (ZCMEI-21-Jap).

OBJECTIVE: In the assessment of chronic otitis media (COM) and its treatment, patient-reported outcomes are becoming increasingly important. The aim of the present study was to translate and validate the Zurich chronic middle ear inventory (ZCMEI-21) in Japanese in order to provide the first Japanese-language instrument for measuring health-related quality of life (HRQoL) in COM. METHODS: The ZCMEI-21 was translated into Japanese according to published guidelines. In order to assess validity, the ZCMEI-21-Jap total score was compared to a question directly addressing HRQoL as well as the five-level version of the EQ-5D questionnaire, a generic measure of HRQoL. RESULTS: Demographic data and validity were assessed in a total of 91 COM patients. The ZCMEI-21-Jap total and subscale scores were well comparable to those of the original validation study. Cronbach's α of the ZCMEI-21-Jap was 0.85, indicating an excellent internal consistency. The ZCMEI-21-Jap total score showed a strong correlation (r=0.68, p<0.0001) to the question directly addressing HRQoL and, as expected, only a moderate correlation to the EQ-5D scores (r=0.49, p<0.0001 for descriptive system score and r=0.44, p<0.0001 for VAS score). CONCLUSION: We successfully translated the ZCMEI-21 into Japanese and were able to obtain sufficient information during the validation process for the use of the ZCMEI-21-Jap to quantify HRQoL in patients with COM. With the current study, we aim to take a step forward towards an international standardization of reporting HRQoL in COM.