2016 ESPO CongressEvaluation of the outcome of CT and MR imaging in pediatric patients with bilateral sensorineural hearing loss
Introduction
Sensorineural hearing loss (SNHL) in pediatric patients may be present at birth or become apparent later during infancy. In both cases, the cause of the hearing loss may be hereditary or acquired. Congenital hearing impairment is the most common birth defect, with an incidence of 1,9 in 1000 newborns in the Netherlands and 1 to 3 per 1000 births worldwide [1,2]. In the Netherlands, congenital hearing loss is detected at a very early age by the current newborn hearing screening program provided by the Dutch Child Health and Welfare service (JGZ) which was implemented from 2002 to 2006 [3]. Currently, 96,5% of the newborn in The Netherlands are screened for hearing impairment [1].
Screening for hearing loss during the newborn period has led to early detection and diagnosis of SNHL, facilitating timely intervention [4,5]. Congenital hearing loss is nowadays generally detected within the first weeks of life in The Netherlands, however hearing loss may also be diagnosed later during infancy, because of a late onset (due to acquired pathologies such as infection or trauma) or a progressive nature of hereditary etiologies.
Whereas adequate and timely revalidation has been the primary goal of newborn hearing screening, it has also sparked the interest in the causes of pediatric SNHL. In addition to genetic and laboratory testing, imaging by computed tomography (CT) and/or magnetic resonance (MR) imaging has become an essential part of the evaluation of pediatric SNHL [[6], [7], [8]]. CT and MR imaging are regarded as complementary modalities. CT is considered a better modality for the identification of bony abnormalities, while MR imaging provides superior information about the cochlear nerve, the intracranial structures and early stages of fibrosis in cases of meningitis [9]. Previous studies of children with SNHL show temporal bone abnormalities in 18–37% when CT is performed, or 24–33% when MR imaging is performed as a single modality [6,7]. Combined, the overall reported diagnostic yield is 25–38% [10,11].
Children with SNHL form a heterogeneous group of patients, with a varying age at detection and varying degrees of hearing loss. These different patient groups may represent different SNHL etiologies, resulting in a different radiologic outcome and yield. Here, we evaluate the prevalence and spectrum of causative radiological abnormalities in children with bilateral SNHL and their associations with the severity of the hearing loss, the symmetry of the hearing loss and the age at diagnosis.
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Patients
Patients between 0 and 18 years of age diagnosed with bilateral SNHL were referred for etiological evaluation to the VU University Medical Center (VUmc) in Amsterdam, The Netherlands. The majority of these children was referred directly after detection of their hearing loss by the Dutch newborn hearing screening, and subsequent bilateral SNHL was confirmed by the audiological center of the VUmc or other regional audiology centers. In some cases, the detection of SNHL or need for etiological
Clinical characteristics
From January 2006 until January 2016, a total of 425 children with SNHL were evaluated by the CDS multidisciplinary team, 303 of which had symmetric or asymmetric bilateral SNHL (>30 dB). Of these 303 children, 96 were excluded because no imaging was performed, either because the cause of the hearing loss was already identified by the pediatric and/or genetic evaluation or by parental request and imaging was deemed of no additional diagnostic value. The main objective of the imaging in the
Discussion
In this study we evaluate the outcome of CT and MR imaging in a large cohort of children with symmetric and asymmetric bilateral SNHL. There are several reasons to perform imaging in children with SNHL, even though it is usually not possible to reverse the SNHL regardless of the outcome of radiology. The prevalence of causative radiological findings in children with bilateral SNHL is considerable: 32% in the current study, similar to previous reports (25–38%) [10,11]. A radiologic diagnosis may
Conclusion
Imaging is an essential part of the etiologic evaluation of children with bilateral SNHL. The highest diagnostic yield is found in children suffering from asymmetric bilateral SNHL and in children suffering from profound SNHL. Based on our findings, MR is the primary imaging modality of choice in the etiological evaluation of children with bilateral SNHL because of its high diagnostic yield.
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