Audiological outcomes after cochlear implantation in a patient with Melnick-Needles syndrome

This is the first report on a successful cochlear implantation in a patient suffering from the Melnick-Needles syndrome (MNS). This case study presents an MNS patient with peri-lingual profound hearing loss, implanted at the age of 38. MNS is a rare X-linked genetic bony dysplasia characterized by skeletal and facial abnormalities. Various soft tissue defects and hearing loss have also been described in MNS; however, this is the first report on bilateral inner ear malformation as a phenotypic feature of MNS. At 15 months after cochlear implantation there was 91% speech identification in open-set monosyllabic CVC test. In noise, the results obtained with the CVC lists were the following: 67% for SNR +15 dB, 52% for SNR +10 dB and 36% for SNR +5 dB. The patient is able to discriminate the differences in all 22 phoneme pairs in the APE phoneme discrimination test. This allows the patient to obtain significant communication ability through the telephone. Conclusions are as follows. 1) Bilateral inner ear malformations and bilateral profound perceptive hearing loss possibly belong to the phenotypic features of MNS. 2) Cochlear implant is potentially a good functional solution for patients suffering from MNS and profound hearing loss. 3) Exceptionally good results on auditory performance have been obtained in this MNS patient with peri-lingual profound hearing loss and almost 36 years duration of deafness.     

Audiological outcomes after cochlear implantation in a patient with Melnick-Needles syndrome

This is the first report on a successful cochlear implantation in a patient suffering from the Melnick-Needles syndrome (MNS). This case study presents an MNS patient with peri-lingual profound hearing loss, implanted at the age of 38. MNS is a rare X-linked genetic bony dysplasia characterized by skeletal and facial abnormalities. Various soft tissue defects and hearing loss have also been described in MNS; however, this is the first report on bilateral inner ear malformation as a phenotypic feature of MNS. At 15 months after cochlear implantation there was 91% speech identification in open-set monosyllabic CVC test. In noise, the results obtained with the CVC lists were the following: 67% for SNR +15 dB, 52% for SNR +10 dB and 36% for SNR +5 dB. The patient is able to discriminate the differences in all 22 phoneme pairs in the APE phoneme discrimination test. This allows the patient to obtain significant communication ability through the telephone. Conclusions are as follows. 1) Bilateral inner ear malformations and bilateral profound perceptive hearing loss possibly belong to the phenotypic features of MNS. 2) Cochlear implant is potentially a good functional solution for patients suffering from MNS and profound hearing loss. 3) Exceptionally good results on auditory performance have been obtained in this MNS patient with peri-lingual profound hearing loss and almost 36 years duration of deafness.     

Conservation of residual acoustic hearing after cochlear implantation.

OBJECTIVE: This study was designed to test the hypothesis that partial hearing conservation is attainable after cochlear implantation with a long perimodiolar electrode. Surgical strategies for hearing conservation during cochlear implantation are described. STUDY DESIGN: Prospective, single-subject, repeated-measures design. SETTING: Academic tertiary care center. PATIENTS: Twenty-eight severely to profoundly hearing-impaired adult cochlear implant recipients who had some measurable hearing preoperatively. INTERVENTION: Cochlear implantation using Nucleus Freedom Contour Advance electrode. MAIN OUTCOME MEASURES: Preimplant and postimplant pure-tone thresholds and speech recognition scores were obtained to determine the incidence and degree of conserved hearing at a mean interval of 9 (+/-3.9) months. RESULTS: Thirty-two percent of subjects experienced complete conservation of hearing (0- to 10-dB loss), and 57% experienced partial conservation of hearing (>11 dB) after implantation. However, open-set speech recognition was partially conserved in only one subject. Cochlear implant performance was not better in patients with conservation of residual hearing. CONCLUSION: Conservation of pure-tone hearing was possible in 89% of implanted patients; however, residual speech perception was not conserved with this long perimodiolar electrode. A ceiling effect tends to inflate the prevalence of hearing conservation in implantation studies of severely to profoundly hearing-impaired patients.     

Cochlear implantation in a child with posttraumatic single-sided deafness

Cochlear implantation has become a standard therapy for children with bilateral profound hearing loss, resulting in substantial and sustainable benefits for the development of expressive and receptive and expressive language skills and cognition. During the last few years, audiologic and otologic criteria for cochlear implantation have been expanded. Recently, patients with profound single-sided deafness with or without tinnitus have received cochlear implants despite normal to near-normal hearing on the contralateral side. This indication, however, has thus far been restricted to adult patients. Although it is known that unilateral hearing has an impact on social-emotional development in children, otologic surgeons have been reluctant to treat children with single-sided deafness with a cochlear implant. We report here on a case of successful cochlear implantation in an 8-year-old boy with acute single-sided deafness due to a lateral skull-base fracture, after an MRI showed signs of imminent fibrosis of the inner ear with possible prevention of cochlear implantation at a later stage. There was normal hearing in the contralateral ear. The child showed rapid development of speech discrimination in the implanted ear, improvements in sound localization and speech perception in noise, and a high degree of patient satisfaction. This experience may encourage using this therapeutic approach in children with chronic profound single-sided deafness.     

Factors contributing to limited open-set speech perception in children who use a cochlear implant

Cochlear implants have enabled many children with severe to profound sensorineural hearing loss to develop speech perception skills. However, some children experience few gains while others develop high levels of speech perception. We identified potential factors contributing to poor performance with an implant by studying implanted children who do not develop functional speech perception. Five children were identified as developing no open-set word recognition skills after at least 2 years of implant use. This study group was compared to a randomly selected control group (n=10) and an age-matched control group (n=5). Pre-implant factors were examined using a Graded Profile Analysis and post-implant factors were assessed in a retrospective chart review. A greater number of pre-implant concerns were raised in the study group than in randomized controls (P<0.01). Chronological age and duration of deafness were pre-implant concerns in all study group subjects. A greater number of post-implant concerns were found in the study group than in randomly selected controls (P<0.005). We conclude that while appropriate selection of candidates for cochlear implantation is important in predicting speech perception outcomes, post-implant follow-up is also essential and must include regular monitoring of equipment, monitoring of stimulation levels with use of objective measures of stimulation levels if necessary, and consistent habilitation.     

Cochlear implantation for children with GJB2-related deafness

OBJECTIVES/HYPOTHESIS: Mutations in GJB2 are a common cause of congenital sensorineural hearing loss. Many children with these mutations receive cochlear implants for auditory habilitation. The purpose of the study was to compare the speech perception performance of cochlear implant patients with GJB2-related deafness to patients without GJB2-related deafness. STUDY DESIGN: Retrospective case review. METHODS: Pediatric cochlear implant recipients who have been tested for GJB2 mutation underwent chart review. All patients received cochlear implantation at a tertiary referral center, followed by outpatient auditory habilitation. Charts were reviewed for cause and duration of deafness, age at time of cochlear implantation, intraoperative and postoperative complications, duration of use, and current age. Results of standard tests of speech perception administered as a part of the patients' auditory habilitation were reviewed. RESULTS: Twenty patients with GJB2 mutations were compared with 27 patients without GJB2 mutations. There was no statistical difference between patients with and without GJB2-related congenital sensorineural hearing loss with regard to open-set and closed-set speech recognition performance at 12, 24, and 36 months after cochlear implantation. Surgical complications were uncommon. CONCLUSION: Pediatric patients with congenital sensorineural hearing loss without other comorbid conditions (eg, developmental delay, inner ear malformations) perform well when they receive cochlear implantation and auditory habilitation. The presence or absence of GJB2 mutation does not appear to impact speech recognition performance at 12, 24, and 36 months after implantation.     

Cochlear implantation in a patient with Paget’s disease

Paget’s disease of the temporal bone can cause hearing loss that eventually develops into profound deafness. However, the treatment options for hearing loss associated with Paget’s disease are limited. Thus far, only 3 cases of cochlear implantation in patients with Paget’s disease have been reported. In this study, we describe a 62-year-old woman who underwent cochlear implantation for progressive bilateral sensorineural hearing loss. The patient gained useful open-set speech perception over a 5-year postoperative period. Cochlear implantation can be beneficial in patients with profound hearing loss associated with Paget’s disease.     

Speech perception in congenitally,pre‐lingually and post‐lingually deaf children expressed in an equivalent hearing loss value

Objectives: To investigate the speech perception performance of children with a cochlear implant (CI) after 3 and 4 years of follow‐up and to study the influence of age at implantation, duration of deafness and communication mode on the variability in speech perception performance. Study design: A broad battery of speech perception tests was administered to 67 children with a CI. The results were reduced into one measure: the ‘equivalent hearing loss (EHL)’. This outcome measure refers to the performance of a reference group of severely and profoundly hearing impaired children with conventional hearing aids. Participants: The population comprised 35 congenitally, 17 pre‐lingually and 15 post‐lingually deaf children implanted between 1989 and 1999. The population was homogeneous with respect to cognition, residual hearing and support at home as a result of conservative inclusion criteria. Results: During the first 2 years after implantation, post‐lingually deaf children showed the fastest rate of improvement in speech perception. After 3 years of implant use, the early implanted pre‐lingually deaf children and congenitally deaf children implanted under the age of 6 years caught up with the post‐lingually deaf children. Pre‐lingually deaf children implanted after a relatively long‐duration of deafness tended to show poorer performance than those with a shorter duration. Performance of congenitally deaf children implanted after the age of 6 years was poorer and progress was slower. In the congenitally deaf children, 36% of the variability in performance was explained by duration of deafness, whereas in the children with pre‐ and post‐lingually acquired deafness, communication mode explained 69% of the variance. Conclusions: All children derived benefit from their CI for speech perception tasks, but performance varied greatly. Several children reached EHL levels around 70 dB; their speech perception was equal to that of a child with conventional hearing aids who has 70 dB HL. After early implantation, the levels of performance that were eventually achieved differed no more than 10 dB, irrespective of whether the onset of deafness was pre‐lingual or postlingual. In congenitally deaf children, duration of deafness played a major role in speech perception performance, whereas in the children with pre‐lingually and post‐lingually acquired deafness together, mode of communication was a major factor.     

Connexin-associated deafness and speech perception outcome of cochlear implantation

OBJECTIVE: To compare performance after cochlear implantation in children with mutations in connexin (Cx) 26 (GJB2) or Cx30 (GJB6) and children with deafness of unknown etiology. DESIGN: Genetic analysis and speech perception evaluation was performed in the children with and without Cx mutations who had undergone cochlear implantation. Speech perception performance was retrospectively analyzed 6, 12, 24, 36, and 48 months after implantation. Test material was selected according to the child's age and cognitive and language abilities. SETTING: The study took place at speech and hearing and genetic centers of a hospital in the central part of Israel and the genetics departments of 3 additional centrally located hospitals. PATIENTS: A total of 30 children who had undergone cochlear implantation were selected for the study, with control patients matched according to age at implantation, duration of implant use, and mode of communication. There was no evidence for additional disabilities or handicaps in either group. MAIN OUTCOME MEASURES: Speech perception measurements included a questionnaire, as well as closed and open-set tests. RESULTS: Overall, the 2 groups showed significant improvement in speech perception results after implantation. Four years after implantation, both groups achieved mean open-set speech perception scores of approximately 60%, 75%, and 90% for monosyllabic, 2 syllables, and words in sentences tests, respectively. CONCLUSIONS: There were no apparent differences in speech perception performance after implantation between the children with Cx mutations and children with deafness of unknown etiology. These data have important implications as a prognostic indicator when counseling candidates for cochlear implantation.     

Barriers to Cochlear Implant Access: Acknowledging the Challenges,Changing the Future: The Limitations of FDA Criteria: Inconsistencies with Clinical Practice,Findings, and Adult Criteria as a Barrier to Pediatric Implantation

Children require greater access to sound than adults as they are learning to communicate using hearing and spoken language. Yet when it comes to cochlear implant candidacy, currently approved Food and Drug Administration (FDA) criteria for adults are much less restrictive than those for children, allowing for greater levels of residual hearing and aided speech recognition in adults. Cochlear implant guidelines for children have changed very little in the 30 years since cochlear implants have been approved for pediatrics, and this lack of change has proven to be a barrier to implantation. Using evidence-based practice, centers have been providing off-label implantation for children who fall outside of current FDA criteria, including children with more residual hearing, children with single-side deafness younger than 5 years, and infants with bilateral profound loss younger than 9 months. The purpose of this article is to outline how these restrictions impede access to implants for children and describe the evidence supporting cochlear implantation in children who fall outside of current criteria.     

Speech, language, and reading skills after early cochlear implantation

OBJECTIVE: To examine whether age at cochlear implantation or duration of implant use is associated with speech, language, and reading skills exhibited at age 8 to 9 years in children who underwent implantation by age 5 years. DESIGN: Performance outcomes in speech perception, speech production, language, and reading were examined in terms of the age at which children first received a cochlear implant (2, 3, or 4 years), the age they received an updated (Spectra) processor, and the duration of use of an implant and an updated processor. SETTING: Data collection was conducted at summer research camps held over 4 consecutive years to maximize the number of children available at a specific age (8-9 years). Children were tested individually by experienced examiners, and their parents and therapists provided background and educational history information. PARTICIPANTS: A total of 181 children from 33 different states and 5 Canadian provinces who received a cochlear implant by age 5 years were tested. A subsample of 133 children with performance IQ scores of 80 or greater and onset of deafness at birth were selected for the age-at-implantation analysis. Another subsample of 39 children with deafness acquired by age 3 years was also examined. OUTCOME MEASURES: A battery of tests of speech perception, speech production, language, and reading was administered to each child and reduced to a single factor score for each skill. RESULTS: Correlation coefficients between age at implantation and duration of use did not reach significance for any of the outcome skills measured. Age at which the updated speech processor (Spectra) was fitted was significantly related to speech production outcome (earlier use of an updated processor was associated with greater speech intelligibility) but not to any other skill area. However, more of the children who underwent implantation at age 2 years (43%) achieved combined speech and language skills commensurate with their age-matched peers with normal hearing than did children who underwent implantation at age 4 years (16%). Furthermore, normal speech and language skills were documented in 80% of children who lost hearing after birth and who underwent implantation within a year of onset of deafness. CONCLUSIONS: For children who receive a cochlear implant between the ages of 2 and 4 years, early cochlear implantation does not ensure better speech perception, speech production, language, or reading skills. However, greater speech and language proficiency may be expected from children who exhibit normal hearing for even a brief period after birth and receive a cochlear implant shortly after losing their hearing. Further research examining the benefits of cochlear implantation before age 2 years will help families and clinicians better understand the time-sensitive nature of the decision to conduct cochlear implant surgery.     

Predictive models for cochlear implantation in elderly candidates

OBJECTIVE: An aging American population carries a high prevalence of profound sensorineural hearing loss. We examined the performance of multichannel cochlear implant recipients in a large database of adult subjects. DESIGN: Nonconcurrent prospective study of a national cohort with multivariate regression analysis of preoperative and postoperative performance variables in multichannel cochlear implant recipients. We applied models of prediction established in previous studies to the observed results. SETTING: Referral centers with active cochlear implant programs. PATIENTS: Adolescents and adults with profound hearing loss (N = 749; age range, 14-91 years). MAIN OUTCOME MEASURE: Postoperative monosyllabic word recognition. RESULTS: The population 65 years and older demonstrated a clinically insignificant 4.6%-smaller postoperative word score compared with the population younger than 65 years. When duration of deafness exceeded 25 years, elderly recipients demonstrated higher word scores than their younger counterparts. A more significant factor affecting outcomes is the ratio of duration of deafness to age at implantation. CONCLUSIONS: Age at implantation carried relatively little predictive value for postoperative performance in subjects 65 years and older. Although a small decrement in mean speech recognition scores was evident, the clinical significance of this difference is questionable when all of the results observed in elderly patients are considered. A shorter percentage of life spent in severe-to-profound sensorineural hearing loss suggests a foundation of acoustic/auditory processing in the elderly cohort that may mitigate potential physiological effects associated with advanced age. This study confirms and extends previous observations that duration of profound deafness and residual speech recognition carry higher predictive value than the age at which an individual receives an implant.     

Residual speech perception and cochlear implant performance in postlingually deafened adults

OBJECTIVE: This study aimed at testing the post-hoc validity of the previously reported predictive index for postoperative cochlear implant performance, based on preoperative duration of deafness, and speech reception. STUDY DESIGN: Adult patients with postlingual severe to profound hearing loss, who were implanted with Cochlear Corporation CI-22 and CI-24 devices were included in this study. We studied the relationship between their postoperative word recognition scores and preoperative factors, namely, duration of deafness, and sentence recognition. We used the same predictive index reported in the previous study to predict their postoperative scores and test the model's agreement with the actual performance. RESULTS: We found that postoperative performance as measured by CNC word scores had an inverse relationship with the duration of deafness, and a direct correlation with the preoperative performance on CID sentence recognition tests. A nonlinear term [Duration / (1+CID)] was shown to improve the correlation coefficient of our predictive index. CONCLUSION: Some predictability of cochlear implant outcome is possible depending on the preoperative duration of deafness and speech recognition abilities. Preoperative residual speech recognition acts as a "trophic factor" that protects the spiral ganglion and/ or the central auditory pathways from degeneration. In other words, it improves the expected postoperative word scores.     

Cochlear implantation in prelingually deafened children with residual hearing

OBJECTIVE: The purpose of this study was to examine the speech perception skills of prelingually deafened French children with preoperative residual hearing who received multichannel cochlear implants. DESIGN: The design of the study incorporated a within-subject, repeated measures design for assessing speech perception skills. SETTING: Montpellier, Toulouse and Lyon Pediatric Cochlear Implant Centers. SUBJECTS: Seven prelingually deafened children demonstrating marginal benefit from conventional amplification prior to implantation with a Nucleus multichannel cochlear implant, served as subjects for the speech perception assessment (a speech recognition score less than 30% defines marginal benefit from acoustic amplification on open set materials). The mean age at implantation was 7 years, 9 months. OUTCOME MEASURES: Speech perception skills were assessed using open set materials and the MUSS and MAIS questionnaires. RESULTS: Open-set speech recognition averaged 21.4% before implantation, and 83.6% after 1 year's cochlear implant experience. All children demonstrated an open-set score over 60% after 12 months of CI use. MAIS test scores averaged 18.1/40 before implantation and 35.1/40 after 9 months of CI use. MUSS test scores averaged 24.4/40 before implantation and 34.1/40 after 9 months of CI use. CONCLUSIONS: Cochlear implantation should be considered for prelingually hearing impaired children demonstrating marginal benefit from hearing aids, with a speech recognition score less than 30% on open set materials, in order to improve their speech discrimination skills.     

Speech recognition in elderly cochlear implant recipients

The benefits of cochlear implantation in the adult and paediatric populations are well established. Cochlear implantation in the geriatric population still remains controversial because of the misconception that elderly patients might perform poorly. The purpose of this study was to report the speech performance of 16 patients over 65 years of age implanted with a Nucleus multichannel cochlear implant and to compare it with that of a control group of 14 adults aged between 41 and 59 years. At the 12 months postoperative evaluation, no significant differences were detected on speech performances between the elderly patients and the control group. The mean word recognition scores were 72.5% for the elderly group and 82% for the control group. The mean everyday sentence recognition scores were 72.5% for the elderly group and 85.7% for the control group. Overall, the results are encouraging and demonstrate that the elderly population with profound hearing loss obtain significant benefits from cochlear implantation despite the age-related auditory processing problems.     

Speech perception in children using cochlear implants: prediction of long-term outcomes

A group of 102 children using the Nucleus multichannel cochlear implant were assessed for open-set speech perception abilities at six-monthly intervals following implant surgery. The group included a wide range of ages, types of hearing loss, ages at onset of hearing loss, experience with implant use and communication modes. Multivariate analysis indicated that a shorter duration of profound hearing loss, later onset of profound hearing loss, exclusively oral/aural communication and greater experience with the implant were associated with better open-set speech perception. Developmental delay was associated with poorer speech perception and the SPEAK signal coding scheme was shown to provide better speech perception performance than previous signal processors. Results indicated that postoperative speech perception outcomes could be predicted with an accuracy that is clinically useful.     

Nucleus 22 Cochlear Implantation Results in Postmeningitic Deafness

Cochlear implant surgery was performed on 13 patients with postmeningitic deafness (seven adults, six children). Two adults and two children (30.8%) had severe labyrinthitis ossificans requiring radical “drill-out.” Five of 13 (38.5%) had some bone growth requiring partial drill-out, and four of 13 (30.8%) had normal insertion with no drill-out. Hearing results for patients with no bone growth were similar to nonmeningitic patients; three of four (75%) had open-set speech recognition. Performance of patients with total drill-out was poor; “auditory only” performance was limited to detection and pattern perception of speech, and no patients had open-set speech recognition. Results for patients with partial drill-out were similar to results in patients with no bone growth. Labyrinthitis ossificans not only presents surgical challenges to cochlear implantation but may also adversely affect hearing outcome.     

1-year postactivation results for sequentially implanted bilateral cochlear implant users.

OBJECTIVE: Evaluate speech recognition in quiet and in noise for a group of 12 children, all of whom underwent sequential bilateral cochlear implantation at various ages (range, 1 yr, 8 mo to 9 yr, 6 mo at time of second implant). STUDY DESIGN: Retrospective. SETTING: Outpatient cochlear implant clinic. PATIENTS: Children who underwent sequential bilateral cochlear implantation. INTERVENTION: Rehabilitative. MAIN OUTCOME MEASURES: Speech recognition in quiet was evaluated for each ear separately using single-word speech recognition assessments (Multisyllabic Lexical Neighborhood Test and Early Speech Perception Test) via recorded presentation. Speech recognition in noise was assessed for each ear separately and in the bilateral condition by obtaining a spondee recognition threshold in the presence of speech-weighted noise presented at 45 dB hearing level. The primary outcome measure for speech recognition in noise assessment was the signal-to-noise ratio for 50% performance, which was calculated by determining the difference between the presentation level of the noise and the presentation level at which the speech recognition threshold was obtained. The results of these assessments were contrasted between children receiving their second cochlear implant before 4 years of age and children receiving their second cochlear implant after 4 years of age. RESULTS: A statistically significant difference for speech recognition scores in quiet was obtained between the early-implanted ear and the late-implanted ears for children receiving their second cochlear implant after 4 years of age. There was not a statistically significant difference in speech recognition scores in quiet between the early-implanted and late-implanted ears of children receiving their second cochlear before 4 years of age. Both groups of children possessed better speech recognition scores in noise (statistically significant at an alpha = 0.05) in the bilateral condition relative to either unilateral condition. However, there was not a statistically significant relationship between speech recognition performance in noise and the duration of deafness of the later implanted ear. CONCLUSION: Bilateral cochlear implantation allowed for better speech recognition in noise relative to unilateral performance for a group of 12 children who underwent sequential bilateral cochlear implantation at various ages. There was not a statistically significant relationship between speech recognition in noise benefit, which was defined as the difference in performance between the first implanted ear and the bilateral condition and the age at which the second implant was received. Children receiving bilateral cochlear implants younger than 4 years of age achieved better speech recognition in quiet performance for the later implanted ear as compared with children receiving their second cochlear implant after 4 year of age.     

Cochlear implantation after surgery for bilateral jugular foramen paragangliomas: a case report

This paper describes a case of cochlear implantation for deafness due to bilateral glomus jugulare tumors (paragangliomas) as well as an unusual complication after cochlear implantation in a 31-year-old male. A 31-year-old male with profound sensorineural hearing loss on the right side and deafness on the left side, caused by bilateral jugular foramen paragangliomas, was implanted with a multi-channel cochlear implant (Combi 40+, Med-El) on the right side during subtotal petrosectomy for removal of the right-sided glomus jugulare tumor. No postoperative medical complications were observed. The patient responded to acoustic stimuli. Postoperative computed tomography (CT) did not show any misplacement of the electrode. Three years after implantation, an acute, rapidly progressive hearing impairment with pain to acoustic stimuli was observed. A CT scan at that time showed cochlear bone resorption. No radiologic evidence of paraganglioma recurrence was observed. The implant was removed and a biopsy of the cochlea was performed which on histologic examination showed fibrosis without any sign of any tumor recurrence. Cochlear implantation can restore hearing in deafness due to bilateral glomus jugulare tumors. Cochlear resorption may occur as a late complication in the implanted side in the patients.     

Outcomes for cochlear implant users with significant residual hearing: implications for selection criteria in children

OBJECTIVES: To develop an evidence-based technique for providing recommendations to candidates for cochlear implantation with significant residual hearing and to assess the efficacy of the approach. DESIGN: Modified selection criteria were derived from an analysis of the postoperative performance for a large group of adult cochlear implant users. In particular, the distributions of results for implant users with significant preoperative open-set speech perception were reviewed. This suggested that the candidates had a good chance (>75%) of overall improvement if they obtained open-set sentence scores in quiet of up to 70% in the best-aided condition and scores of up to 40% in the ear to undergo implantation. PATIENTS: A group of 45 adult implantation candidates who fit the modified criteria and who underwent preimplantation and postimplantation assessment to compare actual results with those predicted from the distributions. RESULTS: The speech perception results showed that 36 subjects (80%) had improved open-set sentence scores with the cochlear implant compared with their best-aided preoperative performance (mean improvement, 20.5%). Forty-four (98%) had improved open-set sentence scores for the ear undergoing implantation (mean improvement, 65.3%). CONCLUSIONS: The general concept of using the distribution of speech perception results to make evidence-based recommendations for candidates for cochlear implants is supported by this study. The approach can be used across different subpopulations, including older children with significant residual auditory skills, and for different outcome measures. It is important that the data used to provide recommendations and modify selection criteria are from an unselected sample of implant users of adequate size. This study highlights the continuing need to evaluate speech perception performance carefully before and after cochlear implantation.