ABSTRACT
Background: An electrophysiological investigation with auditory brainstem response (ABR), round window electrocochleography (RW-ECoG), and electrical-ABR (E-ABR) was performed in children with suspected hearing loss with the purpose of early diagnosis and treatment. The effectiveness of the electrophysiological measures as diagnostic tools was assessed in this study. Methods: In this retrospective case series with chart review, 790 children below 3 years of age with suspected profound hearing loss were tested with impedance audiometry and underwent electrophysiological investigation (ABR, RW-ECoG, and E-ABR). All implanted cases underwent pure-tone audiometry (PTA) of the non-implanted ear at least 5 years after surgery for a long-term assessment of the reliability of the protocol. Results: Two hundred and fourteen children showed bilateral severe-to-profound hearing loss. In 56 children with either ABR thresholds between 70 and 90 dB nHL or no response, RW-ECoG showed thresholds below 70 dB nHL. In the 21 infants with bilateral profound sensorineural hearing loss receiving a unilateral cochlear implant, no statistically significant differences were found in auditory thresholds in the non-implanted ear between electrophysiological measures and PTA at the last follow-up (p > 0.05). Eight implanted children showed residual hearing below 2000 Hz worse than 100 dB nHL and 2 children showed pantonal residual hearing worse than 100 dB nHL (p > 0.05). Conclusion: The audiological evaluation of infants with a comprehensive protocol is highly reliable. RW-ECoG provided a better definition of hearing thresholds, while E-ABR added useful information in cases of auditory nerve deficiency.
ABSTRACT
OBJECTIVE: Cochlear implants are generally considered a contraindication for any procedure requiring electrical stimulation near the implant. We present a case of a patient undergoing intraoperative transcranial electrical motor-evoked potential monitoring with a cochlear implant without adverse outcomes. PATIENT: A 12-year-old girl with a history of VACTERL presented with worsening congenital kyphosis and bilateral severe-to-profound hearing loss. Since age 7 the patient used a cochlear implant in the right ear and hearing aid in the left ear. Physical examination and magnetic resonance imaging in 2016 revealed a left-sided 66-degree thoracolumbar kyphosis at T11 making the patient a candidate for surgical correction. INTERVENTIONS: She underwent a posterior spinal fusion surgery, performed with intraoperative transcranial electrical motor-evoked potential monitoring. Steps were taken to mitigate electrical stimulation of the patient's cochlear implant. MAIN OUTCOME MEASURES: Postoperative impedance of individual channels, audiometry, and neural response testing were compared with preoperative measurements. RESULTS: Significant (>10%) impedance changes were observed postoperatively in channels 1, 2, 4, and 6; however, the net variation across all the channels was low (3%). The patient reported no hearing changes, and no significant changes in hearing threshold were seen in postoperative audiometric testing or neural response testing. CONCLUSION: We present a case of successful posterior spinal fusion with intraoperative neurophysiological monitoring via transcranial electrical stimulation, in a patient with a cochlear implant. With proper precautions, motor-evoked potential monitoring can be safely performed in a patient with a cochlear implant.
