Cochlear Implant Grand Rounds: Across the Lifespan. Facial Nerve Stimulation Case Study

Transcription

1 Cochlear Implant Grand Rounds: Across the Lifespan Presented by Allison Biever, AuD; Jenny Goerhing, AuD; Jordan King, AuD; and Douglas Sladen, PhD June 20, 2013 Facial Nerve Stimulation Presented by Douglas Sladen, PhD Director of the Cochlear Implant Program at Mayo Clinic in Rochester, MN Facial Nerve Stimulation (FNS) Occurrence varies by report, as few as 1% and as many as 14% Onset varies though reports often cite the first observation of FNS within the first two years Cochlear malformations and Otosclerosis are common underlying causes Other causes are high stimulation levels needed in cases of long term deafness Commonly seen in mid-array electrodes, likely due to proximity to facial nerve 1

2 Case Example #1: BACKGROUND 53 year old woman with bilateral CI and bilateral FNS Onset of hearing loss is unknown first taken to the physician at age 3 because she was not talking. No audiometric testing was done Still not talking when entering Kindergarten At this point diagnosed with a severe-to-profound hearing loss Fitted with hearing aid on left side ear only since it was her better ear Her loss was determined to be the result of Mondini malformations Age 14 she lost the left sided hearing and began to use a hearing on the right side She reported the right sided hearing aid provided some benefit, though less than the left Case Example #1: RIGHT CI Wore right sided hearing aid from age 14 to age 41, at which time that all hearing in that ear was lost Implanted in 2001 on the right side with a C1.2 ICS and HiFocus w/positioner at another center First seen at Mayo Oct 2005 reporting chronic FNS CIS 8 channel program (16 paired contacts) using odd electrodes Obvious facial stim on electrodes 7 and 8 (most basal) using a 150 usec pw Pulsewith changed to 225 usec and that eliminated facial stim CUNY sentence score on this day was 62% Case Example #1: LEFT CI Nov 2005 repeat visit to fine tune programming and evaluate candidacy for left sided cochlear implant Speech perception testing, Right sided CI and Auria HINT sentences quiet two list average = 26% BKB-SIN = 21 db SNR 2

3 Case Example #1: FNS April 2006 activation of left CI Initial programming with HiRes-P using a 10.8 usec PW Reported immediate recognition of speech Sept 2006 FNS starts on left 5 months after initial stim of left side FNS occurs on the newly implanted left side, and continues on the right Left sided FNS managed by moving from paired to sequential stim and turning off electrodes 4-7 Right side managed using clipping CNC words CNC phonemes AzBio sentences HINT Q sentences (2lists) BKB-SIN 2007 (Aug) 28% 58% 33% 63% db SNR 2007 (Nov) 40% 66% 23% DNT 16.5 db SNR % 73% 46% 69% 14 db SNR % 63% 34% 64% 15.5 db SNR 2012 Right 10% 28% 2% 10% 23.5 db SNR Left 27% 60% 26% 58% 20 db SNR 3

4 Case Example #1: 2012 Right CI.2 HiFocus w/ positioner Changed stimulating electrode to odd # Changed coupling mode from monopolar to bipolar All channels had facial stim at soft levels Turned off all electrodes and turned them back on one by one Left HiRes 90K/HiFocus Helix Contacts 7 and 8 turned off, clipping applied to adjacent electrodes Turned off all electrodes and turned them back on one by one Increased PW needed higher stim level for loudness 4

5 CNC words CNC phonemes AzBio sentences HINT Q sentences (2lists) BKB-SIN 2007 (Aug) 28% 58% 33% 63% db SNR 2007 (Nov) 40% 66% 23% DNT 16.5 db SNR % 73% 46% 69% 14 db SNR % 63% 34% 64% 15.5 db SNR 2012 Right 10% 28% 2% 10% 23.5 db SNR Left 27% 60% 26% 58% 20 db SNR 5

6 Labyrinthine segment facial nerve Enlarged vestibular aqueduct Axial CT right ear Coronal CT right ear Case Example #1: October 2012 Explanted CI.2 Hifocus w/positioner Re-implanted HiResAdvantage/Hifocus Helix During surgery CI was stimulated on basal, mid and apical electrodes Facial nerve monitor was used to determine if moving electrode improved facial stim Stimulation of each electrode 20 units below current M level held constant Threshold of facial nerve monitor adjusted to audibility Repeated after moving electrode no improvement Repeated with new implant no facial alarm even at high sensitivity Case Example #1: management of current systems Consulting with colleagues/clinical specialists Taking advantage tools (clipping) Participation in aural rehabilitation Psychophysics obtained simultaneously 6

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8 Speech understanding Right CNC = 20% AzBio = 12% HINT quiet = 40% BKB-SIN = 20.5 dbsnr summary Analysis of explanted device concluded device was not defective, though accuracy of test is unknown Likely combination of anatomical and device related conditions Management of FNS requires exhaustive efforts to program around system, and careful consideration of possible benefits of current arrays Allison Biever, AuD Rocky Mountain Cochlear Implant Center 8

9 Case History First seen November year old female Utilizing body worn SPrint Rocky Mountain Cochlear Implant Center Implanted at 3 years, 2 months (had device for 1 year, 2 months) in Peru Difficult time getting information from original implant center Patient had a malformed cochlea, but full insertion was reported by parents Started working with AVT 2 weeks after moving to Colorado Observations Very dependent on her hearing aid Poor speech production; unintelligible Poor speech perception for 4.5 years old 6/12 words correct on ESP SI Consistent responder to CPA Thresholds at 25dB Speech inappropriately loud Excessive facial nerve stimulation Rocky Mountain Cochlear Implant Center Original programming Electrode #16 turned off for unknown reason Programmed in ACE 900 Hz 10 maxima Rocky Mountain Cochlear Implant Center 9

10 Problems Rocky Mountain Cochlear Implant Center 1. How to optimize the implant without facial nerve stimulation and increasing overall volume? 2. Bilateral implantation? Parents were reluctant to implant right side because of residual hearing and narrow IAC. Suggestions for Optimizing Implant Lower rate/ lower maxima Grounding mode (ACE): MP1, MP2 Turn off electrodes Voltage compliance issues? Gain changes/eliminating ADRO Rocky Mountain Cochlear Implant Center Change coding strategy and grounding method (SPEAK & pseduomonopolar) Change pulse width Check placement of internal device Assess integrity of internal device Lower Rate/Maxima Rocky Mountain Cochlear Implant Center 10

11 Rocky Mountain Cochlear Implant Center Ground Mode Changes Turn Off Electrodes Rocky Mountain Cochlear Implant Center Compliance Issues Rocky Mountain Cochlear Implant Center 11

12 Gain Changes Rocky Mountain Cochlear Implant Center Change to Rocky Mountain Cochlear Implant Center SPEAK/pseudomonopolar Change to Rocky Mountain Cochlear Implant Center SPEAK/pseudomonopolar 12

13 Change pulse width Rocky Mountain Cochlear Implant Center Take Home Message Rocky Mountain Cochlear Implant Center Must have adequate C level (loudness level) Many different approaches can be tried to reduce facial nerve stim: Pulse Width Strategy Rate Maxima Grounding Mode Turning Electrodes Off Cochlear Implant Grand Rounds: Working with a child with Autism Spectrum Disorder Jenny L. Goehring, AuD Audiologist, Cochlear Implant Program Boys Town National Research Hospital 13

14 Background Universal newborn hearing screening is now widespread, leading to early identification and treatment of hearing loss Early intervention and early implantation improve communication outcomes JCIH, 1994a; JCIH 1994b; ASHA 2007 Position Statement; Yoshinaga Itano et al. (1998) Background Early CI candidacy criteria required candidates have no cognitive concerns Cochlear implants are FDA approved for children as young as 12 months Additional developmental delays does not necessarily preclude implantation 30 40% of children with hearing loss have another disability in addition to hearing loss Background Autism is a complex brain disorder characterized by difficulties in social interaction, verbal and nonverbal communication and repetitive behaviors. Varying degrees of outcomes, under the larger umbrella of Autism Spectrum Disorders (ASD) or Pervasive Developmental Disorders (PDD) In 2008, prevalence of ASD was 1 in 88 children Appears during early childhood (by 3 yrs) Autism and Developmental Disabilities Monitoring Network (ADDM) 14

15 Background Background Only one study specifically on children with CIs and ASD Donaldson et al. (2004), N=7 All children showed improvement in standardized or raw speechlanguage or auditory scores (improvement in some capacity) MAIS/IT MAIS score improved from 42% pre op to 72% post op Parent survey revealed changes in responses to sound, vocalization, eye contact, responses to requests, and music enjoyment 5/6 families would recommend a CI to another family in a similar situation Irwin et al. (2011) children with ASD were weaker at speech reading than typically developing peers Background Research on children with CIs and other disabilities (some included ASD) Wiley et al. (2008), N = 14 Children with additional disabilities make measurable progress in auditory skills with a CI Children with disabilities had the same rate of auditory skills progress as children without disabilities Wiley et al. (2012), N = 6 PEDI questionnaire (measure of daily functional skills) at pre CI, 6 mos, and 12 mos post CI noted improvement in scaled scores for all three PEDI domains (social function, mobility, self care) 15

16 Background Palmieri et al. (2012), N=50 Examined survey tool (DADQ) to provide an indication of improvement in quality of life Improvements noted in all domains from pre CI to post CI (perceptual skills, preferred communication mode, communicative behaviors, attention and memory, social interaction) Device use pre CI ranged from sometimes to always, increased to always post CI Child birth history: Born full term, 3 weeks in the NICU, had jaundice, immune system problems, received antibiotics, referred on UNHS Diagnostic audiological evaluation (2 months of age): ABR at BTNRH revealed no response at any test frequency, normal high frequency tympanograms, OAEs absent AU Hearing aid fitting (2.5 months of age) Loaner aid alternated between ears Inconsistent use at first, which improved to full time during all waking hours With aid in use, vocalizations increased and one response to the vacuum (could have been vibrotactile) Family was referred to CI Team Lives approximately 1 hour from BTNRH, large household of 7 children Multidisciplinary CI evaluation (9 months of age): Parents very motivated and committed to long term follow up Early intervention team in place MRI of IAC normal AU, no medical contraindications Vestibular testing not obtained d/t patient distress Now utilizing 2 hearing aids full time, responsive to sound with aids, emerging auditory skills (IT MAIS score of 7/40=18%) REEL 3 (Standard Scores = 54 67; first percentiles) DP 3 (66 89, overall development = 71 delayed) 16

17 Underwent CI to right ear (14 months of age) Successful surgery, X ray showed good electrode position Intraoperative impedances WNL; ECAPs obtained for all electrodes at surgery Nucleus CI512 with CP810 speech processor Initial activation 1.5 weeks later (15 months of age) Activation appointments over 2 days Child was rather upset, cried throughout the appointments, some rocking behaviors ECAP based map with conservative stimulation levels (no behavioral info) Parents seemed to be comfortable with equipment First follow up appointment 2 weeks later Wore device for approx. 3 days but would not tolerate for next 1.5 weeks Moved to off the ear option d/t constant removal of processor No signs of sound being too loud Recommended continuing use of left hearing aid, also increase in home services to 1 2 times per week Phone call with deaf educator: Busy household so goal is to include parents in therapy Family needs support with device use 1 month post activation Wearing consistently, reported increase in responses to sound Audiometric responses near db HL (likely suprathreshold) SAT = 50 db HL 17

18 3 months post activation Wearing about 50% of the time, processor comes off when laying on back and rocking, but is now standing and cruising Discussed moving back to ear level, ordered snugfits for retention Speech awareness threshold at 55 db HL Limited auditory progress on IT MAIS Test Administered Pre CI 1 mo post CI 3 mos post CI IT MAIS Parent questionnaire Total Score Age equivalent 7/40 (18%) mos 4/40 (10%).9 mos 6/40 (15%) 1.4 mos Returned at 4 ½ months post activation Parents concerned d/t non use Need to increase use: joybands, initiate speech therapy at BTNRH, increase audiology appointments, family complied with all recommendations 6 months post activation Has not worn CI for 1 month d/t equipment malfunctioning Entire appointment spent getting child to wear CI (upset for appt) Responses to speech in the soundfield around db HL Was seen for 2 Aural Rehab sessions in the past 2 months Returned for audiologic appointments at 7, 8, and 9 months post activation Wearing the processor everyday, but not full time, JoyBand seems to help somewhat, have tried various retention options Responses to speech in the soundfield around db HL Responded to LING sounds in live voice for programming (more readily the /s/ and /sh/) Continues to return for Aural Rehab 1 2 times per month, IT MAIS progress is limited Test Administered Pre CI 1 mo post CI 3 mos post CI 9 mos post CI IT MAIS Total Score Age equivalent 7/40 (18%) mos 4/40 (10%).9 mos 6/40 (15%) 1.4 mos 5/40 (13%) mos 18

19 1 year post activation (2 years, 3 months old) Significant increase in wearing time in last month (nearly full time) Now wearing processor outside of the home SAT = 40 db HL 1 year post activation Results of IT MAIS: increase in vocalizations, vocal play (raspberries), responds to sound consistently Delayed in overall development, not yet walking Test Administered Pre CI 1 mo post CI 3 mos 9 mos 12 mos IT MAIS Total Score Age equivalent 7/40 (18%) mos 4/40 (10%).9 mos 6/40 (15%) 1.4 mos 5/40 (13%) mos 6/40 (15%) 1.4 mos Mother s main concern is rocking behavior and trouble with transitioning to new places outside the home Clinicians also concerned about atypical behaviors, no eye contact, strange manipulation of objects Began discussing need for global skills evaluation Continuing Audiologic appointments every 3 4 months for processor fine tuning Wearing processor consistently Vocalizations are now more speech like Consistently signing more, outside, and no Family is incorporating other signs, although progress is slow Gets very upset when any changes are made to processor programs, Limited audiometric information 19

20 Continuing Aural Rehabilitation sessions twice per month Responses to sound are inconsistent (depending on the day) Limited eye contact Difficulties with transition to clinic room Limited speech language development even with several therapy sessions and home intervention Trying to establish a communication system (CI, sign, PECS) Parents report inappropriate social interactions and manipulation of objects, lining up and spinning objects, rocking behaviors Approximately 1.5 years post CI, Audiology and SLP met with family together Discussed goals and concerns regarding delays in communication and progress with CI Parents have concerns regarding behavior (limited eye contact, limited social interaction) Discussed possibility of multidisciplinary team evaluation with developmental pediatrician Family was not familiar with Autism Spectrum Disorder Discussed completing an updated speech language evaluation and looking into developmental clinic Speech Language Evaluation at 1 year, 8 months post CI (2 years, 11 months old) Test Administered: Developmental Profile 3 Standard Score Percentile Physical <50 (85) [89] <.1 1;8 Adaptive Behavior <50 (60) [81] <.1 1;0 Social Emotional <50 (<50) [83] <.1 0;5 Cognitive <50 (<50) [78] <.1 0;9 Communication <50 (<50) [66] <.1 0;5 General Development Score < 40 (43) [71] <.1 Age Equivalent 20

21 Speech Language Evaluation at 1 year, 8 months post CI (2 years, 11 months old) Test Administered: Mullen Scales of Early Learning Standard Score Percentile Gross Motor 1;5 Visual Reception <55 <1 0;10 Fine Motor <55 <1 1;8 Receptive Language <55 <1 0;1 Expressive Language <55 <1 0;4 Early Learning Composite 49 1 Age Equivalent Speech Language Evaluation at 1 year, 8 months post CI (2 years, 11 months old) Test Administered: Gilliam Autism Rating Scale (GARS) Scaled Score Percentile Probability of Autism Stereotyped Behaviors Average Social Interaction Above Average Developmental Average Autism Quotient Very High Referred to a Developmental Pediatrician Thorough evaluation by Developmental Pediatrician at Boys Town Pediatrics Department (3 years of age): No other medical concerns Chromosome testing normal Diagnosis of Autism Spectrum Disorder Spent extensive time counseling the family as they were not familiar with ASD Medical diagnosis, not educational at this point (not part of IEP) Large burden off the family, better able to understand his needs 21

22 Where are we now? Still struggles to maintain full time use, disrupted with big events Attends local preschool 3 half days a week, will be 5 days next year Attends speech therapy 2 3 times/mo at BTNRH Much time has been spent working on conditioned response to sound & optimizing programming Programming challenges: limited hearing experience, limited language, behavioral issues, understanding the patient (how to optimize apt), room set up, limited attention span Very routine based, does not like changes to CI or environment Recommended a sensory integration evaluation SLP working on establishing a communication system Where are we now? SLP & Audiologist together obtained audiometric information Two audiograms on separate dates were consistent SAT = 30 db HL SAT = 30 db HL Where are we now? IT MAIS: slowly progressing in auditory skills over time More vocal with use of processor Consistently responds to sound, more so in familiar environments Response to name 75% in quiet, 50% in noise Can identify dad and one of sibling s voices Wearing the processor off the ear to alleviate sensory issues Child helps to attach headpiece when it falls off Test Administered IT MAIS Total Score Age equivalent Pre CI 1 mo post 3 mos post 9 mos post 12 mos post 2 yrs post 2 yrs, 7 mos post 7/40 (18%) 4/40 (10%) 6/40 (15%) 5/40 (13%) 6/40 (15%) 11/40 (28%) 14/40 (35%) mos.9 mos 1.4 mos mos 1.4 mos months 3.6 months 22

23 Most recent speech language evaluation at 2.5 years post CI (age 3 years, 9 months) Test Administered: Mullen Scales of Early Learning Standard Score Percentile Age Equivalent Visual Reception ;11 (0;10) Fine Motor ; 2 (1;8) Receptive Language <55 <1 0;11 (0;1) Expressive Language <55 <1 0; 9 (0;4) Early Learning Composite 49 1 Significantly delayed skills in all areas, yet did make some nice gains in age equivalents in past year Why was diagnosis delayed? Complex issues, tough subject matter Child s family expanded, overall resources limited Early intervention team was hesitant regarding diagnosis of ASD Family seemed relieved after discussion & diagnosis was made Recommendations Cases are complex Key into early red flags Counsel families who already have the diagnosis about limited outcomes Rehabilitative approach may differ from child to child (i.e. social story, AAC, picture schedules, and PECS) Build a solid foundation of trust with the family 23

24 Recommendations Audiology specific recommendations: Introduce external hardware prior to activation (for older kids) Introduce sound gradually and slowly Need for objective measures (ESRTs and ECAPs) Modify clinical appointments as needed Longer follow up periods, and likely more appointments needed Recommendations Speech language specific recommendations: Teamwork is crucial (especially between family, educational, and clinical teams) Continue building & exploring communication systems Continue to support listening Don t be afraid to have difficult conversations Help families see child s strengths Seek the help of specialists (behavioral, AAC, etc.) Summary The presence of additional disabilities does not preclude a child from being considered for a cochlear implant (less strict candidacy criteria) Strong need for multidisciplinary evaluations and counseling Outcome with CI may be more limited, and depend on the degree of ASD Children often present with a unique set of challenges Modify and adapt Need for standardized/formal measures for children with ASD 24

25 References American Speech Language Hearing Association; American Speech Language Hearing Association. (2007). Year 2007 Position Statement: Principles and Guidelines for Early Hearing Detection and Intervention Programs. Autism and Developmental Disabilities Monitoring Network (ADDM); Centers for Disease Control and Prevention; Donaldson, A. I., Heavner, K. S., Zwolan, T. A. (2004). Measuring progress in children with Autism Spectrum Disorder who have cochlear implants, Arch Otolaryngol Head Neck Surg, 130, Irwin, J. R., Tornatore, L. A., Brancazio, L., Whalen, D. H. (2011). Can children with Autism Spectrum Disorders hear a speaking face? Child Development, 82(5), Joint Committee on Infant Hearing Position Statement, 1994a; Joint Committee on Infant Hearing Position Statement, 1994b; Palmieri, M., Berrettini, S., Forli, F., Trevisi, P., Genovese, E., Chilosi, A. M., Arslan, E., Martini, A. (2012). Evaluating benefits of cochlear implantation in deaf children with additional disabilities, Ear & Hearing, 33(6), Wiley, S., Meinzen Derr, J., Choo, D. (2008). Auditory skills development among children with developmental delays and cochlear implants, Annals of Otology, Rhinology, & Laryngology, 117(10), Wiley S., Meinzen Derr, J., Grether, S., Choo, D. I., Hughes, M. L. (2012). Longitudinal functional performance among children with cochlear implants and disabilities: A prospective study using the Pediatric Evaluation of Disability Inventory, International Journal of Pediatric Otorhinolaryngology, 76(5), Yoshinaga Itano, C., Sedey, A., Coulter, D., Mehl, A. (1998). Language of Early and Later identified Children With Hearing Loss. Pediatrics, 102, Contact Jenny L. Goehring, AuD Audiologist, Cochlear Implant Program Boys Town National Research Hospital Center for Childhood Deafness Jenny.Goehring@boystown.org REVISITING CANDIDACY: EXPANDING CRITERIA FOR COCHLEAR IMPLANTS Jordan King, AuD Cochlear Implant Audiologist Arkansas Children s Hospital kingje@archildrens.org 25

26 Present Age Adults Adults/ Children (2yrs) Adults/ Children (18 months) Adults / Children (12 months) Adults/ Children (< 12 months) Onset of HL Postlinguistic Postlinguistic Adults, Pre & Postlinguistic Children Adults/ Children Pre & Postlinguistic Adults/ Children Pre & Postlinguistic Adults/ Children Pre & Postlinguistic Degree of HL Bilateral Profound Bilateral Profound Bilateral Severe- Profound Adults ; Profound Children Bilateral Severe- Profound 2 yrs & older; Profound < 2 yrs Bialteral Moderate- Profound Adults; Severeprofound < 2yrs Openset speech scores 0% Sentences 0% Sentences 40% or less Sentences 50% or less in implanted ear; 60% binaural Sentences 50% or less in implanted ear; 60% binaural Sentences/sin gle syllable words FDA Consumer protection and health agency that governs manufacturers Determines device labeling Labeling serves as a guideline for candidacy decisions Based on audiogram and speech recognition Off Label Speech Recognition criteria Determined by aided open set sentence or single syllable word recognition scores. Best aided condition: < 60% correct Implanted ear: < 50% correct Medicare Criteria: < 40% on recorded open set sentences in best aided condition. 26

27 Speech Recognition Testing Presentation level 70 db SPL used historically 50 db SPL is recommended today Representative of soft speech Test materials HINT sentences in quiet used historically (Nilsson et al. 2004) Poor inter-test reliability Ceiling affects Not designed to be given in quiet Single male talker speaking with slow rate and excellent enunciation *Alkaf & Firszt. (2007). JAAA18: ; *Skinner et al. (1997). J Acoust Soc Am 101: Speech Recognition Testing Test Materials Az Bio Sentences in quiet 4 different talkers (2 male/2 female) using normal rate and style of speaking CNC words Monosyllabic word test Single talker Good inter test reliability with Az Bio sentences Az Bio sentences and CNC words used together make an appropriate speech perception test battery for candidacy determination *Gifford et al. (2008). Audiol Neurotol 13: CI teams have begun to Think outside the degree and configuration of the audiogram Consider the whole individual CI centers at the local, national and international level continue to consider individuals who are younger, have more significant degrees of residual hearing, and consequently higher preoperative speech perception scores than indicated by current device labeling. 27

28 CASE STUDY: J.H. Case History 46 year old female Born normal hearing HL first identified at age 27 genetic etiology HL described as very slowly progressive Case History Worn hearing aids in both ears continuously since age 27 Referred from managing ENT due to decrease in speech discrimination Bilateral moderately severe SNHL Reports good and bad hearing days 28

29 Case History Retired Military Volunteer Fire Fighter Husband works as commercial pilot Reliant on husband to help her communicate Patient goals: To be more independent To talk on the phone To be more social Audiologic Evaluation Puretone Air and Bone Conduction SRT OAE s Acoustic Reflexes HA evaluation/verification Loaner HA fitting if needed Bilateral and monaural aided sound-field thresholds Bilateral and monaural aided speech recognition testing 29

30 X X O OX X X O O X O O Audiologic Evaluation Patient seen 4 times over four months Loaner fitting/verification Aided sound-field thresholds remained less than optimal After 3 months of HA work patient and husband reported no noticeable improvement Questions/Concerns Why could acceptable aided sound-field thresholds not be obtained in spite of degree of residual hearing? Patient reports good and bad hearing days. 30

31 Questions/Concerns Etiology can affect counseling regarding expectations of performance outcomes. Completed ABR to rule out ANSD ABR waveforms were typical of SNHL Pre-Op Speech Recognition Pre-operative speech recognition scores Bilaterally aided Sentences at 60 db HL = 85% correct Sentences at 50 db HL = 59% correct CNC words at 50 db HL = 32% correct Right aided Sentences in quiet at 60 db HL = 51% correct Left aided Sentences in quiet at 60 db HL = 30% correct A=pre-op aided binaural CI CI CI CI CI CI=1 week post activation left A A A A CI A 31

32 Speech Recognition Scores Post-op Speech Recognition Scores 3 weeks post activation Sentences at 50 db HL = 82% correct CNC words at 50 db HL = 39% correct 2 months post activation Sentences in quiet at 50 db HL = 100% correct CNC words at 50 db HL = 39% correct 5 months post activation Sentences in noise 50/40 db HL = 64% correct Words in quiet at 50 db HL = 44% Post-op Speech Recognition Scores 1 year post activation Sentences in noise 50/40 db HL = 75% correct Words in quiet at 50 db HL = 64% 2.5 years post activation Words in quiet at 50 db HL =64% 3.5 years post activation Words in quiet at 50 db HL =76% 32

33 Conclusions At lunch my friend from New Jersey spoke very fast. She forgets to turn toward me. She sat across from me instead of beside me but I decided to see how it went. Well not only could I keep up with her conversation, but I could eat and hit my mouth because I could watch my fork rather than read her lips!!! Conclusions Candidacy criteria are set too low Significant benefit is being observed for adult and pediatric patients who do not meet current FDA candidacy criteria for CI Consider Asymmetry Precipitously sloping losses ANSD 33