ORIGINAL ARTICLE
AUDIOLOGICAL FINDINGS AND AIDED PERFORMANCE IN INDIVIDUALS WITH AUDITORY NEUROPATHY SPECTRUM DISORDER (ANSD) – A RETROSPECTIVE STUDY
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Department of Audiology, All India Institute of Speech and Hearing, Mysore, Karnataka, India
 
 
Publication date: 2013-03-31
 
 
Corresponding author
P. M. Jijo   

Jijo P.M., Lecturer in Audiology, Department of Audiology, All India Institute of Speech and Hearing, Mysore, Karnataka, India, Phone: +91 9620467904, e-mail: jijoaudio@gmail.com
 
 
J Hear Sci 2013;3(1):18-26
 
KEYWORDS
ABSTRACT
Background:
There is controversy in the literature regarding the utility of amplification in individuals with auditory neuropathy spectrum disorder (ANSD). Most of the studies regarding the effect of amplification on speech perception in ANSD have been carried out on children. Only a few investigations have studied this aspect in late onset ANSD. The majority of these studies report no aided improvement, with only a few reporting minimal benefit. Further, none of the studies in late onset ANSD evaluated the relation between audiological test results and aided improvement.

Aim:
The study aimed to investigate the effect of amplification on speech identification scores (SISs) in individuals with late onset ANSD. It also determined a relation between audiological test results and aided improvement.

Method:
The retrospective study was carried out by reviewing the clinical records of 64 patients with late onset ANSD.

Results:
Improvement in speech identification with hearing aids was observed in 30% of the ears evaluated. Correlation analysis revealed that there was a significant positive correlation between SISs obtained under headphones and aided performance. However, no relation was found between the degree of hearing loss and the aided performance or on the unaided sound field SIS.

Conclusions:
Unlike earlier studies, it was found that individuals with late onset ANSD did show improvement in speech perception when using hearing aids. The SIS under headphones was a major indicator of aided performance in adults with late onset ANSD.

REFERENCES (28)
1.
1 Starr A, Picton TW, Sininger YS, Hood LJ, Berlin CI. Auditory neuropathy. Brain, 1996; 119: 741–53.
 
2.
2 Berlin CI, Hood LJ, Hurley A, Wen H. Hearing aids: Only for hearing impaired patients with abnormal otoacoustic emissions. In: Berlin CI, (Ed.), Hair Cells and Hearing Aids. Singular Publishing Group, 1996; 99–112.
 
3.
3 Trautwein PG, Sininger YS, Nelson R. Cochlear implantation of auditory neuropathy. J Am Acad Audiol, 2000; 11: 309–15.
 
4.
4 Sininger Y, Oba S. Patients with auditory neuropathy: who are they and what can they hear? In: Sininger YS, Starr A, (eds.), Auditory Neuropathy: A New Perspective on Hearing Disorders. San Diego: Singular-Thomson Learning, 2001; 15–35.
 
5.
5 Katona G, Buki B, Farkas Z, Pytel J, Simon-Nagy E, Hirschberg J. Transitory evoked otoacoustic emission (TEOAE) in a child with profound hearing loss. Int J Pediatr Otorhinolaryngol, 1993; 26: 263–67.
 
6.
6 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: 238–52.
 
7.
7 Starr A, Sininger YS, Pratt H. The varieties of auditory neuropathy. J Basic Clin Physiol Pharmacol, 2000; 11: 215–30.
 
8.
8 Deltenre P, Mansbach AL, Bozet C et al. Auditory neuropathy with preserved cochlear microphonics and secondary loss of otoacoustic emissions. Audiology, 1999; 38: 187–95.
 
9.
9 Berlin CI, Hood LJ, Morlet T et al. Multi-site diagnosis and management of 260 patients with auditory neuropathy/dyssynchrony (auditory neuropathy spectrum disorder). Int J Audiol, 2010; 49: 30–43.
 
10.
10 Cone-Wesson B, Rance G, Sininger YS. Amplification and rehabilitation strategy for patients with auditory neuropathy. In: Sininger YS, Starr A (eds.), Auditory Neuropathy: A New Perspective on Hearing Disorders. San Diego: Singular-Thomson Learning, 2001; 233–50.
 
11.
11 Madden C, Rutter M, Hilbert L, Greinwald J, Choo D. Clinical and audiological features in auditory neuropathy. Arch Otolaryngol Head Neck Surg, 2002; 128: 1026–30.
 
12.
12 Rance G, Cone-Wesson B, Wunderlich J, Dowell RC. Speech perception and cortical event related potentials in children with auditory neuropathy. Ear Hear, 2002; 23: 239–53.
 
13.
13 Rance G, Barker EJ. Speech and language outcome in children with auditory neuropathy/dys-synchrony managed with either cochlear implants or hearing aids. Int J Audiol, 2009; 48: 313–20.
 
14.
14 Vanaja CS, Manjula P. LLR as a measure of benefit derived from hearing devices with auditory dys-synchrony. In: First Conference on Auditory Neuropathy, ed Sivashanker N, Shashikala H.R. Bangalore: Department of Speech Pathology and Audiology, National Institute of Mental Health and Neurosciences, 2004: 136–46.
 
15.
15 Zeng FG, Liu S. Speech perception in individuals with auditory neuropathy. J Speech Lang Hear Res, 2006; 49: 367–80.
 
16.
16 Shallop JK. Auditory neuropathy/dys-synchrony in adults and children. Semin Hear, 2002; 23: 215–22.
 
17.
17 Sininger YS, Hood LJ, Starr A, Berlin CI, Picton TW. Hearing loss due to auditory neuropathy. Audiology Today, 1995; 1: 10–13.
 
18.
18 Widen JE, Ferraro JA, Trouba SE. Progressive neural impairment: case report. J Am Acad Audiol, 1995; 1: 217–24.
 
19.
19 Starr A. The neurology of auditory neuropathy. In: Sininger YS, Starr A (eds.), Auditory Neuropathy: A New Perspective on Hearing Disorders. San Diego: Singular-Thomson Learning, 2001; 37–48.
 
20.
20 Wang Q, Gu R, Han D, Yang W. Familial auditory neuropathy. Laryngoscope, 2003; 113: 1623–29.
 
21.
21 Shivashankar N, Satishchandra P, Shashikala HR, Gore M. Primary auditory neuropathy – an enigma. Acta Neurol Scand, 2003; 108: 130–35.
 
22.
22 Berlin CI, Hood LJ, Morlet T, Wilensky D, St John P et al. Absent or elevated middle ear muscle reflexes in the presence of normal otoacoustic emissions: a universal finding in 136 cases of auditory neuropathy/dys-synchrony. J Am Acad Audiol, 2005; 16: 546–53.
 
23.
23 Jijo PM, Yathiraj A. Audiological characteristics and duration of the disorder in individuals with auditory neuropathy spectrum disorder (ANSD) – a retrospective study. J Ind Sp Lang Hear Ass, 2012; 26: 17–26.
 
24.
24 ANSI: American National Standard Institute. 1999. American national standard: Maximum permissible ambient noise for audiometric rooms. ANSI S3.1-1999. New York, ANSI.
 
25.
25 Yathiraj A, Vijayalakshmi CS. Phonemically balanced wordlist in Kannada. A test developed at the department of audiology, AIISH, Mysore, 2005.
 
26.
26 Young ED, Sachs MB. Representation of steady-state vowels in the temporal aspects of the discharge patterns of populations of auditory nerve fibers. J Acoust Soc Am, 1979; 66: 1381–403.
 
27.
27 Bess HF, Humes LE. Amplification and rehabilitation. In Audiology: the Fundamentals, 4th ed, Lippincott Williams & Wilkins, 2008; 257.
 
28.
28 Van Tassel DJ. Hearing loss, speech and hearing aids. J Speech Hear Res, 1993; 36: 228–44.
 
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