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.

CalibrationPhys: Self-Supervised Video-Based Heart and Respiratory Rate Measurements by Calibrating Between Multiple Cameras.

Video-based heart and respiratory rate measurements using facial videos are more useful and user-friendly than traditional contact-based sensors. However, most of the current deep learning approaches require ground-truth pulse and respiratory waves for model training, which are expensive to collect. In this paper, we propose CalibrationPhys, a self-supervised video-based heart and respiratory rate measurement method that calibrates between multiple cameras. CalibrationPhys trains deep learning models without supervised labels by using facial videos captured simultaneously by multiple cameras. Contrastive learning is performed so that the pulse and respiratory waves predicted from the synchronized videos using multiple cameras are positive and those from different videos are negative. CalibrationPhys also improves the robustness of the models by means of a data augmentation technique and successfully leverages a pre-trained model for a particular camera. Experimental results utilizing two datasets demonstrate that CalibrationPhys outperforms state-of-the-art heart and respiratory rate measurement methods. Since we optimize camera-specific models using only videos from multiple cameras, our approach makes it easy to use arbitrary cameras for heart and respiratory rate measurements.

Zero-Shot Neural Decoding with Semi-Supervised Multi-View Embedding.

Zero-shot neural decoding aims to decode image categories, which were not previously trained, from functional magnetic resonance imaging (fMRI) activity evoked when a person views images. However, having insufficient training data due to the difficulty in collecting fMRI data causes poor generalization capability. Thus, models suffer from the projection domain shift problem when novel target categories are decoded. In this paper, we propose a zero-shot neural decoding approach with semi-supervised multi-view embedding. We introduce the semi-supervised approach that utilizes additional images related to the target categories without fMRI activity patterns. Furthermore, we project fMRI activity patterns into a multi-view embedding space, i.e., visual and semantic feature spaces of viewed images to effectively exploit the complementary information. We define several source and target groups whose image categories are very different and verify the zero-shot neural decoding performance. The experimental results demonstrate that the proposed approach rectifies the projection domain shift problem and outperforms existing methods.

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.

Edema Estimation From Facial Images Taken Before and After Dialysis Via Contrastive Multi-Patient Pre-Training.

Edema is a common symptom of kidney disease, and quantitative measurement of edema is desired. This paper presents a method to estimate the degree of edema from facial images taken before and after dialysis of renal failure patients. As tasks to estimate the degree of edema, we perform pre- and post-dialysis classification and body weight prediction. We develop a multi-patient pre-training framework for acquiring knowledge of edema and transfer the pre-trained model to a model for each patient. For effective pre-training, we propose a novel contrastive representation learning, called weight-aware supervised momentum contrast (WeightSupMoCo). WeightSupMoCo aims to make feature representations of facial images closer in similarity of patient weight when the pre- and post-dialysis labels are the same. Experimental results show that our pre-training approach improves the accuracy of pre- and post-dialysis classification by 15.1% and reduces the mean absolute error of weight prediction by 0.243 kg compared with training from scratch. The proposed method accurately estimate the degree of edema from facial images; our edema estimation system could thus be beneficial to dialysis patients.

Development and validation of an iPad-based Japanese language monosyllable speech perception test (iCI2004 monosyllable).

BACKGROUND: Speech perception tests are commonly used as indices reflecting hearing ability in daily life. In Japan, the CI-2004 test, first developed in 2004, is widely used as standard, but it was not validated against a large number of normal hearing controls and hearing loss patients. AIMS/OBJECTIVES: The primary objective of the present study was to develop and validate iPad-based software for the Japanese monosyllable speech perception test, 'iCI2004'. MATERIAL AND METHODS: Seven universities and two medical centers participated in this study. The hearing threshold and Japanese monosyllable speech perception test results of 77 people with normal hearing and 459 people with hearing loss were collected. RESULTS: All participants with normal hearing achieved almost perfect perception results both in quiet and in noise. For cochlear implant users, the average monosyllable speech perception score was 55.1 ± 19.6% in quiet and 40.3 ± 19.2% in noise (SNR + 10dB). CONCLUSIONS AND SIGNIFICANCE: We developed iPad-based Japanese monosyllable speech perception test software and validated it by testing a large number of controls and hearing loss patients with cochlear implants or hearing aids. The developed monosyllable speech perception test has a sufficiently large dynamic range for assessing improvement in speech perception in Japanese cochlear implant users.

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.

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).

Vestibular evoked myogenic potentials of children with inner ear malformations before and after cochlear implantation.

CONCLUSIONS: Our results show that among the patients with inner ear malformations, there were two patients with sensory cells of sacculus and inferior vestibular neurons and at least five patients with inferior vestibular neurons only, but no patients without sensory cells and vestibular neurons, as determined on the basis of vestibular evoked myogenic potentials (VEMPs). OBJECTIVE: The aim of this study was to investigate whether sensory cells of sacculus or inferior vestibular neurons or both are present in cochlear implant patients with inner ear malformations, particularly common cavity (CC) deformity, using VEMPs. PATIENTS AND METHODS: Seven children with inner ear malformation who underwent cochlear implantation (CI) participated in this study. The patients had CC deformity (n=2), incomplete partition type I (n=2), incomplete partition type II (n=1), or a narrow internal auditory canal (IAC) (n=2). It was possible to record VEMPs before and after CI in three patients and not before but after CI in four patients. After surgery, VEMPs were recorded with the cochlear implant device switched both off and on. RESULTS: Before the CI, two patients showed VEMPs and one patient showed no VEMPs, whereas in four patients no VEMPs could be recorded. All the patients showed VEMPs with the cochlear implant on.

Spontaneous otoacoustic emissions in two infants.

CONCLUSION: We regard spontaneous otoacoustic emission (SOAE) as a unique phenomenon due to spontaneous outer hair cell hyperactivity during the infantile period in these two infants. OBJECTIVES: We report the cases of SOAEs in two infants, which were audible to their parents. We were able to hear continuous sounds from these two infants' ears. We studied their hearing and the emitted sounds objectively. PATIENTS AND METHODS: Patient 1 was a 5-month-old girl and patient 2 was an 11-month-old girl. Their parents heard continuous sounds emanating from their infants' ears. Audiological examinations and frequency analysis were conducted. RESULTS: The distortion-product otoacoustic emissions (DPOAEs) and auditory brainstem responses (ABRs) of the two patients were normal. Analysis of the continuous sounds demonstrated frequencies of 4.2 and 6.5 kHz for patient 1 and 6.7 kHz for patient 2. These audible sounds were diagnosed as SOAEs. The SOAEs in these infants did not disappear with their development in the last 2 years.

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.

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).