# Outologic Manifestations of IgG4-Related Disease: Literature Review and Report of Two Cases
## 3 Case Presentations
### Case 2  
A 48-year-old male was admitted to the emergency department with complaints of left ear otalgia, dizziness, tinnitus, and hearing impairment lasting for a few weeks. On physical examination there was no evidence oforrhea. On otoscopy, retraction of the eardrum was detected, along with granulation tissue on the back wall of the ear canal. Head impulse test was positive to the left side. Blood tests were normal and bacterial cultures of ear discharge were negative.  
Audiometry revealed severe to profound mixed hearing loss of the affected ear, with reduced speech discrimination and SRT of 40 DB.  
On CT scan of the left ear (Figure 4) complete opacification of external and middle ear was noted. In addition, extensive bone erosion, involving the mastoid ear cells, the middle ear structure, and the labyrinth was detected (lateral semicircular canal and the cochlea). The patient underwent a canal wall down mastoidectomy, with the removal of granulation tissue from the mastoid and middle ear; repair of lateral canal fistula was performed. The patient required additional revision surgery due to recurrent otorrhoea and granulation tissue in the mastoid cavity.  
Post-operative management included analgesic treatment and systemic antibiotic treatment (amoxicillin with clavulanic acid). The patient was discharged on the tenth day of hospitalization with significant improvement.  
Four months later, the patient was readmitted to the emergency department with complaints of worsening dizziness and tinnitus, fatigue, or vomiting. On left ear micro-otoscopy, the mastoid cavity was filled with granulation tissue. According to neurological assessment, the patient was suspected as having cerebritis.  
Figure 3: Biopsy of left mastoid—case 1. Hematoxylin and eosin (H&E). Original magnification: A–D 100\(\times\), E 200\(\times\). Fibrous tissue with **(A)** foci of dense lymphoplasacytic infiltration. **(B,C)** Storiform fibrosis is present, as well as areas suggestive of obliterative phlebitis.  
On temporal bone CT scan, post-operative changes were recorded with notable perisinus fluid and narrowing of sigmoidal sinus. Treatment with dexamethasone and pentoxiflline was initiated, resulting in rapid improvement of symptoms. The patient was discharged with a recommendation of ambulatory MRI follow-up in the timeframe of eight weeks and instructed the continuation of pentoxiflline therapy for the same period of time.  
At that point, the initial pathologic sample was revisited. As shown in Figure 5A,B, histologic examination revealed fibrous tissue with foci of dense lymphoplasmacytic inflammation and storiform fibrosis. Upon immunostaining (Figure 5C), up to 25 IgG cells were seen per high powered field, of them <20% were IgG4 positive. Blood serum IgG levels were determined to be within normal limits. Intravenous steroid therapy (methyl-prednisolone sodium succinate) and methotrexate were initiated, resulting in good clinical response and CNS involvement improved on MRI follow-up.  
Figure 4: Pre-operative CT scan—case 2. Axial and coronal reconstruction of high-resolution temporal bone CT scan shows opacification of the mastoid air cells (right picture), and extensive bone destruction of the left mastoid bone and of the posterior wall of the external auditory canal, including the cochlea and vestibule (left picture).