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. Here, we report a case of bilateral progressive profound sensorineural hearing loss diagnosed in a patient with idiopathic HP, where a cochlear implant was effectively used. PATIENT CONCERNS: The patient was a 77-year-old woman. Hearing loss gradually progressed bilaterally, and magnetic resonance imaging showed a space-occupying lesion with a continuous contrast enhancement in the bilateral internal auditory canals, and diffused dural thickening from the middle to the posterior cranial fossa. DIAGNOSES: A trans-labyrinthine biopsy was conducted, and a definite diagnosis of idiopathic HP was made. Thickening of the dura mater in the bilateral internal auditory canals was thought to cause profound hearing loss. INTERVENTIONS AND OUTCOMES: A cochlear implant was implemented 4 months after biopsy, and a favorable hearing response was obtained postoperatively. LESSONS: This is the first report of a cochlear implant in a patient with idiopathic HP. Cochlear implantation was considered a good treatment for profound hearing loss due to idiopathic HP, which provides a reference for patients to receive timely and correct treatment.

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. The monitoring system developed in this study detected highly reliable evoked potentials from the cochlear nerve every two seconds continuously during tumor resection. Near-total tumor resection was achieved, and cochlear implantation was performed successfully after confirming the preservation of cochlear nerve function in a case. The patient's hearing was well compensated by cochlear implantation after surgery. Our novel method continuously achieved real-time monitoring of the cochlear nerve every two seconds during vestibular schwannoma resection. The usefulness of this monitoring system for simultaneous tumor resection and cochlear implantation was demonstrated in the present case. The system developed in this study is compatible with continuous facial nerve monitoring. This highly accurate and novel monitoring method will broaden the number of candidates for this type of surgery in the future.

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. 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.

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. Most of our current knowledge regarding cochlear development is based on rodent models because of the ethical challenges of using human fetal samples for research. However, inter-species differences between rodents and humans have been reported. In this study, we used a primate animal model to investigate the distributions of macrophages in the developing cochlea. The common marmoset (Callithrix jacchus), a small monkey species that inhabits the New World, was used as the model. Methods: We investigated the distribution of macrophages in the developing cochlea of the common marmoset by performing immunohistochemical analyses of cochlear tissue from common marmoset embryos at different development stages. Results: We revealed detailed distribution changes in the macrophages of a primate animal model cochlea. This observation indicates that most of the changes in the general distribution of macrophages were well preserved between rodents and this primate. The distribution changes observed in the common marmoset were also compatible with observations in the human fetus; although, observations in the human fetus are limited. Our observations in this study also revealed several differences between common marmosets and rodents. Conclusion: The time course of immunological development and maturations established in this study will aid in the study of the primate-specific developmental biology of the inner ear. These observations may eventually lead to new therapeutic strategies for hearing loss in humans. In addition, understanding the immunological steady-state of the cochlea may help in the study of age- and genetic-induced hearing loss and in the design of regenerative therapies.

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. 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.

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. 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.

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. 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.

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. The second patient was a 62-year-old female with a foliated polycystic lesion in the petrous bone on the dorsal side of the left internal auditory canal. The third patient was a 68-year-old female with a mass extending from the inner ear to the middle ear, destroying the semicircular canal and cochlea. RESULTS: In two cases, we performed surgical resection successfully with no recurrence. In the other case, the lesion showed shrinkage after chemotherapy for colorectal cancer incidentally found during the examination process. CONCLUSION: Surgical technique and indication for IPT should be based on the location and function of the lesion. In addition, there is room to consider pharmacotherapy as a treatment option for IPT of the temporal bone. LEVEL OF EVIDENCE: 4.

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). Complete recovery of hearing levels was defined as a final pure-tone audiometry of ≤ 20 dB HL or the same level as the contralateral ear. Patients' age; sex; side of hearing loss; initial hearing level; days from onset to treatment; presence of vertigo, diabetes, and hypertension; and hearing improvement on days 3-4 and 6-7 after treatment initiation were analyzed as potential prognostic factors. RESULTS: Overall, 109 patients (32%) had complete recovery. Results of the multivariate logistic regression model identified age (odds ratio [OR] = 0.974), initial hearing level (OR = 0.949), vertigo (OR = 0.409), and hearing improvement on days 6-7 after treatment initiation (OR = 1.11) as significant independent predictors of complete recovery. Age ≥ 60 years, initial hearing level ≥ 72.5 dB HL, and vertigo contributed to poor prognosis. Patients without these three factors and a hearing improvement of ≥ 10 dB HL on days 6-7 post-treatment had a complete recovery rate of 80%. Only 1.5% of the patients with 2-3 of these factors and a hearing improvement of < 10 dB HL on days 6-7 after treatment initiation achieved complete recovery. CONCLUSION: Age, initial hearing level, vertigo, and hearing improvement on days 6-7 after treatment initiation were independent predictors of hearing recovery in ISSHL.