A Rare Case of Candida Mastoiditis in an Immunocompetent Patient With Cholesteatoma Ear Disease

A 25-year-old female reported to us in the ENT outpatient department of our tertiary care teaching hospital with the chief complaints of left ear discharge and hearing loss for the last 10 years along with left facial nerve palsy for the past 7 days. The facial nerve palsy was insidious in onset, and progressive deterioration was seen over a period of time. There was a history of use of steroid ear drops. Patient had no comorbidities such as diabetes mellitus or any other chronic systemic illness. Examination of the ear revealed a posterior–superior perforation in the tympanic membrane involving pars flaccida and pars tensa with characteristic cholesteatoma flakes. Facial nerve palsy assessment by House-Brackmann classification was of grade II (Figure 1). A computed tomography scan was done, which revealed soft tissue density in the left middle ear cavity, involvement of the tympanic segment of the facial nerve, and ossicular erosion with features suggestive of otomastoiditis (Figure 2).

Figure 1.

Clinical photograph of the patient showing facial nerve palsy: (A) preoperative and (B) postoperative.

Figure 2.

Computed tomography scan showing soft tissue density in middle ear cleft and otomastoiditis with involvement of tympanic segment of facial nerve.

A diagnosis of right chronic suppurative otitis media cholesteatoma ear disease with grade II facial nerve palsy was made, and the patient was treated with modified radical mastoidectomy with type III tympanoplasty.

Pathology

Cholesteatoma was seen in antrum, aditus, attic, and extending into the facial recess. Cholesteatoma sac was also seen impinging onto the dehiscent second genu of the facial nerve. Handle of malleus and long process of incus were necrosed.

The excised cholesteatoma sac was sent for microbiology. Culture on Sabouraud dextrose agar showed characteristic yeast growth (Figure 3A). Furthermore, on gram staining, Candida nonalbicans pseudohyphae was observed (Figure 3B). We thus made the final diagnosis of Candida nonalbicans otomastoiditis.

Figure 3.

A, Culture of cholesteatoma on Sabouraud dextrose agar showing yeast growth. B, Gram staining (×1000) showing candida nonalbicans pseudohyphae.

Immediate postoperative period was uneventful. A complete recovery of the facial nerve was seen within a week of surgery (Figure 1). Subsequently, patient was discharged on tablet itraconazole 200 mg twice daily along with fluconazole antifungal ear drops for 6 weeks. The patient is in regular follow-up with us for the past 9 months with no untoward incident to report.

Fungal mastoiditis in an immunocompetent patient is a rarity: Only a few anecdotal case reports are available on the cited subject.^1-3 We could find only 6 cases of fungal mastoiditis in cholesteatoma ear disease reported in the English medical literature, and only one of these cases had a facial nerve palsy.^1-5 In a massive internet search using PubMed/Medline services, the authors could find only one case of otomastoiditis caused by Candida species. ⁶ This case had a bilateral acute otomastoiditis due to Candida auris infection and was treated with antifungals: intravenous fluconazole. No cholesteatoma was seen in this case. In contrast, our case had protracted cholesteatoma ear disease with off and on ear discharge and facial nerve palsy. It was primarily managed surgically followed by antifungal treatment. It would be prudent to note that the drug management guidelines for fungal colonization of cholesteatoma are yet to be defined. To the best of our knowledge, this is the first reported case of Candida mastoiditis in cholesteatoma ear disease.

There are 4 routes of entry of fungal infection to the middle ear: tympanogenic, meningogenic, hematogenic, and nasopharyngeal. ¹ In our case, it is reasonable to conclude that cholesteatoma got infected as a result of environmental spores from the external auditory canal (EAC) or via the eustachian tube.^7-9 Further, the infection could have transcend to the middle ear from the EAC. Use of topical steroid ear drops and maceration of EAC skin as a result of ear discharge could have been important predisposing factors for fungal infestation of EAC. ⁸ Fungi by mycotoxicosis, release of pro-inflammatory cytokines, symbiosis with bacteria, epithelial desquamation, and hypersensitivity reactions (type I and III) may enhance the pathogenicity of the cholesteatoma and thus lead to extracranial complications in immunocompetent patients as in this case. ⁹

There are only 3 studies which have cultured fungus from keratin debris, but they report a high prevalence rate of 27% to 89%.^7-9 It would also be imperative to note that population explosion and advances in medical care have led to a surge in the incidence of opportunistic fungi, especially in the Asian countries. Hence, a high index of suspicion is necessary for early diagnosis of fungal otomastoiditis, especially in patients with protracted cholesteatoma ear disease presenting with facial nerve palsy. The rarity of fungal mastoiditis in medical literature which limits the conclusions that can be drawn on its clinical course and management prompted us to share our modest experience on the cited subject.