direct invasion or metastasis of malignant lymphoma in the middle ear is rare and the clinical features remain unknown [ 1 , 2 ] . we treated a case of non - hodgkin b - cell lymphoma ( nhbl ) in the middle ear presenting as recalcitrant otitis media and mastoiditis associated with transverse sinus thrombosis .
the patient was diagnosed 6 years after the initial symptoms and was treated successfully with surgery and chemotherapy .
we report the clinical characteristics of a rare case of nhbl in the middle ear and review the literature .
a 57-year - old woman presented with chronic temporal headache and right tinnitus , and the symptoms gradually aggravated .
conservative treatment continued at the local otolaryngology clinic based on a diagnosis of otitis media .
slight consciousness disturbance , slight fever and auditory disturbance were noted 1 year after the initial symptoms , and the patient was referred to our department .
neurological findings showed slight consciousness disturbance , and an audiometric hearing test demonstrated 30 db in the right ear , diagnosed as a mild hearing impairment .
magnetic resonance imaging ( mri ) showed edematous brain swelling in the right temporal lobe and right tentorium and dural enhancement around the transverse sinus ( fig .
mri showed a heterogeneous mass lesion in the right middle ear and mastoid air cells with dural enhancement around the lesion ( fig .
she was diagnosed with recalcitrant otitis media invading the mastoid associated with transverse sinus thrombosis and was treated with antibiotics , hyperosmotic fluids , steroids and repeated external drainage of the middle ear .
symptoms of temporal headache , auditory disturbance , slight fever and radiological findings of brain swelling did not disappear , and remission and exacerbation of these symptoms occurred repeatedly . consciousness disturbance occurred again , and chronic temporal headache gradually aggravated 5 years after the first examination in our department .
mri revealed enlargement of the edematous swelling around the right transverse sinus , and the enhanced lesion was also enlarged ( fig .
open drainage of the middle ear with mastoidectomy was performed 6 years after the initial symptoms .
granulation had invaded the eardrum and stapes , and the auditory tube was also filled with granulation ( fig .
pathological examination demonstrated diffuse proliferation , predominantly consisting of small to medium - sized lymphoid cells with slightly irregular nuclei ( fig .
1f ) , which were positive for cd20 , cd79 and bcl-2 , suggesting malignant lymphoma , b - cell type , classified as extranodal marginal zone b - cell lymphoma . computed tomography and blood and bone marrow evaluation did not show other lesions of malignant lymphoma , suggesting that the otitis media lesion was the primary lesion .
she was treated with rchop ( rituximab combined with cyclophosphamide , doxorubicin , vincristine and prednisolone ) chemotherapy .
malignant lymphoma can spread to all areas of the body , including the head and neck .
involvement of the temporal bone as part of generalized lymphoma has been reported ; however , clinical evidence of temporal bone or middle ear involvement is unusual [ 3 , 4 , 5 , 6 ] .
presentation with otoneurological signs prior to systemic involvement of a lymphoproliferative disease is also unusual . to our knowledge ,
only 10 cases of malignant lymphoma originating around the middle ear have been reported ( table 1 ) [ 1 , 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 ] . in the previous 10 reports ,
the lesion was located in the middle ear in 3 , internal in 3 , and external in 4 .
facial nerve palsy was the most common initial symptom and mastoiditis was associated in about 50% of the cases .
there was no direct invasion into the intradural region and no association with sinus thrombosis in 10 cases .
our present case included the following marked characteristics : first , it was difficult to diagnose malignant lymphoma under the suspicion of intractable otitis media .
second , transverse sinus thrombosis caused by dural invasion of a tumor or spread of otogenic infection manifested neurological symptoms .
third , tentorial or dural enhancement adjacent to the transverse sinus did not decrease , and sinus occlusion continued even after remission .
fourth , rchop chemotherapy was effective , and the lesion has not recurred for 4 years .
infectious symptoms and dural enhancement showed repeated remission and abrogation under treatment for otitis media with antibiotic administration and drainage .
there was a possibility that sinus occlusion induced by infection progression might abrogate venous circulation , resulting in brain edema in the early stage , and collateral venous flow might gradually develop during the progression of sinus occlusion .
we treated a rare case of b - cell malignant lymphoma presenting with otitis media and mastoiditis associated with transverse sinus thrombosis .
the authors report no conflicts of interest concerning the case report presented herein or the findings specified in this paper .
 cerebral venous thrombosis as a manifestation of paraneoplastic angitis and otitis media , revealing non - hodgkin b - cell lymphoma ( nhbl ) , is extremely rare . a 57-year - old woman presented with headache , auditory disturbance and recalcitrant otitis media 
 . magnetic resonance imaging showed brain edema in the temporal lobe and transverse sinus thrombosis . 
 external drainage under antibiotic treatment was repeated based on a diagnosis of invasive otitis media and mastoiditis associated with infectious sinus thrombosis , but the condition deteriorated progressively . 
 open surgery for otitis media was performed 6 years after the initial symptoms and after a tumorous lesion had been detected in the middle ear . 
 pathological findings revealed nhbl . 
 we report a rare case of nhbl presenting as otitis media and mastoiditis associated with sinus thrombosis , and a literature review . 
Introduction
Case Report
Discussion
Disclosure Statement