Holly F. B. Teagle

A longitudinal study of electrode impedance, the electrically evoked compound action potential, and behavioral measures in nucleus 24 cochlear implant users.

Objective The primary goal of this study was to examine changes that may occur in electrode impedance, electrically evoked compound action potential (EAP) threshold and slope of the EAP growth function, and behavioral measures of threshold (MAP T-level) and maximum comfort (C-level) over time in both adult and child cochlear implant users. Secondary goals were to determine whether changes in these measures are consistent between children and adults, and to determine whether behavioral measures (MAP T- and C-levels) and electrophysiologic measures (EAP thresholds) exhibit the same trends over time. Design Thirty-five children and 33 adults implanted with the Nucleus CI24M between November 1996 and August 1999 participated in this study. Subjects were included in this study if 1) they had used their implant for at least 1 yr after device connection, and 2) they had participated in the necessary data collection at a minimum number of the time intervals assessed in this study. EAP threshold, slope of the EAP growth function, and common ground electrode impedance measures were collected intraoperatively, at initial stimulation, and at several subsequent visits up to 2 yr post initial stimulation. MAP T- and C-levels were measured at initial stimulation and at the same time intervals as described above. Results Changes in electrode impedance, EAP thresholds, and slope of the EAP growth function from measures made intraoperatively, at initial stimulation, and at 1 to 2 mo post initial stimulation were similar in both children and adults. Beyond the 1- to 2-mo visit, children exhibited significant increases in electrode impedance, EAP thresholds, slope, and MAP T-levels, whereas these same measures in adults remained relatively stable. EAP thresholds in children stabilized by the 3- to 8-mo visit, and electrode impedance stabilized by the 6- to 8-mo visit, while slope of the EAP growth function, MAP T-levels, and MAP C-levels were stable by 1 yr post initial stimulation. C-levels in adults increased up to 1 yr post initial stimulation; however, the amount of increase was much smaller than that seen in children. In both children and adults, longitudinal trends in EAP thresholds mirrored T-level more closely than C-level. Conclusions The results of this study suggest that peripheral changes occur in many children that do not generally occur in adults within the first year of cochlear implant use. One implication of these results is that if EAP thresholds are to be used to assist in programming the speech processor for children, it is best to make those measures at the same time interval as device programming rather than using measures made intraoperatively or at the initial programming session to set MAP levels at later visits.

Cochlear implantation in children with auditory neuropathy spectrum disorder

Objective: To report the patients characteristics, preoperative audiological profiles, surgical outcomes, and postoperative performance for children with auditory neuropathy spectrum disorder (ANSD) who ultimately received cochlear implants (CIs). Design: Prospective, longitudinal study of children with ANSD who received CIs after a stepwise management protocol that included electrophysiologic and medical assessment, documentation of behavioral audiometric thresholds and subsequent fitting of amplification according to Desired Sensation Level targets, auditory-based intervention with careful monitoring of skills development and communication milestones, and finally implantation when progress with the use of acoustic amplification was insufficient. Results: Of 140 children with ANSD, 52 (37%) received CIs in their affected ears (mean duration of use of 41 mos). Many of these children were born prematurely (42%) and impacted by a variety of medical comorbidities. More than one third (38%) had abnormal findings on preoperative magnetic resonance imaging of the brain and inner ear, and 81% had a greater than severe (>70 dB HL) degree of hearing loss before implantation. Although 50% of the implanted children with ANSD demonstrated open-set speech perception abilities after implantation, nearly 30% of them with >6 months of implant experience were unable to participate in this type of testing because of their young age or developmental delays. No child with cochlear nerve deficiency (CND) in their implanted ear achieved open-set speech perception abilities. In a subgroup of children, good open-set speech perception skills were associated with robust responses elicited on electrical-evoked intracochlear compound action potential testing when this assessment was possible. Conclusions: This report shows that children with ANSD who receive CIs are a heterogeneous group with a wide variety of impairments. Although many of these children may ultimately benefit from implantation, some will not, presumably because of a lack of electrical-induced neural synchronization, the detrimental effects of their other associated conditions, or a combination of factors. When preoperative magnetic resonance imaging reveals central nervous system pathology, this portends a poor prognosis for the development of open-set speech perception, particularly when CND is evident. These results also show that electrical-evoked intracochlear compound action potential testing may help identify those children who will develop good open-set speech perception. Instead of recommending CI for all children with electrophysiologic evidence of ANSD, the stepwise management procedure described herein allows for the identification of children who may benefit from amplification, those who are appropriate candidates for cochlear implantation, and those who, because of bilateral CND, may not be appropriate candidates for either intervention.

Auditory neuropathy characteristics in children with cochlear nerve deficiency.

Objective: To describe a group of children exhibiting electrophysiologic responses characteristic of auditory neuropathy (AN) who were subsequently identified as having absent or small cochlear nerves (i.e., cochlear nerve deficiency). Design: A retrospective review of the clinical records, audiological testing results, and magnetic resonance imaging (MRI) studies. Fifty-one of 65 children with AN characteristics on auditory brain stem response (ABR) testing had MRI available for review. Nine (18%) of these 51 children with ABR characteristic of AN have been identified as having small (N = 2; 4%) or absent (N = 7; 14%) cochlear nerves on MRI. Results: Of the nine children with cochlear nerve deficiency, five (56%) were affected unilaterally and four (44%) bilaterally. Eight of nine presented after failing a newborn infant hearing screening, whereas one presented at 3 yr of age. On diagnostic ABR testing, all 9 children (9 of 13 affected ears; 69%) had evidence of a cochlear microphonic (CM) and absent neural responses in at least one ear. In the unilateral cases, AN characteristics were detected in all affected ears. In bilateral cases, at least one of the ears in each child demonstrated the AN phenotype, whereas the contralateral ear had no CM identified. Only one ear with cochlear nerve deficiency had present otoacoustic emissions as measured by distortion-product otoacoustic emissions. In children with appropriate available behavioral testing results, all ears without cochlear nerves were identified as having a profound hearing loss. Only 4 (31%) of the 13 ears with cochlear nerve deficiency had a small internal auditory canal on MRI. Conclusions: Children with cochlear nerve deficiency can present with electrophysiologic evidence of AN. These children frequently refer on newborn screening examinations that use ABR-based testing methods. Similar to other causes of AN, diagnostic ABR testing will show a CM with absent neural responses. Given that 9 (18%) of 51 children with available MRI and electrophysiologic characteristics of AN in our program have been identified as having cochlear nerve deficiency makes this a relatively common diagnosis. These findings suggest that MRI is indicated for all children diagnosed with AN. Moreover, electrophysiologic evidence of unilateral AN in association with a profound hearing loss should make the clinician highly suspicious for this problem. Although children with cochlear nerve deficiency who have a small nerve may benefit from cochlear implantation or amplification, these interventions are obviously contraindicated in children with completely absent cochlear nerves.

Internal auditory canal morphology in children with cochlear nerve deficiency

Objective: To describe the internal auditory canal (IAC) and inner ear morphologic characteristics of children with cochlear nerve (CN) deficiency. Study Design: Retrospective case series. Setting: Tertiary referral center. Patients: Fourteen children with small or absent (deficient) CNs have been identified by means of high-resolution magnetic resonance imaging (MRI). Interventions: MRI of the brain. Clinical evaluation. Main Outcome Measures: Review of medical records, audiological testing results, and imaging studies. Images were evaluated for the structure of the cochlear, vestibular and facial nerves, IACs and inner ears. Audiometric thresholds were evaluated in all subjects. Methods: Fourteen children with small or absent (deficient) CNs have been identified by means of high-resolution MRI. A review of the medical records, audiologic testing results, and imaging studies was undertaken. The images were evaluated for the structure of the cochlear, vestibular and facial nerves, IACs, and inner ears. The audiometric thresholds were evaluated in all subjects. Results: Among the 14 patients, 5 had known syndromes. MRI allowed an exact specification of the nervous structures within all ears with normal-size IACs. Precise characterization of the nerves in ears with small IACs was more difficult, requiring a consideration of both imaging findings and functional parameters. Five children had bilateral deficient CNs, whereas the remaining 9 subjects were affected unilaterally. Thus, 19 ears had CN deficiency (absent CN, 16; small CN, 3). Eleven ears had normal-size IACs and deficient CNs. Of the 9 ears with small IACs, 8 had deficient CNs (absent, 7; small, 1) on the basis of both MRI and functional assessments. Two ears with small IACs had clear morphologic and/or functional evidence for the presence of a CN: one had a small-size CN on MRI, whereas another had a single nerve in a small IAC with present facial and auditory functions. Conclusion: The findings of this study suggest that CN deficiency is not an uncommon cause of congenital hearing loss. The findings that most ears with CN deficiency had normal IAC morphology and that two ears with small IACs had CNs present indicate that IAC morphology is an unreliable surrogate marker of CN integrity. On the basis of these findings, we think that high-resolution MRI, rather than CT imaging, should be performed in all cases of pediatric hearing loss, especially in those cases where profound hearing loss has been documented. For ears with small IACs, the resolution of MRI currently remains limiting. In these cases, the determination of CN status frequently requires a variety of anatomic (CT and MRI) and functional tests (auditory brainstem response, otoacoustic emissions, behavioral audiometry, and physical examination).

Cochlear implantation in children with labyrinthine anomalies and cochlear nerve deficiency: implications for auditory brainstem implantation.

Compare outcomes among children with inner ear malformations and/or cochlear nerve deficiency (CND) who have received a cochlear implant (CI).

Long-term results of cochlear implants in children with residual hearing.

In summary, performance data from 9 children with hearing loss ranging between 60 and 98 dB HL formed 2 distributions based on the 3-frequency PTA: 60 to 82 dB HL and 82 to 98 dB HL. Among the tests administered, the PBK word lists, the lexically hard LNT and MLNT words, and the HINT-C sentences presented in quiet were found to be sensitive measures in differentiating between-group performance. The children in the better hearing group (60 to 82 dB HL) produced higher mean scores on all measures when compared to a group of implanted children. The children in the poorer hearing group (82 to 98 dB HL) yielded either similar or better mean scores than the group of implanted children who used total communication; the children in this group demonstrated lower mean scores on most measures when compared to orally communicating implanted children.

Speech perception by prelingually deaf children after six years of Cochlear implant use: effects of age at implantation.

From the Department of Otolaryngology-Head and Neck Surgery, University of Iowa, Iowa City, Iowa. Supported (in part) by a research grant awarded to the Department of Otolaryngology-Head and Neck Surgery, University of Iowa (No. 2 P50 DC 00242), from the National Institutes of Health/National Institute on Deafness and Other Communication Disorders; grant RR00059 from the General Clinical Research Centers Program, Division of Research Resources, National Institutes of Health; the Lions Clubs International Foundation; and the Iowa Lions Foundation.

Cochlear implants in Waardenburg syndrome.

Objective: Waardenburg syndrome is an autosomal‐dominant syndrome characterized by dystopia canthorum, hyperplasia of the eyebrows, heterochromia irides, a white forelock, and sensorineural hearing loss in 20% to 55% of patients. This patient population accounts for approximately 2% of congenitally deaf children. The purpose of this retrospective case review was to describe the outcomes for those children with Waardenburg syndrome who have undergone cochlear implantation.

Improvements in visual attention in deaf infants and toddlers after cochlear implantation

The aims of this study were to examine the development of visual attention in deaf and hearing infants and toddlers, and assess whether improvements in visual attention were observed in the deaf sample after 12 months of cochlear implantation. A novel puppet task, based on a measure of attention developed with normally hearing infants, was administered to 88 deaf and 42 normal-hearing children at three time points: baseline, six and 12 months post-implantation for the deaf sample. At baseline, deaf children demonstrated significantly more inattentive looks during the puppet skits than hearing children, and these looks were of longer duration, confirming the results of prior studies which have documented deficits in visual attention in deaf children. Longitudinal analyses showed significant decreases in the frequency of inattentive looks for both groups, with a significant decrease in the duration of inattentive looks only for the cochlear implant group. The largest decrease in duration of off-task looks occurred at six months post-implantation, indicating that improvements occurred rapidly after restoration of auditory input. These results provided support for the ‘division of labor’ hypothesis which suggests that deaf children with no access or limited access to sound must monitor their environment visually, making it difficult for them to focus and attend to specific tasks. Cochlear implantation appeared to alter the developmental trajectory of visual attention in a positive manner. The clinical implications of visual attention for the development of early language, reading and social skills are discussed.

Gap detection measured with electrically evoked auditory event-related potentials and speech-perception abilities in children with auditory neuropathy spectrum disorder.

Objectives: This study aimed (1) to investigate the feasibility of recording the electrically evoked auditory event–related potential (eERP), including the onset P1-N1-P2 complex and the electrically evoked auditory change complex (EACC) in response to temporal gaps, in children with auditory neuropathy spectrum disorder (ANSD); and (2) to evaluate the relationship between these measures and speech-perception abilities in these subjects. Design: Fifteen ANSD children who are Cochlear Nucleus device users participated in this study. For each subject, the speech-processor microphone was bypassed and the eERPs were elicited by direct stimulation of one mid-array electrode (electrode 12). The stimulus was a train of biphasic current pulses 800 msec in duration. Two basic stimulation conditions were used to elicit the eERP. In the no-gap condition, the entire pulse train was delivered uninterrupted to electrode 12, and the onset P1-N1-P2 complex was measured relative to the stimulus onset. In the gapped condition, the stimulus consisted of two pulse train bursts, each being 400 msec in duration, presented sequentially on the same electrode and separated by one of five gaps (i.e., 5, 10, 20, 50, and 100 msec). Open-set speech-perception ability of these subjects with ANSD was assessed using the phonetically balanced kindergarten (PBK) word lists presented at 60 dB SPL, using monitored live voice in a sound booth. Results: The eERPs were recorded from all subjects with ANSD who participated in this study. There were no significant differences in test–retest reliability, root mean square amplitude or P1 latency for the onset P1-N1-P2 complex between subjects with good (>70% correct on PBK words) and poorer speech-perception performance. In general, the EACC showed less mature morphological characteristics than the onset P1-N1-P2 response recorded from the same subject. There was a robust correlation between the PBK word scores and the EACC thresholds for gap detection. Subjects with poorer speech-perception performance showed larger EACC thresholds in this study. Conclusions: These results demonstrate the feasibility of recording eERPs from implanted children with ANSD, using direct electrical stimulation. Temporal-processing deficits, as demonstrated by large EACC thresholds for gap detection, might account in part for the poor speech-perception performances observed in a subgroup of implanted subjects with ANSD. This finding suggests that the EACC elicited by changes in temporal continuity (i.e., gap) holds promise as a predictor of speech-perception ability among implanted children with ANSD.