Endolymphatic duct and sac decompression: A new technique for Ménière’s disease treatment

BACKGROUND: The present article aims to introduce the endolymphatic duct and sac decompression technique (DASD) and to give a spotlight on its benefits in Ménière’s disease (MD) treatment. METHODS: Eighty-two patients with intractable MD which met the inclusion criteria were recruited and underwent DASD. This technique allows a meningeal decompression of the duct and the sac from the posterior cranial fossa to the labyrinthine block. The authors considered as main outcomes, the change of the dizziness handicap inventory (DHI) results, with the evaluations of the three sub-scales (Functional scale, Physical scale, and Emotional scale); ear fullness and tinnitus change on the perceptions of the patient; and hearing stage with four-Pure Tone Average (500 hz-1000 hz-2000 hz-4000 hz). The differences between the preoperative and the postoperative score were evaluated. A comparison with the literature was conducted. RESULTS: After a 14-month follow-up, patients that underwent DASD reported a remarkable improvement of the symptoms in all three functional scales, confirmed by the total DHI. The difference between preoperative and postoperative scores is statistically significant. The data describe an ear fullness and tinnitus improvement. The multi-frequency tonal average before and after the surgery does not suggest a worsening of the value for any of 82 patients. CONCLUSION: The modification of sac surgery includes the endolymphatic duct in the decompression area allowing inner ear functional improvement, vertigo control, ear fullness improvement with minimal risk of facial nerve paralysis, and hearing loss. DASD is an improved old surgical technique

Significance of heterozygosis M34T mutation of GJB2 gene in non-syndromic congenital deafness. Retrospective analysis of 12,472 samples of amniotic fluid

Objective: to determinate the role of heterozygosis of M34T mutation of GJB2 gene in non syndromic congenital deafness. Methods: retrospective study between March 2010 and June 2013. Molecular screening for 35delG and M34T mutations of the GJB2 gene was offered to all women undergoing to second trimester genetic amniocentesis. Patients were excluded from the study group if one of the following conditions were present: infections, fetal abnormalities, family history for congenital deafness, diagnosis of chromosomal abnormalities, and consanguinity between parents. Results: a total of 12.472 Caucasian women gave informed consent for this test. Seventy-seven cases were excluded. From the 12.395 amniotic fluid analysis remained, the following was found: 2 cases of 35delG homozygosis and 352 cases of heterozygous carriers (42 M34T mutation, 298 35delG mutation, 12 double heterozygosis M34T/35delG). The follow up in first year of life in the 42 newborns with heterozygosis for M34T mutation showed a mild deafness in 23 cases. Conclusions: in our series, presence of heterozygosis M34T mutation is associated in more than 50% of cases to mild congenital deafness

Autoimmunity in Membranous Nephropathy Targets Aldose Reductase and SOD2

Glomerular targets of autoimmunity in human membranous nephropathy are poorly understood. Here, we used a combined proteomic approach to identify specific antibodies against podocyte proteins in both serum and glomeruli of patients with membranous nephropathy (MN). We detected specific anti–aldose reductase (AR) and anti–manganese superoxide dismutase (SOD2) IgG4 in sera of patients with MN. We also eluted high titers of anti-AR and anti-SOD2 IgG4 from microdissected glomeruli of three biopsies of MN kidneys but not from biopsies of other glomerulonephritides characterized by IgG deposition (five lupus nephritis and two membranoproliferative glomerulonephritis). We identified both antigens in MN biopsies but not in other renal pathologies or normal kidney. Confocal and immunoelectron microscopy (IEM) showed co-localization of anti-AR and anti-SOD2 with IgG4 and C5b-9 in electron-dense podocyte immune deposits. Preliminary in vitro experiments showed an increase of SOD2 expression on podocyte plasma membrane after treatment with hydrogen peroxide. In conclusion, our data support AR and SOD2 as renal antigens of human MN and suggest that oxidative stress may drive glomerular SOD2 expression