An audiological perspective on ''Two further patients with Warsaw breakage syndrome. Is a mild phenotype possible?"

n/

Vestibular anomalies and dysfunctions in children with inner ear malformations: A narrative review

About 20% of children with congenital hearing loss present malformations of the inner ear. In the past few years much has been understood about the morphology and function of the anterior part of the labyrinth, since hearing loss may have a dramatic effect on the overall development of a child. Nowadays, for most of them, a chance for hearing rehabilitation is available, making hearing loss a treatable condition. The anomalies range from the lack of development of the whole inner ear to specific anomalies of isolated structures. Despite the frequent concomitant involvement of the posterior part of the labyrinth, this part of the inner ear is frequently neglected while discussing its morphology and dysfunction. Even though vestibular and balance function/dysfunction may have a significant impact on the global development of children, very little is known about these specific disorders in patients with inner ear malformations. The aim of this review is to summarize the available literature about vestibular anomalies and dysfunctions in children with inner ear malformations, discussing what is currently known about the topic

Application of Patients Reported Outcome measures in Cochlear Implant patients: implications for the design of specific rehabilitation programs

Introduction: Cochlear implants (CI) had been developed to enable a satisfying verbal communication, music experiences have remained in the background of research and development and consequently many CI users are dissatisfied by the music they listen to. Nonetheless, concise indications for clinicians to test music abilities and prescribe rehabilitation programs are still lacking. The main aim of the present study was to test the utility of the application of two different Patient Reporting Outcomes (PRO) measures in a group of CI users. A secondary objective was to identify items capable of driving the indication and design specific music rehabilitation programs for CI patients. Material and Methods: A consecutive series of 73 CI patients referred to the Audiology Unit - University of Padova - was enrolled from November 2021 to May 2022 and evaluated with audiological battery test and PRO measures: Musica e Qualità della Vita (MUSQUAV) and Nijmegen Cochlear Implant Questionnaire (NCIQ) Italian version. Results: The Reliability analysis showed good consistency between the different PRO measures, (Cronbach alpha = 0.873). After accounting for epidemiological and clinical variables, PRO measures showed a correlation with audiological outcomes in only one case (rho=-0.170 for NCIQ-T with CI-Pure Tone Average. A willingness for musical rehabilitation was present in 63% of patients, (Rehab Factor, mean value of 0.791±0.675). Conclusions: we support the role of the application of MUSQUAV and NCIQ to improve the clinical and audiological evaluation of CI patients. Moreover, we proposed a derivative item, called Rehab Factor, which could be used in clinical practice and future studies to clarify the indication and priority of specific music rehabilitation programs

Auditory cortex hypoperfusion: a metabolic hallmark in Beta Thalassemia

Abstract Background Sensorineural hearing loss in beta-thalassemia is common and it is generally associated with iron chelation therapy. However, data are scarce, especially on adult populations, and a possible involvement of the central auditory areas has not been investigated yet. We performed a multicenter cross-sectional audiological and single-center 3Tesla brain perfusion MRI study enrolling 77 transfusion-dependent/non transfusion-dependent adult patients and 56 healthy controls. Pure tone audiometry, demographics, clinical/laboratory and cognitive functioning data were recorded. Results Half of patients (52%) presented with high-frequency hearing deficit, with overt hypoacusia (Pure Tone Average (PTA) > 25 dB) in 35%, irrespective of iron chelation or clinical phenotype. Bilateral voxel clusters of significant relative hypoperfusion were found in the auditory cortex of beta-thalassemia patients, regardless of clinical phenotype. In controls and transfusion-dependent (but not in non-transfusion-dependent) patients, the relative auditory cortex perfusion values increased linearly with age (p < 0.04). Relative auditory cortex perfusion values showed a significant U-shaped correlation with PTA values among hearing loss patients, and a linear correlation with the full scale intelligence quotient (right side p = 0.01, left side p = 0.02) with its domain related to communication skills (right side p = 0.04, left side p = 0.07) in controls but not in beta-thalassemia patients. Audiometric test results did not correlate to cognitive test scores in any subgroup. Conclusions In conclusion, primary auditory cortex perfusion changes are a metabolic hallmark of adult beta-thalassemia, thus suggesting complex remodeling of the hearing function, that occurs regardless of chelation therapy and before clinically manifest hearing loss. The cognitive impact of perfusion changes is intriguing but requires further investigations

A Peculiar Case of Ossicular Chain Fixation and Enlarged Vestibular Aqueduct

We present the case of a patient treated as affected by conductive hearing loss due to recurrent otitis, then as a juvenile otosclerosis, who was finally diagnosed as affected by bilateral ossicular chain fixation and enlarged vestibular aqueduct by means of cone-beam CT

Progetti REDD+: nuove frontiere e vecchie barriere nel mercato forestale del carbonio.

I processi di deforestazione e degradazione delle foreste, in gran parte concentrati nei Paesi tropicali, sono responsabili di almeno il 15% delle emissioni di gas serra (Van der Werf et al., 2009). La Food and Agriculture Organization (FAO) delle Nazioni Unite stima infatti per il periodo 2000-2010 una perdita annuale di foreste pari a 13 milioni di ettari (una superficie pari alla Grecia) concentrata in Sud America, Africa e Sud Est Asiatico, compensata solo in parte da 7,8 milioni di ettari anno tra piantagioni estensive ed espansione naturale delle foreste rispettivamente concentrate in Cina ed Europa. Uno degli strumenti principali sviluppati per mitigare i cambiamenti climatici \ue8 la creazione di mercati delle quote di emissione di gas di serra. I mercati interessano anche il settore forestale: in linea teorica \ue8 del tutto evidente e logico che l\u2019espansione delle aree boscate, il miglioramento degli stock e la prevenzione dei fenomeni di degrado delle foreste sono dei processi analoghi ma di segno opposto rispetto alle emissioni di gas di serra e possono quindi creare \u201ccrediti\u201d di emissione. In effetti nel mercato volontario del carbonio, creato su iniziativa delle imprese, del settore non-profit e supportato occasionalmente da amministrazioni pubbliche locali, i rimboschimenti, i miglioramenti dello stock e i progetti di controllo del degrado delle foreste sono da tempo pienamente riconosciuti. Nel mercato \u201cistituzionale\u201d, legato al Protocollo di Kyoto (PK) e agli accordi ufficiali dei Governi, solo le piantagioni sono inclusi nello scambio di quote, peraltro sotto forti condizioni restrittive. I problemi generali e le potenzialit\ue0 dell\u2019inclusione del settore forestale nel quadro normativo internazionale del mercato del carbonio verranno discussi nella prima parte del presente contributo, mentre nella seconda parte si illustreranno i problemi particolari legati all\u2019attivazione di progetti in grado di ridurre la deforestazione e la degradazione delle foreste tropicali (i progetti Reducing Emissions from Deforestation and Forest Degradation - REDD). Nelle considerazioni finali verr\ue0 nello specifico analizzato il ruolo delle risorse forestali nella strategia italiana di riduzione delle emissioni di gas serra