Makindo Medical Notes

Common in diabetics, patients on high-dose steroids, and immunosuppressed individuals (e.g. HIV/AIDS).

About Gradenigo's Syndrome

• Gradenigo's Syndrome is a rare neurological condition characterized by the classic triad of:
  • Suppurative otitis media
  • Trigeminal nerve pain (usually V1 distribution)
  • Abducens (CN VI) nerve palsy
• First described by Giuseppe Gradenigo in 1907.
• Results from infection spreading to the petrous apex of the temporal bone, affecting adjacent cranial nerves.

Aetiology

Usually secondary to untreated or chronic suppurative otitis media, with infection spreading to the petrous apex → apical petrositis.

• Petrous Apex Involvement: infection/inflammation involving trigeminal ganglion (CN V) and abducens nerve (CN VI).
• Occasional extension: to optic nerve (CN II) or other skull base cranial nerves.

Clinical Presentation

• Ear Symptoms: chronic/recurrent otitis media, otorrhea, hearing loss.
• Facial Pain: trigeminal neuralgia-like pain in forehead/eye (V1 distribution).
• Diplopia: due to abducens palsy (lateral rectus weakness).
• Systemic features: fever, malaise if acute infection ongoing.

Complications if Untreated

• Meningitis or brain abscess.
• Spread to skull base cranial nerves IX, X, XI → dysphagia, hoarseness, shoulder weakness.
• Parapharyngeal or prevertebral abscess → airway compromise.
• Sympathetic plexus involvement → Horner’s syndrome (ptosis, miosis, anhidrosis).

Differential Diagnoses

• Cavernous sinus thrombosis (infective, multiple cranial nerves).
• Tolosa-Hunt syndrome (granulomatous inflammation, steroid responsive).
• Carotid aneurysm/dissection.
• Skull base tumours/metastases.
• Multiple sclerosis or other demyelinating lesions.
• Herpes zoster ophthalmicus.

Investigations

• Bloods: FBC, U&E, LFTs, CRP, ESR.
• Microbiology: Ear swabs/CSF cultures if meningitis suspected.
• Imaging:
  • MRI/CT petrous temporal bone: key to show apical petrositis, bone erosion, inflammation.
  • MR venography: if cavernous sinus thrombosis suspected.
• CSF analysis: if meningitis suspected.

Management

Unlike Tolosa-Hunt, steroids are not primary therapy. Management is infection-focused.

• Antibiotics: IV broad-spectrum antibiotics (covering Gram-positive, Gram-negative, anaerobes) tailored to cultures.
• Surgical intervention: mastoidectomy or apical petrosectomy if abscess or persistent disease.
• Adjunctive steroids: occasionally used to reduce inflammation alongside antibiotics, but not first-line alone.
• Supportive: analgesia, physiotherapy for ophthalmoplegia, seizure management if intracranial spread.

Prognosis

• Favourable if diagnosed early and treated with antibiotics ± surgery.
• Delay risks meningitis, intracranial abscess, and long-term cranial nerve palsies.

Conclusion

Gradenigo’s Syndrome is a rare but serious complication of otitis media. It should be suspected in patients with ear infection, trigeminal neuralgia-like pain, and abducens palsy. Early imaging and antibiotics are lifesaving; steroids only play a limited adjunctive role.

References

• Gradenigo G. Il neurologo in facoltà otorinolaringoiatrica. 1907.
• De Angelis F, et al. Clinical characteristics and outcomes of Gradenigo's syndrome. J Neurol Neurosurg Psychiatry. 2012;83(5):525-528.
• Wolff SM, et al. Gradenigo’s Syndrome: A case report and literature review. Neurol India. 2012;60(2):252-254.
• Osborn AG. Diagnostic Imaging: Brain. Elsevier; 2015.