Case Report
Fatal consequences of an ear infection
Xenia L Stalpers, Mijke Smink-Bol, Paul E Verweij, Pieter Wesseling, Gert W van Dijk
Lancet 2009; 373: 1658 In August, 2006, a 75-year-old non-diabetic, non-alcoholic
Canisius-Wilhelmina Hospital, man with hypertension and atrial fibrillation was admitted
Department of Neurology to the Internal Medicine department with low grade fever,
(X L Stalpers MD,
increasing otalgia, otorrhoea, dysphagia, and hoarseness.
G W van Dijk MD), Department
of Pathology He had suffered from bilateral tympanic membrane
(Prof P Wesseling MD), perforation, intermittent otalgia, and otorrhoea for 12 years.
Nijmegen, Netherlands; In May, 2006, his otolaryngologist had prescribed ofloxacin
Radboud University Nijmegen
ear drops and oral ciprofloxacin for otalgia and otorrhoea.
Medical Center, Department of
Pathology (M Smink-Bol MD, In June, 2006, he was admitted to the Ear, Nose and Throat
Prof P Wesseling MD), department with a 3-week history of left peripheral facial
Department of Medical nerve palsy, left trigeminal sensory loss, and general dis-
Microbiology
(Prof P E Verweij MD),
comfort. He was afebrile, with a serum C-reactive protein
Nijmegen, Netherlands concentration of 72 mg/L; other laboratory tests were
Correspondence to: normal. CT and MRI of the head showed clouding of the
Xenia L Stalpers, left mastoid without intracranial pathology or meningeal
Canisius-Wilhelmina Hospital, enhancement (figure A); chest radiography was normal. A
Department of Neurology,
diagnosis of mastoiditis was made, and ceftazidime and
Weg door Jonkerbos 100,
6532 SZ, Nijmegen, Netherlands flucloxacillin were started. Surgical exploration of the left
x.stalpers@cwz.nl mastoid showed granulation tissue in the external audi-
tory canal and around the ossicles. Microscopically this
tissue showed chronic-active necrotising inflammation;
cultures (including those for fungi) were negative. After
2 weeks, the patient was discharged in fair condition.
In the current admission, besides earlier described
cranial nerve dysfunction, a partial left glossopharyngeal
and vagal nerve palsy were diagnosed by the consulting
otolaryngologist. C-reactive protein was 57 mg/L with
normal white cells. Culture of discharge from the ear
grew Pseudomonas aeruginosa, and amoxicillin (empir-
ically started on admission) was replaced by meropenem.
A B
R likely gained access to the ear and CNS through tympanic
C chance of resolution.
Figure: MRI, macroscopy, and microscopy
(A) Axial T1-weighted magnetic resonance image with intravenous gadolinium showing high signal intensity of
the left mastoid (arrow), without intracranial pathology. (B) Macroscopic view of the anterior side of the brainstem
with a focal purulent exudate (arrow). (C) Extensive active and necrotising inflammation in the left os petrosum
with dispersed septated and branched hyphae (PAS staining, original magnification ×400).
1658
2 weeks later, still in hospital and being treated with
meropenem, he developed headache, neck pain, and a
left abducens nerve palsy. Repeated chest radiography
and MRI of the brain were normal. Cerebrospinal fluid
(CSF) analysis showed only a mild pleocytosis (leucocytes
17×10⁶ per L). The clinical picture suggested a chronic
(basal) meningitis, but blood and CSF cultures remained
negative. The patient refused meningeal biopsy and
further treatment, had progressive difficulty in swal-
lowing, and died 3 weeks later in hospital from pneu-
monia. Autopsy showed a focal purulent prepontine
leptomeningeal exudate (figure B). Microscopically,
multiple septated and branched hyphae were detected in
this exudate and in the left middle and internal ear
(figure C), combined with chronic-active necrotising
inflammation. The inflammation extended into the skull
base and cranial nerves. A mould cultured from the
exudate was characterised as Pseudallescheria boydii by
sequence-analysis of parts of the β-tubulin gene.
P boydii can cause a wide variety of clinical diseases and
is commonly found in soil, polluted water, and manure.
P boydii infections of the central nervous system (CNS),
meninges, and mastoid are very rare and generally occur
in immunocompromised, near drowning, and trauma
patients, but have also been reported in patients without
one of these risk factors.1–3 There was no suspicion of
immunocompromise in our patient, and postmortem HIV
PCR analysis from lymph node and heart tissues were
negative. Diagnosis of CNS involvement by P boydii is
often delayed because of non-specific clinical presentation,
difficulty in obtaining and culturing appropriate specimens,
and because of histological similarity of P boydii to other
fungi, such as aspergillus. In our patient the fungus most
membrane perforation. In patients with P boydii
mastoiditis, surgical intervention, adequate anti-fungal
therapy, and identification and control of underlying
immunological conditions are essential. Voriconazole is
suggested as the treatment of choice.3 Awareness of P boydii
as a cause of chronic mastoiditis and meningitis may allow
early diagnosis and treatment, thereby increasing the
Contributors
All authors contributed to writing and revising this report, XS as main
author and GWvD as senior author. XS had no role in management of
the patient. MSB and PW participated in the autopsy and
histopathological investigations. PV contributed to microbiological
investigations. GWvD contributed to the patient’s management.
References
1 Benham RW, Georg LK. Allescheria boydii, causative agent in a case
of meningitis. J Invest Dermatol 1948; 10: 99–110.
2 Kershaw P, Freeman R, Templeton D, et al. Pseudallescheria boydii
infection of the central nervous system. Arch Neurol 1990; 47: 468–72.
3 Cortez KJ, Roilides E, Quiroz-Telles F, et al. Infections caused by
Scedosporium spp. Clin Microbiol Rev 2008; 21: 157–97.
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