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Hearing Loss Is Common and Often Unrecognized In Preschool Children Following Cardiac Surgery In Infancy

Friday, October 19, 2012: 8:45 AM
Room 275-277 (Morial Convention Center)
Nancy B. Burnham, RN, MSN, CRNP, Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadephia, PA, Richard F. Ittenbach, PhD, Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, Carol Knightly, MA, AuD, CCC-A, Center for Childhood Communications, The Children's Hospital of Philadelphia, Philadephia, PA, Cynthia B. Solot, MA, CCC/SLP, Center for Childhood Communication, The Children's Hospital of Philadelphia, Philadelphia, PA, Marsha Gerdes, PhD, Psychology, The Children's Hospital of Philadelphia, Philadelphia, PA, Judy C. Bernbaum, MD, Pediatrics, The Children's Hospital of Philadelphia, Philadelphia, Gil Wernovsky, MD, FACC, FAAP, Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, Thomas L. Spray, MD, Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadelphia, PA, Susan C. Nicolson, MD, Department of Anesthesia and Critical Care Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA, Robert R. Clancy, MD, Dept. of Pediatrics Division of Neurology, The Children's Hospital of Philadelphia, Philadelphia, PA, Daniel J. Licht, MD, Dept. of Pediatrics, Division of Neurology, Children's Hospital of Philadelphia, Philadelphia, PA, Elaine Zackai, MD, Dept. of Pediatrics Division of Genetics, The Children's Hospital of Philadelphia, Philadelphia, PA and J. William Gaynor, MD, Children's Hospital of Philadelphia, Philadelphia, PA

Purpose: Screening programs reveal a prevalence of hearing loss (HL) of < 1% in the general preschool population. The prevalence of HL in preschool survivors of cardiac surgery in infancy is not known.  HL can be associated with neurodevelopmental (ND) disability, poor language skills and school performance, as well as behavioral problems.  There is an increased risk of HL after extracorporeal membrane oxygenation (ECMO) and in some forms of congenital heart disease (CHD), e.g. with deletions of chromosome 22q11. We investigated the prevalence of HL after cardiac surgery in infancy and the relationship of HL to patient and operative factors.

Methods: Audiologic evaluations were conducted as part of a prospective study evaluating genetic polymorphisms and ND outcome at 4 years of age after repair of CHD with cardiopulmonary bypass (CPB) with or without deep hypothermic circulatory arrest (DHCA) in infancy.  HL status was classified as normal or presence of any type of HL.  HL was sub-typed as conductive [CHL], sensorineural [SNHL], or indeterminate [IDHL].  Statistical analyses were conducted using prevalence estimates and a series of single- and multiple-covariate logistic regression equations

Results: ND evaluations were performed in 381 children and adequate audiologic evaluations obtained in 348 (91.3%). The prevalence (CI0.95%) of HL was 21.6% (17.2, 25.9).  The prevalence of SNHL, CHL, and IDHL were 6.9% (4.1, 9.7), 12.4% (8.8, 16.0), and 2.3% (0.6, 4.0) respectively.  Deletions of 22q11 were present in 15 children (4.3%, 9 with HL [60%]).  HL was present in 3 of 8 subjects who required ECMO (37.5%). Only 15/348 (4.3%) had been screened positive for HL prior to this study and 10 utilized a hearing aid.  After adjusting for patient and operative covariates, younger gestational age, longer postoperative length of stay and a confirmed genetic anomaly were associated with HL, all p<0.01. No operative factors, including duration of CPB and DHCA, were associated with HL.  The presence of HL was associated with worse language skills, p<0.01.

Conclusion: These findings suggest that the prevalence of HL in preschool children following heart surgery in infancy may be 10-fold higher than in the general population and HL is often unrecognized. HL is associated with worse language skills. Periodic surveillance for HL is indicated in survivors of heart surgery in infancy. Further studies are needed to identify potentially modifiable risk factors (environmental noise in ICU and ototoxic drugs).