engleski

Progressive sensorineural hearing loss in a girl with congenital cytomegalovirus infection – case report

Introduction: Congenital cytomegalovirus infection (CMV) affects 1% of the live-born infants. Around 10% of children with conatal infection at birth have severe disease that includes intrauterine growth restriction (IUGR), jaundice, purpura, hepatosplenomegaly, microcephaly, intracerebral calcifications, and retinitis. The most common permanent consequence of the CMV infection - sensorineural hearing loss, can possibly develop even several years after the infection. PURPOSE is to describe clinical course of the congenital CMV infection in a girl with a special overview on sensorineural hearing loss. Case Report: We represent a fifteen-year-old girl born from the first pregnancy in 38th week of gestation. In early newborn period, IUGR, jaundice, the signs of intrauterine and etiologically unproven infection were noticed. Brain US showed granulation of periventricular white matter. Hearing test of early hearing loss, at the time unavailable in Croatia, was not conducted. Due to delay in motor development at the age of 2, 5 years, she was clinically admitted. The girl was included in polyvalent habilitation. At the age of 5, 5 years a hearing impairment was noted. Diagnostics (audiometry, ABR) concluded the complete sensorineural hearing loss on the left side. CMV serology showed positive IgG and negative IgM antibodies result. Brain MRI showed hyperintense lesions in subcortical white matter, the biggest by trigonum of the left lateral ventricle. Metabolic analysis excluded leukodystrophy. At the age of 9 she underwent adenectomy due to recurrent otitis followed by hearing deterioration. Progressive sensorineural hearing loss extended on the right ear. MR brain imaging showed no progression change. The advancement in postnatal diagnostics PCR on CMV from a dried blood drop (Guthrie card) enabled etiological diagnostics. Hearing aid was provided for the right ear. She had adequately developed speech and average level of intellectual functioning. Conclusion: Congenital CMV infection in 15% of the diseased leaves permanent neurological damage, most commonly sensorineural hearing loss and learning difficulties. Gestational age during period of infection determines pathological changes in the brain. It is imperative to recognize the disease on time in order for patient to undergo of treatment. Based on clinical picture, MR brain imaging, serology and subsequent retrograde PCR diagnostics, it was concluded that we were dealing with congenital CMV infection. Reference: 1. Ross SA, Boppana SB. Congenital cytomegalovirus infection: outcome and diagnosis. 5emin Pediatr Infect Dis. 2005 Jan ; 16(1):44-9. 2. Grosse 5D, Ross D5, Dollard SC. Congenital cytomegalovirus (CMV) infection as a cause of permanent bilateral hearing loss: a quantitative assessment. J Clin Virol. 2008 Feb ; 41(2):57-62. 3. Boppana 5B, Fowler KB, Pass RF i sur. Congenital cytomegalovirus infection: association between virus burden in infancy and hearing loss. J Pediatr. 2005 Jun ; 146(6):817-23. 4. Boudewyns A, Declau F, Smets K i sur. Cytomegalovirus DNA detection in Guthrie cards: role in the diagnostic work-up of childhood hearing loss. Otol Neurotol. 2009 Oct ; 30(7):943-9. 5. Christoni Z, Syggelou A, Soldatou A, Karakitsos P, Papaevangelou V. Evaluation of a modified extraction protocol increasing sensitivity in quantification of CMV viremia in Guthrie cards. J Clin Virol. 2012 Dec ; 55(4):360-2.

progressive sensorineural hearing loss ; congenital cytomegalovirus infection

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