The Physician's Duty to Warn Their Patients About the Risks Associated with Medical Intervention: A Review and Discussion.

Since the landmark case of Montgomery v Lanarkshire in 20151, much has been written in medical press regarding the implications for medical practice. The moral duty - varied though it has been over this time, has been discussed since the earliest days of the medical profession. The law has sought to define this duty in response to changes in society, and the nature of the relationship between doctor and patient. The moral and legal duty are intrinsically linked, but the latter must surely follow the former for "the law has little to do with morally required forms of communication in the clinic and in the research environment."2 The common law nature of this process has resulted in an inconsistent and often tortuous path as societal standards have shifted. Accordingly, the ultimate definition of the legal doctrine, "informed consent," has changed since its relatively recent entry into the medicolegal vocabulary. These parallel shifts in the legal and moral duty to disclose risk have resulted in a confusing melee of evidence and recommendations for clinicians. We address the development of the law of "informed consent," as the legal mirror of the moral duty upon a clinician to disclose risk to their patient.

Cochlear implantation in keratitis-ichthyosis-deafness syndrome: 10-year follow-up of two patients.

OBJECTIVE AND IMPORTANCE: The objective of this study was to describe long-term outcomes after cochlear implantation in keratitis-ichthyosis-deafness (KID) syndrome, often caused by GJB2 mutations, in the context of other reported cases. Clinical presentation and intervention: The authors conducted correlative clinical and molecular genetic analysis on two implanted patients with KID syndrome, and tabulated their clinical outcomes. Both children had initially successful surgery. In one case, due to skin-related problems, despite extensive salvage surgery cochlear explantation was required. This patient now communicates with sign language and lip-reading. This contrasts with the outcome for the other patient whereby at post-operative year 10 he is able to easily converse by telephone. Both patients each carry a de novo 148G > A GJB2 mutation. CONCLUSION: Patients with KID syndrome appear to be good candidates for cochlear implantation but may face significant skin-related problems which could disrupt successful post-operative habilitation. Consultation with dermatological colleagues regarding any new therapies may be warranted.

Cochlear Implantation in Brown-Vialetto-Van-Laere syndrome.

OBJECTIVE: To report outcomes for the first known cochlear implantation procedures in two patients with Brown-Vialetto-Van-Laere syndrome. PATIENTS: Two adult patients (a brother and sister) with post-lingual sensorineural deafness associated with Brown-Vialetto-Van-Laere syndrome. The female patient presented with a milder form of the syndrome. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURE: Post-implantation speech discrimination scores. RESULTS: Auditory evoked potential testing suggested pathological changes in both patients' cochleae, auditory nerves, brainstem and (probably) central auditory pathways. In the male patient, despite implantation of the better ear, the Bamford-Kowal-Bench sentence score was zero at 21 months post-implantation. In the female patient, Bamford-Kowal-Bench sentence scores at six months post-implantation were 25 per cent in quiet and 3 per cent in noise. CONCLUSION: These poor clinical outcomes appear to be related to retrocochlear and probable central auditory pathway degeneration.

Treatment of a cochlear implant biofilm infection: a potential role for alternative antimicrobial agents.

OBJECTIVE: This study aimed to investigate antimicrobial treatment of an infected cochlear implant, undertaken in an attempt to salvage the infected device. METHODS: We used the broth microdilution method to assess the susceptibility of meticillin-sensitive Staphylococcus aureus isolate, cultured from an infected cochlear implant, to common antimicrobial agents as well as to novel agents such as tea tree oil. To better simulate in vivo conditions, where bacteria grow as microcolonies encased in glycocalyx, the bactericidal activity of selected antimicrobial agents against the isolate growing in biofilm were also compared. RESULTS: When grown planktonically, the S aureus isolate was susceptible to 17 of the 18 antimicrobials tested. However, when grown in biofilm, it was resistant to all conventional antimicrobials. In contrast, 5 per cent tea tree oil completely eradicated the biofilm following exposure for 1 hour. CONCLUSION: Treatment of infected cochlear implants with novel agents such as tea tree oil could significantly improve salvage outcome.

Auditory sensory memory and working memory processes in children with normal hearing and cochlear implants.

There can be wide variation in the level of oral/aural language ability that prelingually hearing-impaired children develop after cochlear implantation. Automatic perceptual processing mechanisms have come under increasing scrutiny in attempts to explain this variation. Using mismatch negativity methods, this study explored associations between auditory sensory memory mechanisms and verbal working memory function in children with cochlear implants and a group of hearing controls of similar age. Whilst clear relationships were observed in the hearing children between mismatch activation and working memory measures, this association appeared to be disrupted in the implant children. These findings would fit with the proposal that early auditory deprivation and a degraded auditory signal can cause changes in the processes underpinning the development of oral/aural language skills in prelingually hearing-impaired children with cochlear implants and thus alter their developmental trajectory.

A review of cochlear implantation in mitochondrial sensorineural hearing loss.

OBJECTIVE: Mitochondrial sensorineural hearing loss (SNHL) may be nonsyndromic (occurring in isolation), associated with the A1555G mutation in the MTRNR1 gene. Mitochondrial SNHL may also be syndromic, associated with the A3243G point mutation in the MTTL1 gene. In syndromic cases-mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS), maternally inherited diabetes and deafness, Kearns-Sayre syndrome, and chronic progressive external ophthalmoplegia-the SNHL compounds already existing disabilities. The genetic basis for mitochondrial SNHL and postulated sites of pathologic changes are discussed. DATA SOURCES: Sources used were relevant clinical and basic science publications. STUDY SELECTION: A search of the entire databases of Medline and Web of Science, using various subject headings and free-text terms, was used to identify patients with mitochondrial disease having cochlear implants. DATA EXTRACTION: The data from publications were critically reviewed and tabulated to assess implantation outcomes. DATA SYNTHESIS: The data were not amenable to formal meta-analysis or valid data summarization, other than descriptive statistics. CONCLUSIONS: There is an increasing awareness of the prevalence of mitochondrial SNHL and its progressive nature. High-risk candidates warrant genetic testing and family screening. Correlating the data for mitochondrial SNHL as a treatable entity is important, and the authors present an overview of these patients successfully rehabilitated by cochlear implantation.

Management of otoscierosis--an Irish perspective.

The last 30 years have seen a gradual change in the management of otosclerosis. The aim of this study is to evaluate the current practice amongst Irish otolaryngology consultants by a questionnaire and to compare it with the practice currently followed in Great Britain. Thirty-eight responses (67.9%) were available for analysis. The overall trend is towards centralisation with a reduction in the number of surgeons undertaking stapes surgery (39%). The majority of consultants (67%) who undertake stapes surgery would operate for a unilateral conductive loss and 67% would undertake bilateral stapes surgery. Stapedotomy is the only operation performed (100%) with none of the consultants performing partial or total stapedectomies.

Cochlear implantation in the obliterated cochlea.

We present three cases of acquired deafness, associated with obliterated cochleas, in which the apparently radiologically more favourable side was chosen for implantation. In the first case, because of unexpected obliteration, only a partial insertion was possible. Deteriorating performance and non-auditory stimulation of the facial nerve led to removal of the implant and a contralateral implantation with full insertion under the same anaesthetic gave a good postoperative result. In the second case, CT scanning indicated minimal obliteration, but extensive obliteration was encountered at surgery, which required double-array insertion with a delayed but satisfactory outcome. In the third case, extensive unexpected obliteration was noted at surgery and, in light of the experience gained with the first two cases, it was decided not to proceed but to explore the contralateral side. At surgery on the contralateral side, a patent cochlea was noted with full electrode insertion and an excellent outcome. These cases demonstrate a learning curve for this department and our philosophy now is to explore the contralateral ear rather than accept a partial insertion.

Cochlear implantation in maternal inherited diabetes and deafness syndrome.

We describe a rare case of a gradually progressive hearing loss in a patient with maternally inherited diabetes and deafness (MIDD) syndrome successfully rehabilitated with a cochlear implant. The possible aetiology of the hearing loss in these cases is discussed.

Management of otosclerosis in the UK.

The last 30 years has seen a gradual change in the management of otosclerosis. The aim of this study is to evaluate the current practice among British otolaryngology consultants using a questionnaire, and to compare it with the practice reported in a survey 8 years ago. A total of 353 valid responses (64.5%) were available for analysis. The overall trend is towards centralization, with a reduction in the number of surgeons undertaking stapes surgery (49.9%). The majority of consultants (81.3%) who undertake stapes surgery would operate for a unilateral conductive loss and 75.1% would undertake bilateral stapes surgery. Stapedotomy is the operation of choice (82%), with a few consultants performing partial or rarely total stapedectomies. Postoperative restrictions and follow-up times vary widely amongst surgeons, with the senior surgeons tending to be more conservative than the younger consultants.

Bipolar scissors versus cold dissection for pediatric tonsillectomy--a prospective, randomized pilot study.

OBJECTIVE: To evaluate bipolar scissors tonsillectomy by comparing it with traditional cold dissection tonsillectomy. The outcome measures used were: (1) intra-operative bleeding; (2) operative time; (3) post-operative pain; and (4) complication rates including reactionary and secondary hemorrhage. METHOD: A prospective, randomized multiunit study involving three teaching hospitals in Belfast. Fifty consecutive children aged 10-16 years undergoing tonsillectomy for recurrent or chronic tonsillitis, between March 2000 and September 2000 were recruited as a subgroup of 200 patients selected for this study. These children were analysed separately from the adults, in a pilot study for the above parameters. RESULTS: The mean age of the study population was 14.3 years. Sixty-eight percent of the children were girls. Median intra-operative blood loss was 6 ml for bipolar scissors tonsillectomy and 86 ml for cold dissection tonsillectomy (P<0.001). The median operative time was 10.5 min for bipolar scissors tonsillectomy compared to 14.5 min for the cold dissection method (P=0.001). There was no statistically significant difference in the pain scores between the two methods (P>0.05). The overall reactionary hemorrhage rate was 4% while the overall secondary hemorrhage rate was 14%. The hospital readmission rate was 4%. The reactionary and secondary hemorrhage rates were unaffected by the surgical method. CONCLUSIONS: This pilot study has shown that bipolar scissors tonsillectomy is a relatively safe technique in children aged 10-16 years with a similar morbidity to the cold dissection method. Its use is associated with a significant decrease in surgical time and blood loss compared to the cold dissection method. These advantages make it a favourable instrument for pediatric tonsillectomy especially in this age group.

Bipolar scissors versus cold dissection tonsillectomy: a prospective, randomized, multi-unit study.

OBJECTIVE: To evaluate bipolar scissors tonsillectomy by comparing it with traditional cold dissection tonsillectomy. STUDY DESIGN: A prospective, randomized, multi-unit study. SETTINGS: Belfast City Hospital, Royal Victoria Hospital Belfast, and Ulster Hospital Dundonald. PATIENTS: Two hundred consecutive patients undergoing tonsillectomy for recurrent or chronic tonsillitis between March 2000 and September 2000. OUTCOME MEASURES: (1) Intraoperative bleeding, (2) operative time, (3) postoperative pain, and (4) complication rates, including primary and secondary hemorrhage. RESULTS: Seventeen patients were excluded from the study for various reasons. The mean age of the study population was 22 years (range, 10-54 y). Seventy-two percent of patients were female. Twenty-seven percent of patients were children aged 16 years or under. Median intraoperative blood loss was 5 mL for bipolar scissors tonsillectomy and 115 mL for cold dissection tonsillectomy (P < .001). The mean operative time was 13 minutes for bipolar scissors tonsillectomy compared with 20 minutes for the cold dissection method (P < .001). There was no statistically significant difference in the pain scores between the two methods (independent samples t = 1.35; P > .05). The overall primary hemorrhage rate was 2.1%, whereas the overall secondary hemorrhage rate was 16.9%. The hospital readmission rate was 10.3%. The primary and secondary hemorrhage rates were unaffected by the surgical method. CONCLUSIONS: Bipolar scissors tonsillectomy is a safe technique with a similar morbidity to the cold dissection method. Its use is associated with a significant decrease in both surgical time and blood loss compared with the cold dissection method.

Belfast Cochlear Implant Centre; the surgical results of the first 100 implants.

We assess the surgical results of the first one hundred cochlear implant operations at the Belfast Cochlear Implant Centre. This paper is in the form of a retrospective case series, taking into account the surgical technique used and any problems encountered. Initially 28 operations were performed using the standard inverted U-shaped incision. After Gibson and Harrison described a vertical postaural incision we began using a slight modification of this technique for any further implantations. Since November 1994 the vertical post aural skin incision has been used in 71 patients. The surgical complications of cochlear implantation include haematoma, wound infection and flap necrosis of varying degrees of severity. We report a low level of these complications, particularly with the vertical incision.

Cochlear implantation following temporal bone fracture.

Seven cases of profound hearing impairment following either unilateral or bilateral temporal bone fracture are presented who were implanted with the Nucleus 22 channel or Ineraid devices. Six patients suffered bilateral temporal bone fractures. One patient had prior congenital unilateral profound hearing impairment. This patient suffered a unilateral temporal bone fracture. Six patients became regular users of their implants. One gained little benefit and became a non-user. Two of the regular users experienced facial nerve stimulation, which could not be programmed out. In these two cases the implant was removed and the contralateral ear successfully implanted. Implant-aided audiometry demonstrated a hearing threshold of 40-50 dB at nine months after switch-on. The reliability of computed tomography (CT) scanning in predicting cochlear patency in cases of temporal bone fracture will be discussed. The benefit of complimentary imaging with magnetic resonance (MR) is highlighted.

Cochlear implantations in Northern Ireland: an overview of the first five years.

During the last few years cochlear implantation (CI) has made remarkable progress, developing from a mere research tool to a viable clinical application. The Centre for CI in the Northern Ireland was established in 1992 and has since been a provider of this new technology for rehabilitation of profoundly deaf patients in the region. Although individual performance with a cochlear implant cannot be predicted accurately, the overall success of CI can no longer be denied. Seventy one patients, 37 adults and 34 children, have received implants over the first five years of the Northern Ireland cochlear implant programme, which is located at the Belfast City Hospital. The complication rates and the post-implantation outcome of this centre compare favourably with other major centres which undertake the procedure. This paper aims to highlight the patient selection criteria, surgery, post-CI outcome, clinical and research developments within our centre, and future prospects of this recent modality of treatment.

Cochlear implantation under local anaesthesia, the Belfast experience.

The profoundly deaf, who gain little or no benefit from conventional hearing aids and meet various criteria are potential candidates for cochlear implantation. The last two decades have witnessed remarkable progress in this field, and it is now a routine clinical procedure. A few adult patients who are potential candidates for cochlear implantation have an unacceptably high risk for hypotensive general anaesthesia due to other systemic conditions. This group has been successfully implanted under local anaesthesia in our centre. The post-implantation progress of these patients was comparable to those carried out under hypotensive general anaesthesia. Data regarding patient selection criteria, examination, anaesthesia, surgery and the outcome are discussed. It was concluded that cochlear implantation under local anaesthesia is a safe and effective procedure for those patients who otherwise may be denied an implant.

Digital imaging: a valuable technique for the postoperative assessment of cochlear implantation.

Cochlear implantation with a multi-channel electrode array which provides stimulation via the auditory nerve has become a standard treatment for profound deafness. Postoperative radiography demonstrates electrode position and enables confirmation of satisfactory intra-cochlear electrode placement. The number of active electrodes which have been inserted can be determined and possible complications such as electrode kinking or slippage can be assessed. We evaluated digital radiography with confirmation of electrode position by intermittent fluoroscopy and assessed the relative radiation dose of the digital technique, conventional radiography and CT scanning. Radiation dose for this method usually ranges between 40 microGy and 440 microGy compared with a single exposure on the skull stand which produces a dose to the region of the cochlea of 470 microGy and a CT exposure of 950 microGy. The digital technique is comfortable for the patient, easily reproducible and provides images of high diagnostic quality enabling each electrode to be identified, which is especially valuable in association with postoperative electrode mapping. It also involves a lower radiation dose than conventional radiography. We now use digital radiography for all postoperative cochlear implant assessment.

A new approach to surgery for Menière's disease: talking about surgery.

Many patients experience a remission of their Menière's disease between the time that surgery is agreed and their admission to hospital for the procedure. In a series of 23 consecutive patients where surgery appeared to be indicated for control of the vertigo of Menière's disease, 12 settled within 6-8 weeks of agreeing that something would be done, and thereby avoided any surgical procedure. It is suggested that these patients may have been experiencing the same effect as provided by the conservative surgical procedures. In the absence of any clear evidence for a specific surgical effect from any conservative operation for Menière's disease, the authors put forward the suggestion that abandoning all the conservative procedures in favour of the protocol of planned surgery should be considered; wait for 2 months and, if the incapacitating episodic dizziness continues, one should proceed to a differenting procedure.