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Cochlear Implantation in Prelingually Deafened Adolescents

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Cochlear Implantation in Prelingually Deafened Adolescents ARTICLE Cochlear Implantation in Prelingually Deafened Adolescents Daniel M. Zeitler, MD; Abbas Anwar, BS; Janet E. Green, AuD; James S. Babb, PhD; David R. Friedmann, MD; J. Thomas Roland Jr, MD; Susan B. Waltzman, PhD Objectives: To determine the efficacy of cochlear im- 1 (mean change score, 51.10% and 32.23%, respec- plantation (CI) in prelingually deafened adolescent chil- tively; PϽ.001) and from the preoperative testing to EOS dren and to evaluate predictive variables for successful (mean change score, 60.02% and 38.73%, respectively; outcomes. PϽ.001), with a significantly greater increase during the first year (PϽ.001). In addition, there was a highly sig- Design: Retrospective medical record review. nificant correlation between improvements in perfor- mance scores on the CNC word and HINT sentence Participants: Children aged 10 to 17 years with pre- speech perception tests and both age at CI and length of lingual hearing loss (mean length of deafness, 11.5 years) deafness at the year 1 testing (PՅ.009) but not from the who received a unilateral CI (mean age at CI, 12.9 years). year 1 testing to EOS testing. Adolescents with progres- sive deafness and those using oral communication be- Intervention: Unilateral CI. fore CI performed significantly better than age-matched peers. Main Outcome Measures: Standard speech percep- tion testing (Consonant-Nucleus-Consonant [CNC] Conclusions: Adolescents with prelingual deafness un- monosyllabic word test and Hearing in Noise [HINT] sen- dergoing unilateral CI show significant improvement in tence test) was performed preoperatively, 1 year post- objective hearing outcome measures. Patients with shorter operatively (year 1), and at the last follow-up/end of the lengths of deafness and earlier age at CI tend to outper- study (EOS). form their peers. In addition, patients with progressive deafness and those using oral communication have sig- Results: There was a highly significant improvement in nificantly better objective outcomes than their peers. speech perception scores for both HINT sentence and CNC word testing from the preoperative testing to year Arch Pediatr Adolesc Med. 2012;166(1):35-41 ENSORINEURAL HEARING LOSS to a series of electrical impulses that stimu- (SNHL) affects 1 to 3 of ev- late the auditory nerve via a surgically im- ery 1000 children born in the planted electrode in the cochlea. Out- United States and other de- comes in young, prelingually deafened veloped countries, with the patients undergoing CI have been uni- rates likely higher in less developed na- formly excellent.9-11 With the implementa- S1-4 tions. Approximately 4000 infants are tion of universal newborn hearing screen- born each year in the United States with ing in some countries, early diagnosis of bilateral severe to profound hearing loss,5,6 bilateral severe to profound SNHL has mark- Author Affiliations: Denver Ear defined as hearing thresholds above 60 dB edly improved, allowing for early interven- Associates, Englewood, in each ear, and 4 infants per 10 000 births tion in many of these infants. However, Colorado (Dr Zeitler); and are born profoundly deaf in both ears many infants with congenital bilateral se- Department of (thresholds Ͼ80 dB).7,8 vere to profound SNHL are not identified Otolaryngology–Head and Neck early in life owing to the unavailability of Surgery, New York University newborn screening, false-positive screen- Langone Medical Center For editorial comment ing results, or a lack of access to health care. (Drs Green, Friedmann, see page 93 Similarly, a large subset of patients have pro- Roland, and Waltzman), and gressive, bilateral severe to profound SNHL Department of Radiology Cochlear implantation (CI) has be- beginning after infancy owing to a variety (Dr Babb), New York University School of Medicine, New York, come an accepted treatment paradigm for of causes, such as otosclerosis, enlarged ves- New York. Mr Anwar is a individuals 12 months or older who have tibular aqueduct syndrome, labyrinthitis os- medical student at the New bilateral severe to profound SNHL. The ef- sificans, ototoxic medication administra- York University School of ficacy of CI in the rehabilitation of hearing tion, Meniere disease, idiopathic sudden Medicine. relies on the conversion of acoustic sound SNHL, and genetic causes, including pro- ARCH PEDIATR ADOLESC MED/ VOL 166 (NO. 1), JAN 2012 WWW.ARCHPEDIATRICS.COM 35 ©2012 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 gressive nonsyndromic hearing loss. These patients, there- tients (3%) were homeschooled. Fifty of the adolescents (75%) fore, become candidates for CI as adolescents. used oral communication before CI, 7 (10%) used manual com- For adolescents deafened at an early age who do not munication, and 10 (15%) used total or cued communication. undergo CI early in life, speech perception scores after Fifty-four devices were Cochlear Corporation (Sydney, Aus- implantation almost universally improve but are worse tralia), 12 were Advanced Bionics (Valencia, California), and 1 was Med-El (Innsbruck, Austria). All had full insertion of their when compared with age-matched control peers under- 12-15 devices, and there were no postoperative complications. Mean going implantation at an earlier age, ; this duration of follow-up was 60 months (range, 12-168). Eight patients were deafness before CI has been shown to be negatively cor- unavailable for follow-up after CI (moved, changed centers, in- related with the ability to understand and use spoken lan- ternational patients, etc) and were excluded from analysis. Five guage.16-18 Initial hesitation among the parents of many patients were nonusers of their device and were excluded from deaf adolescents to subject their child to CI in the inter- analysis. Fifty-four patients with at least 1 year of follow-up af- est of waiting for long-term data to emerge, “saving the ter CI were included in the data analysis. ear” for new technological advances, or preventing sweep- ing changes in the social, academic, and personal lives METHODS of their children has given way to acceptance and un- derstanding of the CI procedure and the profound ef- Pure-tone and speech audiometry and open-set speech percep- fect it can have on the lives of adolescents. tion testing designed to assess word and sentence recognition were Adolescents represent a unique subgroup of candi- performed preoperatively with the subjects wearing their con- dates for CI that has not been extensively examined. Al- ventional amplification devices and at 3, 6, and 12 months and though early reports19-21 have shown that adolescents ben- then yearly after initial CI device stimulation. Postoperative test- efit from unilateral CI, few studies have examined the ing was performed using the same materials so that each subject effects of factors, such as age at CI, duration of deafness, could be self-compared, allowing for a single-subject repeated- cause of SNHL, and mode of communication, on speech measures design. Scores reported included preoperative, year 1 perception outcomes in this population. The purposes (after 1 year of device use), and end of the study (EOS; requiring Ն2 years of device use). The score at the final evaluation was treated of this study were to determine the efficacy of CI in pre- as missing for subjects who did not undergo evaluation at least 2 lingually deafened children and adolescents aged 10 to years after CI. Changes for these subjects are completely charac- 17 years and to evaluate possible predictive variables. terized by the preoperative to year 1 change score. The changes in word and sentence scores were computed for each subject as METHODS the score at the later time minus the score at the earlier time; a positive change reflects an increase in score. The Consonant-Nucleus-Consonant (CNC) monosyllabic SUBJECTS word test and the Hearing in Noise (HINT) sentence test in quiet and noise were performed preoperatively and postoperatively on We performed an institutional review board–approved retro- all subjects. The CNC test consists of ten 50–monosyllabic word spective review of all children and adolescent patients aged 10 lists that are scored as percentages of phonemes and words cor- to 17 years with bilateral severe to profound SNHL who de- rect. Each list represents the phonemic balance of the English lan- rived minimal to no benefit from conventional amplification guage, and the distribution is matched across lists. The HINT test and underwent unilateral CI from 1986 through 2009. The study is made up of 250 sentences divided into 25 phonetically bal- population included subjects with congenital deafness and those anced lists of 10 sentences each and can be administered in quiet ϩ with postnatal, prelingual progressive or sudden deafness, de- or with competing noise at a signal to noise ratio of 10 dB. All fined as children with severe to profound hearing loss before tests were administered in a standard soundproof suite using re- age 3 years regardless of hearing status at birth or cause of deaf- corded material presented at 60-dB sound pressure level. ness. Patients with postlingual deafness were excluded from the Study end points (CNC word score and HINT sentence score) study. We identified 67 patients who met inclusion criteria. were summarized in terms of the mean(SD) and the median Thirty-three subjects (49%) had congenital deafness of un- (interquartile range); the latter is provided because it is gen- known etiology. Other causes of deafness included progres- erally deemed appropriate when nonparametric analyses are sive hearing loss (n=11), meningitis (n=6), enlarged vestibu- used. A Wilcoxon matched-pairs signed rank test was used to lar aqueduct syndrome and/or Mondini malformation (n=5), assess whether there was a pre-CI to post-CI change in each ototoxic effects (n=4), Waardenberg syndrome (n=3), cyto- study end point.
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