Neuro / Head & Neck for Friday, April 11th, 2025

Contributed by Morehouse School of Medicine, Cincinnati Children's Hospital Medical Center
Hadiya Haq MS2, and Jungwhan John Choi MD.

History

11-year-old boy presenting to the emergency department with ear drainage, headache and blurry vision.  

Images (Click any image to enlarge)

Question

What is the classic triad of symptoms associated with Gradenigo Syndrome?

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Correct answer

Otorrhea, facial pain, and abducens nerve palsy

Discussion

   Gradenigo Syndrome is a rare clinical presentation of acute otomastoiditis complicated by extension of untreated infection to a pneumatized petrous apex, resulting in a suppurative petrous apicitis. The classic triad of symptoms include: 1) otorrhea, 2) deep facial / retro-orbital pain (CNV), and 3) lateral rectus palsy (CNVI), although not all patients display this classic triad. Additional symptoms may include headaches, hearing loss, tinnitus, photophobia, nausea, vomiting, and other vision or hearing disturbances.
   Cranial neuropathies are related to location of the petrous apex and direct spread of infection / inflammation to Meckel’s cave (CNV), Dorello’s canal (CNVI) and sometimes the internal auditory canal (CNVII and CNVIII). Common implicated pathogens include Staphylococcus aureus, Pseudomonas aeruginosa, Klebsiella pneumoniae, Mycobacterium tuberculosis, and Streptococcus pyogenes. Cranial nerve involvement can take 2-3 months to develop after symptom onset dependent on the organism and rate of infectious spread throughout the skull base. 
   Gradenigo Syndrome primarily affects children, with a median onset age of 12 years and a mortality rate of up to 2.6%. With prompt identification and treatment, typically intravenous antibiotics alone, most patients recover within 4-6 weeks. Surgical intervention (e.g. simple vs radical mastoidectomy) may be necessary if presenting symptoms are severe or presentation is complicated by intracranial complications, such as an intracranial abscess as in our presenting case (arrows). 
   Imaging identification and recognition are important for diagnosis. CT is valuable for identifying mastoid and pneumatized petrous apex opacification as well as bony erosion of the petrous apex trabeculae and/or cortex as depicted in our case (arrowheads). MRI is useful for detecting meningeal irritation (arrows), cranial nerve involvement, abscess formation (arrows), skull base osteomyelitis, as well as cavernous sinus and/or dural sinus thrombosis, which would guide clinical management.


Differential diagnosis

  • Variant asymmetric petrous apex pneumatization and/or opacification
  • Cholesteatoma
  • Cholesterol granuloma
  • Tolosa-Hunt Syndrome (clinical presentation)
  • Neoplasms (e.g. sarcomas, LCH)


Additional images

References

  • - Bonavia, L., & Jackson, J. (2022). Gradenigo Syndrome in a 14-Year-old Girl as a Consequence of Otitis Media With Effusion. Journal of Neuro-Ophthalmology: The Official Journal of the North American Neuro-Ophthalmology Society, 42(1), e408–e409. https://doi.org/10.1097/WNO.0000000000001278 - Taklalsingh, N., Falcone, F., & Velayudhan, V. (2017). Gradenigo’s Syndrome in a Patient with Chronic Suppurative Otitis Media, Petrous Apicitis, and Meningitis. American Journal of Case Reports, 18, 1039–1043. https://doi.org/10.12659/AJCR.904648 - Horache, K., Jidal, M., ElOuali, I., Saouab, R., & Elfenni, J. (2024). Insights into Gradenigo syndrome: Case presentation and review. Radiology Case Reports, 19(11), 5442–5446. https://doi.org/10.1016/j.radcr.2024.08.002 - Ali, Z., Alhantoosh, S., Albaqal, Z., Alkhudaimi, R., Alharbi, A. S., & Hamad, T. A. (2024). Gradenigo Syndrome: A Case Report of a Rare Complication of Otitis Media. Cureus, 16(1), e51865. https://doi.org/10.7759/cureus.51865 - Jensen, P. V. F., Avnstorp, M. B., Dzongodza, T., Chidziva, C., & von Buchwald, C. (2017). A fatal case of Gradenigo’s syndrome in Zimbabwe and the Danish-Zimbabwean ENT collaboration. International Journal of Pediatric Otorhinolaryngology, 97, 181–184. https://doi.org/10.1016/j.ijporl.2017.04.014 - Quesada, J., Kong, A., & Tweddle, E. (2021). An unusual case of acute otitis media resulting in Gradenigo syndrome: CT and MRI findings. Radiology Case Reports, 16(12), 3903– 3907. https://doi.org/10.1016/j.radcr.2021.09.026 - Rossor, T. E., Anderson, Y. C., Steventon, N. B., & Voss, L. M. (2011). Conservative management of Gradenigo’s syndrome in a child. Case Reports, 2011(apr19 1), bcr0320113978–bcr0320113978. https://doi.org/10.1136/bcr.03.2011.3978 - Demir, B., Abuzaid, G., Ergenc, Z., & Kepenekli, E. (2020). Delayed diagnosed Gradenigo's syndrome associated with acute otitis media. SAGE open medical case reports, 8, 2050313X20966119. https://doi.org/10.1177/2050313X20966119