Transient Auditory Neuropathy in Infants: How to Conceptualize the Recovery of Auditory Brain Stem Response in the Context of Newborn Hearing Screening?

ABSTRACT

Newborn hearing screening programs are being introduced across the world. Depending on the protocol, these programs usually allow for the identification of neonates and young infants with auditory neuropathy spectrum disorder (ANSD). This article reviews the issues around auditory brain stem response (ABR) recovery in young infants, putting forward the suggestion that it is not necessarily always associated with normal physiological maturational changes. In some cases, it is related to pathological neuromaturational delay but also other factors. The author argues for conceptualizing ABR recovery as transient ANSD and firmly including it as part of the spectrum.

Newborn hearing screening - auditory neuropathy spectrum disorder - transient auditory neuropathy - neuromaturation - prematurity and low birth weight - support for families

REFERENCES

• 1 Starr A, Picton T W, Sininger Y, Hood L J, Berlin C I. Auditory neuropathy.  Brain. 1996;  119 (Pt 3) 741-753
• 2 Berlin CI, Bordelon J, St John P et al.. Reversing click polarity may uncover auditory neuropathy in infants.  Ear Hear. 1998;  19 (1) 37-47
• 3 Rapin I, Gravel J. “Auditory neuropathy”: physiologic and pathologic evidence calls for more diagnostic specificity.  Int J Pediatr Otorhinolaryngol. 2003;  67 (7) 707-728
• 4 Northern J. Guidelines for Identification and Management of Infants and Children with Auditory Neuropathy Spectrum Disorder. Aurora, CO: The Children's Hospital–Colorado; 2008
• 5 Kraus N. Auditory neuropathy: an historical and current perspective. In: Sininger Y, Starr A, eds. Auditory Neuropathy: A New Perspective on Hearing Disorders. London, United Kingdom: Singular; 2001: 1-14
• 6 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 (3) 239-253
• 7 Uus K, Bamford J. Effectiveness of population-based newborn hearing screening in England: ages of interventions and profile of cases.  Pediatrics. 2006;  117 (5) e887-e893
• 8 Varga R, Kelley PM, Keats BJ et al.. Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene.  J Med Genet. 2003;  40 (1) 45-50
• 9 Delmaghani S, del Castillo FJ, Michel V et al.. Mutations in the gene encoding pejvakin, a newly identified protein of the afferent auditory pathway, cause DFNB59 auditory neuropathy.  Nat Genet. 2006;  38 (7) 770-778
• 10 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 (3) 238-252
• 11 Berg AL, Spitzer JB, Towers HM, Bartosiewicz C, Diamond BE. Newborn hearing screening in the NICU: profile of failed auditory brainstem response/passed otoacoustic emission.  Pediatrics. 2005;  116 (4) 933-938
• 12 Starr A, Sininger Y, Winter M, Derebery MJ, Oba S, Michalewski HJ. Transient deafness due to temperature-sensitive auditory neuropathy.  Ear Hear. 1998;  19 (3) 169-179
• 13 Marlin S, Feldmann D, Nguyen Y et al.. Temperature-sensitive auditory neuropathy associated with an otoferlin mutation: deafening fever!.  Biochem Biophys Res Commun. 2010;  394 (3) 737-742
• 14 Nelson KR, Gilmore RL, Massey A. Acoustic nerve conduction abnormalities in Guillain-Barré syndrome.  Neurology. 1988;  38 (8) 1263-1266
• 15 Uus K. The UK context for newborn auditory neuropathy/dys-synchrony.  Presented at: The Auditory Neuropathy Workshop; January 12, 2004; Manchester, England
• 16 Psarommatis I, Riga M, Douros K et al.. Transient infantile auditory neuropathy and its clinical implications.  Int J Pediatr Otorhinolaryngol. 2006;  70 (9) 1629-1637
• 17 Berlin CI, Morlet T, Hood LJ. Auditory neuropathy/dyssynchrony: its diagnosis and management.  Pediatr Clin North Am. 2003;  50 (2) 331-340, vii–viii
• 18 Verhaegen K, Zarowski A, Verstraden N et al.. Delayed maturation of auditory pathways as potential source of mistakes in the diagnostics of hearing loss in neonates. Presented at: Beyond Newborn Hearing Screening Infant and Childhood Hearing in Science and Clinical Practice: Newborn Hearing Screening 2010 Conference; June 8–10, 2010; Como, Italy
• 19 Lafreniere D, Smurzynski J, Jung M, Leonard G, Kim DO. Otoacoustic emissions in full-term newborns at risk for hearing loss.  Laryngoscope. 1993;  103 (12) 1334-1341
• 20 Madden C, Rutter M, Hilbert L, Greinwald Jr JH, Choo DI. Clinical and audiological features in auditory neuropathy.  Arch Otolaryngol Head Neck Surg. 2002;  128 (9) 1026-1030
• 21 Attias J, Raveh E. Transient deafness in young candidates for cochlear implants.  Audiol Neurootol. 2007;  12 (5) 325-333
• 22 Russell SM, Hoffman R, Jafar JJ. Improvement of chronic hearing loss after shunt revision. A case report.  Surg Neurol. 2001;  56 (3) 185-188
• 23 Attias J, Sohmer H, Gold S, Haran I, Shahar A. Noise and hypoxia induced temporary threshold shifts in rats studied by auditory brain stem response.  Hear Res. 1990;  45 (3) 247-252
• 24 Alexander M, Thomas SV, Mohan PK, Narendranathan M. Prolonged brainstem auditory evoked potential latencies in tropical pancreatic diabetics with normal hearing.  Electromyogr Clin Neurophysiol. 1995;  35 (2) 95-98
• 25 Moore JK, Perazzo LM, Braun A. Time course of axonal myelination in the human brainstem auditory pathway.  Hear Res. 1995;  87 (1–2) 21-31
• 26 Moore JK, Linthicum Jr FH. Myelination of the human auditory nerve: different time courses for Schwann cell and glial myelin.  Ann Otol Rhinol Laryngol. 2001;  110 (7 Pt 1) 655-661
• 27 Birnholz JC, Benacerraf BR. The development of human fetal hearing.  Science. 1983;  222 (4623) 516-518
• 28 Kuhlman KA, Burns KA, Depp R, Sabbagha RE. Ultrasonic imaging of normal fetal response to external vibratory acoustic stimulation.  Am J Obstet Gynecol. 1988;  158 (1) 47-51
• 29 Starr A, Amlie RN, Martin WH, Sanders S. Development of auditory function in newborn infants revealed by auditory brainstem potentials.  Pediatrics. 1977;  60 (6) 831-839
• 30 Despland PA, Galambos R. The auditory brainstem response (ABR) is a useful diagnostic tool in the intensive care nursery.  Pediatr Res. 1980;  14 (2) 154-158
• 31 Krumholz A, Felix JK, Goldstein PH, McKenzie E. Maturation of the brainstem auditory evoked potentials in premature infants.  EEE Clin Neurophysiol. 1985;  62 12434
• 32 Hafner H, Pratt H, Blazer S, Sujov P. Critical ages in brainstem development revealed by neonatal 3-channel Lissajous' trajectory of auditory brainstem evoked potentials.  Hear Res. 1993;  66 (2) 157-168
• 33 Ponton CW, Eggermont JJ, Coupland SG, Winkelaar R. The relation between head size and auditory brain-stem response interpeak latency maturation.  J Acoust Soc Am. 1993;  94 (4) 2149-2158
• 34 Engle WA. American Academy of Pediatrics Committee on Fetus and Newborn . Age terminology during the perinatal period.  Pediatrics. 2004;  114 (5) 1362-1364
• 35 Spankovich C, Lustig LR. Restoration of brain stem auditory-evoked potential in maple syrup urine disease.  Otol Neurotol. 2007;  28 (4) 566-569
• 36 Neault M, Kenna M. Auditory dys-synchrony of infancy: best practices for diagnosis, monitoring and guidance. Presented at: The 3rd Annual National Early Hearing Detection and Intervention Meeting; February 18–21, 2004; Washington, DC
• 37 Aldosari M, Mabie A, Husain AM. Delayed visual maturation associated with auditory neuropathy/dyssynchrony.  J Child Neurol. 2003;  18 (5) 358-361
• 38 Antonow-Schlorke I, Helgert A, Gey C et al.. Adverse effects of antenatal glucocorticoids on cerebral myelination in sheep.  Obstet Gynecol. 2009;  113 (1) 142-151
• 39 Daigo M, Arai Y, Oshida K et al.. Effect of hypoxic-ischemic injury on serine palmitoyltransferase activity in the developing rat brain.  Pathobiology. 2008;  75 (6) 330-334
• 40 Sanchez ES, Bigbee JW, Fobbs W, Robinson SE, Sato-Bigbee C. Opioid addiction and pregnancy: perinatal exposure to buprenorphine affects myelination in the developing brain.  Glia. 2008;  56 (9) 1017-1027
• 41 Dror DK, Allen LH. Effect of vitamin B12 deficiency on neurodevelopment in infants: current knowledge and possible mechanisms.  Nutr Rev. 2008;  66 (5) 250-255
• 42 Badaracco ME, Siri MV, Pasquini JM. Oligodendrogenesis: the role of iron.  Biofactors. 2010;  36 (2) 98-102
• 43 McKenna MC, Campagnoni AT. Effect of pre- and postnatal essential fatty acid deficiency on brain development and myelination.  J Nutr. 1979;  109 (7) 1195-1204
• 44 Kjellmer I, Thordstein M, Wennergren M. Cerebral function in the growth-retarded fetus and neonate.  Biol Neonate. 1992;  62 (4) 265-270
• 45 Scherjon SA, Oosting H, de Visser BW, de Wilde T, Zondervan HA, Kok JH. Fetal brain sparing is associated with accelerated shortening of visual evoked potential latencies during early infancy.  Am J Obstet Gynecol. 1996;  175 (6) 1569-1575
• 46 Sarda P, Dupuy RP, Boulot P, Rieu D. Brainstem conduction time abnormalities in small for gestational age infants.  J Perinat Med. 1992;  20 (1) 57-63
• 47 Kim CR, Vohr BR, Oh W. Effects of maternal preeclampsia on brain-stem auditory evoked response in very low birth weight infants.  J Pediatr. 1995;  127 (1) 123-127
• 48 Wilson JMG, Jungner G. Principles and Practice of Screening for Disease. Geneva, Switzerland: World Health Organization; 1968
• 49 Sharma A, Nash AA, Dorman M. Cortical development, plasticity and re-organization in children with cochlear implants.  J Commun Disord. 2009;  42 (4) 272-279