1. 先天性巨細胞病毒感染對於兒童聽損之影響：初報
羅大烜 吳振吉 許巍鐘 呂俊毅 劉殿楨 許權振
台大醫院耳鼻喉部

2. Introduction
Sensorineural hearing loss (SNHL) is the most common sensory defect in children
Among non-genetic causes of SNHL, congenital cytomegalovirus (cCMV) infection is the most prevalent1,2
cCMV infection accounts for 10-20% SNHL in children1,2

3. Congenital CMV infection
( % in industrialized contries) 2-4
~1/10
~9/10
Symptomatic Neonates
Asymptomatic Neonates
• IUGR
• Microcephaly
• Petechiae
• Hepatosplenomegaly
• Encephalitis
• CNS defect (ex. SNHL)

4. Congenital CMV infection
( % in industrialized contries) 2-4
~1/10
~9/10
Symptomatic Neonates
Asymptomatic Neonates
40-50%
13.5%
Sequelae
Sequelae
Hearing loss: most common
Congenital or progressive
Bilateral or unilateral
Treatable w/ antiviral mx

5. Patients cCMV infection N=35 Asymptomatic at birth N= 16 Passed NHS
(2014 ~, NTUH)
Asymptomatic at birth
N= 16
Passed NHS
N=16
Failed NHS
N=0
N=12
N=5
No NHS data
N=2
Symptomatic at birth
N= 19
NHS: New born hearing screen

6. Patients cCMV infection N=35 Asymptomatic at birth N= 16 Passed NHS
(2014 ~, NTUH)
Asymptomatic at birth
N= 16
Passed NHS
N=16
Failed NHS
N=0
N=12
N=5
No NHS data
N=2
Symptomatic at birth
N= 19
NHS: New born hearing screen

7. Assessments CMV viral load Audiologic assessments
Blood serum viral load (cp/ml)
Initial viral load: viral load tested within 1 m after birth
Viral suppression: serum viral load reduced to undetectable
Audiologic assessments
AABR, DPOAEs, ABR, sound field, PTA
Grades of hearing loss5
26-40dB
Mild
41-70dB
Moderate
71-90dB
Severe
>91dB
Profound

8. Demographic data Characteristics N (%) Total 35 Male 24 69% Female 11
31%
Passed NHS 21
60%
Failed NHS 12
34%
No NHS data 2 6%
Asymptomatic 16
46%
Symptomatic 19
54% HL 15
43%
Unilateral HL 7
20%
Bilateral HL 8
23%
No HL
No Hearing test data 1 3%

9. NHS or neonatal symptoms as predictors?
100 (12/12)
83.3 (15/18)
9.5 (2/21)
0 (0/16)
p < 0.01
p < 0.01

10. Unilateral vs. Bilateral HL
Bilateral 較嚴重? => no
P>0.05

11. Initial viral load vs. Hearing features
Initial viral load vs. NHS
Initial viral load vs. HL
p = 0.09
p = 0.07
Pass NHS
Fail NHS
Initial VL (cp/ml)
No HL HL
Initial VL (cp/ml)
Boxplot of Initial VL and for NHS and HL. Box, defined by quartiles Q1 and Q3; bar, median; whiskers, lowest and highest value; o, outliers

12. Viral suppression and HL
Serum viral load decreased gradually in most patients
Most patients achieved serum viral suppression before 1 y (median = 4.5 m)
Most patients showed stable hearing levels as the viral load decreased
Some exceptions
Throat/Urine VL suppression date?

13. Special case -1
Hearing loss deteriorated even after the viral load reached undetectable level

14. Special case -2
A case with immunodeficiency (Activated PI3K-delta syndrome)
Focus 在後面

15. Conclusions
Failing NHS or being symptomatic at birth are predictors of HL development in cCMV infection
Severity of hearing loss was not different between uni- and bilateral cases
Initial viral load was not significantly related to NHS or hearing loss development
Hearing deterioration could occur even in cases with serum viral suppression

16. Thanks for attention!!

17. Reference
Morton CC, Nance WE. Newborn hearing screening—a silent revolution. N Engl J Med 2006;354:
Goderis J, De Leenheer E, et al. Hearing loss and congenital CMV infection: a systematic review. Pediatrics 2014;134:
Dollard SC, Grosse SD, et al. New estimates of the prevalence of neurological and sensory sequelae and mortality associated with congenital cytomegalovirus infection. Rev Med Virol 2007;17:
Kenneson A, Cannon MJ. Review and meta-analysis of the epidemiology of congenital cytomegalovirus infection. Rev Med Virol 2007;17:
Julie G., et al., Hearing in Children with Congenital Cytomegalovirus Infection:Results of a Longitudinal Study, J Pediatr 2016;172:110-5