Our goal was to determine the optimal orientation of insertion of the Slim Modiolar electrode and develop an easy-to-use method to aid implantation surgery. In some instances, the electrode arrays cannot be inserted in their full length. This can lead to buckling, interscalar dislocation or tip fold-over. In our opinion, one of the possible reasons of tip fold-over is unfavourable orientation of the electrode array. Our goal was to determine the optimal orientation of the Slim Modiolar electrode array relative to clear surgical landmarks and present our method in one specified case. For the measurement, we used the preoperative CT scan of one of our cochlear implant patients. These images were processed by an open source and free image visualization software: 3D Slicer. In the first step we marked the tip of the incus short process and then created the cochlear view. On this view we drew two straight lines: the first line represented the insertion guide of the cochlear implant and the second line was the orientation marker (winglet). We determined the angle enclosed by winglet and the line between the tip of the incus short process and the cross-section of previously created two lines. For the calculation we used a self-made python code. The result of our algorithm for the angle was 46.6055°. To validate this result, we segmented, from the CT scan, the auditory ossicles and the membranaceous labyrinth. From this segmentation we generated a 3D reconstruction. On the 3D view, we can see the position of the previous lines relative to the anatomical structures. After this we rotated the 3D model together with the lines so that the insertion guide forms a dot. In this view, the angle was measured with ImageJ and the result was 46.599°. We found that our method is easy, fast, and time-efficient. The surgery can be planned individually for each patient, based on their routine preoperative CT scan of the temporal bone, and the implantation procedure can be made safer. In the future we plan to use this method for all cochlear implantation surgeries, where the Slim Modiolar electrode is used.

[A case of a 61-year-old male patient suffered chronic renal failure and dialysed for 23 years with destructive cervical spondylarthropathy is presented. The patient presented with sudden onset of cervical pain radiating into his shoulders without neurological deficits. CT and MRI of the cervical and thoracic spine revealed severe destructive changes and compressive fractures of C6 and C7 vertebrae which caused the narrowing of the nerve root canals at these levels. A 360-degree fixation was performed to treat the unstable fracture and the patient’s pain (C6 and C7 corpectomy, autolog bone graft replacement of the two vertebral bodies, anterior plate fixation and posterior instrumentation with screws and rods). Postoperatively the patient had no significant pain, no neurological deficit and he was able to manage independent life himself. During the immediate follow-up CT of the neck showed the satisfactory position of the bone graft and the metal implantations. The 6 months follow-up CT revealed the anterior migration of the two screws from the Th1 vertebral body and 2 mm ventral elevation of the caudal end of the plate from the anterior surface of the Th1 vertebral body. The 1-year follow-up could not be performed because the patient died due to cardio-pulmonary insufficiency. This is the second Hungarian report of a chronic dialysis related severe spondylarthropathy which may cause pathologic fractures of the vertebral bodies. The typical radiological and histological findings are discussed. This disease affect patients’ quality of life and the conservative treatment alone seems to be ineffective in most cases. Based on the literature and personal experiences, the authors suggest 360-degree fixation of the spine to provide sufficient stability for the vertebrae of ”bad bone quality”, and early mobilisation of the patient can be achieved.]

Background - Several cochlear implant recipients experience functionality loss due to electrode array mal-positioning. The application of delicate perimodiolar electrodes has many electrophysiological advantages, however, these profiles may be more susceptible to tip fold-over. Purpose - The prompt realization of such complication following electrode insertion would be auspicious, thus the electrode could be possibly repositioned during the same surgical procedure. Methods - The authors present three tip fold-over cases, experienced throughout their work with Slim Modiolar Electrode implants. Implantations were performed through the round window approach, by a skilled surgeon. Standard intraoperative measurements (electric integrity, neural response telemetry, and electrical stapedial reflex threshold tests) were successfully completed. The electrode position was controlled by conventional radiography on the first postoperative day. Results - Tip fold-over was not tactilely sensated by the surgeon. Our subjects revealed normal intraoperative telemetry measurements, only the postoperative imaging showed the tip fold-over. Due to the emerging adverse perception of constant beeping noise, the device was replaced by a CI512 implant after 6 months in one case. In the two remaining cases, the electrode array was reloaded into a back-up sheath, and reinserted into the scala tympani successfully through an extended round window approach. Discussion - Future additional studies using the spread of excitation or electric field imaging may improve test reliability. As all of these measurements are still carried out following electrode insertion, real-time identification, unfortunately, remains questionable. Conclusion - Tip fold-over could be reliably identified by conventional X-ray imaging. By contrast, intraoperative electrophysiology was not sufficiently sensitive to reveal it.

[Primary aldosteronism is a frequent cause of secondary hypertension requiring a specific pharmacological treatment with mineralocorticoid receptor antagonist or with unilateral adrenalectomy. These treatments have shown to reduce the excess of cardiovascular risk characteristically associated with this disease. In this consensus, we discussed the procedures for the diagnosis of primary aldosteronism, we address the strategies for the differential diagnosis of primary aldosteronism subtypes and therapy. We also discuss the evaluation of outcomes and provide suggestions for follow-up as well as cardiovascular and metabolic complications specifically associated with primary aldosteronism. ]

[Pompe disease (PD) is a rare lysosomal disease caused by the deficient activity of acid alpha-glucosidase (GAA) enzyme due to mutations in the GAA gene. The enzymatic deficiency leads to the accumulation of glycogen within the lysosomes. Clinically, the disease has been classically classified in infantile and childhood/adult forms. Presently cc. close to 600 mutations distributed throughout the whole gene have been reported. The c.-32-13T>G splice mutation that is very common in patients of Caucasian origin affected by the childhood/adult form of the disease, with an allelic frequency close to 70%. Enzyme replacement treatment (ERT) is available for the patients with Pompe disease (Myozyme). In this paper, we are presenting the long term follow up of 13 adult onset cases treated more than 5 years. The longest follow up was 15 years. To evaluate the treatment efficacy, the 6 minutes walking test (6MWT) and the respiratory functions were monitored annually. The analysis revealed that at the beginning of ERT for 3-4 years the 6MWT had been generally increasing, then it declined, and after 10 years it was lower in 77% of the cases than it had been at the start of the treatment. In 23% of the cases the 6MWT increased during the follow up time. Only one of the patients become wheelchair dependent during the follow-up period. The respiratory function showed similar results especially in supine position. A high degree of variability was observed among patients in their responses to the treatment, which only partially associated with the antibody titer against the therapeutic protein. The efficacy of the ERT was associated with the type of the disease causing mutation, the baseline status of the disease, the lifestyle and the diet of the patient. The long-term follow up of the patients with innovative orphan drugs is necessary to really understand the value of the treatment and the need of the patients.]