Sudden sensorineural hearing loss: our experience in diagnosis, treatment, and outcome

OBJECTIVES: To describe our experience concerning sudden sensorineural hearing loss (SSNHL) in a large single-institution series of SSNHL patients and to discuss the results. METHOD: This was a retrospective study, and the charts of 156 consecutive inpatients (65 males, mean age 44 years, range 10-74 years; 91 females, mean age 46 years, range 15-75 years) with the diagnosis of SSNHL from 1987 to 2000 were reviewed. One hundred forty-three of 156 patients received multidrug therapy (plasma expanders, antiaggregants, steroids), whereas only 13 SSNHL patients received hyperbaric oxygen therapy. RESULTS: Old age, vascular and metabolic risk factors, and cigarette smoking do not a have a high prevalence in the SSNHL population. An etiologic factor was detected in 23 of 156 (15%) cases (16 cases of acute infection, 4 cases of neurovascular conflicts, 2 cases of cerebellar angiomas, 1 case of cochleovestibular schwannoma). The outcome was not related to the laterality, age, or hearing loss type. On the contrary, a statistically significant association between poor recovery and male sex, both tinnitus and vertigo, and the initial severity of the hearing loss was observed. CONCLUSIONS: Mostly, SSNHL results in idiopathic disease. At present, diagnostic and therapeutic efforts appear to be inadequate to improve the prognosis of SSNHL. Further studies are needed to obtain better knowledge about the etiopathogenesis of SSNHL so that new therapeutic strategies can be considered in the treatment of this challenging ear disease.     

A pilot study of low-dose subcutaneous alemtuzumab therapy for patients with hemotherapy-refractory chronic lymphocytic leukemia

Subcutaneous low-dose alemtuzumab (10 mg t.i.w. for 18 weeks) induced a 50% response rate, including 25% complete response, in 16 patients with refractory chronic lymphocytic leukemia (CLL) patients. The responses were substantial even in patients with unfavorable cytogenetics, fludarabine/rituximab refractoriness, Rai stage IV, previous infections, and age over 65 years. Subcutaneous low-dose alemtuzumab is effective in poor prognosis B-CLL, and has a particularly favourable toxicity profile.     

An alternative immunohistochemical method for detecting Leishmania amastigotes in paraffin-embedded canine tissues

Canine visceral leishmaniasis (CVL) is a zoonosis and a chronic systemic disease of the dog caused by a protozoan by the species Leishmania infantum in the Old World and Leishmania chagasi in the New World. Several methods are currently employed for the diagnosis of CVL including microscopic detection of the parasite in bone marrow and lymph node aspirates, demonstration of specific antibodies anti-Leishmania in sera from infected animals, and isolation of the parasite by in vitro culture or by inoculation of laboratory animals. However, a definitive diagnosis is based on the actual detection of the parasite, which is conventionally achieved by examining Giemsa-stained smears or histopathological sections stained with hematoxylin and eosin. These methods have a low sensitivity, and therefore, they are often inconclusive. This is particularly true in canine organs that have a low level of parasitism such as kidneys, lungs, central nervous system, and testis, or, in some cases, the skin. The technique for immunohistochemical detection of leishmanial amastigotes in canine tissues has been reported previously and has proved to be undoubtedly efficient for the diagnosis. In this paper, we describe a straightforward and inexpensive immunohistochemical approach for Leishmania detection in formalin-fixed paraffin-embedded canine tissues. Amastigote forms of Leishmania were easily observed within macrophages in several organs from naturally infected dogs using the streptavidin-biotin immunohistochemical method with canine hyperimmune serum as the primary antibody. In addition, the secondary antibody used was not specific to canine immunoglobulin, characterizing a cross-immune reaction. Our results indicate that this technique could be a useful tool for epidemiological, clinical, and histopathological studies.     

Intestinal ischemia reperfusion injury and multisystem organ failure

Intestinal ischemia-reperfusion is a common pathway for many diseases in infants, children, and adults, and this may lead to multiple organ dysfunction syndrome and death. While several studies have investigated reperfusion injury in cardiac, cerebral, and hepatic disease, limited work has been published on intestinal ischemia-reperfusion and its multiorgan effects.The authors have developed models of intestinal ischemia-reperfusion in rats and have demonstrated that intestinal reperfusion causes liver energy failure at normothermia. This is followed by 100% mortality within 4 hours of reperfusion. Moderate hypothermia (32 degrees C to 33 degrees C) induced throughout ischemia and reperfusion prevents liver energy failure, intestinal damage, and neutrophil infiltration in the lungs. Moderate hypothermia in this model of intestinal ischemia and reperfusion prevents mortality. Further studies are needed to establish whether therapeutic hypothermia is a useful intervention in the treatment of infants and children with intestinal injuries caused by ischemia and reperfusion.     

Cytologic aspects of the nasal respiratory epithelium throughout the menstrual cycle

A prospective study was performed to investigate the changes in nasal cytology that occur in healthy premenopausal women throughout the menstrual cycle. Eighty-eight women with an ovulatory menstrual cycle underwent nasal sampling with a cytobrush by direct vision of the middle and inferior nasal turbinates during the follicular, periovular, and luteal phases of the menstrual cycle, and the specimens were evaluated with the maturation index. Hematoxylin-eosin staining showed the cytologic aspects of the nasal respiratory epithelium and of vaginal smears according to the three different phases of the menstrual cycle. Along with the vaginal cells, the nasal respiratory epithelium is an ovarian steroid target.     

Ileocolic Arteriovenous Fistula with Superior Mesenteric Vein Aneurism: Endovascular Treatment

We report a case of a venous aneurysm secondary to an acquired ileocolic arteriovenous fistula in a 64-year-old woman with recurrent abdominal pain and history of appendectomy. The aneurysm was diagnosed by ultrasound and computed tomography. Angiography showed an arteriovenous fistula between ileocolic branches of the superior mesenteric artery and vein. This vascular abnormality was successfully treated with coil embolization.     

Deficit of in vivo mitochondrial ATP production in OPA1-related dominant optic atrophy

Dominant optic atrophy has been associated with mutations in the OPA1 gene, which encodes for a dynamin-related GTPase, a mitochondrial protein implicated in the formation and maintenance of mitochondrial network and morphology. We used phosphorus magnetic resonance spectroscopy to assess calf muscle oxidative metabolism in six patients from two unrelated families carrying the c.2708-2711delTTAG deletion in exon 27 of the OPA1 gene. The rate of postexercise phosphocreatine resynthesis, a measure of mitochondrial adenosine triphosphate production rate, was significantly delayed in the patients. Our in vivo results show for the first time to our knowledge a deficit of oxidative phosphorylation in OPA1-related DOA.     

The ND1 gene of complex I is a mutational hot spot for Leber's hereditary optic neuropathy

A novel mitochondrial DNA (mtDNA) transition (3733G-->A) inducing the E143 K amino acid change at a very conserved site of the NADH dehydrogenase subunit 1 (ND1) was identified in a family with six maternally related individuals with Leber's hereditary optic neuropathy (LHON) and in an unrelated sporadic case, all negative for known mutations and presenting with the canonical phenotype. The transition was not detected in 1,082 control mtDNAs and was heteroplasmic in several individuals from both pedigrees. In addition, the mtDNAs of the two families were found to belong to different haplogroups (H and X), thus confirming that the 3733G-->A mutation occurred twice independently. Phosphorus magnetic resonance spectroscopy disclosed an in vivo brain and skeletal muscle energy metabolism deficit in the four examined patients. Muscle biopsy from two patients showed slight mitochondrial proliferation with abnormal mitochondria. Biochemical investigations in platelets showed partially insensitive complex I to rotenone inhibition. We conclude that the 3733G-->A transition is a novel cause of LHON and, after those at positions 3460 and 4171, is the third ND1 mutation to be identified in multiple unrelated families. This finding shows that, in addition to ND6, the ND1 subunit gene is also a mutational hot spot for LHON.     

Cytologic aspects of the nasal respiratory epithelium in postmenopausal women treated with hormone therapy

OBJECTIVE: To investigate the effects of hormone therapy (HT) on nasal respiratory epithelium in postmenopausal women. DESIGN: Prospective open clinical trial. SETTING: Outpatient menopausal clinic. PATIENT(S): One hundred three healthy postmenopausal women, of whom 55 treated with HT, and 48 untreated women (controls). INTERVENTION(S): Different regimens of HT by patch, gel, or oral administration. MAIN OUTCOME MEASURE(S): Cytologic changes of nasal, middle and inferior turbinate cells compared with vaginal cytologic findings by using the maturation index. RESULT(S): Hematoxylin-eosin staining for the maturation index confirmed similar trophic cytologic aspects between the nasal respiratory epithelium and vaginal smears in HT-treated women and controls. Women treated with sequential HT or estrogen therapy (ET) showed better trophic characteristics in the nasal cytological samples compared with women treated with continuous combined HT. CONCLUSION(S): Along with vaginal cells the nasal respiratory epithelium is an estrogen target. The activity of HT in the nasal respiratory epithelium may depend on the type of hormone regimen used.