Sara Ghiselli

Correlation of adverse effects of cisplatin administration in patients affected by solid tumours: A retrospective evaluation

Cisplatin is the most common antineoplastic drug used for the therapy of solid tumours. To date, researchers have focused on the dosage to be administered for each specific tumour, mainly considering the local adverse effects. The aim of this study was to correlate the severity of the adverse effects with: i) the dosage of cisplatin; ii) the specific site of the tumour; iii) the association with other drugs; and iv) the symptoms. We analysed data from 123 patients with 11 different tumour classes undergoing therapy from 2007 to 2008 at St. Anna Hospital (Ferrara, Italy), using the Spearman non-parametric correlation index. Even though significant correlations were found among the variables, the overall results showed that the main factor influencing the severity of the adverse effects was the dosage of cisplatin administered.

Coenzyme Q10 plus Multivitamin Treatment Prevents Cisplatin Ototoxicity in Rats

Cisplatin (Cpt) is known to induce a high level of oxidative stress, resulting in an increase of reactive oxygen species damaging the inner ear and causing hearing loss at high frequencies. Studies on animal models show that antioxidants may lower Cpt-induced ototoxicity. The aim of this study is to evaluate the ototoxic effects of two different protocols of Cpt administration in a Sprague-Dawley rat model, and to test in the same model the synergic protective effects of a solution of coenzyme Q10 terclatrate and Acuval 400®, a multivitamin supplement containing antioxidant agents and minerals (Acu-Qter). The Cpt was administered intraperitoneally in a single dose (14 mg/kg) or in three daily doses (4.6 mg/kg/day) to rats orally treated or untreated with Acu-Qter for 5 days. The auditory function was assessed by measuring auditory brainstem responses from 2 to 32 kHz at day 0 and 5 days after treatment. Similar hearing threshold and body weight alterations were observed in both Cpt administration protocols, but mortality reduced to zero when Cpt was administered in three daily doses. The Acu-Qter treatment was able to prevent and completely neutralize ototoxicity in rats treated with three daily Cpt doses, supporting the synergic protective effects of coenzyme Q terclatrate and Acuval 400® against Cpt-induced oxidative stress. The administration protocol involving three Cpt doses is more similar to common human chemotherapy protocols, therefore it appears more useful for long-term preclinical studies on ototoxicity prevention.

The written language of children with cochlear implant

Abstract In the era of cochlear implants (CIs) and newborn hearing screening programmes, the oral language skills of deaf children have improved significantly. The literature documents sizable gains in their speech perception and receptive and expressive spoken language. However, the improvements in the area of reading and writing are less remarkable, and reading and writing continue today to be a major challenge for children with CIs. The article discusses the role of two cognitive factors that may contribute to make writing difficult to achieve for children with CIs: poor executive functions and verbal working memory. Recommendations for assessment and intervention are derived from this analysis.

Is very early hearing assessment always reliable in selecting patients for cochlear implants? A case series study

INTRODUCTION This study concerns a case series of 23 infants with a diagnosis of severe-to-profound hearing loss at 3 months old, who significantly improved (even reaching a normal auditory threshold) within their first year of life. METHODS All infants were routinely followed up with audiological tests every 2 months after being fitted with hearing aids as necessary. A reliable consistency between the various test findings (DPOAE, ABR, behavioral responses, CAEP and ECoG) clearly emerged in most cases during the follow-up, albeit at different times after birth. RESULTS The series of infants included 7 cases of severe prematurity, 6 of cerebral or complex syndromic malformations, 5 healthy infants, 2 with asymptomatic congenital CMV infection, and 1 case each of hyperbilirubinemia, hypoxia, and sepsis. All term-born infants showed a significant improvement over their initial hearing threshold by 6 months of age, while in most of those born prematurely the first signs of threshold amelioration occurred beyond 70 weeks of gestational age, and even beyond 85 weeks in one case. CONCLUSIONS Cochlear implantation (CI) should only be considered after a period of auditory stimulation and follow-up with electrophysiological and behavioral tests, and an accurate analysis of their correlation. In our opinion, CI can be performed after a period of 8 months in all term-born infants with persistent severe-to-profound hearing loss without risk of diagnostic error, whereas the follow-up for severely preterm infants should extend to at least 80 weeks of gestational age.

Improvement of the video analysis method for the evaluation of communication skills in deaf children with complex needs

Abstract Several papers report important benefits from cochlear implants for deaf children with complex needs but these improvements are difficult to assess with standard tests. The communication strategies of deaf children with complex needs are comparable to those of very young children without comorbidities because both are in a preverbal stage of communication. For this reason we propose a new use of video analysis in deaf children with associated disabilities. The set-up of the recording is the same as the standard method and 20 communication turns are evaluated. The adult stays in front of the child at eye level (especially if a child has a visual impairment). The abilities and progress of the children are evaluated in the standard four areas: turn-taking, autonomy, awareness and eye contact. Non-verbal behaviours are examined. When it is possible to evaluate the category of the eye contact, the most important parameter to take is the adequacy of the use of this ability. For deaf children with complex needs, it is very important to add to the standard parameters the analysis of the maternal communicative style. In conclusion, video analysis can be a useful tool of evaluation when dealing with deaf children with associated disabilities even if not applied in its standard form.

Cochlear implantation in post-lingually deafened adults and elderly patients: analysis of audiometric and speech perception outcomes during the first year of use

SUMMARY The aim of this study was to analyse audiometric and speech perception outcomes after cochlear implantation (CI) in adult and elderly patients in the first year post-CI activation. We evaluated 42 subjects who underwent CI at the Otorhinolaryngological Clinic of Padua Hospital. The subjects enrolled were post-lingually deafened patients who were unilaterally implanted for bilateral, severe-to-profound hearing loss. The overall sample was divided into three groups according to the age at the time of implantation: group A (35-49 years), group B (50-64 years) and group C (≥ 65 years). The subjects were assessed, both before and after surgery (at months 1, 3, 6 and 12), using pure tone audiometry, speech audiometry and speech perception tests and the CAP questionnaire. Statistical analysis of outcomes was using a Students t-test for paired data. In all study groups a significant improvement was demonstrated in auditory performance examinations post-CI compared to the pre-operative scores. All subjects in all age groups obtained significant improvements in PTA scores before surgery and post-CI activation. Comparison of PTA values among the three age groups did not reveal any significant difference. Considerable improvement was obtained even in the speech audiometry thresholds in all groups at follow-up, with no significant differences between groups. The speech perception examination and CAP questionnaire showed good progress in all study groups, although younger patients tended to achieve more complex categories than older ones. In conclusion, CI is an effective treatment for severe-to-profound hearing loss with no significant differences in auditory performances between older and younger CI recipients. Even if somewhat slower, subjects older than 65 reached good performance and therefore are good candidates for a cochlear implant.

Audiological and clinical management of children with oculo-auriculo-vertebral spectrum

Abstract 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 first 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 specific 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.

Heart problems and deafness: Are they more common than supposed?

In 2005, the World Health Organization (WHO) estimated that 12.5 million people worldwide are affected by bilateral profound hearing loss. Congenital hearing loss is the hereditary sensorial defect most commonly observed in newborns; it affects 1.12 per 1000 children. If we also consider acquired forms and late-onset forms of hearing loss, prevalence grows to 1.33 per 1000 children. In Italy the overall prevalence is 0.78 per 1000 for males and 0.69 per 1000 for females (1). Fifty percent of sensorineural deafness is due to genetic causes, most of which are inherited in an autosomal recessive manner. Genetic deafness is very heterogeneous and can occur in non-syndromic form (70% of cases) or in syndromic form (30% of cases). Several syndromes are associated with deafness, heart anomalies and other diseases: Charge syndrome, Leopard syndrome, Fabry syndrome, Refsum syndrome, SALL4 syndrome and Mobius syndrome. Until recently only one syndrome in which deafness and heart problems are the sole features has been described – Jervell and Lange-Nielsen syndrome (J-LNS). This condition, if not timely discovered and treated, may culminate in sudden death. However, in December 2010, Nature Neuroscience published an article about a new channelopathy termed SANDD syndrome, characterized by sinoatrial node dysfunction and deafness (2). These recent data underline the importance of a cardiac investigation in cases of suspected genetic deafness of unknown cause. It is probable that in the future other syndromes involving both the heart and the hearing system will be found. We have to consider that many of the causative genes of deafness have not yet been identifi ed. Furthermore, genes involved in hearing impairment encode proteins of different classes, such as cell adhesion and cytoskeleton proteins, transcription factors, extracellular matrix components, membrane

Correction: Coenzyme Q10 plus Multivitamin Treatment Prevents Cisplatin Ototoxicity in Rats

[This corrects the article DOI: 10.1371/journal.pone.0162106.].

Check-list for the assessment of functional impairment in children with congenital aural atresia

OBJECTIVES Congenital Aural Atresia (CAA) is a deformity of the external ear and it is commonly associated with malformations of middle and inner ear and, in some cases, with other facial deformities. Very few assessment measures exist for evaluating the functional impairment in children with CAA. Purpose of this study is to introduce and describe an assessment Checklist, (nominated FOS Checklist) that covers feeding abilities (F), oralmotor skills (O), communication/language development (S) in children with CAA. FOS wants to offer a range of assessment providing a profile of the child in comparison to hearing peers and it aims to make clinicians able to identify additional problems and areas of difficulties as well as specific abilities and skills. Secondary, we want to investigate the presence of correlations between disorders and side of CAA. METHODS a new Checklist (FOS Checklist) was administered to 68 children with CAA. RESULTS Feeding abilities are age-adequate in 94,3% of all patients. 54,4% of all patients are in need for further assessment of their oral-motor skills; delays in language development were found in 44,1% of cases. Orofacial development delays have been observed in 57.2% of subjects among the bilateral CAA group, in 53.9% among the right CAA group and in 53.4% among the left CAA group. Patients referred for further language evaluation were 42,9% in the bilateral CAA group, 33.3% in the right CAA group and 33.3% in the left CAA group. According to the χ2 analysis, referral for further assessment is independent from side of aural atresia. CONCLUSIONS Subjects with bilateral CAA are more likely to be referred for further assessment, both for oral motor aspects and for speech perception and language development. However, there is not a significant statistical difference between the performances of children with bilateral or unilateral CAA. FOS Checklist is simple, reliable and time effective and can be used in everyday clinical practice. FOS enable clinicians to identify additional problems and areas of difficulties as well as specific abilities and skills; moreover, FOS allows to determine appropriate referrals and intervention strategies.