Noise-induced hearing loss (NIHL) is a common type of sensorineural hearing loss caused by exposure to high-intensity noise that leads to irreversible cochlear damage. Despite extensive research on cochlear pathophysiology, the precise mechanisms remain unclear, and no established treatment exists. This is due to the challenges in imaging and the inability to perform biopsies in human patients. Consequently, animal models, particularly mice, have been widely used to study NIHL. Clinically, NIHL presents as either a temporary threshold shift, in which hearing recovers, or a permanent threshold shift, which results in an irreversible loss. Histopathological studies have identified the key features of NIHL, including outer hair cell loss, auditory nerve degeneration, and synaptic impairment. Recent findings suggest that oxidative stress and inflammation are major contributors to NIHL, highlighting the potential for therapeutic interventions, such as antioxidants and anti-inflammatory agents. Given the increasing prevalence of NIHL owing to occupational noise exposure and personal audio device use, addressing this issue is a pressing public health challenge. This review summarizes the clinical features, underlying mechanisms, and emerging treatment strategies for NIHL while identifying current knowledge gaps and future research directions.

There are no established indications for facial nerve schwannoma treatment, including surgery, radiation and follow-up observation, and it is difficult to determine treatment policy uniformly. The treatment policy was examined from each treatment course. Data of patients with facial nerve schwannomas at our hospital from 1987 to 2018 were retrospectively examined. Their age, sex, clinical symptoms, tumor localization, treatment policies and outcomes were reviewed. In total, 22 patients underwent surgery and 1 patient underwent radiotherapy; 22 patients were followed up without treatment. After total resection, there were no tumor recurrences, and most patients had grade 3 or 4 postoperative facial paralysis. After subtotal resection, tumor regrowth was observed in four patients and reoperation was required in two patients. Facial nerve function was maintained in four patients and was decreased in two patients. During follow-up, six patients showed tumor growth. Only one patient had worsening facial nerve paralysis; four patients underwent facial nerve decompression owing to facial nerve paralysis during follow-up. If the tumor compresses the brain or it is prone to growth, surgery may be indicated, and when the preoperative facial nerve function is grade ≤ 3, consideration should be given to preserving facial nerve function and subtotal resection should be indicated. If the preoperative facial nerve function is grade ≥ 3, total resection with nerve grafting is an option to prevent regrowth. If there is no brain compression or tumor growth, the follow-up is a good indication, and decompression should be considered in facial nerve paralysis cases.

Surgery for vestibular schwannoma includes various techniques such as the middle cranial fossa, suboccipital, translabyrinthine, and retrolabyrinthine approaches. The retrolabyrinthine approach does not impair the labyrinth and allows postoperative hearing preservation. Previously, we reported an endoscope-assisted retrolabyrinthine approach under reinforced continuous intraoperative monitoring for preservation of hearing and facial nerve function. However, the hearing preservation rate was relatively low in patients with long-wave V latency in the auditory brainstem response or poor otoacoustic emission response under this approach. Thus, the hearing preservation rate in such patients remains to be improved. To overcome this limitation, we modified the previous approach. In 26 of the 33 consecutive cases (79%) over the last three years, preservation of hearing equivalent to that before surgery or improved hearing was achieved. Postoperative deafness was observed in only two of the 33 cases (6%). According to previous reports, the rate of hearing preservation using the retrolabyrinthine approach is approximately 30–50%. Therefore, we have achieved a higher hearing preservation rate than that previously reported using the endoscopy-assisted retrolabyrinthine approach under reinforced continuous intraoperative monitoring. These improvements in our department are considered to be helpful for hearing preservation.

Tumoral lesions of the temporal bone include benign or malignant tumors and congenital or inflammatory lesions. Temporal bone lesions are difficult to approach. Therefore, making a preoperative diagnosis and considering whether the lesions require treatment are necessary; if they require treatment, then the type of treatment requires consideration. These tumors cannot be observed directly and must be diagnosed based on symptoms and imaging findings. However, the differentiation of temporal bone lesions is difficult because they are rare and large in variety. In this pictorial review, we divided temporal bone lesions by location such as along the facial nerve, along the internal jugular vein, around the endolymphatic sac, in the internal auditory canal/cerebellopontine angle, petrous apex, middle ear, and mastoid, focusing on the imaging findings of temporal bone lesions. Then, we created a diagnostic flowchart that suggested that the systematic separation of imaging findings is useful for differentiation. Although it is necessary to make comprehensive judgments based on the clinical symptoms, patient background, and imaging findings to diagnose temporal bone mass lesions, capturing imaging features can be a useful differentiation method.

Cartilage conduction hearing aids (CC-HA), bone anchored hearing aids (Baha), and ADHEAR are good choices to improve hearing in patients who cannot use air conduction hearing aids because of aural atresia or continuous otorrhea. As there are many overlaps in the characteristics of these devices, we conducted a comparative trial of CC-HA, Baha, and ADHEAR. We provided patients with the opportunity to select their devices. The data of 6 patients who underwent comparative trials in our department between October 2021 and August 2022 were retrospectively examined. The gains of Baha and CC-HA outweighed those of ADHEAR. Regarding the sound localization test, there was no significant tendency for any of the hearing devices. Regarding the Glasgow Benefit Inventory, there was no clear tendency among the three devices in the quality of life evaluation. The most satisfactory model was the one subjectively chosen by the patient, regardless of the gain and score of the sound source localization test. Therefore, we believe that it is necessary to provide an opportunity for comparative trials and a consultation with each patient in the process of selecting a device.

Surgery for vestibular schwannoma can be divided into hearing-preserving and nonhearing-preserving surgeries. Hearing-preserving surgery is usually not considered in patients with deafness due to vestibular schwannoma, because hearing is unlikely to improve, and surgery aims to maximize the tumor resection at the expense of hearing. We report an extremely rare case of a 46-year-old man with unilateral profound hearing loss due to a vestibular schwannoma with marked cystic degeneration in the left cistern, which significantly recovered to near-normal hearing levels after hearing-preserving surgery. Hearing loss gradually worsened, and preoperative pure-tone evaluation showed complete hearing loss in the left ear. However, the response to the distortion product otoacoustic emission was preserved, and hearing loss was considered to be retrocochlear. Tumor resection was performed using the retrolabyrinthine approach with continuous monitoring using dorsal cochlear nucleus action potential, auditory brainstem response, and facial nerve function muscle action potential. The cistern portion of the tumor was almost completely resected along with the wall. Postoperatively, the pure-tone threshold on the left side markedly improved. The present case clearly demonstrates the possibility of hearing recovery in patients with retrocochlear hearing loss. We should consider expanding the indications for hearing-preserving surgery. Cite this article as: Sakaki Y, Hosoya M, Nishiyama T, et al. A case of vestibular schwannoma with deafness showing remarkable hearing recovery following hearing-preserving surgery. J Int Adv Otol. 2023;19(5):426-430.