Clinical profile of hearing loss in children with congenital cytomegalovirus (CMV) infection: CMV DNA diagnosis using preserved umbilical cord

Figures & data

Table I. Summary of children with bilateral and unilateral hearing loss.

Hearing loss	Gender (n)	Hearing level (dB)	Affected side (n)	Severe to profound hearing loss		Mild to moderate hearing loss
				n	Age at diagnosis (months)	n	Age at diagnosis (months)
Total (n = 134)	M = 70, F = 64			101 (75.4%)	34.4 ± 34.7	33 (24.6%)	48.8 ± 38.7
Bilateral (n = 46)	M = 31, F = 15	71.8 (R) 71.7 (L)		28 (20.9%)	16.6 ± 19.9	18 (13.4%)	11.1 ± 39.1
Unilateral (n = 88)	M = 39, F = 49	89.5 (W) 13.6 (B)	R = 43, L = 45	73 (54.5%)	41.2 ± 36.6	15 (11.2%)	40.3 ± 36.8

M, male; F, female; R, right; L, left; B, better hearing ear; W, worse hearing ear.

Figure 1. An original amplification plot of real-time PCR in case no. 12 with positive CMV DNA. CMV DNA in positive control and case 12 (A) and genomic DNA (GJB2 gene) in positive control, case no. 12 and negative control (B) are amplified. These results show that our real-time PCR method is precise. Blank, sample without any added DNA.

Table II. Results of CMV DNA test combined with genetic deafness testing in bilateral sensorineural hearing loss (SNHL).

Test		Severe to profound SNHL (n = 28)	Mild to moderate SNHL (n = 18)	Total (n = 46)
CMV DNA test	Positive	4 (14.3%)	0 (0%)	4 (8.7%)
Deafness gene test	GJB2	6 (21.4%)	1 (5.6%)	7 (15.2%)
	SLC26A4	3 (10.7%)	0 (0%)	3 (6.5%)
	Other	0 (0%)	0 (0%)	0 (0%)
	Total	9 (32.1%)	1 (5.6%)	10 (21.7%)
Total diagnostic rate		46.4%	5.6%	30.4%

CMV, cytomegalovirus; SNHL, sensorineural hearing loss.

Table III. Results of CMV DNA test combined with genetic deafness test in unilateral sensorineural hearing loss (SNHL).

Test		Severe to profound SNHL (n = 73)	Mild to moderate SNHL (n = 15)	Total (n = 88)
CMV DNA test	Positive	7 (9.6%)	1 (6.7%)	8 (9.1%)
Deafness gene test	GJB2	0 (0%)	0 (0%)	0 (0%)
	Other	0 (0%)	0 (0%)	0 (0%)
	Total	0 (0%)	0 (0%)	0 (0%)
Total diagnostic rate		9.6%	6.7%	9.1%

Table IV. Clinical data of children positive for CMV DNA.

Case no.	Sex	Age at diagnosis (months)	Bilateral/unilateral	Affected side	Severity	Average HL (R/L) (dB)	FL/P/NA	Onset	NHS	Delta Ct
1	F	60	Bilateral	R/L	Profound	87.5/108.8	FL/P	Late	Pass	7.52
2	F	52	Bilateral	R/L	Profound	87.5/110	FL/P	Late	Pass	8.58
3	M	50	Bilateral	R/L	Profound	100.0/100.0	P	Late	Pass	12.94
4	M	62	Bilateral	R/L	Profound	110/46.3	−	Likely late	−	0.07
5	M	6	Unilateral	L	Profound	32.5/103.8	−	Congenital	Refer (L)	8.01
6	M	65	Unilateral	R	Profound	107.5/17.5	−	Unknown	−	11.64
7	M	50	Unilateral	L	Profound	6.3/100.0	−	Unknown	−	13.59
8	F	98	Unilateral	R	Profound	110/15	−	Unknown	−	11.67
9	F	55	Unilateral	L	Profound	15.0/92.5	−	Late	Pass	8.3
11	F	2	Unilateral	R	Profound	90.0/18.3	NA	Congenital	Refer (R)	0.46
10	M	80	Unilateral	L	Severe	13.3/70.0	−	Unknown	−	14.32
12	F	44	Unilateral	L	Moderate	15.0/58.3	FL/P	Late	Pass	9.41

NHS, Newborn Hearing Screening; FL, fluctuation of hearing loss; P, progressive hearing loss; NA, not applicable.

Table V. Review of previous reports.

Reference	Year	Subjects	CMV positive rate			Progression (%)	Late-onset (%)	Study design	Diagnostic methods	Country
			Total	Bilateral	Unilateral
Barbi et al. [12]	2003	>40 dB HL	9/79 (11.4%)	1/37 (2.7%)	8/42 (19%)	NR	NR	Retrospective	DBS, qPCR	Italy
Ogawa et al. [3]	2007	>20 dB, nonsyndromic SNHL	10/67 (10.5%)	9/63 (14.3%)	1/4 (25%)	NR	NR	Retrospective	UC, PCR	Japan
Samileh et al. [15]	2008	>40 dB HL	33/95 (34.7%)	NR/75	NR/20	NR	NR	Prospective	Serologic test	Iran
Stehel et al. [16]	2008	NHS refer	16/256 (6%)	16/256 (6%)	NR	NR	NR	Prospective	DNA from urine	USA
Walter [20]	2008	Unexplained SNHL	8/35 (22.9%)	NR	NR	NR	NR	Retrospective	DBS, qPCR	UK
Mizuno [21]	2008	Only bilateral	3/45 (6.7%)	3/45 (6.7%)	0	NR	NR	Prospective	UC, qPCR	Japan
Jakubikova et al. [14]	2009	>60 dB HL, NHS refer	4/71 (5.6%)	4/55 (7.3%)	0/16 (0%)	NR	NR	Prospective	Serologic test	Slovak Republic
Boudewyns [22]	2009	NHS refer, >20 dB	4/55 (7.3%)	NR	NR	NR	NR	Retrospective	DBS, qPCR	Belgium
Choi [23]	2009	NHS refer	13/479 (2.7%)	13/479 (2.7%)	NR	NR	NR	Retrospective	DBS, qPCR	USA
Tagawa [24]	2009	>70 dB, deaf schoolchildren	3/26 (11.5%)	3/26 (11.5%)	0 (0%)	2 (7.7%)	1 (3.9%)	Retrospective	UC, qPCR	Japan
Kimani [25]	2010	NHS refer	11/109 (10.1%)	8/92 (8.8%)	3/17 (17.6%)	NR	NR	Retrospective	DBS, qPCR	USA
Adachi [26]	2010	NHS refer, >35 dB, bilateral	13/77 (17%)	13/77 (17%)	0	NR	NR	Retrospective	UC, qPCR	Japan

NR, not reported; DBS, dried blood spot; UC, umbilical cord; qPCR, quantitative PCR.

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