A 26 year-old female presents with therapy-resistant otitis media acutissima on both
sides and an upper airway infection. Previous, she has been treated with grommets,
iv antibiotics and prednisolone. After getting better for a short period of time,
the hearing loss proceeded after leaving the hospital.
In the clinical examination, the tympanic membrane was inflamed and with grommets
on both sides, she presented as well a rhinosinusitis. The audiometry revealed a loss
of the inner ear function till 40 – 70 dB on both sides. We immediately started iv-prednisolone
again and changed the antibiotics, nevertheless there was no remission.
Next step was a serologic testing for neurotropic germs as well as a search for autoimmune
antibodies. The findings revealed a highly positive amount of cANCA, additionally
a biopsy of the nasal mucosa showed a necrotizing inflammation. We diagnosed granulomatosis
with polyangiitis (GPA).
The therapy with cortisone was intensified and the patient improved immediately. Further
diagnostics and therapy was hold by the rheumatologists who, to that moment, didn't
find any other manifestations.
Due to a resting deafness on the right ear she was provided with a cochlear implant.
At the same time, the CT scan of the thorax revealed infiltrations in both lungs.
A treatment with Rituximab lead to a remission.
Conclusion:
Age, localization and severity of the case are not typical for GPA. Nevertheless,
it should always be considered as a differential diagnosis.
