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.

Mastoiditis in adults: a 19-year retrospective study.

The objective of our study was to review retrospectively the clinical, radiological and therapeutic findings in 62 adults with acute mastoiditis treated at the ENT Department of Ferrara from 1992 to 2010. 62 adult cases fulfilled the following inclusion criteria: otoscopical evidence of co-existent or recent otitis media; postauricular swelling, erythema or tenderness; protrusion of the auricle; fever and/or significant radiological findings of mastoiditis. Conservative treatment comprehended antibiotic ± ventilation tube. Surgical procedures comprehended mastoidectomy or mastoido-tympanoplasty. The incidence of adult's mastoiditis in our district (0.99 cases/year/100.000 inhabitants) has maintained quite stable during the considered 19-year period. The typical clinical presentation was observed in 48% of cases. Complications were meningitis (15 cases), meningo-encephalitis (1), meningitis associated with lateral sinus thrombosis (1), facial nerve paralysis (11), and labyrinthitis (8). In all cases except one, the facial palsy recovered completely and no mortality was observed due to these complications. Complete cure was obtained with conservative treatment in 69% of uncomplicated cases and in 24% of patients with intracranial complications. Mastoiditis in adults may present as the acute classical form, as well as latent forms which often have prolonged and insidious development followed by a rapid clinical deterioration. Clinical features are frequently atypical, while incidence of meningitis and other complications is still high particularly in the most elderly. Thus, great care is required from clinicians to make an early diagnosis in order to promote adequate treatment.

Pseudomonas and Aspergillus interaction in malignant external otitis: risk of treatment failure.

Malignant external otitis (MEO) is a rare infection of the temporal bone primarily affecting elderly patients and diabetics or immunocompromised individuals, which may have dismal prognosis if treatment is not prompt and adequate. Almost 95% of MEO cases reported in the literature are attributed to Pseudomonas aeruginosa, and this pathogen is isolated from aural drainage in > 90% of cases. However, in recent years fungal and polymicrobial temporal bone infections have been reported with increasing frequency. The aim of this paper is to discuss a possible pitfall in MEO treatment using an unusual clinical case. In this patient, bacteriological data positive for Pseudomonas delayed correct diagnosis of Aspergillus infection, which was obtained after surgical debridement and biopsy of the infra-temporal space.

High frequency hearing loss in the elderly: effect of age and noise exposure in an Italian group.

OBJECTIVE: To describe the effect of age and noise on high frequency hearing thresholds in an Italian population aged 70 years and older, in order to investigate the interaction between presbycusis and noise exposure. METHODS: We compared 460 subjects: 367 affected by presbycusis alone (204 women and 163 men) and 93 affected by presbycusis and noise exposure (eight women and 85 men). Pure tone average hearing thresholds, for each ear, were compared between groups, and between sexes and ages within groups. RESULTS: A slight threshold difference was found between the two groups at 4 kHz. After adjusting for age and gender, this difference was found to be related only to differing patient age. Men's and women's thresholds differed significantly in both groups, especially at high frequencies, at which threshold deterioration was worse in men than women. CONCLUSION: The threshold differences between patients with presbycusis with and without noise exposure were limited. Larger studies are needed to assess the relative effects of ageing and noise exposure on hearing thresholds.

Bioelectric brain maturation in fullterm infants and in healthy and pathological preterm infants at term post-menstrual age.

At the same post-menstrual age (39-41 weeks), EEG maturation assessed according to the Nolte and Haas method (Nolte, R. and Haas, H.G. (1978) Dev. Med. Child Neurol., 20, 167-182) was studied in 16 fullterm infants (FT), 17 healthy preterm infants (HP) and 18 pathological preterm infants (PP) affected by brain lesions (haemorrhage and/or leucomalacia). There were no significant differences in respect to EEG maturational codes, EEG types or bioelectrical age between the FT and HP groups. The preterm infants affected by brain lesions presented higher percentages of younger EEG codes (i.e. 36 weeks) in State 1 and a higher number of fluctuations between one maturation code and another in both State 1 and State 2, with respect to the HP group. Also, although the PP infants with young EEG codes did not present serious background EEG abnormalities, they did reveal minor EEG abnormalities, such as excessive asynchrony of the 'tracé alternant', lack of frontal sharp transients and monomorphic TA 'bouffees' with little activity at 2-6 c/s. However, no relationship between young EEG codes and onset-offset or duration of the states was found: young codes were often randomly distributed in successive State 1-State 2 epochs, regardless of groupings. Bioelectric age appropriate to the post-menstrual age precedes a normal development or only minor handicap at 24 months, while EEG immaturity of more than 2 weeks corresponds to later major handicaps. The prognostic value of EEG immaturity of between 1.1 and 2 weeks is uncertain.

Ultrastructural observations on autolytic changes of atrial specific granules in pig heart.

The progressive autolytic changes of atrial specific granules (ASGS) up to 24 hours after death were studied electron microscopically. Tissue samples taken from the left auricular myocardium of four pig hearts at 0, 0.5, 1.5, 3.5, 6, 12 and 24 hours post mortem were fixed for 24 hours by immersion in 2.5 per cent cacodylate buffered glutaraldehyde (pH 7.4). Two pig hearts were kept at 20 degrees C and two at 4 degrees C. Although typical autolytic changes were seen in many organelles, no remarkable modifications in aspect, distribution, content and diameter of ASGS were observed in all samples. The results of this study indicate that ASGS are not morphologically affected by the autolytic processes, at least during the first 24 hours. Hence, the electron microscopic evaluation of ASGS does not need a particular fixation method such as perfusion. This may eliminate technical problems when slaughterhouse material is used.

Epilepsy with typical absence seizures with onset during the first year of life.

Absence epilepsy with multiple daily seizures and onset at the age of 6 and 1/2 months in a healthy female child with normal development is described. EEG-video recording revealed typical absence seizures (vacant staring and interruption of motor activity) and complex absences (as above, plus raising of the eyeballs, slight beatings of the eyebrows, and forward propulsion of head and shoulders). The absences were accompanied by bilateral symmetrical 3-Hz spike-wave discharges preceded, and at times followed, by bilateral frontoparietal theta activity coinciding with onset and termination of the absence seizures. The seizures regressed with nitrazepam therapy. At age 3-years, the child is seizure-free and shows normal neurologic development.

Late febrile convulsions: a clinical follow-up.

A joint study was performed on patients from the Pediatric Clinics of Catania and Modena. Two hundred and twenty-two children who presented with febrile convulsions (FC) after the age of six years (LFC) were followed up in order to evaluate the risk of recurrence and type of convulsions. The overall results showed that 94 patients out of 222 (42.3%) had subsequent fits, both febrile and afebrile. The present study suggests that the risk of subsequent afebrile crises in LFC is higher (15.8%) than observed after "simple" FC (2-3%) and similar to that reported after "complex" FC (13-17%).

[Griffith test in the evaluation of psychomotor development in the 1st year of life. A longitudinal study]. / Il test di Griffith per la valutazione dello sviluppo psicomotorio nel primo anno di vita. Studio longitudinal.

Griffith psychometric test has been experimented on a group of 20 healthy newborn infants, monitored during their 1st year of life with quarterly check-ups. It was clear that the development general rates were constantly higher than the original values reported by the English author. In particular, in 12 months old infants, the rates appeared significantly higher than the rates observed on the same Italian specimen in past periods. A transverse and longitudinal study of the psychomotor development of subjects included in the specimen has given the opportunity to verify some of his features. The development outlines, traced by employing the average rates relevant to the 5 different aspects monitored (motor, personal-social, language, eye-hand co-ordination, practical reasoning), appeared to be well-balanced, that is without dissynchronisms between the different scales. The only exception being the scale of the social behaviour, were the average rates appear to be sensibly higher at every age. Individual development outlines, on the contrary, appeared to be disjointed in all aspects (except for 4 cases). In the longitudinal analysis then, some noteworthy oscillations in both general and partial rates, were observed among the controls carried out in subsequent periods in all the subjects.

[Apnea during sleep and wakefulness in term newborns]. / Le apnee nel sonno e nella veglia del neonato a termine.

Apneas in the different sleep states are commonly observed in the full-term healthy newborn infant. Central and short apneas prevail whilst apneas greater than 15 sec. are rare; a marked incidence of short apneas (less than 10 sec.) was observed in active sleep, even though apneas are not exclusive of active sleep. There is a strong inter-individual variability of apnea incidence. Apnea incidence in a state is not positively correlated to apnea incidence in different behavioural states: on the contrary there seems to be an opposite correlation between incidence in AS and QS. Apnea occurrence is positively correlated, in individuals, to the periodic breathing percentage. Apneas number and their duration is markedly lowered already in the second month of life. Periodic breathing must be considered a feature of immaturity. Obstructive apneas are less frequent than central apneas: their survey requires sophisticated technics with the aid of simultaneous recording of several breathing parameters. Relationship between central apneas/ obstructive apneas and mixed apneas is not known. Certainly obstructive and mixed apneas occurrence has been underestimated because of technical difficulties deriving from their survey. The meaning of an incidence of short apneas markedly higher than normal in full-term newborn infants is controversial and not clear, individuals with long apneas and subjects with short apneas in excess have been considered infants at SIDS risk. It is not clear whether periodic breathing and apneas depend on a common pathogenesis; the correlation between high incidence of periodic breathing in postnatal period and SIDS risk is still controversial. Few Authors suggest to treat newborn infants with extended apneas in sleep and considerable percentage of periodic breathing with aminophylline. The relation between gastro-oesophageal reflux and apnea has been recently evidenced. Central apneas and obstructive apneas during breast and bottle feeding have also been documented. Differently from pre-term infant apneas, bradycardia, although not exceptional, is not frequent during apneas in full-term newborn infants.

The use of flunarizine in pediatric epilepsy.

Using the same technique as Declerk's and Wauquier's, we added flunarizine to standard therapy in 14 patients, 7 of whom were treated with the sequence flunarizine-placebo and 7 with the sequence placebo-flunarizine. Treatment averaged between 75 and 85 days, with medication administered in a single dose (orally, in drops), 5-10 mg/day. The patients comprised 7 males and 7 females, from 13 to 17 years with the following diagnoses: epileptic encephalopathy 6 cases, grand mal, myoclonic epilepsy, multifocal epilepsy, epilepsy with polymorphic seizures, 2 cases each. Age, sex and disease were distributed equally in both sequences. During treatment with flunarizine we observed the following: 7 cases--no effect on number or severity of seizures; 2 cases--moderate results; 5 cases--good results.

[Up-date on the subject of neonatal convulsions]. / Attualità in tema di convulsioni neonatali.

New experimental, clinical and therapeutic results in the field of convulsive disorders of the newborn have recently come to light. Experimental studies on animals have shown that, contrary to what was assumed in the past, the immature brain is highly excitable, and during the first weeks of life excitation processes predominate over inhibitory processes. Over the last ten years, benign idiopathic convulsions in newborns, familial convulsions and benign convulsions on the fifth day of life, have been defined. Both types develop and disappear spontaneously during the first week of life and have a favourable prognosis, although they may appear at the start (especially the benign fifth-day convulsions) as status epilepticus. Recently, two types of convulsive status epilepticus in newborns have been identified: a severe idiopathic status epilepticus and focal status epilepticus. The myoclonic syndromes which occur during quiet sleep and which are not accompanied by EEG discharges should be distinguished from convulsions and do not require anti-convulsive therapy. The various EEG monitoring techniques have shown an unexpectedly high number of convulsions, especially in the form of sub-clinical convulsions and/or atypical convulsive seizures. The atypical seizures distinctly predominate in status epilepticus. The clinical evidence of the seizures in reduced by administration of anti-convulsants, which seem to block typical seizures and, viceversa, to be less active for atypical seizures and EEG seizures. In the therapeutic field, the use of phenobarbital and/or phenytoin at a high initial dosage (20 mg/kg) has been well affirmed. Drugs such as lidocaine and thiopental are currently being experimented and have given encouraging results in severe status epilepticus.

Visual orientation to the human face in the premature and fullterm newborn.

Visual orientation to the human face was studied by the method of Brazelton in 15 fullterm newborns on the 4th-5th day of life and in 21 premature newborns (gestational age 27-37 weeks) tested weekly up to 40 weeks conceptional age. No evidence of visual orientation was found before 33 weeks. Performances on a par with those of fullterm newborns were not attained until 37-38 weeks on average, though in a few cases this was achieved at 35 weeks. The quality of orientation improved gradually from 32-33 weeks to 38 weeks, but with wide inter- and intra-individual variations. Noteworthy were the discontinuity and transient worsening of visual orientation of the extremely premature subjects (gestational age less than or equal to 31 weeks) in the weeks following birth. Neurological status at the time of the test, rather than pre-and perinatal risk factors, correlated positively with the quality of the visual responses. No statistically significant differences in orientation were found between premature newborns at term age and fullterm newborns.

[Value of computerized tomography in childhood epilepsy]. / Importanza della tomografia computerizzata nelle epilessie infantili.

272 cases of childhood epilepsy were studied with brain TC scan to correlate the type of seizure to the possible neurological damage. Children with generalized convulsions had a low incidence of abnormal TC findings if in absence of neurological signs (3/80) (Group A), but a more relevant incidence (13/34) in presence of neurological signs (Group B). 65 children had epileptic encephalopathy (Group C): 15/17 West syndromes and 33/48 Lennox syndromes presented TC abnormalities. The remnant 93 children had partial seizures. In Group D partial epilepsy was free of neurological signs; the incidence of abnormal TC findings was 17/76. An higher incidence (9/17) was found in Group E, where partial epilepsy was accompanied by neurological disturbances.

[Febrile convulsions and epilepsy]. / Convulsioni febbrili ed epilessia.

We can divide the epilepsies connected to febrile convulsions (F.C.) in five groups: Early epileptic syndromes with generalized febrile and non febrile seizures. Early epileptic syndromes with polymorphic febrile and non febrile seizures. Generalized epilepsies occurring after F.C. Partial epilepsies (whether with elementary or complex fits) occurring after F.C. H.H.E. syndromes. The first two types of epilepsies are rather uncommon and mostly with poor prognosis (especially type 2). The epilepsies occurring after F.C. start at different ages and are characterized from various clinical and prognostical features. The most frequent type are the generalized forms, which present mostly a good evolution, as well as the partial forms with elementary fits. The partial ones with complex fits occur rather frequently and often evolue badly: in some of these cases we can have cerebral damage, as occurs in H.H.E. syndromes.

Clinical course of ulcerative colitis in Italy.

The prognosis of ulcerative colitis has been studied in 122 patients with this disease who had been referred to Bolgna from various parts of Italy and were then followed up. The overall mortality rate was 4.9% and the percentage of patients who were treated by total colectomy was 15.5%. Postoperative mortality was 10.5%, and the percentage of patients who developed carcinoma of the colon was 0.8%. All these figures fall within the range of values for the relevant variables quoted by previous authors from different countries. It is concluded that the prognosis of ulcerative colitis in the 122 Italian patients in the present study was similar to that reported previously in patients of other nationalities.