Bringing Hearing to the Deaf: A technical and personal account 1 TALK OUTLINE How we hear: The physiology of natural hearing Causes of Deafness (27 million Americans cannot hear well) Solutions for hearing loss The cochlear implant: How to get one. What is it like? How well do they work? Why do they work? Political & social issues What cochlear implants are telling us about the functioning of the brain The future 2 ca. 550 B.C. Sound Pythagoras reasons that sound is a vibration of air. 3 Physical and perceptual characteristics of sound Physical • Amplitude • Frequency • Complexity , and phase relationship of constituent frequencies Perceptual • Loudness • Pitch • Timbre 4 Acoustic Pressure is measured in decibels (dB) • 1 atm = 100,000 pascals = 1011 micropascals • Threshold: the softest sound detectable is 20 micropascals (at 1000 Hz). 2 parts in 10 billion of an atmosphere • We hear sounds 1-10 million times more intense than threshold • dB are logarithmic units with 0 dB at threshold • adding 20 dB = factor of 10 increase in pressure – 6 dB approximately doubles the pressure • 40 dB SPL = 20 x 100 = 2,000 micropascals 5 loud Hearing threshold of a severely deaf person Hearing threshold of a profoundly deaf person (ex: the speaker) soft 6 The Ear Has Three Distinct Regions ca. 175 A.D. Galen ca. 550 B.C. Pythagoras & successors Nerve transmits sound to the brain It has taken until the present to unravel the rest 7 The Outer Ear The videos shown in this talk are based on Auditory Transduction by Brandon Pletcsh which was awarded 1st place in the NSF/AAAS Science and Engineering Visualization Challenge 2003 8 Tympanic Vibrations 9 The tympanic membrane & ossicles 1543 Anatomist Andreas Vesalius describes the structure of the middle ear. 10 The tympanic membrane & ossicles 11 Bony Labyrinth stapes and round window 12 Why is our “sound sensor” not on the outside of our head? Hermann Ludwig von Helmholtz first to understand the role of the ossicles Impedance mismatch overcome by ratio of areas and lever action 13 The cochlea and it chambers The cochlea is about the size of a pea 1561 Gabriello Fallopio discovers the snail-shaped cochlea of the inner ear. 14 The Cochlea houses the Organ of Corti Auditory Nerve 15 Organ of Corti Hair Cells are a mechano-electric transducer 1st detailed study of Organ of Corti by Alfonso Corti Original figures (scanned) from: 16 Zeitschrift für wissenschaftliche Zoologie (1851) The Basilar Membrane is a Frequency Analyzer 17 Tonotopic Organization 18 End of Early History The Middle Ages Georg von Békésy (Nobel 1961) Hermann Ludwig von Helmholtz first theory of the role of BM as a spectrum analyzer providing a frequency-position map of sound Fourier components. Experimentally measured traveling wave profiles published by von Békésy in Experiment in Hearing, McGraw-Hill Inc., 1960. base apex 19 The Copernican Revolution Von Békésy's findings stimulated the production of numerous cochlear models that reproduced the observed wave shapes, but were in contrast with psychophysical data on the frequency selectivity of the cochlea. displacement Davies (1983): a revolutionary new hypothesis there exists an active process within the organ of Corti that increases the vibration of the basilar membrane. 20 Active amplification Careful measurements on living animal cochlea Same animal post mortem Johnstone et al (1986) What causes the amplification? 21 Rows of Hair Cells in the healthy cochlea Inner hair cells 10,000 afferent (signals go the brain) Outer Hair Cells 30,000 Sparsely innervated Hair 5m 30mHair cell 22 Hair cells are mechano-electrical transducers 1980’s 500 nm Both inner and outer hair cells work this way 2nm diameter 23 The inner hair cells send signals to the brain that are interpreted as sound. What do the outer hair cells do? Outer hair cells exhibit electro motility they are also electro-mechanical 1987-2003 transducers 24 The Five Main Causes of Hearing Loss 1. 2. 3. 4. 5. Heredity. At least 100 hereditary syndromes can result in hearing loss. Infections, such as bacterial meningitis and rubella (German measles). Acoustic trauma produced by acute or chronic exposure to loud sounds. Prescription drugs, such as ototoxic antibiotics (streptomycin and tobramycin) and chemotherapeutic agents, such as cisplatin. Presbycusis, the hearing loss of old age, All of us Me in 1990 27 million Americans cannot hear well 25 The main types of hearing loss and treatments 1) Conductive (the ossicles no longer function) BAHA 2) Sensorineural (loss of hair cells) (a) (70%) loss of some hair cells (mild, moderate hearing loss) hearing aids(1940’s-present) / MEIHD (in clinical trials) (b) (4%) Loss of large numbers of hair cells (severe (3%) profound (1%)) Hearing aids do not help: no matter how loud the amplified sound there is little electricity reaching the brain Cochlea Implant (CI) 3) Loss of the auditory nerve Auditory Brain Stem Implant (ABI) (a cochlea implant at the cochlea nucleus) (Experimental) 26 Action of ototoxic antibiotics on hair cells Loud noise also destroys hair cells 27 Don’t lose your hair…. cells Many of the differences in perception between natural hearing and hearing in people with cochlear Normal hearing loss can be accounted for in terms of Hearing a loss or reduction in basilar compression. * Loss of gain (can’t hear softer sounds) * Reduced dynamic range * Loss of frequency sensitivity * Preferential loss of high frequency sensitivity. (Since hair cells at the base of the cochlea are more prone to damage.) 28 The first cochlea implant (1800)…. Volta placed two metallic probes in both ears and connected the end of two probes to a 50volt battery, and observed that: "... at the moment when the circuit was completed, I received a shock in the head, and some moments after I began to hear a sound, or rather noise in the ears, which I cannot well define: it was a kind of crackling with shocks, as if some paste or tenacious matter had been boiling... The disagreeable sensation, which I believe might be dangerous because of the shock in the brain, prevented me from repeating this experiment..." Alessandro Volta, Philosophical Transactions, Vol. 90 (1800), Part 2, pp. 403-431. 29 Cochlea Implant 1. 2. 3. 4. 5. 6. Sounds are picked up by a microphone & turned into an electrical signal. The signal passes to a speech processor (ASIC) where the spectrum is analyzed and “coded” (turned into a special digital pattern of electrical pulses). These pulses are sent to a coil antenna, then transmitted across the intact skin (by radio waves) to a receiver in the implant. The implant (ASIC) reads the program (data) and follows the instructions sending a pattern of analog electrical pulses to multiple electrodes in the cochlea. The auditory nerve picks up the electrical pulses and sends them to the brain. The brain recognizes the signals as sound. Unlike hearing aids, which make sounds louder, a Cochlear Implant bypasses the non-functional hair cells of the ear and delivers weak electrical signals directly to the auditory nerve. 30 Multiple electrodes at separate locations to exploit the tonotopic arrangement of the cochlea amplitude time AmplitudeCurrent 31 Channel Interaction The cochlea is filled with conductive salt solutions which allow current to spread. Current spread is detrimental to speech recognition when currents from different electrodes interact. Interleaved pulsing of the electrode array minimizes channel interaction 18,800 pulses per second 32 Sentence Recognition (% correct) 100 90 Cochlea implants have improved dramatically in twenty years SPEAK 80 CA/CIS 70 60 50 CIS CIS speech coding strategies Multipeak 40 F0F1F2 30 CA F0F2 20 10 SingleChannel 0 3M Nucleus Nucleus Nucleus Nucleus Ineraid Ineraid Clarion House WSP WSP II MSP Spectra 22 MIT RTI ABC 1980 1982 1985 1989 1994 1992 1993 1996 1 electrode multi- electrodes MedEL Combi 1996 Indication for Cochlear Implant • Adults (I’ll discuss children separately) – 18 years old and older (no limitation by age) – Bilateral severe-to-profound sensori-neural hearing loss (70 dB hearing loss or greater with little or no benefit from hearing aids for 6 months) ~1 million citizens now qualify but only ~20,000 CI’s in U.S.) – Psychologically suitable – No anatomic contraindications – Medically not contraindicated 34 General Workup • Audiological exam with binaural amplification • CT scan/MRI of temporal bones (cochlea structure) • Extended trial with state of the art high-powered hearing aids • Psychological evaluation • Medical evaluation • Any necessary tests to discover etiology of hearing loss • Patient chooses device: 3 major manufacturers of state of the art multi channel implants: Cochlear (Australia), MEDEL (Austria), Clarion (U.S.). All devices have similar performance the patient is the largest variable in the outcome 35 • Wait for surgery (can be many months….) Surgical Technique Surgery 2-4 hrs under general anesthesia 36 Postoperative Management • • • • • • • Complication rate <5% Wound infection/breakdown Facial nerve injury Vertigo Device failure—re-implantation usually successful Avoid MRI Wait ~8 weeks for wound to heal before activation day Porter & Gadre (Galveston, TX) 37 The cost of a CI: Insurance Issues A CI costs $50,000 including evaluation, surgery post operative hospital care, extensive audiological (re)habilitation. Medicare/Medicaid pays <$20,000. Some private insurers refuse to cover the devices, others provide excellent coverage. In general coverage is probably easier under a traditional plan than a HMO “The reimbursement levels have forced eight hospital to close CI programs due to the cost of subsidizing the implants.” (B. March President Cochlear America) Other hospitals ration services by putting children on waiting lists Currently 45,000 US children are CI eligible but only 9,000 have a CI And yet the cost of CI is small compared to the cost in government aid for education and training estimated at $1 million over the course of a lifetime (not to mention the massive human cost). “Ultimately this is about the way society views hearing..Being deaf is not going to kill you and so the insurance companies do not view this as necessary.” D. Sorkin, VP Consumer Affairs, Cochlear Corp. (A manufacturer).“ In my case, the cost of the device was fully covered by insurance 38 How well does it work? My experience 0 Normal dB Now BEFORE Pre-op 100 125 Hz Frequency 8000 Hz Speech tests pre-op now 39 Comparison of CI’s from the three main manufacturers Comparative Test Scores Percent Words/Sentences Correctly Understood 100% 90% Now Clarion HiFocus 80% Nucleus 24 Contour 70% Med-El Combi 40+ 60% 50% 40% 30% 20% 10% 0% CNC (1 month) CNC (3 mo.) CNC (6 mo.) HINT N (3 mo.) HINT Q (3 mo.) Test HINT Q (6 CUNY Q (3 CUNY Q (6 mo.) mo.) mo.) HSM (3 mo.) Types of word & sentence recognition Pre-op My test scores are no longer exceptional. 75% of recent patients with state of the art devices can use the phone. Why does the CI work so well 10,000 inner hair cells 10 electrodes ?40 Hearing doesn’t end at the cochlea Perception (visual or auditory) is a Dynamic Combination of Top-down and Bottom-Up Processing • The need for sensory detail depends on the distinctiveness of the object and the level of familiarity • “If you see a huge gray animal in the distance you don’t need much detail to know that it is an elephant” Visual examples… 41 42 43 44 45 46 47 48 49 50 51 52 Speech pattern recognition problem Vowel perception by normal hearing listeners. F1 and F2 values of English vowels (Peterson and Barney, 1952) Vowels are quite distinct •What features of the pattern of neural output from the cochlea •are most critical? Amplitude? Temporal? Place? 53 “CHOICE” SPECTROGRAPH TIME ELECTRODOGRAPH (SPEAK STRATEGY)) TIME 54 Input Dynamic Range Amplitude Study Pain 50 dB gives best result Cf normal hearing: 120 dB (20dB) (but speech falls in a ~50 dB Just audible range) 1 0 0 A . C o c h l e a r I m p l a n t L i s t e n e r s 9 0 Implants Normal Hearing B : N o r m a l H e a r i n g L i s t e n e r s 8 0 Compression 7 0 % 6 0 1 0 0 OutpAmplitude(DynamicRange%) 8 0 4 0 PercntCorect(%) O 5 0 p = 0 . 1 0 p = 0 . 2 0 p = 0 . 3 0 L O G 3 0 p = 0 . 5 6 0 2 0 p = 0 . 8 4 0 V O W E L S C O N S O N A N T S 1 0 p = 1 . 0 p = 1 . 5p = 2 . 0p = 3 . 0 p 2 0 Output = (Input) 0 A m i n 0 2 0 0 4 0 0 6 0 0 I n p u t A m p l i t u d e ( U n i t ) I 8 0 0 A m a x 1 0 0 0 0 0 . 0 5 0 . 1 0 . 2 P 0 . 5 P 0 . 30 . 50 . 8 1 23 P o w e r f u n c t i o n e x p o n e n t( P ) •Speech recognition is only mildly affected by large distortions in amplitude55 100 Temporal Study DAIP Consonants (20) Quiet n=7 Information Transferred 80 60 % Correct 16 Electrode CIS 40 12 Electrode CIS 8 Electrode CIS 4 Electrode CIS 16 Electrode QPS 20 12 Electrode QPS 8 Electrode QPS Types of implant with variable numbers of channels & speech coding strategies 4 Electrode QPS +10 dB SNR High stimulation pulse rates •High rates should better represent temporal features in speech. •No improved use of temporal cues in speech at higher rates observed •A significant effect for number of channels was seen for vowels (not shown) but not for consonants,monosyllabic words or sentences 0 500/s 1000 1000 /s 10000 10000 Stimulation Rate/s(ppse) 11 S/phase 1000 10000 56 Spectral Resolution (Number of Channels) Study 1-channel 2-channel 4-channel 8-channel 16-channel Original 57 Spectral Resolution (Number of Channels) Study % Correct •Most important factor is the number of spectral channels of information –number of distinct channels •Number of effective channels is proportional to, but not the same as, the number of electrodes (due to interactions between channels) 58 Place study Partial insertion Typical insertion 22 Apex 20 18 16 14 12 10 9 8 7 6 5 4 3 2 1 0 5 10 15 20 25 20 184 513 1168 2476 5085 Base 30 35 mm 10290 20677 Hz Frequency is logarithmically distributed along cochlea Typically pitch from 500- 5000 Hz is covered (speech is 250- 6800 Hz) Partial insertion can lead to more dramatic shift Frequencies of speech may not go to the correct tonotopic place for that sound tonotopic shift 59 60 61 62 63 64 65 Place study It takes time to adjust to the tonotopic shift i.e.to learn how to understand speech with an implant N = 67 100 % Percent Correct 90 80 70 60 50 40 30 20 10 0 PREOP 2 1 WEEKS MONTH 3 MONTHS 6 Time MONTHS Trials indicate that it may be possible to reduce the learning time by gradually shifting the frequencies Talavage (Purdue)/Svirsky (Indiana) The adult brain is quite plastic All of the adults in this study were post lingually deaf, What about prelingually deaf children? 66 The Deaf Community and Cochlear Implants • Until recently, strong opposition to pediatric implants while generally neutral towards adult implantation. • An implant will delay a deaf child’s acquisition of sign language (a deaf child’s “natural language”) and assimilation into the deaf community. • 1991 position statement National Association of the Deaf: “deplores the FDA decision to approve pediatric implantation as being unsound scientifically, procedurally, and ethically.” • 2000 position statement (www.nad.org): – Emphasizes taking advantage of technological advancements that have the potential to improve the quality of life for deaf and hard of hearing persons, and “strongly supports the development of the whole child and of language and literacy.” 67 Language Development in Profoundly Deaf Children With Cochlear Implants (Svirsky, Miyamoto et al. Indiana ) N=23 HEARING 60 LANGUAGE AGE (months) LANGUAGE AGE (months) 96 72 48 24 DEAF 0 0 24 48 72 96 Without CI (predicted) With CI 48 36 24 12 0 0 12 24 36 48 60 CHRONOLOGICAL AGE (months) AGE (months) Before & at 3 intervals after implant To be implanted Figure 1 Figure 2 “Despite a large amount of individual variability, the best performers in the implanted group seem to be developing an oral linguistic system based largely on auditory input from a cochlear implant” 68 Alien tries to figure out how a car works from the outside; By observation develops model: pressing gas pedal causes car to accelerate But alien would not know from this how internal combustion engine works. To do that they would need to take a look under the hood “Cochlear implants Enable us to study the role of the cochlea in perception (i.e. to take a look under the hood ”) 69 Physical stimulus Cochlea Normal Cochlea Auditory Nerve CI Auditory Nerve Neural coding ABI Brain “c a Perception t” Cochlear Nucleus Brain Using hearing impaired listeners to probe normal auditory functions Pitch estimate 100 90 80 70 60 50 40 30 20 10 0 100 Ineraid implant: DC 10 basal electrode apical electrode Monopolar Bipolar 2 4 6 8 10 12 14 16 18 20 22 1 10 Electrode Position (base to apex) 100 300 1000 5000 Pulse rate (Hz) Place pitch resolution is very poor it can be even be reversed for adjacent electrodes. Temporal coding for pitch = 300 Hz But in steps of 20 Hz Original (normal hearing (NH) discriminates in steps of 1-2Hz at 100 Hz NH uses tonotopic code to obtain frequency resolution at low frequencies CI Melody recognition is extremely difficult for CI users (lyrics help) Improving Cochlear Implants 1) Combined electric and acoustic stimulation Targets patients with reasonable low frequency hearing (usually with hearing aid) add shallow CI electrode for high frequency stimulation 72 2) Bilateral cochlear implants are 2 implants better than one? With one CI there is no directionality Localization NH 10 Bilateral CI 160 (Helms & Muller) 50% correct Bilateral 73 Bilateral cochlear implants Benefit #2 Better speech recognition in noise 100% Hearing subjects score 100% in all three tests For patients who do poorly with one implant the effects are much more dramatic 74 Plans to improve cochlear implants * * * * Combining acoustic and electric hearing Bilateral Cochlear implants Increasing the number of channels/greater cochlea coverage Reducing power fully implantable device (Use latest CMOS technology for ASIC or stay analog as long as possible. Microphone under skin or transducer attached to ossicles ) * Regeneration of neurons: molecular scaffold and electrical stimulation (nanotechnology) 75 Summary: Implants, Neuroscience & Bio-engineering Implants enable the postlingually deaf to hear & provide sufficient information to support language development in children Implants are a probe of speech recognition –Amplitude and temporal cues are not critical –Spectral/tonotopic cues are the key •number of effective channels (not electrodes) •frequency assignments to electrodes learning curve adult brain is plastic Music/speech quality (male/female & accents) Fine spectral information “The ear is is critical (not there yet) made for music” Implants are teaching us how the brain hears Example: pitch Implants, as the first prosthesis to successfully restore neural function, are a benchmark for biomedical engineering. 76 Final Thoughts A Cochlear Implant is a wonderful example of electrical engineering, computer science, mechanical engineering, physics, biology all working together in a tiny package inside a human being A wonderful example of why it is important to invest in basic research. There are 60,000 implantees worldwide. With the latest devices ¾ of post lingually deaf adults can use a telephone, and small children can hear their parents voices and learn to understand them At a personal level 357 days ago I had my hearing restored. It has enabled me to more easily conduct research & teach, and hear my wife’s voice for the first time in 12 years and my 11 year old daughter’s voice for the first time. 77 Acknowledgements This talk could not have been put together without the essential help of the following: At Purdue: Kirk Arndt & Steve Lichti (Physics) Donna Fekete (Biology) Beth Strickland (Audiology) Tom Talavage (ECE) At MedEl: Peter Knopp (Vienna) Jason Edwards (US), Amy Barco (US) Elsewhere: David Ashmore (London), Bill Brownell (Baylor), Phil Louzoi (UT Dallas), Richard Miyamoto (Indiana), Brandon Pletsch (IowaMed), Bob Shannon (House Ear Institute), Mario Svirsky (Indiana), Fan-Gang Zeng (UC Irvine) 78 Cochlea Implants are a hot topic PubMed Search: "Cochlear AND Implant” (Total=2086) 250 150 100 50 0 1972 73 74 75 76 77 78 79 1980 81 82 83 84 85 86 87 88 89 1990 91 92 93 94 95 96 97 98 99 2000 N umbe r of pape rs 200 1970s = 32 : 0 1980s = 453 : 1 1990s = 1557 : 5 Y e ar Pitch encoding Place code Timing code Vowel perception by cochlear implant users. 20 filters associated with 20 stimulation channels Stimulation channels 20 19 18 17 16 15* 14 13 12 11 10 9 8 7 6 5 4 3 2 1 Filters Amplitude 200 Hz 10 kHz Frequency *Stimulation mode: BP+1 81 Bilateral cochlear implants Benefit #2 Better speech recognition in noise: Bilateral implant vs. Monaural implant with noise on opposite side. (Modest gain) Bilateral implant vs. Monaural implant with noise on same side. (Enormous gain) 82 Loudness encoding L=f(A) E L=f[g(E)] A Loudness growth and balance functions L=exp(E) 100 A 600 Electric level (uA) Loudness estimate 700 B 500 400 10 300 200 0.29 0.29 L=3.65[(I+Io) -Io ]] r=0.99 1 E(uA)=-72.98+8.02 A(dB) r=0.98 100 0 20 40 60 80 100 Acoustic level (dB HL) 20 40 60 80 100 Acoustic level (dB HL) Auditory Brain Cochlear nerve expansion compression transmission 20-dB transmission line log ( ) exp ( ) Cochlear compression followed by central expansion 80-dB internal world 100-dB external world Loudness coding scheme 86 87 Standard Electrode 3) Better electrodes positions – closer to the target cells Comparison: a significant improvement: easier to hit the target when closer. Standard Electrode closer to target 88 The Copernican Revolution Von Békésy's findings stimulated the production of numerous cochlear models that reproduced the observed wave shapes, but were in contrast with psychophysical data on the frequency selectivity of the cochlea. Davies (1983): a revolutionary new hypothesis there exists an active process within the organ of Corti that increases the vibration of the basilar membrane. 89 Mountain November 2003 90 At near-threshold stimulus levels, the frequency tuning of auditory nerve fibers closely parallel that of basilar membrane displacement Only minor signal transformations intervene between mechanical vibration and auditory nerve excitation. In mammals, cochlear frequency selectivity is fully expressed in the vibrations of the basilar membrane. 91 Hydrodynanic Model of the Basilar Membrane f 0 =frequency of stimulus Z (1/ A2 ) (M 0 K / 0 )2 D2 M mass 0 =frequency of stimulus D = damping K stiffness A = area 0 = k 2 / m low Z resonance 92