Auditory Neuropathy Spectrum Disorder among DPOAEs Screened Infants
DOI:
https://doi.org/10.12970/2311-1917.2014.02.02.2Keywords:
Auditory neuropathy spectrum disorders, auditory brainstem response, otoacoustic emissions, hearing loss.Abstract
Objective: Auditory neuropathy spectrum disorder (ANSD) characterized by absent or severely abnormal auditory brainstem response (ABR) with intact outer hair cells functions, as evidenced by the presence of evoked otoacoustic emissions (OAEs) and/or cochlear microphonics. In Universal Newborn Hearing Screening (UNHS), all infants screened for hearing loss either by OAEs or ABR or both of them. The purpose of this work was to study ANSD among infants passed OAEs based UNHS program.
Design: UNHS distortion product OAEs (DPOAEs) based program were done for 10281 infants within six years duration. Click evoked ABR were performed to high risk for hearing loss infants and on parents concern about hearing loss for their passed UNHS program children among attendee of audiology and ORL clinics. Tympanograms and behavioral thresholds were performed for the study group.
Results: Thirteen infants met the inclusion criteria of ANSD were discovered among passed UNHS program infants (0.12%). Unilateral ANSD were diagnosed in 2 infants (the other ears were normal in one infant and ABR was preserved down to mild hearing loss in the other infant). All ANSD infants had absent click evoked ABR at the maximum presentation level of 100 dBnHL, the acoustic reflexes were absent while DPOAEs were presented in all affected ears.
Discussion and Conclusion: In spite of passing UNHS OAEs based program still some infants are missed for the early diagnosis of ANSD which mandate special concern in the screening procedures to be more inclusive for the diagnosis of those infants.
References
Mencher GT, Davis AC, Devoe SJ, Beresford D, Bamford JM. Universal neonatal hearing screening: past, present, and future. Am J Audiol 2001; 10: 3-12. http://dx.doi.org/10.1044/1059-0889(2001/002)
Kennedy C, McCann D, Campbell MJ, Campbell M, Thornton A. Universal newborn screening for permanent childhood hearing impairment: an 8-year follow-up of a controlled trial. Lancet 2005; 366: 660-2. http://dx.doi.org/10.1016/S0140-6736(05)67138-3
Joint Committee on Infant Hearing 1994 Position Statement; American Academy of Pediatrics Joint Committee on Infant Hearing. Pediatrics 1995; 95: 152-6.
Welch D, Greville KA, Thorne PR, Pudy SC. Influence of acquisition parameters on the measurement of click evoked otoacoustic emissions in neonates in a hospital environment. Audiology 1996; 35: 143-57. http://dx.doi.org/10.3109/00206099609071937
McPherson B, Li SF, Shi B-X, Tang JLF, Wong BYK. Neonatal hearing screening: evaluation of tone-burst and click-evoked otoacoustic emission test criteria. Ear Hear 2006; 27: 256-62. http://dx.doi.org/10.1097/01.aud.0000215971.18998.9d
Buchman CA, Roush PA, Teagle H, Brown CJ, Zdanski CJ, Grose JH. Characteristics of auditory neuropathy in children with cochlear nerve deficiency. Ear Hear 2006; 27: 399-408. http://dx.doi.org/10.1097/01.aud.0000224100.30525.ab
Rance G, Cone-Wesson B, Wunderlich J, Dowell R. Speech perception and cortical event related potentials in children with auditory neuropathy. Ear Hear 2002; 23: 239-53. http://dx.doi.org/10.1097/00003446-200206000-00008
Auditory neuropathy spectrum disorder (ANSD) guidelines. Guide Development Conference on the Identification and Management of Infants with Auditory Neuropathy. International Newborn Hearing Screening Conference, Como, Italy, 2008.
Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI. Auditory neuropathy. Brain 1996; 119: 741-53. http://dx.doi.org/10.1093/brain/119.3.741
Berlin CI, Morlet T, Hood LJ. Auditory neuropathy/ dyssynchrony: its diagnosis and management. Pediatr Clin North Am 2003; 50: 331-40. http://dx.doi.org/10.1016/S0031-3955(03)00031-2
Madden C, Rutter M, Hilbert L, Greinwald JH Jr., Choo D. Clinical and audiological features in auditory neuropathy. Arch Otolaryngol Head Neck Surg 2002; 128: 1026-30. http://dx.doi.org/10.1001/archotol.128.9.1026
Korver AMH, van Zanten GA, Meuwese-Jongejeugd A, van Straaten HLM, Oudesluys-Murphy AM. Auditory neuropathy in a low-risk population: A review of the literature. Int J Pediatr Otorhinolaryngol 2012; 76: 1708-11. http://dx.doi.org/10.1016/j.ijporl.2012.08.009
Foerst A, Beutner D, Lang-Roth R, Huttenbrink K, von Wedel H, Walger M. Prevalence of auditory neuropathy/ synaptopathy in a population of children with profound hearing loss. Int J Pediatr Otorhinolaryngol 2006; 70: 1415-22. http://dx.doi.org/10.1016/j.ijporl.2006.02.010
Beutner D, Foerst A, Lang-Roth R, von Wedel H, Walger M. Risk factors for auditory neuropathy/ auditory synaptopathy. ORL 2007; 69: 239-44. http://dx.doi.org/10.1159/000101545
Rance G, Beer DE, Cone-Wesson B, et al. Clinical findings for a group of infants and young children with auditory neuropathy. Ear Hear 1999; 20: 238-52. http://dx.doi.org/10.1097/00003446-199906000-00006
Amatuzzi MG, Northrop C, Liberman MC, et al. Selective inner hair cell loss in premature infants and cochlea pathological patterns from neonatal intensive care units autopsies. Arch Otolaryngol Head Neck Surg 2001; 127: 629-36. http://dx.doi.org/10.1001/archotol.127.6.629
Dowley AC, Whitehouse WP, Mason SM, Cope Y, Grant J, Gibbin KP. Auditory neuropathy: unexpectedly common in a screened newborn population. Dev Med Child Neurol 2009; 51: 642-6. http://dx.doi.org/10.1111/j.1469-8749.2009.03298.x
Corley VM, Crabbe LS. Auditory neuropathy and a mitochondrial disorder in a child: case study. J Am Acad Audiol 1999; 10: 484-8.
