Simon K W Lloyd

Central Manchester University Hospitals NHS Foundation Trust, Manchester, England, United Kingdom

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Publications (36)43.5 Total impact

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    ABSTRACT: Neurofibromatosis type 2 (NF2) is an autosomal dominant condition with high spontaneous mutation rate which predisposes to the development of multiple nerve sheath tumours (schwannomas), meningiomas and ependymoma. The cardinal feature and main diagnostic criterion for the diagnosis of NF2 remains the development of bilateral vestibular schwannoma (BVS). With increasing use of MRI screening the possibility of a 'chance' diagnosis of BVS has been mooted with a potential frequency of one in two million people in their lifetime. Until now, however, no evidence for such an event has been published. We aimed to demonstrate that chance occurrence can occur and to estimate its frequency among those with just BVS late in life. Two vestibular schwannomas from the same patient were DNA sequenced and underwent loss of heterozygosity analysis. We show that a man who developed BVS, at ages 52 and 67 years developed these tumours sporadically by demonstrating that there were no molecular events in common between the two tumours. Furthermore from a database of over 1200 patients with NF2, we have estimated that ∼25% of cases of BVS over 50 years and 50% over 70 years of age where no other features of NF2 are present represent a chance occurrence rather than due to an underlying mosaic or constitutional NF2 mutation. Patients presenting with BVS later in life should be appraised of the potential likelihood they may not have NF2 and the resultant further reduction in risks of transmission to offspring. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.
    Journal of medical genetics. 02/2015;
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    ABSTRACT: Balance disequilibrium is a significant contributor to falls in the elderly. The most common cause of balance dysfunction is loss of sensory cells from the vestibular sensory epithelia of the inner ear. However, inaccessibility of inner ear tissue in humans severely restricts possibilities for experimental manipulation in order to develop therapies to ameliorate this loss. Here we provide a structural and functional analysis of human vestibular sensory epithelia harvested at trans-labyrinthine surgery. We demonstrate the viability of the tissue and labelling with specific markers of hair cell function and of ion homeostasis in the epithelium. Samples obtained from the oldest patients revealed a significant loss of hair cells across the tissue surface, but we found immature hair bundles present in epithelia harvested from patients over 60 years of age. These results suggest that the environment of the human vestibular sensory epithelium could be responsive to stimulation of developmental pathways in order to enhance hair cell regeneration, as has been demonstrated successfully in the vestibular organs of adult mice.
    Neurobiology of Aging. 02/2015;
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    ABSTRACT: 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. 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). 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. 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.
    Ontology & Neurotology 12/2014; · 1.60 Impact Factor
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    ABSTRACT: To determine differences in speech perception outcomes for patients who received a CI422 and a Contour cochlear implant. Retrospective case review. Tertiary referral center. Thirty-two adults who underwent cochlear implantation. Cochlear implantation using a CI422 or Contour device. Bamford-Kowal-Bench (BKB) speech perception scores at 3 and 9 months after activation. 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. 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.
    Cochlear implants international. 11/2014; 16 Suppl 1.
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    ABSTRACT: 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.
    European archives of oto-rhino-laryngology. Supplement 10/2014;
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    ABSTRACT: Objective: To present a review of all patients diagnosed with a facial nerve schwannoma (FNS) managed in our center over almost two decades, and suggest guidelines for their classification and management. Study Design: Retrospective case review Setting: Tertiary referral center Patients: Twenty-eight patients with a facial nerve schwannoma Intervention: Conservative or surgical management depending on clinical and radiological features Main Outcome Measure: Patient demographics, site of tumor, and clinical symptoms, including facial nerve function (House-Brackmann score) at baseline and follow-up. In those managed surgically, operative approach and surgical outcomes were also recorded. Results: Of 28 patients, 16 were male. Mean age at presentation was 46 years. The majority presented with either facial weakness or hearing loss. The internal auditory canal segment of the facial nerve was the most commonly affected (19/28, 68%). Multi-segmental lesions were found in almost half (46%) of patients. Facial weakness was most commonly associated with involvement of the labyrinthine segment (89%). Overall, 16 (57%) patients were managed surgically. Conclusion: FNS may be difficult to distinguish on both clinical and imaging grounds from other cerebellopontine pathologies on the basis of audiovestibular symptoms alone. The presence of facial weakness in combination with imaging findings suggestive of FNS is highly suggestive for FNS. In patients with brainstem compression, rapid tumor growth, or House-Brackmann greater than 4, we suggest a surgical approach based on preoperative audiovestibular status, helping optimize long-term facial function and minimize morbidity. Facial nerve reanimation at the time of primary surgery is preferred. Copyright (C) 2015 by Otology & Neurotology, Inc. Image copyright (C) 2010 Wolters Kluwer Health/Anatomical Chart Company
    Ontology & Neurotology 10/2014; · 1.60 Impact Factor
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    ABSTRACT: This study describes our experience of cochlear implantation (CI) with hearing preservation in adolescents. Our aim was to determine if hearing preservation is successful in this population, if the preserved hearing is maintained, and what the potential benefit of preserving hearing in this population is.
    Ontology & Neurotology 08/2014; 35(9). · 1.60 Impact Factor
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    ABSTRACT: DesignTwo round Delphi survey.SettingEmail questionnaire.ParticipantsStakeholders with a vested interest in ENT undergraduate education.Main Outcomes MeasuredMode and median scores for 232 learning outcomes.ResultsThe individual learning objectives that scored most highly were related to history taking and examination, red flag symptoms, common ENT conditions including all forms of otitis, acute and chronic rhinosinusitis, thyroid disease, pharyngeal infection and airway compromise and formulation of differential diagnoses.Conclusions Using a Delphi technique, a structured, evidence based curriculum has been developed. This should assist those medical schools who do not currently have ENT in their curriculum but wish to re-instate it to produce a high quality teaching programme. It may also assist those medical schools who do have ENT in their curriculum to continue to develop their curriculum.This article is protected by copyright. All rights reserved.
    Clinical otolaryngology: official journal of ENT-UK; official journal of Netherlands Society for Oto-Rhino-Laryngology & Cervico-Facial Surgery 08/2014; · 2.27 Impact Factor
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    ABSTRACT: To review the postoperative surgical outcomes of cystic vestibular schwannomas (CVSs), especially facial nerve outcomes, and compare these results with those from matched solid vestibular schwannomas (SVS) resected during the same period at a tertiary referral center.
    Ontology & Neurotology 05/2014; · 1.60 Impact Factor
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    ABSTRACT: Objective: To systematically summarise the peer-reviewed literature relating to the aetiology, clinical presentation, investigation and treatment of geniculate neuralgia. Data sources: Articles published in English between 1932 and 2012, identified using Medline, Embase and Cochrane databases. Methods: The search terms 'geniculate neuralgia', 'nervus intermedius neuralgia', 'facial pain', 'otalgia' and 'neuralgia' were used to identify relevant papers. Results: Fewer than 150 reported cases were published in English between 1932 and 2012. The aetiology of the condition remains unknown, and clinical presentation varies. Non-neuralgic causes of otalgia should always be excluded by a thorough clinical examination, audiological assessment and radiological investigations before making a diagnosis of geniculate neuralgia. Conservative medical treatment is always the first-line therapy. Surgical treatment should be offered if medical treatment fails. The two commonest surgical options are transection of the nervus intermedius, and microvascular decompression of the nerve at the nerve root entry zone of the brainstem. However, extracranial intratemporal division of the cutaneous branches of the facial nerve may offer a safer and similarly effective treatment. Conclusion: The response to medical treatment for this condition varies between individuals. The long-term outcomes of surgery remain unknown because of limited data.
    The Journal of Laryngology & Otology 05/2014; · 0.70 Impact Factor
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    ABSTRACT: Aim To present the outcomes of cochlear implantation (CI) in children with auditory neuropathy spectrum disorders (ANSD). Materials and methods The pre- and post-CI hearing outcomes in children with ANSD were retrospectively evaluated. Performance was assessed with categories of auditory performance (CAP) and the Manchester spoken language development scale (MSLDS). Results Full data were available in 27 implanted children with ANSD with average age at implantation 35.4 months (range 19-68 months). Nine children were implanted bilaterally, while 13 were bimodal. The pre-CI CAP and MSLDS scores were 2.5 (range 0-5) and 2.5 (range 0-6), while the post-CI scores 5.8 (range 2-9) and 7.7 (range 3-10), respectively. Conclusions Although the outcome of CI in children with ANSD might vary, it is favourable in most of the cases. CI seems a justified hearing rehabilitation option for children with ANSD and limited benefits from conventional hearing aids.
    Cochlear Implants International 05/2014; 15 Suppl 1:S51-4.
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    ABSTRACT: To date, only a very limited number of lipomas of the cerebellopontine angle (CPA) have been reported. Our objective was to examine clinical and radiologic features of CPA lipomas and determine the most appropriate management plan. Retrospective case series. Tertiary referral center. Patients with CPA lipomas were identified through the skull base multidisciplinary meeting database. Radiologic surveillance and clinical assessment. Tumor growth, assessed through radiologic measurements on serial magnetic resonance imaging, demographics, presenting symptoms, and any correlation between weight gain and lipoma growth were among the examined factors. Of the 15 patients with CPA lipomas, six were female and nine were male, with an average age at presentation of 50.2 years (range, 31.7-76.4 yr) and an average follow-up time of 51.7 months (range, 6-216 mo). The lipomas were unilateral in all cases, nine on the right (60%) and six on the left (40%) side. None of the lipomas increased in size. All patients were treated conservatively. Sensorineural hearing loss was the main presenting symptom (80%) followed by tinnitus (46.7%) and vertigo (20%). None of the patients suffered from facial nerve dysfunction. There was no correlation between weight gain and tumor growth. CPA lipomas can be diagnosed accurately with appropriate magnetic resonance imaging techniques and be managed conservatively with safety. Cochleovestibular are the most common presenting symptoms, whereas facial nerve involvement is rare. CPA lipomas do not tend to grow and can be monitored on a less regular basis.
    Otology & neurotology: official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 03/2014; · 1.44 Impact Factor
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    ABSTRACT: 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.
    Otology & neurotology: official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 03/2014; · 1.44 Impact Factor
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    ABSTRACT: 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. Retrospective case series. Single tertiary referral NF2 center. Six patients with NF2. 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. Surgical and audiometric outcomes using Bamford-Kowal-Bench (BKB) and City of New York University (CUNY) sentence scores. 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. 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.
    Otology & neurotology: official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 01/2014; 35(1):43-51. · 1.44 Impact Factor
  • Simon Lloyd, Deborah Mawman, Chris Raine
    Cochlear implants international 11/2013; 14 Suppl 4:1-2.
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    ABSTRACT: The assessment process is critical in deciding whether a profoundly deaf child with cochlear nerve deficiency (CND) will be suitable for a cochlear or auditory brainstem implant (ABI). Magnetic resonance imaging (MRI) using submillimetric T2 weighted gradient echo or turbo spin echo sequences is mandatory for all profoundly deaf children to diagnose CND. Evidence of audition on behavioural or electrophysiological tests following both auditory and electrical stimulation sometimes allows identification of significant auditory tissue not visible on MRI. In particular electric auditory brainstem response (EABR) testing may allow some quantification of auditory tissue and help decide whether a cochlear implant will be beneficial. Age and cognitive development are the most critical factors in determining ABI benefit. Hearing outcomes from both cochlear implants and ABIs are variable and likely to be limited in children with CND. A proportion of children will get no benefit. Usually the implants would be expected to provide recognition of environmental sounds and understanding of simple phonetics. Most children will not develop normal speech and they will often need to learn to communicate with sign language. The ABI involves a major neurosurgical procedure and at present the long term outcomes are unknown. It is therefore essential that parents who are considering this intervention have plenty of time to consider all aspects and the opportunity for in depth discussion.
    Cochlear implants international 11/2013; 14 Suppl 4:27-31.
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    ABSTRACT: 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. Consensus statement. English NF2 Service. Clinicians from all 4 lead NF2 units in England. 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. 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.
    Otology & neurotology: official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 10/2013; · 1.44 Impact Factor
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    ABSTRACT: Objectives. To analyse the long-term outcome of translabyrinthine surgery for vestibular schwannoma (VS) in neurofibromatosis type 2 (NF2). Research type. Retrospective cohort study. Setting. Two tertiary referral NF2 units. Patients. One hundred and forty eight translabyrinthine operations for patients with VS were performed. Preoperative stereotactic radiotherapy had been performed on 12(9.4%) patients. Results. Mean tumour size was 3.1 cm. Total tumour excision was achieved in 66% of cases, capsular remnants were left in 24% of cases, and subtotal excision was achieved in 5% and partial removal was achieved in 5%. The radiological residual/recurrence rate was 13.9%. The perioperative mortality was 1.6%. At 2 years postoperatively, facial function was expressed in terms of House-Brackmann score (HB): HB 1 in 53.4%, HB 1/2 in 61.3%, HB 1-3 in 83.2% and HB 4-6 in 16.8%. All nine patients who underwent surgery following failed stereotactic radiotherapy had HB 3 function or better. Among 9.5% of the cases, 14 facial nerves were lost during surgery and repaired using direct anastomosis or grafting. There was no tinnitus present preoperatively in 27% of the cases, and 22% of patients developed tinnitus postoperatively. In patients with preoperative tinnitus, 61% remained the same, 17% got it resolved and only in 21% it worsened. The preoperative hydrocephalus rate was 26%, and among 15% of the cases five ventriculo-peritoneal (VP) shunts were performed. The cerebrospinal fluid leak rate was 2.5%. Fifty-six patients underwent auditory brainstem implantation (ABI) and two patients had cochlear implant (CI) sleepers inserted. Conclusions. The management of patients with NF2 presents the clinician with a formidable challenge with many patients still presenting themselves late with the neurological compromise and a large tumour load. There is still an argument for the management by observation until the neurological compromise dictates interventional treatment particularly with the option of hearing rehabilitation with ABI or CI. The translabyrinthine approach provides a very satisfactory means of reducing the overall tumour volume.
    British Journal of Neurosurgery 03/2013; · 0.86 Impact Factor
  • Simon K W Lloyd, D Gareth R Evans
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    ABSTRACT: Neurofibromatosis type 2 (NF2) is an autosomal dominant inherited tumor predisposition syndrome caused by mutations in the NF2 gene on chromosome 22. Affected individuals develop schwannomas typically involving both vestibular nerves leading to hearing loss and eventual deafness. Rehabilitation with brainstem implants and in some cases cochlear implants is improving this outcome. Schwannomas also occur on other cranial nerves, on spinal nerve roots and peripheral nerves, and intracutaneously as plaques. Cranial and spinal meningiomas and spinal ependymomas are other common tumors. Fifty to sixty percent of patients represent de novo mutations and as many as 33% of these are mosaic for the underlying disease causing mutation. Truncating mutations (nonsense, frameshift insertions/deletions) are the most frequent germline events and cause the most severe disease, whilst single and multiple exon deletions are common and are usually associated with milder NF2. Neurological deficits are a major feature of the condition and neurologists have a pivotal role in assigning symptoms to lesions and in managing neuropathies. NF2 represents a difficult management problem with most patients facing substantial morbidity and reduced life expectancy. Surgery remains the focus of current management although watchful waiting and occasionally radiation treatment have a role. We are seeing the advent of tailored drug therapies aimed at the genetic level and these are likely to provide huge improvements for this devastating, life-limiting condition.
    Handbook of Clinical Neurology 01/2013; 115:957-67.
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    ABSTRACT: 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. Retrospective case review. The Manchester Cochlear Implant Programme, University of Manchester. Profoundly deafened patients after head injury who proceeded to cochlear implantation for auditory rehabilitation. 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. 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). 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.
    Otology & neurotology: official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 08/2012; 33(8):1328-32. · 1.44 Impact Factor

Publication Stats

82 Citations
43.50 Total Impact Points

Institutions

  • 2010–2013
    • Central Manchester University Hospitals NHS Foundation Trust
      Manchester, England, United Kingdom
  • 2012
    • The University of Manchester
      Manchester, England, United Kingdom
  • 2009–2012
    • Cambridge University Hospitals NHS Foundation Trust
      Cambridge, England, United Kingdom