Pigmented Villonodular Synovitis Presenting as Unilateral Hearing Loss: Review of the Literature and Case Report

Background/Aims: To review the existing literature on pigmented villonodular synovitis (PVNS) of the temporomandibular joint (TMJ) and report a rare case of PVNS of the TMJ presenting with unilateral hearing loss. Methods: Review of the existing literature and a description of personal experience with PVNS of the TMJ presenting with unilateral hearing loss. Results: Review of the existing literature revealed 76 reported cases of PVNS of the TMJ. The most common presenting symptom was of a slowly enlarging mass or swelling of the preauricular area, with dysfunctional TMJ also frequently reported. All patients underwent surgical excision with some pursuing radiation as adjuvant therapy. Presented Patient: A 46-year-old man presented with several months of unilateral subjective hearing loss and aural fullness. Imaging revealed a mass centered along the superior TMJ with expansion through the squamous temporal bone and extra-axial intracranial extension into the middle cranial fossa. Imaging characteristics and fine-needle aspiration biopsy were consistent with PVNS. Intervention: The patient underwent near-total excision of the mass via frontotemporal craniectomy and lateral temporal bone resection. Follow-Up: At the 16-month follow-up there was no evidence of disease recurrence. Conclusion: PVNS of the TMJ represents a rare entity that can present with a variety of symptoms including unilateral hearing loss.