COCHLEAR IMPLANTATION IN CONGENITAL RUBELLA SYNDROME

Devira Zahara

ENT Department Medical Faculty of Universitas Sumatera Utara Medan

Cochlear implantation in children with congenital rubella syndrome can be a

fundamental and supportive intervention. Congenital rubella syndrome is a condition
that occurs in children at birth when the pregnant mother is infected with rubella virus
affecting the development of the fetus. Children born with this condition may suffer
multiple disabilities and health complications. The three main symptoms of congenital
rubella syndrome are deafness, eye problems, such as cataracts; and heart defects.
The importance of early detection of hearing loss in a child and intervention is made
as early as possible. Deafness also has implications for cognitive, emotional and social
development.

Treatment of congenital rubella syndrome involves managing any disorders

which are present. Surgery may be required to correct eye and heart defects, and
deafness can be treated using cochlear implants. Cochlear implant surgery is a
surgical process in which a device is inserted into the inner ear to replace its functions.
It can be an option for people who have severe to profound hearing loss from inner
ear damage and who receive limited benefit with hearing aids.

We have 3 cases with congenital rubella syndrome, 1 case also with autism
who underwent cochlear implant surgery. All cases showed variable degrees of
auditory and speech outcomes. Long-term follow-up is recommended for those cases.

Keywords: Congenital rubella syndrome, cochlear implant, auditory and speech

outcomes
 Congenital rubella syndrome (CRS) is a group of symptoms affecting infants
caused by rubella virus transmitted from infected mothers during pregnancy. Rubella
infection can cause fatal consequences in early pregnancy, such as: miscarriages,
stillbirths, and a variety of serious birth defects in infants. Congenital heart disease,
cataracts, developmental delay and hearing impairment are common birth defects
occurred in infants with rubella infection. Hearing impairment is the most common
defect. (Lanzieri, Redd, Abernathy, & Icenogle, n.d.)

Figure 1.Common clinical manifestation of Congenital Rubella Syndrome (CRS)

World Health Organization (WHO) predict that there are more than 100.000
children are born with CRS worldwide (Sao & Nayva, 2017). Meanwhile, in developing
countries, it was reported that there are 0,6-2,2 infants with CRS per 1000 live births
(Sabellha & Hager, 2012). Arumugam et.al (2014) found that CRS was the most
common syndrome associated with congenital profound deafness in Madras, India
(34% from 35 study participants) as shown in figure 1 (Arumugam, et al., 2015) Herini
et.al (2017) did a cross sectional study looking at CRS prevalence in Yogyakarta,
Indonesia. They estimated the ratio of cases to normal infants was 1:1500 during the
study period. Hearing impairment was found in all study participants who are
confirmed by laboratory test, as shown in Table 1 (Herini, Gunadi, Triono, & Mulyadi,
2017).

Figure 2. Distribution of selected hereditary syndrome commonly associated with Sensoru Neural Hearing Loss
(SNHL) in Madras, India (Arumugam et.al. 2017)
Table 1. Characterization of children with congenital rubella syndrome in Yogyakarta, Indonesia (Herini et.al.
2017)

Hearing loss typically occurs during the 6 to 12 months of life, however, it can
also occur at birth (Cohen, et al., 2014). Additionally, sensorineural hearing loss often
happen when maternal rubella infection develops within the first 16 weeks of
pregnancy. Previous study found that inner ear is most affected to damage during six
to twelve weeks of pregnancy. Thus, sensorineural hearing loss in newborns with CRS
is caused by hemorrhagic damage of organ corti, which is a sensory end organ of
hearing. Other than that, CRS can also disrupt further development of different parts
of inner ear and auditory nerve. Wild et al found that profound hearing loss was more
evident in children with CRS than mild to moderate one (Sao & Nayva, 2017).

The treatment depends on the severity of hearing loss. The treatment options
are cochlear implantation and use of hearing aids (Cohen, Durstenfeld, & Roehm,
2014). Arumugam et al. mentioned that deaf children with syndromic association,
including congenital rubella syndrome, do not have any contraindication for cochlear
implantation. It is safe to perform cochlear implantation in children with syndromic
association given that they are diagnosed early treated properly (Arumugam, et al.,
2015).

A cochlear implant is an electronis prosthesis in the inner ear. It consists of a

receiver, a head piece and a speech processor. The head piece is worn behind the
auricle, as shown in figure 3 below, and contains a microphone to gather the sound
and the equipment to transmit them to the internal part of the implant. The internal part
is put into the inner ear via surgery. The surgery to implant the receiver and electrodes
takes approximately two to three hours. The implant will be activated four weeks after
surgery. A cochlear implant can assist congenitally deaf children to achieve speech,
to assess the environmental souncd anf to have audioverbal communication skills
within one year. However, cochlear implantation does not mean the children will
successfully use the device. The implant will only improve sound perception, but does
not necessarily result in the correct processing of the implant. Therefore, the
exceptional support of the family determine the result of the implanted device a
successful communication aid. (Kanchanalarp, Cheewaruangroj, Thawin, &
Lertsukprasert, 2006).

Figure 3.Parts of cochlear implant

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