Case Presentation: 71-year-old African American female with history of chronic right ear infection/mastoiditis, hypertension, hyperlipidemia, stroke with residual expressive aphasia, diabetes mellitus type II and atrial fibrillation, presented to the ED for diplopia and pain to the right face. Initially the diagnosis of giant-cell arteritis was entertained and temporal artery biopsy showed no evidence of vasculitis. The patient was treated with high-dose steroids at that time and was discharged on prednisone.Following discharge patient followed up with ENT as outpatient and was treated with ciprofloxacin ear drops for chronic ear infection. Two days after discharge patient returned to the emergency room after sustaining a fall at home and was complaining of worsening slurred speech and left-sided facial droop. On exam she had right ear discharge and sixth nerve palsy. Computed tomography (CT) of the head showed no evidence of acute stroke but showed opacification of the right mastoid and middle ear. Magnetic resonance imaging (MRI) of the brain showed no evidence of acute stroke, but there was evidence of severe infection that was extending into the ventricles with evidence of ventriculitis, as well as osteomyelitis of the skull base, inflammatory changes of the right middle ear and mastoid with other extensive changes including the clivus and the right carotid region. Diagnosis of Gradenigo’s syndrome with ventriculitis was made and broad-spectrum antibiotic treatment was started with Vancomycin, Cefepime, Flagyl and Voriconazole. ENT evaluated the patient and there was no surgical intervention needed. Cerebral spinal fluid did not grow any fungal, viral, tubercular or bacterial culture. Repeat MRI of the brain demonstrated no improvement with persistent foci of restricted diffusion within the occipital horns compatible with purulent material, and ventriculitis. Also had unchanged, right mastoid opacification, abnormal enhancement involving the clivus, right occipital condyles and inferior occipital bone, as well as petrous abscesses and thrombosis of the right sigmoid sinus. After extended conservative treatment patient improved clinically.

Discussion: Gradenigo’s syndrome is a terminology often used in the past to describe the triad of suppurative otitis media, ipsilateral sixth (abducens) cranial nerve palsy and facial pain in the distribution of the fifth (trigeminal) cranial nerve. Gradenigo’s syndrome is rare, and the diagnosis is easily missed.

Conclusions: It is important to highlight that Gradenigo’s syndrome can masquerade as other entities like vasculitis and also can advance to other more severe disease like ventriculitis. In cases with chronic otitis media or six nerve palsy we should consider Gradenigo’s syndrome as a differential diagnosis. In a diabetic patient concern is raised regarding pseudomonas aeruginosa and rhinocerebral mucormycosis.