Critical Issues in the Management of Newborn Hearing Screening in the Time of COVID-19 in Umbria, Italy.

Hearing impairment is the most frequent of the sensorial defects in humans, and if not treated promptly, can severely impair cognitive and spoken language skills. For this reason, a universal newborn hearing screening (UNHS) has been established. The purpose of our study is to examine, by means of a retrospective analysis, the results of the UNHS program in the Umbria region during the spread of COVID-19 (2020-2021), comparing the same data from the years 2011-2012, to understand if the program has improved. Our study has shown how the coverage rate of well born babies' (WB) screening has significantly increased to currently meet the JCIH benchmark. The percentage of WB referrals significantly decreased in 2020-2021, another indicator of the screening program's greater efficiency in Umbria. However, a critical issue has emerged: the percentage of those lost to follow-up is greater than 30%, well above the benchmark. As far as the COVID-19 pandemic has certainly had a significant impact, it is necessary to carefully monitor those who do not access the diagnostic level. To emphasize the importance of a proper screening program, it will be helpful to strengthen the computerized data collection system and create an information network between audiologists, pediatricians and families.

Long-Term Results of Tinnitus Retraining Therapy in Patients Who Failed to Complete the Program.

Purpose: We aimed to evaluate the results of Tinnitus Retraining Therapy (TRT) in patients who did not complete the program. Methods: We divided 90 patients who failed to complete the TRT program were into 3 groups: 36 patients who only completed the first phase of the TRT program (Missing group; M), 34 patients who attended counselling for less than 6 months (Noncompliant group; NC) and 20 patients who attended counselling for more than 6 months but did not complete the TRT program (Compliant group; C). The Tinnitus Handicap Inventory (THI), tinnitus Visual Analogue Scales (VAS) and a questionnaire regarding the reasons for dropout were obtained through a telephone survey. Results: Telephonic THI and VAS scores were significantly lower than the initial scores in the M and C groups but not in the NC group. Patients who were unsure about the effectiveness of TRT were prevalent in the NC group, and the poorest long-term THI results were registered in those patients. Conclusions: A fundamental cause of very poor TRT results was when patients were unsure about TRT. On the other hand, a single counselling session could be effective in reducing tinnitus annoyance in patients who accepted the TRT approach and trusted its efficacy.

Sensorineural Hearing Loss in Newborns Hospitalized in Neonatal Intensive Care Unit: An Observational Study.

Children hospitalized in Neonatal Intensive Care Units (NICU) present an increased risk for Sensorineural Hearing Loss (SNHL) due to prematurity, hypoxia-ischemia, hyperventilation, low birth weight and the use of ototoxic drugs. The aim of this study was to assess the prevalence of SNHL in newborns hospitalized in a NICU using Transient Evoked Otoacoustic Emissions (TEOAE) and Automated Auditory Brainstem Responses (A-ABR) and analyze the associated risk factors. A sample of 153 newborns hospitalized in NICU underwent TEOAE, A-ABR and clinical ABR to evaluate the presence of hearing deficits. Prevalence of SNHL was calculated and odds ratio for specific risk factors was measured. One-hundred fifteen babies (86.7%) presented normal hearing at TEOAE and A-ABR. Fifteen children had a REFER response at TEOAE and a PASS response at A-ABR. Twenty-five children (16.3%) had a REFER A-ABR and were addressed to clinical ABR. A diagnosis of SNHL was made in 12 (7.8%) newborns. An increased risk of SNHL was observed in preterm children <28 weeks (p=0.0135), in children with neurological disorders (p=0.02), that underwent surgery (p=0.0002), affected from premature retinopathy (p=0.0006), craniofacial malformation (p=0.007) and that had sepsis (p=0.04). Additional risk factors for SNHL in our sample were a maternal disease during pregnancy (p=0.0011), cesarean delivery (p<0.0001) and a twin pregnancy (p<0.0001). SNHL in newborns is correlated with hospitalization in NICU. An accurate hearing screening associated to a rigorous clinical medical collection of data is necessary to promptly identify cases of SNHL in children with a special attention to those hospitalized in NICU and plan proper intervention.

Universal newborn hearing screening in Umbria region, Italy.

OBJECTIVE: In Italy, universal newborn hearing screening (UNHS) was first introduced in some regions from 1997. Umbria Region has launched a UNHS program in all hospitals, which has been implemented throughout the region since July 2010. Before UNHS implementation in Umbria region, the average age of identification of congenital hearing loss was around 32 months of age with an average age of initial amplification treatment at least 2 months later. The coverage rate of newborn screening was only 34.4% in 2006. The aim of this study was to examine the results of this program and its evolution in the first 2.5 years since implementation in our region. METHODS: Since July 2010, all 11 birth centers and hospitals in Umbria region have been involved in a UNHS program. The screening involves the automated otoacoustic emissions (AOAE) test and automatic auditory brain stem response (AABR) audiometry. The number of screening stages and tests used were different depending on whether the infants had audiological risk factors or not. RESULTS: A total of 20,841 babies were born in the hospitals involved of whom 20,051 were well born babies (WB), while 790 babies (3.8%) presented identified audiological risk factors (BRF). The overall coverage rate in the study period was 93.8%. The prevalence of hearing loss was 2‰ for WB infants and 4.3% for BRF. Mean age at diagnosis was 5.31±3.95 and 11.28±7.73 months in the WB and BRF groups, respectively. CONCLUSIONS: UNHS has allowed us to substantially increase the coverage rates and decrease the mean age at diagnosis and subsequent treatment. The identification of audiological risk factors is very important for adequate screening and follow-up. However the Joint Committee on Infant Hearing 2007 quality indicators and benchmarks for screening have not yet been fully achieved and there is still scope for some improvement. This could be achieved with a closer cooperation among institutions, parents, pediatricians, and ENT doctors.

Impact of identifying factors which trigger bothersome tinnitus on the treatment outcome in tinnitus retraining therapy.

The aim of this work was to ascertain any differences in the effectiveness of rehabilitation therapy in relation to the presence or absence of a known negative reinforcement responsible for the tinnitus-related pathology. Between 1 January 2001 and 31 December 2008, we recruited 294 subjects suffering from incapacitating tinnitus and/or hyperacusis. The patients underwent tinnitus retraining therapy (TRT) according to the methods described by Jastreboff and Hazell [Tinnitus Retraining Therapy: Implementing the Neurophysiological Model. Cambridge, Cambridge University Press, 2004, pp 121-133]. We clinically assessed the presence or absence of known phenomena of associative learning, regarding the presence of adverse events temporally correlated with tinnitus and the treatment outcome. The separate analysis of the 2 subgroups shows a statistically significant difference in the improvement rate between the group with a known triggering factor and the group without a triggering factor, with a preponderance of the former with a 91% improvement rate versus approximately 56% for the latter. In our study, the inability to identify factors triggering bothersome tinnitus negatively affected the treatment outcome in TRT.

Cochlear implantation in children with "CHARGE syndrome": surgical options and outcomes.

CHARGE syndrome is a rare, polymalformative disease, representing one of the major causes of associated blindness and deafness. Bilateral, severe-profound, sensorineural hearing loss is common in CHARGE children. Aim of this study is to present our results in children with "CHARGE syndrome" submitted to cochlear implantation (CI). The frequency of anatomic anomalies, possible variations in the surgical technique of CI, and the audiological/rehabilitative benefits attained in our patients are reported. we submitted 5 children affected by CHARGE syndrome with profound, bilateral, sensorineural hearing loss to CI. Otoacoustic emissions, auditory brainstem response, acoustic impedance testing, cranial computed tomography and magnetic resonance were carried out preoperatively in all children. CI was performed using the mastoidotomy-posterior tympanotomy approach in two cases, and the suprameatal approach in three children. Infant toddler-meaningful auditory integration scale was used to evaluate kid's audiological performance before and after CI. Intra-operatory findings and postsurgical complications were evaluated. Among our patients, intraoperative anatomical malformations were cochlear hypoplasia (100 %), ossicles malformations (100 %), semicircular canals aplasia (100 %), oval window atresia (60 %), round window atresia (40 %), widening of the aqueduct of the vestibule (20 %), and aberrant course of the facial nerve (20 %). No intra- or postoperative complication was recorded in relation to implant positioning. After a follow-up ranging from 1 to 4.5 years, only 2/5 patients used oral language as the sole mean of communication, 1 started utilizing oral language as the main mean of communication, while 2 patients did not develop any linguistic ability. In conclusion, CI in patients with CHARGE association is feasible and, despite results variability, it should be carried out in CHARGE children with severe hearing loss as soon as possible. Although the selection of a specific surgical technique does not seem to influence the audiological outcome, the suprameatal access is valuable when important surgical landmarks (i.e. lateral semicircular canal and incus) are absent.

Personal experience with tinnitus retraining therapy.

We present the results of tinnitus retraining therapy (TRT) in a group of patients suffering from tinnitus and/or hyperacusia. Based on the scores from a specific questionnaire and the Tinnitus Handicap Inventory (THI), the patients were classified into five categories and began therapy according to Jastreboff's criteria. Depending on the individual case, therapy envisaged counselling sessions, ambient sound enrichment, sound generators and hearing aids. At the end of the 18-month period, therapeutic success was observed in 79% of the patients. The initial numerical values of the scale of the symptoms and the THI seem predictive of treatment outcome. The use of instruments (sound generators) increases the success rate, but the study also demonstrates the effectiveness of counselling and ambient sound enrichment. Failures mainly involved patients with hypacusia who refused to wear hearing aids, as this influenced the effectiveness of ambient sound enrichment and counselling. Paralleling the data in the literature, the results demonstrate the effectiveness of TRT, which cannot be attributed to a placebo effect given the extended duration of treatment.

Determining subjective visual vertical: dynamic versus static testing.

OBJECTIVE: Comparative evaluation of two tests that, as a visual reference, respectively use a static light source (static testing) and a dynamic one (dynamic testing) to analyze subjective visual vertical in patients with unilateral vestibular dysfunction. STUDY DESIGN: Prospective study. SETTING: Otolaryngology and Cervicofacial Surgery Division, Department of Medical-Surgical Specialization, University of Perugia, Italy. PATIENTS: Forty-two patients with unilateral vestibular dysfunction. INTERVENTIONS: Determination of subjective visual vertical 1 to 2 days (first test) after the onset of vertigo. Repetition of 8 to 10 days (second test) and 90 days (third test) after onset. MAIN OUTCOME MEASURES: Test sensitivity and specificity. RESULTS: The sensitivity of the static test was 85.7, 73.3, and 59.5% during the first, second, and third test, respectively. The dynamic test showed a sensitivity of 91.3, 86.8, and 78.1%, respectively. Specificity was 100% for the static test and 96.7% for the dynamic test. CONCLUSION: The dynamic test proved to be more useful in the weeks after the onset of dysfunction. Given the greater sensitivity of this test, the reduction in perception error time makes it more suitable for revealing false negatives that emerged when using the static test alone.

Study of vestibular evoked myogenic potentials in unilateral vestibulopathy: Otolithic versus canal function testing.

OBJECTIVE: The study provides a qualitative evaluation of unilateral vestibulopathy by comparing otolithic and canal function, to establish possible relationships between the type of dysfunction observed and the evolving clinical pictures associated with it. STUDY DESIGN: Retrospective study of a series of cases. SETTING: Department of Medical-Surgical Specialization, Otolaryngology and Cervicofacial Surgery Division, University of Perugia, Perugia, Italy. PATIENTS: Twenty patients whose medical history showed at least one episode corresponding to the clinical parameters of acute vestibulopathy. INTERVENTIONS: Study of vestibular function by recording VEMPs and repeating canal function testing at least 6 months after the first episode of vertigo. MAIN OUTCOME MEASURES: Relationship between the type of vestibulopathy (canal and otolithic) and the clinical pictures observed. RESULTS: Paroxysmal positional vertigo, observed in 4 patients, was correlated with the presence of vestibular evoked myogenic potentials (VEMPs) and the absence of an ipsilateral canal response in all cases (100%). Persistent dizziness was observed in nine patients, and VEMPs were absent in all of them (100%); three (33.3%) showed the recovery of previously absent canal function. Comparison of responses in six patients with recurrent acute vestibulopathy showed persistent and complete loss of canal function in five cases (83.3%), whereas impairment of otolithic response was less constant (40%). CONCLUSION: The combined VEMPs-canal test study shows predictive value regarding certain evolving clinical pictures of vestibulopathy. The absence of VEMPs confirms the role of otolithic dysfunction in the onset of dizziness. Likewise, it suggests that a vestibular origin of these disorders should be considered in cases that have shown aspecific symptoms since onset, without frank vertigo and with normal vestibular response to canal function testing.