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Pathology of Granulomatosis with Polyangiitis (Wegener's Granulomatosis) of the Ear

Dr Sampurna Roy MD



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 Granulomatosis with Polyangiitis (Wegener's granulomatosis) is defined as a granulomatous inflammation of the upper and lower respiratory tract and systemic vasculitis of small and medium sized vessels which is often accompanied by a necrotizing glomerulonephritis.

The etiology of the disease is still unknown.

According to recent literature, involvement of the ears, nose, and throat can in many instances be the only early manifestation of the disease.

It is important that audiologists be aware of the early signs in order to make appropriate referral for treatment, particularly since they are usually among the first to see these patients. 

Common sites of involvement include the middle ear, nose and sinuses and subglottis.

Granulomatous lesions in the nose, paranasal sinuses and pharynx are very characteristic and often mark the beginning of the disease. 

Visit: Granulomatosis with Polyangiitis of the Lung

Otologic manifestations of Granulomatosis with Polyangiitis : 

- A painful otitis media with combined sensorineural hearing loss may be the first sign of this disease in some cases ;

- Tympanic membrane perforation, 

- Perforation of ear lobes, 

- External otitis ,

Diagnosis is made by a combination of physical examination, laboratory studies and tissue biopsy. 

Differential diagnosis:  Rheumatoid arthritis ; polyarteritis nodosa.


Further reading

Wegener's granulomatosis: current trends in diagnosis and management.

Head and neck manifestations of Wegener's granulomatosis.

Wegener's granulomatosis presenting as otomastoiditis. A case report.

An unusual case of otitis media.

How can the diagnostic value of head and neck biopsies be increased in Wegener's granulomatosis: a clinicopathologic study of 49 biopsies in 21 patients .

Wegener's granulomatosis in the head and neck region .

Protracted superficial Wegener's granulomatosis.

Cutaneous Wegener's granulomatosis: a variant or atypical localized form? 

Hearing impairment and earache as initial symptoms of Wegener's granulomatosis.

Otologic signs and symptoms as first manifestations of Wegener's granulomatosis with very severe clinical course--review of the literature and case report.

Necrotising granulomatous inflammation with middle ear the only localisation.


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Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)






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