1 History of Auditory Implantation Studying the history of auditory implantation and following the developments that have taken place in only some 50 years is exciting and teaches us about the courage, vision, and endurance of some special individuals in and from different parts of the globe. It also makes us realize that the cochlear implant (CI) has been developed through multidisciplinary efforts and would not have existed if other developments had not paved the way; for example, without the experience with silicone-covered leads in pacemakers, biocompatibility studies, and the existence of antibiotics, this development would not have been possible. The reactions of disbelief and skepticism in the community of auditory professionals, and later the reactions of anxiety and anger in the deaf community to these new developments—which felt like a threat and an offense to their culture and way of living—also demonstrate the sociological impact that the cochlear implant has had and sometimes still has. Finally, this chain of development could not have taken place without serendipity lending a hand, and without some very courageous patients who decided in close consultation with their doctors to become objects of research for this possible new treatment. Five Eras in the Development of Auditory Implants • Forerunners • Pioneers and experimentation: 1957–1960s • Feasibility studies, safety studies, evaluation of auditory gain: 1970s • Development of the commercial multielectrode cochlear implant: 1980s • Development of the auditory brainstem implant: 1990s Interest in stimulating hearing by use of electricity started in 1790 when Alessandro Volta, an Italian physicist and Count of the region of Lombardia in northern Italy, stimulated his own auditory system by connecting a battery with potential difference of ~50 volts to two metal rods that were inserted into his ears. Apparently he heard a “boom” within his head and then a hissing sound as if “soup was boiling”; he found the experience quite uncomfortable and moreover it lacked tonal quality! Volta had had an interest in electricity from a very young age and at the age of 18 years was already corresponding with various academics and scientists on the subject. He invented the electrical battery (the voltaic cell; 1800), and also developed an interest in chemistry. He was also the person who discovered methane in a nearby swamp as a gas that could be used as fuel (1778). The son of a family of nine children who was expected to become a priest like five of his siblings, Alessandro Volta contributed significantly as a scientist and became professor in physics at the University of Pavia in 1779 and professor in philosophy at Padova (Padua) in 1815. In his recognition, the unit of potential difference (or “voltage”)—the volt—has been named after him since 1881. Some other experiments involving electrical stimulation of the auditory system were described in those years, all without tonal quality of sound. In 1855 Duchenne of Boulogne (1806–1875), based in Paris, stimulated the ear with alternating current instead of direct current, leading to a sensation of “the beating of a fly’s wings”; still an unsatisfactory outcome. Several discoveries in the early 20th century (around the 1930s), the era of the development of the telephone, influenced the final development of the auditory implant.1 Working as a researcher at the Bell Telephone Laboratories in New York, Dudley described and designed in 1939 a real-time voice synthesizer that produced intelligible speech. The fundamental frequency of speech, the intensity of its spectral components, and its overall power could be extracted using a specially designed circuit. This synthesizer was named the vocoder (encoding the voice) and its operating principles formed the basis for the early speech processing schemes in auditory implants.2 In 1930, Ernest Glen Wever and Charles Bray recorded electrical potentials in the cochlea that reproduced the sound stimulus and described this phenomenon, which was later called the Wever–Bray effect.3 These experiments were performed on a cat, with an electrode introduced into the auditory nerve. While they thought to record the discharges of the auditory nerve, following the “telephone theory” (signals carried along the cable of the ear, i.e., the auditory nerve), in fact it was the cochlear microphonic that they recorded, produced by the outer hair cells of the cochlea. The telephone theory was later rejected, but Wever and Bray did inspire several CI pioneers.4 In the 1930s Stanley Smith Stevens and his colleagues described “electrophonic hearing,” thought to be the mechanism by which cochlear structures respond to electrical stimulation to produce hearing and therefore only present in intact cochleae. It is now known that electrophonic hearing is a result of the mechanical oscillation of the basilar membrane responding to voltage changes. Before 1957, efforts to stimulate hearing electrically were performed on patients with at least a partially functioning cochlea. The results therefore could have been based on electro-phonic hearing instead of on direct stimulation of the auditory nerve. The early pioneers had to prove the effect of their auditory implant as being a result of stimulation of the auditory nerve and not electrophonic hearing. The first direct electrical stimulation of the auditory nerve was performed in the 1950s by the French-Algerian surgeons Andre Djourno (1904–1996) and Charles Eyries (1908–1996). They each had a different interest and background. Whereas Djourno was a scientist who had studied medicine, and had been interested in medical applications of electricity and nerve stimulation for a long time, Eyries was a trained otolaryngologist and was more of a clinician. He had more interest in the facial nerve embryology and function and ways to restore this function. Before they worked together, Djourno had already produced a device to continuously measure pulse, had used electroencephalogram to study narcolepsy, and had originated the use of electricity for removal of metal pieces from bones. He also invented some sort of artificial respiration by direct stimulation of the phrenic nerve. With these inventions he showed his deep interest in neural stimulation by the use of prostheses. His next project of interest was the making and testing of “implantable induction coils” (which he called “microbobinages”). He made these coils himself to use for “telestimulation,” and tested them in rabbits. The induction coil was placed under the skin and the stimulation was delivered transcutaneously. Subsequently, the rabbit jumped after stimulation of the sciatic nerve. He studied several aspects of telestimulation, among which were electrode biocompatibility, the effect of long-term telestimulation of a nerve, and the effect of stimulus frequency. Because with higher frequency the muscle did not contract and with lower frequency the contraction was painful, he determined that the ideal stimulus frequency was around 450 Hz, finally using his own voice as the telestimulus because it was of the appropriate frequency. This finding might have prepared him for the idea of stimulating the auditory nerve to restore hearing. A 57-year-old male patient, who presented postoperatively with both bilateral loss of hearing and bilateral loss of facial mobility after bilateral resection of large cholesteatomas, brought Djourno and Eyries together. In the quest for a possible nerve graft, Eyries met Djourno at the laboratories of the medical school in Paris and they decided together with the patient on a surgical procedure with the purpose of restoring facial as well as acoustic nerve function. This procedure was performed on February 25, 1957, by Eyries, during which the induction coil was placed under the temporal muscle and the active electrode was placed in the stump of the auditory nerve, while at the same time the facial nerve graft was applied. The procedure restored facial nerve function by the graft. The reports on the auditory outcome, also partly tested intraoperatively, showed encouraging responses. After extensive rehabilitation with a speech therapist, auditory sensations were present; discrimination between lower and higher frequencies was detected, but there was no speech perception. Unfortunately, after some weeks the implanted electrode broke down, and a subsequently implanted second device also broke down. Eyries held Djourno responsible for the breakdown of the electrodes and refused to perform a third implantation; this marked the end of their working partnership and communication. Djourno, however, went on and was approached by a colleague who proposed to organize funding and engineering support for further development of the implant in collaboration with industry, in exchange for exclusivity. A true academician, Djourno refused; he was not interested in profiting from his inventions and detested industry. Out of principle he implanted one more patient together with a different, third surgeon. This implantation was not successful and because of lack of funding Djourno finally stopped working in the field of auditory prostheses. The activities of Djourno and Eyries were followed up in Paris by Claude-Henri Chouard, a student in Eyries’s laboratory. He became instrumental in the development of one of the first functional multichannel implants, the Chorimac-12 (now Neurelec/MXM-Oticon). He considered Charles Eyries as his major source of inspiration. Because of the work and implantation of Djourno and Eyries in 1957, that is seen as the year in which the development of auditory implants started. The discoveries of Djourno and Eyries did not reach the outside world or the United States quickly: they published their findings in a French medical journal and the connections of Djourno, the scientist rather than the surgeon, with American otolaryngologists were not close. Eyries, the clinician, on the other hand did not show much enthusiasm for the project and that only briefly. William House in California serendipitously was given a translated English summary of the French manuscript via a patient around 1959. The manuscript was very positive on the findings and House became inspired. At that time, House (1923–2012) was a young dentist-turned-otologist who had completed his residency in 1956 and had just started working in Los Angeles at the Otologic Medical Group, with his half-brother Howard House. Although early in his career, he had already made some significant contributions to the field of otology/neuro-otology by developing the facial recess approach and later he also developed the middle fossa approach. Bill’s bar at the fundus of the internal auditory canal, a landmark for the identification of the labyrinthine segment of the facial nerve, carries his (nick) name. When the manuscript from Paris reached him he was working with John Doyle, a neurosurgeon, on the middle fossa approach. They were working on recording the cochlear nerve response to sound, associated with tinnitus. James Doyle, the brother of John Doyle and an electrical engineer, took charge of the electrical recordings during surgery. Having found success recording sound-induced potentials from the cochlear nerve, they planned on stimulating the nerve to restore hearing. They first experimented with electrical stimulation to restore hearing during stapes surgery, using promontory or opened oval window stimulation. This was successful: these patients reported that they were able to hear the stimuli. This stimulated the researchers to implant a patient with a device. The first patient who consented to being implanted was a 40-year-old man with severe deafness due to otosclerosis. Promontory stimulation of the right ear showed responses on January 5, 1961, and 4 days later a gold wire electrode was inserted under local anesthesia via retroauricular approach through the round window into the cochlea. The electrode left the ear via the retroauricular skin. The patient heard electrical stimuli but had poor tolerance of loud noise. After several weeks the implant was removed. A second patient, a woman suffering from congenital syphilis, followed during the same month: she also heard the stimulus. Eventually the wire was removed because of fear of infection. Hoping to be able to produce and study discrimination of the higher frequencies, the first patient was implanted again, this time with a five-wire electrode: the results were not very encouraging and this device also needed to be removed because of risk of infection. This problem with the biocompatibility of the materials used presented a major concern for the follow-up of their experimental procedures. The thought behind implanting a five-wire electrode was to be able to spread the high-frequency stimuli along spatially separated electrodes; subpopulations of nerve fibers would thus be stimulated, summing to a high-frequency response along the whole nerve. An early patent application for this multielectrode implant was submitted by James Doyle and Earle Ballentyne in 1961. The patent process took time and it was only accepted in 1969. The supporting theory, however, was proven to be wrong because the same signal was applied to all electrodes and thus did not lead to pitch discrimination.
1.1 Forerunners
1.1.1 Alessandro Volta (1745–1827) (Fig. 1.1)
1.1.2 Homer Dudley: the Vocoder
1.1.3 Wever and Bray: the Cochlear Microphonic
1.1.4 S.S. Stevens: Electrophonic Hearing
1.2 Pioneers and Experimentation
1.2.1 In France
Andre Djourno and Charles Eyries (1957)
1.2.2 Early American Years
William F. House (Fig. 1.2)