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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 25  |  Issue : 1  |  Page : 40-42

Hearing loss in infant with congenital cytomegalovirus infection


Department of Otorhinolaryngology-Head and Neck Surgery, Faculty of Medicine, Dr. Cipto Mangunkusumo General Hospital, University of Indonesia, Jakarta, Indonesia

Date of Web Publication19-Jun-2019

Correspondence Address:
Dr. Tri Juda Airlangga
Department of Otorhinolaryngology-Head and Neck Surgery, Faculty of Medicine, Dr. Cipto Mangunkusumo General Hospital, University of Indonesia, Jakarta
Indonesia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.INDIANJOTOL_121_18

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  Abstract 


Hearing loss is one of the sequels that often happened as a result of congenital cytomegalovirus (CMV) infection. Difficulties in diagnosing congenital CMV infection cause late detection of hearing loss which affects the patient's management and prognosis. We present three cases of hearing loss in infants with congenital CMV infection with other risk factors such as hyperbilirubinemia, low birth weight, premature birth, and ototoxic drug usage. The results of hearing assessment, imaging findings, and antiviral treatments are discussed. Hearing assessments in patient with congenital CMV infection should be checked routinely because hearing loss in this disease can be progressive and fluctuating.

Keywords: Brainstem evoked response audiometry, congenital cytomegalovirus infection, hearing loss


How to cite this article:
Airlangga TJ, Bashiruddin JE. Hearing loss in infant with congenital cytomegalovirus infection. Indian J Otol 2019;25:40-2

How to cite this URL:
Airlangga TJ, Bashiruddin JE. Hearing loss in infant with congenital cytomegalovirus infection. Indian J Otol [serial online] 2019 [cited 2019 Jul 20];25:40-2. Available from: http://www.indianjotol.org/text.asp?2019/25/1/40/260718




  Introduction Top


Cytomegalovirus (CMV) is one of the causes of congenital infections, especially in developing countries. The prevalence of congenital CMV infection varies in each country with 0.64% overall around the world. The prevalence in developing countries is around 0.6%–6.1%.[1] Congenital CMV infection can occur as a result of transplacental transmission of virus from primary infection or maternal recurrent infection during pregnancy.[2] Complications of congenital CMV infections such as hearing loss, vision loss, and neurodevelopmental sequelae affect patient's developmental growth. Infants with congenital CMV infection are categorized into symptomatic and asymptomatic based on the clinical findings.[3] Almost 90% of neonates infected with CMV do not have clinical findings at birth, but around 10% cases are at risk for sensorineural hearing loss during childhood.[4] Infants with symptomatic infection are at much higher risk for hearing loss and neurodevelopmental sequelae.[3] We will discuss three cases of infants with congenital CMV infection and hearing loss that occurs.


  Case Report Top


The first case – a 2-month-old baby boy referred for auditory evaluation after previously diagnosed with congenital CMV infection and obtained abnormal results in both ears of otoacoustic emissions (OAEs) and brain stem evoked response audiometry (BERA) screening. He was born at 30-week gestation with a weight of 1350 g and a body length of 40 cm by cesarean section for indications of placental insufficiency. During pregnancy, the patient's mother was known to suffer CMV infection from a positive result of anti-CMV IgM. He was suffering from hyperbilirubinemia; from the analysis of bilirubin, his test value for total was 10.86 mg/dl, direct was 1.91 mg/dl, and indirect was 8.95 mg/dl. The patient got blue light therapy for 3 days. He was known to suffer congenital CMV infection from positive results of anti-CMV IgM and CMV polymerase chain reaction (PCR) which was examined when the patient was 3 days old. There were ventriculomegaly and calcification from head ultrasonography. The patient received intravenous ganciclovir 7.5 mg twice daily for 2 weeks. According to the patient's mother, he still showed a response such as following the direction of the voice and was surprised to hear loud voices. On physical examination at 2 months old, he obtained a weight of 2800 g and body length of 44 cm. His head size is normal. The result of the examination on both ears, nose, and mouth was within the normal limits. On the OAE examination, there was no disturbance emission in the cochlear outer hair cell in both patients' ears. He obtained positive results from repeat CMV PCR test and was given oral valganciclovir 45 mg twice a day for 6 weeks. After oral valganciclovir treatment, the patient got a negative result from repeat CMV. On follow-up at the age of 5 months, there was an emission disturbance in the cochlear outer hair cell on the left ear from OAE examination results. From the tympanometry examination, he obtained Type A in both ears. At the age of 8 months, OAE was performed. From the results of OAE, it was found that there was an emission disturbance in the cochlear outer hair cell on the left ear.

The second case – a 15-day-old baby girl referred for auditory examination. The patient was diagnosed with congenital CMV infection from a positive result of CMV PCR screening. The patient was treated for ileal atresia, and laparotomy was performed. During treatment, the patient got intravenous antibiotics of ampicillin and gentamicin for 7 days. There is no history of jaundice. The laboratory test results of patients are within the normal limits. On head ultrasonography, there was no abnormality. OAE and BERA screening examinations obtained within normal limits on both patients' ears. The patient was born with a body weight of 3710 g and body length of 50 cm. Head circumference of the patient is 34 cm. Hearing assessment was done when the patient was at 3 months old; from OAE examination, there was no disturbance of cochlear outer hair cell emission.

The third case – a baby boy aged 2 months who was consulted for auditory evaluation after previously being diagnosed with congenital CMV infection and obtained abnormal results of BERA and OAE screening. BERA click examination was done and obtained V wave at 60 dB nHL intensity for both ears. At 8 weeks of the gestation, the mother had a high fever for almost an entire week without knowing the cause. From the history of childbirth, the patient known to be born through normal delivery with the help of a midwife at the 34 weeks of gestation. When the baby was born, he did not cry immediately. The patient was born with a weight of 2200 g. At the age of 1 month, the patient appeared yellow and suffered from continuous vomits. The patient was hospitalized and when treated, he was known to have congenital CMV infection from a positive result of PCR CMV with hyperbilirubinemia. Analysis of bilirubin gave values for total 19.29 mg/dl, direct 16.67 mg/dl, and indirect 2.62 mg/dl. On head ultrasonography, there was no calcification or ventriculomegaly. He received intravenous ganciclovir 7.5 mg twice daily for 2 weeks and then was given oral valganciclovir 36 mg twice daily for 6 weeks. He got a negative result from repeat PCR CMV test after oral valganciclovir treatment. On physical examination, he obtained a weight of 2200 g, a body length of 44 cm, and a head circumference of 33 cm. The results of the examination on both ears, nose, and mouth are within the normal limits. The patient had a hearing assessment at the age of 5 months; from OAE examination, there was no disturbance of cochlear outer hair cell emission.

BERA click examination and noncontrast mastoid (cochlear) magnetic resonance imaging (MRI) were conducted to the three cases. The results of BERA click examination were presented in [Table 1], and the results of MRI were shown in [Table 2]. For the first cases, it was found T2-weighted (T2WI) hyperintensity in white matter at the bilateral temporal and occipital region. Written informed consent was obtained from the patient for publication of the report.
Table 1: Results of brain stem-evoked response audiometry click examination in 40 dB nHL intensity

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Table 2: Diameters of cochlear nerve and facial nerve from noncontrast cochlear magnetic resonance imaging

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  Discussion Top


Three cases were diagnosed with congenital CMV infection based on CMV PCR examination results before the patient was 21 days old. In the first and third cases, they were found to suffer from symptomatic congenital CMV infection with hyperbilirubinemia, one of which, was the first case ones, obtained calcifications and ventriculomegaly from head ultrasonography, whereas the second case was an infant with asymptomatic congenital CMV infection. It is because there were no clinical symptoms and abnormalities from laboratory or other imaging findings. Each case in this report had other risk factors of hearing loss beside congenital CMV infection.[5] For the first and third cases, premature birth, low birth weight, and hyperbilirubinemia were the risk factors. For the second case, the risk factor was the usage of ampicillin and gentamicin as ototoxic drugs.

Congenital CMV infection is one of the most common causes of hearing loss in children. Hearing loss occurs in 5%–10% of children with asymptomatic CMV infection, and 40%–50% of children with congenital CMV infection are symptomatic.[6] In the first case, from the BERA click examination at 5-month-old follow-up, there was an extension of the V-wave latency at 40 dB HL intensity in both ears compared to 2 months old. At 8-month-old follow-up, there was a shortening of the V-wave latency compared to the time when 5 months old. It can be concluded that there is a fluctuation in the latency of wave V which is one of the characteristics of hearing loss in congenital CMV infection.[7] For the third case, a V wave at 60 dB nHL intensity was obtained from the BERA click when the baby was 2 months old, and there was an improvement in hearing function with a shortening of the V-wave latency at 5-month-old follow-up compared to time when 2 months old. In the second case, the patient with asymptomatic congenital CMV infection, the BERA click examination at 3 months old found the shortening of the V wave compared to 0 month old. In Teissier et al.'s study, infants with symptomatic congenital CMV infection are having a greater risk of hearing loss compared with asymptomatic ones.[8]

In the case of symptomatic congenital CMV infection, hearing loss often occurs from severe to very severe and requires the use of hearing aids.[8] In this case report, two cases experienced mild-to-moderate hearing loss at 2 months old of BERA examination where both cases later experienced improved hearing function at follow-up. This is thought to be the effect of early antiviral treatment of valganciclovir which given for 6 weeks. In Yilmaz Çiftdogan and Vardar's study, there was a progressive improvement in hearing function for a year in the patient with congenital CMV infection who receive oral valganciclovir therapy.[9] Patients with congenital CMV infection are at risk of developing progressive and fluctuating hearing loss.[7] Some children who are infected with CMV may have normal hearing assessment result at birth and then develop into hearing lost during follow-up.[10] Hearing assessment needs to be carried out in routine every 6 months to 3 years and then annually to assess the development of hearing impairment caused by congenital CMV infection.[11]

The cochlear nerve size is called normal if it has a size equal to or greater than the VII nerve diameter seen on the MRI examination.[12] In the three cases above, from the noncontrast MRI examination of the mastoid, the size of the cochlear nerve is within normal limits where the size of the cochlear nerve is greater than the nerve VII size. In the first case, T2WI hyperintensity in white matter was found in regional temporal and occipital bilateral, which is one of the MRI images that indicate congenital CMV infection.[8]


  Conclusion Top


Newborns with symptomatic congenital CMV infection are more easily diagnosed than in infants with asymptomatic congenital CMV infection. From this case report, it could be concluded that congenital CMV infection needs further evaluation of hearing function because hearing loss in this disease is progressive and fluctuating. A multidisciplinary approach is needed to detect and treat early to reduce the risk of neurological development disorders, especially sensorineural hearing loss that affects individual and social development, and also improve the quality of life of the patients.

Financial support and sponsorship

This study was financially supported by HIBAH TADOK by DRPM Universitas Indonesia.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Lanzieri TM, Dollard SC, Bialek SR, Grosse SD. Systematic review of the birth prevalence of congenital cytomegalovirus infection in developing countries. Int J Infect Dis 2014;22:44-8.  Back to cited text no. 1
    
2.
Krause PR, Bialek SR, Boppana SB, Griffiths PD, Laughlin CA, Ljungman P, et al. Priorities for CMV vaccine development. Vaccine 2013;32:4-10.  Back to cited text no. 2
    
3.
Boppana SB, Ross SA, Fowler KB. Congenital cytomegalovirus infection: Clinical outcome. Clin Infect Dis 2013;57 Suppl 4:S178-81.  Back to cited text no. 3
    
4.
Li LQ, Tan JJ, Zhou Y, Yu JL. Does congenital cytomegalovirus infection lead to hearing loss by inducing mutation of the GJB2 gene? Pediatr Res 2013;74:121-6.  Back to cited text no. 4
    
5.
Karaca CT, Oysu C, Toros SZ, Naiboǧlu B, Verim A. Is hearing loss in infants associated with risk factors? Evaluation of the frequency of risk factors. Clin Exp Otorhinolaryngol 2014;7:260-3.  Back to cited text no. 5
    
6.
Korver AM, de Vries JJ, Konings S, de Jong JW, Dekker FW, Vossen AC, et al. DECIBEL study: Congenital cytomegalovirus infection in young children with permanent bilateral hearing impairment in the netherlands. J Clin Virol 2009;46 Suppl 4:S27-31.  Back to cited text no. 6
    
7.
Fowler KB. Congenital cytomegalovirus infection: Audiologic outcome. Clin Infect Dis 2013;57 Suppl 4:S182-4.  Back to cited text no. 7
    
8.
Teissier N, Bernard S, Quesnel S, Van Den Abbeele T. Audiovestibular consequences of congenital cytomegalovirus infection. Eur Ann Otorhinolaryngol Head Neck Dis 2016;133:413-8.  Back to cited text no. 8
    
9.
Yilmaz Çiftdogan D, Vardar F. Effect on hearing of oral valganciclovir for asymptomatic congenital cytomegalovirus infection. J Trop Pediatr 2011;57:132-4.  Back to cited text no. 9
    
10.
Foulon I, Naessens A, Faron G, Foulon W, Jansen AC, Gordts F, et al. Hearing thresholds in children with a congenital CMV infection: A prospective study. Int J Pediatr Otorhinolaryngol 2012;76:712-7.  Back to cited text no. 10
    
11.
Swanson EC, Schleiss MR. Congenital cytomegalovirus infection: New prospects for prevention and therapy. Pediatr Clin North Am 2013;60:335-49.  Back to cited text no. 11
    
12.
McClay JE, Booth TN, Parry DA, Johnson R, Roland P. Evaluation of pediatric sensorineural hearing loss with magnetic resonance imaging. Arch Otolaryngol Head Neck Surg 2008;134:945-52.  Back to cited text no. 12
    



 
 
    Tables

  [Table 1], [Table 2]



 

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