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.

An innovative method for trans-impedance matrix interpretation in hearing pathologies discrimination.

Trans-impedance measurement is a novel methodology for assessing the positioning of a cochlear implant (CI). This study proposes an innovative use of trans-impedance measurements to characterize specific hearing pathologies by means of the trans-impedance matrix (TIM) quantitative analysis. Three indices are used: Shannon Entropy, the Exponential Decay constant and Spatial Correlation. These indices were computed on the TIMs of two groups of patients, clustered in terms of hearing pathology: (i) congenital hearing loss (CONG) and (ii) otosclerosis (OTO). The study aimed to demonstrate the sensitivity of the above synthetic indices in relation to the considered hearing pathologies. Furthermore, the first two indices were employed to explore the influence of the positioning of the electrode, either over (i) the basal or (ii) the apical regions, on the TIMs patterns. The results suggest that the indices were statistically different for the patient groups and the positioning impacted solely on OTO patients. In particular: (i) CONG patients displayed significantly higher Shannon Entropy (p = 0.0002) and (ii) a lower Exponential Decay constant than OTO patients (p = 0.001); (iii) the OTO patients exhibited a lower Shannon Entropy and a higher Exponential Decay constant over the basal regions than the apical regions (p < 0.008); (iv) Spatial Correlation demonstrated that TIMs had specific patterns according to the hearing pathology (p < 0.008).

Cochlear implantation in post-lingually deafened adults and elderly patients: analysis of audiometric and speech perception outcomes during the first year of use.

The aim of this study was to analyse audiometric and speech perception outcomes after cochlear implantation (CI) in adult and elderly patients in the first year post-CI activation. We evaluated 42 subjects who underwent CI at the Otorhinolaryngological Clinic of Padua Hospital. The subjects enrolled were post-lingually deafened patients who were unilaterally implanted for bilateral, severe-to-profound hearing loss. The overall sample was divided into three groups according to the age at the time of implantation: group A (35-49 years), group B (50-64 years) and group C (≥ 65 years). The subjects were assessed, both before and after surgery (at months 1, 3, 6 and 12), using pure tone audiometry, speech audiometry and speech perception tests and the CAP questionnaire. Statistical analysis of outcomes was using a Student's t-test for paired data. In all study groups a significant improvement was demonstrated in auditory performance examinations post-CI compared to the pre-operative scores. All subjects in all age groups obtained significant improvements in PTA scores before surgery and post-CI activation. Comparison of PTA values among the three age groups did not reveal any significant difference. Considerable improvement was obtained even in the speech audiometry thresholds in all groups at follow-up, with no significant differences between groups. The speech perception examination and CAP questionnaire showed good progress in all study groups, although younger patients tended to achieve more complex categories than older ones. In conclusion, CI is an effective treatment for severe-to-profound hearing loss with no significant differences in auditory performances between older and younger CI recipients. Even if somewhat slower, subjects older than 65 reached good performance and therefore are good candidates for a cochlear implant.

Cranial Nerve Abnormalities in Oculo-Auriculo-Vertebral Spectrum.

BACKGROUND AND PURPOSE: Cranial nerve abnormalities might be observed in hemifacial microsomia and microtia (oculo-auriculo-vertebral spectrum), but the rate, features, and relationship with functional impairment or phenotype severity have not yet been defined. This study aimed at investigating absence/asymmetry, abnormal origin, morphology and course of cranial nerves, and presence/asymmetry of the foramen ovale and inferior alveolar nerve canal in a cohort of oculo-auriculo-vertebral spectrum patients. MATERIALS AND METHODS: Twenty-nine patients with oculo-auriculo-vertebral spectrum (mean age, 7 years; age range, 0.2-31 years; 12 females) underwent brain MR imaging, CT, and neurologic evaluation; 19 patients had a more severe phenotype (Goldenhar syndrome). RESULTS: Cranial nerve abnormalities were detected only in patients with Goldenhar syndrome (17/19, bilaterally in 8) and were involved the second (4/19), third (1/18), fifth (11/19), sixth (8/16), seventh (11/18), and eighth (8/18) cranial nerves. Multiple cranial nerve abnormalities were common (11/17). Eleven patients showed bone foramina abnormalities. Trigeminal and facial nerve dysfunctions were common (44% and 58%, respectively), especially in patients with Goldenhar syndrome. Trigeminal abnormalities showed a good correlation with ipsilateral dysfunction (P = .018), which further increased when bone foramina abnormalities were included. The facial nerve showed a trend toward correlation with ipsilateral dysfunction (P = .081). Diplopia was found only in patients with Goldenhar syndrome and was associated with third and sixth cranial nerve abnormalities (P = .006). CONCLUSIONS: Among patients with oculo-auriculo-vertebral spectrum, cranial nerve morphologic abnormalities are common, correlate with phenotype severity, and often entail a functional impairment. The spectrum of cranial nerve abnormalities appears wider than simple hypo-/aplasia and includes an anomalous cisternal course and partial/complete fusion of diverse cranial nerves.

Is very early hearing assessment always reliable in selecting patients for cochlear implants? A case series study.

INTRODUCTION: This study concerns a case series of 23 infants with a diagnosis of severe-to-profound hearing loss at 3 months old, who significantly improved (even reaching a normal auditory threshold) within their first year of life. METHODS: All infants were routinely followed up with audiological tests every 2 months after being fitted with hearing aids as necessary. A reliable consistency between the various test findings (DPOAE, ABR, behavioral responses, CAEP and ECoG) clearly emerged in most cases during the follow-up, albeit at different times after birth. RESULTS: The series of infants included 7 cases of severe prematurity, 6 of cerebral or complex syndromic malformations, 5 healthy infants, 2 with asymptomatic congenital CMV infection, and 1 case each of hyperbilirubinemia, hypoxia, and sepsis. All term-born infants showed a significant improvement over their initial hearing threshold by 6 months of age, while in most of those born prematurely the first signs of threshold amelioration occurred beyond 70 weeks of gestational age, and even beyond 85 weeks in one case. CONCLUSIONS: Cochlear implantation (CI) should only be considered after a period of auditory stimulation and follow-up with electrophysiological and behavioral tests, and an accurate analysis of their correlation. In our opinion, CI can be performed after a period of 8 months in all term-born infants with persistent severe-to-profound hearing loss without risk of diagnostic error, whereas the follow-up for severely preterm infants should extend to at least 80 weeks of gestational age.