Children with hearing impairment and early cochlear implant: A pragmatic assessment.

Extensive research has demonstrated the benefits of cochlear implants (CI) in contributing to improve the linguistic skills of children with hearing impairment; however, few studies have focused on the development of pragmatic ability and its relationship with age of implantation. Pragmatics is the ability to use language in different contexts and its development has crucial implications, e.g., social inclusion and professional attainments. In this study, we conducted a comprehensive assessment of pragmatic ability using the Language Pragmatic Abilities (APL Medea), a battery composed by five different tasks: Comprehension of Metaphors, Implicit meaning, Comics, Situations and Colors Game (a perspective taking task). Eighteen children with early CI, belonging to 3 different age groups (6; 11-7; 11, 8; 0-8; 11 and 9; 0-9; 11 years-old), and twenty-four children with typical development (Control Group) participated to the study. We also investigated how the precocity of CI, i.e., age of first implantation, may affect the pragmatic development. Globally, children with CI obtained lower scores in the APL Medea battery than typically hearing children. However, focusing on the Medea tasks separately, children with CIs differed from their hearing peers only in Comics and Colors Game tasks. Finally, age of implantation was a moderate but significant predictor of pragmatic performance.

Congenital cytomegalovirus infection and audiological follow-up: electrophysiological auditory threshold before 3 months of age as a predictor of hearing outcome at 3 years of age.

OBJECTIVE: We aimed to evaluate whether electrophysiological auditory thresholds (EATs) before 3 month of age, as assessed by the auditory brainstem responses (ABR) test and the auditory steady state responses (ASSR) test, can predict hearing outcome at 3 years of age among children born with congenital cytomegalovirus (cCMV) infection. STUDY DESIGN: Audiological assessment was performed before 3 months of age, and every 6 months thereafter until 3 years of age, in a population of 63 children (126 ears). EATs before 3 months of age and at 3 years of age were compared. RESULT: No ear with an EAT of ≤30 dBHL (i.e. normal hearing) before 3 months of age showed worsening EATs at 3 years of age. CONCLUSION: An EAT of ≤30 dBHL obtained by ABR and ASSR tests before 3 months of age is predictive of a normal hearing at 3 years of age in children born with cCMV.

Kawasaki disease triggered by parvovirus infection: an atypical case report of two siblings.

BACKGROUND: There are reports of the familial occurrence of Kawasaki disease but only a few reports described Kawasaki disease in siblings. However, the familial cases were not simultaneous. In these patients the idea of infective agents as trigger must be considered. CASE PRESENTATION: We describe two siblings with atypical presentations of Kawasaki disease; the sister was first diagnosed as having parvovirus infection with anemia and the brother was diagnosed as having myocarditis. The first patient was a 9-month-old Caucasian girl with fever, conjunctivitis, rash, and pharyngitis, and later she had cervical adenopathy, diarrhea and vomiting, leukocytosis, and anemia, which were explained by positive immunoglobulin M against parvovirus. However, coronary artery lesions with aneurysms were documented at day 26 after fever onset. An infusion of intravenous immunoglobulin and high doses of steroids were not efficacious to resolve the coronary lesions. She was treated with anakinra, despite a laboratory test not showing inflammation, with prompt and progressive improvement of coronary lesions. Her 7-year-old Caucasian brother presented vomiting and fever at the same time as she was unwell, which spontaneously resolved after 4 days. Four days later, he again presented with fever with abdominal pain, associated with tachypnea, stasis at the pulmonary bases, tachycardia, gallop rhythm, hypotension, secondary anuria, and hepatomegaly. An echocardiogram revealed a severe hypokinesia, with a severe reduction of the ejection fraction (20%). He had an increase of immunoglobulin M anti-parvovirus, tested for the index case of his sister, confirming the suspicion of viral myocarditis. He received dopamine, dobutamine, furosemide plus steroids, with a progressive increase of the ejection fraction to 50%. However, evaluating his sister's history, the brother showed a myocardial dysfunction secondary to Kawasaki shock syndrome. CONCLUSIONS: We report on familial Kawasaki disease in two siblings which had the same infectious trigger (a documented parvovirus infection). The brother was diagnosed as having post-viral myocarditis. However, in view of the two different and simultaneous evolutions, the girl showed Kawasaki disease with late coronary artery lesions and aneurysms, whereas the brother showed Kawasaki shock syndrome with myocardial dysfunction. We stress the effectiveness of anakinra in non-responder Kawasaki disease and the efficacy on coronary aneurysms.

Quantitative criteria for the diagnosis of the congenital absence of pericardium by cardiac magnetic resonance.

BACKGROUND: Congenital absence of the left ventricular pericardium (LCAP) is a rare and poorly known cardiac malformation. Cardiac Magnetic Resonance (CMR) is generally used for the diagnosis of LCAP because of its high soft tissue contrast, multiplanarity and cine capability, but the diagnosis is usually made by only qualitative criteria. The aim of the present study was to establish quantitative criteria for the accurate diagnosis of LCAP on CMR. METHODS: We enrolled nine consecutive patients affected by LCAP (mean age 26±8years, 7 males), 13 healthy controls, 13 patients with dilated cardiomyopathy (DCM), 12 patients with hypertrophic cardiomyopathy (HCM) and 13 patients with right ventricular overload (RVO). All patients underwent CMR. The whole-heart volume was measured in end-systole and end-diastole. Whole-heart volume change (WHVC), was the systo-diastolic change of volume, expressed percentage of the end-diastolic volume. The angle of clockwise-rotation of the heart was also measured in the end-diastolic phase of the axial cine stack. RESULTS: The WHVC was significantly higher in LCAP (21.9±5.4), compared to healthy subjects (8.6±2.4, p<0.001), DCM (7.1±1.8, p<0.001), HCM (9.3±2.4, p<0.001) and RVO (8±2.4, p<0.001). The clockwise-rotation was significantly higher in LCAP (76±14°) than healthy controls (40±11°, p<0.001), DCM (41±5°, p<0.001), HCM (30±6°, p<0.001) and RVO (49±8°, p<0.001). WHVC had the highest sensitivity (100%) and specificity (100%) for diagnosing LCAP, using a threshold of >13%. CONCLUSIONS: In LCAP the systo-diastolic WHVC was significantly higher than controls, DCM, HCM and RVO patients and resulted an optimal quantitative criteria for the diagnosis of LCAP.

Chemotherapy-induced cardiotoxicity: role of the conventional echocardiography and the tissue Doppler.

AIM: The cardiotoxicity of anticancer drugs is an emerging problem and only an identification of the early signs of cardiotoxicity by conventional echocardiography and not (tissue Doppler imaging, TDI), will limit and contain the long-term cardiotoxicity effects. The aim of this study was to identify, through conventional echocardiography and TDI, parameters to use as early "signs" of cardiotoxicity. METHODS: A prospective study was performed using patients with breast cancer (72 women, median age 57 ± 12) treated with anticancer drugs (adjuvant chemotherapy). All patients underwent a careful cardiological evaluation before starting treatment (T0) and during follow-up at 3 months (T1), 6 months (T2) and 1 year (T3). Electrocardiography and echocardiography were performed in all patients in these times. Echocardiography evaluation considered the following parameters: systolic and diastolic diameters and volumes, LVEF, MAPSE, TAPSE, E/A TDI (Em, Am, Sm, IVCT, IVRT, ET, TEI index). On the basis of chemotherapy treatment, patients were divided into 5 groups: A=FEC (fluorouracil, epirubicin, cyclophosphamide), B=FEC+trastuzumab, C=trastuzumab, D=FEC+taxotere, E=FEC+taxolo+trastuzumab. RESULTS: A significant reduction in the echo parameters of TDI was observed. TDI appears to offer important advantages over traditional techniques in revealing the presence of early signs of cardiotoxicity. CONCLUSION: The TDI should be utilized to complement conventional echocardiography in the assessment of cardiotoxicity.

Nasal cheek flap in ethmoidal and skull base tumour surgery: results and complications.

Surgery is the treatment of choice for the management of malignant nasal/ethmoidal tumours, followed, in most cases, by external radiotherapy. Two main procedures are adopted to resect these tumours depending upon stage and extension: ethmoidectomy and medial maxillectomy, via a transfacial approach, or craniofacial resection with a combined transcranial and transfacial approach. The nasal cheek flap technique allows complete nasal swing thus obtaining a wide access to both the nasal fossae and the ethmoidal labyrinth. Furthermore, this approach can also be used in the management of small intracranial tumours extended through the skull base to the nasal cavity, paranasal sinuses, upper and middle clivus. We have used the nasal cheek flap since 1992 with good aesthetic and functional results. Aim of the present study was to analyse personal experience, focusing on complications, aesthetic results and self-evaluation expressed by the patients.

Dysmorphism of the middle ear: case report.

Although there are numerous publications in the literature describing the wide range of diagnosis, classifications and treatment of malformations of the hearing apparatus, even more variations can be found in clinical practice. Indeed, each individual case is unique as far as concerns pathogenesis, clinical course and treatment. The case reported herein describes a 12-year-old boy affected by cranio-facial dysmorphism and monolateral conductive hearing loss in the right ear: followed from radiological diagnosis--carried out to study a malformation of the ear pinna--to surgical treatment.

Oropharyngeal carcinoma during pregnancy: clinical and psycho-oncological aspects.

Cancer is uncommon in pregnancy, occurring in approximately one out of 1000 pregnancies, although it has been noted that one out of 118 women diagnosed with cancer is pregnant at the time of diagnosis. In the last 10 years, two oropharyngeal carcinomas, (1 squamous cell carcinoma and 1 adenoid cystic carcinoma) which developed during pregnancy, have been diagnosed and treated in our Department. No cases of oropharyngeal cancer, during pregnancy, have so far been reported in the literature with the exception of one case of oral cancer. This report focuses not only on the clinical history but also the management of oropharyngeal carcinoma during pregnancy, in terms of choice and timing of treatment. A scrupulous psycho-oncological analysis was also carried out in order to throw further light on psychological repercussions of head and neck cancer in the pregnant woman.

[Endoscopic follow-up of translaryngeal Fantoni tracheostomy]. / Follow-up endoscopico di tracheotomie translaringee sec. Fantoni.

Dilatational tracheotomy techniques are widely used in the long-term management of the respiratory tract in patients in intensive care units (ICU). The translaryngeal tracheotomy technique (TLT) was first described by Fantoni in 1993 and rapidly asserted itself, especially in Europe. This technique basically differs from the other percutaneous techniques in that it involves a progressive, retrograde, dilatation of the trachea in a single session conducted from inside the trachea, working outward, simultaneously exerting a counter-pressure on the pre-tracheal soft tissues with the fingers. The present study involves an endoscopy follow-up of 130 patients who had undergone TLT at the Intensive Care Unit of our Hospital between November 2000 and May 2001. The pre-operative oro-tracheal intubation time varied from 1 to 42 days. All patients filled out a brief questionnaire containing validated questions on their general health and quality of life with particular attention focused on respiratory conditions. Then, after receiving informed consent, the patients underwent laryngo-tracheoscopy with local anesthetic using a flexible tracheobronchoscope. All tests were recorded and viewed later by two operators in order to identify and divide the patients according to the level of execution of the tracheotomy and the presence of sequelae. The results obtained have shown that, like other percutaneous tracheotomy techniques, TLT provides some benefits including the fact that procedure can be performed at the bedside in a short time, with few post-operative complications, simpler nursing and fewer sequelae in time. Analysis of data concerning time of tracheostomy execution, tracheal level of stomia and nursing times has revealed three factors that determine severe sequelae: delay in tracheostomy execution, high level of execution with cricoid involvement and onset of problems during first tracheal cannula change.

Is pressure dressing necessary after ear surgery?

Compressive bandages carry intrinsic risks and complications. The purpose of this study was to assess whether compressive head bandages are necessary after ear surgery to prevent complications such as hematoma or wound infection. A prospective, randomized, controlled study was conducted from August 1, 1993, to June 1, 1999. We randomly assigned 420 consecutive middle ear or mastoid operations to either a pressure bandage group or to a no-pressure bandage group. A careful follow-up was planned, and complications were recorded. In the pressure bandage group, 3 patients had bruising over the pinna and 70 patients had minor skin erythema when the dressing was removed. No patient had bruising or erythema in the no-pressure bandage group. No patient had hematoma, wound infection, or any other wound complication in either group. As a result of this study, we have decided to abandon the use of compression bandages after uncomplicated ear surgery.

[Reconstructive strategies in head and neck cancer: case history review from 1992 to 1997 (154 cases)]. / Strategie ricostruttive in oncologia cervico-cefalica: revisione della casistica (154 casi) degli anni 1992-97.

Technological progress in reconstructive surgery, in particular the use of pedunculated or free flaps, has given new impetus to head and neck dissection. This stems from the fact that such techniques provide greater oncological radicality, reduce the number of severe post-operative complications and give better quality of life. The present study examines 154 patients suffering from upper aero-digestive tract neoplasms (131 males and 23 females; age range 23-82 years) who had undergone radical surgery. Reconstruction was performed with flaps in 154 cases: 119 pedunculated flaps (102 large myocutaneous pectoral and 17 temporal muscle flaps) and 35 free flaps (18 radial osteofasciocutaneous, 13 radial fasciocutaneous and 4 omentum flaps). Analysis of the individual districts showed that the flap of choice was the temporal muscle flap when surgery involved the soft parts of the orbital-maxilly-zigomatic area and the rhinopharynx. This is because it is highly moldable and reliable. In surgery of the oral cavity and oropharynx the grand pectoral flap is most frequently used as it provides enough tissue for the reconstruction, adequately protects the vascular-nerve axis in the neck and it is quick and easy. However, the functional results are not the best and there is some alteration in the initial phases of deglutition. To reduce these problems, the authors encourage the use of free flaps which provide good results from both the functional and esthetic points of view. They are, however, more difficult to perform and this leads the authors to conclude that they should only be selected for certain patients (long life expectancy, female, young, etc.). In the center where the authors work the flap of choice is the radial fasciocutaneous or osteofasciocutaneous flap. In surgery of the hypopharynx and larynx reconstruction is normally performed with a grand pectoral myocutaneous flap, sculpted as needed for the individual case. In this region, reconstruction proves functionally satisfactory even when there is a minimum of residual mucosa. Finally, for reconstructive surgery of the apex, the omentum free flap was used as it is malleable and can be used to reconstruct broad areas of dissection. The esthetic and functional results, the low incidence of complications and the greater quality of life suggest that this type of flap be extended to the surgery of locally advanced tumors in combination with an accurate, valid reconstructive solution.

[Nasal hyperreactivity and neuropeptides]. / Iperreattività nasosinusale e neuropeptidi.

The Authors define nasal-sinusal hyperreactivity and closely investigate the influence it has on the relationship existing among the Central Nervous System, Peripheric Nervous System, Immunitary System and Nasal Mucosa. Particular reference is made to neuropeptides that seem to be true "bridge" substances between the Nervous System and some components of the Immunitary System. Importance of the P-Substance is emphasized.