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Cochlear Implantation Joseph L. Russell, MD Faculty Advisors: Dayton Young, MD and Tomoko Makishima, MD,PhD Department of Otolaryngology, University of Texas Medical Branch Grand Rounds Presentation October 29, 2012 Overview History of cochlear implantation Current implant systems Pre-operative workup and planning Otologic Audiologic Radiologic Overview Surgical Approach Complications Outcomes Recent advances Bilateral cochlear implants Electrical and acoustic stimulation (EAS) Future directions History of Cochlear Implantation 1800 Volta (Italy) 1957 Djourno and Eyries (France) Implanted a coil electrode in a patient who had bilateral deafness and facial paralysis from cholesteatoma surgeries High/low frequency discrimination; few words understood 1961 House (USA) Implanted three patients with single electrodes into the scala tympani through the round window; infection prompted early removal within weeks in all patients Auditory results similar to Djourno and Eyries 1960’s basic science objections Dynamic range of electric stimulation (10 dB) vs normal ear (120 dB) Insertional trauma Neural degeneration from electrical stimulation History of Cochlear Implantation 1967 Simmons (USA) Demonstrated in cat models that: Electrodes could be inserted atraumatically Long-term electrical stimulation did not lead to any significant neural degeneration in the cochlea 1972 House (USA) Developed (with 3M Corporation) the first FDA-approved single-channel cochlear implant Some improvement in speech discrimination, improved voice modulation, ability to hear environmental sounds No open set speech discrimination Over 1000 devices implanted from 1972 to mid-1980’s 1978 Clark (Australia) Implanted the first multi-channel electrode array Some open set speech discrimination obtained 1985 FDA approval for multi-channel implants in adults 1990 FDA approval for multi-channel implants in children 2-18 years old 2000 FDA approval for use of cochlear implants in children as young as 12 months old Current Implant Technology Three companies currently have FDA approved implants Advanced Bionics (California) —HR90 K Cochlear (Australia) —Nucleus 5 Med-El (Austria) —Sonata ti100 Current Implant Technology General design of cochlear implant systems Sound is received by a microphone located on the BTE sound processor (1); it is processed and coded, then sent via the transcutaneous radiofrequency link to the implanted receiver-stimulator (2); data are decoded and sent to the multi-electrode array (3), stimulating spiral ganglion neurons, which then transmit the signal via the auditory nerve (4) toward higher processing centers Current Implant Technology Special electrode arrays A—cannot see here, standard size with electrodes distributed over 26.4 mm B—compressed array with electrodes distributed over 13 mm C—medium array with electrodes distributed over 21 mm D—split array, one with 5 pairs of electrodes, other with 7 pairs—for severely ossified cochleas E—thin and shortened electrode array for EAS F—common cavity electrode Candidate Selection and Preoperative Evaluation Adult selection criteria Best-aided scores on open-set sentence tests of <50% in the ear to be implanted and <60% in contralateral ear For Medicare patients, <30% in the ear to be implanted and <40% in the contralateral ear Failure with conventional hearing aids No evidence of central auditory lesions or lack of auditory nerve No evidence of contraindications to surgery in general NOTES: Hearing level used to be used as criteria, but recent shifts have moved to focus on speech discrimination, as these scores most accurately reflect the patient’s disability Pediatric selection criteria Patient age 12 months to 17 years 11 months Lack of auditory progression with minimal benefit from hearing aids (after 3-6 month trial) In children <2 year old, determined by lack of auditory milestones In children ≥2 years old, scores of <30% on single-syllable word tests (MLNT/LNT) Profound SNHL with unaided pure tone average of ≥90 dB HL for children 12 to 24 months old and ≥70 dB HL for children ≥2 years old (reference points, not strict criteria) No evidence of central auditory lesions or lack of an auditory nerve No evidence of contraindications to surgery in general NOTES: MLNT = Multisyllabic Lexical Neighborhood Test LNT = Lexical Neighborhood Test Absolute contraindications for CI Cochlear aplasia Absence of the auditory nerve Otologic assessment History Onset and progression of hearing loss Etiology of hearing loss History of amplification use History of meningitis Ear infections—past/current Previous otolgic surgeries Exam Active infection Perforations Tympanostomy tubes NOTES: Preferable to remove ear tubes and close perforations prior to implantations, though implants have been performed in these conditions without adverse events Audiologic assessment Adults Unaided and aided thresholds for pure tones Minimum Speech Test Battery (MSTB) Used at many cochlear implant centers to assess pre and post- implant performance Set of compact disc recordings for standardization Includes the following: Consonant-Nucleus-Consonant (CNC) Monosyllable Word Test Arizona Biomedical (AzBio) Sentences (in quiet and in noise) Bamford-Kowal-Bench Sentences in Noise (BKB-SIN) HINT sentences were previously part of the MSTB but have fallen out of favor due to ceiling effect Audiologic assessment Children ABR and OAEs Implant candidates typically have no response at limits of the testing equipment Findings/implications in auditory neuropathy Unaided and aided thresholds for pure tones Speech perception tests Meaningful Auditory Integration Scale (MAIS) Early Speech Perception (ESP) Test Lexical Neighborhood Test (LNT), multisyllabic LNT (MLNT) NOTES: Auditory neuropathy—all must have MRI due absence of auditory nerve in 16% MAIS—questionnaire for family of children too young to participate in speech perception tests ESP—word is spoken without visual cues, patient selects correct object or picture of the stimulus LNT—50 monosyllabic words, ranging from “easy” (high frequency, few lexical neighbors) to “hard” (low frequency, many lexical neighbors); MLNT 2-3 syllable words; open-set test Imaging: CT vs. MRI High-resolution computed tomography (HRCT) Traditionally the gold-standard imaging modality Superior visualization of the bony structure of the otic capsule and the course of the facial nerve Weakness: can miss cochlear fibrosis, retrocochlear pathology, CNS abnormalities, and cochlear nerve hypoplasia/absence Magnetic resonance imaging (MRI) More effective at identifying cochlear fibrosis Able to identify presence/absence of cochlear nerve and caliber Weakness: inferior visualization of bony anatomy, particularly of the fallopian canal; inability to detect the presence of the round window, oval window, or an enlarged vestibular aqueduct; often requires anesthesia for young patients Imaging: CT vs. MRI In recent retrospective studies, MRI has been shown to be both more sensitive and specific than CT in identifying inner ear abnormalities that affect surgical planning MRI is now the preferred imaging modality in some centers HRCT is still advocated in cases of malformed external canals, semicircular canals, or vestibule due to the high incidence of an anomalous facial nerve in these patients Vaccination Children with cochlear implants are at higher risk for meningitis, though overall rate is low (<0.6%) Streptococcus pneumoniae has been the most common organism isolated in the children with cochlear implants who developed meningitis Current vaccine recommendations: Patients <2 years old Prevnar (7-valent) only Patients 2-5 years old Prevnar and Pneumovax (23-valent) Patients >5 years old Pneumovax only Additionally, all patients <5 year old should receive the Hib vaccine Vaccination should be completed at least 2 weeks prior to surgery Surgical Approach Surgical Approach Transmastoid facial recess approach Continuous facial nerve monitoring Skin marking with dummy sound processor and transmitter • • Incision is modification of standard post-auricular incision with a posterosuperior extension to provide exposure to seat the receiver-stimulator Methylene blue can be injected with 18g needle to mark position of the receiver-stimulator package Placement of incisions 1. 1. Postauricular crease, 2. skin incision, 3. periosteal incision 2. Must have at least 1 cm between edge of incision and receiverstimulator 3. Carry incision to temporalis fascia superiorly and to mastoid periosteum inferiorly Flap elevation Skin flaps are developed anteriorly to EAC and posteriorly to allow for placement of receiver-stimulator Exposure of the temporal bone Create a musculoperiosteal flap—incise temporalis fascia, muscle and periosteum vertically, then raise anteriorly to the bony EAC, revealing the spine of Henle; raise posteriorly to create pocket for receiver-stimulator Cortical mastoidectomy Superior and posterior margins are not saucerized to aid in containment of the electrode within the mastoid cavity Facial recess Use short process of incus as a pointer to define the level at which to open the facial recess; too medial= into facial nerve, too lateral= into canal wall; keep incus buttress thin to optimize exposure Round window niche usually visible just inferior to stapedius tendon—a small diamond burr is used to remove the lip of the niche to expose the RW membrane Cochleostomy vs round window insertion (RWI) • History—why RWI was abandoned— buckling of electrodes, cochlear trauma • Proponents for cochleostomy—”straight shot,” less chance of trauma • Proponents for RWI—less chance of scala vestibuli insertion, improved hearing preservation • Recent study showed no difference in hearing outcomes and complications between RWI and cochleostomy • Cochleostomy is made at the anteroinferior aspect of the round window; should be as small as possible Placement of the electrode array • • • • • Into scala tympani Insertional trauma must be minimized Do not force electrode array Cochelostomy is sealed with muscle or fascia after electrode placement At this point, only bipolar cautery should be used Implanting the receiver-stimulator • • A well is drilled in the calvarium to accommodate the receiver-stimulator—avoid dural compromise, especially in children Many advocate securing the implant with sutures to the calvarium, while others do not Final result prior to closure • • • Coiled electrode in mastoid allows for the 1.7 cm increase in the distance between the electrode array and receiver stimulator between infancy and adulthood Close musculoperiosteal flap, followed by deep dermal sutures to close the skin flaps, then close skin Standard mastoid dressing is placed and removed on the first post-operative day Complications Wound complications—most common, about 4% Infection, flap necrosis, extrusion of receiver-stimulator Placement of incisions relative to receiver Flap thickness—6 to 7 mm ideal Otitis media (2%) Damaged or misplaced electrodes (1%) Persistent CSF leak (1%) Facial paresis (0.5%) Complications Meningitis NEJM in 2003 showed incidence of Streptococcal meningitis in children with cochlear implants was >30 times the incidence in age-matched controls Study limitations 11.5% of children with implants had prior history of meningitis 8.5% of children had labyrinthine dysplasia Advanced Bionics positioner device, discontinued in 2002 Later studies in rats showed that cochlear implants do increase risk of meningitis; this risk was mitigated with Pneumovax vaccine Streptococcal and Haemophilus vaccination now required prior to implantation Revision cochlear implantation Rates Adults 5.4 to 7% Children 8 to 12% Reasons Hard failure (46% of cases) Medical-surgical (37% of cases) Wound healing, malposition, cholesteatoma Soft failure (15% of cases) In general, patients perform as well after reimplanation as their best performance prior to revision Outcomes Like beauty, are often in the eye of the beholder Challenges in tracking outcomes Benefits from cochlear implantation vary widely across individuals Study methodology and outcome metrics vary considerably Most studies are relatively small due to rapid changes in technology Outcomes Speech perception/spoken word recognition Adults, after 6 months of implantation Open-set word test scores 30 to 60%, up to 75% with most recent speech processing strategies Words-in-sentence testing scores >75% Children >75% achieve substantial open-set speech recognition after 3 years of implant use Implanted patients have, on average, language-learning rates that match normal-hearing peers >50% who use early education intervention exhibit age-appropriate vocabulary scores by kindergarten After 5 years post-implantation, implant users have a 75% rate of assignment to mainstream classrooms, compared to 12% of similar-hearing peers with hearing aids Cost Outcomes Cost-utility highly favorable in adults, better than knee replacement and heart transplant Cost-benefit highly favorable in children, with estimated net savings of $30, 000 to $200,000 per child if implanted at age 3 years Factors affecting implant performance Age at implantation -- the earlier the better, definitely by age 3, preferably by age 2 Duration of profound loss -- the shorter, the better Duration of cochlear implant use -- maximum benefit not seen until at least 3-5 years post-implant Training with amplification/early linguistic experience -- if some residual hearing present and used, results are better with CI Communication environment -- patients in oral only environment have better open-set word recognition than those in total communication environment Presence of other disabilities -- reduced performance in word recognition compared to patients without other disabilities, though benefits are realized in speech and language skills for those with other disabilities Family support Recent Advances Bilateral cochlear implantation A majority of centers are currently implanting the majority of children bilaterally Improved sound localization and understanding speech in noise have been shown in small studies Other potential advantages include more natural hearing, reduced listening effort, and improved quality of life Disadvantages: cost, potential exclusion from future innovations, such as hair cell regeneration Large multi-center long-term investigations underway Electric and acoustic stimulation For patients with residual low frequency hearing A shortened electrode array is inserted as atraumatically as possible into the cochlea A cochlear implant and hearing aid are then used on the same side A subgroup of 11 patients at the University of Iowa improved their average CNC word scores from 32% correct with binaural hearing aids to 75% correct with one implant and binaural hearing aids at 9 months post-implant Improved hearing in noise and appreciation of music over standard cochlear implant Future Directions Totally implantable cochlear implants In 2008, Briggs reported results of three adult subjects implanted with a modified Cochlear Corporation receiverstimulator that contained an internal microphone and rechargeable battery All had improved hearing results at 12 months However, implantees performed twice as well on CNC word scores when using an external (regular) processor compared to the fully implanted mode Swallowing and breathing also noted to interfere with hearing Robot-assisted/image-guided cochlear implantation Research groups in Hanover Medical School, Germany and Vanderbilt Percutaneous postauricular transmastoid access to the basal turn of the cochlea with either an image-guided frame through which a powered drill is guided (USA), or with an image-guided robot-controlled drill (Germany) Cadaver studies with 6 to 10 specimens have been promising, showing no facial nerve injuries; two planned stapes injuries and three planned chorda tympani sacrifices Foundation for minimally-invasive cochlear implants Robot-assisted/image-guided cochlear implantation References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 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Online. www.expertconsult.com Luxford WL and Cullen RD. Chapter 31—Surgery for cochlear implantation. In Otologic Surgery, 3rd ed. Eds. Brackmann DE et al. Saunders Elsevier, 2010. Online. www.expertconsult.com Gudis DA, Montes M, Bigelow DC, Ruckenstein MJ. The round window: is it the “cochleostomy” of choice? Experience in 130 consecutive cochlear implants. Otol Neurotol. 2012 Sep 11. [Epub ahead of print] Balkany TJ, Brown KD, and Gantz BJ. Cochlear implanation: medical and surgical considerations. . In Cummings Otolaryngology: Head & Neck Surgery. 5th ed. Ed. Flint PW, et al. China: Mosby Elsevier, 2010. 2234-42. Print. Kirk KI, Choi S. Clinical investigations of cochlear implant performance. In Cochlear Implants: Principles & Practice. 2nd ed. Ed. Niparko JK. Philadelphia, PA: Lippincott Williams & Wilkins, 2009. 191-222. Print. Lin FR, Niparko JK, Francis HW. 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Percutaneous cochlear implant drilling via customized frames: an in vitro study. Otolaryngol Head Neck Surg 2010;142:421-6.