Ireneusz Bielecki1, Anna Horbulewicz, Teresa Wolan. 1. Department of Pediatric Surgery, Division of Otolaryngology, Medical University of Silesia, Upper Silesian Center for Child Health, Medyków 16 street, 40-752 Katowice, Poland. ibielecki@sum.edu.pl
Abstract
OBJECTIVE: To determine the prevalence of Auditory Neuropathy Spectrum Disorder (ANSD) among infants with sensorineural hearing loss (SNHL) and discuss the risk factors. METHODS: The study group was comprised of children diagnosed with ANSD. Criteria for inclusion in the study group were as follows: permanent sensorineural hearing loss (SNHL); normal transient evoked or distortion product OAEs; absent or elevated middle-ear muscle reflexes (MEMR); severely abnormal or absent ABR waveform. All newborns were screened via a two-stage protocol, involving OAEs and ABR for the repeatedly referred cases and all neonates with one or more risk factors. RESULTS: From 2002 to 2011, 9419 infants whose hearing ability was uncertain or who had risk factors for hearing loss were investigated. From this population, 352 were diagnosed with SNHL. Of these 352 children, 18 (5.1%) were diagnosed with ANSD. In the ANSD group, prematurity and low birth weight (<1500 g) were observed in 5 cases; ototoxical medication in 8 cases; mechanical ventilation in excess of 5 days in 5 cases. Hyperbilirubinemia was observed in 7 cases, but severe hyperbilirubinemia requiring an exchange transfusion was not observed; 4 patients had no risk factors, 6 patients had only one risk factor, and the 8 remaining had two or more risk factors. CONCLUSION: Auditory Neuropathy Spectrum Disorder is not an extremely rare hearing disorder. Screening for hearing loss via OAEs, MEMR and ABR examinations concurrently are essential for the accurate diagnosis of ANSD.
OBJECTIVE: To determine the prevalence of Auditory Neuropathy Spectrum Disorder (ANSD) among infants with sensorineural hearing loss (SNHL) and discuss the risk factors. METHODS: The study group was comprised of children diagnosed with ANSD. Criteria for inclusion in the study group were as follows: permanent sensorineural hearing loss (SNHL); normal transient evoked or distortion product OAEs; absent or elevated middle-ear muscle reflexes (MEMR); severely abnormal or absent ABR waveform. All newborns were screened via a two-stage protocol, involving OAEs and ABR for the repeatedly referred cases and all neonates with one or more risk factors. RESULTS: From 2002 to 2011, 9419 infants whose hearing ability was uncertain or who had risk factors for hearing loss were investigated. From this population, 352 were diagnosed with SNHL. Of these 352 children, 18 (5.1%) were diagnosed with ANSD. In the ANSD group, prematurity and low birth weight (<1500 g) were observed in 5 cases; ototoxical medication in 8 cases; mechanical ventilation in excess of 5 days in 5 cases. Hyperbilirubinemia was observed in 7 cases, but severe hyperbilirubinemia requiring an exchange transfusion was not observed; 4 patients had no risk factors, 6 patients had only one risk factor, and the 8 remaining had two or more risk factors. CONCLUSION:Auditory Neuropathy Spectrum Disorder is not an extremely rare hearing disorder. Screening for hearing loss via OAEs, MEMR and ABR examinations concurrently are essential for the accurate diagnosis of ANSD.
Authors: Shuman He; John H Grose; Holly F B Teagle; Jennifer Woodard; Lisa R Park; Debora R Hatch; Patricia Roush; Craig A Buchman Journal: Ear Hear Date: 2015 May-Jun Impact factor: 3.570
Authors: William J Riggs; Joseph P Roche; Christopher K Giardina; Michael S Harris; Zachary J Bastian; Tatyana E Fontenot; Craig A Buchman; Kevin D Brown; Oliver F Adunka; Douglas C Fitzpatrick Journal: Front Neurosci Date: 2017-07-19 Impact factor: 4.677