Key Words Auditory Neuropathy Spectrum Disorder Diagnosis Management Hearing Aids Cochlear Implants Rehabilitation

Background. Auditory neuropathy spectrum disorder (ANSD) has been reported in up to 10% of all children with hearing loss. It is widely accepted that it can be difficult to identify and manage children with ANSD through typical clinical procedures, in comparison to children with sensorineural hearing loss (SNHL). An important limitation in managing children with ANSD is that behavioral pure-tone audiograms are less predictive of a child’s intervention needs with respect to amplification and early rehabilitation. The objective of this study was to characterize the clinical profiles and audiological management of children with ANSD followed through a provincial universal newborn hearing screening (UNHS) program in one region of Canada from 2003-2013. Methods. Population-based data were collected at the Children’s Hospital of Eastern Ontario (CHEO), Ottawa, Canada between 2003 and 2013 for all children who were diagnosed with ANSD. Detailed characteristics were extracted including screening status, etiology, risk factors, and severity of hearing loss. Diagnostic, amplification, and intervention outcomes were also documented. Results. Analyses of 418 children indicated that 22 (5.3%) children were identified with ANSD and more than 30% had other disabilities. Children were identified at a median age of 7.5 months (IQR: 4.7, 22.5), fitted with amplification at a median age of 17.3 months (IQR: 12.1, 26.6) and 12 underwent cochlear implantation at a median age 26.4 months (IQR: 18.0, 47.3). Conclusion. More than 85.0% of children with ANSD are currently fitted with hearing aids or cochlear implants and have achieved some degree of open-set word recognition and communication development. Carmen Barreira-Nielsen, Ph.D., Audiology and Speech Language Pathology Department of Federal University of Espírito Santo BRAZIL Elizabeth Fitzpatrick, Ph.D., Faculty of Health Sciences, University of Ottawa, Ottawa, ON CANADA JoAnne Whittingham, M.Sc., Child Hearing Laboratory, Children’s Hospital of Eastern Ontario Research Institute, Ottawa, ON CANADA AUDITORY NEUROPATHY SPECTRUM DISORDER Carmen Barreira-Nielsen Elizabeth M. Fitzpatrick JoAnne Whittingham Characteristics and outcomes of children with Auditory Neuropathy Spectrum Disorder Caractéristiques et évolution des enfants ayant un trouble du spectre de neuropathie auditive