Apparent cochlear nerve aplasia: To implant or not to implant?

Frank M. Warren, Richard H. Wiggins, Cache Pitt, H. Ric Harnsberger, Clough Shelton

Research output: Contribution to journalArticle

40 Citations (Scopus)

Abstract

Objective: To describe the imaging findings and clinical outcomes of children with apparent cochlear nerve aplasia undergoing cochlear implantation. Study Design: Retrospective case review. Setting: Tertiary care center. Patients: Three patients with imaging findings consistent with absent cochlear nerve canal on diagnostic imaging and questionable audiometric responses on testing who underwent promontory stimulation and subsequent cochlear implantation. Intervention(S): Magnetic resonance imaging and computed tomography, audiologic assessment, and cochlear implantation. Main Outcome Measure(S): Audiologic performance after cochlear implantation. RESULTS: Three patients were identified to have hearing loss on newborn hearing screening and underwent auditory brainstem response testing revealing absent brainstem responses. ASSR testing was inconclusive when performed. Imaging in all cases identified 1 ear with a small internal auditory canal with 2 nerves present, one of which seems to enter the vestibule in each case and the other is assumed to be the functioning facial nerve. There was a bony plate present over the entrance to the cochlea in 2 of the 3 patients. Over time, 2 of the families reported responses to auditory stimuli with amplification. Promontory stimulation testing showed reproducible responses to electrical stimuli in the ears in question. After cochlear implantation, all 3 patients have shown responses to auditory stimuli. Conclusion: The absence of a visible cochlear nerve or cochlear nerve canal on radiologic imaging does not preclude auditory innervation of the cochlea. Cochlear implantation can be a viable option for patients with apparent cochlear nerve aplasia who have undergone appropriate testing. Electronically evoked auditory brainstem response is critical in the evaluation of this patient group.

Original languageEnglish (US)
Pages (from-to)1088-1094
Number of pages7
JournalOtology and Neurotology
Volume31
Issue number7
DOIs
StatePublished - Sep 2010
Externally publishedYes

Fingerprint

Cochlear Nerve
Cochlear Implantation
Brain Stem Auditory Evoked Potentials
Cochlea
Ear
Facial Nerve
Diagnostic Imaging
Hearing Loss
Tertiary Care Centers
Hearing
Brain Stem
Retrospective Studies
Tomography
Magnetic Resonance Imaging
Outcome Assessment (Health Care)
Newborn Infant

Keywords

  • Cochlear implants
  • Cochlear nerve aplasia
  • Cochlear nerve canal
  • Evoked auditory potentials

ASJC Scopus subject areas

  • Otorhinolaryngology
  • Clinical Neurology
  • Sensory Systems
  • Medicine(all)

Cite this

Warren, F. M., Wiggins, R. H., Pitt, C., Harnsberger, H. R., & Shelton, C. (2010). Apparent cochlear nerve aplasia: To implant or not to implant? Otology and Neurotology, 31(7), 1088-1094. https://doi.org/10.1097/MAO.0b013e3181eb3272

Apparent cochlear nerve aplasia : To implant or not to implant? / Warren, Frank M.; Wiggins, Richard H.; Pitt, Cache; Harnsberger, H. Ric; Shelton, Clough.

In: Otology and Neurotology, Vol. 31, No. 7, 09.2010, p. 1088-1094.

Research output: Contribution to journalArticle

Warren, FM, Wiggins, RH, Pitt, C, Harnsberger, HR & Shelton, C 2010, 'Apparent cochlear nerve aplasia: To implant or not to implant?', Otology and Neurotology, vol. 31, no. 7, pp. 1088-1094. https://doi.org/10.1097/MAO.0b013e3181eb3272
Warren FM, Wiggins RH, Pitt C, Harnsberger HR, Shelton C. Apparent cochlear nerve aplasia: To implant or not to implant? Otology and Neurotology. 2010 Sep;31(7):1088-1094. https://doi.org/10.1097/MAO.0b013e3181eb3272
Warren, Frank M. ; Wiggins, Richard H. ; Pitt, Cache ; Harnsberger, H. Ric ; Shelton, Clough. / Apparent cochlear nerve aplasia : To implant or not to implant?. In: Otology and Neurotology. 2010 ; Vol. 31, No. 7. pp. 1088-1094.
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