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Early experience and health related quality of life outcomes following auditory brainstem implantation in children
International Journal of Pediatric Otorhinolaryngology 113 (2018) 140–149
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International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Early experience and health related quality of life outcomes following auditory brainstem implantation in children☆
T
Leena Asfour, David R. Friedmann∗, William H. Shapiro, J. Thomas Roland Jr., Susan B. Waltzman New York University School of Medicine, 550 1st Ave, NY, NY 10016, USA
A R T I C LE I N FO
A B S T R A C T
Keywords: Auditory brainstem implantation Cochlear nerve deficiency Health related quality of life Quality of life Speech perception Pediatric Cochlear implantation
Objective: To assess auditory brainstem implant (ABI) outcomes in children with a prospective study. Methods: Twelve patients with cochlear nerve deficiency received an auditory brainstem implant. Patients were evaluated with age appropriate speech perception and production assessments, and health related quality of life (HRQoL) surveys for parents of subjects and for subjects if age appropriate. Results: Twelve patients received an ABI without major complications. Eleven out of twelve received some auditory benefit from their ABI. Parental HRQoL ratings were positive for all domains with the exception of communication. Self reported overall HRQoL metrics from two subjects were also positive. Conclusions: ABI is a good option for patients who are not eligible for or fail CI. Our findings show that despite varying degrees of postoperative performance, HRQoL ratings were positive. The presence of additional disabilities and health problems resulted in less positive HRQoL outcomes. Our results emphasize the need to assess outcomes in these patients beyond speech perception and communication.
1. Introduction Over the last several decades, cochlear implants (CI) have been used as a treatment for individuals with profound hearing loss often with tremendous benefit in speech and communication abilities [1]. Some with congenital profound hearing loss are not candidates for cochlear implantation because of absent cochleae or deficient cochlear nerves. The auditory brainstem implant (ABI) was initially designed for use in Neurofibromatosis 2 (NF2) patients with bilateral vestibular schwannomas and loss of their cochlear nerves [1]. Of patients undergoing ABI surgery in the US, 81% acquire auditory sensations [1]. At our center, we have implanted 40 deafened NF2 adults with an ABI. Adequate data from 31 patients, reveals 21 patients have sound detection, of which 9 have closed set discrimination and 2 have some open set speech perception. More recently, the ABI is being explored as a treatment option for pediatric patients with cochlear nerve deficiency, bilateral cochlear ossification, and absent or severely malformed cochleae; conditions that preclude benefit from a CI. Centers with approval for investigational use of this device have published data concluding that ABI surgery is safe although outcomes have been variable [2–6]. In one study, 29 out
of 35 children who received ABI had closed set word discrimination and 12 had open set discrimination above 50% [7]. A study by Colletti et al. reported that all 21 patients with cochlear nerve deficiency who initially failed CI and went on to ABI achieved environmental awareness and responded to speech sounds. Of the 21, 41% achieved open set speech perception [8]. In one United States institution, four pediatric patients implanted with an ABI achieved environmental sound awareness. One patient had spontaneous device failure. Another patient had device failure due to blunt trauma, a revision ABI and device failure a second time due to blunt trauma [3]. At another center in the United States, only one out five patients is frequently responding to environmental stimuli at the one-year post implantation stage [2]. While speech and hearing outcomes are a core part of ABI evaluation, they give a limited picture of a subject's outcomes. Hearing loss impacts psychosocial aspects of a person's life, such as communication, self-esteem and social relationships [9]. CI literature has explored these domains by creating CI specific tools to measure health related quality of life (HRQoL). HRQoL is defined as an individual's perceived mental and physical health and has become an increasingly important way of measuring outcomes and value of health interventions.
☆ ∗
This study was reviewed and approved by the institutional review board of New York University School of Medicine. Corresponding author. Skirball Suite 7q, 550 1st AveNY, NY 10016, USA. E-mail address: [email protected] (D.R. Friedmann).
https://doi.org/10.1016/j.ijporl.2018.07.037 Received 20 March 2018; Received in revised form 16 July 2018; Accepted 22 July 2018 Available online 24 July 2018 0165-5876/ © 2018 Published by Elsevier B.V.
International Journal of Pediatric Otorhinolaryngology 113 (2018) 140–149
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were programmed to provide optimal auditory stimulation without non-auditory side effects.
In attempt to better encapsulate outcomes in other domains in ABI pediatric patients, we collected data from the subjects' families regarding the HRQoL of their child following ABI. We used a validated CI parental survey to collect the data to report HRQoL data on this population.
2.4. Hearing and speech and language outcomes Post-operative auditory function was evaluated using behavioral audiometry and commonly used, developmentally appropriate measures of speech perception, speech production and language. Subjects were evaluated at 1, 3, 6, 12, 18, 24, 30, and 36-month intervals using one or more of the following measures when appropriate. (1) The Infant-Toddler Meaningful Auditory Integration of Sound (IT-MAIS) is a parent report scale for children under the age of four years where parents report their child's communication abilities [12]. (2) The Ling Six Sound test presents speech sounds (ah, oo, ee, sh, s, m) via live voice and assesses the subject's ability to detect and discriminate the sounds [13]. (3) The Early Speech Perception (ESP) test assesses pattern perception, spondaic word identification and closed-set monosyllabic word identification. Subjects are placed in one of four categories, ranging from detection to consistent word identification [14]. (4) The Glendonald Auditory Screening Procedure (GASP) uses phonemes, words or sentences to assess identification and comprehension in a closed set [15]. (5) The Common Phrases test assesses the ability of subjects to repeat a phrase or at least the key word in a phrase given to them in an open set [16]. (6) The Minimal Auditory Capabilities (MAC) battery is a modified open set speech perception test that evaluates the subject's ability to distinguish the difference between noise/voice, male/female and same/different sounds [17]. (7) The Oral and Written Language Scales (OWLS) is a speech and language evaluation that assesses both comprehension and expression of language for subjects aged 3–21 years [18]. Results are compared to normative results in age-matched peers without hearing loss.
2. Materials and methods This study was reviewed and approved by the institutional review board of New York University School of Medicine. Informed consent was obtained from the parents of all subjects. 2.1. Inclusion criteria Patients ranged in age from 18 months to 18 years. Preoperative evaluation included MRI with or without a CT that demonstrated at least one of the following: cochlear nerve deficiency, cochlear aplasia or severe hypoplasia, severe inner ear malformation or post-meningitis ossification precluding CI. If the patients had a cochlea, a CI was placed first, given that imaging modalities cannot adequately predict CI performance in patients with cochlear nerve deficiency [10]. Patients that did not derive benefit from or progress with the CI after six months of consistent use were evaluated for ABI. Patients without a cochlea bilaterally did not need to receive a CI first. 2.2. Subject demographics Patients were evaluated from other institutional referrals or from patients at our center with suspected cochlear nerve deficiency who did not benefit from their CI. Subjects were evaluated by a multidisciplinary ABI team to determine their baseline capabilities and eligibility. Twelve pediatric patients were determined to be eligible for an ABI and their families underwent extensive counseling. Six patients were male and six were female. Their ages at implantation ranged from 22 months to 17 years, with the mean age at five years. All patients had bilateral profound hearing loss. Patent demographics are outlined in Table 1.
2.5. HRQoL measures from parents/caretakers To collect data on HRQoL, we adapted the Children with Cochlear Implants: Parental Perspectives survey, a validated HRQoL tool for pediatric CI patients [19–21]. The survey consists of 74 questions and is completed by the parents of CI recipients. Some questions address one of eight HRQoL domains: communication, general functioning, self-reliance, well-being, social interactions, education, effects of implantation and supporting the child. The rest of the questions address the process of implantation and decision-making. The survey questions are answered on a 5 point Likert scale (ie Strongly agree, Agree, Neither agree nor disagree, Disagree, Strongly Disagree). Although the survey was designed for parents of CI recipients, the majority of the questions were relevant to families of ABI recipients. Some questions were determined to be not suitable to our population or relevant to our implant center and were excluded; they are listed in Appendix 1. The final survey was 65 questions, reproduced in Appendix 2. The survey were distributed at a single point in time, such that parents filled out the surveys based on differing periods of device use and experience since their child's implantation. To analyze the surveys, responses to positive statements were ranked 1–5 and responses to negative statements were ranked on a reverse scale. The average response to questions within a domain was determined for each subject.
2.3. Surgical implantation The pre operative evaluation consisted of a thorough medical evaluation, a high resolution MRI of the brain and internal auditory canals. An electrically evoked auditory brainstem response (EABR) exam under anesthesia was done in cases where an absent cranial nerve was suspected and there were no auditory responses on behavioral testing. A retrosigmoid approach was used in all pediatric subjects and the surgical team consisted of our multi-disciplinary skull base team (neurosurgery and neurotology) as well as a pediatric neurosurgeon through a technique previously published [11]. Intraoperatively, facial and lower cranial nerves were monitored. The Nucleus ABI541 was used in all patients. If the ABI was to be implanted on the same side as a previous CI, the CI was explanted during the same operation. The cochlear nucleus was identified and the device was placed. An EABR was used to optimize the position of the electrodes and assess for non-auditory responses. Postoperatively, all patients were admitted to the pediatric intensive care unit (PICU) for at least 24 h and were transferred to the regular floor when appropriate. A non-contrast CT scan was obtained prior to discharge to evaluate device placement, and screen for postoperative hydrocephalus or intracranial hematomas. Patients were followed closely for any complications. Initial device activation was performed under general anesthesia in the operating room with cranial nerve monitoring between 3 and 7 weeks following implantation. Electrodes that produced an EABR without non-auditory side effects were noted. Additional stimulation sessions were performed in subsequent days in our clinic. The devices
2.6. HRQoL measures from pediatric subjects To collect self-reported HRQoL data from the subjects, the Kid KINDLR Questionnaire for Measuring Health-Related Quality of Life for children aged 8–11-year olds was used. It is an established HRQoL questionnaire that has been tested for reliability, validity and internal consistency [22]. It has 24 questions and covers six subscales: physical well-being, emotional well-being, self-esteem, family, friends, and school. The questions are answered on a 5 point Likert scale (i.e. very often, often, sometimes, seldom, never). An overall score and a score for 141
142
Questionable R CN VIII. No L CN VIII.
Ventricular septal defect, Atrial septal defect
Partial mastoidectomy
Goldenhar Syndrome. Delayed walking due to balance issues and low tone. Microtia left ear. Bilateral PE tube placement. Autistic features Bilateral CI at 20 mo
CHARGE, cleft lip and palate
L CI at 13 mo; removed after 3 mo for infection (HA at 9 mo) Pontine hypoplasia, abnormally developed cochlea bilaterally, and absent cochlear nerve bilaterally
Failed
Failed
Passed
Right dysplastic vestibular apparatus with absence of normal cochlea. Absent R CN VIII. Left complete aplasia of inner ear structures. Right dysplastic cochlea, left cochlea absent. Bilateral absence of vestibule and semicircular canals. 2 neural structures in R cisternal space, one structre in IAC. 1 neural structure in L cisternal space and IAC. Bilateral hypoplastic IAC with no cochlear nerve. Dysplastic lateral semicircular canals and vestibules. Bilateral CN VIII bundle but no nerve at the modiolus of the cochlea. Absent cochlea and CN VIII bilaterally. Dysplastic vestibule and stenotic IACs bilaterally. Dysplastic cochlea and vestibular labyrinth bilaterally. Thin R CN VIII and no L CN VIII. Left cochlear aplasia, vestibular dysplasia and severe labyrinthine hypoplasia. Right labyrinthine aplasia. CN VIII and CN VII not clearly distinguished bilaterally. 1.5 cm arachnoid cyst in right cerebellopontine angle. R CN VIII emerges from brainstem but stenotic R IAC appears to only contain CNVII.
N/A
IACs stenotic bilaterally. CN VIII not seen well at the brain stem. IACs are filled with CSF; normal cochlea.
none R CI at 28 mo; 1st revision at 8 y; 2nd revision at 9 y (HA at 26 mo)
L CI at 28 mo
R CI at 12 mo
none
R CI at 14 mo (HA at 6 mo)
HA at 4 mo
R CI at 14 mo and L CI at 23 mo (HA at 12 mo)
L CI at 41 mo (HA at 30 mo)
none
none
none
Hydrocephalus at 10 mo, torticollis
Duane's Retraction Syndrome, Marcus Gunn Syndrome, vetebrae/rib anomalies, patent ductus arteriousus, mitral valve prolapse
Passed
Female Ex term, normal pregnancy and delivery; NICU for 2 wks.
17 y
S12
Autism, perinatal hypothyroidism, gross motor delay, developmental delay, acid reflux, repaired bilateral strabismus.
N/A
Female N/A, patient was adopted at age 2.5 y
9y
S11
none
Passed
Female Ex term, normal pregnancy and delivery
5y
S10
Factor XI deficiency, myringotomy tubes
Failed
Failed
Female Ex term, normal pregnancy and delivery
5y
S9
Failed
Male Ex term, uncomplicated twin pregnancy, C- section
4y
S8
Failed
2y
S7
Female 29 wks gestation, twin gestation with intrauterine growth restriction and prenatal vascular shunting to the brain. 11 wks in NICU, required CPAP, blood transfusions and phototherapy. Failed
1y
S6
Male Ex term, born in breech position; NICU for 2 days.
Male Ex term, normal pregnancy and delivery
4y
S5
L CI at 22 mo
Male N/A
Failed
Female NICU for 5 days after birth for hypoglycemia and jaundice.
Male Ex term, Csection, low tone at birth, NICU for 2 wks.
Male Ex term, Csection, Norwood procedure, Gtube, Glenn procedure.
4y
S4
2y
2y
S3
18 mo
S2
Abbreviations y, years; R, right; L, left; N/A, not available; wk, week; CSF, cerebrospinal fluid. CN, cranial nerve; mo, month; IAC, internal auditory canal; HA, hearing aides; CI, cochlear implant; NICU, neonatal intensive care unit.
Imaging Findings
Prior interventions
Results of Newborn hearing screening Other Medical History
Preoperative Age at evaluation Gender Birth History
S1
Table 1 Patient demographics and preoperative data.
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International Journal of Pediatric Otorhinolaryngology 113 (2018) 140–149
143
4.5 y 4y 2.5 y
none
3y
Abbreviations: y, years; R, right; L, left; N/A, not available; wk, week; CSF, cerebrospinal fluid. CN, cranial nerve; mo, month; VP, ventriculoperitoneal.
2.5 y 7 mo 3y 2.5 y
none
CSF leak at 3 wks post op managed with left frontal tunneled ventriculostomy, VP shunt (6wks post op). 2y Postoperative
23 mo Right Atrophic CN VIII Age at Operation Side Intraoperative nerve findings at brainstem
Perioperative
Table 2 Peri- and postoperative data.
2 y 8 mo Right Absent CN VIII
The parental survey was completed and returned by the parents of ten out of the twelve implanted patients for a response rate of 83%. Surveys were not completed for S11 and S12. The grand mean for subjects in each HRQoL domain is plotted in Fig. 1. The average rating for each domain was greater than three on the five-point scale for all domains except communication. This indicates that overall, parents rated their child's HRQoL either as average or on the positive side of the scale for seven out of eight domains. Parents rated supporting the child (M = 3.69, SD = 0.58, range 3–4.67), well-being (M = 3.67, SD = 0.60, range = 2.6–4.67), and effects of implantation (M = 3.62, SD = 0.31, range = 3.14–4.14) the most positively. Education (M = 3.55, SD = 0.65, range = 2.43–4.43), self-reliance (M = 3.48, SD = 0.67, range 2.5–4.25), and social relations (M = 3.43, SD = 0.56, range = 2.2–4) were also rated positively. General functioning (M = 3.12, SD = 0.75, range 2–4) and communication (M = 2.60, SD = 1.22, range = 1–4.4) domains produced the lowest ratings. A breakdown of the means for each subject in each domain is shown in Table 4. Means that fell below the average rating of three are bolded and starred. For the domains, effects of implantation and supporting the child, no parent rated the HRQoL of their child below average. In the
Duration of ABI Use
3.2. Parental Perspectives survey
none
2 y 11 mo Left Absent CN VIII; CN VII appeared to be running with CN V and entering the petrous bone through an anomalous root.
none
4 y 11 mo Right N/A
4y Right Absent CN VIII
CSF leak, resolved with compression dressing.
22 mo Right 1.5 cm arachnoid cyst anteriorly displaced CN VII and CN III complex.
CSF leak at 2 wks post op after fall at home; surgically repaired. 5y
none
4y Right CN VIII present.
5y Right Absent CN VIII
CSF leak at 1 wk post op, managed with lumbar drain and mastoid dressing. 2y
5y Left Thin CN VIII
Twelve patients underwent ABI surgery. Preoperative data is outlined for each subject in Table 1. All subjects had cochlear nerve deficiency. S6, S8 and S12 also had cochlear aplasia and did not have a CI trial. S1, S2 and S7 had complicated additional medical history or developmental difficulties. Nine patients trialed and failed a CI prior to ABI. The patients were determined to fail CI when they failed to make auditory or language progress on appropriate assessments after using the CI for at least 6 months. Perioperative data is outlined in Table 2. The age of implantation ranged from 22 months to 17 years, with the mean age at five years. Imaging findings correlated with intraoperative findings at the brainstem in 8 out of 11 cases. Postoperative data is outlined in Table 2. The length of hospital stay was less than four days in all cases except for S1 who required further treatment secondary to his complicated cardiac conditions and S11 for prolonged postoperative ileus. Four subjects had pseudomeningoceles or CSF leaks; one was managed with mastoid compression dressings and the other three required further procedures, specified in Table 2. There were no other observed complications. Devices were activated between 3 and 7 weeks after the ABI surgery. Non-auditory side effects occurred in two out of twelve subjects. Table 3 outlines the status of auditory progress for all subjects. Eleven out of the twelve subjects obtained auditory sound awareness from their ABI. S5, S10, S11 and S12 perform with > 50% accuracy on open set speech perception tests. S9 and S8 perform with > 50% accuracy on closed set word discrimination. S1, S4, S6 and S7 can detect the 6 Ling sounds. S3 does not consistently respond to environmental stimuli but does use the device. S2 does not demonstrate objective benefit from the ABI.
none
6y Left Absent CN VIII
3.1. Surgical data and perception outcomes
Complications
3. Results
none
9y Right Atrophic CN VIII
Prolonged post op ileus
17 y Right CN VIII stump. Abnormal flocculus. Brainstem posteriorly placed and recessed in the cerebellopontine angle.
each subscale is calculated. The scores are reported from 0 to 100, where 0 represents the lowest and 100 represents the highest HRQoL score. While the KINDLR survey has developed disease specific modules, none exists for children with CIs. A preliminary six question CI specific subscale was created by Warner-Czyz et al. [23]. The questions are relevant to ABI recipients as well and were included in the Kid KINDLR survey. The responses were converted to a score on a 100-point scale using the same method given for the generic Kid KINDLR subscales. The full survey used in our Kid KINDLR survey is reproduced in Appendix 3.
5y
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144
Minimal Auditory Capabilities (MAC) Battery AD (ABI): Noise/Voice: 57% Same/ Different: 50% Male/Female: 60% Common Phrases: Key Word 80% (16/ 20) Phrase 70% (7/ 10) Oral and Written Language Scales 2(OWLS-2): percentile rank amongst age matched peers Listening comprehension: 12 Oral Expression: 47 Oral Language composite: 32 Common Phrases: Key Word 100% Phrase 100% Early Speech Perception Test: Category 2 (Pattern Perception) GASP Words: Audio: 40% Audiovisual: 83% Early Speech Perception Test: Category 2 (Pattern Perception)
Detects Ling sounds. Inconsistent detection of environmental sounds.
Detects Ling sounds. Early Speech Perception Test Category 1 (No pattern perception).
Scored 80% on open set speech testing using a bisyllabic word list at 2.5 y.
2 y, 35
100% Ling sound detection at 30–40 dB. 80% discrimination. Produced his first word at 4.5 mo post op.
4 y, 20
No consistent responses to auditory stimuli; does not like to remove device. 1 y IT MAIS = 17/40
2 y, 25
No consistent responses to auditory stimuli.
1 y, 25
S1 takes device off. Aware of speech and nonspeech stimuli. Detects ling sounds. 6 mo IT Mais = 9
2 y, 30
2 y, 40
2 y, 40
4 y, 10
4 y, 45
1 y, 25
6 mo, 25
1.5 y, 10
N/A
2 y, 15
2 y, 60
2 y, 35
S12
4 y, 45
S11
N/A
S10
N/A
S9
2 y, 60
S8
2.5 y, 60
S7
6 mo, 35
S6
2.5 y, 60
S5
6 mo, 45
S4
Abbreviationsy, years; N/A, not available; mo, month; PTA, pure tone audiometry; dB, decibels.
Visit period; lowest PTA, dB Visit period; lowest speech detection threshold, dB Status of auditory and speech and language progress
S3
S2
S1
Table 3 Status of auditory progress.
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International Journal of Pediatric Otorhinolaryngology 113 (2018) 140–149
International Journal of Pediatric Otorhinolaryngology 113 (2018) 140–149
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Fig. 1. Average parental ratings of HRQoL across eight domains.
4. Summary and discussion
education, well-being and social interaction domains, only one or two subjects (S1 and/or S2) reported below average means. Communication, general functioning and self-reliance had the most subjects with means below average. Four patients (S4, S5, S8 and S10) had average or above average ratings for all HRQoL domains. The results for the survey questions pertaining to the process of implantation and decision-making are displayed in Table 5. The most common responses are bolded. In general, parents agreed that having a positive attitude and being well informed were an important part of the implantation process. Almost all the parents proceeded with the implant in hopes that their child would become part of the hearing world.
Eleven out of the twelve subjects receive varying amounts of subjective and objective benefit from their ABI. The auditory outcomes for subjects ranged from no environmental sound awareness in one case to four cases of open set speech perception. No patients had any long-term complications. Our results are in agreement with other studies, which have found pediatric ABI surgery to be safe among experienced adult NF2 teams and provide varying degrees of auditory input to patients. Overall, parents rated HRQoL as positive for seven out of eight domains. Examination of the HRQoL data in context of each subject's audiologic outcomes and other health issues reveal some patterns. S1, S2 and S7, who had additional disabilities, were the only subjects that had three or more domain ratings less than 3 out of 5, indicating a below average rating. Given that HRQoL intends to capture physical and mental health perceptions, this is not surprising. S4, S5, S8 and S10 all produced parental HRQoL ratings above 3 in all domains. While S10 has open set speech perception, S4 is still developing his ability to accurately detect and discriminate the six Ling sounds. This finding may indicate that positive HRQoL outcomes may be evident even when speech perception outcomes are poor. Comparison of the parental ratings in CI recipients reveals some differences. The communication, social relations and self-reliance domains received the most positive ratings for CI patients across several studies [24]. In contrast communication was the most poorly rated domain for
3.3. Kid KINDLR survey Of the three subjects who were old enough to complete the Kid KINDLR survey, two completed and returned it (response rate 66%). The sub scale scores, overall generic HRQoL score and ABI specific score are displayed for S9 and S10 in Fig. 2. Scores were greater than 50 in all cases with the exception of S9 regarding her ABI specific QoL, which she rated at 45/100. Both subjects scored similarly in each of the categories with the exception of school. Importantly, S9 attends a school for the deaf, while S10 attends public school.
Table 4 Mean parental rating of HRQoL for each subject in each domain.
Communication General Functioning Self reliance Well being Social Interactions Education Effects of Implantation Supporting the Child
S1
S2
S3
S4
S5
S6
S7
S8
S9
S10
1* 2* 2.75* 2.6* 3 2.43* 3.14 3
1.2* 2.17* 2.5* 3.2 2.2* 2.86* 3.5 3.17
2* 2.33* 3.75 3.8 3.67 4.29 3.29 3.5
3 3.5 4 4.67 3.5 3.17 3.67 4.4
4.4 3.67 4.25 3.8 4 3.86 4.14 4.67
2.2* 4 4.25 3.6 3.83 3.43 4 3.83
1.6* 2.67* 2.5* 3 3 3.14 3.71 3
3.4 3.33 3.5 3.8 3.6 3.86 3.5 4
2.8* 3.67 3.5 4 3.5 4 3.43 3.33
4.4 3.83 3.75 4.2 4 4.43 3.86 4
Means rated below average are bolded and starred. 145
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Table 5 Parental ratings of process of decision-making and implantation.
S22 S24 S25 S28 S34 S36 S38 S40 S42 S43 S46 S49 S54 S55 S57 S58 S60 S63 S68
Question
Strongly Agree
Agree
Neither agree nor disagree
Disagree
Strongly Disagree
Access to the implant center is a problem. Signing support is helpful for a considerable time after implantation. I wish to participate in meetings with other families who have an implanted child. Making the decision to proceed with the implantation was the most difficult part for me. We feel the need for advice from the implant center concerning his future. We are reliant on the implant center for technical advice about his implant. The process of implantation was no more intrusive than expected. It is essential that he is encouraged to wear the processor all the time. A positive attitude is a great help towards successful use of the implant. Regular checking of the implant system is essential. Before proceeding with implantation, parents should obtain as much information and advice as possible. It was useful to speak to another family with an implanted child before deciding on an implant. I am confident that the long term electrical stimulation will not be a problem. The whole process of implantation is sill stressful. I worry that ultimately he may be neither part of the deaf nor the hearing world. It was important to me that my child could hear sounds from traffic for safety reasons. I believe now that my child will have reasonable prospects for employment. It has been hard to take time off work for the appointments at the implant center. I chose implantation for my child so he would have a chance to become part of the hearing world.
0 4 0 1 1 2 1 5 8 6 8
0 5 7 4 6 6 5 4 1 4 2
2 0 2 1 2 1 2 0 1 0 0
3 0 1 4 1 1 1 0 0 0 0
5 1 0 0 0 0 0 1 0 0 0
5
3
2
0
0
1 1 1 4 2 0 6
3 4 0 6 4 4 2
6 0 4 0 3 1 1
0 5 4 0 0 5 0
0 0 1 0 1 0 0
Looking at self-reported HRQoL in ABI and studies of CI populations, Warner-Czyz et al. reported a mean overall Kid KINDLR score of 75.4 (SD = 9.2) and a mean CI module specific score of 77.8 for a sample of 54 patients [24]. In comparison, our two subjects scored below the overall Kid KINDLR average of their age matched CI peers with scores of 63.5 and 72.9. The ABI specific module scores appear to be much lower when compared to CI peers with scores of 45 and 60. This may indicate that HRQoL outcomes are not as positive in ABI recipients as CI recipients, however a larger sample size is needed for a meaningful comparison. Families of patients who have undergone ABI are heavily invested emotionally in their outcomes. Unlike, CI, the surgical procedure requires a neurosurgical procedure with nontrivial risk of serious injury or death. As such, subjective reports of benefit are likely to be subject to reporter bias and a sincere desire on their part to demonstrate that their efforts have benefited their child. Our study is limited due to low power. A more rigorous statistical analysis of the results is not meaningful without a larger sample size.
ABI patients. The least positive ratings amongst CI patients were in the domains of education, effects of implantation and supporting the child24. Amongst ABI parents, effects of implantation and supporting the child were two of the three most highly rated domains. This may be related to the expectations of the ABI parents. A crucial step in the ABI process is extensive counseling regarding realistic expectations. All patients were provided with this counseling prior to proceeding. Despite this, eight out of nine parents strongly agreed or agreed that they choose the ABI in hopes that their child would become a part of the hearing world. It is unclear if this is a realistic expectation even in the most carefully chosen candidates. However given that effect of implantation is highly rated, parents seem to view any auditory benefit as a positive outcome from HRQoL perspective. This underlies the fact that communication, the very reason patients undergo interventions such as CI, need to be viewed differently when considering the broader benefits of ABI. So too does our way of measuring success in such patients and QoL may be one such area to further explore these domains that are not accessible through traditional means of evaluating patients.
Fig. 2. Self reported ratings of six generic HRQoL subscales, overall generic HRQoL, and ABI specific HRQoL. 146
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to be somewhat more positive. HRQoL ratings are lower for subjects with additional disabilities and while the limits of this technology in this population continue to be explored, confounding can be mitigated by not considering ABI in those with additional disabilities until clear benefit is demonstrated consistently in appropriate candidates.
Moreover comparison of outcomes between subjects and with their age match CI peers is difficult given the small sample size and age range. Developmentally appropriate evaluations change frequently with age and capabilities. 5. Conclusions
Conflicts of interest An ABI may be considered in those children without other apparent disabilities who do not benefit from a cochlear implant or cannot receive one because of anatomic constraints. Appropriate preoperative counseling as to possible outcomes is imperative so that parents can make an informed choice. Our cohort demonstrates a range of auditory outcomes, with all but one receiving some benefit. While we found limited auditory and linguistic benefit from ABI, HRQoL measures seem
Dr. Roland is on the Cochlear Americas Surgical Advisory Board and Dr. Shapiro is on the Cochlear Americas Audiological Advisory Board. Acknowledgements This work was supported in part by the Children's Hearing Institute.
Appendix 1. Questions excluded from the Children with Cochlear Implants: Parental Perspectives
S5 S10 S13 S21 S27 S29 S44 S52 S56
He or she does not have a close relationship with her grandparents. It has been a problem getting someone to look after the family when we go to the implant center. The program at the implant center should emphasize speaking and listening. Only experienced teams should carry out cochlear implantation. We can now chat even when he or she cannot see my face (for example in the car or in the dark) It was a difficult time waiting for the results of the assessments before implantation. At least one visit per year by the implant center staff to home/school is essential. The important factor in choosing an implant device is its reliability. I expected him or her to learn to talk once he or she had his or her implant.
Appendix 2. Questions used in the Children with Cochlear Implants: Parental Perspectives
S1 S2 S3 S4 S6 S7 S8 S9 S11 S12 S14 S15 S16 S17 S18 S19 S20 S22 S23 S24 S25 S26 S28 S30 S31 S32 S33 S34 S35 S36 S37 S38 S39 S40
Communication is difficult even with people he knows well. Immediately after implantation his ability to communicate was poorer. The help I give him has become more productive now that he has his implant. Before implantation he obtained no benefit at all from other hearing devices (i.e. cochlear implant, hearing aids). He is totally reliant on his implant all the time. He knows when I want his attention because he can hear me call. I worry the implant will break down. He is unable to cope with mainstream schooling. Progress during the first few months seemed very slow. I can seldom leave him to do something on his own. I worry that he will blame me for my decision for him to have an implant. He has needed more help from me since he received his implant. He still shows signs of frustration in his behavior. I am concerned that my child will be rejected by the deaf community because of the implant. The quality of his speech gives me cause for concern. A lot of help at first means a child needs less help later. I get more time to myself because of his increased independence. Access to the implant center is a problem. He is keeping up well with the children of his own age at school. Signing support is helpful for a considerable time after implantation. I wish to participate in meetings with other families who have an implanted child. Progress after implantation has exceeded my expectations. Making the decision to proceed with the implantation was the most difficult part for me. He was socially isolated before getting his implant. The local school and support services adequately meet all our needs concerning the use of his implant at school. A significant change has been improvement in his confidence. He was very dependent on us before the implantation. We feel the need for advice from the implant center concerning his future. He can now entertain himself listening to music or watching TV or playing games. We are reliant on the implant center for technical advice about his implant. I am concerned about his future school placement. The process of implantation was no more intrusive than expected. He does not make friends easily outside of the family. It is essential that he is encouraged to wear the processor all the time.
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S41 S42 S43 S45 S46 S47 S48 S49 S50 S51 S53 S54 S55 S57 S58 S59 S60 S61 S62 S63 S64 S65 S66 S67 S68 S69 S70 S71 S72 S73 S74
He is sociable within the family. A positive attitude is a great help towards successful use of the implant. Regular checking of the implant system is essential. He shares in family situations more than before implantation. Before proceeding with implantation, parents should obtain as much information and advice as possible. He is as independent as most children his age. Parents should have a choice in the use of sign language at school. It was useful to speak to another family with an implanted child before deciding on an implant. I am so happy about his progress at school. I can now let him play outside as he is aware of the sound of traffic. He is still unable to cope in new situations. I am confident that the long term electrical stimulation will not be a problem. The whole process of implantation is still stressful. I worry that ultimately he may be neither part of the deaf nor the hearing world. It was important to me that my child could hear sounds from traffic for safety reasons. His behavior has improved since he had his implant. I believe now that my child will have reasonable prospects for employment. He has become argumentative since getting his implant. A parent of a child with an implant needs to be patient as the benefits may take time to show. It has been hard to take time off work for the appointments at the implant center. He is less frustrated than before he had the implant. He takes part in family relationships on an equal footing with other members. I find it easier to communicate with him by speaking than signing. I give the same amount of help as before his implant. I chose implantation for my child so he would have a chance to become part of the hearing world. He is totally reliant on his implant at school. He continues to be a happy child and good fun to be with. His use of spoken language has developed greatly. Now he is talkative and engages others in conversation. Other children in the family resented the time and attention taken up by the implant (Tick here if there are no other children.) His relationship with his siblings has improved. (Tick here if there are no other children.)
Appendix 3. Questions used in the Kid KINDLR
Physical Health During the past week, I felt ill. During the past week, I had a headache or tummy ache. During the past week, I was tired and worn-out. During the past week, I felt strong and full of energy. General feeling During the past week, I had fun and laughed a lot. During the past week, I was bored. During the past week, I felt alone. During the past week, I was scared. Feelings About Self During the past week, I was proud of myself. During the past week, I felt on top of the world. During the past week, I felt pleased with myself. During the past week, I had lots of good ideas. Family During the past week, I got on well with my parents. During the past week, I felt fine at home. During the past week, we quarreled at home. During the past week, my parents stopped me from doing certain things. Friends During the past week, I played with friends. During the past week, other kids liked me. During the past week, I got along well with my friends. During the past week, I felt different from other children. School During the last week in which I was at school, doing my schoolwork was easy. During the last week in which I was at school, I enjoyed my lessons. During the last week in which I was at school, I worried about my future. During the last week in which I was at school, I worried about bad marks or grades. 148
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ABI During During During During During
the the the the the
last last last last last
week, week, week, week, week,
how how how how how
often often often often often
did did did did did
you wear your implant? your implant bother you? you like the way you looked with your implant? your friends think your implant was cool? you hear everything you needed to?
[11] S.E. Heman-Ackah, M.K. Cosetti, S. Gupta, J.G. Golfinos, J.T. Roland, Retrosigmoid approach to cerebellopontine angle tumor resection: surgical modifications, Laryngoscope 122 (11) (2012) 2519–2523. [12] S. Zimmerman-Phillips, A.M. Robbins, M.J. Osberger, Assessing cochlear implant benefit in very young children, Ann. Otol. Rhinol. Laryngol. 185 (2000) 42–43. [13] D. Ling, The Six-sound Test, The Listener, 2002/2003, pp. 52–53. [14] J.S. Moog, A.E. Geers, Early Speech Perception Test for Profoundly Hearing- impaired Children, Central Institute for the Deaf, St. Louis, 1990. [15] N.P. Erber, Glendonald Auditory Screening Procedure, Auditory training, 1982, pp. 47–71. [16] A.M. Robbins, J.J. Renshaw, M.J. Osberger, Common Phrases Test, Indiana University School of Medicine, Indiannapolis, 1995. [17] E. Owens, D. Kessler, C. Telleen, E. Schubert, The minimal auditory capabilities (MAC) battery, Hear. Aid J. 9 (1981) 32. [18] E. Carrow-Woolfolk, The Oral and Written Language Scales (OWLS), Pro-Ed, Austin, 2007. [19] S.M. Archbold, M.E. Lutman, S. Gregory, et al., Parents and their deaf child: their perceptions 3 years after cochlear implantation, Deaf. Educ. Int. 4 (2002) 12–40. [20] S.M. Archbold, T.P. Nikolopoulos, M. Tait, et al., Approach to communication, speech perception and intelligibility after paediatric cochlear implantation, Br. J. Audiol. 34 (2000) 257–264. [21] S.M. Archbold, T.H. Sach, C. O'Neill, et al., Outcomes from cochlear implantation for child and family: parental Perspectives, Deaf. Educ. Int. 10 (2008) 120–142. [22] U. Ravens-Sieberer, M. Bullinger, Kindl-r English: Questionnaire for Measuring Health-related Quality of Life in Children and Adolescents, Revised Version, (2000). [23] A.D. Warner-Czyz, B. Loy, E.A. Tobey, P. Nakonezny, P.S. Roland, Health-related quality of life in children and adolescents who use cochlear implants, Int. J. Pediatr. Otorhinolaryngol. 75 (1) (2011) 95–105. [24] R. Kumar, A. Warner-Czyz, C.H. Silver, B. Loy, E. Tobey, American parent perspectives on quality of life in pediatric cochlear implant recipients, Ear Hear. 36 (2) (2015) 269–278.
References [1] J.T. Roland, S.B. Waltzman, Cochlear Implants, Thieme, New York, 2014. [2] E.P. Wilkinson, L.S. Eisenberg, M.D. Krieger, M.S. Schwartz, M. Winter, J.L. Glater, A.S. Martinez, L.M. Fisher, R.V. Shannon, Initial results of a safety and feasibility study of auditory brainstem implantation in congenitally deaf children, Otol. Neurotol. 38 (2017) 212–220. [3] S.V. Puram, S.R. Barber, E.D. Kozin, P. Shah, A. Remenschneider, B.S. Herrmann, A.C. Duhaime, F.G. Barker, D.J. Lee, Outcomes following pediatric auditory brainstem implant surgery, Otolaryngology-head Neck Surg. (Tokyo) 155 (2016) 133–138. [4] P.V. Shah, E.D. Kozin, A.B. Kaplan, D.J. Lee, Pediatric auditory brainstem implant surgery: a New option for auditory habilitation in congenital deafness? J. Am. Board Fam. Med. 29 (2016) 286–288. [5] S.V. Puram, D.J. Lee, Pediatric auditory brainstem implant surgery, Otolaryngol. Clin. 48 (2015) 1117–1148. [6] K.S. Noij, E.D. Kozin, R. Sethi, P.V. Shah, A.B. Kaplan, B. Herrmann, A. Remenschneider, D.J. Lee, Systematic review of nontumor pediatric auditory brainstem implant outcomes, Otolaryngology-head Neck Surg. (Tokyo) 153 (2015) 739–750. [7] L. Sennaroğlu, G. Sennaroğlu, E. Yücel, B. Bilginer, G. Atay, M.D. Bajin, B.Ö. Mocan, M. Yaral, F. Aslan, B.Ç. Çnar, et al., Long-term results of ABI in children with severe inner ear malformations, Otol. Neurotol. 37 (2016) 865–872. [8] L. Colletti, E.P. Wilkinson, V. Colletti, Auditory brainstem implantation after unsuccessful cochlear implantation of children with clinical diagnosis of cochlear nerve deficiency, Ann. Otol. Rhinol. Laryngol. 122 (10) (2013) 605–612. [9] J.G. Nicholas, A.E. Geers, Personal, social, and family adjustment in school-aged children with a cochlear implant, Ear Hear. 24 (1 Suppl) (2003) 69S–81S. [10] N.M. Young, F.M. Kim, M.E. Ryan, E. Tournis, S. Yaras, Pediatric cochlear implantation of children with eighth nerve deficiency, Int. J. Pediatr. Otorhinolaryngol. 76 (2012) 1442–1448.
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Contents lists available at ScienceDirect
International Journal of Pediatric Otorhinolaryngology journal homepage: www.elsevier.com/locate/ijporl
Early experience and health related quality of life outcomes following auditory brainstem implantation in children☆
T
Leena Asfour, David R. Friedmann∗, William H. Shapiro, J. Thomas Roland Jr., Susan B. Waltzman New York University School of Medicine, 550 1st Ave, NY, NY 10016, USA
A R T I C LE I N FO
A B S T R A C T
Keywords: Auditory brainstem implantation Cochlear nerve deficiency Health related quality of life Quality of life Speech perception Pediatric Cochlear implantation
Objective: To assess auditory brainstem implant (ABI) outcomes in children with a prospective study. Methods: Twelve patients with cochlear nerve deficiency received an auditory brainstem implant. Patients were evaluated with age appropriate speech perception and production assessments, and health related quality of life (HRQoL) surveys for parents of subjects and for subjects if age appropriate. Results: Twelve patients received an ABI without major complications. Eleven out of twelve received some auditory benefit from their ABI. Parental HRQoL ratings were positive for all domains with the exception of communication. Self reported overall HRQoL metrics from two subjects were also positive. Conclusions: ABI is a good option for patients who are not eligible for or fail CI. Our findings show that despite varying degrees of postoperative performance, HRQoL ratings were positive. The presence of additional disabilities and health problems resulted in less positive HRQoL outcomes. Our results emphasize the need to assess outcomes in these patients beyond speech perception and communication.
1. Introduction Over the last several decades, cochlear implants (CI) have been used as a treatment for individuals with profound hearing loss often with tremendous benefit in speech and communication abilities [1]. Some with congenital profound hearing loss are not candidates for cochlear implantation because of absent cochleae or deficient cochlear nerves. The auditory brainstem implant (ABI) was initially designed for use in Neurofibromatosis 2 (NF2) patients with bilateral vestibular schwannomas and loss of their cochlear nerves [1]. Of patients undergoing ABI surgery in the US, 81% acquire auditory sensations [1]. At our center, we have implanted 40 deafened NF2 adults with an ABI. Adequate data from 31 patients, reveals 21 patients have sound detection, of which 9 have closed set discrimination and 2 have some open set speech perception. More recently, the ABI is being explored as a treatment option for pediatric patients with cochlear nerve deficiency, bilateral cochlear ossification, and absent or severely malformed cochleae; conditions that preclude benefit from a CI. Centers with approval for investigational use of this device have published data concluding that ABI surgery is safe although outcomes have been variable [2–6]. In one study, 29 out
of 35 children who received ABI had closed set word discrimination and 12 had open set discrimination above 50% [7]. A study by Colletti et al. reported that all 21 patients with cochlear nerve deficiency who initially failed CI and went on to ABI achieved environmental awareness and responded to speech sounds. Of the 21, 41% achieved open set speech perception [8]. In one United States institution, four pediatric patients implanted with an ABI achieved environmental sound awareness. One patient had spontaneous device failure. Another patient had device failure due to blunt trauma, a revision ABI and device failure a second time due to blunt trauma [3]. At another center in the United States, only one out five patients is frequently responding to environmental stimuli at the one-year post implantation stage [2]. While speech and hearing outcomes are a core part of ABI evaluation, they give a limited picture of a subject's outcomes. Hearing loss impacts psychosocial aspects of a person's life, such as communication, self-esteem and social relationships [9]. CI literature has explored these domains by creating CI specific tools to measure health related quality of life (HRQoL). HRQoL is defined as an individual's perceived mental and physical health and has become an increasingly important way of measuring outcomes and value of health interventions.
☆ ∗
This study was reviewed and approved by the institutional review board of New York University School of Medicine. Corresponding author. Skirball Suite 7q, 550 1st AveNY, NY 10016, USA. E-mail address: [email protected] (D.R. Friedmann).
https://doi.org/10.1016/j.ijporl.2018.07.037 Received 20 March 2018; Received in revised form 16 July 2018; Accepted 22 July 2018 Available online 24 July 2018 0165-5876/ © 2018 Published by Elsevier B.V.
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were programmed to provide optimal auditory stimulation without non-auditory side effects.
In attempt to better encapsulate outcomes in other domains in ABI pediatric patients, we collected data from the subjects' families regarding the HRQoL of their child following ABI. We used a validated CI parental survey to collect the data to report HRQoL data on this population.
2.4. Hearing and speech and language outcomes Post-operative auditory function was evaluated using behavioral audiometry and commonly used, developmentally appropriate measures of speech perception, speech production and language. Subjects were evaluated at 1, 3, 6, 12, 18, 24, 30, and 36-month intervals using one or more of the following measures when appropriate. (1) The Infant-Toddler Meaningful Auditory Integration of Sound (IT-MAIS) is a parent report scale for children under the age of four years where parents report their child's communication abilities [12]. (2) The Ling Six Sound test presents speech sounds (ah, oo, ee, sh, s, m) via live voice and assesses the subject's ability to detect and discriminate the sounds [13]. (3) The Early Speech Perception (ESP) test assesses pattern perception, spondaic word identification and closed-set monosyllabic word identification. Subjects are placed in one of four categories, ranging from detection to consistent word identification [14]. (4) The Glendonald Auditory Screening Procedure (GASP) uses phonemes, words or sentences to assess identification and comprehension in a closed set [15]. (5) The Common Phrases test assesses the ability of subjects to repeat a phrase or at least the key word in a phrase given to them in an open set [16]. (6) The Minimal Auditory Capabilities (MAC) battery is a modified open set speech perception test that evaluates the subject's ability to distinguish the difference between noise/voice, male/female and same/different sounds [17]. (7) The Oral and Written Language Scales (OWLS) is a speech and language evaluation that assesses both comprehension and expression of language for subjects aged 3–21 years [18]. Results are compared to normative results in age-matched peers without hearing loss.
2. Materials and methods This study was reviewed and approved by the institutional review board of New York University School of Medicine. Informed consent was obtained from the parents of all subjects. 2.1. Inclusion criteria Patients ranged in age from 18 months to 18 years. Preoperative evaluation included MRI with or without a CT that demonstrated at least one of the following: cochlear nerve deficiency, cochlear aplasia or severe hypoplasia, severe inner ear malformation or post-meningitis ossification precluding CI. If the patients had a cochlea, a CI was placed first, given that imaging modalities cannot adequately predict CI performance in patients with cochlear nerve deficiency [10]. Patients that did not derive benefit from or progress with the CI after six months of consistent use were evaluated for ABI. Patients without a cochlea bilaterally did not need to receive a CI first. 2.2. Subject demographics Patients were evaluated from other institutional referrals or from patients at our center with suspected cochlear nerve deficiency who did not benefit from their CI. Subjects were evaluated by a multidisciplinary ABI team to determine their baseline capabilities and eligibility. Twelve pediatric patients were determined to be eligible for an ABI and their families underwent extensive counseling. Six patients were male and six were female. Their ages at implantation ranged from 22 months to 17 years, with the mean age at five years. All patients had bilateral profound hearing loss. Patent demographics are outlined in Table 1.
2.5. HRQoL measures from parents/caretakers To collect data on HRQoL, we adapted the Children with Cochlear Implants: Parental Perspectives survey, a validated HRQoL tool for pediatric CI patients [19–21]. The survey consists of 74 questions and is completed by the parents of CI recipients. Some questions address one of eight HRQoL domains: communication, general functioning, self-reliance, well-being, social interactions, education, effects of implantation and supporting the child. The rest of the questions address the process of implantation and decision-making. The survey questions are answered on a 5 point Likert scale (ie Strongly agree, Agree, Neither agree nor disagree, Disagree, Strongly Disagree). Although the survey was designed for parents of CI recipients, the majority of the questions were relevant to families of ABI recipients. Some questions were determined to be not suitable to our population or relevant to our implant center and were excluded; they are listed in Appendix 1. The final survey was 65 questions, reproduced in Appendix 2. The survey were distributed at a single point in time, such that parents filled out the surveys based on differing periods of device use and experience since their child's implantation. To analyze the surveys, responses to positive statements were ranked 1–5 and responses to negative statements were ranked on a reverse scale. The average response to questions within a domain was determined for each subject.
2.3. Surgical implantation The pre operative evaluation consisted of a thorough medical evaluation, a high resolution MRI of the brain and internal auditory canals. An electrically evoked auditory brainstem response (EABR) exam under anesthesia was done in cases where an absent cranial nerve was suspected and there were no auditory responses on behavioral testing. A retrosigmoid approach was used in all pediatric subjects and the surgical team consisted of our multi-disciplinary skull base team (neurosurgery and neurotology) as well as a pediatric neurosurgeon through a technique previously published [11]. Intraoperatively, facial and lower cranial nerves were monitored. The Nucleus ABI541 was used in all patients. If the ABI was to be implanted on the same side as a previous CI, the CI was explanted during the same operation. The cochlear nucleus was identified and the device was placed. An EABR was used to optimize the position of the electrodes and assess for non-auditory responses. Postoperatively, all patients were admitted to the pediatric intensive care unit (PICU) for at least 24 h and were transferred to the regular floor when appropriate. A non-contrast CT scan was obtained prior to discharge to evaluate device placement, and screen for postoperative hydrocephalus or intracranial hematomas. Patients were followed closely for any complications. Initial device activation was performed under general anesthesia in the operating room with cranial nerve monitoring between 3 and 7 weeks following implantation. Electrodes that produced an EABR without non-auditory side effects were noted. Additional stimulation sessions were performed in subsequent days in our clinic. The devices
2.6. HRQoL measures from pediatric subjects To collect self-reported HRQoL data from the subjects, the Kid KINDLR Questionnaire for Measuring Health-Related Quality of Life for children aged 8–11-year olds was used. It is an established HRQoL questionnaire that has been tested for reliability, validity and internal consistency [22]. It has 24 questions and covers six subscales: physical well-being, emotional well-being, self-esteem, family, friends, and school. The questions are answered on a 5 point Likert scale (i.e. very often, often, sometimes, seldom, never). An overall score and a score for 141
142
Questionable R CN VIII. No L CN VIII.
Ventricular septal defect, Atrial septal defect
Partial mastoidectomy
Goldenhar Syndrome. Delayed walking due to balance issues and low tone. Microtia left ear. Bilateral PE tube placement. Autistic features Bilateral CI at 20 mo
CHARGE, cleft lip and palate
L CI at 13 mo; removed after 3 mo for infection (HA at 9 mo) Pontine hypoplasia, abnormally developed cochlea bilaterally, and absent cochlear nerve bilaterally
Failed
Failed
Passed
Right dysplastic vestibular apparatus with absence of normal cochlea. Absent R CN VIII. Left complete aplasia of inner ear structures. Right dysplastic cochlea, left cochlea absent. Bilateral absence of vestibule and semicircular canals. 2 neural structures in R cisternal space, one structre in IAC. 1 neural structure in L cisternal space and IAC. Bilateral hypoplastic IAC with no cochlear nerve. Dysplastic lateral semicircular canals and vestibules. Bilateral CN VIII bundle but no nerve at the modiolus of the cochlea. Absent cochlea and CN VIII bilaterally. Dysplastic vestibule and stenotic IACs bilaterally. Dysplastic cochlea and vestibular labyrinth bilaterally. Thin R CN VIII and no L CN VIII. Left cochlear aplasia, vestibular dysplasia and severe labyrinthine hypoplasia. Right labyrinthine aplasia. CN VIII and CN VII not clearly distinguished bilaterally. 1.5 cm arachnoid cyst in right cerebellopontine angle. R CN VIII emerges from brainstem but stenotic R IAC appears to only contain CNVII.
N/A
IACs stenotic bilaterally. CN VIII not seen well at the brain stem. IACs are filled with CSF; normal cochlea.
none R CI at 28 mo; 1st revision at 8 y; 2nd revision at 9 y (HA at 26 mo)
L CI at 28 mo
R CI at 12 mo
none
R CI at 14 mo (HA at 6 mo)
HA at 4 mo
R CI at 14 mo and L CI at 23 mo (HA at 12 mo)
L CI at 41 mo (HA at 30 mo)
none
none
none
Hydrocephalus at 10 mo, torticollis
Duane's Retraction Syndrome, Marcus Gunn Syndrome, vetebrae/rib anomalies, patent ductus arteriousus, mitral valve prolapse
Passed
Female Ex term, normal pregnancy and delivery; NICU for 2 wks.
17 y
S12
Autism, perinatal hypothyroidism, gross motor delay, developmental delay, acid reflux, repaired bilateral strabismus.
N/A
Female N/A, patient was adopted at age 2.5 y
9y
S11
none
Passed
Female Ex term, normal pregnancy and delivery
5y
S10
Factor XI deficiency, myringotomy tubes
Failed
Failed
Female Ex term, normal pregnancy and delivery
5y
S9
Failed
Male Ex term, uncomplicated twin pregnancy, C- section
4y
S8
Failed
2y
S7
Female 29 wks gestation, twin gestation with intrauterine growth restriction and prenatal vascular shunting to the brain. 11 wks in NICU, required CPAP, blood transfusions and phototherapy. Failed
1y
S6
Male Ex term, born in breech position; NICU for 2 days.
Male Ex term, normal pregnancy and delivery
4y
S5
L CI at 22 mo
Male N/A
Failed
Female NICU for 5 days after birth for hypoglycemia and jaundice.
Male Ex term, Csection, low tone at birth, NICU for 2 wks.
Male Ex term, Csection, Norwood procedure, Gtube, Glenn procedure.
4y
S4
2y
2y
S3
18 mo
S2
Abbreviations y, years; R, right; L, left; N/A, not available; wk, week; CSF, cerebrospinal fluid. CN, cranial nerve; mo, month; IAC, internal auditory canal; HA, hearing aides; CI, cochlear implant; NICU, neonatal intensive care unit.
Imaging Findings
Prior interventions
Results of Newborn hearing screening Other Medical History
Preoperative Age at evaluation Gender Birth History
S1
Table 1 Patient demographics and preoperative data.
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4.5 y 4y 2.5 y
none
3y
Abbreviations: y, years; R, right; L, left; N/A, not available; wk, week; CSF, cerebrospinal fluid. CN, cranial nerve; mo, month; VP, ventriculoperitoneal.
2.5 y 7 mo 3y 2.5 y
none
CSF leak at 3 wks post op managed with left frontal tunneled ventriculostomy, VP shunt (6wks post op). 2y Postoperative
23 mo Right Atrophic CN VIII Age at Operation Side Intraoperative nerve findings at brainstem
Perioperative
Table 2 Peri- and postoperative data.
2 y 8 mo Right Absent CN VIII
The parental survey was completed and returned by the parents of ten out of the twelve implanted patients for a response rate of 83%. Surveys were not completed for S11 and S12. The grand mean for subjects in each HRQoL domain is plotted in Fig. 1. The average rating for each domain was greater than three on the five-point scale for all domains except communication. This indicates that overall, parents rated their child's HRQoL either as average or on the positive side of the scale for seven out of eight domains. Parents rated supporting the child (M = 3.69, SD = 0.58, range 3–4.67), well-being (M = 3.67, SD = 0.60, range = 2.6–4.67), and effects of implantation (M = 3.62, SD = 0.31, range = 3.14–4.14) the most positively. Education (M = 3.55, SD = 0.65, range = 2.43–4.43), self-reliance (M = 3.48, SD = 0.67, range 2.5–4.25), and social relations (M = 3.43, SD = 0.56, range = 2.2–4) were also rated positively. General functioning (M = 3.12, SD = 0.75, range 2–4) and communication (M = 2.60, SD = 1.22, range = 1–4.4) domains produced the lowest ratings. A breakdown of the means for each subject in each domain is shown in Table 4. Means that fell below the average rating of three are bolded and starred. For the domains, effects of implantation and supporting the child, no parent rated the HRQoL of their child below average. In the
Duration of ABI Use
3.2. Parental Perspectives survey
none
2 y 11 mo Left Absent CN VIII; CN VII appeared to be running with CN V and entering the petrous bone through an anomalous root.
none
4 y 11 mo Right N/A
4y Right Absent CN VIII
CSF leak, resolved with compression dressing.
22 mo Right 1.5 cm arachnoid cyst anteriorly displaced CN VII and CN III complex.
CSF leak at 2 wks post op after fall at home; surgically repaired. 5y
none
4y Right CN VIII present.
5y Right Absent CN VIII
CSF leak at 1 wk post op, managed with lumbar drain and mastoid dressing. 2y
5y Left Thin CN VIII
Twelve patients underwent ABI surgery. Preoperative data is outlined for each subject in Table 1. All subjects had cochlear nerve deficiency. S6, S8 and S12 also had cochlear aplasia and did not have a CI trial. S1, S2 and S7 had complicated additional medical history or developmental difficulties. Nine patients trialed and failed a CI prior to ABI. The patients were determined to fail CI when they failed to make auditory or language progress on appropriate assessments after using the CI for at least 6 months. Perioperative data is outlined in Table 2. The age of implantation ranged from 22 months to 17 years, with the mean age at five years. Imaging findings correlated with intraoperative findings at the brainstem in 8 out of 11 cases. Postoperative data is outlined in Table 2. The length of hospital stay was less than four days in all cases except for S1 who required further treatment secondary to his complicated cardiac conditions and S11 for prolonged postoperative ileus. Four subjects had pseudomeningoceles or CSF leaks; one was managed with mastoid compression dressings and the other three required further procedures, specified in Table 2. There were no other observed complications. Devices were activated between 3 and 7 weeks after the ABI surgery. Non-auditory side effects occurred in two out of twelve subjects. Table 3 outlines the status of auditory progress for all subjects. Eleven out of the twelve subjects obtained auditory sound awareness from their ABI. S5, S10, S11 and S12 perform with > 50% accuracy on open set speech perception tests. S9 and S8 perform with > 50% accuracy on closed set word discrimination. S1, S4, S6 and S7 can detect the 6 Ling sounds. S3 does not consistently respond to environmental stimuli but does use the device. S2 does not demonstrate objective benefit from the ABI.
none
6y Left Absent CN VIII
3.1. Surgical data and perception outcomes
Complications
3. Results
none
9y Right Atrophic CN VIII
Prolonged post op ileus
17 y Right CN VIII stump. Abnormal flocculus. Brainstem posteriorly placed and recessed in the cerebellopontine angle.
each subscale is calculated. The scores are reported from 0 to 100, where 0 represents the lowest and 100 represents the highest HRQoL score. While the KINDLR survey has developed disease specific modules, none exists for children with CIs. A preliminary six question CI specific subscale was created by Warner-Czyz et al. [23]. The questions are relevant to ABI recipients as well and were included in the Kid KINDLR survey. The responses were converted to a score on a 100-point scale using the same method given for the generic Kid KINDLR subscales. The full survey used in our Kid KINDLR survey is reproduced in Appendix 3.
5y
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Minimal Auditory Capabilities (MAC) Battery AD (ABI): Noise/Voice: 57% Same/ Different: 50% Male/Female: 60% Common Phrases: Key Word 80% (16/ 20) Phrase 70% (7/ 10) Oral and Written Language Scales 2(OWLS-2): percentile rank amongst age matched peers Listening comprehension: 12 Oral Expression: 47 Oral Language composite: 32 Common Phrases: Key Word 100% Phrase 100% Early Speech Perception Test: Category 2 (Pattern Perception) GASP Words: Audio: 40% Audiovisual: 83% Early Speech Perception Test: Category 2 (Pattern Perception)
Detects Ling sounds. Inconsistent detection of environmental sounds.
Detects Ling sounds. Early Speech Perception Test Category 1 (No pattern perception).
Scored 80% on open set speech testing using a bisyllabic word list at 2.5 y.
2 y, 35
100% Ling sound detection at 30–40 dB. 80% discrimination. Produced his first word at 4.5 mo post op.
4 y, 20
No consistent responses to auditory stimuli; does not like to remove device. 1 y IT MAIS = 17/40
2 y, 25
No consistent responses to auditory stimuli.
1 y, 25
S1 takes device off. Aware of speech and nonspeech stimuli. Detects ling sounds. 6 mo IT Mais = 9
2 y, 30
2 y, 40
2 y, 40
4 y, 10
4 y, 45
1 y, 25
6 mo, 25
1.5 y, 10
N/A
2 y, 15
2 y, 60
2 y, 35
S12
4 y, 45
S11
N/A
S10
N/A
S9
2 y, 60
S8
2.5 y, 60
S7
6 mo, 35
S6
2.5 y, 60
S5
6 mo, 45
S4
Abbreviationsy, years; N/A, not available; mo, month; PTA, pure tone audiometry; dB, decibels.
Visit period; lowest PTA, dB Visit period; lowest speech detection threshold, dB Status of auditory and speech and language progress
S3
S2
S1
Table 3 Status of auditory progress.
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Fig. 1. Average parental ratings of HRQoL across eight domains.
4. Summary and discussion
education, well-being and social interaction domains, only one or two subjects (S1 and/or S2) reported below average means. Communication, general functioning and self-reliance had the most subjects with means below average. Four patients (S4, S5, S8 and S10) had average or above average ratings for all HRQoL domains. The results for the survey questions pertaining to the process of implantation and decision-making are displayed in Table 5. The most common responses are bolded. In general, parents agreed that having a positive attitude and being well informed were an important part of the implantation process. Almost all the parents proceeded with the implant in hopes that their child would become part of the hearing world.
Eleven out of the twelve subjects receive varying amounts of subjective and objective benefit from their ABI. The auditory outcomes for subjects ranged from no environmental sound awareness in one case to four cases of open set speech perception. No patients had any long-term complications. Our results are in agreement with other studies, which have found pediatric ABI surgery to be safe among experienced adult NF2 teams and provide varying degrees of auditory input to patients. Overall, parents rated HRQoL as positive for seven out of eight domains. Examination of the HRQoL data in context of each subject's audiologic outcomes and other health issues reveal some patterns. S1, S2 and S7, who had additional disabilities, were the only subjects that had three or more domain ratings less than 3 out of 5, indicating a below average rating. Given that HRQoL intends to capture physical and mental health perceptions, this is not surprising. S4, S5, S8 and S10 all produced parental HRQoL ratings above 3 in all domains. While S10 has open set speech perception, S4 is still developing his ability to accurately detect and discriminate the six Ling sounds. This finding may indicate that positive HRQoL outcomes may be evident even when speech perception outcomes are poor. Comparison of the parental ratings in CI recipients reveals some differences. The communication, social relations and self-reliance domains received the most positive ratings for CI patients across several studies [24]. In contrast communication was the most poorly rated domain for
3.3. Kid KINDLR survey Of the three subjects who were old enough to complete the Kid KINDLR survey, two completed and returned it (response rate 66%). The sub scale scores, overall generic HRQoL score and ABI specific score are displayed for S9 and S10 in Fig. 2. Scores were greater than 50 in all cases with the exception of S9 regarding her ABI specific QoL, which she rated at 45/100. Both subjects scored similarly in each of the categories with the exception of school. Importantly, S9 attends a school for the deaf, while S10 attends public school.
Table 4 Mean parental rating of HRQoL for each subject in each domain.
Communication General Functioning Self reliance Well being Social Interactions Education Effects of Implantation Supporting the Child
S1
S2
S3
S4
S5
S6
S7
S8
S9
S10
1* 2* 2.75* 2.6* 3 2.43* 3.14 3
1.2* 2.17* 2.5* 3.2 2.2* 2.86* 3.5 3.17
2* 2.33* 3.75 3.8 3.67 4.29 3.29 3.5
3 3.5 4 4.67 3.5 3.17 3.67 4.4
4.4 3.67 4.25 3.8 4 3.86 4.14 4.67
2.2* 4 4.25 3.6 3.83 3.43 4 3.83
1.6* 2.67* 2.5* 3 3 3.14 3.71 3
3.4 3.33 3.5 3.8 3.6 3.86 3.5 4
2.8* 3.67 3.5 4 3.5 4 3.43 3.33
4.4 3.83 3.75 4.2 4 4.43 3.86 4
Means rated below average are bolded and starred. 145
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Table 5 Parental ratings of process of decision-making and implantation.
S22 S24 S25 S28 S34 S36 S38 S40 S42 S43 S46 S49 S54 S55 S57 S58 S60 S63 S68
Question
Strongly Agree
Agree
Neither agree nor disagree
Disagree
Strongly Disagree
Access to the implant center is a problem. Signing support is helpful for a considerable time after implantation. I wish to participate in meetings with other families who have an implanted child. Making the decision to proceed with the implantation was the most difficult part for me. We feel the need for advice from the implant center concerning his future. We are reliant on the implant center for technical advice about his implant. The process of implantation was no more intrusive than expected. It is essential that he is encouraged to wear the processor all the time. A positive attitude is a great help towards successful use of the implant. Regular checking of the implant system is essential. Before proceeding with implantation, parents should obtain as much information and advice as possible. It was useful to speak to another family with an implanted child before deciding on an implant. I am confident that the long term electrical stimulation will not be a problem. The whole process of implantation is sill stressful. I worry that ultimately he may be neither part of the deaf nor the hearing world. It was important to me that my child could hear sounds from traffic for safety reasons. I believe now that my child will have reasonable prospects for employment. It has been hard to take time off work for the appointments at the implant center. I chose implantation for my child so he would have a chance to become part of the hearing world.
0 4 0 1 1 2 1 5 8 6 8
0 5 7 4 6 6 5 4 1 4 2
2 0 2 1 2 1 2 0 1 0 0
3 0 1 4 1 1 1 0 0 0 0
5 1 0 0 0 0 0 1 0 0 0
5
3
2
0
0
1 1 1 4 2 0 6
3 4 0 6 4 4 2
6 0 4 0 3 1 1
0 5 4 0 0 5 0
0 0 1 0 1 0 0
Looking at self-reported HRQoL in ABI and studies of CI populations, Warner-Czyz et al. reported a mean overall Kid KINDLR score of 75.4 (SD = 9.2) and a mean CI module specific score of 77.8 for a sample of 54 patients [24]. In comparison, our two subjects scored below the overall Kid KINDLR average of their age matched CI peers with scores of 63.5 and 72.9. The ABI specific module scores appear to be much lower when compared to CI peers with scores of 45 and 60. This may indicate that HRQoL outcomes are not as positive in ABI recipients as CI recipients, however a larger sample size is needed for a meaningful comparison. Families of patients who have undergone ABI are heavily invested emotionally in their outcomes. Unlike, CI, the surgical procedure requires a neurosurgical procedure with nontrivial risk of serious injury or death. As such, subjective reports of benefit are likely to be subject to reporter bias and a sincere desire on their part to demonstrate that their efforts have benefited their child. Our study is limited due to low power. A more rigorous statistical analysis of the results is not meaningful without a larger sample size.
ABI patients. The least positive ratings amongst CI patients were in the domains of education, effects of implantation and supporting the child24. Amongst ABI parents, effects of implantation and supporting the child were two of the three most highly rated domains. This may be related to the expectations of the ABI parents. A crucial step in the ABI process is extensive counseling regarding realistic expectations. All patients were provided with this counseling prior to proceeding. Despite this, eight out of nine parents strongly agreed or agreed that they choose the ABI in hopes that their child would become a part of the hearing world. It is unclear if this is a realistic expectation even in the most carefully chosen candidates. However given that effect of implantation is highly rated, parents seem to view any auditory benefit as a positive outcome from HRQoL perspective. This underlies the fact that communication, the very reason patients undergo interventions such as CI, need to be viewed differently when considering the broader benefits of ABI. So too does our way of measuring success in such patients and QoL may be one such area to further explore these domains that are not accessible through traditional means of evaluating patients.
Fig. 2. Self reported ratings of six generic HRQoL subscales, overall generic HRQoL, and ABI specific HRQoL. 146
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to be somewhat more positive. HRQoL ratings are lower for subjects with additional disabilities and while the limits of this technology in this population continue to be explored, confounding can be mitigated by not considering ABI in those with additional disabilities until clear benefit is demonstrated consistently in appropriate candidates.
Moreover comparison of outcomes between subjects and with their age match CI peers is difficult given the small sample size and age range. Developmentally appropriate evaluations change frequently with age and capabilities. 5. Conclusions
Conflicts of interest An ABI may be considered in those children without other apparent disabilities who do not benefit from a cochlear implant or cannot receive one because of anatomic constraints. Appropriate preoperative counseling as to possible outcomes is imperative so that parents can make an informed choice. Our cohort demonstrates a range of auditory outcomes, with all but one receiving some benefit. While we found limited auditory and linguistic benefit from ABI, HRQoL measures seem
Dr. Roland is on the Cochlear Americas Surgical Advisory Board and Dr. Shapiro is on the Cochlear Americas Audiological Advisory Board. Acknowledgements This work was supported in part by the Children's Hearing Institute.
Appendix 1. Questions excluded from the Children with Cochlear Implants: Parental Perspectives
S5 S10 S13 S21 S27 S29 S44 S52 S56
He or she does not have a close relationship with her grandparents. It has been a problem getting someone to look after the family when we go to the implant center. The program at the implant center should emphasize speaking and listening. Only experienced teams should carry out cochlear implantation. We can now chat even when he or she cannot see my face (for example in the car or in the dark) It was a difficult time waiting for the results of the assessments before implantation. At least one visit per year by the implant center staff to home/school is essential. The important factor in choosing an implant device is its reliability. I expected him or her to learn to talk once he or she had his or her implant.
Appendix 2. Questions used in the Children with Cochlear Implants: Parental Perspectives
S1 S2 S3 S4 S6 S7 S8 S9 S11 S12 S14 S15 S16 S17 S18 S19 S20 S22 S23 S24 S25 S26 S28 S30 S31 S32 S33 S34 S35 S36 S37 S38 S39 S40
Communication is difficult even with people he knows well. Immediately after implantation his ability to communicate was poorer. The help I give him has become more productive now that he has his implant. Before implantation he obtained no benefit at all from other hearing devices (i.e. cochlear implant, hearing aids). He is totally reliant on his implant all the time. He knows when I want his attention because he can hear me call. I worry the implant will break down. He is unable to cope with mainstream schooling. Progress during the first few months seemed very slow. I can seldom leave him to do something on his own. I worry that he will blame me for my decision for him to have an implant. He has needed more help from me since he received his implant. He still shows signs of frustration in his behavior. I am concerned that my child will be rejected by the deaf community because of the implant. The quality of his speech gives me cause for concern. A lot of help at first means a child needs less help later. I get more time to myself because of his increased independence. Access to the implant center is a problem. He is keeping up well with the children of his own age at school. Signing support is helpful for a considerable time after implantation. I wish to participate in meetings with other families who have an implanted child. Progress after implantation has exceeded my expectations. Making the decision to proceed with the implantation was the most difficult part for me. He was socially isolated before getting his implant. The local school and support services adequately meet all our needs concerning the use of his implant at school. A significant change has been improvement in his confidence. He was very dependent on us before the implantation. We feel the need for advice from the implant center concerning his future. He can now entertain himself listening to music or watching TV or playing games. We are reliant on the implant center for technical advice about his implant. I am concerned about his future school placement. The process of implantation was no more intrusive than expected. He does not make friends easily outside of the family. It is essential that he is encouraged to wear the processor all the time.
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S41 S42 S43 S45 S46 S47 S48 S49 S50 S51 S53 S54 S55 S57 S58 S59 S60 S61 S62 S63 S64 S65 S66 S67 S68 S69 S70 S71 S72 S73 S74
He is sociable within the family. A positive attitude is a great help towards successful use of the implant. Regular checking of the implant system is essential. He shares in family situations more than before implantation. Before proceeding with implantation, parents should obtain as much information and advice as possible. He is as independent as most children his age. Parents should have a choice in the use of sign language at school. It was useful to speak to another family with an implanted child before deciding on an implant. I am so happy about his progress at school. I can now let him play outside as he is aware of the sound of traffic. He is still unable to cope in new situations. I am confident that the long term electrical stimulation will not be a problem. The whole process of implantation is still stressful. I worry that ultimately he may be neither part of the deaf nor the hearing world. It was important to me that my child could hear sounds from traffic for safety reasons. His behavior has improved since he had his implant. I believe now that my child will have reasonable prospects for employment. He has become argumentative since getting his implant. A parent of a child with an implant needs to be patient as the benefits may take time to show. It has been hard to take time off work for the appointments at the implant center. He is less frustrated than before he had the implant. He takes part in family relationships on an equal footing with other members. I find it easier to communicate with him by speaking than signing. I give the same amount of help as before his implant. I chose implantation for my child so he would have a chance to become part of the hearing world. He is totally reliant on his implant at school. He continues to be a happy child and good fun to be with. His use of spoken language has developed greatly. Now he is talkative and engages others in conversation. Other children in the family resented the time and attention taken up by the implant (Tick here if there are no other children.) His relationship with his siblings has improved. (Tick here if there are no other children.)
Appendix 3. Questions used in the Kid KINDLR
Physical Health During the past week, I felt ill. During the past week, I had a headache or tummy ache. During the past week, I was tired and worn-out. During the past week, I felt strong and full of energy. General feeling During the past week, I had fun and laughed a lot. During the past week, I was bored. During the past week, I felt alone. During the past week, I was scared. Feelings About Self During the past week, I was proud of myself. During the past week, I felt on top of the world. During the past week, I felt pleased with myself. During the past week, I had lots of good ideas. Family During the past week, I got on well with my parents. During the past week, I felt fine at home. During the past week, we quarreled at home. During the past week, my parents stopped me from doing certain things. Friends During the past week, I played with friends. During the past week, other kids liked me. During the past week, I got along well with my friends. During the past week, I felt different from other children. School During the last week in which I was at school, doing my schoolwork was easy. During the last week in which I was at school, I enjoyed my lessons. During the last week in which I was at school, I worried about my future. During the last week in which I was at school, I worried about bad marks or grades. 148
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ABI During During During During During
the the the the the
last last last last last
week, week, week, week, week,
how how how how how
often often often often often
did did did did did
you wear your implant? your implant bother you? you like the way you looked with your implant? your friends think your implant was cool? you hear everything you needed to?
[11] S.E. Heman-Ackah, M.K. Cosetti, S. Gupta, J.G. Golfinos, J.T. Roland, Retrosigmoid approach to cerebellopontine angle tumor resection: surgical modifications, Laryngoscope 122 (11) (2012) 2519–2523. [12] S. Zimmerman-Phillips, A.M. Robbins, M.J. Osberger, Assessing cochlear implant benefit in very young children, Ann. Otol. Rhinol. Laryngol. 185 (2000) 42–43. [13] D. Ling, The Six-sound Test, The Listener, 2002/2003, pp. 52–53. [14] J.S. Moog, A.E. Geers, Early Speech Perception Test for Profoundly Hearing- impaired Children, Central Institute for the Deaf, St. Louis, 1990. [15] N.P. Erber, Glendonald Auditory Screening Procedure, Auditory training, 1982, pp. 47–71. [16] A.M. Robbins, J.J. Renshaw, M.J. Osberger, Common Phrases Test, Indiana University School of Medicine, Indiannapolis, 1995. [17] E. Owens, D. Kessler, C. Telleen, E. Schubert, The minimal auditory capabilities (MAC) battery, Hear. Aid J. 9 (1981) 32. [18] E. Carrow-Woolfolk, The Oral and Written Language Scales (OWLS), Pro-Ed, Austin, 2007. [19] S.M. Archbold, M.E. Lutman, S. Gregory, et al., Parents and their deaf child: their perceptions 3 years after cochlear implantation, Deaf. Educ. Int. 4 (2002) 12–40. [20] S.M. Archbold, T.P. Nikolopoulos, M. Tait, et al., Approach to communication, speech perception and intelligibility after paediatric cochlear implantation, Br. J. Audiol. 34 (2000) 257–264. [21] S.M. Archbold, T.H. Sach, C. O'Neill, et al., Outcomes from cochlear implantation for child and family: parental Perspectives, Deaf. Educ. Int. 10 (2008) 120–142. [22] U. Ravens-Sieberer, M. Bullinger, Kindl-r English: Questionnaire for Measuring Health-related Quality of Life in Children and Adolescents, Revised Version, (2000). [23] A.D. Warner-Czyz, B. Loy, E.A. Tobey, P. Nakonezny, P.S. Roland, Health-related quality of life in children and adolescents who use cochlear implants, Int. J. Pediatr. Otorhinolaryngol. 75 (1) (2011) 95–105. [24] R. Kumar, A. Warner-Czyz, C.H. Silver, B. Loy, E. Tobey, American parent perspectives on quality of life in pediatric cochlear implant recipients, Ear Hear. 36 (2) (2015) 269–278.
References [1] J.T. Roland, S.B. Waltzman, Cochlear Implants, Thieme, New York, 2014. [2] E.P. Wilkinson, L.S. Eisenberg, M.D. Krieger, M.S. Schwartz, M. Winter, J.L. Glater, A.S. Martinez, L.M. Fisher, R.V. Shannon, Initial results of a safety and feasibility study of auditory brainstem implantation in congenitally deaf children, Otol. Neurotol. 38 (2017) 212–220. [3] S.V. Puram, S.R. Barber, E.D. Kozin, P. Shah, A. Remenschneider, B.S. Herrmann, A.C. Duhaime, F.G. Barker, D.J. Lee, Outcomes following pediatric auditory brainstem implant surgery, Otolaryngology-head Neck Surg. (Tokyo) 155 (2016) 133–138. [4] P.V. Shah, E.D. Kozin, A.B. Kaplan, D.J. Lee, Pediatric auditory brainstem implant surgery: a New option for auditory habilitation in congenital deafness? J. Am. Board Fam. Med. 29 (2016) 286–288. [5] S.V. Puram, D.J. Lee, Pediatric auditory brainstem implant surgery, Otolaryngol. Clin. 48 (2015) 1117–1148. [6] K.S. Noij, E.D. Kozin, R. Sethi, P.V. Shah, A.B. Kaplan, B. Herrmann, A. Remenschneider, D.J. Lee, Systematic review of nontumor pediatric auditory brainstem implant outcomes, Otolaryngology-head Neck Surg. (Tokyo) 153 (2015) 739–750. [7] L. Sennaroğlu, G. Sennaroğlu, E. Yücel, B. Bilginer, G. Atay, M.D. Bajin, B.Ö. Mocan, M. Yaral, F. Aslan, B.Ç. Çnar, et al., Long-term results of ABI in children with severe inner ear malformations, Otol. Neurotol. 37 (2016) 865–872. [8] L. Colletti, E.P. Wilkinson, V. Colletti, Auditory brainstem implantation after unsuccessful cochlear implantation of children with clinical diagnosis of cochlear nerve deficiency, Ann. Otol. Rhinol. Laryngol. 122 (10) (2013) 605–612. [9] J.G. Nicholas, A.E. Geers, Personal, social, and family adjustment in school-aged children with a cochlear implant, Ear Hear. 24 (1 Suppl) (2003) 69S–81S. [10] N.M. Young, F.M. Kim, M.E. Ryan, E. Tournis, S. Yaras, Pediatric cochlear implantation of children with eighth nerve deficiency, Int. J. Pediatr. Otorhinolaryngol. 76 (2012) 1442–1448.
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