Audiological Performance of ADHEAR Systems in Simulated Conductive Hearing Loss: A Case Series with a Review of the Existing Literature.

A new non-invasive adhesive bone conduction hearing device (ABCD) has been proposed as an alternative solution for reversible bilateral conductive hearing loss in recurrent or long-lasting forms of otitis media with effusion (OME) in children that cannot undergo surgical treatment. Our aim was to assess the effectiveness of ABCD in children with OME. Twelve normal-hearing Italian-speaking volunteers, in whom a conductive hearing loss was simulated, participated in the study. The free-field average hearing threshold was determined and, to evaluate binaural hearing skills, loudness summation and the squelch effect were assessed. Five conditions were tested: (1) unaided without earplugs, (2) unaided with bilateral earplugs, (3) aided right ear with bilateral earplugs, (4) aided left ear with bilateral earplugs, and (5) bilateral aid with bilateral earplugs. Post-hoc analysis showed a significant statistical difference between plugged, unplugged, and each aided condition. The main results were a better loudness summation and a substantial improvement of the squelch effect in the bilaterally aided. Our results suggest that ABCD is a valid treatment for patients with conductive hearing loss that cannot undergo bone conduction implant surgery. It is also important to consider bilateral aids in order to deal with situations in which binaural hearing is fundamental.

Modeling Postnatal Hearing Case Finding Within the Italian National Health System.

Objective: Despite the successful implementation of newborn hearing screening (NHS), a debate is emerging as to what should be the best means of enabling timely diagnosis and intervention for preschoolers with educationally significant sensorineural or conductive hearing impairment (HI) missed at the time of NHS or occurred after birth. Our study aims to document the proportion and characteristics of HIs diagnosed in children in need of audiologic assessment, in order to outline the optimization areas of an operational framework for auditory surveillance during preschool age. Method: The referral routes and outcomes of 730 audiological assessments performed in 3 years within the framework of the early hearing identification program in Trieste (Italy) were retrospectively analyzed. Results: Among 570/595 completed evaluations, an HI was diagnosed in 114 children, 73.7% of which presenting an exclusively conductive HI due to middle ear effusion. HIs were found in 36/141 who failed NHS, and 60/385 preschoolers who were referred by the primary care pediatrician's surveillance activity during well-child visits, with diagnostic yield of 25.5 and 15.5%, respectively. Conclusion: Ongoing preschool surveillance in primary care setting integrated into a NHS program is feasible to conduct and may effectively identify HIs that missed NHS or were related with a risk factor. New triage instruments and protocols for immediate audiology referral could allow to obtain the diagnosis of educationally significant conductive and sensorineural HIs ahead of the development concern and in the same way reduce patient mobility, thus optimizing timing efficiency and economic impact of the program.

Endoscopic-assisted pediatric transcutaneous bone-anchored hearing implant: how I do it.

BACKGROUND: Conventional technique for transcutaneous bone-anchored hearing implants surgery requires the elevation of a large retroauricular skin flap to expose the site of implantation, that may cause a large scar and the interruption of retroauricular vasculature. METHODS: A less invasive, endoscopic-assisted modification of the surgical technique is described, which provides access to the implant site through two small skin incisions and the creation of a pocket in the retroauricular area. CONCLUSION: Endoscopic-assisted bone-anchored hearing implant surgery could be a viable option for transcutaneous bone-anchored hearing implant surgery, especially for pediatric patients.

Correlation of cochlear aperture stenosis with cochlear nerve deficiency in congenital unilateral hearing loss and prognostic relevance for cochlear implantation.

The use of neonatal hearing screening has enabled the identification of congenital unilateral sensorineural hearing loss (USNHL) immediately after birth, and today there are several intervention options available to minimize potential adverse effects of this disease, including cochlear implantation. This study aims to analyze the characteristics of the inner ear of a homogeneous group of congenital non-syndromic USNHL to highlight the features of the inner ear, which can help in clinical, surgical, and rehabilitative decision-making. A retrospective chart review was carried out at a tertiary referral center. Systematic diagnostic work-up and rigorous inclusion-exclusion criteria were applied to 126 children with unilateral hearing impairment, leading to a selection of 39 strictly congenital and non-syndromic USNHL cases, undergoing computed tomography (CT) and magnetic resonance (MR) imaging studies. The frequency and type of malformations of the inner ear in USNHL and unaffected contralateral ears were assessed, with an in-depth analysis of the deficiency of the cochlear nerve (CND), the internal auditory canal (IAC) and the cochlear aperture (CA). Inner ear anomalies were found in 18 out of 39 (46%) of the USNHL patients. In 1 subject, the anomalies were bilateral, and the CND resulted in the predominant identified defect (78% of our abnormal case series), frequently associated with CA stenosis. Only 3 out of 14 children with CND presented stenosis of the IAC. CND and CA stenosis (and to a much lesser extent IAC stenosis) are a frequent association within congenital and non-syndromic USNHL that could represent a distinct pathological entity affecting otherwise healthy infants. In the context of a diagnostic work-up, the evaluation with CT and MRI measurements should take place in a shared decision-making setting with thorough counseling. Both imaging techniques have proven useful in differentiating the cases that will most likely benefit from the cochlear implant, from those with potentially poor implant performance.

Who misses the newborn hearing screening? Five years' experience in Friuli-Venezia Giulia Region (Italy).

INTRODUCTION: Permanent hearing impairment is the most common sensory disorder in newborns. The Universal Newborn Hearing Screening (UNHS) is widely adopted as a cost-effective procedure to achieve early identification and treatment of congenital hearing impairment, with the final goal of an improved linguistic and cognitive outcome for hearing impaired children. The Italian Ministry of Health has recently comprised UNHS in the Essential Level of Health Assistance. Nevertheless, programs still vary both across and within Italian Regions in terms of coverage, testing, referral and tracking protocols. In Friuli-Venezia Giulia region the program for the early identification of newborn and childhood hearing impairment is operative since 2012. In order to minimize the lost to follow-up cases, UNHS and childhood hearing surveillance activities have been organized in close collaboration among birth centres, paediatric audiology services, territorial Family Paediatricians and the sole regional centre for paediatric hearing loss management. MATERIAL AND METHODS: We performed a five years' retrospective analysis of the UNHS experience in Friuli-Venezia Giulia comparing the UNHS activity of year 2013 and year 2017. The focus of the study concerns the "missing" cases. Three different typologies of "miss" cases ("documentation-miss", "access-miss" and "pathway-miss") have been defined in correspondence with main reasons for their occurrence. RESULTS: Births in Friuli-Venezia Giulia were 9465 and 8432, respectively in 2013 and 2017. International quality indicators improved with a gain of efficiency in 5 years' experience. However, "missing" cases were 486 in 2013 and 321 in 2017, mainly due to the lack of an efficient documentation system. CONCLUSION: UNHS programs have proven to be valuable and cost-effective in Friuli-Venezia Giulia and other Italian regions. New resources and efforts are required to achieve a complete standardization and informatisation of the UNHS data to avoid documentation gaps. A possible strategy would point to the opportunity to unify data management systems for all the ongoing newborn screening programs (metabolic, hearing and visual), linking the integrated IT system with the regional repository of current datasets.

Uncommon Post-Meningitis Hearing Threshold Improvement: A Case Report.

Bacterial meningitis may cause inner ear fibrosis and progressive cochlear ossification with irreversible profound hearing loss (HL). Recognition of potential ossification is essential for effective management. We present a clinical case of a 4 year old boy who developed a progressive HL starting 3 weeks after meningitis. For the prospective risk of cochlear ossification, bilateral cochlear implantation (CI) was performed. Unexpectedly, unaided hearing threshold began to show improvement on the left ear, starting 4 months after meningitis and continuing for years post CI surgery. In order to explore the residual cochlear function, a trial of exclusively acoustic amplification was performed on the improved left side 5 years post implantation, providing good results. A certain degree of hearing recovery may be expected after meningitis related deafness. This case encourages surgeons to always adopt atraumatic surgical techniques that can enable the preservation of cochlear structure and residual function after CI surgery.