Our patient was born prematurely at 26 4/7 weeks with a birth weight of 1050 g as the first of dichorionic diamniotic twins. The early neonatal course was complicated by respiratory insufficiency, due to hyaline membrane disease, treated with surfactant and mechanical ventilation for 7 days. In addition, as a bacterial infection could not be ruled out, antibiotics were routinely administered. A transient circulatory insufficiency was treated with volume expansion. Hyperglycaemia was treated with insulin and mild hyperbilirubinaemia (maximum bilirubin level 250 μmol/l) was treated with phototherapy. Brain ultrasound showed a bilateral grade II intraventricular haemorrhage in the first days of life; a mild posthaemorrhagic ventricular dilatation resolved spontaneously. Recurrent anaemia was treated with several CMV-free erythrocyte top-up transfusions.
At 3 weeks of age, when the child was already respiratory stable, increasing signs of a pulmonary disease became evident. The diagnostic programme included a virological work-up, and CMV was cultured from the urine. CMV PCR diagnostics in the Guthrie card at day 3 was negative. Breast milk culture was initially negative, but a second test 6 weeks after birth was positive for CMV. Maternal IgG antibodies 1 week after birth were positive; IgM antibodies were negative. The relationship between the timing and results of the CMV diagnostics and the clinical course are presented in . Lung problems were treated with nasal positive airway pressure, supplemental oxygen and diuretics (a thiazide and an aldosterone antagonist) for a period of 4 weeks. No antiviral medication was prescribed.
CMV diagnostics in relation to clinical signs and postconceptional age.
Routine, automated, auditory brain response hearing screening failed around term age. Further audiological diagnostics at the corrected age of 6 weeks showed negative responses on the classic auditory brainstem response (ABR) on both sides, slightly lowered oto-acoustic emissions (OAEs) on the left side and normal OAEs on the right side. A normal tympanogram was recorded. These results are consistent with a diagnosis of hearing loss due to AN. The hearing level during observational audiometry at 1 year of age was 90 dB. At 3.5 years of age, the hearing level was 35–40 dB until 3000 Hz. No reaction occurred above 3000 HZ. Initially the child communicated only through sign language. At 2 years follow-up no signs of a generalised neurological disease were present. Except for a delay in the speechlanguage domain, there was normal neurodevelopment. During the intake procedure for a cochlear implant procedure, the CT scan showed no congenital malformation of the inner ear and direct stimulation of the acoustic nerve showed no response. At 4 years of age, there is normal language perception and a mildly delayed language expression.
The twin sister did not develop any clinical or laboratory sign of acquired early CMV infection.