Faculty Bibliography

OBJECTIVES/HYPOTHESIS:: Medulloblastoma is the most common pediatric malignant tumor of the central nervous system in children. Treatment includes surgical excision, external beam radiation, and multiagent chemotherapy. Otologic sequelae are common and may result from radiation and/or chemotherapy. Profound sensorineural hearing loss (SNHL) is a known complication of neuro-oncologic treatment and may render these patients eligible for cochlear implantation (CI). Issues of CI in this population, including diagnosis, treatment of preoperative middle ear disease, operative and postoperative course, performance data, and long-term tumor surveillance are highlighted and reviewed. STUDY DESIGN:: Retrospective chart review. METHODS:: Three patients treated for pediatric medulloblastoma with surgical resection, postoperative hyperfractioned craniospinal radiotherapy, and multiagent adjuvant chemotherapy who underwent cochlear implantation were identified. Details of neuro-oncologic treatment and associated otologic complications are presented and analyzed. Primary outcome assessment includes treatment of middle ear pathology, perioperative cochlear implant course, and postimplantation performance data. RESULTS:: Each patient required surgical treatment of chronic ear disease 4 to 16 years after chemoradiation. All progressed to profound SNHL and were implanted 8 to 17 years post-neuro-oncologic treatment. There were no intraoperative complications, and full insertion of the cochlear implant electrode array was achieved in each patient. One patient developed postoperative wound dehiscence requiring operative closure. Postimplantation performance data support significant benefit in all patients. CONCLUSIONS:: Patients treated for pediatric medulloblastoma develop otologic sequelae, including profound SNHL, and may require cochlear implantation. Successful management of middle ear and mastoid pathology involves consideration of potential future cochlear implantation. Postoperative performance data supports cochlear implantation in this population. Laryngoscope, 2009

OBJECTIVES/HYPOTHESIS: Widespread universal newborn hearing screening has led to increased identification of infant hearing loss. Supported by improved diagnostic tools allowing more definitive diagnosis of profound sensorineural hearing loss in young children, cochlear implantation in children <12 months of age is now common. Literature supports short-term safety and improved auditory outcomes in these young children, however long-term data is lacking. The study examines issues of long-term safety and efficacy in cochlear implant patients implanted <1 year of age. STUDY DESIGN: Retrospective chart review. METHODS: Fifty children who received cochlear implants before 1 year of age were followed for up to 7 years. Age at implantation ranged from 5 to 11 months with a mean of 9.1 months. Three patients had simultaneous bilateral implantation at 8 to 9 months of age. Medical records were reviewed for complications incurred during length of device usage, including time of complication, management, and resolution. Auditory assessment included both the Infant-Toddler Meaningful Auditory Integration Scale and tests of speech perception. RESULTS: All 50 patients had full insertions of the electrode array. There were a total of eight complications (16%) in seven patients, three major (6%) and five minor (10%), which occurred at or before 10 months postoperatively. There were no perioperative anesthetic complications. CONCLUSIONS: Cochlear implantation in children <12 months of age is safe and efficacious over an extended period of time. Rates and nature of both major and minor complications are comparable to studies in adults and older children and support continued monitoring of these patients over the long-term

OBJECTIVE: Bilateral sequential cochlear implantation outcomes are dependent on many different factors. Newer technology in the second implanted ear may also contribute to outcome. This study examines the effect of cochlear implant technology on speech recognition outcomes in a population of adult patients who have undergone bilateral sequential implantation using different technologies in each ear. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Twenty adults who underwent bilateral sequential cochlear implantation with different technologies and processing strategies in each ear were patients for this study. Control Group A included patients (n = 8) who were simultaneously implanted, and Control Group B (n = 3) were patients who were sequentially implanted with the same technology. INTERVENTION: Bilateral sequential cochlear implantation. OUTCOME MEASURES: The outcome measure was the Consonant-Nucleus-Consonant monosyllabic word test administered in each implanted ear and in the binaural condition before and 1 year after operation. A multivariate analysis was performed to account for factors including duration of deafness, length of device usage, and severity of deafness. RESULTS: There was significant improvement from before to 1 year after the operation in word scores for the individual ears and in the binaural condition for all groups. All patients were consistent users of both devices, and the use of different technology in the second implanted ear did not affect the patients' ability to benefit from bilateral implantation despite the use of different devices and processing strategies. CONCLUSION: Bilateral sequential implantation with newer and/or differing technology in the second implanted ear did not reduce the benefits of bilateral stimulation and should not be considered a deterrent to second-sided implantation

OBJECTIVE: To examine speech perception outcomes as related to a reduction in the number of functional electrodes postimplantation and to determine the effect of electrode reduction on subsequent device failure. STUDY DESIGN: Retrospective review. SETTING: Tertiary academic referral center. PATIENTS: Of 1,520 children and adults with full insertions of the Advanced Bionics, Med El, and Nucleus devices, 15 (1%) were patients. Patients were included in the study if all electrodes were functional at initial stimulation, but the number of electrodes in use was subsequently reduced at follow-up programming sessions. Exclusion criteria included partial and split-array electrode insertions. INTERVENTION(S): Patients with bilateral severe to profound sensorineural hearing loss underwent either unilateral or bilateral cochlear implantation. MAIN OUTCOME MEASURE(S): Postimplantation speech perception tests obtained with a full complement of functional electrodes were performed and the results compared to those obtained with 1 or more electrodes removed from the user program. Electrode deactivation was also correlated with device failure. RESULTS AND CONCLUSION: The results of this study indicate that deactivation of cochlear implant electrodes is relatively uncommon, and although the deactivation does not have a direct influence on speech performance outcomes, the loss of 5 or more electrodes can suggest impending device failure. Additionally, those patients with electrode deactivation coupled with a decline in speech perception scores should also be considered at risk for device failure

OBJECTIVES: The purpose of this study is to determine if the performance of the first implanted member of a family predicts the performance of subsequently implanted family members. STUDY DESIGN: Retrospective chart review. METHODS: Seventy-one cochlear implant recipients, each belonging to a family with two or more implanted members, were the subjects of this study. Routine audiometric measurements and age-appropriate speech perception tests were performed pre- and postoperatively. In addition, length of deafness, age at implantation, etiology, and length of device usage were correlated to outcome. RESULTS: All implant recipients within a family showed improvement postimplantation, and the predictive component between family members was strong. Presence of GJB2 mutations and greater age at implantation were predictive of poorer rehabilitative outcome, while length of device usage was associated with improved speech perception scores. CONCLUSIONS: On average, if the first implanted family member performs well with a cochlear implant, those following will do well; however, GJB2-related deafness and increased age at implantation are associated with poorer outcome. These findings have important implications for members of families considering cochlear implantation

This study investigates the effect of the Nucleus CI24RE implant's neural response telemetry (NRT) system, which has less internal noise compared to its predecessor, the CI24M/R implant, on the NRT threshold (TNRT) profile across the array. CI24M/R measurements were simulated by ignoring CI24RE measurements with response amplitudes below 50 uV. Comparisons of the estimated TNRTs from the CI24RE measurements and the CI24M/R simulations suggest that, apart from a constant level difference, the TNRT profiles from the newer implant generally would not have differed very much from those of its predecessor. This view was also reflected by principal component analysis (PCA) results which revealed a 'shift' component similar to that reported by Smoorenburg et al (2002). On the whole, there is no indication that current practices of using the TNRT profiles for assisting with speech processor programming need to be revised for the CI24RE implant

OBJECTIVE: To review our experience with partial standard and double-array cochlear implantation in the ossified cochlea. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Twenty cochleae in 19 adult and pediatric patients with ossified cochleae. INTERVENTION(S): Patients underwent either partial insertion of a standard electrode array or double-array electrode insertion for their cochlear implantation. MAIN OUTCOME MEASURE(S): Number of electrodes inserted, number of active electrodes at follow-up, and open-set speech performance. RESULTS: Twelve patients (four children and eight adults) had partial insertion of standard array electrode. The mean number of electrodes inserted and active at follow-up was 14.8 and 11.3, respectively. Eight patients (four children and four adults) had a double-array electrode implanted. The mean number of electrodes inserted and active at follow-up was 18.1 and 16.3, respectively. Open-set speech recognition was better in children regardless of duration of deafness. There were no complications. CONCLUSION: These findings suggest that the double array allows for more usable electrodes than in partially inserted cochlear implant. Compared with adults, children with ossified cochlea do well both in partial standard and double-array insertion. Both insertion techniques are safe, with no complications occurring in our patients. Straight and double arrays should be available in the operating suite for any patient in whom suspected cochlear ossification is confronted

OBJECTIVE: To describe a safe and effective technique for the implantation of common cavity cochleae. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Fifteen cochleae in 9 adults and children with common cavity deformities implanted with and without the use of intraoperative fluoroscopy. INTERVENTION(S):: Fluoroscopically assisted cochlear implantation. MAIN OUTCOME MEASURE(S): Rates of extracochlear placement, bending, kinking, intrameatal placement, and complications were analyzed. Numbers of electrodes inserted, number of active electrodes, and speech performance were compared between the 2 groups. RESULTS: Half of the nonfluoroscopy group had suboptimal placement of their electrode arrays. This group also had a higher rate of complications, lower numbers of electrodes implanted, and lower number of active electrodes at follow-up. Results of speech perception testing suggest that fluoroscopically assisted implantation of the common cavity results in improved performance. CONCLUSION: Intraoperative fluoroscopy is an easy, safe, and effective technique that results in improved outcomes for cochlear implant patients with common cavity deformities

Objective: To examine speech perception outcomes and determine the impact of length of deafness and time between implants on performance in the sequentially bilateral implanted population. STUDY DESIGN: Retrospective review. SETTING: Tertiary academic referral center. PATIENTS: Forty-three children (age, <18 yr) and 22 adults underwent sequential bilateral implantation with at least 6 months between surgeries. The mean age at the time of the second implant in children was 7.83 years, and mean time between implants was 5.16 years. Five children received the first side implant (C1) below 12 months of age; 16, at 12 to 23 months; 9, between the ages of 24 and 35 months; and 11, at 36 to 59 months; 2 were implanted above the age of 5 years. In adults, mean age at second implant was 46.6 years, and mean time between implants was 5.6 years. INTERVENTION: Sequential implantation with 6 months or more between implantations. MAIN OUTCOME MEASURES: Speech perception tests were performed preoperatively before the second implantation and at 3 months postoperatively. RESULTS: Results revealed significant improvement in the second implanted ear and in the bilateral condition, despite time between implantations or length of deafness; however, age of first-side implantation was a contributing factor to second ear outcome in the pediatric population. CONCLUSION: Sequential bilateral implantation leads to significantly better speech understanding. On average, patients improved, despite length of deafness, time between implants, or age at implantation