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Otherwise, even if you say something, the person may think that you have never
contacted them because they cannot hear you, and they may be hurt.
People may need emotional support whether they have a problem/disability or
not. When it comes to the emotional needs of people with a disability, this sensitivity becomes even more important. The more families, the environment, and peers
communicate in a friendly way with a hearing-impaired individual, the better the
person will feel and achieve accordingly.
D. T. Eroğlu and C. C. Cingi
5.3.2 Positive Parenting
When families are informed that their child is deaf, they might stop interacting or
reduce their communication with the child during the psychological stages they go
through and this may negatively affect the child’s communication with the community and his social and emotional development is negatively affected, too. However,
if the child is not in communication, and a part of the social world, s/he will not be
in harmony with his/her close environment, which will result in problems in terms
of self-esteem and communication abilities. If the child is left alone in early childhood and s/he is withdrawn from the environment, the child will have difculty in
communicating in all social settings and it is inevitable to experience adaptation
problems.
Families should be quick about acknowledging their child’s problem. Make them
feel valued in every situation. Thus, the child will think that hearing loss has nothing
to do with being less valuable and will not have to worry about being accepted by
society [2].
When it comes to family, not only parents but also other children come to mind.
All family members should be open and accepting regarding this issue. It is natural
for the other children to ask questions about the topic and talking to them openly is
crucial [2].
The hearing-impaired child should feel included in all activities and communication environments. Exclusionary or impatient responses are not welcome [2].
It is the families’ responsibility to help such kids to eliminate the feeling of loneliness and nurture the feeling of belongingness and get the support of others to make
their kids become a part of society.
It is very important to discuss the Individual Education Plan (IEP) with the
school administration before your child starts school [2].
It is important to talk to the teachers about how the child’s social and academic
development is going on and what can be done more if there is any gap [2].
In a le belonging to the child, all things done, reports, the manual of the devices
used, the warranty certicate, developmental features, and other related things
should be recorded [2]. The parents will ensure nothing is neglected in this way.
Being knowledgeable about the rights of the disabled and the laws will both
enable the child to act knowing their rights and enable them to be advocated appropriately where necessary [2].

5 Communication intheFamily ofaHearing-Impaired Child
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Communication is not only communicating with others. It also covers inner person communication. When an individual is happy with himself, he is happy
with others.
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5.3.3 Support andSelf-confidence
Peers and teachers of hearing-impaired children are as important as their families.
Hence, families should use adequate gathering opportunities with those to teach
how to communicate effectively being aware of the social rules, speech rules, and
different situations.
It is of great importance in learning how to react appropriately and act properly
in society for those groups, too. By establishing close relationships with those people, impaired children will be integrated into society and that will result in condence and happiness.
Hearing-impaired children growing up in a restricted environment due to their
disability are unable to communicate well as a result of lack of communication.
That will result in low self-esteem and personality problems. Support of others is
the most crucial thing in the development of communication skills and personality
order for those children, so public awareness and consciousness should be given
urgent consideration.
In order to have healthy communication with the child, it is recommended that
parents cooperate with other parents [3]. No single method is perfect. It is wise to
learn about other people’s methods as well [3].
5.4 Impacts onFamilies
Having a disabled child inevitably affects the family sadly [4, 5]. In a family with
hearing impairment, the way of life is affected [6]. Often a deaf child leads to a
change in relationships among family members [7]. Family members often behave
differently than usual to include the disabled child in communication. Usually, the
mother of a deaf child tries to set healthy communication among the family members [8]. All these attempts sometimes have adverse effects that affect the psychological health of the family members and can sometimes cause problems in marriage
[9]. Parenting skills can create a big difference in well-being and communication
skills of parents and children. So the aim of Positive Parenting Program (Triple-P)
is rendering parents–child interaction improvement [10]. Being deaf creates the illusion that people in the world are divided into hearing and deaf people [11]. Hearing
impairment is associated with behavioral change and communication change [12].
Family members of the hearing-impaired individual are thought to be unhappy and
in difculty [13]. It is associated with negative things such as low self-condence,
behavioral problem, social life deprivation, and psychological disorder. [14–16].
Hearing-impaired individuals may prevent communication within the family [17],

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or due to the adverse effects on the mother; she may cause poor communication
within the family [9, 18].
D. T. Eroğlu and C. C. Cingi
5.5 Positive Parenting Program
Considering the impacts of having a disabled child, the issue seems so devastating.
Nevertheless, with some awareness and education parents can take it more softly.
Parenting is the most effective factor in the development of the child, in the formation of his personality, and in gaining the mental, physical, emotional, and social
skills that he will need in his life [6]. Triple-P is based on social learning to support
the development of the child in the best way, to help him gain the skills he needs in
the best way, and to guide him in the formation of his personality [19]. The program
is educating parents by the principles designed by Sanders which are based on
Bandura’s theory [10, 20]. Triple-P, which was created in Australia, covers develop-
mental, cognitive, and behavioral elements [21]. It can be used to help with the
emotional and behavioral problems of the child as well as the mother, and the child’s
communication and interaction via creating awareness about the different needs of
both parties and the relationship between them [22]. The program contributes to
family relations and their social needs [20]. The program is mostly recommended
for children aged 0–16 [23, 24] to improve their social skills including developmental, cognitive, and behavioral management [10]. Triple-P was designed aiming to
improve self-esteem, knowledge, and self-efcacy and to nurture parent–child relations [18, 22, 25].
5.6 Conclusion
If we want to talk about the positive effect on children with hearing problems in
terms of communication skills, social relations, education, and so on, they must be
given full support by their families, peers, teachers, and society. It has to be reminded
once again that family–child interaction is such an important factor in the social and
emotional development of the hearing impaired.
During infancy and early childhood, individuals interact with the people around
them and have access to auditory inputs that will support the development of language and communication skills. Hence, each and every chance of input access
should be used by the family to have a child who is happy in the society of which s/
he a part and can communicate with others with great self-esteem.
References
1. What are the communication considerations for parents of deaf and hard-of hearing children?
https://les.eric.ed.gov/fulltext/ED496562.pdf. Accessed 23 Nov 2022.

5 Communication intheFamily ofaHearing-Impaired Child
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2. Dehgan L.Helpful tips for parents of children with hearing loss. 2017. https://hearinghealth-
foundation.org/blogs/helpful- tips- for- parents- of- children- with- hearing- loss. Accessed 23
Nov 2022.
3. Parenting tips for deaf children with additional needs. https://www.aussiedeafkids.org.au/
parenting- tips- for- deaf- children- with- additional- needs.html. Accessed 23 Nov 2022.
4. Fullana J, Pallisera M, Vilà M, Valls MJ, Díaz Garolera G.Intellectual disability and independent living: professionals’ views via a Delphi study. J Intellect Disabil. 2019;23:29–38.
5. Ashori M, Norouzi G, Jalil Abkenar SS.The effect of positive parenting program on mental
health in mothers of children with intellectual disability. J Intellect Disabil. 2019;23:1–15.
6. Antonopoulou K, Hadjikakou K, Stampoltzis A, Nicolaou N. Parenting styles of mothers with deaf or hard-of-hearing children and hearing siblings. J Deaf Stud Deaf Educ.
2012;17(3):306–18.
7. Szarkowski A, Brice P. Positive psychology in research with the deaf community: an idea
whose time has come. J Deaf Stud Deaf Educ. 2018;23(2):111–7.
8. Barr M, Duncan J, Dally K.A systematic review of services to DHH children in rural and
remote regions. J Deaf Stud Deaf Educ. 2018;23(2):118–30.
9. Broekhof E, Bos MGN, Camodeca M, Rieffe C.Longitudinal associations between bullying and
emotions in deaf and hard of hearing adolescents. J Deaf Stud Deaf Educ. 2018;23(1):17–27.
10. Sanders MR, Kirby JN, Tellegen CL.Towards a public health approach to parenting: a systematic review and meta-analysis of the Triple P-positive parenting program. Clin Psychol Rev.
2014;34(4):337–57.
11. Hallahan DP, Kauffman JM, Pullen PC.Exceptional learners: an introduction to special education, USA. 2018.
12. Rudner M, Seeto M, Keidser G, Johnson B, Rönnberga J.Poorer speech reception threshold
in noise is associated with lower brain volume in auditory and cognitive processing regions. J
Speech Lang Hear Res. 2019;62(4S):1117–30.
13. Ashori M, Jalil Abkenar SS.The effectiveness of cognitive rehabilitation program on auditory
perception and verbal intelligibility of deaf children. Am J Otolaryngol. 2019;40:693–702.
14. Zaidman Zait A, Most T, Tarrasch R, Haddad-eid E, Brand D.The impact of childhood hearing
loss on the family: mothers’ and fathers’ stress and coping resources. J Deaf Stud Deaf Educ.
2016;21(1):23–33.
15. Shin HY, Hwang HJ. Mental health of the people with hearing impairment in Korea: a
population- based cross-sectional study. Korean J Fam Med. 2017;38(2):57–63.
16. Lawyer G. Deaf education and deaf culture: lessons from Latin America. Am Ann Deaf.
2018;162(5):486–8.
17. Kirk S, Gallagher G, Coleman MR.Educating exceptional children. 14th ed. Boston: Cengage
Learning; 2015.
18. Ashori M, Abkenar SSJ.The effect of positive parenting program on interaction of mother and
deaf child. W J Yoga Phys Ther Rehabil. 2019;1(2). WJYPR.MS.ID.000506.
19. Fujiwara T, Kato N, Matthew R.Effectiveness of group positive parenting program (triple p)
in changing child behavior, parenting style, and parental adjustment: an intervention study in
Japan. J Child Fam Stud. 2011;20(6):804–13.
20. Ruane A, Carr A.Systematic review and meta-analysis of steppingstones Triple P for parents
of children with disabilities. Fam Process. 2018;58(1):232–46.
21. Kleefman M, Jansen DEMC, Stewart RE.The effectiveness of steppingstones Triple P parenting support in parents of children with borderline to mild intellectual disability and psychosocial problems: a randomized controlled trial. BMC Med. 2014;12:191.
22. Sanders MR.Development, evaluation, and multinational dissemination of the Triple P-positive
parenting program. Annu Rev Clin Psychol. 2012;8:345–79.
23. Sanders MR, Pickering JA, Kirby JN.A commentary on evidenced-based parenting programs:
redressing misconceptions of the empirical support for Triple P.BMC Med. 2012;10:145.
24. Schappin R, de Graaf IM, Reijneveld SA.Effectiviteit van Triple P in Nederland: stand van
zaken en controverse. Kind en Adolescent. 2017;38(2):75–90.
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25. Lohan A, Mitchell AE, Filus A.Positive parenting for healthy living (Triple P) for parents
of children with type 1 diabetes: protocol of a randomised controlled trial. BMC Pediatr.
2016;16(1):158.
D. T. Eroğlu and C. C. Cingi

Part II
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Congenital and Neonatal Infections

Congenital Infections andHearing Loss:
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AnOverview
FatmaLevent, AyşeEnginArısoy,
andGailJ.Demmler-Harrison
6.1 Introduction
Hearing loss (HL), dened as a partial or total loss of the ability to detect sound, can
appear at any age and occurs when a part of the ear or auditory system has suffered
a structural or functional deviation from the norm [1]. Three of the major categories
of HL include (1) conductive: secondary to a mechanical concern preventing sound
from traveling efciently to the inner ear; (2) sensorineural: secondary to the inner
ear or eighth cranial nerve damage); and (3) mixed: a combination of conductive
and sensorineural [2]. Hearing loss can be mild to profound, may be unilateral or
bilateral, and may be stable, progressive, or uctuating.
Congenital HL is any hearing impairment identied at or shortly after birth and
is typically sensorineural [3]. Congenital HL may be due to hereditary or nonhereditary causes, including congenital infections and ototoxic medications (Table6.1) [4].
6
F. Levent (*)
Division of Pediatric Infectious Diseases, Department of Pediatrics, School of Medicine,
Texas Tech University, Lubbock, TX, USA
e-mail: fatma.levent@ttuhsc.edu
A. E. Arısoy
Division of Neonatology, Department of Pediatrics, Faculty of Medicine, Kocaeli University,
Kocaeli, Türkiye
e-mail: arisoyengin@yahoo.com
G. J. Demmler-Harrison
Division of Infectious Diseases, Department of Pediatrics, Baylor College of Medicine,
Houston, TX, USA
Infectious Disease Service, Texas Children’s Hospital, Houston, TX, USA
e-mail: gdemmler@bcm.edu
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2023
A. E. Arısoy et al. (eds.), Hearing Loss in Congenital, Neonatal and Childhood
Infections, Comprehensive ENT, https://doi.org/10.1007/978-3-031-38495-0_6
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Table 6.1 Leading causes of congenital and acquired sensorineural hearing loss
Congenital
Hereditary Multiple syndromes e.g., Alport, Pendred,
Usher, and Waardenburg
syndromes
Nonhereditary
Congenital infections Cytomegalovirus infection
is the most common, and
others include congenital
toxoplasmosis, rubella,
syphilis, and Zika virus
infections
Acquired
Inner ear dysplasia or
malformation
Perilymph stula A leak of inner ear uid
Prematurity Perinatal complications,
hyperbilirubinemia, noise,
and ototoxic drugs increase
the risk
Hyperbilirubinemia The highest risk with levels
above the threshold for
exchange transfusion
Bacterial
meningitis
Ototoxic drugs e.g., aminoglycosides,
Noise exposure Can occur over time with
Trauma Trauma to the temporal bone
Tumor Vestibular schwannoma
Heavy metals Lead poisoning is the most
Occurs early during the
infection
high-dose loop diuretics,
certain chemotherapeutic
agents (cisplatin),
salicylates, and antimalarial
drugs
constant or repeated
exposure
can cause sensorineural or
mixed hearing loss
occurs mostly in children
with neurobromatosis
type 2
common, but cadmium,
mercury, and arsenic also
may have toxic effects on
cochlear cells
Ranges from mild to
progressive
through a defect in the otic
capsule
F. Levent et al.

6 Congenital Infections andHearing Loss: AnOverview
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Infections, including cytomegalovirus (CMV), rubella, Zika virus, lymphocytic
choriomeningitis (LCMV), varicella-zoster virus (VZV), herpes simplex virus
(HSV) infections, toxoplasmosis, and syphilis, transmitted to the fetus or newborn
during pregnancy or childbirth may cause congenital HL [5]. The HL resulting from
congenital infections may be identied at birth but is frequently progressive or
delayed, emphasizing the need for universal newborn hearing screening (NHS) and
continued close monitoring.
Congenital HL affects approximately 2–3 of every 1000 newborns in one or both
ears [6]. The universal NHS in the United States of America (USA) in 2007 improved
HL detection with earlier speech and language intervention [7–11]. Universal NHS
programs have also been available in Canada, Europe, and most middle- and highincome countries [12]. Prevalence estimates are higher in countries where universal
NHS programs are not implemented [13].
Many childhood HL cases can be detected right after birth through NHS.However,
passing this test does not always lead to normal hearing. Thus, postnatal identication of HL will depend on later interactions and interventions [14].
The outcomes of HL, mainly if classied at a severe or profound degree, may
include behavioral difculties and learning and cognitive delays, and negatively
affect the quality of life of children [15]. Therefore, early detection of HL and intervention are critical.
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6.2 Pathophysiology
The pathophysiology leading to HL in viral infections is likely multifactorial.
Inammation may damage the cochlea, resulting in sensorineural HL (SNHL) [16].
A murine model study of HL induced in newborn mice infected with CMV found a
signicant inammatory component [17]. Another study suggested that CMV infection (CMVI) associated with HL may be related to reactive oxygen species (ROS)
induced inammation [18]. Human CMVI increases ROS levels and activates
inammatory bodies of nucleotide-binding oligomerization domain-like receptor
protein 3 (NLRP3) in the cochlea [19]. A study with newborn mice infected with
murine CMV reported virus-induced cochlear inammation during auditory development instead of direct virus-cytopathic effects that might contribute to cochlea
pathology and altered auditory function [20].
6.3 Infections
Congenital infections are among the primary causes of SNHL.Table 6.2 summarizes the general characteristics of leading congenital infections.

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Table 6.2 The general characteristics of leading congenital infections
Mode of
Infectious agent
Toxoplasma
gondii:
Intracellular
protozoan
Rubella virus:
RNA virus,
Togaviridae
family
Cytomegalovirus
(CMV): DNA
virus,
Herpesviridae
family
Herpes simplex
virus (HSV):
DNA virus,
Herpesviridae
family
Treponema
pallidum:
Spirochete
Zika virus:
Flaviviridae
CSF cerebrospinal uid, DNA deoxyribonucleic acid, Ig immunoglobulin, PCR polymerase chain
reaction, RNA ribonucleic acid
acquisition Transmission
Foodborne Higher risk of
transmission in
later gestation
More severe
symptoms in
earlier gestation
Inhalation Highest in the
rst trimester
Bodily
uids
Bodily
uids
Sexual
contact
Mosquitoborn
Transplacental PCR in blood,
Intrapartum HSV 1–2 PCR
Highest in the
third trimester
Highest in the
rst and second
trimesters
Diagnosis
(postnatal)
T. gondii specic
IgG, IgM, and
IgA, and PCR in
blood and CSF
Serum IgM and
PCR in blood,
nasopharyngeal
swab, urine, and
CSF
urine, and saliva
surface, blood,
and CSF
Non-treponemal
test in blood
PCR in blood,
urine, and CSF,
serum IgM
F. Levent et al.
Clinical
manifestations, in
addition to
sensorineural hearing
loss
Intracranial
calcications,
hydrocephalus, and
chorioretinitis;
asymptomatic >60%
of cases
Cataracts, cardiac
defects,
microcephaly, and
microphthalmia
Retinitis, intracranial
calcications,
microcephaly, and
mental retardation;
asymptomatic in 85%
of cases
Microcephaly,
intracranial
calcications, skin,
and ocular ndings
Multiple organ
systems and skin rash
Severe microcephaly,
ocular, and cardiac
abnormalities
6.3.1 Congenital Cytomegalovirus Infection
6.3.1.1 Epidemiology
Congenital CMVI (cCMVI) is the most common intrauterine infection in children,
with an incidence of approximately 0.5–1.3% and 40,000 new cases annually in the
USA and 0.64–0.7% of neonates worldwide [21–24]. The prevalence varies among
populations. It is estimated that cCMVI leads to about 13–22% of all cases of neonatal HL, making it one of the most common nonhereditary causes [25]. Congenital
CMVI is also the primary cause of long-term neurological and sensory sequelae, the
most common of which is SNHL [26]. Congenital CMVI-related HL is underestimated because most newborns are undiagnosed, which might be related to no
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