Colm Madden1, Michael Rutter, Lisa Hilbert, John H Greinwald, Daniel I Choo. 1. Center for Hearing and Deafness Research, Department of Pediatric Otolaryngology, Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229, USA. John.Greinwald@chmcc.org
Abstract
OBJECTIVE: To medically and audiologically characterize a population of children diagnosed as having auditory neuropathy (AN). STUDY DESIGN: Retrospective medical chart review. SETTING/ SUBJECTS: We identified 22 patients from a pediatric otology clinic in a tertiary care pediatric hospital setting. RESULTS: A genetic factor in AN is suggested by our identification of 3 families with 2 affected children and 2 other children with family histories that were positive for hearing loss. Clinical features common among our population included a history of hyperbilirubinemia (n = 11 [50%]), prematurity (n = 10 [45%]), ototoxic drug exposure (n = 9 [41%]), family history of hearing loss (n = 8 [36%]), neonatal ventilator dependence (n = 8 [36%]), and cerebral palsy (n = 2 [9%]). Full clinical and audiological data were available for 18 of the 22 children, including otoacoustic emissions, auditory brainstem responses with cochlear microphonics, and age-appropriate audiometric findings. Significantly, 9 of these 18 patients showed improvement in behavioral thresholds over time, indicating that a subset of children with AN may recover useful hearing levels. Also significant was the success of cochlear implantation in 4 children. CONCLUSIONS: Management of AN in children requires serial clinical and audiometric evaluations, with a prominent role for behavioral testing. Prematurity, genetics, and hyperbilirubinemia appear to be significant factors in the development of AN; hyperbilirubinemia can be associated with spontaneous improvement of hearing thresholds. For those children not benefiting from amplification or FM systems, cochlear implantation remains a potentially successful method of habilitation.
OBJECTIVE: To medically and audiologically characterize a population of children diagnosed as having auditory neuropathy (AN). STUDY DESIGN: Retrospective medical chart review. SETTING/ SUBJECTS: We identified 22 patients from a pediatric otology clinic in a tertiary care pediatric hospital setting. RESULTS: A genetic factor in AN is suggested by our identification of 3 families with 2 affected children and 2 other children with family histories that were positive for hearing loss. Clinical features common among our population included a history of hyperbilirubinemia (n = 11 [50%]), prematurity (n = 10 [45%]), ototoxic drug exposure (n = 9 [41%]), family history of hearing loss (n = 8 [36%]), neonatal ventilator dependence (n = 8 [36%]), and cerebral palsy (n = 2 [9%]). Full clinical and audiological data were available for 18 of the 22 children, including otoacoustic emissions, auditory brainstem responses with cochlear microphonics, and age-appropriate audiometric findings. Significantly, 9 of these 18 patients showed improvement in behavioral thresholds over time, indicating that a subset of children with AN may recover useful hearing levels. Also significant was the success of cochlear implantation in 4 children. CONCLUSIONS: Management of AN in children requires serial clinical and audiometric evaluations, with a prominent role for behavioral testing. Prematurity, genetics, and hyperbilirubinemia appear to be significant factors in the development of AN; hyperbilirubinemia can be associated with spontaneous improvement of hearing thresholds. For those children not benefiting from amplification or FM systems, cochlear implantation remains a potentially successful method of habilitation.
Authors: Joseph P Roche; Benjamin Y Huang; Mauricio Castillo; Marc K Bassim; Oliver F Adunka; Craig A Buchman Journal: Otol Neurotol Date: 2010-07 Impact factor: 2.311