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To review the outcomes of cochlear implants (CI) in patients with neurofibromatosis type 2 (NF2) in a large cohort, and identify factors associated with poor hearing benefit.Fifteen-year retrospective national observational case series.United Kingdom regional NF2 multidisciplinary teams.Consecutive patients with NF2 receiving a CI.CI for hearing rehabilitation.1) Audiometric performance at 9 to 12 months after implantation using City University of New York (CUNY) sentence recognition score, and Bamford- Kowal-Bench (BKB) word recognition score in quiet (BKBq), and in noise (BKBn). 2) CI use at most recent review.Sixty four consecutive patients, median age 43 years, were included. Nine to 12 months mean audiometric scores were: CUNY 60.9%, BKBq 45.8%, BKBn 41.6%. There was no difference in audiometric outcomes between VS treatment modalities. At most recent review (median 3.6 years from implantation), 84.9% with device in situ/available data were full or part-time users. Between 9 and 12 months and most recent review there was an interval reduction in mean audiometric scores: CUNY -12.9%, BKBq -3.3%, BKBn -4.9%. Larger tumor size and shorter duration of profound hearing loss were the only variables associated with poorer audiometric scores. Tumor growth at the time of surgery was the only variable associated with CI non-use. Individual patient response was highly variable.CI can provide significant and sustained auditory benefits to patients with NF2 independent of tumor treatment modality, with the majority of those implanted becoming at least part-time users. Larger datasets are required to reliably assess the role of independent variables.

Objective: The National Institute of Clinical Excellence recommends that adult patients with severe to profound deafness are eligible for simultaneous bilateral cochlear implantation if they are bl...

Background It is common for patients with neurofibromatosis type 2 to develop bilateral profound hearing loss hearing loss, and this is one of the main determinants of quality of life in this patient group. Objectives The aim of this systematic review was to review the current literature regarding hearing outcomes of treatments for vestibular schwannomas in neurofibromatosis type 2 including conservative and medical management, radiotherapy, hearing preservation surgery and auditory implantation in order to determine the most effective way of preserving or rehabilitating hearing. Search strategy A MESH search in PubMed using search terms (('Neurofibromatosis 2' [Mesh]) AND 'Neuroma, Acoustic'[Mesh]) AND 'Hearing Loss' [Mesh] was performed. A search using keywords was also performed. Studies with adequate hearing outcome data were included. With the exception of the cochlear implant studies (cohort size was very small), case studies were excluded. Evaluation method The GRADE system was used to assess quality of publication. Formal statistical analysis of data was not performed because of very heterogenous data reporting. Results Conservative management offers the best chance of hearing preservation in stable tumours. The use of bevacizumab probably improves the likelihood of hearing preservation in growing tumours in the short term and is probably more effective than hearing preservation surgery and radiotherapy in preserving hearing. Of the hearing preservation interventions, hearing preservation surgery probably offers better hearing preservation rates than radiotherapy for small tumours but recurrence rates for hearing preservation surgery were high. For patients with profound hearing loss, cochlear implantation provides significantly better auditory outcomes than auditory brainstem implantation. Patients with untreated stable tumours are likely to achieve the best outcomes from cochlear implantation. Those who have had their tumours treated with surgery or radiotherapy do not gain as much benefit from cochlear implantation than those with untreated tumours. Conclusions This review summarises the current literature related to hearing preservation/rehabilitation in patients with NF2. Whilst it provides indicative data, the quality of the data was low and should be interpreted with care. It is also important to consider that the management of vestibular schwannomas in NF2 is complex and decision-making is determined by many factors, not just the need to preserve hearing.

The aim of this study was to quantify the benefit gained from cochlear implantation in pre- or peri-lingually deafened patients who were implanted as adults Methods: This was a retrospective case-control study. Auditory (BKB/CUNY/3AFC/Environmental sounds), quality of life (GBI/HUI3) and cognitive (customized questionnaire) outcomes in 26 late implanted pre- or peri-lingually deafened adults were compared to those of 30 matched post-lingually deafened, traditional cochlear implant users.There was a statistically significant improvement in all scores in the study group following cochlear implantation. BKB scores for cases was 49.8% compared to 83.6% for controls (p=0.037). CUNY scores for cases was 61.7% compared to 90.3% for controls (p=0.022). The 3AFC and environmental sounds scores were also better in controls compared to cases but the difference was not statistically significant. Quality of life scores improved following implantation in cases and controls but the improvement was only statistically significant in the controls. There was a 7.7% non-user rate in the cases. There were no non-users in the control group.Early deafened,,late implanted patients can benefit audiologically from cochlear implantation and in this study the improvement in speech discrimination scores was greater than expected perhaps reflecting careful selection of patients. Nevertheless, audiological benefits are limited compared to traditional cochlear implant recipients with the implant acting as an aid to lip reading in most cases.With careful selection of candidates, cochlear implantation is beneficial in early deafened, late implanted patients.

Aim To assess the long-term subjective benefits of auditory brainstem implants (ABIs) and investigate the extent to which ABI users perceive it to be a useful device. Methods Using the Manchester database, all 31 living patients registered as ABI users were identified and sent the ABI performance questionnaire. Data regarding daily duration of use, auditory fatigue, ability to differentiate between speech and environmental sounds, and subjective usefulness of the ABI in different listening conditions were collected. Patients were asked to rate the usefulness of the device in various settings on a scale of 1 (not useful) to 6 (very useful). The mean age at implantation was 33 years (13-73 yr), and the mean follow-up period was 6 years (1.5-15 yr). Results Between March 1994 and September 2009, 57 patients underwent ABI insertion. A total of 26 patients were not eligible for inclusion in the study (19 nonusers, 5 deceased, and 2 sleepers). Of the 31 eligible patients identified as ABI users, 23 returned the questionnaire, 1 was excluded as the questionnaire had been completed on his or her behalf, giving a net response rate of 71%. Mean duration of usage per day was 12.62 hours (range, 8-16 h). Seventy-one percent of the patients turned the processor off at one or more points during the day. Differentiation between speech and environment was achieved in 95%, and 70% were able to differentiate between gender and adult and pediatric voices. The ABI was perceived as most beneficial when dealing with a familiar voice in a quiet place, with a median usefulness score of 4, rising to 5 when used in conjunction with lip reading. The ABI was least useful when dealing with an unfamiliar voice in a loud place, with a mean score of 1, rising to 2 when used in conjunction with lip reading. In all environments, combining the ABI with lip reading served to increase usefulness ratings by at least 1 point. Conclusion This study demonstrates that ABI users make use of their device for at least 12 hours per day on average and obtain considerable subjective benefit in speech discrimination when using the device with familiar speakers in a quiet environment. Benefit is less significant in noise and with an unfamiliar speaker. Lip reading enhances subjective benefit by at least 1 point, and lip reading training may have a role to play in maximizing subjective benefit.

OBJECTIVE: To determine differences in speech perception outcomes for patients who received a CI422 and a Contour cochlear implant. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Thirty-two adults who underwent cochlear implantation. INTERVENTION: Cochlear implantation using a CI422 or Contour device. MAIN OUTCOME MEASURE: Bamford-Kowal-Bench (BKB) speech perception scores at 3 and 9 months after activation. RESULTS: The mean BKB scores at 3 months for the CI422 device were 86.0% in quiet and 55.1% in noise. This compares with 86.0% in quiet and 62.3% in noise for the Contour device. At 9 months, the mean BKB scores were 85.9% in quiet and 67.1% in noise for the CI422 and 90.1% in quiet and 77.6% in noise for the Contour device. There was no statistically significant difference (p > 0.05) between speech perception outcomes at 3 or 9 months. CONCLUSION: This study suggests that CI422 and Contour electrode both improve speech perception outcomes postoperatively, and there does not appear to be any significant difference in outcome between the two types of devices.

The objective of this study was to describe changes in hearing over time in patients with neurofibromatosis type 2 (NF2) treated conservatively. A retrospective case review was conducted in a tertiary referral centre. Pure tone audiometry, speech discrimination scores, serviceable hearing (American Academy of Otolaryngology class A or B) and measurement of vestibular schwannoma (VS) size on magnetic resonance imaging were evaluated in 56 patients (89 ears) with NF2 with at least one conservatively managed VS. Over a mean follow-up period of 7 years (range 0.8-21 years) pure tone average thresholds increased gradually with a mean annual rate of 1.3 dB for the right ear (p = 0.0003) and 2 dB for the left ear (p = 0.0009). Speech discrimination scores dropped with an average annual rate of 1.3 and 0.34% in the right and left ear, respectively. Patients maintained serviceable hearing for an average of 7.6 years (range 2.7-19.3 years). The average annual VS growth was 0.4 mm without any correlation with hearing loss. There was a correlation between patients' age and pure tone threshold increase (p < 0.05 for both ears). In this selected population of patients with NF2, hearing threshold increases were very slow. In NF2 patients with indolently behaving tumours, serviceable hearing can be maintained for a significant length of time, making conservative management an attractive option.

Audits were carried out to assess the clinical effectiveness of historical annual review appointments and a new patient-led review system.The written records of 100 patients who attended for an annual review appointment were retrospectively analysed. A further 50 patients who did not respond to the patient-led review offer letter were contacted to determine why they had not responded. The records of patients who subsequently attended for review were also analysed to evaluate their outcome.Ninety-one per cent of patients tested showed no change or an improvement in aided thresholds. Eighty-nine per cent of patients tested showed no change or an improvement in speech discrimination scores. Ninety-three per cent of patients tested showed no change in impedances. Fifty-two per cent of patients required a map check and 36% of patients needed some equipment changed. One patient out of 100 needed referral to Ear, Nose and Throat (ENT). Twenty per cent of the patients who did not respond to the review offer letter reported they had not received the letter. The remainder did not require an appointment or did not understand the system.The results indicate that the majority of patients' implant and functional performance remains stable over time. The process of patient-led review has been optimized, so patients fully understand the system and are not missed if they do not receive the letter.Patient-led review allows patients to take more responsibility, so they can participate more proactively in the maintenance of their implant and the centre is able to focus their resources on the patients who need it.

OBJECTIVE: To describe the experience of auditory brainstem implantation (ABI) in patients with Neurofibromatosis type 2 (NF2).STUDY DESIGN: Retrospective case review.SETTING: Tertiary referral centre.PATIENTS: Implanted with a Cochlear ABI22 or ABI24M between 1994 and 2009 because of NF2 disease.INTERVENTION(S): Rehabilitative.MAIN OUTCOME MEASURE(S): Surgical complication rate; audiological outcomes.RESULTS: There were 50 primary ABI insertions in 49 patients, including 16 inserted at the time of first side tumor removal as a sleeper, and two revision repositionings which failed to improve outcome. Postoperatively three patients had cerebrospinal fluid leaks which did not require reoperation, one patient had meningitis, and eleven patients suffered either temporary or permanent lower cranial nerve dysfunction. Twenty-nine patients became full time users; a further 12 patients became non-users. Three patients died while their device was inactive. Five patients retain serviceable contralateral hearing. Audiological open set testing of users showed means of: environmental sounds discrimination 51%; phoneme discrimination: with ABI alone 22%/lip reading (LR) 45%/ABI with LR 65%; sentence testing: with ABI alone 13%/LR 19%/ABI with LR 54%.CONCLUSIONS: The majority of patients with NF2 implanted with an ABI find the device a useful aid to communication in conjunction with LR and in recognizing common environmental sounds. A small proportion gain open set discrimination. Almost a third of patients may end up as non-users. There is probably an increased risk of postoperative lower cranial nerve dysfunction so careful preoperative assessment is advised.

Background: Adult cochlear implant (CI) candidacy is assessed in part by the use of speech perception measures. In the United Kingdom the current cut-off point to fall within the CI candidacy range is a score of less than 50% on the BKB sentences presented in quiet (presented at 70 dBSPL). Goal: The specific goal of this article was to review the benefit of adding the AB word test to the assessment test battery for candidacy. Results: The AB word test scores showed good sensitivity and specificity when calculated based on both word and phoneme scores. The word score equivalent for 50% correct on the BKB sentences was 18.5% and it was 34.5% when the phoneme score was calculated; these scores are in line with those used in centres in Wales (15% AB word score). Conclusion: The goal of the British Cochlear Implant Group (BCIG) service evaluation was to determine if the pre-implant assessment measures are appropriate and set at the correct level for determining candidacy, the future analyses will determine whether the speech perception cut-off point for candidacy should be adjusted and whether other more challenging measures should be used in the candidacy evaluation.

OBJECTIVE There is very little information in the literature regarding outcomes of cochlear implantation in patients profoundly deafened after head injury. The aim of this study was to assess outcomes in this group of patients. STUDY DESIGN Retrospective case review. SETTING The Manchester Cochlear Implant Programme, University of Manchester. PATIENTS Profoundly deafened patients after head injury who proceeded to cochlear implantation for auditory rehabilitation. MAIN OUTCOME MEASURES Mean age and duration of deafness at implantation. Preimplantation and postimplantation speech perception outcomes were measured using Bench Kowel Bamford (BKB) sentences in quiet and noise, City University of New York sentences with lip reading and Arthur Boothroyd words scoring the percentage phonemes correct. RESULTS Twenty patients received 23 cochlear implants. Mean age at implantation was 51 years (standard deviation, 12 yr). Mean duration of deafness at implantation was 12 years (range, 1-30 yr).Preimplantation BKB score in quiet of 0%. Mean postimplantation BKB score in quiet was 64% (range, 0%-100%) and in noise was 61% (range, 0%-97%). Three were nonusers, and 1 required reimplantation. There was a moderately negative correlation between outcome and age at implantation (r = -0.41, p < 0.05) and between outcome and duration of deafness (r = -0.52, p < 0.05). CONCLUSION Cochlear implantation is an effective method for hearing rehabilitation in profoundly deafened patients after head injury. However, negative factors, such as significant injury to the central auditory pathway, basal turn obliteration, long duration of deafness, and nonauditory stimulation, should be considered in the preoperative assessment of these patients.

The suggestion that the depth of insertion of the electrode into the cochlea is critical to hearing preservation has led to the development of a generation of short electrodes designed to minimize intracochlear trauma and avoid contact with the apical region of the cochlea. This study aims to describe our experience of hearing preservation surgery using a deeply inserted standard length electrode array covering the region of residual hearing.A retrospective case note review was performed identifying cases of attempted hearing preservation using standard length electrodes.Study based at Manchester Royal Infirmary, a tertiary referral center.Fourteen cochlear implants in 13 patients were identified for further analysis from the Manchester Cochlear Implant Programme database.Each patient received the same design of implant using a "soft" surgical technique.Preoperative and postoperative air conduction thresholds were compared to assess the degree of hearing preservation.Successful hearing preservation was demonstrated in 12 of 14 cases, and the postoperative residual hearing thresholds in 3 adolescents receiving a standard length electrode array were found to have improved. Preservation of speech recognition was not measured in this study, rather hearing was tested by pure tone audiogram. Follow-up at the time of this study ranged from 1 week to 23 months.This study demonstrates that deep insertion of the electrode into the cochlea does not preclude successful hearing preservation. It also highlights that residual hearing can be consistently preserved using a "cochleostomy" approach.

The Manchester Cochlear Implant Programme was established in 1988 and over 1000 patients have been implanted to date. Developments and improvements in cochlear implant technology over the past 21 years have resulted in new generations of implants and speech processors becoming available for use with patients. Since 2004, our patients have been asked to choose a device, based on their own preferences.This study looks at the speech discrimination scores of adults fitted with the latest generation of speech processors, including the Freedom (n = 40), the Harmony (n = 3) and the Opus 2 (n = 7). These scores are also compared to those of patients using earlier generations of speech processors, including the Esprit 3G (n = 89), the Auria (n = 9) and the Tempo+ (n = 38).Speech discrimination assessments (CUNY sentences, BKB sentences (in quiet and in noise) and AB words) were undertaken at the following intervals: 1 week, 3 months, 9 months and 21 months post switch-on. Patients were grouped according to...

A review of sporadic and NF2-related vestibular schwannoma surgery in children (under 18 years of age) with a specific interest in resection rates, recurrence, facial nerve outcomes, hearing preservation, hearing rehabilitation and genetic analysis.A retrospective analysis of prospectively collected data of 35 consecutively operated vestibular schwannomas in 29 paediatric patients that underwent 38 operations between 1992 and 2007. Pre- and post-operative radiology, facial nerve function, pure tone audiogram and speech discrimination tests were performed with a mean follow-up of 4.5 years. Tumour and blood mutations were analysed in 86% of patients.Total resection was achieved in all sporadic cases and 68% of NF2 cases. Near total resection led to tumour recurrence in 5 out of 10 cases. The facial nerve was anatomically preserved in 92%. Facial nerve function was excellent to good (Grades 1-3) in 88% with outcome related to tumour size. Hearing preservation was successful in 3 of 11 cases.Surgery with complete resection results in excellent tumour control, but it is more difficult to attain total resection in NF2 with a relatively high recurrence rate of persistently growing tumours. A better facial outcome is associated with smaller tumours, near-total resection and first time surgery. Hearing preservation is possible in a minority. Hearing rehabilitation can be successful by utilising cochlear implants and auditory brain stem implants (ABI) as appropriate. Overall there is a low complication rate and results are comparable with adult series.

OBJECTIVES: To objectively evaluate the clinical and functional outcomes of cochlear implantation in an elderly population. DESIGN: Retrospective comparative study. SETTING: Neurotology unit at Manchester Royal Infirmary, a supraregional tertiary referral centre in collaboration with Adult Cochlear Implant Programme at The University of Manchester. PARTICIPANTS: All cochlear implant procedures (38) undertaken on post-lingually deafened elderly patients (age range at the time of implantation 65-80 years, n = 34) in the period from 1989 to 2002. MAIN OUTCOME MEASURES: Medical and surgical outcomes. Audiological performance outcomes for isolated words, words in sentences in quiet and noise. Functional outcome measures used are self-reported measures of the social, psychological and emotional aspects of quality of life, and the differences between expectations for functional outcomes and the realization of functional outcomes. They included expectation profiles, Glasgow Benefit inventory (GBI) and Glasgow Health Status Inventory Questionnaire (GHSI). RESULTS: There was statistically significant improvement post-implantation of both open and closed set test scores (P < 0.01). Eighty-two percentage of patients were completely satisfied with their cochlear implants. Patients judged that implantation restored half the loss of quality of life that they had experienced as a result of severe-profound deafness with a highly significant (P < 0.001) improvement in overall quality of life after implantation. The commonest post-operative observation was transient mild pyrexia. CONCLUSIONS: The age of a cochlear implant candidate should not be a factor in the candidacy decision-making process. The quality of life of our elderly recipients was significantly improved after cochlear implant.

This retrospective study evaluated the psychophysical parameters of 29 postlingually deafened adults who had received a Nucleus Contour or Contour Advance implant during the first month of fitting in order to determine how many sessions were required to obtain a stable implant MAP. The T-levels did not differ significantly across the five fitting sessions, but the C-levels as well as the dynamic range showed an increase up to the fourth session. For all psychophysical parameters the basal, medial and apical portion of the electrode array differed significantly across sessions. Subjects with a Nucleus Contour implant had higher comfortable levels than subjects with a Contour Advance implant. The duration of deafness, presence of preoperative tinnitus, aetiology, frequency of fitting, stimulus rate and number of active channels did not significantly influence the psychophysical levels. These results suggested that four fitting sessions within the first three weeks after switch on should initially suffice and the fifth session could be planned beyond one month.

To evaluate the speech perception benefits of bilateral implantation for subjects who already have one implant.Repeated measures.Thirty adult cochlear implant users who received their second implant from 1 to 7 years with a mean of 3 years after their first device. Ages ranged from 29 to 82 years with a mean of 57 years.Tertiary referral centers across the United Kingdom.Monosyllabic consonant-nucleus-consonant words and City University of New York sentences in quiet with coincident speech and noise and with the noise spatially separated from the speech by +/-90 degrees .At 9 months, results showed the second ear in noise was 13.9 +/- 5.9% worse than the first ear (p0.001); a significant binaural advantage of 12.6 +/- 5.4% (p0.001) over the first ear alone for speech and noise from the front; a 21 +/- 6% (p0.001) binaural advantage over the first ear alone when noise was ipsilateral to the first ear; no binaural advantage when noise was contralateral to the first ear.There is a significant bilateral advantage of adding a second ear for this group. We were unable to predict when the second ear would be the better performing ear, and by implanting both ears, we guarantee implanting the better ear. Sequential implantation with long delays between ears has resulted in poor second ear performance for some subjects and has limited the degree of bilateral benefit that can be obtained by these users. The dual microphone does not provide equivalent benefit to bilateral implants.

Objective: To examine the causes and prevalence of previous and current device nonuse among adults who have received cochlear implants.Study Design: Retrospective case review.Setting: Adult tertiary referral center for cochlear implantation.Methods: Two hundred fourteen consecutively implanted adult patients. The length of implant use ranged from I month to 14 years.Main Outcome Measure: A period of 4 consecutive weeks of nonuse of cochlear implant, including both obligatory and elective nonuse.Results: Twenty-nine adults (13.6% of implantees) were identified as having at some stage not used their device for a period of more than 4 consecutive weeks. The main reason was device failure (n = 11). Ten adults are current nonusers (4.7% of implantees). Reasons include surgical complication necessitating explantation (n = 3), comorbid illness (n = 3), elective nonuse (n 2), audiologic complication (n = 1), and device failure (n = 1).Conclusion: The overall prevalence of device nonuse was noted to increase slowly with time. The role of psychologic factors in contributing to the decision of an individual to elect to opt out of device use remains unproven.

The adult cochlear implant programme in Manchester was established in 1988, initially using funding obtained from the HEAR (Help Ear and Allied Research: charity number: 519784) charity before government resources became available in the mid-1990s. Manchester was the first centre in the UK to implant multichannel devices on a regular basis. To date, over 250 adults have been implanted, including nine bilateral and eight deaf-blind patients.(1) All the patients have a postlingual onset of severe-profound hearing loss; 73% (n = 175) of the implants performed used a Nucleus multichannel implant and 24% (n = 58) used a Medel multichannel implant. In addition, the team has implanted three Medel single channel devices, two Ineraid devices and one Clarion High Focus II device. This study is a retrospective analysis of the trends and outcomes in implant fitting during the first 14 years (1988-2002) of the programme. The paper describes the patient demographics and audiological complications for 240 implantations performed on 214 patients. Speech perception outcomes are reported for a subset of the patients. The average score for the Bench, Kowal, Bamford sentence test at the post-18-month stage of implant use is 66% and for Arthur Boothroyd words 53%. Trends in the series are analysed with respect to the change in criteria for adult implantation, the move towards bilateral implantation and the rate of uptake of cochlear implants by different ethnic groups.

Cochlear implantation is the principal treatment for restoring hearing and speech understanding in individuals with sensorineural hearing loss who gain limited benefit from hearing aids. Cochlear i...

Objectives: To compare speech perception outcomes between bilateral implantation (cochlear implants [CIs]) and bimodal rehabilitation (one CI on one side plus one hearing aid [HA] on the other side) and to explore the clinical factors that may cause asymmetric performances in speech intelligibility between the two ears in case of bilateral implantation.Design: Retrospective data from 2247 patients implanted since 2003 in 15 international centers were collected. Intelligibility scores, measured in quiet and in noise, were converted into percentile ranks to remove differences between centers. The influence of the listening mode among three independent groups, one CI alone (n = 1572), bimodal listening (CI/HA, n = 589), and bilateral CIs (CI/CI, n = 86), was compared in an analysis taking into account the influence of other factors such as duration of profound hearing loss, age, etiology, and duration of CI experience. No within-subject comparison (i.e., monitoring outcome modifications in CI/HA subjects becoming CI/CI) was possible from this dataset. Further analyses were conducted on the CI/CI subgroup to investigate a number of factors, such as implantation side, duration of hearing loss, amount of residual hearing, and use of HAs that may explain asymmetric performances of this subgroup.Results: Intelligibility ranked scores in quiet and in noise were significantly greater with both CI/CI and CI/HA than with a CI-alone group, and improvement with CI/CI (+11% and +16% in quiet and in noise, respectively) was significantly better than with CI/HA (+6% and +9% in quiet and in noise, respectively). From the CI/HA group, only subjects with ranked preoperative aided speech scores >60% performed as well as CI/CI participants. Furthermore, CI/CI subjects displayed significantly lower preoperative aided speech scores on average compared with that displayed by CI/HA subjects. Routine clinical data available from the present database did not explain the asymmetrical results of bilateral implantation.Conclusions: This retrospective study, based on basic speech audiometry (no lateralization cues), indicates that, on average, a second CI is likely to provide slightly better postoperative speech outcome than an additional HA for people with very low preoperative performance. These results may be taken into consideration to refine surgical indications for CIs.

ObjectiveTo report a case of deafness and blindness caused by ethylene glycol poisoning and treated by bilateral simultaneous cochlear implantation.Study designCase report.SettingUniversity teaching hospital, tertiary referral center.Patient37 years old man poisoned with ethylene glycol.InterventionBilateral simultaneous Medel Sonata cochlear implants.Outcome measureBKB sentences (Bamford–Kowal–Bench sentence tests); AB words (Arthur Boothroyd monosyllabic words).ResultsSuccessful rehabilitation of hearing in a case of deafness and blindness of acute onset.ConclusionBilateral CI is an effective method of hearing rehabilitation in presence of additional disability (blindness).

To determine the indications for, and auditory outcomes following, cochlear reimplantation in adults and investigate factors influencing outcome.Retrospective case series.Cochlear implant program in a tertiary care hospital.Thirty adults (32 ears) who have undergone cochlear reimplantation in the ipsilateral ear.Explantation and reimplantation of cochlear implant.Speech discrimination as measured using Bamford-Kowal Bench sentence testing in quiet (BKBq) and noisy (BKBn) environments.Best BKBq improved from 58.5% to 71.4% (p = 0.0242), and BKBn improved from 60.9% to 67.2% (p = 0.826) after reimplantation. Device failure was the most common indication for reimplantation. There was no significant difference in failure rate or outcome between implant manufacturers. The mean time to reimplantation was 4.7 years, and this was not related to auditory outcome. Otosclerosis and Ménière's disease may predispose to a worse auditory outcome after reimplantation.Cochlear reimplantation does not have a detrimental effect on auditory outcomes and in some cases results in improved speech perception.

Nine patients are presented who underwent cochlear implantation in the presence of chronic suppurative otitis media. Four had a simple tympanic membrane perforation, four had a pre-existing mastoid cavity and one had cholesteatoma in the ear chosen for implantation. Patients with a simple perforation had a staged procedure with myringoplasty followed by cochlear implantation after an interval of three months. Patients with cholesteatoma or with an unstable mastoid cavity were also staged. A mastoidectomy or revision mastoidectomy was performed with obliteration of the middle ear and mastoid using a superiorly pedicled temporalis muscle flap and blind sac closure of the external meatal skin. After a further six months a second stage procedure was performed to confirm that the middle-ear cleft was healthyand to insert the implant. Patients presenting with a stable mastoid cavity underwent obliteration of the cavity and implantation of the electrode as a one-staged procedure. To date there have been no serious problems such as graft breakdown, recurrence of disease or implant extrusion, and all patients are performing well.

A case is reported in which a Nucleus 22 channel cochlear implant was inserted into the basal turn of the cochlea of a patient with advanced otosclerosis. It then passed out of the anterior end of the basal turn into an otospongiotic cavity related to the cochlea. Seven electrodes were located in the basal turn and it was possible to map them sufficiently well for the patient to derive considerable benefit from the implant. The problem of implant induced facial nerve stimulation in otospongiosis is also discussed.

OBJECTIVE: To investigate the outcomes from ipsilateral simultaneous or sequential cochlear implantation in patients with neurofibromatosis type 2 (NF2) after vestibular schwannoma removal with cochlear nerve preservation. STUDY DESIGN: Retrospective case series. SETTING: Single tertiary referral NF2 center. PATIENTS: Six patients with NF2. INTERVENTION: Removal of vestibular schwannoma (VS) with preservation of the cochlear nerve and cochlear implantation. Four patients had their surgery via a translabyrinthine approach. Two patients had a retrosigmoid approach. A cochlear implant was inserted at the same time as tumor removal in 4 cases and sequentially in 2 cases. MAIN OUTCOME MEASURES: Surgical and audiometric outcomes using Bamford-Kowal-Bench (BKB) and City of New York University (CUNY) sentence scores. RESULTS: The average age at implantation was 24 years (range, 15-36 yr). Follow-up ranged from 5 to 93 months, with an average of 38 months. All patients had useful hearing in the contralateral ear before surgery. One patient gained no benefit from cochlear implantation and proceeded to have an auditory brainstem implant. Of those that had functional cochlear nerves, the average BKB score in quiet was 64%, BKB score in noise was 42%, and CUNY score with lipreading was 97%. Results varied within the group, but all patients gained significant benefit and continue to use their CI at least intermittantly. CONCLUSION: The present series demonstrates that in selected cases, cochlear implantation can be successful after a translabyrinthine approach for VS removal and for restoring hearing after failed retrosigmoid hearing preservation surgery. All patients found the cochlear implant offered useful hearing even in the presence of contralateral hearing.

OBJECTIVE: Hearing loss resulting from bilateral vestibular schwannomas (VSs) has a significant effect on the quality of life of patients with neurofibromatosis Type 2 (NF2). A national consensus protocol was produced in England as a guide for cochlear implantation (CI) and auditory brainstem implantation (ABI) in these patients. STUDY DESIGN: Consensus statement. SETTING: English NF2 Service. PARTICIPANTS: Clinicians from all 4 lead NF2 units in England. MAIN OUTCOME MEASURES: A protocol for the assessment, insertion and rehabilitation of CI and ABI in NF2 patients. RESULTS: Patients should undergo more detailed hearing assessment once their maximum aided speech discrimination score falls below 50% in the better hearing ear. Bamford-Kowal-Bench sentence testing scores below 50% should trigger assessment for auditory implantation, as recommended by the National Institute for Clinical Excellence guidelines on CI. Where this occurs in patients with bilateral stable VS or a unilateral stable VS where the contralateral cochlear nerve was lost at previous surgery, CI should be considered. Where VS surgery is planned, CI should be considered where cochlear nerve preservation is thought possible, otherwise an ABI should be considered. Intraoperative testing using electrically evoked auditory brainstem responses or cochlear nerve action potentials may be used to determine whether a CI or ABI is inserted. CONCLUSION: The NF2 centers in England agreed on this protocol. Multisite, prospective assessments of standardized protocols for auditory implantation in NF2 provide an essential model for evaluating candidacy and outcomes in this challenging patient population.

To investigate the outcome of cochlear implantation (CI) in patients deafened by ototoxic drugs and to compare this, with the outcome of CI in sudden sensorineural hearing loss (SSNHL) with a similar duration of deafness.The Manchester Auditory Implant Centre database was reviewed to identify patients who were implanted to rehabilitate profound sensorineural hearing loss resulting from treatment with ototoxic agents and patients with SSNHL group. A retrospective case note review of selected patients was carried out. Primary outcome measure was post-implantation Bamford-Kowal-Bench (BKB) score in quiet in both the groups. Secondary outcome measure was any significant complications following implantation.We identified 14 patients in the ototoxic group, which were matched with 13 patients in the SSNHL group. The post-operative BKB score in the ototoxic group ranged from 33 to 100% (median score 91%). One patient had bilateral CI. One patient required explantation following an infection. The post-operative BKB score in the SSNHL group ranged from 16 to 100% (median score 88%). One patient in this group could not be tested using this method as they did not have open set speech discrimination. Two patients in this group had bilateral CI. The data were analysed using Mann-Whitney U test. There was no statistically significant difference in the BKB scores in the two groups of patients (P value -0.983).Patients with profound hearing loss secondary to ototoxic agents can be rehabilitated successfully with CI. The outcomes may be variable and may be dependent on the underlying pathology for which the ototoxic agents were prescribed.

Objective: To update a 15-year-old study of 800 postlinguistically deaf adult patients showing how duration of severe to profound hearing loss, age at cochlear implantation (CI), age at onset of severe to profound hearing loss, etiology and CI experience affected CI outcome. Study Design: Retrospective multicenter study. Methods: Data from 2251 adult patients implanted since 2003 in 15 international centers were collected and speech scores in quiet were converted to percentile ranks to remove differences between centers. Results: The negative effect of long duration of severe to profound hearing loss was less important in the new data than in 1996; the effects of age at CI and age at onset of severe to profound hearing loss were delayed until older ages; etiology had a smaller effect, and the effect of CI experience was greater with a steeper learning curve. Patients with longer durations of severe to profound hearing loss were less likely to improve with CI experience than patients with shorter duration of severe to profound hearing loss. Conclusions: The factors that were relevant in 1996 were still relevant in 2011, although their relative importance had changed. Relaxed patient selection criteria, improved clinical management of hearing loss, modifications of surgical practice, and improved devices may explain the differences.

Objective: To test the influence of multiple factors on cochlear implant (CI) speech performance in quiet and in noise for postlinguistically deaf adults, and to design a model of predicted auditory performance with a CI as a function of the significant factors.Study Design: Retrospective multi-centre study.Methods: Data from 2251 patients implanted since 2003 in 15 international centres were collected. Speech scores in quiet and in noise were converted into percentile ranks to remove differences between centres. The influence of 15 pre-, per- and postoperative factors, such as the duration of moderate hearing loss (mHL), the surgical approach (cochleostomy or round window approach), the angle of insertion, the percentage of active electrodes, and the brand of device were tested. The usual factors, duration of profound HL (pHL), age, etiology, duration of CI experience, that are already known to have an influence, were included in the statistical analyses.Results: The significant factors were: the pure tone average threshold of the better ear, the brand of device, the percentage of active electrodes, the use of hearing aids (HAs) during the period of pHL, and the duration of mHL.Conclusions: A new model was designed showing a decrease of performance that started during the period of mHL, and became faster during the period of pHL. The use of bilateral HAs slowed down the related central reorganization that is the likely cause of the decreased performance. Citation: Lazard DS, Vincent C, Venail F, Van de Heyning P, Truy E, et al. (2012) Pre-, Per- and Postoperative Factors Affecting Performance of Postlinguistically Deaf Adults Using Cochlear Implants: A New Conceptual Model over Time. PLoS ONE 7(11):e48739. doi:10.1371/journal.pone.0048739

Cochlear implantation in patients with known central nervous system conditions can result in wide-ranging outcomes. The aim of this study is to report two cases of cochlear implantation outcomes in patients with acquired cerebellar ataxia following cerebellar surgery. The first is a female implanted with the Nucleus 24 implant in September 2000 and the second is a male implanted with a MED-EL Sonata Flexsoft electro-acoustic stimulation in July 2009. Programming these patients resulted in significant non-auditory stimulation which resulted in less than optimum map fittings. The patients did not gain any open set speech perception benefit although both of them gained an awareness of sound with the device. However, patient 2 elected to become a non-user because of the limited benefit.

Objectives: To analyse the speech perception performance of 53 cochlear implant recipients with otosclerosis and to evaluate which factors influenced patient performance in this group. The factors included disease-related data such as demographics, pre-operative audiological characteristics, the results of CT scanning and device-related factors. Methods: Data were reviewed on 53 patients with otosclerosis from 4 cochlear implant centres in the United Kingdom and the Netherlands. Comparison of demographics, pre-operative CT scans and audiological data revealed that the patients from the 4 different centres could be considered as one group. Speech perception scores had been obtained with the English AB monosyllable tests and Dutch NVA monosyllable tests. Based on the speech perception scores, the patients were classified as poor or good performers. The characteristics of these subgroups were compared. Results: There was wide variability in the speech perception results. Similar patterns were seen in the phoneme scores and BKB sentence scores between the poor and good performers. The two groups did not differ in age at onset of hearing loss, duration of hearing loss, progression, age at onset of deafness, or duration of deafness. Conclusions: The clinical presentation of the otosclerosis (rapid or slow progression) did not influence speech perception. Better performance was related to less severe signs of otosclerosis on CT scan, full insertion of the electrode array, little or no facial nerve stimulation and little or no need to switch off electrodes.

This study attempts to answer the question of whether there is a 'critical age' after which a second contralateral cochlear implant is less likely to provide enough speech perception to be of practical use. The study was not designed to predict factors that determine successful binaural implant use, but to see if there was evidence to help determine the latest age at which the second ear can usefully be implanted, should the first side fail and become unusable.Outcome data, in the form of speech perception test results, were collected from 11 cochlear implant programmes in the UK and one centre in Australia. Forty-seven congenitally bilaterally deaf subjects who received bilateral sequential implants were recruited to the study. The study also included four subjects with congenital unilateral profound deafness who had lost all hearing in their only hearing ear and received a cochlear implant in their unilaterally congenitally deaf ear. Of those 34 subjects for whom complete sets of data were available, the majority (72%) of those receiving their second (or unilateral) implant up to the age of 13 years scored 60 per cent or above in the Bamford Kowal Bench (BKB) sentence test, or equivalent. In contrast, of those nine receiving their second or unilateral implant at the age of 15 or above, none achieved adequate levels of speech perception on formal testing: two scored 29 per cent and 30 per cent, respectively, and the rest seven per cent or less.A discriminant function analysis performed on the data suggests that it is unlikely that a second contralateral implant received after the age of 16 to 18 years will, on its own, provide adequate levels of speech perception. As more children receive sequential bilateral cochlear implants and the pool of data enlarges the situation is likely to become clearer.The results provide support for the concept of a 'critical age' for implanting the second ear in successful congenitally deaf unilateral cochlear implant users. This would argue against 'preserving' the second ear beyond a certain age, in order to use newer models of cochlear implant or for the purpose of hair cell regeneration and similar procedures in the future. The results suggest a new and more absolute reason for bilateral implantation of congenitally deaf children at an early age.

This is a retrospective case review of all 163 adults to have received multichannel cochlear implants on the Manchester University/Manchester Royal Infirmary programme between 1988 and 1998. The aims were to investigate the incidence of unwanted non-auditory effects of electrical stimulation (NAS) of the cochlea, and to try to identify any factors that seemed to be related to these effects. The effectiveness of programming strategies in eliminating unwanted effects was also studied. Most of the devices were Nucleus CI 22M or CI 24M. The remainder were Med-el Combi 40 or Combi 40+. NAS occurred in 23.9% of implantees. There were 20 cases of facial nerve stimulation (12.3%), 18 cases of pain in the ear or throat (11.0%) and one case of vestibulospinal spinal stimulation (0.6%). Two aetiologies were significantly associated with NAS. Otosclerosis tended to be associated with facial nerve stimulation and skull base fracture was associated with pain. Pain was associated with electrodes stimulated in the base turn of the cochlea and facial nerve stimulation tended to occur with more distally situated electrodes, close to labyrinthine segment of the nerve. There was no association with one particular make of device. The T and C levels for the rogue electrodes were in the normal range. It is concluded that the unwanted effects result from shorting of current through areas of low electrical resistance in the temporal bone. A number of different strategies were employed to prevent the effect, including alteration of current levels, removal of electrodes from the map and changing the stimulation mode, and this was successfully achieved in all cases. There was no difference between the performance of patients who had had NAS and those who had not, as assessed on open-set BKB sentence scores.

Objective:To report on combined ipsilateral electrical and acoustic stimulation in a subset of conventional candidates for cochlear implantation where preoperative pure tone thresholds were ≤60 dB HL for 250 and 500 Hz. Methods: Subjects were 10 adults implanted with the Nucleus 24 Contour Advance perimodiolar electrode array. Soft surgical procedures were strictly observed: ≤1.5-mm cochleostomy hole placed anterior and inferior to the round window, a Healon bubble placed over the opening to prevent entry of foreign bodies, and no suction applied. The electrode array was inserted 17 mm to the first marker rib using the recommended ‘advance-off-stylet’ technique. Pure tone hearing threshold levels were recorded pre-, and postoperatively at 1–2 and 6–12 months. Speech recognition was tested for cochlear implant (CI) alone and combined with ipsilateral hearing aid for 7 subjects who retained significant residual hearing in the implanted ear at 1–2 months after operation. Results: There were 3/10 cases where 1–2 months after operation low-frequency responses were considered vibrotactile only (>85–110 dB HL, 250–500 Hz). In the remaining 7 cases, residual hearing was maintained up to at least 6 months after operation with minor changes. Insertion depth angles in these cases ranged from 285 to 420°. For these subjects, the mean preoperative score for words presented at 65 dB SPL was 22%. Mean postoperative scores were 56% for CI alone, and 68% for CI plus ipsilateral hearing aid (p < 0.05, paired t). For sentences presented in multitalker babble noise at 5 dB SNR, mean scores were 61% CI alone, and 75% CI+IpsiHA (p < 0.01, paired t). Conclusions: Hearing was conserved during surgery and over time in 70% of conventional candidates implanted with the Nucleus 24 Contour Advance CI who had significant levels of preoperative low-frequency residual hearing (≤60 dB HL). These conventional candidates for CI also benefited from improved speech recognition in noise when using combined ipsilateral electrical and acoustic stimulation.

The adult cochlear implant programme in Manchester was established in 1988 and the evaluation of the cochlear implant service involved the first 58 implants users (mean age = 51.65 years, range 19–75 years). Questionnaires were sent to implant users and their partners to evaluate the service with regard to provision of information, clinical care during in-patient assessments, waiting times, operation for cochlear implant and postoperative rehabilitation. The results show that the majority of patients (78 per cent) felt that the implant gave them as much or more benefit than expected. Areas identified for improvements include provision of more written information about cochlear implants; reduction in waiting times for first appointments; more information about the surgical risks and more instruction about home auditory training exercises for family and friends.As a consequence of the audit results the clinical practice and service provision for cochlear implantation in Manchester has been modified.

We present a unique case in which a multichannel cochlear implant device was explanted and the same device was re-implanted into the contralateral ear. A patient with bilateral total deafness secondary to head injury received an implant in his left ear but developed severe facial nerve stimulation. Because this stimulation could not be eliminated effectively with change of programming, it was decided to implant the contralateral ear. Since the device itself was functioning well, it was explanted from the left ear and re-implanted successfully into the right ear. Facial nerve stimulation was minimal in this ear and the patient demonstrated very good speech discrimination. To our knowledge, this is the first description of this strategy.