Table Of Content

Cochlear implants in the United Kingdom: Awareness and utilization Chris Raine Yorkshire Cochlear Implant Service, Bradford Royal Infirmary, Bradford, UK Introduction:EverychildandadultintheUnitedKingdomwhofulfilsthecriteriaforcochlearimplantationis entitled to receive treatment under the National Health Service (NHS); children since 2009 are eligible for bilateral simultaneous implants and adults single implants unlesstheyhave additional sensory needs. History:Duringaperiodbetween1982and1990,whenanumberofindividualteamsranprogrammesusing charitable funding, the British Cochlear Implant Group approached the UK Department of Health, who agreed to set up a 4-year pilot study of 10 programmes, including one children’s programme. The outcomes were collected and analysed by the Medical Research Council’s Institute of Hearing Research. The results, showing positive outcomes for adults and children, were published in 1995 and subsequently funding was provided directly by theNHS. Access: Between 2001 and 2006 the Universal Newborn Hearing Screen (UNHS) was implemented in England and Wales and also in Scotland and Northern Ireland. Data from UNHS and also data from the three main cochlear implant manufacturers have allowed estimates of access to cochlear implants for children and adults within thecriteriafor implantation. Children: Between 2006 and 2011 the figures show that 74% of estimated eligible children aged 0–3 years havereceived implants and 94% by the ageof 17. Adults: For adults the figures are considerably lower, with only about 5% of those eligible for an implant actually receiving one. The reasons for this include, to a lesser degree, the fact that guidelines by the National Institute of Clinical Excellence (NICE) are stricter than in some other European countries, but chiefly because of lack of awareness among candidates and professionals, both of criteria for eligibility and of thepotential advantages from cochlear implantation. Keywords:Cochlearimplantation,History,Surgicalaccess,Adults,Children,Incidence,Universalnewbornhearingscreening Introduction History In the United Kingdom (UK) all adults and children In the UK the field of CI started from humble begin- who fulfil the criteria for cochlear implantation (CI) ningswithbasicresearchwithexternalcochlearstimu- are eligible for this treatment under the National lation in 1978. Six patients were fitted with the Health Service (NHS). They are entitled to be External Pattern Input system funded by the Medical assessed, implanted, and subsequently managed, Research Council. This had a spring-loaded electrode entirely free of charge. positioned onto the promontory that stimulated the WhileCIisnowanestablishedandrecognizedtreat- inner ear with an analogue signal from an in-the-ear mentforpatientswithseveretoprofoundsensorineural removabledevice.Thepatientseitherhadnotympanic hearinglossitisrelevanttoappreciatethe background membrane or had had the membrane surgically as to how it was established in the UK. It is acknowl- attached to the promontory. edgedthatfollowingDjournoandEyries’directstimu- During the 1980s attitudes to CI in the UK lationoftheauditorynerveinParisin1957,significant were ‘conservative’ strideswere made in the USA,initially in Los Angeles In 1982 the University College Hospital group (UCH) and subsequently in San Francisco. Transformation in collaboration with the University College of San from experimental treatment to actual clinical service Francisco (UCSF) implanted a multichannel device, took place when the US Food and Drug sadlywithoutmuchlong-termsuccessduetoreliability Administration (FDA)approved theuse ofthe House- factors.Subsequently,nofurtherSanFranciscodevices 3Msingle channelimplantsystem in1984. were available, while the San Francisco team explored options for commercial manufacturing of their device, Correspondence to: Professor C H Raine, Yorkshire Cochlear Implant which eventually led tothe AdvancedBionicsimplants. Service, Listening for Life Centre, Bradford Royal Infirmary, BD9 6RJ. Email:[email protected] The UCH team during the 1980s started to use the ©W.S.Maney&SonLtd2013 MOREOpenChoicearticlesareopenaccessanddistributedunderthetermsoftheCreativeCommonsAttributionLicense3.0 S32 DOI10.1179/1467010013Z.00000000077 CochlearImplantsInternational 2013 VOL.14 NO.S1 Raine CochlearimplantsintheUnitedKingdom Vienna single channel extra cochlear devices. Six over 70 years (Davis, 1989). Various reasons may be patients were implanted before manufacturing stopped ascribed to this – unwillingness to commit to evalu- which stimulated the UCH team headed by Graham ation, surgery and extensive rehabilitation. During Fraser together with Michael Conway to approach the selection it was recognized that physical fitness and Department of Health and the Royal National intellectual ability were factors. Institute for Deaf People (RNID – now known as In 1990 the FDA approved the use of the Nucleus Action on Hearing Loss). From charitable funding 22 in children 2–17 years. By 1994 111 children had Finetech manufactured 80 single channel UCH/ been implantedwithin the UK. RNID devices (Cooper et al., 1989). The team were The report from the Institute of Hearing Research: able to demonstrate that their first 30 patients treated ‘Cochlear Implantation in the UK 1990–1994’ by could gain considerable improvements in their lip Summerfield and Marshall (1995) was pivotal in reading and communication ability (Cooper et al., showingthatCIservicesdeliveredthroughmultidisci- 1989). All acquired useful awareness of environmental plinary teams produced effective outcomes for sounds with improvement in their qualityof life. None both adults and children. There was also a ‘learning achievedopensetwithoutlipreading. curve’ in patient assessment and selection, surgical By 1985 the Nucleus 22-channel implant, designed complications and rehabilitation. by Graeme Clark in Melbourne, was commercially Following on from the 1990/1994 programmes, available and the Food and Drug Administration various funding streams were established through the (FDA) of the USA approved its use for Adults in the NHS. The sources of funding were varied as the same year. The use of these implants in the UK NHS went through structural changes. Some local started in Manchester and Kilmarnock in 1988 with purchasers had policies of not funding adults, others several centres commencing shortly after, with had strict allocations. This not only let to frustration support from charitable sources. but developed a potential backlog of patients poten- Around this time the British Cochlear Implant tiallysuitable fortreatment. Group(BCIG)wasformedbymembersoftheexisting Annual numbers were initially collected by the cochlear implant teams, in all disciplines. One of its Medical Research Council Institute of Hearing initial functions was to attempt to persuade the UK Research (MRC IHR). Fig. 1 shows the annual and Department of Health to fund cochlear implants in cumulative figures foradults and children supplied to the UK. In spring 1990 the Department of Health the MRC IHR by clinical coordinators of CI centres invited bids from existing and potential service provi- from 1988/1989 to 2003/2004. ders to bid for a 3-year centrally funded programme Data were also collected by members of the for CIs for adults and children, using the Nucleus 22 National Cochlear Implant Users Association multichannel implant and with each programme (NCIUA) Table 1 shows continued growth in the implanting about 10 patients a year. The Medical numberof patientstreated (NCUIA, 2010). ResearchCouncil’sInstitute ofHearingResearchwas commissionedtoevaluatetheservices.Theevaluation hadthreegoals:(1)toestablishifCIcouldbeaneffec- tive routine treatment. (2) Assess initial benefits to young children and (3) derive recommendations for the future form and scale of services nationally. This ‘National Cochlear Implant Programme’ was also rolled out to Scotland in 1991 by the Home and Health Department in selected hospitals and by HealthandSocialServicesinNorthernIrelandin1992. In addition to the 10 funded programmes several teamscontinuedtoprovideCIservicesbyindependent charitable funding. Prior to the programme about 80 people had been implanted, of whom the majority had received the UCH/RNID device, most of the others the Nucleus 22-channel device. At the end of the programme nearly 300 patients over10yearsofagehadbeenimplanted.Itisinterest- ingtonotethatdespitethefactthatsome60%ofthose Figure1 MRCIHRcumulativeandannualdataofcochlear withaprofoundhearinglossareaged70yearsorolder implantationinchildrenandadults(courtesyProf.AQ yet only 6% of those implanted in the study were Summerfield). CochlearImplantsInternational 2013 VOL.14 NO.S1 S33 Raine CochlearimplantsintheUnitedKingdom Table1 CIsurgicaldatacollectedbyNCIUA Table2 BCIGSurveyNovember2011 Year Adults Children Adults Children Total 2005 239 332 HowmanyunilateralCIswereyou 4959 2525 7484 2006 292 375 supportingon31March2011? 2007 350 429 HowmanybilateralCIswereyou 186 1259 1445 2008 504 448 supportingon31March2011? 2009 498 527 Howmany‘new’unilateralcochlear 494 121 615 implantprocedureswerecarried outinthepast12months? Howmany‘new’simultaneousCI 14 276 290 CI is currently delivered through 17 NHS units in procedureswerecarriedoutin the UK. thelast12months? The National Institute of Health and Clinical HowmanysequentialCI 14 258 272 procedureswereperformedin Excellence (NICE) initiated a technology appraisal thecontralateralearinthelast process into the clinical and cost effectiveness of CI 12months? in adults and children. The reviewevaluated evidence on pre-lingually deafened children; post-lingually deafened children; and adults with acquired and pro- Do patients with severe to profound hearing gressive deafness and adults born deaf. The final loss have access to CIs? appraisal document was published in February 2009 The most recent BCIG review of results was in (TAG166, 2009). November 2011 (Table 2). Some of the main outcomes were: Globally the figures show that a significant number (cid:129) UnilateralCIisrecommendedasanoptionforpeople of children now have access to bilateral implantation. with severe to profound deafness who do not receive Over the last couple of years significant additional adequatebenefit fromacoustichearingaids. workloadhasbeenplacedoncentrestoreviewchildren (cid:129) Simultaneous bilateral CI is recommended as an with unilateral devicesto see if they would be suitable option for the following groups of people with for sequential surgery, especially as there appears to severe to profound deafness who do not receive ade- be evidence of a ‘critical age’ after which a second, quatebenefit fromacoustichearingaids: (cid:129) children sequential implant may be relatively ineffective for a (cid:129) adultswhoareblindorwhohaveotherdisabilities congenitally deaf child who is already making good thatincreasetheirrelianceonauditorystimuliasa use of an implant in the original ear (Graham et al., primarysensorymechanismforspatialawareness. 2009).Thisprocessinmostcentreshasbeencompleted. Sequential CI was not recommended as an option WhiletherearenowseveralthousandpatientswithCIs for people with severe to profound deafness. weneedtoexplorewhetherwearemeetingclinicalneed. However, peoplewho had a unilateral implant before publication of the guidance, and who fall into one of Children the categories described for bilateral implantation, To try and assess this, two sources of data were should have the option of an additional implant accessed. The first is the annual reported incidence in the other ear if this is considered to provide suffi- of hearing loss as detected from the Universal cient benefit by the responsible clinician after an Newborn Hearing Screen (UNHS). From 2001 to informed discussion with the individual person and 2006 the Department of Health rolled out a pro- theircarers. gramme of UNHS in England. Wales was fully The guidance also set clinical criteria for implan- implemented by 2004 and Scotland developed their tation for severe to profound deafness is defined as service in approximately 2005. It has been recognized the ability to hear only sounds that are louder than thatsuchscreeningisefficient (Davisetal.,2001)and 90dB HL at frequencies of 2 and 4kHz without cost effective (Stevens et al., 1998). acoustic hearing aids. Also the absence of adequate Fortnum et al. (2001) assessed the levels of perma- benefitfromacoustichearingaids,defined asfollows: nent childhood hearing impairment (PCHI) based (cid:129) for adults, a score of 50% or greater on Bamford–Kowal–Bench (BKB) sentence testing at a on hearing levels >40dB averaged over 0.5, 1, 2 and 4kHz in children born from 1980 to 1995, resident soundintensityof70dBSPL; (cid:129) for children, speech, language, and listening skills in the UK in 1998. Prevalence rose from 0.91 (95% appropriate to age, developmental stage, and cogni- confidence interval 0.85 to 0.98) for 3 year olds to tiveability. 1.65(1.62to1.68)forchildrenaged9–16years.Acap- TheclinicalcriteriaforCIintheUKarequitestrict ture–recapture technique was used to estimate comparedwithsomeothercountries.However,oncea the extent of under-ascertainment in the population patient has been assessed by a multidisciplinary team survey.Adjustmentforunder-ascertainmentincreased funding has been relativelysecure. estimatesto1.07(1.03to1.12)and2.05(2.02to2.08). S34 CochlearImplantsInternational 2013 VOL.14 NO.S1 Raine CochlearimplantsintheUnitedKingdom Table3 RegistrationdatabyagegroupforCIperformedinEnglandandWales <3 3–17 18–29 30–49 50–64 65–80 >80 Unknown 2006 141 173 28 69 71 74 10 19 2007 147 179 40 99 89 69 5 30 2008 186 202 49 124 104 120 20 21 2009 182 180 35 −13 78 97 16 25 2010 161 178 54 134 105 103 25 41 2011 149 189 27 123 104 101 26 57 The prevalence of confirmed severe and profound The surgicalregistrationsforthefirst3yearsoflife PCHI was shown to uniformly increase until the age in England and Wales were plotted against UNHS of 9 years (Fortnum et al., 2001). figures of children of a similarage (Fig. 2). Thenumberofdetectedchildrenbornwithaperma- On average the rate of implantation was 74% of nent hearing impairment of ≥40dB in the better ear estimated eligible children with severe to profound was supplied by the English and Welsh agencies. hearing loss for this age group. Using these data the prevalence for children with Similarly, surgical registrations up to 17 years were severe (71–95dB HL) and profound (>95dB HL) plotted against UNHS figures for the cohort of chil- lossescanbeestimated.However,currentNICEguide- dren with estimated severe to profound loss for this lines onlyallow implantation if hearing is ≥90dB at 2 age range (Fig. 3). and4kHz.Totryand‘estimate’apotentialpopulation For the cohort 0–17 years the implantation rate on suitableforCIsurgeryfor0–3yearsand0–16yearsall averagewas 94% of the estimated numberof children the‘profounds’and20%ofthe‘severes’werecalculated with severe to profound hearing loss from UNHS. fromUNHSforEnglandandWalesfrom2006to2011. Toassesstherateofimplantationbyyears,thethree Adults majorimplantcompaniesAdvanceBionics,Cochlear, AdultsonlyhaveaccesstoasingleCIunlesstheyhave andMedElwereapproachedandtheykindlysupplied additionalsensoryimpairment.(TAG166,2009).Prior data on ‘Surgical Registrations’ (the numbers of totheNICEreport,patientswhobecamedeaffollow- people receiving implants) for the financial years ing meningitis or ossifying conditions affecting the 2006–2011 (Table 3). Patient registrations were allo- cochlea could usually have bilateral CI. Fig. 4 high- cated into the age groups at the time of surgery. lights the surgery performed within the UK between Simultaneous implants were counted as one implant 2006 and 2011. and sequential, second implants omitted; only the year in which the first implant was inserted was used. Discussion Datawere supplied for thewhole of the UK (Fig. 4). ItisimportanttotryandevaluateaccesstoCIsasthey As UNHS data were not readily available for haveanimportantroleinthemanagementofpatients Scotland, surgical data from the only implant centre, with severe to profound hearing loss. in Kilmarnock, were kindly supplied and deducted Forchildren:theearlyrecognitionofPCHIbyUNHS from the total data. This allowed a comparison to be has been very important in the early detection and made in the data from England and Wales between initiation of appropriate management. Fortnum et al. the number of children with hearing loss likely to (2001) showed a prevalence of 1.07/1000 to the age of benefit from an implant and the number of children 3 with a betterear hearing loss ≥40dB HL increasing whowere implanted. to 2.05/1000 by 9–16 years. The prevalence of Figure2 Estimateofthenumberofchildren(0–3years)in Figure3 Estimateofthenumberofchildren(0–16years)in EnglandandWaleswithseveretoprofoundhearinglossand EnglandandWaleswithseveretoprofoundhearinglossand registeredashavinghadCIperformed. registeredashavinghadCIperformed. CochlearImplantsInternational 2013 VOL.14 NO.S1 S35 Raine CochlearimplantsintheUnitedKingdom Figure4 SurgicalregistrationdatabyyearsforCIperformedintheUK. confirmedhearingimpairmentincreasesto9years,then Not all children are necessarily referred to a regional levelsout.Possiblereasonsforthisinclude:non-diagno- unit or are found to be suitable once assessment has sis at screening; post-natal acquisition of hearing loss; been performed. Not all parents agree to referral. In late onset of progressive hearing impairment; and chil- theevaluationofthegroup0–16yearstheannualsur- drennowresidentintheUKbutbornabroad. gical registration is about 340 new cases and is The use of cochlear implants has steadily grown approaching the anticipated average incidence of 370 over the years. Clinical criteria have also changed representing about 93% of suitable children. over the years with recognition that early detection It is known that the incidence of acquired and pro- and implantation give superior results. In the UK, gressive losses steadily rise to about 9 years of age comprehensive clinical data on UNHS have been then levels out. From MRC IHR data about 280 sur- collected on children with PCHI ≥40dB HL since geries took place in 2003/2004. The NCIUA reported the mid-2000s. Using data supplied by services in a significant rise from 2005 to 2009 (NCUIA, 2010) EnglandandWalesthenumberofpotentialcandidates and what we might be seeing in reducing numbers are with a hearing loss of ≥90dB HL was estimated. related to the catching up of a previous ‘backlog’. During the years 2006–2011 the number of children Again the ‘backlog’ may be due to various factors: identified was approximately 225 by 3 years, and 375 detection, awareness, and improvement in funding. by 9–16 years. Assuming that the prevalence is con- Clearlythe currenttrendsneedtobemonitored. stant, then those children <3 years of agewho might Continuedclinicalsurveillanceofchildren’shearing have their implant at any time within a 3-year period needs to be encouraged to allow earlier referral of were compared with the actual annual registrations children with progressive hearing losses to cochlear of CI surgery. Using surgical data based on regis- implant programmes. tration was thought to be the most accurate way of For adults: the incidence of hearing impairment comparing use with anticipated use. The current rate increases with age: 4.6% of 18–40 year olds are of implantation in the 0–3 years of age was on affected by a loss of at least 25dB, this rises to 60% average 74%. This increased during childhood to in 71–80 year olds (Shield, 2006). There is consider- 93% by the time the children reached 17 years. able variation in both qualitative and quantitative Thereappears(Fig.2)tobeanincreasingrateofCI descriptions of deafness. Care must therefore be surgery in the under threes possibly due to the ‘roll taken when considering or reviewing literature on out’ of UNHS. This improvement also predates the any aspects of deafness where grades of hearing loss clinicalapprovalbyNICEin2009intreatingchildren are discussed. NICE (TAG166, 2009) reported that andadultswithseveretoprofounddeafness(TAG166, there are approximately 613000 people older than 2009). The impact of NICE on children has been the 16 years with severe to profound deafness in access to bilateral simultaneous CI for new patients England and Wales. Davis’s national study in 1995 and sequential implants for those implanted prior to reported that in 18–80 year olds 0.7% had a severe 2009 afterappropriate clinical assessment. hearing loss (70–94dB HL) and 0.2% a profound It may be logical that the rate of surgery is below (>95dB HL) (Davis, 1995). With a population of andisparalleltotheincidenceofhearingimpairment. 51.4 million over 15 years of age in the UK (Office S36 CochlearImplantsInternational 2013 VOL.14 NO.S1 Raine CochlearimplantsintheUnitedKingdom of National Statistics, 2011) there is an estimated address and understand the needs of the adult popu- 0.103 million with a profound loss and 0.36 million lation. We also continue to collect accurate annual with a severe loss. It would be difficult to estimate age-based data on surgery. how many adults in the severe group would be suit- IntheUKcochlearimplantsareavailableandfreeof able for CI. charge for all UK residents, children and adults, who CochlearimplantunitsintheUKarecurrentlysup- fulfil the NICE criteria for cochlear implants. The portingover5000adults.Withannualdatashowinga recent figures comparing rates of surgery against slowbutsteadygrowthinnumbersofadultsimplanted numbers of potentially suitable cases suggest that per year from about 240 in 2003/2004 to about 500 approximately 74% of suitable children aged 0–3 in 2010/2011. This would appear to be significantly yearsofagehavereceivedcochlearimplants,increasing belowany predictions of need. to94%ofthesechildrenbythetimetheyhavereached What factors might be affecting such a potential 17 years of age. For adults the comparable figures reduction in perceived access foradults in the UK? suggest that only about 5% of the anticipated popu- The UK appears to perform half the number per lation of suitable adults receive cochlear implants, million of population as compared with Germany or depending on what figures are used to estimate need. Austria (van Hardeveld, 2010). However, the clinical The next tasks will be to improve the figures for criteria for CI set by NICE set specific targets. With adultsandtoincreasetheproportionofeligiblechildren appropriate hearing aid provision a score of less than receivingtheirimplantbefore the ageof4. 50% on BKB sentence testing at 65dB SPL in quiet, Acknowledgement and Pure Tone thresholds of 90dB or higher at 2 A.Q. Summerfield foradvice and guidance. and 4kHz are the criteria for eligibility. Clinically it is appreciated that this does not reflect the ‘real’ References world as nobody speaks at such a high intensity. Cooper H.R., Carpenter L., Aleksy W., Booth C.L., Read T.E., Testing in noise and assessment of performance with GrahamJ.M.,etal.1989.UCH/RNIDsinglechannelextraco- monosyllabic words would be more appropriate. chlearimplant:resultsinthirtyprofoundlydeafenedadults.The JournalofLaryngologyandOtologySupplement,18:22–38. Guidelines published on behalf of the Board of DavisA.1995.Hearinginadults:theprevalenceanddistributionof the German Society for Oto-Rhino-Laryngology, hearing impairment and reported hearing disability in the Head and Neck Surgery (http://www.acir.de/ MRC Institute of Hearing Research’s National Study of Hearing.London:WhurrPublishers. ADANO_ci_leitlinie.pdf, 2012) do not specify audio- DavisA.,BamfordJ.,StevenJ.2001.Performanceofneonataland infanthearingscreens:sensitivityandspecificity.BritishJournal logicalcriteria,givingtheclinicianpossiblymoreclini- ofAudiology,35(1):3–15. calfreedom.ThiswouldbeasuitabletaskforBCIGto Davis A.C. 1989. The prevalence of hearing impairment and reassess and review in order to advise NICE when its reported hearing disability among adults in Great Britain. InternationalJournalofEpidemiology,18(4):911–917. criteria are next reviewed. Fortnum H.M., Summerfield A.Q., Marshall D.H., Davis A.C., In many situations hearing loss is progressive in Bamford J.M. 2001. Prevalence of permanent childhood hearingimpairmentintheUnitedKingdomandimplications adults. Patients may have grown to accept their pro- for universal neonatal hearing screening: questionnaire based blems; families can support and reduce the effect of ascertainmentstudy.BMJ,323(7312):536–540. GrahamJ.,VickersD.,EylesD.,BrintonJ.,AlMalkyJ.,AleksyG., hearing loss. Patients accept deterioration as a et al. 2009. Bilateral sequential cochlear implantation in the natural ageing process and may be reluctant to congenitally deaf child: evidence to support the concept of a ‘critical age’ after which the second ear is less likely to undergo surgery. provide an adequate level of speech perception on its own. General practitioners may not be aware of treat- CochlearImplantsInternational,10(3):119–141. ments but this should not be a barrier for referral to http://www.acir.de/ADANO_ci_leitlinie.pdf. 2012. Cochlea- ImplantatVersorgungundzentral-auditorischeImplantate. ENT/Audiology units. Sudden loss has significantly NCUIA. 2010. Summer news letter. National Cochlear Implant more impact, and is more likely to initiate a referral. UsersAssociation.http://www.nciua.org.uk OfficeofNationalStatistics.2011.NationalPopulationProjections BCIGiscurrentlyembarkingonaposter‘awareness 2010-based Statistical Bulletin. http://www.ons.gov.uk/ons/rel/ campaign’ targeting audiological units. It would also npp/national-population-projections/2010-based-projections/ stb-2010-based-npp-principal-and-key-variants.html be appropriate for them to press for more representa- Shield B. 2006. Evaluation of the social and economic costs of tive tests offunctional listening. hearingimpairment.AreportforHear-It. StevensJ.C.,HallD.M.,DavisA.,DaviesC.M.,DixonS.1998.The costsofearlyhearingscreeninginEnglandandWales.Archives Conclusion ofDiseaseinChildhood,78(1):14–19. This review raises more questions than it answers. SummerfieldA.Q.,MarshallD.H.1995.Cochlearimplantationin theUK1990–1994.HerMajesty’sStationeryOffice,UK. What is required is closer evaluation of children TAG166. 2009. Cochlear implants for children and adults with detected by UNHS with moderate to profound severetoprofounddeafness.http://www.nice.org.uk/nicemedia/ pdf/TA166Guidancev2.pdf hearingimpairmenttoregisterwithspecializedaudiol- van Hardeveld R. 2010. Special bulletin – May 2010. Results ogy/CIunits.Clearlywedonothavesufficientdatato enquiry2009.EURO-CIU. CochlearImplantsInternational 2013 VOL.14 NO.S1 S37