Temporal Bone Paraganglioma Presenting as a Bezold’s Abscess and Necrotizing Fasciitis

Background Though paragangliomas of the temporal bone are usually benign histopathologically, locally aggressive behavior can be mistaken for malignancy. Due to the consequences of associated bone erosion in areas of the temporal bone that also contain squamous epithelium, cholesteatoma can form alongside a tumor and alter the expected symptom profile of a tympanic paraganglioma. The present report describes a case of a Glasscock–Jackson’s grade IV tympanic paraganglioma that led to cholesteatoma formation, a Bezold’s abscess and necrotizing fasciitis of the neck.

Case Presentation A 66-year-old woman presented to the emergency room with a 2-month history of progressive right ear pain, which had recently extended to involve her neck. She was a poor historian, though she reported frequent foul smelling drainage from the right ear during this time, right-sided hearing loss, tinnitus, and limited neck range of motion. She denied vertigo, fevers, or weight loss. Her history was notable for cervical cancer for which she was undergoing chemotherapy. On examination, there was purulent debris and polypoid tissue obstructing the lateral aspect of the ear canal. Fluctuance was palpated over the mastoid tip, and inferiorly, the skin of the neck was erythematous and edematous down to the clavicle with distinct areas of crepitus palpable. Contrast-enhanced computed tomography of the neck and temporal bone were performed, which showed erosion of the tympanic, mastoid, and petrous portions of the temporal bone, as well as a large multiloculated abscess in the right neck with air tracking from the skull base to the clavicle. No clear jugular bulb erosion was seen, though the carotid canal was partially eroded in the middle ear. Operative intervention revealed an abscess in the infra-auricular soft tissue as well as necrotic soft tissue deep to the sternocleidomastoid tracking down anteriorly and inferiorly to the sternal notch. A mastoidectomy was performed, revealing a red, pulsatile mass filling the EAC middle ear and mastoid. In areas of bone erosion, cholesteatoma debris and purulence were identified. Histopathology revealed the mass to be a paraganglioma.

Conclusion Tympanic paragangliomas are the most common benign primary middle ear neoplasm. They are generally slow growing, and the associated symptom profile is usually auditory. The present case is an example of a rare case in which associated cholesteatoma, abscess, and necrotizing fasciitis developed, demonstrating that relatively insidious tumor growth can ultimately have significant consequences.