Electrical Stapedial Reflex Thresholds in Cochlear Implant Recipients with Cochlear Nerve Deficiency: A Retrospective Study

Abstract

Introduction

Cochlear nerve deficiency (CND) is characterized by a thin or absent cochlear branch of the vestibulocochlear nerve, leading to sensorineural hearing loss (SNHL). The effectiveness of cochlear implants (CIs) in patients with CND is debated, with some studies indicating poor outcomes and others showing limited speech detection and discrimination abilities. Accurate objective measures are essential for a successful CI mapping.

Objective

To investigate the use of global stimulation with live speech to measure electrically evoked stapedial reflex thresholds (eSRTs) in CI recipients with CND and compare the results with behavioral comfort levels.

Methods

This retrospective study reviewed the CI database from January 2015 to December 2023 to identify patients with CND. The eSRT measurements following CI were conducted using a standard procedure. These levels were compared with the behavioral comfort levels from their mapping data.

Results

From the CI database of 273 recipients, six children with CND were identified whose eSRT levels were documented. Among these six, two children exhibited presence of electrical stapedial reflexes that correlated well with behavioral comfort levels in response to global stimulation of electrodes. None of them presented reflexes to individual electrode stimulation.

Conclusion

Global stimulation of electrodes has the potential to elicit eSRT in patients with CND more than individual electrode stimulation. Further research is crucial to validate the effectiveness of global stimulation in eliciting eSRT in a larger cohort of patients with CND. This would be a feasible test as an outcome measure, and it would help provide better prognostic counselling.

Keywords

cochlear nerve deficiency - electrical stapedial reflex - post operative - children - cochlear implant

Publication History

Received: 16 July 2024

Accepted: 11 March 2025

Article published online:
10 September 2025

• References

• 1 Valero J, Blaser S, Papsin BC, James AL, Gordon KA. Electrophysiologic and behavioral outcomes of cochlear implantation in children with auditory nerve hypoplasia. Ear Hear 2012; 33 (01) 3-18
• 2 Wu CM, Lee LA, Chen CK, Chan KC, Tsou YT, Ng SH. Impact of cochlear nerve deficiency determined using 3-dimensional magnetic resonance imaging on hearing outcome in children with cochlear implants. Otol Neurotol 2015; 36 (01) 14-21
• 3 Li Y, Yang J, Li Y. [Constitute, imaging and auditory characteristics of pediatric patients with congenital malformations of inner ear in sensorineural hearing loss]. Lin Chuang Er Bi Yan Hou Tou Jing Wai Ke Za Zhi 2011; 25 (01) 1-5
• 4 Mafong DD, Shin EJ, Lalwani AK. Use of laboratory evaluation and radiologic imaging in the diagnostic evaluation of children with sensorineural hearing loss. Laryngoscope 2002; 112 (01) 1-7
• 5 Ren C, Lin Y, Xu Z, Fan X, Zhang X, Zha D. Audiological characteristics and cochlear implant outcome in children with cochlear nerve deficiency. Front Neurol 2022; 13: 1080381
• 6 Gray RF, Ray J, Baguley DM, Vanat Z, Begg J, Phelps PD. Cochlear implant failure due to unexpected absence of the eighth nerve–a cautionary tale. J Laryngol Otol 1998; 112 (07) 646-649
• 7 Brown CJ, Abbas PJ, Gantz B. Electrically evoked whole-nerve action potentials: data from human cochlear implant users. J Acoust Soc Am 1990; 88 (03) 1385-1391
• 8 Lee ER, Friedland DR, Runge CL. Recovery from forward masking in elderly cochlear implant users. Otol Neurotol 2012; 33 (03) 355-363
• 9 Turner C, Mehr M, Hughes M, Brown C, Abbas P. Within-subject predictors of speech recognition in cochlear implants: a null result. Acoust Res Lett Online 2002; 3: 95-100 10.1121/1.1477875
• 10 He S, Shahsavarani BS, McFayden TC, Wang H, Gill KE, Xu L. et al. Responsiveness of the Electrically Stimulated Cochlear Nerve in Children With Cochlear Nerve Deficiency. Ear Hear 2018; 39 (02) 238-250
• 11 Chao X, Wang R, Luo J, Wang H, Fan Z, Xu L. Relationship between electrically evoked compound action potential thresholds and behavioral T-levels in implanted children with cochlear nerve deficiency. Sci Rep 2023; 13 (01) 4309
• 12 Raine C, Ajayi F, Cruikshank H, Khan S, Beesley P. Relationship of intraoperative stapedial reflex thresholds and programming thresholds and comfort levels. Paper presented at the XVI World Congress of Otorhinolaryngology Head and Neck Surgery. 1997
• 13 Jerger J, Oliver TA, Chmiel RA. Prediction of dynamic range from stapedius reflex in cochlear implant patients. Ear Hear 1988; 9 (01) 4-8
• 14 Bresnihan M, Norman G, Scott F, Viani L. Measurement of comfort levels by means of electrical stapedial reflex in children. Arch Otolaryngol Head Neck Surg 2001; 127 (08) 963-966
• 15 Lorens A, Walkowiak A, Piotrowska A, Skarzynski H, Anderson I. ESRT and MCL correlations in experienced paediatric cochlear implant users. Cochlear Implants Int 2004; 5 (01) 28-37
• 16 Raghunandhan S, Ravikumar A, Kameswaran M, Mandke K, Ranjith R. Electrophysiological Correlates of Behavioral Comfort Levels in Cochlear Implantees: A Prospective Study. Indian J Otolaryngol Head Neck Surg 2015; 67 (03) 210-222
• 17 Charroó LE, Bermejo S, Cordovez ASP, Rodríguez C, Finley CC, Saoji AA. Effect of Number of Electrodes Used to Elicit Electrical Stapedius Reflex Thresholds in Cochlear Implants. Audiol Neurootol 2021; 26 (03) 164-172
• 18 Zhang Z, Li Y, Hu L, Wang Z, Huang Q, Wu H. Cochlear implantation in children with cochlear nerve deficiency: a report of nine cases. Int J Pediatr Otorhinolaryngol 2012; 76 (08) 1188-1195
• 19 Young NM, Kim FM, Ryan ME, Tournis E, Yaras S. Pediatric cochlear implantation of children with eighth nerve deficiency. Int J Pediatr Otorhinolaryngol 2012; 76 (10) 1442-1448
• 20 Vincenti V, Ormitti F, Ventura E, Guida M, Piccinini A, Pasanisi E. Cochlear implantation in children with cochlear nerve deficiency. Int J Pediatr Otorhinolaryngol 2014; 78 (06) 912-917
• 21 Arumugam SV, Nair G, Paramasivan VK, Goyal S, Murali S, Kameswaran M. A Study of Outcome of Pediatric Cochlear Implantation in Patients with Cochleovestibular Nerve Deficiency. J Int Adv Otol 2020; 16 (02) 147-152
• 22 Othman IA, Abdullah A, See GB, Umat C, Tyler RS. Auditory Performance in Early Implanted Children with Cochleovestibular Malformation and Cochlear Nerve Deficiency. J Int Adv Otol 2020; 16 (03) 297-302
• 23 Shakhtour LB, Song S, Orobello NC, Garrett S, Ambrose T, Behzadpour HK. et al. Evaluation of Cochlear Implantation in Children With Cochlear Nerve Absence or Deficiency. Otolaryngol Head Neck Surg 2024; 171 (04) 1197-1204
• 24 Stephan K, Welzl-Müller K. Post-operative stapedius reflex tests with simultaneous loudness scaling in patients supplied with cochlear implants. Audiology 2000; 39 (01) 13-18
• 25 Hodges AV, Butts SL, King JE, Balkany TJ. Electrically elicited stapedial reflexes in Nucleus 24 cochlear implant users. VI International Conference on Cochlear Implants. 2002
• 26 Kutz Jr JW, Lee KH, Isaacson B, Booth TN, Sweeney MH, Roland PS. Cochlear implantation in children with cochlear nerve absence or deficiency. Otol Neurotol 2011; 32 (06) 956-961
• 27 Margolis RH. Detection of hearing impairment with the acoustic stapedius reflex. Ear Hear 1993; 14 (01) 3-10