{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T20:59:30Z","timestamp":1771275570084,"version":"3.50.1"},"reference-count":417,"publisher":"Georg Thieme Verlag KG","issue":"S 01","license":[{"start":{"date-parts":[[2021,4,30]],"date-time":"2021-04-30T00:00:00Z","timestamp":1619740800000},"content-version":"vor","delay-in-days":29,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Laryngorhinootologie"],"published-print":{"date-parts":[[2021,4]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>Seltene Erkrankungen der Nase, der Nasennebenh\u00f6hlen und der vorderen Sch\u00e4delbasis stellen aufgrund der niedrigen Inzidenz und den daraus resultierend eingeschr\u00e4nkten diagnostischen Kriterien sowie therapeutischen Optionen eine Herausforderung dar. Der Schwellenwert, ab dem eine Erkrankung als selten zu werten ist, liegt bei h\u00f6chstens 5 Betroffenen pro 10 000 Personen. Innerhalb dieser Erkrankungen gibt es allerdings extreme Schwankungen. So geh\u00f6ren einige seltene Erkrankungen, wie z.B. das invertierte Papillom, zu den regelm\u00e4\u00dfig diagnostizierten und therapierten Krankheitsbildern einer gr\u00f6\u00dferen Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde. Andererseits liegen bei anderen seltenen Erkrankungen und Fehlbildungen weltweit deutlich unter 100 Fallberichte vor, was die Notwendigkeit einer B\u00fcndelung der verf\u00fcgbaren Erfahrung zu Diagnostik und Therapie unterstreicht.<\/jats:p><jats:p>Die vorliegende Arbeit gibt eine \u00dcbersicht zu seltenen Erkrankungen der Nase, der Nasennebenh\u00f6hlen und der vorderen Sch\u00e4delbasis aus den \u00fcbergeordneten Bereichen Erkrankungen \/ Syndrome des olfaktorischen Systems, Fehlbildungen der Nase und Nasennebenh\u00f6hlen, Bel\u00fcftungs- und Funktionsst\u00f6rungen sowie benigne und maligne Tumore. Die Einordnung sowie Angaben zu Diagnostik und Therapiem\u00f6glichkeiten wurden unter Ber\u00fccksichtigung der aktuellen Literatur erstellt.<\/jats:p>","DOI":"10.1055\/a-1331-2469","type":"journal-article","created":{"date-parts":[[2021,4,30]],"date-time":"2021-04-30T23:32:31Z","timestamp":1619825551000},"page":"S1-S44","source":"Crossref","is-referenced-by-count":6,"special_numbering":"S","title":["Seltene Erkrankungen der Nase, der Nasennebenh\u00f6hlen und der vorderen Sch\u00e4delbasis"],"prefix":"10.1055","volume":"100","author":[{"given":"Fabian","family":"Sommer","sequence":"additional","affiliation":[{"name":"Klinik f\u00fcr Hals-Nasen-Ohrenheilkunde, Kopf- und Hals-Chirurgie, Universit\u00e4tsklinik Ulm"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2021,4,30]]},"reference":[{"key":"ref2","first-page":"1","article-title":"Pr\u00e4valenzen en und Inzidenzen seltener Krankheiten: Bibliografische Angaben","author":"I 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