Douglas P. Sladen

Cochlear implantation in children 12 months of age and younger.

Objective To investigate surgical, anesthetic, and device-related complications as well as auditory and speech-language development outcomes associated with cochlear implantation (CI) in children 12 months of age and younger. Study Design Retrospective chart review. Setting Tertiary academic referral center. Patients All children with severe-to-profound sensorineural hearing loss who underwent cochlear implantation at 12 months of age or younger and an audiometric control group implanted between 13 and 24 months of age. Main Outcome Measures Anesthetic and surgical course; major and minor surgical, anesthetic and device-related complications; postoperative disposition; postoperative auditory receptive and expressive language development. Results Twenty-six patients (41 ears) met criteria. The median duration of follow-up was 58 months. No major surgical or anesthetic complications occurred. One patient (4%) experienced device failure, which required revision surgery and implant exchange. Two other patients (8%) had individual electrode anomalies that were treated with map exclusion. At the last recorded follow-up, 73% of patients were performing at or above the level of normal-hearing age-matched peers. Patients that were implanted at 12 months of age or younger reached age-appropriate speech and language skills by 24 months of age compared with 40 months for the older pediatric control group. Conclusion The current study demonstrates that CI provides substantial benefit among infant recipients. Furthermore, when performed by an experienced cochlear implant and pediatric anesthesia team, the surgical and anesthetic risks are similar to that expected with both older pediatric and adult patients.

Hearing Preservation Among Patients Undergoing Cochlear Implantation

Introduction Despite successful preservation of low-frequency hearing in patients undergoing cochlear implantation (CI) with shorter electrode lengths, there is still controversy regarding which electrodes maximize hearing preservation (HP). The thin straight electrode array (TSEA) has been suggested as a full cochlear coverage option for HP. However, very little is known regarding its HP potential. Methods A retrospective review was performed at two tertiary academic medical centers, reviewing the electronic records for 52 patients (mean, 58.2 yr; range, 11–85 yr) implanted with the Cochlear Nucleus CI422 Slim Straight (Centennial, CO, USA) electrode array, referred to herein as the thin straight electrode array or TSEA. All patients had a preoperative low-frequency pure-tone average (LFPTA) of 85 dB HL or less. Hearing thresholds were measured at initial activation (t1) and 6 months after activation (t2). HP was assessed by evaluating functional HP using a cutoff level of 85 dB HL PTA. Results At t1, 54% of the subjects had functional hearing; 33% of these subjects had an LFPTA between 71 and 85 dB HL, and 17% had an LFPTA between 56 and 70 dB HL. At t2, 47% of the patients had functional hearing, with 31% having an LFPTA between 71 and 85 dB HL. Discussion Preliminary research suggests that the TSEA has the potential to preserve functional hearing in 54% of patients at t1. However, 22% (n = 6) of the patients who had functional hearing at t1 (n = 28) lost their hearing between t1 and t2. Further studies are needed to evaluate factors that influence HP with the TSEA electrode and determine the speech perception benefits using electric and acoustic hearing over electric alone.

Older and Younger Adult Cochlear Implant Users: Speech Recognition in Quiet and Noise, Quality of Life, and Music Perception

PURPOSE To determine whether older cochlear implant (CI) listeners differ from younger CI listeners on measures of speech understanding, music perception, and health-related quality of life (HRQoL). In the study, the authors hypothesized that speech recognition would be more difficult for older adults, especially in noisy conditions. Performance on music perception was expected to be lower for older implanted listeners. No differences between age groups were expected on HRQoL. METHOD Twenty older (>60 years) and 20 younger (<60 years) implanted adults participated. Speech understanding was assessed using words and sentences presented in quiet, and sentences presented at +15, +10, and +5 dB signal-to-noise ratio conditions. Music perception was tested using the University of Washington Clinical Assessment of Music, and HRQoL was measured using the Njimegen CI survey. RESULTS Speech understanding was significantly lower for the older compared with the younger group in all conditions. Older implanted adults showed lower performance on music perception compared with younger implanted adults on 1 of 3 subtests. Older adults reported lower HRQoL benefit than younger adults on 3 of 6 subdomains. CONCLUSION Data indicate that older CI listeners performed more poorly than younger CI listeners, although group differences appear to be task specific.

Accuracy of a Tablet Audiometer for Measuring Behavioral Hearing Thresholds in a Clinical Population

Objective To examine the validity of a tablet computer–based audiometer for measuring hearing thresholds in a moderately noisy environment. Study Design A prospective single-cohort repeated measures study. Setting Tertiary care institution providing hearing health care to a diverse population. Subjects and Methods Subjects included 49 participants (44 adults, 5 children) with all degrees of hearing sensitivity. Potential participants were excluded if they were <4 years old or had cognitive deficits or ear drainage. Participants were tested with established audiometric tests and a tablet audiometer. Threshold values were measured for both ears using various stimuli (500, 1000, 2000, and 4000 Hz). Testing with the tablet audiometer was conducted in a non-sound-treated room; room properties were characterized by ambient noise level and reverberation testing. Calibration assessment of the tablet audiometer was performed on 3 occasions. Results Within the test thresholds for hearing level, results from the tablet device were within 10 dB of those determined by conventional audiometry for 164 of 172 hearing levels and did not show proportional bias over the testing range. Calibration assessment showed accurate proximity between output and target values, though crosstalk and linearity failed initial assessments. Conclusion The tablet-based automated audiometer presents a new method for threshold hearing assessment outside conventional sound booths.

Developmental Outcomes in Early School-Age Children with Minimal Hearing Loss

PURPOSE Previous research suggests that school-age children with minimal hearing loss (CMHL) are at risk for a variety of psychoeducational problems. However, CMHL are a heterogeneous group, and the profile of at-risk children is unknown. Data regarding the characteristics of early school-age CMHL are needed to extend previous findings and determine potential risk factors associated with psychoeducational difficulties. METHOD Psychoeducational outcomes were evaluated at baseline and longitudinally in age-matched groups of 27 CMHL (ages 4–10 years) and 26 children with normal hearing (CNH) using assessments of language, reading, behavior, speech recognition in noise, and cognition. Additional analyses were used to identify demographic characteristics among CMHL that are associated with psychoeducational difficulties. RESULTS At the earliest age tested, CMHL had greater teacher-rated attention difficulties in the classroom than CNH. Differences in the rate of psychoeducational development were not observed between groups. Among CMHL, psychoeducational difficulties were associated with delays in identification of hearing loss and low maternal education. CONCLUSIONS Classroom attention abilities should be monitored for early school-age CMHL. Late-identified CMHL and CMHL with low maternal education levels may be in particular need of academic and social support. Continued efforts for early identification of CMHL should be made to improve outcomes for these children.

Cochlear Implantation for Single-Sided Deafness : A Multicenter Study

To report the preliminary outcomes of patients with single‐sided deafness and asymmetric hearing loss undergoing cochlear implantation at two centers.

Early outcomes after cochlear implantation for adults and children with unilateral hearing loss

This study was designed to examine speech recognition and self‐perceived health‐related quality of life (HRQoL) received from cochlear implantation among a cohort of adults and children with a short duration of unilateral hearing loss greater than 6 months, but less than 2 years.

Cochlear implantation in patients with intracochlear and intralabyrinthine schwannomas

Objective: Schwannomas may arise primarily within the inner ear, or invade the cochlea or labyrinth from the distal internal auditory canal through transmodiolar or transmacular extension, respectively. To date, very limited data exists regarding cochlear implant (CI) outcomes in this unique population. Study Design: Retrospective case review. Patients: Ten ears (nine patients) with inner ear schwannomas that underwent CI at a single tertiary referral center. Intervention(s): Cochlear implantation. Main Outcome Measure(s): Surgical approach, CI performance. Results: Ten ears (nine patients) were implanted with conventional CI arrays. Three cases had primary inner ear schwannomas, while seven were in patients with neurofibromatosis type 2 (NF2) having transmodiolar invasion of the inner ear from a vestibular schwannoma (VS). In all cases, intracochlear tumor was left in situ to preserve cochlear anatomy and a full electrode insertion was achieved. Use of a styleted electrode with late deployment aided advancement through the intracochlear tumor. In all cases, the ipsilateral internal auditory canal and inner ear could be visualized on postoperative magnetic resonance imaging (MRI) for tumor surveillance. Eight ears achieved good open-set word recognition (median, Consonant-Nucleus-Consonant [CNC] 50% [range, 28–88%], median, AzBio 73% [range, 60–91%]); two patients with NF2 and prolonged deafness (15 and 22 years) received limited benefit. Conclusions: Cochlear implantation in patients with inner ear schwannomas and an intact cochlear nerve is feasible. Leaving intracochlear schwannoma in situ preserves cochlear architecture and use of a styleted electrode may aid in achieving a full insertion when obstructing tumor is present. Postoperative MRI surveillance is still adequate after CI and a high percentage of patients achieve good open-set speech perception performance.

Survey of the American Neurotology Society on Cochlear Implantation: Part 1, Candidacy Assessment and Expanding Indications

OBJECTIVE To examine practice variance of cochlear implant candidacy assessment and off-label indications across centers in the United States. METHODS Cross-sectional survey of the American Neurotology Society (ANS). RESULTS A total of 81 surveys were returned from ANS members who report regular involvement in cochlear implant care. Overall there was a broad distribution in age and clinical experience, with most respondents reporting ACGME accreditation in neurotology and employment at an academic center. The annual volume of cochlear implant surgeries varied considerably across centers.Seventy-eight percent of respondents performed cochlear implantation for at least one of the following indications within the last 2 years: profound hearing loss in children less than 12 months of age (35, 43%), children with asymmetrical hearing loss where at least one ear was better than performance cutoff for age (25, 31%), adults with asymmetrical hearing where at least one ear was better than the performance cutoff for adult criteria (49, 61%), single-sided deafness (37, 46%), and ipsilateral vestibular schwannoma (28, 35%). Centers with a higher annual implant volume more frequently performed off-label implantation in all queried populations (all, p≤0.001), and performed surgery on infants with congenital deafness at a younger age (p = 0.013), compared with centers with lower surgical volume.When surveyed regarding speech perception testing practices for adult candidacy assessment, 75 (100%) respondents who answered this question reported routine use of AzBio sentences, 42 (56%) CNC word scores, and 26 (35%) HINT testing; only 7 (9%) reported using BKB-SIN testing and 6 (8%) reported using CUNY scores. Fifty-one (68%) reported routine use of speech-in-noise testing to determine adult cochlear implant candidacy, 21 (28%) reported selective use only when patient scores were borderline in quiet, and 3 (4%) reported that their center does not currently use testing in noise for candidacy determination. Nineteen (26%) solely used +10 dB signal-to-noise ratio (SNR), 12 (16%) solely used +5 dB SNR, and 41 (55%) used both +10 and +5 dB SNR. Overall, 19% (N = 14) only perform unilateral implantation in the Medicare population, while 81% (N = 58) consider bilateral implantation. CONCLUSION Significant variation in cochlear implant candidacy assessment and off-label implantation exists across centers and providers in the United States resulting in healthcare inequities. The high percentage of surgeons performing implantations for off-label or nontraditional indications reflects the overly restrictive and dated status of current implant guidelines. With greater adoption of more difficult speech perception testing in noise, careful clinical judgment is needed to maintain a favorable risk-benefit balance for prospective implant candidates.

Evaluation of a revised indication for determining adult cochlear implant candidacy

To evaluate the use of monosyllabic word recognition versus sentence recognition to determine candidacy and long‐term benefit for cochlear implantation.