Sex-specific enlarged vestibular aqueduct morphology and audiometry.

OBJECTIVE: Enlargement of the vestibular aqueduct (EVA) is one of the most common congenital malformations in pediatric patients presenting with sensorineural or mixed hearing loss. The relationship between vestibular aqueduct (VA) morphology and hearing loss across sex is not well characterized. This study assesses VA morphology and frequency-specific hearing thresholds with sex as the primary predictor of interest. MATERIALS AND METHODS: A retrospective, longitudinal, and repeated-measures study was used. 47 patients at an academic tertiary care center with hearing loss and a record of CT scan of the internal auditory canal were candidates, and included upon meeting EVA criteria after confirmatory measurements of vestibular aqueduct midpoint and operculum widths. Audiometric measures included pure-tone average and frequency-specific thresholds. RESULTS: Of the 47 patients (23 female and 24 male), 79 total ears were affected by EVA; the median age at diagnosis was 6.60 years. After comparing morphological measurements between sexes, ears from female patients were observed to have a greater average operculum width (3.25 vs. 2.70 mm for males, p = 0.006) and a greater average VA midpoint width (2.80 vs. 1.90 mm for males, p = 0.004). After adjusting for morphology, male patients' ears had pure-tone average thresholds 17.6 dB greater than female patients' ears (95% CI, 3.8 to 31.3 dB). CONCLUSIONS: Though females seem to have greater enlargement of the vestibular aqueduct, this difference does not extend to hearing loss. Therefore, our results indicate that criteria for EVA diagnoses may benefit from re-evaluation. Further exploration into morphological and audiometric discrepancies across sex may help inform both clinician and patient expectations.

Predictive factors and outcomes of cochlear implantation in patients with connexin 26 mutation: a comparative study.

PURPOSE: To compare hearing outcomes in patients with connexin 26 (Cx 26) mutations undergoing cochlear implantation to age matched controls and to examine whether age at implantation, gender and type of mutation were correlated with hearing outcome. MATERIALS AND METHODS: Retrospective chart review of 21 patients with Cx 26 mutations that underwent cochlear implantation compared to 18 age-matched controls. Patients' characteristics, type of mutation and pre- and postoperative short and long-term hearing thresholds, word and sentence scores were analyzed. RESULTS: There was no statistically significant difference between the Cx 26 and control group in the mean short term and mean long term post-operative pure tone averages (PTA), speech reception thresholds (SRT), word and sentence scores. Gender, age at implantation and type of connexin 26 mutation did not predict hearing outcomes. CONCLUSIONS: In patients with connexin 26 mutation, cochlear implantation provides an effective mean of auditory habilitation. Mutational status, age and gender do not seem to predict hearing outcomes.

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.

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 loss patterns in enlarged vestibular aqueduct syndrome: Do fluctuations have clinical significance?

OBJECTIVES: To reveal hearing loss patterns in patients with enlarged vestibular aqueduct (EVA) syndrome according to demographic and clinical characteristics. METHODS: A retrospective, longitudinal study design was utilized to identify patients with EVA. Ears of patients were categorized into one of four cohorts: progressive fluctuating, progressive non-fluctuating, stable fluctuating, and stable non-fluctuating patterns. Pairwise and group comparisons were performed with non-parametric tests to assess vestibular aqueduct (VA) morphology, clinical, and demographic variables between hearing loss pattern cohorts. Rates of hearing loss in the subgroups were determined utilizing a mixed linear effects model. RESULTS: 44 patients (25 female, 19 male, median diagnosis age: 8.06 years) met inclusion criteria. 16 individuals demonstrated unilateral EVA and 29 had bilateral EVA, resulting in 74 total ears with EVA. Amongst the four cohorts, differences in operculum widths amongst groups were statistically significant (p = 0.049) while VA midpoint widths were not (p = 0.522). Progressive hearing loss ears without fluctuations demonstrated a 3.20 dB per year (p < 0.001) progression while progressive hearing loss ears with fluctuations reported a rate of 3.52 dB loss per year (p < 0.001). CONCLUSION: Hearing fluctuations occur similarly in EVA patients with stable and progressive hearing loss. With the exception of increased rates of hearing loss progression for fluctuating progressive hearing loss patients, vestibular aqueduct morphology, demographic, and clinical characteristics commonly reported are likely not strong predictors for whether patients will or will not experience fluctuating patterns of hearing loss.

Cochlear Implantation in Patients With Menière's Disease: Does Disease Activity Affect the Outcome?

OBJECTIVE: Menière's disease (MD) is characterized by episodes of vertigo, tinnitus, and sensorineural hearing loss. In the setting of bilateral deafness due to MD alone or contralateral pathology, cochlear implantation (CI) improves hearing. Active MD is characterized by fluctuating auditory symptoms and vertigo; whereas remittance of vertiginous symptoms and severe, permanent sensorineural hearing loss characterizes the inactive disease state. This study evaluates outcomes for MD patients compared with the general CI population and assesses if disease activity affects implant outcomes. STUDY DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: Twenty-four patients with MD that received CI (7 active, 16 inactive, and 1 Probable Menière's), and 24 age-matched controls. INTERVENTIONS: Cochlear implantation. MAIN OUTCOME MEASURES: Word Recognition Score, Sentence Recognition Score (SRS), and Speech Reception Threshold. RESULTS: Best-aided preoperative and postoperative audiometric data were compared per ear between MD patients and controls and stratified by disease status using descriptive statistics with mixed-effects modeling. Patients with MD derived significantly more benefit from CI than controls when comparing differences between preoperative and postoperative levels for Word Recognition Score (12.2%, p = 0.0236), SRS (12.8%, p = 0.0375), and Speech Reception Threshold (-14.4 dB, p = 0.0188). Active disease status does not negatively impact CI outcomes and patients with active MD may benefit from greater gains in SRS (23.5%, p = 0.0107). CONCLUSIONS: CI provides greater gains in functional hearing for patients with MD compared with age-matched controls. Patients with active MD seem to perform better with respect to SRS following CI than patients with inactive status.

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.

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.

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 Children's 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.