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The Forgotten Syndrome? Four Cases of Gradenigo’s Syndrome and a Review of the Literature

ABSTRACT Background: Gradenigo’s Syndrome (GS) is defined as the clinical triad of acute otitis media, ipsilateral sixth nerve palsy, and pain in the distribution of the first and the second branches of the fifth nerve. The purpose of this study is to review the literature and report 4 cases of GS. Methods: The study is a retrospective case series and a review of the literature. Four consecutive patients (aged 5–70 years) treated by otolaryngologists and ophthalmologists for GS in the Capital region of Denmark from 2003 to 2015 are presented. Diagnosis is based on the clinical triad, and in 3 of 4 patients, neuroimaging supports the diagnosis. Follow-up was continued until both the sixth nerve palsy and the ear infection had resolved. Diagnostic work-up and treatment profile are described. Results: In 3 of our 4 reported patients, the presentation of GS was classic with a history of acute otitis media and ipsilateral sixth nerve palsy. One case presented as a chronic case with a sixth nerve palsy secondary to chronic suppurative otitis media (CSOM), with a relapse 6 years later. Conclusion: GS is a rare and potentially life-threatening complication to otitis media. GS can present in an acute and chronic form, and should be a differential diagnosis in the workup of unexplained sixth nerve palsy.