Bone-anchored hearing aids and unilateral sensorineural hearing loss: why do patients reject them?

OBJECTIVES: This study aimed to report the bone-anchored hearing aid uptake and the reasons for their rejection by unilateral sensorineural deafness patients. METHODS: A retrospective review of 90 consecutive unilateral sensorineural deafness patients referred to the Greater Manchester Bone-Anchored Hearing Aid Programme between September 2008 and August 2011 was performed. RESULTS: In all, 79 (87.8 per cent) were deemed audiologically suitable: 24 (30.3 per cent) eventually had a bone-anchored hearing aid implanted and 55 (69.6 per cent) patients declined. Of those who declined, 26 (47.3 per cent) cited perceived limited benefits, 18 (32.7 per cent) cited reservations regarding surgery, 13 (23.6 per cent) preferred a wireless contralateral routing of sound device and 12 (21.8 per cent) cited cosmetic reasons. In all, 32 (40.5 per cent) suitable patients eventually chose the wireless contralateral routing of sound device. CONCLUSION: The uptake rate was 30 per cent for audiologically suitable patients. Almost half of suitable patients did not perceive a sufficient benefit to proceed to device implantation and a significant proportion rejected it. It is therefore important that clinicians do not to rush to implant all unilateral sensorineural hearing loss patients with a bone-anchored hearing aid.

Bone-anchored hearing aids and chronic pain: a long-term complication and a cause for elective implant removal.

OBJECTIVES: To report a case series of elective removal of bone-anchored hearing aid implants, and reasons for removal. DESIGN: Retrospective review of a prospectively collected database. SETTING: Two tertiary referral centres in the Manchester area: Manchester Royal Infirmary and Salford Royal University Hospital. PARTICIPANTS: A series of 499 adults and children who had undergone a total of 602 implant insertions (1984-2008). MAIN OUTCOME MEASURES: Implant removal rates, and reasons. RESULTS: Twenty-seven of the 602 implants (4.5 per cent) required removal. Of these, 12 were due to pain (2.0 per cent), seven to persistent infection (1.2 per cent), three to failure of osseointegration (0.5 per cent), three to trauma (0.5 per cent) and two to other reasons (0.4 per cent). CONCLUSION: Chronic implant site pain represents the main reason why implants are removed electively, and affects 2 per cent of all implants. This complication has important medico-legal implications and should be discussed when obtaining informed consent for implantation.

Cochlear implantation in a child with CINCA syndrome who also has wide vestibular aqueducts.

Chronic infantile neurological cutaneous and articular (CINCA) syndrome is a severe auto-inflammatory disease, due to mutation of the CIAS1 gene. CINCA syndrome should be considered the most severe of a spectrum of three disorders all due to mutation of the CIAS1 gene. CINCA syndrome produces a triad of symptoms of neonatal onset: maculopapular urticarial rash, chronic meningitis, and chronic non-inflammatory arthropathy with recurrent fever. CINCA syndrome is also associated with sensory organ damage, especially progressive hearing loss and loss of vision. In this case report, we present the first case of cochlear implantation in a 13-year-old child with CINCA syndrome. Cochlear implantation was successful at rehabilitating the hearing loss with the child able to continue mainstream education, with her academic performance and speech discrimination both showing marked improvement. Anakinra (an interleukin 1 receptor antagonist) is now in widespread use to treat CINCA syndrome and is known to rapidly reverse the inflammatory features of CINCA syndrome. However, current evidence suggests that anakinra has limited effectiveness in reversing the sensorineural hearing loss seen in CINCA syndrome. We therefore propose that cochlear implantation is a viable treatment option in this rare yet severe auto-inflammatory disease, if the patient has failed to respond to anakinra. Owing to the unknown pathogenesis of the progressive hearing loss seen in CINCA syndrome and the limited effectiveness of anakinra in reversing the progressive hearing loss, we suggest that cochlear implantation is the modality of choice in rehabilitating severe-to-profound hearing loss not responsive to anakinra.

Predictors of audiological outcome following cochlear implantation in adults.

The objective of this study was to examine variables that may predict open set speech discrimination following cochlear implantation. It consisted of a retrospective case review conducted in a tertiary referral centre with a cochlear implant programme. The patients were 117 postlingually deafened adult cochlear implant recipients. The main outcome measures were Bench, Kowal, Bamford (BKB) sentence scores recorded nine months following implant activation. The variables studied were age at the time of surgery, sex, duration of hearing loss, aetiology of hearing loss, residual hearing, implant type, speech processor strategy, number of active electrodes inserted. Variables found to have a significant effect on BKB following univariate analysis were entered into a multivariate analysis to determine independent predictors. Multivariate ordinal regression analysis gave an odds ration of 1.09 for each additional year of deafness prior to implantation (confidence interval 1.06-1.13; p < 0.001). Duration of deafness prior to implantation is an independent predictor of implant outcome. It accounted for 9% of the variability. Other factors must influence implant performance.

Cerebrospinal fluid leak rate after the use of BioGlue in translabyrinthine vestibular schwannoma surgery: a prospective study.

OBJECTIVE: To determine the effectiveness of BioGlue surgical adhesive in dural and middle ear closure after translabyrinthine vestibular schwannoma surgery. STUDY DESIGN: A prospective study. SETTING: Tertiary neurotological referral center. PATIENTS: There were 24 patients in the BioGlue series. BioGlue was used in the same manner in all cases. All patients received similar postoperative care. INTERVENTIONS: We studied the use of BioGlue and its possible effect on further reducing our department's cerebrospinal fluid leak rate for translabyrinthine vestibular schwannoma surgery. MAIN OUTCOME MEASURES: Postoperative events were documented that enabled us to determine the overall cerebrospinal fluid leak rate (including incidence of various leak routes and morbidity). RESULTS: The overall cerebrospinal fluid leak rate was 62.5% (15 of 24). Rhinorrhoea was the commonest route (80%), followed by postaural wound leak (33.3%) and external auditory canal otorrhoea (33.3%). Forty percent of cases had more than one cerebrospinal fluid leak route; 73.3% of leak cases required lumbar drain insertion, 40% needed pressure bandaging, and 66.7% had to undergo formal surgical repair. Forty percent had recurrent leaks after the initial episode had completely ceased. The mean extra stay in hospital as a result of the cerebrospinal fluid leak was 13.3 days. CONCLUSION: Our preliminary prospective study of the use of BioGlue for dural and middle ear closure in translabyrinthine vestibular schwannoma surgery demonstrated poor results. The high cerebrospinal fluid leak rate associated with the unusual presentations and ensuing management difficulties in controlling these leaks lead us to recommend that BioGlue not be used in translabyrinthine vestibular schwannoma surgery. The manufacturers have noted our results and have considered adding our recommendation to the product data sheet.

Epilepsy following middle-fossa extradural retraction: implications for driving.

New-onset epilepsy has several social and financial repercussions. Development of seizures after middle-fossa surgery is uncommon. We present two subjects who developed epilepsy following middle-fossa surgery, requiring treatment with anticonvulsants, and discuss the implications.

Device nonuse among adult cochlear implant recipients.

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 1 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.

Complications following adult cochlear implantation: experience in Manchester.

Cochlear implantation is regarded as a safe and effective treatment for the profoundly deaf. However, a proportion of patients suffer complications after implant surgery. This paper examines the complications encountered in 240 adult cochlear implant operations performed in Manchester between June 1988 and June 2002. Minor complications were defined as those that either settled spontaneously or with conservative management. The total number of minor complications was 61 (25.4 per cent of cases). Non-auditory stimulation, which resolved with implant reprogramming, was present in 53 cases (22.1 per cent). Major complications were defined as those requiring further surgery, explantation or causing a significant medical problem, and occurred in 15 patients (6.25 per cent). These included implant extrusion, implant sepsis, electrode migration, flap-related problems, and persistent non-auditory stimulation. Nine of the 15 patients suffering a major complication required explantation. There were no post-operative deaths, cases of meningitis, nor persistent facial palsies in the series.