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Audiological changes over time in adolescents and young adults with auditory neuropathy spectrum disorder

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Abstract

Auditory neuropathy spectrum disorder (ANSD) describes a condition in which a patient’s otoacoustic emissions (OAE) are (or were at one time) present and auditory brainstem responses (ABR) are abnormal or absent. ANSD is also diagnosed based on the presence of cochlear microphonics and abnormal or absent ABRs with or without abnormalities of OAE. We noted the changes in audiological characteristics over time with respect to pure tone thresholds, OAEs and Speech Identification Scores (SIS) in seven individuals with ANSD. The results indicated that all the individuals with ANSD had decreased SIS over time, whereas there was subsequent reduction in pure tone thresholds only in nine out of fourteen ears. There was absence of OAEs for two individuals in both ears during the follow-up evaluations. There was no regular pattern of changes in pure tone thresholds or SIS across all individuals. This indicates that there may be gradual worsening of hearing abilities in individuals with ANSD. Thus, regular follow-up and monitoring of audiological changes are necessary for individuals with ANSD. Also, longitudinal studies need to be done to further add evidence to the audiological changes over time in individuals with ANSD.

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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. Starr A, Picton T, Sininger YS, Hood LJ, Berlin CI (1996) Auditory neuropathy. Brain 119:741–753

    Article  PubMed  Google Scholar 

  3. Deltenre P, Mansbach A, Bozet C, Christiaens F, Barthelemy P, Paulissen D, Renglet T (1999) Auditory neuropathy with preserved cochlear microphonic and secondary loss of otoacoustic emissions. Audiology 38:187–195

    Article  CAS  PubMed  Google Scholar 

  4. Rance G, Beer DE, Cone-Wesson B, Shepherd HK, Dowell RLC, 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 

  5. 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 

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

    Google Scholar 

  7. Starr A, Sininger YS, Pratt H (2000) The varieties of auditory neuropathy. J Basic Clin Physiol Pharmacol 11:215–230

    Article  CAS  PubMed  Google Scholar 

  8. Shivashankar N, Satishchandra P, Shashikala HR, Gore M (2003) Primary auditory neuropathy—an enigma. Acta Neurol Scand 108:130–135

    Article  CAS  PubMed  Google Scholar 

  9. Prabhu P, Avilala VKY, Manjula P (2012) Predisposing factors in individuals with late-onset auditory dys-synchrony. Asia Pac J Speech Lang Hear 15(1):41–50

    Article  Google Scholar 

  10. Jijo PM, Yathiraj A (2012) Audiological characteristics and duration of the disorder in individuals with auditory neuropathy spectrum disorder (ANSD)—a retrospective study. J Indian Speech Hear Assoc 26(1):17–26

    Google Scholar 

  11. Narne VK, Prabhu P, Chandan HS, Deepthi M (2014) Audiological profiling of 198 individuals with auditory neuropathy spectrum disorder. Hear Bal Commun 12(3):112–120

    Article  Google Scholar 

  12. Picton TW (2011) Auditory neuropathy—when time is broke. Human auditory-evoked potentials. Plural Publishing Inc, San Diego

    Google Scholar 

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

    Article  PubMed  Google Scholar 

  14. Madden C, Hilbert L, Rutter M, Greinwald J, Choo D (2002) Clinical and audiological features in auditory neuropathy. Arch Otolaryngol Head Neck Surg 18:1026–1030

    Article  Google Scholar 

  15. Hood L (1998) Auditory neuropathy: what is it and what can we do about it? Hear J 51(8):10–18

    Article  Google Scholar 

  16. Hood L, Berlin C, Morlet T, Brashears S, Rose K, Tedesco S (2002) Considerations in the clinical evaluation of auditory neuropathy/auditory dys-synchrony. Semin Hear 23(3):201–208

    Article  Google Scholar 

  17. Hood L (2002) Auditory neuropathy/auditory dys-synchrony: new insights. Hear J 55(2):10–18

    Article  Google Scholar 

  18. Carhart R, Jerger JF (1959) Preferred method for clinical determination of pure-tone thresholds. J Speech Hear Disord 24:330–345

    Article  Google Scholar 

  19. Yathiraj A, Vijayalakshmi CS. (2005). Phonemically balanced wordlist in Kannada. A test developed at the Department of Audiology, AIISH, Mysore

  20. Mayadevi S. (1974).The development and standardization of a common speech discrimination test for Indians. Unpublished dissertation submitted to University of Mysore, in part fulfillment of Masters Degree in Speech and Hearing

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

    Article  PubMed Central  PubMed  Google Scholar 

  22. Rance G, Beer DE, Cone-Wesson B, Shepherd 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 

  23. Amatuzzi MG, Northrop C, Liberman MC, Thornton A, Halpin C, Herrmann B et al (2001) Selective inner hair cell loss in premature infants and cochlea pathological patterns from neonatal intensive care unit autopsies. Arch Otolaryngol Head Neck Surg 127:629–636

    Article  CAS  PubMed  Google Scholar 

  24. Berlin C, Bordelon J, St. John P, Wilensky D, Hurley A, Kluka E, Hood L (1998) Reversing click polarity may uncover auditory neuropathy in infants. Ear Hear 19(1):37–47

    Article  CAS  PubMed  Google Scholar 

  25. Starr A, Sininger Y, Nguyen T, Michalewski J, Oba S, Abdala C (2001) Cochlear receptor (microphonic and summating potentials, otoacoustic emissions) and auditory pathway (auditory brain stem potentials) activity in auditory neuropathy. Ear Hear 22:91–99

    Article  CAS  PubMed  Google Scholar 

  26. Arnesen AR, Osen KK (1978) The cochlear nerve in the cat: topography, cochleotopy and fiber spectrum. J Comp Neurol 178:661–678

    Article  CAS  PubMed  Google Scholar 

  27. El-Badry MM, McFadden S (2009) Evaluation of inner hair cell and nerve fiber loss as sufficient pathologies underlying auditory neuropathy. Hear Res 255:84–90

    Article  PubMed Central  PubMed  Google Scholar 

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Acknowledgments

The authors acknowledge with gratitude Prof. S. R. Savithri, Director, All India Institute of Speech and Hearing, Mysore for permitting to conduct the study at the institute. The authors would also like to thank Dr. Vijaya Kumar Narne for his constant support, motivation and guidance in carrying out the research on this topic. The authors also like to acknowledge the participants for their co-operation.

Conflict of interest

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.

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Authors and Affiliations

  1. Department of Speech Language and Hearing Science, College of Health and Human Science, Purdue University, West Lafayette, USA

    Hunsur Suresh Chandan

  2. Department of Audiology, All India Institute of Speech and Hearing, Manasagangothri, Naimisham Campus, Mysore, 570006, Karnataka, India

    Prashanth Prabhu

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  1. Hunsur Suresh Chandan
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  2. Prashanth Prabhu
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Correspondence to Prashanth Prabhu.

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Chandan, H.S., Prabhu, P. Audiological changes over time in adolescents and young adults with auditory neuropathy spectrum disorder. Eur Arch Otorhinolaryngol 272, 1801–1807 (2015). https://doi.org/10.1007/s00405-015-3505-0

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  • DOI: https://doi.org/10.1007/s00405-015-3505-0

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