Case Report
Outcomes of cochlear implantations for mumps deafness: A report of four pediatric cases

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Abstract

Mumps virus occasionally causes bilateral hearing loss. We report 4 cases of bilateral mumps deafness in whom cochlear implantations (CI) were performed. The age at the onset of hearing loss was 1–9 years. CI surgery was performed within 6 months from the onset of hearing loss in 3 cases and after 9 years in the other case, showing good speech perception in the early intervention cases and a poor outcome after later implantation. Early CI surgery is highly recommended in sudden onset deafness by mumps in childhood.

Introduction

Mumps is an infection caused by the mumps virus, typically presenting with flu-like symptoms, followed by bilateral swelling of the parotid glands [1]. However, subclinical infection is a possible presentation and some patients may not experience parotid swelling [2]. Mumps infection can lead to a number of complications including meningitis, encephalitis, pancreatitis, orchitis, oophoritis, infertility, epididymitis, and deafness [3,4].

The prevalence of mumps deafness is estimated to be as low as 0.5–5.0 per 100000 cases [5]. However, due to the low penetration of mumps vaccination in Japan, mumps is endemic with an incidence of deafness as high as 1 per 1000 cases [6].

Most cases of mumps deafness present with a profound unilateral hearing loss; however in some cases, it is bilateral [2]. There are few studies examining the pathogenesis of mumps deafness. The human temporal bone findings, as reported by Lindsay [7], were primarily of the cochlear duct. The stria vascularis, organ of Corti, and tectorial membrane were severely degenerated in the basal coil, diminishing progressively towards the apex. On the other hand, ganglion cells were near normal with minimal degeneration of the peripheral cochlear nerve in the basal coil. Experimental studies using guinea pigs and monkeys, revealed that the mumps virus has a high affinity for the stria vascularis and the outer hair cells of the ear, mainly disrupting the stria vascularis and the organ of Corti [8,9]. Although steroids have been clinically administered as a treatment for mumps deafness, the prognosis for patients is generally poor [2]. As a result, CI surgery is the only effective treatment for mumps deafness. However, its performance has rarely been reported [[10], [11], [12], [13], [14], [15]].

Herein, we present 4 pediatric cases with bilateral mump deafness who underwent CIs, and report their clinical findings and outcomes.

Section snippets

Case1

A 6-year old girl had bilateral hearing loss and vomiting, 3 days following the onset of right parotid swelling. Mumps-specific IgM and IgG antibody levels were high (13.8 mg/dL and 28.1 mg/dL, respectively; Positive assessment was made at ≧1.21 IgM and ≧4.0 IgG antibody levels). She had no history of mumps vaccination. The patient was diagnosed with mumps deafness and treated with intravenous prednisolone (21 mg/day). However, treatment was not effective and she was referred to our hospital

Discussion

We performed unilateral CI in 4 cases of bilateral mumps deafness. No cases and their families requested bilateral CI. Of the 4 cases, 3 had CI surgery performed within 6 months from the onset of hearing loss, and 1 after 9 years. Good speech perception was achieved in the early intervention cases, while a poor outcome was found after late implantation. Our results suggest that early cochlear implantation in sudden onset deafness by mumps in childhood is highly recommended.

Table 2 lists the 14

Conclusions

To conclude, we performed CI surgery in 4 cases of bilateral mumps deafness. Of the 4 cases, 3 had CI surgery performed within 6 months from the onset of hearing loss, and 1 after 9 years. Good speech perception was reported in the early intervention cases, while a poor outcome was reported after later implantation. This suggests that early implantation is highly recommended in sudden onset deafness by mumps in childhood. However, the possibility of a poor outcome should be discussed

Conflicts of interest

None.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

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