The influence of auditory selective attention on linguistic outcomes in deaf and hard of hearing children with cochlear implants.

PURPOSE: Auditory selective attention (ASA) is crucial to focus on significant auditory stimuli without being distracted by irrelevant auditory signals and plays an important role in language development. The present study aimed to investigate the unique contribution of ASA to the linguistic levels achieved by a group of cochlear implanted (CI) children. METHODS: Thirty-four CI children with a median age of 10.05 years were tested using both the "Batteria per la Valutazione dell'Attenzione Uditiva e della Memoria di Lavoro Fonologica nell'età evolutiva-VAUM-ELF" to assess their ASA skills, and two Italian standardized tests to measure lexical and morphosyntactic skills. A regression analysis, including demographic and audiological variables, was conducted to assess the unique contribution of ASA to language skills. RESULTS: The percentages of CI children with adequate ASA performances ranged from 50 to 29.4%. Bilateral CI children performed better than their monolateral peers. ASA skills contributed significantly to linguistic skills, accounting alone for the 25% of the observed variance. CONCLUSIONS: The present findings are clinically relevant as they highlight the importance to assess ASA skills as early as possible, reflecting their important role in language development. Using simple clinical tools, ASA skills could be studied at early developmental stages. This may provide additional information to outcomes from traditional auditory tests and may allow us to implement specific training programs that could positively contribute to the development of neural mechanisms of ASA and, consequently, induce improvements in language skills.

The Spectrum of Neurological and Sensory Abnormalities in Gaucher Disease Patients: A Multidisciplinary Study (SENOPRO).

Gaucher disease (GD) has been increasingly recognized as a continuum of phenotypes with variable neurological and sensory involvement. No study has yet specifically explored the spectrum of neuropsychiatric and sensory abnormalities in GD patients through a multidisciplinary approach. Abnormalities involving the nervous system, including sensory abnormalities, cognitive disturbances, and psychiatric comorbidities, have been identified in GD1 and GD3 patients. In this prospective study, named SENOPRO, we performed neurological, neuroradiological, neuropsychological, ophthalmological, and hearing assessments in 22 GD patients: 19 GD1 and 3 GD3. First, we highlighted a high rate of parkinsonian motor and non-motor symptoms (including high rates of excessive daytime sleepiness), especially in GD1 patients harboring severe glucocerebrosidase variants. Secondly, neuropsychological evaluations revealed a high prevalence of cognitive impairment and psychiatric disturbances, both in patients initially classified as GD1 and GD3. Thirdly, hippocampal brain volume reduction was associated with impaired short- and long-term performance in an episodic memory test. Fourthly, audiometric assessment showed an impaired speech perception in noise in the majority of patients, indicative of an impaired central processing of hearing, associated with high rates of slight hearing loss both in GD1 and GD3 patients. Finally, relevant structural and functional abnormalities along the visual system were found both in GD1 and GD3 patients by means of visual evoked potentials and optical coherence tomography. Overall, our findings support the concept of GD as a spectrum of disease subtypes, and support the importance of in-depth periodic monitoring of cognitive and motor performances, mood, sleep patterns, and sensory abnormalities in all patients with GD, independently from the patient's initial classification.

Hyperacusis in Children: A Preliminary Study on the Effects of Hypersensitivity to Sound on Speech and Language.

There is a growing awareness that children may experience hyperacusis, a condition that is often associated with behavioral and developmental disorders. This preliminary study was aimed to investigate the effects of hyperacusis alone on various components of speech and language in children without developmental disorders. This study was conducted on 109 children aged between 4 and 7 years attending kindergarten and primary school. Hyperacusis was assessed through behavioral observation of children and questionnaires for parents. Different components of speech and language were assessed through specific tests. Hyperacusis was diagnosed in fifteen children (13.8%); ten (66.7%) were attending primary school and five (33.3%) kindergarten. A significant difference between children with and without hyperacusis was found for tests evaluating the average number of words in a sentence and phonemic fluency; older children appeared to have more difficulties. Several differences in education profiles were found: parents of children with hyperacusis spent less time with their children compared to parents of children without hyperacusis. Our preliminary results suggest some difficulties in lexical access and the use of shorter sentences by children with hypersensitivity to sound; however, the small size of our sample and the largely unknown interactions between hyperacusis and developmental disorders suggest caution when interpreting these results. Further studies on larger samples are necessary to gain additional knowledge on the effects of hyperacusis on speech and language in children without developmental disorders.

Somatic Tinnitus.

Modulation of tinnitus characteristics such as pitch and loudness has been extensively described following movements of the head, neck and limbs, vertical or horizontal eye gaze, pressure on myofascial trigger points, cutaneous stimulation of the hands, electrical stimulation of the median nerve, and transcranial direct current stimulation. Modulation of tinnitus follows complex interactions between auditory and somatosensory afferents and can be favored by underlying somatic disorders. When tinnitus appears to be preceded or strictly linked to a somatic disorder, and therefore related to problems of the musculoskeletal system rather than of the ear, it is defined somatic tinnitus. A correct diagnosis and treatment of somatic disorders underlying tinnitus play a central role for a correct management of somatic tinnitus. However, the identification of somatic tinnitus may be complex in some cases. In this paper, after a general review of the current evidences for somatic tinnitus available in the literature, we present and discuss some cases of patients in which somatic modulation of tinnitus played a role-although different from case to case-in their tinnitus, describing the diagnostic and therapeutic approaches followed in each individual case and the results obtained, also highlighting unexpected findings and pitfalls that may be encountered when approaching somatic tinnitus patients.

Vestibular Schwannoma and Ipsilateral Endolymphatic Hydrops: An Unusual Association.

Vestibular schwannoma and endolymphatic hydrops are two conditions that may present with similar audio-vestibular symptoms. The association of the two in the same patient represents an unusual finding that may lead clinicians to errors and delays in diagnosis and clinical management of affected subjects. We discuss the case of a patient with an intrameatal vestibular schwannoma reporting symptoms suggestive for ipsilateral endolymphatic hydrops. The patient presented with fluctuating hearing loss, tinnitus, and acute rotatory vertigo episodes, and underwent a full audiological evaluation and imaging of the brain with contrast-enhanced Magnetic Resonance Imaging. Clinical audio-vestibular and radiological examination confirmed the presence of coexisting vestibular schwannoma and endolymphatic hydrops. Hydrops was treated pharmacologically; vestibular schwannoma was monitored over time with a wait and scan protocol through conventional MRI. The association of vestibular schwannoma and endolymphatic hydrops is rare, but represents a possible finding in clinical practice. It is therefore recommended investigating the presence of inner ear disorders in patients with vestibular schwannoma and, similarly, to exclude the presence of this condition in patients with symptoms typical of inner ear disorders.

Somatosensory Tinnitus: Correlation between Cranio-Cervico-Mandibular Disorder History and Somatic Modulation.

In a subpopulation of patients, tinnitus can be modulated by movements of the jaw or head and neck due to complex somatosensory-auditory interactions. In some of these subjects, tinnitus could be related to an underlying temporomandibular (TMJ) or craniocervical (NECK) dysfunction that, if correctly identified, could streamline treatment and increase chances of tinnitus improvement. However, it is still unclear whether somatic modulation of tinnitus could be used as a screening tool for identifying such patients. In this study, we included 310 tinnitus patients with normal hearing, no psychiatric comorbidities, and a positive history of TMJ and/or NECK dysfunction and/or a positive modulation of tinnitus to evaluate the characteristics of somatic modulation, investigate the relationship between positive history and positive modulation, and identify factors most strongly associated with somatic modulation. Tinnitus modulation was present in 79.67% of the patients. We found a significant association within the same subjects between a positive history and a positive tinnitus modulation for the same region, mainly for TMJ in unilateral tinnitus patients and for TMJ + NECK in bilateral tinnitus patients. A strong correlation between history and modulation in the same somatic region within the same subgroups of subjects was also identified. Most TMJ maneuvers resulted in an increased loudness, while NECK maneuvers showed an increase in tinnitus loudness in about 59% of cases. High-pitched tinnitus and male gender were associated with a higher prevalence of modulation; no differences were found for tinnitus onset, Tinnitus Handicap Inventory score, and age. In this paper, we report a strong association between history and modulation for the same regions within the same patients; such an association should always be investigated to improve chances of a correct diagnosis of somatosensory tinnitus.

Tinnitus Holistic Simplified Classification (THoSC): A New Assessment for Subjective Tinnitus, With Diagnostic and Therapeutic Implications.

OBJECTIVE: One of the most debated topics in tinnitus is its standard and practical classification. The most popular classification distinguishes subjective from objective tinnitus. Other classifications are based on different features. On the whole, they seem incomplete, and the diagnostic and therapeutic algorithms are often difficult for practical purposes. The aim of this work is to develop a new diagnostic and therapeutic algorithm. METHODS: Our model is based on 10 years of experience. In particular, the starting point is the data retrieved from 212 consecutive patients in our Tinnitus Unit between May and December 2013: We found a clear auditory disorder in 74.5% of the population, muscolo-skeletal disorders and/or trigeminal disease in 57.1%, and psychiatric comorbidities in 43.8%. Different features coexisted in 59.9% of the population. RESULTS: Following such data we propose the Tinnitus Holistic Simplified Classification, which takes into account the different tinnitogenic mechanisms and the interactions between them. It differentiates tinnitus that arises from: (1) auditory alterations (Auditory Tinnitus), (2) complex auditory-somatosensory interactions (Somatosensory Tinnitus), (3) psychopathological-auditory interactions (Psychopathology-related Tinnitus), and (4) 2 or all of the previous mechanisms (Combined Tinnitus). CONCLUSIONS: In our opinion this classification provides an accurate and easy tailored path to manage tinnitus patients.

Sophono Alpha System and subtotal petrosectomy with external auditory canal blind sac closure.

Recently, a new acoustic device, the so-called Sophono Alpha System, has been introduced into clinical practice. The aim of this study was to assess Sophono Alpha System hearing aids in ten patients suffering from recurrent chronic middle ear disease who underwent subtotal petrosectomy. Presence of mixed hearing loss with bone conduction thresholds better than or equal to 45 dB was present in each patient. Audiometric tests were performed before and after Sophono implantation and using a conventional bone conduction hearing aid (hearing glasses). Speech audiometry data (speech recognition threshold and word recognition score) were also collected. Speech recognition threshold in dB and percentage of word recognition score at 65 dB were subsequently calculated. After implantation and activation of the Sophono Alpha System, audiological data showed an average air conduction value of 42.1 dB. By comparing this data with the values of air conduction following subtotal petrosectomy, an average acoustic improvement of 29.7 dB could be calculated. The hearing results showed significantly better outcomes of Sophono Alpha System vs. conventional bone conduction aid. Indications to MRI use in patients undergoing Sophono Alpha System implantation are also provided.

Comparative Analysis of Intellectual Quotient in Developmental Population with Severe Hearing Loss: Hearing Aids vs. Cochlear Implant Users.

The development of language, memory and intellectual functions is linked to normal hearing and correct sounds interpretation. Hearing loss (HL), especially in its severe form, negatively affects the development of these functions. This prospective study aimed at comparing the Intelligent Quotients (IQ) of children with cochlear implants (CI) with the ones of people wearing hearing aids (HA) after one year of hearing rehabilitation. 21 subjects with severe/profound bilateral hearing loss (deafness) were included in this study. Eleven children with congenital profound HL underwent CI and ten children with moderate to severe HL (congenital and acquired) were rehabilitated by HA. Children's IQs were assessed at enrolment (T0) and 12 months after hearing aids/CI use plus speech therapy. Statistical analyses were performed to analyze the data within and between groups. Comparison of IQs showed no statistically significant differences between CI and HA none at T0 and T1. The subtests showed lower scores in verbal comprehension and process speed index in patients treated with HA when compared to CI. This study showed that auditory rehabilitation can support the normal development of cognitive function in children between six and eight years of age. The use of the correct hearing aids based on the patient's hearing thresholds is important to maximize the rehabilitation outcomes. Due to the small sample size, although stratified for age, our results must be considered preliminary and further analyses on larger samples are needed to confirm our data.

Sudden sensorineural hearing loss: a vascular cause? Analysis of prothrombotic risk factors in head and neck.

OBJECTIVE: This aim of this study was to determine the prevalence of thrombophilic risk factors in sudden sensorineural hearing loss, central retinal vein occlusion, and stroke associated with small vessel disease, with the purpose of investigating and reinforcing the vascular hypothesis in the pathogenesis of sudden sensorineural hearing loss. DESIGN: Case-control study. Genetic and acquired risk factors of these three groups were compared with healthy controls. STUDY SAMPLE: Forty-nine, 60, and 101 patients affected respectively by sudden sensorineural hearing loss, central retinal vein occlusion, or stroke associated with small vessel disease, enrolled during a three-year period were compared with 210 healthy controls. RESULTS: The frequency of hyperhomocysteinemia (homocysteine ≥ 15 µmol/L) was higher in each disease group than in controls. A statically significant, albeit weak, correlation between the MTHFR C677T mutation and hyperhomocysteinemia was found in all three diseases. CONCLUSIONS: Hyperhomocysteinemia proved to be a risk factor for sudden sensorineural hearing loss. Based on these results, we propose to analyse homocysteine in sudden sensorineural hearing loss patients and, if its values are high, to evaluate the presence of MTHFR C677T mutation.

Audiological and radiological characteristics of a family with T961G mitochondrial mutation.

OBJECTIVE: The aim of this study was to describe audiological and radiological characteristics, and other secondary aspects, in a family carrying a T961G mutation in the 12S rRNA mitochondrial gene. DESIGN: Case report. STUDY SAMPLE: Six members of a family participated in an audiological evaluation that included pure-tone audiometry, immittance tests, auditory brainstem responses (ABR), and otoacoustic emissions (OAE). The radiological evaluation was conducted through temporal bone CT scans using a Toshiba 16 channels Aquilon Spirale. Neuropsychiatric evaluation was also administered. RESULTS: Three participants were diagnosed with severe sensorineural hearing loss of cochlear origin and cochlear malformations visible in CT scans. One participant had a mild mixed-hearing loss and no cochlear malformations. Two participants had normal audiological and radiological findings. CONCLUSIONS: We believe our study can provide helpful insight on the clinical findings of a rare mutation, of which few data have been presented in literature.

Hearing attention and quality of listening in children with unilateral hearing loss with and without hearing aid.

Objective: To analyse hearing attention and quality of listening in a cohort of children affected by moderate to severe unilateral hearing loss, comparing a group of hearing aid users to children with no hearing aid. Methods: Twenty-four children (12 hearing aid users, and 12 without hearing rehabilitation) underwent audiological evaluation with speech audiometry in quiet and noise, hearing attention tests, and the speech, spatial and quality of hearing questionnaire in the version for parents. Results: Concerning speech audiometry in noise, in the most difficult condition no one in the unaided group achieved a normal speech recognition threshold score (0% vs 11.6 ± 2.7% in the aided group). The selective hearing attention and shifting tests showed fewer errors in the aided group vs. the non-aided group. The questionnaire showed a significant difference between the quality of listening perceived by the parents of the two groups. The mean total scores were 152.8 ± 2.7 in the aided group and 116.1 ± 2.6 in the non-aided group (p > 0.001). Conclusions: Children with unilateral hearing loss without hearing rehabilitation show a lower quality of hearing, especially with regards to divided auditory attention. Hearing rehabilitation should be proposed as soon as possible in children with moderate to severe unilateral hearing loss.

Hearing Loss After Cisplatin-based Chemoradiotherapy for Locally Advanced Head and Neck Cancer: A Prospective Single-institution Study.

BACKGROUND/AIM: A single-institution prospective study was conducted to evaluate hearing loss rate after intensity modulated radiotherapy with concomitant cisplatin-based chemotherapy (CRT) for locally advanced head and neck cancer and identify cochlear dosimetric parameters associated with hearing loss risk. PATIENTS AND METHODS: Hearing assessment, patients' characteristics, tumor-related variables, and cochlear quantitative dosimetric factors for adults with locally advanced head and neck cancer treated with CRT were prospectively collected. Each patient repeated audiometry at baseline (pre-CRT), 1 month after CRT, and then every 3 to 6 months. For each cochlea minimum dose (Dmin), mean dose (Dmean), and maximum dose (Dmax) were extracted from treatment plans. Logistic analysis was used for multivariate modeling. The relation between cochlear dosimetric factors and significant hearing loss was also analyzed with receiver operating characteristic (ROC) curves. RESULTS: Between January 2016 and December 2018, 35 patients (70 cochleae) were included. Most patients (n=29; 82.9%) had primary cancer in a low-risk region (oral cavity, oropharynx, larynx). All patients completed the programmed CRT. During follow-up, significant hearing loss was recorded in 13 cases (37.1%). The ROC areas for significant hearing loss in relation to Dmin, Dmean, and Dmax were 0.70, 0.66, and 0.66, respectively. A dose-dependent relationship was noted between cochlear Dmin and significant hearing loss. CONCLUSION: Dmin <14.4 Gy is associated with reduced rates of significant hearing loss after concomitant cisplatin-based CRT in patients with locally advanced head and neck cancer.

Benefits of Parent Training in the Rehabilitation of Deaf or Hard of Hearing Children of Hearing Parents: A Systematic Review.

The present study is a systematic review on the effectiveness of Parent Training (PT) and coaching in deaf and hard of hearing (DHH) rehabilitation programs which reviews and synthesizes the existing body of evidence to assess the benefits of these programs in enhancing parents' sensitivity, responsivity and promoting language development in DHH children during the first years after HA fitting or CI activation. Five published studies met the Population, Intervention, Comparison and Outcomes (PICO) inclusion criteria and were eligible to be included, but heterogeneity in terms of the study design, interventions and outcomes did not allow for performing a meta-analysis. All included studies shared the view that a parent's learning is a circular (rather than frontal) process, and the results appear promising in terms of enhancing parents' responsiveness and promoting DHH child language development. Nevertheless, the available evidence was judged to not be robust enough due to limitations in the studies' designs. Further high-quality evidence is needed to evaluate the true degree of clinical value and the cost effectiveness of PT programs aimed at increasing parents' responsiveness to their DHH children.

The safety of sublingual immunotherapy, can the rare systemic reactions be prevented?

Introduction: The safety of subcutaneous immunotherapy (SCIT), and particularly the dramatic issue of fatal reactions, has been an obstacle that limited the implementation of a therapy with unique characteristics of action on the causes of allergy. The introduction of sublingual immunotherapy (SLIT) was aimed at solving safety problems while maintaining clinical efficacy.Areas covered: For more than 20 years, SLIT has been based on allergen extracts in drops at low average doses. As evidenced by meta-analyses, the typical adverse events (AE) have consisted of local reactions in the mouth and throat. Unlike the injection route, no correlation was observed between the administered dose and AEs. The development of SLIT products in tablets, based on higher doses than drops, has somewhat changed the concept of SLIT safety. Although large trials, performed to obtain regulatory agency approval, have shown overall high safety, rare anaphylactic reactions have been described.Expert opinion: SLIT is globally safe, and no fatal reactions have ever been reported, but with currently available high biological potency products it is necessary to follow prudential rules, such as the administration of the first dose under medical supervision and the thorough education of patients to avoid taking of higher doses than recommended.

Hyperacusis in Children with Attention Deficit Hyperactivity Disorder: A Preliminary Study.

The association between hyperacusis and developmental disorders such as autism spectrum disorders has been extensively reported in the literature; however, the specific prevalence of hyperacusis in attention deficit hyperactivity disorder (ADHD) has never been investigated. In this preliminary study, we evaluated the presence of hyperacusis in a small sample of children affected by ADHD compared to a control group of healthy children. Thirty normal hearing children with a diagnosis of ADHD and 30 children matched for sex and age were enrolled in the study. All children underwent audiological and multidisciplinary neuropsychiatric evaluation. Hearing was assessed using pure tone audiometry and immittance test; ADHD was diagnosed following the Diagnostic and Statistical Manual of Mental Disorder criteria. Hyperacusis was assessed through the administration of a questionnaire to parents and an interview with children. Hyperacusis was diagnosed in 11 children (36.7%) in the study group and in four children (13.3%) in the control group; this difference was statistically significant (p = 0.03). The preliminary results of this study suggest a higher presence of hyperacusis in children with attention deficit hyperactivity disorder compared to control children. More studies on larger samples are necessary to confirm these results.

Isolated auditory neuropathy at birth in congenital cytomegalovirus infection.

BACKGROUND: Congenital cytomegalovirus (cCMV) infection is the most frequent non-genetic cause of sensorineural hearing-loss (SNHL) (i.e., hearing loss due to a cochlear and/or auditory nerve damage). It is widely accepted that SNHL at birth, when associated to cCMV symptomatic infection involving the central nervous system, benefits from antiviral therapy started in the neonatal period. Conversely, there is no consensus for antiviral treatment in congenitally infected infants diagnosed with isolated SNHL (i.e., SNHL in an otherwise asymptomatic infant) at birth. Our aim was to assess the frequency and the auditory outcome of isolated SNHL at birth due to auditory neuropathy (AN) (i.e., SNHL in a patient with normal cochlear function and auditory nerve dysfunction) in infants with cCMV infection. METHODS: We retrospectively reviewed the clinical history of 60 infants, born at term, with cCMV asymptomatic infection, without additional risk factors for SNHL, and exhibiting bilateral "pass" otoacustic emissions (OAE). None of them underwent antiviral therapy. Hearing thresholds were assessed by means of Auditory Brainstem Responses (ABR). AN affected children were followed up until possible normalization of the hearing thresholds or definitive diagnosis of AN. Each infant diagnosed with monolateral or bilateral AN was classified according to the worst ear threshold. RESULTS: In our population, the first ABR was performed at a mean age of 5.00 ± 2.79 (SD) months and AN was diagnosed in 16/60 (26.67%) infants; in 4 infants the AN was defined as mild (4/4 monolateral), moderate in 11 (5/11 bilateral), and severe in 1 (bilateral). The mean age at first ABR was 3.69 ± 2.80 (SD) months in the 16 babies with AN and 5.48 ± 2.66 (SD) months in the 44 infants with normal hearing (p = 0.007). All AN cases spontaneously recovered a normal auditory threshold over time. The mean length of the audiological follow-up was 32.44 ± 17.58 (SD) months (range 5-60 months). CONCLUSION: A delayed maturation of the auditory pathways should be considered when a mild/moderate isolated AN at birth is detected in cCMV infected infants. Prospective studies conducted on larger populations, and with a longer audiological follow-up, are needed to confirm our findings.

Transcutaneous bone conductive implants in patients with conductive/mixed hearing loss: audiological outcomes in noise condition.

BACKGROUND: Recently, the use of transcutaneous bone conduction implants (BCIs) has been increased. However, scarce data about BCI hearing recovery in noise conditions have been reported. OBJECTIVES: To investigate the audiological benefits obtained with transcutaneous BCI-Sophono Alpha System in noise conditions. To evaluate post-implantation clinical outcomes and patient satisfaction levels. MATERIALS AND METHODS: Fourteen patients suffering from conductive or mixed hearing loss implanted with the Sophono Alpha System were evaluated. Patients underwent physical examination, free-field pure-tone and speech audiometry both in unaided and aided conditions. The matrix sentence test was employed with fixed noise at 65 dB, and with a fluctuating primary signal, in three different conditions of noise presentations (S0/N0, S0/Ncontra, S0/Nipsi). RESULTS: Hearing gain, expressed as the difference between pre-implant AC and post-implant SAS free field, was on average 26.7 dB. The unaided speech recognition score in quiet conditions had a mean value of 64.6%, and improved after SAS implantation, achieving mean values of 98.2%. SRT50 with the matrix sentence test improved in all three conditions of noise presentation. CONCLUSIONS: Sophono Alpha System devices represent a valid treatment option for hearing rehabilitation of patients with conductive or mixed hearing loss. The audiological results regarding hearing gain in noise conditions were good.

Universal newborn hearing screening in the Lazio region, Italy.

BACKGROUND: The introduction of Universal Newborn Hearing Screening (UNHS) programs has drastically contributed to the early diagnosis of hearing loss in children, allowing prompt intervention with significant results on speech and language development in affected children. UNHS in the Lazio region has been initially deliberated in 2012; however, the program has been performed on a universal basis only from 2015. The aim of this retrospective study is to present and discuss the preliminary results of the UNHS program in the Lazio region for the year 2016, highlighting the strengths and weaknesses of the program. METHODS: Data from screening facilities in the Lazio region for year 2016 were retrospectively analyzed. Data for Level I centers were supplied by the Lazio regional offices; data for Level II and III centers were provided by units that participated to the study. RESULTS: During 2016, a total of 44,805 babies were born in the Lazio region. First stage screening was performed on 41,821 children in 37 different birth centers, with a coverage rate of 93.3%. Of these, 38.977 (93.2%) obtained a "pass" response; children with a "refer" result in at least one ear were 2844 (6.8%). Data from Level II facilities are incomplete due to missing reporting, one of the key issues in Lazio UNHS. Third stage evaluation was performed on 365 children in the three level III centers of the region, allowing identification of 70 children with unilateral (40%) or bilateral (60%) hearing loss, with a prevalence of 1.6/1000. CONCLUSIONS: The analysis of 2016 UNHS in the Lazio region allowed identification of several strengths and weaknesses of the initial phase of the program. The strengths include a correct spread and monitoring of UNHS among Level I facilities, with an adequate coverage rate, and the proper execution of audiological monitoring and diagnosis among Level III facilities. Weakness, instead, mainly consisted in lack of an efficient and automated central process for collecting, monitoring and reporting of data and information.