Auditory neuropathy spectrum disorder (ANSD) in referrals from neonatal hearing screening at a well-baby clinic
- Resource Type
- Authors
- Sara Dirckx; An Boudewyns; Paul Van de Heyning; Jenneke van den Ende; Frank Declau; Anouk Hofkens
- Source
- European journal of pediatrics
- Subject
- Male
medicine.medical_specialty
Hearing loss
Population
Comorbidity
Audiology
Congenital hearing loss
03 medical and health sciences
Neonatal Screening
0302 clinical medicine
Auditory neuropathy spectrum disorder
Risk Factors
030225 pediatrics
Evoked Potentials, Auditory, Brain Stem
otorhinolaryngologic diseases
Humans
Medicine
Hearing Loss, Central
030223 otorhinolaryngology
education
Referral and Consultation
Retrospective Studies
education.field_of_study
medicine.diagnostic_test
business.industry
Incidence
Infant, Newborn
Cochlear nerve
Infant
medicine.disease
Magnetic Resonance Imaging
Audiometry, Evoked Response
Hearing disorder
Child, Preschool
Pediatrics, Perinatology and Child Health
Female
Human medicine
medicine.symptom
Audiometry
business
- Language
- English
- ISSN
- 0340-6199
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. Little data are available regarding the prevalence of this condition in healthy newborns. 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. Hearing screening was performed by automated auditory brain stem response (ABR) testing. A diagnosis of ANSD was established when ABR tracings were absent in the presence of otoacoustic emissions and/or a cochlear microphonic. Amongst 201 infants with confirmed congenital hearing loss, 13 infants were diagnosed with ANSD. The condition was unilateral in six and bilateral in seven infants. A risk factor for hearing loss could be identified in three infants. Abnormalities on magnetic resonance imaging were found in six infants; five of them had cochlear nerve deficiency.The prevalence of ANSD was 6.5 % amongst well babies with confirmed congenital hearing loss identified through UNHS. The estimated incidence of ANSD in our population of newborns at the well-baby clinic was 0.09/1000 live births. Magnetic resonance revealed an underlying anatomical abnormality in about half of the patients.• Auditory neuropathy dyssynchrony spectrum disorder (ANSD) is a particular form of hearing loss, mostly encountered in neonatal intensive care unit (NICU) graduates. • Little data are available on the prevalence and risk factors for ANSD in healthy newborns. What is new: • The estimated prevalence of ANSD in healthy newborns is 0.09/1000 live births. • In about half of the healthy newborns with ANSD, a structural abnormality was detected on magnetic resonance imaging of the posterior fossa/brain.