Progressive, Long-Term Hearing Loss in Congenital CMV Disease After Ganciclovir Therapy
Tatiana M. Lanzieri, A. Chantal Caviness, Peggy Blum, Gail J. Demmler‐Harrison, Congenital Cytomegalovirus Longitudinal Study Group, Shahzad Ahmed, Hanna Baer, Amit Bhatt, Peggy Blum, Frank R. Brown, Francis I. Catlin, A. Chantal Caviness, David K. Coats, Jane C Edmonds, Marily Flores, Daniel Franklin, Cindy Gandaria, Jewel M. Greer, Carol Griesser, Mohamed A. Hussein, Isabella Iovino, A S Istas, Haoxing D. Jin, Mary K Kelinske, Joseph T. Klingen, Antone Laurente, Thomas Littman, Mary Murphy, Jerry A. Miller, Christopher T. Nelson, Daniel E. Noyola, Evelyn A. Paysse, Alan K. Percy, Sara Reis, Ann Reynolds, Judith C Rozelle, O’Brien Smith, Paul G. Steinkuller, Marie Turcich, Sherry Vinson, Robert G. Voigt, Bethann F. Walmus, Jill Williams, Daniel Williamson, Kimberly G. Yen, Martha D. Yow, Gail J. Demmler‐Harrison
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.