Simultaneous cochlear implantation and removal of acoustic neuroma: implications for hearing.

OBJECTIVE: To present our data evaluating the feasibility of simultaneous cochlear implantation with resection of acoustic neuroma. METHODS: This paper describes a case series of eight adult patients with a radiologically suspected acoustic neuroma, treated at a tertiary referral centre in Newcastle, Australia, between 2012 and 2015. Patients underwent cochlear implantation concurrently with removal of an acoustic neuroma. The approach was translabyrinthine, with facial nerve monitoring and electrically evoked auditory brainstem response testing. Standard post-implant rehabilitation was employed, with three and six months' follow-up data collected. The main outcome measures were: hearing, subjective benefit of implant, operative complications and tumour recurrence. RESULTS: Eight patients underwent simultaneous cochlear implantation with resection of acoustic neuroma over a 3-year period, and had 25-63 months' follow up. There were no major complications. All patients except one gained usable hearing and were daily implant users. CONCLUSION: Simultaneous cochlear implantation with resection of acoustic neuroma has been shown to be a safe treatment option, which will be applicable in a wide range of clinical scenarios as the indications for cochlear implantation continue to expand.

Extracorporeal photochemotherapy in mycosis fungoides.

OBJECTIVES: Extracorporeal photo-chemotherapy (ECP, photopheresis) is an approved treatment modality for mycosis fungoides (MF). Our aim is to present our ECP data for MF. METHODS: We retrospectively evaluated 50 MF patients who received ECP for clinical activity, toxicity, and response and outcome rates, and we compared these with combination therapies. RESULTS: The overall response rate (ORR) was 42% (21/50), while the median time to response was 11months (range, 3-48months). Ten of the responders (48%) had 3 or more treatment lines prior to ECP. Eight patients (16%) had adverse events related to ECP. The overall survival (OS) of 50 patients was 72months (range, 3-211). There was no statistically significant difference in the OS in early-stage vs late-stage patients (77 vs 69months, P=0.077). The stage 3 and 4 patients received an average of 31 cycles compared to 55 cycles in stage 1 and 2 patients (P=0.006). The increased extent of ECP was not correlated with the response. Combined treatment with ECP significantly improved the OS (84months vs 62months, P=0.005). DISCUSSION: A low frequency of side effects and improved OS observed in combination therapy makes ECP a favorable option for treating MF.

Black hairy tongue after allogeneic stem cell transplantation: an unrecognized cutaneous presentation of graft-versus-host disease.

Black hairy tongue (BHT) is an unusual condition in adults characterized by marked accumulation of keratin on the dorsum of the tongue, resulting in a hair-like appearance. Herein, we have described 15 patients developing BHT after allogeneic stem cell transplantation (allo-SCT). BHT was generally accompanied by other cutaneous manifestations of cutaneous graft-versus-host disease (GVHD) or a precursor of GVHD. Our experience in this series emphasized that histopathologic evaluation is required for seemingly harmless eruptions like BHT in the posttransplantation period. Given the important prognostic implications of GVHD, physicians should be careful when confronted with BHT.

Nailfold capillary abnormalities are prevalent in sclerodermoid graft-versus-host disease and readily detected with dermatoscopy.

BACKGROUND: Well-recognized videocapillaroscopic patterns have been described in systemic sclerosis (SS). However, no studies have described the capillary abnormalities of sclerodermoid chronic graft-versus-host disease (Scl GVHD) developed after allogeneic haematopoietic stem cell transplantation (allo-HSCT). OBJECTIVES: The aims of this study were to find the characteristics of nailfold capillary changes in Scl GVHD after allo-HSCT. PATIENTS AND METHODS: Eighteen patients affected by Scl GVHD and a control group of 15 patients with lichenoid GVHD were evaluated. Duration and type of sclerodermoid GVHD, Raynaud phenomenon (RP), dysphagia, joint contractures, antinuclear antibodies (ANA), anti-Scl-70 and anticentromere (ACA) antibodies were investigated parameters. A nailfold capillary examination using a standard dermatoscope was performed on all fingers of each subject. RESULTS: Twelve patients were male and six were female with a mean age of 37 +/- 11.6 years. Joint retractions and dysphagia developed in 27.8% and 38.9% of the patients, respectively. Three (16.7%) patients had RP. Autoimmune markers like anti-Scl-70 and ACA were negative in all. Capillaroscopy was abnormal in 15 patients with Scl GVHD. A regular disposition of the capillary loops along with avascular whitish linear areas at the level of the last row, neovascularization with reticular pattern, capillary disorganization, haemorrhages, enlarged capillaries and avascular areas were the main features. No capillary abnormalities were observed in patients with lichenoid GVHD. There was no statistically significant correlation between ANA positivity, RP, joint retractions, dysphagia, extensiveness of Scl GVHD, duration of sclerodermoid lesions and nailfold capillaroscopy analysis. CONCLUSIONS: This study shows the identification of distinct nailfold capillaroscopy patterns in patients with Scl GVHD but it does not confer special risk for any other specific clinical symptoms of the disease.

Postirradiation linear morphoea.

Postirradiation morphoea is an uncommon side-effect of radiotherapy. We report a 74-year-old woman who was treated with radiotherapy for endometrial carcinoma. About 3.5 years after the first dose of radiotherapy, the patient developed linear morphoea starting from the radiation port and affecting distant, nonirradiated skin. Lesions of radiation-induced morphoea are generally described as well-demarcated, indurated plaques with varying amounts of associated erythema; however, there is no previous publication of unilateral band-like distribution of morphoea associated with radiotherapy, to our knowledge.

Cortical potentials as predictors of outcomes with adult cochlear implant recipients - preliminary findings.

A proportion of adult cochlear implant recipients report an inability to use the signal from their cochlear implant effectively at varying post-operative intervals following cochlear implantation. Some of these recipients report deterioration in their ability to use the implant signal and do not benefit from map optimization. Others never attain the level of outcome that they had expected. Speech perception, functional listening in certain circumstances and EABR may demonstrate high performance with the cochlear implant. However recipients report extreme difficulty in noise and distortion of the signal with the cochlear implant. Commonly, environmental sounds overshadow speech. Cortical evoked potentials were measured in a group of recipients who had been reporting such difficulties. They revealed potential processing abnormalities at the level of the cortex in some of these cases. This paper will review the five result profiles that were obtained based on pre-operative, intra-operative and post-operative outcomes. It will begin to explore the value of predictive factors that may indicate the difficulties these recipients would experience post-operatively. Further, management strategies to evaluate and assist in optimizing performance will be addressed.

Biodiesel production from various feedstocks and their effects on the fuel properties.

Biodiesel, which is a new, renewable and biological origin alternative diesel fuel, has been receiving more attention all over the world due to the energy needs and environmental consciousness. Biodiesel is usually produced from food-grade vegetable oils using transesterification process. Using food-grade vegetable oils is not economically feasible since they are more expensive than diesel fuel. Therefore, it is said that the main obstacle for commercialization of biodiesel is its high cost. Waste cooking oils, restaurant greases, soapstocks and animal fats are potential feedstocks for biodiesel production to lower the cost of biodiesel. However, to produce fuel-grade biodiesel, the characteristics of feedstock are very important during the initial research and production stage since the fuel properties mainly depend on the feedstock properties. This review paper presents both biodiesel productions from various feedstocks and their effects on the fuel properties.

Cutaneous, pulmonary and hepatic sarcoidosis associated with autoimmune complications during interferon-alpha treatment for hepatitis C virus infection.

A patient with hepatitis C virus (HCV) infection was diagnosed with cutaneous, pulmonary and hepatic sarcoidosis following interferon alpha therapy. There are only a few cases of sarcoidosis associated with this treatment. This is the first case who not only developed sarcoidosis, but also autoimmune hypothyroidism and thrombocytopenia during interferon alpha therapy due to the immunomodulatory effects of the drug.

Granuloma annulare on herpes zoster scars in a Hodgkin's disease patient following autologous peripheral stem cell transplantation.

Various cutaneous lesions including granulomatous reactions may occur at sites of resolved herpes zoster infection. A 46-year-old man with Hodgkin's disease developed localized granuloma annulare lesions on herpes zoster scars 3 months after allogeneic peripheral stem cell transplantation. This is the first case of granuloma annulare localized on herpes zoster scars that developed following peripheral stem cell transplantation.

Post-transfusion acute graft versus host disease in a 17-year-old girl with a malignant mesenchymal tumor--report of a case.

Post-transfusion graft-versus-host disease (PT-GVHD) is seldom, but it has a high mortality rate, exceeding 90 percent. There is no standard treatment for PT-GVHD and irradiation of blood and its components before transfusion is broadly accepted for the prevention of PT-GVHD. In this report we present a case (a 17-year-old female) of PT GVHD, who died in spite of the use of all available therapeutic options.

A case of Netherton's syndrome with cerebral infarction.

Netherton's syndrome, a rare congenital disease of childhood, is characterized by variable cutaneous erythematous eruptions with different manifestations. A five-year-old boy, who presented with ichthyosis linearis circumflexa, atopic manifestations and pili torti, had spastic hemiparesia due to cerebral infarction. Netherton's syndrome can easily be misdiagnosed as Leiner's disease, generalized psoriasis or nonbullous congenital ichthyosiform erythroderma, especially in the neonatal period, because of its nonspecific clinical and histological features. Pediatricians should consider this syndrome in the differential diagnosis of the generalized erythematous skin disorders of childhood associated with various abnormalities.

Abnormal positive potentials in round window electrocochleography.

OBJECTIVE: To describe an atypical waveform, termed an abnormal positive potential (APP), on round window electrocochleograms (RW ECochG) of children and to relate its occurrence to clinical history. STUDY DESIGN: APPs were identified prospectively, and a retrospective analysis was made of these patients' clinical histories, audiograms, and auditory outcomes (hearing aid, cochlear implant, or nonauditory communication) SETTING: Tertiary referral teaching hospital, day surgery and clinics. PATIENTS: All 431 children <110 months of age suspected of a severe to profound hearing loss who underwent RW ECochG from January 1993 to August 1997. INTERVENTION: Diagnostic RW ECochG for auditory threshold estimation. MAIN OUTCOME MEASURE: The presence on the RW ECochG of the APP: an early positive potential in the absence of a compound action potential (CAP). RESULTS: An APP was observed in 34 children. The APP was most marked in response to clicks and 8-kHz tones. The APP click threshold averaged 70 dB hearing loss. The brainstem evoked potential of these children showed an absence of waves, or a broad positive wave with no subsequent waves. Twenty-nine of 30 behavioral audiograms obtained were indicative of severe to profound hearing loss. Auditory outcomes were available from 26 children; 45% of them derived no help from a hearing aid, and 8 children received a cochlear implant. Clinical factors frequently associated with APP were prematurity in combination with kemicterus or hypoxia. CONCLUSIONS: APP thresholds were lower than neural thresholds or behavioral thresholds. Children with APP need close follow-up, because half of those studied needed nonauditory strategies to develop effective communication.

Waveforms of cochlear implant-evoked auditory brain stem responses in anesthetized young children, recorded with a new preamplifier.

An ultra-low-noise preamplifier has been specifically designed for the conditions in lightly anesthetized young children, to record cochlear implant--evoked auditory brain stem responses (impEABRs). The preamplifier linearly filters out the signal from the radio-frequency implant-driver. The preamplifier provides simultaneous accurate records of the stimulus-current artifact and impEABR, both ipsilaterally and contralaterally. It is convenient to label electrically evoked vertex-positive peaks, eg, eII, on probable analogy with acoustically evoked potentials. In order to label all the commonly observed peaks with latency less than 5 milliseconds, it is necessary at times to use labels eIIa, eIIb, eIIIa, eIIIb, eIVa, eIVb, eVa, and eVb. The records also show two distinct nadirs, with latencies about 2.5 milliseconds and about 6 milliseconds. The details of the waveforms are preserved as the stimulus strength descends towards threshold.

Use of transtympanic round window electrocochleography for threshold estimations in children.

OBJECTIVE: This study aimed to evaluate the efficacy of round window electrocochleography for the estimation of hearing thresholds in difficult-to-test children. STUDY DESIGN: The study was a retrospective analysis. SETTING: A standard day-stay operating room was used. PATIENTS: Round window electrocochleography was performed on 198 children between January 1993 and January 1996. INTERVENTION: The intervention was diagnostic. MAIN OUTCOME MEASURE: Clinically reliable pure-tone audiograms were obtained in 101 patients (50.9%) for comparisons of electrocochleography and behavioral thresholds. RESULTS: The mean differences between electrocochleography and behavioral thresholds at 0.5, 1, 2, and 4 kHz were less than 6 dB. Ninety-seven percent of the results were within +30 to -30 dB, and approximately 86% of the results were within +20 to -20 dB. Good correlation coefficients of 0.83, 0.84, 0.91 and 0.88 were found between electrocochleography and behavioral thresholds at 0.5, 1, 2, and 4 kHz, respectively. Only 2 of 395 ears were complicated postoperatively by suppurative otitis media. There were no anesthetic complications. CONCLUSION: Round window electrocochleography can be used in conjunction with other audiometric methods for threshold estimations in the difficult-to-test children and as part of the preoperative assessment for cochlear implantation.

Waveforms of cochlear implant-evoked auditory brain stem responses in anesthetized young children, recorded with a new preamplifier.

An ultra-low-noise preamplifier has been specifically designed for the conditions in lightly anesthetized young children, to record cochlear implant-evoked auditory brain stem responses (impEABRs). The preamplifier linearly filters out the signal from the radio-frequency implant-driver. The preamplifier provides simultaneous accurate records of the stimulus-current artifact and impEABR, both ipsilaterally and contralaterally. It is convenient to label electrically evoked vertex-positive peaks, eg, eII, on probable analogy with acoustically evoked potentials. In order to label all the commonly observed peaks with latency less than 5 milliseconds, it is necessary at times to use labels eIIa, eIIb, eIIIa, eIIIb, eIVa, eIVb, eVa, and eVb. The records also show two distinct nadirs, with latencies about 2.5 milliseconds and about 6 milliseconds. The details of the waveforms are preserved as the stimulus strength descends toward threshold.