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Objectives: Early hearing detection and intervention (EHDI) is a newborn hearing screening system created to detect infants with hearing loss (HL) and intervene to reduce language and communication impairment. Early hearing detection (EHD) consists of three sequential stages: identification, screening, and diagnostic testing. This study longitudinally reviews each stage of EHD in each state and proposes a framework to improve utilization of EHD data., Design: A retrospective public database review was conducted, accessing publicly available data from the Centers for Disease Control and Prevention. Summary descriptive statistics were utilized to generate a descriptive study of EHDI programs in each U.S. state from 2007 to 2016., Results: Data over 10 years from 50 states as well as Washington, DC were included in this analysis, creating up to 510 data points per analysis. Hundred percent (85 to 105) (median [min to max]) of newborns were identified by and entered EHDI programs. Ninety-eight percent (51 to 100) of identified infants completed screening. Of the infants who screened positive for HL, the proportion that received diagnostic testing was 55% (1 to 100). The overall proportion of infants who failed to complete EHD was 3% (1 to 51). Of the infants who fail to complete EHD 70% (0 to 100) are from missed screenings, 24% (0 to 95) are from missed diagnostic testing, and 0% (0 to 93) are from missed identification. Although there are more infants missed at screening, it was estimated, with limitations, that there is an order of magnitude more infants with HL among those who did not complete diagnostic testing compared with those who did not complete screening., Conclusions: Analysis demonstrates high completion rates at both identification and screening stages, whereas the diagnostic testing stage demonstrates low and highly variable completion rates. The low completion rates at diagnostic testing create a bottleneck in the EHD process and the large variability impedes the comparison of HL outcomes across states. Analysis also demonstrates that among all stages of EHD, whereas the largest number of infants are missed at screening, the largest number of children with HL are likely missed at diagnostic testing. Therefore, a focus by individual EHDI programs on addressing causes of low diagnostic testing completion rates would yield the greatest increase in the identification of children with HL. Potential causes of low diagnostic testing completion rates are further discussed. Finally, a new vocabulary framework is proposed to facilitate further study of EHD outcomes., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: The objectives of this study were to demonstrate the safety of auditory brainstem implant (ABI) surgery and document the subsequent development of auditory and spoken language skills in children without neurofibromatosis type II (NFII)., Design: A prospective, single-subject observational study of ABI in children without NFII was undertaken at the University of North Carolina at Chapel Hill. Five children were enrolled under an investigational device exemption sponsored by the investigators. Over 3 years, patient demographics, medical/surgical findings, complications, device mapping, electrophysiologic measures, audiologic outcomes, and speech and language measures were collected., Results: Five children without NFII have received ABIs to date without permanent medical sequelae, although 2 children required treatment after surgery for temporary complications. All children wear their device daily, and the benefits of sound awareness have developed slowly. Intra-and postoperative electrophysiologic measures augmented surgical placement and device programming. The slow development of audition skills precipitated limited changes in speech production but had little impact on growth in spoken language., Conclusions: ABI surgery is safe in young children without NFII. Benefits from device use develop slowly and include sound awareness and the use of pattern and timing aspects of sound. These skills may augment progress in speech production but progress in language development is dependent upon visual communication. Further monitoring of this cohort is needed to better delineate the benefits of this intervention in this patient population.

Objectives: This preliminary study aimed (1) to assess longitudinal changes in electrically evoked auditory event-related potentials (eERPs) in children with auditory brainstem implants (ABIs) and (2) to explore whether these changes could be accounted for by maturation in the central auditory system of these patients., Design: Study participants included 5 children (S1 to S5) with an ABI in the affected ear. The stimulus was a train of electrical pulses delivered to individual ABI electrodes via a research interface. For each subject, the eERP was repeatedly measured in multiple test sessions scheduled over up to 41 months after initial device activation. Longitudinal changes in eERPs recorded for each ABI electrode were evaluated using intraclass correlation tests for each subject., Results: eERPs recorded in S1 showed notable morphological changes for five ABI electrodes over 41 months. In parallel, signs or symptoms of nonauditory stimulation elicited by these electrodes were observed or reported at 41 months. eERPs could not be observed in S2 after 9 months of ABI use but were recorded at 12 months after initial stimulation. Repeatable eERPs were recorded in S3 in the first 9 months. However, these responses were either absent or showed remarkable morphological changes at 30 months. Longitudinal changes in eERP waveform morphology recorded in S4 and S5 were also observed., Conclusions: eERP responses in children with ABIs could change over a long period of time. Maturation of the central auditory system could not fully account for these observed changes. Children with ABIs need to be closely monitored for potential changes in auditory perception and unfavorable nonauditory sensations. Neuroimaging correlates are needed to better understand the emergence of nonauditory stimulation over time in these children.

Objectives/hypothesis: To determine if prolongation of the interdevice interval in children receiving bilateral cochlear implants adversely affects speech perception outcomes., Study Design: Retrospective chart review., Methods: Retrospective review of our pediatric cochlear implant database was performed. Children who had undergone revision surgery or had less than 12 months listening experience with either the first or second implant were excluded. The interdevice interval, best Phonetically Balanced Kindergarten word lists (PBK) score from each ear, and demographic data about each patient were collected. A ratio of PBK was generated (PBK second side/PBK first side) to minimize potential confounding from other individual patient factors that affect speech outcomes., Results: Two hundred forty children met the study criteria. Mean age at first cochlear implantation (CI) was 3.2 years (0.6-17.9), and the second was 6.6 years (0.8-22.4). Mean best PBK score from the first CI side was 83.8% (0-100), and the second was 67.5% (0-100) (P < .001). When the PBK ratio was plotted against interdevice interval, R(2) was 0.47 (P < .001). When analyzed for hearing stability, those with a progressive loss history demonstrated less influence of prolonged interdevice interval on performance. Multivariate analysis did not identify other factors influencing the ratio. A line of best fit for those with stable hearing loss suggested best outcomes were with an interdevice interval less than 3 to 4 years. Beyond 7 to 8 years, very few achieved useful speech recognition from the second CI., Conclusions: Where possible, the second implant should be received within 3 to 4 years of the first to maximize outcome in those with stable, severe to profound sensorineural hearing loss., Level of Evidence: 4. Laryngoscope, 126:2389-2394, 2016., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: This study explored the feasibility of measuring electrically evoked cortical auditory event-related potentials (eERPs) in children with auditory brainstem implants (ABIs)., Design: Five children with unilateral ABIs ranging in age from 2.8 to 10.2 years (mean: 5.2 years) participated in this study. The stimulus was a 100-msec biphasic pulse train that was delivered to individual electrodes in a monopolar stimulation mode. Electrophysiological recordings of the onset eERP were conducted in all subjects., Results: The onset eERP was recorded in four subjects who demonstrated auditory perception. These eERP responses showed variations in waveform morphology across subjects and stimulating electrode locations. No eERPs were observed in one subject who received no auditory sensation from ABI stimulation., Conclusions: eERPs can be recorded in children with ABIs who develop auditory perception. The morphology of the eERP can vary across subjects and also across stimulating electrode locations within subjects.

Objectives: To compare the results of a "no response" (NR) result on auditory brainstem response (ABR) testing with those of behavioral pure-tone audiometry and ultimate clinical tracking to cochlear implantation (CI)., Design: Retrospective review of pediatric patients who underwent multifrequency ABR testing in a 5 year span. Total of 1143 pediatric patients underwent ABR testing during the study period and 105 (9.2%) were identified with bilateral NR based on absent responses to both click and tone burst stimuli. For the children with NR, various clinical parameters were evaluated as these children progressed through the CI evaluation process. Children were grouped based on whether they underwent ABRs for diagnostic or for confirmatory purposes., Results: Of the 105 children who met inclusion criteria, 94 had sufficient follow-up to be included in this analysis. Ninety-one (96.8%) of 94 children with bilateral NR ABRs were ultimately recommended for and received a CI. Three (3.2%) children were not recommended for implantation based on the presence of multiple comorbidities rather than auditory factors. None of the children (0%) had enough usable residual hearing to preclude CI. For those who had diagnostic ABRs, the average time at ABR testing was 5.4 months (SD 6.2, range 1-36) and the average time from ABR to CI was 10.78 months (SD 5.0, range 3-38)., Conclusions: CI should tentatively be recommended for children with a bilateral NR result with multifrequency ABR, assuming confirmatory results with behavioral audiometric testing. Amplification trials, counseling, and auditory-based intervention therapy should commence but not delay surgical intervention, as it does not appear to change the eventual clinical course. Children not appropriate for this "fast-tracking" to implantation might include those with significant comorbidities, auditory neuropathy spectrum disorder, and unreliable or poorly correlated results on behavioral audiometric testing.

Hypotheses: Electrocochleography (ECoG) to acoustic stimuli can differentiate relative degrees of cochlear responsiveness across the population of cochlear implant recipients. The magnitude of the ongoing portion of the ECoG, which includes both hair cell and neural contributions, will correlate with speech outcomes as measured by results on CNC word score tests., Background: Postoperative speech outcomes with cochlear implants vary from almost no benefit to near normal comprehension. A factor expected to have a high predictive value is the degree of neural survival. However, speech performance with the implant does not correlate with the number and distribution of surviving ganglion cells when measured postmortem. We will investigate whether ECoG can provide an estimate of cochlear function that helps predict postoperative speech outcomes., Methods: An electrode was placed at the round window of the ear about to be implanted during implant surgery. Tone bursts were delivered through an insert earphone. Subjects included children (n = 52, 1-18 yr) and postlingually hearing impaired adults (n = 32). Word scores at 6 months were available from 21 adult subjects., Results: Significant responses to sound were recorded from almost all subjects (80/84 or 95%). The ECoG magnitudes spanned more than 50 dB in both children and adults. The distributions of ECoG magnitudes and frequencies were similar between children and adults. The correlation between the ECoG magnitude and word score accounted for 47% of the variance., Conclusion: ECoGs with high signal-to-noise ratios can be recorded from almost all implant candidates, including both adult and pediatric populations. In postlingual adults, the ECoG magnitude is more predictive of implant outcomes than other nonsurgical variables such as duration of deafness or degree of residual hearing.

Objectives/hypothesis: Although it is clear that early auditory stimulation through cochlear implantation (CI) has been shown to improve speech and language development trajectories for children with prelingual hearing loss, data supporting implantation in postlingual children are mostly lacking. The purpose of this study was to characterize speech perception abilities following CI in a group of children with previously well-developed language abilities., Study Design: Retrospective analysis., Methods: Twenty-eight hearing-impaired children who received CIs were selected for study based on the presence of well-developed spoken language skills before implantation. Fifteen children with prelingual hearing loss served as a control group. Speech perception skills were assessed using developmentally appropriate measures., Results: Children with postlingual hearing loss showed a statistically significant improvement in open-set speech perception scores as early as 6 months following CI, whereas prelingual children demonstrated significant improvements only after 24 months of use. Despite these early disparities in performance, the two groups were similar 36 months after implantation and beyond (60 months of implant use)., Conclusions: Children with well-developed language abilities before CI showed substantial (and statistically significant) early improvements in open-set speech perception abilities following implantation that continued beyond 2 years of follow-up. These results suggest that postlingual children are excellent candidates for CI., (Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.)

Objective: Binaural hearing has been shown to support better speech perception in normal-hearing listeners than can be achieved with monaural stimulus presentation, particularly under noisy listening conditions. The purpose of this study was to evaluate whether bilateral electrical stimulation could confer similar benefits for cochlear implant listeners., Design: A total of 26 postlingually deafened adult patients with short duration of deafness were implanted at five centers and followed up for 1 yr. Subjects received MED-EL COMBI 40+ devices bilaterally; in all but one case, implantation was performed in a single-stage surgery. Speech perception testing included CNC words in quiet and CUNY sentences in noise. Target speech was presented at the midline (0 degrees), and masking noise, when present, was presented at one of three simulated source locations along the azimuth (-90, 0, and +90 degrees)., Results: Benefits of bilateral electrical stimulation were observed under conditions in which the speech and masker were spatially coincident and conditions in which they were spatially separated. Both the "head shadow" and "summation" effects were evident from the outset. Benefits consistent with "binaural squelch" were not reliably observed until 1 yr after implantation., Conclusions: These results support a growing consensus that bilateral implantation provides functional benefits beyond those of unilateral implantation. Longitudinal data suggest that some aspects of binaural processing continue to develop up to 1 yr after implantation. The squelch effect, often reported as absent or rare in previous studies of bilateral cochlear implantation, was present for most subjects at the 1 yr measurement interval.

A 31-year-old male patient presented with progressive mixed hearing loss since birth. A stapedectomy was attempted and was unsuccessful because of perilymph gushing. CT of the temporal bones showed bulbous dilatation of the fundi of the internal auditory canals and absence of the bone plates separating them from the base of the cochleas. This unusual abnormality was found after the attempted stapedectomy and explains the clinical findings. The findings in male patients are fairly typical X-linked congenital deafness.

Cochlear implantation has become widely accepted as an effective means of hearing rehabilitation in severely and profoundly deaf individuals. In the elderly, cochlear implantation involves a number of unique issues that can affect patient outcomes. These factors include age-related changes in the auditory system, prolonged durations of deafness, diminished communication abilities, and coexisting medical and psychosocial problems. In general, the results of cochlear implantation in the elderly have been comparable with those of younger adults. Perioperative attention to medical and surgical details allows for safe insertion and a minimum of postoperative complications. Patients older than 65 have obtained excellent results by both audiologic and quality-of-life measures.