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.

The music-related quality of life: Italian validation of MuRQoL into MUSQUAV questionnaire and preliminary data from a cohort of postlingually deafened cochlear implant users.

PURPOSE: Cochlear implant (CI) users do not receive much of the auditory information necessary for an accurate perception of music. This usually entails a dissatisfaction with the music they hear, so that their quality of life may potentially be affected. The main aim of this paper was to translate and validate into Italian an instrument to evaluate these aspects-The Music-Related Quality of Life Questionnaire (MuRQoL)-to help the work of clinicians and therapists. METHODS: The translation of the MuRQoL into "Questionario Musica e Qualità della Vita" (MUSQUAV) was done according to the international guidelines. The translated questionnaire was administered to normal hearing (NH) and CI users adults. Exploratory factor analysis, confirmatory factor analysis and known group method were used to confirm construct validity and applicability of MUSQUAV. RESULTS: We retrieved 225 results. The MUSQUAV questionnaire was acceptable according to the goodness-of-fit indices. The correlation between the items, evaluated using Cronbach's α coefficient, indicates a good internal consistency (> 0.80). The non-parametric Mann-Whitney test showed significant differences in the distinct populations tested. CONCLUSIONS: The MUSQUAV questionnaire is a valid, low-cost and rapid instrument for professional workers in the audiological field, especially useful in the assessment of the patients' perception and musical engagement.

Aspiration: diagnostic contributions from bedside swallowing evaluation and endoscopy.

The aim of this study was to identify which characteristics, collected by bedside swallowing evaluation (BSE) and fiberoptic endoscopic evaluation of swallowing (FEES), are a risk or a protective factor for aspiration. This retrospective study included data on 1577 consecutive patients, collected by BSE and FEES. Bivariate analysis was performed to verify the association of each variable with aspiration (Chi-Square test). The variables associated with aspiration were entered into a multivariate logistic model to quantify this association. Several variables were significantly associated (p < 0.05) with aspiration; cooperation, sensation, laryngeal elevation and direct therapy were found to be protective factors against aspiration. The regression model identified the most variables related with aspiration, among which tracheotomy, material pooling and spillage. Patients able to perform dry swallows were 77% less likely to aspirate (protective factor). Several variables are involved in protection of airways during swallowing. Their interaction, in patients with swallowing disorders, offers the clinician the best means of interpreting BSE and FEES.

A mild phenotype of sensorineural hearing loss and palmoplantar keratoderma caused by a novel GJB2 dominant mutation.

Dominant GJB2 mutations are known to cause a syndromic form of sensorineural hearing loss associated with palmo-plantar skin manifestations. We present the genotype/phenotype correlations of a new GJB2 mutation identified in three generations of an Italian family (proband, mother and grandfather) whose members are affected by sensorineural hearing impairment associated with adult-onset palmoplantar keratoderma. In all affected members we identified a new heterozygous GJB2 mutation (c.66G > T, p.Lys22Asn) whose segregation, population frequency and in silico prediction analysis have suggested a pathogenic role. The p.Lys22Asn GJB2 mutation causes a dominant form of hearing loss associated with variable expression of palmoplantar keratoderma, representing a model of full penetrance, with an age-dependent effect on the phenotype.

Pacemaker and ICD oversensing induced by movements near the MRI scanner bore.

PURPOSE: The effect of the movement near the MRI scanner bore for people with a pacemaker (PM) or an implantable cardioverter defibrillator (ICD) is experimentally evaluated and discussed. METHODS: The authors performed in vitro measurements on a saline-filled human-shaped phantom (male, 170 cm height), equipped first with an MR-conditional PM (bicameral configuration, DDD programming), then with an MR-conditional ICD (biventricular configuration, detection algorithms enable but shock delivery disable). Both the devices were able to transmit in real-time the detected cardiac activity (electrograms) while moving the phantom around the MRI scanner. The phantom was also equipped with an accelerometer and a magnetic field probe to measure the angular velocity and the magnetic field variation during the experiment. Unipolar versus bipolar sensing mode and maximum sensitivity versus nominal settings were tested. RESULTS: The sensing functions of the PM and ICD systems began to react to motion induced electromagnetic interference starting at an angular velocity as low as 2 rad/s (|dB/dT| = 2 T/s). The motion induced EMI in PM and ICD systems was interpreted as sensed intrinsic heartbeats which resulted in inappropriate pacing inhibition and arrhythmia classification. At the maximum speed of about 6 rad/s (|dB/dT| = 3 T/s), the induced EMI affected classification of ectopic beats and two episodes of VF were inappropriately recorded. CONCLUSIONS: These results demonstrate that motion in and around an MR scanner can induce EMI significant enough to be misinterpreted by implanted PMs and ICDs leading to inappropriate changes in therapy. These findings highlight that PM or ICDs, including MR-conditional systems should not enter the MRI room, except in case of an examination under specified conditions.

Nimodipine in otolaryngology: from past evidence to clinical perspectives.

As L-type voltage-gated calcium channels (VGCCs) control Ca(2+) influx and depolarisation of cardiac and vascular smooth muscle, they represent a specific therapeutic target for calcium channel blockers (CCBs), which are approved and widely used to treat hypertension, myocardial ischaemia and arrhythmias. L-type currents also play a role in calcium entry in the sensory cells of the inner ear. In hair cells of both cochlea and labyrinth, calcium cytoplasmic influx is the first physiological process that activates complex intracellular enzymatic reactions resulting in neurotransmitter release. Excessive calcium ion entry into sensory cells, as a consequence of L-VGCCs malfunction is responsible for over-activation of phospholipase A2 and C, protein kinase II and C, nitric oxide synthase and both endonucleases and depolymerases, which can cause membrane damage and cellular death if the cytoplasmic buffering capacity is overcome. Nimodipine, a highly lipophilic 1-4 dihydropyridine that easily crosses the brain-blood barrier, is generally used to reduce the severity of neurological deficits resulting from vasospasm in patients with subarachnoid haemorrhage. Moreover, due to its selective blocking activity on L-channel calcium currents, nimodipine is also suggested to be an effective countermeasure for cochlear and vestibular dysfunctions known as channelopathies. Indeed, experimental data in amphibians and mammalians indicate that nimodipine has a stronger efficacy than other CCBs (aminopyridine, nifedipine) on voltage-dependent whole-cell currents within hair cells at rest and it is the only agent that is also effective during their mechanically induced depolarisation. In humans, the efficacy of nimodipine is documented in the medical management of peripheral vestibular vertigo, sensorineural hearing loss and tinnitus, even in a pathology as complex as Ménière's disease. Nimodipine is also considered useful in the prophylaxis of damage to the facial and cochlear nerves caused by ablative surgery of cerebellopontine tumours; it has been recently hypothesised to accelerate functional recovery of recurrent nerve lesions during thyroid cancer surgery. Further trials with adequate study design are needed to test the efficacy of nimodipine in the treatment of vertigo due to cerebrovascular disease and vestibular migraine.

Choosing the correct treatment for acute aortic type B dissection.

Acute type B aortic dissection is a life threatening disease process, which remains a clinical dilemma despite advances in technology, surgical technique and postoperative management. The variability of presenting symptoms, lack of a consensus on indications for treatment and differing opinions about the optimal timing for repair have added to the management confusion. Medical management has been the standard of care for acute uncomplicated type B dissection. Surgical repair and endovascular intervention are reserved for those who present with, or subsequently develop, dissection-related complications. Complicated dissections occur in 25% of cases and may include organ malperfusion, aortic rupture, periaortic hematoma, and uncontrolled hypertension. In the past decade thoracic endovascular aortic repair (TEVAR) has gained widespread acceptance as the modality of choice for the treatment of complicated type B dissection. This transition is representative of advances in technology, physician experience with aortic endografts and lower morbidity and mortality rates associated with TEVAR. The best medical therapy remains the standard of care for uncomplicated dissection, however this strategy fails to prevent long-term aortic-related morbidity and mortality. Recent data suggest that early TEVAR lowers aortic-related events and improves long-term aortic specific survival by covering the entry tear, promoting false lumen thrombosis and inducing aortic wall remodeling. The paucity of supporting data has created controversy surrounding the optimal treatment strategy for acute type B dissection. Nonetheless, recent healthcare trends show a paradigm shift towards the utilization of early TEVAR in acute type B dissection.

The Pooling-score (P-score): inter- and intra-rater reliability in endoscopic assessment of the severity of dysphagia.

This study evaluated the intra- and inter-rater reliability of the Pooling score (P-score) in clinical endoscopic evaluation of severity of swallowing disorder, considering excess residue in the pharynx and larynx. The score (minimum 4 - maximum 11) is obtained by the sum of the scores given to the site of the bolus, the amount and ability to control residue/bolus pooling, the latter assessed on the basis of cough, raclage, number of dry voluntary or reflex swallowing acts (< 2, 2-5, > 5). Four judges evaluated 30 short films of pharyngeal transit of 10 solid (1/4 of a cracker), 11 creamy (1 tablespoon of jam) and 9 liquid (1 tablespoon of 5 cc of water coloured with methlyene blue, 1 ml in 100 ml) boluses in 23 subjects (10 M/13 F, age from 31 to 76 yrs, mean age 58.56±11.76 years) with different pathologies. The films were randomly distributed on two CDs, which differed in terms of the sequence of the films, and were given to judges (after an explanatory session) at time 0, 24 hours later (time 1) and after 7 days (time 2). The inter- and intra-rater reliability of the P-score was calculated using the intra-class correlation coefficient (ICC; 3,k). The possibility that consistency of boluses could affect the scoring of the films was considered. The ICC for site, amount, management and the P-score total was found to be, respectively, 0.999, 0.997, 1.00 and 0.999. Clinical evaluation of a criterion of severity of a swallowing disorder remains a crucial point in the management of patients with pathologies that predispose to complications. The P-score, derived from static and dynamic parameters, yielded a very high correlation among the scores attributed by the four judges during observations carried out at different times. Bolus consistencies did not affect the outcome of the test: the analysis of variance, performed to verify if the scores attributed by the four judges to the parameters selected, might be influenced by the different consistencies of the boluses, was not significant. These initial data validate the clinical use of the P-score in the management of patients with deglutition disorders by a multidisciplinary team.

Imaging assessment of groin pain.

Groin pain is a common condition in athletes, especially those who play certain sports, for instance soccer, ice hockey, fencing which request rapid acceleration and frequent changes of movement. This condition represents a diagnostic difficulty for the radiologist due to either the anatomical pubic region complexity than the many causes that can be a source of pain, because the groin pain can be determined by conditions affecting the bony structures, cartilage and muscle tendons that are part of the proper pubis but also from those involving the hip. The approach to the groin through diagnostic imaging is multidisciplinary: The study of the patient is performed by traditional radiographs, ultrasound examination, magnetic resonance imaging or computed tomography, based on clinical suspicion, and each of these methods provides different results depending on the disease in question. The purpose of this article is to examine what are the optimal imaging techniques to investigate the various diseases affecting the patient with groin pain.

Long head of the biceps tendon and rotator interval.

The term "biceps brachii" is a Latin phrase meaning "two-headed (muscle) of the arm." As its name suggests, this muscle has two separate origins. The short head of biceps is extraarticular in location, originates from the coracoid process of the scapula, having a common tendon with the coracobrachialis muscle. The long head of biceps tendon (LBT) has a much more complex course, having an intracapsular and an extracapsular portion. The LBT originates from the supraglenoid tubercle, and in part, from the glenoid labrum; the main labral attachments vary arising from the posterior, the anterior of both aspects of the superior labrum (Bletran et al. in Top Magn Reson Imaging 14:35-49, 2003; Vangsness et al. in J Bone Joint Surg Br 76:951-954, 1994). Before entering the bicipital groove (extracapsular portion), the LBT passes across the "rotator cuff interval" (intracapsular portion). Lesions of the pulley system, the LBT, and the supraspinatus tendon, as well as the subscapularis, are commonly associated (Valadie et al. in J Should Elbow Surg 9:36-46, 2000). The pulley lesion can be caused by trauma or degenerative changes (LeHuec et al. in J Should Elbow Surg 5:41-46, 1996). MR arthrography appears to be a promising imaging modality for evaluation of the biceps pulley, through the distention of the capsule of the rotator interval space and depiction of the associated ligaments.

Femoroacetabular impingement: role of imaging.

The femoroacetabular impingement (FAI) is an impingement characterized by repetitive abutment between the femur and the acetabular rim during hip motion due to loss of joint clearance (Imam and Khanduja in Int Orthop 35(10):1427-1435, 2011; James et al. in AJR Am J Roentgenol 187(6):1412-1419, 2006). Femoroacetabular impingement (FAI) can be classified as either cam or pincer type, and it can be differentiated on the basis of a predominance of either a femoral or an acetabular abnormality (Pfirrmann et al. in Radiology 244(2):626, 2007; Ganz et al. in Clin Orthop Relat Res 466(2):264-272, 2008). In cases of cam FAI, the nonspherical shape of the femoral head at the femoral head-neck junction and reduced depth of the femoral waist lead to abutment of the femoral head-neck junction against the acetabular rim. In cases of pincer FAI, acetabular overcoverage limits the range of motion and leads to a conflict between the acetabulum and the femur. The most important role of preoperative MR evaluation in patients affected by FAI is the accurate assessment of the damage's extension.

Mechanism of traumatic knee injuries and MRI findings.

Bone bruises are focal abnormalities in subchondral bone marrow due to trabecular microfractures as a result of traumatic force. These trauma-induced lesions are better detected with magnetic resonance (MR) imaging using water-sensitive sequences. Moreover, the pattern of bone bruise is distinctive and allows us to understand the dynamics of trauma and to predict associated soft injuries. This article discusses the mechanism of traumatic injury and MR findings.

Diagnostic imaging of ankle impingement syndromes in athletes.

The chronic ankle pain is a very frequent clinical problem, which is often characterized by a painful mechanical limitation of full-range ankle movement. A large amount of causes are involved in its pathogenesis, but the most common forms are secondary to an osseous or soft tissue abnormality. Especially for professional athletes, impingement lesions are the most important causes of chronic pain; however, this symptomatology can also affect ordinary people, mostly in those who work in environments that cause severe mechanical stress on the joints. This group of pathologies is characterized by a joint conflict secondary to an abnormal contact among bone surfaces or between bones and soft tissues. Diagnosis is mainly clinic and secondly supported by imaging in order to localize the critical area of impingement and determine the organic cause responsible for the joint conflict. Treatments for different forms of impingement are similar. Usually, the first step is a conservative approach (rest, physiotherapy, ankle bracing, shoe modification and local injection of corticosteroids), and only in case of unsuccessful response, the second step is the operative treatment with open and arthroscopic techniques. The aim of the study is to describe different MR imaging patterns, comparing our data with those reported in the literature, in order to identify the best accurate diagnostic protocol.

Ankle impingement: a review of multimodality imaging approach.

Ankle impingement is defined as entrapment of an anatomic structure that leads to pain and decreased range of motion of the ankle and can be classified as either soft tissue or osseous (Bassett et al. in J Bone Joint Surg Am 72:55-59, 1990). The impingement syndromes of the ankle are a group of painful disorders that limit full range of movement. Symptoms are due to compression of soft-tissues or osseous structures during particular movements (Ogilvie-Harris et al. in Arthroscopy 13:564-574, 1997). Osseous impingement can result from spur formation along the anterior margin of the distal tibia and talus or as a result of a prominent posterolateral talar process, the os trigonum. Soft-tissue impingement usually results from scarring and fibrosis associated with synovial, capsular, or ligamentous injury. Soft-tissue impingement most often occurs in the anterolateral gutter, the medial ankle, or in the region of the syndesmosis (Van den Bekerom and Raven in Knee Surg Sports Traumatol Arthrosc 15:465-471, 2007). The main impingement syndromes are anterolateral, anterior, anteromedial, posterior, and posteromedial impingement. These conditions arise from initial ankle injuries, which, in the subacute or chronic situation, lead to development of abnormal osseous and soft-tissue thickening within the ankle joint. The relative contributions of the osseous and soft-tissue abnormalities are variable, but whatever component is dominant there is physical impingement and painful limitation of ankle movement. Conventional radiography is usually the first imaging technique performer and allows assessment of any potential bone abnormality, particularly in anterior and posterior impingement. Computed tomography (CT) and isotope bone scanning have been largely superseded by magnetic resonance (MR) imaging. MR imaging can demonstrate osseous and soft-tissue edema in anterior or posterior impingement. MR imaging is the most useful imaging modality in evaluating suspected soft-tissue impingement or in excluding other ankle pathology such as an osteochondral lesion of the talus. MR imaging can reveal evidence of previous ligamentous injury and also can demonstrate thickened synovium, fibrosis, or adjacent reactive soft-tissue edema. Studies of conventional MR imaging have produced conflicting sensitivities and specificities in assessment of anterolateral impingement. CT and MR arthrographic techniques allow the most accurate assessment of the capsular recesses, albeit with important limitations in diagnosis of clinical impingement syndromes. In the majority of cases, ankle impingement is treated with conservative measures, with surgical debridement via arthroscopy or an open procedure reserved for patients who have refractory symptoms. In this article, we describe the clinical and potential imaging features, for the four main impingement syndromes of the ankle: anterolateral, anterior, anteromedial, posterior, and posteromedial impingement.

Lesions of the rotator cuff footprint: diagnostic performance of MR arthrography compared with arthroscopy.

BACKGROUND: To evaluate the diagnostic performance of magnetic resonance arthrography (MR-A) of the shoulder in the diagnosis of rotator cuff tears involving the humeral insertion of the supraspinatus and infraspinatus tendon (footprint), using arthroscopy as the reference standard. MATERIALS AND METHODS: The study population included 90 consecutive patients with history and clinical diagnosis of instability of the shoulder, rotator cuff tear or posterosuperior glenoid impingement. A total of 108 MR arthrograms were performed, since 18 patients had undergone a bilateral procedure. Arthroscopy, which was performed within 45 days after MR-A, was used as the reference standard. Sensitivity, specificity, accuracy, positive and negative predictive values were then calculated. RESULTS: Magnetic resonance arthrography showed a sensitivity of 92 % and a specificity of 78 % for the overall detection of tears involving the rotator cuff footprint. The diagnostic accuracy was 90 %, and the positive and negative predictive values were 95 and 64 %, respectively. Ten lesions were non-classifiable on surgery, of which eight were non-classifiable on MR-A also. CONCLUSIONS: Magnetic resonance arthrography is extremely accurate for the detection and classification of rotator cuff footprint tears. Most of these lesions are articular-sided (partial articular-sided supraspinatus tendon avulsion lesions) with predominance in younger patients and concealed type of tear (concealed interstitial delamination lesions).

Non-enhanced MR angiography of renal arteries: comparison with contrast-enhanced MR angiography.

BACKGROUND: The main causes of renal artery stenosis (RAS) are atherosclerosis and fibromuscular dysplasia. Despite contrast-enhanced magnetic resonance angiography (CE-MRA) being a safe and reliable method for diagnosis of RAS especially in young individuals, recently it has been possible to adopt innovative technologies that do not require paramagnetic contrast agents. PURPOSE: To assess the accuracy of steady-state free-precession (SSFP) non-contrast-enhanced magnetic resonance angiography (NC-MRA) by using a 1.5 T MR scanner for the detection of renal artery stenosis, in comparison with breath-hold CE-MRA as the reference standard. MATERIAL AND METHODS: Sixty-three patients (33 men, 30 women) with suspected renovascular hypertension (RVHT) were examined by a 1.5T MR scanner; NC-MRA with an electrocardiography (ECG)-gated SSFP sequence was performed in 58.7% (37/63) of patients; in 41.3% (26/63) of patients a respiratory trigger was used in addition to cardiac gating. CE-MRA, with a three-dimensional gradient echo (3D-GRE) T1-weighted sequence, was performed in all patients within the same session. Maximum intensity projection (MIP) image quality, number of renal arteries, and the presence of stenosis were assessed by two observers (independently for NC-MRA and together for CE-MRA). The agreement between NC-MRA and CE-MRA as well as the inter-observer reproducibility were calculated with Bland-Altman plots. RESULTS: MIP image quality was considered better for NC-MRA. NC-MRA identified 143 of 144 (99.3%) arteries detected by CE-MRA (an accessory artery was not identified). Fourteen stenoses were detected by CE-MRA (11 atherosclerotic, 3 dysplastic) with four of 14 (28.5%) significant stenosis. Bland-Altman plot demonstrated an excellent concordance between NC-MRA and CE-MRA; particularly, the reader A evaluated correctly all investigated arteries, while over-estimation of two stenoses occurred for reader B. Regarding NC-MRA, inter-observer agreement was excellent. CONCLUSION: NC-MRA is a valid alternative to CE-MRA for the assessment of renal arteries.

No thyroid abnormalities in patients submitted to cardiac catheterization in the first eighteen months of life.

BACKGROUND: No data are available about the risk of thyroid disturbance after exposure to low-dose radiation due to the use of cardiac catheterization in the first years of life. AIM: To determine the risk of functional and morphological thyroid abnormalities in a homogeneous cohort of patients who underwent diagnostic low-dose radiation for heart catheterization during the first 18 months of life. SUBJECTS AND METHODS: Fifty-five patients, submitted to cardiac catheterization during the first 18 months of life, underwent evaluation of the thyroid function and structure after a median period of 13 yr since the first radiation exposure. Sixty-eight unexposed controls matched for age and sex, underwent the same protocol. Twenty-two patients were then re-evaluated after a median period of 22 yr. RESULTS: Thyroid function resulted normal in both patients and controls. The prevalence of small thyroid nodules and inhomogeneous structures in ultrasound study was not augmented in irradiated patients compared to controls. No thyroid tumors or reduced thyroid volume were observed. CONCLUSIONS: Neither functional nor morphological disorders of the thyroid gland were demonstrated after a period up to 24 yr in patients exposed to diagnostic ionizing radiation for cardiac catheterization during the first 18 months of life.

[Etiological, clinical and neuroradiological investigation of deaf children with additional neuropsychiatric disabilities]. / Sordita complicata nel bambino: correlati eziologici, clinici e neuroradiologici.

AIM: Sensorineural hearing loss (SNHL) is complicated by additional disabilities in about 30% of cases, but the epidemiology of associated disorders, in terms of type, frequency and aetiology is still not clearly defined. Additional disabilities in a deaf child have important consequences in assessing and choosing a therapeutic treatment, in particular when considering cochlear implantation (CI) or hearing aids (HA). The aim of this paper was to evaluate frequency, type and severity of additional neurodevelopmental disabilities in children with profound bilateral sensorineural hearing loss and to investigate the relationship between disability and the etiology of deafness. METHODS: Eighty children with profound bilateral sensorineural hearing loss (mean age 5.4 years) were investigated by means of a diagnostic protocol including clinical, neurodevelopmental, and audiological procedures together with genetic and neurometabolic tests and neuroradiological investigation by brain MRI. RESULTS: Fifty-five percent of the sample exhibited one or more disabilities in addition to deafness, with cognitive, behavioural-emotional and motor disorders being the most frequent. The risk of additional disabilities varied according to aetiology, with a higher incidence in hereditary syndromic deafness, in cases due to pre-perinatal pathology (in comparison to unknown and hereditary non syndromic forms) and in the presence of major brain abnormalities at MRI. CONCLUSION: Our results suggest that the aetiology of deafness may be a significant risk indicator for the presence of neuropsychiatric disorders. A multidimensional evaluation, including aetiological, neurodevelopmental and MRI investigation is needed for formulating prognosis and for planning therapeutic intervention, especially in those children candidated to cochlear implant.