Optical coherence tomography angiography to assess for retinal vascular changes in Neuro-Sjögren.

Background: Sjögren's syndrome is an autoimmune disease characterized by sicca symptoms and various extraglandular manifestations including vasculitis. Neurological involvement occurs frequently (Neuro-Sjögren) and often mimics immune neuropathies such as chronic inflammatory demyelinating polyneuropathy (CIDP).

Objectives: We aim to assess relevant differences in vessel density (VD) in Optical Coherence Tomography Angiography (OCTA) in those diseases to use it as an easily available diagnostic tool.

Acoustic stimulation of the human round window by laser-induced nonlinear optoacoustics.

The feasibility of low frequency pure tone generation in the inner ear by laser-induced nonlinear optoacoustic effect at the round window was demonstrated in three human cadaveric temporal bones (TB) using an integral pulse density modulation (IPDM). Nanosecond laser pulses with a wavelength in the near-infrared (NIR) region were delivered to the round window niche by an optical fiber with two spherical lenses glued to the end and a viscous gel at the site of the laser focus. Using IPDM, acoustic tones with frequencies between 20 Hz and 1 kHz were generated in the inner ear.

Relapsing Polychondritis with Tracheobronchial Involvement: A Detailed Description of Two Pediatric Cases and Review of the Literature.

Relapsing polychondritis (RP) is a rare immune-mediated disease that primarily affects the cartilaginous structures of the ears, nose and airways. The clinical spectrum ranges from mild to severe disease characterized by progressive destruction of cartilage in the tracheobronchial tree leading to airway obstruction and acute respiratory failure. Early diagnosis is crucial to prevent irreversible airway damage and life-threatening complications.

Deep intracochlear injection of triamcinolone-acetonide with an inner ear catheter in patients with residual hearing.

Introduction: In a previous study, an inner ear catheter was used to deliver low- and high-dose steroids into the cochlea prior to cochlear implant electrode insertion. With this approach, more apical regions of the cochlea could be reached and a reduction of electrode impedances in the short term was achieved in cochlear implant recipients. Whether intracochlear application of drugs via the catheter is a safe method also for patients with residual hearing has not been investigated hitherto.

Estimating vibration artifacts in preclinical experimental assessment of actuator efficiency in bone-conduction hearing devices.

Objectives: Test feasibility of a means to distinguish artifact from relevant signal in an experimental method for pre-clinical assessment of bone conduction (BC) stimulation efficiency based on measurement of intracochlear pressure (ICP).

Methods: Experiments were performed on fresh-frozen human temporal bones and cadaver heads. In a first step, fiber optic pressure sensors inserted into the cochlea through cochleostomies were intentionally vibrated to generate relative motion versus the stationary specimen, and the resulting ICP artifact recorded, before and after attaching the sensor fiber to the bone with glue.

Sjögren's syndrome with and without neurological involvement.

Objective: Neurological manifestations of Sjögren's syndrome can be severe but also treatment-responsive. We aimed to systematically evaluate neurological manifestations of primary Sjögren's syndrome and find clinical features allowing sufficient identification of affected patients (pSSN) among those with Sjögren's syndrome without neurological involvement (pSS).

Methods: Para-/clinical features of patients with primary Sjögren's syndrome (2016 ACR/EULAR classification criteria) were compared between pSSN and pSS.

Clinical and paraclinical features of small fiber neuropathy in Sjögren's syndrome.

Sjögren's syndrome is a potentially treatable cause of Small Fiber Neuropathy (SFN)-a condition that severely affects patients' quality of life. We therefore aimed to characterize patients with SFN and Sjögren's syndrome to raise awareness of this disease and facilitate its early recognition as an essential step for appropriate treatment. In 97 SFN patients (median age 48 years, 77% female), we studied the clinical features associated with Sjögren's syndrome compared to the idiopathic SFN subtype.

Cochlear Implantation in Obliterated Cochlea: A Retrospective Analysis and Comparison between the IES Stiff Custom-Made Device and the Split-Array and Regular Electrodes.

Anatomical malformations, obliterations of the cochlea, or re-implantations pose particular challenges in cochlear implantation. Treatment methods rely on radiological and intraoperative findings and include incomplete insertion, the implantation of a double array, and radical cochleostomy. In addition, a stiff electrode array, e.

Trigeminal Nerve Affection in Patients with Neuro-Sjögren Detected by Corneal Confocal Microscopy.

Background: Patients with Sjögren's syndrome and polyneuropathy more frequently develop cranial nerve affection when compared to patients with chronic inflammatory demyelinating polyneuropathy (CIDP). We therefore aimed to analyze trigeminal corneal nerve fibre characteristics in both patient groups.

Methods: A total of 26 patients with Sjögren's syndrome associated neuropathy and 29 patients with CIDP were recruited at our university hospital and compared to 6 healthy controls.

Proteome profile of patients with excellent and poor speech intelligibility after cochlear implantation: Can perilymph proteins predict performance?

Modern proteomic analysis and reliable surgical access to gain liquid inner ear biopsies have enabled in depth molecular characterization of the cochlea microenvironment. In order to clarify whether the protein composition of the perilymph can provide new insights into individual hearing performance after cochlear implantation (CI), computational analysis in correlation to clinical performance after CI were performed based on the proteome profile derived from perilymph samples (liquid biopsies). Perilymph samples from cochlear implant recipients have been analyzed by mass spectrometry (MS).

Output performance of the novel active transcutaneous bone conduction implant Sentio at different stimulation sites.

Objectives: The output performance of a novel semi-implantable transcutaneous bone conduction device was compared to an established percutaneous bone-anchored hearing system device using cadaver heads. The influence of actuator position, tissue growth below the actuator and mounting it on the surface or in a flattened bone bed on the performance of the implanted actuator was investigated.

Materials And Methods: The percutaneous and the new transcutaneous device were sequentially implanted at two sites in five human cadaver heads: 55 mm superior-posterior to the ear canal opening (position A) and, closer to the cochlea, about 20 mm inferior-posterior to the ear canal opening behind the pinna on the mastoid (position B).

Personalized Proteomics for Precision Diagnostics in Hearing Loss: Disease-Specific Analysis of Human Perilymph by Mass Spectrometry.

Despite a vast amount of data generated by proteomic analysis on cochlear fluid, novel clinically applicable biomarkers of inner ear diseases have not been identified hitherto. The aim of the present study was to analyze the proteome of human perilymph from cochlear implant patients, thereby identifying putative changes of the composition of the cochlear fluid perilymph due to specific diseases. Sampling of human perilymph was performed during cochlear implantation from patients with clinically or radiologically defined inner ear diseases like enlarged vestibular aqueduct (EVA; = 14), otosclerosis ( = 10), and Ménière's disease ( = 12).

Nerve ultrasound findings in Sjögren's syndrome-associated neuropathy.

Background And Purpose: The phenotype of Sjögren's syndrome-associated neuropathy has been better characterized in recent years. However, Sjögren's syndrome-associated neuropathy remains an underdiagnosed entity with only few insights considering the pathomechanisms of nerve damage. Nerve ultrasound has proven to be a useful and efficient tool in detecting nerve damage of autoimmune origin.

First-in-human intracochlear application of human stromal cell-derived extracellular vesicles.

Extracellular vesicles (EVs) derived from the secretome of human mesenchymal stromal cells (MSC) contain numerous factors that are known to exert anti-inflammatory effects. MSC-EVs may serve as promising cell-based therapeutics for the inner ear to attenuate inflammation-based side effects from cochlear implantation which represents an unmet clinical need. In an individual treatment performed on a 'named patient basis', we intraoperatively applied allogeneic umbilical cord-derived MSC-EVs (UC-MSC-EVs) produced according to good manufacturing practice.

Performance Evaluation of Coupling Variants for an Active Middle Ear Implant Actuator: Output, Conductive Losses, and Stability of Coupling With Ambient Pressure Changes.

Introduction: This study aims to investigate the performance of an active middle ear implant actuator for various coupling configurations. Actuator output and conductive losses were measured, and the stability of coupling was evaluated by challenging the link between actuator and ossicles through pressure events in magnitudes that occur in daily life.

Methods: Actuator coupling efficiency and the occurrence of conductive losses were measured in 10 temporal bones through laser Doppler vibrometry on the stapes footplate for various coupling types (incus short process with and without laser hole, incus long process, stapes head).

Improved Speech Intelligibility in Subjects With Stable Sensorineural Hearing Loss Following Intratympanic Dosing of FX-322 in a Phase 1b Study.

Objectives: There are no approved pharmacologic therapies for chronic sensorineural hearing loss (SNHL). The combination of CHIR99021+valproic acid (CV, FX-322) has been shown to regenerate mammalian cochlear hair cells ex vivo. The objectives were to characterize the cochlear pharmacokinetic profile of CV in guinea pigs, then measure FX-322 in human perilymph samples, and finally assess safety and audiometric effects of FX-322 in humans with chronic SNHL.

CIDP associated with Sjögren's syndrome.

Background: This study addresses the challenging characterisation and differentiation of CIDP versus CIDP in association with Sjögren's syndrome to facilitate the process in clinical routine.

Methods: Patients with both CIDP and Sjögren's syndrome and CIDP without Sjögren's syndrome were compared concerning relevant differences in clinical, laboratory and electrophysiological findings. 154 patients who fulfilled the diagnostic EFNS/PNS criteria for CIDP were included in the analysis.

Extracellular vesicles from human multipotent stromal cells protect against hearing loss after noise trauma in vivo.

The lack of approved anti-inflammatory and neuroprotective therapies in otology has been acknowledged in the last decades and recent approaches are heralding a new era in the field. Extracellular vesicles (EVs) derived from human multipotent (mesenchymal) stromal cells (MSC) can be enriched in vesicular secretome fractions, which have been shown to exert effects (eg, neuroprotection and immunomodulation) of their parental cells. Hence, MSC-derived EVs may serve as novel drug candidates for several inner ear diseases.

Hearing dysfunction in patients with Neuro-Sjögren: a cross-sectional study.

Background: Sjögren's syndrome is an immunologically mediated disease with salivary and lacrimal gland destruction characterised by typical sicca symptoms of dry mouth and eyes. Awareness of extraglandular neurological manifestations such as polyneuropathy and affection of cranial nerves is rising. Hearing loss as consequence of involvement of the vestibulocochlear nerve presents a severe disability.

Impedance Values Do Not Correlate With Speech Understanding in Cochlear Implant Recipients.

Objective: To evaluate a possible correlation between impedance values and speech perception after cochlear implantation.

Study Design: Retrospective chart review.

Setting: Tertiary referral center.

Dose-Dependent Transient Decrease of Impedances by Deep Intracochlear Injection of Triamcinolone With a Cochlear Catheter Prior to Cochlear Implantation-1 Year Data.

Administration of low-dose steroids via a catheter inserted into the cochlea to apply pharmaceuticals to more apical regions was previously shown not to be sufficient for long-term reduction of electrode impedances. The aim of the present study was to investigate the effect of intra-cochlear high-dose triamcinolone application on impedances in cochlear implant recipients. Patients received low-dose (4 mg/ml; = 5) or high-dose (20 mg/ml; = 5) triamcinolone via a cochlear catheter just prior to the insertion of a Med-El Flex28 electrode.

Endogenous α1-antitrypsin levels in the perilymphatic fluid correlates with severity of hearing loss.

Objectives: To determine the levels of endogenous α1-antitrypsin in the perilymph of patients undergoing cochlear implant (CI), and its reverse association with the severity of hearing loss.

Study Design: Retrospective study.

Setting: Tertiary care university hospital.

Neuro-Sjögren: Peripheral Neuropathy With Limb Weakness in Sjögren's Syndrome.

Sjögren's syndrome is a heterogeneous inflammatory disorder frequently involving peripheral nerves with a wide spectrum of sensory modalities and distribution patterns. The objective of this cross-sectional study was to determine characteristics of Sjögren's syndrome as a cause for severe neuropathy with limb weakness. One hundred and eighty four patients with polyneuropathy associated with limb weakness underwent routine diagnostics including investigations for Sjögren's syndrome.

Defining the Inflammatory Microenvironment in the Human Cochlea by Perilymph Analysis: Toward Liquid Biopsy of the Cochlea.

The molecular pathomechanisms in the majority of patients suffering from acute or progressive sensorineural hearing loss cannot be determined yet. The size and the complex architecture of the cochlea make biopsy and in-depth histological analyses impossible without severe damage of the organ. Thus, histopathology correlated to inner disease is only possible after death.