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Outcomes of visually impaired patients who received cochlear implantations
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ANL-2013; No. of Pages 5 Auris Nasus Larynx xxx (2015) xxx–xxx
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Outcomes of visually impaired patients who received cochlear implantations Kenichi Takano *, Aya Kaizaki, Etsuko Saikawa, Ayami Konnno, Noriko Ogasawara, Tetsuo Himi Department of Otolaryngology, Sapporo Medical University School of Medicine, Japan
A R T I C L E I N F O
A B S T R A C T
Article history: Received 25 March 2015 Accepted 26 August 2015 Available online xxx
Objective: Patients with multiple sensory deficits, including hearing loss and visual impairment, present a unique problem. We evaluated the clinical outcomes of cochlear implantation in patients with severe to profound sensorineural hearing loss and visual impairment. Methods: We retrospectively reviewed eight patients with severe sensorineural hearing loss and visual impairment who underwent cochlear implantation at our institution between 1993 and 2014. The follow-up period was between 2 and 20 years. We evaluated the case histories, etiologies of hearing loss and visual impairment, pre- and postoperative pure-tone thresholds, speech perception rates after CI using the Japanese CD speech discrimination scoring system (CI-2004 test) for words and sentences, and pre- and postoperative communication means. Postoperative speech discrimination scores were compared between patients with and without visual impairment who underwent cochlear implantation. Results: The outcomes of cochlear implantation were good in all patients, with seven showing the ability to hold a conversation with others. The average proportion of correct answers for words and sentences in the CI-2004 test was 72.3 19.1% and 86.0 16.1%, respectively, for the patients with visual impairment and 62.1 21.7% and 78.5 20.9%, respectively, for those without visual impairment (based on auditory senses only). There were no significant differences in results between the patients with and without visual impairment. Conclusions: Cochlear implantation is important for the rehabilitation of patients with severe auditory loss and visual impairment. Medical staff members require additional skills to perform auditory evaluations and rehabilitate patients with multiple sensory deficits. ß 2015 Elsevier Ireland Ltd. All rights reserved.
Keywords: Cochlear implantation Visual impairment Communication abilities Profound hearing loss
1. Introduction Cochlear implantation (CI) has become an established method for the auditory rehabilitation of qualified patients with severe to profound sensorineural hearing loss (SNHL), and it is widely accepted as a safe procedure [1,2]. Dual-sensory impairment or SNHL with visual impairment is a relatively rare condition, and affected patients present a unique problem, have limited options for communication, and mainly rely on tactile stimulation [3]. These patients cannot depend on other individuals who are willing to learn how to communicate with them, so they learn special finger spelling, hand writing, and tactile communication methods. The magnitude of the disability caused by dual-sensory
* Corresponding author at: Department of Otolaryngology, Sapporo Medical University School of Medicine, S1W16, Chuo-ku, Sapporo 060-8543, Japan. Tel.: +81 11 688 9655; fax: +81 11 615 5405. E-mail address: [email protected] (K. Takano).
impairment presents great difficulties for individuals with normal hearing and sight [4]. In the present study, we evaluated the outcomes of CI in eight adult patients with severe to profound SNHL and severe visual impairment. 2. Materials and methods The records of 270 patients who participated in the adult and pediatric cochlear implant programs at our institutions from 1993 to 2014 were retrospectively reviewed. Eight adult patients with SNHL and significant visual impairment were identified (Table 1). The age range of the patients was 57–82 years (average, 65.6 8.9 years), while their duration of deafness ranged from 1 to 46 years. The follow-up period was between 2 and 20 years. The causes of deafness and visual impairment in these patients are shown in Table 1. The patients’ visual acuities as measured by Japanese ophthalmologists and their Snellen notations converted from decimal notations are shown in Table 2. Four patients received a CI24 and one
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Please cite this article in press as: Takano K, et al. Outcomes of visually impaired patients who received cochlear implantations. Auris Nasus Larynx (2015), http://dx.doi.org/10.1016/j.anl.2015.08.005
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2 Table 1 Subject characteristics. Patient no.
Age at CI
Gender
Causes of deafness (onset years)
Causes of visual impairment (onset years)
Implant device
Sound processor (type)
1 2
77 59
F M
High fever (12) Glaucoma, cataract (late 20s)
CI24RE CI22M
N5 (ear hunging) Spectra (box)
3 4 5 6 7 8
57 82 61 58 70 61
M F F F F F
Idiopathic progressed (60s) Sudden deafness (29) Menier’s disease (36) Idiopathic progressed (50) Sudden deafness (60 on right, 81 on left) Idiopathic progressed (55) Idiopathic progressed (56) Idiopathic progressed (67) Idiopathic progressed (15)
Retinitis pigmentosa (30s) Retinochoroidal atrophy (40s) Unknown (10s) Injury (51) Congenital Glaucoma, cataract (50s)
CI24RE COMBI40+ N22 M24 CI24R(CS) CI24M
Freedom (box/ear hunging) OPUS (box) MSP (box) SPrint (box) ESPrint 3G (ear hunging) 3G (box)
Table 2 Pre- and post-operative evaluations of the communication means. Patient no.
Visual acuity
Average of preoperative Pure-tone thresholds (dB)
Preoperative communication means
Postoperative communication means
Using a phone
Right
Left
1 2
0 20/1000
0 0
110/110 110/110
Voice conversation Voice conversation
Possible Possible
20/1000
20/1000
105/110
Voice conversation
Possible
20/1000 20/1000 20/800 20/800 20/50
HM 20/800 0 20/1000 0
110/110 105/105 110/105 130/130 110/110
Finger braille Handwritten letters Bold and bigger letters (visual field center part only) Bold and bigger letters (visual field center part only) Bold and bigger letters Bold and bigger letters Bold and bigger letters Bold and bigger letters Bigger letters
3 4 5 6 7 8
Voice conversation Voice conversation Environmental sound Voice conversation Voice conversation
Possible Impossible Impossible Possible Possible
each received a CI22, Nucleus 22, Nucleus 24, and COMBI40+. All electrodes were inserted into the cochlea without any intraoperative problems. In addition, 90 adult patients, over fifty years of age (average, 62.0 8.4 years; range, 50–84 years, 25 men 65 women), without visual impairment, who underwent CI at our institution during the same period and are currently alive, were included as a control group. The duration of deafness ranged from 4 months to 46 years, with a follow-up period of 1 to 27 years. We evaluated the following: (1) etiology of hearing loss and visual impairment, (2) pre- and postoperative pure-tone thresholds, (3) speech perception rates after CI using the Japanese CD speech discrimination scoring system (CI-2004 test) for words and sentences, and (4) pre- and postoperative communication means. Briefly, pure-tone thresholds and the average frequency (standard deviation) at 250, 500, 1000, 2000, and 4000 Hz were measured after CI in the eight patients with visual impairment. The proportion of correct answers for words (70 dB) and sentences (70 dB) in the Japanese CI-2004 test (speech discrimination scores) were compared between patients with and without visual impairment. Student’s t-test was used to evaluate significant differences in the average frequency and CI-2004 test results between the two groups. Data are expressed as means standard deviations. P-values of <0.05 were considered to indicate statistical significance. 3. Results
abilities. She was able to converse without finger braille and handwritten letters, could hold a conversation with others on the telephone, and could enjoy listening to radio or television. 3.1.2. Case 2 A 59-year-old man with visual impairment due to bilateral glaucoma and cataracts since his late 20s was diagnosed with hearing loss after experiencing sudden deafness in his left ear at 29 years of age. At 36 years of age, he developed Meniere’s disease in the right ear. He was barely able to read bold, large letters. He received a Nucleus CI22M device, and his response to the rehabilitation program has been excellent. He is doing well with regard to hearing approximately 18 years after CI. 3.1.3. Case 3 A 57-year-old man developed visual impairment in his 30s because of retinitis pigmentosa. His hearing level had decreased since he was 10 years of age, and he began using hearing aids at 13 years of age. His hearing loss progressed, and he became completely deaf 7 years before CI. Although he was suspected to be Usher syndrome, he did not hope to undergo genetic testing. He received a Nucleus CI24RE device and reported subjective improvement in communication as early as 4 weeks after activation. Six months after activation, his CI-2004 score was 81% for sentences, and approximately 6 years later, he was able to communicate faceto-face with family and friends.
3.1. Case histories 3.1.1. Case 1 A 77-year-old woman lost her sight after she developed high fever (details unknown) at the age of 12 years. She developed SHNL in her 60s and lost her hearing at 74 years of age. Her husband was also blind with moderate hearing loss. She desired relief from her auditory impairment and the ability to communicate with her husband. Before implantation, her only means of communication were finger braille and handwritten letters. She received a Nucleus CI24RE device and immediately acquired effortless speech tracking
3.1.4. Case 4 An 82-year-old woman developed visual impairment in her 40s because of retinochoroidal atrophy. She developed sudden deafness in her right ear at 60 years of age and in her left ear at 81 years of age. She scored 0% in the speech discrimination test using closed-set sentences in the best-aided condition. Subsequently, she received a COMBI40+ device and scored 84% for words in the CI-2004 test. She is now 87 years old, fit, able to communicate even with unfamiliar speakers without difficulty, and able to use the telephone.
Please cite this article in press as: Takano K, et al. Outcomes of visually impaired patients who received cochlear implantations. Auris Nasus Larynx (2015), http://dx.doi.org/10.1016/j.anl.2015.08.005
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3.1.5. Case 5 A 61-year-old woman developed visual impairment at the age of 10 years from an unknown cause. She was diagnosed with hearing loss in her 40s that progressed to profound bilateral SNHL at 55 years of age. She received a Nucleus 22 device and is now able to communicate with unfamiliar speakers; however, she experiences some difficulty in understanding speech in a noisy environment and is unable to use a telephone. 3.1.6. Case 6 A 58-year-old woman developed visual impairment at 51 years of age after an accidental fall in her apartment. Her hearing level decreased after the accident and progressed to profound bilateral SNHL at 56 years of age. She received a Nucleus 24 device and scored 30% for words and 50% for sentences in the CI-2004 test. Although she is able to hear all environmental sound, she experiences some difficulty in understanding speech in a noisy environment. 3.1.7. Case 7 A 70-year-old woman with visual impairment since birth developed hearing loss that progressed to an advanced stage by 67 years of age. She was barely able to read bold, large letters. Subsequently, she received a CI24RCS device and was able to communicate with her family and friends. She continued to show satisfactory hearing outcomes and could use the telephone; unfortunately, she suffered subarachnoid hemorrhage and was declared brain dead. 3.1.8. Case 8 A 61-year-old woman developed visual impairment in her 50s because of bilateral glaucoma and cataracts. Her hearing loss was diagnosed at 15 years of age and progressed to profound bilateral hearing loss at 60 years. She was barely able to read large letters and received a CI24M device. Subsequently, she was able to communicate even with unfamiliar speakers and use the telephone without difficulty. 3.2. Hearing evaluations The results of hearing evaluation for the patients with SNHL and visual impairment before and after CI are presented in Tables 2 and 3, respectively. Pure-tone thresholds after CI are shown in Table 3. The average frequency ( standard deviation) for the patients with visual impairment was 37.5 11.6 dBHL (250 Hz), 34.4 10.2 dBHL (500 Hz), 32.5 8.0 dBHL (1000 Hz), 33.8 10.3 dBHL (2000 Hz), and 35.0 11.6 dBHL (4000 Hz; Fig. 1 and Table 3). As shown in Fig. 2, the average proportion of correct answers provided by the patients with visual impairment in the CI2004 test was 72.3 19.1% for words and 86.0 16.1% for sentences, while that provided by the patients without visual impairment on the basis of auditory senses only was 62.1 21.7% for words and
Fig. 1. Postoperative pure-tone thresholds at 250, 500, 1000, 2000, and 4000 Hz for eight patients who underwent cochlear implantation.
78.5 20.9% for sentences. There were no significant differences in the average proportion of correct answers between the two groups (P = 0.28 for words, P = 0.35 for sentences). 4. Discussion The combination of hearing loss and visual impairment presents a unique and complex problem. For most patients without visual impairment, CI still serves as an adjunct to lip reading; however, the results of CI in some patients have been so encouraging that the selection net has widened and includes selected marginal hearing aid users with visual impairment and associated disabilities. CI in patients with visual impairment may be considered a risky undertaking because the original indication is in patients with normal sight. Since Martin et al. reported a case of a deaf–blind patient who underwent CI in 1988 [5], a few authors have reported their experiences with patients with profound SNHL and visual impairment [3,6–10]. It is believed that such patients generally respond well to CI. The patients with profound SNHL and visual impairment described in this study demonstrated satisfactory hearing outcomes after CI, proving that CI was able to improve and enhance their quality of life. In the present study, most of the patients were elderly individuals living alone or had spouses with visual impairment. Our findings suggest that the benefits of QOL improvement and safe living outweigh the risks associated with CI in patients with a social handicap. Considering the present increase in the aging population in developed countries, the number of individuals with hearing loss and visual impairment is expected to increase in the
Table 3 Postoperative evaluations of the hearing level. Patient no.
1 2 3 4 5 6 7 8
Postoperative pure-tone thresholds with CI (dBHL)
Speech perception scores (Japanese CI2004 test)
250 Hz
500 Hz
1000 Hz
2000 Hz
4000 Hz
Words (%)
Sentence (%)
20 45 25 30 45 50 35 50
20 40 25 25 40 40 35 50
20 35 30 25 40 35 30 45
20 30 25 30 40 45 30 50
20 30 25 30 45 50 30 50
64 68 76 84 88 30 84 84
93 80 81 95 98 50 96 95
Please cite this article in press as: Takano K, et al. Outcomes of visually impaired patients who received cochlear implantations. Auris Nasus Larynx (2015), http://dx.doi.org/10.1016/j.anl.2015.08.005
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Fig. 2. Proportions of accurate answers (speech discrimination scores) for words (70 dB) and sentences (70 dB) in the Japanese CI-2004 test (based on auditory senses only) for patients with and without visual impairment. There is no significant difference in the proportions between the two groups.
future. It is difficult for such patients to acquire information about CI; our patients acquired information about CI from other experienced patients or medical staff. Therefore, sufficient information should be provided to patients with hearing loss and visual impairment as well as medical staff. Furthermore, educational programs explaining the benefits of CI to such patients should be conducted. The causes of visual impairment were diverse, whereas the cause of deafness in six of the eight patients was idiopathic progression. Six patients had developed progressive or sudden deafness. One patient (case 6) experienced an almost simultaneous onset of deafness and visual impairment, while one (case 8) showed progressive visual impairment. In our case series, because all the patients developed postlingual profound SNHL, their responses to CI were good. Pre- and postoperative evaluation and rehabilitation of the patients were more challenging for the families and medical staff. Because the communication means used by each patient were different, such as tentacles or large, bold letters, selection of individualized communications means was required, occasionally in cooperation with a specialized interpreter, after obtaining preoperative informed consent and gaining familiarity with the tactile methods of communication used by each individual. We confirmed whether the patient was being understood several times. We also allowed the patients to repeatedly touch the actual implantation device and select the sound processor that was the easiest to use according to them. They were also given time to learn basic operations such as battery exchange and mounting of the speech processor. Thus, evaluation and rehabilitation sessions are more time-consuming and labor-intensive for CI patients with visual impairment than for those with normal sight. Furthermore, old age increases the difficulty in operating the cochlear implant and related attachments for patients with visual impairment; therefore, a careful follow-up at short intervals is important. Nevertheless, the efforts made by the patients in our study showed satisfactory outcomes in terms of hearing and quality of life. The National Institute for Health and Clinical Excellence (NICE) in the UK conducted a systematic review of the clinical outcomes of unilateral and bilateral CI in children and adults [11], and since this review, more evidence has been published on the effects of
bilateral CI in adults, which was found to provide additional improvements in speech perception and localization [1,12]. For speech perception in noise, a significant bilateral benefit is provided when the noise is presented from the unilaterally aided side [13–15]. Our patients did not undergo bilateral CI; furthermore, their visual input was limited. Bilateral CI is believed to be useful and secures the safety of patients with visual impairment, particularly in noisy conditions. In conclusion, we reported the outcomes of CI in patients with severe to profound SNHL and severe visual impairment. After surgery, seven of the eight CI patients with visual impairment were able to communicate with other individuals through voice conversation. Unfortunately, one patient experienced some difficulty in understanding speech in noisy environments. We are confident that CI can have a significant positive impact on the quality of life of patients with SNHL and visual impairment. Professionals and caregivers need to acquire certain additional rehabilitative skills and take the cultural backgrounds of potential candidates into consideration. Conflicts of interest None. References [1] Gaylor JM, Raman G, Chung M, Lee J, Rao M, Lau J, et al. Cochlear implantation in adults: a systematic review and meta-analysis. JAMA Otolaryngol Head Neck Surg 2013;139:265–72. [2] Mudry A, Mills M. The early history of the cochlear implant: a retrospective. Otolaryngol Head Neck Surg 2013;139:446–53. [3] El-Kashlan HK, Boerst A, Telian SA. Multichannel cochlear implantation in visually impaired patients. Otol Neurotol 2001;22:53–6. [4] Saeed SR, Ramsden RT, Axon PR. Cochlear implantation in the deaf-blind. Am J Otol 1998;19:774–7. [5] Martin EL, Burnett PA, Himelick TE, Phillips MA, Over SK. Speech recognition by a deaf-blind multichannel cochlear implant patient. Ear Hear 1988;9:70–4. [6] Kawasaki E, Tomoda K, Iwano T, Kitahara H, Yamashita T. Cochlear implantation in a blind and deaf patient. Adv Otorhinolaryngol 1997;52:89–91. [7] Cullington HE. Cochlear implantation of a deaf blind patient with mitochondrial cytopathy. J Laryngol Otol 1999;113:353–4. [8] Damen GW, Pennings RJ, Snik AF, Mylanus EA. Quality of life and cochlear implantation in Usher syndrome type I. Laryngoscope 2006;116:723–8.
Please cite this article in press as: Takano K, et al. Outcomes of visually impaired patients who received cochlear implantations. Auris Nasus Larynx (2015), http://dx.doi.org/10.1016/j.anl.2015.08.005
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ANL-2013; No. of Pages 5 K. Takano et al. / Auris Nasus Larynx xxx (2015) xxx–xxx [9] Wiley S, Meinzen-Derr J, Stremel-Thomas K, Schalock M, Bashinski SM, Ruder C. Outcomes for children with deaf-blindness with cochlear implants: a multisite observational study. Otol Neurotol 2013;34:507–15. [10] Takasaki K, Kanda Y, Kumagami H, Yashida H, Yamamoto-Fukuda T, Miyamoto I, et al. Cochlear implantations in visually impaired patients. Eur Arch Otorhinolaryngol 2007;264:363–7. [11] Bond M, Mealing S, Anderson R, Elston J, Weiner G, Taylor RS, et al. The effectiveness and cost-effectiveness of cochlear implants for severe to profound deafness in children and adults: a systematic review and economic model. Health Technol Assess 2009;13:1–330. [12] van Schoonhoven J, Sparreboom M, van Zanten BG, Scholten RJ, Mylanus EA, Dreschler WA, et al. The effectiveness of bilateral cochlear implants for
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severe-to-profound deafness in adults: a systematic review. Otol Neurotol 2013;34:190–8. [13] Laszig R, Aschendorff A, Stecker M, Mu¨ller-Deile J, Maune S, Dillier N, et al. Benefits of bilateral electrical stimulation with the nucleus cochlear implant in adults: 6-month postoperative results. Otol Neurotol 2004;25:958–68. [14] Ramsden R, Greenham P, O’Driscoll M, Mawman D, Proops D, Craddock L, et al. Evaluation of bilaterally implanted adult subjects with the nucleus 24 cochlear implant system. Otol Neurotol 2005;26:988–98. [15] Litovsky R, Parkinson A, Arcaroli J, Sammeth C. Simultaneous bilateral cochlear implantation in adults: a multicenter clinical study. Ear Hear 2006;27: 714–31.
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ANL-2013; No. of Pages 5 Auris Nasus Larynx xxx (2015) xxx–xxx
Contents lists available at ScienceDirect
Auris Nasus Larynx journal homepage: www.elsevier.com/locate/anl
Outcomes of visually impaired patients who received cochlear implantations Kenichi Takano *, Aya Kaizaki, Etsuko Saikawa, Ayami Konnno, Noriko Ogasawara, Tetsuo Himi Department of Otolaryngology, Sapporo Medical University School of Medicine, Japan
A R T I C L E I N F O
A B S T R A C T
Article history: Received 25 March 2015 Accepted 26 August 2015 Available online xxx
Objective: Patients with multiple sensory deficits, including hearing loss and visual impairment, present a unique problem. We evaluated the clinical outcomes of cochlear implantation in patients with severe to profound sensorineural hearing loss and visual impairment. Methods: We retrospectively reviewed eight patients with severe sensorineural hearing loss and visual impairment who underwent cochlear implantation at our institution between 1993 and 2014. The follow-up period was between 2 and 20 years. We evaluated the case histories, etiologies of hearing loss and visual impairment, pre- and postoperative pure-tone thresholds, speech perception rates after CI using the Japanese CD speech discrimination scoring system (CI-2004 test) for words and sentences, and pre- and postoperative communication means. Postoperative speech discrimination scores were compared between patients with and without visual impairment who underwent cochlear implantation. Results: The outcomes of cochlear implantation were good in all patients, with seven showing the ability to hold a conversation with others. The average proportion of correct answers for words and sentences in the CI-2004 test was 72.3 19.1% and 86.0 16.1%, respectively, for the patients with visual impairment and 62.1 21.7% and 78.5 20.9%, respectively, for those without visual impairment (based on auditory senses only). There were no significant differences in results between the patients with and without visual impairment. Conclusions: Cochlear implantation is important for the rehabilitation of patients with severe auditory loss and visual impairment. Medical staff members require additional skills to perform auditory evaluations and rehabilitate patients with multiple sensory deficits. ß 2015 Elsevier Ireland Ltd. All rights reserved.
Keywords: Cochlear implantation Visual impairment Communication abilities Profound hearing loss
1. Introduction Cochlear implantation (CI) has become an established method for the auditory rehabilitation of qualified patients with severe to profound sensorineural hearing loss (SNHL), and it is widely accepted as a safe procedure [1,2]. Dual-sensory impairment or SNHL with visual impairment is a relatively rare condition, and affected patients present a unique problem, have limited options for communication, and mainly rely on tactile stimulation [3]. These patients cannot depend on other individuals who are willing to learn how to communicate with them, so they learn special finger spelling, hand writing, and tactile communication methods. The magnitude of the disability caused by dual-sensory
* Corresponding author at: Department of Otolaryngology, Sapporo Medical University School of Medicine, S1W16, Chuo-ku, Sapporo 060-8543, Japan. Tel.: +81 11 688 9655; fax: +81 11 615 5405. E-mail address: [email protected] (K. Takano).
impairment presents great difficulties for individuals with normal hearing and sight [4]. In the present study, we evaluated the outcomes of CI in eight adult patients with severe to profound SNHL and severe visual impairment. 2. Materials and methods The records of 270 patients who participated in the adult and pediatric cochlear implant programs at our institutions from 1993 to 2014 were retrospectively reviewed. Eight adult patients with SNHL and significant visual impairment were identified (Table 1). The age range of the patients was 57–82 years (average, 65.6 8.9 years), while their duration of deafness ranged from 1 to 46 years. The follow-up period was between 2 and 20 years. The causes of deafness and visual impairment in these patients are shown in Table 1. The patients’ visual acuities as measured by Japanese ophthalmologists and their Snellen notations converted from decimal notations are shown in Table 2. Four patients received a CI24 and one
http://dx.doi.org/10.1016/j.anl.2015.08.005 0385-8146/ß 2015 Elsevier Ireland Ltd. All rights reserved.
Please cite this article in press as: Takano K, et al. Outcomes of visually impaired patients who received cochlear implantations. Auris Nasus Larynx (2015), http://dx.doi.org/10.1016/j.anl.2015.08.005
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ANL-2013; No. of Pages 5 K. Takano et al. / Auris Nasus Larynx xxx (2015) xxx–xxx
2 Table 1 Subject characteristics. Patient no.
Age at CI
Gender
Causes of deafness (onset years)
Causes of visual impairment (onset years)
Implant device
Sound processor (type)
1 2
77 59
F M
High fever (12) Glaucoma, cataract (late 20s)
CI24RE CI22M
N5 (ear hunging) Spectra (box)
3 4 5 6 7 8
57 82 61 58 70 61
M F F F F F
Idiopathic progressed (60s) Sudden deafness (29) Menier’s disease (36) Idiopathic progressed (50) Sudden deafness (60 on right, 81 on left) Idiopathic progressed (55) Idiopathic progressed (56) Idiopathic progressed (67) Idiopathic progressed (15)
Retinitis pigmentosa (30s) Retinochoroidal atrophy (40s) Unknown (10s) Injury (51) Congenital Glaucoma, cataract (50s)
CI24RE COMBI40+ N22 M24 CI24R(CS) CI24M
Freedom (box/ear hunging) OPUS (box) MSP (box) SPrint (box) ESPrint 3G (ear hunging) 3G (box)
Table 2 Pre- and post-operative evaluations of the communication means. Patient no.
Visual acuity
Average of preoperative Pure-tone thresholds (dB)
Preoperative communication means
Postoperative communication means
Using a phone
Right
Left
1 2
0 20/1000
0 0
110/110 110/110
Voice conversation Voice conversation
Possible Possible
20/1000
20/1000
105/110
Voice conversation
Possible
20/1000 20/1000 20/800 20/800 20/50
HM 20/800 0 20/1000 0
110/110 105/105 110/105 130/130 110/110
Finger braille Handwritten letters Bold and bigger letters (visual field center part only) Bold and bigger letters (visual field center part only) Bold and bigger letters Bold and bigger letters Bold and bigger letters Bold and bigger letters Bigger letters
3 4 5 6 7 8
Voice conversation Voice conversation Environmental sound Voice conversation Voice conversation
Possible Impossible Impossible Possible Possible
each received a CI22, Nucleus 22, Nucleus 24, and COMBI40+. All electrodes were inserted into the cochlea without any intraoperative problems. In addition, 90 adult patients, over fifty years of age (average, 62.0 8.4 years; range, 50–84 years, 25 men 65 women), without visual impairment, who underwent CI at our institution during the same period and are currently alive, were included as a control group. The duration of deafness ranged from 4 months to 46 years, with a follow-up period of 1 to 27 years. We evaluated the following: (1) etiology of hearing loss and visual impairment, (2) pre- and postoperative pure-tone thresholds, (3) speech perception rates after CI using the Japanese CD speech discrimination scoring system (CI-2004 test) for words and sentences, and (4) pre- and postoperative communication means. Briefly, pure-tone thresholds and the average frequency (standard deviation) at 250, 500, 1000, 2000, and 4000 Hz were measured after CI in the eight patients with visual impairment. The proportion of correct answers for words (70 dB) and sentences (70 dB) in the Japanese CI-2004 test (speech discrimination scores) were compared between patients with and without visual impairment. Student’s t-test was used to evaluate significant differences in the average frequency and CI-2004 test results between the two groups. Data are expressed as means standard deviations. P-values of <0.05 were considered to indicate statistical significance. 3. Results
abilities. She was able to converse without finger braille and handwritten letters, could hold a conversation with others on the telephone, and could enjoy listening to radio or television. 3.1.2. Case 2 A 59-year-old man with visual impairment due to bilateral glaucoma and cataracts since his late 20s was diagnosed with hearing loss after experiencing sudden deafness in his left ear at 29 years of age. At 36 years of age, he developed Meniere’s disease in the right ear. He was barely able to read bold, large letters. He received a Nucleus CI22M device, and his response to the rehabilitation program has been excellent. He is doing well with regard to hearing approximately 18 years after CI. 3.1.3. Case 3 A 57-year-old man developed visual impairment in his 30s because of retinitis pigmentosa. His hearing level had decreased since he was 10 years of age, and he began using hearing aids at 13 years of age. His hearing loss progressed, and he became completely deaf 7 years before CI. Although he was suspected to be Usher syndrome, he did not hope to undergo genetic testing. He received a Nucleus CI24RE device and reported subjective improvement in communication as early as 4 weeks after activation. Six months after activation, his CI-2004 score was 81% for sentences, and approximately 6 years later, he was able to communicate faceto-face with family and friends.
3.1. Case histories 3.1.1. Case 1 A 77-year-old woman lost her sight after she developed high fever (details unknown) at the age of 12 years. She developed SHNL in her 60s and lost her hearing at 74 years of age. Her husband was also blind with moderate hearing loss. She desired relief from her auditory impairment and the ability to communicate with her husband. Before implantation, her only means of communication were finger braille and handwritten letters. She received a Nucleus CI24RE device and immediately acquired effortless speech tracking
3.1.4. Case 4 An 82-year-old woman developed visual impairment in her 40s because of retinochoroidal atrophy. She developed sudden deafness in her right ear at 60 years of age and in her left ear at 81 years of age. She scored 0% in the speech discrimination test using closed-set sentences in the best-aided condition. Subsequently, she received a COMBI40+ device and scored 84% for words in the CI-2004 test. She is now 87 years old, fit, able to communicate even with unfamiliar speakers without difficulty, and able to use the telephone.
Please cite this article in press as: Takano K, et al. Outcomes of visually impaired patients who received cochlear implantations. Auris Nasus Larynx (2015), http://dx.doi.org/10.1016/j.anl.2015.08.005
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3.1.5. Case 5 A 61-year-old woman developed visual impairment at the age of 10 years from an unknown cause. She was diagnosed with hearing loss in her 40s that progressed to profound bilateral SNHL at 55 years of age. She received a Nucleus 22 device and is now able to communicate with unfamiliar speakers; however, she experiences some difficulty in understanding speech in a noisy environment and is unable to use a telephone. 3.1.6. Case 6 A 58-year-old woman developed visual impairment at 51 years of age after an accidental fall in her apartment. Her hearing level decreased after the accident and progressed to profound bilateral SNHL at 56 years of age. She received a Nucleus 24 device and scored 30% for words and 50% for sentences in the CI-2004 test. Although she is able to hear all environmental sound, she experiences some difficulty in understanding speech in a noisy environment. 3.1.7. Case 7 A 70-year-old woman with visual impairment since birth developed hearing loss that progressed to an advanced stage by 67 years of age. She was barely able to read bold, large letters. Subsequently, she received a CI24RCS device and was able to communicate with her family and friends. She continued to show satisfactory hearing outcomes and could use the telephone; unfortunately, she suffered subarachnoid hemorrhage and was declared brain dead. 3.1.8. Case 8 A 61-year-old woman developed visual impairment in her 50s because of bilateral glaucoma and cataracts. Her hearing loss was diagnosed at 15 years of age and progressed to profound bilateral hearing loss at 60 years. She was barely able to read large letters and received a CI24M device. Subsequently, she was able to communicate even with unfamiliar speakers and use the telephone without difficulty. 3.2. Hearing evaluations The results of hearing evaluation for the patients with SNHL and visual impairment before and after CI are presented in Tables 2 and 3, respectively. Pure-tone thresholds after CI are shown in Table 3. The average frequency ( standard deviation) for the patients with visual impairment was 37.5 11.6 dBHL (250 Hz), 34.4 10.2 dBHL (500 Hz), 32.5 8.0 dBHL (1000 Hz), 33.8 10.3 dBHL (2000 Hz), and 35.0 11.6 dBHL (4000 Hz; Fig. 1 and Table 3). As shown in Fig. 2, the average proportion of correct answers provided by the patients with visual impairment in the CI2004 test was 72.3 19.1% for words and 86.0 16.1% for sentences, while that provided by the patients without visual impairment on the basis of auditory senses only was 62.1 21.7% for words and
Fig. 1. Postoperative pure-tone thresholds at 250, 500, 1000, 2000, and 4000 Hz for eight patients who underwent cochlear implantation.
78.5 20.9% for sentences. There were no significant differences in the average proportion of correct answers between the two groups (P = 0.28 for words, P = 0.35 for sentences). 4. Discussion The combination of hearing loss and visual impairment presents a unique and complex problem. For most patients without visual impairment, CI still serves as an adjunct to lip reading; however, the results of CI in some patients have been so encouraging that the selection net has widened and includes selected marginal hearing aid users with visual impairment and associated disabilities. CI in patients with visual impairment may be considered a risky undertaking because the original indication is in patients with normal sight. Since Martin et al. reported a case of a deaf–blind patient who underwent CI in 1988 [5], a few authors have reported their experiences with patients with profound SNHL and visual impairment [3,6–10]. It is believed that such patients generally respond well to CI. The patients with profound SNHL and visual impairment described in this study demonstrated satisfactory hearing outcomes after CI, proving that CI was able to improve and enhance their quality of life. In the present study, most of the patients were elderly individuals living alone or had spouses with visual impairment. Our findings suggest that the benefits of QOL improvement and safe living outweigh the risks associated with CI in patients with a social handicap. Considering the present increase in the aging population in developed countries, the number of individuals with hearing loss and visual impairment is expected to increase in the
Table 3 Postoperative evaluations of the hearing level. Patient no.
1 2 3 4 5 6 7 8
Postoperative pure-tone thresholds with CI (dBHL)
Speech perception scores (Japanese CI2004 test)
250 Hz
500 Hz
1000 Hz
2000 Hz
4000 Hz
Words (%)
Sentence (%)
20 45 25 30 45 50 35 50
20 40 25 25 40 40 35 50
20 35 30 25 40 35 30 45
20 30 25 30 40 45 30 50
20 30 25 30 45 50 30 50
64 68 76 84 88 30 84 84
93 80 81 95 98 50 96 95
Please cite this article in press as: Takano K, et al. Outcomes of visually impaired patients who received cochlear implantations. Auris Nasus Larynx (2015), http://dx.doi.org/10.1016/j.anl.2015.08.005
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Fig. 2. Proportions of accurate answers (speech discrimination scores) for words (70 dB) and sentences (70 dB) in the Japanese CI-2004 test (based on auditory senses only) for patients with and without visual impairment. There is no significant difference in the proportions between the two groups.
future. It is difficult for such patients to acquire information about CI; our patients acquired information about CI from other experienced patients or medical staff. Therefore, sufficient information should be provided to patients with hearing loss and visual impairment as well as medical staff. Furthermore, educational programs explaining the benefits of CI to such patients should be conducted. The causes of visual impairment were diverse, whereas the cause of deafness in six of the eight patients was idiopathic progression. Six patients had developed progressive or sudden deafness. One patient (case 6) experienced an almost simultaneous onset of deafness and visual impairment, while one (case 8) showed progressive visual impairment. In our case series, because all the patients developed postlingual profound SNHL, their responses to CI were good. Pre- and postoperative evaluation and rehabilitation of the patients were more challenging for the families and medical staff. Because the communication means used by each patient were different, such as tentacles or large, bold letters, selection of individualized communications means was required, occasionally in cooperation with a specialized interpreter, after obtaining preoperative informed consent and gaining familiarity with the tactile methods of communication used by each individual. We confirmed whether the patient was being understood several times. We also allowed the patients to repeatedly touch the actual implantation device and select the sound processor that was the easiest to use according to them. They were also given time to learn basic operations such as battery exchange and mounting of the speech processor. Thus, evaluation and rehabilitation sessions are more time-consuming and labor-intensive for CI patients with visual impairment than for those with normal sight. Furthermore, old age increases the difficulty in operating the cochlear implant and related attachments for patients with visual impairment; therefore, a careful follow-up at short intervals is important. Nevertheless, the efforts made by the patients in our study showed satisfactory outcomes in terms of hearing and quality of life. The National Institute for Health and Clinical Excellence (NICE) in the UK conducted a systematic review of the clinical outcomes of unilateral and bilateral CI in children and adults [11], and since this review, more evidence has been published on the effects of
bilateral CI in adults, which was found to provide additional improvements in speech perception and localization [1,12]. For speech perception in noise, a significant bilateral benefit is provided when the noise is presented from the unilaterally aided side [13–15]. Our patients did not undergo bilateral CI; furthermore, their visual input was limited. Bilateral CI is believed to be useful and secures the safety of patients with visual impairment, particularly in noisy conditions. In conclusion, we reported the outcomes of CI in patients with severe to profound SNHL and severe visual impairment. After surgery, seven of the eight CI patients with visual impairment were able to communicate with other individuals through voice conversation. Unfortunately, one patient experienced some difficulty in understanding speech in noisy environments. We are confident that CI can have a significant positive impact on the quality of life of patients with SNHL and visual impairment. Professionals and caregivers need to acquire certain additional rehabilitative skills and take the cultural backgrounds of potential candidates into consideration. Conflicts of interest None. References [1] Gaylor JM, Raman G, Chung M, Lee J, Rao M, Lau J, et al. Cochlear implantation in adults: a systematic review and meta-analysis. JAMA Otolaryngol Head Neck Surg 2013;139:265–72. [2] Mudry A, Mills M. The early history of the cochlear implant: a retrospective. Otolaryngol Head Neck Surg 2013;139:446–53. [3] El-Kashlan HK, Boerst A, Telian SA. Multichannel cochlear implantation in visually impaired patients. Otol Neurotol 2001;22:53–6. [4] Saeed SR, Ramsden RT, Axon PR. Cochlear implantation in the deaf-blind. Am J Otol 1998;19:774–7. [5] Martin EL, Burnett PA, Himelick TE, Phillips MA, Over SK. Speech recognition by a deaf-blind multichannel cochlear implant patient. Ear Hear 1988;9:70–4. [6] Kawasaki E, Tomoda K, Iwano T, Kitahara H, Yamashita T. Cochlear implantation in a blind and deaf patient. Adv Otorhinolaryngol 1997;52:89–91. [7] Cullington HE. Cochlear implantation of a deaf blind patient with mitochondrial cytopathy. J Laryngol Otol 1999;113:353–4. [8] Damen GW, Pennings RJ, Snik AF, Mylanus EA. Quality of life and cochlear implantation in Usher syndrome type I. Laryngoscope 2006;116:723–8.
Please cite this article in press as: Takano K, et al. Outcomes of visually impaired patients who received cochlear implantations. Auris Nasus Larynx (2015), http://dx.doi.org/10.1016/j.anl.2015.08.005
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ANL-2013; No. of Pages 5 K. Takano et al. / Auris Nasus Larynx xxx (2015) xxx–xxx [9] Wiley S, Meinzen-Derr J, Stremel-Thomas K, Schalock M, Bashinski SM, Ruder C. Outcomes for children with deaf-blindness with cochlear implants: a multisite observational study. Otol Neurotol 2013;34:507–15. [10] Takasaki K, Kanda Y, Kumagami H, Yashida H, Yamamoto-Fukuda T, Miyamoto I, et al. Cochlear implantations in visually impaired patients. Eur Arch Otorhinolaryngol 2007;264:363–7. [11] Bond M, Mealing S, Anderson R, Elston J, Weiner G, Taylor RS, et al. The effectiveness and cost-effectiveness of cochlear implants for severe to profound deafness in children and adults: a systematic review and economic model. Health Technol Assess 2009;13:1–330. [12] van Schoonhoven J, Sparreboom M, van Zanten BG, Scholten RJ, Mylanus EA, Dreschler WA, et al. The effectiveness of bilateral cochlear implants for
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severe-to-profound deafness in adults: a systematic review. Otol Neurotol 2013;34:190–8. [13] Laszig R, Aschendorff A, Stecker M, Mu¨ller-Deile J, Maune S, Dillier N, et al. Benefits of bilateral electrical stimulation with the nucleus cochlear implant in adults: 6-month postoperative results. Otol Neurotol 2004;25:958–68. [14] Ramsden R, Greenham P, O’Driscoll M, Mawman D, Proops D, Craddock L, et al. Evaluation of bilaterally implanted adult subjects with the nucleus 24 cochlear implant system. Otol Neurotol 2005;26:988–98. [15] Litovsky R, Parkinson A, Arcaroli J, Sammeth C. Simultaneous bilateral cochlear implantation in adults: a multicenter clinical study. Ear Hear 2006;27: 714–31.
Please cite this article in press as: Takano K, et al. Outcomes of visually impaired patients who received cochlear implantations. Auris Nasus Larynx (2015), http://dx.doi.org/10.1016/j.anl.2015.08.005