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Epidermal nevus syndrome (ENS) comprises a heterogeneous group of neurocutaneous syndromes associated with the presence of epidermal nevi and variable extracutaneous manifestations. Postzygotic activating HRAS pathogenic variants were previously identified in nevus sebaceous (NS), keratinocytic epidermal nevus (KEN), and different ENS, including Schimmelpenning–Feuerstein–Mims and cutaneous‐skeletal‐hypophosphatasia syndrome (CSHS). Skeletal involvement in HRAS‐related ENS ranges from localized bone dysplasia in association with KEN to fractures and limb deformities in CSHS. We describe the first association of HRAS‐related ENS and auricular atresia, thereby expanding the disease spectrum with first branchial arch defects if affected by the mosaic variant. In addition, this report illustrates the first concurrent presence of verrucous EN, NS, and nevus comedonicus (NC), indicating the possibility of mosaic HRAS variation as an underlying cause of NC. Overall, this report extends the pleiotropy of conditions associated with mosaic pathogenic variants in HRAS affecting ectodermal and mesodermal progenitor cells. [ABSTRACT FROM AUTHOR]

To provide guiding principles to deliver high-quality treatment with implantable bone conducting hearing devices and to guarantee the best possible outcomes for each patient in Belgium. A consensus meeting was convened on March 26, 2019, including surgeons and audiologists from different bone conducting hearing devices hospitals and fitting centers in Belgium, and an independent moderator. First, different care models for treatment with bone conducting hearing devices, currently applied in Belgium, were identified and discussed. It was agreed that bone conducting hearing devices surgery and fitting should be provided by clinicians with adequate training and experience and that bone conducting hearing devices care should be centralized as much as possible. Preferably sound processor fitting is carried out within the bone conducting hearing devices hospital or at a specialized fitting center outside the hospital. In any case, a close interdisciplinary collaboration between both the bone conducting hearing devices surgeon and the bone conducting hearing devices audiologist was considered critical to ensure good patient outcomes (e.g., to facilitate appropriate treatment in the event of complications). Second, general guidelines were debated and agreed upon to improve the quality of care for the different phases of the patient journey (referral, assessment, treatment, and follow-up). Providing a standard of care means that every person, regardless of the type or degree of hearing loss, the region in which they reside and the healthcare professional they see, has access to a standardized assessment and treatment process, resulting in the most efficient hearing solution for his or her indication. This consensus statement was a first step towards a more standardized approach for treatments with bone conducting hearing devices in Belgium. [ABSTRACT FROM AUTHOR]

With the introduction of more flexible and thinner electrodes, such as Cochlear's Slim Modiolar Electrode, there is a higher risk of electrode insertion problems, in particular the tip foldover. Timely intraoperative detection of the problem would allow for direct intraoperative correction. This paper describes a non-radiological method for intraoperative tip foldover detection that is applicable in all surgical centers and can quickly deliver accurate results. Postoperative radiographs of 118 CI-recipients implanted with Nucleus devices were retrospectively analyzed on the presence of a tip foldover. Electrode Voltage Telemetry (EVT), also called Electric Field Imaging, was performed by means of Cochlear's EVT software tool, which is now integrated into Custom Sound–EP as the Trans-Impedance-Matrix measurement option. Tip foldover detection was automated by using the linear Hough transform for extracting straight-line patterns in the Trans-Impedance Matrix's heatmap. The six cases of electrode tip foldover were accurately identified by the EVT measurements, including two cases with folding location very close to the electrode tip (contact 20). Electrode Voltage Telemetry measures the Trans-Impedance Matrix, which can accurately detect tip foldovers of the cochlear implant electrodes within 1 min. This method can be reliably applied in all patients with normal cochlear anatomy and is able to intraoperatively detect foldovers localized even very close to the electrode tip. Application of the linear Hough transform allows for automatic detection of electrode tip foldovers that shows excellent agreement with visual evaluation of the radiological images and the transimpedance matrix's heatmap. [ABSTRACT FROM AUTHOR]

Relapsing polychondritis is a rare connective tissue disorder, of unknown etiology, characterized by episodic inflammation of cartilaginous and connective tissue structures, leading to permanent destruction. About 90% of patients develop auricular manifestations in the course of the disease. The main treatment of relapsing polychondritis is medical, and until now, surgery was only performed for cosmetic reasons. We present a case wherein M-meatoplasty is proposed as a functional treatment option in auricular relapsing polychondritis, preventing secondary external otitis and accompanying conductive hearing loss. [ABSTRACT FROM AUTHOR]

• Electrode voltage telemetry offers advanced impedance recording in clinical setting. • Electrode voltage telemetry allows decomposing impedance at cochlear implant electrode. • Decomposing impedance unravels postoperative phenomena at cochlear implant electrode. • Timescale of resistance changes near cochlear implant electrode hints to fibrosis. • Increased postoperative near-field resistance correlates to cochlear implant usage. Measurement of the complex electrical impedance of the electrode contacts can provide new insights into the factors playing a role in the preservation of residual hearing with cochlear implants (CIs). However, unraveling the contributions related to the different phenomena from impedance data necessitates more advanced measurement and analysis techniques. The present study explores a new impedance measurement option recently included into the cochlear-implant programming software and aims to contribute to a more solid basis for the clinical use of impedance measures as a biomarker for fibrous tissue formation. Twenty adult CI-recipients were followed from surgery until 1 year after implantation by means of Electrode Voltage Telemetry (EVT), also called Electric Field Imaging or TransImpedance-Matrix measurement, and a 4-point technique for probing the voltage between adjacent electrode contacts. The data were compared to the electrode location derived from computed tomography, and to the device usage log. Using our impedance model for electrical stimulation of the cochlea, the polarization impedance related the electrode–tissue interface was determined, and the bulk impedance (access resistance) was split into a near-field and a far-field component. On average, the polarization impedance increased abruptly after surgery, indicating a strong passivation of the electrode contacts before cochlear-implant initiation. Its initial rise resolved almost completely soon after device switchon (2–4 weeks). The gradual increase of the access resistance mainly happened during the first 40 days on a time scale very similar to that observed in a guinea-pig study correlating impedance changes to fibrous tissue growth. The higher increase towards the round window is consistent with the higher amount of tissue observed in histological animal studies close to the electrode entry point. While the initial changes were due to the near-field resistance, the far-field resistance began to rise only after one month for half of the study group, once the near-field component had reached its critical value. This suggests indeed fibrosis initiating near the electrode contacts and spreading thereafter farther away. The near-field resistance positively correlated to device usage. EVT data allow for a further decomposition of the impedance at a cochlear-implant electrode, yielding a more detailed description of the postoperative intracochlear phenomena, such as fibrosis. [ABSTRACT FROM AUTHOR]