Objectives: Congenital cytomegalovirus (cCMV) infection is the most common nongenetic cause of sensorineural hearing loss in children. Due to the close anatomical relationship between the auditory and the vestibular sensory organs, cCMV can also be an important cause of vestibular loss. However, the prevalence and nature of cCMV-induced vestibular impairment is still underexplored. The aim of this study was to investigate the occurrence and characteristics of vestibular loss in a large group of cCMV-infected children, representative of the overall cCMV-population. Design: Ninety-three children (41 boys, 52 girls) with a confirmed diagnosis of cCMV were enrolled in this prospective longitudinal study. They were born at the Ghent University Hospital or referred from another hospital for multidisciplinary follow-up in the context of cCMV. The test protocol consisted of regular vestibular follow-up around the ages of 6 months, 1 year, 2 years, and 3 years with the video Head Impulse Test, the rotatory test, and the cervical Vestibular Evoked Myogenic Potential test. Results: On average, the 93 patients (52 asymptomatic, 41 symptomatic) were followed for 10.2 months (SD: 10.1 mo) and had 2.2 examinations (SD: 1.1). Seventeen (18%) patients had sensorineural hearing loss (7 unilateral, 10 bilateral). Vestibular loss was detected in 13 (14%) patients (7 unilateral, 6 bilateral). There was a significant association between the occurrence of hearing loss and the presence of vestibular loss (p < 0.001), with 59% (10/17) vestibular losses in the group of hearing-impaired children compared to 4% (3/76) in the group of normal-hearing subjects. In the majority of the cases with a vestibular dysfunction (85%, 11/13), both the semicircular canal system and the otolith system were affected. The remaining subjects (15%, 2/13) had an isolated semicircular canal dysfunction. Sixty-one patients already had at least one follow-up examination. Deterioration of the vestibular function was detected in 6 of them (10%, 6/61). Conclusions: cCMV can impair not only the auditory but also the vestibular function. Similar to the hearing loss, vestibular loss in cCMV can be highly variable. It can be unilateral or bilateral, limited or extensive, stable or progressive, and early or delayed in onset. As the vestibular function can deteriorate over time and even normal-hearing subjects can be affected, vestibular evaluation should be part of the standard otolaryngology follow-up in all children with cCMV.
Due to the close anatomical relationship between the auditory and vestibular end organs, hearing-impaired children have a higher risk for vestibular dysfunction, which can affect their (motor) development. Unfortunately, vestibular dysfunction often goes unnoticed, as vestibular assessment in these children is not standard of care nowadays. To timely detect vestibular dysfunction, the Vestibular Infant Screening–Flanders (VIS–Flanders) project has implemented a basic vestibular screening test for hearing-impaired infants in Flanders (Belgium) with a participation rate of 86.7% during the first year and a half. The cervical Vestibular Evoked Myogenic Potentials (cVEMP) test was applied as vestibular screening tool to map the occurrence of vestibular (mainly saccular) dysfunction in this population. At the age of 6 months, 184 infants were screened. No refers on vestibular screening were observed in infants with permanent conductive hearing loss. In infants with permanent sensorineural hearing loss, a cVEMP refer rate of 9.5% was observed. Failure was significantly more common in infants with severe-profound compared to those with mild-moderate sensorineural hearing loss (risk ratio = 9.8). Since this is the first regional study with a large sample size and successful participation rate, the VIS–Flanders project aims to set an example for other regions worldwide.
Objectives: Given the close interconnection between the auditory and vestibular end organs, the increasingly broad application of (bilateral) cochlear implantation (CI) in children raises concern about its impact on the vestibular function. Unfortunately, literature on this matter is inconclusive and subject to several limitations. Therefore, this study aimed to elucidate the impact of pediatric CI on the vestibular function in a large sample of children, representative for the current CI population. Design: Fifty hearing-impaired children followed in the Ghent University Hospital were included in this prospective study. Twenty-seven patients underwent unilateral CI, and 23 were bilaterally implanted (9 sequentially, 14 simultaneously), adding up to 73 implanted ears. Children’s median age at first implantation was 29 (range 8 to 194) months. Vestibular assessment was scheduled on average 2.8 months (SD: 3.6) before and 4.6 (SD: 4.0) months after implantation and consisted of video Head Impulse Testing of the lateral semicircular canals, rotatory testing (0.16, 0.04, and 0.01 Hz) and cervical vestibular evoked myogenic potential (cVEMP) testing with bone conduction stimulation. Caloric testing was added in children older than 3 years of age. Results: Overall, group analysis in our sample of 73 CI-ears did not reveal any significant impact on the vestibular function, except for a significantly shortened ipsilateral N1 latency of the cVEMP responses (p = 0.027) after CI. Complete ipsilateral loss of function after implantation was seen in 5% (3/54) of all CI-ears on the video head impulse testing, in 0% (0/10) on the caloric test and in 2% (1/52) on the cVEMP, notably all patients deafened by a congenital cytomegalovirus infection. Conclusions: The impact of CI on the vestibular function in our dataset was limited. Therefore, the many advantages of simultaneous bilateral implantation may outweigh the risk for vestibular damage postoperatively. However, the impact on the vestibular function may be dependent on various factors (e.g., etiology of the hearing loss), and the clinical outcome is still difficult to predict. Vestibular assessment remains thus an important aspect in the pediatric CI population; first because the vestibular function should be considered in the decision-making process on (simultaneous or sequential bilateral) CI and second because it is essential to reveal a possible additional sensory deficit, allowing an opportunity for rehabilitation to improve the overall outcome of these children.
Objectives: Although vestibular deficits can have severe repercussions on the early motor development in children, vestibular assessment in young children has not yet been routinely integrated in clinical practice and clear diagnostic criteria to detect early vestibular deficits are lacking. In young children, specific adjustments of the test protocol are needed, and normative data are age-dependent as the vestibular pathways mature through childhood. Therefore, this study aims to demonstrate the feasibility of an extensive age-dependent vestibular test battery, to provide pediatric normative data with the concurrent age trends, and to offer a clinical framework for pediatric vestibular testing. Design: This normative study included 133 healthy children below the age of 4 years (mean: 22 mo, standard deviation: 12.3 mo, range: 5–47 mo) without history of hearing loss or vestibular symptoms. Children were divided into four age categories: 38 children younger than 1 year old, 37 one-year olds, 33 two-year olds, and 25 three-year olds. Children younger than 3 years of age were examined with the video Head Impulse Test (vHIT) of the horizontal semicircular canals, cervical vestibular evoked myogenic potentials (cVEMP) with bone conduction stimuli, and the rotatory test at 0.16, 0.04, and 0.01 Hz. In 3-year old children, the vHIT of the vertical semicircular canals and ocular vestibular evoked myogenic potentials (oVEMP) using a minishaker were added to the protocol. Results: The horizontal vHIT appeared to be the most feasible test across age categories, except for children younger than 1-year old in which the success rate was the highest for the cVEMP. Success rates of the rotatory test varied the most across age categories. Age trends were found for the vHIT as the mean vestibulo-ocular reflex (VOR) gain increased significantly with age (r = 0.446, p < 0.001). Concerning the cVEMP, a significant increase with age was found for latency P1 (r = 0.420, p < 0.001), rectified interpeak amplitude P1-N1 (r = 0.574, p < 0.001), and averaged electromyographic (EMG) activity (r = 0.430, p < 0.001), whereas age trends for the latency N1 were less pronounced (r = 0.264, p = 0.004). Overall, the response parameters of the rotatory test did not show significant age effects (p > 0.01), except for the phase at 0.01 Hz (r = 0.578, p < 0.001). Based on the reported success rates and age-dependent normative vestibular data, straightforward cutoff criteria were proposed (vHIT VOR gain < 0.7, cVEMP rectified interpeak amplitude < 1.3, oVEMP interpeak amplitude < 10 µV) with accompanying clinical recommendations to diagnose early vestibular impairment. Conclusions. In this large cohort of typically developing children below the age of 4 years, the vHIT and cVEMP were the most feasible vestibular tests. Moreover, the age-dependent normative vestibular data could specify age trends in this group of young children. Finally, based on the current results and clinical experience of more than ten years at the Ghent University Hospital (...
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