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Semin Speech Lang 2012; 33(04): 354-366
DOI: 10.1055/s-0032-1326917
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

A Tutorial on Auditory Neuropathy/Dyssynchrony for the Speech-Language Pathologist and Audiologist

Kristin Uhler
1   University of Colorado, Denver—Medical Campus, Denver, Colorado
,
Amanda Heringer
2   University of Colorado, Boulder, Boulder, Colorado
,
Nanette Thompson
3   University Hospital, Aurora, Colorado
,
Christine Yoshinaga-Itano
2   University of Colorado, Boulder, Boulder, Colorado
› Author Affiliations
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Publication History

Publication Date:
18 October 2012 (online)

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Abstract

This article presents information about developmental outcomes of children with auditory neuropathy/auditory dyssynchrony (AN). Colorado data on the number of children screened and the number of children identified with unilateral and bilateral AN will be described. Descriptive information about the percent of children with AN with cognitive disability and disabilities other than hearing loss will be presented. Language outcomes of children with normal cognitive development will be presented. This article will also provide information about etiologies and audiological information of children with AN. It includes assessment tools that have been useful in decision making for children with AN.

Keywords

Auditory neuropathy - auditory dyssynchrony - auditory neural hearing loss - auditory neuropathy spectrum disorder - language development
 

  • References

  • 1 Ngo RYS, Tan HKK, Balakrishnan A, Lim SB, Lazaroo DT. Auditory neuropathy/auditory dys-synchrony detected by universal newborn hearing screening. Int J Pediatr Otorhinolaryngol 2006; 70: 1299-1306
    CrossrefPubMedGoogle Scholar
  • 2 Akman I, Özek E, Kulekci S, Türkdogan D, Cebeci D, Akdaş F. Auditory neuropathy in hyperbilirubinemia: is there a correlation between serum bilirubin, neuron-specific enolase levels and auditory neuropathy?. Int J Audiol 2004; 43: 516-522
    CrossrefPubMedGoogle Scholar
  • 3 Hinchcliffe R, Osuntokun BO, Adeuja AO. Hearing levels in Nigerian ataxic neuropathy. Audiology 1972; 11: 218-230
    CrossrefPubMedGoogle Scholar
  • 4 Starr A, Picton TW, Sininger Y, Hood LJ, Berlin CI. Auditory neuropathy. Brain 1996; 119 (Pt 3) 741-753
    CrossrefPubMedGoogle Scholar
  • 5 Berlin CI, Hood L, Morlet T, Rose K, Brashears S. Auditory neuropathy/dys-synchrony: diagnosis and management. Ment Retard Dev Disabil Res Rev 2003; 9: 225-231
    CrossrefPubMedGoogle Scholar
  • 6 Berlin CI, Hood LJ, Morlet T , et al. Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony (auditory neuropathy spectrum disorder). Int J Audiol 2010; 49: 30-43
    CrossrefPubMedGoogle Scholar
  • 7 Foerst A, Beutner D, Lang-Roth R, Huttenbrink KB, 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: 1415-1422
    CrossrefPubMedGoogle Scholar
  • 8 Manchaiah VKC, Zhao F, Danesh AA, Duprey R. The genetic basis of auditory neuropathy spectrum disorder (ANSD). Int J Pediatr Otorhinolaryngol 2011; 75: 151-158
    CrossrefPubMedGoogle Scholar
  • 9 Rapin I, Gravel J. “Auditory neuropathy”: physiologic and pathologic evidence calls for more diagnostic specificity. Int J Pediatr Otorhinolaryngol 2003; 67: 707-728
    CrossrefPubMedGoogle Scholar
  • 10 Psarommatis I, Riga M, Douros K , et al. Transient infantile auditory neuropathy and its clinical implications. Int J Pediatr Otorhinolaryngol 2006; 70: 1629-1637
    CrossrefPubMedGoogle Scholar
  • 11 Kirkim G, Serbetcioglu B, Erdag TK, Ceryan K. The frequency of auditory neuropathy detected by universal newborn hearing screening program. Int J Pediatr Otorhinolaryngol 2008; 72: 1461-1469
    CrossrefPubMedGoogle Scholar
  • 12 Kumar UA, Jayaram MM. Prevalence and audiological characteristics in individuals with auditory neuropathy/auditory dys-synchrony. Int J Audiol 2006; 45: 360-366
    CrossrefPubMedGoogle Scholar
  • 13 Xoinis K, Weirather Y, Mavoori H, Shaha SH, Iwamoto LM. Extremely low birth weight infants are at high risk for auditory neuropathy. J Perinatol 2007; 27: 718-723
    CrossrefPubMedGoogle Scholar
  • 14 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: 642-646
    CrossrefPubMedGoogle Scholar
  • 15 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: 1026-1030
    CrossrefPubMedGoogle Scholar
  • 16 Rapin I, Gravel J. “Auditory neuropathy”: physiologic and pathologic evidence calls for more diagnostic specificity. Int J Pediatr Otorhinolaryngol 2003; 67: 707-728
    CrossrefPubMedGoogle Scholar
  • 17 Sanyelbhaa Talaat H, Kabel AH, Samy H, Elbadry M. Prevalence of auditory neuropathy (AN) among infants and young children with severe to profound hearing loss. Int J Pediatr Otorhinolaryngol 2009; 73: 937-939
    CrossrefPubMedGoogle Scholar
  • 18 Sininger Y, Starr A. Auditory Neuropathy: A New Perspective on Hearing Disorders. Singular, Thomsen Learning: San Diego, CA, 2001
    Google Scholar
  • 19 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: 238-252
    CrossrefPubMedGoogle Scholar
  • 20 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: 737-742
    CrossrefPubMedGoogle Scholar
  • 21 Zadro C, Ciorba A, Fabris A , et al. Five new OTOF gene mutations and auditory neuropathy. Int J Pediatr Otorhinolaryngol 2010; 74: 494-498
    CrossrefPubMedGoogle Scholar
  • 22 Wang DY, Wang YC, Weil D , et al. Screening mutations of OTOF gene in Chinese patients with auditory neuropathy, including a familial case of temperature-sensitive auditory neuropathy. BMC Med Genet 2010; 11: 79
    PubMedGoogle Scholar
  • 23 Rodríguez-Ballesteros M, Reynoso R, Olarte M , et al. A multicenter study on the prevalence and spectrum of mutations in the otoferlin gene (OTOF) in subjects with nonsyndromic hearing impairment and auditory neuropathy. Hum Mutat 2008; 29: 823-831
    CrossrefPubMedGoogle Scholar
  • 24 Romanos J, Kimura L, Fávero ML , et al. Novel OTOF mutations in Brazilian patients with auditory neuropathy. J Hum Genet 2009; 54: 382-385
    CrossrefPubMedGoogle Scholar
  • 25 Gardner-Barry K. (2012). Cochlear implants in infants and children with ANSD: Experiences at the Sydney Cochlear Implant Center. Paper presented at: Diagnosis and Management of Auditory Neuropathy Spectrum Disorder Conference; March 15–17, 2012; St. Petersburg, FL
    PubMedGoogle Scholar
  • 26 Walton J, Gibson WPR, Sanli H, Prelog K. Predicting cochlear implant outcomes in children with auditory neuropathy. Otol Neurotol 2008; 29: 102-309
    PubMedGoogle Scholar
  • 27 Teagle HFB, Roush PA, Woodard JS , et al. Cochlear implantation in children with auditory neuropathy spectrum disorder. Ear Hear 2010; 31: 325-335
    CrossrefPubMedGoogle Scholar
  • 28 American Academy of Pediatrics, Joint Committee on Infant Hearing. Year 2007 position statement: principles and guidelines for early hearing detection and intervention programs. Pediatrics 2007; 120: 898-921
    CrossrefPubMedGoogle Scholar
  • 29 Cantle Moore R. The Infant Monitor of Vocal Production, 2004, 2006, 2008. Available at: http://www.ridbcrenwickcentre.com/imp . Accessed on September 7, 2012
    PubMedGoogle Scholar
  • 30 Meinzen-Derr J, Wiley S, Creighton J, Choo D. Auditory Skills Checklist: clinical tool for monitoring functional auditory skill development in young children with cochlear implants. Ann Otol Rhinol Laryngol 2007; 116: 812-818
    CrossrefPubMedGoogle Scholar
  • 31 Uhler K, Yoshinaga-Itano C, Gabbard SA, Rothpletz AM, Jenkins H. Longitudinal infant speech perception in young cochlear implant users. J Am Acad Audiol 2011; 22: 129-142
    Article in Thieme ConnectPubMedGoogle Scholar
  • 32 Fenson L, Marchman VA, Thal DJ, Dale PS, Reznick S, Bates E. MacArthur Bates Communicative Development Inventories (CDIs), 2nd ed. Baltimore, MD: Paul H. Brookes Publishing Co., Inc; 2007
    Google Scholar
  • 33 Ireton H. Child Development Inventory (CDI) manual. Minneapolis, MN: Minneapolis Behavior Science System; 1992
    Google Scholar
  • 34 Young A. Families as they are families as they become. Bad Ischl: Austria. Paper presented at: 1st International Congress on Family-centered Early Intervention for Children who are Deaf or Hard of Hearing. May 30–June 1, 2012. Available at: http://www.fcei2012.org . Accessed on September 7, 2012
    PubMedGoogle Scholar
  • 35 Aragon M, Yoshinaga-Itano C. Using Language ENvironment analysis to improve outcomes for children who are deaf or hard of hearing. Semin Speech Lang 2012; 33: 340-353
    Article in Thieme ConnectPubMedGoogle Scholar