Williams syndrome is a rare genetic disease that affects elastin production, leading to medium and large vessel stenoses and other abnormalities. Cardiac manifestations of Williams syndrome are the most life-threatening, occurring in 80% of children. Children with Williams syndrome are known to be at risk for sudden cardiac death. These tragic events are often precipitated by diagnostic or therapeutic procedures requiring anaesthesia or sedation, such as cardiac catheterisation. We present the case of a 3-month-old infant with Williams syndrome who suffered sudden cardiac arrest during cardiac catheterisation and subsequent arrest approximately 48 hours after the procedure. We also review the current literature focused on children with Williams syndrome who have suffered sudden cardiac arrest during or after cardiac catheterisation procedures.

Objectives: Some experts have promoted preparticipative cardiovascular screening programs for young athletes and have claimed that such programs were cost-effective without performing a critical analysis of studies supporting this statement. In this systematic review, a critical assessment of economic evaluations on these programs is performed to determine if they really provide value for money.

Methods: A systematic review of economic evaluations was performed on December 24, 2014. Web sites of health technology assessment agencies, the Cochrane database of systematic review, the National Health Service Economic Evaluation Database of the Cochrane Library, EMBASE, Medline, Psychinfo, and EconLit were searched to retrieve (reviews of) economic evaluations. No language or time restrictions were imposed and predefined selection criteria were used. Selected studies were critically assessed applying a structured data extraction sheet.

Results: Five relevant economic evaluations were critically assessed. Results of these studies were mixed. However, those in favor of screening made (methodological) incorrect choices, of which the most important one was not taking into account a no-screening alternative as comparator. Compared with no screening, other strategies (history and physical examination or history and physical examination plus electrocardiogram) were not considered cost-effective.

Conclusions: Results of primary economic evaluations should not be blindly copied without critical assessment. Economic evaluations in this field lack the support of robust evidence. Negative consequences of screening (false positive findings, overtreatment) should also be taken into account and may cause more harm than good. A mass screening of young athletes for cardiovascular diseases does not provide value for money and should be discouraged.

Objectives: Semiautomated external defibrillators (AEDs) should be considered as a means to facilitate in-hospital early defibrillation (IHED) in areas where advanced life support rescuers are not readily available. In this study, we aimed to develop a checklist and a measurement protocol to evaluate and compare AEDs by assessing factors that may affect IHED.

Methods: A clinical and technical comparison of six AEDs was performed. Technical specifications were analyzed, while an emergency team evaluated ergonomics and appropriateness for IHED at Bambino Gesù Children's Hospital. A measurement protocol was implemented, which aimed to assess the ability of defibrillators to recognize shockable and nonshockable rhythms, accuracy of delivered energy, and charging time.

Results: Designs of AEDs differed in several features which influence their appropriateness for IHED. Some units showed poor ergonomics and instructions/feedback for cardiopulmonary resuscitation. Differences between defibrillators in recognizing shockable and nonshockable rhythms emerged for polymorphic ventricular tachycardia waveforms and when the frequency and amplitude of input signals varied. Tests for accuracy revealed poor performances at low and high impedance levels for most AEDs. Notably, differences greater than 20 seconds were found in the time from power-on to “ready for discharge.”

Conclusions: The approach we used to assess AEDs allowed us to evaluate their appropriateness with respect to the organizational context, to measure their parameters, and to compare models. Results showed that ergonomics and/or performances (timing and accuracy) could be improved in each device.

The aim of this study was to investigate the efficacy of hyperbaric oxygen (HBO) therapy in idiopathic sudden sensorineural hearing loss (ISSHL) by comparing hearing gain and improvement rate in patients who have been placed on both HBO and medical treatment (MT) (37 patients), and patients who have received MT only (17 patients). Both groups were compared with reference to pure tone average (PTA) and the number of patients who experienced hearing gain. Of 37 patients (40 ears) who received HBO + MT, 24 (60 per cent) experienced ≥ 10 decibels (dB) improvement in PTA compared to 13 (76.4 per cent) of 17 patients who were placed on MT only. Inter- or intra-group comparison of age stratification (<50 and ≥50 ages) did not produce significant differences in PTA and in the number of patients who experienced hearing gain.

Although there are numerous studies showing efficacy of HBO therapy; this study did not reveal a trend in favour of HBO therapy.

Objective: We investigated the possible role of hypotension and related autonomic phenomena in the pathogenic mechanism of sudden sensorineural hearing loss.

Methods: Forty-nine patients belonging to the ASA I-II classes of anaesthesiological risk and submitted to a non-otological surgical procedure were examined. Each operation was performed under general anaesthesia by controlled hypotension technique. Hearing function of the patients was evaluated before and after surgery by means of a pure tone audiometry recorded by the same clinician with the same instrument.

Results: No cases of bilateral hearing worsening were recorded after surgery.

Conclusions: An induced and controlled steady hypotension under general anaesthesia did not affect the hearing function of any of the patients. It may be supposed, therefore, that an adverse effect on the cochlear oxygenation is more likely to be caused by the sympathetic changes induced by a consistent decrease of blood pressure rather than to hypotension itself.

Autoimmune inner ear disease is a well described entity. We report a case of sudden-onset sensorineural hearing loss in association with psoriatic arthritis, which has not been reported in the literature. The case satisfies the criteria for the presumptive diagnosis of autoimmune hearing loss. A high index of suspicion, with early diagnosis and aggressive treatment with steroids and/or immunosuppressive agents, is essential to prevent irreversible hearing loss. The condition of psoriatic arthritis must be added to the pantheon of autoimmune diseases that can lead to sensorineural hearing loss.

Cardiac arrest is a dramatic condition leading to sudden death if someone cannot perform two interventions, basic life support and early defibrillation, that have been proved to improve long-term survival. The modern era of cardiopulmonary resuscitation began with a description of closed chest cardiac massage by Kouwenhoven in 1960. Research to make advances in cardiac arrest is a very difficult task. Experts set up a series of guidelines that represent a keystone for educational needs and evolving technology. There is a consensus that the highest potential survival rate from cardiac arrest can only be achieved when the following sequence of events occurs as rapidly as possible: (1) recognition of early warning signs, (2) activation of the emergency medical services, (3) basic cardiopulmonary resuscitation, (4) cardiac defibrillation, (5) management of the airway and ventilation of the lungs, and (6) intravenous (i.v.) administration of medications. These events are necessary for any success of emergency cardiovascular care. They are linked in a chain of survival. The links in the adult chain of survival are: early access; early CPR; early defibrillation; and early acute cardiac life support. The 'Utstein style', recently introduced, represents a standard of practice both inside and outside hospital with recommended guidelines for the uniform reporting of clinical data from the patient suffering cardiac arrest. According to the concept of the chain of survival, the Utstein style delineates time and establishes a set of core and supplemental times to be recorded that provide important characteristics of the response of victims of cardiac arrest.

Adult presentation of the Dandy-Walker syndrome is extremely rare. We report a case where the patient was first diagnosed at 75 years of age because of sudden onset of unilateral senorineural deafness and episodic vertigo. He remained well for a further three years until developing the more classical adult presenting features of cognitive impairment, disordered gait and ataxia. The radiological and otological investigative findings are discussed, together with their implications.

A questionnaire was sent to 100 consultant otolaryngologists from three selected health regions in the United Kingdom enquiring about their assessment and management of a patient presenting with sudden-onset sensorineural hearing loss. Seventy – six replied, of whom 42 stated a sub – specialist interest in otology. Seventy – eight percent of respondents would order routine blood tests including the erythrocyte sedimentation rate (ESR) and syphilis serology; in addition 38 per cent would order a magnetic resonance image (MRI) scan at the first presentation. The vast majority would treat such a patient (89 per cent), although the method and combination varied widely. A review of the literature confirmed that the level of evidence for treating this condition is limited and therefore the wide disparity in consultants’ practice is not surprising.

In this study, we analysed the clinical and audiological features of 24 vestibular schwannoma patients presenting with sudden deafness who visited our hospital within 10 days after its onset. The relationships between the prognosis of hearing loss and the clinical and audiological features including the pure-tone average (PTA), the presence of dizziness and/or vertigo, the tumour size and the auditory brain stem response (ABR) findings were evaluated. Hearing recovery depended on the PTA and tumour size, but even a small tumour induced sudden deafness. Impaired hearing completely recovered in five our of 24 patients. ABR findings indicated that the aetiology of sudden deafness in the vestibular schwannoma patients might vary in each patient.

The retrospective analysis of 1700 forensic autopsies over 17 years (1981–97) following unexpected sudden cardiac death revealed a group of 50 cases that could have been related to surgery and/or anaesthesia. Patients were young with no history of cardiac disease. Surgery was performed for uncomplicated disorders, all classified as ASA 1. Cardiac arrest took place at induction of anaesthesia in 16% of cases, during surgery in 64% and at the end of surgery in 20%. Investigation and expertise reports ordered by the public prosecutor revealed none of the typical causes of death usually associated with surgery or anaesthesia. Pathological examination showed cardiac lesions in 47 cases: arrhythmogenic right ventricular cardiomyopathy in 18 cases, coronary artery disease in 10 cases, cardiomyopathy in eight cases, structural abnormalities of the His bundle in nine cases, mitral valve prolapse in one case, and acute myocarditis in one case. Identification of the cause of death of patients at low risk may provide major relief to the family of the patient and the medical staff.

We report the case of a female patient who presented with sudden deafness as the first symptom of a cerebellar tumour which was not localized strictly in the cerebellopontine angle and did not show direct compression on the extrabulbar portion of the Vlllth cranial nerve. The clinical picture contained a number of signs and symptoms typical of cerebellar involvement.

Surgical intervention restored the hearing and caused the symptoms to disappear.

We also review the association between tumours and sudden deafness in the literature.

Twelve per cent of a series of 284 patients with vestibular schwannoma presented with sudden deafness. If sudden sensorineural hearing loss is present then it is very likely to be the main presenting symptom. The mean length of patients' history is eight months shorter in this group than in the non-sudden deafness group. Sixteen per cent of vestibular schwannoma patients without sudden deafness present with a 'dead' ear whereas 29.5 per cent of those presenting with sudden deafness have total hearing loss. There was no significant difference between the sudden deafness group and the 'all others' group with regard to tumour size, udiogram shape, caloric test, imbalance, and facial numbness. Although the numbers of patients with sudden deafness in this series were too small to reach significance, on the basis of the clinical correlation of vestibular schwannoma morphology it is possible to postulate that compression of the vasculature within the bony internal auditory canal by a laterally arising tumour may be the aetiological factor and may be more likely to occur than in more medially arising tumours.

Sickle cell disease is a world-wide problem which has been noted to cause high tone sensorineural hearing loss. We report a case with sudden onset bilateral hearingloss which progressed to total sensorineural deafness. To our knowledge there has been noreport of such a case occurring previously.

One hundred and four larynges removed from infants registered as dying fromSudden Infant Death Syndrome (SIDS) and 20 control larynges from infants of matching ages, were examined histologically. Excessive amounts of subglottic submucosal glandular tissue had reduced the available airway by more than half in 35 per cent of the SIDS larynges within the age group two to four months. In 30 per cent of this group the airway reduction was more than 60 per cent. It is suggested that hyperplasia of subglottic mucous glands is one cause of fatal hypoxia in sudden unexpected infant death.

An 18-year-old woman, while suffering from acute acquired toxoplasmosis, experienced sudden deafness and a total loss of vestibular function first in the right ear and three months later also in the left. Following treatment with sulphadiazine and pyrimethamine, hearing was retrieved to such a degree that the patient was enabled to communicate by means of a body-worn hearing aid and lip-reading. Taking the differential diagnostic possibilities into account, we believe that toxoplasmosis was the cause of the severe hearing loss. Since effective treatment seems to be available, we recommend that patients with acute bilateral sensorineural hearing loss of unknown origin are examined for acute toxoplasmosis with a view to instituting chemotherapy.