Comparing linear and non-linear models to estimate the appropriate cochlear implant electrode array length-are current methods precise enough?

PURPOSE: In cochlear implantation with flexible lateral wall electrode arrays, a cochlear coverage (CC) range between 70% and 80% is considered ideal for optimal speech perception. To achieve this CC, the cochlear implant (CI) electrode array has to be chosen according to the individual cochlear duct length (CDL). Here, we mathematically analyzed the suitability of different flexible lateral wall electrode array lengths covering between 70% and 80% of the CDL. METHODS: In a retrospective cross-sectional study preoperative high-resolution computed tomography (HRCT) from patients undergoing cochlear implantation was investigated. The CDL was estimated using an otosurgical planning software and the CI electrode array lengths covering 70-80% of the CDL was calculated using (i) linear and (ii) non-linear models. RESULTS: The analysis of 120 HRCT data sets showed significantly different model-dependent CDL. Significant differences between the CC of 70% assessed from linear and non-linear models (mean difference: 2.5 mm, p < 0.001) and the CC of 80% assessed from linear and non-linear models (mean difference: 1.5 mm, p < 0.001) were found. In up to 25% of the patients none of the existing flexible lateral wall electrode arrays fit into this range. In 59 cases (49,2%) the models did not agree on the suitable electrode arrays. CONCLUSIONS: The CC varies depending on the underlying CDL approximation, which critically influences electrode array choice. Based on the literature, we hypothesize that the non-linear method systematically overestimates the CC and may lead to rather too short electrode array choices. Future studies need to assess the accuracy of the individual mathematical models.

Internal auditory canal volume in normal and malformed inner ears.

PURPOSE: A narrow bony internal auditory canal (IAC) may be associated with a hypoplastic cochlear nerve and poorer hearing performances after cochlear implantation. However, definitions for a narrow IAC vary widely and commonly, qualitative grading or two-dimensional measures are used to characterize a narrow IAC. We aimed to refine the definition of a narrow IAC by determining IAC volume in both control patients and patients with inner ear malformations (IEMs). METHODS: In this multicentric study, we included high-resolution CT (HRCT) scans of 128 temporal bones (85 with IEMs: cochlear aplasia, n = 11; common cavity, n = 2; cochlear hypoplasia type, n = 19; incomplete partition type I/III, n = 8/8; Mondini malformation, n = 16; enlarged vestibular aqueduct syndrome, n = 19; 45 controls). The IAC diameter was measured in the axial plane and the IAC volume was measured by semi-automatic segmentation and three-dimensional reconstruction. RESULTS: In controls, the mean IAC diameter was 5.5 mm (SD 1.1 mm) and the mean IAC volume was 175.3 mm3 (SD 52.6 mm3). Statistically significant differences in IAC volumes were found in cochlear aplasia (68.3 mm3, p < 0.0001), IPI (107.4 mm3, p = 0.04), and IPIII (277.5 mm3, p = 0.0004 mm3). Inter-rater reliability was higher in IAC volume than in IAC diameter (intraclass correlation coefficient 0.92 vs. 0.77). CONCLUSIONS: Volumetric measurement of IAC in cases of IEMs reduces measurement variability and may add to classifying IEMs. Since a hypoplastic IAC can be associated with a hypoplastic cochlear nerve and sensorineural hearing loss, radiologic assessment of the IAC is crucial in patients with severe sensorineural hearing loss undergoing cochlear implantation.

Volumetry improves the assessment of the vestibular aqueduct size in inner ear malformation.

OBJECTIVES: Enlarged vestibular aqueduct (EVA) is a common finding associated with inner ear malformations (IEM). However, uniform radiologic definitions for EVA are missing and various 2D-measurement methods to define EVA have been reported. This study evaluates VA volume in different types of IEM and compares 3D-reconstructed VA volume to 2D-measurements. METHODS: A total of 98 high-resolution CT (HRCT) data sets from temporal bones were analyzed (56 with IEM; [cochlear hypoplasia (CH; n = 18), incomplete partition type I (IPI; n = 12) and type II (IPII; n = 11) and EVA (n = 15)]; 42 controls). VA diameter was measured in axial images. VA volume was analyzed by software-based, semi-automatic segmentation and 3D-reconstruction. Differences in VA volume between the groups and associations between VA volume and VA diameter were assessed. Inter-rater-reliability (IRR) was assessed using the intra-class-correlation-coefficient (ICC). RESULTS: Larger VA volumes were found in IEM compared to controls. Significant differences in VA volume between patients with EVA and controls (p < 0.001) as well as between IPII and controls (p < 0.001) were found. VA diameter at the midpoint (VA midpoint) and at the operculum (VA operculum) correlated to VA volume in IPI (VA midpoint: r = 0.78, VA operculum: r = 0.91), in CH (VA midpoint: r = 0.59, VA operculum: r = 0.61), in EVA (VA midpoint: r = 0.55, VA operculum: r = 0.66) and in controls (VA midpoint: r = 0.36, VA operculum: r = 0.42). The highest IRR was found for VA volume (ICC = 0.90). CONCLUSIONS: The VA diameter may be an insufficient estimate of VA volume, since (1) measurement of VA diameter does not reliably correlate with VA volume and (2) VA diameter shows a lower IRR than VA volume. 3D-reconstruction and VA volumetry may add information in diagnosing EVA in cases with or without additional IEM.

[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.

Comparison of two disease­specific instruments assessing health-related quality of life in patients with chronic otitis media.

PURPOSE: Evaluating the current health state in chronic otitis media (COM), audiologic results are complemented by subjective outcomes, such as health-related quality of life (HRQoL). Two disease-specific instruments assessing HRQoL in COM in German-speaking patients exist, i.e., the chronic otitis media outcome test (COMOT-15) and the Zurich chronic middle ear inventory (ZCMEI-21). Since the psychometric properties of these questionnaires in a concurrent application are unknown, the aim of this study was to compare the COMOT-15 and the ZCMEI-21. METHODS: HRQoL was assessed in adult COM patients using the COMOT-15 and the ZCMEI-21. Psychometric properties were determined, including response distribution, concurrent validity, internal consistency, correlation to hearing and gender differences. RESULTS: In 173 patients (mean age 51.5 years), both questionnaires showed normally distributed scores without strong floor and ceiling effects. The total scores and subscores of both questionnaires exhibited satisfactory internal consistency (Cronbach's α 0.7-0.9) with the exception of the COMOT-15 hearing subscore (α = 0.94) and the ZCMEI-21 medical resource subscore (α = 0.66). Fair correlations between the air conduction pure-tone average and the total scores were found (COMOT-15: r = 0.36, p < 0.0001; ZCMEI-21: r = 0.34, p < 0.0001). CONCLUSION: In the first study comparing the COMOT-15 and the ZCMEI-21, both questionnaires exhibited satisfactory psychometric properties with several subtle differences. The COMOT-15 has a strong focus on hearing with a probably redundant content of the hearing subscore and may be suited for hearing-focused research questions. The ZCMEI-21 provides a comprehensive assessment of the COM symptom complex and may therefore also be used in research settings, where ear discharge, vertigo or pain should be covered.

[The professional ear user-implications for the prevention, diagnosis, and treatment of ear diseases]. / Der "Professional Ear User" ­ Implikationen für die Prävention, Diagnostik und Therapie von Ohrerkrankungen.

BACKGROUND: Perfect hearing is crucial to the practice of various professions, such as instrument makers, musicians, sound engineers, and other professions not related to music, such as sonar technicians. For people of these occupational groups, we propose the term "professional ear user" (PEU) in analogy to "professional voice user". PEUs have special requirements for their hearing health, as they have well-known above-average auditory perceptual abilities on which they are professionally dependent. OBJECTIVE: The purpose of this narrative review is to summarize selected aspects of the prevention, diagnosis, and treatment of ear disorders in PEUs. RESULTS AND CONCLUSION: Prevention of hearing disorders and other ear diseases includes protection from excessive sound levels, avoidance of ototoxins and nicotine, and a safe manner of cleaning the outer auditory canal. Diagnosing hearing disorders in PEUs can be challenging, since subclinical but relevant changes in hearing cannot be reliably objectified by conventional audiometric methods. Moreover, the fact that a PEU is affected by an ear disease may influence treatment decisions. Further, physicians must be vigilant for non-organic ear diseases in PEUs. Lastly, measures to promote comprehensive ear health in PEUs as part of an educational program and to maintain ear health by means of a specialized otolaryngology service are discussed. In contrast to existing concepts, we lay the attention on the entirety of occupational groups that are specifically dependent on their ear health in a professional setting. In this context, we suggest avoiding a sole focus on hearing disorders and their prevention, but rather encourage the maintenance of a comprehensive ear health.

[Mastoid cavity obliteration with allogenic and autologous material]. / Allogenes und autologes Material führt bei Mastoidhöhlenobliteration zu vergleichbaren Rezidivraten.

INTRODUCTION: Surgical obliteration is the treatment of choice for symptomatic mastoid cavities. Various methods and materials are available. Allogeneic material is easily available but can lead to wound infections due to rejection, granulation and inflammatory reactions. Consequently, autologous material is often preferred due to its good biocompatibility. The aim of this study was to compare the long-term outcomes of patients after mastoid cavity obliteration with allogeneic and autologous material with regard to the occurrence of recurrent cholesteatoma, the number of revision surgeries and the impact of surgery on the health-related quality of life. METHODS: Patients who underwent mastoid cavity obliteration with an allogeneic material (hydroxyapatite matrix [HMM]) were retrospectively included in the study. In a prospective part, patients who underwent mastoid cavity obliteration with autologous reconstruction material (AutoM) were included. In addition to a detailed chart review, all patients underwent pure tone audiometry and completed the Zurich Chronic Middle Ear Inventory (ZCMEI-21) at the postoperative examination to assess health-related disease-specific quality of life. RESULTS: A total of 22 patients with a mean age of 56.9 (SD 18.7 years) receiving HMM (mean follow-up interval: 88.3 months [SD 21.9 months]) and 25 patients with a mean age of 52.4 years (SD 13.7 years) receiving AutoM (mean follow-up interval: 13.5 months [SD 9.5 months]) were included in the study. The revision-free rate for audiological indication after one year was 100% for HMM and 85% for AutoM. The recurrence-free rate at one year was 95% for HMM and 100% for AutoM. 55% of the patients with alloplastic material and 8% of the patients with autologous material required revision surgery due to ear discharge or recurrent cholesteatoma. The groups did not differ in terms of postoperative hearing outcome or postoperative quality of life. DISCUSSION: Obliteration of mastoid cavities is a surgical challenge. There was no relevant difference between the materials used in terms of recurrence rate, hearing and health-related quality of life within the 13.5-month observation period of both groups. The lack of subjective symptoms together with the high rate of recurrent cholesteatoma and revision indications emphasises the need for regular clinical follow-up including ear microscopy in chronic middle ear disease and strongly suggests regular follow-up examinations.

Characteristics of health-related quality of life in different types of chronic middle ear disease.

PURPOSE: Different types of chronic middle ear disease (CMED) present with differing severity of single symptoms, such as discharge, vertigo or hearing loss, which impair health-related quality of life (HRQoL). Here, we aimed to (i) investigate characteristics of HRQoL and (ii) evaluate the association between HRQoL and hearing among different types of CMED. METHODS: In this prospective longitudinal cohort study, we included adult patients undergoing surgical treatment for CMED. Primary outcomes included HRQoL assessed by the Zurich Chronic Middle Ear Inventory (ZCMEI-21) and hearing assessed by pure-tone audiometry. RESULTS: In the included 108 patients (51% females), CMED were chronic otitis media (COM) with (n = 46) or without (n = 22) cholesteatoma, persistent mastoid cavity (with [n = 15] or without [n = 10] recurrent cholesteatoma), revision ossiculoplasty for hearing restoration (n = 14), and postinflammatory meatal fibrosis (n = 1). Preoperatively, the mean ZCMEI-21 score showed statistically significant differences among different types of CMED (p = 0.007) with persistent mastoid cavity without cholesteatoma exhibiting the highest score (34.1, SD 7.7) indicating poor HRQoL. At a mean follow-up period of 183 days, no statistically significant differences in the ZCMEI-21 scores among different types of CMED were observed (p = 0.67). CONCLUSION: This study objectifies differences in HRQoL among different types of CMED. In patients with indication for functional surgery only, e.g., persistent mastoid cavity without cholesteatoma, the worst HRQoL was observed. Yet, in these types of CMED, HRQoL guides decision for treatment. Moreover, differences in HRQoL among different types of CMED were not closely associated with hearing, but largely depended on other symptoms, such as discharge or vertigo.

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.

Establishing the minimal clinically important difference (MCID) of the Zurich Chronic Middle Ear Inventory (ZCMEI-21) in patients treated for chronic middle ear disease.

AIM: To determine the minimal clinically important difference (MCID) of the Zurich Chronic Middle Ear Inventory (ZCMEI-21), a questionnaire assessing health-related quality of life (HRQoL) in chronic otitis media (COM). METHODS: In this longitudinal study, 103 patients completed the ZCMEI-21 before and after surgery for chronic otitis media. An anchor-based method including a global rating of change (GRC) was used to assess the MCID of the ZCMEI-21. RESULTS: A total of 103 patients were included. The mean preoperative and postoperative ZCMEI-21 scores were 28.6 (SD 13.6), and 21.8 (SD 12.8), respectively. The mean change was 6.8 (SD 0.8; p < 0.0001). A significant correlation between the ZCMEI-21 scores and the GRC was found (r = - 0.5; p < 0.001). Using the anchor-based method, the MCID of the ZCMEI-21 was estimated at 5.3 (SD 12.0). CONCLUSIONS: Knowledge of values indicating a clinically relevant change in patient-reported outcome measures is important when interpreting effects of different treatment modalities. This is the first study assessing the MCID of a questionnaire measuring HRQoL in COM, i.e. the ZCMEI-21. We recommend a MCID of 5 in COM patients undergoing surgical treatment. This information substantially increases the usefulness of the ZCMEI-21 as an outcome measure in COM as changes can be assessed with regard to their clinical meaningfulness.

Mastoid cavity obliteration leads to a clinically significant improvement in health-related quality of life.

OBJECTIVE: To assess the change in health-related quality of life (HRQoL) in patients undergoing mastoid cavity obliteration. METHODS: Patients who had undergone canal wall-down mastoidectomy for chronic otitis media with creation of a persistent mastoid cavity and underwent revision tympanomastoid surgery including mastoid cavity obliteration using autologous material were included. Audiological measurements including air conduction (AC) and bone conduction (BC) pure-tone averages (PTA) and the air-bone gap (ABG) were assessed. Health-related Quality of Life (HRQoL) was assessed by the Zurich Chronic Middle Ear Inventory (ZCMEI-21) pre- and postoperatively. RESULTS: A total of 25 patients (16 females and 9 males; mean age 51.6 years, 14 right and 11 left ears) were included. Patients were reexamined after a mean follow-up period of 9.2 months (SD = 6.5) after obliteration of the mastoid cavity. Compared to the preoperative visit, patients showed a significantly reduced AC PTA at the postoperative visit (mean difference: - 4.1; SD = 10.4, p = 0.045). The mean ZCMEI-21 score changed from 31.7 (SD = 14.5) preoperatively to 17.4 (SD = 15.1) postoperatively (mean difference: - 14.3; SD = 19.1; p = 0.0002). The mean ZCMEI-21 score changes were neither correlated to the AC PTA shift (p = 0.60) nor to the ABG shift (p = 0.66). CONCLUSIONS: This is the first study reporting a highly significant and clinically important improvement in HRQoL after mastoid cavity obliteration in a prospective setting. The improvement in HRQoL was not correlated to the hearing improvement. As a clinical implication, we provide evidence for a substantial subjective benefit of the surgical obliteration of a symptomatic mastoid cavity and, therefore, encourage this surgical procedure.

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.

Immunolocalization of calcium sensing and transport proteins in the murine endolymphatic sac indicates calciostatic functions within the inner ear.

An exceptionally low calcium (Ca2+) concentration in the inner ear endolymph ([Ca2+]endolymph) is crucial for proper auditory and vestibular function. The endolymphatic sac (ES) is believed to critically contribute to the maintenance of this low [Ca2+]endolymph. Here, we investigated the immunohistochemical localization of proteins that are presumably involved in the sensing and transport of extracellular Ca2+ in the murine ES epithelium. Light microscopic and fluorescence immunolabeling in paraffin-embedded murine ES tissue sections (male C57BL/6 mice, 6-8 weeks old) demonstrated the presence of the calcium-sensing receptor CaSR, transient receptor potential cation channel subtypes TRPV5 and TRPV6, sarco/endoplasmic reticulum Ca2+-ATPases SERCA1 and SERCA2, Na+/Ca2+ exchanger NCX2, and plasma membrane Ca2+ ATPases PMCA1 and PMCA4 in ES epithelial cells. These proteins exhibited (i) membranous (apical or basolateral) or cytoplasmic localization patterns, (ii) a proximal-to-distal labeling gradient within the ES, and (iii) different distribution patterns among ES epithelial cell types (mitochondria-rich cells (MRCs) and ribosome-rich cells (RRCs)). Notably, in the inner ear membranous labyrinth, CaSR was exclusively localized in MRCs, suggesting a unique role of the ES epithelium in CaSR-mediated sensing and control of [Ca2+]endolymph. Structural loss of the distal ES, which is consistently observed in Meniere's disease, may therefore critically disturb [Ca2+]endolymph and contribute to the pathogenesis of Meniere's disease.

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.

English translation and validation of the Zurich chronic middle ear inventory (ZCMEI-21-E) assessing quality of life in chronic otitis media: A prospective international multicentre study.

OBJECTIVES: To translate and validate the Zurich Chronic Middle Ear Inventory (ZCMEI-21) for the English language in order to provide an English instrument to assess health-related quality of life in chronic otitis media (COM). DESIGN: Pilot translation study including cognitive debriefings, prospective multicentre cross-sectional psychometric validation study. SETTING: Four tertiary referral centres in three different English-speaking countries (UK, USA and Australia). PARTICIPANTS: Adult patients suffering from COM. MAIN OUTCOME MEASURES: The English translation of the ZCMEI-21 (ZCMEI-21-E) and the five-level version of the EQ-5D questionnaire. The EQ-5D, which constitutes a generic measure of health-related quality of life, consists of a descriptive system score and a visual analogue scale. Statistical outcomes included single-item descriptive statistics, internal consistency (Cronbach's α) as an indicator of reliability, as well as construct validity. RESULTS: A total of 124 patients suffering from COM were included. The mean age was 50.1 years (SD 16.9 years), and 72 (58.1%) were males. The Cronbach's α of the ZCMEI-21-E was 0.91, suggesting an excellent internal consistency. The Spearman's correlation coefficient of the ZCMEI-21-E total score was 0.55 (P < 0.0001) for convergent construct validity with EQ-5D descriptive system score and 0.57 (P < 0.0001) with the EQ-5D visual analogue scale. CONCLUSIONS: The ZCMEI-21-E is a new validated questionnaire that provides clinicians with a short, comprehensive and reliable instrument to quantify health-related quality of life in patients suffering from COM. The ZCMEI-21-E may be of use in clinical routine as well as in outcome research and monitoring.

Development and validation of the Zurich chronic middle ear inventory (ZCMEI-21): an electronic questionnaire for assessing quality of life in patients with chronic otitis media.

Because existing patient-reported outcome measures (PROMs) specific for chronic otitis media (COM) are lacking certain relevant symptoms and dimensions of health-related quality of life (HRQoL), we aimed to develop and validate a new questionnaire for comprehensively measuring HRQoL in adult patients with COM. An expert panel and patients were involved in developing the first version of the Zurich chronic middle ear inventory, containing 33 items (ZCMEI-33). An electronic application was chosen not only to provide maximal data quality, but also to facilitate and accelerate data analysis. Item reduction was performed by testing the questionnaire in a first cohort (n = 85). Using sequential statistical analysis, the ZCMEI-33 was reduced to 21 items (ZCMEI-21). Subsequently, the ZCMEI-21 was validated in a second cohort (n = 76). Validation revealed a Cronbach's α of 0.91, indicating excellent internal consistency. Moreover, the ZCMEI-21 was able to discriminate between patients with COM and healthy participants (p < 0.0001), thus possessing good discrimination validity. Assessing criterion validity, the ZCMEI-21 total score was compared to a question directly addressing HRQoL and the EQ-5D descriptive system score, a generic measure of HRQoL. Whereas the ZCMEI-21 total score and the EQ-5D descriptive system score were only moderately correlated (r = 0.60, p < 0.0001), the ZCMEI-21 total score and the question directly addressing HRQoL showed a strong correlation (r = 0.74, p < 0.0001). In conclusion, sufficient information on reliability and validity was obtained to propagate the application of the ZCMEI-21 to quantify HRQoL in patients with COM.