title: Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony (auditory neuropathy spectrum disorder).
abstract: Test results and management data are summarized for 260 patients with diagnoses of Auditory Neuropathy Spectrum Disorder (ANSD). Hearing aids were tried in 85 of these patients, and 49 patients tried cochlear implants. Approximately 15% reported some benefit from hearing aids for language learning, while improvement in speech comprehension and language acquisition was reported in 85% of patients who were implanted. Approximately 5% (13/260) of the total population developed normal speech and language without intervention. Patients were diagnosed at our laboratory (n=66) or referred from other sites (n=194), and all showed absent/grossly abnormal auditory brainstem responses (ABR), often 'ringing' cochlear microphonics, and the presence or history of otoacoustic emissions. Etiologies and co-existing conditions included genetic (n=41), peripheral neuropathies (n=20), perinatal jaundice and/or anoxia and/or prematurity (n=74). These patients comprise 10% or more of hearing impaired patients; their language acquisition trajectories are generally unpredictable from their audiograms.
authors.affiliation: Kresge Hearing Research Laboratory, LSUHSC, New Orleans, Louisiana, USA. cberlin@cas.usf.edu
authors.forename: Charles I
authors.initials: CI
authors.lastname: Berlin
authors.forename: Linda J
authors.initials: LJ
authors.lastname: Hood
authors.forename: Thierry
authors.initials: T
authors.lastname: Morlet
authors.forename: Diane
authors.initials: D
authors.lastname: Wilensky
authors.forename: Li
authors.initials: L
authors.lastname: Li
authors.forename: Kelly Rose
authors.initials: KR
authors.lastname: Mattingly
authors.forename: Jennifer
authors.initials: J
authors.lastname: Taylor-Jeanfreau
authors.forename: Bronya J B
authors.initials: BJ
authors.lastname: Keats
authors.forename: Patti St
authors.initials: PS
authors.lastname: John
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Montgomery
authors.forename: Jon K
authors.initials: JK
authors.lastname: Shallop
authors.forename: Benjamin A
authors.initials: BA
authors.lastname: Russell
authors.forename: Stefan A
authors.initials: SA
authors.lastname: Frisch
mesh_terms: D000293:Adolescent; D000328:Adult; D001304:Auditory Diseases, Central / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003054:Cochlear Implants; D016208:Databases, Factual; D016057:Evoked Potentials, Auditory, Brain Stem; D005260:Female; D006310:Hearing Aids; D006801:Humans; D007223:Infant; D007804:Language Development; D008297:Male; D017084:Otoacoustic Emissions, Spontaneous; D013067:Speech Perception; D016896:Treatment Outcome; D055815:Young Adult