1. DISCHER J, ESTEVE-FRAYSSE MJ , CASPER C, BENARD M, CALMELS MN
VESTIBULAR DISORDERS IN CONGENITAL CYTOMEGALOVIRUS INFECTION: EVALUATION AND IMPACT
Good morning
I’m Julie Discher, from the ENT department of Pr Deguine, Toulouse, France
I’m going to present you an evaluation of vestibular disorders in congenital cytomegalovirus infection.
DISCHER J, ESTEVE-FRAYSSE MJ , CASPER C, BENARD M, CALMELS MN
20 – 06 – 2016

2. CONGENITAL CMV
1st infectious cause of congenital hearing loss and neurological disability
Developmental delay without neurological disability Clinical vestibular impairments ?
Nassar MN
Congenital CMV is the first infectious cause of congenital hearing loss and neurological disability.
Histologic vestibular lesions have been described in fetuses with congenital CMV.
Yet, a lack of vestibular informations affects psychomotor development.
In some children with congenital CMV, a developmental delay without neurological disability has been observed.
Our study’s objective was to assess the prevalence of vestibular impairments in a population of children with congenital CMV infection and their impact on main stages of postural development.
Assess the prevalence of vestibular impairments in a population of children with congenital CMV infection and their impact on main stages of postural development.

3. METHODS Prospective, single-center, transversal study
Vestibular assessment proposed to children born between 2005 and with a congenital CMV infection
(positive urine culture between 0-15th day and/or positive dried blood spot and/or body of clinical and radiological evidence)
Exclusion criteria:
- Refusal
- Age < 3 months
- Cochlear implant
It was a prospective, transversal study.
We proposed a vestibular assessment to parents of children born between 2005 and 2015, followed at the university hospital center in toulouse, diagnosed with a positive urine culture between birth and 15th day and/or positive dried blood spot and/or body of clinical and radiological evidence.
Exclusion criteria were parents’ refusal, less than 3 months of age, a cochlear implant.

4. ASSESSMENT Posturomotor control milestone Hearing evaluation
< 6 months
Clinical vestibular assessment
Paraclinical assessment
Head holding
Sitting without support
Independant walking
Otoscopy
Walking observation
Oculomotricity
Balance tests
VNS
Video Head Impulse Test (VHIT)
Cervical vestibular evoked myogenic potentials (cVEMP)
Rotatory chair test
Caloric tests
ABR
TEOAE
Behavorial audiometry test
For the assessment,
We asked to parents the age of head holding, sitting without support and independant walking.
A recent hearing evaluation was necessary, and we performed an otoscopy.
A vestibular clinical assessment was performed : walking and oculomotricity observation, balance tests and a videonystagmoscopy.
This assessment was completed with paraclinical vestibular examinations : a video head impulse test, cervical vestibular evoked myogenic potentials, rotatory chair tests and caloric tests.

5. RESULTS - POPULATION
24 patients Mean age : 2 years 11 months (3 months – 10 years) Warning signs of CMV infection: - maternal: 12 (54,2%) - fetal/neonatal: 11 (45,8%) Clinical form: 7 (29%) symptomatic at birth, 8 (33,3%) symptomatic at examination 5 (20,8%) with hearing loss Brain MRI (17 patients, 12 antenatal, 6 postnatale): - abnormal (hyperintense signal in the white matter ± ventriculomegaly, abnormal gyration, microcephaly): 5 (29,4%) - normal: 12 (70,6%)
24 patients participated.
Mean age was 35 months.
29% were symptomatic at birth, one third were symptomatic at the examination.
Diagnosis was done on maternal infectious signs or on a systematic blood test in 54,2% of cases, and on fetal/neonatal signs such as sonographic findings, suggestive symptoms at birth or a developmental delay in 45,8% of cases.
20,8% had a hearing loss, 2 had a bilateral hearing loss.
17 patients had a brain MRI, 5 had anomalies (hyperintense signal in the white matter, abnormal gyration…)

6. RESULTS PARACLINICAL VESTIBULAR ASSESSMENT
Horizontal canal function
Saccular otolith function
VHIT
High frequencies
Rotatory chair test
Medium frequencies
Caloric test
Low frequencies
cVEMP n 19 9 24 16
Normal
13 (69%)
7 (78%)
15 (62%)
9 (56%)
Unilateral damage
5 (26%) -
5 (21%)
5 (31%)
Bilateral damage
1 (5%)
4 (17%)
2 (13%)
Abnormal test
6 (31%)
2 (22%)
9 (38%)
7 (44%)
31% had an impaired VHIT (high frequencies for horizontal canal), 22% had an abnormal rotatory chair test (medium frequencies), 38% had abnormal caloric test (low frequencies). 44% of patients had an impaired saccular otolith function.
Among them, 3 had a unilateral and partial impaired saccular otholith function, without any other vestibular impairment : we considered their vestibular assessment as normal, the results of this examination depends on the quality of the muscle contraction.
One patient had discordant results due to the inflammatory local state in a context of serous otitis media, which weren’t included for data processing.
34,8% had an impaired horizontal canal function.
Partial unilateral response for cVEMP without canalar dysfonction : normal assessment
One excluded patient: conflicting results – OME
Horizontal canal dysfunction: 34,8% (8/23)

7. VESTIBULAR IMPAIRMENTS AND CMV CLINICAL EXPRESSION
AT BIRTH
AT EVALUATION
CMV clinical expression at birth (N=7)
Frequency
Microcephaly
3 (12,5%)
Hearing loss
1 (4,2%)
Hematologic disorders
2 (8,3%)
Growth delay
Neurological disorders
Organomegaly
Hepatitis
Hypotonia
CMV pneumonitis
CMV clinical expression at evaluation (N=8)
Frequency
Hearing loss
5 (20,8%)
Posturo-motor delay
6 (25%)
Neurological disorders
4 (16,7%)
Ophtalmologic disorders
2 (8,3%)
We observed all bilateral vestibular impairments in children with clinical expression of CMV infection at birth. More than half of them had a vestibular impairment, while only one quarter in children asymptomatic at birth.
Likewise,
All bilateral impairments were diagnosed in children with a clinical expression of CMV at the evaluation. Three quarters had an altered vestibular function, compared with only 13,3% of asymptomatic children.
All bilateral vestibular impairments were diagnosed among symptomatic children.

8. VESTIBULAR IMPAIRMENTS AND HEARING LOSS
Hearing tresholds
(loss in decibels in bone conduction)
Number of ears (N=48)
Normal
41 (85,4%)
Mild hearing loss (21 to 40 dB)
Moderate hearing loss (41 to 70 dB)
1 (2,1%)
Severe hearing loss (71 to 90 dB)
3 (6,2%)
Profound hearing loss (>90 dB)
2 (4,2%)
Cophosis (no sound perception)
80% of children with hearing loss and only 22,2% of children with normal hearing had a vestibular impairment.
Vestibular impairment :
80% of children with hearing loss
22,2% of children with normal hearing tresholds

9. VESTIBULAR IMPAIRMENTS AND POSTUROMOTOR DEVELOPMENT
At 4 months, 93,3% of patients with normal vestibular function hold their head while only 50% of patients with horizontal canal function alteration.
At 9 months, 92,9% of patients with normal vestibular fuction sit without support while only 28,6% of patients with horizontal canal function alteration.
Among all patients, at 16 months, 92,9% with normal evaluation walk independantly against 16,7% with horizontal canal function alteration.
Months
Head holding
at 4 months
Sitting without support
At 9 months
Independant walking
at 16 months

10. VESTIBULAR IMPAIRMENTS AND POSTUROMOTOR DEVELOPMENT
INDEPENDANT WALKING
The distribution of age of independant walking suggests an actual impact of vestibular function on this stage : none of the children with neurological disability walks, so there is no neurological bias for this particular stage.
Thus, the median age of independant walking is 12 months for children with normal assessment, versus 18 months in case of horizontal canal function’s alteration.
Median age of acquisition of the stages of posturo-motor development by type of vestibular impairment

11. RESULTS 20,8% (5/24) of hearing loss
34,8% (8/23) with horizontal canal dysfunction including:
- 57,1% of children symptomatic at birth
- 25% of children asymptomatic at birth
Vestibular impairment is often linked to hearing loss (80%), but can occur when hearing is normal (22,2%).
Vestibular impairments impact the age of independant walking.
All bilateral impairments are seen in children with clinical expression of CMV infection.
In our 23 cases’ set, 34,8% of children have a horizontal canal dysfunction, 20,8% have a hearing loss.
80% of patients with hearing loss have a vestibular impairment, versus 22,2% of children with normal hearing.
57,1% of children with clinical expression at birth and 25% of asymptomatic children had a horizontal canal function alteration.
All bilateral impairments were observed in children with clinical expression of CMV, at birth or at examination.

12. DISCUSSION
1st study assessing the prevalence of vestibular impairments on asymptomatic children
Results are consistent with other studies:
Zagólski % (infants)
Karltorp % (profound hearing loss, after cochlear implantation)
Bernard ,3% (children with hearing loss)
Adaptability of paraclinical vestibular assessment for children
Methodological limitations
This study is, to our knowledge, the first assessing vestibular impairments and their impact on posturo-motor development on a group of asymptomatic children,
It allowed us to confirm the adaptability of paraclinical vestibular assessment for children.
The small sample size prevented us from making a statistical survey, and thus remains merely descriptive.

13. CONCLUSION
Vestibular impairments seem more frequent than hearing impairments.
Vestibular impairments are more frequently bilateral in children with a clinical expression of CMV congenital infection at birth or at examination.
To conclude, we can say that prevalence of vestibular impairments seems higher than of hearing impairments ; we need to confirm those results with larger studies.
These impairments are more serious in children with a clinical expression of CMV infection.
In asymptomatic children, vestibular impairments are unilateral, rare, and have no impact on posturomotor development.
A vestibular assessment lasts on average one hour and includes tests which can be hard for children and parents. The analysis of our results defines a target population and a vestibular assessment should be recomended in all children with clinical expression of CMV, between 6 and 12 months, to guide their psychomotor education.
Vestibular assessment should be recomended in all children with clinical expression of congenital CMV infection between 6 and 12 months
Psychomotor education

14. Thanks to team and families!