Clinical features of hearing loss in congenital CMV and risk factor for progression

Objective: In congenital cytomegalovirus (cCMV), risk factors for sensorineural hearing loss (SNHL)or deterioration remain unclear, and little is known about specific patterns of sensorineural hearing loss (SNHL). We describe SNHL in a cohort of cCMV infants; we reviewed clinical features and their correlation with distribution, timing, and progression of SNHL. Design: Anonymised data regarding the first and the last available audiological assessment, along with clinical and treatment information from children with cCMV and SNHL followed up at Imperial College Healthcare NHS Trust (ICHT) and IRCSS-Meyer Children’s University Hospital (MCH) were reviewed. Study sample: Sixty-one children with cCMV and SNHL (both treated and untreated) were included. Results: Congenital SNHL was found in 47/61 (77%) children, and 14/61 (23%) developed late-onset SNHL (LO-SNHL) at a median of 4.7 years (IQRs 2.2–6.8). Considering the age at development of LO-HL significant differences were found between the curves of untreated and treated for 6 weeks or 6 months (log rank p = 0.04, 0.0007 respectively); no differences were found among patients treated for 6 weeks or 6 months. On audiological assessment, all tested frequencies were usually affected. The majority of children had abnormal brain imaging at baseline, and those with normal imaging were less likely to have deterioration. Conclusions: In this clinical cohort, LO-SNHL occurred more often in untreated children and the median age at deterioration was 4.7 years (IQRs 2.2–6.8), later than in other cohorts, highlighting the importance of ongoing audiological assessment until 5–6 years of age.