I bring the following case to discussion and would appreciate your thoughts on how to manage this difficult case:
This is a 63-year-old female patient from Mozambique who has had recurrent episodes of bilateral otorrhea for about ten years. In 2013 she underwent bilateral otologic surgery in another hospital. According to previous medical report, CT scan of the temporal bones was performed before surgery and revealed: "bilateral middle ear filling with destruction of the ossicular chain. Erosion of the otic capsule on the right side at the level of the cochlear basal turn and vestibule". According to the same clinical record, she was submitted to simultaneous bilateral mastoidectomy. The middle ear was filled with "soft tissue" and a sample was sent to histopathological analysis whose result was inconclusive. Microbiological analysis (bacteriological and mycological) was also negative.
She was sent to our hospital in 2018 and by that time she was already bilaterally severely deafened.
Otoscopy revealed bilateral stenosis of the membranous portion of the external auditory canal, making it impossible to visualize the tympanic membrane. There were no inflammatory exudates. The retro-auricular regions had no inflammatory signs and operative scars were present bilaterally.
She performed pure-tone audiometry that showed bilateral profound sensorineural hearing loss. A recent CT scan of the temporal bones revealed bilateral complete filling of both mastoid and middle ear spaces with nonspecific soft tissue mass and extensive demineralization / erosion of middle ear structures, labyrinth and otic capsules (images in attached files).
She has a pacemaker which makes it difficult to perform magnetic resonance imaging.
Blood analysis showed normal hemogram, renal and liver functions. Analysis targeting autoimmune diseases revealed a positive ANA (antinuclear antibodies) with centromere pattern and a small monoclonal IgG kappa peak. Serum IgG subclasses were within the normal range. Rheumatoid factor, ANCA (anti-neutrophil cytoplasmic antibodies), ESR (erythrocyte sedimentation rate) and ACE (angiotensin-converting enzyme) levels were normal. Serological testing for HIV, Hepatitis B and C and syphilis were negative. Urinalysis was normal.
This patient was recently sent to a rheumatology consultation and is still being studied (autoimmune disease? IgG4 related disease?).
For now we are considering surgery to perform only tissue biopsy that could help targeting diagnosis and treatment.
In the long term, what is your advice to this situation (considering its aggressive and destructive behavior)? Any thoughts on differential diagnosis?
Thank you for your help