Cochlea implantation in patients with superficial hemosiderosis.

INTRODUCTION: Superficial hemosiderosis is a sub-form of hemosiderosis in which the deposits of hemosiderin in the central nervous system damage the nerve cells. This form of siderosis is caused by chronic cerebral hemorrhages, especially subarachnoid hemorrhages. The diversity of symptoms depends on the respective damage to the brain, but in most of the cases it shows up as incipient unilateral or bilateral hearing loss, ataxia and signs of pyramidal tracts. We are investigating the question of whether cochlear implantation is a treatment option for patients with superficial hemosiderosis and which strategy of diagnostic procedure has to be ruled out preoperatively. MATERIALS AND METHODS: In a tertiary hospital between 2009 and 2018, we examined (N = 5) patients with radiologically confirmed central hemosiderosis who suffered from profound hearing loss to deafness were treated with a cochlear implant (CI). We compared pre- and postoperative speech comprehension (Freiburg speech intelligibility test for monosyllables and HSM sentence test). RESULTS: Speech understanding improved on average by 20% (monosyllabic test in the Freiburg speech intelligibility test) and by 40% in noise (HSM sentence test) compared to preoperative speech understanding with optimized hearing aids. DISCUSSION: The results show that patients with superficial siderosis benefit from CI with better speech understanding. The results are below the average for all postlingual deaf CI patients. Superficial siderosis causes neural damages, which explains the reduced speech understanding based on central hearing loss. It is important to correctly weigh the patient's expectations preoperatively and to include neurologists within the therapy procedure.

[Technical advancements in cochlear implants : State of the art]. / Technische Entwicklungen bei Cochleaimplantaten : Stand der Technik.

Twenty years ago, cochlear implants (CI) were indicated only in cases of profound hearing loss or complete deafness. While from today's perspective the technology was clumsy and provided patients with only limited speech comprehension in quiet scenarios, successive advances in CI technology and the consequent substantial hearing improvements over time have since then resulted in continuous relaxation of indication criteria toward residual hearing. While achievements in implant and processor electronics have been one key factor for the ever-improving hearing performance, development of electro-acoustic CI systems-together with atraumatic implantation concepts-has led to enormous improvements in patients with low-frequency residual hearing. Manufactures have designed special processors with integrated hearing aid components for this patient group, which are capable of conveying acoustic and electric stimulation. A further milestone in improvement of hearing in challenging listening environments was the adoption of signal enhancement algorithms and assistive listening devices from the hearing aid industry. This article gives an overview of the current state of the art in the abovementioned areas of CI technology.

Cochlear implant programming: a global survey on the state of the art.

The programming of CIs is essential for good performance. However, no Good Clinical Practice guidelines exist. This paper reports on the results of an inventory of the current practice worldwide. A questionnaire was distributed to 47 CI centers. They follow 47600 recipients in 17 countries and 5 continents. The results were discussed during a debate. Sixty-two percent of the results were verified through individual interviews during the following months. Most centers (72%) participated in a cross-sectional study logging 5 consecutive fitting sessions in 5 different recipients. Data indicate that general practice starts with a single switch-on session, followed by three monthly sessions, three quarterly sessions, and then annual sessions, all containing one hour of programming and testing. The main focus lies on setting maximum and, to a lesser extent, minimum current levels per electrode. These levels are often determined on a few electrodes and then extrapolated. They are mainly based on subjective loudness perception by the CI user and, to a lesser extent, on pure tone and speech audiometry. Objective measures play a small role as indication of the global MAP profile. Other MAP parameters are rarely modified. Measurable targets are only defined for pure tone audiometry. Huge variation exists between centers on all aspects of the fitting practice.

Intrauterine growth retardation associated with precocious puberty and sertoli cell hyperplasia.

The original description of patients with Russell-Silver syndrome included precocious puberty, the mechanism of which was unclear. We describe a child with a Russell-Silver syndrome-like phenotype who presented with precocious puberty that was associated with hyperplasia of the Sertoli cells. The patient was found to have an immature cryptorchid testicle; hyperplastic Sertoli cells were also aneuploid carrying trisomy 8. This chromosomal abnormality was present in Sertoli cells only and could not be detected in peripheral lymphocytes, tunica vaginalis, or other, normal, testicular tissue. Sertoli cells in culture showed excess aromatization providing an explanation for the rapid advancement of the patient's bone age. We conclude that in a patient with a Russell-Silver syndrome-like phenotype, Sertoli cell hyperplasia was associated with somatic trisomy 8, increased aromatization, and gonadotropin-independent precocious puberty.

Peroxisomal and mitochondrial defects in the cerebro-hepato-renal syndrome.

The cerebro-hepato-renal syndrome is a rare familial malady with cerebral, renal, and skeletal abnormalities, severe hypotonia, cirrhosis, iron and lipid storage, and death within 6 months. Correlated electron microscopic, histochemical, and biochemical studies demonstrate defects in two oxidative organelles. Peroxisomes cannot be found in hepatocytes and renal proximal tubules. In hepatocytes and cortical astrocytes, mitochondria are distorted in their appearance and glycogen stores are increased. Oxygen consumnption of brain and liver mitochondrial preparations with succinate and with substrates reducing nicotinamide adenine dinucleotide is markedly diminished, but the consumption is normal with ascorbate and tetramethylphenylenediamine, which suggests a defect in electron transport prior to the cytochromes. Histochemical studies of mitochondrial oxidation point to a defect between the succinate dehydrogenase flavoprotein and coenzyme Q, possibly in the region of nonheme iron protein.

Practice patterns in neonatal hyperbilirubinemia.

OBJECTIVE: To determine practice patterns of office-based pediatricians and neonatologists in the treatment of neonatal hyperbilirubinemia in healthy, term newborns during 1992, before the publication of the practice guideline for treatment of neonatal jaundice by the American Academy of Pediatrics (AAP). The survey was undertaken to inform the AAP's Subcommittee on Hyperbilirubinemia on current practices and to aid it in its preparation of the guidelines. It was also anticipated that this survey would serve as a basis for comparison for a second survey to be performed several years after the publication of the practice guidelines. METHODS: A self-administered questionnaire describing a single case of a jaundiced, breastfed 36-hour-old healthy, full-term infant with a total serum bilirubin concentration of 11.0 mg/dL (188 microM/L) was sent to a random sample of 600 office-based pediatricians and 606 neonatologists who were members of the AAP. The final response rate was 74%. Respondents were asked to answer questions regarding treatment of the case based on their actual practices. Ranges of total serum bilirubin concentration were provided as possible answers to questions on initiation of phototherapy and exchange transfusion, and interruption of breastfeeding. Respondents were also queried about frequency of serum bilirubin testing, locations of phototherapy administration, and factors influencing their therapeutic decisions. RESULTS: Four hundred forty-two office-based pediatricians and 444 neonatologists completed the survey. There was a tendency for neonatologists to initiate both phototherapy and exchange transfusions at lower serum bilirubin concentrations than office-based general pediatricians. At a serum bilirubin of 13 to 19 mg/dL (222 to 325 microM/L), 54% of office-based pediatricians stated they would initiate phototherapy whereas 76% of neonatologists would do so. Forty percent of office-based practitioners said they would perform exchange transfusions at serum bilirubin levels of 20 to 25 mg/dL (342 to 428 microM/L), whereas 60% of neonatologists said they would. Only a small percentage of both office-based practitioners (13%) and neonatologists (16%) indicated they would interrupt breastfeeding at 8 to 13 mg/dL (137 to 222 microM/L); but with each incremental level of serum bilirubin, an increasing proportion of neonatologists would interrupt breastfeeding. Little correlation was found between treatment practices and demographic characteristics except for years in practice; physicians with the fewest years in practice (5 years or less) differed significantly from all other groups of physicians in initiating exchange transfusions at higher serum bilirubin concentrations. CONCLUSIONS: The results of this survey indicated a wide range of variation of opinion among both groups of physicians, most likely a reflection of the uncertainty and controversy surrounding these issues. The data may also reflect a possible wide range of "acceptable practice" as opposed to a narrow treatment standard. Office-based practitioners more closely approximated the new 1994 recommendations than neonatologists.

Comparative measurements of phototherapy: a practical guide.

Increasing the dose of administered phototherapy has been shown to increase the rate of bilirubin decrement up to a saturation point beyond which no further increases in bilirubin degradation are observed. This study provides irradiance measurements which can be used to interpret phototherapy dose-response and saturation data clinically and to determine an optimal phototherapy lamp combination for maximal bilirubin degradation.

Determinants of neurobehavioral outcome in low-birth-weights infants.

The relative importance of (1) birth weight, gestational age, and head circumference at birth, and (2) appropriateness of birth weight and head circumference to gestational age in the predictability of neurobehavioral outcome was evaluated in 127 low-birth-weight infants at 7 months of age. Lower absolute birth weights, shorter gestational ages, and smaller head circumferences at birth correlated with poorer outcome (Bayley Scales of Infant Development and abnormal neurologic examination) at the corrected chronologic age of 7 months (r = .28 to .42, all P less than .005). The incidence of low scores on the Mental Development Index and of severe neurologic deficit was significantly higher in small head circumference for gestational age infants than in appropriate head circumference for gestational age infants (both, P less than .05). In the absence of small head circumference, small for gestational age infants had similar incidences of low Bayley scores and abnormal neurologic examinations as did appropriate for gestational age infants. These observations suggest that head circumference at birth may be the single most important variable for subsequent neurobehavioral outcome, and that both birth weight and gestational age may simply be markers of fetal head growth in their relationship to later outcome.

The association of kernicterus with bacterial infection in the newborn.

A total population of 29,395 neonates cared for in the six-year period from 1971 to 1976 was reviewed for evidence of autopsy-proven kernicterus. A total of 327 neonates died and 232 were autopsied. The only cases of kernicterus occurred in four near-term infants with antemortem proven sepsis. All four of these infants weighed more than 2,200 gm and were delivered after gestations of either 36 or 37 weeks. These cases of kernicterus occurred during a period when more aggressive management of hyperbilirubinemia in low-birth-weight infants had apparently eliminated immaturity as a predisposing factor in the development of kernicterus, uncovering bacterial infection as the major remaining etiologic co-factor.

Respiratory distress syndrome of the newborn and complications of pregnancy.

A group of 412 infants with birthweights between 501 and 2,500 gm and gestational ages of 36 weeks or less were studied for the influence of both prolonged ruputre of amniotic membranes and maternal hypertension on the incidence of idiopathic respiratory distress syndrome (IRDS). The occurrence of these complications was associated with a significant decrease in the incidence of IRDS only in infants with birthweights between 1,501 and 2,500 gm (37.4% in the no complications group versus 12.8% in the complications group; P less than .01) or gestational ages of 33 to 36 weeks (35.2% in the no complications group versus 13.1% in the complications group; P less than .01). In infants with birthweights of 1,500 gm or less or gestational ages of 32 weeks or less, the specific antecedent complications of pregnancy did not alter the incidence of IRDS. Rupture of the membranes for more than 72 hours had no greater effect on the incidence of IRDS than those lasting 24 to 72 hours.

Differential effects of maternal heroin and methadone use on birthweight.

An analysis of birthweights of 337 neonates in relation to history of maternal narcotic usage was undertaken. Mean birthweight of infants born to mothers abusing heroin during the pregnancy was 2,490 gm, an effect primarily of intrauterine growth retardation. Low mean birthweight (2,615 gm) was also seen in infants born to mothers who had abused heroin only prior to this pregnancy, and mothers who had used both heroin and methadone during the pregnancy (2,535 gm). Infants born to mothers on methadone maintenance during the pregnancy had significantly higher mean birthweights (2.961 gm), but lower than the control group (3,176 gm). A highly significant relationship was observed between maternal methadone dosage in the first trimester and birthweight, i.e., the higher the dosage, the larger the infant. Heroin causes fetal growth retardation, an effect which may persist beyond the period of addiction. Methadone may promote fetal growth in a dose-related fashion after maternal use of heroin.

Intelligence at six years in relation to neonatal bilirubin levels: follow-up of the National Institute of Child Health and Human Development Clinical Trial of Phototherapy.

Results of the National Institute of Child Health and Human Development Randomized Controlled Trial of Phototherapy were examined for the relationship of neonatal bilirubin level to neurological and developmental outcome at 6-year follow-up. This analysis focused on 224 control children with birth weight of less than 2000 g. Bilirubin levels were maintained below previously specified levels by the use of exchange transfusion only (24%). Rates of cerebral palsy were not significantly higher for children with elevated maximum bilirubin level than for those whose level remained low. No association was evident between maximum bilirubin level and IQ (Full Scale, Verbal, or Performance) by simple correlation analysis (r = -.087, P = .2 for Full Scale) or by multiple linear regression adjusting for factors that covary with IQ (beta = -.15, P = .58). IQ was not associated with mean bilirubin level, time and duration of exposure to bilirubin, or measures of bilirubin-albumin binding. Thus, over the range of bilirubin levels permitted in this clinical trial, there was no evidence of bilirubin toxicity to the central nervous system. Measures used to control the level of bilirubin in low birth weight neonates appear to prevent effectively the risk of bilirubin-induced neurotoxicity.

The fine structural morphology of the midgut of aged Drosophila: a morphometric analysis.

The midguts of 1-day and 72 day-old fruitflies were examined morphometrically at the electron microscopic level. The major alterations noted were that the number of supranuclear mitochondria decreased by approximately 50%, while the volume of individual mitochondria doubled as a function of age. Moreover, approximately 29% of the nuclear volume of old flies, was occupied by inclusion bodies as was 19% of the supranuclear cytoplasmic volume. Additionally, the surface density of rough endoplasmic reticulum was reduced to more than half that of young flies. It is suggested that the functional capability of the parenchymal cells become debilitated due to the presence of these inclusion bodies, and that the cell's ability to manufacture proteins and produce energy are seriously hindered by the mitochondrial alterations and reduction in the surface density of the rough endoplasmic reticulum.

Two thousand years of medical advice on breastfeeding: comparison of Chinese and western texts.

This discussion introduces only a few aspects of the historical writings on breastfeeding in the two cultures. Chinese writings seem to be closer in orientation to modern worldwide medical advice, approaching breastfeeding from a more natural and supportive perspective. Ancient and not-so-ancient western medical advice on breastfeeding often implies the inadequacy of the mother to breastfeed her own infant, especially in the early weeks of life. One can only speculate as to what the historical basis for this may be. European medicine emphasizes the testing of milk for its adequacy. Again, the scientific basis for this is not evident. Modern clinical science finds that the milk of virtually all mothers, even those suffering from significant malnutrition, is adequate for the growth and development of the infant. This focus on the "testing" of milk may represent an early example of the reliance on laboratory diagnosis that has so heavily dominated western medicine in recent years. Finally, western medicine seems more managerial with regard to breastfeeding than Chinese medicine, and has perhaps "medicalized" breastfeeding, a compliant often voiced even now in late 20th century America. Nonetheless, both literatures demonstrate that throughout the history of recorded medicine, physicians have been concerned with promoting optimal breastfeeding and have understood the importance of human milk for the survival, growth, and development of the infant.

PIP: Chinese and Western pediatric scholarship is compared based on published textbook material over the past 2000 years. Modern, late 20th century teachings on breastfeeding are organized around the concept that breastfeeding is a natural, biological behavior that should be initiated immediately after birth and the belief that human milk is almost always the perfect food for the infant, even when the mother is less than adequately nourished or is suffering from some disease. In contrast, ancient and not so ancient Western medical advice on breastfeeding often implies the inadequacy of the mother to breastfeed her own infant, especially in the early weeks of life. This concept continues unquestioned through 1700 years of European medical advice on breastfeeding. One the other hand, William Cadogan's advice in 1750 is remarkably similar to that of the 12th century Chinese physician: absence of medical intervention and a natural and rapid onset of nursing by the biological mother. Chinese writings seem to be closer in orientation to modern worldwide medical advice, approaching breastfeeding from a more natural and supportive perspective. The ancient Chinese medical texts, but not the early European texts, address the origins of human milk. A Chinese work by Sun Simiao (581 to 682) of the Tang Dynasty describes human milk as the product of vital energies. On the initiation of breastfeeding, a 12th century Chinese writing sounds remarkably similar to the advice one would give today to a mother who had just delivered a child. However, Chinese physicians are not without their concepts of bad milk. They describe types of milk that they associate with the induction of various diseases in nurslings. Finally, Western medicine seems more managerial with regard to breastfeeding than Chinese medicine, and has perhaps medicalized breastfeeding. Throughout the 2000 years, both literatures express concern that substitutes for human milk are being used too early and too often.

The narcotic-dependent mother: fetal and neonatal consequences.

During the years 1971--1974, 230 infants born to drug-dependent women and 33 infants born to ex-addicts were studied. Heroin abuse declined while methadone usage increased during those years. Compared to heroin abuse, methadone maintenance treatment during pregnancy was associated with more consistent prenatal care, more normal fetal growth and reduced fetal mortality. Meconium staining of amniotic fluid was increased in the heroin and heroin-methadone groups; this was not associated, however, with an increase in meconium aspiration or a reduction in Apgar scores. Of special note was the equally severe intrauterine growth retardation of infants of former heroin addicts who were free of narcotic use during pregnancy. Neonatal withdrawal from methadone appeared to be more severe than from heroin, as judged by amount of medication required to control symptoms and duration of treatment. In all groups, central nervous system signs were the most common manifestations of withdrawal. Severity of withdrawal did not correlate with late pregnancy maternal methadone dosage. Neonatal seizures occurred in 1.5% of the heroin group and 10% of the methadone group. Discharge of an infant to a parent rather than to an alternate care-taker was more likely if the mother was enrolled in a methadone treatment program. Methadone maintenance programs appear to offer significant therapeutic benefits, balancing the untoward effects of the drug on the newborn infant.

Embryotoxicity and teratogenicity of formocresol on developing chick embryos.

White Leghorn chick embryos were used to investigate the embryotoxicity and teratogenicity of 25 and 50% Buckley's formocresol. Embryos were injected at 48 h and sacrificed on the ninth day of incubation. The percentage of mortality of sham and vehicle controls was 4.8 and 17.7%, respectively, whereas eggs injected with 25 and 50% formocresol displayed a 40 and 100% mortality rate. Gross morphological abnormalities, weights, and crown-rump lengths were determined, demonstrating that Buckley's formocresol is embryotoxic and teratogenic in chick embryos. Gross morphological changes were noted to include cranial hematomas, facial abnormalities, eye and beak deformities as well as alterations in feather germ appearance. Histological changes included alterations in the organization of the eyes, and formation of the beak, palate, vasculature, musculature, cartilage, and bone. Moreover, experimental embryos displayed a retardation of development in that they lagged behind their controls by approximately 24 to 36 h.