Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies

Background. Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the management of language developmental disorders. Methods. The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations) on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests. Results. Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years). Among these, 143 patients (64 females and 79 males) presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children) ranged from 9 months and 16 years (average = 4.4; median = 3.0). Conclusions. Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities, even if central nervous system anomalies represent a negative prognostic factor that is made worse by the concomitant presence of cochlear malformations. Common cavity and stenosis of the internal auditory canal (less than 2 mm) are negative prognostic factors even if brain lesions are absent

Bilateral osseous stenosis of the internal auditory canal: case report

Osteomas as well as exostoses of the internal auditory canal are rare, benign, usually slow-growing lesions. The most common localizations of these temporal bone lesions are the mastoid cortex and the external auditory canal. A rare case is reported of bilateral osseous stenosis of the internal auditory canal, in the absence of clinical (auditory, vestibular and facial nerve) symptoms. In the absence of auditory, vestibular and/or facial nerve symptoms, long-term follow-up should be assessed; surgical intervention may be warranted only if symptoms are present

Malignant otitis externa in the antibiotic resistance era: key to successful treatment

Malignant otitis externa in the antibiotic resistance era: key to successful treatment. Objective: Malignant otitis externa (MOE) is a rare aggressive, necrotizing infection of the external auditory canal and the temporal bone. MOE may have a poor prognosis when it is not treated promptly and adequately. It is most commonly reported in males, older individuals, patients with diabetes, or patients that are immunocompromised. Pseudomonas aeruginosa is the main pathogenic agent involved. This study aimed to evaluate a clinical series of patients with MOE and discuss the current literature on the topic. Methodology: This retrospective study included 8 patients with MOE that were evaluated and treated, medically and/ or surgically, at the University Hospital of Ferrara between January 2012 and December 2016. We retrieved data from medical records on the clinical history, imaging, and treatment. Results: In all cases, a microbiological examination disclosed the presence of P. aeruginosa. The infection was eventually controlled in all cases, after a median of 6 months of therapy. All patients were followed-up for an average of 12 months after infection resolution. Conclusion: Currently, no specific guidelines for MOE treatment are available in the literature. Based on our findings, we proposed a diagnostic and therapeutic flow-chart for managing this infection

Sindrome di charge: risultati a lungo termine della riabilitazione audiologica

Obiettivo del presente lavoro è valutare i risultati della riabilitazione audiologica su un gruppo, numericamente consistente, di bambini affetti da sindrome di CHARGE. Lo studio è stato eseguito retrospettivamente, utilizzando il database dei pazienti pediatrici, presso l’Audiologia dell’Azienda Ospedaliero-Universitaria di Padova e di Ferrara. Sono stati individuati 31 bambini in totale, che hanno presentato diversi gradi di disabilità uditiva associata alla sindrome di CHARGE. La valutazione audiologica è stata eseguita utilizzando i potenziali evocati uditivi (ABR) e/o l’elettrococleografia, oppure le tecniche di audiometria infantile (VRA o play audiometry). Sono stati valutati anche i risultati percettivi, in termini di capacità di comunicazione e linguaggio espressivo. Sono quindi stati studiati gli effetti della riabilitazione uditiva (con apparecchio acustico o impianto cocleare) e in particolare lo sviluppo del linguaggio nel corso di un lungo follow-up. Gli esiti degli interventi riabilitativi sono risultati diversi in relazione alle eterogenee e spesso gravi disabilità associate alla sindrome di CHARGE (ad esempio, ritardo di sviluppo psico-fisico, gravi disturbi visivi concomitanti, disfunzioni uditive retrococleari per neuropatia uditiva/dissincronia associata). Anche dopo lungo follow-up, lo sviluppo del linguaggio è risultato gravemente compromesso nella maggior parte dei casi, suggerendo quindi la necessità di sviluppare modalità di comunicazione alternative in questo gruppo di piccoli Pazienti. L’identificazione precoce della sordità neurosensoriale e l’accurata pianificazione di trattamenti riabilitativi mirati, è in ogni caso fondamentale nei bambini con sindrome di CHARGE

Dolore post-operatorio nei pazienti affetti da neoplasia testa-collo: fattori predittivi ed efficacia della terapia

Negli anni è aumentata l’attenzione verso i molteplici aspetti associati alla “sfera” dolore, anche nei pazienti oncologici sottoposti a chirurgia testa-collo. Il dolore, definito infatti da diverse caratteristiche, quali l’esperienza personale, gli aspetti qualitativi della percezione, l’intensità, l’impatto emotivo, riconosce un’eziologia “multifattoriale”. Scopo del presente lavoro è stato: (i) valutare l’efficacia della terapia analgesica in pazienti affetti da tumore testa-collo e sottoposti a trattamento chirurgico; (ii) studiare le possibili variabili ed i fattori predittivi che possano influenzare l’insorgenza di dolore. Sono stati studiati 164 pazienti, affetti da neoplasia maligna del distretto testa-collo, trattati chirurgicamente tra il dicembre 2009 ed il dicembre 2013. I dati raccolti comprendono l’età, il sesso, la valutazione del rischio anestesiologico, la sede del tumore, la stadiazione TNM, il tipo di intervento effettuato, la complessità e la durata dell’intervento, le eventuali complicanze post-operatorie, i giorni di degenza post-intervento, la valutazione del dolore nei giorni 0, 1, 3 e 5 post-chirurgia. L’adeguatezza della terapia analgesica è stata espressa in termini di incidenza e prevalenza del dolore post-operatorio, le variabili legate al paziente, alla malattia, al trattamento chirurgico e farmacologico, sono state poi associate all’insorgenza del dolore così da poter descrivere eventuali fattori predittivi. Dai dati ottenuti emerge che la popolazione studiata ha ricevuto un’adeguata terapia antalgica, sia nell’immediato post-operatorio che nei giorni successivi. Non sono risultate associazioni statisticamente significative tra sesso, età ed incidenza del dolore post-chirurgico, mentre lo stadio del tumore, la complessità dell’intervento chirurgico e la sede della neoplasia hanno presentano correlazione significativa con il rischio di insorgenza di dolore post-operatorio. L’elevata prevalenza del dolore in ambito oncologico testa-collo, fa sì che un’appropriata ed attenta gestione del dolore risulti fondamentale. Nel futuro pertanto si auspica una sempre migliore comprensione dei fattori biologici, sociali e psicologici che caratterizzano la percezione del dolore ai fini di migliorarne il controllo

A Multicenter Clinical Evaluation of Data Logging in Cochlear Implant Recipients Using Automated Scene Classification Technologies

Currently, there are no studies assessing everyday use of cochlear implant (CI) processors by recipients by means of objective tools. The Nucleus 6 sound processor features a data logging system capable of real-time recording of CI use in different acoustic environments and under various categories of loudness levels. In this study, we report data logged for the different scenes and different loudness levels of 1,366 CI patients, as recorded by SCAN. Monitoring device use in cochlear implant recipients of all ages provides important information about the listening conditions encountered in recipients' daily lives that may support counseling and assist in the further management of their device settings. The findings for this large cohort of active CI users confirm differences between age groups concerning device use and exposure to various noise environments, especially between the youngest and oldest age groups, while similar levels of loudness were observed

A case of temporal arteritis presenting with sensorineural hearing loss

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Immune-mediated inner ear disease

The incidence of autoimmune inner ear disease (AIED) is difficult to determine: probably it is a rare disease, accounting for <1% of all cases of hearing impairment or dizziness. Nevertheless, the diagnosis of AIED might be overlooked because of the lack of a specific diagnostic test. The hallmark of this clinically diagnosed condition is the presence of a rapidly progressive, often fluctuating, bilateral sensorineural hearing loss (SNHL) over a period of weeks to months. The progression of hearing loss is too rapid to be diagnostic for presbycusis and too slow to conclude a diagnosis of sudden SNHL. Vestibular symptoms, such as generalized imbalance, ataxia, positional vertigo and episodic vertigo may be present in almost 50% of patients. Occasionally only one ear is affected initially, but bilateral hearing loss occurs in most patients, with symmetric or asymmetric audiometric thresholds. Almost 25-50% of patients also have tinnitus and aural fullness, which can fluctuate. Systemic autoimmune diseases coexist in 15-30% of patients