The value of moderate dose escalation for re-irradiation of recurrent or second primary head-and-neck cancer.

BACKGROUND: Treatment for local and locoregional recurrence or second head-and-neck (H&N) cancers after previous radiotherapy is challenging, and re-irradiation carries a significantly increased risk for radiotherapy-related normal tissue toxicities and treatment failure due to a radioresistant tumor phenotype. Here, we analyzed re-irradiation management and outcomes in patients with recurrent or second primary H&N carcinoma using state-of-the-art diagnostic procedures and radiotherapy techniques. METHODS: Between 2010 and 2019, 48 patients with recurrent or second primary H&N carcinoma received re-radiotherapy at the University of Freiburg Medical Center and were included in this study. Overall survival (OS) and progression-free survival (PFS) were calculated with the Kaplan-Meier method, and univariate Cox-regression analyses were performed to assess the effects of clinico-pathological factors on treatment outcomes. Acute and chronic treatment-related toxicities were quantified using the Common Terminology Criteria for Adverse Events (CTCAE v4.03). RESULTS: Thirty-one patients (64.6%) received definitive and 17 (35.4%) adjuvant radiotherapy. Simultaneous chemotherapy was administered in 28 patients (58.3%) with cetuximab as the most commonly used systemic agent (n = 17, 60.7%). After a median time of 17 months (range 4 months to 176 months) between first and second radiotherapy, patients were re-irradiated with a median of 58.4 Gy and a treatment completion rate of 87.5% (n = 42). Median OS was 25 months with a 1-year OS amounting to 62.4%, and median PFS was 9 months with a 1-year PFS of 37.6%. Univariate analyses demonstrated that both a lower rT-status and a radiotherapy boost were associated with improved OS (p < 0.05). There was a trend towards superior OS for patients who received > 50 Gy (p = 0.091) and who completed the prescribed radiotherapy (p = 0.055). Five patients (10.4%) suffered from at least one grade 3 toxicities, while 9 patients (27.3%) experienced chronic higher-grade toxicities (≥ grade 3) with one (3.0%) grade 4 carotid blowout and one (3.0%) grade 4 osteoradionecrosis. CONCLUSION: Re-irradiation of recurrent or second primary H&N cancer with modern radiation techniques such as intensity-modulated radiotherapy resulted in promising survival rates with acceptable toxicities compared to historical cohorts. Increased re-irradiation doses, utilization of a radiotherapy boost and completion of the re-irradiation treatment were found to result in improved survival.

Radiotherapeutic management of cervical lymph node metastases from an unknown primary site - experiences from a large cohort treated with modern radiation techniques.

PURPOSE: To analyze management and outcomes following (chemo)radiation therapy in patients with cervical lymph node metastases from an unknown primary site (CCUP) in a large single-center cohort. METHODS: Between 2008 and 2019, 58 patients with CCUP were treated with (chemo)radiation therapy at the University of Freiburg Medical Center and were included in this analysis. Overall survival (OS), locoregional progression-free survival (PFS) and distant metastasis-free survival (DMFS) were calculated using the Kaplan-Meier method. The use of diagnostic procedures and their impact on oncological outcomes was analyzed by Cox regression, and treatment-related toxicities were quantified. RESULTS: Median follow-up was 29.9 months (range 4.6-121.9). Twenty-one patients (36.2%) received definitive RT, 35 (60.3%) underwent adjuvant RT, and 2 (3.4%) were treated for oligometastatic disease. Concurrent chemotherapy was prescribed in 40 patients (69.0%). 89.6% of patients completed the prescribed RT, and 65.0% completed the prescribed simultaneous chemotherapy. Locoregional recurrence was observed in 7 patients (12.1%) and distant metastases in 13 cases (22.4%). OS was 81,1, 64.9% and 56,6% after 1, 3 and 5 years, respectively. Univariate analysis of age, gender, extracapsular spread, tumor grading, neck dissection, diagnostic utilization of 18F-fluorodeoxyglucose positron-emission tomography and concomitant chemotherapy showed no effect on OS (p > 0.05 for all), while smoking was significantly associated with decreased survival (p < 0.05). There was a trend towards impaired OS for patients with advanced nodal status (pN3) (p = 0.07). Three patients (5.2%) experienced grade 3 radiation dermatitis, and 12 (22.4%) developed grade 3 and 1 (1.7%) grade 4 mucositis. CONCLUSIONS: RT of the panpharynx and cervical lymph nodes with concurrent chemotherapy in case of risk factors demonstrated good locoregional control, but the metachronous occurrence of distant metastases limited survival and must be further addressed.

Speech Recognition in Noise in Single-Sided Deaf Cochlear Implant Recipients Using Digital Remote Wireless Microphone Technology.

BACKGROUND: Previous research in cochlear implant (CI) recipients with bilateral severe-to-profound sensorineural hearing loss showed improvements in speech recognition in noise using remote wireless microphone systems. However, to our knowledge, no previous studies have addressed the benefit of these systems in CI recipients with single-sided deafness. PURPOSE: The objective of this study was to evaluate the potential improvement in speech recognition in noise for distant speakers in single-sided deaf (SSD) CI recipients obtained using the digital remote wireless microphone system, Roger. In addition, we evaluated the potential benefit in normal hearing (NH) participants gained by applying this system. RESEARCH DESIGN: Speech recognition in noise for a distant speaker in different conditions with and without Roger was evaluated with a two-way repeated-measures design in each group, SSD CI recipients, and NH participants. Post hoc analyses were conducted using pairwise comparison t-tests with Bonferroni correction. STUDY SAMPLE: Eleven adult SSD participants aided with CIs and eleven adult NH participants were included in this study. DATA COLLECTION AND ANALYSIS: All participants were assessed in 15 test conditions (5 listening conditions × 3 noise levels) each. The listening conditions for SSD CI recipients included the following: (I) only NH ear and CI turned off, (II) NH ear and CI (turned on), (III) NH ear and CI with Roger 14, (IV) NH ear with Roger Focus and CI, and (V) NH ear with Roger Focus and CI with Roger 14. For the NH participants, five corresponding listening conditions were chosen: (I) only better ear and weaker ear masked, (II) both ears, (III) better ear and weaker ear with Roger Focus, (IV) better ear with Roger Focus and weaker ear, and (V) both ears with Roger Focus. The speech level was fixed at 65 dB(A) at 1 meter from the speech-presenting loudspeaker, yielding a speech level of 56.5 dB(A) at the recipient's head. Noise levels were 55, 65, and 75 dB(A). Digitally altered noise recorded in school classrooms was used as competing noise. Speech recognition was measured in percent correct using the Oldenburg sentence test. RESULTS: In SSD CI recipients, a significant improvement in speech recognition was found for all listening conditions with Roger (III, IV, and V) versus all no-Roger conditions (I and II) at the higher noise levels (65 and 75 dB[A]). NH participants significantly benefited from the application of Roger in noise for higher levels, too. In both groups, no significant difference was detected between any of the different listening conditions at 55 dB(A) competing noise. There was also no significant difference between any of the Roger conditions III, IV, and V across all noise levels. CONCLUSIONS: The application of the advanced remote wireless microphone system, Roger, in SSD CI recipients provided significant benefits in speech recognition for distant speakers at higher noise levels. In NH participants, the application of Roger also produced a significant benefit in speech recognition in noise.

Speech Perception in Quiet and Noise With an Off the Ear CI Processor Enabling Adaptive Microphone Directionality.

OBJECTIVE: To investigate the impact of the wearing position of an off-the-ear-processor (OTE) on speech perception in quiet and noise. PATIENTS: The study group consisted of 16 adult subjects with bilateral severe-to-profound sensorineural hearing loss, 2 of them unilaterally, and 14 bilaterally provided with cochlear implants. MAIN OUTCOME MEASURES: Speech perception in quiet and noise was measured for frontal presentation with the recipients behind-the-ear processor CP810 or CP910 and the OTE processor Kanso (Cochlear Limited, Sydney, Australia). Additionally, speech performance in noise with the OTE for spatially separated signal and noise sources was assessed. RESULTS: The recipients showed monosyllabic word recognition scores in quiet between 65 and 95% and speech reception thresholds in noise between 2.4 and -5.5 dB SNR with the OTE. For frontal presentation of speech and noise, application of the adaptive directional microphone (Beam) yielded a slight median decrement of 0.6 dB for the speech reception threshold compared with standard directionality. However, huge median improvements, ranging from -3.7 to -11.6 dB, for the three tested conditions with spatially separated sources (S0NIL, S0NCL, S0N180) were observed. CONCLUSION: The beamforming algorithm in the investigated OTE processor provides similar benefits as described in previous studies for behind-the-ear processors in conditions with spatially separated speech and noise sources. Adaptive microphone directionality can be successfully implemented in an OTE processor. The OTE processor's potential to increase usability, comfort, and cosmetics might not be compromised by a deterioration of speech performance.

Experimental Simulation of Clinical Borderline Situations in Temporal Bone Specimens After Ossiculoplasty.

OBJECTIVES: One reason for insufficient hearing improvement with a distinct air-bone gap after ossiculoplasty with implantation of partial or total ossicular replacement prostheses can be the dislocation or minimal shifting of the prosthesis. The aim of this study was the simulation of common clinical borderline situations with minimal shifting of the prosthesis in temporal bone specimens after ossiculoplasty. It was furthermore the goal to identify these specific situations through imaging by cone beam computed tomography (cbCT) and direct visual inspection using the operation microscope. Additionally, the functional status was evaluated using laser-Doppler vibrometry (LDV). DESIGN: We used a total of four temporal bone specimens for this study. A reconstruction with a partial ossicular replacement prostheses was performed in three specimens and with a total ossicular replacement prostheses in one specimen, with good initial acoustic properties. Subsequently, one specific type of prosthesis failure was simulated in each specimen, respectively, by minimally shifting, tilting, or bending the prostheses from their initial positions. These changes were introduced step-by-step until a borderline situation just short of complete acoustic decoupling was reached. Each step was examined using both LDV and cbCT and observed through the operation microscope. RESULTS: LDV was able to quantify the mechanic function of the ossicular chain after most of the manipulation steps by demonstrating the effect of any shifting of the prosthesis on the middle ear transfer function. However, in some situations, the middle ear transfer function was better with a visually more advanced failure of the prosthesis. In addition, cbCT showed most of the steps with excellent resolution and was able to delineate changes in soft tissue (e.g., cartilage covering). CONCLUSION: cbCT seems to be a promising imaging technique for middle ear problems. As cbCT and LDV exhibited slightly different advantages and disadvantages regarding the demonstration of borderline situations, the combination of both techniques allowed for a more precise evaluation of middle ear reconstructions. Knowledge of the specific characteristics of these methods and their possible combination might help otologists and otosurgeons to refine indications for revision surgery and improve their personal patient counseling.

Navigation accuracy after automatic- and hybrid-surface registration in sinus and skull base surgery.

OBJECTIVE: Computer-aided-surgery in ENT surgery is mainly used for sinus surgery but navigation accuracy still reaches its limits for skull base procedures. Knowledge of navigation accuracy in distinct anatomical regions is therefore mandatory. This study examined whether navigation accuracy can be improved in specific anatomical localizations by using hybrid registration technique. STUDY DESIGN: Experimental phantom study. SETTING: Operating room. SUBJECTS AND METHODS: The gold standard of screw registration was compared with automatic LED-mask-registration alone, and in combination with additional surface matching. 3D-printer-based skull models with individual fabricated silicone skin were used for the experiments. Overall navigation accuracy considering 26 target fiducials distributed over each skull was measured as well as the accuracy on selected anatomic localizations. RESULTS: Overall navigation accuracy was <1.0 mm in all cases, showing the significantly lowest values after screw registration (0.66 ± 0.08 mm), followed by hybrid registration (0.83± 0.08 mm), and sole mask registration (0.92 ± 0.13 mm).On selected anatomic localizations screw registration was significantly superior on the sphenoid sinus and on the internal auditory canal. However, mask registration showed significantly better accuracy results on the midface. Navigation accuracy on skull base localizations could be significantly improved by the combination of mask registration and additional surface matching. CONCLUSION: Overall navigation accuracy gives no sufficient information regarding navigation accuracy in a distinct anatomic area. The non-invasive LED-mask-registration proved to be an alternative in clinical routine showing best accuracy results on the midface. For challenging skull base procedures a hybrid registration technique is recommendable which improves navigation accuracy significantly in this operating field. Invasive registration procedures are reserved for selected challenging skull base operations where the required high precision warrants the invasiveness.

Perception of Interaural Phase Differences With Envelope and Fine Structure Coding Strategies in Bilateral Cochlear Implant Users.

The ability to detect a target signal masked by noise is improved in normal-hearing listeners when interaural phase differences (IPDs) between the ear signals exist either in the masker or in the signal. To improve binaural hearing in bilaterally implanted cochlear implant (BiCI) users, a coding strategy providing the best possible access to IPD is highly desirable. In this study, we compared two coding strategies in BiCI users provided with CI systems from MED-EL (Innsbruck, Austria). The CI systems were bilaterally programmed either with the fine structure processing strategy FS4 or with the constant rate strategy high definition continuous interleaved sampling (HDCIS). Familiarization periods between 6 and 12 weeks were considered. The effect of IPD was measured in two types of experiments: (a) IPD detection thresholds with tonal signals addressing mainly one apical interaural electrode pair and (b) with speech in noise in terms of binaural speech intelligibility level differences (BILD) addressing multiple electrodes bilaterally. The results in (a) showed improved IPD detection thresholds with FS4 compared with HDCIS in four out of the seven BiCI users. In contrast, 12 BiCI users in (b) showed similar BILD with FS4 (0.6 ± 1.9 dB) and HDCIS (0.5 ± 2.0 dB). However, no correlation between results in (a) and (b) both obtained with FS4 was found. In conclusion, the degree of IPD sensitivity determined on an apical interaural electrode pair was not an indicator for BILD based on bilateral multielectrode stimulation.

Prospects and limitations of different registration modalities in electromagnetic ENT navigation.

The present study examined electromagnetic tracking technology for ENT navigation. Five different registration modalities were compared and navigation accuracy was assessed. Four skull models were individually fabricated with a three-dimensional printer, based on patients' computer tomography datasets. Individual silicone masks were fitted for skin and soft tissue simulation. Five registration modalities were examined: (1) invasive marker, (2) automatic, (3) surface matching (AccuMatch), (4) anatomic landmarks, and (5) oral splint registration. Overall navigation accuracy and accuracy on selected anatomic locations were assessed by targeting 26 titanium screws previously placed over the skull. Overall navigation accuracy differed significantly between all registration modalities. The target registration error was 0.94 ± 0.06 mm (quadratic mean ± standard deviation) for the invasive marker registration, 1.41 ± 0.04 mm for the automatic registration, 1.59 ± 0.14 mm for the surface matching registration, and 5.15 ± 0.66 mm (four landmarks) and 4.37 ± 0.73 mm (five landmarks) for the anatomic landmark registration. Oral splint registration proved itself to be inapplicable to this navigation system. Invasive marker registration was superior on most selected anatomic locations. However, on the ethmoid and sphenoid sinus the automatic registration process revealed significantly lower target registration error values. Only automatic and surface registration met the accuracy requirements for noninvasive registration. Particularly, the automatic image-to-world registration reaches target registration error values on the anterior skull base which are comparable with the gold standard of invasive screw registration.

Cochlear implantation for hearing rehabilitation in single-sided deafness after translabyrinthine vestibular schwannoma surgery.

The aim of the study was to investigate the option of cochlear implantation (CI) in resultant single-sided deafness associated with unilateral translabyrinthine resection of sporadic vestibular schwannoma (VS). This is a retrospective study performed at Tertiary Care Academic Centre. Following extensive counselling regarding the potential for delayed CI, translabyrinthine VS resection was performed and an intracochlear placeholder was inserted to allow later CI in 11 patients who showed intraoperative microscopic confirmation of preserved cochlear nerve anatomy. Follow-up magnetic resonance imaging (MRI) and promontory testing were performed 1 year after surgery to confirm the absence of VS recurrence and viable cochlea. Confirmed CI candidates underwent a second procedure where the placeholder was removed and the CI inserted (4/11). Preimplant unaided and CI-aided evaluations at 12 and 24 months were performed for subjective and objective hearing outcomes. Tinnitus suppression was also measured for implant on and off effects. Available audiological data for three patients demonstrated significant hearing benefits for 'speech from deaf/implanted side, noise from the normal-hearing side' in all three patients and localisation ability improved for 2/3 patients. Subjective findings presented similar results. For the two patients with preimplant tinnitus, complete suppression occurred during active CI. CI is beneficial for hearing rehabilitation and tinnitus reduction in SSD patients with remaining viable cochlear nerve after translabyrinthine VS surgery. Counselling on the risks of intracochlear placeholder insertion and the inherent limitations for ongoing MRI investigations of VS recurrence is essential.

Cochlear implantation in children with single-sided deafness: does aetiology and duration of deafness matter?

For adult patients with single-sided deafness (SSD), treatment with a cochlear implant (CI) is well established as an acceptable and beneficial hearing rehabilitation method administered routinely in clinical practice. In contrast, for children with SSD, CI has been applied less often to date, with the rationale to decide either on a case-by-case basis or under the realm of clinical research. The aim of our clinical study was to evaluate the longitudinal benefits of CI for a group of children diagnosed with SSD and to compare their outcomes with respect to patient characteristics. Evaluating a pool of paediatric SSD patients presenting for possible CI surgery revealed that the primary aetiology of deafness was congenital cochlear nerve deficiency. A subgroup of children meeting the CI candidacy criteria for the affected ear (the majority with acquired hearing loss) were enrolled in the study. Preliminary group results suggest substantial improvements in speech comprehension in noise and in the ability to localise sound, which was demonstrated through objective and subjective assessments after CI treatment for the group, with results varying from patient to patient. Our study shows a trend towards superior outcomes for children with acquired hearing loss and a shorter duration of hearing loss compared to congenitally deafened children who had a longer duration of SSD. This indicates an interactive influence of the age at onset, aetiology and duration of deafness upon the restoration of binaural integration and the overall benefits of sound stimulation to two ears after CI treatment. Continued longitudinal investigation of these children and further studies in larger groups may provide more guidance on the optimal timing of treatment for paediatric patients with acquired and congenital SSD.

Binaural integration of periodically alternating speech following cochlear implantation in subjects with profound sensorineural unilateral hearing loss.

In cochlear implant (CI) recipients with unilateral hearing loss (UHL) and normal hearing (NH) in the contralateral ear, the central auditory system receives signals of different auditory modalities, i.e. electrically via the CI ear as well as acoustically via the NH ear. The present study investigates binaural integration of bimodal stimulation in the central auditory system of 10 CI subjects with UHL by applying a modified version of the Rapidly Alternating Speech Perception (RASP) test to characterise speech recognition ability under monotic and dichotic listening arrangements. Subsequently, the results for each monotic and dichotic test condition were compared to quantify the binaural benefit from CI usage. The study results demonstrate significantly improved speech recognition under dichotic compared to monotic listening conditions, providing evidence that there is binaural integration of acoustically and electrically transmitted speech segments in the central nervous system at brainstem and cortical levels. In contrast to more commonly used tests of binaural integration, such as localisation, the RASP test provides the clinical option to investigate binaural integration involving structures at the cortical level.

Radiologic Results and Hearing Preservation With a Straight Narrow Electrode via Round Window Versus Cochleostomy Approach at Initial Activation.

OBJECTIVE: To evaluate the potential influence of two different cochlear implant (CI) electrode carrier approaches to the scala tympani in terms of insertion depth and angle as well as hearing preservation. STUDY DESIGN: Retrospective follow-up study. SETTING: Tertiary care academic center. PATIENTS: Forty-one adult subjects underwent cochlear implantation using the Cochlear Nucleus CI 422 slim straight electrode (Cochlear Limited, New South Wales, Australia) with the intent of achieving atraumatic electrode insertion and hearing preservation. INTERVENTIONS: Fourteen subjects were implanted using a cochleostomy approach and 27 via a round window approach. MAIN OUTCOME MEASURES: Radiologic assessment of insertion depth and angle was performed on postoperative rotational tomography. Postoperative low-frequency hearing preservation, complete loss of residual low-frequency hearing, and speech perception in quiet measures were obtained in defined intervals for both groups. RESULTS: Mean insertion depth reached 21.5 mm (standard deviation, 1.1), whereas mean insertion angle of 388 degrees (standard deviation, 34.7) was revealed for all CI recipients, with no significant difference between the cochleostomy cohort and the round window cohort. The CI-aided speech perception in quiet showed significantly higher scores 3 to 4 months after activation compared with the preoperative speech perception results. The audiologic data demonstrated no statistically significant difference in probability of complete loss of residual low-frequency hearing at initial activation between the cochleostomy group and the round window group. CONCLUSION: The present results suggest that similar insertion depth and angle as well as similar low-frequency hearing preservation can be achieved in cochlear implantation with this straight narrow electrode via either cochleostomy approach or round window approach at least at initial activation. This fact gives the surgeon the opportunity to adapt access to the scala tympani as needed.

Comparison of 3D C-arm-based registration to conventional pair-point registration regarding navigation accuracy in ENT surgery.

OBJECTIVE: Navigation surgery on the skull base requires high navigation accuracy. The registration process is related to the main loss in accuracy. This study compared titanium screw registration with an inbuilt registration process of a 3-dimensional (3D) C-arm. STUDY DESIGN: Experimental phantom study. SETTING: Operating room. SUBJECTS AND METHODS: Four skull models were fabricated with a 3D printer based on the patient's computed tomography (CT) data sets and fitted with an individually customized silicone skin. A 3D-isocentric C-arm fluoroscopic image intensifier system combined with a flat panel detector performed scans of petrous bones (PB) and paranasal sinuses (PS). The navigation accuracy of pair-point registration (PPR) with titanium screws was compared with C-arm-based registration. RESULTS: Overall navigation accuracy was 1.53 ± 0.51 mm after PPR and 1.26 ± 0.12 mm after C-arm registration (P = .0259). PPR showed the best accuracy results on PS (1.28 ± 0.69 mm), followed by right PB (1.43 ± 0.52 mm) and left PB (1.74 ± 0.69 mm). A significant difference was seen only between PS and left PB (P = .0206). In contrast, C-arm registration revealed significantly lower target registration errors (TREs) on PB (0.99 ± 0.23 mm right PB, P < .0001; 1.2 ± 0.35 mm left PB, P = .0412) compared with PS. When comparing both registration modalities, C-arm registration was significantly superior on PB. With respect to specific anatomic locations, C-arm-based registration showed significantly lower TREs on the frontal and lateral skull base than PPR. CONCLUSION: C-arm-based navigation shows higher navigation accuracy on the skull base compared with PPR. As the 3D C-arm allows real-time imaging and real-time navigation, it will be a helpful tool for skull base surgeons.

The new mid-scala electrode array: a radiologic and histologic study in human temporal bones.

HYPOTHESIS: To analyze the quality of insertion of the newly developed midscala (MS) electrode, which targets a midscalar electrode position to reduce the risk of trauma to the lateral wall and the modiolus. BACKGROUND: Modern cochlear implant surgery aims for a safe intracochlear placement of electrode arrays with an ongoing debate regarding cochleostomy or round window (RW) insertion and the use of lateral wall or perimodiolar electrode placement. Intracochlear trauma after insertion of different electrodes depends on insertion mode and electrode design and may result in trauma to the delicate structures of the cochlear. METHODS: We performed a temporal bone (TB) trial with insertion of the MS electrode in n = 20 TB's after a mastoidectomy and posterior tympanotomy. Insertion was performed either via the RW or a cochleostomy. Electrode positioning, length of insertion, and angle of insertion were analyzed with rotational tomography (RT). TBs were histologically analyzed. Results of RT and histology were compared. RESULTS: Scala tympani (ST) insertion could be accomplished reliably by both RW and via a cochleostomy approach. In 20 TBs, 1 scala vestibuli insertion, 1 incomplete (ST), and 1 elevation of basilar membrane were depicted. No trauma was found in 94.7% of all ST insertions. RT allowed determination of the intracochlear electrode position, which was specified by histologic sectioning. CONCLUSION: The new MS electrode seems to fulfill reliable atraumatic intracochlear placement via RW and cochleostomy approaches. RT is available for evaluation of intracochlear electrode position, serving as a potential quality control instrument in human implantation.

A novel homozygous mutation in the EC1/EC2 interaction domain of the gap junction complex connexon 26 leads to profound hearing impairment.

To date, about 165 genetic loci or genes have been identified which are associated with nonsyndromal hearing impairment. In about half the cases, genetic defects in the GJB2 gene (connexin 26) are the most common cause of inner-ear deafness. The genes GJB2 and GJB6 are localized on chromosome 13q11-12 in tandem orientation. Connexins belong to the group of "gap junction" proteins, which form connexons, each consisting of six connexin molecules. These are responsible for the exchange of ions and smaller molecules between neighboring cells. Mutational analysis in genes GJB2 and GJB6 was brought by direct sequencing of the coding exons including the intron transitions. Here we show in the participating extended family a homozygous mutation c.506G>A, (TGC>TAC) p.Cys169Tyr, in the GJB2 gene, which could be proven for the first time and led to nonsyndromal severe hearing impairment in the afflicted patients. The mutation is located in the EC1/EC2 interaction complex of the gap junction connexon 26 complex and interrupts the K(+) circulation and therefore the ion homeostasis in the inner ear. The homozygous mutation p.Cys169Tyr identified here provides a novel insight into the structure-function relationship of the gap junction complex connexin/connexon 26.

Revision surgery due to magnet dislocation in cochlear implant patients: an emerging complication.

OBJECTIVES: To analyze the cause and effect of magnet dislocation in cochlear implant (CI) recipients requiring magnet revision surgery for treatment. STUDY DESIGN: Retrospective study. SETTING: Tertiary referral center. INTERVENTIONS: Case reports from 1,706 CI recipients consecutively implanted from January 2000 to December 2011 were reviewed. The number of cases requiring magnet revision surgery was assessed. RESULTS: Revision surgery involving magnet removal or replacement was indicated in 1.23% (21/1,706), of all CI recipients. Magnet dislocation occurring during magnetic resonance tomography (MRI), at 1.5 Tesla (T), with the magnet in place and with the application of compression bandaging around the head, was the main cause for revision surgery in 47.62% (10/21) of the affected cases. All 10 cases were implanted with Cochlear Nucleus cochlear implants. These events occurred, despite adherence to current recommendations of the manufacturer. CONCLUSION: The present study underlines that MRI examination is the main cause of magnet dislocation. The use of compressive bandaging when using 1.5-T MRI does not eliminate the risk of magnet dislocation. Additional cautionary measures are for required for conditional MRI. We recommend X-ray examination after MRI to determine magnet dislocation and avoid major complications in all cases reporting pain during or after MRI. Additional research regarding silicon magnet pocket design for added retention is needed. Effective communication of guidelines for precautionary measures during MRI examination in CI patients is mandatory for all clinicians involved. MRI in CI recipients should be indicated with caution.