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Published on: August 30, 2019
Evidence of vestibular dysfunction in children with enlarged vestibular aqueduct
Guangwei Zhou1, Alicia Wang2, Jacob Brodsky1
1Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, Boston, MA, USA; Department of Otolaryngology-Head and Neck Surgery, Harvard Medical School, Boston, MA, USA.
Insights
Vestibular dysfunction is common in children with enlarged vestibular aqueduct (EVA). Early objective testing is crucial for identifying balance and vestibular impairments to guide rehabilitation.
Area of Science:
- Pediatric Otolaryngology
- Neuroscience
- Audiology
Background:
- Enlarged vestibular aqueduct (EVA) is a congenital inner ear malformation.
- EVA is associated with hearing loss and can impact balance and vestibular function.
- Understanding vestibular deficits in pediatric EVA is critical for management.
Purpose of the Study:
- To investigate the prevalence and characteristics of balance and vestibular deficits in children diagnosed with EVA.
- To highlight the importance of comprehensive vestibular evaluation in this population.
Main Methods:
- Retrospective review of 53 pediatric patients with EVA.
- Utilized a battery of vestibular function tests including VNG, rotary chair, vHIT, VEMP, SVV, and SOT.
- Included genetic testing for associated conditions like Pendred syndrome.
Main Results:
- Abnormal vestibular testing was frequent, with the highest rates in rotary chair (67%) and VEMP (55%).
- Specific deficits varied across tests: SOT (58%), vHIT (30%), SVV (39%), and VNG (8%).
- 37 out of 53 children had bilateral EVA, with 5 confirmed cases of Pendred syndrome.
Conclusions:
- Vestibular dysfunction is a significant and common finding in children with EVA.
- Clinicians must be vigilant for signs of balance and vestibular impairments in these patients.
- Objective vestibular testing is essential for early detection and to facilitate appropriate vestibular rehabilitation and balance retraining.
Objective:
To investigate the occurrence and characteristics of balance and vestibular deficits in pediatric patients with enlarged vestibular aqueduct (EVA).
Materials And Methods:
Retrospective review of 53 children with EVA who underwent a comprehensive vestibular evaluation in our pediatric balance and vestibular program. Laboratory testing included videonystagmography (VNG), rotary chair, video head impulse testing (vHIT), vestibular evoked myogenic potential (VEMP), subjective visual vertical (SVV) and Sensory Organization Test (SOT) in posturography.
Results:
The mean age of these children, 31 girls and 22 boys, was 7.1 years (SD = 4.8). Among these 53 children, 16 had unilateral EVA (7 on the left side and 9 on the right side) and 37 had bilateral EVA, in which genetic testing confirmed 5 cases of Pendred syndrome. Abnormal testing results were found in 58% (11/19) on SOT, 67% (32/48) on rotary chair, 55% (48/88 of ears) on VEMP, 30% (8/27) on vHIT, 39% (7/18) on SVV, and 8% (4/53) on VNG.
Conclusions:
Vestibular dysfunction may be a common finding in children with EVA. Clinicians who provide medical care for children with EVA need to be familiar with signs of potential balance and vestibular impairments. Although performing vestibular evaluation on young children with EVA can be difficult, objective testing is important in order to identify any potential vestibular deficit in these pediatric patients so that proper vestibular rehabilitation and balance retraining can be provided.
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