Digital live imaging of intraoperative electrocochleography during cochlear implantation: the first 50 patients.

INTRODUCTION: Real-time visualization of intraoperative electrocochleography (ECochG) potentials via a digital microscope during cochlear implantation can provide direct feedback during electrode insertion. The aim of this prospective, randomized study of 50 patients was to obtain long-term data with a focus on residual hearing preservation and speech understanding. MATERIAL AND METHODS: Cochlear implantations were performed in 50 patients (26 female, 24 male) with residual hearing using a digital microscope. Patients were randomized into two groups. Intraoperative ECochG potentials were either displayed directly in the surgeon's field of view (picture-in-picture display, PiP) or not directly in the field of view (without picture-in-picture display, without PiP). Residual hearing preservation and speech comprehension were recorded within a 1-year follow-up period, compared between groups (PiP versus without PiP) and to a control group of 26 patients implanted without ECochG. RESULTS: Mean insertion time was significantly longer in the picture-in-picture group (p = 0.025). Residual hearing preservation after 6 weeks at 250 Hz was significantly better in the picture-in-picture group (p = 0.017). After one year, 76% of patients showed residual hearing in the picture-in-picture group (62% without picture-in-picture technique, p = n.s.). Use of the picture-in-picture technique resulted in better long-term pure tone residual hearing preservation at 250, 500, and 1000 Hz. Speech intelligibility improved by 46% in the picture-in-picture group (38% without picture-in-picture). DISCUSSION: This study is the first to describe long-term results in a large cohort of cochlear implant patients in whom digital visualization of intraoperative ECochG was used. Our results show that visualization of intraoperative ECochG has a positive effect on residual hearing preservation.

Two different methods to digitally visualize continuous electrocochleography potentials during cochlear implantation: a first description of feasibility.

PURPOSE: The study explores the potential of real-time electrocochleographic potentials (ECochG) visualization during electrode insertion using digital microscopes such as RoboticScope (BHS®). Collaborative software development of the MAESTRO Software (MED-EL®) offers continuous ECochG monitoring during implantation and postoperative hearing evaluation, addressing previous time constraints. The study aims to assess software applicability and the impact of real-time visualization on long-term residual hearing preservation. METHODS: Eight patients with residual hearing underwent cochlear implantation with Flex26 or Flex28 electrode according to the Otoplan evaluation. ECochG responses were measured and visualized during electrode insertion, with insertion times recorded. Two randomized display methods (graph and arrows) tracked ECochG potentials. Postoperative behavioral thresholds determined hearing preservation. Successful real-time intraoperative ECochG visualization was achieved in all cases, enabling surgeon adaptation. Mean electrode insertion time was 114 s, with postoperative thresholds comparable to preoperative values. Visualization did not affect surgeon workload. ECochG amplitudes differed between patients with and without residual hearing. CONCLUSION: The study demonstrates effective implementation of advanced ECochG software combined with real-time visualization, enabling residual hearing preservation during CI. Visualization had no apparent effect on surgeon performance or workload. Future investigation involving a larger population will assess the long-term impact of ECochG on hearing threshold and structure preservation.

Cognitive deficits in patients with peripheral vestibular dysfunction.

BACKGROUND AND PURPOSE: Previous studies demonstrated cognitive deficits in patients with peripheral vestibulopathy (PVP) with dysfunction of spatial navigation and orientation, but also documented cognitive decline in nonspatial abilities. This study evaluates cognitive deficits in patients with unilateral vestibulopathy (UVP) as well as bilateral vestibulopathy (BVP) in multiple cognitive domains using common screening tests to reliably detect these deficits in clinical practice. METHODS: This prospective study compared patients with UVP and BVP to age- and sex-matched healthy controls (HC). Tests included the Alzheimer's Disease Assessment Scale (ADAS), Mini-Mental Status Examination (MMSE), Trail Making Test Part A and B, Clock Drawing Task, Executive Interview-25 (EXIT25), Dementia Detection (DemTect), and the Judgment of Line Orientation (JLO). The Montgomery-Åsberg Depression Rating Scale was used to control for depression. Videonystagmography objectively reconfirmed PVP. The Vertigo Symptoms Scale and the Dizziness Handicap Inventory were used to assess for symptom severity and restrictions of activities of daily living. RESULTS: Eighty-one patients (65 UVP, 16 BVP) were compared to 55 HC. Patients showed impairment in ADAS, MMSE, DemTect, EXIT25, and JLO. No differences between UVP and BVP were detected. The relative risk (RR) estimates of developing cognitive deficits following PVP were increased. The RR for the ADAS was higher in BVP (RR = 4.91, 95% confidence interval [CI] = 1.87-12.9, p = 0.001) than in UVP (RR = 3.75, 95% CI = 1.65-8.51, p = 0.002), but was similar for the MMSE and DemTect between groups. CONCLUSIONS: Patients with PVP showed deficits in multiple cognitive domains including nonspatial cognitive abilities. Vestibulopathy could be a risk factor for the development of cognitive impairment.

Visualizing Contralateral Suppression of Hearing Sensitivity via Acoustic and Electric Brainstem Audiometry in Bimodal Cochlear Implant Patients: A Feasibility Study.

INTRODUCTION: The medial olivocochlear reflex (MOCR) is a part of the binaural processing strategies and influences the efferent auditory pathway in normal-hearing individuals. Patients with asymmetric hearing loss often benefit from a bimodal hearing solution with a cochlear implant (CI) and a hearing aid (HA). However, hearing performances may vary with some surprisingly high- or low-performing CI/HA users. A potential role of the MOCR among these patients warrants further investigation. Otoacustics emissions are an established method to visualize the reflex; however, this technique implies some disadvantages. To visualize the MOCR via auditory brainstem response (ABR) could be a promising alternative. METHODS: Twenty-three bimodal CI/HA users were enrolled. Experimental setup was as follows: I. electrical ABR on the CI side was recorded with and without simultaneous contralateral noise signal at the HA side, II. acoustic ABR was recorded on the HA side with and without simultaneous contralateral noise at the CI side. Brainstem thresholds and amplitudes of waveforms I-V with and without contralateral noise were compared. Potential correlations of patient-related factors and hearing performances were analysed. RESULTS: In four individuals, a reduction of brainstem audiometry thresholds could be observed at the acoustic brainstem audiometry. In these cases, results could be reproduced. Summarizing ABR measurements at the HA side of all individuals, no relevant changes of ABR thresholds (dB nHL) or waveform amplitude reductions (nV) could be observed irrespective of the presence or absence of a contralateral suppression signal. CONCLUSION: Threshold changes of acoustic ABR upon presentation of a contralateral suppression signal could not generally be measured in bimodal CI users. However, in a subgroup, a highly reproducible effect was demonstrated if a contralateral suppression signal was applied. A reactivated rather than rehabilitated MOCR may have accounted for this effect in this subgroup. One could speculate that in these patients, bimodal fitting could be affected by the MOCR efferents.

Evaluation of hearing preservation in adults with a slim perimodiolar electrode.

PURPOSE: Numerous endeavors have been undertaken to preserve hearing in cochlear implant (CI) patients. Particularly, optimization of electrode array design aims at preservation of residual hearing (RH). This study examines whether a slim perimodiolar (PM) electrode array could bear the capability to preserve hearing. METHODS: A total of 47 patients underwent cochlear implantation receiving the PM electrode. (i) Patients with pure tone audiogram (PTA) thresholds better than 85 dB and/or hearing loss for Freiburg speech test numbers less than 60 dB and more than 50% maximum monosyllabic understanding were assigned to the RH group (n = 17), while all others belonged to the noRH group (n = 30). (ii) Another group implanted with a slim straight, lateral wall (LW) electrode was recruited for comparison. RESULTS: We compared 17 RH-30 noRH patients all receiving the PM electrode. RH in PM recipients decreased faster than in LW recipients. No significant differences were observed between both (RH v/s noRH) groups in NRT thresholds, Freiburg speech test and A§E® phonemes. Analogous satisfaction levels were indicated through the questionnaires in terms of sound quality, hearing in silence, noise and directional hearing in both groups. CONCLUSIONS: The results suggest that hearing preservation is influenced not only by electrode shape but various factors. This study opens an avenue for further investigations to elucidate and enumerate the causes for progressive hearing loss.

Assessing Medial Olivocochlear Reflex Strengths via Auditory Brainstem Response: Measurement and Variability in Normal-Hearing Individuals.

PURPOSE: Optimal measurement settings to measure the medial olivocochlear reflex (MOCR) in humans have not yet been defined. The purpose of this study was to advance the representation of the MOCR in auditory brainstem response (ABR) as an addition to the current diagnostic portfolio. PARTICIPANTS AND METHOD: Twelve female and 14 male normal-hearing adults participated in the study. Potential effects of a contralateral acoustic stimulus (CAS) on amplitude changes were investigated by recording ABR waveform profiles on the left side at click intensities of 50/60/70 dB nHL with and without CAS (60 dB SPL). Secondly, to detect potential chronological order influences, measurement settings were rearranged on the right side and measurements were repeated. Additionally, ABR thresholds were recorded with and without a CAS in 10 patients. RESULTS: When the effect of contralateral suppression was analyzed on the basis of amplitude changes, there was a change under administration of the CAS signal that was statistically significant. Interestingly, the order of recordings affected the degree of amplitude change. In three out of 10 patients, reproducible suppression effects on ABR thresholds were detectable upon CAS presentation. CONCLUSIONS: To our knowledge, this is the largest study dealing with the recording of the MOCR elicited by a contralateral noise via ABR in normal-hearing individuals. Effects of MOCR are measurable via amplitude changes upon CAS administration. Chronological orders influence the impact of this effect on amplitude changes. Optimal measurement settings have not yet been defined. However, experiments such as this study may help to further improve measurements, and thus advance the representation of the MOC reflex in ABR as an addition to the current diagnostic portfolio.

Suitability of auditory speech sound evaluation (A§E®) in German cochlear implant patients.

A§E® is an audiological evaluation tool based on speech sounds as stimuli and was developed by The Eargroup, Antwerp. It consists of three levels: detection, discrimination and identification of phonemes and is a sufficient tool to measure supraliminal auditory capacities in Flemish language of children 10 months and older as well as adults. The aim of this study was to test the suitability of patients for cochlear implant in Germany, using 245 consecutive measurements (141 adults, 79 children, 25 controls) in a standard test protocol for phoneme detection and discrimination, Mainzer and Göttinger in children and Freiburger in adults pre-, 3 and 6 months and 1 year after implantation. Results in detection and discrimination regarding frequency spectrum and fitting parameters were evaluated using the following statistical methods: t test and correlation analysis. Detection improves first, followed by numbers, phoneme discrimination and monosyllables. Test results compared pre- to 3, 6 months and later, postoperatively, differ significantly (p < 0.05). A "ceiling-effect" is obtained between 6 months and 1 year (reproduction in A§E® >90%). Development of detection and discrimination correlates directly with results in numbers and monosyllables (r = 0.92). Non-German speaking patients cope better with A§E® than with numbers and monosyllables. Evaluation of phoneme discrimination enables better frequency specific fitting. Results indicated that A§E® is a valuable diagnostic supplement in fitting and therapy of cochlear implant patients regardless of cognitive level, age and language. Important information on frequency-resolving power of cochlear nerve after implantation is gained. Use of A§E® enables comparability and evaluation of study results in different countries.

Transimpedance Matrix Measurements Reliably Detect Electrode Tip Fold-over in Cochlear Implantation.

OBJECTIVE: During cochlear implantation, electrophysiological tests are performed to document safe technical functioning of implant and electrodes. In rare cases, the apical part of the electrode folds over during insertion. The data from transimpedance matrix (TIM) measurements enable the generation of a heat map or TIM profile measuring the spatial distribution of voltage. The aim of this study was to determine the accuracy of heat-map TIM profiles and compare them with spread of excitation (SOE) measurements and intraoperative imaging for prediction of electrode malposition. STUDY DESIGN: Non-randomized study. SETTING: Tertiary referral center. PATIENTS AND INTERVENTIONS: One hundred patients who underwent cochlear implantation with completed TIM measurements, SOE data and perioperative imaging met the inclusion criteria and were enrolled. MAIN OUTCOME MEASURE: The electrophysiological data on the electrode array positioning was compared with temporal bone imaging. RESULTS: In seven cases, TIM measurements showed irregular results. In two cases, irregular TIM profiles were registered, but SOE data and 3D x-ray of the temporal bone didn't display deviated electrode positioning. A 3D x-ray of the skull displayed electrode tip fold-over in four cases and electrode buckling in one case. Sensitivity of TIM measurements and SOE data was 100%, specificity of TIM measurements was 97.89%, and specificity of SOE data was 98.93%. CONCLUSION: Out of 100 patients using TIM measurements for detection of electrode malpositioning, no false negative cases were detected. TIM measurements successfully detect electrode malposition in an intraoperative setting. Different heat map patterns may be observed depending on location and type of malposition.

Digital Live Imaging of Intraoperative Electrocochleography - First Description of Feasibility and Hearing Preservation During Cochlear Implantation.

INTRODUCTION: Intraoperative electrocochleography (ECochG) during cochlear implantation is a promising tool to preserve residual hearing. However, the time gap between insertion of the electrode and acoustic feedback from the audiologist to the surgeon can cause delay and subsequently irreparable damage to cochlear structures. In this feasibility study, for the first time, real-time visualization of intraoperative ECochG via digital microscope display directly to the surgeon was successfully performed in four patients. MATERIALS AND METHODS: Four patients with residual hearing underwent cochlear implantation. Intraoperative electrocochleography responses were collected and direct visualization during the time of electrode insertion into the surgeon's field of view in the binoculars using augmented real-time digital imaging was realized. The time of electrode insertion was recorded. Hearing preservation was determined by testing postoperative changes in behavioral thresholds. RESULTS: Digital live visualization of intraoperative ECochG using image augmentation in a digital microscope was successfully performed in all cases and enabled direct adaptation of the surgeon's insertion behavior. Mean time of electrode insertion was 129.8 seconds. Postoperative behavioral thresholds were comparable to preoperative taken thresholds. Preservation of residual hearing in the low frequency range was possible. DISCUSSION: This study is the first to describe digital visualization of intraoperative electrocochleography as a new method enabling the surgeon to directly react to changes in amplitude of the cochlea microphonics. Our results show that augmentation of the intraoperative live imaging with electrical potentials could add to hearing preservation during cochlear implantation.

Acute arterial hemorrhage following radiotherapy of oropharyngeal squamous cell carcinoma.

BACKGROUND AND PURPOSE: Vascular erosion is a rare but life-threatening complication after radiotherapy. The authors report on acute arterial bleeding and its therapy following radiotherapy of oropharyngeal tumors. PATIENTS AND METHODS: Ten patients with oropharyngeal squamous cell carcinoma of any stage developed foudroyant acute arterial hemorrhage 3-46 months (14.4 +/- 5.1 months) after primary (5/10) or adjuvant radio(chemo)therapy (R[C]T). RESULTS: All patients had a history of recurrent minor bleeding episodes and showed deep mucosal ulcerations also outside the primary tumor region. A life-threatening arterial hemorrhage appeared in the area of these mucosal defects in the pharyngeal region. Affected vessels were the common carotid artery as well as the internal and the external portion with branches like the ascending pharyngeal and superior thyroid arteries. Treatment consisted of emergency intubation or tracheotomy followed by exposure and package of the pharynx and surgical ligature and/or embolization. 6/10 patients (all hospitalized) survived the episode, however, lethal outcome in 4/10 patients (outpatients) was related to asphyxia as a result of blood aspiration or exsanguination. None of the patients revealed evidence of persistent or recurrent tumor disease as proven by biopsy/autopsy and imaging technique. CONCLUSION: Vascular erosion following primary or adjuvant R(C)T represents a rare and potentially life-threatening complication requiring immediate emergency treatment involving head and neck surgeons, anesthesiologists and neuroradiologists. For patients with oropharyngeal neoplasms treated by R(C)T and showing recurrent bleeding episodes and mucosal ulceration particularly after the acute treatment phase, hospitalization with prophylactic surgical ligature or embolization of affected arteries is recommended.

Aligning Hearing Aid and Cochlear Implant Improves Hearing Outcome in Bimodal Cochlear Implant Users.

OBJECTIVE: Patients with unilateral deafness and residual hearing on the contralateral ear can benefit from a cochlear implant (CI) on one side and a hearing aid (HA) on the other. However, hearing improvement among these patients is heterogenous. Interindividual differences in bimodal benefit may be caused by a mismatch of CI and HA. The aim of this study was to clinically apply a HA fitting strategy and to evaluate hearing outcome with and without a dedicated bimodal fitting formula. STUDY DESIGN: Prospective non-randomized study. SETTING: Tertiary referral center. PATIENTS: Twelve patients using a CI processor and a conventional HA were enrolled. Before and after the new HA had been adjusted to the patient and linked to the CI, pure-tone audiometry and localization tests were performed. Speech perception was determined in quiet and noise. Tests were repeated after 6 and 12 weeks. To evaluate the subjective listening comfort two questionnaires (Oldenburg Inventory and HISQUI19) were assessed. INTERVENTION: Therapeutic. RESULTS MAIN OUTCOME MEASURE: Word recognition in quiet, sentence recognition in noise. Speech perception in noise improved significantly: directed suppression of noise helped to segregate the target speech signal from a mixture of sounds or competing speakers. Evaluation of the questionnaires revealed a positive subjective hearing experience compared with patients' initial settings of the devices. CONCLUSION: By linking CI and HA hearing and speech perception can be improved. However, good counselling at the outset is essential to obtain enhanced outcome.

Imaging studies of bacterial biofilms on cochlear implants-Bioactive glass (BAG) inhibits mature biofilm.

The capability of Pseudomonas aeruginosa and Staphylococcus aureus to form biofilm on varying CI component materials differs in the presence and absence of bioactive glass (BAG). The application of BAG induces significant changes in biofilm morphology which can be visualized via scanning electron microscopy (SEM). Bacterial biofilm formation on medical devices, such as cochlear implants (CI), can lead to chronic infections. Interestingly, BAG of type S53P4 seems to be a promising tool for use in the reduction of biofilm development. Primarily, four bacterial species known to cause implant-related infections, P.aeruginosa (ATCC9027), S. aureus (ATCC6538), Staphylococcus epidermidis (ATCC12228) and Streptococcus pyogenes (ATCC19615) were analyzed regarding their capacity to form biofilm on CI components manufactured from three kinds of material: silicone, platinum and titanium. Subsequently, P. aeruginosa and S. aureus biofilms were visualized using scanning electron microscopy, comparing BAG-treated biofilm with non-treated biofilm. The four bacterial species presented biofilm-forming capabilities in a species and surface dependent manner. Metal CI components allowed for the greatest proliferation of biofilm. S. aureus and P. aeruginosa showed the highest rate of biofilm formation on polystyrene surfaces. For both species, SEM revealed altered biofilm morphology after treatment of S53P4 BAG. This study indicates that bacterial biofilm formation and structure on CI components is dependent on the surface composition, altering between metal and silicone surfaces. After application of BAG, changes in biofilm morphology on CI components were observed. These data highlight the impact of BAG on bacterial biofilm morphology.

Impact of cochlear tonotopy on electrically evoked compound action potentials (ECAPs).

BACKGROUND: A wide range of cochlear implant electrode designs exists. Lateral wall electrodes may be favored for their potential to preserve residual hearing by virtue of being thin and delicate; whereas perimodiolar electrodes may have advantages in case of profound hearing loss, due to electrode positioning in close proximity to the auditory nerve fibers. AIM: The aim of this study was to investigate the impact of these two array designs on the interaction between electrodes and the auditory nerve in different tonotopic regions of the cochlea. PATIENTS AND METHODS: A retrospective study of both adult and pediatric cochlear implant recipients (CI24RE/CI512 or CI422, Cochlear®) was undertaken. The differences of threshold Neural Response Telemetry (tNRT) acquired 12 months after surgery were analyzed with respect to the tonotopic location. RESULTS: The results of 168 implants showed that perimodiolar arrays had lowest thresholds in the basal region whereas straight arrays had lowest thresholds in the apex. Highest thresholds for both array types were encountered in the medial parts. CONCLUSIONS AND SIGNIFICANCE: tNRTs differ depending on electrode type and location inside the cochlea. This should be considered pre implantation when choosing the electrode array type and post-implantation when mapping the CI program.

18 F-FDG PET/MRI vs MRI in patients with recurrent adenoid cystic carcinoma.

OBJECTIVES: To evaluate and compare the diagnostic potential of 18 F-fluorodeoxyglucose positron emission tomography/magnetic resonance imaging (18 FDG-PET/MRI) and MRI for recurrence diagnostics after primary therapy in patients with adenoid cystic carcinoma (ACC). METHODS: A total of 32 dedicated head and neck 18 F-FDG PET/MRI datasets were included in this analysis. MRI and 18 F-FDG PET/MRI datasets were analyzed in separate sessions by two readers for tumor recurrence or metastases. RESULTS: Lesion-based sensitivity, specificity, positive predictive value, negative predictive value and diagnostic accuracy were 96%, 84%, 90%, 93%, and 91% for 18 F-FDG PET/MRI and 77%, 94%, 95%, 73%, and 84% for MRI, resulting in a significantly higher diagnostic accuracy of 18 F-FDG PET/MRI compared to MRI (P < .005). CONCLUSION: 18 F-FDG PET/MRI is superior to MRI in detecting local recurrence and metastases in patients with ACC of the head and neck. Especially concerning its negative predictive value, 18 F-FDG PET/MRI outperforms MRI.

Bioactive Glass Granules Inhibit Mature Bacterial Biofilms on the Surfaces of Cochlear Implants.

HYPOTHESIS: Biofilm formation on cochlear implant (CI) surfaces differs between bacterial species and can be reduced by the application of S53P4 bioactive glass. BACKGROUND: The formation of bacterial biofilms on medical devices, such as cochlear implants, can lead to chronic infections resulting in the need for implant removal. In this study, various surfaces of three CI implant kits from different manufacturers were examined for bacterial biofilm formation and reduction of a pre-existing biofilm by the application of bioactive glass. METHODS: Biofilm formations of 4 bacterial species causing implant-related infections were tested on 17 different surfaces: Pseudomonas aeruginosa (ATCC9027), Staphylococcus aureus (ATCC6538), Staphylococcus epidermidis (ATCC12228), and Streptococcus pyogenes (ATCC19615). For P. aeruginosa and S. aureus biofilm reduction after application of S53P4 bioactive glass was evaluated. RESULTS: All tested microbial species formed biofilms on the examined CI surfaces in a strain-dependent manner. For S. aureus, a significantly higher biofilm formation on metal components compared with silicone was found whereas the other strains did not show a material specific biofilm formation. Application of S53P4 bioactive glass resulted in a significant reduction of P. aeruginosa and S. aureus mature biofilm. CONCLUSION: The four bacteria species displayed biofilm formation on the CI surfaces in a species- and material-specific manner. The results show that bioactive glass can reduce biofilm formation on CI materials in vitro. Future studies are necessary to confirm the results in vivo.

Cochlear Implantation in Siblings With Refsum's Disease.

OBJECTIVES: Whether the origin of severe hearing loss in Refsum's syndrome is caused by cochlear impairment or retrocochlear degeneration remains unclear. This case report aims to investigate hearing performance before and after cochlear implantation to shed light on this question. Also, identification of new mutations causing Refsum's syndrome would be helpful in generating additional means of diagnosis. METHODS: A family of 4 individuals was subjected to genetic testing. Two siblings (56 and 61 years old) suffered from severe hearing and vision loss and received bilateral cochlear implants. Genetic analysis, audiological outcome, and clinical examinations were performed. RESULTS: One new mutation in the PHYH gene (c.768del63bp) causing Refsum's disease was found. Preoperative distortion product otoacoustic emissions (DPAOEs) were absent. Postoperative speech perception in Freiburger speech test was 100% for bisyllabic words and 85% (patient No. 1) and 65% (patient No. 2), respectively, for monosyllabic words. Five years after implantation, speech perception remained stable for bisyllabic words but showed decreasing capabilities for monosyllabic words. DISCUSSION: A new mutation causing Refsum's disease is presented. Cochlear implantation in case of severe hearing loss leads to an improvement in speech perception and should be recommended for patients with Refsum's disease, especially when the hearing loss is combined with a severe loss of vision. Decrease of speech perception in the long-term follow-up could indicate an additional retrocochlear degeneration.

Radiological and NRT-Ratio-Based Estimation of Slim Straight Cochlear Implant Electrode Positions: A Multicenter Study.

OBJECTIVES: An intraoperative neural response telemetry-ratio (NRT-ratio) was established, which can provide information about the intraoperative intracochlear electrode array position for perimodiolar electrodes. METHODS: In a retrospective controlled study in 2 tertiary referral centers, the electrophysiological data sets of 50 patients with measured intraoperative auto-NRTs and postoperative radiological examinations were evaluated. All patients were implanted with Nucleus slim straight electrodes. The NRT-ratio was calculated by dividing the average auto-NRT data from electrodes 16 to 18 with the average from electrodes 5 to 7. Using a flat panel tomography system or a computed tomography, the position of the electrode array was certified radiological. RESULTS: Radiologically, 2 out of 50 patients were identified with an electrode translocated from the scala tympani into the scala vestibuli. The radiologically estimated electrodes indicating a scalar change showed a regular NRT-ratio but nonspecific NRT-level changes at the localization of translocation.

Loss of Functional Osteoprotegerin: More Than a Skeletal Problem.

Introduction: Juvenile Paget's disease (JPD), an ultra-rare, debilitating bone disease due to loss of functional osteoprotegerin (OPG), is caused by recessive mutations in TNFRFSF11B. A genotype-phenotype correlation spanning from mild to very severe forms is described. Aim: This study aimed to describe the complexity of the human phenotype of OPG deficiency in more detail and to investigate heterozygous mutation carriers for clinical signs of JPD. Patients: We investigated 3 children with JPD from families of Turkish, German, and Pakistani descent and 19 family members (14 heterozygous). Results: A new disease-causing 4 bp-duplication in exon 1 was detected in the German patient, and a microdeletion including TNFRFSF11B in the Pakistani patient. Skeletal abnormalities in all affected children included bowing deformities and fractures, contractures, short stature and skull involvement. Complex malformation of the inner ear and vestibular structures (2 patients) resulted in early deafness. Patients were found to be growth hormone deficient (2), displayed nephrocalcinosis (1), and gross motor (3) and mental (1) retardation. Heterozygous family members displayed low OPG levels (12), elevated bone turnover markers (7), and osteopenia (6). Short stature (1), visual impairment (2), and hearing impairment (1) were also present. Conclusion: Diminished OPG levels cause complex changes affecting multiple organ systems, including pituitary function, in children with JPD and may cause osteopenia in heterozygous family members. Diagnostic and therapeutic measures should aim to address the complex phenotype.

Long-term outcome of vertigo and dizziness associated disorders following treatment in specialized tertiary care: the Dizziness and Vertigo Registry (DiVeR) Study.

To investigate the long-term outcome of interdisciplinary treatment in a tertiary care neuro-otology institution after 2 years as part of the Dizziness and Vertigo Registry study. Risk factors associated with unfavourable outcome were assessed. 3113 consecutive patients with disorders of vertigo and dizziness were recruited prospectively between March 2010 and February 2012. Patients were clinically assessed and treated according to their diagnosis. Standardized instruments were used at baseline and at 2-year follow-up [Dizziness Handicap Inventory (DHI), Quality of Life Questionnaire, General Depression Scale, Stait-Trait Anxiety Index], as well as a custom health-related questionnaire. The primary outcome variable of this observational study was the change in DHI after 2 years. Patients suffered from phobic postural vertigo (23%), benign peripheral paroxysmal vertigo (14.4%), unilateral vestibulopathy (10.5%), central vestibular disorders (8%), Menière's disease (9.8%), vestibular migraine (6.9%), bilateral vestibulopathy (5.5%), and vestibular paroxysmia (3.1%). Mean disease duration was 4.6 ± 6.3 years. 1272 patients were available for follow-up, 1159 completed the DHI score. 72.1% of patients improved in DHI score from baseline to 2 years follow-up. Mean reduction in DHI score was 14 points (p = 0.02). Long-term outcome following diagnosis and treatment in a specialized tertiary care centre is good and persistent after 2 years. Risk factors for an unfavourable outcome were advanced age, severe disability, constant vertigo or dizziness, and concomitant back pain, while depression and anxiety did not contribute to this risk considerably.

Influence of etiologic factors on speech perception of cochlear-implanted children.

OBJECTIVE: Rehabilitation success in cochlear implant patients is influenced by many factors. Influence of different etiologies of deafness on rehabilitation outcome is assessed. STUDY DESIGN: Retrospective survey of patients. SETTING: University hospital. Secondary referral center. PATIENTS: One hundred and sixty-three between 1996 and 2008 implanted children (0-18 years, mean 5.17 years). INTERVENTIONS: Evaluation of patients' data: origin of deafness, hearing and speech test results. Access Data Base; Wilcoxon and t-test. RESULTS: Mean follow-up: 65.4 months. Etiology of deafness in children found in 104 cases: 69 (51.9%) suffered from hereditary hearing loss. All children showed improvement in their auditory performance, children with connexin-26 mutation performed significantly best, Usher and CHARGE-syndrome children significantly worst. Post-meningitic and post-septic children developed slower but reached same levels later. DISCUSSION: Primary cause of deafness, among other factors, has a considerable impact on outcome of rehabilitation. This offers possibilities to influence the outcome by etiology-adjusted therapy modules.