Utilization of diagnostic testing for pediatric sensorineural hearing loss

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Abstract

Background/Objective

Sensorineural hearing loss is a common diagnosis among children. The diagnostic workup varies widely among practitioners. This study's aim was to assess the utilization of diagnostic testing for SNHL and determine the yield of each test.

Study design

Retrospective chart review.

Setting

Tertiary care center.

Subjects

827 patients with a diagnosis of SNHL from January 1, 2011 to January 1, 2015.

Results

746 patients met inclusion criteria. Temporal bone imaging was performed on 561 (75%) of patients with 224 (40%) having positive results that explained the etiology of the SNHL. Congenital SNHL was more likely to be associated with abnormal imaging than acquired SNHL (109/299 versus 106/316 respectively) (p = 0.001). Unilateral SNHL was more likely to be associated imaging abnormalities than bilateral SNHL (101/221 and 123/340 respectively) (p = 0.028). Genetic testing was performed on 244 (33%) patients, of which 94 (39%) had abnormalities. Positive genetics results were more common with bilateral than unilateral SNHL (82/191 and 12/53 respectively) (p = 0.007). There was no statistically significant difference in the utility of genetic testing for congenital and acquired SNHL (p = 0.0836). Cytomegalovirus (CMV) testing was available for 104 (14%) of patients with 13 (12.5%) being positive and consistent with congenital CMV. Electrocardiogram, urinalysis, and Lyme titers were less useful.

Conclusions

Imaging and genetic testing had the highest yield in the evaluation of children with SNHL and were the most commonly performed. CMV testing was valuable in neonates who failed newborn hearing screening.

Introduction

Sensorineural hearing loss (SNHL) in children is common in developed countries. The estimated incidence of SNHL is 1–4/1000 [1,2] children at birth and 6/1000 by the age of 18 [3]. A wide range of conditions can lead to SNHL that can be generally categorized genetic versus non-genetic and congenital versus acquired. Genetic etiologies account for approximately 50% of cases of congenital SNHL [2]. Approximately 70% of genetic hearing losses are non-syndromic and 30% are associated with an underlying syndrome [2]. Non-genetic etiologies include infections such as congenital cytomegalovirus (CMV), ototoxic medications, maternal drug use, low Apgar scores, prematurity, and other environmental insults [2,4]. Early detection and treatment is important for speech and language development and meeting academic and social milestones [5,6].

Given the challenging nature of finding an etiology for the hearing loss, a thoughtful diagnostic approach is necessary. Although a complete history and physical examination are important, these often have low diagnostic yield. Therefore, further workup including genetic testing, imaging, lab work, and other diagnostic studies is often employed [1,7]. Many efforts have been directed to provide a diagnostic algorithm [2,4,8], yet different practices have different diagnostic approaches. There is no clear dominant strategy for any specific set of tests [[9], [10], [11], [12]]. Choosing to perform a test can depend on many factors, including severity and type of hearing loss, associated signs and symptoms, and family preference [13].

This study demonstrates the utility of different diagnostic approaches to SNHL in children at a tertiary care center. Particular attention was paid to the types of testing employed and the results of such testing. The purpose of this study is to assess the protocol for diagnosis and evaluation of SNHL at this institution. Findings were compared to the recent recommendations by the International Pediatric Otolaryngology Group (IPOG). [8] The study then looked at the diagnostic yield of the testing performed, comparing these results across different SNHL categories (congenital versus acquired and unilateral versus bilateral) and SNHL levels.

Section snippets

Methods

A retrospective chart review was performed for patients presenting to our institution from January 1, 2011 to January 1, 2015. 827 charts were available for review from the practices of five otolaryngologists. The Massachusetts Eye and Ear Institutional Review Board (IRB) approved this study (IRB protocol 15–025H).

Patients who were newborn to age 18 with a diagnosis of SNHL or hearing loss were included in the initial chart review. The presence of one audiogram in the chart to confirm the

Results

Of the 827 charts reviewed, 746 met inclusion criteria. Patient demographics are listed in Table 1. Fig. 1 shows the levels of hearing loss broken down into unilateral, bilateral, congenital, and acquired. Of note, 20 patients did not have information in the chart to classify the hearing loss as congenital or acquired. Table 2 demonstrates the utilization and diagnostic yield of CT, MRI, and genetic testing across the different levels of hearing loss broken down into unilateral, bilateral,

Discussion

Selective diagnostic workup for patients with SNHL can be very useful in determining the etiology and identifying any associated conditions. Information regarding the patient's birth history should be obtained which includes gestational age, Apgar scores, and any problems with pregnancy. It is important to note if an ICU stay was required because these patients have a higher risk of SNHL [14]. A history of jaundice or IV antibiotic use should be elicited. It is also important to ask about

Conclusion

When determining the etiology of sensorineural hearing loss in children, there is a wide variation in diagnostic approaches. At our institution, we feel the most important diagnostic testing modalities include imaging and genetic evaluation in combination with patient history. CMV testing should be performed on any infant who does not pass the NBHS. In settings where this is not possible, CMV testing should be performed immediately after an abnormal confirmatory hearing test. Other testing

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    This paper has not been presented at any professional meetings. It has not been published elsewhere. There are no conflicts of interest.

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