IgG4-Related Disease Presenting as an Erosive Inflammatory Process of the Middle Ear and Mastoid

Orr Dimitstein ¹ Daniel M Kaplan ¹ Ohad Hilly ² Elisheva Pokroy-Shapira ³ Yulia Strenov ⁴

¹Department of Otolaryngology - Head and Neck Surgery, Soroka University Medical Center and the Faculty of Health Sciences, Ben Gurion University in the Negev
²Otolaryngology Department, Rabin Medical Center, Sackler Faculty of Medicine, Tel Aviv University
³Rheumatology Unit, Rabin Medical Center, Sackler Faculty of Medicine, Tel Aviv University
⁴Pathology Department, Rabin Medical Center, Sackler Faculty of Medicine, Tel Aviv University

A 49 year old patient was evaluated for vertigo and tinnitus persisting for several months. Otoscopy showed retraction and middle ear fluid and the head thrust exam was positive to the left side. The patient’s audiogram showed a left-sided moderate to severe mixed hearing loss. A canal wall down mastoidectomy was performed under suspicion of a cholesteatoma. Intraoperative findings included ample granulations and bone erosion of the tegmen and the horizontal semicircular canal filling the mastoid, the tympanic cavity, extending to the fallopian canal, with no evidence of a cholesteatoma.

Three months after surgery the patient continued his initial symptoms and headaches. His audiogram progressed to a left-sided profound hearing loss. Head CT showed edema in the left temporal lobe. Further evaluation included head MRI, consults from the neurosurgery, neurology and infectious disease services and performing a lumbar puncture. All these exams showed no additional findings and he was treated with a course of corticosteroids. No change in symptoms was noted during another three-month follow-up, and the patient was admitted for revision mastoidectomy. The middle ear and mastoid cavity were, once again, full of granulations which most notably, eroded the bone over the sigmoid sinus and the carotid canal.

A revision of the histopathological slides from the mastoid granulations demonstrated storiform fibrosis with dense lymphoplasmacytic infiltrates that stained positive (25-30 cell/hpf) for IgG4 plasma cells. A course of Prednisone and Methotrexate was started with impressive clinical and radiological improvement.

The case we present is an example of a relatively uncommon manifestation of IgG4 related disease, which was treated initially as chronic otitis media, but was unresponsive to repeated surgery. In our presentation we review the literature regarding otolaryngology manifestations of this systemic disease and suggest conditions where the possibility of this diagnosis should be explored.