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Auditory neuropathy spectrum disorder: a new approach to hearing aid fitting



The aim of this study was to explore the outcome of hearing aid amplification in children with auditory neuropathy spectrum disorder (ANSD) using speech P1 cortical auditory evoked potential (P1-CAEP).

Study design

Forty-five children were divided into three groups: the ANSD group (n=15), the sensorineural hearing loss (SNHL) group (n=15), and the normal hearing group (n=15). The ANSD group had a mean age of 48.2 (±29.4) months and included children with moderate-to-severe hearing loss, a history of bilateral hearing aid use for at least 6 months, and absence of comorbid disorders. The SNHL group was closely matched to the ANSD group. Verification of hearing aids was carried out twice with 6 months of interval and included evaluation of aided sound field and P1-CAEP, and evaluation using the Arabic version of the Infant Toddler Meaningful Auditory Integration Scale (IT-MAIS). The P1-CAEPs were elicited using the temporally modified synthetic ‘ba’ and ‘da’ syllables.


In the initial evaluation, 80% of ANSD children showed P1 response to the ‘ba’ stimulus and 87% of children to the ‘da’ stimulus. Only one child from the SNHL group did not show P1-CAEP responses to the ‘da’ stimulus. The latency of P1 was prolonged in both groups compared with the normal hearing loss group. At 6-month evaluation, the P1-CAEP latencies improved equally in the ANSD and SNHL groups. Children with absent responses persisted to have absent responses. The mean IT-MAIS scores was initially 45.5 (±20) in the ANSD group and 79 (±9) in the SNHL group and increased after 6 months in both groups. The IT-MAIS scores negatively correlated with the P1-CAEP latency in the two evaluation sessions and positively correlated with the age of hearing aid fitting.


Around 50% of ANSD children demonstrated benefit from amplification. They showed evidence of normal central auditory maturation and progress in auditory skill development. Longitudinal P1 recording is recommended in the comprehensive audiological test battery in ANSD population using temporally modified speech stimuli.


  1. 1

    Sininger YS. Identification of auditory neuropathy in infants and children. Paper presented at the Seminars in Hearing. Como, Italy: Guidelines Development Conference at National Health Service; 2008.

  2. 2

    Rance G. Auditory neuropathy/dys-synchrony and its perceptual consequences. Trends Amplif 2005; 9:1–43.

    Article  Google Scholar 

  3. 3

    Vlastarakos PV, Nikolopoulos TP, Tavoulari E, Papacharalambous G, Korres S. Auditory neuropathy: endocochlear lesion or temporal processing impairment? Implications for diagnosis and management. Int J Pediatr Otorhinolaryngol 2008; 72:1135–1150.

    Article  Google Scholar 

  4. 4

    Sharma A, Cardon G, Henion K, Roland P. Cortical maturation and behavioral outcomes in children with auditory neuropathy spectrum disorder. Int J Audiol 2011; 50:98–106.

    Article  Google Scholar 

  5. 5

    Walker E, McCreery R, Spratford M, Roush P. Children with ANSD fitted with hearing aids applying the AAA Pediatric Amplification Guideline: current practice and outcomes. J Am Acad Audiol 2016; 27:204–218.

    Article  Google Scholar 

  6. 6

    Shallop JK, Peterson A, Facer GW, Fabry LB, Driscoll CL. Cochlear implants in five cases of auditory neuropathy: postoperative findings and progress. Laryngoscope 2001; 111(Pt 1):555–562.

    Article  CAS  Google Scholar 

  7. 7

    Jeong S-W, Kim L-S, Kim B-Y, Bae W-Y, Kim J-R. Cochlear implantation in children with auditory neuropathy: outcomes and rationale. Acta Otolaryngol Suppl 2007; 127:36–43.

    Article  Google Scholar 

  8. 8

    Breneman AI, Gifford RH, Dejong MD. Cochlear implantation in children with auditory neuropathy spectrum disorder: long-term outcomes. J Am Acad Audiol 2012; 23:5–17.

    Article  Google Scholar 

  9. 9

    Rance G, Cone-Wesson B, Wunderlich J, Dowell R. Speech perception and cortical event related potentials in children with auditory neuropathy. Ear Hear 2002; 23:239–253.

    Article  Google Scholar 

  10. 10

    Teagle HF, Roush PA, Woodard JS, Hatch DR, Zdanski CJ, Buss E, Buchman CA. Cochlear implantation in children with auditory neuropathy spectrum disorder. Ear Hear 2010; 31:325–335.

    Article  Google Scholar 

  11. 11

    Rance G, Barker EJ. Speech perception in children with auditory neuropathy/dyssynchrony managed with either hearing aids or cochlear implants. Otol Neurotol 2008; 29:179–182.

    Article  Google Scholar 

  12. 12

    Ching TY, Day J, Dillon H, Gardner-Berry K, Hou S, Seeto M, et al. Impact of the presence of auditory neuropathy spectrum disorder (ANSD) on outcomes of children at three years of age. Int J Audiol 2013; 52 Suppl 2: S55–S64.

    Article  Google Scholar 

  13. 13

    American Academy of Audiology. Clinical practice guidelines: pediatric amplification. Reston, VA: American Academy of Audiology; 2013.

  14. 14

    Cardon G, Sharma A. Central auditory maturation and behavioral outcome in children with auditory neuropathy spectrum disorder who use cochlear implants. Int J Audiol 2013; 52:577–586.

    Article  Google Scholar 

  15. 15

    Kraus N, Bradlow AR, Cheatham MA, Cunningham J, King CD, Koch DB, et al. Consequences of neural asynchrony: a case of auditory neuropathy. J Assoc Res Otolaryngol 2000; 1:33–45.

    Article  CAS  Google Scholar 

  16. 16

    Hassan DM. Perception of temporally modified speech in auditory neuropathy. Int J Audiol 2011; 50:41–49.

    Article  Google Scholar 

  17. 17

    Northern L, Downs P. Hearing in children. In: Katz J, Burkard R, editors. Handbook of clinical audiology. 5th ed. USA: Lippincott Williams & Wilkins; 1991. 469–480.

    Google Scholar 

  18. 18

    Soliman S, El-Mahalawi T. Simple speech test as a predictor for speech reception threshold (SRT) in preschool children [Master thesis]. Egypt: Faculty of Medicine, Ain Shams University; 1984. Available at:, [Last accessed 2016 Apr 20].

  19. 19

    Kamal N, Tawfik S, Bassiony S, Hazzaa N, Shalaby A, Omar P. Efficacy of auditory processing training in cochlear implant children: a new approach [Doctoral thesis in Audiology]. Egypt: Audiology Unit, ORL Department, Ain Shams University; 2009. Available at: [Last accessed 2016 Apr 20].

  20. 20

    Zimmerman-Phillips S, Robbins AM, Osberger MJ. Assessing cochlear implant benefit in very young children. Ann Otol Rhinol Laryngol Suppl 2000; 185:42–43.

    Article  CAS  Google Scholar 

  21. 21

    Sharma A, Dorman MF, Spahr AJ. A sensitive period for the development of the central auditory system in children with cochlear implants: implications for age of implantation. Ear Hear 2002; 23:532–539.

    Article  Google Scholar 

  22. 22

    Nossair A, El Danasoury I, ElKholy W, Hassan D. Study of p1 speech evoked response in different pediatric age groups [Master thesis]. Egypt: Faculty of Medicine, Ain Shams University; 2012. Available at: [Last accessed 2016 Apr 20].

  23. 23

    Alvarenga KF, Amorim RB, Agostinho-Pesse RS, Costa OA, Nascimento LT, Bevilacqua MC. Speech perception and cortical auditory evoked potentials in cochlear implant users with auditory neuropathy spectrum disorders. Int J Pediatr Otorhinolaryngol 2012; 76:1332–1338.

    Article  Google Scholar 

  24. 24

    El Kholi W, Hegazi M. Auditory and language profi les in infants and children with auditory neuropathy/dyssynchrony. Sc J Az Med Fac (Girls) 2007; 28:729–740.

    Google Scholar 

  25. 25

    Tallal P. Temporal or phonetic processing defi cit in dyslexia? That is the question. Appl Psycholinguist 1984; 5:167–169.

    Article  Google Scholar 

  26. 26

    Nagarajan SS, Wang X, Merzenich MM, Schreiner CE, Johnston P, Jenkins WM, et al. Speech modifications algorithms used for training language learning-impaired children. IEEE Trans Rehabil Eng 1998; 6:257–268.

    Article  CAS  Google Scholar 

  27. 27

    Starr A, Picton TW, Kim R. Pathophysiology of auditory neuropathy. In: Sininger Y, Starr A, editors. Auditory neuropathy: a new perspective on hearing disorders. San Diego: Singular; 2001.67–82.

    Google Scholar 

  28. 28

    Sharma A, Gilley PM, Dorman MF, Baldwin R. Deprivation-induced cortical reorganization in children with cochlear implants. Int J Audiol 2007; 46:494–499.

    Article  Google Scholar 

  29. 29

    Sharma A, Nash AA, Dorman M. Cortical development, plasticity and re-organization in children with cochlear implants. J Commun Disord 2009; 42:272–279.

    Article  Google Scholar 

  30. 30

    Cardon G, Campbell J, Sharma A. Plasticity in the developing auditory cortex: evidence from children with sensorineural hearing loss and auditory neuropathy spectrum disorder. J Am Acad Audiol 2012; 23:396–411. quiz 495

  31. 31

    De Villers-Sidani E, Chang EF, Bao S, Merzenich MM. Critical period window for spectral tuning defined in the primary auditory cortex (A1) in the rat. J Neurosci 2007; 27:180–189.

    Article  CAS  Google Scholar 

  32. 32

    Berlin CI, Hood L, Morlet T, Rose K, Brashears S. Auditory neuropathy/dyssynchrony: diagnosis and management. Ment Retard Dev Disabil Res Rev 2003; 9:225–231.

    Article  Google Scholar 

  33. 33

    Raveh E, Buller N, Badrana O, Attias J. Auditory neuropathy: clinical characteristics and therapeutic approach. Am J Otolaryngol 2007; 28:302–308.

    Article  Google Scholar 

  34. 34

    Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol 2000; 11:309–315.

    PubMed  CAS  Google Scholar 

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Correspondence to Dalia Mohamed Hassan.

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Hassan, D.M. Auditory neuropathy spectrum disorder: a new approach to hearing aid fitting. Egypt J Otolaryngol 33, 67–77 (2017).

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  • auditory neuropathy spectrum disorder
  • cortical auditory evoked potential
  • hearing aids
  • temporally modified speech