A primate model animal revealed the inter-species differences and similarities in the subtype specifications of the spiral ganglion neurons.

Type I spiral ganglion neurons are peripheral neurons essential for hearing perception. While they can be subdivided in mice based on characteristic gene expression patterns, detailed examinations of these subtypes in primates and humans are lacking. In this study, we investigated the developmental subtypes of spiral ganglion neurons in the common marmoset (Callithrix jacchus).

Detailed characterization of auditory neuropathy in perrault syndrome with TWNK variants.

Perrault syndrome is an autosomal recessive condition characterized by hearing loss and ovarian failure. Hearing loss in Perrault syndrome has been reported as sensorineural; however, only two cases in a single report have comprehensively investigated hearing in Perrault syndrome with TWNK variant, and the association between this variant and auditory neuropathy has not been established. The proband presented with hearing difficulties and primary amenorrhea.

Effective cochlear implantation for idiopathic hypertrophic pachymeningitis with bilateral profound hearing loss: A case report.

Rationale: Hypertrophic pachymeningitis (HP) is a local or diffuse fibrous thickness of the dura mater of the brain or spinal cord, caused by infection or connective tissue disease. Headache is the most common clinical symptom, followed by various cranial nerve disorders such as visual impairment, diplopia, and hearing loss. HP can be classified into secondary and idiopathic.

A novel intraoperative continuous monitoring method combining dorsal cochlear nucleus action potentials monitoring with auditory nerve test system.

Highly accurate real-time cochlear nerve monitoring to preserve cochlear nerve function is essential for simultaneous cochlear implantation and ipsilateral vestibular schwannoma resection. In the present study, we developed a novel real-time monitoring system that combines dorsal cochlear nucleus action potential monitoring with intracochlear stimulating electrodes (Auditory Nerve Test System, ANTS). We used this system for a case with vestibular schwannoma resection via the translabyrinthine approach.

A Case of Vestibular Schwannoma with Deafness Showing Remarkable Hearing Recovery Following Hearing-Preserving Surgery.

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.

Distribution of macrophages in the developing cochlea of the common marmoset, a primate model animal.

Introduction: Macrophages are essential immune cells in the cochlea that contribute to inflammation, tissue repair, and homeostasis. They also play an important role in local cochlear immunity. The developmental immigration and maturation of macrophages in the cochlea have been investigated and are considered essential for normal hearing acquisition.

Imaging of Temporal Bone Mass Lesions: A Pictorial Review.

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.

Vestibular Schwannoma Surgery with Endoscope-Assisted Retrolabyrinthine Approach under Modified Reinforced Continuous Intraoperative Monitoring for Hearing Preservation: Experience of 33 Cases in a Single Center.

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.

Intratemporal Facial Nerve Schwannomas: A Review of 45 Cases in A Single Center.

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.

Labyrinthine destruction caused by inflammatory pseudotumor of the temporal bone: A report of three cases and review of the literature.

Background: Inflammatory pseudotumors (IPTs) are rare, idiopathic, and inflammatory lesions that are histopathologically benign. Here, we present three cases of labyrinthine destruction caused by an IPT.

Methods: The first patient was a 74-year-old male with a mass lesion extending from the inner ear to the external ear canal.

Early hearing improvement predicts the prognosis of idiopathic sudden sensorineural hearing loss.

Purpose: To determine pre- and post-treatment factors that are useful for predicting the prognosis of hearing improvement in idiopathic sudden sensorineural hearing loss (ISSHL).

Methods: This retrospective study included 332 patients with ISSHL. Patients received intravenous steroid treatment (prednisolone sodium succinate; 120 mg/day followed by dose tapering).