Review article
Kawasaki disease is associated with sensorineural hearing loss: A systematic review

https://doi.org/10.1016/j.ijporl.2014.05.026Get rights and content

Abstract

Context

Kawasaki Disease (KD), a systemic vasculitis of unknown etiology, has been associated with the development of sensorineural hearing loss (SNHL). KD is primarily a disease of young children, who are the most susceptible to complications from even minimal hearing loss. If there is a connection between KD and the development of SNHL, a better understanding of this relationship may improve our management of this disease and its complications.

Objective

To perform a systematic review according to a standardized guideline to evaluate the possible association between KD and SNHL.

Data sources

Medline and PubMed online databases were reviewed for appropriate articles.

Study selection

All studies available in English discussing KD and SNHL were included.

Data extraction

Studies were assessed primarily for the incidence of SNHL. Where possible, they were assessed for the degree and laterality of the loss, length of follow up and change in hearing over time.

Results

8 studies meeting the criteria were assessed. 3 were case reports, 1 was a case series and the remaining 4 were prospective control trials. 8 patients have been reported as cases, and 240 assessed in PCT. 36% of patients assessed had some degree of SNHL, and overall 14% had evidence of persistent SNHL at follow up.

Conclusions

This systematic review would suggest there is an association between KD and SNHL. It is important for physicians caring for patients with KD to be aware of this complication and consider screening these patients given possible complications of hearing loss in this age group.

Introduction

Kawasaki disease (KD) and atypical KD (AKD) are a systemic vasculitis of unknown etiology that occur almost exclusively in pediatric patients [1]. The diagnostic criteria for KD and AKD are outlined in Table 1 [2], [3], [4]. Over 75% of cases occur in children less than 5 years of age and the incidence is highly varied, ranging from 4 to 216 per 100,000 children less than 5 years of age worldwide [1]. In Canada and the Unites States, the incidence of KD is 26.2 per 100,000 and 20.8 per 100,000 children less than 5 years of age, respectively [5], [6]. KD is associated with a high incidence of severe cardiac complications, including coronary artery aneurysms, which can occur in up to 20–25% of untreated patients [7]. With treatment, the incidence of coronary artery abnormalities decreases to 1–5% [8]. Because of this potential for significant morbidity and mortality, all children should receive a screening echocardiogram as a component of their treatment [1], [2], [4], [7], [8], [9].

In 1988, a Japanese physician reported the first cases of sensorineural hearing loss (SNHL) potentially associated with KD [10]. Over the last 20 year, little research has been published on this possible relationship, and the results of those studies have been inconsistent. Overall, these studies suggest that KD is associated with SNHL, but with varying degrees of severity and persistence [11], [12], [13], [14], [15], [16], [17], [18].

The effects of hearing loss in pediatric patients have been well studied. Normal hearing is an essential component of speech and language development, especially from birth to five years of age. Even mild SNHL may cause problems with normal speech and language development [19]. Affected children who are identified earlier have also been shown to have better outcomes than children with delayed diagnoses, which provides the basis for newborn hearing screening examinations [20]. SNHL is not a well-defined complication of KD, and there is currently no recommendation regarding the need for audiology screening in these patients. If KD is associated with an increased risk of developing hearing loss, an audiology assessment may be a necessary component in routine follow up to identify and treat hearing loss early, to minimize the potential impact on speech and language development.

The goal of this systemic review is to examine the literature on the association between KD and SNHL in an effort to help clarify a potential correlation. A better understanding of this relationship may improve management of this disease and its complications.

Section snippets

Materials and methods

All relevant scientific publications were identified though Ovid/Medline and PubMed searches. Search terms included “Kawasaki disease”, “hearing”, and “hearing loss”. The details of the search strategy are detailed in Table 2. Inclusion criteria included any paper discussing KD and SHNL. Exclusion criteria included papers that were not available in English. All papers meeting these criteria were included, including case reports, case series and prospective control trials (PCT). While this does

Results

In total, 8 articles were found that met inclusion criteria. The articles were published between 1990 and 2012. 3 of the articles were single case reports. 1 of the articles was a case series of 5 individual patients. The remaining 4 articles were PCT. The studies are grouped for discussion by study design. Table 4 details the data from the studies.

Sundel et al. published the first case English case series of 5 patients with KD and SNHL [17]. 3 of the patients had bilateral SNHL and 2 patients

Discussion

Since the initial association between KD and SNHL was made in 1988, a total of 248 patients have been assessed in an attempt to define the possible association. 8 of these patients were noted in case reports, and the other 240 patients were involved in PCT. Of the PCT patients, 36% were noted to have some degree of SNHL, presenting in the first 30 days of illness. Overall, 41% of patients with SNHL had SNHL at follow up, which represents a degree of persistent SNHL of 14% (Fig. 2). The length

Conclusions

The results of this systematic review would suggest that there is an association between KD and SNHL. Given the possible complications arising from hearing loss in this age group it is important for physicians caring for patients with KD to be aware of this complication. As such, we believe strong consideration should be given to audiometric assessment in these patients. Further research into the pathophysiology and natural history of the hearing loss in these patients is necessary.

Funding

No funding was secured for this study.

Financial disclosure

Kristine Anne Smith and Warren Yunker have no disclosures.

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