Wegener's Granulomatosis of the ear

Wegener’s Granulomatosis with Otological Disorders as Primary Symptoms

“…How ever, serous otitis media may occur as an ini tial sign of Wegener's granulomatosis, as al ready reported [2,7,8], Our cases illustrate that Wegener's granulomatosis may also manifest first as otitis externa or sensorineu ral hearing loss soon followed, as in the 3rd case, by facial palsy; thus, otological mani festations may precede involvement of other organ systems for several years.…”

“…In several respects, the course of the disease is similar to that of malignant external otitis [2], which mani fests itself in diabetics by progressing, necro tizing and destructive affection of the tem poral bone, associated with cranial nerve palsy, a poor general condition and high ESR [6], This condition is due to Pseudomonas infection, which was demonstrated a few times in swabs from the ear in our material. It is important to distinguish between these two states, as the treatment differs decisive ly.…”

“…In addition, the diagnosis of tuberculous otitis should also be ruled out in any patient with a persistent otalgia not responding to the conventional forms of therapy [12], Serous Otitis media. This condition is caused by nasopharyngeal ulceration and ob struction of the Eustachian tube; destruction of the middle ear and mastoid with 'chronic otitis media' caused by actual involvement of the middle ear and mastoid cavity by Wegener's granulomatosis [2,7,8].…”

“…The cause of the deficit is unknown. Suggested causes include granu loma pressing on the acoustic nerve [2,8], vasculitis of the cochlear vessels [14], dispo sition of immune complexes in the cochlea [12] or involvement of the vessels of the endolymphatic sac [11], Facial Palsy. Facial palsy may respond to immunosuppressive treatment [3], Experi mental studies on dogs suggest that facial palsy may be caused by a localized immuno logic or hypersensitivity reaction [15].…”

“…More re cently, it has been said that a complete or typical histological picture is not always es sential for a diagnosis of Wegener's granulo matosis and that, if the clinical picture is suggestive of the diagnosis, a less typical his tological picture can be acceptable [2,9] and treatment started. It is well recognized that an early diagnosis and treatment can lead to a marked improvement in the patient's mor bidity and survival [2]. However, no mini mum requirements for the diagnosis of We gener's granulomatosis based upon clinical findings have yet been established.…”