Eur Arch Otorhinolaryngol DOI 10.1007/s00405-015-3646-1
OTOLOGY
Delayed-onset hearing loss in pediatric candidates for cochlear implantation Sung-Wook Jeong1 • Min-Young Kang1 • Jae-Ryong Kim2 • Lee-Suk Kim1
Received: 1 March 2015 / Accepted: 1 May 2015 Ó Springer-Verlag Berlin Heidelberg 2015
Abstract The objective of this study was to evaluate the clinical significance of delayed-onset hearing loss in children. Seventy-three children who underwent cochlear implantation (CI) were included. They were divided into a congenital hearing loss group (n = 50) and a delayed-onset hearing loss group (n = 23). The age at diagnosis of hearing loss, age at the beginning of auditory habilitation, the age at CI, and the postimplant speech perception abilities were compared between the two groups. Children in the congenital hearing loss group were confirmed to have hearing loss at a mean age of 0.3 years, and those in the delayed-onset hearing loss group were diagnosed with hearing loss at a mean age of 2.0 years. Auditory habilitation began at a mean age of 0.4 and 2.0 years, and CI was performed at a mean age of 1.4 and 2.6 years, respectively. Children in the congenital hearing loss group had better scores on speech perception tests than those in the delayed-onset hearing loss group, but the differences were not significant. About half of the children with delayedonset hearing loss (57 %) had risk factors associated with delayed-onset hearing loss. A high prevalence of delayedonset hearing loss was noted in the group of children who underwent CI. Risk factors for hearing loss were not found in 43 % of children with delayed-onset hearing loss. Universal screening for delayed-onset hearing loss needs to be performed during early childhood.
& Lee-Suk Kim [email protected] 1
Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Dong-A University, 3-1 Dongdaeshindong, Seo-gu, Busan 602-715, Korea
2
Department of Otolaryngology-Head and Neck Surgery, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
Keywords Delayed-onset hearing loss Newborn hearing screening Sensorineural hearing loss Cochlear implants
Introduction Of every 1000 children born, 1–3 have congenital sensorineural hearing loss (SNHL) [1]. In infants who have risk factors for hearing loss or who are treated in an intensive care unit, the risk of congenital hearing loss can be increased 10- to 50-fold [2–4]. Fortunately, children with congenital hearing impairment can achieve useful auditory verbal abilities if their hearing loss is detected early during the newborn period and an intervention using a hearing aid or cochlear implant is performed soon after detection of hearing loss. Because of the high incidence of congenital hearing loss and the significance of early detection and intervention for congenital hearing loss, most countries run universal newborn hearing screening programs. However, newborns who have mild hearing loss or hearing loss at specific frequencies (e.g. low-frequency hearing loss) may not be detected by newborn hearing screening even though they have congenital permanent hearing loss. Furthermore, some children who have normal hearing or who pass the newborn hearing screening because of mild hearing loss at birth can lose their hearing in early childhood [5]. Because these children pass the newborn hearing screening, early detection of their hearing loss is difficult soon after onset without special attention and intervention. Auditory input during early childhood is crucial for the development of normal auditory verbal abilities [6, 7], so special attention or screening for delayed-onset hearing loss is required. About three of 1000 children lose their hearing during infancy or early childhood [8]. The Joint Committee on Infant Hearing (JCIH) identified 11 risk factors for permanent
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Eur Arch Otorhinolaryngol
congenital, delayed-onset, and/or progressive hearing loss, and recommended diagnostic audiological evaluation at the age of 24–30 months for children who pass the newborn hearing screening, but have these risk factors to detect delayed-onset hearing loss [9]. Several studies concerned with the epidemiology of delayed-onset hearing loss have been published [3, 4, 10–12], but reports on the epidemiology of delayed-onset hearing loss in Asian people are lacking. Therefore, we performed this study to examine the epidemiology of delayed-onset hearing loss in Korean children and evaluate the significance of delayed hearing loss. About 480,000 children are born in South Korea each year. Given that one in every 1000 newborns is born with severe-to-profound SNHL, about 480 children are thought to be born with severe-to-profound SNHL each year in South Korea. Cochlear implantation (CI) surgery has been performed for about 300–400 children with hearing loss every year during the last 10 years in Korea. Therefore, most children born with severe-to-profound SNHL can be assumed to receive CI surgery. One of the reasons for the high rate of CI surgery in Korea is the availability of a National Health Insurance system, which is run by the Ministry of Health. Funding for the insurance is made by compulsory contributions from all residents, employers, and the government. All Korean residents pay about 2.95 % of their income as their insurance fee and their insurance covers 80 % of the cost of inpatient hospital care. Furthermore, several public corporations aid the poorest people who have financial difficulty in paying the 20 % gap in cost for inpatient hospital care. Therefore, most children with severeto-profound SNHL can receive CI surgery without financial difficulty in Korea. Because the annual number of pediatric CI surgeries in the young population under the age of 3 years is an approximation of the annual number of newborns who are born with severe-to-profound SNHL, a cohort of pediatric CI recipients is similar to the cohort of children with severe-to-profound SNHL. Furthermore, the medical records of CI recipients, including birth history, family history, and results of audiological tests and speech perception tests, are well documented. For this reason, we performed a retrospective review of the medical records of children who received CI surgery to assess the clinical significance of delayed-onset hearing loss among the children with severe-to-profound SNHL by comparing the outcome of CI between children with delayed-onset hearing loss and those with congenital hearing loss.
Subjects and methods A retrospective review was performed of the medical records of 233 children who underwent CI between May 2007 and December 2013 at Dong-A University Hospital.
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Among them, children who had documented results of newborn hearing screening were included in this study. The pass criterion for transient evoked otoacoustic emission was a response amplitude of 5 dB with a reproducibility of 50 % or greater, and the pass criterion for the automated auditory brainstem response was 35 dB nHL. The children were classified into two groups depending on the results of newborn hearing screening. The first group comprised children with congenital hearing loss who did not pass the newborn hearing screening and were confirmed to have hearing loss on diagnostic audiological tests. The second group comprised children with delayed-onset hearing loss. Children who passed the newborn hearing screening and those who were confirmed as having normal hearing on diagnostic audiological tests after failing to pass the newborn hearing screening were included in a delayed-onset hearing loss group. Birth history, risk factor for hearing loss, results of newborn hearing screening, or reason for referral for hearing tests were reviewed. The age at diagnosis of hearing loss, the age at the beginning of auditory habilitation, the age at CI, and the postimplant speech perception abilities were compared between the congenital hearing loss group and the group with delayed-onset hearing loss. The scores for categories of auditory performance (CAP) and an open-set monosyllabic word test at 3 years after CI were chosen for comparison. Children with congenital cytomegalovirus infection or severe cochlear malformation, which can interfere with speech perception, were excluded from the performance analysis. Statistical analyses were performed using SPSS for Windows version 18.0 (SPSS Inc, Chicago, IL), and p \ 0.05 was considered significant.
Results Epidemiologic characteristics of the congenital hearing loss group and the delayed-onset hearing loss group Seventy-three children (45 boys and 28 girls) who had documented results of newborn hearing screening were included in the analysis. Fifty children (68 %) had congenital hearing loss and 23 children (32 %) had delayedonset hearing loss (Fig. 1). Birth history was not remarkable in children of either group. There were no children with prematurity or low birth weight in either group. Two children in the congenital hearing loss group and one child in the delayed-onset hearing loss group had been treated in a neonatal intensive care unit. Two children in the congenital hearing loss group and ten children in the delayedonset hearing loss group had inner-ear malformations. The detailed epidemiological data for the two groups are presented in Tables 1 and 2.
Eur Arch Otorhinolaryngol
No. of patients
60 50
Moderate to severe / Progressive (n=10)
40 30 20
Profound (n=40)
Profound (n=16)
10 0
Moderate to severe / Progressive (n=7)
Congenital HL group (n=50, 68%)
Delayed onset HL group (n=23, 32%)
Fig. 1 Results of initial diagnostic audiological tests in the congenital hearing loss (congenital HL) group and the delayed-onset hearing loss (delayed-onset HL) group
Timing of diagnosis of hearing loss, beginning of auditory habilitation and CI of children in the congenital hearing loss group Children with congenital hearing loss (n = 50) were confirmed to have hearing loss at a mean age of 0.3 years (range 0.1–1.8 years) (Table 1). Forty children (80 %) were confirmed to have profound hearing loss and ten children (20 %) were diagnosed with moderate-to-severe hearing loss through initial diagnostic audiological tests. Children with profound hearing loss began auditory habilitation at a mean age of 0.4 years (range 0.1–0.9 years) and underwent CI at a mean age of 1.4 years (range 0.8–4.7 years). Children who were diagnosed with moderate-to-severe hearing loss through initial diagnostic audiological tests began auditory habilitation at a mean age of 1.0 years (range 0.1–2.4 years). They showed progressive deterioration of their hearing to a profound degree, so underwent CI at a mean age of 3.9 years (range 1.2–6.3 years). Timing of diagnosis of hearing loss, beginning of auditory habilitation, and CI of children in the delayed-onset hearing loss group
with profound hearing loss and seven (30 %) with moderate-to-severe hearing loss. Children with profound hearing loss began auditory habilitation using hearing aids after confirmation of their hearing impairment and underwent CI at a mean age of 2.6 years (range 1.2–4.8 years). Children who were diagnosed with moderate-to-severe hearing loss began auditory habilitation at a mean age of 2.1 years (range 1.1–4.1 years). Their hearing deteriorated progressively and they underwent CI at a mean age of 3.9 years (range 2.1–7.2 years) when their hearing worsened to a profound degree and no more improvements in auditory development were expected. The diagnosis of hearing loss, the beginning of auditory habilitation, and CI occurred later in children with delayed-onset hearing loss than in children with congenital hearing loss (Fig. 2). Thirteen (57 %) of 23 children with delayed-onset hearing loss had risk indicators that were proposed by the JCIH in their 2007 position statement to be associated with permanent congenital, delayed-onset, or progressive hearing loss. The risk indicators included a family history of hearing loss for four children, cytomegalovirus infection in one child, and inner-ear malformation in ten children. However, ten children (43 %) had no risk indicators for hearing loss (Table 2). Comparison of postimplant speech perception abilities between the congenital hearing loss group and the delayed-onset hearing loss group Children who had used cochlear implants for 3 years or more (11 in the congenital hearing loss group and seven in the delayed-onset hearing loss group) were included for comparison of their postimplant speech perception abilities. Children in the congenital hearing loss group achieved better mean scores on CAP and monosyllabic word tests than those in the delayed-onset hearing loss group, but the differences were not statistically significant (Mann–Whitney test, p [ 0.05) (Fig. 3).
Discussion Children with delayed-onset hearing loss (n = 23) were confirmed to have hearing loss at a mean age of 2.0 years (range 0.2–4.0 years) (Table 2). The children had passed the newborn hearing screening (n = 20) or had been confirmed to have normal hearing (n = 3) through diagnostic audiological tests performed during their early infancy. However, they were referred for audiological evaluation because their caregivers had concerns about hearing impairment in the children because of inconsistent responses to environmental sounds or delay in speech–language development (n = 22), or a physician recommended the hearing tests for a child who had perinatal cytomegalovirus infection (n = 1). Sixteen children (70 %) were diagnosed
The prevalence of delayed-onset hearing loss has been reported to be as high as that of congenital hearing loss. Lu¨ et al. [10] reported that 16 of 21,427 preschool children (0.75 persons per 1000) who had passed newborn hearing screening were confirmed to have permanent delayed-onset hearing loss. Weichbold et al. [11] showed that 23 (22 %) of 105 children with bilateral permanent hearing loss had delayed-onset hearing loss. Almost every newborn who is born with hearing loss can be identified and managed early after birth because of the newborn hearing screening program. Early management for newborn hearing loss can prevent negative
123
123
F
F
F
M
M
M
M
F
M
M
F
M M
M
M
F
F
F
F
M
F
M
M
M
7
8
9
10
11
12
13
14
15
16
17
18 19
20
21
22
23
24
25
26
27
28
29
30
F
M
6
33
M
5
F
F F
3 4
M
M
2
31
M
1
32
Sex
No.
39
35
39
37
36
38
34
40
38
38
38
39
39
40
41 40
40
38
40
39
39
38
37
40
38
41
41
39
39
38 40
40
40
GA (months)
3.2
2.6
3.1
3.2
2.7
3.3
2.5
3.5
5.2
3.0
2.6
2.7
3.1
3.8
3.5 2.9
2.6
2.9
3.9
3.0
2.7
3.3
3.1
3.0
3.0
3.1
3.8
3.2
4.0
3.2 2.8
3.4
3.6
Birth weight (kg)
Both ears refer
Both ears refer
Both ears refer
Both ears refer (AABR)
Both ears refer
Both ears refer
Both ears refer
Both ears refer (AABR)
Both ears refer
Both ears refer (AOAE)
Both ears refer
Both ears refer
Both ears refer
Both ears refer (AABR)
Both ears refer Both ears refer
Both ears refer
Both ears refer
Both ears refer
Both ears refer (AABR)
Both ears refer
Both ears refer
Both ears refer (AABR)
Both ears refer (AABR)
Both ears refer (AABR)
Both ears refer (AABR)
Both ears refer
Both ears refer (AABR)
Both ears refer (AABR)
Both ears refer (AOAE) Both ears refer (AABR)
Both ears refer (AABR)
Both ears refer (AABR)
Result of NHS
0.1
0.2
0.9
0.1
0.4
0.4
0.3
0.3
0.4
0.3
0.1
0.7
0.1
0.1
0.3 0.7
0.3
0.1
0.1
0.1
0.1
0.3
0.4
0.3
0.1
0.1
0.1
0.1
0.1
0.2 0.7
0.4
0.2
Age at diagnosis of HL (years)
Table 1 Epidemiological and audiological characteristics of 50 children with congenital hearing loss
NR/NR
NR/NR
NR/90
100/100
NR/90
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
100/NR
90/90
100/100
90/90 NR/NR
NR/90
100/100
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR NR/NR
NR/NR
NR/NR
Hearing thresholda at initial hearing test (Rt/Lt, dB nHL)
0.4
0.3
0.9
0.3
0.4
0.6
0.7
0.6
0.6
0.5
0.3
0.5
0.3
0.2
0.3 0.8
0.3
0.5
0.3
0.3
0.3
0.6
0.4
0.4
0.3
0.2
0.3
0.1
0.1
0.3 0.8
0.5
0.3
Age at beginning of abilitation (years)
2.1
2
1.6
1
1.1
1.3
1.1
1.4
1.8
0.9
0.9
0.9
3.1
1.1
1.1 1.4
1.3
0.9
0.9
0.9
1.1
1.6
0.9
0.9
1.3
2.5
0.9
1.1
0.8
0.9 1.8
2.1
1.4
Age at CI (years)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-) (-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-) (-)
(-)
(-)
Risk factor of hearing loss
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F
M
M
M
M M
M
F
F
M
35
36
37
38
39 40
41b
42b
43b
44b
M
50b
38
40
40
38
40
39
38
40
40
39
40 35
38
37
40
39
40
GA (months)
3.8
2.9
3.8
3.6
3.5
2.8
2.6
3.2
3.1
3.4
3.6 2.5
3.2
4.0
3.5
2.8
3.1
Birth weight (kg)
Both ears refer
Both ears refer
Both ears refer
Both ears refer
Both ears refer
Both ears refer
Right ears refer (AABR)
Both ears refer
Both ears refer (AABR)
Both ears refer
Both ears refer Both ears refer
Both ears refer
Both ears refer (AABR)
Both ears refer
Both ears refer
Both ears refer
Result of NHS
0.3
0.4
0.3
0.1
0.1
0.3
0.6
0.3
0.1
0.1
0.1 1.8
0.1
0.1
0.1
0.1
0.5
Age at diagnosis of HL (years)
80/80
50/50
55/60
NR/70
60/30
60/80
NR/60
80/90
80/80
80/80
NR/NR NR/NR
NR/100
NR/NR
NR/NR
90/NR
100/NR
Hearing thresholda at initial hearing test (Rt/Lt, dB nHL)
0.4
2.1
2.4
2.2
1
0.8
0.7
0.3
0.2
0.1
0.5 0.7
0.3
0.5
0.3
0.2
0.5
Age at beginning of abilitation (years)
1.2
2.8
6.3
2.5
5.6
5.3
3.3
3.6
5.8
3
4.7 2.1
1
1
1
2
1.1
Age at CI (years)
(-)
NICU care for tachypnea
IEM (IP II, EVA)
(-)
IEM (EVA)
Family history
(-)
(-)
(-)
(-)
(-) (-)
(-)
(-)
(-)
(-)
Exchange transfusion
Risk factor of hearing loss
b
a
Hearing deteriorated progressively to a profound degree on follow up hearing tests
Hearing thresholds were measured using auditory brainstem response
GA gestational age, NHS newborn hearing screening, AABR automated auditory brainstem response, AOAE automated otoacoustic emission, HL hearing loss, Rt right, Lt left, CI cochlear implantation, NR no response, IEM inner-ear malformation, IP incomplete partition, EVA enlarged vestibular aqueduct
M
49b
48
F
M
47b
b
M
46b
45
F
M
34
b
Sex
No.
Table 1 continued
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Sex
F
M
F
M
M
M
F
F
M F
M
M
M
F
F
M
F
M
F
No.
1
123
2
3
4
5
6
7
8
9 10
11
12
13
14
15
16c
17c
18c
19c
40
40
40
40
40
38
37
39
38
40 39
39
40
NA
38
39
40
34
38
GA (months)
2.9
3.3
3.7
3.5
3.0
3.0
2.6
2.6
3.0
3.1 3.0
3.1
3.9
NA
2.9
3.7
3.5
2.9
3.0
Birth weight (kg)
Both ears pass
Both ears pass
Right ear refer ABR Rt. 20 dB (1 month of age) Both ears refer (AABR) ABR Rt. 20 dB, Lt. 40 dB (1 months of age) Both ears pass
Both ears pass
Both ears pass
Both ears pass
Both ears pass
Both ears pass Both ears pass
Both ears pass
Both ears pass
Both ears pass
Both ears pass
Both ears pass (AOAE)
Both ears pass (AOAE)
Both ears pass
Both ears pass
Result of NHS
Poor response to environmental sound Poor response to environmental sound
Caregiver’s concern
Poor response to environmental sound Poor response to environmental sound Poor response to environmental sound Poor response to environmental sound Poor response to environmental sound Delay in speechlanguage development Poor response to environmental sound Delay in speechlanguage development Caregiver’s concern Delay in speechlanguage development Delay in speechlanguage development Poor response to environmental sound Delay in speechlanguage development Delay in speechlanguage development Poor response to environmental sound Delay in speechlanguage development
Reason for referral
0.9
1.3
1.3
2.0
3.8
3.3
3.0
0.8
3.3
1.1 1.4
2.2
2.0
2.1
1.9
0.2
1.4
0.8
2.4
Age at diagnosis of HL (years)
Table 2 Epidemiological and audiological characteristics of 23 children with delayed-onset hearing loss
70/90
90/70
75/75
80/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR NR/NR
NR/80
NR/NR
NR/NR
NR/NR
90/NR
NR/NR
NR/NR
90/100
Hearing thresholda (Rt/Lt, dB nHL)
1.1
1.4
1.3
2.0
4.0
3.0
2.9
0.8
3.4
1.2 1.5
2.3
2.1
2.2
2.0
0.3
1.5
0.8
2.5
Age at beginning of habilitation (years)
2.1
2.5
3.7
2.8 m
4.8
3.3
3.3
1.3
3.8
1.5 2.0
2.9
2.4
2.4
2.6
2.1
2.4
1.2
3.0
Age at CI (years)
IEM (IP II, EVA), family history
IEM (EVA), family history IEM (EVA)
(-)
IEM (IP II, EVA)
(-)
(-)
(-)
IEM (IP I, EVA)
Family history IEM (IP II, EVA)
(-)
(-)
(-)
(-)
IEM (IP II, EVA)
(-)
(-)
Family history
Risk factor
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CMV 2.1
Average threshold of pure tone audiometry
Hearing thresholds were measured using auditory brainstem response
Hearing deteriorated progressively to a profound degree on follow up hearing tests c
b
a
Left ear refer (AOAE) ABR Rt. 20 dB, Lt. 20 dB (1 months of age) 2.4 M 23c
36
Both ears pass NA M 22c
NA
Both ears pass 3.1 41 M 21c
ABR auditory brainstem response, NA not available, CMV cytomegalovirus; see Table 1 for other abbreviations
1.1 80/70 1.1
IEM (IP III) 5.6 3.1 80/60 2.8
IEM (CH) 4.3 2.7 70/60 2.6
IEM (EVA) 7.2 4.1 56/115b 4.0
Poor response to environmental sound Delay in speechlanguage development Delay in speechlanguage development Recommendation of physician Both ears pass 3.4 NA M 20c
GA (months) Sex No.
Table 2 continued
Birth weight (kg)
Result of NHS
Reason for referral
Age at diagnosis of HL (years)
Hearing thresholda (Rt/Lt, dB nHL)
Age at beginning of habilitation (years)
Age at CI (years)
Risk factor
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impacts of hearing loss on speech–language development and academic achievement. However, because newborn hearing screening has focused on early identification of congenital hearing loss, this program cannot identify infants with delayed-onset hearing loss. When a newborn passes a newborn hearing screening, referral for audiological evaluation is made only when there is caregiver concern about hearing or a delay in the child’s language development. However, it is not easy for the caregiver to detect early signs of hearing loss in very young children. In addition, a pass result of screening at birth may make the caregiver consider that their child has permanently normal hearing and ignore the early signs of delayed hearing loss. Therefore, detection and intervention of delayed-onset hearing loss can be easily delayed. According to Weichbold et al., age at hearing loss confirmation in children with delayed-onset hearing loss was from 0.9 to 63 months. Young et al. [12] demonstrated that 32 of 108 cochlearimplanted children had delayed-onset hearing loss and these children underwent CI at a mean age of 2.6 years, which was about 11 months later than children with congenital hearing loss. In our series, children with delayedonset hearing loss were confirmed to have hearing loss at a mean age of 2.0 years, whereas children who had congenital hearing loss were diagnosed with hearing loss at a mean age of 0.3 years. It is evident that children with unrecognized and unmanaged hearing loss have significant speech–language delays, negative educational consequences and behavioral problems, and the adverse effects of hearing loss increase with increasing delay in intervention. Delayed detection and intervention of delayed-onset hearing loss can impact adversely on long-term speech and language development. Furthermore, risk factors associated with development of delayed-onset hearing loss can influence negatively on the outcome of intervention using a hearing aid or cochlear implant. To our knowledge, there is no published report on outcome of intervention for children with delayed-onset hearing loss. This study compared the outcome of CI between children with delayed-onset hearing loss and those with congenital hearing loss. Although the differences were not significant, the delayed-onset hearing loss group showed slightly lower speech perception test scores 3 years after cochlear implantation than the congenital hearing loss group despite their older chronological age (mean age at test: 5.7 years in the delayed-onset hearing loss group, 4.2 years in the congenital hearing loss group), and demonstrated a wider range of test scores than the congenital hearing loss group. The small difference in speech perception abilities during early childhood may make a significant difference in educational and vocational achievements in the future. Therefore, children with delayed-onset hearing loss should be identified as early as possible after they lose their hearing. For this, the JCIH proposed 11 risk factors which are associated
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Eur Arch Otorhinolaryngol Fig. 2 Age at diagnosis of hearing loss (HL), beginning of auditory habilitation, and cochlear implantation (CI) of children who diagnosed to have profound hearing loss at initial diagnostic audiological evaluation. n = 40 and n = 16 correspond, respectively, to the congenital HL group and delayed-onset HL group. Statistical analyses were performed using an independent t test
Fig. 3 Comparison of auditory performance between congenital hearing loss group and delayed-onset hearing loss group at 3 years after cochlear implantation. Implanted children with congenital hearing loss achieved slightly higher scores in speech perception tests than those with delayed-onset hearing loss, but the differences were not statistically significant. Statistical analyses were performed using Mann– Whitney test. CAP categories of auditory performance, MWT open-set monosyllabic word test
with permanent congenital, delayed-onset or progressive hearing loss in childhood, and suggested that the following conditions are of greater concern for delayed-onset hearing loss: caregiver concern regarding hearing, speech, language, or developmental delay; a family history of permanent childhood hearing loss; extracorporeal membrane oxygenation or chemotherapy; in utero cytomegalovirus infection; neurodegenerative disorders; head trauma, especially basal skull or temporal bone fracture; syndromes associated with hearing loss; and culture-positive postnatal infections associated with sensorineural hearing loss [9]. The JCIH recommends that infants who pass the newborn hearing screening but have one of these risk factors should have additional diagnostic audiology assessment by 24–30 months of age to detect delayed-onset hearing loss [9]. However, previous studies and this study demonstrate that every child who passes newborn hearing screening
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needs to receive additional audiological assessment to detect delayed-onset hearing loss, regardless of the presence of risk factors, and that the audiological assessment is better performed earlier than the timing recommended by the JCIH. Studies have shown that many children with delayed-onset hearing loss do not have any risk factors raised by the JCIH. Lu¨ et al. [10] reported that ten children out of 16 with delayed-onset hearing loss had no risk factors for delayed-onset hearing loss. Weichbold et al. [11] showed that risk factors were present in half of the 23 children with delayed-onset hearing loss. In our series, 43 % (10/23) of children with delayed-onset hearing loss had no risk factors. Furthermore, any of several conditions, including asymptomatic cytomegalovirus infection, innerear malformations, or nonsyndromic genetic hearing loss, which can cause delayed-onset hearing loss, are nearly impossible to recognize without specific evaluation
Eur Arch Otorhinolaryngol
procedures. Therefore, risk factor-based screening for delayed-onset hearing loss can lose many children with hearing loss. Age at hearing loss confirmation in children with delayed-onset hearing loss was reported to be from 0.9 to 63 months [11] and children with delayed-onset hearing loss in this study were confirmed to have hearing loss at an age from 3 to 48 months. The JCIH recommended an additional audiological assessment by 24–30 months of age, but some children may lose their hearing much earlier than this time. Given that the correlation between the presence of risk factors and the development of delayed-onset hearing loss is weak, and that hearing loss can develop during early infancy, earlier and more frequent additional audiological assessment than the JCIH recommendation should be performed for all children who pass the newborn hearing screening to detect delayed-onset hearing loss. If universal screening for delayed-onset hearing loss is difficult to perform because of financial concerns, an awareness program for delayed-onset hearing loss needs to be instigated. Physicians should have thorough knowledge about delayed-onset hearing loss and parents of newborns should be informed about the possibility of development of delayed hearing loss. Children who have risk factors for delayed-onset hearing loss should receive periodic audiological assessment. Even though children have no risk factors for delayed-onset hearing loss, progress of speech and language skills should be monitored by parents, and if children do not show age-appropriate development in speech–language skills or if they show any symptom of hearing loss, audiological evaluation should be employed.
Conclusion Thirty-two percent of 73 children who underwent CI had delayed-onset hearing loss. The diagnosis of hearing loss, beginning of auditory habilitation, and CI surgery for children with delayed-onset hearing loss was far later than for those with congenital hearing loss. Risk factors for hearing loss were not found in 43 % of children with delayed-onset hearing loss. Universal screening for delayed hearing loss needs to be performed during early childhood
for early detection and intervention of delayed-onset hearing loss. Acknowledgments This study was supported by research funds from Dong-A University. Conflict of interest of interest.
All the authors certify that they have no conflict
References 1. Smith RJH, Bale JF, White KR (2005) Sensorineural hearing loss in children. Lancet 365:879–890 2. Martines F, Salvago P, Bentivegna D, Bartolone A, Dispenza F, Martines E (2012) Audiologic profile of infants at risk: experience of a Western Sicily tertiary care centre. Int J Pediatr Otorhinolaryngol 76:1285–1291 3. Watkin PM, Baldwin M, McEnery G (1991) Neonatal at risk screening and the identification of deafness. Arch Dis Child 66:1130–1135 4. Meyer C, Witte J, Hildmann A, Hennecke KH, Schunck KU, Maul K, Franke U, Fahnenstich H, Rabe H, Rossi R, Hartmann S, Gortner L (1999) Neonatal screening for hearing disorders in infants at risk: incidence, risk factors, and follow-up. Pediatrics 104:900–904 5. Watkin PM, Baldwin M (2011) Identifying deafness in early childhood: requirements after the newborn hearing screen. Arch Dis Child 96:62–66 6. Yoshinaga-Itano C, Sedey AL, Coulter DK, Mehl AL (1998) Language of early- and later-identified children with hearing loss. Pediatrics 102:1161–1171 7. Colletti L, Mandala` M, Colletti V (2012) Cochlear implants in children younger than 6 months. Otolaryngol Head Neck Surg 147:139–146 8. Northern J, Downs M (2002) Hearing in children, 5th edn. Lippincott, Philadelphia 9. American Academy of Pediatrics, Joint Committee on Infant Hearing (2007) Year 2007 position statement: principles and guidelines for early hearing detection and intervention programs. Pediatrics 2007(120):898–921 10. Lu¨ J, Huang Z, Yang T, Li Y, Mei L, Xiang M et al (2011) Screening for delayed-onset hearing loss in preschool children who previously passed the newborn hearing screening. Int J Pediatr Otorhinolaryngol 75:1045–1049 11. Weichbold V, Nekahm-Heis D, Welzl-Mueller K (2006) Universal newborn hearing screening and postnatal hearing loss. Pediatrics 117:631–636 12. Young NM, Reilly BK, Burke L (2011) Limitations of universal newborn hearing screening in early identification of pediatric cochlear implant candidates. Arch Otolaryngol Head Neck Surg 137:230–234
123
OTOLOGY
Delayed-onset hearing loss in pediatric candidates for cochlear implantation Sung-Wook Jeong1 • Min-Young Kang1 • Jae-Ryong Kim2 • Lee-Suk Kim1
Received: 1 March 2015 / Accepted: 1 May 2015 Ó Springer-Verlag Berlin Heidelberg 2015
Abstract The objective of this study was to evaluate the clinical significance of delayed-onset hearing loss in children. Seventy-three children who underwent cochlear implantation (CI) were included. They were divided into a congenital hearing loss group (n = 50) and a delayed-onset hearing loss group (n = 23). The age at diagnosis of hearing loss, age at the beginning of auditory habilitation, the age at CI, and the postimplant speech perception abilities were compared between the two groups. Children in the congenital hearing loss group were confirmed to have hearing loss at a mean age of 0.3 years, and those in the delayed-onset hearing loss group were diagnosed with hearing loss at a mean age of 2.0 years. Auditory habilitation began at a mean age of 0.4 and 2.0 years, and CI was performed at a mean age of 1.4 and 2.6 years, respectively. Children in the congenital hearing loss group had better scores on speech perception tests than those in the delayed-onset hearing loss group, but the differences were not significant. About half of the children with delayedonset hearing loss (57 %) had risk factors associated with delayed-onset hearing loss. A high prevalence of delayedonset hearing loss was noted in the group of children who underwent CI. Risk factors for hearing loss were not found in 43 % of children with delayed-onset hearing loss. Universal screening for delayed-onset hearing loss needs to be performed during early childhood.
& Lee-Suk Kim [email protected] 1
Department of Otolaryngology-Head and Neck Surgery, College of Medicine, Dong-A University, 3-1 Dongdaeshindong, Seo-gu, Busan 602-715, Korea
2
Department of Otolaryngology-Head and Neck Surgery, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea
Keywords Delayed-onset hearing loss Newborn hearing screening Sensorineural hearing loss Cochlear implants
Introduction Of every 1000 children born, 1–3 have congenital sensorineural hearing loss (SNHL) [1]. In infants who have risk factors for hearing loss or who are treated in an intensive care unit, the risk of congenital hearing loss can be increased 10- to 50-fold [2–4]. Fortunately, children with congenital hearing impairment can achieve useful auditory verbal abilities if their hearing loss is detected early during the newborn period and an intervention using a hearing aid or cochlear implant is performed soon after detection of hearing loss. Because of the high incidence of congenital hearing loss and the significance of early detection and intervention for congenital hearing loss, most countries run universal newborn hearing screening programs. However, newborns who have mild hearing loss or hearing loss at specific frequencies (e.g. low-frequency hearing loss) may not be detected by newborn hearing screening even though they have congenital permanent hearing loss. Furthermore, some children who have normal hearing or who pass the newborn hearing screening because of mild hearing loss at birth can lose their hearing in early childhood [5]. Because these children pass the newborn hearing screening, early detection of their hearing loss is difficult soon after onset without special attention and intervention. Auditory input during early childhood is crucial for the development of normal auditory verbal abilities [6, 7], so special attention or screening for delayed-onset hearing loss is required. About three of 1000 children lose their hearing during infancy or early childhood [8]. The Joint Committee on Infant Hearing (JCIH) identified 11 risk factors for permanent
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congenital, delayed-onset, and/or progressive hearing loss, and recommended diagnostic audiological evaluation at the age of 24–30 months for children who pass the newborn hearing screening, but have these risk factors to detect delayed-onset hearing loss [9]. Several studies concerned with the epidemiology of delayed-onset hearing loss have been published [3, 4, 10–12], but reports on the epidemiology of delayed-onset hearing loss in Asian people are lacking. Therefore, we performed this study to examine the epidemiology of delayed-onset hearing loss in Korean children and evaluate the significance of delayed hearing loss. About 480,000 children are born in South Korea each year. Given that one in every 1000 newborns is born with severe-to-profound SNHL, about 480 children are thought to be born with severe-to-profound SNHL each year in South Korea. Cochlear implantation (CI) surgery has been performed for about 300–400 children with hearing loss every year during the last 10 years in Korea. Therefore, most children born with severe-to-profound SNHL can be assumed to receive CI surgery. One of the reasons for the high rate of CI surgery in Korea is the availability of a National Health Insurance system, which is run by the Ministry of Health. Funding for the insurance is made by compulsory contributions from all residents, employers, and the government. All Korean residents pay about 2.95 % of their income as their insurance fee and their insurance covers 80 % of the cost of inpatient hospital care. Furthermore, several public corporations aid the poorest people who have financial difficulty in paying the 20 % gap in cost for inpatient hospital care. Therefore, most children with severeto-profound SNHL can receive CI surgery without financial difficulty in Korea. Because the annual number of pediatric CI surgeries in the young population under the age of 3 years is an approximation of the annual number of newborns who are born with severe-to-profound SNHL, a cohort of pediatric CI recipients is similar to the cohort of children with severe-to-profound SNHL. Furthermore, the medical records of CI recipients, including birth history, family history, and results of audiological tests and speech perception tests, are well documented. For this reason, we performed a retrospective review of the medical records of children who received CI surgery to assess the clinical significance of delayed-onset hearing loss among the children with severe-to-profound SNHL by comparing the outcome of CI between children with delayed-onset hearing loss and those with congenital hearing loss.
Subjects and methods A retrospective review was performed of the medical records of 233 children who underwent CI between May 2007 and December 2013 at Dong-A University Hospital.
123
Among them, children who had documented results of newborn hearing screening were included in this study. The pass criterion for transient evoked otoacoustic emission was a response amplitude of 5 dB with a reproducibility of 50 % or greater, and the pass criterion for the automated auditory brainstem response was 35 dB nHL. The children were classified into two groups depending on the results of newborn hearing screening. The first group comprised children with congenital hearing loss who did not pass the newborn hearing screening and were confirmed to have hearing loss on diagnostic audiological tests. The second group comprised children with delayed-onset hearing loss. Children who passed the newborn hearing screening and those who were confirmed as having normal hearing on diagnostic audiological tests after failing to pass the newborn hearing screening were included in a delayed-onset hearing loss group. Birth history, risk factor for hearing loss, results of newborn hearing screening, or reason for referral for hearing tests were reviewed. The age at diagnosis of hearing loss, the age at the beginning of auditory habilitation, the age at CI, and the postimplant speech perception abilities were compared between the congenital hearing loss group and the group with delayed-onset hearing loss. The scores for categories of auditory performance (CAP) and an open-set monosyllabic word test at 3 years after CI were chosen for comparison. Children with congenital cytomegalovirus infection or severe cochlear malformation, which can interfere with speech perception, were excluded from the performance analysis. Statistical analyses were performed using SPSS for Windows version 18.0 (SPSS Inc, Chicago, IL), and p \ 0.05 was considered significant.
Results Epidemiologic characteristics of the congenital hearing loss group and the delayed-onset hearing loss group Seventy-three children (45 boys and 28 girls) who had documented results of newborn hearing screening were included in the analysis. Fifty children (68 %) had congenital hearing loss and 23 children (32 %) had delayedonset hearing loss (Fig. 1). Birth history was not remarkable in children of either group. There were no children with prematurity or low birth weight in either group. Two children in the congenital hearing loss group and one child in the delayed-onset hearing loss group had been treated in a neonatal intensive care unit. Two children in the congenital hearing loss group and ten children in the delayedonset hearing loss group had inner-ear malformations. The detailed epidemiological data for the two groups are presented in Tables 1 and 2.
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No. of patients
60 50
Moderate to severe / Progressive (n=10)
40 30 20
Profound (n=40)
Profound (n=16)
10 0
Moderate to severe / Progressive (n=7)
Congenital HL group (n=50, 68%)
Delayed onset HL group (n=23, 32%)
Fig. 1 Results of initial diagnostic audiological tests in the congenital hearing loss (congenital HL) group and the delayed-onset hearing loss (delayed-onset HL) group
Timing of diagnosis of hearing loss, beginning of auditory habilitation and CI of children in the congenital hearing loss group Children with congenital hearing loss (n = 50) were confirmed to have hearing loss at a mean age of 0.3 years (range 0.1–1.8 years) (Table 1). Forty children (80 %) were confirmed to have profound hearing loss and ten children (20 %) were diagnosed with moderate-to-severe hearing loss through initial diagnostic audiological tests. Children with profound hearing loss began auditory habilitation at a mean age of 0.4 years (range 0.1–0.9 years) and underwent CI at a mean age of 1.4 years (range 0.8–4.7 years). Children who were diagnosed with moderate-to-severe hearing loss through initial diagnostic audiological tests began auditory habilitation at a mean age of 1.0 years (range 0.1–2.4 years). They showed progressive deterioration of their hearing to a profound degree, so underwent CI at a mean age of 3.9 years (range 1.2–6.3 years). Timing of diagnosis of hearing loss, beginning of auditory habilitation, and CI of children in the delayed-onset hearing loss group
with profound hearing loss and seven (30 %) with moderate-to-severe hearing loss. Children with profound hearing loss began auditory habilitation using hearing aids after confirmation of their hearing impairment and underwent CI at a mean age of 2.6 years (range 1.2–4.8 years). Children who were diagnosed with moderate-to-severe hearing loss began auditory habilitation at a mean age of 2.1 years (range 1.1–4.1 years). Their hearing deteriorated progressively and they underwent CI at a mean age of 3.9 years (range 2.1–7.2 years) when their hearing worsened to a profound degree and no more improvements in auditory development were expected. The diagnosis of hearing loss, the beginning of auditory habilitation, and CI occurred later in children with delayed-onset hearing loss than in children with congenital hearing loss (Fig. 2). Thirteen (57 %) of 23 children with delayed-onset hearing loss had risk indicators that were proposed by the JCIH in their 2007 position statement to be associated with permanent congenital, delayed-onset, or progressive hearing loss. The risk indicators included a family history of hearing loss for four children, cytomegalovirus infection in one child, and inner-ear malformation in ten children. However, ten children (43 %) had no risk indicators for hearing loss (Table 2). Comparison of postimplant speech perception abilities between the congenital hearing loss group and the delayed-onset hearing loss group Children who had used cochlear implants for 3 years or more (11 in the congenital hearing loss group and seven in the delayed-onset hearing loss group) were included for comparison of their postimplant speech perception abilities. Children in the congenital hearing loss group achieved better mean scores on CAP and monosyllabic word tests than those in the delayed-onset hearing loss group, but the differences were not statistically significant (Mann–Whitney test, p [ 0.05) (Fig. 3).
Discussion Children with delayed-onset hearing loss (n = 23) were confirmed to have hearing loss at a mean age of 2.0 years (range 0.2–4.0 years) (Table 2). The children had passed the newborn hearing screening (n = 20) or had been confirmed to have normal hearing (n = 3) through diagnostic audiological tests performed during their early infancy. However, they were referred for audiological evaluation because their caregivers had concerns about hearing impairment in the children because of inconsistent responses to environmental sounds or delay in speech–language development (n = 22), or a physician recommended the hearing tests for a child who had perinatal cytomegalovirus infection (n = 1). Sixteen children (70 %) were diagnosed
The prevalence of delayed-onset hearing loss has been reported to be as high as that of congenital hearing loss. Lu¨ et al. [10] reported that 16 of 21,427 preschool children (0.75 persons per 1000) who had passed newborn hearing screening were confirmed to have permanent delayed-onset hearing loss. Weichbold et al. [11] showed that 23 (22 %) of 105 children with bilateral permanent hearing loss had delayed-onset hearing loss. Almost every newborn who is born with hearing loss can be identified and managed early after birth because of the newborn hearing screening program. Early management for newborn hearing loss can prevent negative
123
123
F
F
F
M
M
M
M
F
M
M
F
M M
M
M
F
F
F
F
M
F
M
M
M
7
8
9
10
11
12
13
14
15
16
17
18 19
20
21
22
23
24
25
26
27
28
29
30
F
M
6
33
M
5
F
F F
3 4
M
M
2
31
M
1
32
Sex
No.
39
35
39
37
36
38
34
40
38
38
38
39
39
40
41 40
40
38
40
39
39
38
37
40
38
41
41
39
39
38 40
40
40
GA (months)
3.2
2.6
3.1
3.2
2.7
3.3
2.5
3.5
5.2
3.0
2.6
2.7
3.1
3.8
3.5 2.9
2.6
2.9
3.9
3.0
2.7
3.3
3.1
3.0
3.0
3.1
3.8
3.2
4.0
3.2 2.8
3.4
3.6
Birth weight (kg)
Both ears refer
Both ears refer
Both ears refer
Both ears refer (AABR)
Both ears refer
Both ears refer
Both ears refer
Both ears refer (AABR)
Both ears refer
Both ears refer (AOAE)
Both ears refer
Both ears refer
Both ears refer
Both ears refer (AABR)
Both ears refer Both ears refer
Both ears refer
Both ears refer
Both ears refer
Both ears refer (AABR)
Both ears refer
Both ears refer
Both ears refer (AABR)
Both ears refer (AABR)
Both ears refer (AABR)
Both ears refer (AABR)
Both ears refer
Both ears refer (AABR)
Both ears refer (AABR)
Both ears refer (AOAE) Both ears refer (AABR)
Both ears refer (AABR)
Both ears refer (AABR)
Result of NHS
0.1
0.2
0.9
0.1
0.4
0.4
0.3
0.3
0.4
0.3
0.1
0.7
0.1
0.1
0.3 0.7
0.3
0.1
0.1
0.1
0.1
0.3
0.4
0.3
0.1
0.1
0.1
0.1
0.1
0.2 0.7
0.4
0.2
Age at diagnosis of HL (years)
Table 1 Epidemiological and audiological characteristics of 50 children with congenital hearing loss
NR/NR
NR/NR
NR/90
100/100
NR/90
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
100/NR
90/90
100/100
90/90 NR/NR
NR/90
100/100
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR NR/NR
NR/NR
NR/NR
Hearing thresholda at initial hearing test (Rt/Lt, dB nHL)
0.4
0.3
0.9
0.3
0.4
0.6
0.7
0.6
0.6
0.5
0.3
0.5
0.3
0.2
0.3 0.8
0.3
0.5
0.3
0.3
0.3
0.6
0.4
0.4
0.3
0.2
0.3
0.1
0.1
0.3 0.8
0.5
0.3
Age at beginning of abilitation (years)
2.1
2
1.6
1
1.1
1.3
1.1
1.4
1.8
0.9
0.9
0.9
3.1
1.1
1.1 1.4
1.3
0.9
0.9
0.9
1.1
1.6
0.9
0.9
1.3
2.5
0.9
1.1
0.8
0.9 1.8
2.1
1.4
Age at CI (years)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-) (-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-)
(-) (-)
(-)
(-)
Risk factor of hearing loss
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F
M
M
M
M M
M
F
F
M
35
36
37
38
39 40
41b
42b
43b
44b
M
50b
38
40
40
38
40
39
38
40
40
39
40 35
38
37
40
39
40
GA (months)
3.8
2.9
3.8
3.6
3.5
2.8
2.6
3.2
3.1
3.4
3.6 2.5
3.2
4.0
3.5
2.8
3.1
Birth weight (kg)
Both ears refer
Both ears refer
Both ears refer
Both ears refer
Both ears refer
Both ears refer
Right ears refer (AABR)
Both ears refer
Both ears refer (AABR)
Both ears refer
Both ears refer Both ears refer
Both ears refer
Both ears refer (AABR)
Both ears refer
Both ears refer
Both ears refer
Result of NHS
0.3
0.4
0.3
0.1
0.1
0.3
0.6
0.3
0.1
0.1
0.1 1.8
0.1
0.1
0.1
0.1
0.5
Age at diagnosis of HL (years)
80/80
50/50
55/60
NR/70
60/30
60/80
NR/60
80/90
80/80
80/80
NR/NR NR/NR
NR/100
NR/NR
NR/NR
90/NR
100/NR
Hearing thresholda at initial hearing test (Rt/Lt, dB nHL)
0.4
2.1
2.4
2.2
1
0.8
0.7
0.3
0.2
0.1
0.5 0.7
0.3
0.5
0.3
0.2
0.5
Age at beginning of abilitation (years)
1.2
2.8
6.3
2.5
5.6
5.3
3.3
3.6
5.8
3
4.7 2.1
1
1
1
2
1.1
Age at CI (years)
(-)
NICU care for tachypnea
IEM (IP II, EVA)
(-)
IEM (EVA)
Family history
(-)
(-)
(-)
(-)
(-) (-)
(-)
(-)
(-)
(-)
Exchange transfusion
Risk factor of hearing loss
b
a
Hearing deteriorated progressively to a profound degree on follow up hearing tests
Hearing thresholds were measured using auditory brainstem response
GA gestational age, NHS newborn hearing screening, AABR automated auditory brainstem response, AOAE automated otoacoustic emission, HL hearing loss, Rt right, Lt left, CI cochlear implantation, NR no response, IEM inner-ear malformation, IP incomplete partition, EVA enlarged vestibular aqueduct
M
49b
48
F
M
47b
b
M
46b
45
F
M
34
b
Sex
No.
Table 1 continued
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123
Sex
F
M
F
M
M
M
F
F
M F
M
M
M
F
F
M
F
M
F
No.
1
123
2
3
4
5
6
7
8
9 10
11
12
13
14
15
16c
17c
18c
19c
40
40
40
40
40
38
37
39
38
40 39
39
40
NA
38
39
40
34
38
GA (months)
2.9
3.3
3.7
3.5
3.0
3.0
2.6
2.6
3.0
3.1 3.0
3.1
3.9
NA
2.9
3.7
3.5
2.9
3.0
Birth weight (kg)
Both ears pass
Both ears pass
Right ear refer ABR Rt. 20 dB (1 month of age) Both ears refer (AABR) ABR Rt. 20 dB, Lt. 40 dB (1 months of age) Both ears pass
Both ears pass
Both ears pass
Both ears pass
Both ears pass
Both ears pass Both ears pass
Both ears pass
Both ears pass
Both ears pass
Both ears pass
Both ears pass (AOAE)
Both ears pass (AOAE)
Both ears pass
Both ears pass
Result of NHS
Poor response to environmental sound Poor response to environmental sound
Caregiver’s concern
Poor response to environmental sound Poor response to environmental sound Poor response to environmental sound Poor response to environmental sound Poor response to environmental sound Delay in speechlanguage development Poor response to environmental sound Delay in speechlanguage development Caregiver’s concern Delay in speechlanguage development Delay in speechlanguage development Poor response to environmental sound Delay in speechlanguage development Delay in speechlanguage development Poor response to environmental sound Delay in speechlanguage development
Reason for referral
0.9
1.3
1.3
2.0
3.8
3.3
3.0
0.8
3.3
1.1 1.4
2.2
2.0
2.1
1.9
0.2
1.4
0.8
2.4
Age at diagnosis of HL (years)
Table 2 Epidemiological and audiological characteristics of 23 children with delayed-onset hearing loss
70/90
90/70
75/75
80/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR
NR/NR NR/NR
NR/80
NR/NR
NR/NR
NR/NR
90/NR
NR/NR
NR/NR
90/100
Hearing thresholda (Rt/Lt, dB nHL)
1.1
1.4
1.3
2.0
4.0
3.0
2.9
0.8
3.4
1.2 1.5
2.3
2.1
2.2
2.0
0.3
1.5
0.8
2.5
Age at beginning of habilitation (years)
2.1
2.5
3.7
2.8 m
4.8
3.3
3.3
1.3
3.8
1.5 2.0
2.9
2.4
2.4
2.6
2.1
2.4
1.2
3.0
Age at CI (years)
IEM (IP II, EVA), family history
IEM (EVA), family history IEM (EVA)
(-)
IEM (IP II, EVA)
(-)
(-)
(-)
IEM (IP I, EVA)
Family history IEM (IP II, EVA)
(-)
(-)
(-)
(-)
IEM (IP II, EVA)
(-)
(-)
Family history
Risk factor
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CMV 2.1
Average threshold of pure tone audiometry
Hearing thresholds were measured using auditory brainstem response
Hearing deteriorated progressively to a profound degree on follow up hearing tests c
b
a
Left ear refer (AOAE) ABR Rt. 20 dB, Lt. 20 dB (1 months of age) 2.4 M 23c
36
Both ears pass NA M 22c
NA
Both ears pass 3.1 41 M 21c
ABR auditory brainstem response, NA not available, CMV cytomegalovirus; see Table 1 for other abbreviations
1.1 80/70 1.1
IEM (IP III) 5.6 3.1 80/60 2.8
IEM (CH) 4.3 2.7 70/60 2.6
IEM (EVA) 7.2 4.1 56/115b 4.0
Poor response to environmental sound Delay in speechlanguage development Delay in speechlanguage development Recommendation of physician Both ears pass 3.4 NA M 20c
GA (months) Sex No.
Table 2 continued
Birth weight (kg)
Result of NHS
Reason for referral
Age at diagnosis of HL (years)
Hearing thresholda (Rt/Lt, dB nHL)
Age at beginning of habilitation (years)
Age at CI (years)
Risk factor
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impacts of hearing loss on speech–language development and academic achievement. However, because newborn hearing screening has focused on early identification of congenital hearing loss, this program cannot identify infants with delayed-onset hearing loss. When a newborn passes a newborn hearing screening, referral for audiological evaluation is made only when there is caregiver concern about hearing or a delay in the child’s language development. However, it is not easy for the caregiver to detect early signs of hearing loss in very young children. In addition, a pass result of screening at birth may make the caregiver consider that their child has permanently normal hearing and ignore the early signs of delayed hearing loss. Therefore, detection and intervention of delayed-onset hearing loss can be easily delayed. According to Weichbold et al., age at hearing loss confirmation in children with delayed-onset hearing loss was from 0.9 to 63 months. Young et al. [12] demonstrated that 32 of 108 cochlearimplanted children had delayed-onset hearing loss and these children underwent CI at a mean age of 2.6 years, which was about 11 months later than children with congenital hearing loss. In our series, children with delayedonset hearing loss were confirmed to have hearing loss at a mean age of 2.0 years, whereas children who had congenital hearing loss were diagnosed with hearing loss at a mean age of 0.3 years. It is evident that children with unrecognized and unmanaged hearing loss have significant speech–language delays, negative educational consequences and behavioral problems, and the adverse effects of hearing loss increase with increasing delay in intervention. Delayed detection and intervention of delayed-onset hearing loss can impact adversely on long-term speech and language development. Furthermore, risk factors associated with development of delayed-onset hearing loss can influence negatively on the outcome of intervention using a hearing aid or cochlear implant. To our knowledge, there is no published report on outcome of intervention for children with delayed-onset hearing loss. This study compared the outcome of CI between children with delayed-onset hearing loss and those with congenital hearing loss. Although the differences were not significant, the delayed-onset hearing loss group showed slightly lower speech perception test scores 3 years after cochlear implantation than the congenital hearing loss group despite their older chronological age (mean age at test: 5.7 years in the delayed-onset hearing loss group, 4.2 years in the congenital hearing loss group), and demonstrated a wider range of test scores than the congenital hearing loss group. The small difference in speech perception abilities during early childhood may make a significant difference in educational and vocational achievements in the future. Therefore, children with delayed-onset hearing loss should be identified as early as possible after they lose their hearing. For this, the JCIH proposed 11 risk factors which are associated
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Eur Arch Otorhinolaryngol Fig. 2 Age at diagnosis of hearing loss (HL), beginning of auditory habilitation, and cochlear implantation (CI) of children who diagnosed to have profound hearing loss at initial diagnostic audiological evaluation. n = 40 and n = 16 correspond, respectively, to the congenital HL group and delayed-onset HL group. Statistical analyses were performed using an independent t test
Fig. 3 Comparison of auditory performance between congenital hearing loss group and delayed-onset hearing loss group at 3 years after cochlear implantation. Implanted children with congenital hearing loss achieved slightly higher scores in speech perception tests than those with delayed-onset hearing loss, but the differences were not statistically significant. Statistical analyses were performed using Mann– Whitney test. CAP categories of auditory performance, MWT open-set monosyllabic word test
with permanent congenital, delayed-onset or progressive hearing loss in childhood, and suggested that the following conditions are of greater concern for delayed-onset hearing loss: caregiver concern regarding hearing, speech, language, or developmental delay; a family history of permanent childhood hearing loss; extracorporeal membrane oxygenation or chemotherapy; in utero cytomegalovirus infection; neurodegenerative disorders; head trauma, especially basal skull or temporal bone fracture; syndromes associated with hearing loss; and culture-positive postnatal infections associated with sensorineural hearing loss [9]. The JCIH recommends that infants who pass the newborn hearing screening but have one of these risk factors should have additional diagnostic audiology assessment by 24–30 months of age to detect delayed-onset hearing loss [9]. However, previous studies and this study demonstrate that every child who passes newborn hearing screening
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needs to receive additional audiological assessment to detect delayed-onset hearing loss, regardless of the presence of risk factors, and that the audiological assessment is better performed earlier than the timing recommended by the JCIH. Studies have shown that many children with delayed-onset hearing loss do not have any risk factors raised by the JCIH. Lu¨ et al. [10] reported that ten children out of 16 with delayed-onset hearing loss had no risk factors for delayed-onset hearing loss. Weichbold et al. [11] showed that risk factors were present in half of the 23 children with delayed-onset hearing loss. In our series, 43 % (10/23) of children with delayed-onset hearing loss had no risk factors. Furthermore, any of several conditions, including asymptomatic cytomegalovirus infection, innerear malformations, or nonsyndromic genetic hearing loss, which can cause delayed-onset hearing loss, are nearly impossible to recognize without specific evaluation
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procedures. Therefore, risk factor-based screening for delayed-onset hearing loss can lose many children with hearing loss. Age at hearing loss confirmation in children with delayed-onset hearing loss was reported to be from 0.9 to 63 months [11] and children with delayed-onset hearing loss in this study were confirmed to have hearing loss at an age from 3 to 48 months. The JCIH recommended an additional audiological assessment by 24–30 months of age, but some children may lose their hearing much earlier than this time. Given that the correlation between the presence of risk factors and the development of delayed-onset hearing loss is weak, and that hearing loss can develop during early infancy, earlier and more frequent additional audiological assessment than the JCIH recommendation should be performed for all children who pass the newborn hearing screening to detect delayed-onset hearing loss. If universal screening for delayed-onset hearing loss is difficult to perform because of financial concerns, an awareness program for delayed-onset hearing loss needs to be instigated. Physicians should have thorough knowledge about delayed-onset hearing loss and parents of newborns should be informed about the possibility of development of delayed hearing loss. Children who have risk factors for delayed-onset hearing loss should receive periodic audiological assessment. Even though children have no risk factors for delayed-onset hearing loss, progress of speech and language skills should be monitored by parents, and if children do not show age-appropriate development in speech–language skills or if they show any symptom of hearing loss, audiological evaluation should be employed.
Conclusion Thirty-two percent of 73 children who underwent CI had delayed-onset hearing loss. The diagnosis of hearing loss, beginning of auditory habilitation, and CI surgery for children with delayed-onset hearing loss was far later than for those with congenital hearing loss. Risk factors for hearing loss were not found in 43 % of children with delayed-onset hearing loss. Universal screening for delayed hearing loss needs to be performed during early childhood
for early detection and intervention of delayed-onset hearing loss. Acknowledgments This study was supported by research funds from Dong-A University. Conflict of interest of interest.
All the authors certify that they have no conflict
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