Slide 1 REVISITING CANDIDACY: EXPANDING CRITERIA FOR COCHLEAR IMPLANTS. Slide 2. Slide 3. Cochlear Implant History. Cochlear Implant History

Transcription

1 Slide 1 REVISITING CANDIDACY: EPANDING CRITERIA FR CCHLEAR IMPLANTS Jordan King, Au.D. CCC-A Cochlear Implant Audiologist Arkansas Children s Hospital kingje@archildrens.org Slide 2 Cochlear Implant History s: experimentation Dijourno and Eyries William House F. Blair Simmons 1970 s: feasibility study Is this safe and useful? William House and Graeme Clark Slide 3 Cochlear Implant History Bilger Report 1980 s to present: commercial use and development Bilger, R. C. (1977). Psychoacoustic evaluation of present prostheses. Arch torhinolaryngol, 86,

2 Slide 4 Time Line st human subject implanted with multichannel device 1981 subjects demonstrated some open set speech understanding without lipreading 1985 FDA approval for adult patients 1990 FDA approval for children 2 years and older 2000 FDA approval for children 12months+ Slide 5 Where are we now? Commercially available multi-electrode cochlear implants from three manufacturer s Cochlear Corporation (1984) Advanced Bionics Corporation (1996) MED-EL Corporation (2001) Slide 6 Internal Devices Advanced Bionics HiRes 90K Cochlear Corporation 512 Med El Syncrony

3 Slide 7 External Devices Cochlear Corporation Nucleus 6 Kanso Aqua+ Slide 8 External Devices Med El Sonnet Rondo Slide 9 External Devices Advanced Bionics Naida Q90 sound processor & Phonak Naida Link hearing aid Aquacase

4 Slide 10 Candidacy Slide 11 Conventional vs. Expanding 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 ff Label Slide 12 Conventional vs. Expanding ff Label Implanting at less than 12 months of age Implanting with greater degrees of residual hearing Asymmetrical or unilateral losses Implanting with higher degrees of speech perception performance Simultaneous bilateral implantation

5 Slide Present Age Adults Adults/ Adults/ Adults / Adults/ Children (2yrs) Children (18 Children (12 Children months) months) (< 12 months) nset of HL Postlinguistic Postlinguistic Adults/ Adults/ Adults/ Adults, Pre & Children Pre & Children Pre & Children Pre & Postlinguistic Postlinguistic Postlinguistic Postlinguistic Children 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 penset speech scores 0% Sentences 0% Sentences 40% or less Sentences 50% or less in 50% or less in implanted implanted ear; 60% ear; 60% binaural binaural Sentences Sentences/sin gle syllable words Slide 14 Adult Selection Criteria > 18 years of age Bilateral, moderate to profound, sensorineural hearing loss Pre or post-linguistic onset Slide 15 Adult Selection Criteria Limited benefit from appropriately fit amplification 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.

6 Slide 16 Pediatric Selection Criteria Months Severe to profound bilateral sensorineural hearing loss Slide 17 Pediatric Selection Criteria months Limited benefit from binaural amplification Lack of progress in basic auditory skill development Amplification and aggressive intervention Slide 18 Pediatric Selection Criteria 2 Years - 17 Years 11 Months Severe-to-profound bilateral sensorineural hearing loss Lack of auditory skill development in spite of: ptimized hearing aids Intensive aural habilitation < 30% Multi-syllabic Lexical Neighborhood (MLNT) or Lexical Neighborhood Test (LNT) Holding children more closely to adult criteria *

7 Slide 19 Speech Perception 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 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: Slide 20 Speech Perception 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: Slide 21 teams have begun to Think outside the degree and configuration of the audiogram Consider the whole individual 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.

8 Slide 22 A word about Hybrid candidacy Slide 23 Hybrid Technology Slide 24 Candidacy for Hybrid The system is indicated for unilateral use in patients aged 18 years and older who have residual low frequency hearing and a severe to profound HF SNHL and who obtain limited benefit from HAs. Pre-operative hearing of candidates range from normal to moderate HL in the LFs (thresholds no poorer than 60 db HL up to and including 500 Hz) Severe to profound mid-to high-frequency hearing loss (threshold average of 2000, 3000 and 4000 Hz 75 db HL) in the ear to be implanted.

9 Slide 25 Slide 26 Speech Perception Testing with HAs The CNC word recognition score will be between 10% and 60%, inclusively in the ear to be implanted The contralateral ear will be equal to or better than that of the ear to be implanted by not more than 80% correct. Moderately severe to profound mid to high frequency HL (threshold averages of 2000, 3000 and 4000 Hz 60 db HL) in the contralateral ear. Slide 27 Cochlear Implant Vs. Hybrid

10 Slide 28 CASE STUDIES Slide 29 Case #1- Residual Hearing (symmetrical) 44 year old female Born normal hearing HL first identified at age 24 unknown etiology HL progressive in nature Worn hearing aids in both ears continuously since age 26 Slide 30 Case #1 Bilateral moderate to severe SNHL Works as an accountant Came to our program as a last resort before quitting her job Very uncomfortable in social situations unless her husband is present

11 Slide 31 Case #1 Patients goals: Be more at ease socially Talk on the phone Maintain employment Slide 32 Slide 33 Case #1 Pre-operative sentence scores Binaural aided Sentences in quiet at 60 db HL = 76% correct Sentences in quiet at 50 db HL = 41% correct Right aided Sentences in quiet at 60 db HL = 81% correct Left aided Sentences in quiet at 60 db HL = 37% correct

12 Slide 34 Case #1 120% 100% 80% 60% 40% Pre-op 1 month 6 months 9 months 20 months 20% 0% HINT-Q HINT +10 CNC words 9 years Slide 35 A = pre-op aided A A A A A =1 week post activation Slide 36 Case #1 1 month post activation Sentences in quiet at 60 db HL = 100% correct Sentences in quiet at 50 db HL = 64% correct 3 months post activation Sentences in quiet at 50 db HL = 77% correct 6 months post activation Sentences in quiet at 50 db HL = 86% correct Words in quiet at 60 db HL = 72% Words in quiet at 50 db HL = 36%

13 Slide 37 Case #1 9 months post activation (HINT sentences) Sentences in quiet at 50 db HL = 100% Sentences in noise at 50/40 db HL = 89% Sentences in quiet at 50/45 db HL = 31% 20 months post activation (CNC words) Words in quiet at 60 db HL = 76% Words in quiet at 50 db HL = 60% Slide 38 Case #1 9 years post activation CNC Words in quiet at 50 db HL = 84% AzBio sentences at 50 db HL = 93% AzBio sentences at 40 db HL = 88% Slide 39 Case #2 - Residual Hearing (symmetrical) 46 year old female Born normal hearing HL first identified at age 27 genetic etiology HL described as very slowly progressive

14 Slide 40 Case #2 Worn hearing aids in both ears continuously since age 27 Referred from managing ENT due to decrease in speech discrimination Bilateral moderately severe SNHL Slide 41 Case #2 Retired Military Volunteer Fire Fighter Husband works as commercial pilot Reliant on husband to help her communicate Slide 42 Case #2 Patient goals: To be more independent To talk on the phone To be more social

15 Slide 43 Slide 44 Case #2 Patient seen 4 times over four months Loaner fitting/verification After 3 months of HA work patient and husband reported no noticeable improvement Completed ABR to rule out ANSD Slide 45 Case #2 Pre-operative speech perception scores Binaural 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

16 Slide 46 A=pre-op aided binaural A A A A A =1 week post activation left Slide 47 Case #2 Slide 48 Case #2 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%

17 Slide 49 Case #2 1 year post activation Sentences in noise 50/40 db HL = 75% correct Words in quiet at 50 db HL = 64% 4 years post activation Using AzBio sentences instead of HINT In quiet at 50dB HL = 95% In noise at 50/40 = 73% In noise at 50/45 = 65% Slide 50 Case #2 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!!! Slide 51 Case #3 Residual hearing (sloping) 1 year 10 month old male ABR at 3 months old showed bilateral severe HL Fit HA s at 3 ½ months old Achieved full time HA easily Began discussing at 1 year 7 months Language age of 11 months Severe receptive/expressive delay

18 Slide 52 NR NR Slide 53 Case #3 Implanted right ear at 2 years old Implanted left ear at 2 ½ years old IT-MAIS scores Pre-op = 72% 1 year post activation = 95% Slide 54 R = right R L HA L R L R L = left L L L HA = pre op hearing aid R R responses binaural R HA HA HA SRT = 30 db 83% correct on MLNT

19 Slide 55 Case #3 Chronological age Listening Age Langauge Age Rate of Progress 3 ½ months 1 months 3 months (WNL) (HA) 1 yr 10 months 1 yr 8 months (HA) 11 months 8 months in 12 months 2 yrs 5 months 2 yrs 3 months (both) 18 months 7 months in 7 months 3 months () 3 yrs 2 yrs 10 months (both) 11 months () 3 yrs 7 months 3 yrs 5 months (both) 18 months ( s AU) 20 months 2 months in 6 months 30 months 10 months in 6 months Slide 56 Case #3 3 years 20% intelligibility in conversation 3 years 7 months 50-60% intelligibility in conversation Consonant production improved from 20% to 46% Vowel production improved from 54% to 88% 6 years 7 months 80% correct on LNT single syllable words 100% intelligible in conversation Slide 57 Case #3 Today: 10.7 years old; 9 years post activation PBK words at 50 db HL Right only = 84% at 50 db HL Left only = 84% at 40 db HL s bilaterally = 76% Pediatric AzBio Sentences at 30 db HL s bilaterally = 96%

20 Slide 58 Case #4 6 ½ year old female Born 6 weeks premature Moderate SNHL in the right ear and moderately severe SNHL in the left ear at age 2 ½. Progressive HL Slide 59 Case #4 Asymmetrical Wore 2 hearing aids consistently since time of diagnosis Hearing loss often had a mixed component do to recurrent persistent middle ear effusion. At time of evaluation patient exhibited moderately severe HL in the right ear and profound loss in the left ear Implanted left ear at age 7 Slide 60 NR NR NR

21 Slide 61 R = right HA pre-op L R L R R L R R L = left HA pre-op = 3 weeks post-op SRT = 35 db NR NR Slide 62 Case #4 Speech and Language progress CELF-4 Concepts and following directions Chronoloigcal Age Language Age Rate of Progress 6 years old <4 years 7 years old 4 years 9 months 9 months in 12 months 8 years old 7 years 6 months 2 years 9 months in 1 year Slide 63 Case #4 Speech and Language progress WLS ral Expression Chronological age Language Age Rate of progress 7 years old 4 years 10 months 8 years old 8 years 4 months 3 years 6 months in 1 year

22 Slide 64 Case #4 Speech and Language progress WLS Listening Comprehension Chronological age Language Age Rate of progress 7 years old 6 years 6 months 8 years old 8 years 10 months 2 years 4 months in 1 year Slide 65 Case #5 Asymmtrical 7 year old male (2010) Identified at birth with a normal hearing right ear and profound SNHL in the left ear Right ear progressed to severe rising to normal SNHL at 8 months old and has remained stable since Had worn right hearing aid only since progression of right ear Slide 66 NR NR NR NR NR NR

23 Slide 67 R = right HA R R R R R = present (6 yrs post Sx) SRT = 25 db with 30 db with HA Slide 68 Case #5 Counseled that would likely always to supplemental to the HA ear but not dominant. PBK words HA only at 50 db HL = 56% only at 50 db HL = 80% +HA at 50 db HL = 88% Pediatric AzBio Sentences in noise At 45/35 db HL = 97% At 45/40 db HL = 85% Slide 69 Cochlear Implants and Unilateral SNHL An Evidence Base

24 Slide 70 Cochlear Implants and Unilateral SNHL Unilateral SNHL is a common condition that has been indicated to significantly impact the development and academic success of children. For mild-moderate SNHL traditional amplification is a viable treatment option. For severe-profound SNHL, contralateral routing of the signal may be necessary (CRS/BAI), however this does not improve localization or provide true binaural hearing as only one cochlea is functional. Slide 71 Cochlear Implantation may represent a future treatment for unilateral SNHL. s can approximate the true benefits of binaural hearing. Head shadow effect Squelch effect Summation effect Slide 72 Dornhoffer, J.R., & Dornhoffer J.L. (December 2016). Pediatric Unilateral Sensorineural Hearing Loss: Implications and Management. Curr pin tolaryngol Head and Neck Surg, 24, Firszt, J.B., Reeder, R.M., Holden, L.K., (2016). Unilateral Hearing Loss: Understanding Speech Recognition and Localization Variability Implications for Cochlear Implant Candidacy, Ear and Hearing, 38, Sladen, D.P., Frisch, C.D., Carlson, M.L., et al. (January 2017). Cochlear Implantation for Single-Sided Deafness: A Multicenter Study. Laryngoscope, 127,

25 Slide 73 Sladen et al. Significant improvement in monosyllabic word recognition scores in quiet Significant improvement in sentence recognition scores in quiet Improvement noted in binaural sentence in noise recognition but difference wasn t statically significant. 13 of 23 participants reported tinnitus and of those 13, 12 reported improvement in tinnitus post implantation Slide 74 Case #6 Unilateral (SSD) 2 year 8 month old male Normal birth history Failed newborn hearing screening Father has unilateral SNHL (SSD) Mother is an audiologist Identified at 13 days old with a normal hearing left ear and a moderate to severe right ear. Slide 75 A A A

26 Slide 76 Case #6 HA use was initiated at 4 months of age Chronic middle ear infection/effusion Good HA use when ear was healthy Right ear SNHL progressed into the profound range at 1year 8 months of age Poor HA benefit after progression Parents pursued unilateral cochlear implantation Slide 77 Case #6 Implanted in the right (profound) ear at 2 years of age. Current age is 2 years 8 months and chronic middle ear infection/effusion remains problematic specifically in his normal hearing ear. Slide 78 > > > >

27 Slide 79 Case #6 SRT with = 35 db HL WIPI score in only condition = 72% Slide 80 Prognostic Indicators Previous auditory experience* Length of deafness* Age at implantation IQ Status of cochlea Presence of additional disabilities Consistency of device use Mode of communication Quality habilitative environment Slide 81 Conclusions Candidacy criteria are set too low. Significant benefit from cochlear implantation is being observed for adult and pediatric patients who do not meet current FDA candidacy criteria for.

28 Slide 82 Considerations for implanting under expanded candidacy criteria: Asymmetry Precipitously sloping losses Unilateral losses (SSD) ANSD Masking is necessary to isolate the for sound field verification Insurance coverage could be problematic Slide 83 Questions?