Search

ImportanceOtologic disease is common among people with primary ciliary dyskinesia, yet little is known about its spectrum and severity.ObjectiveWe characterized otologic disease among participants with primary ciliary dyskinesia using data from the Ear-Nose-Throat Prospective International Cohort of PCD patients (EPIC-PCD).DesignCross-sectional analysis of baseline cohort data.SettingTwelve specialized primary ciliary dyskinesia centers in 10 countries.ParticipantsWe prospectively included children and adults with primary ciliary dyskinesia diagnoses, routine ENT examinations, and completed symptom questionnaires at the same visit or within 2 weeks.ExposuresPotential risk factors associated with increased risk of ear disease.Main outcomes and measuresWe describe the prevalence and characteristics of patient-reported otologic symptoms and findings from otologic examinations; we identify potential factors associated with increased risk of ear inflammation and hearing impairment.ResultsWe included 397 (211 males) participants with median age 15 (range 0–73). A total of 204 (51%) reported ear pain, 110 (28%) ear discharge, and 183 (46%) hearing problems. Adults reported ear pain and hearing problems more frequently when compared with children.Otitis media with effusion—usually bilateral—from otoscopy was most common among 121 (32%) of 384 participants. Retracted tympanic membrane and tympanic sclerosis were more commonly seen among adults. Tympanometry was performed on 216 participants and showed pathologic type B results for 114 (53%). Audiometry was performed on 273 participants and showed hearing impairment in at least 1 ear, most commonly mild.Season of visit was the strongest risk factor for problems related to ear inflammation (autumn compared with spring odds ratio, 95% confidence interval: 2.4, 1.5–3.8) and age 30 and older of hearing impairment (age 41–50 compared with age 10 years and younger odds ratio, 95% confidence interval: 3.3, 1.1–9.9).Conclusion and relevanceMany people with primary ciliary dyskinesia suffer from ear problems yet frequency varies, highlighting disease expression differences and possible clinical phenotypes. Understanding differences in otologic disease expression and progression during lifetime may inform clinical decisions about follow-up and medical care. We recommend multidisciplinary primary ciliary dyskinesia management includes regular otologic assessments for all ages even without specific complaints.Key PointsQuestionWhat are the characteristics of otologic disease among patients with primary ciliary dyskinesia (PCD)?FindingsBaseline data from a large multicenter cohort of patients with PCD showed frequent reports of ear pain and reduced hearing with age as the main factor associated with hearing impairment. Otitis media with effusion was the most common otoscopic finding; adults often presented with tympanic sclerosis following history of previous ear infections.MeaningSince otologic disease is an important yet underreported part of PCD’s clinical expression, we recommend otologic assessments for all age groups as part of regular clinical follow-up.

Delayed complications after cochlear implant (CI) surgery can result in the need for revision surgery. Temporal bone computed tomography (tCT) is frequently used in the diagnostic work-up for these patients.We aimed to test for the value of tCT in delayed complications after CI surgery.Retrospective chart review including all patients with CI who were consecutively enrolled in our academic tertiary care centre and received a tCT due to delayed complications between January 2008 and December 2017.A total of 85 patients with a mean age of 28 years at CI surgery were identified. Mean time between CI and tCT was 4.3 years. Reasons for performance of tCT were device malfunction (20%), cephalgia (20%), scalp hematoma/seroma (15.3%), device failure (11.8%), vertigo (10.6%), local infection (7.1%), trauma (5.9%), meningism (3.5%), tinnitus (3.5%) and facial palsy (2.4%). No abnormalities in tCT were detected in 82.4%. However, an abnormal tCT was a predictor for surgical therapy (We recommend the performance of tCT in CI patients with delayed complications to identify or rule out pathology in order to choose the most ideal and relevant individual treatment.

Purpose To give an overview of the current knowledge about COVID-19 pandemic and its impact on otolaryngology clinical practice. Methods Recent findings about SARS-CoV-2 virus and the COVID-19 infection it causes are reviewed. In addition to international databases and in the absence of hard scientific data, literature search included reports published online from scientific societies and other institutions. Results The role of anosmia as a COVID-19-related symptom is presented. Further, considerations about steroid administration in ENT-related conditions are also discussed. Due to the close work with mucosa surfaces of the upper aerodigestive tract, otolaryngologists and surrounding staff are considered high risk for coronavirus transmission. Hence, staff protection measures for ENT examinations, surgeries and other procedures during COVID-19 pandemic are recommended. Conclusion Knowledge and evidence about the impact of COVID-19 infection on otolaryngology clinical practice are accumulating rapidly. Additionally to patient’s management, safety of health care professionals should be a main goal right now by following strict safety guidelines.

Diffuse idiopathic skeletal hyperostosis (DISH) is a rare and potentially life-threatening syndrome. We present the case of a patient complaining about severe dyspnoea and diagnosed with vocal cord paresis. An emergency tracheotomy was performed to restore his breathing. Diagnostic imaging revealed large mass-occupying cervical osteophytes compressing the larynx. The osteophytes were removed via an anterior cervical approach, and vital signs were normalized. However, postoperatively, a fistula was discovered between the upper part of the oesophagus and the trachea. As a result, a gastrostomy tube had to be placed indefinitely. Literature review confirms the rare frequency of emergency tracheostomy due to DISH syndrome. The aim of the present study is to expand on our knowledge of a rare pathological entity that can frequently be misdiagnosed.

Introduction Primary ciliary dyskinesia (PCD) is a rare, genetic, multiorgan disease with an estimated prevalence of 1 in 10 000. It affects mainly the upper and lower airways due to impaired mucociliary clearance. Almost all patients have sinonasal or otologic (ear–nose–throat, ENT) problems, although the ENT clinical phenotype may present great variability. Despite that, data on PCD ENT manifestations are scarce and based on small single-centre studies. To date, we know little about the spectrum and severity of PCD ENT disease, its association with lung disease, its course over life and its determinants of prognosis. This study protocol describes the aims and methods of the first prospective, observational, multinational cohort study focusing on ENT disease in patients with PCD. Methods and analysis The ENT prospective international cohort of patients with PCD (EPIC-PCD) is a prospective standardised observational clinical cohort set up as a multinational multicentre study, embedded into routine patient care. It aims to longitudinally characterise ENT disease in patients with PCD and its association with lung disease, and to identify determinants of its prognosis. Patients of all ages, diagnosed with PCD who undergo an ENT clinical assessment at least once a year at one of the participating centres will be invited to participate. Collected data include diagnostic test results, results of ENT examinations, lung function measurements, information on management of ENT disease and patient-reported data on clinical symptoms and health-related quality of life (QoL). Data are collected using the standardised PCD-specific FOLLOW-PCD form and the validated QoL-PCD questionnaire. Ethics and dissemination The study has been reviewed and approved by the Human Research Ethics Committees at all participating centres, based on local legislation. The results of the study will be published in scientific journals, presented at scientific conferences and disseminated to participants and national patient organisations. Trial registration NCT04611516.

To examine the possible correlation between the loss of short-term residual hearing and vestibular function after cochlear implantation (CI).Retrospective patient review.Academic tertiary referral center.One hundred twenty patients with normal vestibular function (VF) in the caloric testing and residual hearing (RH) at the frequencies 250, 500, and 1000 Hz on the surgery side between 2008 and 2016 were included in the study.Primary CI on the first side via round window with a conventional full-length electrode.Changes of RH and VF 7 weeks after surgery were analyzed. Preservation of RH was defined as measurable postoperative thresholds at the frequencies 250, 500, and 1000 Hz in the pure-tone audiogram. Preservation of the VF after CI was assessed both by an absolute and relative threshold in the caloric testing.Seven weeks after implantation, the preservation of RH was achieved in 52 (43.3 %) patients and the preservation of VF in 95 (79.2 %) patients on the operated side. There was no significant statistical correlation between these two parameters.The loss of RH was more than twice as frequent as the loss of VF. However, no statistical correlation between the failure of the two inner ear functions was found. The exact causes are still unknown, but our data could give an indication that there may be different underlying pathomechanisms.

Purpose: To give an overview of the current knowledge about COVID-19 pandemic and its impact on otolaryngology clinical practice. Methods: Recent findings about SARS-CoV-2 virus and the COVID-19 infection it causes are reviewed. In addition to international databases and in the absence of hard scientific data, literature search included reports published online from scientific societies and other institutions. Results: The role of anosmia as a COVID-19-related symptom is presented. Further, considerations about steroid administration in ENT-related conditions are also discussed. Due to the close work with mucosa surfaces of the upper aerodigestive tract, otolaryngologists and surrounding staff are considered high risk for coronavirus transmission. Hence, staff protection measures for ENT examinations, surgeries and other procedures during COVID-19 pandemic are recommended. Conclusion: Knowledge and evidence about the impact of COVID-19 infection on otolaryngology clinical practice are accumulating rapidly. Additionally to patient’s management, safety of health care professionals should be a main goal right now by following strict safety guidelines. © 2020, Springer-Verlag GmbH Germany, part of Springer Nature.

BACKGROUND NOXA and MCL1 are involved in the intrinsic pathway of apoptosis, where Noxa selectively binds to MCL1 and prevents it from inhibiting apoptosis. Both factors are considered as potential tumour biomarkers, while MCL1 has attracted interest as target in cancer. The purpose of this study was to investigate the expression of NOXA and MCL1 in 160 CRC tumour samples, to investigate their significance, also in combination with IAPs, DR5 expression and KRAS gene mutations in CRC. MATERIALS AND METHODS Fresh frozen colorectal tissue was obtained from patients undergoing surgery for CRC. Real-time quantitative PCR was performed for the determination of mRNA expression levels. Protein expression was determined immunohistochemically. Differences in the mRNA expression profile were evaluated with the nonparametric Wilcoxon signed ranks test. Statistical analysis was performed with the use of Mann-Whitney U test and receiver-operating characteristic (ROC) curve. RESULTS NOXA was found to be overexpressed in CRC tumours (P

Objective The involvement of the dura is a rare yet potentially life-threatening complication during cholesteatoma surgery. Thus, the knowledge about treatment and consequences of this issue is of great importance to every ear surgeon. Methods This retrospective study analyzed the dura involvement with regard to the type of defect, reconstruction method used, and the post-operative complications of 1291 pediatric and adult cholesteatoma surgeries performed at an academic tertiary care center over a twelve-year period. Results From a total of 1291 cholesteatoma surgeries, we identified 84 patients (6.5%) with dura involvement intraoperatively, most of them adult patients. The majority of the reported cases were bony defects and exposed dura without CSF leakage (79.73%, 67 out of 84). In 14.28% of the cases (12 out of 84) a meningo(encephalo)cele or dura defect with liquorrhea were detected. In 30 surgeries (35.7%, 30 out of 84) no reconstruction of the lateral skull base was considered necessary. The most common material used for reconstruction was conchal cartilage (25.0%, 21 out of 84), followed by polydioxanone (PDS)-foil (11.9%, 10 out of 84), bone pâte (9.5%, 8 out of 84) and a combination of materials (17.9%, 15 out of 84). Revision surgery of the reconstruction was necessary in 16.7% (14 out of 84) of the cases. Long-term evaluation (mean of 19.3 months) showed no complication related to the skull base defect. Conclusion During cholesteatoma surgery, bony and dura defects can be managed effectively, with good long-term reliability. No intracranial or mastoidal complications are expected.