Pediatric Unilateral Sensorineural Hearing Loss Research Articles

Evaluating diagnostic yield of computed tomography (CT) and magnetic resonance imaging (MRI) in pediatric unilateral sensorineural hearing loss

IntroductionOptions for imaging for evaluation of pediatric patients with unilateral sensorineural hearing loss (USNHL) include computed tomography (CT) and magnetic resonance imaging (MRI). Although both CT and MR imaging provide valuable information in the evaluation of pediatric patients with USNHL, debate remains regarding which imaging modality is most ideal and should be the preferred study for these children. The objective of this study is to evaluate and compare the diagnostic yield of CT versus MRI in children with USNHL. MethodsA multi-institutional retrospective chart review was conducted. Pediatric patients with hearing loss (diagnosis codes 389.00–389.22) seen between 2010 and 2012 at three tertiary care centers were identified. Only patients with USNHL and imaging studies were reviewed and results of CT and MRI for each patient were examined and compared. Cochleovestibular or central nervous system findings known to directly correlate to SNHL were noted as positive findings on imaging. McNemar's test was used to compare patients with positive CT and MRI results. ResultsA total of 219 patients between the ages of 0–18 years with USNHL who underwent CT and/or MRI were identified. Imaging abnormalities were found in 41/96 patients who underwent MRI with overall diagnostic yield of 42.7% and 69 of 188 patients who underwent CT with overall diagnostic yield of 36.7%. For patients who underwent both imaging modalities (n = 65), there was no statistically significant difference in positive findings detected by CT vs MRI (p > 0.05). ConclusionsBoth CT and MR imaging have similar overall diagnostic yield when used to evaluate children with USNHL. Parents and patients should be counseled regarding cost, test duration, radiation exposure, need for sedation, and diagnostic accuracy associated with each imaging modality and these factors should be considered to select the appropriate diagnostic study.

Bony cochlear nerve canal stenosis in pediatric unilateral sensorineural hearing loss

ObjectivesThis study was performed to evaluate the frequency of bony cochlear nerve canal (BCNC) stenosis and its clinical significance in pediatric patients with unilateral sensorineural hearing loss (SNHL) of unknown etiology. Materials and methodsWe analyzed the medical records and temporal bone computed tomography (CT) results of patients less than 13 years of age with a diagnosis of unilateral SNHL of unknown etiology between July 2007 and July 2017. We compared the BCNC diameter between both sides and analyzed the age at diagnosis, degree of hearing loss, and accompanying inner ear anomalies. ResultsIn 42 patients, the mean age at diagnosis was 7.4 ± 3.6 years, and the average hearing level in the affected ear was 87.9 ± 20.0 dB HL (decibels hearing level). The average diameter of the BCNC was 1.22 ± 0.75 mm on the affected side and 1.96 ± 0.52 mm on the normal side. The most suitable criterion for BCNC stenosis appeared to be a diameter of 1.2 mm by the recursive partitioning procedure. With application of this criterion, the rate of BCNC stenosis was significantly greater on the affected side than on the normal side (52.4% vs. 4.8%, respectively; P < 0.05). A narrow internal acoustic canal was found in two patients, and vestibular and cochlear anomalies were found in three patients each. ConclusionsOur results suggest that it is reasonable to set a diameter of 1.2 mm as a cutoff for BCNC stenosis, and also that BCNC stenosis is a common cause of unilateral SNHL of unknown etiology in childhood.

Computed Tomography and Magnetic Resonance Imaging Evaluation in Pediatric Unilateral Sensorineural Hearing Loss

Computed Tomography and Magnetic Resonance Imaging Evaluation in Pediatric Unilateral Sensorineural Hearing Loss

Pediatric unilateral sensorineural hearing loss: implications and management.

The purpose of this review is to summarize current studies detailing the impact of unilateral sensorineural hearing loss in children and the most current modalities of treatment used in its management. Current studies corroborate historic views on the impact of unilateral sensorineural hearing loss on patient wellbeing and academic success and stress the use of additional surveillance and studies to diagnose those patients that may pass standard screening practices and suffer from lack of prompt and proper care. With respect to management, notable findings include the continuous development of improved conventional and contralateral routing of signal amplification devices that may act to provide alternatives to percutaneous bone-anchored hearing aid implantation. These include improvements in more conventional hearing aid technology, so as to bridge the performance gap with the classical bone-anchored hearing aid implant, and the development of partially implanted transcutaneous bone conduction hearing devices. Due to dissatisfaction with sound localization, a new and significant development is the burgeoning accumulation of research on cochlear implantation for the treatment of unilateral sensorineural hearing loss in children. With advances in technology in historic modalities of treatment, and the advent of new modalities such as cochlear implantation, the clinician has a wide armamentarium by which to provide treatment to patients based on clinical circumstances and patient desires.

The Yield of Multigene Testing in the Management of Pediatric Unilateral Sensorineural Hearing Loss.

To explore the role and yield of multigene evaluation in children recently diagnosed with unilateral sensori-neural hearing loss (SNHL). Retrospective cohort study. Tertiary level pediatric institution. Sixty children diagnosed with unilateral SNHL between January 2005 and December 2015. Targeted mutation analysis was performed in 51 children using DNA microarray for genotyping 11 different genes known to be correlated with nonsyndromic SNHL. Multigene testing results. Average age at diagnosis was 4.3 years. All children appeared to have nonsyndromic hearing loss (HL). HL was categorized as mild (17 children), moderate (17 children), severe (7 children), or profound (19 children). Genetic testing was performed in 51 (85%) children. Sixteen children (31.3%) were tested positive to connexin 26 (GJB2). One patient (2%) from this subgroup was homozygous and 15 were heterozygous. The average age of GJB2 positive children was 6 years and their average pure tone audiometry (PTA) was 75 dB. Computed tomography (CT) and/or magnetic resonance imaging (MRI) scans were performed in 43 children (71.66%). Out of the children who had a scan, 27 patients (62.8%) had negative findings on either CT or MRI scans. Sixteen (37.2%) patients had positive scan finding/s. Significant proportion of children with unilateral SNHL may have positive genetic testing while the vast majority of these children present with heterozygous mutations of connexin 26 (GJB2). Findings suggest that genetic evaluation has a role as a complementary modality in HL evaluation for pediatric unilateral SNHL although it may not be necessary to analyze for various abnormalities other than connexin 26 when practising in a limited resources environment.

Management of Pediatric Unilateral Sensorineural Hearing Loss

Management of Pediatric Unilateral Sensorineural Hearing Loss

Epidemiology of unilateral sensorineural hearing loss with universal newborn hearing screening

Compare the epidemiology of pediatric unilateral sensorineural hearing loss before and after implementation of universal newborn hearing screening in Missouri. Inception cohort. Charts of 134 children born between January 1, 1990 and December 31, 2007, diagnosed with unilateral sensorineural hearing loss at a single institution in Missouri were reviewed to determine the effects of universal newborn hearing screening on age of detection and etiology of hearing loss. Mean age of detection declined from 4.4 (standard deviation [SD] 1.8) to 2.6 (SD 2.6) years of age, whereas the rate of detection by 6 months of age increased from 3% to 42%. The majority (58%) of cases had normal hearing at birth. The most common etiological category was unknown (41%) before screening and congenital (45%) after screening. The use of magnetic resonance imaging has increased by 21% (2-fold), whereas use of computed tomography has declined by 8% since 2002. Yields of connexin, Pendred, electrocardiogram, and syphilis testing were 0/48 and 2/31 before and after screening, respectively. Implementation of universal newborn hearing screening in Missouri is associated with a decrease in age of detection of unilateral sensorineural hearing loss. The majority of cases are either not present or not detectable at birth. The combination of hearing status at birth and imaging findings suggests that the majority of cases are congenital rather than of unknown etiology.

小児一側性難聴児における CT 所見の検討

小児一側性難聴児における CT 所見の検討

08:18: Pediatric Unilateral Sensorineural Hearing Loss

08:18: Pediatric Unilateral Sensorineural Hearing Loss