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At a glance

A rare postinfectious syndrome caused by the extension of a middle ear infection (otitis media and/or mastoiditis) to the petrous apex leading to abducens palsy. A triad of symptoms consisting of periorbital unilateral pain related to trigeminal nerve involvement, diplopia due to sixth nerve palsy and persistent otorrhea, associated with bacterial otitis media with apex involvement of the petrous part of the temporal bone (petrositis). The classical syndrome related to otitis media has become very rare after the antibiotic era.

Synonyms

Gradenigo Petrosum Syndrome; Gradenigo Triad; Gradenigo-Lannois Syndrome; Abducens Nerve Palsy-Petrous Osteomyelitis Syndrome; Petrous Osteomyelitis-Abducens Nerve Palsy Syndrome; Petrous Apicitis Disease.

History

Guiseppe Conte Gradenigo, an Italian Otolaryngologist (1859-1926), reported in 1904 a triad of symptoms consisting of periorbital unilateral pain related to trigeminal nerve involvement, diplopia due to sixth nerve palsy and persistent otorrhea, associated with bacterial otitis media with apex involvement of the petrous part of the temporal bone (petrositis).

Incidence

Incidence has widely decreased since the development of antibiotics.

Genetic inheritance

No genetic inheritance.

Pathophysiology

Caused by extension of an infection of the middle ear, mastoid sinus, or both to the petrous apex that occurs because of extensive pneumatization and the presence of bone marrow. The proximity of the venous sinuses to the petrous apex is the reason for the historically high incidence of venous sinus thrombosis associated with petrous apicitis. Petrous apicitis is believed to result when organisms, typically pseudomonas, become trapped within the complex air cell system of the petrous apex. Blockage of this air cell system may result from acute or chronic inflammation or from mechanical blockage by an obstructing lesion. The inflammation may extend into the Dorello canal, which contains cranial nerve (CN) VI and the Gasserian ganglion (CN V). Inflammation of the canal produces the triad of symptoms recognized by Gradenigo: lateral rectus (CN VI) palsy, retroorbital pain, and otorrhea.

Diagnosis

Based on the clinical findings of a coexisting ear infection and ipsilateral paralysis of the abducens nerve and otorrhea. Severe pain in the area supplied by the ophthalmic branch of the trigeminal nerve is generally associated. The MRI can show focal enhancement of the meninges over the petrous apex and extension of the infection into Meckel cave (the cavity that harbors the trigeminal ganglion).

Clinical aspects

Pain is typically severe, centered within the ear, burning or throbbing, worse at night, aggravated by jaw movement, pressure over the tragus, and traction upon the auricle. Pain can be observed simultaneously in the frontal and parietal regions. In the largest series of patients with petrous apicitis, the most common presenting symptoms were hearing loss in 60% of patients, deep pain (50%), CN VIII involvement (50%), ...

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