Use of 3D-FLAIR MRI at 3 T may contribute to the elucidation of pathologic conditions in the inner ears of patients with ISSNHL and provide new radiologic indicators (mild hemorrhage, acute inflammation, presence or absence of BLB breakdown) that might assume the role of prognostic factors.

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The issue of cochlear implantation in deaf children with associated disabilities is an emerging subject. Currently, there is no consensus on whether to implant children with multiple impairments; moreover, it may be difficult to evaluate these children with standard tests pre- or post-implantation. In addition, these children often have poor speech perception and language skills, making assessment more difficult. Despite these factors, these children often receive important benefits in daily life, with an overall improvement in quality of life. In the present study, post-implant outcomes of 23 profoundly deaf children with neuropsychiatric disorders were analysed, using objective measures of speech perception, and a questionnaire administered to the parents, aimed at evaluating the benefits in daily life after implantation. The results were quite variable, but overall positive, in terms of speech perception, communication abilities, and improvement in quality of life. The findings add an additional piece of evidence to support the effectiveness of cochlear implantation in these special cases.

We did not record any surgical relevant complication in any of the patients, nor any significant postoperative variation in hearing thresholds, for air conduction or bone conduction, indicating the absence of surgical damage to the cochlea. All the patients demonstrated improvements in speech perception abilities with the device functioning and reported subjective benefits. With regard to the postoperative adverse effects, we had problems with feedback noise, which resolved with minor fitting adjustments in seven cases, while it required a second surgery to change the microphone position in the other patient. In one case a minimal extrusion of the microphone cable occurred requiring a revision surgery, a device failure occurred in one case, requiring substitution, and one patient decided on explantation of the device owing to psychological problems.

PDD Pervasive developmental disorder SNHL Sensorineural hearing lossAIM The effects of sensorineural hearing loss (SNHL) are often complicated by additional disabilities, but the epidemiology of associated disorders is not clearly defined. The aim of this study was to evaluate the frequency and type of additional neurodevelopmental disabilities in a sample of children with SNHL and to investigate the relation between these additional disabilities and the aetiology of deafness.METHOD One hundred children with severe ⁄ profound SNHL (60 males, 40 females; mean age 5y 7mo, SD 3y 6mo, range 8mo-16y) were investigated using a diagnostic protocol including neurodevelopmental, genetic, neurometabolic, and brain magnetic resonance imaging (MRI) assessment.RESULTS Forty-eight per cent of the sample exhibited one or more additional disabilities, with cognitive, behavioural-emotional, and motor disorders being the most frequent. The risk of additional disabilities varied according to the type of aetiology. Thirty-seven out of 80 individuals with available MRIs showed signal abnormalities, in particular brain malformations (46%) and white matter abnormalities (54%). Frequency and type of disability were associated with aetiology (p=0.015) and MRI data (p<0.001).INTERPRETATION A multidimensional evaluation, including aetiological, neurodevelopmental, and MRI investigation, is needed for planning therapeutic intervention, such as cochlear implantation in children with severe to profound hearing impairment. The aetiology of deafness is a relevant risk indicator for the presence of an associated disorder.According to the literature, sensorineural hearing loss (SNHL) is complicated by additional disabilities in about 30% of individuals, 1-5 but the epidemiology of associated disorders, in terms of type, frequency, and aetiology, is still not clearly defined. A wide variety of additional disabling conditions have been described in children with severe to profound hearing impairment, sometimes as part of a named syndrome (i.e. CHARGE [coloboma, heart defects, choanal atresia retarded growth and development, ear anomaly ⁄ deafness], Waardenburg, Usher, and Goldenhar syndromes), or as comorbid to deafness of known or unknown aetiology. Studies on additional disabilities are difficult to perform and interpret. The heterogeneity of definition and inclusion criteria (such as severity of cognitive disability) has led to widely differing results from various studies. Moreover, the relation between deafness and disability can raise contradictory interpretations that may be related to aetiology or a secondary association.Additional disabilities in a child with severe to profound hearing impairment have important consequences when assessing and choosing a therapeutic treatment, in particular when considering cochlear implantation or hearing aids.Given the costs of cochlear implantation, clear criteria for evaluating possible risks and benefits and meeting parents' expectations are indispensable. 6 The preoperative assessment and the postope...

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The early identification of pre-lingual deafness is necessary to minimize the consequences of hearing impairment on the future communication skills of a baby. According to the most recent international guidelines the deafness diagnosis must occur before the age of three months and the prosthetic-rehabilitative treatment with a traditional hearing aid should start within the first six months. When a Cochlear implant becomes necessary, the treatment should start between the age of 12 months and 18 months. The only way to diagnose the problem early is the implementation of universal neonatal audiological screening programs. Transient evoked otoacoustic emissions (TEOAE) is the most adequate test because it's accurate, economic and of simple execution. Automatic auditory brainstem response (AABR) is necessary to identify patients with auditory neuropathy but it is also important to reduce the number of false-positives.The 20-30% of infant hearing impairment is represented by progressive or late-onset hearing loss (HL) so it's also necessary to establish an audiological follow up program, especially in infants at risk.From November 2005 all neonates born in the University hospital of Pisa undergo newborn hearing screening. From 2008 the screening program follows the guidelines for the execution of the audiological screening in Tuscany which have been formulated by our group according to the 2007 JCIH Position Statement and adaptated to our regional reality by a multidisciplinary effort. From November 2005 to April 2009 8113 neonates born in the Neonatal Unit of Santa Chiara Hospital (Pisa) have undergone newborn hearing screening. 7621 neonates (93.9%) without risk factors executed only the TEOAE test. 492 (6.1%) neonates had audiological risk factors and thus underwent TEOAE and AABR. 84 patients (1,04%) failed both TEOAE and AABR tests. 78 of them underwent further investigations. 44 patients resulted falsepositives (the 0,54% of the screened newborns). 34 neonates (4,2 ‰) had a final diagnosis of hearing impairment. 8 patients (0.99 ‰) had unilateral hearing loss (HL). 26 patients (3,2 ‰) had bilateral hearing impairment.In our screening program the percentage of false-positives was quite low (0.54%) while the incidence of bilateral HL (3.2 ‰) is a little higher than that found in literature reports. In most of our patients premature birth or neonatal suffering represent the main cause of HL.

From a rehabilitative viewpoint, our results suggest the importance of implementing rehabilitation in lexical comprehension, even when expressive vocabulary appears to be within normal range. Moreover, assessment of language acquisition in CI Italian children should focus on those grammar aspects that are more vulnerable to early acoustic deprivation (such as free and bound morphology) to ensure enhanced language therapy planning.