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.

Quantitative colorectal cancer perfusion measurement by multidetector-row CT: does greater tumour coverage improve measurement reproducibility?

The purpose of this study was to determine if greater z-axis tumour coverage improves the reproducibility of quantitative colorectal cancer perfusion measurements using CT. A 65 s perfusion study was acquired following intravenous contrast administration in 10 patients with proven colorectal cancer using a four-detector row scanner. This was repeated within 48 h using identical technical parameters to allow reproducibility assessment. Quantitative tumour blood volume, blood flow, mean transit time and permeability measurements were determined using commercially available software (Perfusion 3.0; GE Healthcare, Waukesha, WI) for data obtained from a 5 mm z-axis tumour coverage, and from a 20 mm z-axis tumour coverage. Measurement reproducibility was assessed using Bland-Altman statistics, for a 5 mm z-axis tumour coverage, and 20 mm z-axis tumour coverage, respectively. The mean difference (95% limits of agreement) for blood volume, blood flow, mean transit time and permeability were 0.04 (-2.50 to +2.43) ml/100 g tissue; +8.80 (-50.5 to +68.0) ml/100 g tissue/min; -0.99 (-8.19 to +6.20) seconds; and +1.20 (-5.42 to +7.83) ml/100 g tissue/min, respectively, for a 5 mm coverage, and -0.04 (-2.61 to +2.53) ml/100 g tissue; +7.40 (-50.3 to +65.0) ml/100 g tissue/min; -2.46 (-12.61 to +7.69) seconds; and -0.23 (-8.31 to +7.85) ml/100 g tissue/min, respectively, for a 20 mm coverage, indicating similar levels of agreement. In conclusion, increasing z-axis coverage does not improve reproducibility of quantitative colorectal cancer perfusion measurements.

Protective effect of bilirubin in ischemia-reperfusion injury in the rat intestine.

BACKGROUND: Although bilirubin, which crosses the blood-brain barrier, can cause irreversible brain damage, it also possesses antioxidant properties that may be protective against oxidative stress. Intestinal ischemia-reperfusion (IR) injury results in cell destruction, mediated via the generation of reactive oxygen species. Although increased serum bilirubin is correlated with increased antioxidant potential in the face of hyperoxia, evidence of bilirubin-associated protective effect against IR injury remains nonspecific. We therefore sought to investigate whether hyperbilirubinemia would be protective against IR injury to the intestine. METHODS: Young adult rats were randomly assigned to one of three groups: 1) IR/control (n = 12); 2) IR/hyperbilirubinemia (n = 10), in which IR was generated while the rats were treated with a continuous infusion of bilirubin; and 3) hyperbilirubinemia controls (n = 10). Blood and intestinal tissue samples were obtained to determine serial thiobarbituric acid reducing substances (index of lipid peroxidation) and for xanthine oxidase/xanthine dehydrogenase and glutathione/glutathione disulfide ratios. Intestinal histopathology was graded from 1 (normal) to 4 (severe necrotic lesions). RESULTS: Histopathologic scoring and circulating and tissue thiobarbituric acid reducing substances were highest in the IR/control animals compared with either the IR/hyperbilirubinemics or the controls. All of these are consistent with the most severe injury in this group. Xanthine oxidase/xanthine dehydrogenase ratios were not significantly different among the groups. CONCLUSION: Hyperbilirubinemia ameliorates the extent of intestinal IR injury in our model and appears to act as an antioxidant. This study supports the concept that bilirubin possesses some beneficial properties in vivo, although no direct clinical conclusions can be drawn from these data.

Jaundice and breastfeeding.

Optimal management of breastfeeding does not eliminate neonatal jaundice and elevated serum bilirubin concentrations. Rather, it leads to a pattern of hyperbilirubinemia that is normal and, possibly, beneficial to infants. Excessive frequency of exaggerated jaundice in a hospital or community population of breastfed infants may be a warning that breastfeeding policies and support are not ideal for the establishment of good breastfeeding practices. The challenge to clinicians is to differentiate normal patterns of jaundice and hyperbilirubinemia from those that indicate an abnormality or place an infant at risk.

Breastfeeding and jaundice.

In the breastfed infant, prolongation of unconjugated hyperbilirubinemia into the third and later weeks of life in the healthy newborn is a normal and regularly occurring extension of physiologic jaundice. This is known as breastmilk jaundice. A factor in human milk increases the enterohepatic circulation of bilirubin. Insufficient caloric intake resulting from maternal and/or infant breastfeeding difficulties may also increase serum unconjugated bilirubin concentrations. This is the infantile equivalent of adult starvation jaundice. It is known as breastfeeding jaundice or "breast-nonfeeding jaundice." This increase in severity of physiologic jaundice of the newborn also results from increased enterohepatic circulation of bilirubin, but not because of a factor in human milk. In extreme cases, it may place the infant at risk for development of bilirubin encephalopathy. Optimal breastfeeding practices, which result in minimal initial weight loss and early onset of weight gain, are associated with both reduced breastfeeding jaundice and minimization of the intensity of breastmilk jaundice.

Jaundice in the breastfed infant.

Maternal perception of insufficient milk is a widespread phenomenon in modern breastfeeding. This article addresses underlying physiology, feeding patterns, growth patterns, and medical complications as they impact milk supply and infant growth. The complexity of mother-infant factors leads to a broad differential diagnosis. Problem-oriented management is discussed with the goal of preventing low milk supply, intervening promptly for feeding problems, promoting infant growth, and preserving the breastfeeding relationship.

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.

Effects of nicotine on tongue development in the CD-1 mouse.

Fetuses of pregnant CD-1 mice, exposed to 0.1% nicotine sulfate at a dose of 1.67 mg/kg body weight from the 6th through the 15th gestational days were compared with control fetuses to assess the effects of nicotine on tongue development. Mothers were sacrificed on the 18th gestational day. The heads of a total of 130 nicotine-treated and 348 control fetuses were embedded in paraffin and sectioned in the frontal plane. 9.6% of the nicotine-treated fetuses had palatal clefts and their tongue development was much retarded compared to the controls. The tongues of the clefted fetuses were misshaped, reduced in size, had no filiform or fungiform papillae, and their myotubes were just in the process of formation. The circumvallate papilla of these fetuses were present but neither taste buds nor glands of von Ebner had as yet developed. Tongue development of nicotine-treated, non-clefted fetuses were closer to those of the controls. The anlagen of their filiform and fungiform papillae were developing, their myotubes were longer and better arranged, their circumvallate papilla was present but without taste buds, and their glands of von Ebner were not developed. It is suggested that nicotine interferes with both palatal and mesenchymal components of tongue development.