doi: 10.3109/14992020903160892īeutner D, Foerst A, Lang-Roth R, von Wedel H, Walger M (2007) Risk factors for auditory neuropathy/auditory synaptopathy. doi: 10.1542/peds.2004-2806īerlin CI, Hood LJ, Morlet T, Wilensky D, Li L, Mattingly KR, Taylor-Jeanfreau J, Keats BJ, John PS, Montgomery E, Shallop JK, Russell BA, Frisch SA (2010) Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony (auditory neuropathy spectrum disorder). doi: 10.1159/000103271īerg AL, Spitzer JB, Towers HM, Bartosiewicz C, Diamond BE (2005) Newborn hearing screening in the NICU: profile of failed auditory brainstem response/passed otoacoustic emission. doi: 10.1542/peds.2007-2333Īttias J, Raveh E (2007) Transient deafness in young candidates for cochlear implants. Magnetic resonance revealed an underlying anatomical abnormality in about half of the patients.Īmerican Academy of Pediatrics, Joint Committee on Infant Hearing (2007) Year 2007 position statement: principles and guidelines for early hearing detection and intervention programs. The estimated incidence of ANSD in our population of newborns at the well-baby clinic was 0.09/1000 live births. Abnormalities on magnetic resonance imaging were found in six infants five of them had cochlear nerve deficiency.Ĭonclusion: The prevalence of ANSD was 6.5 % amongst well babies with confirmed congenital hearing loss identified through UNHS. A risk factor for hearing loss could be identified in three infants. The condition was unilateral in six and bilateral in seven infants. Amongst 201 infants with confirmed congenital hearing loss, 13 infants were diagnosed with ANSD. A diagnosis of ANSD was established when ABR tracings were absent in the presence of otoacoustic emissions and/or a cochlear microphonic. Hearing screening was performed by automated auditory brain stem response (ABR) testing. We performed a retrospective medical records review of 791 referrals from universal neonatal hearing screening (UNHS) at a well-baby clinic to investigate the prevalence of ANSD. Little data are available regarding the prevalence of this condition in healthy newborns. Auditory neuropathy spectrum disorder (ANSD) is a particular kind of hearing disorder characterised by normal outer hair cell function and abnormal or absent auditory brain stem responses.
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