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Vestibular Dysfunction in Pediatric Patients With Congenital Cytomegalovirus Infection and Hearing Loss: Occurrence
Guangwei Zhou, Hannah Peterson1, Alice Yun1
1Department of Otolaryngology and Communication Enhancement, Boston Children's Hospital, Waltham, Massachusetts.
Insights
Children with congenital cytomegalovirus (CMV) infection often experience significant vestibular dysfunction, frequently bilateral. Early balance and vestibular evaluation is crucial for managing this common complication beyond hearing loss.
Area of Science:
- Pediatric Otolaryngology
- Neuroscience
- Infectious Diseases
Background:
- Congenital cytomegalovirus (CMV) infection is a leading cause of non-genetic sensorineural hearing loss in children.
- Vestibular dysfunction is an underrecognized consequence of congenital CMV infection.
- The prevalence and characteristics of vestibular loss in this population require further elucidation.
Purpose of the Study:
- To investigate the incidence of vestibular dysfunction in pediatric patients diagnosed with congenital CMV infection.
- To characterize the nature of vestibular loss, including laterality and affected vestibular end-organs.
- To compare vestibular function in congenital CMV patients with a control group having hearing loss due to GJB2 mutations.
Main Methods:
- Retrospective study conducted at a tertiary pediatric referral center.
- Included 50 pediatric patients with congenital CMV infection and hearing loss, and 48 controls with GJB2-associated hearing loss.
- Utilized comprehensive vestibular evaluations, including Videonystagmography, rotary chair testing, and cervical vestibular evoked myogenic potentials.
Main Results:
- 70% of children with congenital CMV infection exhibited bilateral vestibular loss, and 14% had unilateral loss.
- In contrast, only 19% of the GJB2 control group showed bilateral vestibular loss, and 4% had unilateral loss.
- Developmental and motor delays were noted in 70% of the congenital CMV cohort.
Conclusions:
- Congenital CMV infection poses a high risk for significant vestibular loss, affecting both semicircular canals and otoliths.
- Vestibular dysfunction is a common comorbidity alongside hearing loss in congenital CMV.
- Routine, comprehensive vestibular and balance assessments are recommended for early detection and management in children with congenital CMV infection.
Objective:
To explore the occurrence of vestibular dysfunction in pediatric patients with congenital cytomegalovirus (CMV) infection and define the characteristics of vestibular loss in this population.
Study Design:
Retrospective study with controls.
Setting:
Tertiary pediatric referral center.
Patients:
Pediatric patients with hearing loss associated with congenital CMV infection and GJB2 mutation(s).
Interventions:
Balance and vestibular evaluation.
Main Outcome Measures:
Normal versus abnormal results of laboratory vestibular testing.
Results:
A total of 50 pediatric patients with congenital CMV infection, average age = 3.4 years (ranging from 7 mo to 12 yr, SD = 2.9 yr), underwent vestibular workup, including Videonystagmography, rotary chair test, and cervical vestibular evoked myogenic potential test. Of these children with CMV infection, 35 (70%) had bilateral vestibular loss, 7 (14%) had unilateral loss, and only 8 (16%) had a normal vestibular workup. In contrast, among the 48 pediatric patients without a history of CMV infection and with hearing loss associated with GJB2 mutation(s), only 9 (19%) had bilateral vestibular loss, 2 (4%) had unilateral loss, while 37 (77%) had a normal vestibular workup. Developmental/motor delay was documented in 35 (70%) patients with congenital CMV infection and hearing loss.
Conclusions:
Children with congenital CMV infection are at high risk for vestibular loss, which is frequently bilateral, impacting the semicircular canals and otolith organs, not just the well-known hearing loss. A comprehensive balance and vestibular workup is warranted for children with congenital CMV infection with the goal of early identification of vestibular dysfunction and earlier management.
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