Alessandro Castiglione - Academia.edu (original) (raw)

Papers by Alessandro Castiglione

Acta otorhinolaryngologica Italica : organo ufficiale della Società italiana di otorinolaringologia e chirurgia cervico-facciale, 2014

Kaposi's sarcoma (KS) is a multicentric, malignant neoplastic vascular disease, mainly involv... more Kaposi's sarcoma (KS) is a multicentric, malignant neoplastic vascular disease, mainly involving skin and mucosae, characterised by the proliferation of endothelial cells. The aetiology of KS still is unknown. Nonetheless, it has been reported that several epidemiological and environmental factors may play a role in its pathogenesis. Viral factors (i.e. human herpes virus 8, HHV-8) have also been claimed to play a role in the onset of KS. Four main clinical presentations of KS have been described: classic (sporadic), African (endemic), iatrogenic (immunosuppression-associated) and AIDS-associated (epidemic). The authors present a case of KS involving the external ear of a HIVnegative patient with a history of non-Hodgkin lymphoma and tuberculosis.

Introduction. Restoring a sensory function in older adults might allow a significant improvement ... more Introduction. Restoring a sensory function in older adults might allow a significant improvement in their cognitive status. Although specific clinical conditions could compromise management and drastically reduce the chance of acceptable outcomes, auditory rehabilitation with cochlear implants or hearing aids still remains one of the most effectiveness procedure. Advances in research and technology suggest a functional “adaptation” in central processes that could influence other related or not strictly related activities, such as memory and working memory, frontal and pre-frontal processes, orientation, calculation, logic and executive functions. Since the link between hearing loss and cognitive decline has been clarified, scientific community is currently finding out the lacking evidence of effectiveness of auditory rehabilitation in reducing or counteracting cognitive decline. Material and Methods. Hearing impaired patients with more than 65 years of age, affected with mild to pro...

Hearing loss in older adults is associated with both cognitive impairment and aging. Consequently... more Hearing loss in older adults is associated with both cognitive impairment and aging. Consequently, the auditory rehabilitation of the elderly should not be limited to restore hearing function and should take into careful consideration the effects, causes, and consequences of the sensorial deprivation on central processes in terms of speech perception and quality of life. Auditory rehabilitation can reduce the cognitive “load” (the neural activity needed to achieve a task, in particular understanding/recognizing the spoken word), social isolation, anxiety, and depression. Dizziness represents largely a problem in the elderly, being the most common reason patients over the age of 75 years seek medical attention. Different causes for such sensations are reported. Cardiovascular, neurological, or locomotor disease, deterioration in sensory organs, vestibular dysfunction, and adverse drug effects are the most common. A multifactorial approach could be the best solution for a number of el...

Hearing, Balance and Communication, 2019

Purpose: A lot of energy has been spent in advances in audiology to define a new strategy of diag... more Purpose: A lot of energy has been spent in advances in audiology to define a new strategy of diagnosis and rehabilitation in hearing-impaired patients, in particular for those rehabilitated with cochlear implants or hearing aids. The OLSA test or Matrix Sentence test is a new testing tool able to precisely define a signal-to-noise ratio in different clinical or diagnostic conditions. Spreading of this new test has a standardized level of reference which can help clinicians in comparing patients and data. Method: Implanted patients with contralateral hearing aid use were enrolled in the recent Italian Matrix Sentence test validated for the Italian language. Patients with good perceptual outcomes without post-operative complications were asked to undergo the Italian Matrix Sentence test twice in a day section with or without a cochlear implant and/or contralateral hearing aids. Results: Forty-five cochlear implanted patients with good outcomes and without major complications were enrolled in the present study to compare speech discrimination in noise with cochlear implants and/or hearing aids during bimodal or unimodal stimulation. Obviously, a significant difference was noticed in patients with cochlear implants or hearing aids when compared with patients without digital devices, as well as a statistically significant difference was found in patients with a cochlear implant only when compared with patients with hearing aids. Nevertheless, significant differences were found when comparing bimodal stimulation to unimodal stimulation with the cochlear implant: an ameliorative trend has been reported among patients with either the devices (hearing aids and cochlear implant). Conclusion: The Italian Matrix Sentence test is useful and automated speech audiometry allows a comprehensive evaluation of perceptual abilities in hearing impaired patient. Nevertheless, gives important information among implanted patients especially in the adaptive mode for central comprehension of the signal in noise.

Annales françaises d'Oto-rhino-laryngologie et de Pathologie Cervico-faciale, 2012

This study is the first recognition of genetic implications (i.e. SNPs) in the field of sudden se... more This study is the first recognition of genetic implications (i.e. SNPs) in the field of sudden sensorineural hearing loss. In detail, SNPs of iron homeostasis genes seem to have a role in sudden sensorineural hearing loss establishment. Pharmacogenetics aspects have been discussed

International Journal of Pediatric Otorhinolaryngology, 2013

Cochlear implantation is a relatively safe procedure with a low complication rate. The overall ra... more Cochlear implantation is a relatively safe procedure with a low complication rate. The overall rate of complications among cochlear implant patients ranges from 6% to 20%. Major complications are those that are life-threatening or require surgery, whereas minor complications are those that can be medically treated. Nonetheless, certain complications, even if highly rare, may require specific investigations and treatments. Among these rare complications are those with endocochlear involvement, such as cochleitis or labyrinthitis, with fibrosis or ossification that could lead to explantation. The aims of the present study were to report a particular case of post-operative cochleitis and to review the rate of complications after cochlear implantation, emphasising those conditions with proven endocochlear involvement. Methods: We refer to the case of an eight-year-old Italian boy affected by the sudden onset of headache, ipsilateral otalgia and facial paresis, who presented to our clinic for inexplicable worsening of the performance of his implant and his residual hearing, six years after surgery. A complete investigation including (clinical history, routine, autoimmune and serological blood tests, electrophysiological measurements from the cochlear implant and neuroimaging) was performed and is herein described. Additionally, a comprehensive review of the literature was conducted using internet search engines; 274 papers were selected, 88 of which were best suited to our purposes. Results: In our case, the progression of the symptoms and the performance decrement required explantation, followed by a complete recovery. Reviewing the literature revealed only three reports concerning cases of proven endocochlear phlogosis that required revision surgery. Wound swelling/ infection and vertigo remain the two most common complications of cochlear implantation. Failure of the device is the third most frequent complication (10.06% of all complications and 1.53% of cochlear implantations). Other rare conditions (such as granulating labyrinthitis with cochlear fibrosis, ossification and erosion, silicone allergy and the formation of a biofilm around the internal device) are possible and unpredictable. Although rare (approximately 1%), such cases may require explantation. Conclusions: Despite efforts by both surgeons and manufacturers, device-related and surgical complications still occur. These and other rare conditions demand specific management, and their frequency may be underestimated. Further studies are needed to assess more realistic rates of complications and devise more efficient strategies for early diagnosis and treatment.

Mutations in the SLC26A4 gene (7q22.3-7q31.1) are considered one of the most common causes of gen... more Mutations in the SLC26A4 gene (7q22.3-7q31.1) are considered one of the most common causes of genetic hearing loss. There are two clinical forms related to these mutations: syndromic and non-syndromic deafness. The first one is named Pendred Syndrome (PS) when deafness is associated with thyroid goiter; the second is called DFNB4, when no other symptoms are present. Both are transmitted as an autosomal recessive trait, but simple heterozygotes can develop both forms of deafness. Actually it is thought that Pendred Syndrome occurs when both alleles of SLC26A4 gene are mutated; DFNB4 seems due to monoallelic mutations. PS and DFNB4 can be associated with inner ear malformations. In most of the cases (around 80%), these consist in Enlarged Vestibular Aqueduct (EVA). EVA can also be present without SLC26A4 mutations. Understanding the role of new SLC26A4 variants should facilitate clinical assessment, as well as diagnostic and therapeutic approaches. This investigation aims to detect and report genetic causes of two unrelated Italian boys with hearing loss. Patients and family members underwent clinical, audiological and genetic evaluations. To identify genetic mutations, DNA sequencing of SLC26A4 gene (including all 21 exons, exon-intron boundaries and promoter region) was carried out. Both probands were affected by congenital, progressive and fluctuating mixed hearing loss. Temporal bone imaging revealed a bilateral EVA with no other abnormalities in both cases. Probands were heterozygotes for previously undescribed mutations in the SLC26A4 gene: R409H/IVS2+1delG (proband 1) and L236P/K590X (proband 2). No other mutations were detected in GJB2, GJB6 genes or mitochondrial DNA (mit-DNA). The IVS2+1delG and K590X mutations have not yet been described in literature but there is some evidence to suggest that they have a pathological role. The results underlined the importance of considering the complete DNA sequencing of the SLC26A4 gene for differential molecular diagnosis of deafness, especially in those patients affected by congenital, progressive and fluctuating mixed hearing loss with bilateral EVA.

Hearing, Balance and Communication, 2014

Oculo-auriculo-vertebral spectrum is a rare, heterogeneous congenital condition, of unknown aetio... more Oculo-auriculo-vertebral spectrum is a rare, heterogeneous congenital condition, of unknown aetiology, mainly involving the ear (microtia) and jaw (hemifacial microsomia). In addition to those structures originating from the fi rst and second pharyngeal arches, multiple systems can be affected. ENT specialists and audiologists may focus their attention on the microtia and its effect on the hearing capacity of the child, but it may be only a sign of a more complex spectrum of abnormalities. A complete study of auditory function is crucial, but not exclusively. Proper management would consider the neuroradiological study not only of the whole hearing organ, but also of the cranial nerves, soft tissues and craniofacial structures. A geneticist should investigate the family history in order to identify a specifi c pattern of inheritance. A maxillofacial surgeon and orthodontic assessment may be appropriate when patients present with hemifacial microsomia; an ophthalmological evaluation should be considered when ocular impairment is evident or suspected. A total body examination is crucial to disclose abnormalities involving other organs or systems. If further malformations are suspected, then additional radiological investigation and a specialist examination may be warranted.

European Journal of Clinical Investigation, 2009

Figure 1 Contrast-enhanced T1-weighted coronal MR image: meningeal enhancement on T1C+ in supra a... more Figure 1 Contrast-enhanced T1-weighted coronal MR image: meningeal enhancement on T1C+ in supra and infra-tentorial region extended into the left IAC (arrow).

European Archives of Oto-Rhino-Laryngology, 2011

Our objective is to determine the complication rate in a population of infants, children, adolesc... more Our objective is to determine the complication rate in a population of infants, children, adolescents and adults, from a University Hospital Cochlear Implant program and to discuss their causes and treatments. The methods include a retrospective study of 438 consecutive patients in a tertiary referral centre, the Audiology Department of the University Hospital of Ferrara. All patients receiving cochlear implants, between 1 January 2003 and 31 December 2009, have been included. All complications and treatments were systematically reviewed with an average duration of follow-up of 46 months (range 10-84 months). The results reveal that the overall rate of complications in our group was 9.1% (40 of 438), and most of them were minor. Wound swelling and infections represent the most common complication occurred. There were no cases of transient or permanent facial palsy following surgery, and also we did not register any case of postsurgical meningitis. Thirteen patients (3.0%) underwent explantation followed by reimplantation. In conclusion, we find that Cochlear implantation is a safe low-morbility technique with a relatively low complication rate in the presented population.

Acta Oto-Laryngologica, 2010

Conclusions: Despite the need for special fitting strategies, improvements in speech discriminati... more Conclusions: Despite the need for special fitting strategies, improvements in speech discrimination tests support the use of cochlear implantation (CI) for patients with Cogan syndrome. Adequate preimplant counselling is mandatory, to prevent high expectations and to stress the necessity for bilateral implantation. Objective: In 60% of patients with Cogan syndrome, CI remains the only treatment option. Literature data agree that once the electrode array is properly inserted, functional outcomes are very good. Nevertheless, results may deteriorate due to progressive cochlear ossification. A few studies have documented the outcomes of CI in these patients, but none have reported the long-term results. Methods: This was a retrospective study describing the outcomes of 3 implanted patients with Cogan syndromeamong 300 adult patients who received a cochlear implant, 3 had become deaf due to Cogan syndrome. Results: In one patient the cochlear ossification advanced and the speech perception abilities worsened from the highest category to identification of words in closed set. The second patient complained of an abrupt reduction of loudness at 18 months post-implant, which required an increased electrical stimulation. The third patient reached the identification category probably due to auditory dyssynchrony, as an atypical consequence of the syndrome.

Aging Clinical and Experimental Research, 2021

The population undergoing dialysis is aging worldwide, particularly in Japan. The clinical condit... more The population undergoing dialysis is aging worldwide, particularly in Japan. The clinical condition of frailty is the most problematic expression in the elderly population. Potential pathophysiological factors of frailty present in patients with CKD and are accentuated in patients with ESRD. The aim of this study was to identify the prevalence and predictors of frailty in Japanese HD patients. This study was a multicenter, cross-sectional and observational investigation conducted at 6 institutions. To evaluate frailty, the modified Fried's frailty phenotype adjusted for Japanese as the self-reported questionnaire was used. Of the 542 patients visiting each institution, 388 were enrolled in this study. In total, 26.0% of participants were categorized as notfrailty, 52.6% as pre-frailty and 21.4% as frailty. The prevalence of frailty increased steadily with age and was more prevalent in females than in males and the subjects with frailty received polypharmacy. A multivariate logistic regression analysis revealed that the factors independently associated with frailty were the following: female gender (odds ratio [OR] = 3.661, 95% confidence interval [CI] 1.398-9.588), age (OR = 1.065, 95% CI 1.014-1.119), age ≥ 75 years old (OR = 4.892, 95% CI 1.715-13.955), body mass index (BMI) < 18.5 (OR = 0.110, 95% CI 0.0293-0.416), number of medications being taken (OR = 1.351, 95% CI 1.163-1.570), diabetes mellitus (DM) (OR = 2.765, 95% CI 1.081-7.071) and MNA-SF ≤ 11 (OR = 7.405, 95% CI 2.732-20.072). Frailty was associated with the accumulation of risk factors. The prevalence of frailty in Japanese patients with HD was relatively lower than that previously reported in Western developed countries; however, it was extremely high compared to the general population regardless of age. Our findings suggest that frailty might be associated with an increase in the prevalence of adverse health outcomes in patients with HD.

Frontiers in Neuroscience, 2019

Epidemiological studies show increasing prevalence rates of cognitive decline and hearing loss wi... more Epidemiological studies show increasing prevalence rates of cognitive decline and hearing loss with age, particularly after the age of 65 years. These conditions are reported to be associated, although conclusive evidence of causality and implications is lacking. Nevertheless, audiological and cognitive assessment among elderly people is a key target for comprehensive and multidisciplinary evaluation of the subject's frailty status. To evaluate the use of tools for identifying older adults at risk of hearing loss and cognitive decline and to compare skills and abilities in terms of hearing and cognitive performances between older adults and young subjects, we performed a prospective cross-sectional study using supraliminal auditory tests. The relationship between cognitive assessment results and audiometric results was investigated, and reference ranges for different ages or stages of disease were determined. Patients older than 65 years with different degrees of hearing function were enrolled. Each subject underwent an extensive audiological assessment, including tonal and speech audiometry, Italian Matrix Sentence Test, and speech audiometry with logatomes in quiet. Cognitive function was screened and then verified by experienced clinicians using the Montreal Cognitive Assessment Score, the Geriatric Depression Scale, and further investigations in some. One hundred twenty-three subjects were finally enrolled during 2016-2019: 103 were >65 years of age and 20 were younger participants (as controls). Cognitive functions showed a correlation with the audiological results in post-lingual hearing-impaired patients, in particular in those affected by slight to moderate hearing loss and aged more than 70 years. Audiological testing can thus be useful in clinical

European Archives of Oto-Rhino-Laryngology, 2019

Purpose To assess preoperative features that could predict the audiological outcome after cochlea... more Purpose To assess preoperative features that could predict the audiological outcome after cochlear implantation in the elderly, in terms of pure tone audiometry, speech audiometry, and speech perception performance. Methods All available records of patients with cochlear implants aged 65 or more at the time of their implantation at our Institution were reviewed (50 patients, mean age 70.76 ± 4.03 years), recording preoperative clinical features. Pure tone audiometry, speech audiometry, and speech perception performance 1 year after cochlear implant activation and fitting were used as outcome measures. Results No statistically significant association emerged between clinical features and pure tone audiometry. On univariate analysis, progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech audiometry and speech perception performance (p = 0.035 and p = 0.033, respectively). On multivariate analysis, progressive sensorineural hearing loss retained its independent prognostic significance in terms of speech perception performance (p = 0.042). The discriminatory power of a two-variable panel (age and etiology of hearing loss) featured an AUC (ROC) of 0.738 (an acceptable discriminatory power according to the Hosmer-Lemeshow scale). Conclusions A progressive sensorineural hearing loss of unknown origin was associated with a better outcome in terms of speech perception in the elderly in our case study. Further features that can predict audiological outcome achievable with cochlear implants in the elderly are desirable to perform adequate counselling and rehabilitation programs.

Clinical oral investigations, Jan 22, 2017

Feeding and swallowing impairment are present in up to 80% of oculo-auriculo-vertebral spectrum (... more Feeding and swallowing impairment are present in up to 80% of oculo-auriculo-vertebral spectrum (OAVS) patients. Salivary gland abnormalities have been reported in OAVS patients but their rate, features, and relationship with phenotype severity have yet to be defined. Parotid and submandibular salivary gland hypo/aplasia was evaluated on head MRI of 25 OAVS patients (16 with severe phenotype, Goldenhar syndrome) and 11 controls. All controls disclosed normal salivary glands. Abnormal parotid glands were found exclusively ipsilateral to facial microsomia in 21/25 OAVS patients (84%, aplasia in six patients) and showed no association with phenotype severity (14/16 patients with Goldenhar phenotype vs 7/9 patients with milder phenotype, p = 0.6). Submandibular salivary gland hypoplasia was detected in six OAVS patients, all with concomitant ipsilateral severe involvement of the parotid gland (p < 0.001). Submandibular salivary gland hypoplasia was associated to Goldenhar phenotype (...

Neuroradiology, Jan 31, 2017

In the article "Oculo-auriculo-vertebral spectrum: going beyond the first and second pharyngeal a... more In the article "Oculo-auriculo-vertebral spectrum: going beyond the first and second pharyngeal arch involvement^, the list of author names was incorrect. Author names were inverted with the surname preceding first name.

Audiology and Neurotology, 2016

A growing interest in cognitive effects associated with speech and hearing processes is spreading... more A growing interest in cognitive effects associated with speech and hearing processes is spreading throughout the scientific community essentially guided by evidence that central and peripheral hearing loss is associated with cognitive decline. For the present research, 125 participants older than 65 years of age (105 with hearing impairment and 20 with normal hearing) were enrolled, divided into 6 groups according to their degree of hearing loss and assessed to determine the effects of the treatment applied. Patients in our research program routinely undergo an extensive audiological and cognitive evaluation protocol providing results from the Digit Span test, Stroop color-word test, Montreal Cognitive Assessment and Geriatric Depression Scale, before and after rehabilitation. Data analysis was performed for a cross-sectional and longitudinal study of the outcomes for the different treatment groups. Each group demonstrated improvement after auditory rehabilitation or training on sho...

BioMed Research International, 2015

Background. Specific clinical conditions could compromise cochlear implantation outcomes and dras... more 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 neuroi...

Hearing, Balance and Communication, 2015

B-ENT, 2010

To describethe occurrence of a cavernous hemangioma of the external auditory meatus extending to ... more To describethe occurrence of a cavernous hemangioma of the external auditory meatus extending to the middle ear and the retroauricolar region in an adult patient, and to review the relevant literature. Case report. We report the clinical presentation, imaging studies and surgical procedures used in the study. The angiographic study showed that the blood supply of the mass originated from the posterior auricular artery. Via a retroauricular approach, this artery was isolated and ligated in order to control the intra-operative bleeding. A canal wall up mastoidectomy with posterior tympanotomy and a tympano-canaloplasty were performed, permitting a complete excision. At 36 months of follow-up, no clinical and radiological signs of recurrence were detected. Surgical removal is the treatment of choice, with rare recurrence of the hemangiomas. Pre-operative evaluation is based on computed tomography (CT scan), but should be followed by magnetic resonance angiography (MR-angio) or intra-ar...