Cochlear Implantation in Children with Enlarged Vestibular Aqueduct: A Systematic Review of Surgical Implications and Outcomes.

OBJECTIVES: This study investigated age at implantation, improvement in hearing and speech perception outcomes, as well as surgical complications in pediatric cochlear implant recipients with Pendred Syndrome (PS) or non-syndromic enlarged vestibular aqueduct (NSEVA). DESIGN: A systematic review of the literature between 1984 and 2021 was performed. Two independent reviewers performed abstract and full-text screening using the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. The inclusion criteria were: English language, cochlear implant, age at implantation available, age <18 years, PS, Mondini malformation, and enlarged vestibular aqueduct. Full-text analysis was completed using the National Institute of Health assessment tool for case series and case-control studies. Studies were also graded according to the Oxford Centre for Evidence-Based Medicine grading system. RESULTS: In total, 198 studies were identified and screened, and 55 studies were included for analysis. Audiological outcomes were available in 46 studies, and the four-frequency pure-tone audiogram average improved by 60 to 78 dB HL due to cochlear implantation. Auditory performance and speech intelligibility scores increased by 44%. The overall average implantation age was 60 months. The implantation age was 21 months lower in the studies where individuals were implanted after the year 2000 compared with those implanted before the year 2000. Perilymph gusher/oozing was the most common surgical incident reported, occurring in 187 of 1572 implantations. CONCLUSIONS: In children with PS/NSEVA, cochlear implantation improves pure-tone average by 60 to 78 dB HL and capacity of auditory performance/speech intelligibility by 44%. The implantation age for these children has decreased during the last two decades but is still somewhat higher than reported for unselected pediatric cochlear implantation. Perilymph gusher/oozing is the most common surgical complication.

Life-threatening angio-oedema after the first dose of an ACE inhibitor-not an anaphylactic reaction.

We present a case of a 60-year-old Caucasian woman, with no prior history of swellings, who was admitted to a hospital due to life-threatening angio-oedema. She had, the previous day, been prescribed an ACE inhibitor for her essential hypertension. She had taken one tablet at night-time, and awoke in the morning with a swollen face progressing to involve the tongue and throat within a few hours. On arrival at her doctor's office, her voice had altered. Corticosteroids and antihistamine were administered while awaiting an ambulance. Arriving at the emergency department, she had dyspnoea due to increasingly severe angio-oedema of the upper airways. Neither adrenaline inhalations, intravenously administrated corticosteroids, atropine nor furosemide were effective and the patient soon become bradycardic. A tracheotomy was performed and the patient was placed on a ventilator. She eventually made a full recovery. It was concluded that she had suffered from life-threatening angio-oedema due to her new medication.

Angioedema--assessment and treatment.

BACKGROUND: Angioedema has numerous hereditary, acquired and iatrogenic causes. A number of studies show that angioedema is inadequately assessed and treated during its acute phase as well as in the follow-up period. We present an algorithm for the assessment and treatment of patients with angioedema. KNOWLEDGE BASE: The article is based on a literature search in PubMed, a review of bibliographies and the authors' clinical experience and research. RESULTS: The majority of angioedema patients have accompanying urticaria. Pathophysiologically, angioedemas are divided into histaminergic and non-histaminergic forms. In a large group of patients no positive trigger is identified. On assessment in hospital the most frequently identified cause is drug intake, normally angiotensin-converting-enzyme inhibitors and NSAIDs , while allergic/pseudoallergic and idiopathic reactions are more commonly seen in general practice. There are a number of rare causes of angioedema, all of which are important to keep in mind. The acute and prophylactic treatment will depend on the subtype of angioedema and is best provided through cross-disciplinary collaboration. INTERPRETATION: Angioedema is a potentially life-threatening condition and should be assessed and treated systematically. It is important to remember that angioedema is either histaminergic or non-histaminergic, as the treatment of the two types is different.