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Objective: To assess the audiological profile in a cohort of children affected by syndromic craniosynostosis., Methods: Eleven children with Apert syndrome (n=4), Saethre-Chotzen syndrome (n=3), Muenke syndrome (n=2), Crouzon syndrome (n=1) and Pfeiffer syndrome type 1 (n=1) were submitted to a complete audiologic evaluation including otoscopy, pure-tone audiometry, tympanometry and acoustic reflex testing, ABR, otoacustic emissions, temporal bone High Resolution CT (HRCT) scan. The main outcome measures were prevalence, type and severity of hearing loss, prevalence of chronic otitis media, correlation with the time of first surgical correction., Results: Seven of 11 patients (64%) presented hearing loss (HL), conductive in 3/7 patients (43%) and mixed in 4/7 (57%). No patients showed a purely sensorineural HL. All hearing impaired patients displayed middle ear disorders: the patients with conductive HL had otitis media with effusion (OME) and 3/4 patients with mixed HL showed tympanic alterations or cholesteatoma. A bilateral vestibular aqueduct enlargement was detected by HRCT scan in one normal hearing patient. The ABRs resulted normal in all cases., Conclusion: Our study confirms the high prevalence of otologic diseases in such patients. In contrast with previous studies, middle ear disorders were responsible for the hearing impairment also in patients with mixed HL due to secondary inner ear damage. These findings restate the necessity of a close audiologic follow-up. We did not detect the specific ABR abnormalities previously reported, possibly because of an early correction of the cranial vault malformations., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Objective: To assess the role of the efferent auditory system by inhibition of contralateral otoacoustic emission in dyslexic children with auditory processing disorders., Methods: The study sample was 34 children: 17 with dyslexia and 17 age-matched controls. Sensitive speech tests (low-pass filtered, time-compressed, distorted and dichotic) were performed to assess coexisting auditory processing disorder. Distortion-product otoacoustic emission (DPOAE) values were measured in basal condition and with contralateral broadband noise signal delivered via an earphone transducer at 60 dB SPL., Results: The lower scores at sensitive speech testing confirmed the association of an auditory processing disorder in the dyslexic children. DPOAE values were significantly attenuated by contralateral inhibition only in the control group (p=0.001; dyslexics, p=0.19); attenuation was not significant at any frequency in the dyslexic group., Conclusions: The differences in DPOAE attenuation between the groups, although not statistically significant, suggest alterations in the auditory efferent system in the dyslexic population. These alterations may affect language perception. If confirmed in further studies with larger samples, these results could provide insight into a possible pathophysiological background of dyslexia., (Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.)

Objectives: Flexible fiberoptic endoscopes have made pediatric laryngeal examinations an everyday practice, even though fiberoptic-flexible laryngoscopy (FFL) is not always well tolerated in young children because of limited cooperation. Laryngeal ultrasonography (LUS) has been applied to normal and pathological findings in infants and children, allowing the assessment of subglottic hemangiomas, laryngeal stenosis and paralysis. No previous study assessed benign vocal folds lesions by LUS in children. The aim of this study is to evaluate the possibility of LUS to detect benign vocal fold lesions in children by comparing the results of FFL in 16 children with those of LUS., Methods: Sixteen children (9 males and 7 females) with a mean age of 7.5+/-4.0 years were included in the study. Each child underwent FFL performed by a skilled phoniatrician and LUS performed blindly by an expert radiologist., Results: On FFL bilateral vocal folds nodules were found in 9 patients, vocal fold cyst in 2 other patients, while in 2 children the vocal folds appeared normal. Laryngeal papyllomatosis, vocal fold polyp and vocal fold irregularity were found in only one patient. LUS enabled the diagnosis in all the 14 patients with vocal fold lesions. Bilateral hyperechoic lesions were visible in 10 patients, while hypoechoic lesions were found in three patients. No lesion were found in two children, while one patient presented with a monolateral hyperechoic lesion., Conclusions: LUS was accurate, safe, well accepted and tolerated. LUS appears to be a useful diagnostic tool for supplementing FFL in the assessment of benign vocal fold lesions in children and may represent an interesting alternative in everyday clinical practice.

Objective: Aim of the study was to assess the mean age at diagnosis of bilateral congenital hearing loss in the Audiology and Phoniatry Centre of the University of Turin, pointing out, by North-West Italy experience, the role of the newborn hearing screening in anticipating the age of diagnosis., Methods: This was a retrospective study. Forty-six congenital deaf babies were reviewed and age at diagnosis was assessed for each, taking in consideration the role of hearing loss risk factors. Eighteen babies (39%) were sent by the centres that participate to the newborn hearing screening program while 28 (61%) came for parental or pediatrician suspicion of hearing loss and for general language delay. Sixteen babies (35%) presented risk factors for hearing loss., Results: The mean age of identification of severe to profound hearing loss was 20.5 months (S.D.=15.3) in the whole group; considering the group of 28 babies not screened the mean age was 29.3 months (S.D.=13.4). This value decreased to 6.8 months (S.D.=3.6) in the group which underwent screening programme. This difference was statistically significant at Student's t-test (p<0.001). The average ages of diagnosis for healthy versus high risk children were significantly different only in the group of screened babies (p<0.05)., Conclusions: Childhood hearing impairment is one of the most common of congenital disorders, and even though there is a general trend of early identification, in reality age of diagnosis is as yet still too late even in developed countries. Our results show that newborn hearing screening could reduce the age at which infants with hearing loss are diagnosed and treated; this would improve speech, language, auditory outcome and the quality of parents and infant life.

External irradiation of different head and neck cancers may involve parts of the ear. The vestibular function of 25 patients in which the inner ear was comprised in the irradiated volume was investigated by electronystagmography (ENG). Doses administered to the vestibular system ranged between 2800 and 5120 cGy. Five patients suffered subjective vertigo or dizziness. Eleven patients (three out of five with vertigo) showed vestibular abnormalities to ENG (44% of the total). Altered responses to specific tests were as follow: six patients to the bithermal caloric stimulation, two to the pendular-sinusoidal test and the other three to both of them. Patients were evaluated 3 and 6 months after the ending of the radiation therapy course. At the first evaluation, abnormalities to caloric test were noted in three patients (12%) and to sinusoidal rotatory test in one patient (4%). At the second evaluation, rates of abnormal response increased to 36% and 20%, respectively. Vestibular disorders seemed to be scantly related to the total radiation dose. Data of literature are discussed in order to identify possible implications on treatment planning.