Anatomical and audiological considerations in branchiootorenal syndrome: A systematic review.

Objective: Establish anatomical considerations, audiological outcomes, and optimal management in patients with branchiootic/branchiootorenal syndrome (BO/BOR). Methods: Databases reviewed: Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. Clinical or radiological studies of patients with BOR syndrome describing either the audiological profile or anatomical changes were included. Articles in which BOR syndrome was associated with other syndromes, and those that were focused only on general and genetic aspects of BOR syndrome were excluded. Articles were assessed using Oxford Centre for Evidence-Based Medicine (OCEBM) grading system and the Brazzelli risk of bias tool for nonrandomized studies. Results: Searches identified 379 articles. Of these, 64 studies met the inclusion criteria, reporting outcomes in 482 patients from at least 95 families. In 308 patients, hearing loss was categorized as sensorineural (29%), conductive (20%), and mixed (51%). Hearing outcomes were variable in terms of onset, pattern, and severity; ranging from mild to profound deafness. One hundred sixty-nine patients presented with inner ear anomalies, 145 had middle, and 151 had external ear abnormalities. In 44 studies, 58 ear operations were described. Mixed outcomes were reported in patients managed with hearing aids or middle ear surgery; however, successful cochlear implantation was described in all five cases. Conclusion: The anatomical and audiological profiles of patients with BO/BOR are variable. A range of surgical procedures were described, however lacked objective outcome measures. Given the range of anatomical variants, management decisions should be made on an individual basis including full audiological and radiological assessment. Level of evidence: NA.

Mechanical dilatation of the stenosed cervix under local anesthesia: A prospective case series.

AIM: Cervical stenosis is traditionally managed by mechanical dilatation under general anesthesia (GA). We aimed to assess the safety, effectiveness, and patient acceptability of dilatation in the outpatient setting under local anesthesia (LA). METHODS: Data were collected prospectively from all patients attending the outpatient department with cervical stenosis from March 20, 2015 to September 23, 2020. Mechanical dilatation of the cervix was performed using Hegar dilators under LA. Subsequent colposcopic assessment, cytology, histology, and management were recorded. RESULTS: One hundred forty-nine cases were referred for cervical dilatation, 63 (43%) of which had complete stenosis. One hundred eighteen (79%) patients had previously undergone cervical procedures. Successful dilatation under LA was achieved in 119 (83%) patients; 5 (3%) declined (requesting GA), 6 (4%) did not tolerate speculum examination, and 19 (13%) had unsuccessful procedures. The median Hegar size used was 8 mm. Dilatation under LA was acceptable in 93% attempted procedures. Thirteen episodes of restenosis were recorded with no major adverse events. Younger age (p = 0.045) and severe (compared to complete) stenosis (p < 0.0001) were associated with procedure success, with improved results over time (p = 0.003). Successful dilatation permitted cervical assessment; eight patients required cervical excisions, two underwent hysterectomies, with one confirmed case of adenocarcinoma. CONCLUSION: Rigid cervical dilatation in the outpatient setting provides effective, instantaneous treatment for women who have failed cytological or colposcopic assessment. For the vast majority of women, the procedure was well tolerated and preferred to using GA. However, given that 1 in 10 women experienced restenosis, patients should be counseled about the possibility of requiring further management.

Hearing loss in inner ear and systemic autoimmune disease: A systematic review of post-cochlear implantation outcomes.

OBJECTIVES: To assess outcomes following cochlear implantation (CI) in patients with hearing loss secondary to primary or secondary autoimmune inner ear disease (AIED). METHODS: A systematic review and narrative synthesis was completed according to PRISMA guidelines. Databases searched included MEDLINE, PubMed, EMBASE, Web of Science, Cochrane Collection, and ClinicalTrials.gov. No limits were placed on year of publication or language. RESULTS: A total of 551 studies were identified, of which 29 were included after removal of duplicates, and screening the title, abstract, and full text. All except one study were OCEBM grade IV. 114 of 115 patients displayed improvement in hearing following cochlear implantation. With implant use, roughly a third of these patients had hearing that improved over time, a third improved and plateaued, and a third remained stable. There was no additional risk of perioperative complications found in AIED patients compared what is generally accepted in general cochlear implantation, although two episodes of device failure after 6 months were noted, and four patients with secondary AIED displayed poor initial audiological outcomes. CONCLUSION: CI in both primary and secondary AIED provides marked improvement in hearing. Early CI may be a valid management option, provide long-lasting hearing in patients and reduce the side effects of long-term systemic immunosuppressants. However, patients should be counseled residual hearing may be lost if there is cochlear ossification or fibrosis which may make implant insertion more traumatic. LEVEL OF EVIDENCE: NA.

Outcomes of Cochlear Implantation in Patients with Temporal Bone Trauma: A Systematic Review and Narrative Synthesis.

Establish outcomes following cochlear implantation (CI) in patients following temporal bone trauma. Systematic review and narrative synthesis. Medline, Pubmed, Embase, Web of Science, Cochrane Collection, and ClinicalTrials.gov. No limits are placed on language or year of publication. The review conducted in accordance with the PRISMA statement. Searches identified 223 abstracts and 64 full texts. Of these, 23 studies met the inclusion criteria reporting outcomes in 77 patients with at least 96 implants. Hearing outcomes were generally good with most patients demonstrating improved audiological and functional outcomes. Complications were reported in 14 cases with 10 of these being major. The methodological quality of included studies was modest, predominantly consisting of case reports and non-controlled case series with small numbers of patients. All studies were OCEBM grade IV. Hearing outcomes following CI in temporal bone trauma are good with useful functional improvement demonstrated in the majority of patients. It appears to be an effective method of aural rehabilitation and should be considered in selected cases following hearing loss due to temporal bone fracture.

Outcomes of Cochlear Implantation in Patients with Pendred syndrome: A Systematic Review and Narrative Synthesis.

Establish outcomes following cochlear implantation (CI) in patients with Pendred syndrome. Systematic review and narrative synthesis. Databases searched: Medline, Pubmed, Embase, Web of Science, Cochrane Collection and ClinicalTrials.gov. No limits placed on language or year of publication. Review conducted in accordance with the PRISMA statement. Searches identified 251 abstracts and 242 full texts. Of these, 22 studies met inclusion criteria reporting outcomes in 231 patients with at least 234 implants. Hearing outcomes were generally good with patients experiencing useful functional improvement. A total of 46 minor complications were reported in 78 cases. The methodological quality of included studies was modest, predominantly consisting of case reports and non-controlled case series with small numbers of patients. All studies were OCEBM grade III-IV. Hearing outcomes following CI in Pendred syndrome are generally good with useful functional improvement. However, outcomes reported in published studies lack long term follow up.

Are the doctors of the future ready to support breastfeeding? A cross-sectional study in the UK.

BACKGROUND: Currently there is no published data on the inclusion of breastfeeding education within the UK medical school curriculum. This study aims to address this knowledge gap and explore students' perceptions of their readiness to support breastfeeding. METHODS: An online survey was used to collect data from 32 UK undergraduate medical schools and their students. All students in their final two years of study at the 30 universities offering a 5- or 6-year medicine course, were eligible. RESULTS: Curriculum data was obtained from 26 (81%) institutions. Compulsory breastfeeding education was provided by 85% (N = 22) institutions with 81% (n = 21) providing lecture-based teaching and 19% (n = 5) offering formal clinical education. Overall, 411 students from 22 institutions participated. A moderate ability to identify the benefits of breastfeeding was observed; however, self-rated confidence in practical skills was poor. Assisting with latching was the least confident skill, with confidence in only 3% (14/411) students. Most students (93%) viewed doctors as playing an important role in breastfeeding, with those interested in either women's health, paediatrics or general practice perceiving the role of doctors as more important. Overall, 93% (381/411) students requested further breastfeeding education. CONCLUSIONS: This study suggests UK medical schools are not adequately preparing students to support breastfeeding patients. Further studies should explore the competency of doctors to meet the needs of lactating women, and design optimal training for UK medical students.

Formula Milk Supplementation on the Postnatal Ward: A Cross-Sectional Analytical Study.

Breastfeeding rates are low in the UK, where approximately one quarter of infants receive a breastmilk substitute (BMS) in the first week of life. We investigated the reasons for early BMS use in two large maternity units in the UK, in order to understand the reasons for the high rate of early BMS use in this setting. Data were collected through infant feeding records, as well as maternal and midwife surveys in 2016. During 2016, 28% of infants received a BMS supplement prior to discharge from the hospital maternity units with only 10% supplementation being clinically indicated. There was wide variation in BMS initiation rates between different midwives, which was associated with ward environment and midwife educational level. Specific management factors associated with non-clinically indicated initiation of BMS were the absence of skin-to-skin contact within an hour of delivery (p = 0.01), and no attendance at an antenatal breastfeeding discussion (p = 0.01). These findings suggest that risk of initiating a BMS during postnatal hospital stay is largely modifiable. Concordance with UNICEF Baby Friendly 10 steps, attention to specific features of the postnatal ward working environment, and the targeting of midwives and mothers with poor educational status may all lead to improved exclusive breastfeeding rates at hospital discharge.