Rate of Neural Recovery in Implanted Children with Auditory Neuropathy Spectrum Disorder

Abstract

Objectives. The study objectives were to compare the rate of neural recovery and speech perception performance in children with auditory neuropathy spectrum disorder (ANSD) and children with sensorineural hearing loss (SNHL) from other etiologies.

Study Design. Cohort study.

Setting. Academic hospital and cochlear implant center.

Subjects and Methods. Ten children with ANSD were matched based on type of implant and age at implantation with peers diagnosed with SNHL. Electrically evoked compound action potential (ECAP) recovery functions were obtained to measure neural refractory behaviors in response to stimulation from the cochlear implant. Speech perception performance was measured using speech recognition thresholds (SRTs) for monosyllable and spondee word stimuli. These outcome measures were compared between groups.

Results. There was no difference in average recovery function exponent in children with ANSD compared to children with SNHL. Similarly, there were no differences in average SRTs in quiet and in noise in children with ANSD compared to children with SNHL. Relationships between SRT and recovery rate were not present within groups or for all subjects for SRT in quiet, but a significant relationship was found for all subjects for SRT in noise (P = .04).

Conclusions. Dyssynchronous neural activity in ANSD may affect temporal encoding of electrical stimulation from a cochlear implant. As a group, children with ANSD did not demonstrate slower neural recovery compared to those with SNHL, but there was slower neural recovery observed for some subjects. The utility of ECAP recovery functions on optimizing the stimulation rate for individual patients with ANSD requires further investigation.

Keywords

otology/neurotology cochlear implants pediatric otolaryngology

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References

Starr

Picton

Sininger

Hood

Berlin

. Auditory neuropathy. Brain. 1996;119(pt 3):741-753.

Rance

Beer

Cone-Wesson

. Clinical findings for a group of infants and young children with auditory neuropathy. Ear Hear. 1999;20(3):238-252.

Berlin

Hood

Morlet

. 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(8):546-553.

Starr

Sininger

Pratt

. The varieties of auditory neuropathy. J Basic Clin Physiol Pharmacol. 2000;11(3):215-230.

Pareyson

Scaioli

Berta

Sghirlanzoni

. Acoustic nerve in peripheral neuropathy: a BAEP study. Brainstem Auditory Evoked Potentials. Electromyogr Clin Neurophysiol. 1995;35(6):359-364.

Rodriguez-Ballesteros

del Castillo

Martin

. Auditory neuropathy in patients carrying mutations in the otoferlin gene (OTOF). Hum Mutat. 2003;22(6):451-456.

Rodriguez-Ballesteros

Reynoso

Olarte

. A multicenter study on the prevalence and spectrum of mutations in the otoferlin gene (OTOF) in subjects with nonsyndromic hearing impairment and auditory neuropathy. Hum Mutat. 2008;29(6):823-831.

Varga

Kelley

Keats

. Non-syndromic recessive auditory neuropathy is the result of mutations in the otoferlin (OTOF) gene. J Med Genet. 2003;40(1):45-50.

Rance

Cone-Wesson

Wunderlich

Dowell

. Speech perception and cortical event related potentials in children with auditory neuropathy. Ear Hear. 2002;23(3):239-253.

10.

Rance

McKay

Grayden

. Perceptual characterization of children with auditory neuropathy. Ear Hear. 2004;25(1):34-46.

11.

Zeng

Shannon

. Psychophysical laws revealed by electric hearing. Neuroreport. 1999;10(9):1931-1935.

12.

Berlin

Hood

Morlet

. Multi-site diagnosis and management of 260 patients with auditory neuropathy/dys- synchrony (auditory neuropathy spectrum disorder). Int J Audiol. 2010;49(1):30-43.

13.

Madden

Hilbert

Rutter

Greinwald

Choo

. Pediatric cochlear implantation in auditory neuropathy. Otol Neurotol. 2002;23(2):163-168.

14.

Teagle

Roush

Woodard

. Cochlear implantation in children with auditory neuropathy spectrum disorder. Ear Hear. 2010;31(3):325-335.

15.

Peterson

Shallop

Driscoll

. Outcomes of cochlear implantation in children with auditory neuropathy. J Am Acad Audiol. 2003;14(4):188-201.

16.

Brown

Hughes

Luk

Abbas

Wolaver

Gervais

. The relationship between EAP and EABR thresholds and levels used to program the nucleus 24 speech processor: data from adults. Ear Hear. 2000;21(2):151-163.

17.

Franck

Norton

. Estimation of psychophysical levels using the electrically evoked compound action potential measured with the neural response telemetry capabilities of Cochlear Corporation’s CI24M device. Ear Hear. 2001;22(4):289-299.

18.

Hughes

Brown

Abbas

Wolaver

Gervais

. Comparison of EAP thresholds with MAP levels in the nucleus 24 cochlear implant: data from children. Ear Hear. 2000;21(2):164-174.

19.

Garadat

Litovsky

. Speech intelligibility in free field: spatial unmasking in preschool children. J Acoust Soc Am. 2007;121(2):1047-1055.

20.

Levitt

. Transformed up-down methods in psychoacoustics. J Acoust Soc Am. 1971;49(suppl 2):467-477.

21.

Botros

Psarros

. Neural response telemetry reconsidered: II. The influence of neural population on the ECAP recovery function and refractoriness. Ear Hear. 2010;31(3):380-391.

22.

Brown

Abbas

Gantz

. Electrically evoked whole-nerve action potentials: data from human cochlear implant users. J Acoust Soc Am. 1990;88(3):1385-1391.

23.

Kiefer

Hohl

Sturzebecher

. Comparison of speech recognition with different speech coding strategies (SPEAK, CIS, and ACE) and their relationship to telemetric measures of compound action potentials in the nucleus CI 24M cochlear implant system. Audiology. 2001;40(1):32-42.

24.

Abbas

Brown

. Electrically evoked auditory brainstem response: refractory properties and strength-duration functions. Hear Res. 1991;51(1):139-147.

25.

Kraus

Bradlow

Cheatham

. Consequences of neural asynchrony: a case of auditory neuropathy. J Assoc Res Otolaryngol. 2000;1(1):33-45.

26.

Rance

Barker

Mok

Dowell

Rincon

Garratt

. Speech perception in noise for children with auditory neuropathy/dys-synchrony type hearing loss. Ear Hear. 2007;28(3):351-360.

27.

Shallop

Peterson

Facer

Fabry

Driscoll

. Cochlear implants in five cases of auditory neuropathy: postoperative findings and progress. Laryngoscope. 2001;111(4, pt 1):555-562.

28.

Zeng

Liu

. Speech perception in individuals with auditory neuropathy. J Speech Lang Hear Res. 2006;49(2):367-380.

29.

Shpak

Berlin

Luntz

. Objective measurements of auditory nerve recovery function in nucleus CI 24 implantees in relation to subjective preference of stimulation rate. Acta Otolaryngol. 2004;124(6):679-683.