[The otologic manifestation of Wegener's granulomatosis--review of contemporary achievements in diagnostics and treatment].
OBJECTIVE
Wegener granulomatosis (WG) presents nasal, laryngeal and otologic symptoms as an initial signs of the disease and finally can affect the ENT region in 3/4 patients. The authors want to analyze 7 cases of WG with early aural symptoms, connected with the diagnostics dilemma, discuss the problems of otologic manifestation in WG and present the newest concepts of diagnostics and treatment of this condition.


METHODS
All patients were treated in the ENT University Department in Poznań in the years 2002-2010. Three patients manifested isolated aural symptoms at the time of the initial clinical presentation (otitis media with effusion, facial palsy and sensorineural profound hypoacusis), whereas in four aural problems were the first, the most expressed and pronounced. In the last four cases neither the initial stage of respiratory nor renal failure were the immediate reason for applying to the doctor, but in two the rapid onset of hypoacusis combined with purulent discharge and facial nerve palsy and in two quick progression of mixed type hypoacusis.


RESULTS
The age of the patients ranged from 32 to 46. The outcome of initially otologic cases, which developed generalized form of WG was poor (first died after 2 months, second after 7 days, third in a two-month observation had the pulmonary insufficiency, fourth in one month had severe renal failure), whereas other 3 patients with localized form of the disease have been successfully under control for 3-5 years.


CONCLUSIONS
As Wegeners granulomatosis in some cases presents ENT problems as an initial sign, it is important to take it into consideration in atypical inflammatory states of the ear. The otologic onset of WG is very insidious and prompt diagnosis in early stage of disease is a challenge.
Klinika Otolaryngologii i Onkologii Laryngologicznej, Uniwersytet Medyczny im. K. Marcinkowskiego w Poznaniu, Poland.
Małgorzata
M
Wierzbicka
Mariusz
M
Puszczewicz
Anna
A
Bartochowska
Witold
W
Szyfter
D000328:Adult; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D005158:Facial Paralysis / Q000188:drug therapy* / Q000209:etiology*; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000175:diagnosis*; D006319:Hearing Loss, Sensorineural / Q000209:etiology*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010034:Otitis Media with Effusion / Q000188:drug therapy* / Q000209:etiology*; D000069283:Rituximab
D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab