title: Clinical and audiological features in auditory neuropathy.
abstract: OBJECTIVE
  To medically and audiologically characterize a population of children diagnosed as having auditory neuropathy (AN).
  
  
  METHODS
  Retrospective medical chart review.
  
  
  METHODS
  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.affiliation: 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
authors.forename: Colm
authors.initials: C
authors.lastname: Madden
authors.forename: Michael
authors.initials: M
authors.lastname: Rutter
authors.forename: Lisa
authors.initials: L
authors.lastname: Hilbert
authors.forename: John H
authors.initials: JH
authors.lastname: Greinwald
authors.forename: Daniel I
authors.initials: DI
authors.lastname: Choo
mesh_terms: D001299:Audiometry; D002648:Child; D002675:Child, Preschool; D003056:Cochlear Nerve / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D017063:Outcome Assessment, Health Care; D011859:Radiography; D012189:Retrospective Studies; D012307:Risk Factors; D012720:Severity of Illness Index; D000160:Vestibulocochlear Nerve Diseases / Q000000981:diagnostic imaging* / Q000503:physiopathology* / Q000628:therapy