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Case Reports in Otolaryngology

Volume 2014, Article ID 192318, 5 pages
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Case Report
Fungal Malignant Otitis Externa with Facial Nerve Palsy:
Tissue Biopsy Aids Diagnosis

Jenny Walton and Chris Coulson

Department of Otorhinolaryngology, Queen Elizabeth Hospital Birmingham, Mindelsohn Way, Edgbaston, Birmingham B15 2WB, UK

Correspondence should be addressed to Jenny Walton; jennywalton@[Link]

Received 2 December 2013; Accepted 23 December 2013; Published 5 February 2014

Academic Editors: M.-K. Chen, D. K. Chhetri, C. F. Hwang, and L.-F. Wang

Copyright 2014 J. Walton and C. Coulson. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
cited.

Fungal malignant otitis externa (FMOE) is a serious and potentially life-threatening condition that is challenging to manage.
Diagnosis is often delayed due to the low sensitivity of aural swabs and many antifungal drugs have significant side effects.
We present a case of FMOE, where formal tissue sampling revealed the diagnosis and the patient was successfully treated with
voriconazole, in addition to an up to date review of the current literature. We would recommend tissue biopsy of the external
auditory canal in all patients with suspected FMOE in addition to routine microbiology swabs.

1. Introduction 2. Case
Malignant otitis externa (MOE) remains a relatively uncom- An 83-year-old man with type 2 diabetes presented with right
mon condition but can lead to serious morbidity or mortality sided otalgia and otorrhoea; examination revealed granula-
as diagnosis is often delayed leading to initially ineffec- tion tissue inferiorly in his ear canal at the Osseochondral
tive treatment [1]. The infection begins in the external junction. A clinical diagnosis of malignant otitis externa
auditory canal, typically presenting with severe otalgia and was made. Pseudomonas aeruginosa was grown on microbi-
purulent otorrhoea; it can rapidly spread via the ear canal ological culture of the discharge, sensitive to ciprofloxacin.
soft tissue to the temporal bone resulting in osteomyelitis His condition was successfully treated over a period of
and subsequent cranial nerve palsies and intracranial infec- three months with oral and topical ciprofloxacin and he
tion. The prevalence of bacterial MOE is not accurately was discharged from clinic pain-free with an otoscopically
documented [2] and it is unknown whether fungal MOE normal canal.
is a repercussion of unsuccessfully treated bacterial otitis One year later, he presented with left-sided grade III
externa or if it represents a de novo presentation of fungal facial nerve palsy and a painful left ear (note this is the
disease. contralateral side to the original presentation). The left ear
The most commonly reported pathogen in malignant was initially otoscopically normal. CT and MR imaging
otitis externa is Pseudomonas aeruginosa [2]. Aspergillus demonstrated a left skull base osteomyelitis with involvement
species and Candida albicans have been implicated in fungal of the temporal bone and extension to the clivus with an
MOE. There are 32 previously reported cases of fungal associated small epidural abscess. Over the next few months,
MOE, usually occurring in patients with some form of the VII nerve palsy progressed to complete paralysis (grade
immunosuppressiontypically diabetes, acquired immun- VI) and was unresponsive to treatment with ciprofloxacin
odeficiency, or malignancy. We present a case of fungal and meropenem. He developed inflammation of his left
malignant otitis externa complicated by a facial nerve palsy external canal with an associated otorrhoea. Numerous
that proved very difficult to achieve a formal diagnosis. aural swabs revealed no definitive microbiological diagnosis.
 2
Table 1: Summary of published cases of FMOE. M: male, F: female, DM: diabetes mellitus, N: no, Y: yes, NIDDM: noninsulin-dependent diabetes mellitus, AML: acute myeloid leukaemia,
tx: transplant, CRF: chronic renal failure, HTN: hypertension, AIDS: acquired immunodeficiency syndrome, and ALL: acute lymphocytic leukaemia. Numbers in round brackets represent
separate patients reported in the same paper.
Year of Age Relevant Surgical
Main author M/F Pathogen Nerve palsy Treatment Symptom resolution
paper (years) comorbidities debridement
Type 2 DM (poorly Amphotericin then
Tarazi et al. (1) [3] 2012 77 M Aspergillus No N Y
controlled) voriconazole
Type 2 DM (poorly Amphotericin then
Tarazi et al. (2) [3] 2012 85 F Aspergillus VII N Y
controlled) voriconazole
Type 2 DM (poorly
Tarazi et al. (3) [3] 2012 70 F Aspergillus No Voriconazole N Y
controlled)
Amphotericin B NoCN palsies
Halsey et al. [4] 2011 62 M AML Aspergillus wentii VII Y
then itraconazole persisted
Parize et al. (1) [5] 2009 48 M Polychondritis Aspergillus niger No Voriconazole N Yes
IDDM, renal tx,
Parize et al. (2) [5] 2009 40 F Aspergillus niger No Voriconazole Y Nhearing loss
CRF
NIDDM, HTN, Candida, Voriconazole,
Ling and Sader [6] 2008 77 M VII Y Yes
gout Aspergillus flavus caspofungin
VII, IX, X, Amphotericin B,
Narozny et al. [7] 2006 65 M NIDDM Aspergillus Y Ndied
XII itraconazole
Scedosporium
Tzuku et al. [8] 2006 17 F IDDM No Amphotericin B Y Nodied on day 7
apiospermum
Amphotericin B
Bellini et al. [9] 2003 73 M NIDDM Aspergillus niger Unspecified N Yes
then itraconazole
Shelton et al. [10] 2002 58 M Nil Aspergillus niger Unspecified Itraconazole N Yes
Amphotericin B
Finer et al. [11] 2002 7 M Neuroblastoma Aspergillus flavus Unspecified N Yes
then itraconazole
Malassezia VII, IX, X, Amphotericin B NoCN palsies
Chai et al. [12] 2000 53 M NIDDM N
sympodialis XII then fluconazole persisted
Aspergillus Amphotericin B
Chen et al. (1) [13] 1999 41 M AIDS VII Y Yes
fumigatus then itraconazole
NoCN palsies

Case Reports in Otolaryngology

Chen et al. (2) [13] 1999 36 M AIDS Unknown VII Amphotericin B Y
persisted
A. flavus, niger, Amphotericin B
Slack et al. [14] 1999 14 F ALL Unspecified Y Yes
fumigatus then itraconazole
Aspergillus Itraconazole then
Munoz and Martnez-Chamorro [15] 1998 27 F AIDS Unspecified N Ndied
fumigatus amphotericin B
Aspergillus Timentin-
Ress et al. [16] 1997 41 M AIDS Unspecified N Ndied
fumigatus gentamicin
NoCN palsies
Kountakis et al. [17] 1997 65 M NIDDM Aspergillus flavus VII Amphotericin B Y
persisted
 Case Reports in Otolaryngology
Table 1: Continued.
Year of Age Relevant Surgical
Main author M/F Pathogen Nerve palsy Treatment Symptom resolution
paper (years) comorbidities debridement
Aspergillus
Yates [18] 1997 18 M AIDS Unspecified Itraconazole Y Yes
fumigatus
Harley et al. [19] 1995 20 M ALL Aspergillus flavus VII Amphotericin B Y Ndied
Amphotericin B
Gordon and Giddings (1) [20] 1994 67 F NIDDM, HTN Aspergillus flavus No Y No
then itraconazole
Ceftazidime,
ciprofloxacin,
Gordon and Giddings (2) [20] 1994 82 M Nil Aspergillus flavus No Y No
tobramycin,
itraconazole
Aspergillus flavus,
Hanna et al. [21] 1993 61 M NIDDM VII Amphotericin B Y Yes
Candida
Aspergillus Amphotericin B
Reiss et al. (1) [22] 1991 42 M AIDS Unspecified Y Yes
fumigatus then itraconazole
Aspergillus Amphotericin B
Reiss et al. (2) [22] 1991 30 M AIDS Unspecified Y Yes
fumigatus then itraconazole
Amphotericin B
Phillips et al. [23] 1990 64 F NIDDM, AML Aspergillus VII N Yes
then itraconazole
Menachof and Jackler [24] 1990 46 F AML Aspergillus flavus VII Amphotericin B Y Y
Aspergillus
Denning et al. [25] 1989 38 M Nil No Itraconazole Y Yes
fumigatus
Aspergillus Amphotericin B,
Cunningham et al. [26] 1988 85 M Nil VII Y Yes
fumigatus rifampicin
Bickley et al. [27] 1988 80 M Myelodysplasia Aspergillus No Amphotericin B Y Yes
Aspergillus NoCN palsies
Petrak et al. [28] 1985 68 M AML VII Amphotericin B Y
fumigatus persisted

3
4 Case Reports in Otolaryngology

Tissue biopsies were taken directly from the canal and, Voriconazole is currently recommended as first line
in contrast to the previous microbiology, Aspergillus flavus treatment in cases of invasive aspergillosis [31] and its use
was isolated. Treatment was commenced with intravenous is increasing since its launch in 2002. The intravenous form
voriconazole for 2 weeks, followed by subsequent conver- is recommended for use in systemically unwell patients,
sion to oral voriconazole. Within a month of commencing with the oral form being reserved for those who are stable
antifungal therapy, he became pain-free and his facial palsy or have improved following initial intravenous treatment.
resolved completely. Oral voriconazole therapy was contin- Voriconazole is widely distributed throughout tissues and,
ued for 10 months after resolution of symptoms. The initial in its oral form, is not usually associated with worsening of
plan had been to treat him for 12 months, although the patient renal function. This is particularly important as it is often
developed worsening liver function; hence, the therapy was patients with comorbidities affecting renal function such
discontinued. as diabetes, who develop aspergillosis FMOE and therefore
require treatment with voriconazole.
The most commonly reported side effects of voriconazole
3. Discussion include visual disturbance, particularly to colour vision,
abnormal liver function tests, deranged renal function, and
Cranial nerve palsies are frequently seen in fungal MOE electrolyte abnormalities [32]. As noted in the literature,
[4]; however, many reported cases have not demonstrated a amphotericin B was most frequently used, although it has a
significant improvement following treatment, possibly due to significantly poorer side effect profile, including derangement
the delayed isolation of the pathogen and initiation of anti- of renal function [5]. Itraconazole and caspofungin have also
fungal treatment. Halsey et al. [4] reported the presence of been used in cases reported in the literature.
VII nerve palsy in 75% of patients with Aspergillus infection, Antibiotic therapy for the majority of cases of MOE is
compared with only 34% in MOE due to Pseudomonas. guided by the results of aural swabs sent for microbiological
Aspergillus fumigatus was thought to be the most com- culture. Diagnosis of FMOE in our case and other cases in the
mon fungal pathogen in MOE [6], with Aspergillus flavus literature required formal tissue sampling from the external
assumed to be a less frequent cause of the condition. However, auditory canal in order to identify the pathogen [4, 20, 33].
review of published cases reveals A. flavus to be a roughly as The patient in our case presented one year following
prevalent as A. flavus in MOE. A. flavus has been observed treatment of bacterial MOE with symptoms in the contralat-
to be 100 times more virulent than A. fumigatus and has an eral ear. Although we found no evidence that the causative
optimum temperature for growth of 37 C, which may explain organism was the same on both sides, we note one particular
its particular pathogenicity in humans [29]. case in the literature where the disease spread from one side
In MOE, the infection spreads through the fissures of to the other via the clivus [31].
Santorini, small perforations in the cartilaginous floor of the
external auditory canal, and then medially until it reaches the
skull base [1]. Here, it causes bony destruction and further 5. Conclusion
continues its medial progression triggering cranial nerve
We would recommend biopsy of tissue from the external
palsies with the VII nerve being most commonly affected due
auditory canal in any patient with a presumed diagnosis of
to the proximity of the stylomastoid foramen to the ear canal.
MOE in addition to aural swabs. The importance of this is
The presence of a facial nerve palsy has been suggested to
to obtain early confirmation of a fungal pathogen thereby
represent a poor prognosis in patients with MOE along with
allowing timely treatment to be commenced.
coexisting immunosuppression [30]. Table 1 summarizes the
cases of fungal MOE (FMOE) present in the literature.
Conflict of Interests
4. Summary of the Literature The authors declare that there is no conflict of interests
regarding the publication of this paper.
The mean age of patients in the literature was 52 years with
a male : female ratio of 7 : 3. Aspergillus species were the
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