Search Results (6)

Hearing loss can result from impairments in structures that support endocochlear potential, as they play a crucial role in the transduction and transmission of auditory waves. This aspect has been the subject of several studies to date. In our review, the role of ion transport channels and pumps involved in hearing function has been highlighted, emphasizing how important the Kir4.1 channel is in maintaining the endocochlear potential. The Kir4.1 channel, a member of the inwardly rectifying potassium channel (Kir) family, plays a key role in the regulation of cell electrical activity and potassium ion homeostasis. The cochlear expression of these channels is at the level of the intermediate cells of the vascular stria, in the root cells of the outer sulcus, and in the glial cells of the spiral ganglion. In development, its expression demonstrates its involvement in the progression of pathologies related to potassium channel dysfunction, and its activation in the stria vascularis is directly related to the generation of endocochlear potential. Kir4.1 is fundamental in stabilizing the resting membrane potential of cells and modulating their excitability, as it facilitates a greater influx of potassium into cells compared to efflux when the membrane potential is negative. Mutations in the K⁺ channel gene KCNJ10 (Kir4.1) have been associated with several disorders, with the most significant studies on EAST/SeSAME syndrome and Pendred syndrome. Recent research has explored the metabolic importance of potassium channel changes associated with stria vascularis degeneration in the progression of age-related hearing loss. Furthermore, in ototoxicity studies, the Kir4.1 channel has been shown to have the ability to compensate for the deficiency of other K⁺ channels, as it maintains the cochlear homeostasis by correcting the imbalanced K⁺ concentration. Full article

Background: Spontaneous orbital cephaloceles are a rare condition. The purpose of this study is to provide a description of a clinical case and to carry out a systematic literature review. Methods: A systematic review of the English literature published on the Pubmed, Scopus, and Web of Science databases was conducted, according to the PRISMA recommendations. Results: A 6-year-old patient was admitted for right otomastoiditis and thrombosis of the sigmoid and transverse sinuses, as well as the proximal portion of the internal jugular vein. Radiological examinations revealed a left orbital mass (22 × 14 mm) compatible with asymptomatic orbital meningocele (MC) herniated from the superior orbital fissure (SOF). The child underwent a right mastoidectomy. After the development of symptoms and signs of intracranial hypertension (ICH), endovascular thrombectomy and transverse sinus stenting were performed, with improvement of the clinical conditions and reduction of the orbital MC. The systematic literature review encompassed 29 publications on 43 patients with spontaneous orbital MC. In the majority of cases, surgery was the preferred treatment. Conclusions: The present case report and systematic review highlight the importance of ICH investigation and a pathophysiological-oriented treatment approach. The experiences described in the literature are limited, making the collection of additional data paramount. Full article

Despite the spread of novel-generation cochlear-implant (CI) magnetic systems, access to magnetic resonance imaging (MRI) for CI recipients is still limited due to safety concerns. The aim of this study is to assess and record the experiences of Hires Ultra 3D (Advanced Bionics) recipients who underwent an MRI examination. A multicentric European survey about this topic was conducted focusing on safety issues, and the results were compared with the current literature. We collected a total of 65 MRI scans performed in 9 otologic referral centers for a total of 47 Hires Ultra 3D recipients, including, for the first time, 2 children and 3 teenagers. Preventive measures were represented by scanning time and sedation for children. Head wrapping was used in eight cases, and six of the eight cases received local anesthesia, even if both measures were not needed. Only three patients complained of pain (3/65 examinations, 4.6%) due to the tight head bandage, and one of the three cases required MRI scan interruption. No other adverse events were reported. We believe that these results should encourage MRI execution in accordance with manufacturer recommendations for Ultra 3D recipients. Full article

Objectives: The objectives were to present the real-time estimated values of cochlear implant (CI) electrode insertion speed (IS) during intraoperative sessions using the Cochlear Nucleus SmartNav System to assess whether this measure affected CI outcomes and to determine whether real-time feedback assists expert surgeons in achieving slow insertion. Methods: The IS was measured in 52 consecutive patients (65 implanted ears) using the CI632 electrode. The IS values were analyzed in relation to procedure repetition over time, NRT ratio, and CI audiological outcomes. Results: The average IS was 0.64 mm/s (SD = 0.24); minimum and maximum values were 0.23 and 1.24 mm/s, respectively. The IS significantly decreased with each array insertion by the operator (p = 0.006), and the mean decreased by 24% between the first and last third of procedures; however, this reduction fell within the error range of SmartNav for IS (+/−0.48 mm/s). No correlation was found between IS and the NRT ratio (p = 0.51), pure-tone audiometry (PTA) at CI activation (p = 0.506), and PTA (p = 0.94) or word recognition score (p = 0.231) at last evaluation. Conclusions: The estimated IS reported by SmartNav did not result in a clinically significant reduction in insertion speed or an improvement in CI hearing outcomes. Real-time feedback of IS could potentially be used for training, but its effectiveness requires confirmation through additional studies and more accurate tools. Implementation of IS assessment in clinical practice will enable comparisons between measurement techniques and between manual and robot-assisted insertions. This will help define the optimal IS range to achieve better cochlear implant (CI) outcomes. Full article

Intralabyrinthine schwannomas (ILS) are rare benign tumours arising from the peripheral branches of the cochlear or vestibular nerves in the membranous labyrinth, intracochlear schwannomas being the most frequent ones. When hearing is no longer feasible on the affected side, surgical removal along with simultaneous cochlear implantation can be proposed to the patient. We hereby present a systematic review of the literature on the topic, as well as two original cases from our centre (Ospedale Università degli Studi di Padova). Cochlear implantation in intracochlear schwannomas is feasible, with overall satisfactory hearing outcomes in accordance with the evidence found in the literature. Full article

With the recent scientific and technical developments, hearing preservation surgery is becoming a growing objective in inner ear pathologies, especially for vestibular schwannomas. In this review, we aim to describe the pros and cons of the following cochlear nerve monitoring techniques: ABRs (auditory brainstem responses), DENM (direct eighth cranial nerve monitoring), EcochG (electrocochleography), CNAP (cochlear compound nerve action potentials), DPOAE (distortion product otoacoustic emissions), PAMRs (postauricular muscle responses). The Cochrane library, Scopus, DynaMed, and PubMed databases were screened to obtain any relevant papers from October 2009 to the present day. Due to the heterogeneity of the existing studies in the literature, there is no way to tell whether a technique is better than another. All authors reported satisfactory outcomes with the cochlear nerve monitoring techniques tested, either alone or in combination. Full article