Sex steroid action is critical to form sexually dimorphic nuclei, although it is not fully understood. We previously reported that masculinization of the principal nucleus of the bed nucleus of the stria terminalis (BNSTp), which is larger and has more neurons in males than in females, involves aromatized testosterone that acts via estrogen receptor-α (ERα), but not estrogen receptor-β (ERβ). Here, we examined sex steroid action on the formation of the anteroventral periventricular nucleus (AVPV) that is larger and has more neurons in females. Morphometrical analysis of transgenic mice lacking aromatase, ERα, or ERβ genes revealed that the volume and neuron number of the male AVPV were significantly increased by deletion of aromatase and ERα genes, but not the ERβ gene. We further examined the AVPV and BNSTp of androgen receptor knockout (ARKO) mice. The volume and neuron number of the male BNSTp were smaller in ARKO mice than those in wild-type mice, while no significant effect of ARKO was found on the AVPV and female BNSTp. We also examined aromatase, ERα, and AR mRNA levels in the AVPV and BNSTp of wild-type and ARKO mice on embryonic day (ED) 18 and postnatal day (PD) 4. AR mRNA in the BNSTp and AVPV of wild-type mice was not expressed on ED18 and emerged on PD4. In the AVPV, the aromatase mRNA level was higher on ED18, although the ERα mRNA level was higher on PD4 without any effect of AR gene deletion. Aromatase and ERα mRNA levels in the male BNSTp were significantly increased on PD4 by AR gene deletion. These results suggest that estradiol signaling via ERα during the perinatal period and testosterone signaling via AR during the postnatal period are required for masculinization of the BNSTp, whereas the former is sufficient to defeminize the AVPV.

Cartilage conduction (CC) is a form of conduction that allows a relatively loud sound to be audible when a transducer is placed on the aural cartilage. The CC transmission mechanism has gradually been elucidated, allowing for the development of CC hearing aids (CC-HAs), which are clinically available in Japan. However, CC is still not fully understood. This review summarizes previous CC reports to facilitate its understanding. Concerning the transmission mechanism, the sound pressure level in the ear canal was found to increase when the transducer was attached to the aural cartilage, compared to an unattached condition. Further, inserting an earplug and injecting water into the ear canal shifted the CC threshold, indicating the considerable influence of cartilage–air conduction on the transmission. In CC, the aural cartilage resembles the movable plate of a vibration speaker. This unique transduction mechanism is responsible for the CC characteristics. In terms of clinical applications, CC-HAs are a good option for patients with aural atresia, despite inferior signal transmission compared to bone conduction in bony atretic ears. The advantages of CC, namely comfort, stable fixation, esthetics, and non-invasiveness, facilitate its clinical use.

Severe conductive hearing loss due to unilateral aural atresia leads to auditory and developmental disorders, such as difficulty in hearing in challenging situations. Bone conduction devices compensate for the disability but unfortunately have several disadvantages. The aim of this study was to evaluate the benefits of cartilage conduction (CC) hearing aids for speech perception in unilateral aural atresia. Eleven patients with unilateral aural atresia were included. Each participant used a CC hearing aid in the atretic ear. Speech recognition scores in the binaural hearing condition were obtained at low speech levels to evaluate the contribution of aided atretic ears to speech perception. Speech recognition scores were also obtained with and without presentation of noise. These assessments were compared between the unaided and aided atretic ear conditions. Speech recognition scores at low speech levels were significantly improved under the aided atretic ear condition (p < 0.05). A CC hearing aid in the unilateral atretic ear did not significantly improve the speech recognition score in a symmetrical noise presentation condition. The binaural hearing benefits of CC hearing aids in unilateral aural atresia were predominantly considered a diotic summation. Other benefits of binaural hearing remain to be investigated.

Various prognostic factors for idiopathic sudden sensorineural hearing loss (SSNHL) have been reported. Hearing loss directly derived from idiopathic SSNHL is important for understanding underlying pathogenesis and outcomes. We assessed the usefulness of evaluating hearing loss and recovery of idiopathic SSNHL on the basis of estimated hearing loss. The study included 115 patients whose characteristics and outcomes of hearing loss were investigated. The effects of vertigo/dizziness and age on hearing thresholds before/after treatment, nonaffected ear threshold, estimated hearing loss, improvement of hearing loss, and estimated remaining hearing loss were investigated. Vertigo/dizziness was a significant prognostic factor for hearing. In vertigo/dizziness patients, significantly more severe hearing loss and poorer improvement of hearing loss were observed above 500 Hz and below 1000 Hz, respectively. Severe hearing disorder remained at all frequencies. Conversely, post-treatment thresholds were significantly higher in the older population (≥65 years), although no differences in pretreatment thresholds were observed between the younger (≤64 years) and older populations. However, on the basis of nonaffected ear thresholds, previously existing hearing loss could have influenced the outcome. Thus, comparison of hearing outcomes between affected and nonaffected ears is essential for understanding hearing loss and outcomes in idiopathic SSNHL cases with existing hearing disorder.

The ear molds and shells of hearing devices are generally designed using ear impressions. Computed tomography (CT) can provide information concerning ear structure, which can be utilized in computer-aided design to create hearing devices. This study aimed to compare the differences in hearing devices designed using either ear impressions or CT images. The ear chip of a cartilage conduction (CC) hearing device was designed based on the ear impression and CT images of patients with aural atresia. Three patients with bilateral (n = 1) or unilateral (n = 2) aural atresia participated in this study. The aided thresholds and subjective evaluations were compared. There were no serious problems with the use of either device, and no remarkable differences were observed regarding their respective audiological and subjective benefits. Regarding the subjective patient preferences, the hearing aid with the device designed via CT imaging was better in three ears of two patients, whereas the wearing comfort of the transducer was better for the device designed via the ear impression. The subjective evaluation of appearance varied among the cases. Finally, a device that was designed using CT findings was purchased for one patient. CT images can be utilized in computer-aided design to create CC hearing devices.