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Hearing Disorders: HELP
Articles by Francesca Forli
Based on 22 articles published since 2010
(Why 22 articles?)
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Between 2010 and 2020, F. Forli wrote the following 22 articles about Hearing Disorders.
 
+ Citations + Abstracts
1 Review A review of new insights on the association between hearing loss and cognitive decline in ageing. 2016

Fortunato, S / Forli, F / Guglielmi, V / De Corso, E / Paludetti, G / Berrettini, S / Fetoni, A R. ·Otolaryngology Audiology and Phoniatric Unit, University of Pisa, Pisa, Italy; · Department of Neuroscience, Institute of Neurology, Catholic University of the Sacred Heart, Rome, Italy; · Department of Head and Neck Surgery - Otorhinolaryngology, Catholic University of the Sacred Heart, Rome, Italy. ·Acta Otorhinolaryngol Ital · Pubmed #27214827.

ABSTRACT: Age-related hearing loss (ARHL) has a multifactorial pathogenesis and it is an inevitable hearing impairment associated with reduction of communicative skills related to ageing. Increasing evidence has linked ARHL to more rapid progression of cognitive decline and incidental dementia. Many aspects of daily living of elderly people have been associated to hearing abilities, showing that hearing loss (HL) affects the quality of life, social relationships, motor skills, psychological aspects and function and morphology in specific brain areas. Epidemiological and clinical studies confirm the assumption of a relationship between these conditions. However, the mechanisms are still unclear and are reviewed herein. Long-term hearing deprivation of auditory inputs can impact cognitive performance by decreasing the quality of communication leading to social isolation and depression and facilitate dementia. On the contrary, the limited cognitive skills may reduce the cognitive resources available for auditory perception, increasing the effects of HL. In addition, hearing loss and cognitive decline may reflect a 'common cause' on the auditory pathway and brain. In fact, some pathogenetic factors are recongised in common microvascular disease factors such as diabetes, atherosclerosis and hypertension. Interdisciplinary efforts to investigate and address HL in the context of brain and cognitive ageing are needed. Surprisingly, few studies have been adressed on the effectiveness of hearing aids in changing the natural history of cognitive decline. Effective interventions with hearing aids or cochlear implant may improve social and emotional function, communication, cognitive function and positively impact quality of life. The aim of this review is to overview new insights on this challenging topic and provide new ideas for future research.

2 Review [Cochlear implant in children: rational, indications and cost/efficacy]. 2013

Martini, A / Bovo, R / Trevisi, P / Forli, F / Berrettini, S. ·Dipartimento di Neuroscienze e Organi di Senso, Università di Padova, Padova, Italia. alessandromartini@unipd.it ·Minerva Pediatr · Pubmed #23685383.

ABSTRACT: A cochlear implant (CI) is a partially implanted electronic device that can help to provide a sense of sound and support speech to severely to profoundly hearing impaired patients. It is constituted by an external portion, that usually sits behind the ear and an internal portion surgically placed under the skin. The external components include a microphone connected to a speech processor that selects and arranges sounds pucked up by the microphone. This is connected to a transmitter coil, worn on the side of the head, which transmits data to an internal receiver coil placed under the skin. The received data are delivered to an array of electrodes that are surgically implanted within the cochlea. The primary neural targets of the electrodes are the spiral ganglion cells which innervate fibers of the auditory nerve. When the electrodes are activated by the signal, they send a current along the auditory nerve and auditory pathways to the auditory cortex. Children and adults who are profoundly or severely hearing impaired can be fitted with cochlear implants. According to the Food and Drug Administration, approximately 188,000 people worldwide have received implants. In Italy it is extimated that there are about 6-7000 implanted patients, with an average of 700 CI surgeries per year. Cochlear implantation, followed by intensive postimplantation speech therapy, can help young children to acquire speech, language, and social skills. Early implantation provides exposure to sounds that can be helpful during the critical period when children learn speech and language skills. In 2000, the Food and Drug Administration lowered the age of eligibility to 12 months for one type of CI. With regard to the results after cochlear implantation in relation to early implantation, better linguistic results are reported in children implanted before 12 months of life, even if no sufficient data exist regarding the relation between this advantage and the duration of implant use and how long this advantage persists in the subsequent years. With regard to cochlear implantation in children older than 12 months the studies show better hearing and linguistic results in children implanted at earlier ages. A sensitive period under 24-36 months has been identified over which cochlear implantation is reported to be less effective in terms of improvement in speech and hearing results. With regard to clinical effectiveness of bilateral cochlear implantation, greater benefits from bilateral implants compared to monolateral ones when assessing hearing in quiet and in noise and in sound localization abilities are reported to be present in both case of simultaneous or sequential bilateral implantation. However, with regard to the delay between the surgeries in sequential bilateral implantation, although benefit is reported to be present even after very long delays, on average long delays between surgeries seems to negatively affect the outcome with the second implant. With regard to benefits after cochlear implantation in children with multiple disabilities, benefits in terms of speech perception and communication as well as in quality of the daily life are reported even if benefits are slower and lower in comparison to those generally attained by implanted children without additional disabilities. Regarding the costs/efficacy ratio, the CI is expensive, in particular because of the cost of the high technological device, long life support, but even if healthcare costs are high, the savings in terms of indirect costs and quality of life are important. The CI, in fact, has a positive impact in terms of quality of life.

3 Article 3D fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging at different stages of otosclerosis. 2018

Berrettini, Stefano / Lombardo, Francesco / Bruschini, Luca / Ciabotti, Annalisa / Raffaello, Canapicchi / De Cori Sara, ? / De Marchi Daniele, ? / Forli, Francesca. ·ENT Audiology and Phoniatrics Unit, Department of Surgical, Medical, Molecular Pathology and Emergency Medicine, University of Pisa, Via Paradisa 2, 56100, Pisa, Italy. s.berrettini@med.unipi.it. · Division of ENT Diseases, Karolinska Institutet, Stockholm, Sweden. s.berrettini@med.unipi.it. · Neuroradiology Unit, Fondazione CNR Regione Toscana "G. Monasterio", Pisa, Italy. · ENT Audiology and Phoniatrics Unit, Department of Surgical, Medical, Molecular Pathology and Emergency Medicine, University of Pisa, Via Paradisa 2, 56100, Pisa, Italy. ·Eur Arch Otorhinolaryngol · Pubmed #30191304.

ABSTRACT: PURPOSE: The aim of this work is to study otosclerotic patients by 3D-FLAIR (fluid attenuated inversion recovery) sequence magnetic resonance imaging (MRI) with and without Gadolinium administration (-/+ Gd), to understand whether there is a direct relationship between radiological findings at 3D FLAIR MRI sequences and some clinical features of otosclerosis, such as the presence and entity of sensorineural involvement, duration of disease, patient gender, and other factors. METHODS: 38 patients affected by different stages of unilateral or bilateral otosclerosis underwent 3D FLAIR MRI+/- Gd. 11 subjects with normal hearing, previously submitted to 3T MRI for other minor diseases, unrelated with otosclerosis, had been retrospectively enrolled as control group. RESULTS: We found significant correlations between 3D FLAIR MRI findings and some clinical features of otosclerosis, such as severity of cochlear damage (in terms of entity of sensorineural loss) and duration of disease. These findings indicate that at 3D-FLAIR MRI different patterns may depend on the level of blood labyrinth barrier damage in the cochlea, and be related to different stages of cochlear involvement in otosclerotic patients. CONCLUSIONS: We believe that our findings may contribute in understanding the pathogenesis of cochlear damage in otosclerosis and may have further prognostic value. Our results led us to consider the possible use of 3D-FLAIR sequences in monitoring the effectiveness of any medical therapy of otosclerosis and in selecting the patients eligible for treatment.

4 Article Transtympanic Hearing Aid: exploratory study on a new device. 2018

Berrettini, S / Bruschini, L / DE Vito, A / Gnocco, T / Rosica, N C / Pizzoli, L / Forli, F. ·ENT Audiology and Phoniatric Unit, University Hospital of Pisa, Pisa, Italy. · CRAI S.p.A., Bovolenta, Italy. · ENT freelance doctor, Rome, Italy. · Engineer freelance, Rome, Italy. ·Acta Otorhinolaryngol Ital · Pubmed #29984801.

ABSTRACT: SUMMARY: In this paper, we present the preliminary results achieved with a transtympanic hearing aid (THA). This is a modified digital, open-fit external hearing aid (HA) designed for acute study only, which allows coupling with a pre-implanted ventilation tube. The THA conveys amplified sound directly onto the round window, bypassing the ossicular chain, in contrast with traditional HAs that convey sound onto the second or third portion of the external auditory canal. The THA has been developed as an alternative to standard HAs and active middle ear implants for patients who are unsatisfied with traditional HA outcomes and want to avoid middle-ear implantation. The results achieved using the THA were compared to those obtained with an equivalent device, the Latitude 8 Moxi 13 (Moxi), uncoupled from the ventilation tube, and placed onto the outer ear. For this purpose, 12 patients with conductive (1/12), sensorineural (3/12), or mixed (8/12) hearing loss from moderate to severe, with a pre-implanted ventilation tube, underwent audiological evaluation with both the THA and the Latitude 8 Moxi 13 (Moxi). Our initial results showed that the THA provided significant improvement in the warble tone results in comparison to the Moxi. Moreover, patients with a PTA between 41 and 90 also achieved better results in terms of speech recognition using the THA in comparison to the Moxi. In conclusion, these outcomes provide the first evidence of the potential benefits of the THA over standard open-fit HAs. Nevertheless, these preliminary outcomes require further confirmation.

5 Article How does a bilingual environment affect the results in children with cochlear implants compared to monolingual-matched children? An Italian follow-up study. 2018

Forli, Francesca / Giuntini, Giada / Ciabotti, Annalisa / Bruschini, Luca / Löfkvist, Ulrika / Berrettini, Stefano. ·Otorhinolaryngology, Audiology and Phoniatrics Operative Unit, Azienda Ospedaliero Universitaria Pisana, Department of Surgical, Medical, Molecular Pathology and Emergency Medicine, University of Pisa, 56124 Pisa, Italy. Electronic address: francesca.forli@gmail.com. · Otorhinolaryngology, Audiology and Phoniatrics Operative Unit, Azienda Ospedaliero Universitaria Pisana, Department of Surgical, Medical, Molecular Pathology and Emergency Medicine, University of Pisa, 56124 Pisa, Italy. · Department of Special Needs Education, University of Oslo, Norway. · Otorhinolaryngology, Audiology and Phoniatrics Operative Unit, Azienda Ospedaliero Universitaria Pisana, Department of Surgical, Medical, Molecular Pathology and Emergency Medicine, University of Pisa, 56124 Pisa, Italy; Department of Clinical Science, Intervention and Technology, Karolinska Institutet, 17177 Stockholm, Sweden. ·Int J Pediatr Otorhinolaryngol · Pubmed #29447820.

ABSTRACT: OBJECTIVES: To compare the results after cochlear implantation achieved by monolingual and bilingual deaf children implanted at our Institution, with the aim of understanding if there are any differences between the two groups and if there is a correlation between the outcomes and some patients' variables. METHODS: The study group was composed by 14 bilingual deaf children and the control group by the same number of monolingual children implanted at our Institution. The control group was obtained by matching to each bilingual child a monolingual one with a similar clinical history regarding age at hearing loss diagnosis, age at first hearing-aids fitting and age at CI procedure. Children received a speech perception and linguistic development evaluation through specific structured tests. The linguistic competence of the patients both in mainstream and native language was determined by the Student Oral Language Observation Matrix (SOLOM). RESULTS: We did not find any statistically significant differences between bilingual and monolingual children in speech perception outcomes. Nevertheless, we obtained different results concerning language skills: bilingual implanted children scored lower at structured language tests, even if the difference was not statistically relevant. Bilingual children scored significantly lower than monolingual ones at the SOLOM scale for linguistic competence. CONCLUSION: The results reported in the present study show better language skills after cochlear implant in Italian monolingual cases than in bilingual ones. This seems to be related to the condition of bilingualism in Italy, mainly related to immigration, and frequently associated with low socio-economic levels, poor competence in the mainstream language and poor social integration, with a suboptimal exposure to the mainstream language and difficulties in following the rehabilitative program.

6 Article Cochlear implant in prelingually deafened oralist adults: speech perception outcomes, subjective benefits and quality of life improvement. 2017

Forli, F / Turchetti, G / Giuntini, G / Bellelli, S / Fortunato, S / Bruschini, L / Barillari, M R / Berrettini, S. ·ENT, Audiology and Phoniatric Unit, University of Pisa, Italy. · Istituto di Management, Scuola Superiore Sant'Anna, Pisa, Italy. · Dipartimento di Salute Mentale e Fisica e Medicina Preventiva, Divisione di Audiologia e Foniatria, II Università di Napoli, Italy. · Department of Clinical Science, Intervention and Technology, Karolinska Institutet, 17177, Stockholm, Sweden. ·Acta Otorhinolaryngol Ital · Pubmed #29165436.

ABSTRACT: The aim of this study is to report our results in a group of prelingually deafened adults, who followed an oralist rehabilitation programme, and submitted to cochlear implant at our institution. We evaluated 30 prelingually deafened adult patients, 18 males and 12 females, median age 35 years, of a group of 36 prelingually deafened adult patients consecutively submitted to unilateral cochlear implantation at the ENT Unit of the University of Pisa. After implantation, patients achieved significant benefits in terms of speech perception skills, including the ability to have telephone conversations in some cases, quality of life and their own perception of disability. According to literature data, the results herein reported are quite variable but generally satisfactory. Procedures other than traditional speech perception measures should be used to evaluate the benefits of cochlear implant in such patients, to compressively evaluate the global benefits, not only in terms of speech perception, but also in terms of quality of life and daily life.

7 Article Audio-visual temporal perception in children with restored hearing. 2017

Gori, Monica / Chilosi, Anna / Forli, Francesca / Burr, David. ·U-VIP Unit for Visually Impaired People, Center for Human Technologies, Fondazione Istituto Italiano di Tecnologia, Via Enrico Melen 83, Genoa, Italy. Electronic address: monica.gori@iit.it. · Department of Developmental Neuroscience, Stella Maris Scientific Institute, Pisa, Italy. · Azienda Ospedaliero-Universitaria Pisana, Pisa, Italy; Dipartimento di Psicologia, Università Degli Studi di Firenze, Via S. Salvi 12, 50125, Florence, Italy. · School of Psychology, University of Western Australia, Perth, WA, Australia. ·Neuropsychologia · Pubmed #28365363.

ABSTRACT: It is not clear how audio-visual temporal perception develops in children with restored hearing. In this study we measured temporal discrimination thresholds with an audio-visual temporal bisection task in 9 deaf children with restored audition, and 22 typically hearing children. In typically hearing children, audition was more precise than vision, with no gain in multisensory conditions (as previously reported in Gori et al. (2012b)). However, deaf children with restored audition showed similar thresholds for audio and visual thresholds and some evidence of gain in audio-visual temporal multisensory conditions. Interestingly, we found a strong correlation between auditory weighting of multisensory signals and quality of language: patients who gave more weight to audition had better language skills. Similarly, auditory thresholds for the temporal bisection task were also a good predictor of language skills. This result supports the idea that the temporal auditory processing is associated with language development.

8 Article Idiopathic sensorineural hearing loss in the only hearing ear. 2016

Berrettini, S / De Vito, A / Bruschini, L / Fortunato, S / Forli, F. ·ENT, Audiology and Phoniatrics Universitary Unit of "Azienda Universitaria Ospedaliera Pisana" Pisa, Italy; · Guest Professor at Division of ENT Diseases, Karolinska Institutet, Stockholm, Sweden. ·Acta Otorhinolaryngol Ital · Pubmed #27196076.

ABSTRACT: A retrospective chart review was used for 31 patients with sudden, progressive or fluctuating sensorineural hearing loss (SHL) in the only hearing ear who had been consecutively evaluated at the ENT, Audiology and Phoniatrics Unit of the University of Pisa. The group of patients was evaluated with a complete history review, clinical evaluation, imaging exam (MRI, CT), audiologic tests (tone and speech audiometry, tympanometry, study of stapedial reflexes, ABR and otoacoustic emission) evaluation. In order to exclude genetic causes, patients were screened for CX 26 and CX30 mutations and for mitochondrial DNA mutation A1555G. Patients with sudden or rapidly progressive SHL in the only hearing ear were treated with osmotic diuretics and corticosteroids. In patients who did not respond to intravenous therapy we performed intratympanic injections of corticosteroid. Hearing aids were fitted when indicated and patients who developed severe to profound SHL were scheduled for cochlear implant surgery. The aim of this study is to report and discuss the epidemiology, aetiopathogenesis, therapy and clinical characteristic of patients affected by SHL in the only hearing hear and to discuss the issues related to the cochlear implant procedure in some of these patients, with regard to indications, choice of the ear to implant and results.

9 Article Early care in children with permanent hearing impairment. 2016

Giuntini, G / Forli, F / Nicastro, R / Ciabotti, A / Bruschini, L / Berrettini, S. ·U.O. ORL Audiologia e Foniatria Universitaria, Università di Pisa; · U.O. ORL Audiologia e Foniatria Universitaria, Università di Pisa, Operative Unit of Otorhinolaryngology, Audiology and Phoniatrics, University of Pisa, Italy, Guest Professor at Division of Ear, Nose and Throat Diseases, Dept. of Clinical Science, Intervention and Technology, Karolinska Istituet, Stockholm, Sweden. ·Acta Otorhinolaryngol Ital · Pubmed #27054391.

ABSTRACT: The implementation of regional protocols for newborn hearing screening and early audiologic diagnosis represent the first step of the entire diagnostic, rehabilitative and prosthetic programme for children with permanent hearing impairment. The maximum benefit of early diagnosis can indeed be obtained only by prompt rehabilitation aimed at fostering the child's communicative, linguistic and cognitive development. Within the framework of the CMM 2013 project of the Ministry of Health entitled "Preventing Communication Disorders: a Regional Program for Early Identification, Intervention and Care of Hearing Impaired Children", the problems concerning the promotion of the global development of children with PHI through an early rehabilitation project based on shared knowledge and scientific evidence. In this project, our specific aim was to define the features and modes of access to a precise and specialised rehabilitation project for the small hearing-impaired child within three months from audiologic diagnosis. Three main recommendations relative to assessment and rehabilitation aspects of early care emerged from the study.

10 Article Aetiologic diagnosis of hearing loss in children identified through newborn hearing screening testing. 2016

Forli, F / Giuntini, G / Bruschini, L / Berrettini, S. ·Operative Unit of Otorhinolaryngology, Audiology and Phoniatrics, University of Pisa, Italy; · Operative Unit of Otorhinolaryngology, Audiology and Phoniatrics, University of Pisa, Italy, Guest Professor at Division of Ear, Nose and Throat Diseases, Dept. Of Clinical Science, Intervention and Technology, Karolinska Istituet, Stockholm, Sweden. ·Acta Otorhinolaryngol Ital · Pubmed #27054388.

ABSTRACT: With the implementation of universal newborn hearing screening (UNHS) programmes and early diagnosis and treatment of hearing problems, the need has clearly emerged to implement and carry out a systematic and coordinated protocol for the aetiological diagnosis of permanent hearing impairment (PHI). Within the framework of the Italian Ministry of Health project CCM 2013 "Preventing Communication Disorders: a Regional Program for early Identification, Intervention and Care of Hearing Impaired Children", it has been decided to consider the problems relative to aetiological diagnosis of child PHI within UNHS programmes. The specific objective was to apply a shared diagnostic protocol that can identify the cause in at least 70% of cases of PHI. For this part of the project, four main recommendations were identified that can be useful for an efficient aetiological diagnosis in children affected by PHI and that can offer valid suggestions to optimise resources and produce positive changes for third-level audiologic centres.

11 Article The Carina© middle ear implant: surgical and functional outcomes. 2016

Bruschini, Luca / Berrettini, Stefano / Forli, Francesca / Murri, Alessandra / Cuda, Domenico. ·ENT Audiology and Phoniatric Unit, University Hospital of Pisa, Pisa, Italy. l.bruschini@gmail.com. · ENT Audiology and Phoniatric Unit, University Hospital of Pisa, Pisa, Italy. · Division of ENT Diseases, Karolinska Institute, Stockholm, Sweden. · ENT Unit, "Guglielmo da Saliceto" Hospital of Piacenza, Piacenza, Italy. ·Eur Arch Otorhinolaryngol · Pubmed #27007286.

ABSTRACT: The Carina© system (Cochlear Ltd, Sydney, Australia) is a totally implantable prosthesis designed to meet the needs of acoustic amplification in adults with moderate-to-severe sensorineural or mixed hearing loss. It mechanically stimulates the ossicles or labyrinthine windows. The object of this study was to update surgical and functional results obtained in a consistent sample of Carina© recipients. The data sheets of 26 patients who underwent Carina© surgery at two ENT units [i.e., ENT Audiology and Phoniatrics Unit of the University Hospital of Pisa (Pisa, Italy) and ENT Unit of "Guglielmo da Saliceto" Hospital of Piacenza (Piacenza, Italy)] were evaluated for the surgical and audiological outcomes. Twenty patients received a unilateral device, whereas six patients were bilaterally implanted (simultaneously in one patient and sequentially in the remaining five patients). The total number of ears implanted in this study was 32. Most implanted patients expressed a high degree of satisfaction with the prosthesis, which highlights the improvement in hearing in different environmental conditions, the cosmetic advantages, and greater freedom in performing common activities in daily living. Audiological examination demonstrated valid functional gain and significant improvement in speech perception in quiet. Based on this experience, the Carina© is a valid alternative to hearing aids. Moreover, a bilateral implantation, even a simultaneous implantation, is a viable option for selected motivated patients because the intervention is generally well tolerated and the surgery time is relatively fast.

12 Article Mitochondrial DNA (mtDNA) haplotypes and dysfunctions in presbyacusis. 2014

Mostafa, H / Saad, M / El-Attar, A / Ahmed, G / Berrettini, S / Forli, F / Siciliano, G / Mancuso, M. ·Audiology Unit, ENT Department, Faculty Of Medicine, Assiut University, Egypt; · Department of Genetics, Faculty of Agriculture, Assiut University, Egypt; · Department of otolaryngology, Faculty of Medicine, University of Pisa, Italy; · Department of Neurosciences, Faculty of Medicine, University of Pisa, Italy. ·Acta Otorhinolaryngol Ital · Pubmed #24711684.

ABSTRACT: The aim of this study was to investigate the presence of mitochondrial DNA (mtDNA) alterations and metabolic dysfunctions in patients with presbyacusis, and to discover correlations between presbyacusis and the degree of hearing loss and mitochondrial damage. Seventy patients with presbyacusis were examined, including 40 Egyptian patients and 30 Italian patients. Forty eight normal subjects were included as control group, including 24 Egyptians and 24 Italians. There was no common point mutation, and A1555G, A3243G, A7445G not were detected in any patients or controls. Haplogroup U was significantly common in patients in comparison to controls. Mutation of antioxidant genes (GSTT1, GSTM1) were significantly present in only Italian patients compared to Italian controls.

13 Article Cerebral lateralization for language in deaf children with cochlear implantation. 2014

Chilosi, Anna Maria / Comparini, Alessandro / Cristofani, Paola / Turi, Marco / Berrettini, Stefano / Forli, Francesca / Orlandi, Giovanni / Chiti, Alberto / Giannini, Nicola / Cipriani, Paola / Cioni, Giovanni. ·Dipartimento di Neuroscienze dell'Età Evolutiva, IRCCS Fondazione Stella Maris, Pisa, Italy. Electronic address: achilosi@inpe.unipi.it. · Dipartimento di Neuroscienze dell'Età Evolutiva, IRCCS Fondazione Stella Maris, Pisa, Italy. · Dipartimento di Neuroscienze, Psicologia, Area del Farmaco e Salute del Bambino (NEUROFARBA), Università di Firenze, Piazza San Marco 4, 50100 Firenze, Italy. · Dipartimento di Ricerca Traslazionale e delle Nuove Tecnologie in Medicina e Chirurgia, Università di Pisa, Via Paradisa 2, 56100 Pisa, Italy. · Dipartimento di Medicina Clinica e Sperimentale, Università di Pisa, Via Roma 67, 56100 Pisa, Italy. · Dipartimento di Neuroscienze dell'Età Evolutiva, IRCCS Fondazione Stella Maris, Pisa, Italy; Dipartimento di Medicina Clinica e Sperimentale, Università di Pisa, Via Roma 67, 56100 Pisa, Italy. ·Brain Lang · Pubmed #24463309.

ABSTRACT: Functional Transcranial Doppler ultrasonography (fTCD) was used to investigate the effects of early acoustic deprivation and subsequent reafferentation on cerebral dominance for language in deaf children provided with Cochlear Implantation (CI). Twenty children with CI (13 in right ear and 7 in left ear) and 20 controls matched for age, sex and handedness were administered a fTCD animation description task. Left hemisphere dominance for language with comparable mean Laterality Indexes (LIs) was found in children with CI and controls; right-ear implanted subjects showed cerebral activation controlateral to implanted ear more frequently than left-ear implanted ones. Linguistic proficiency of CI recipients was below age expectation in comparison to controls; language scores did not significantly differ between children with left and right LI, whereas both age and side of implantation were significantly related to language outcome. Theoretical implication and potential clinical application of fTCD in CI management are discussed.

14 Article Analysis of the 3-dimensional fluid-attenuated inversion-recovery (3D-FLAIR) sequence in idiopathic sudden sensorineural hearing loss. 2013

Berrettini, Stefano / Seccia, Veronica / Fortunato, Susanna / Forli, Francesca / Bruschini, Luca / Piaggi, Paolo / Canapicchi, Raffaello. ·Otolaryngology, Audiology, and Phoniatrics Unit, Department of Neuroscience, University of Pisa, Italy. s.berrettini@med.unipi.it ·JAMA Otolaryngol Head Neck Surg · Pubmed #23681028.

ABSTRACT: IMPORTANCE: The unpredictability of idiopathic sudden sensorineural hearing loss (ISSNHL) presents a challenge to preventive care. Our study confirms the potentially important role of the 3-T magnetic resonance imaging (MRI), and in particular of the 3-dimensional fluid-attenuated inversion-recovery (3D-FLAIR) sequence, in the diagnosis and prognosis of ISSNHL to guide medical treatment. OBJECTIVE: To confirm the diagnostic, clinical, and prognostic role of 3D-FLAIR MRI in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). DESIGN, SETTING, AND PATIENTS: Retrospective study in a tertiary referral center with a consecutive sample of 23 patients diagnosed as having unilateral ISSNHL from January 2010 to March 2011. EXPOSURES: Patients underwent 3D-FLAIR MRI at 3 T to evaluate ISSNHL, and the MRI images were compared with those belonging to a random group of 20 age-matched healthy patients. MAIN OUTCOMES AND MEASURES: Precontrast and postcontrast high-intensity 3D-FLAIR MRI findings in patients with ISSNHL and the correlation with clinical findings. RESULTS: Thirteen patients showed high-intensity signals in the affected inner ear on precontrast and postcontrast 3D-FLAIR MRI (57%). From the analysis of different MRI sequences, we posited 3 radiologic patterns likely correlated with mild hemorrhage, acute inflammation, and presence or absence of blood-labyrinth or nerve barrier (BLB) breakdown. Hypersignal on 3D-FLAIR MRI was positively associated with pretreatment hearing loss (P = .04) and presence of vertigo (P = .04). A strict correlation also existed between distribution of the signal (vestibule, semicircular canals) and clinical features (vertigo) (P = .04). CONCLUSIONS AND RELEVANCE: Use of 3D-FLAIR MRI at 3 T may contribute to the elucidation of pathologic conditions in the inner ears of patients with ISSNHL and provide new radiologic indicators (mild hemorrhage, acute inflammation, presence or absence of BLB breakdown) that might assume the role of prognostic factors.

15 Article A longitudinal study of lexical and grammar development in deaf Italian children provided with early cochlear implantation. 2013

Chilosi, Anna Maria / Comparini, Alessandro / Scusa, Maria Flora / Orazini, Laura / Forli, Francesca / Cipriani, Paola / Berrettini, Stefano. ·Department of Developmental Neuroscience, Istituto Di Ricovero e Cura a Carattere Scientifico, Stella Maris, Pisa, Italy. achilosi@inpe.unipi.it ·Ear Hear · Pubmed #23615459.

ABSTRACT: OBJECTIVE: A growing number of studies on deaf children with cochlear implant (CI) document a significant improvement in receptive and expressive language skills after implantation, even if they show language delay when compared with normal-hearing peers. Data on language acquisition in CI Italian children are still scarce and limited to only certain aspects of language. The purpose of this study is to prospectively describe the trajectories of language development in early CI Italian children, with particular attention to the transition from first words to combinatorial speech and to acquisition of complex grammar in a language with rich morphology, such as Italian. DESIGN: Six children, with profound prelingual deafness, provided with CI, between 16 and 24 months of age were prospectively assessed and followed over a mean period of up to 34.8 months postimplant. During follow-up, each child received between four to five individual language evaluations through a combination of indirect procedures (parent reports of early lexical and grammar development) and direct ones (administration of standardized receptive and expressive language tests with Italian norms and collection of spontaneous language samples). RESULTS: In relation to chronological age, the acquisition of expressive vocabulary was delayed. However, considering the duration of hearing experience, most CI participants showed an earlier start and faster growth of expressive rather than receptive vocabulary in comparison with typically developing children. This quite atypical result persisted right up until the end of the follow-up. The acquisition of expressive grammar was delayed relative to chronological age, though all but one CI participant achieved the expected grammar level after approximately 3 years of CI use. In addition, the rate of grammar acquisition was not homogeneous during development, showing two different paces: one comparable with normal hearing in the transition from holophrastic to primitive combinatorial speech and a much slower one to attain more advanced levels of morphosyntactic control. CONCLUSION: From a rehabilitative viewpoint, our results suggest the importance of implementing rehabilitation in lexical comprehension, even when expressive vocabulary appears to be within normal range. Moreover, assessment of language acquisition in CI Italian children should focus on those grammar aspects that are more vulnerable to early acoustic deprivation (such as free and bound morphology) to ensure enhanced language therapy planning.

16 Article Evaluating benefits of cochlear implantation in deaf children with additional disabilities. 2012

Palmieri, Maria / Berrettini, Stefano / Forli, Francesca / Trevisi, Patrizia / Genovese, Elisabetta / Chilosi, Anna Maria / Arslan, Edoardo / Martini, Alessandro. ·Otology-Cochlear Implant Centre, Department of Neuroscience, University of Pisa, Pisa, Italy. ·Ear Hear · Pubmed #22785571.

ABSTRACT: OBJECTIVES: Cochlear-implanted deaf children having additional disabilities may develop speech perception and language skills at a slower pace than their implanted peers without such disorders. Nevertheless, it has been shown that, even for these special cases, cochlear implantation (CI) provides benefits for a larger range of neuropsychological functions including social and relational skills. These benefits are frequently mentioned by parents, but rarely objectively measured by tests. This article presents a new evaluation tool aimed at assessing the global benefits offered by CI in these special cases. DESIGN: The new tool has been designed as a closed-format questionnaire, divided into five areas. It is based on observing the frequency of preselected behaviors in daily activities, which imply specific social, neuropsychological, and perceptual skills. The questionnaire has been presented to the parents of 50 deaf children with additional disabilities, before and at least 6 months after CI. RESULTS: The data show significant improvements in all investigated areas. However, not all skills improve in the same way, and only those skills related to language and communication correlate positively with time after implantation. The present article further discusses changes in skills for which parents have higher expectations, such as the preferred communication mode, speech intelligibility, and the ability to communicate on the telephone. CONCLUSIONS: The questionnaire has a simple-to-use format, and it has been proven to be sufficiently sensitive for the detection of changes in each examined area. Because the questionnaire is based on observed behaviors, it can be used even when other existing tests involve tasks that are too complex for these children.

17 Article [Etiological, clinical and neuroradiological investigation of deaf children with additional neuropsychiatric disabilities]. 2012

Chilosi, A M / Scusa, M F / Comparini, A / Genovese, E / Forli, F / Berrettini, S / Cipriani, P. ·Dipartimento di Neuroscienze dello Sviluppo, IRCCS Stella Maris, Calambrone, Pisa, Italia. achilosi@inpe.unipi.it ·Minerva Pediatr · Pubmed #22495195.

ABSTRACT: AIM: Sensorineural hearing loss (SNHL) is complicated by additional disabilities in about 30% of cases, but the epidemiology of associated disorders, in terms of type, frequency and aetiology is still not clearly defined. Additional disabilities in a deaf child have important consequences in assessing and choosing a therapeutic treatment, in particular when considering cochlear implantation (CI) or hearing aids (HA). The aim of this paper was to evaluate frequency, type and severity of additional neurodevelopmental disabilities in children with profound bilateral sensorineural hearing loss and to investigate the relationship between disability and the etiology of deafness. METHODS: Eighty children with profound bilateral sensorineural hearing loss (mean age 5.4 years) were investigated by means of a diagnostic protocol including clinical, neurodevelopmental, and audiological procedures together with genetic and neurometabolic tests and neuroradiological investigation by brain MRI. RESULTS: Fifty-five percent of the sample exhibited one or more disabilities in addition to deafness, with cognitive, behavioural-emotional and motor disorders being the most frequent. The risk of additional disabilities varied according to aetiology, with a higher incidence in hereditary syndromic deafness, in cases due to pre-perinatal pathology (in comparison to unknown and hereditary non syndromic forms) and in the presence of major brain abnormalities at MRI. CONCLUSION: Our results suggest that the aetiology of deafness may be a significant risk indicator for the presence of neuropsychiatric disorders. A multidimensional evaluation, including aetiological, neurodevelopmental and MRI investigation is needed for formulating prognosis and for planning therapeutic intervention, especially in those children candidated to cochlear implant.

18 Article Cochlear implantation in patients affected by superficial hemosiderosis of the central nervous system. 2012

Berrettini, S / De Vito, A / Bruschini, L / Fortunato, S / Forli, F. ·Otology and Cochlear Implant Unit, University-Hospital of Pisa, Cisanello, Via Paradisa 2, 56124 Pisa, Italy. s.berrettini@med.unipi.it ·Eur Arch Otorhinolaryngol · Pubmed #21431954.

ABSTRACT: The objectives of the study were to: report three more cases of cochlear implantation (CI) in patients affected by superficial hemosiderosis of the central nervous system (SH-CNS); assess whether CI may be a viable option in this disease. The study was conducted in a tertiary referral center. Pre-operative and post-operative clinical notes of three patients with SH-CNS were reviewed. Two out of three cases showed very good results with CI in sentence perception in noise over 90%. For the other case, hearing performance was very low. He showed only disyllabic word identification abilities in a closed set (40%). Cochlear implantation may be a viable option for patients with severe to profound sensorineural hearing loss due to SH-CNS. In these cases, an adequate pre-operative counseling, explaining the possibility of poor post-operative results and/or the worsening of the outcomes in the following years, is important.

19 Article Universal neonatal audiological screening: experience of the University Hospital of Pisa. 2011

Ghirri, Paolo / Liumbruno, Annalisa / Lunardi, Sara / Forli, Francesca / Boldrini, Antonio / Baggiani, Angelo / Berrettini, Stefano. ·Mother and Child Department, Neonatology Unit and Section of Neonatal Endocrinology and Dysmorphology, University Hospital of Pisa, Pisa, Italy. pghirri@med.unipi.it ·Ital J Pediatr · Pubmed #21481246.

ABSTRACT: The early identification of pre-lingual deafness is necessary to minimize the consequences of hearing impairment on the future communication skills of a baby. According to the most recent international guidelines the deafness diagnosis must occur before the age of three months and the prosthetic-rehabilitative treatment with a traditional hearing aid should start within the first six months. When a Cochlear implant becomes necessary, the treatment should start between the age of 12 months and 18 months. The only way to diagnose the problem early is the implementation of universal neonatal audiological screening programs. Transient evoked otoacoustic emissions (TEOAE) is the most adequate test because it's accurate, economic and of simple execution. Automatic auditory brainstem response (AABR) is necessary to identify patients with auditory neuropathy but it is also important to reduce the number of false-positives.The 20-30% of infant hearing impairment is represented by progressive or late-onset hearing loss (HL) so it's also necessary to establish an audiological follow up program, especially in infants at risk.From November 2005 all neonates born in the University hospital of Pisa undergo newborn hearing screening. From 2008 the screening program follows the guidelines for the execution of the audiological screening in Tuscany which have been formulated by our group according to the 2007 JCIH Position Statement and adaptated to our regional reality by a multidisciplinary effort. From November 2005 to April 2009 8113 neonates born in the Neonatal Unit of Santa Chiara Hospital (Pisa) have undergone newborn hearing screening. 7621 neonates (93.9%) without risk factors executed only the TEOAE test. 492 (6.1%) neonates had audiological risk factors and thus underwent TEOAE and AABR. 84 patients (1,04%) failed both TEOAE and AABR tests. 78 of them underwent further investigations. 44 patients resulted false positives (the 0,54% of the screened newborns). 34 neonates (4,2 ‰) had a final diagnosis of hearing impairment. 8 patients (0.99 ‰) had unilateral hearing loss (HL). 26 patients (3,2 ‰) had bilateral hearing impairment.In our screening program the percentage of false-positives was quite low (0.54%) while the incidence of bilateral HL (3.2 ‰) is a little higher than that found in literature reports. In most of our patients premature birth or neonatal suffering represent the main cause of HL.

20 Article Fully implantable Otologics MET Carina(™) device for the treatment of sensorineural and mixed hearing loss: Audio-otological results. 2010

Bruschini, Luca / Forli, Francesca / Passetti, Susanna / Bruschini, Paolo / Berrettini, Stefano. ·Otology-Cochlear Implant Centre, Department of Neuroscience, University of Pisa, Italy. ·Acta Otolaryngol · Pubmed #20380547.

ABSTRACT: CONCLUSION: Our results attest that the Otologics MET Carina(™) is a viable treatment for moderate to severe sensorineural hearing loss (SNHL) and for cases of mixed hearing loss, and that in selected cases it could represent an alternative to conventional hearing aids. OBJECTIVES: To describe our experience with the fully implantable Carina(™) in eight adult patients, seven with moderate to severe SNHL and one with mixed hearing loss. METHODS: Eight implanted adult patients were submitted to a comprehensive audiological evaluation. RESULTS: We did not record any surgical relevant complication in any of the patients, nor any significant postoperative variation in hearing thresholds, for air conduction or bone conduction, indicating the absence of surgical damage to the cochlea. All the patients demonstrated improvements in speech perception abilities with the device functioning and reported subjective benefits. With regard to the postoperative adverse effects, we had problems with feedback noise, which resolved with minor fitting adjustments in seven cases, while it required a second surgery to change the microphone position in the other patient. In one case a minimal extrusion of the microphone cable occurred requiring a revision surgery, a device failure occurred in one case, requiring substitution, and one patient decided on explantation of the device owing to psychological problems.

21 Article Neurodevelopmental disorders in children with severe to profound sensorineural hearing loss: a clinical study. 2010

Chilosi, Anna M / Comparini, Alessandro / Scusa, Maria F / Berrettini, Stefano / Forli, Francesca / Battini, Roberta / Cipriani, Paola / Cioni, Giovanni. ·Department of Developmental Neuroscience, IRCCS Stella Maris, Pisa, Italy. achilosi@inpe.unipi.it ·Dev Med Child Neurol · Pubmed #20345959.

ABSTRACT: AIM: The effects of sensorineural hearing loss (SNHL) are often complicated by additional disabilities, but the epidemiology of associated disorders is not clearly defined. The aim of this study was to evaluate the frequency and type of additional neurodevelopmental disabilities in a sample of children with SNHL and to investigate the relation between these additional disabilities and the aetiology of deafness. METHOD: One hundred children with severe/profound SNHL (60 males, 40 females; mean age 5 y 7 mo, SD 3 y 6 mo, range 8 mo-16 y) were investigated using a diagnostic protocol including neurodevelopmental, genetic, neurometabolic, and brain magnetic resonance imaging (MRI) assessment. RESULTS: Forty-eight per cent of the sample exhibited one or more additional disabilities, with cognitive, behavioural-emotional, and motor disorders being the most frequent. The risk of additional disabilities varied according to the type of aetiology. Thirty-seven out of 80 individuals with available MRIs showed signal abnormalities, in particular brain malformations (46%) and white matter abnormalities (54%). Frequency and type of disability were associated with aetiology (p=0.015) and MRI data (p<0.001). INTERPRETATION: A multidimensional evaluation, including aetiological, neurodevelopmental, and MRI investigation, is needed for planning therapeutic intervention, such as cochlear implantation in children with severe to profound hearing impairment. The aetiology of deafness is a relevant risk indicator for the presence of an associated disorder.

22 Minor Newborn hearing screening protocol in tuscany region. 2017

Berrettini, Stefano / Ghirri, Paolo / Lazzerini, Francesco / Lenzi, Giovanni / Forli, Francesca. ·Otolaryngology Audiology and Phoniatric Unit, University of Pisa, Pisa, Italy. · Mother and Child Department, Neonatology Unit and Section of Neonatal Endocrinology and Dysmorphology, University Hospital of Pisa, Pisa, Italy. pghirri@med.unipi.it. · Paediatrician, National Coordinator of FIMP Audiology section, Grosseto, Italy. ·Ital J Pediatr · Pubmed #28931415.

ABSTRACT: BACKGROUND: Newborn hearing screening has to be considered the first step of a program for the identification, diagnosis, treatment and habilitation/rehabilitation of children with hearing impairment. MAIN PART: In Tuscany Region of Italy, the universal newborn hearing screening is mandatory since november 2007. The first guidelines for the execution of the screening have been released in June 2008; then many other Italian regions partially or totally adopted these guidelines. On the basis of the experience from 2008 and according to the recent evidences in the scientific literature, a new screening protocol was released in Tuscany region. The new protocol is an evolution of the previous one. Some issues reported in the previous protocol and in the Joint Committee on Infant Hearing statement published in 2007 were revised, such as the risk factors for auditory neuropathy and for late onset, progressive or acquired hearing loss. The new updated guidelines were submitted to the Sanitary Regional Council and then they have been approved in August 2016. The updated screening protocol is mainly aimed to identify newborns with a congenital moderate-to-profound hearing loss, but it also provides indications for the audiological follow-up of children with risk's factor for progressive or late onset hearing loss; further it provides indications for the audiological surveillance of children at risk for acquired hearing impairment. Then, in the new guidelines the role of the family paediatrician in the newborn hearing screening and audiological follow-up and surveillance is underscored. Finally the new guidelines provide indications for the treatment with hearing aids and cochlear implant, in accordance with the recent Italian Health Technology Assessment (HTA) guidelines. CONCLUSIONS: In the paper we report the modality of execution of the universal newborn hearing screening in the Tuscany Region, according to the recently updated protocol. The main features of the protocol and the critical issues are discussed.