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Hearing Disorders: HELP
Articles by Seza Ozen
Based on 3 articles published since 2010
(Why 3 articles?)

Between 2010 and 2020, S. Ozen 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 Evaluation of hearing in pediatric familial Mediterranean fever patients during attack period and attack-free period. 2019

Salimov, Asif / Akyol, Umut / Cildir, Bunyamin / Batu, Ezgi Deniz / Ozen, Seza. ·Department of Otolaryngology, Hacettepe University Faculty of Medicine, 06100, Sihhiye, Ankara, Turkey. Electronic address: asifselimov2003@hotmail.com. · Department of Otolaryngology, Hacettepe University Faculty of Medicine, 06100, Sihhiye, Ankara, Turkey. · Department of Audiology, Hacettepe University Faculty of Health Sciences, 06100, Sihhiye, Ankara, Turkey. · Department of Pediatrics, Division of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, 06100, Sihhiye, Ankara, Turkey. ·Int J Pediatr Otorhinolaryngol · Pubmed #30743166.

ABSTRACT: OBJECTIVES: Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease worldwide. It usually has a childhood onset and is characterized with recurrent attacks with irregular intervals. Few studies have been performed to investigate hearing in FMF patients ran with various tests and showed different results. In this study, first time in the literature, we aimed to evaluate and compare auditory functions in pediatric FMF patients during the attack periods and attack-free periods to see the possible effects of autoinflammation that was caused by FMF attacks. METHODS: 40 pediatric FMF patients (80 ears) enrolled in the study as study group and 21 matching (42 ears) healthy children as a control group. Blood samples were taken from patients who presented clinical attack symptoms. Control group and FMF patients that were in attack period underwent audiometric evaluation including pure tone audiometry (0.25, 0.5, 1, 2, 4, 8 kHz) and Distortion product otoaoustic emission (DPOAE) (1, 1.4, 2, 2.8, 4 kHz) test. The tests were repeated in attackfree period. Correlations between hearing results and C-reactive protein, erythrocyte sedimentation rate levels, duration of disease, age at disease onset, colchicine usage time, cumulative dosage of colchicine and the number of attacks in the last six months were studied. RESULTS: Hearing thresholds of FMF patients, compared to control group, were found to be increased at most frequencies. Lower signal/noise ratios (SNR) were detected at most frequencies in the DPAOE test. In the attack period compared to attack-free period, hearing thresholds in the audiometry were found to be increased at some frequencies (p < 0.05). However, this was not supported by the DPOAE test. In correlation analysis, increased colchicine usage time and colchicine cumulative dosage were associated with decreased thresholds in audiogram and increased SNR values in DPOAE. Detailed statistical analyses of all parameters were included in the study. CONCLUSION: Results demonstrated cochlear involvement in FMF patients. Acute changes in hearing thresholds in the attack period may be suggesting the effect of acute inflammation on cochlea. The difference between the audiometry and otoacoustic emission test results of FMF patients and healthy controls suggests the cumulative effect of recurrent inflammation attacks on cochlea. Positive affect of treatment of the disease with long term colchicine in hearing were also demonstrated.

3 Article Phenotypic and genotypic characteristics of cryopyrin-associated periodic syndrome: a series of 136 patients from the Eurofever Registry. 2015

Levy, R / Gérard, L / Kuemmerle-Deschner, J / Lachmann, H J / Koné-Paut, I / Cantarini, L / Woo, P / Naselli, A / Bader-Meunier, B / Insalaco, A / Al-Mayouf, S M / Ozen, S / Hofer, M / Frenkel, J / Modesto, C / Nikishina, I / Schwarz, T / Martino, S / Meini, A / Quartier, P / Martini, A / Ruperto, N / Neven, B / Gattorno, M / Anonymous6350800. ·Paediatric Rheumatology, Hôpital Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France. · Department of Clinical Immunology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux de Paris, Paris, France. · Division of Pediatric Rheumatology, University Hospital Tübingen, Tuebingen, Germany. · National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK. · Paediatric Rheumatology, CEREMAI, CHU de Bicêtre, Assistance Publique-Hôpitaux de Paris, Paris, France. · Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy. · Centre of Paediatric and Adolescent Rheumatology-UCL, London, UK. · Pediatria II, Reumatologia, Istituto Giannina Gaslini, Genoa, Italy. · Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy. · Department of Pediatric, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. · Department of Paediatric Nephrology and Rheumatology, Hacettepe University, Ankara, Turkey. · Paediatric Rheumatology Unit of Western Switzerland, CHUV, University Hospital of Lausanne, Lausanne, Switzerland. · Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands. · Reumatologia, Hospital Valle de Hebron, Barcelona, Spain. · Children's Department, Institute of Rheumatology RAMS, Moscow, Russian Federation. · Section of Paediatric Rheumatology and Osteology, University School of Medicine Children's Hospital, Würzburg, Germany. · Dip.to di Scienze Pediatriche e dell'Adolescenza, Clinica Pediatrica Universita' di Torino, Turin, Italy. · Pediatric Immunology and Rheumatology Unit, Pediatric Clinic, Spedali Civili and University of Brescia, Brescia, Italy. · Pediatria II, Reumatologia, Istituto Giannina Gaslini, Genoa, Italy Department of Paediatrics, University of Genoa, Italy. ·Ann Rheum Dis · Pubmed #25038238.

ABSTRACT: OBJECTIVE: To evaluate genetic, demographic and clinical features in patients with cryopyrin-associated periodic syndrome (CAPS) from the Eurofever Registry, with a focus on genotype-phenotype correlations and predictive disease severity markers. METHODS: A web-based registry retrospectively collected data on patients with CAPS. Experts in the disease independently validated all cases. Patients carrying NLRP3 variants and germline-mutation-negative patients were included. RESULTS: 136 patients were analysed. The median age at disease onset was 9 months, and the median duration of follow-up was 15 years. Skin rash, musculoskeletal involvement and fever were the most prevalent features. Neurological involvement (including severe complications) was noted in 40% and 12% of the patients, respectively, with ophthalmological involvement in 71%, and neurosensory hearing loss in 42%. 133 patients carried a heterozygous, germline mutation, and 3 patients were mutation-negative (despite complete NLRP3 gene screening). Thirty-one different NLRP3 mutations were recorded; 7 accounted for 78% of the patients, whereas 24 rare variants were found in 27 cases. The latter were significantly associated with early disease onset, neurological complications (including severe complications) and severe musculoskeletal involvement. The T348M variant was associated with early disease onset, chronic course and hearing loss. Neurological involvement was less strongly associated with V198M, E311 K and A439 V alleles. Early onset was predictive of severe neurological complications and hearing loss. CONCLUSIONS: Patients carrying rare NLRP3 variants are at risk of severe CAPS; onset before the age of 6 months is associated with more severe neurological involvement and hearing loss. These findings may have an impact on treatment decisions.