Skip to main content
Log in

Prevalence and Audiological Characteristics of Auditory Neuropathy Spectrum Disorder in Pediatric Population: A Retrospective Study

  • Original Article
  • Published:
Indian Journal of Otolaryngology and Head & Neck Surgery Aims and scope Submit manuscript

Abstract

Auditory neuropathy spectrum disorder (ANSD) is a type of hearing disorder which is challenging for assessment and rehabilitation. This disorder has been studied over a decade and prevalence of the disorder is variable. The study aimed at estimating the prevalence and audiological characteristics of ANSD in children. A retrospective study was conducted from the medical records of pediatric patients evaluated at Rajiv Gandhi Government General Hospital and Madras Medical College, Chennai to estimate the prevalence of ANSD. Medical records of 2,624 children evaluated during the period of November 2010 to October 2012 within the age range of 6 months to 12 years were analyzed. Out of 2,624 pediatric population assessed 217 (8.26 %) of them had unilateral or bilateral sensory neural hearing loss with varying degrees. Out of 217 children with sensory neural hearing loss 5.06 % (N = 11) had ANSD. Audiological characteristics varied among the group. Children with ANSD had varied degree of hearing thresholds from normal to profound hearing impairment. All of them had ‘A’ type tympanogram with absent stapedial reflexes. DPOAEs or TEOAEs were observed in 54 % of population with ANSD. All of them had abnormal auditory brainstem responses (ABR). Replicable cochlear microphonics was observed in 46 % of children with ANSD. These results indicate that ANSD is not a rare condition among children and we emphasize the use of objective tests like tympanometry, Stapedial Reflex test, otoacoustic emissions and ABR in routine hearing assessment procedure for all children to identify ANSD.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2

Similar content being viewed by others

References

  1. Berlin CI, Hood LJ, Morlet T, Wilensky D, Li L, Mattingly KR et al (2010) Multi site diagnosis and management of 260 patients with auditory neuropathy/dys-synchrony (auditory neuropathy spectrum disorder). Int J Audiol 49:30–43

    Article  PubMed  Google Scholar 

  2. Davis H, Hirsh S (1979) A slow brainstem response for low frequency audiometry. Audiology 18:445–461

    Article  CAS  PubMed  Google Scholar 

  3. Foerst A, Beutner D, Lang-Roth R, Huttenbrink KB, von Wedel H et al (2006) Prevalence of auditory neuropathy/synaptopathy in a population of children with profound hearing loss. Int J Pediatr Otorhinolaryngol 70:1415–1422

    Article  PubMed  Google Scholar 

  4. Kraus N (2001) Auditory neuropathy: an historical and current perspective. In: Sininger YS, Starr A (eds) Auditory neuropathy: a new perspective on hearing disorders. Singular-Thomson learning, Samdiago, pp 1–14

    Google Scholar 

  5. Kraus N, Bradlow MA, Cunningham CJ, King CD, Koch DB, Nicol TG et al (2000) Consequences of neural synchrony: a case of auditory neuropathy. J Assoc Res Otolaryngol 1:33–45

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Kumar AU, Jayaram M (2006) Prevalence and audiological characteristics in individuals with auditory neuropathy/auditory dys-synchrony. Int J Audiol 45:360–366

    Article  PubMed  Google Scholar 

  7. Lee JSM, McPherson B, Yuen KCP, Wong LLN (2001) Screening for auditory neuropathy in a school for hearing impaired children. Int J Pediatr Otorhinolaryngol 61:39–46

    Article  CAS  PubMed  Google Scholar 

  8. Maris M, Venstermans C, Boudewyns AN (2011) Auditory neuropathy/dys-synchrony as a cause of failed neonatal hearing screening. Int J Pediatr Otorhinolaryngol 75:973–975

    Article  CAS  PubMed  Google Scholar 

  9. Mittal R, Ramesh AV, Panwar SS, Nilkanthan A, Nair S, Mehra PR (2012) Auditory neuropathy spectrum disorder: its prevalence and audiological characteristics in an Indian tertiary care hospital. Int J Pediatr Otorhinolaryngol 76(9):1351–1354

    Article  PubMed  Google Scholar 

  10. Rance G, Beer DE, Cone-wesson B, Shephard RK, Dowell RC, King AM et al (1999) Clinical findings for a group of infants and young children with auditory neuropathy. Ear Hear 20:238–252

    Article  CAS  PubMed  Google Scholar 

  11. Rance G, Barker EJ, Sarant JZ, Ching TY (2007) Receptive language and speech production in children with auditory neuropathy/dys-synchrony type hearing loss. Ear Hear 28:694–702

    Article  PubMed  Google Scholar 

  12. Rea PA, Gibson WPR (2003) Evidence for surviving outer hair cell function in congenitally deaf ears. Laryngoscope 113:230–234

    Google Scholar 

  13. Rodríguez Domínguez FJ, Cubillana Herrero JD, Cañizares Gallardo N, Pérez Aguilera R (2007) Prevalence of auditory neuropathy: prospective study in a tertiary-care center. Acta Otorinolaryngol 58(6):239–245

    Article  Google Scholar 

  14. Sanyelbhaa Talaat H, Kabel AH, Samy H, Elbadry M (2009) Prevalence of auditory neuropathy among infants and young children with severe to profound hearing loss. Int J Pediatr Otorhinolaryngol 73(7):937–939

    Article  PubMed  Google Scholar 

  15. Starr A, Picton TW, Sininger Y, Hood L, Berlin CI (1996) Auditory neuropathy. Brain 119:741–753

    Article  PubMed  Google Scholar 

  16. Sininger YS, Oba S (2001) Patients with auditory neuropathy: who are they and what can they hear? In: In Sininger YS, Starr A (eds) Auditory neuropathy: a new perspective on hearing disorders. Singular-Thomson Learning, Samdiago, pp 15–35

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to S. S. Vignesh.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Vignesh, S.S., Jaya, V. & Muraleedharan, A. Prevalence and Audiological Characteristics of Auditory Neuropathy Spectrum Disorder in Pediatric Population: A Retrospective Study. Indian J Otolaryngol Head Neck Surg 68, 196–201 (2016). https://doi.org/10.1007/s12070-014-0759-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12070-014-0759-6

Keywords

Navigation