Yuika Sakurai

Cochlear implantation in patients with chronic otitis media

OBJECTIVE Patients with complications of otitis media present a significant challenge to safe cochlear implantation. We describe our experience of cochlear implantation in patients with chronic ear disease, and propose management principles according to the presenting status of the ear. METHODS Cochlear implantations were performed as treatment for complications of otitis media in seven patients. They consisted of four patients with adhesive otitis media, two patients with an open cavity after surgery for otitis media and one patient with eosinophilic otitis media. The electrodes were inserted by an approach via the external auditory canal in patients with poor growth of the mastoid antrum or adhesion of the tympanum. For the patients with an open cavity, we created a posterior wall for the external auditory canal and perform the mastoid obliteration. Modified Rambos technique with blind sac closure of the external auditory canal was performed for the case of eosinophilic otitis media as a single-stage procedure. RESULTS The post-operative courses were good. However, a post-operative infection developed in one patient who had previously undergone radiation therapy following surgical excision of a cerebellar tumor. CONCLUSION Transcanal approach is effective in a poorly pneumatized mastoid or severe adhesive otitis media. A decision whether implantation as a single-stage or multiple stages depends on the condition of each cases. But there is a possibility of infection even if we selected a stage operation.

Mutations in the NOG gene are commonly found in congenital stapes ankylosis with symphalangism, but not in otosclerosis

Usami S, Abe S, Nishio S, Sakurai Y, Kojima H, Tono T, Suzuki N. Mutations in the NOG gene are commonly found in congenital stapes ankylosis with symphalangism, but not in otosclerosis.

The Patients Associated With TMPRSS3 Mutations Are Good Candidates for Electric Acoustic Stimulation

Objectives: To clarify the frequency of TMPRSS3 mutations in the hearing loss population, genetic analysis was performed, and detailed clinical characteristics were collected. Optical intervention for patients with TMPRSS3 mutations was also discussed. Methods: Massively parallel DNA sequencing (MPS) was applied for the target exon-sequencing of 63 deafness genes in a population of 1120 Japanese hearing loss patients. Results: Hearing loss in 5 patients was found to be caused by compound heterozygous TMPRSS3 mutations, and their detailed clinical features were collected and analyzed. Typically, all of the patients showed ski slope type audiograms and progressive hearing loss. Three of the 5 patients received electric acoustic stimulation (EAS), which showed good results. Further, the onset age was found to vary, and there were some correlations between genotype and phenotype (onset age). Conclusions: MPS is a powerful tool for the identification of rare causative deafness genes, such as TMPRSS3. The present clinical characteristics not only confirmed the findings from previous studies but also provided clinical evidence that EAS is beneficial for patients possessing TMPRSS3 mutations.

The hearing status in 12 female and 15 male Japanese Fabry patients

OBJECTIVE Fabry disease (FD) is an x-linked inherited disease that causes a lack of a lysosomal enzyme, alpha-galactosidaseA, leading to cellular accumulation of glycosphingolipids of the whole body. This accumulation in the inner ear causes hearing loss also. However, FD is rare, and the frequency is lower in females than in males. Thus, there have been few comparative studies between women and men for hearing loss in FD. Accordingly, we examined the hearing status of both male and female FD patients and elucidated the similarities and differences. We also analyzed for correlations between the hearing status and renal and heart disorders. METHODS 12 women and 15 men were studied by means of pure tone audiometry, the relationships between the hearing status and the renal and cardiac functions. RESULTS The audiogram type was the flat type in a majority of both women and men, followed by the high type and low type, while the U-shaped type was rare. Examination of the thresholds average showed abnormality in one woman and four men. Comparison to threshold for each age bracket of normal subjects showed abnormality in three women and nine men. No correlations were found between the hearing loss and either the renal or cardiac function. All the patients with renal dysfunction had abnormal hearing. CONCLUSIONS The frequency of hearing loss in FD was higher in men than in women. Also, there may be some relationship between renal function and hearing loss in FD.

Inner ear pathology of alpha-galactosidase A deficient mice, a model of Fabry disease.

OBJECTIVE Fabry disease is characterized by genetic alpha-galactosidase A deficiency, resulting in accumulation of glycolipids (GL-3) and tissue damage. Hearing loss is also common and attributed to GL-3 accumulation in the inner ear. The only reported histological studies dealt with murine and human specimens. Accordingly, histopathological studies of the cochlea were performed on an alpha-galactosidase A deficient murine model of Fabry disease, using C57BL6/J mice as the controls. METHODS The hearing ability was evaluated using the ABR threshold, while cochlear specimens were observed light microscopically and ultrathin temporal bone sections by TEM. RESULTS HE staining showed no accumulation of GL-3 or abnormal cochlear morphology in the alpha-galactosidase A deficient mice, but toluidine blue staining and TEM revealed GL-3 accumulation in the stria vascularis and kidney. No GL-3 accumulation was detected in the C57BL6/J controls by either HE staining or TEM. The alpha-galactosidase A deficient mice and the controls showed no clear differences in the ABR threshold (hearing acuity), but for older animals the threshold was higher in the C57BL6/J controls. CONCLUSION In summary, although the alpha-galactosidase A deficient mice showed no clear hearing loss, GL-3 accumulation was demonstrated in the cochlea.

Bilateral Deafness as a Complication of the Vaccination-A Case Report

The vaccination has much profit for an infectious disease. On the other hand, there is little frequency, side effects may appear. It includes severe complication. We reported the case that resulted in bilateral acute profound hearing loss after mumps alone and measles and rubella (MR) vaccination. The case was a 5 years old girl. She inoculated mumps alone and MR vaccine. After 18days later, both sides profound hearing loss occurred in her. The hearing loss was not improved by the intravenous feeding of the steroid. Three months later, cochlea implantation was carried out to her right ear. She got hearing again. As for the hearing loss, mumps vaccine was considered as a cause from a latency period until the onset. The bilateral profound hearing loss that was a very rare complication was occurred by vaccination. The care of the hearing is important, but the mental care of an affected child and the parent is important, too.