Evaluation of the outcome of CT and MR imaging in pediatric patients with bilateral sensorineural hearing loss

Calkoen, E.A. van Beeck; Merkus, Paul; Goverts, S. Theo; Kamp, Jiddeke M. van de; Mulder, Margot F.; Hensen, Erik F.

doi:10.1016/j.ijporl.2018.02.022

Cited by 19 publications

(16 citation statements)

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“…Abnormal imaging findings were more frequent in the SSD group compared with the ASH group. Our findings are consistent with those of previous reports of imaging findings in bilateral SNHL, reporting similar rates of 20% to 30% …”

Section: Discussionsupporting

confidence: 93%

“…Our findings are consistent with those of previous reports of imaging findings in bilateral SNHL, reporting similar rates of 20% to 30%. 4,11,14 We observed bilateral anomalies not only in the ASH group but also in the SSD group. A previous study described a wider cochlear basal turn lumen and abnormal lateral SCC bony island in the contralateral normal hearing ears of children with unilateral hearing loss, compared with controls.…”

Section: Discussionmentioning

confidence: 65%

“…[4][5][6][7][8][9] A prior publication by the senior author has suggested a higher diagnostic yield for imaging with increased hearing loss severity in bilateral SNHL, and this has also been observed in a recent study. 4,11 In unilateral SNHL, Song et al found a higher rate of positive CT findings in 322 children with unilateral profound SNHL compared with nonprofound SNHL (31.6% vs. 23.8%). 8 Two additional studies that examined this association could not repeat these findings, yet those publications included a smaller sample size.…”

Section: Discussionmentioning

confidence: 99%

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Imaging findings in pediatric single‐sided deafness and asymmetric hearing loss

et al. 2019

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Objective To examine the imaging findings on computer tomography (CT) and magnetic resonance imaging (MRI) in pediatric single‐sided deafness (SSD) and asymmetric hearing loss (ASH). Methods The medical records of 189 pediatric patients with SSD and ASH were retrospectively reviewed, and imaging findings were compared. SSD was defined as unilateral profound hearing loss and contralateral normal hearing ear. In the ASH group, ASHw was defined as the worse hearing ear with profound hearing loss, while ASHb was defined as the better hearing ear with mild‐moderate hearing loss. Results There were 170 patients with SSD and 19 patients with ASH. In the SSD group, 83 patients (48.8%) had imaging findings associated with hearing loss. In the ASH group, such imaging findings were found in six (31.6%) of the ASHw and in five (26.3%) of the ASHb ears. The most common finding in the SSD group was cochlear nerve deficiency (50.6%), followed by cochlear dysplasia (39.8%) and enlarged vestibular aqueduct (26.5%). In the ASH groups, cochlear dysplasia was seen in three (50%) of ASHw ears and in two (40%) of the ASHb ears, and enlarged vestibular aqueduct was seen in three (50%) of ASHw ears and in two (40%) of the ASHb ears. Conclusion Imaging studies identified the etiology in half of the cases of SSD and in one‐third of ASH patients. Our findings strongly support the use of imaging studies in the evaluation of pediatric SSD and ASH. Level of Evidence 4 Laryngoscope, 130:1007–1010, 2020

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Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 65%

Section: Discussionmentioning

confidence: 99%

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Imaging findings in pediatric single‐sided deafness and asymmetric hearing loss

et al. 2019

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“…Of all identified abnormalities, 60% was located within the labyrinth, 15% in the cochlear nerve, and 25% in the brain. Detailed description of the type of abnormality has been reported elsewhere [22, 23].…”

Section: Resultsmentioning

confidence: 99%

“…The protocols for the diagnostic evaluation of the etiology of SNHL in children are based on Dutch guidelines and the experience of the CDS team [24], and include radiology, pediatric and genetic evaluation. The outcome of radiology alone in children with SNHL has been described in more detail elsewhere [22, 23]. During the 10-year period reviewed in this study, some diagnostic protocols were altered or added to the diagnostic battery due to technological development or the evolving understanding of SNHL in children.…”

Section: Methodsmentioning

confidence: 99%

The etiological evaluation of sensorineural hearing loss in children

et al. 2019

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This study aims to evaluate the etiology of pediatric sensorineural hearing loss (SNHL). A total of 423 children with SNHL were evaluated, with the focus on the determination of causative genetic and acquired etiologies of uni- and bilateral SNHL in relation to age at diagnosis and severity of the hearing loss. We found that a stepwise diagnostic approach comprising of imaging, genetic, and/or pediatric evaluation identified a cause for SNHL in 67% of the children. The most common causative finding in children with bilateral SNHL was causative gene variants (26%), and in children with unilateral SNHL, a structural anomaly of the temporal bone (27%). The probability of finding an etiologic diagnosis is significantly higher in children under the age of 1 year and children with profound SNHL. Conclusions : With our stepwise diagnostic approach, we found a diagnostic yield of 67%. Bilateral SNHL often has a genetic cause, whereas in unilateral SNHL structural abnormalities of the labyrinth are the dominant etiologic factor. The diagnostic yield is associated with the age at detection and severity of hearing loss: the highest proportion of causative abnormalities is found in children with a young age at detection or a profound hearing loss. What is Known: • Congenital sensorineural hearing loss is one of the most common congenital disorders • Determination of the cause is important for adequate management and prognosis and may include radiology, serology, and DNA analysis What is New: • Using a stepwise diagnostic approach, causative abnormalities are found in 67% both in uni- and bilateral SNHL, with the highest diagnostic yield in very young children and those suffering from profound hearing loss • Bilateral SNHL often has a genetic cause, whereas in unilateral SNHL structural abnormalities of the labyrinth are the dominant etiologic factor Electronic supplementary material The online version of this article (10.1007/s00431-019-03379-8) contains supplementary material, which is available to authorized users.

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Comprehensive medical evaluation of pediatric bilateral sensorineural hearing loss

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Bouzaher

Cohen

et al. 2021

Laryngoscope Investig Oto

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Children with bilateral sensorineural hearing loss (SNHL) should undergo a comprehensive medical evaluation to determine the underlying etiology and help guide treatment and counseling. In this article, we review the indications and rationale for medical evaluation of pediatric bilateral SNHL, including history and physical examination, imaging, genetic testing, specialist referrals, cytomegalovirus (CMV) testing, and other laboratory tests. Workup begins with a history and physical examination, which can provide clues to the etiology of SNHL, particularly with syndromic causes. If SNHL is diagnosed within the first 3 weeks of life, CMV testing should be performed to identify patients that may benefit from antiviral treatment. If SNHL is diagnosed after 3 weeks, testing can be done using dried blood spots samples, if testing capability is available. Genetic testing is oftentimes successful in identifying causes of hearing loss as a result of recent technological advances in testing and an ever-increasing number of identified genes and genetic mutations. Therefore, where available, genetic testing should be performed, ideally with next generation sequencing techniques. Ophthalmological evaluation must be done on all children with SNHL. Imaging (high-resolution computed tomography and/or magnetic resonance imaging) should be performed to assess for anatomic causes of hearing loss and to determine candidacy for cochlear implantation when indicated. Laboratory testing is indicated for certain etiologies, but should not be ordered indiscriminately since the yield overall is low.

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Evaluation of the outcome of CT and MR imaging in pediatric patients with bilateral sensorineural hearing loss

Cited by 19 publications

References 10 publications

Imaging findings in pediatric single‐sided deafness and asymmetric hearing loss

Imaging findings in pediatric single‐sided deafness and asymmetric hearing loss

The etiological evaluation of sensorineural hearing loss in children

Comprehensive medical evaluation of pediatric bilateral sensorineural hearing loss

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