Cortical Auditory Evoked Potentials Recorded Directly Through the Cochlear Implant in Cochlear Implant Recipients: a Feasibility Study.

OBJECTIVES: The use of objective measures in cochlear implant (CI) mapping, has greatly contributed to the refinement of the setting of audible and comfortable stimulation levels, which serve as the basis of the mapping process, especially in cases of infants and young children. In addition, objective measures can also confirm the integrity of the CI system. Current CI objective measures mainly reflect neural activity from the auditory nerve and brainstem site. An objective cortical CI measure that reflects directly central auditory activity is greatly needed, especially since it is closely related to CI outcomes in both children and adults. Recording the brain activity currently requires an external evoked potential (EP) system including scalp electrodes, rendering it impractical for widespread clinical use. This study aimed to assess the feasibility of recording cortical auditory evoked potentials (CAEPs) directly and solely through the cochlear implant in response to external acoustic stimulation in the non-implanted ear. DESIGN: A total of nine CI users (four females and five males) participated, including seven post-lingual adults (23 to 72 years), and two pediatric cases, one teenager (15 years), and one child (8 years)-both pre-lingual. All participants had a residual hearing in the ear contralateral to the ear with CI. CAEPs were recorded in the implanted ear in response to acoustic stimulation of the non-implanted ear, consisting of a brief tonal stimulus at comfortable listening levels. Recordings used an intracranial montage consisting of an intracochlear apical electrode (active) and one of the two ( case and ring ) extra-cochlear implanted electrodes serving as reference electrodes. The CI CAEPs were compared with a single-channel conventional CAEP recording obtained simultaneously via scalp electrodes (Fz-mastoid) using a standard EP system and an external trigger from the CI system. Statistical comparisons were made between the CI and the scalp recorded CAEPs and for differences between the CI CAEP measures acquired using the ring and the case as the reference electrode. RESULTS: CAEPs recorded directly and solely through the CI were equivalent to the standard scalp recorded CAEP responses. CAEP responses acquired using the case electrode as the reference were highly correlated in terms of morphology, latencies, and amplitudes of the CAEP components. The CI CAEP latencies of the two pediatric cases were consistent with their normal developed age group and delayed relative to adult CAEP latencies, as expected. CONCLUSIONS: This study demonstrated the feasibility of recording long latency CAEPs directly and solely through CI in adults with residual hearing, in response to acoustic stimulation of the non-implanted ear. The CI CAEPs closely resembled the CAEPs recorded simultaneously by an external EP system and via scalp electrodes. The ability to record directly from the implant, without the need of an external recording system, presents an innovative method with many clinical and research implications.

The effect of environmental noise isolation on bronchiolitis severity in hospitalised children.

AIM: Bronchiolitis is an infectious disease, with no effective treatment. Music and Mozart's works specifically are known to have a positive effect on physiological parameters, while noise is considered harmful. We aim to evaluate the short-term effect of environmental noise detachment and/or music listening on the course of bronchiolitis in hospitalised children. METHODS: This is a prospective, double-blinded randomised controlled trial. Patients were divided into three intervention groups: 1-Mozart's Sonata, 2-instrumental music, 3-silence. Music was heard via media players and soundproof headphones. Disease severity was evaluated before and after intervention using the Modified Tal score. RESULTS: Seventy music sessions were included in the analysis (Mozart n = 23, instrumental n = 22, silence n = 25). A one-point drop in the average bronchiolitis severity score was observed in all three groups from 7.1 (CI 95%, 5 to 9.2) to 6.1 (CI 95%, 4.3 to 7.9), p < 0.001. No significant difference was found between the three groups with respect to change in the severity score before and after the intervention. CONCLUSION: Isolation from disturbing sounds heard in paediatric departments could be considered a simple non-invasive intervention in children hospitalised with bronchiolitis. Further studies are warranted to evaluate long-term effects of this intervention and the specific effect of music.

Postoperative Intracochlear Electrocochleography in Pediatric Cochlear Implant Recipients: Association to Audiometric Thresholds and Auditory Performance.

OBJECTIVES: The aim of this study was to compare intracochlear-recorded cochlear microphonics (CM) responses to behavioral audiometry thresholds in young children, with congenital hearing loss, 2 to 5 years after cochlear implantation early in life. In addition, differences in speech and auditory outcomes were assessed among children with and without residual hearing. DESIGN: The study was conducted at a tertiary, university-affiliated, pediatric medical center. CM responses by intracochlear electrocochleography technique were recorded from 102 implanted ears of 60 children and those responses correlated to behavioral audiometry thresholds at 0.125 to 2 kHz frequencies. All children had received Advanced Bionics cochlear implant with High Focus J1 or MidScala electrodes, along with extensive auditory rehabilitation before and after implantation, including the use of conventional hearing aids. Speech Spatial and Hearing Qualities, Category of Auditory Performance scale, and educational settings information were obtained for each participant. Those cochlear implantation (CI) outcomes were compared between children with or without residual CM responses. RESULTS: Two distinctive CM responses patterns were found among the implanted children. Of all ears diagnosed with cochlear hearing loss (n = 88), only in 29 ears, clear CM responses were obtained. In all other ears, no CM responses were obtained at the maximum output levels. The CM responses were highly correlated with coefficients ranging from 0.7 to 0.83 for the audiometric behavioral thresholds at 0.125 to 2 kHz frequency range. Of all ears diagnosed with auditory neuropathy syndrome disorder (n = 14), eight ears had residual hearing and recordable CM postimplantation. The other six ears showed no recordable CM responses at maximum output levels for all tested frequencies. Those showed supposedly better CM responses than the behavioral audiometry threshold, however with poor correlations with tested frequency thresholds. Children with residual hearing showed significantly better auditory outcomes with CI, compared with those without residual hearing. CONCLUSIONS: In children with congenital cochlear hearing loss, the objective CM intracochlear responses can reliably predict the residual audiometric threshold. However, in children with auditory neuropathy syndrome disorder, the CM thresholds did not match the behavioral audiometric responses. Postoperatively, children with recordable CM responses, showing preserved residual hearing, demonstrated better CI outcomes.

Treatment of congenital cytomegalovirus beyond the neonatal period: an observational study.

Recently, valganciclovir treatment of symptomatic congenital cytomegalovirus (cCMV) disease, commenced during the neonatal period (≤ 4 weeks), was found to improve hearing and developmental outcome. However, many children (symptomatic or asymptomatic at birth) present only after 4 weeks of age. The purpose of this observational retrospective study was to describe the outcome and safety of valganciclovir therapy in infants with cCMV who started treatment > 4 weeks of life. Of the 91children who started antiviral treatment > 4 weeks of age, 66/298 (22.2%) were symptomatic at birth; 25/217 (11.5%) were asymptomatic at birth. Treatment was initiated on average at 14 weeks of age (range 5-77 weeks) and at 53.3 weeks (range 12-156 weeks), respectively. Of the 45 affected ears in the symptomatic group, 30 (66.7%) improved and only 2 (4.4%) deteriorated, with most of the improved ears (27/30, 90%) returning to normal. In the asymptomatic group, late-onset treatment was initiated and out of the 42 deteriorated ears, 38 (90.5%) improved after at least 1 year of follow-up. Hematological adverse events, i.e., neutropenia, were noted in a minority of cases (4.4%).Conclusion: Our study demonstrates the benefits and safety aspects of treating symptomatic and asymptomatic children with cCMV even beyond the recommended neonatal period.What is Known:• Valganciclovir treatment of symptomatic congenital cytomegalovirus (cCMV) disease, commenced during the neonatal period, is beneficial in improving hearing and developmental outcome.• However, data of treatment started beyond the neonatal period is lacking.What is New:• Our study demonstrates the benefits of treating symptomatic children with cCMV as well as asymptomatic children that develop late-onset hearing loss even beyond the recommended neonatal period.• This was true for symptomatic children who presented > 4 weeks as well as to those were asymptomatic at birth but experienced late hearing deterioration.

[COCHLEAR IMPLANTS IN SINGLE-SIDED DEAFNESS].

INTRODUCTION: In normal hearing, the brain receives bilateral auditory input from both ears. In individuals with only one functioning ear listening in noisy environments and sound localization may become difficult. Historically, the impact of unilateral hearing loss in children had typically been minimized by clinicians, as it was assumed that one normal hearing ear provided sufficient auditory input for speech development and normal hearing experience. Data supporting the negative effects of unilateral deafness has been accumulating during the last decades. The effects of unilateral deafness extend beyond spatial hearing to language development, slower rates of educational progress, problems in social interaction and in cognitively demanding tasks. Until recently, treatments for single sided deafness were limited to routing signals from the deaf ear to the contralateral hearing ear either through conventional CROS aids or through bone anchored technologies. These technologies simply transfer sounds to the single functioning ear which allow sound awareness from the deaf side and minor improvement in hearing in noisy environments and localization. The cochlear implant is a surgically implanted electronic device that contains an array of electrodes which is placed into the cochlea, and stimulates the cochlear nerve. The cochlear implant bypasses the injured parts of the inner ear. Currently it is the only treatment to restore binaural hearing. This review aims to discuss the different aspects, the benefits and disadvantages of cochlear implantation in children with single sided deafness.

Cochlear Implantation and Social-Emotional Functioning of Children with Hearing Loss.

This study examined the contribution of cochlear implants (CIs) to the social-emotional functioning of children who are deaf or hard of hearing (dhh). Sixty-three parents of children who are dhh participated in the study. Thirty children were CI users and 32 used hearing aids (HAs). They completed the Strengths and Difficulties Questionnaire and a background questionnaire. Parents of children with CIs reported lower levels of hyperactivity/inattention and higher levels of pro-social behavior compared to parents of children with HAs. Additionally, older age when hearing loss was detected was related to more pro-social behavior, and age at implantation among CI users was negatively correlated with children's hyperactivity/inattention and conduct problems. These findings add to the existing knowledge about the many benefits of CIs for individuals with hearing loss and emphasize the possible impact of early implantation to children's social-emotional functioning.

Valganciclovir Is Beneficial in Children with Congenital Cytomegalovirus and Isolated Hearing Loss.

OBJECTIVE: To evaluate the efficacy of antiviral treatment for infants with congenital cytomegalovirus (cCMV) with isolated sensorineural hearing loss (SNHL). STUDY DESIGN: Data were reviewed retrospectively for infants with isolated SNHL who received prolonged antiviral treatment between 2005 and 2017. Hearing status was evaluated for infants who had been followed for >1 year. RESULTS: Among 329 infants treated for cCMV, 59 (18%) were born with isolated SNHL. Hearing impairment was unilateral in 38 (64.4%) infants and bilateral in 21 (35.6%). Of the 80 affected ears at baseline, 55 (68.8%) improved, and only 2 (2.5%) deteriorated. Most of the improved ears (53/55 = 96.3%) returned to normal hearing with no deterioration observed in the ears that were unaffected at baseline. On best ear evaluation, of 21 infants who had bilateral hearing loss, 16 (76.1%) improved (93.7% regaining normal functional hearing); none deteriorated. CONCLUSION: Infants born with isolated SNHL due to cCMV were found to benefit from prolonged antiviral treatment. These children (and ears) showed significant improvement in hearing status and no deterioration of unaffected ears at baseline. Our data serve as observational evidence of the benefits of antiviral treatment in these children. Avoiding treatment of these children due to the lack of prospective data is debatable.

Pediatric Cochlear implant soft failure.

PURPOSE: Hard cochlear implant failures are diagnosed by objective tests whereas soft failures are suspected on the basis of clinical signs and symptoms. This study reviews our experience with children in tertiary pediatric medical center who underwent revision cochlear implantation, with emphasis on soft failures. MATERIALS AND METHODS: Children (age<18years) who underwent revision cochlear implantation from 2000 to 2012 were identified by database search. Pre- and post-explantation data were collected. RESULTS: Twenty-six revision surgeries were performed, accounting for 7.4% of all cochlear implant surgeries at our center during the study period. The pre-explantation diagnosis was hard failure in 7 cases (27%), soft failure in 12 (46%), and medical failure in 7 (27%). On post-explantation analysis, 7/12 devices from the soft-failure group with a normal integrity test had abnormal findings, yielding a 63% false-negative rate (12/19) for the integrity test. All children regained their initial performance. Compared to hard failures, soft failures were associated with a shorter median time from first implantation to symptom onset (8 vs 25months) but a significantly longer time from symptom onset to revision surgery (17.5 vs 3months; P=0.004). CONCLUSIONS: Soft cochlear implant failure in young patients poses a diagnostic challenge. A high index of suspicion is important because a delayed diagnosis may have severe consequences for language development. A normal integrity test does not unequivocally exclude device failure and is unrelated to functional outcome after revision surgery. Better education of parents and rehabilitation teams is needed in addition to more accurate diagnostic tests.

Auditory Performance and Electrical Stimulation Measures in Cochlear Implant Recipients With Auditory Neuropathy Compared With Severe to Profound Sensorineural Hearing Loss.

OBJECTIVES: The aim of the study was to compare auditory and speech outcomes and electrical parameters on average 8 years after cochlear implantation between children with isolated auditory neuropathy (AN) and children with sensorineural hearing loss (SNHL). DESIGN: The study was conducted at a tertiary, university-affiliated pediatric medical center. The cohort included 16 patients with isolated AN with current age of 5 to 12.2 years who had been using a cochlear implant for at least 3.4 years and 16 control patients with SNHL matched for duration of deafness, age at implantation, type of implant, and unilateral/bilateral implant placement. All participants had had extensive auditory rehabilitation before and after implantation, including the use of conventional hearing aids. Most patients received Cochlear Nucleus devices, and the remainder either Med-El or Advanced Bionics devices. Unaided pure-tone audiograms were evaluated before and after implantation. Implantation outcomes were assessed by auditory and speech recognition tests in quiet and in noise. Data were also collected on the educational setting at 1 year after implantation and at school age. The electrical stimulation measures were evaluated only in the Cochlear Nucleus implant recipients in the two groups. Similar mapping and electrical measurement techniques were used in the two groups. Electrical thresholds, comfortable level, dynamic range, and objective neural response telemetry threshold were measured across the 22-electrode array in each patient. Main outcome measures were between-group differences in the following parameters: (1) Auditory and speech tests. (2) Residual hearing. (3) Electrical stimulation parameters. (4) Correlations of residual hearing at low frequencies with electrical thresholds at the basal, middle, and apical electrodes. RESULTS: The children with isolated AN performed equally well to the children with SNHL on auditory and speech recognition tests in both quiet and noise. More children in the AN group than the SNHL group were attending mainstream educational settings at school age, but the difference was not statistically significant. Significant between-group differences were noted in electrical measurements: the AN group was characterized by a lower current charge to reach subjective electrical thresholds, lower comfortable level and dynamic range, and lower telemetric neural response threshold. Based on pure-tone audiograms, the children with AN also had more residual hearing before and after implantation. Highly positive coefficients were found on correlation analysis between T levels across the basal and midcochlear electrodes and low-frequency acoustic thresholds. CONCLUSIONS: Prelingual children with isolated AN who fail to show expected oral and auditory progress after extensive rehabilitation with conventional hearing aids should be considered for cochlear implantation. Children with isolated AN had similar pattern as children with SNHL on auditory performance tests after cochlear implantation. The lower current charge required to evoke subjective and objective electrical thresholds in children with AN compared with children with SNHL may be attributed to the contribution to electrophonic hearing from the remaining neurons and hair cells. In addition, it is also possible that mechanical stimulation of the basilar membrane, as in acoustic stimulation, is added to the electrical stimulation of the cochlear implant.

Clinical Implications for Children Born With Congenital Cytomegalovirus Infection Following a Negative Amniocentesis.

BACKGROUND: Recently, congenital cytomegalovirus (cCMV) infection was reported irrespective of a negative amniotic fluid prenatal analysis for cytomegalovirus (CMV). The question of whether this phenomenon represents low sensitivity of the test or late development of fetal infection (after amniocentesis) was discussed, but not answered. However, if late transmission is the rule, then infants born with cCMV after negative amniocentesis would be expected to carry better prognosis than those who tested positive. METHODS: Data of all infants with cCMV infection, followed in 2 pediatric centers from 2006 to 2015, were reviewed. Infant outcome after birth of symptomatic vs asymptomatic disease was compared with infants born after a negative amniocentesis (study group) and those with a positive amniocentesis (control group). RESULTS: Amniocentesis was performed in 301 pregnancies of our cohort of infants with cCMV and was negative for CMV in 47 (15.6%). There were fewer symptomatic cCMV neonates in the study group than in the control group (4.3% vs 25%; P < .001). Hearing impairment at birth was also less frequent in the study group (2.2% vs 17.4%; P = .012). None of the children in the study group had neurologic sequelae at long-term follow up, compared with 13 (14.1%) in the control group (P < .001). CONCLUSIONS: Although negative amniocentesis does not exclude cCMV, infants with cCMV born after a negative amniocentesis seldom present with mild clinical symptoms or cerebral ultrasound features at birth. These children also have a very good long-term outcome. Our findings support the theory of a late development of fetal infection, after the time of the amniocentesis.

Hearing loss patterns after cochlear implantation via the round window in an animal model.

PURPOSE: The mechanism and the type of hearing loss induced by cochlear implants are mostly unknown. Therefore, this study evaluated the impact and type of hearing loss induced by each stage of cochlear implantation surgery in an animal model. STUDY DESIGN: Original basic research animal study. SETTING: The study was conducted in a tertiary, university-affiliated medical center in accordance with the guidelines of the Institutional Animal Care and Use Committee. SUBJECTS AND METHODS: Cochlear implant electrode array was inserted via the round window membrane in 17 ears of 9 adult-size fat sand rats. In 7 ears of 5 additional animals round window incision only was performed, followed by patching with a small piece of periosteum (control). Hearing thresholds to air (AC) and bone conduction (BC), clicks, 1 kHz and 6 kHz tone bursts were measured by auditory brainstem evoked potential, before, during each stage of surgery and one week post-operatively. In addition, inner ear histology was performed. RESULTS: The degree of hearing loss increased significantly from baseline throughout the stages of cochlear implantation surgery and up to one week after (p<0.0001). In both operated groups, the greatest deterioration was noted after round window incision. Overall, threshold shift to air-conduction clicks, reached 61 dB SPL and the bone conduction threshold deteriorated by 19 dB SPL only. Similar losses were found for 1-kHz and 6-kHz frequencies. The hearing loss was not associated with significant changes in inner ear histology. CONCLUSIONS: Hearing loss following cochlear implantation in normal hearing animals is progressive and of mixed type, but mainly conductive. Changes in the inner-ear mechanism are most likely responsible for the conductive hearing loss.

Cognitive autonomy among adolescents with and without hearing loss: Associations with perceived social support.

Cognitive autonomy is a skill which may help adolescents prepare for important decisions in adulthood. The current study examined the associations between cognitive autonomy and perceived social support among adolescents with and without hearing loss. Participants were 177 students: 55 were deaf and hard of hearing (dhh) and 122 were hearing. They completed the Cognitive Autonomy and Self-Evaluation Inventory, the Multidimensional Scale of Perceived Social Support, and a demographic questionnaire. Significant positive correlations were found between some of the cognitive autonomy variables and some of the perceived social support variables. However, among the dhh group, they were fewer and weaker. Family support was found to be a significant predictor of three out of the five cognitive autonomy variables. In addition, significant differences were found between the dhh and hearing participants in some of the cognitive autonomy variables, but not in perceived social support. Implications for theory and practice are discussed.

Lenticulostriated vasculopathy is a high-risk marker for hearing loss in congenital cytomegalovirus infections.

AIM: This study investigated the relationship between lenticulostriated vasculopathy (LSV) and hearing loss in 141 infants with congenital cytomegalovirus (cCMV) infection. METHODS: We included all infants with cCMV infection who were followed in our clinic for more than a year with only LSV signs of brain involvement on initial brain ultrasound. Group one comprised 13 infants with no hearing impairment at birth who were not treated with gan/valganciclovir during 2006-2009. Group two was 51 infants with LSV and no hearing impairment who had been treated since mid-2009. Group three was 25 infants born with LSV and hearing loss, who had been treated from birth. Group four was 52 control infants born during the same period with asymptomatic cCMV. Hearing tests were performed during the neonatal period and every four to six months until four years of age. RESULTS: Hearing deterioration was more extensive in group one (85%) than in group two (0%, p < 0.001) and the asymptomatic group (10%, p < 0.001) and occurred more often in group four (10%) than in group two (0%, p = 0.008). CONCLUSION: Lenticulostriated vasculopathy was common in infants with cCMV infection and may serve as a sign of central nervous system involvement and further hearing deterioration. Antiviral treatment may be prudent in such infants.

Pattern of hearing loss following cochlear implantation.

Cochlear implantation is associated with deterioration in hearing. Despite the fact that the damage is presumed to be of sensory origin, residual hearing is usually assessed by air-conduction thresholds alone. This study sought to determine if surgery may cause changes in air- and bone-conduction thresholds producing a mixed-type hearing loss. The sample included 18 patients (mean age 37 years) with an air-bone gap of 10 dB over three consecutive frequencies and measurable masked and reliable bone-conduction thresholds of operated and non-operated ears who underwent cochlear implant surgery. All underwent comprehensive audiologic and otologic assessment and imaging before and after surgery. The air-bone gap in the treated ears was 17-41 dB preoperatively and 13-59 dB postoperatively over 250-4,000 Hz. Air-conduction thresholds in the treated ears significantly deteriorated after surgery, by a mean of 10-21 dB. Bone-conduction levels deteriorated nonsignificantly by 0.8-7.5 dB. The findings indicate that the increase in air-conduction threshold after cochlear implantation accounts for most of the postoperative increase in the air-bone gap. Changes in the mechanics of the inner ear may play an important role. Further studies in larger samples including objective measures of inner ear mechanics may add information on the source of the air-bone gap.

Predicting hearing thresholds in occupational noise-induced hearing loss by auditory steady state responses.

OBJECTIVES: Currently available behavioral tools for the assessment of noise-induced hearing loss (NIHL) depend on the reliable cooperation of the subject. Furthermore, in workers' compensation cases, there is considerable financial gain to be had from exaggerating symptoms, such that accurate assessment of true hearing threshold levels is essential. An alternative objective physiologic tool for assessing NIHL is the auditory steady state response (ASSR) test, which combines frequency specificity with a high level of auditory stimulation, making it applicable for the evaluation of subjects with a moderate to severe deficit. The primary aim of the study was to assess the value of the multifrequency ASSR test in predicting the behavioral warble-tone audiogram in a large sample of young subjects with NIHL of varying severity or with normal hearing. The secondary goal was to assess suprathreshold ASSR growth functions in these two groups. DESIGN: The study group included 157 subjects regularly exposed to high levels of occupational noise, who attended a university-associated audiological clinic for evaluation of NIHL from 2009 through 2011. All underwent a behavioral audiogram, and on the basis of the findings, were divided into those with NIHL (108 subjects, 216 ears) or normal hearing (49 subjects, 98 ears). The accuracy of the ASSR threshold estimations for frequencies of 500, 1000, 2000, and 4000 Hz was compared between groups, and the specificity and sensitivity of the ASSR test in differentiating ears with or without NIHL was calculated using receiver operating characteristic analysis. Linear regression analysis was used to formulate an equation to predict the behavioral warble-tone audiogram at each test frequency using ASSR thresholds. Multifrequency ASSR amplitude growth as a function of stimulus intensity was compared between the NIHL and normal-hearing groups for 1000 Hz and 4000 Hz carrier frequencies. RESULTS: In the subjects with NIHL, ASSR thresholds to various frequencies were significantly and highly correlated with the behavioral warble-tone thresholds; Pearson correlation coefficients ranged from 0.6 to 0.8 over the four frequencies tested. Differences between thresholds ranged from 10 to 13 dB. The configuration of the ASSR waveforms closely approximated the behavioral audiogram. The sensitivity for screening hearing thresholds was 92%; by frequency, sensitivity ranged between 92.7 and 98.4%, but specificity was lower, especially at the low frequencies. ASSR accurately predicted moderate and severe NIHL. The mean ASSR growth amplitude to increasing stimulus level for 1000 and 4000 Hz was significantly steeper in the NIHL than in the normal-hearing group, with no significant difference between frequencies. CONCLUSIONS: The ASSR test has a high sensitivity to detect moderate to severe hearing loss in subjects with NIHL. Its use can facilitate the early identification of noise-exposed workers with NIHL. It may also serve an important medico-legal function in cases of workers' compensation. The ASSR test is not, by itself, an appropriate tool for hearing screening in the general population.

Long-term outcomes and electrophysiological measures of children with inner ear malformations and cochlear implants.

OBJECTIVES: To compared auditory and speech performance outcomes of children with cochlear implants (CI), between those with inner ear malformations (IEMs) and with normal ear anatomy; and to describe differences in electrophysiological measurements. STUDY DESIGN: A retrospective study. SETTING: A tertiary care pediatric medical center. PATIENTS: Forty-one children with IEMs who underwent CI during 2003-2017, and 41 age-matched CI recipients with normal ear anatomy (control group). MAIN OUTCOME MEASURES: Post-CI auditory performance outcomes including educational setting, Categories of Auditory Performance (CAP), and Speech Intelligibility Rating (SIR); and electrophysiological measurements, Including maximal comfortable electrical levels (CLs) and impedances along CI electrodes. RESULTS: The ANOVA on ranks revealed lower CAP scores in the study than control group: H3 = 18.8, P < 0.001. Among children with IEMs, CAP scores were better in children with enlarged vestibular aqueduct (EVA) (P < 0.04). SIR scores of the control group did not differ from those with isolated EVA; however, SIR scores of the IEMs without EVA subgroup were lower than all the other study subgroups (P < 0.01). The proportion of the control group that was integrated with full inclusion educational settings into the regular mainstream schools was higher than for those with IEMs without EVA (47 % vs. 15 %, P < 0.05), but similar to those with isolated EVA. For the study group versus control group, maximal comfortable electrical levels (CLs) were higher)P > 0.03) while impedance measurements were similar. CONCLUSIONS: Outcomes of pediatric recipients with normal anatomy were better than those with IEMs. Among pediatric recipients of CI with IEMs, auditory performance was better and CLs were lower among children with isolated EVA than all other IEM subgroups.

The protective effect of aspirin-induced temporary threshold shift in an animal model of cisplatin-related ototoxicity.

PURPOSE: The purpose of this study was to evaluate whether induction of temporary threshold shift (TTS) with aspirin prior to cisplatin exposure can prevent or minimize cisplatin detrimental effects on hearing. METHODS: We randomly divided BALB mice into three groups: (1) cisplatin only, (2) aspirin only, and (3) combined aspirin/cisplatin. Cisplatin was administered as a single intraperitoneal injection of 14 mg/kg. Aspirin was administered for three weeks via intraperitoneal injection of 200 mg/kg sodium salicylate, twice daily. Air conduction thresholds were recorded using Auditory Brainstem Responses (ABR). Cochleae were harvested and cochlear hair cells were counted using a scanning electron microscope (SEM). RESULTS: Aspirin-induced TTS have reached an average of 30.05±16.9 dB after 2 weeks. At 60 days, cisplatin-only treated mice experienced an average threshold shifts of 50.7 dB at 4 kHz, 35.16 dB at 8 kHz, 70 dB at 16 kHz, 53.1 dB at 32 kHz. All threshold shifts were significantly worse than for cisplatin/aspirin treated mice with TTS of 11.85 dB at 4 kHz, 3.58 dB at 8 kHz, 16.58  dB at 16 kHz, 20.41 dB at 32 kHz (p < 0.01). Cochlear cell count with SEM has shown reduction in the number of both inner and outer hair cells in the mid-turn in cisplatin treated mice. CONCLUSION: Aspirin induced TTS can protect from cisplatin-induced ototoxicity. This beneficial effect was demonstrated by auditory thresholds as well as SEM. Larger pre-clinical and clinical studies are still needed to confirm these findings.

Cochlear hearing loss in patients with Laron syndrome.

The aim of this prospective clinical study was to test auditory function in patients with Laron syndrome, either untreated or treated with insulin-like growth factor I (IGF-I). The study group consisted of 11 patients with Laron syndrome: 5 untreated adults, 5 children and young adults treated with replacement IGF-I starting at bone age <2 years, and 1 adolescent who started replacement therapy at bone age 4.6 years. The auditory evaluation included pure tone and speech audiometry, tympanometry and acoustic reflexes, otoacoustic emissions, loudness dynamics, auditory brain stem responses and a hyperacusis questionnaire. All untreated patients and the patient who started treatment late had various degrees of sensorineural hearing loss and auditory hypersensitivity; acoustic middle ear reflexes were absent in most of them. All treated children had normal hearing and no auditory hypersensitivity; most had recordable middle ear acoustic reflexes. In conclusion, auditory defects seem to be associated with Laron syndrome and may be prevented by starting treatment with IGF-I at an early developmental age.

Prediction of aided and unaided audiograms using sound-field auditory steady-state evoked responses.

OBJECTIVE: To assess sound field auditory thresholds of hearing-impaired adults by using auditory steady-state evoked responses (ASSRs). DESIGN: ASSRs were recorded to carrier frequencies of 500, 1000, 2000, and 4000 Hz, each uniquely modulated at a single frequency of 80-100 Hz. ASSR thresholds were compared to behavioral auditory thresholds. STUDY SAMPLE: Twenty adults (11 male, age 35.6 years) with moderate-severe sensorineural hearing loss who had used hearing aids, and 10 normal-hearing subjects (mean age 22.4 years). RESULTS: For most frequencies, behavioral sound-field thresholds were slightly lower than ASSR thresholds in both aided and unaided conditions, with a significant correlation between them. Differences between ASSR and behavioral thresholds ranged between 516 dB in the unaided and between 5-16 dB in the aided condition. The ASSR amplitude growth function to 2000 Hz was steeper in both the aided and unaided conditions than in the normal-hearing group. CONCLUSIONS: Sound-field ASSRs can predict behavioral auditory thresholds in both the unaided and aided condition, as well as behavioral functional gains. The ASSR growth function for 2000 Hz is suggested to reflect an underlying mechanism of intensity encoding common to abnormal loudness perception frequently reported in cases of cochlear hearing loss.