TY - JOUR T1 - Achieving early functional auditory access in paediatric cochlear implantation. JF - Acta Otorhinolaryngol Ital Y1 - 2016 A1 - Orzan, E A1 - Muzzi, E A1 - Marchi, R A1 - Falzone, C A1 - Battelino, S A1 - Ciciriello, E AB -

Cochlear implantation (CI) is a viable option for providing access to auditory stimulation in severe-to-profound hearing loss/impairment of cochlear origin. It has been demonstrated that CI is safe and effective for deaf children. Younger age at activation after CI is linked with better outcomes. It is important to study variables and issues that can interfere with an early fitting and access to sound after CI. They range from patient characteristics, family compliance and support, to technical, medical or organisational problems. A SWOT analysis and a subsequent TOWS matrix was conducted to discuss issues and propose recommendations to be considered when operating an early switch on of the CI.

VL - 36 IS - 1 U1 - http://www.ncbi.nlm.nih.gov/pubmed/27054390?dopt=Abstract ER - TY - JOUR T1 - Achieving effective hearing aid fitting within one month after identification of childhood permanent hearing impairment. JF - Acta Otorhinolaryngol Ital Y1 - 2016 A1 - Bastanza, G A1 - Gallus, R A1 - De Carlini, M A1 - Picciotti, P M A1 - Muzzi, E A1 - Ciciriello, E A1 - Orzan, E A1 - Conti, G AB -

Diagnosis of child permanent hearing impairment (PHI) can be made with extreme timeliness compared to the past thanks to improvements in PHI identification through newborn hearing screening programmes. It now becomes essential to provide an effective amplification as quickly as possible in order to restore auditory function and favour speech and language development. The early fitting of hearing aids and possible later cochlear implantation indeed prompts the development of central auditory pathways, connections with secondary sensory brain areas, as well as with motor and articulatory cortex. The aim of this paper is to report the results of a strategic analysis that involves identification of strengths, weaknesses, opportunities and threats regarding the process of achieving early amplification in all cases of significant childhood PHI. The analysis is focused on the Italian situation and is part 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".

VL - 36 IS - 1 U1 - http://www.ncbi.nlm.nih.gov/pubmed/27054389?dopt=Abstract ER - TY - JOUR T1 - Childhood hearing surveillance activity in Italy: preliminary recommendations. JF - Acta Otorhinolaryngol Ital Y1 - 2016 A1 - Orzan, E A1 - Ruta, F A1 - Bolzonello, P A1 - Marchi, R A1 - Ceschin, F A1 - Ciciriello, E AB -

Following the positive outcomes of the newborn hearing screening programmes already underway in several Italian regions, it is now necessary to address the identification of childhood hearing impairments that missed the neonatal screening programme or have delayed onset. Within the framework of the Ministry of Health project CCM 2013 "Preventing Communication Disorders: a Regional Program for early Identification, Intervention and Care of Hearing Impaired Children", a group of professionals identified three main recommendations that can be useful to improve hearing surveillance activity within the regional and state Italian Health System. The family paediatrician is recognised as having a key role in ongoing monitoring of hearing capacity and development of the growing child.

VL - 36 IS - 1 U1 - http://www.ncbi.nlm.nih.gov/pubmed/27054386?dopt=Abstract ER - TY - JOUR T1 - Detection of Malawi polyomavirus sequences in secondary lymphoid tissues from Italian healthy children: a transient site of infection. JF - Virol J Y1 - 2016 A1 - Papa, N A1 - Zanotta, N A1 - Knowles, A A1 - Orzan, E A1 - Comar, M AB -

BACKGROUND: The novel Malawi polyomavirus (MWPyV) was initially detected in stool specimens from healthy children and children with gastrointestinal symptoms, mostly diarrhea, indicating that MWPyV might play a role in human gastroenteric diseases. Recently, MWPyV sequences were additionally identified in respiratory secretions from both healthy and acutely ill children suggesting that MWPyV may have a tropism for different human tissues. This study was designed to investigate the possible sites of latency/persistence for MWPyV in a cohort of healthy Italian children.

METHODS: Specimens (n° 500) of tonsils, adenoids, blood, urines and feces, from 200 healthy and immunocompetent children (age range: 1-15 years) were tested for the amplification of the MWPyV LT antigen sequence by quantitative real-time PCR. Samples (n° 80) of blood and urines from 40 age-matched children with autoimmune diseases, were screened for comparison. Polyomaviruses JC/BK and Epstein-Barr Virus (EBV) were also tested as markers of infection in all samples using the same molecular technique.

RESULTS: In our series of healthy children, MWPyV was detected only in the lymphoid tissues showing a prevalence of 6 % in tonsils and 1 % in adenoids, although with a low viral load. No JCPyV or BKPyV co-infection was found in MWPyV positive samples, while EBV showed a similar percentage of both in tonsils and adenoids (38 and 37 %). Conversely, no MWPyV DNA was detected in stool from babies with gastroenteric syndrome. With regards to autoimmune children, neither MWPyV nor BKPyV were detected in blood, while JCPyV viremia was observed in 15 % (6/40) of children treated with Infliximab. Urinary BKPyV shedding was observed in 12.5 % (5/40) while JCPyV in 100 % of the samples.

CONCLUSIONS: The detection of MWPyV sequences in tonsils and adenoids of healthy children suggests that secondary lymphoid tissues can harbour MWPyV probably as transient sites of persistence rather than actual sites of latency.

VL - 13 U1 - http://www.ncbi.nlm.nih.gov/pubmed/27287743?dopt=Abstract ER - TY - JOUR T1 - Empowering the family during the first months after identification of permanent hearing impairment in children. JF - Acta Otorhinolaryngol Ital Y1 - 2016 A1 - Ciciriello, E A1 - Bolzonello, P A1 - Marchi, R A1 - Falzone, C A1 - Muzzi, E A1 - Orzan, E AB -

The latest international guidelines highlight the importance of involving the family in the diagnostic and rehabilitation process of children affected by permanent hearing impairment. This emphasises how meaningful this approach is for the development of the deaf child. So far, there is very little evidence about this approach in Italy, and there are still some barriers to its practical management. The aim of this paper is to report the results of a strategic analysis, which identifies the strengths, weaknesses, opportunities and threats of the family empowerment process during early auditory diagnosis and rehabilitation. The audiology programme should have the goal to offer information and support to families in order to achieve a conscious decision about the use and type of auditory prosthesis and rehabilitation choice within three months after audiologic diagnosis. Within the framework of the Ministry of Health project CCM 2013 "Preventing Communication Disorders: a Regional Program for Early Identification, Intervention and Care of Hearing Impaired Children", a group of professionals identified three main recommendations that can be useful to foster the natural communicative development of the child by strengthening the therapeutic alliance and empowerment of the family. The recommendations obtained with this analysis can help to develop new Italian guidelines with the aim to foster natural communicative development of the child by strengthening the therapeutic alliance and empowerment of the family.

VL - 36 IS - 1 U1 - http://www.ncbi.nlm.nih.gov/pubmed/27054393?dopt=Abstract ER - TY - JOUR T1 - Improving regional universal newborn hearing screening programmes in Italy. JF - Acta Otorhinolaryngol Ital Y1 - 2016 A1 - Molini, E A1 - Cristi, M C A1 - Lapenna, R A1 - Calzolaro, L A1 - Muzzi, E A1 - Ciciriello, E A1 - Della Volpe, A A1 - Orzan, E A1 - Ricci, G AB -

The Universal Newborn Hearing Screening (UNHS) programme aims at achieving early detection of hearing impairment. Subsequent diagnosis and intervention should follow promptly. Within the framework of the Ministry of Health project CCM 2013 "Preventing Communication Disorders: a Regional Program for early Identification, Intervention and Care of Hearing Impaired Children", the limitations and strengths of current UNHS programs in Italy have been analysed by a group of professionals working in tertiary centres involved in regional UNHS programmes, using SWOT analysis and a subsequent TOWS matrix. Coverage and lost-to-follow up rates are issues related to UNHS programmes. Recommendations to improve the effectiveness of the UNHS programme have been identified. The need for homogeneous policies, high-quality information and dissemination of knowledge for operators and families of hearing-impaired children emerged from the discussion.

VL - 36 IS - 1 U1 - http://www.ncbi.nlm.nih.gov/pubmed/27054385?dopt=Abstract ER - TY - JOUR T1 - Planning early childhood audiologic intervention programs on a regional scale: introduction to an Italian study. JF - Acta Otorhinolaryngol Ital Y1 - 2016 A1 - Orzan, E A1 - Ciciriello, E AB -

Non-uniform, late, or inappropriate care of childhood with permanent hearing impairment (PHI) predisposes many children to develop communicative- behaviour problems and impaired psychosocial adjustment that can persist in adolescence and adulthood.In March 2014, the CCM (Centro Controllo Malattie or Disease Control Centre) of the Italian Ministry of Health funded a project entitled " Preventing Communication Disorders: a Regional Program for Early Identification, Intervention and Care of Hearing Impaired Children". The project involved 5 tertiary centres with UNHS programs formally approved by the Region. The main purpose of the project is to define and launch an integrated regionally-based public health model for identification, diagnosis and intervention of childhood PHI. The first phase of the project investigated the state of art and produced recommendations for positive changes in identification, diagnosis, therapy and care of childhood PHI in Italy, taking into account diagnostic and treatment innovations, family empowerment, treatment alliance and an interdisciplinary approach. Recommendations drawn from this initial phase will represent the basis for a regional system for early intervention that is validated, integrated and shared between the five regions.

VL - 36 IS - 1 U1 - http://www.ncbi.nlm.nih.gov/pubmed/27054384?dopt=Abstract ER - TY - JOUR T1 - Life-threatening unilateral hearing impairments. Review of the literature on the association between inner ear malformations and meningitis. JF - Int J Pediatr Otorhinolaryngol Y1 - 2015 A1 - Muzzi, E A1 - Battelino, S A1 - Gregori, M A1 - Pellegrin, A A1 - Orzan, E AB -

BACKGROUND: Bacterial meningitis is a life threatening disease that can be triggered by a CSF leak through an inner ear malformation. Early identification of the specific type of cochleovestibular dysplasia and the associated risk of meningitis is of vital importance.

OBJECTIVES: The objective of this review is to collect and discuss available data on the association between inner ear malformations and meningitis in children.

METHODS: Electronic databases were crosschecked for obtaining relevant papers published in the last 20 years, and further cases were identified by hand searching through the references. Demographic data were extracted from full texts, together with information on the severity of hearing impairment, the type of inner ear anomaly, the site of cerebrospinal fluid leak, the number of recurrent meningitis episodes.

RESULTS: Sixty-seven cases of meningitis related to inner ear malformation have been identified among 45 papers. Mean age at presentation is 3.60±3.00 (range 0.1-14) years. Average diagnostic delay from the first episode of meningitis is 3.44±3.41 (range 0.00-10.00) years. The number of meningitis episodes that occurred before the correct diagnosis and definitive surgical treatment is 3.27±1.81 (range 1.00-10.00). Unilateral hearing impairment affects 70% of patients. Six patients had normal hearing at presentation. Two children are dead from inner-ear-malformation-related meningitis among reviewed reports.

CONCLUSION: A high number of paediatric patients carrying inner ear malformations, especially when associated with unilateral hearing impairment, could be at risk to develop recurrent bacterial meningitis. Universal newborn hearing screening programs should prompt a diagnostic work-up even in the case of unilateral hearing impairment, in order to prevent inner ear malformation-related meningitis.

VL - 79 IS - 12 U1 - http://www.ncbi.nlm.nih.gov/pubmed/26453271?dopt=Abstract ER -