A regional-based newborn hearing screening program: the Emilia-Romagna model after ten years of legislation.

Background: Hearing loss, occurring in 1-3/1,000 newborns in the well-babies population, is one of the most common congenital diseases, and hearing screening at birth still represents the only means for its early detection. Since 2011 the Emilia Romagna Regional Health Agency has recommended Newborn Hearing Screening for all babies at its birth points and for newborns moving to the region. The aims of this study are to analyze the results of this regional-based Newborn Hearing Screening program and to discuss the impact of the legislative endorsement on the organization. Material and methods: This is an observational retrospective chart study. The recordings of well-babies and babies at Neonatal Intensive Care Units were collected during the period from January 1st 2015 to December 31st 2020. The following data were included: Newborn Hearing Screening coverage, percentage of refer at otoacoustic emissions, prevalence and entity of hearing loss, unilateral/bilateral rate, presence of audiological risk factors. Results: More than 99% of a total of 198,396 newborns underwent the Newborn Hearing Screening test during the period January 1st 2015 to December 31st 2020, with a coverage ranging between 99.6% and 99.9%. Overall, the percentage of confirmed hearing loss cases was about 17-30 % of refer cases, 745 children received a diagnosis of hearing loss (prevalence 3.7/1,000). Considering profound hearing loss cases, these represent 13% of bilateral hearing loss. Conclusion: A regional-based Newborn Hearing Screening program is valuable and cost-effective. In our experience, the centralization of the data system and of the data control is crucial in order to implement its efficiency and effectiveness. Healthcare policies, tracking systems and public awareness are decisive for a successful programme implementation.

Can lateral semicircular canal dysplasia play a role in the genesis of hyperacusis.

Hyperacusis can be a prominent and disabling symptom of superior semicircular canal dehiscence associated with autophony and the Tullio phenomenon. We report three clinical cases characterized by disabling hyperacusis in which semicircular canals dehiscence was excluded by temporal bone high-resolution computed tomography. The images disclosed lateral semicircular canal dysplasia, characterized by a small bony island, and dilatation of both the anterior and the posterior arms of the lateral semicircular canal. Cochleo-vestibular examinations (pure tone audiometry, infra-red videonystagmoscopy, vibration-induced nystagmus test, vestibular evoked myogenic potentials) will also be described. To verify the transtympanic ventilation tube effect, bilateral myringotomies tubes were performed in one patient but no long lasting subjective benefit was noted. Concerning the pathophysiology of this condition, we hypothesized that the increased volume of inner ear liquid can modify the micromechanical function of the cochlea and the labyrinthine hydrodynamics. In conclusion, in the case of specific symptoms, such as hyperacusis, it is important to consider the possibility of an inner ear morphological alteration involving the lateral canal and vestibule structures, as well as the existence of bony semicircular canal dehiscence.

Tympanometric findings in superior semicircular canal dehiscence syndrome.

The diagnostic role of audio-impedancemetry in superior semicircular canal dehiscence (SSCD) disease is well known. In particular, since the first reports, the presence of evoked acoustic reflexes has represented a determining instrumental exhibit in differential diagnosis with other middle ear pathologies that are responsible for a mild-low frequencies air-bone gap (ABG). Even though high resolution computed tomography (HRCT) completed by parasagittal reformatted images still represents the diagnostic gold standard, several instrumental tests can support a suspect of labyrinthine capsule dehiscence when "suggestive" symptoms occur. Objective and subjective audiometry often represents the starting point of the diagnostic course aimed at investigating the cause responsible for the so-called "intra-labyrinthine conductive hearing loss". The purpose of this study is to evaluate the role of tympanometry, in particular of the inter-aural asymmetry ratio in peak compliance as a function of different mild-low frequencies ABG on the affected side, in the diagnostic work-up in patients with unilateral SSCD. The working hypothesis is that an increase in admittance of the "inner-middle ear" conduction system due to a "third mobile window" could be detected by tympanometry. A retrospective review of the clinical records of 45 patients with unilateral dehiscence selected from a pool of 140 subjects diagnosed with SSCD at our institution from 2003 to 2011 was performed. Values of ABG amplitude on the dehiscent side and tympanometric measurements of both ears were collected for each patient in the study group (n = 45). An asymmetry between tympanometric peak compliance of the involved side and that of the contralateral side was investigated by calculating the inter-aural difference and the asymmetry ratio of compliance at the eardrum. A statistically significant correlation (p = 0.015 by Fisher's test) between an asymmetry ratio ≥ 14% in favour of the pathologic ear and an ABG > 20 dB nHL on the same side was found. When "evocative" symptoms of SSCD associated with important ABG occur, the inter-aural difference in tympanometric peak compliance at the eardrum in favour of the "suspected" side could suggest an intra-labyrinthine origin for the asymmetry. Tympanometry would thus prove to be a useful instrument in clinical-instrumental diagnosis of SSCD in detection of cases associated with alterations of inner ear impedance.

Meniere's disease: update of etiopathogenetic theories and proposal of a possible model of explanation.

Meniere's Disease (MD) is an affection consisting of an association of sensorineural hearing loss, tinnitus and vertigo initially presenting by crises. A review of the most considered possible causative factors and pathophysiologic interpretations allows us to underline the uncertainties which still exist about the genesis of this illness. We propose a mechanistic model based on the effect of a haemodynamic imbalance leading to transient ischaemia which could have an effect on the pH of the inner ear as well as on the work of the inner ear proton pumps. It is hypothesized that under ischaemic conditions and consequent metabolic acidity a preserved proton pump activity can generate an overload of anions in the endolymphatic partition, which is a closed system, thus resulting in an enhancement of osmolarity and consequently in the formation of a hydrops resulting in the development of fluctuating hearing loss, tinnitus and vertigo which characterize Meniere's Disease.

Langerhans cell histiocytosis: bilateral temporal bone involvement in an adult with diabetes insipidus.

OBJECTIVE: To present a clinical case of an adult affected by Langerhans cell histiocytosis with bilateral, non-simultaneous, involvement of the temporal bone, associated with diabetes insipidus and to review the literature. METHODOLOGY: A rare case of bilateral temporal bone involvement of Langerhans cell histiocytosis in a 42-year-old woman affected by diabetes insipidus is reported. We present patient's clinical history supported by radiologic, histopathologic and audiologic findings. RESULTS: The patient was submitted to a series of otologic surgical procedures due to the progression of the disease. Ossicular chain was always preserved, so that conservative surgery (canal wall-up technique) was performed, permitting the achievement of good hearing results, bilaterally. CONCLUSIONS: Temporal bone involvement of Langerhans cell histiocytosis may lead to a progressive chronic disease. However, the ossicular chain can remain uninvolved, making a conservative surgical treatment possible. Careful follow-up is essential for detecting new lesions and serial CT scans are mandatory.

[Cisplatin and carboplatin-induced ototoxicity in children: clinical aspects and perspectives for prevention]. / Ototossicità da cisplatino e carboplatino in età pediatrica: aspetti clinici e prospettive di prevenzione.

In 70% of the cases of malignant neoplasms in pediatric patients antiblast therapy is used. The administration of platinum compounds, Cisplatin (CDDP) or Carboplatin (CBDCA), often at high cumulative doses, necessarily implies a certain degree of toxicity which normally takes on secondary significance in the healing of the child. Among the side effects, early and delayed ototoxicity is well known and, in the child, take on particular aspects. While not abandoning the treatment of the base pathology, the therapeutic findings, increasingly comforting in terms of long-term survival, require more accurate evaluation and overall control of the quality of life of these young patients. Since initial cochlear damage can be reversible, auditory function must be carefully monitored in order to prevent the lesions from becoming permanent, in particular prior to the onset of speech. For this reason a retrospective study was made of a group of 26 children affected by malignant neoplasms all of whom had undergone a polychemotherapy protocol with the administration of CDDP in 14 cases and CBDCA in 12 cases. All these young patients were monitored with conventional audiometry. The presence of different variables (antiblastic drug administration schedule, course of the disease, general conditions) only permitted evaluation of a single correlation: between auditory function at the end of the treatment (or after a few cycles of therapy) and the overall dose of CDDP or CBDCA administered. In 16 cases (62%) typical bilateral perceptive deafness was detected progressively involving the hyperacute and acute frequencies. The finding of hearing loss was significantly greater in the patients treated with CDDP (86%) vs. those treated with CBDCA (33%). Moreover, analysis of the results showed that, within certain limits, ototoxicity can depend more on individual sensitivity to the drug than to the total dose administered. The results confirm the well-known ototoxicity of platinum compounds, in particular CDDP. As ever improved therapeutic results are achieved in the treatment of malignant neoplasms in pediatric patients, greater attention can be paid to the quality of life of these young patients after the disease has been healed. Careful monitoring of auditory function can be extremely important in the pursuit this objective; indeed, this makes it possible to adjust antiblast drug administration, particularly in the later stages of treatment, customizing the treatment schedule to individual patient sensitivity.

Risco profissional do anestesiologista de Buenos Aires: investigacion e estudo comparativo com um grupo-controle / Professional risk of the anesthesiologist of Buenos Aires. Research and comparative study with a control-group

Na ultima decada, cientistas de reconhecida hierarquia internacional publicaram importantes investigacoes sobre a contaminacao ambiental nos centros cirurgicos e o risco profissional do anestesiologista decorrente ao trabalho desenvolvido nesse meio. Membros da diretoria da Associacao Argentina de Anestesiologia - Departamento de Buenos Aires decidiram pesquisar seu associados, com a finalidade de verificar condicoes socio-economicas, ambiente de trabalho e se determinadas patologias teriam relacao com a interacao desses fatores. Os autores analisaram 401 pesquisas, representando estas 69,13% dos anestesiologistas de Buenos Aires. Como grupo-controle comparativo foram investigados 65 medicos da Sociedade Argentina de Enfermidades Transmissiveis (83,33% ), que habitualmente nao frequentam centros cirurgicos. Os resultados permitem afirmar que os anestesiologistas, comparados com os colegas do grupo "nao exposto", sofrem das seguintes patologias, resultados estatisticamente significativos: infeccoes cutaneas e oftalmicas, sintomas relacionados com a contaminacao ambiental (cefaleia, nauseas, sonolencia, anorexia, enjoos) e situacoes de estresse pelo trabalho diario (ulcera gastroduodenal, precordialgia, taquicardia Outras patologias, como a hepatite, o infarto do miocardio e anormalidades congenitas em filhos de mulheres anestesiologistas, embora nao tenham sido significativas do ponto de vista estatistico, mostraram uma maior tendencia em relacao ao grupo-controle