SURGERY OF THE EAR “halt a million dollars per child for corse ines th Fhe eost of eoclcar implantation ‘ives (ranging, from $40,000 Inlewr tpn Iassromt, th cont pay allot the casts of ‘sant recipients, Cuttent evidence han Lautat Wchikren wont fonnse and thet 2 ynaintiean «Lass iets ane npfoyanent Fates seC0 pena eating fos ea) Sjeapre ns plantation, vot cocoa implantation nal Association for the Deaf (NAD) isan onganization ° eve iy “teat culture.” According He deat vant to be seat and af beatiness a8 ‘a individuals live and fempts to eliminate vent genocide.” sis use American Sign from jost party deaf culturalis Lan ASL). ASL isa distinctively sifferent ei geammatical structore, Faility English with an entirely A aie inte fest) with even written English a at ASL is “natural” communication shat (dhe basis for this claim of “naturalness” is unclear) for Sperchildren and that spoken, written, or even signed English sec Snaturals” Deaf culturalists believe that deaf children are | members of deaf culture, and to restore their hearing is, to deny them their natural birthright." ‘Deaf culturalists, while claiming on the one hand that deaf- ness ie nota disability, derive support and benefit from billions ef dollars in disability benefits. Insofar as cochlear implants sHfective, deaf individuals who decline to use them have an lective disability.” Tucker has questioned the extent to which jndividuals with elective disabilities may call on society to pro~ ‘ride supportive services and accommodations." ‘The strongest advocates of deaf culture are quite explicit bout the implications of their views: hearing parents have no, “right” to make decisions about their deaf children if those decisions might result in hearing restoration. That right, they Claim, belongs to the culture to which they “naturally belong” ‘and should be made by deaf culturists on their behalf.” Some of these deaf culture activists regard cochlear implants in chil- dren as a form of “child abuse.” These issues have been care~ fully examined by Balkany and colleagues with the following However, the arguments of these leaders are internally contradictory: They hold that deafness is not a disability but upport disability benefit fr the deafs they maintain both that cochlear implants do not work and that they work-so well that they sre “genocidal” (ie, they will eliminate deafness), Their position fe ethical principles of beneficence and autonomy as ‘opposes th on and privacy. Ethical standaeds they relate to self-determi soars est its the cid presides he nett of weiner iar hve the respon areca ei eis est meres" Cost Effectiveness studies use the 1@Uality-adjusteantine” effectiveness al Most cost diane the life expectancy of cochlear imp) vel oschuange as a result of implantation, any ‘result directly from improved health tility. Heal wt be measured using a variety of differ. ses ineluding a visual analog seale and the Health 2) ‘These scales have been used commonly iveness of cochlear implants. The time teen used only recently to assess the of perte tents isnot antici increase in QAI Utility Index-2 (1 ins assessing the cost ef traule-otf instrument ha ‘oflife for children. ni colleagues compared 45 postlingually deafened i cochlear implant users with 46 deaf candi- srincon the waiting list for cochlear implants. Three health- tise quality of life instruments were utilized: a specially Jeveloped cochlear implant questionnaire, the SF-36 health Status questionnaire, and the HUI-2, All three questionnaires Getected improvements in health-related quality of life attrib utable to cochlear implant use."* in 1999 Palmer and colleagues prospectively evaluated ‘cost effectiveness in 62 adult cochlear recipients. Adults who cectived the implant had a health utility gain (using HUT) of 6.2. Ninety percent of the gain occurred within 6 months of implantation. Using standard models, a 0.2 improvement in health utility resulted in a cost of $14,670 per QALY. Cheng, and colleagues published a series of articles assessing the cost, Effectiveness of cochlear implants. In their studies, half of the Substantial loss of health utility (0.6) is recovered by a cochlear implant. Based on their estimates, the cost per QALY for 2 pediatric cochlear implant recipient varied between $5,197 tnd $9,029 depending on which survey instrument was used. Generally speaking a cost of $20,000 to $25,000 per QALY is considered a cost-effective intervéition (ie;“a good deal”). For example, placement of a defibrillator has a cost of $34,836 per ‘QALY. total knee replacement is $59,292 per QALY. Cochlear implantation has been demonstrated to have one of the highest cost-effectiveness ratings of any current intervention. Overall, including indirect costs such as reduced educational expenses, the cochlear implant provides a savings to society of $53:198, per child.*-” quali Krabbe adult multichamn @ PREOPERATIVE EVALUATION Initial screening for cochlear implant candidacy in postlingually deafened adults begins wi netry and speekCHAPTER 95; CoCr EAR IMPLANT de nd Equilibrium of the olaryngology—Head ' American A and Neck Surgery has recor nal candidacy determination beeen ee IN’ smnmended crmination be made using Hearing in esting and Morphology of cochton and samicireular jeal candidacy purposes, nut the dlaptive mode Patoney of cochloar duct * to assess results amd comp, laptive mode is use ompare outcomes, The CNC test is. | Status. of eoshloae norwe—, . open-set word recognition test that hay th ple vontanze pevforimance-meanirements re ak! poniern, | Blestol ne media > R ool Dofoct of eribiform area bee Eniargod vestibular aqueduct 2A ++ That testing chitaven with pre : ng children with prelingual hear- | presence of round or oval window oad special tests. Indeed, in the early years of cochlear implanta. [GUS abacemalites tlon, adequate tests for assessing cochlear tmplamt seldegy in besingal children had to be developed. Existing tests were has become clear that MRI is the most sensitive technique inadequate, hi fvinyeeaaiy labyrinthitis ossificans, Even high-resolution: ‘The Early Speech Perception (ESP) test assesses spesch CF scanning may miss cochlene obstruction in up te SO ef perception ability and is available in both a low verbal and 7 : whic seanning cannot detect standard vemion, Speech perception ability i divided int hres Fe Cpetrction until frank ossification has developed Sutests toassess the child's capa ; pessence or absence of a assess the child's capac Omthe other hand, M esence speech (“ball” versus “cookie” versus. “ise fluid signal within the labyrinthine bone. ‘Consequently, any- cream cone Te Dear ainmnates aid trom within the cochlea eae palk- notably anossiied fans tissue sil oS bla abmnemaline!"| Consequenty, MRI has ‘eal de epost modality of choice forthe detection of vSumeningiic endocachlear obstruction" @ Hee naz imaging can-demonsteat{ an absent Posilinguatly deatened children are asuires as aul oped some anguage dlls. A numberof other tests are also used Tethe evaluation of young and prelingvally deafened children, including the Craig Lip Inventory, the Meaningful Auditory Integration Scale (MAIS), and the Infant Taddlsr MAIS. This or hypoplastic cochlear nerve, Using currently available ‘estingis specialized andeequisesa specially traingd.clipisian. 1'5-T magnets, precise measurements of the size of the cochlear Medical Evaluation uit more exact quantification of cochlear nerve diameter” Once it has been determined that a person is a good audio- Since cochlear nerve diameter appears to correlate with the logic candidate, a medical evaluation is necessary. The medi- niimber.o-surviving spial gs anglicn cells quanbiicanan ache cal evaluation should determine that a candidate can undergo sizeof the cochlear nerve ma} wediction of the operative procedure with acceptable risks. Radiographic posupeineotcomeaftes cochlear En imaging of the temporal bone should be obtained inorderto cochlear nerve is ope of the few absolute contraingiations ‘0 daar ee atentiglanatomic variations that mightcontasin. cozhlearimplantation, Although small imernal auditors cana) dicate the operation or rea Theusualaugical on CT scan-auggests an absent cochlear nerve, absence of the peacednte, coils necvecan define be tered on Mh eal saat nally, radiographic evaluation of cochlear implant agittal reconstructions through the infernal auditory canal candidates has been performed using high-resolution com- (Figure 35-1). putefized tomographic (CT) scanning, Recent ef ‘Defects in the xibeit ofthe cochlea, which present cee Te eeaeaar imeging (MI permit greatly enhanced heJikeihood ofan intraoperative “gushen” can be identified a aa ee Mie in also very useful in the preopera-” MBLscanaing and warn the surgeon about this potential diffi- RES Mtl cocblear implant candidates, There are pros culty. Central nervous system (CNS) abnormalities that could Gnd cons to both techniques. (Table 35-1) Each technique a affect th of implantation can also be well is capable of providing important information not provided identified by the other. Some thought should be given to the individual High-resolution CT scanning, however. permits more com= patient's circumstances and the potential difficulties that may pl ete characterization of hypoplasia, a incomplete beencountered in that individual before the decision is made partitioning defect nity) (Figure 35-2 as to which type of scan should be requested. Sometimes both ai larged vestibular aqueducts, High" 8 types of imaging will be needed. CT scanning permits fairly precise mapping of the fallopianINE 95-1 + Satta raconatvctn ermal aucitry canal showing an In and arietation of thane al auditory canal a ay 5-2 + Axial section on the right demonstrates Genal and facial nerve. A clear understanding of facial nerve 2patomy Sletde e paoclosicas Evaluation A pefcliouocial evaluation $ no t evaluation of the pediatsie patient bun sh Fa routine part of the iould be considered in special circumstance Paychosoci tion i6 0 determine the intellectual ability ofthe eh lish the child's expectations for postimplantp identify fasnily issues that may affect in plant performance. 1 the child's intel mal, the goals for the child, along with ex be scaled back. Rehabilitati Tenia may be achieved more slowly. Iscvere 1 prof Se The purpose of evalua estah- formance, Plantation oF post ‘@ severe cochlear malformation in this case a common "y. (white arrow), There is no internal auditory canal visible through this section The image Lore 1s small canal present that is just large enough to ‘ular nerves are absent as isthe vestibule and semicircular canals, onthe lett ‘accommodate the facial nerve, The cochlear results, the psy pathologist and audiologist, hhas developed th ge. Intensive therapy can be directed towa ‘ular deticits to help him/her develop the ne essary skill set, ily issues that may atfect the success of the impla ital stress, depression, or abuse must be Ment For example, an lolescent well integrated into asig tunity may age toan implant as a result of parennerve, A nderstanding of facial nerve vy i especully imPOrtani in those persons with tempo be developmental anomalies (eg, dysplastic emiirculor that increase the likelihood of anomalous facial nerye ab | ological Evaluation evalustion of the pediattic patient but should beg aN special circumstances, The paras af eeeee to determine the inilectan ai arin a athe child's expectations for pouiinglon rene idensify family scones shat may plant performence, the cuit’ ntelty abe goal for he clang Jed back. Hehabilvative milestones sncial e c part of the considered in affect implantation ‘Axial section on the right demonstrates a severe cochlear malformatica "y (White arrow), There ts no internal aultory canal vi ‘sere fa small canal present that is just large enough to a ‘nerves are absent as is the vestibule and semiciecular figuration andl orertat i the internal auditory cara ate ater ann, 1 0n tha irathy Baty e through ths section. The mage on the left a ceommadate the facial nerve: The secninas canals th or the appropri havior to emerge. Intensive therapy can be directed towa the child's particular deticits to help him/her develop the n esnary skill et, Family issues that may affect the success of the For example, an adolescent well integrated into asi ee to an implant as a result of parett CHAPTER 95: COCHLEAR IMPLANTS IN ADULTS AND CHILOREN + 587 a pressure when he/she really does not want one. Ifimplanted, he! Shes likely to become a nonuser. - suit somettMeS EATicant problems of familys interaction ate reedgnized that can be Improved with appropriate therapy. Such therapy often continues long afer the implant has been placed and successfully programmed gssesesd rue ics/can’significantly enhancesa child's ability to become a suc- cecful implant usr. Perhaps the most imp lint part of the psychosocial eval ation is to assess both thegeeipient’sand his/her family’sexpec- tations for the device. Almost nothing creates more trouble in, the postoperative period as do unrealistic expectations on the ppart of cochlear implant recipients or their family members. The evaluating clinician must assess both the qpen and the “hidden” expectations of potential recipients and or thei families for the device. If expectations are unrealistic, they should be modi- fied prior to implantation. When expectations are realistic, the chance af disappointment, anger, and rejection of the device is _gfcatly diminished. ~ hhas changed, As experience with cochlear implants has grown, handicapped Candidates ihe assessment of multihandicapped individuals can be espe cially challenging. Handicaps most commonly associated with Congenital hearing loss include mental retardation, vival gnd motor delavs, epilepsy autism, cerebral palsy altention-deficit disorder, and a variety of syndromic abnormalities meluding CHARGE assosiatinn (coloboma of the eve, heartanomaly,cho- HTSUS cetardavion and genital and eat anomalies), Usher's Tesinski and colleagues have evaluated 47 children who were implanted and had one ot more associated handicaps. Eighty- two percent ofthese children were succesfully programmed. Isaacson and colleagues evaluated five children with significant FIGURE 35-3 + The axlal section on the right shows the complete absence of the cochlea. There is ny ON lis bone whee the coches sould be Theresa completo absence ofthe eral auctory canal on Pots flat athe coronal section (mage onthe ight though 90 {on ese ims, the pant had normal acta neveunetoninleain hatte facia nerve was na ce of the nternal audory canal is apparent tenet worried the chide with iss cia rej cos well overall” Waltzman and colleagues es bute dren betwen the ages of 2a 12 Yeats a ees handleaps. hese children recived significant wnatdevopncnt was sower andes a6 E wa 7 ‘Cochlear implantation, children SRE ipl fandiaps shouldbe carefully evaluated byateam Soot nly te nnture o The handicaps ue a oeaingand scent weoTs coca og Which Ear to Implant “The selection of which eat to implant can be difficult. tn. the Sas generally selected. twas argued that che implantation ise vvfould destroy residual hearing (and it does in at least 30 % of Coy ond hot the beter Rearing ear should be conserved iy ‘case the implant did not work.” Over the years that philosophy confidence in them has increased. Experience to date indicates that the ability to eliminate patients who will not benefit from the implant has become quite good. Consequently, many pro- grams currently select the better hearing ear. Lis seasoned that the better hearing ear is ikely to have a higher population of ‘AREA ue clementsandt hence offer the possiblity of bei tbat of implanting the worse hearing eat, with the result that the tablent tov acheves-na isnt. tn such cases one i inclined 10 carhad been implanted, Moteoxer it itis now often believed that, if the residual hearing in the better hearing ear could provide enefit, then the patient would not be an appropri- ate implant candidate. Despite such reasoning, it has not been588 + SURGERY OF THE EAR Mh itative eadata,thyt at produces superior results. Teed, there icsomeevidevce tht results are just as good wl he poorer ear s implanted. especially ifthe difference between Mihough this may well be nll, the qu enve hotween the & when the diff sis Large. von coclilear implantation have shown reps ith a fairly high degree of reliability th tion of deafness, the worse the postoperative perfor- ) c2Bkate ston used to alec dhe cart recive The uty leatened eat is casen, STTone may guide side selection, If one a antl dysplastic or hypoplastic, the contealateral St O°P “THERETO cochlear pateney, when present, are often ive. While long-standing labysinthitis ossificans is initially may progress more rapid nth should TATA THS OTHE The Teast obstructed Tab —Sonictimes a previous procedure in one ear makes the other ear more desirable. Canal wall down mastoidectomy none ealing sar would make the scisamarked dfeencein vestibular fanc- nicanly reduced lnbyrinthing function on one tle If sa that side shouldbe chosen so aso peste hee oh thebeterseatbular Gh Ritinvane tae eee ere Ultimately, auditory information must reach the cerebral sprtex to be useful, Even when peripheral a is identical, there may be significant differences in the amount of (CNS activation obtained by stimulating ane side as opposed to the other. New techniques in brain imaging such as single-pho~ {on emission CT (SPECT), functional MRI, PET, and refined cortical auditory electrophysiology may allow differences in CNS activation to be identified preoperatively. Age of Implantation Cochlear implantation in children began in the second half of the 1980s under the close supervision ofthe US Food and Drug Administration (FDA). Iwas initially Timited to postlingually deafeied children beraus deb wh. This concern was addressed by Roland and co) position on serial postoperative radiographs. Children were gy Ihed ron 1 to 7S months and o change in electrode pongo as noted.” 5 hearea of speech and language dey lene, onan inte basis hat Youre lowly, evidence to support their intuitions is accumulating 3, anc nwccrtsted100chitren who ceed mers between 1 and 10 years of age. Growth curve analysis indicate that there was an additional value for earlier implantation oyee and above advantages attributed to length of use. They con cluded that there were clearly advantages to implanting childreg before the age of 2% years tl conto iconitent withthe information devel ‘oped by Sharma and her colleagues using long latency cortical: evoked responses. Maturation of long-latency cortical response, (as measured by decreased latency) occurs reliably when ch siren are implanted before the age of 3-1/2 years and occur, rarely in children implanted after the age of 7 years" Researchers at the University of Michigan have demon. strated that the postimplantation speech recognition scores of 48 seven-year-olds varied according to the length of time the child had been implanted. The longer the child had had the implant, the better the speech recognition scores. They ea, uated an additional 53 children 36 months after their implany had been placed. Holding the length of use fixed, they dem onstrated thi ildren’s performance as age at loreover, ata fixed post implant time of implant decreased. Mg ph ag children TpIGMETSA younger ages demonstrated better performance Soya oes ee colleagues have shown That postim. Plantation, the rate of expressive and receptive language earning approaches that of normal-hearing children. He was, however tunable to demonstrate “catch-up effects,” Consequently, youn, Ber age at implantation would leave a narrower gap between ormal-hearing and implanted children. Moogand Geershave shown that normal levels of language and reading are associated with earlier age of implantation.** Cheng and colleagues’ meta- analysis shossed th tapi gains in speech perception are associated with earlier age in implantation,” Conaisrsral have shown that there is i J iced about implanting older patients. Concerns about effectiveness and cost utility have been. Tabed loeph gulgacd tech eae Tes Boo about aise peli cel nelson: bab Data oh speech recngnition scores in elderly patien ognition, Data atients ihalihedace lire eck cla nels sa aS Suld have litlewlity for children with-congental esteg eae Seen mba tbat the dele 1S. Over the decades, the indications have expanded basedcy documented outcomes submitted to and reviewed by the FDA" age Of implantation has slowly been lowered from 2 years through 18 months to 1 year of age. Initial objections tg patients, = ‘A number of other concerns have been raised about the geriatric population. It has been suggested that they are at greater risk for soft tissue complications because of decreased ‘zuplamting very young children were partially based onthew>=, bjood flow in scalp tissues, related not only to-microvascn- lar disease but also to an increasing incidence of diabetes, «cived potential for electrode migration/extrusion secondary ty’ See ESst ¢ st CHAPTER a5: COCHLEAR IMPLAN!9 " idi- Se ‘ stimulating the audi (AZ FHowevet no such increase of soi issue complications hay bypassing dhe-coeblear hai eels an 5) pte we VE jeen verified 2 a ae ecmmtcatcns has bass Ay an srncbronousio™ ae promptly stibular injuries, it has been hypothesized Teggutes have reported thatth ; an 37 Garheimps ablation and fling could be disproportionately severe in. an elderly population, from an average ents with auditor {fir postvochlear implantation in four patier th Y So data have been produced to support this concerns and Mats andealeagics have shorn vo deren i beep fal atays between geriati Patients. I enough Ceetibolar function was destroyed to affect ambulation, one proud expecta delay 1 scharge. It his been noted Mone in both the deaf and the chery. and it has heen spec areied that the combination of both could Success! rehabilitation sera stadics have evaluated the effectvenes semplonts i the elderly, Labadie and coleagu touted dht both geriatric and younger patients have statistially Significant increases in Central Institute for the Deaf (CID) SETCNC scores (there was no difference between groups) Sutistction with the device has also been cemonstrated as increased self-confidence and improved quality of fife.” te against Candidacy Guidelines [As cochlear implants have achieved documented improve iments in open-set speech recognition scores, FDA guidelines for implantation have been expanded. AL first,FDA guidelines suggested that potential recipients should have pure-tone aver- ‘ages (PTAs) of 90 dB or greater, The guideline has been Jow- ‘ered to 70 4B in recent clinical trials, It was initially suggested ‘By the FDA that appropriate implant candidates should have ee of ess than 2006 m-auist THs cre rion has now been substantially relaxed, and individuals with. less than 50% correct responses to HINT sentences in quiet are considered appropriate candidates. It is worth emphasiz- ing that FDA-approved criteria are guidelines and do not con- strain an experienced implant team from making thoughtful exceptions. There is a move toward using CNC words as a cri terion, primarily to avoid ceiling effects during postoperative valuation. Auditory Neuropathy ‘Auditory neuropathy (auditory dys-synchrony) is a recently {identified type of sensorineural hearing loss. [tis defined as Lae Nasnl a ae delays amd Interaural_intenss es 10 localize gc sain Noma earsienescanatetaclulearL eitsorms of posta wed detectable waveorTs ST pba ern stimulation.” Salon 2 a en reso cca seplanation it Wty neuropathy at event follow i rents in sound detection, 5] yeech, Shllop and clleng tea five children with aud ‘lL badsis perception, terpreted the pres xi nilication that synchrony was at least telemetry (NRT) as am leat partially restored, Otoacoustic emissions remained 79 the secur bat were eliminated in the operated ear 2 contralate implantation.** Bilateral Implantation ; venene aie deverat potential benefits from using, 0 implants” 1. Bilateral listeners bet at head ado any ie a with diferent signal-to-noise ratios (SNRs). ilaveral listeners can pick the ¢ the best SNR and. 2. Bilateral list vith bat SN ¢_y ‘enhance theix ability f b s ; fzsmes apparent in noisy environments, in which indi- ae aeee with unilateral hearing experience greater difficult in speech understanding. 3, Unitateral hearing makes sound localization almost impos- 2 degrees of difference in the origin of a sound signal. Ithas ‘been documented that ilateral implant users can s8i0 38° nificant sound localization using both time discrimination ‘ind interaural intensity cues. I-has been demonstrated that 7 four of eight bilaterally implanted patients, which shoul ‘be adequate for 10 degrees of angle resolution in a free-feld cefvironment. One patient had a 25 us resolution, Normal- hearing patients have, at best, 9us of resolution. On average, itis about 15 us, The extent to which improved sound locali- ruditory brain stem response (ABR) waveforms are absentin ‘S| the context of normal middlecar functions, Itis hypothesized charging dys-synchronously, such that no identifiable action fotentiat develops in the cochlear nerve. Hearing loss in this Eondition variable, perhaps because of variable degrees. of oF638 + SURGERY OF THE EAR it ‘The facial nerve is located incision with a cervical extension. located c 1e-stylomastoid foramen after raising a sol fe ne coecaud he goa rye is identified 1¢ flap over the parotid; thi nerve is id d aoe ee hypoglossal ures partially ames ya ally. The hypoglossalnerve is partially transected in preparation for grafting i raft ngerthan_the gap is harvested.’ After preparing the graft it lone ¢ to the proximal portion of the transected hypoglos- sal nerve in end-to-side fashion followed by end-to-end anas- tomosis of the graft with the distal facial nerve as described above. Recovery of facial movement begins at approximately ple procedure that provides strong neural input and results in \cceptable-dynamie-funetion. The disadvantages of this tech- nique include mass movement and potential tongue hemiat- rophy, which rar sin chewing, swallowing, or speech difficulties. The Vike omosis is preferable to the facial to spinal accessory nerve anastomosis. If there is no functional neuromuscufar svsteit, Siirgical struction 3 op father than Panay nett rept er RRR a tha = Avvaricty of transposition procedures have been described, including trans- position of both iclec lis, mas: ‘and free muscle (grgeilis-rectug al ius, abductor is isminor, cSt sed. Free guuscle transfer withaneore ean et omosis (using the contralateral facial nerve fo: he mainstay of treat i 01 7 (such as Mabius’ syndrome or Goldenhar’s syndrome).’ The crdss-face Kraft usually interposes the sural tween the distal buccal branch on the functioning si jonfunc- tioning side; however, variati ist. Unfortuy = cle transfer procedures are of limited effectiveness. Muscle transposition most commonly employs the tem- poralis muscle because of its good location, length, contractil. ibys and vector of pull. The masseter and digastric muscles are Used less often. The temporalis muscle is good for reanimatins pf the mouth in patients with long-standing (at lease 1 year in length) paralysis.” It isa proven and useful technique for facial Substitarion 28 Patients in who nerve grafting or cranial nerve substitution procedures are not possible. Tt hes ala been used gold eeiarn On with other procedures, such as placement ara gold weight in the upper eyelid. Temperance pacement a 3 dynamic technique that allows patients to Inve a voluntary ome Sate aap this techn ie versions ofthis technique eee or fascia lata, which ji attached to t] - faci iii atlacheo the SOO itaneoush louth to support the i 4 relaxation of the tissues with eee, iy Sa aoe Fey Although static slings afford relative symmetry at rest, they provide ne movement. © ADJUNCTIVE MEASURES The patient and the surgeon must realize that, unfortunately, Snot achieve perfection in