: Congenital cytomegalovirus (cCMV) infection is the most common non-genetic cause of sensorineural hearing loss (SNHL) in children. In cases of severe-to-profound SNHL, cochlear implantation (CI) is a widely used intervention, but outcomes remain variable due to possible neurodevelopmental comorbidities. This study aimed to evaluate the long-term auditory and language outcomes in children with cCMV after CI and to explore clinical and radiological predictors of post-CI performance.
View Article and Find Full Text PDFActa Otorhinolaryngol Ital
June 2025
Objective: Management of advanced otosclerosis (AO) has evolved over the past 20 years with the availability of cochlear implant (CI) in addition to stapes surgery. Both procedures are reliable treatment options for AO with similar success rates and currently there are no standard guidelines regarding the surgical treatment of AO. The aim of this paper is to report the outcomes and complications of a series of patients with AO submitted to CI at our Institution.
View Article and Find Full Text PDFActa Otolaryngol
July 2025
Background: Single-sided deafness (SSD) encompasses the presence of a profoundly deaf ear with a normal, contralateral one. Patients with SSD may have difficulty with speech intelligibility in noise and localizing sounds.
Aims/objectives: This retrospective study aims to evaluate the long-term effectiveness of bone conduction implant (BCI) in a group of patients with SSD.
Acta Otorhinolaryngol Ital
April 2025
Objective: Cochlear implant (CI) is a well-established treatment for children with sensorineural hearing loss without benefit from hearing aids. The Italian guidelines date back 15 years; given the expansion of indications for CI (including single side deafness and asymmetrical hearing loss) it became necessary to establish updated guidelines.
Methods: Thirteen experts and 2 patient representatives selected the key questions and drew up recommendations.
Acta Otorhinolaryngol Ital
February 2025
Objective: Cochlear implant (CI) is a well-established treatment for adults with sensorineural hearing loss and without benefit from hearing aids. The Italian guidelines date back 15 years; given the expansion of indications for CI, including single side deafness and asymmetrical hearing loss, it became necessary to establish updated guidelines.
Methods: Thirteen experts and 2 patient representatives selected the key questions and drew up recommendations.
BackgroundPersistent cortical deafness in the pediatric population is rarely reported, and there is limited information on its implications for early intervention.ObjectivesThis study aims to (1) conduct a scoping review on pediatric cortical deafness and (2) present a case report of a 7-year-old girl with left unilateral spastic cerebral palsy and cortical deafness resulting from presumed perinatal bilateral stroke.MethodsA search of PubMed, Scopus, and Web of Science identified 407 manuscripts.
View Article and Find Full Text PDFInt J Pediatr Otorhinolaryngol
September 2024
Introduction: Purpose of our study was to compare two competing methods of performing bisyllabic word speech audiometry for the detection of the 50% speech reception threshold in noise (SRT50).
Methods: Classic method is performed submitting multiple word lists at a fixed signal-to-noise ratio. A newer Fast method - Italian Fast Speech Reception Threshold 50 (IFastSRT50) - is performed by means of program software with a single list of bisyllabic words and noise intensity shifting.
Bioengineering (Basel)
February 2024
Bone defects are a significant health problem worldwide. Novel treatment approaches in the tissue engineering field rely on the use of biomaterial scaffolds to stimulate and guide the regeneration of damaged tissue that cannot repair or regrow spontaneously. This work aimed at developing and characterizing new piezoelectric scaffolds to provide electric bio-signals naturally present in bone and vascular tissues.
View Article and Find Full Text PDFTitanium middle ear (ME) prostheses are widely used in surgical practice due to their acoustic properties. However, they present a significant drawback shared by all synthetic materials currently in use for ME reconstruction: they can be rejected by the organism of the host. In this study, we aim to review the current literature on titanium partial ossicular replacement prostheses (PORPs) and total ossicular replacement prostheses (TORPs) extrusion and dislocation.
View Article and Find Full Text PDFCongenital unilateral hearing loss (UHL) represents a contemporary audiologic challenge. Children with UHL can struggle with understanding speech in noise, localizing sounds, developing language, and maintaining academic performance, leading to low self-esteem, anxiety, and decreased social support. Two specific conditions related to UHL in children are single-sided deafness (SSD) and unilateral auris atresia (UAA).
View Article and Find Full Text PDFActa Otorhinolaryngol Ital
April 2023
Objective: To establish the safety and effectiveness of subtotal petrosectomy with cochlear implantation in patients affected by chronic middle ear disorders to refractory to previous surgical treatments.
Methods: A multicentre, retrospective study was conducted on patients affected by recalcitrant chronic middle ear disorders who underwent cochlear implantation in combination with subtotal petrosectomy. Patients' details were collected from databases of 11 Italian tertiary referral centres.
Background: Congenital cytomegalovirus (cCMV) infection is the leading cause of non-hereditary sensorineural hearing loss in children. While about 10% of children reportedly display symptoms at birth, 85-90% of cCMV infection cases are asymptomatic. However, 10-15% of these asymptomatic infants may later develop hearing, visual, or neurodevelopmental impairments.
View Article and Find Full Text PDF: Otoferlin is a multi-C2 domain protein implicated in neurotransmitter-containing vesicle release and replenishment of the cochlear inner hair cell (IHC) synapses. Mutations in the OTOF gene have been associated with two different clinical phenotypes: a prelingual severe-to-profound sensorineural hearing loss (ANSD-DFNB9); and the peculiar temperature-sensitive auditory neuropathy (TS-ANSD), characterized by a baseline mild-to-moderate hearing threshold that worsens to severe-to-profound when the body temperature rises that returns to a baseline a few hours after the temperature has fallen again. The latter clinical phenotype has been described only with a few OTOF variants with an autosomal recessive biallelic pattern of inheritance.
View Article and Find Full Text PDFBackground: The Carina system (Cochlear Ltd., Sydney, Australia) is a totally implantable device providing acoustic amplification in adult patients with moderate-to-severe sensorineural or mixed hearing loss. One of the main concerns about such a totally implantable device has been represented by the subcutaneous battery lifespan.
View Article and Find Full Text PDFBackground: Bone-anchored hearing aids represent a valid alternative for patients with conductive/mixed hearing loss who cannot use hear- ing aids. To date, these devices have given good audiological results, thanks to various fitting prescription programs (i.e.
View Article and Find Full Text PDFPerrault syndrome (PRLTS) is a rare autosomal recessive disorder characterised by ovarian failure in females and sensorineural hearing loss (SNHL) in both genders. In the present paper we describe a child affected by PRLTS3, due to CLPP homozygous mutations, presenting auditory neuropathy spectrum disorder (ANSD) with bilateral progressive SNHL. This is the first case reported in the literature of an ANSD in PRLTS3.
View Article and Find Full Text PDFThe demand for bone substitutes is increasing in Western countries. Bone graft substitutes aim to provide reconstructive surgeons with off-the-shelf alternatives to the natural bone taken from humans or animal species. Under the tissue engineering paradigm, biomaterial scaffolds can be designed by incorporating bone stem cells to decrease the disadvantages of traditional tissue grafts.
View Article and Find Full Text PDFMinerva Pediatr (Torino)
October 2024
Background: The importance of neonatal screening for congenital cytomegalovirus infection (cCMV) is widely shared in the world scientific literature. However, currently, no programs for universal neonatal screening for cCMV have been reported in the literature, and only a few experiences of hearing targeted cCMV screening have been published. In the Tuscany Region, Italy, screening for cCMV has been mandatory since 2008 for each newborn that resulted as "refer" at the Transient Evoked Otoacoustics Emissions (TEOAE) Test, and in our university hospital since 2012, it has been extended to some categories at risk of cCMV.
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