Patricia A. Roush

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).

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.

Effects of Age and Hearing Impairment on the Ability to Benefit from Temporal and Spectral Modulation

Objectives: At poor signal-to-noise ratios, speech understanding may depend on the ability to combine speech fragments that are distributed across time and frequency. The goal of this study was to determine the effects of development and hearing impairment on this ability. Design: Listeners in the present study included adults and children with normal hearing and with hearing impairment. The children with normal hearing included a younger group (aged 4.6–6.9 yr, N = 10) and an older group (aged 7.3–11.1 yr, N = 11). The adults with normal hearing were aged 19 to 27 yr (N = 10). Adults (aged 19–54 yr, N = 9) and children (aged 7.2–10.7 yr, N = 8) with hearing impairment were also tested. The two groups with hearing impairment had comparable mild/moderate bilateral sensorineural hearing impairment. Masked speech reception thresholds for sentences were determined in a baseline condition of steady speech-shaped noise and in noise that was temporally modulated, spectrally modulated, or both temporally and spectrally modulated. Results: The results of normal-hearing listeners indicated higher masked speech reception thresholds for children than adults in steady noise. Adults and children showed the same magnitude of masking release for spectral modulation. Adults showed more masking release than the younger children for temporal modulation and showed more masking release than both the younger and older children for combined temporal/spectral modulation. Comparing normal-hearing and hearing-impaired listeners, the hearing-impaired listeners had higher masked speech reception thresholds in the steady noise condition and reduced masking release in the modulated noise conditions. Neither the two-way interaction between age and hearing impairment nor the three-way interaction between age, hearing impairment, and masking configuration was significant. Conclusions: Although the reduced masking release for temporal modulation shown by the younger children with normal hearing could be a result of poor temporal resolution, it more likely reflects inefficient use of speech cues in temporal gaps or factors stemming from higher signal-to-noise ratios required by children in the baseline condition. The reduced masking release for combined temporal/spectral modulation demonstrated by both the younger and older children with normal hearing may indicate that children in the age range tested here have some difficulty in combining speech information that is distributed across temporal and spectral gaps. Hearing impairment was associated with higher thresholds and reduced masking release in all modulation conditions. Children with hearing impairment showed the poorest performance of any group, consistent with additive effects of hearing loss and development.

Characteristics of hearing aid fittings in infants and young children.

Objectives: Hearing aids (HAs) provide the basis for improving audibility and minimizing developmental delays in children with mild to severe hearing loss. Multiple guidelines exist to recommend methods for optimizing amplification in children, but few previous studies have reported HA fitting outcomes for a large group of children. The present study sought to evaluate the proximity of the fitting to prescriptive targets and aided audibility of speech, as well as survey data from pediatric audiologists who provided HAs for the children in the present study. Deviations from prescriptive target were predicted to have a negative impact on aided audibility. In addition, children who were fitted using verification with probe microphone measurements were expected to have smaller deviations from prescriptive targets and greater audibility than cohorts fitted without these measures. Design: HA fitting data from 195 children with mild to severe hearing losses were analyzed as part of a multicenter study evaluating outcomes in children with hearing loss. Proximity of fitting to prescriptive targets was quantified by calculating the average root-mean-square (RMS) error of the fitting compared with Desired Sensation Level prescriptive targets for 500, 1000, 2000, and 4000 Hz. Aided audibility was quantified using the Speech Intelligibility Index. Survey data from the pediatric audiologists who fit amplification for children in the present study were collected to evaluate trends in fitting practices and relate those patterns to proximity of the fitting to prescriptive targets and aided audibility. Results: More than half (55%) of the children in the study had at least 1 ear that deviated from prescriptive targets by more than 5 dB RMS on average. Deviation from prescriptive target was not predicted by pure-tone average, assessment method, or reliability of assessment. Study location was a significant predictor of proximity to prescriptive target with locations that recruited participants who were fit at multiple clinical locations (University of Iowa and Boys Town National Research Hospital) having larger deviations from target than the location where the participants were recruited primarily from a single, large pediatric audiology clinic (University of North Carolina). Fittings based on average real-ear to coupler differences resulted in larger deviations from prescriptive targets than fittings based on individually measured real-ear to coupler differences. Approximately 26% of the children in the study has aided audibility less than 0.65 on the Speech Intelligibility Index (SII). Aided audibility was significantly predicted by the proximity to prescriptive targets and pure-tone average, but not age in months. Conclusions: Children in the study had a wide range of fitting outcomes in terms of proximity to prescriptive targets (RMS error) and aided speech audibility (SII). Even when recommended HA verification strategies were reported, fittings often exceeded the criteria for both proximity to the prescriptive target and aided audibility. The implications for optimizing amplification for children are also discussed.

Monitoring of Cochlear Function During Cochlear Implantation

Objective: To report the feasibility of monitoring cochlear function during cochlear implantation.

Trends and Predictors of Longitudinal Hearing Aid Use for Children Who Are Hard of Hearing.

Objectives: Children who are hard of hearing (CHH) have restricted access to acoustic and linguistic information. Increased audibility provided by hearing aids (HAs) influences language outcomes, but the advantages of appropriately fit HAs can only be realized if children wear their devices on a consistent basis. The purpose of this article was to characterize long-term HA use in CHH, based on parent-report measures, and identify factors that influence longitudinal trends in HA use. Design: Participants were parents of 290 children with mild to severe hearing loss. At every visit, parents estimated the average amount of time the child used HAs per day during the week and on the weekends. Parent reports of daily HA use were analyzed to determine if different patterns of HA use were observed longitudinally during the study. Independent predictor variables were then related to longitudinal trends in HA use within three age groups (infant, preschool, school age). Results: On average across multiple visits, parents reported that their children wore their HAs for 10.63 hr per day (SD = 3.29). Data logging values were lower than parent-report measures (M = 8.44, SD = 4.06), suggesting that parents overestimated daily HA use. The majority of children in each age group wore HAs at least 8 hr per day from their first research testing interval to their last, based on parent-report measures. Maternal education level predicted longitudinal trends in HA use for infants and school-age CHH. Degree of hearing loss was related to trends in school-age children only. Conclusions: These results indicated that the majority of CHH increased HA use over time, but a sizable minority demonstrated a low level of use or decreased use in the time period studied. Maternal education level influenced longitudinal trends in daily HA use. Degree of hearing loss influenced trends in school-age children only. Audiologists and early intervention service providers might aid in improving HA use by providing regular hands-on training with the HAs and individualized problem-based strategies to address the challenges families experience with attaining a high level of use. Families may also benefit from practical demonstrations of the benefits of consistent HA use, such as hearing loss simulations, examples of listening in noise with and without HAs, or listening to malfunctioning HAs.

Nonlinear frequency compression in hearing aids: impact on speech and language development.

Objectives: The research questions of this study were: (1) Are children using nonlinear frequency compression (NLFC) in their hearing aids getting better access to the speech signal than children using conventional processing schemes? The authors hypothesized that children whose hearing aids provided wider input bandwidth would have more access to the speech signal, as measured by an adaptation of the Speech Intelligibility Index, and (2) are speech and language skills different for children who have been fit with the two different technologies; if so, in what areas? The authors hypothesized that if the children were getting increased access to the speech signal as a result of their NLFC hearing aids (question 1), it would be possible to see improved performance in areas of speech production, morphosyntax, and speech perception compared with the group with conventional processing. Design: Participants included 66 children with hearing loss recruited as part of a larger multisite National Institutes of Health–funded study, Outcomes for Children with Hearing Loss, designed to explore the developmental outcomes of children with mild to severe hearing loss. For the larger study, data on communication, academic and psychosocial skills were gathered in an accelerated longitudinal design, with entry into the study between 6 months and 7 years of age. Subjects in this report consisted of 3-, 4-, and 5-year-old children recruited at the North Carolina test site. All had at least at least 6 months of current hearing aid usage with their NLFC or conventional amplification. Demographic characteristics were compared at the three age levels as well as audibility and speech/language outcomes; speech-perception scores were compared for the 5-year-old groups. Results: Results indicate that the audibility provided did not differ between the technology options. As a result, there was no difference between groups on speech or language outcome measures at 4 or 5 years of age, and no impact on speech perception (measured at 5 years of age). The difference in Comprehensive Assessment of Spoken Language and mean length of utterance scores for the 3-year-old group favoring the group with conventional amplification may be a consequence of confounding factors such as increased incidence of prematurity in the group using NLFC. Conclusions: Children fit with NLFC had similar audibility, as measured by a modified Speech Intelligibility Index, compared with a matched group of children using conventional technology. In turn, there were no differences in their speech and language abilities.