Gail S. Murray

Lack of Financial Barriers to Pediatric Cochlear Implantation: Impact of Socioeconomic Status on Access and Outcomes

OBJECTIVES (1) To analyze if socioeconomic status influences access to cochlear implantation in an environment with adequate Medicaid reimbursement. (2) To determine the impact of socioeconomic status on outcomes after unilateral cochlear implantation. DESIGN Retrospective cohort study. SETTING University Hospitals Case Medical Center and Rainbow Babies and Childrens Hospital (tertiary referral center), Cleveland, Ohio. PARTICIPANTS Pediatric patients (age range, newborn to 18 years) who received unilateral cochlear implantation during the period 1996 to 2008. MAIN OUTCOME MEASURES Access to cochlear implantation after referral to a cochlear implant center, postoperative complications, compliance with follow-up appointments, and access to sequential bilateral cochlear implantation. RESULTS A total of 133 pediatric patients were included in this study; 64 were Medicaid-insured patients and 69 were privately insured patients. There was no statistical difference in the odds of initial cochlear implantation, age at referral, or age at implantation between the 2 groups. The odds of prelingual Medicaid-insured patients receiving sequential bilateral cochlear implantation was less than half that of the privately insured group (odds ratio [OR], 0.43; P = .03). The odds of complications in Medicaid-insured children were almost 5-fold greater than the odds for privately insured children (OR, 4.6; P = .03). There were 10 complications in 51 Medicaid-insured patients (19.6%) as opposed to 3 in 61 privately insured patients (4.9%). Medicaid-insured patients missed substantially more follow-up appointments overall (35% vs 23%) and more consecutive visits (1.9 vs 1.1) compared with privately insured patients. CONCLUSIONS In an environment with adequate Medicaid reimbursement, eligible children have equal access to cochlear implantation, regardless of socioeconomic background. However, lower socioeconomic background is associated with higher rates of postoperative complications, worse follow-up compliance, and lower rates of sequential bilateral implantation, observed herein in Medicaid-insured patients. These findings present opportunities for cochlear implant centers to create programs to address such downstream disparities.

BIOELECTRONIC MICROPHONE OPTIONS FOR A TOTALLY IMPLANTABLE HEARING DEVICE FOR PARTIAL AND TOTAL HEARING LOSS

For the past 2 to 3 decades, several institutions in the United States, Europe, and Japan have carried out experimental and clinical research in implantable hearing devices. Currently devices have been developed that are safe and functional. Longterm efficiency has yet to be proven in patients implanted suffering from partial sensorineural hearing 10~s.~ Indeed, as yet, not even the best design and engineering practices to be applied in the construction and testing of these devices have been determined. Two approaches have been tested, electromagnetic and piezoelectric: each has advantages and disadvantages. In investigative studies and for clinical applications approved by the United States Food and Drug Administration (FDA), most of the devices implanted in humans are electromagnetic. The piezoelectric device developed in Japan has been implanted in about 90 patients in Japan; it has been granted approval in Japan as an investigative device to be implanted only in university medical centers. For the past 2 or 3 decades, no new surgical procedures have been added to the armentarium of otologic surgeons. The development of totally implantable hearing devices has been eagerly awaited. Progress has been made in Germany with the European community approval of the totally implantable communication assistance device (TICA) by implex in Munich?* Such devices must be safe and efficient enough to surpass the performance of acoustic hearing aids, and any complications arising from their implantation

Cochlear implantation outcomes in patients with autoimmune and immune-mediated inner ear disease.

Objective: To compare hearing outcomes in patients with autoimmune inner ear disease (AIED) undergoing cochlear implantation to a group of controls, postlingually deafened by non-immune-mediated causes. Hypothesis: Hearing performance in AIED patients who receive unilateral or bilateral cochlear implants is comparable to similarly treated postlingually deafened adults without AIED. Study Design: Retrospective chart review. Setting: Academic neurotologic tertiary referral center. Patients: Ten patients with AIED with 12 implanted ears who met the audiological criteria for cochlear implantation were compared with 12 randomly selected controls, postlingually deafened by non-immune-mediated causes. Intervention: Cochlear implantation using commercially available devices. Main Outcome Measures: Preoperative and postoperative hearing thresholds, words, and sentence scores. A note was made regarding the presence or absence of ossification or fibrosis noted within the scala tympani at the time of implant. Results: The mean age was 49.6 ± 14 years in the AIED group and 56.8 ± 17 years in the control group (p = 0.31). The mean duration of deafness was 14 ± 26 months in the AIED group and 6.5 ± 4 months in the control group (p = 0.34). In the AIED group, 42% were men and 58% were women. In the control group, 33% were men and 67% women. Five patients in the AIED group (6 implanted ears) were found to have cochlear fibrosis and variable degrees of ossification. Two patients in that group required drill-out procedures. All patients had full insertion. The mean preoperative pure-tone averages in the AIED and control groups were 102 ± 18 and 90 ± 13 dB, respectively (p = 0.13). In the AIED, the mean short-term (≤12 mo of follow-up) postoperative word and sentence scores were 74.8% ± 15% and 94% ± 6%, respectively. In the control group, the mean short-term postoperative words and sentence scores were 72% ± 12% and 96% ± 4%, respectively. No statistical difference was present in the short-term postimplantation words (p = 0.7) and sentence scores (p = 0.49) between both groups. The mean long-term (after 12 mo of follow-up) postoperative word and sentence scores in the AIED group were 87.2% ± 11% and 96.8% ± 4%, respectively. In the control group, the long-term words and sentence scores were 77.2% ± 14% and 77.2% ± 7%, respectively. No statistical significance was found in the long-term postimplantation words (p = 0.17) and sentence scores (p = 0.7) between both groups. Conclusion: Cochlear implantation is a safe and viable option for hearing rehabilitation in patients deafened by progressive AIED. Hearing outcomes in AIED patients are excellent and support transition to implantation when hearing is lost or long-term steroid therapy becomes undesirable. Cochlear fibrosis or ossification seems to affect nearly 50% of implanted ears (41.6% of patients) in this study and implies that the cochlea is at risk for ossification changes long term. In appropriate candidates, earlier implantation may be indicated before postinflammatory obliterative changes in the cochlea.

Cochlear implantation outcomes in patients with far advanced otosclerosis.

OBJECTIVES To compare hearing outcomes in patients with far advanced otosclerosis (FAO) undergoing cochlear implantation to an age-matched group of controls, to describe the effects of cochlear ossification on hearing, and to review the adverse effects of implantation in patients with FAO. HYPOTHESIS Hearing performance in patients with FAO after cochlear implantation is comparable to similarly treated postlingually deafened adults without FAO. Ossification or retrofenestral otosclerosis does not predict poor hearing outcomes. Modiolar-hugging technology reduces postoperative facial nerve stimulation. STUDY DESIGN Retrospective chart review. SETTING Academic neurotologic tertiary referral center. PATIENTS Thirty patients with FAO, who metaudiological criteria for cochlear implantation, were compared to 30 age-matched controls, postlingually deafened by non-otosclerotic causes. MAIN OUTCOME MEASURES Audiometric pre- and postoperative speech reception threshold, word, and sentence scores were analyzed. The presence of retrofenestral findings on computed tomography or intraoperative cochlear ossification were noted. RESULTS In the FAO group, radiographic abnormalities were noted in 26.4% of patients. Intraoperative ossification requiring drillout was seen in 29.4% of patients. None developed postoperative facial nerve stimulation. There was no difference between the FAO and control groups in the mean short-term and long-term postoperative speech reception threshold, word, and sentence scores (P = .77). The presence of radiographic abnormalities did not predict hearing outcome. Intraoperative cochlear ossification was not associated with worse short-term word and sentence scores (P = .58 and 0.79, respectively), and for the long-term hearing outcome (P = .24). CONCLUSIONS In patients with FAO, effective and safe hearing rehabilitation can be accomplished with cochlear implantation.

Hearing and neurologic impairment: insult timing indicated by primary tooth enamel defects.

The association between hearing loss, neurologic impairment, and primary tooth enamel defects was examined in a group of 88 children presenting for hearing evaluation at a tertiary care childrens hospital. Forty-one had classifiable enamel defects, reflecting the time and duration of prenatal or perinatal insult. Hearing loss was more prevalent and severe in 19 children with enamel defects of the incisal tooth edge (mean = 61 dB) reflecting a systemic insult at 14 to 16 weeks gestation, than in 7 children with cervical third tooth defects (mean = 23 dB) reflecting insult near term (X2 = 4.08; p less than 0.05). Audiometric findings among the 15 children with incisor defects of the middle third varied; 7 had significant hearing loss and 8 were normal. A correlation was observed between severity of hearing loss (in dB) and estimated time of systemic insult (in weeks gestational age) determined by tooth defect site (r = -0.48; p less than 0.01). The neurologic data revealed similar trends. The group with early systemic insults more frequently had moderate or severe neurologic deficits known to originate early in fetal development. The two groups with third trimester or term insults tended to have mild or no neurologic impairment. A differential susceptibility for developing auditory and neurologic structures based on insult timing is supported.

The Prevalence of Congenital Hearing Loss in Neonates with Down Syndrome

OBJECTIVE To determine the prevalence of hearing loss in newborns with Down syndrome. STUDY DESIGN We performed a cross-sectional, retrospective chart review of all infants with Down syndrome born at a university-affiliated hospital (n = 77) or transferred in to the associated pediatric hospital (n = 32) following birth at an outlying hospital between 1995 and 2010. We determined the rate of failure of newborn hearing screens, the proportion of infants lost to follow-up, and the rate of confirmed hearing loss, as well as the associations of risk factors for hearing loss with confirmed hearing loss. RESULTS Of the 109 patients with hearing screening data, 28 failed their newborn hearing screen. Twenty-seven infants were referred for audiologic evaluation, and 19 completed the evaluation. Fifteen of these 19 infants (79%) had confirmed hearing loss. The prevalence of congenital hearing loss in this sample of neonates with Down syndrome was 15%. Exposure to mechanical ventilation was the sole known risk factor associated with hearing loss. In this study, the loss to follow-up rate for infants with positive hearing screens was 32%. CONCLUSION Newborns with Down syndrome have a higher prevalence of congenital hearing loss compared with the total neonatal population (15% vs 0.25%). Continued monitoring of hearing is needed in children with Down syndrome.

Deep Brain Stimulation of Heschl Gyrus: Implantation Technique, Intraoperative Localization, and Effects of Stimulation.

BACKGROUND Tinnitus is a source of considerable morbidity, and neuromodulation has been shown to be a potential treatment option. However, the location of the primary auditory cortex within Heschl gyrus in the temporal operculum presents challenges for targeting and electrode implantation. OBJECTIVE To determine whether anatomic targeting with intraoperative verification using evoked potentials can be used to implant electrodes directly into the Heschl gyrus (HG). METHODS Nine patients undergoing stereo-electroencephalogram evaluation for epilepsy were enrolled. HG was directly targeted on volumetric magnetic resonance imaging, and framed stereotaxy was used to implant an electrode parallel to the axis of the gyrus by using an oblique anterolateral-posteromedial trajectory. Intraoperative evoked potentials from auditory stimuli were recorded from multiple electrode contacts. Postoperatively, stimulation of each electrode was performed and participants were asked to describe the percept. Audiometric analysis was performed for 2 participants during subthreshold stimulation. RESULTS Sounds presented to the contralateral and ipsilateral ears produced evoked potentials in HG electrodes in all participants intraoperatively. Stimulation produced a reproducible sensation of sound in all participants with perceived volume proportional to amplitude. Four participants reported distinct sounds when different electrodes were stimulated, with more medial contacts producing tones perceived as higher in pitch. Stimulation was not associated with adverse audiometric effects. There were no complications of electrode implantation. CONCLUSION Direct anatomic targeting with physiological verification can be used to implant electrodes directly into primary auditory cortex. If deep brain stimulation proves effective for intractable tinnitus, this technique may be useful to assist with electrode implantation. ABBREVIATIONS DBS, deep brain stimulatorEEG, electroencephalographyHG, Heschl gyrus.

Surgical duration of cochlear implantation in an academic university-based practice

OBJECTIVE Establish the time to safely and efficiently perform cochlear implantation (CI) in a university-based academic center. STUDY DESIGN Case series with chart review. SETTING Academic neurotologic referral center. PATIENTS 424 patients who underwent CI surgery between 2002 and 2010. INTERVENTION Unilateral, bilateral or revision CI using commercially available devices approved for use in the United States. MAIN OUTCOME MEASURES mean surgical duration (SD) and mean total operative room time (TORT). RESULTS Overall mean SD for all 424 patients was 83 ± 30 min (min) whereas the mean TORT was 135 ± 56 min. The mean SD for unilateral CI was 84 ± 18 min for the first implant and 82 ± 22 min for the second implant (p=0.55). The SD for primary and revision CI was 83 ± 18 min and 85 ± 36 min, respectively (p=0.51). The mean SD for pediatric and adult CI was 83 ± 21 min and 83 ± 18 min, respectively (p=0.92). The mean SD without resident assistance was 74 ± 14 min whereas with the assistance of a resident the mean SD was 84 ± 20 min (p=0.02). When ossification was encountered the mean SD was 90 ± 32 min compared to 82 ± 19 min when absent (p<0.001). An association was found between TORT or SD, and the year of surgery, presence of ossification and the involvement of an assistant. CONCLUSION In a university-based academic center, CI surgery can be safely and efficiently performed, supporting future cost-effectiveness analysis of its current practice.

Hearing deficits correlated with the timing of systemic disturbance as indicated by primary incisor defects

A correlation of hearing deficits and enamel defects was investigated in 18 children presenting to a pediatric neurology service for hearing evaluation. Eleven had enamel defects. Five had defects consistent with a systemic insult occurring as early as 14 weeks gestation, two between 29 to 33 weeks gestation, and four near term. Hearing loss was more severe-in the five with enamel defects occurring in the mid-trimester (&OV0398; = 70 dB) than in the four subjects with defects occurring around term (&OV0398; = 23 dB) (t = 3.8; p <0.01). Of the remaining two subjects, one had normal hearing and the other had a moderate loss. A correlation was found between the average degree of hearing loss (in dB) versus the estimated time of systemic insult in weeks gestational age as indicated by position of the tooth defect (r = −0.78; p <0.01). Neurological profiles also differed with those having early defects being more severe. These findings suggest a differential susceptibility for developing audiological (and possibly other neurological) structures based on insult timing.

Bilateral Sequential cochlear implantation in patients with enlarged vestibular aqueduct (EVA) syndrome

Objectives: To analyze audiometric outcomes after bilateral cochlear implantation in patients with isolated enlarged vestibular aqueduct (EVA) syndrome and associated incomplete partition (IP) malformations. Secondary objective was to analyze rate of cerebrospinal fluid (CSF) gusher in patients with IP-EVA spectrum deformities and compare this with the existing literature. Study Design: Retrospective chart review. Methods: Thirty-two patients with EVA syndrome who received unilateral or bilateral cochlear implants between June 1999 and January 2014 were identified in the University Hospitals Case Medical Center cochlear implant database. Isolated EVA (IEVA) and Incomplete Partition Type II (IP-II) malformations were identified by reviewing high-resolution computed tomography (HRCT) imaging. Demographic information, age at implantation, surgical details, postimplantation audiometric data including speech reception thresholds (SRT), word, and sentence scores were reviewed and analyzed. Intra- and postoperative complications were analyzed as well and compared with the literature. Results: Seventeen patients (32 implanted ears) had pediatric cochlear implantation for EVA-associated hearing loss. Data from 16 controls (32 implanted ears) were used to compare audiometric and speech outcomes of EVA cohort. Mean age at implantation was 6.8 years for EVA cohort and 6.0 years for controls. There was no statistically significant difference in long-term postoperative SRT, monaurally aided word scores, and binaurally tested word scores between pediatric EVA group and controls. The EVA patients had a long-term mean sentence score of 85.92%. A subset of EVA patients implanted at mean age of 3.18 years (n = 15 ears) had similar audiometric outcomes to another control group with Connexin 26 mutations (n = 20 ears) implanted at a similar age. Further subset analysis revealed no significant differences in age at implantation, SRT, and word scores in patients with IEVA and IP-II malformation. There was no significant association between size of vestibular aqueduct and age at implantation. There was no CSF gusher or other intra- or postoperative complications reported in our series. Conclusion: Bilateral sequential cochlear implantation can be performed safely in patients with EVA. Audiometric outcomes are excellent and comparable to pediatric cochlear implant patients with no malformations. CSF gusher rates can be minimized by trans-round window approach. Further long-term studies are needed to identify differences within IP-EVA spectrum deformities, audiometric outcomes, and proportions of EVA patients who will need cochlear implantation for hearing rehabilitation.