Children with residual hearing have become eligible for consideration as CI candidates in some pediatric programs because of the positive clinical research outcomes of cochlear implant (CI). However, decision-making about CIs for children with residual hearing is difficult for parents because they experience uncertainty when their children show auditory benefits and are developing language through hearing aids (HAs). Clinicians may be uncomfortable recommending CI for these children due to variability in audiometric candidacy criteria in individual clinical practice. However, there is very limited information about the CI decision-making process and needs to assist the parents of these children and practitioners. We conducted a comprehensive study to better understand and support the CI decision-making experiences of families and practitioners. The objectives of our study were to: 1) explore the clinical characteristics and outcomes of children with residual hearing who received CIs, 2) summarize the evidence about the benefits and risks of CIs compared to HAs in children with residual hearing, and 3) explore the decision-making process and needs for children with residual hearing from the perspective of parents and practitioners. This research project combined quantitative and qualitative research designs. A retrospective chart review was conducted to address the first objective. Data on the clinical characteristics of children with residual hearing were extracted from medical charts from a tertiary care pediatric CI center in Ottawa, Canada. A systematic review was performed on the benefits and risks of CIs versus HAs for children with residual hearing to address the second objective. The third objective was addressed through two sub-studies. The first sub-study involved qualitative semi-structured interviews. A total of 12 parents participated in individual interviews. In the second sub-study, 17 practitioners at a pediatric CI center in Ottawa, Canada, and specialized teachers of the deaf and hard of hearing at local school boards were recruited, and four focus groups and one individual interview were conducted. This study showed that a total of 100 of 389 (25.7%) children who received CIs from 1992 to 2018 at the Children's Hospital of Eastern Ontario (CHEO) had residual hearing, representing more than half the children who were implanted in the last two years covered by the study. As documented in our study, overall, children with residual hearing demonstrated benefits in auditory functioning following cochlear implantation. Approximately 70% of these children achieved open-set word perception scores of 80% or more post-CI. In the systematic reviews, a total of 3265 citations were identified, of which eight studies met inclusion criteria. The articles consisted of four moderate and two weak quality pre-post cohort studies and two weak quality cross-sectional studies. The systematic review confirmed that children with CIs showed significantly better speech perception scores than those with HAs. Limited evidence of improvement in auditory performance and non-significant improvement in speech intelligibility was found. Two aspects of social-emotional functioning (hyperactivity/inattention and pro-social behaviour) showed significant improvement with CIs. Our finding also contributes new information about the loss of residual hearing and device use. Four studies provided data on risks following CIs; a total of 16 of 43 (37.2%) children showed loss of residual hearing and 14.0% (8/57) of children had discontinued or limited use of their CI or HA. The qualitative interviews revealed that both parents and practitioners identified child’s everyday functioning as an important factor that influenced their decision-making. It was clear through the qualitative research with parents that they held a strong preference for child’s inclusion into hearing society. Spoken communication was a core value for the parents of these children and some parents expressed high expectations that their children’s hearing would become ‘normal’. We found that practitioners primarily supported parental decision-making by providing information on the practical aspects of the benefits and risks of CIs. Overall parents were satisfied with the decision-making process and decision support from practitioners. However, parents stressed the importance of receiving more personalized information that considered their specific concerns, values and preferences related to their child and family’s circumstances. Practitioners also noted that more research among children with residual hearing is needed to guide parental CI decision-making. To our knowledge, the findings from this dissertation are the first to examine decision-making for children with residual hearing. Our study contributes new information about the characteristics of children receiving CIs, the potential benefits and risks for children with residual hearing, and decision-making needs from the perspectives of families and practitioners. In addition, our research is a useful first step in understanding what families need to make better decisions to assist in the CI decision-making process for this specific population.

A common cause of profound deafness is hair cell dysfunction in the cochlea. Cochlear implants (CI) bypass the hair cells via an electrode and stimulate the cochlear nerve directly. Nowadays, it is possible to preserve residual hair cell function and hearing through flexible electrodes and a-traumatic CI surgery techniques; called hearing preservation CI surgery. This may suit partially deaf patients who can use natural low frequency hearing in combination with electric high frequency hearing; so-called hybrid hearing. The aim of this thesis was to elucidate the effectiveness of hearing preservation CI surgery. The thesis demonstrates human cochlear anatomy in relation to CI and evaluates hearing and patient satisfaction after hearing preservation CI surgery. Analyses of human cochlear moulds belonging to the Uppsala collection showed large variations in dimensions and coiling characteristics of the cochlea. Each cochlea was individually shaped. The size and shape of the cochlea influences the position of the electrode. The diameter of the basal cochlear turn could predict insertion depth of the electrode, which is crucial for hearing preservation. The first 21 patients operated with hearing preservation CI surgery in Uppsala, showed preserved hearing. Nine-teen partially deaf patients receiving implants intended for hybrid hearing, were evaluated concerning pure tone audiometry, monosyllables (MS) and hearing in noise test (HINT). They also responded to a questionnaire, consisting of the IOI-HA, EQ-5D VAS and nine questions about residual hearing. The questionnaire results indicated a high degree of patient satisfaction with improved speech perception in silence and noise. This was also reflected by improved results in MS and HINT. Hearing was preserved in all patients, but there was an on-going deterioration of the residual hearing in the operated ear which surpassed the contralateral ear. There were no correlations between the amount of residual hearing and patient satisfaction or speech perception results. Electric stimulation provides a major contribution to speech comprehension in partially deaf patients. All the patients showed a high degree of satisfaction with their CI, regardless of varying hearing preservation.

INTRODUÇÃO: As Respostas Auditivas de Estado Estável permitem avaliação frequência específica em intensidades de até 120 dB NA e a detecção de audição residual em pacientes com perda auditiva severo-profunda. O objetivo deste estudo é comparar os limiares à RAEE e os resultados da avaliação comportamental em crianças com suspeita de surdez severo-profunda. MÉTODO: Estudo transversal para comparar respostas à RAEE e por audiometria com reforço visual (VRA) em 63 crianças candidatas ao implante coclear (126 orelhas) com idade entre 6 e 72 meses. Foram incluídas crianças com otomicroscopia normal, ausência de respostas ao PEATE clique a 90 dB NA e às emissões otoacústicas. Foram excluídas crianças com malformações de orelha interna, doenças do espectro da neuropatia auditiva, ou que não completaram a avaliação comportamental ou não atingiram ruído eletroencefalográfico < 30 nV durante a RAEE. Foram utilizados estímulos com tons contínuos sinusoidais (100% AM e 20% FM) nas frequências de 500, 1000, 2000 e 4000 Hz em intensidade máxima de 110 dB NA. Os limiares à VRA foram obtidos por tom warble nas frequências de 500, 1000, 2000 e 4000 Hz em cada orelha através de fones de inserção (ER-5A) ou tipo casco (TDH-39). A intensidade máxima de estimulação foi de 120 dB NA em cada frequência. RESULTADOS: Limiares comportamentais foram obtidos em 36,7% (185/504) de todas as frequências em todas as crianças, 9% em intensidade maior que 110 dB NA. Entre as 504 medidas da RAEE em 63 indivíduos, 53 limiares foram obtidos (10,5%). Ao todo, 89,5% das frequências testadas não apresentaram nenhuma resposta em 110 dB NA. A distribuição dos limiares à RAEE foi semelhante à da avaliação comportamental. A maioria das respostas foram em 500 Hz, diminuindo nas frequências agudas. A diferença média entre os limiares à VRA e à RAEE variou entre 0,09 e 8,94 dB. Foram realizadas 27 comparações entre RAEE e VRA: 12 em 500 Hz, 9 em 1000 Hz, 5 em 2000 Hz e 1 em 4000 Hz. Respostas ausentes foram observadas em ambos os testes em 38,1% em 0,5 KHz, 52,45% em 1 KHz, 74,6% em 2 KHz e 81,0% em 4 KHZ. A especificidade foi > 90% em 1, 2 e 4 KHz. Nas orelhas sem resposta comportamental em 120 dB NA, todos os limiares à RAEE estavam na faixa de perda profunda, 90% deles > 110 dB NA. CONCLUSÃO: A ausência de respostas nas altas intensidades na RAEE foi o principal achado (especificidade > 90%) o que prediz limiares comportamentais na faixa de surdez profunda / Introduction and Objective: ASSR allows frequency-specific evaluation in intensities up to 120 dB HL and detection of residual hearing in patients with severe-toprofound hearing loss. The aim of this study was to compare ASSR thresholds and behavioral test results in children with suspected severe-to-profound hearing loss. Methods: A cross sectional study was carried out to compare ASSR and Visual Reinforcement Audiometry (VRA) responses in 63 pediatric cochlear implant candidates (126 ears) aged between 6 to 72 months. We included children with normal otomicroscopy findings, absent responses to click-ABR at 90 dB HL and otoaccoustic emissions. We excluded children with inner ear malformations, auditory neuropathy spectrum disorder or who did not complete VRA or achieve EEG noise < 30 nV during the ASSR test. Air-conduction ASSR stimuli were continuous sinusoidal tones (100% AM and 20% FM) presented at 0.5, 1, 2 and 4 kHz starting at the maximum presentation level of 110 dB HL. VRA thresholds were acquired with warble tones presented at 0.5, 1, 2 and 4 KHz in each ear through ER-tone 5A or TDH-39 phones. Maximum presentation level was 120 dB HL for each frequency. Results: Behavioral thresholds were obtained in 36.7% (185/504) of all frequencies in all subjects, 9% were in intensities > 110 dB HL. Among 504 ASSR measurements from 63 subjects, 53 thresholds were obtained (10.5%). Overall 89.5% of the tested frequencies did not show any response at 110 dB HL. The distribution of ASSR responses was similar to the behavioral test results. Most responses were at 500 Hz, decreasing among the higher frequencies. Mean differences between behavioral and ASSR thresholds varied from 0.09 to 8.94 dB. Overall, 27 comparisons of behavioral and ASSR thresholds were obtained: 12 at 0.5 KHz, 9 at 1 KHz, 5 at 2 KHz and 1 at 4 KHz. Absent responses were observed in both tests in 38.1% at 0.5 KHz, 52.4% at 1 KHz, 74.6% at 2 KHz and 81.0% at 4 KHz. The specificity was > 90% at 1, 2 and 4 KHz. In ears with no behavioral response at 120 dB HL all ASSR thresholds were in the profound hearing loss range, 90% of them were equal or > than 110 dB HL. Conclusion: Among 63 pediatric CI candidates, absent responses to high-intensity ASSR was the major finding (specificity > 90%) predicting behavioral thresholds in the profound hearing loss range. These findings can be helpful to confirm the decision for cochlear implantation

INTRODUÇÃO: As Respostas Auditivas de Estado Estável permitem avaliação frequência específica em intensidades de até 120 dB NA e a detecção de audição residual em pacientes com perda auditiva severo-profunda. O objetivo deste estudo é comparar os limiares à RAEE e os resultados da avaliação comportamental em crianças com suspeita de surdez severo-profunda. MÉTODO: Estudo transversal para comparar respostas à RAEE e por audiometria com reforço visual (VRA) em 63 crianças candidatas ao implante coclear (126 orelhas) com idade entre 6 e 72 meses. Foram incluídas crianças com otomicroscopia normal, ausência de respostas ao PEATE clique a 90 dB NA e às emissões otoacústicas. Foram excluídas crianças com malformações de orelha interna, doenças do espectro da neuropatia auditiva, ou que não completaram a avaliação comportamental ou não atingiram ruído eletroencefalográfico < 30 nV durante a RAEE. Foram utilizados estímulos com tons contínuos sinusoidais (100% AM e 20% FM) nas frequências de 500, 1000, 2000 e 4000 Hz em intensidade máxima de 110 dB NA. Os limiares à VRA foram obtidos por tom warble nas frequências de 500, 1000, 2000 e 4000 Hz em cada orelha através de fones de inserção (ER-5A) ou tipo casco (TDH-39). A intensidade máxima de estimulação foi de 120 dB NA em cada frequência. RESULTADOS: Limiares comportamentais foram obtidos em 36,7% (185/504) de todas as frequências em todas as crianças, 9% em intensidade maior que 110 dB NA. Entre as 504 medidas da RAEE em 63 indivíduos, 53 limiares foram obtidos (10,5%). Ao todo, 89,5% das frequências testadas não apresentaram nenhuma resposta em 110 dB NA. A distribuição dos limiares à RAEE foi semelhante à da avaliação comportamental. A maioria das respostas foram em 500 Hz, diminuindo nas frequências agudas. A diferença média entre os limiares à VRA e à RAEE variou entre 0,09 e 8,94 dB. Foram realizadas 27 comparações entre RAEE e VRA: 12 em 500 Hz, 9 em 1000 Hz, 5 em 2000 Hz e 1 em 4000 Hz. Respostas ausentes foram observadas em ambos os testes em 38,1% em 0,5 KHz, 52,45% em 1 KHz, 74,6% em 2 KHz e 81,0% em 4 KHZ. A especificidade foi > 90% em 1, 2 e 4 KHz. Nas orelhas sem resposta comportamental em 120 dB NA, todos os limiares à RAEE estavam na faixa de perda profunda, 90% deles > 110 dB NA. CONCLUSÃO: A ausência de respostas nas altas intensidades na RAEE foi o principal achado (especificidade > 90%) o que prediz limiares comportamentais na faixa de surdez profunda / Introduction and Objective: ASSR allows frequency-specific evaluation in intensities up to 120 dB HL and detection of residual hearing in patients with severe-toprofound hearing loss. The aim of this study was to compare ASSR thresholds and behavioral test results in children with suspected severe-to-profound hearing loss. Methods: A cross sectional study was carried out to compare ASSR and Visual Reinforcement Audiometry (VRA) responses in 63 pediatric cochlear implant candidates (126 ears) aged between 6 to 72 months. We included children with normal otomicroscopy findings, absent responses to click-ABR at 90 dB HL and otoaccoustic emissions. We excluded children with inner ear malformations, auditory neuropathy spectrum disorder or who did not complete VRA or achieve EEG noise < 30 nV during the ASSR test. Air-conduction ASSR stimuli were continuous sinusoidal tones (100% AM and 20% FM) presented at 0.5, 1, 2 and 4 kHz starting at the maximum presentation level of 110 dB HL. VRA thresholds were acquired with warble tones presented at 0.5, 1, 2 and 4 KHz in each ear through ER-tone 5A or TDH-39 phones. Maximum presentation level was 120 dB HL for each frequency. Results: Behavioral thresholds were obtained in 36.7% (185/504) of all frequencies in all subjects, 9% were in intensities > 110 dB HL. Among 504 ASSR measurements from 63 subjects, 53 thresholds were obtained (10.5%). Overall 89.5% of the tested frequencies did not show any response at 110 dB HL. The distribution of ASSR responses was similar to the behavioral test results. Most responses were at 500 Hz, decreasing among the higher frequencies. Mean differences between behavioral and ASSR thresholds varied from 0.09 to 8.94 dB. Overall, 27 comparisons of behavioral and ASSR thresholds were obtained: 12 at 0.5 KHz, 9 at 1 KHz, 5 at 2 KHz and 1 at 4 KHz. Absent responses were observed in both tests in 38.1% at 0.5 KHz, 52.4% at 1 KHz, 74.6% at 2 KHz and 81.0% at 4 KHz. The specificity was > 90% at 1, 2 and 4 KHz. In ears with no behavioral response at 120 dB HL all ASSR thresholds were in the profound hearing loss range, 90% of them were equal or > than 110 dB HL. Conclusion: Among 63 pediatric CI candidates, absent responses to high-intensity ASSR was the major finding (specificity > 90%) predicting behavioral thresholds in the profound hearing loss range. These findings can be helpful to confirm the decision for cochlear implantation

La surdité de perception est généralement la conséquence de la mort des cellules ciliées et de la dégénérescence supplémentaire des innervations afférentes induite par une absence de stimulation. Les indications de l’implant cochléaire se sont élargies en direction des patients sourds qui présentent un reliquat d’audition exploitable dans les fréquences graves. La persistance d’un reliquat auditif suffisant après l’intervention permet parfois une stimulation électrique acoustique, amplifiée acoustique sur les fréquences graves préservées, et électrique sur les fréquences aiguës. Actuellement, plusieurs protocoles de préservation de l’audition sont en investigation. Chaque protocole vise à appliquer des procédures qui minimisent les deux mécanismes du traumatisme, à savoir trauma immédiat et trauma différé. En particulier, une insertion atraumatique du porte-électrode (Flex EAS et Flex Soft ; Med-El) a été proposée pour minimiser le traumatisme chirurgical intracochléaire. L’application local et concomitante de médicament devrait améliorer la tolérance du tissu et donc réduire les dommages intracochléaire au niveau cellulaire. Un cathéter (Med-El) intra cochléaire pour la délivrance de médicaments intracochléaire a été développé, destiné à l’utilisation d’agents pharmacologiques in situ avant l'insertion du porte-électrode. Les propriétés mécaniques de ce cathéter n’induit pas de traumatisme dans la cochlée. Une insertion séquentielle de cathéter et de porte-électrodes est réalisable et souvent atraumatique / Sensorineural deafness is generaly the result of hair cell death and additional degeneration of afferent innervation. In recent years the candidacy criteria for cochlear implantation have been expanding, and now include patients with severe to profound high-frequency hearing loss along with mild to moderate low-frequency loss. The single most important prerequisite for providing both electric and acoustic stimulation in the same ear is the preservation of acoustic hearing following the surgical procedure. Currently, several hearing preservation protocols are under investigation. Each protocol attempts to implement procedures that minimize both immediate and delayed mechanisms. Specifically, atraumatic approaches and electrode insertions (Flex EAS and Flex Soft) have been proposed that aim at minimizing the surgical aspect of intracochlear trauma. The concomitant application local of drugs should enhance tissue tolerability and thus reduce intracochlear damage on a cellular level. Acute and topical, intracochlear drug delivery prior to electrode array insertion with a disposable single-use catheter (Med-El) has been evaluated and developed. The flexible properties of this catheter are enough to be inserted without trauma in the cochlea. Sequential insertion of intracochlear catheters and electrode arrays is feasible and often atraumatic

INTRODUÇÃO: As Respostas Auditivas de Estado Estável permitem avaliação frequência específica em intensidades de até 120 dB NA e a detecção de audição residual em pacientes com perda auditiva severo-profunda. O objetivo deste estudo é comparar os limiares à RAEE e os resultados da avaliação comportamental em crianças com suspeita de surdez severo-profunda. MÉTODO: Estudo transversal para comparar respostas à RAEE e por audiometria com reforço visual (VRA) em 63 crianças candidatas ao implante coclear (126 orelhas) com idade entre 6 e 72 meses. Foram incluídas crianças com otomicroscopia normal, ausência de respostas ao PEATE clique a 90 dB NA e às emissões otoacústicas. Foram excluídas crianças com malformações de orelha interna, doenças do espectro da neuropatia auditiva, ou que não completaram a avaliação comportamental ou não atingiram ruído eletroencefalográfico < 30 nV durante a RAEE. Foram utilizados estímulos com tons contínuos sinusoidais (100% AM e 20% FM) nas frequências de 500, 1000, 2000 e 4000 Hz em intensidade máxima de 110 dB NA. Os limiares à VRA foram obtidos por tom warble nas frequências de 500, 1000, 2000 e 4000 Hz em cada orelha através de fones de inserção (ER-5A) ou tipo casco (TDH-39). A intensidade máxima de estimulação foi de 120 dB NA em cada frequência. RESULTADOS: Limiares comportamentais foram obtidos em 36,7% (185/504) de todas as frequências em todas as crianças, 9% em intensidade maior que 110 dB NA. Entre as 504 medidas da RAEE em 63 indivíduos, 53 limiares foram obtidos (10,5%). Ao todo, 89,5% das frequências testadas não apresentaram nenhuma resposta em 110 dB NA. A distribuição dos limiares à RAEE foi semelhante à da avaliação comportamental. A maioria das respostas foram em 500 Hz, diminuindo nas frequências agudas. A diferença média entre os limiares à VRA e à RAEE variou entre 0,09 e 8,94 dB. Foram realizadas 27 comparações entre RAEE e VRA: 12 em 500 Hz, 9 em 1000 Hz, 5 em 2000 Hz e 1 em 4000 Hz. Respostas ausentes foram observadas em ambos os testes em 38,1% em 0,5 KHz, 52,45% em 1 KHz, 74,6% em 2 KHz e 81,0% em 4 KHZ. A especificidade foi > 90% em 1, 2 e 4 KHz. Nas orelhas sem resposta comportamental em 120 dB NA, todos os limiares à RAEE estavam na faixa de perda profunda, 90% deles > 110 dB NA. CONCLUSÃO: A ausência de respostas nas altas intensidades na RAEE foi o principal achado (especificidade > 90%) o que prediz limiares comportamentais na faixa de surdez profunda / Introduction and Objective: ASSR allows frequency-specific evaluation in intensities up to 120 dB HL and detection of residual hearing in patients with severe-toprofound hearing loss. The aim of this study was to compare ASSR thresholds and behavioral test results in children with suspected severe-to-profound hearing loss. Methods: A cross sectional study was carried out to compare ASSR and Visual Reinforcement Audiometry (VRA) responses in 63 pediatric cochlear implant candidates (126 ears) aged between 6 to 72 months. We included children with normal otomicroscopy findings, absent responses to click-ABR at 90 dB HL and otoaccoustic emissions. We excluded children with inner ear malformations, auditory neuropathy spectrum disorder or who did not complete VRA or achieve EEG noise < 30 nV during the ASSR test. Air-conduction ASSR stimuli were continuous sinusoidal tones (100% AM and 20% FM) presented at 0.5, 1, 2 and 4 kHz starting at the maximum presentation level of 110 dB HL. VRA thresholds were acquired with warble tones presented at 0.5, 1, 2 and 4 KHz in each ear through ER-tone 5A or TDH-39 phones. Maximum presentation level was 120 dB HL for each frequency. Results: Behavioral thresholds were obtained in 36.7% (185/504) of all frequencies in all subjects, 9% were in intensities > 110 dB HL. Among 504 ASSR measurements from 63 subjects, 53 thresholds were obtained (10.5%). Overall 89.5% of the tested frequencies did not show any response at 110 dB HL. The distribution of ASSR responses was similar to the behavioral test results. Most responses were at 500 Hz, decreasing among the higher frequencies. Mean differences between behavioral and ASSR thresholds varied from 0.09 to 8.94 dB. Overall, 27 comparisons of behavioral and ASSR thresholds were obtained: 12 at 0.5 KHz, 9 at 1 KHz, 5 at 2 KHz and 1 at 4 KHz. Absent responses were observed in both tests in 38.1% at 0.5 KHz, 52.4% at 1 KHz, 74.6% at 2 KHz and 81.0% at 4 KHz. The specificity was > 90% at 1, 2 and 4 KHz. In ears with no behavioral response at 120 dB HL all ASSR thresholds were in the profound hearing loss range, 90% of them were equal or > than 110 dB HL. Conclusion: Among 63 pediatric CI candidates, absent responses to high-intensity ASSR was the major finding (specificity > 90%) predicting behavioral thresholds in the profound hearing loss range. These findings can be helpful to confirm the decision for cochlear implantation

INTRODUÇÃO: As Respostas Auditivas de Estado Estável permitem avaliação frequência específica em intensidades de até 120 dB NA e a detecção de audição residual em pacientes com perda auditiva severo-profunda. O objetivo deste estudo é comparar os limiares à RAEE e os resultados da avaliação comportamental em crianças com suspeita de surdez severo-profunda. MÉTODO: Estudo transversal para comparar respostas à RAEE e por audiometria com reforço visual (VRA) em 63 crianças candidatas ao implante coclear (126 orelhas) com idade entre 6 e 72 meses. Foram incluídas crianças com otomicroscopia normal, ausência de respostas ao PEATE clique a 90 dB NA e às emissões otoacústicas. Foram excluídas crianças com malformações de orelha interna, doenças do espectro da neuropatia auditiva, ou que não completaram a avaliação comportamental ou não atingiram ruído eletroencefalográfico < 30 nV durante a RAEE. Foram utilizados estímulos com tons contínuos sinusoidais (100% AM e 20% FM) nas frequências de 500, 1000, 2000 e 4000 Hz em intensidade máxima de 110 dB NA. Os limiares à VRA foram obtidos por tom warble nas frequências de 500, 1000, 2000 e 4000 Hz em cada orelha através de fones de inserção (ER-5A) ou tipo casco (TDH-39). A intensidade máxima de estimulação foi de 120 dB NA em cada frequência. RESULTADOS: Limiares comportamentais foram obtidos em 36,7% (185/504) de todas as frequências em todas as crianças, 9% em intensidade maior que 110 dB NA. Entre as 504 medidas da RAEE em 63 indivíduos, 53 limiares foram obtidos (10,5%). Ao todo, 89,5% das frequências testadas não apresentaram nenhuma resposta em 110 dB NA. A distribuição dos limiares à RAEE foi semelhante à da avaliação comportamental. A maioria das respostas foram em 500 Hz, diminuindo nas frequências agudas. A diferença média entre os limiares à VRA e à RAEE variou entre 0,09 e 8,94 dB. Foram realizadas 27 comparações entre RAEE e VRA: 12 em 500 Hz, 9 em 1000 Hz, 5 em 2000 Hz e 1 em 4000 Hz. Respostas ausentes foram observadas em ambos os testes em 38,1% em 0,5 KHz, 52,45% em 1 KHz, 74,6% em 2 KHz e 81,0% em 4 KHZ. A especificidade foi > 90% em 1, 2 e 4 KHz. Nas orelhas sem resposta comportamental em 120 dB NA, todos os limiares à RAEE estavam na faixa de perda profunda, 90% deles > 110 dB NA. CONCLUSÃO: A ausência de respostas nas altas intensidades na RAEE foi o principal achado (especificidade > 90%) o que prediz limiares comportamentais na faixa de surdez profunda / Introduction and Objective: ASSR allows frequency-specific evaluation in intensities up to 120 dB HL and detection of residual hearing in patients with severe-toprofound hearing loss. The aim of this study was to compare ASSR thresholds and behavioral test results in children with suspected severe-to-profound hearing loss. Methods: A cross sectional study was carried out to compare ASSR and Visual Reinforcement Audiometry (VRA) responses in 63 pediatric cochlear implant candidates (126 ears) aged between 6 to 72 months. We included children with normal otomicroscopy findings, absent responses to click-ABR at 90 dB HL and otoaccoustic emissions. We excluded children with inner ear malformations, auditory neuropathy spectrum disorder or who did not complete VRA or achieve EEG noise < 30 nV during the ASSR test. Air-conduction ASSR stimuli were continuous sinusoidal tones (100% AM and 20% FM) presented at 0.5, 1, 2 and 4 kHz starting at the maximum presentation level of 110 dB HL. VRA thresholds were acquired with warble tones presented at 0.5, 1, 2 and 4 KHz in each ear through ER-tone 5A or TDH-39 phones. Maximum presentation level was 120 dB HL for each frequency. Results: Behavioral thresholds were obtained in 36.7% (185/504) of all frequencies in all subjects, 9% were in intensities > 110 dB HL. Among 504 ASSR measurements from 63 subjects, 53 thresholds were obtained (10.5%). Overall 89.5% of the tested frequencies did not show any response at 110 dB HL. The distribution of ASSR responses was similar to the behavioral test results. Most responses were at 500 Hz, decreasing among the higher frequencies. Mean differences between behavioral and ASSR thresholds varied from 0.09 to 8.94 dB. Overall, 27 comparisons of behavioral and ASSR thresholds were obtained: 12 at 0.5 KHz, 9 at 1 KHz, 5 at 2 KHz and 1 at 4 KHz. Absent responses were observed in both tests in 38.1% at 0.5 KHz, 52.4% at 1 KHz, 74.6% at 2 KHz and 81.0% at 4 KHz. The specificity was > 90% at 1, 2 and 4 KHz. In ears with no behavioral response at 120 dB HL all ASSR thresholds were in the profound hearing loss range, 90% of them were equal or > than 110 dB HL. Conclusion: Among 63 pediatric CI candidates, absent responses to high-intensity ASSR was the major finding (specificity > 90%) predicting behavioral thresholds in the profound hearing loss range. These findings can be helpful to confirm the decision for cochlear implantation