Hostname: page-component-cd9895bd7-dk4vv Total loading time: 0 Render date: 2024-12-26T21:35:03.476Z Has data issue: false hasContentIssue false

Auditory steady state response in auditory neuropathy

Published online by Cambridge University Press:  14 April 2010

A A Emara*
Affiliation:
MD Audiology Unit, ENT Department, Tanta University, Cairo, Egypt
T A Gabr
Affiliation:
MD Audiology Unit, ENT Department, Tanta University, Cairo, Egypt
*
Address for correspondence: Dr Afaf Ahmed Emara, Assistant Professor of Audiology, Tanta University, 11 Hassan Radwan Street, Tanta, Egypt. E-mail: [email protected]

Abstract

Review:

Auditory neuropathy is a disorder characterised by preservation of outer hair cell function, with normal otoacoustic emissions and/or cochlear microphonics, but an absent or distorted auditory brainstem response.

Purpose:

This study aimed to objectively assess hearing thresholds in patients with auditory neuropathy, using the auditory steady state response.

Materials and methods:

Thirteen patients with auditory neuropathy and 15 normal hearing subjects were examined. Audiological evaluation included basic audiological tests, otoacoustic emissions, auditory brainstem response and auditory steady state response.

Results:

In the auditory neuropathy patients, the auditory brainstem response was absent in 11 patients, while the auditory steady state response was absent in only three.

Conclusion:

The auditory steady state response may serve as a valuable objective measure for assessing the hearing threshold across different frequencies in patients with auditory neuropathy. We recommend that auditory steady state response be used to complete the evaluation of patients with auditory neuropathy.

Type
Main Articles
Copyright
Copyright © JLO (1984) Limited 2010

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Footnotes

Presented at the 6th Tanta International Conference on Advanced Otorhinolaryngology, 15–16 January 2009, Scientific Sessions (Cairo Sheraton Hotel), Tanta, Egypt.

References

1Starr, A, Picton, TW, Siniger, Y, Hood, LJ, Berlin, CI. Auditory neuropathy. Brain 1996;119:741–53CrossRefGoogle ScholarPubMed
2Kraus, N, Bradlow, AR, Cheatham, MA, Cunningham, J, King, CD, Koch, TG et al. Consequences of neural asynchrony: a case of auditory neuropathy. J Assoc Res Otolaryngol 2000;1:3345CrossRefGoogle ScholarPubMed
3Simmons, JL, Beauchaine, KL. Auditory neuropathy: case study with hyperbilirubinemia. J Am Acad Audiol 2000;11:337–47Google ScholarPubMed
4Madden, C, Hilbert, L, Rutter, M, Greinwald, J, Choo, D. Pediatric cochlear implantation in auditory neuropathy. Otol Neurotol 2002;23:163–8CrossRefGoogle ScholarPubMed
5Starr, A, Sininger, YS, Pratt, H. The varieties of auditory neuropathy. J Basic Clin Physiol Pharmacol 2000;11:215–30CrossRefGoogle ScholarPubMed
6Vlastarakos, 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;72:1135–50CrossRefGoogle ScholarPubMed
7Sininger, Y, Oba, S. Patients with auditory neuropathy: who are they and what can they hear? In: Sininger, Y, Starr, A, eds. Auditory Neuropathy: a New Perspective on Hearing Disorders. San Diego: Singular Publishing, 2001;1535Google Scholar
8Picton, TW, John, MS, Dimitrijevic, A, Purcell, D. Human auditory steady-state responses. Int J Audiol 2003;42:177219CrossRefGoogle ScholarPubMed
9Regan, D. Human Brain Electrophysiology: Evoked Potentials and Evoked Magnetic Fields in Science and Medicine. Amsterdam: Elsevier, 1989;41–2Google Scholar
10Attias, J, Buller, N, Rubel, Y, Raveh, E. Multiple auditory steady-state responses in children and adults with normal hearing, sensorineural hearing loss, or auditory neuropathy. Ann Otol Rhinol Laryngol 2006;115:268–76CrossRefGoogle ScholarPubMed
11Dowlev, AC, Whitehouse, WP, Mason, SN, Cope, Y, Grant, J, Gibbin, KP. Auditory neuropathy: unexpectedly common in a screened newborn population. Dev Med Child Neurol 2009;51:642–6CrossRefGoogle Scholar
12Rance, G, Dowell, RC, Rickards, FW, Beer, DE, Clark, GM. Steady-state evoked potential and behavioral hearing thresholds in a group of children with absent click-evoked auditory brainstem response. Ear Hear 1998;8:4861CrossRefGoogle Scholar
13Toca, T, Savio Lope, SG. Stable auditory evoked potentials in the study of two patients with auditory neuropathy. Acta Otorrinolaringol Esp 2005;56:240–5Google Scholar
14Kraus, N, Ozdamar, O, Stein, L, Reed, N. Absent auditory brainstem response: peripheral hearing loss or brain stem dysfunction? Laryngoscope 1984;94:400–6CrossRefGoogle ScholarPubMed
15Sininger, Y, Hood, LJ, Starr, A, Berlin, CI, Picton, TW. Hearing loss due to auditory neuropathy. Audiol Today 1995;7:1113Google Scholar
16Beutner, D, Foerst, A, Lang-Roth, R, von Wedel, H, Walger, M. Risk factors for auditory neuropathy/auditory synaptopathy. ORL J Otorhinolaryngol Relat Spec 2007;69:239–44CrossRefGoogle ScholarPubMed
17Starr, A, Picton, TW, Kim, R. Pathophysiology of auditory neuropathy. In: Sininger, Y, Starr, A, eds. Auditory Neuropathy: a New Perspective on Hearing Disorders. San Diego: Singular Publishing, 2001;6782Google Scholar
18Dimitrijevic, A, John, MS, Van Roon, P.Estimating the audiogram using multiple auditory steady-state responses. J Am Acad Audiol 2002;2:205–24Google Scholar
19Rickards, FW, Tan, IE, Cohen, IX, Wilson, OJ, Drew, JH, Clark, GM. Auditory steady-state evoked potential in newborns. Br J Audiol 1994;28:327–37CrossRefGoogle ScholarPubMed
20Rance, G, Rickards, FW, Cohen, LT, De, Vidi, Clark, GM. The automated prediction of hearing thresholds in sleeping subjects using auditory steady-state evoked potentials. Ear Hear 1994;16:499507CrossRefGoogle Scholar
21Rance, G, Beer, DE, Cone-Wesson, B, Shepher, RK, Dowell, RC, King, AM et al. Clinical findings for a group of infants and young children with auditory neuropathy. Ear Hear 1999;20:238–52CrossRefGoogle ScholarPubMed