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Hearing Disorders: HELP
Articles by Pascal N. Tyrrell
Based on 3 articles published since 2010
(Why 3 articles?)
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Between 2010 and 2020, Pascal N. Tyrrell wrote the following 3 articles about Hearing Disorders.
 
+ Citations + Abstracts
1 Review Diagnostic criteria for cryopyrin-associated periodic syndrome (CAPS). 2017

Kuemmerle-Deschner, Jasmin B / Ozen, Seza / Tyrrell, Pascal N / Kone-Paut, Isabelle / Goldbach-Mansky, Raphaela / Lachmann, Helen / Blank, Norbert / Hoffman, Hal M / Weissbarth-Riedel, Elisabeth / Hugle, Boris / Kallinich, Tilmann / Gattorno, Marco / Gul, Ahmet / Ter Haar, Nienke / Oswald, Marlen / Dedeoglu, Fatma / Cantarini, Luca / Benseler, Susanne M. ·Division of Pediatric Rheumatology, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany. · Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey. · Department of Medical Imaging, University of Toronto, Toronto, Ontario, Canada. · Department of Pediatric Rheumatology, Reference Centre for Autoinflammatory Disorders CEREMAI, Bicêtre Hospital, University of Paris SUD, Paris, France. · Translational Autoinflammatory Disease Section, NIAMS/NIH, Bethesda, Maryland, USA. · National Amyloidosis Centre, University College London Medical School, London, UK. · Haematologie, Onkologie und Rheumatologie, Universitaetsklinikum Heidelberg, Heidelberg, Germany. · University of California at San Diego, San Diego, California, USA. · Kinderrheumatologische Ambulanz, Universitaetsklinikum Eppendorf, Hamburg, Germany. · German Center for Pediatric and Adolescent Rheumatology, Garmisch-Partenkirchen, Germany. · Department of Rheumatology, Charité, University Medicine Berlin, Berlin, Germany. · UO Pediatria 2, G. Gaslini Institute, Genoa, Italy. · Istanbul University, Istanbul, Turkey. · Laboratory for Translational Immunology, University Medical Center Utrecht, Utrecht, The Netherlands. · Department of Rheumatology, Boston Children's Hospital, Boston, Massachusetts, USA. · Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA. · Rheumatology Unit, Policlinico Le Scotte, University of Sienna, Italy. · Rheumatology, Department of Paediatrics, Alberta Children's Hospital, University of Calgary, Calgary, Alberta, Canada. ·Ann Rheum Dis · Pubmed #27707729.

ABSTRACT: Cryopyrin-associated periodic syndrome (CAPS) is a rare, heterogeneous disease entity associated with

2 Article Early detection of sensorineural hearing loss in Muckle-Wells-syndrome. 2015

Kuemmerle-Deschner, Jasmin B / Koitschev, Assen / Tyrrell, Pascal N / Plontke, Stefan K / Deschner, Norbert / Hansmann, Sandra / Ummenhofer, Katharina / Lohse, Peter / Koitschev, Christiane / Benseler, Susanne M. ·Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tuebingen, Hoppe-Seyler-Str. 1, D-72076, Tuebingen, Germany. kuemmerle.deschner@uni-tuebingen.de. · Department of Otorhinolaryngology, Head and Neck Surgery, Klinikum Stuttgart, Stuttgart, Germany. a.koitschev@klinikum-stuttgart.de. · Department of Medical Imaging, University of Toronto, Toronto, Canada. pascal.tyrrell@utoronto.ca. · Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital Halle (Saale), Halle (Saale), Germany. stefan.plontke@uk-halle.de. · Department of Anesthesiology and Intensive Care Medicine, University Hospital Tuebingen, Tuebingen, Germany. norbert.deschner@uni-tuebingen.de. · Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tuebingen, Hoppe-Seyler-Str. 1, D-72076, Tuebingen, Germany. sandra.hansmann@med.uni-tuebingen.de. · Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tuebingen, Hoppe-Seyler-Str. 1, D-72076, Tuebingen, Germany. katharina.ummenhofer@googlemail.com. · CeGaT, Center for Genomics and Transcriptomics, Tuebingen, Germany. peter.lohse@cegat.de. · Department of Otorhinolaryngology, Head and Neck Surgery, Klinikum Stuttgart, Stuttgart, Germany. ch_haferkamp@yahoo.de. · Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tuebingen, Hoppe-Seyler-Str. 1, D-72076, Tuebingen, Germany. susa.benseler@gmail.com. · Rheumatology, Department of Pediatrics, Alberta Children's Hospital, University of Calgary, Calgary, Canada. susa.benseler@gmail.com. ·Pediatr Rheumatol Online J · Pubmed #26531310.

ABSTRACT: BACKGROUND: Muckle-Wells-syndrome (MWS) is an autoinflammatory disease characterized by systemic and organ-specific inflammation due to excessive interleukin (IL)-1 release. Inner ear inflammation results in irreversible sensorineural hearing loss, if untreated. Early recognition and therapy may prevent deafness. The aims of the study were to characterize the spectrum of hearing loss, optimize the otologic assessment for early disease and determine responsiveness to anti-IL-1-therapy regarding hearing. METHODS: A single center prospective cohort study of children and adults with MWS was performed. Standardized clinical, laboratory and otologic assessments including standard pure tone audiometry, additional high tone thresholds, vestibular organ testing, tinnitus evaluation and functional disability classes were determined serially. Pure-tone-average models were developed and evaluated. Risk factors for hearing loss and the impact of anti-IL-1 treatment were determined. RESULTS: A total of 23 patients with genetically confirmed MWS were included, of whom 63 % were females; 52 % were children. At baseline all patients had active MWS; 91 % reported clinically impaired hearing with 74 % having an abnormal standard assessment (0.5-4 kHz). In contrast, high frequency pure tone averages (HF-PTA) were abnormal in all symptomatic patients including those with early hearing loss (sensitivity 100 %). Females were at highest risk for hearing loss even after adjustment for age (p = 0.008). Treatment with IL-1 blockade resulted in improved or stable hearing in 91 % of patients. CONCLUSIONS: Early inner ear inflammation in MWS primarily affects the high frequencies, beyond the range of standard otologic assessment tools. The HF-PTA is a sensitive tool to detect imminent hearing loss and monitor treatment response.

3 Article Risk factors for severe Muckle-Wells syndrome. 2010

Kümmerle-Deschner, Jasmin B / Tyrrell, Pascal N / Reess, Fabian / Kötter, Ina / Lohse, Peter / Girschick, Hermann / Huemer, Christian / Horneff, Gerd / Haas, Johannes-Peter / Koitschev, Assen / Deuter, Christoph / Benseler, Susanne M. ·University Hospital Tuebingen, Tuebingen, Germany. ·Arthritis Rheum · Pubmed #20722029.

ABSTRACT: OBJECTIVE: Muckle-Wells syndrome (MWS) is an inherited autoinflammatory disease resulting in excessive interleukin-1 release. It is unknown whether demographic, clinical, or laboratory characteristics at the time of diagnosis may identify patients who are at high risk for severe disease activity. This study was undertaken to analyze clinical and laboratory features of MWS, compare genetically defined subcohorts, and identify risk factors for severe MWS. METHODS: A multicenter cohort study of consecutive MWS patients was performed. Parameters assessed included clinical features, MWS Disease Activity Score (MWS-DAS), inflammation markers, and cytokine levels. E311K mutation-positive patients were compared with E311K mutation-negative patients. Putative risk factors for severe MWS (defined as an MWS-DAS score of ≥10) were assessed in univariate analyses, and significant predictors were entered into a multivariate model. RESULTS: Thirty-two patients (15 male and 17 female) were studied. The most frequent organ manifestations were musculoskeletal symptoms and eye and skin disorders. Renal disease and hearing loss were seen in >50% of the patients. Genetically defined subcohorts had distinct phenotypes. Severe disease activity was documented in 19 patients (59%). Predictors of severe MWS identified at the time of diagnosis were female sex, hearing loss, musculoskeletal disease, increased erythrocyte sedimentation rate, and low hemoglobin level. Female sex and hearing loss remained significant after adjustment for age in a multivariate model (relative risk 1.8 and 2.6, respectively). CONCLUSION: MWS patients at high risk for severe disease can be identified at the time of diagnosis. Female patients presenting with hearing loss have the highest likelihood of manifesting severe MWS and should be considered a high-risk group.