Risk factors for auditory neuropathy/auditory synaptopathy.
OBJECTIVE
It was the aim of this study to describe risk factors in auditory neuropathy/auditory synaptopathy (AN/AS).


METHODS
Between 1997 and 2005, we diagnosed 37 children with AN/AS. They underwent a critical chart review for risk factors and etiological coincidences in this idiosyncratic disorder.


RESULTS
Eighteen neonates had a history of prematurity and low birth weight. Hyperbilirubinaemia was present in 13 children. Three patients had evidence of infection during pregnancy, and AN/AS was associated with complex syndromal diseases in 2 cases. A congenital, familial pattern was seen in 2 siblings. Seven patients had idiopathic AN/AS.


CONCLUSIONS
Rather than being a single etiological entity, AN/AS comprises a spectrum of risk factors and associated problems affecting the cochlea and the auditory pathway. This study shows that the majority of AN/AS in children is the result of perinatal problems and is not genetic in origin. Hyperbilirubinaemia is a common and etiologically significant finding in infants suffering from AN/AS. Thus, early hearing screening for AN/AS including transient evoked otoacoustic emissions and auditory brainstem response assessment among neonates with risk factors for AN/AS is crucial in order to better manage patients suffering from this disorder.
Beutner
Dirk
D
Department of Otorhinolaryngology, Head and Neck Surgery, University of Cologne, Cologne, Germany. dirk.beutner@uni-koeln.de
Foerst
Astrid
A
Lang-Roth
Ruth
R
von Wedel
Hasso
H
Walger
Martin
M
D000293:Adolescent; D001301:Audiometry, Pure-Tone; D001304:Auditory Diseases, Central / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D003051:Cochlea / Q000503:physiopathology*; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D006304:Health Status; D006319:Hearing Loss, Sensorineural / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology*; D006801:Humans; D007223:Infant; D007230:Infant, Low Birth Weight; D007231:Infant, Newborn; D007234:Infant, Premature; D008297:Male; D015997:Neonatal Screening / Q000379:methods*; D017084:Otoacoustic Emissions, Spontaneous / Q000502:physiology*; D012307:Risk Factors