Unmasking Villaret's syndrome: a diagnostic challenge of glomus jugulare mimicking mastoiditis

Villaret's syndrome involves unilateral palsy of cranial nerves IX to XII, often with Horner's sign, commonly caused by benign neoplasms or vascular issues. This report highlights glomus jugulare-induced Villaret syndrome, stressing careful evaluation for persistent otologic symptoms.

A 43-year-old woman with a history of chronic suppurative otitis media (CSOM) presented with recurrent ear discharge, pain, and severe mixed hearing loss. Despite a left tympanoplasty, symptoms persisted, leading to a canal wall down mastoidectomy. Six months later, she developed facial numbness, hoarseness, and dysphagia. Physical exam showed unilateral cranial nerve palsies with Horner's syndrome, consistent with Villaret's syndrome. Imaging confirmed a glomus jugulare tumor. After digital subtraction angiography and balloon occlusion test, surgical resection was performed. Postoperatively, she showed gradual cranial nerve recovery and remains under follow-up.

Jugular foramen syndrome involves unilateral cranial nerve palsies, including Villaret's syndrome, where cranial nerves IX-XII and cervical sympathetic fibers are affected. Most often linked to benign neoplasms like paragangliomas, glomus jugulare tumors are slow-growing, often benign, and predominantly affect middle-aged females. Diagnosis relies on MRI's "salt-and-pepper" imaging, with high-resolution CT for bony involvement. Treatment, including surgery with embolization, aims to reduce cranial nerve deficits. Radiotherapy and radiosurgery are options for non-surgical candidates or recurrence cases.

This case underscores the diagnostic challenges of Villaret's syndrome caused by glomus jugulare. Initially masked by symptoms of chronic otitis media and mastoiditis, cranial nerve deficits eventually revealed the underlying tumor, highlighting the importance of vigilant assessment and imaging in persistent otologic cases.