© The American Journal of Case Reports, 2010; 11: 102-105 WWW. AMJCASEREP .

COM
Case Report

Received: 2009.08.07
Accepted: 2010.04.21 Management of Gradenigo syndrome in a child
Published: 2010.05.14

Charalampos Iliadis1, Greta Wozniak2, Marianna Vlychou2, Venetia Barkatsa1,

Sofia Sotirakou3, Alexandra Kunz4
1
Department of Neurosurgery, University Hospital of Larissa, Larissa, Greece
2
Department of Radiology, University Hospital of Larissa, Larissa, Greece
3
Paediatric Outpatients Word, University Hospital of Larissa, Larissa, Greece
4
Harvard University (Extention), Boston, MA, U.S.A.

Summary
Background: Gradenigo syndrome is a rare presentation of acute petrositis characterized by medial otitis, ret-
ro-orbital pain and abducens nerve palsy (CN VI palsy) affecting the lateral rectus muscle (CN VI
palsy), due to inflammation of this nerve at Dorello’s canal. Treatment usually consists of mastoid-
ectomy, and antibiotics. Successful treatments with less aggressive interventions have occasionally
been reported.
Case Report: We describe the clinical and radiological findings and the conservative management of a 14-year-old
who developed Gradenigo syndrome as a complication of otitis media, with a favorable outcome.
Conclusions: Gradenigo syndrome has a characteristic clinical presentation and requires immediate therapeu-
tic management due to potential fatal complications; however, conservative treatment can achieve
a complete recovery without major surgery.

key words: Gradenigo syndrome • abducens nerve palsy • otitis media • myringotomy • mastoidectomy

Full-text PDF: http://www.amjcaserep.com/fulltxt.php?ICID=880574

Word count: 1783
Tables: —
Figures: 2
References: 10

Author’s address: Greta Wozniak, Department of Radiology, University Hospital of Thesally, Larissa, Greece, e-mail: greta@med.uth.gr

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Background
Gradenigo syndrome is a rare complication of otitis me-
dia, more common in pediatric population, which devel-
ops as a spreading infection from the middle ear medially
to the petrous portion of the temporal bone. Prior to the
introduction of widely available antibiotics, apical petrosi-
tis has been a frequent complication of otitis media [1–4].
Gradenico syndrome, first reported in 1907, is also known as
Gradenigo-Lannois syndrome (1904), named after Maurice
Lannois, and Giuseppe Conte Gradenigo [1].
Since the increased use of antibiotics for the management
of otitis media, the prevalence of both acute and chronic
forms of the disease has decreased. The syndrome is charac-
terized by an ipsilateral paralysis of the sixth cranial nerve,
impairment of the trigeminal nerve, including retro-orbital
pain (the area supplied by the ophthalmic branch), and mid-
dle ear infection. The cranial nerve dysfunction is caused by
an osteitis and local leptomeningitis near the apex of the os
petrosum. In this region the trigeminal nerve ganglion and
the abducens nerve lie closely together, separated from the
petrous apex (PA) only by the dura mater [2,5–7]. The pe-
trous apex bears a mucosally-lined pneumatized cell system,
which communicates with the middle ear. These air cells are
a route for the spread of infection, and may be the cause of
petrositis, petrous apicitis, cavernous thrombophlebitis and
hypertrophic parechymeningitis with communicating hydro-
cephalus [6,8]. Treatment depends on the form of acute or
chronic course and the extension of the disease. Occasionally,
a neurosurgical consultation should be obtained.
The case of a young adolescent who developed Gradenigo
syndrome as a complication of otitis media is described, fol-
lowed by discussion of the relevant conservative manage-
ment for complete recovery.
Case Report
A 14-year old girl, with no significant past otological his-
tory or other neurological problems, was admitted to the
Emergency Department (ED) with 3-weeks right side otal-
gia, headache, double vision, vomiting, and refractory
to oral antibiotic therapy. On physical examination, cen-
tral perforation was noted in the tympanic membrane of
the right ear. Ipsilateral sixth nerve palsy was also present.
There were no vertigo or meningeal signs or decrease in
facial sensation. The remainder of the cranial nerve exam-
ination was normal.
At admission the blood pressure was 135/76 mm Hg, 70 puls-
es per minute, and the temperature was 37.3°C. The patient
was alert and well oriented. There was no conjunctival in-
jection. The funduscopic examination was normal. The to-
tal leukocyte count was 12,000/mm3 with 71% neutrophils,
1% band forms, 14% lymphocytes, 6% eosinophils, and 4%
monocytes. The erythrocyte sedimentation rate was 50mm at
the end of first hour, and a C-reactive protein was 6.2 mg/l.
The patient was treated with a high dose of wide-spectrum
intravenous antibiotics IV for 4 weeks. The dosage of amoxi-
cillin given was doubled because of the increased incidence
of Streptococcus pneumonia. Group A b-hemolytic strep-
tococci, and S. aureus are the causative organisms in this
Iliadis C et al – Management of Gradenigo syndrome in a child
age range (>14 of years), with intermediary resistance. She
was given 90mg/kg per day in 3 administrations as recom-
mended by the Centers for Disease Control and Prevention.
In order to improve Eustachian tube function, topical va-
soconstrictors (phenylephrine 0.25% 3 drops q 3 h) were
instilled into each nasal cavity for 1 week while the patient
was supine with the neck extended. In order to prevent life-
threatening complications such as thrombosis of the sinous,
the patient was given inhibitor of platelets aggregation,
with acetyl salicylic acid 50 mg per day and low dose intra-
venous, methylprednisolone, 250mg twice per day. After 5
days of antibiotics IV, a complete recovery of facial pain was
obtained, and 10 days after the initiation of treatment the
neurological examination showed a partial recovery of the
sixth nerve palsy. For this reason, we decided to proceed to
tympanostomy tube placement on the right ear in order to
decompress it and improve the nerve palsy.
Because of tympanic membrane bulging and severe and
persistent otalgia, for the first 2 weeks at the department
the patient underwent a right myringotomy with ventila-
tion tube insertion, which showed some fluid in the middle
ear. There was also mucous in the aspiration. The bacteri-
ological study revealed altered white blood cells. No bacte-
ria were identified. This might have been due to prior ad-
ministration of antibacterial agents.
Her audiogram demonstrated normal hearing in both ears
on pure tone averages. Her discrimination score was 86%
in the right ear and 92% in the left ear. Tympanogram in
the right ear was improved after tube removal 1 month lat-
er. Post-treatment discrimination score was 90%.
The CT showed sclerosis of the left right antrum and pe-
trous bone, loss of pneumatisation of the mastoid air cells
and destruction of the petrous apex, with an enhancing
mass around the petrous apex and the left parasellar re-
gion (Figure 1). MRI confirmed the presence of extensive
changes with abnormal enhancement located at the right
PA extending into Meckel’s cave and along the dura of the
middle cranial fossa floor. The ventricular system was slight-
ly enlarged. Inflammatory debris was detected in the right
mastoid (Figure 2).
Her pyrexia and otalgia returned to normal 3 weeks after
initial commencement of the intravenous antibiotic therapy
and 2 weeks after myringotomy. Her inflammatory markers
return to normal. The follow-up MRI 1 year later showed
full recovery in right petrous apex, middle ear structures
and mastoid air cells.
Discussion
Petrous apicitis, also known as petrositis, is divided into
acute and chronic forms. In acute otitis media, the most
frequently isolated organisms are Streptococci, Staphylococcus
species, Haemophilus influenza, Pseudomonas species, Moraxella
catarrhal and various anerobes. Mycobacterium tuberculosis has
also been previously implicated. In chronic suppurative
disease, frequent findings tend to be anerobes and Gram-
negative organisms; Haemophilus influenza type B is a major
etiological factor in cases of otitis media-associated menin-
gitis. Suppurative otitis media associated with mastoiditis is
usually associated with Bacteroides [3,6].
103
Case Report
Figure 1. CT scan (bone window) shows sclerosis of the right mastoid
antrum and petrous bone, obliteration of the definition of
the middle and inner ear structures.
Most cases of petrous apicitis are considered to occur in pa-
tients with well-developed air cell systems extending into
the apex. However, cases of petrous apicitis have been de-
scribed in non-pneumatized temporal bones. These cases
may represent a direct spread of infection from an acute
process in the middle ear, and thus represent a true osteo-
myelitis, rather than infection of a cell system [9].
Due to the number of vital structures that pass near the PA,
infection in this area can either result in symptomatology
consistent with Gradenigo syndrome or may run under-
detected for long periods of time due to vague symptoms.
Symptoms include vertigo, facial paralysis, and hearing loss
due to involvement of the otic capsule, as well as sensory dis-
turbances or facial pain result from involvement of cranial
nerve V due to compression and/or irritation of the gasse-
rian ganglion. Dysfunction of the III and IV nerve has oc-
casionally been reported due to anterior involvement and
also dysfunction of IX through XII nerve due to posteri-
or involvement.
As the technology of diagnostic radiology improves, more
petrous apex lesions are being identified. Current imag-
ing not only offers accurate localization of the lesion, but
also in the majority of cases is also able to give important
diagnostic information about the nature of the abnormali-
ty [1,3,5,9]. Computed tomography (CT) is considered the
imaging examination of choice to evaluate petrous apici-
tis, due to its sensitivity and low false positive rate. Findings
on CT include opacification or coalescence of the air cells
of the petrous apex. Acute petrous apicitis that occurs into
well developed air cells can generally appear as an expans-
ile lesion within the apex, and enhance with contrast if an
abscess is present. Chronic apicitis results in a destructive
bone appearance that resembles osteomyelitis of the scull
base [4]. CT scans demonstrate fluid-filled mastoid air cells
and sclerosis of the bones, and the degree of periosteal
104
Figure 2. Coronal MR Image confirms the presence of extensive soft
tissue along the middle and inner ear.
reaction and status of the middle ear structures can be as-
sessed based on CT scan findings [4,8].
MRI scans are best for soft tissue lesion assessment [8]. Acute
petrous apicitis with abscess formation can be appreciated
as a low intensity lesion on T1-weighted images that may en-
hance post-contrast. Chronic apicitis appears as an irregular
heterogeneous appearance that is best demonstrated on T2
images. MRI is also utilized to establish the extent of meninge-
al and cerebral involvement, and helps differentiate between
pathologies that may be encountered at the petrous apex.
MRI with angiography is the gold standard in the diagnosis
of dura sinus thrombosis, providing excellent visualization
both of large deep and superficial cerebral veins.
Treatment of Gradenigo syndrome with intratemporal com-
plications involves intravenous antibiotics and myringotomy
in those cases with an intact tympanic membrane. The de-
cision whether to insert a ventilation tube depends on the
findings during myringotomy (e.g., pus in the middle ear).
McHugh et al. (1994) reported a case of Gradenigo syn-
drome in which CT scan showed a small mass in the left
IAC and MRI showed evidence of petrositis. These lesions
markedly improved without aggressive treatment. The report
also underlined the utility of gadolinium-enhanced MRI in
identifying soft tissue inflammation and intraosseus disease
in the absence of bone destruction [3,6,9].
Debridement (myringotomy) is a less aggressive surgical in-
tervention, reserved for those patients unresponsive to in-
travenous antibiotics or those with resistant organisms but
with bulging tympanic membrane.

Surgery (wide-mastoidectomy) is indicated in 2 settings
[3,4]: firstly, in cases with signs and symptoms of mastoid-
itis, abscess formation within the petrous apex as well as
in the epidural space or brain parenchyma, and second-
ly, if there is no improvement despite adequate antibiotic
therapy or antibiotics in combination with myringotomy.
Mastoidectomy is performed as far as possible in the direc-
tion of the petrous apex cells to drain the petrous abscess
through the surgically opened cells and to stop the trap-
ping phenomenon [2–4].
Once sufficient treatment has been established, decisions
regarding the follow-up procedure and when to end anti-
biotic treatment are crucial.
In the recent literature, intravenous antibiotics are recom-
mended for at least 2–3 weeks. Patients with an associated
osteomyelitis may require up to 6 weeks of medical treat-
ment. [3,5,7,10].
In patients who have been treated surgically, a follow-up
CT or MRI is recommended in order to establish eradica-
tion of the infection, since abnormalities on MRI can per-
sist for prolonged periods even though the patient presents
as fully recovered. Normalization of finding indicates good
healing. Negative values are the only reliable indication for
stopping medical treatment.
Conclusions
Gradenigo syndrome may complicate acute otitis media, and
should be suspected in case of unilateral headache and ab-
ducens nerve palsy. The symptoms and signs can be subtle,
Iliadis C et al – Management of Gradenigo syndrome in a child
so the clinician must always have a high index of suspicion
in order not to miss a critical diagnosis. Conservative man-
agement, including myringotomy and prolonged antibiot-
ic treatment without major surgery, may be considered as
a treatment of choice. Surgical intervention should be per-
formed if there is a life-threatening complication or a pus
sample is obtained by mastoid drainage.
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