Cochlear implantation for progressive hearing loss caused by an A8296G mutation in mitochondrial DNA.

Mitochondrial DNA mutations such as A3243G or A1555G are widely reported to cause hearing loss, but few reports exist on the A8296G mutation, which can also cause hearing loss. This report presents the case of a patient with the A8296G mutation and severe bilateral sensorineural hearing loss (SNHL) that progressed over two decades. The patient had no history of diabetes, but did have a family history of SNHL in her father and maternal grandmother. She was first diagnosed with SNHL at 45 years of age, and an A8296G mutation was found. The hearing threshold in the low-frequency range of the right ear was preserved at diagnosis, but eventually declined resulting in severe bilateral hearing loss by the age of 66 years, and cochlear implantation (CI) was performed in the left ear. The hearing threshold three months after CI was 25-45 dB HL, and the phoneme speech discrimination score in the left ear improved from 20% without CI to 74% with CI. SNHL patients with the A8295G mutation are good candidates for treatment with CI.

Cochlear Implantation in Patients with Mitochondrial Gene Mutation: Decline in Speech Perception in Retrospective Long-Term Follow-Up Study.

Clinical evidence of the effectiveness of cochlear implantation for hearing loss with mitochondrial DNA mutation is limited. Most reports have only described short-term postoperative speech perception, which may not reflect the limitations of cochlear implantation caused by progressive retrocochlear dysfunction. The present study aimed to investigate long-term speech perception after cochlear implantation in patients with severe to profound hearing loss associated with mitochondrial DNA mutation. A retrospective chart review was performed on patients with mitochondrial DNA mutation who had undergone cochlear implantation at the Department of Otolaryngology and Head and Neck Surgery at the University of Tokyo Hospital. We extracted data on causative mutations, clinical types, clinical course, perioperative complications, and short-term and long-term postoperative speech perception. Nine patients with mitochondrial DNA mutation underwent cochlear implantation. The mean observation period was 5.5 ± 4.2 years (range, 1-13 years), and seven patients were followed for more than 3 years. Two of the seven patients who initially showed good speech perception exhibited deterioration during long-term follow-up. The absence of an acute progression of cognitive decline in patients, showing a gradual decrease in speech perception, suggests that the deterioration of speech perception was caused by progressive retrocochlear degeneration. Although most patients with mitochondrial DNA mutation maintained good speech perception for more than 3 years after cochlear implantation, retrocochlear degeneration could cause the deterioration of speech perception during long-term follow-up.

Vestibular evoked myogenic potentials evoked by multichannel cochlear implant - influence of C levels.

CONCLUSIONS: This study showed that vestibular evoked myogenic potentials (VEMPs) evoked by cochlear implant (CI), could be related to the comfortable level (C level), particularly in the channels that are closer to the apical turn of the cochlea. OBJECTIVE: The purpose of this study was to investigate the correlation between VEMPs and C level of each channel. SUBJECTS AND METHODS: We investigated 24 children who underwent cochlear implantation. VEMPs were recorded from the operated ears with the CI switched 'off' or 'on'. To investigate the correlation between VEMPs and C level, we selected 13 patients with Nucleus 24 (SPrint), and divided them into group A (normal VEMPs) and B (absence of VEMPs). In these children, all the 22 electrodes were active, and were mapped in the same frequency range for each channel. RESULTS: Twenty children (83%) showed no VEMPs with the CI 'off'. Among them, 10 elicited VEMPs with the CI 'on', but the other 10 did not. In all channels, the mean C levels of CI were higher in group A than in group B. The p values in channels 1-12 were >0.10, in channels 13-16 were 0.06-0.09, and in channels 17-22 were 0.05-0.06, which were lower but not statistically significant.

Outcomes of cochlear implantations for mumps deafness: A report of four pediatric cases.

Mumps virus occasionally causes bilateral hearing loss. We report 4 cases of bilateral mumps deafness in whom cochlear implantations (CI) were performed. The age at the onset of hearing loss was 1-9 years. CI surgery was performed within 6 months from the onset of hearing loss in 3 cases and after 9 years in the other case, showing good speech perception in the early intervention cases and a poor outcome after later implantation. Early CI surgery is highly recommended in sudden onset deafness by mumps in childhood.

A magnetoencephalographic study of Japanese vowel processing.

Magnetic brain responses were recorded to clarify the cortical representation of vowel processing in Japanese. We investigated the peak latencies and equivalent current dipoles of the auditory N1m responses to the Japanese vowels [a], [i], [o], and [u]. In intraindividual analyses for a single participant, well-replicated results for the dipole parameters supported the existence of phoneme-specific cortical maps for vowels. In the interindividual analyses for the eight participants, [a] and [i] elicited significantly earlier N1m responses than [u], and the dipole for [i] was more posteriorly oriented than [a] in the left hemisphere. The results of the current study suggest left hemispheric predominance in vowel processing and that factors associated with a different language system may modify the cortical map.

Development of auditory skills after cochlear implantation in children with inner ear malformations.

CONCLUSIONS: CI improves hearing thresholds and auditory skills in children with most types of inner ear malformations. However, the development of sound detection skills is not as good as it is in children without inner ear malformations. OBJECTIVES: To investigate the influence of inner ear malformations on development of auditory skills after cochlear implantation (CI). METHODS: Records of 20 children with inner ear malformations who underwent cochlear implantation before 4 years of age and followed up for more than 2 years were retrospectively reviewed. Hearing thresholds, the Meaningful Auditory Integration Scale (MAIS), and Meaningful Use of Speech Scale (MUSS) scores before and after CI were analyzed and compared with 20 age-matched deaf children who underwent CI. RESULTS: The children with inner ear malformations showed significant improvements in hearing thresholds and the MAIS and MUSS scores 1 year after CI (p < 0.01). However, their development of the MAIS scores was significantly delayed compared to children without inner ear malformations (p < 0.05), while there was no significant difference in development of the MUSS scores between them. Significant improvements in hearing thresholds and the MAIS and MUSS scores were observed in cochlear hypoplasia and incomplete partition (p < 0.05).

Environmental sound perception in adult patients with cochlear implants: a comparison with central auditory disorders.

The aim of this study is to investigate the perception mechanism of environmental sounds in postlingual patients with cochlear implants compared with that in patients with central auditory disorders. Seventeen postlingual patients with cochlear implants were studied; six patients with auditory nerve disease (auditory neuropathy) and ten patients with cortical deafness were selected for the comparison. A taperecorded environmental sound perception test of 24 environmental sounds was carried out. This test is divided into two categories: the category of voice includes human voice, animal and bird sounds, and the category of non-voice includes musical instrument sound, natural sound and artificial sound. The percentage of correct perception of environmental sounds in postlingually deaf patients with cochlear implants was markedly higher than that in patients with cortical deafness, but was similar to that in patients with auditory nerve disease (auditory neuropathy).