An efficient strategy for establishing a model of sensorineural deafness in rats

Ototoxic drugs can be used to produce a loss of cochlear hair cells to create animal models of deafness. However, to the best of our knowledge, there is no report on the establishment of a rat deafness model through the combined application of aminoglycosides and loop diuretics. The aim of this study was to use single or combined administration of furosemide and kanamycin sulfate to establish rat models of deafness. The rats received intravenous injections of different doses of furosemide and/or intramuscular injections of kanamycin sulfate. The auditory brainstem response was measured to determine the hearing threshold after drug application. Immunocytochemistry and confocal microscopy were performed to evaluate inner ear morphology. In the group receiving combined administration of furosemide and kanamycin, the auditory brainstem response threshold showed significant elevation 3 days after administration, higher than that produced by furosemide or kanamycin alone. The hair cells showed varying degrees of injury, from the apical turn to the basal turn of the cochlea and from the outer hair cells to the inner hair cells. The spiral ganglion cells maintained a normal morphology during the first week after the hair cells completely disappeared, and then gradually degenerated. After 2 months, the majority of spiral ganglion cells disappeared, but a few remained. These findings demonstrate that the combined administration of furosemide and kanamycin has a synergistic ototoxic effect, and that these drugs can produce hair cell loss and hearing loss in rats. These findings suggest that even in patients with severe deafness, electronic cochlear implants may partially restore hearing.

A Novel De novo GATA-binding Protein 3 Mutation in a Patient with Hypoparathyroidism, Sensorineural Deafness, and Renal Dysplasia Syndrome

INTRODUCTION Hypoparathyroidism, sensorineural deafness, and renal dysplasia （HDR） syndrome, also called Barakat syndrome, is an autosomal dominant genetic disease caused by haploinsufficiency of the GATA-binding protein 3 （GATA3） gene located on the 10pl 5 chromosome.

The effect of acupuncture at Jǐngjiājǐ(颈夹脊) on the repair and regeneration of cochlear hair cells of rats with sensorineural deafness

Objective： To observe the effect of acupuncture at Jǐngjiājǐ（颈夹脊）on the repair and regeneration of cochlear hair cells of guinea pigs with sensorineural deafness.Methods： Sixty healthy guinea pigs were selected, 20 guinea pigs were randomly assigned to the normal control group（group A）, and other guinea pigs were randomly divided into model control group（group B） and acupuncture treatment group（group C） after injection with gentamicin sulfate in order to induce deafness. No intervention was given to the guinea pigs in group A and group B, and acupuncture at Jǐngjiājǐ was given to the guinea pigs in group C for 30 days. ABR threshold, DPOAE amplitudes and hair cells counting of guinea pigs in each group were recorded after intervention for 30 days.Results： After intervention for 30 days, ABR threshold in group C was significantly lower than that in group B（38.46 ±7.36 vs 82.94 ±6.47, P〈0.01）. and the DPOAE amplitudes in group C were obviously higher than that in group B（28.06 ±5.64 vs 25.23 ±5.38, P〈0.01）. The number of cochlear hair cells in group C increased significantly, over 50% of the hair cells survived, accounting for 66.67% of the observation cases. The number of cochlear hair cells in the 3 rd and 4 th gyri was close to the normal level, and plenty of proliferous sustentacular cells can be seen. Compared with group B, the number of cochlear outer hair cells in each gyrus in group C significantly increased（36.76 ±1.97 vs 28.59±2.24, P〈0.01）, indicating that acupuncture at Jǐngjiajǐ can promote the repair and regeneration of cochlear hair cells.Conclusion： Acupuncture at Jǐngjiajǐ can promote the repair and regeneration of cochlear hair cells, thus improving the hearing of guinea pigs with deafness.

Clinical and Experimental Observation on Treatment of Sensorineural Deafness with Bushen Conger Tablet (补肾聪耳片)

Objective: To observe the effect of Bushen Conger （BSCE） tablet, a pure Chinese herbal medicine for reinforcing Kidney, removing blood stasis and opening apertures, in treating sensorineural deafness. Methods: Three hundred and ten patients were treated by BSCE tablet and the effects were observed. At the same time the effects of BSCE tablet on hearing, SOD, LPO, etc. were also assayed in guinea pigs. Results: The clinical total effective rate of BSCE tablet was 71.9 %. Animal experiment proved that the drug was effective in preventing and treating toxic deafness induced by aminoglycoside antibiotics. Conclusion: BSCE tablet could cure sensorineural deafness through adjusting the cochlear cyclic neucleotides as well as its SOD level, reducing the LPO level, and raising the thyroid and sexual glandular functions.

HDR syndrome presented with nephrotic syndrome in a Chinese boy: A case report

BACKGROUND HDR syndrome is a rare genetic disease caused by variants in the GATA3 gene and is phenotypically defined by the triad of hypoparathyroidism(H),deafness(D),and renal disease(R).Renal disorders of HDR are mainly developmental ab-normalities,although renal functional abnormalities can also be observed.Ne-phrotic syndrome or nephrotic-level proteinuria is rare in HDR syndrome.Here,we report a Chinese infant with HDR syndrome who presented with early-onset nephrotic syndrome.We suggest that variants in the GATA3 gene might be asso-ciated with nephrotic syndrome.(p.Pro235 Leu),in exon 3 of the GATA3 gene.CONCLUSION We report an infant with HDR syndrome who presented with early-onset nephrotic syndrome in China.We suggest that variants in the GATA3 gene might be associated with infant-onset nephrotic syndrome.