Perilymph Fistula as a Complication of Eustachian Tube Dilation and Tympanoplasty.

Eustachian tube dilation (ETD) is an established, minimally invasive therapeutic approach for chronic eustachian tube dysfunction. The complications associated with performing a ETD are rare. A 22-year-old female patient presented with chronic otitis media on the right side and chronic obstructive tube dilation disorder on both sides. A type I tympanoplasty was performed on the right side because of a tympanic membrane perforation after a ETD on both sides without apparent complications. On the 5th postoperative day, she presented with headache, dizziness and hearing loss on the right side. There was a decrease of hearing threshold on the right side in the pure-tone audiogram and vHIT, cVEMP, and SVV were irregular. The ß-2-transferrin test was positive. Since a right-sided perilymph fistula was suspected, an emergency tympanotomy was performed with a round window membrane cover with fascia on the right side. Intraoperatively, a regular, intact ossicular chain was found with a slightly moist middle ear mucosa. The round window membrane was covered by the promontorial lip. Under these measures, the patient's dizziness regressed. The right ear pure-tone threshold vHIT, cVEMP, and SVV normalized.

Ipsilateral Vestibular Schwannoma after Cochlear Implantation.

OBJECTIVE: The vestibular schwannoma incidence rate is approximately 4.2 per 100,000/year. Thus far, about 700,000 cochlear implantations have been performed worldwide; therefore, the occurrence of vestibular schwannoma postcochlear implantations can be assumed to be infrequent. Recent developments allow safe observation and surveillance of the implanted-side internal auditory canal (IAC) and cochlea by magnetic resonance imaging (MRI), even after cochlear implantation. Patients. A 71-year-old woman with sudden hearing loss and a contralateral vestibular schwannoma without clinical and genetic signs of neurofibromatosis type II. Intervention(s). Ipsilateral cochlear implantation and contralateral vestibular schwannoma extirpation with regular tumor follow-up. Main Outcome Measure(s). Comparison of ipsilateral pre and postcochlear implantation 3T MRI T1 GAD. RESULTS: We observed a tumor growing at the fundus of the internal auditory canal 1 year after cochlear implantation on the ipsilateral side. Although first detected after cochlear implantation beside a known vestibular schwannoma on the contralateral side, a scan slice thickness of 2 mm cannot fully exclude the preoperative persistence of a small tumor. Based on the clinical findings and after genetic exclusion of NFII, the patient was classified as a NFII mosaic type. CONCLUSION: Even after cochlear implantation, tumors in the IAC causing vertigo, facial palsy, and affecting the audiologic outcome can be detected by MRI. The MRI slice thickness used before cochlear implantation should be under 2 mm.

Evaluation of cochlear implant electrode scalar position by 3 Tesla magnet resonance imaging.

The estimation of scalar electrode position is a central point of quality control during the cochlear implant procedure. Ionic radiation is a disadvantage of commonly used radiologic estimation of electrode position. Recent developments in the field of cochlear implant magnets, implant receiver magnet position, and MRI sequence usage allow the postoperative evaluation of inner ear changes after cochlear implantation. The aim of the present study was to evaluate the position of lateral wall and modiolar cochlear implant electrodes using 3 T MRI scanning. In a prospective study, we evaluated 20 patients (10× Med-El Flex 28; 5× HFMS AB and 5× SlimJ AB) with a 3 T MRI and a T2 2D Drive MS sequence (voxel size: 0.3 × 0.3 × 0.9 mm) for the estimation of the intracochlear position of the cochlear implant electrode. In all cases, MRI allowed a determination of the electrode position in relation to the basilar membrane. This observation made the estimation of 19 scala tympani electrode positions and a single case of electrode translocation possible. 3 T MRI scanning allows the estimation of lateral wall and modiolar electrode intracochlear scalar positions.

Malignant Transformation of Temporal Bone Schneiderian Papilloma Associated with HPV-6.

INTRODUCTION: Temporal bone Schneiderian papillomas (TBSPs) rarely present as a primary tumors arising from the middle ear and mastoid process. The clinical findings and imaging of TBSPs are not specific. Therefore, diagnosis can only reliably be definitively established by histopathology. OBJECTIVE: To report a novel case of a malignant transformation of TBSP associated with HPV-6 and to present its management. Case Report. A 68-year-old woman presented with conductive hearing loss and recurrent right-sided otorrhoea. Initially, we performed a lateral temporal bone resection and obliteration with abdomen fat. Early histology described TBSP associated with HPV-6. Follow-up detected malignant transformation of the Schneiderian papillomatous variant. Postoperative radiotherapy combined with extended temporal bone resection resulted in a disease-free 17-month period of follow-up. Discussion. TBSPs are not very specific, and the diagnosis can only reliably be established by histopathology. There is a risk of malignant transformation, and due to the absence of reliable prognostic markers, strict postoperative follow-up is mandatory and should consist of regular otoscopy, nasal endoscopy, and imaging. This case also supports the importance of extended temporal bone resections as salvage surgery, combining radical surgery with radiotherapy for improved survival rates.

Covid-19 dermatoses: Acral vesicular pattern evolving into bullous pemphigoid.

Bullous pemphigoid (BP) appears to be rising in incidence across the Western World, especially in the elderly. Some of the pathogenetic mechanisms involving antigen mimicry and antibody cross-reactivity have been elucidated for cases associated with neurological disease and certain drugs. There have been reports of cutaneous manifestations of Covid-19 (SARS-Cov2 infection) as the pandemic has raged across the world. We report here a case of prolonged Covid-19, symptomatic with dermatoses only, which was seen to evolve initially from a maculo-papular exanthema with acral vesicular dermatitis, into classical BP disease. This was confirmed histologically by positive skin autoantibody serology, direct IMF on peri-lesional skin and also salt-split IMF. Although possible that the development of BP could be a purely co-incidental finding during Covid-19, we suggest that it is more likely that prolonged SARS-Cov2 infection triggered an autoimmune response to the basement membrane antigens, BP 180 and 230. To our knowledge, this is the first case of BP developing during concurrent Covid-19 disease. It will be necessary to continue dermatological surveillance as the pandemic continues, to collate data on BP incidence and to test these patients for Covid-19 disease. As the pandemic continues, even potential and rare associations such as this will be clarified eventually. What's already known about this topic? Covid-19 disease has been associated with a spectrum of dermatosesCommon presentations in up to 20% of patients include exanthema, pseudo-chilblain like acral lesions 'Covid toes', livedo-/retiform purpuric/necrotic vascular lesions, acute urticarial lesions, and vesicular/varicella-like lesionsA multi-system inflammatory syndrome in children akin to Kawasaki syndrome has been described What does this study add? To our knowledge, this is the first description of classic Bullous Pemphigoid evolving from vesicular lesions caused by prolonged SARS-Cov2 induced skin inflammation.

Effect of head position on cochlear implant MRI artifact.

PURPOSE: A new generation of cochlear implant (CI) magnets and specific surgical techniques (e.g., implant positioning) has changed the relationship between a CI and magnet resonance imaging (MRI). MRI allows a pain free in vivo evaluation of the inner ear fluid state and internal auditory canal after the insertion of an electrode. The aim of this study is to evaluate how the patient's head position in the MRI scanner influences the CI magnet-related artefact. METHODS: We performed in vivo measurement of MRI artefacts at 3 T with a CI system containing a bipolar diametrical magnet. The implant magnet was positioned with a head bandage at different positions from the nasion and external auditory canal in three volunteers. We used a turbo spin echo (TSE) T2w sequence on the axial and coronal planes and observed three positions: (1) regular position, (2) chin to chest (anteflexion), and (3) hyperextension (retroflexion). RESULTS: By comparing the positions, anteflexion of the cervical spine in a chin-to-chest position allowed us to place the artefact in a more apical position from the IAC in the coronal plane. The hyperextension of the cervical spine position shifts the artefact father towards the cochlea's direction. CONCLUSION: The head's position can influence the location of MRI artefacts. In cases where the artefact diminished the IAC or cochlea, anteflexion of the cervical spine in the chin-to-chest position of the head in the MRI scanner should be attempted to allow a visualization of the IAC.

Electrophysiological effects of slim straight intracochlear electrode position.

OBJECTIVE: The electrical current distribution of a cochlear implant electrode within the cochlea is essential for post-operative hearing performance. The slim straight electrode is designed to enable the placement of contacts in a lateral or medial direction to the modiolus. The electrophysiological effect of this different contact direction is so far unknown. The aim of this study was to determine the influence of intracochlear laterally or medially directed electrode contacts on electrophysiological behaviour. METHOD: A slim straight electrode was inserted into the cochleae of five patients, and the neural response threshold was measured in a laterally and medially directed contact position. The cochleae in five temporal bone specimens were de-capped allowing an insertional observation of the contact position (lateral versus medial) of the electrode. RESULTS: There was no difference in neural response threshold between a lateral and a medial position of the contacts. Temporal bone study indicated no intracochlear torsion of the electrode. CONCLUSION: Our study provides evidence that the intracochlear position of slim straight electrode contacts does not affect the neural response threshold.

Comparison of Cochlear Implant Magnets and Their MRI Artifact Size.

INTRODUCTION: Recent developments regarding cochlear implant magnets (e.g., a bipolar diametral magnet) and refined surgical techniques (e.g., implant positioning) have had a significant impact on the relation between cochlear implants and MRIs, making the reproducible visibility of cochlea and IAC possible. MRI scanning has changed from a contraindication to a diagnostic tool. Magnet artifact size plays a central role in the visual assessment of the cochlea and IAC. OBJECTIVE: The aim of this study is to compare the CI magnet-related maximum artifact sizes of various cochlea implant systems. MATERIALS AND METHODS: We performed an in vivo measurement of MRI artifacts at 1.5 and 3 Tesla with three cochlear implant magnet systems (AB 3D, Medel Synchrony, and Oticon ZTI). The implant, including the magnet, was positioned with a head bandage 7.0 cm and 120° from the nasion, external auditory canal. We used a TSE T2w MRI sequence on the axial and coronal plains and compared the artifacts in two volunteers for each tesla strength. RESULTS: Intraindividual artifact size differences between the three magnets are smaller than interindividual maximum artifact size differences. 3 T MRI scans, in comparison to 1.5 T MRI scans, show a difference between soft artifact areas. CONCLUSION: We observed no major difference between maximum implant magnet artifact sizes of the three implant magnet types.

Electrode Afterload: A Valuable Technique in a Case of Short Electrode Insertion.

Introduction. The location of the electrode inside the cochlea is important for speech performance. However, many variables, including array length, insertion depth, and individual anatomy, may affect the intracochlear position of the electrode. Insertion deeper than 20 mm and revision surgery are critical situations in which residual hearing and electrode integrity may be at risk. This case report challenges this hypothesis and raises the following question: is it possible to achieve a better speech understanding with an electrode afterload without compromising residual hearing? Case Report. A 73-year-old female patient showed up for evaluation of hearing loss. The patient was operated four times in an external hospital due to cholesteatoma formation in the right ear. Related to a poor aided speech understanding, a CI-surgery was performed. 5 months after the surgery, the subject returned with poor speech understanding. A revision surgery was performed, where the first white marker of the electrode was seen in the round window (20 mm). The electrode was inserted 4 mm deeper into the cochlea. After six and twelve months, the results of the Freiburger monosyllabic speech test improved till 25% and 45%, respectively. Discussion. Hearing preservation is possible with a revisional deeper insertion from 20 mm to 24 mm. In this case, a partial obliteration of an open cavity made the electrode surgically easily accessible. This allowed the deeper insertion during the revision surgery. In a regular surgical field with a posterior tympanotomy, the revision surgery is more challenging and brings the electrode into the risk of an iatrogenic destruction. Conclusion. This case of an electrode afterload after having inserted the electrode initially to mm, demonstrates that hearing can be preserved and speech perception can improve after performing this maneuver.

Immunotherapeutics for head and neck squamous cell carcinoma stem cells.

Cancer stem cell (CSC)-related therapy resistance has become a new obstacle to the successful application of cancer treatment and head and neck squamous cell carcinoma (HNSCC) is no exception to this finding. Head and neck squamous cell carcinoma is highly immune-suppressive, and recently the immune suppression and invasion of HNSCC-CSCs have been characterized. These characteristics have received research and clinical attention because they would enable the stratification of patients into specific cancer subtypes and, consequently, the establishment of new therapeutic approaches with improved efficacy. This review discusses the feasibility of CSC-targeted strategies and their incorporation with nanotechnology to improve the efficacy of cancer immunotherapy.

[Ectopic thyroid tissue after total thyroidectomy]. / Ektopes Schilddrüsengewebe nach erfolgter Thyreoidektomie.

Ectopic thyroid gland tissue can occur either as the only detectable thyroid gland tissue or in addition to a normotopic thyroid gland. After a total thyroidectomy thyroid-stimulating hormone (TSH) can induce a compensatory volume increase of previously asymptomatic ectopic tissue. This hyperplastic ectopic tissue can occur as an unclear cervical space-occupying lesion. Prior to surgical exploration of an unclear cervical mass the possibility of ectopic thyroid tissue should be included in the differential diagnostic considerations.

[A rare pathology of the petrous part of the temporal bone: adenoma of the middle ear]. / Eine seltene Pathologie des Felsenbeins: Das Adenom des Mittelohres.

Adenomas are very rare tumors of the middle ear. They are benign neoplasms originating from the glandular components of the mucous membrane of the middle ear. The middle ear adenoma was first described by Hyams and Michaels in 1976, which was named an adenomatous tumor. This article reports the case of a 50-year-old female patient, who presented with recurrent right-sided dull otalgia and pulse synchronous tinnitis, which began 1 year prior to presentation, with the suspected diagnosis of a glomus tympanicum tumor. Following the otorhinolaryngological examination and imaging an unclear mesotympanal space-occupying lesion was detected. A transmeatal endoscopic complete removal of the tumor was carried out. The histopathological investigations enabled the diagnosis of an adenoma of the middle ear. Adenomas are a rare differential diagnosis of tumors of the middle ear. In cases with a suitable localization an adequate exposure and removal of this rare tumor can be achieved by a transmeatal endoscopic access.

Dynamic intracochlear pressure measurement during cochlear implant electrode insertion.

Background: Electrode insertion into the cochlea can cause significant pressure changes inside the cochlea with assumed effects on the cochlea's functionality regarding residual hearing. Model-based intracochlear pressure (ICP) changes were performed statically at the cochlear helix. Aims/objectives: The aim of this study was to observe dynamic pressure measurements during electrode insertion directly at the cochlear implant electrode. Material and methods: The experiments were performed in an uncurled cochlear model that contained a volume value equivalent to a full cochlea. A microfibre pressure sensor was attached at one of two positions on a cochlear implant electrode and inserted under different insertional conditions. Results: We observed the ICP increase depending on the insertional depth. A sensor-position-specific pressure change is insertional-depth dependent. Interval insertion did not lead to a lower peak insertional ICP. Conclusions and significance: In contrast to the static pressure-sensor measurement in the artificial model's helix, a dynamic measurement directly at the electrode shows the pressure profile to increase based on the insertional depth. A mechanical traumatic relevance of the observed pressure values cannot be fully excluded.

The Pull-Back Technique for the 532 Slim Modiolar Electrode.

INTRODUCTION: The distance between the modiolus and the electrode array is one factor that has become the focus of many discussions and studies. Positioning the electrode array closer to the spiral ganglion with the goal of reducing the current spread has been shown to improve hearing outcomes. The perimodiolar electrode arrays can be complemented with a surgical manoeuvre called the pull-back technique. This study focuses its attention on the recently developed 532 slim modiolar electrode. OBJECTIVE: To investigate the intracochlear movements and pull-back technique for the 532 slim modiolar electrode. MATERIAL AND METHODS: A decapping procedure of the cochlea was performed on 5 temporal bones. The electrode array was inserted, and the intracochlear movements were microscopically examined and digitally captured. Three situations were analysed: the initial insertion, the overinsertion, and the pull-back position. The position of the three white markers of the electrode array in relation to the round window (RW) was evaluated while performing these three actions. RESULTS: The initial insertion achieved an acceptable perimodiolar position of the electrode array, but a gap was still observed between the mid-portion of the array and the modiolus (the first white marker was seen in the RW). When we inserted the electrode more deeply, the mid-portion of the array was pushed away from the modiolus (the second and third white markers were seen in the RW). After applying the pull-back technique, the gap observed during the initial insertion disappeared, resulting in an optimal perimodiolar position (the first white marker was once again visible in the RW). CONCLUSION: This temporal bone study demonstrated that when applying the pull-back technique for the 532 slim modiolar electrode, a closer proximity to the modiolus was achieved when the first white marker of the electrode array was visible in the round window.

Labyrinthectomy after Cochlear Implantation: A Case of a Novel Approach for Vertigo Control.

Vertigo control in cases of Ménière disease and deafness can be achieved by labyrinthectomy before or as a single-stage procedure during cochlear implantation. The aim was to describe a case in which a labyrinthectomy was performed after cochlear implantation. The scar tissue was removed from the electrode cable, and the receiver was removed from the periostal pocket and placed out without electrode dislocation. Labyrinthectomy was performed after securing the electrode at the external canal. The patient disclaimed after three months no disabling vertigo. Intraoperatively, the electrode was not dislocated. A labyrinthectomy can be performed even after cochlear implantation to treat vertigo.

[Perspectives of genome editing in otorhinolaryngology]. / Perspektiven der Genomeditierung in der Hals-Nasen-Ohren-Heilkunde.

BACKGROUND: Recent advances in DNA sequencing technology have enabled researchers to identify the genetic background underlying human illness. In addition, the latest genome editing technology, CRISPR-Cas9 (clustered regularly interspaced short palindromic repeats and CRISPR-associated protein 9), provides great potential to edit genomic DNA sequences precisely with high efficiency. This technology has been evaluated for treatment of genetic diseases in recently published preclinical studies. Since many such genetic disorders can affect functional structures in the head and neck area, the technology bears high therapeutic potential in otorhinolaryngology. OBJECTIVE: In this article, we summarize the concept of CRISPR-Cas9-based therapies, recent achievements in preclinical applications, and future challenges for the implementation of this technology in otolaryngology. MATERIALS AND METHODS: Genetic targeting strategies were analyzed or established using genome sequencing data derived from online databases and literature. RESULTS: Recent research on animal models has shown that genome editing can be used to treat genetic diseases by specifically targeting mutant genomic loci. For example, one preclinical study in the field of otolaryngology has demonstrated that inherited autosomal dominant deafness in mice can be treated using CRISPR-Cas9. Moreover, the same strategies can be used to establish applications for the treatment of head and neck cancer. The greatest challenge appears to be establishment of a system for the safe and efficient delivery of therapeutic nucleotides in clinics. CONCLUSIONS: In theory, genome editing could be used in otolaryngology to target disease-causing genomic loci specifically. However, various challenges have to be overcome until applications can be used clinically.

Induction chemotherapy (IC) followed by radiotherapy (RT) versus cetuximab plus IC and RT in advanced laryngeal/hypopharyngeal cancer resectable only by total laryngectomy-final results of the larynx organ preservation trial DeLOS-II.

Background: The German multicenter randomized phase II larynx organ preservation (LOP) trial DeLOS-II was carried out to prove the hypothesis that cetuximab (E) added to induction chemotherapy (IC) and radiotherapy improves laryngectomy-free survival (LFS; survival with preserved larynx) in locally advanced laryngeal/hypopharyngeal cancer (LHSCC). Patients and methods: Treatment-naïve patients with stage III/IV LHSCC amenable to total laryngectomy (TL) were randomized to three cycles IC with TPF [docetaxel (T) and cisplatin (P) 75 mg/m2/day 1, 5-FU (F) 750 mg/m2/day days 1-5] followed by radiotherapy (69.6 Gy) without (A) or with (B) standard dose cetuximab for 16 weeks throughout IC and radiotherapy (TPFE). Response to first IC-cycle (IC-1) with ≥30% endoscopically estimated tumor surface shrinkage (ETSS) was used to define early responders; early salvage TL was recommended to non-responders. The primary objective was 24 months LFS above 35% in arm B. Results: Of 180 patients randomized (July 2007 to September 2012), 173 fulfilled eligibility criteria (A/B: larynx 44/42, hypopharynx 41/46). Because of 4 therapy-related deaths among the first 64 randomized patients, 5-FU was omitted from IC in the subsequent 112 patients reducing further fatal toxicities. Thus, IC was TPF in 61 patients and TP in 112 patients, respectively. The primary objective (24 months LFS above 35%) was equally met by arms A (40/85, 47.1%) as well as B (41/88, 46.6%). One hundred and twenty-three early responders completed IC+RT; their overall response rates (TPF/TP) were 94.7%/87.2% in A versus 80%/86.0% in B. The 24 months overall survival (OS) rates were 68.2% and 69.3%. Conclusions: Despite being accompanied by an elevated frequency in adverse events, the IC with TPF/TP plus cetuximab was feasible but showed no superiority to IC with TPF/TP regarding LFS and OS at 24 months. Both early response and 24 months LFS compare very well to previous LOP trials and recommend effective treatment selection and stratification by ETSS. Clinical trial information: NCT00508664.