Progressive, Long-Term Hearing Loss in Congenital CMV Disease After Ganciclovir Therapy

Abstract

Background: Long-term hearing outcomes among children with symptomatic congenital cytomegalovirus (CMV) disease who received 6-week ganciclovir therapy early in life are unknown.

Methods: Longitudinal study of 76 children with symptomatic congenital CMV disease, born 1983-2005, who were categorized into three groups: group A treated with ganciclovir; group B untreated who had microcephaly, chorioretinitis, or sensorineural hearing loss (SNHL; ≥25 dB) diagnosed in the first month of life (congenital); and group C untreated who did not meet criteria for group B.

Results: Patients in groups A (n = 17), B (n = 27), and C (n = 32) were followed to median age of 13, 11, and 13 years, respectively. In group A, patients received ganciclovir for median of 40 (range, 11-63) days; 7 (41%) had grade 3 or 4 neutropenia. Congenital SNHL was diagnosed in 11 (65%) patients in group A, 15 (56%) in group B, and none in group C. Early-onset SNHL was diagnosed between ages ≥1-12 months in an additional 4 (24%), 6 (22%), and 8 (25%) patients in groups A, B, and C, respectively. By the end of follow-up, 12 (71%), 16 (59%), and 7 (22%) of patients in groups A, B, and C, respectively, had severe (>70 dB) SNHL in the better-hearing ear.

Conclusions: In this study, most patients with symptomatic congenital CMV disease and congenital or early-onset SNHL eventually developed hearing loss severe enough to have been potential candidates for cochlear implantation, with or without 6-week ganciclovir therapy. Understanding long-term hearing outcomes of patients treated with 6-month oral valganciclovir (current standard of care) is needed.

Keywords: congenital cytomegalovirus; ganciclovir; sensorineural hearing loss.

Figure 1.

Proportion of patients with normal hearing and mild, moderate, and severe hearing loss in the poorer- and better-hearing ears by patient group and age. Hearing level was categorized based on the click ABR or average of the pure-tone thresholds at 0.5, 1, 2, and 4 kHz (four-frequency average), as follows: ≤20 dB as normal hearing (white), and 21-45 dB as mild (light pink), 46-70 dB as moderate (dark pink), and >70 dB as severe (red) hearing loss; number of children in each category is shown inside the bars. We categorized the ear with higher thresholds at the initial assessment with SNHL as the poorer-hearing ear. Patients were not included until the first assessment, and patients who died were excluded from age intervals after death occurred. Patients with missing assessment for a given interval were considered to have hearing level of prior assessment. One patient whose first ABR showed severe and moderate hearing loss in the poorer- and better-hearing ears, had normal hearing bilaterally during follow-up. Five patients whose first ABR showed mild hearing loss in the poorer-hearing ears had normal hearing bilaterally during follow-up. One treated patient who retained normal hearing throughout the follow-up did not have microcephaly or chorioretinitis at birth, and if untreated would have been categorized as group C. Abbreviations: ABR, auditory brainstem response; SNHL, sensorineural hearing loss.