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Summary: To calculate the prevalence of sinonasal and ear involvement in an Erdheim‐Chester disease (ECD) population, to describe the different ear, nose and throat (ENT) manifestations and to study the association between ENT involvement, other organ involvement, and BRAF mutations. We led a retrospective monocentric study in the national referral center for ECD. One hundred and sixty‐two patients with ECD and ENT data were included between January 1, 1980 and December 31, 2020. Ear and nose clinical and radiological findings were noted. We described and studied the prevalence of ENT involvement in ECD population. The association between sinonasal and ear involvement, other organ involvement, and BRAF mutations was calculated. The prevalence of ENT manifestations is around 45%. No clinical rhinologic or otologic signs were specific to ECD. Sinus imaging was abnormal in 70% of cases. A bilateral maxillary sinus frame osteosclerosis was highly specific of ECD. Associations were found between the sinus MRI imaging type and BRAF status, central nervous system involvement, cerebellum involvement and xanthelasma. Sinonasal or ear involvement is frequent in ECD and has specific imaging features for sinuses. Trial registration: #2011‐A00447‐34. [ABSTRACT FROM AUTHOR]

Background: Paranasal sinus fungus balls (PSFB) are a common form of surgically treatable, noninvasive mycosis. To date, no guidelines have standardized PSFB treatment or management of difficult cases (eg, immunocompromised or fragile patients). The clinical consensus statement presented herein aims to provide a comprehensive management guide to PSFB based on current evidence. Methods: A multidisciplinary, international panel of 19 specialists judged statements in 3 rounds of a modified Delphi method survey. Statements encompassed the following PSFB management issues: definition, diagnostic workup; treatment indications and modalities; and follow‐up. Otolaryngologists, maxillofacial surgeons, infectious disease specialists, and transplant physicians were considered the target audience. Results: Among the 23 statements, 7 reached strong consensus and 16 reached consensus. Consensus was reached on the definition, diagnosis, and treatment modalities for PSFB. Postoperative follow‐up modalities and scenarios with bacterial superinfection were the most debated issues. Conclusion: Until further data are available, these points provide a framework for the management of PSFB. Moreover, PSFB should be considered a noninvasive mycosis that is not necessarily symptomatic or related to odontogenic conditions. Although diagnosis may be incidental, endoscopy and single imaging (computed tomography or magnetic resonance imaging, with distinctive features) are required for diagnosis, whereas contrast medium would allow for differential diagnosis. Although treatment of PSFB should be considered mandatory before sinus augmentation and is recommended for symptomatic patients, immunosuppressed patients, or patients with planned immunosuppression, watchful waiting could be considered for asymptomatic patients with chronic rhinosinusitis who are provided with appropriate advice and assessment. [ABSTRACT FROM AUTHOR]