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 language | English (US) |
|---|---|
| Pages (from-to) | 1088-1094 |
| Number of pages | 7 |
| Journal | Otology and Neurotology |
| Volume | 31 |
| Issue number | 7 |
| DOIs | |
| State | Published - Sep 2010 |
| Externally published | Yes |
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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
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 journal › Article
}
TY - JOUR
T1 - Apparent cochlear nerve aplasia
T2 - To implant or not to implant?
AU - Warren, Frank M.
AU - Wiggins, Richard H.
AU - Pitt, Cache
AU - Harnsberger, H. Ric
AU - Shelton, Clough
PY - 2010/9
Y1 - 2010/9
N2 - 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.
AB - 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.
KW - Cochlear implants
KW - Cochlear nerve aplasia
KW - Cochlear nerve canal
KW - Evoked auditory potentials
UR - http://www.scopus.com/inward/record.url?scp=77956344774&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=77956344774&partnerID=8YFLogxK
U2 - 10.1097/MAO.0b013e3181eb3272
DO - 10.1097/MAO.0b013e3181eb3272
M3 - Article
C2 - 20634773
AN - SCOPUS:77956344774
VL - 31
SP - 1088
EP - 1094
JO - Otology and Neurotology
JF - Otology and Neurotology
SN - 1531-7129
IS - 7
ER -