The prevalence of allergic rhinitis, especially that of Japanese cedar pollinosis, has increased in recent years in Japan. Furthermore, spontaneous remission of Japanese cedar pollinosis is rare until old age. Allergen immunotherapy (AIT) is currently considered to be the only effective therapy with the potential to alter the natural course of allergic rhinitis. In many placebo-controlled clinical trials of AIT for allergic rhinitis, significant improvements of symptoms have been observed in the AIT group as compared to the placebo group. Regular allergen administrations over a period of years are needed to obtain stable long-term efficacy of AIT; however, some patients still show poor efficacy. Therefore, if the therapeutic efficacy could be predicted at an early time point of the treatment, the burden of non-responders would be reduced. There are no generally accepted predictive biomarkers until date, however, it would be desirable to identify a biomarker for predicting the therapeutic efficacy of AIT and evaluate its usefulness in clinical settings.

To obtain clues to improve the present status of (re)habilitation for hearing-impaired children in Japan, we visited several hearing rehabilitation centers in Los Angeles between January 6 and January 8, 2020.

The following important findings were obtained.

1) The number of specialists, represented by speech-language pathologists and audiologist, involved in the (re)habilitation of hearing-impaired children was far higher in the United States (US), and this seems to be one of the biggest problems in Japan. To improve this situation, certification of speech therapists (STs) specializing in hearing should be promoted.

2) To provide higher-level auditory-verbal education to hearing-impaired children, training for specialists in auditory-verbal therapy should be promoted in medical schools.

3) The legislated system of free new-born hearing screening in the US is associated with an almost 100% coverage.

4) The legislated educational programs for early interventions in hearing-impaired children in the US, such as Early Intervention Program and Indivisual Education Plan, in which multi-occupational specialists are involved in seamless care of the children, probably represent the biggest difference between our two countries, and appears to be among the biggest causes of problems in (re)habilitation of hearing-impaired children in Japan.

5) Inclusive education, where both normal-hearing and hearing-impaired children are educated in the same class, was another impressive educational measure for hearing-impaired children in the US.

6) A system for deciding on the communication method for each child is operated in an organized manner by multi-occupational specialists, leading to early precise orientation of communication for each child.

7) We are amazed to note that most of those centers and schools are administered financially by donation as well as (re)habilitation fees.

8) For those specialists in the (re)habilitation of hearing-impaired children in Japan such as STs to be evaluated properly, the fees for (re)habilitation by hearing and speech training in Japan should be similar to the fees in the US.

Spontaneous cerebrospinal fluid (CSF) effusion of the temporal bone is extremely rare in adults. Its local findings are similar to those of otitis media with effusion (OME) and the condition is often diagnosed by an intractable serous otorrhea that persists after paracentesis of the tympanic membrane (TM). We report two cases of spontaneous CSF effusion of the temporal bone which showed different clinical courses. Both patients presented with sudden aural fullness, without any trigger. They were initially diagnosed as having OME, however, pulsatile serous otorrhea persisted even after paracentesis of the TM/tube insertion. Temporal bone CT showed bone defects of the middle cranial fossa in both cases. The first case had a large amount of glucose-positive otorrhea, which led to the diagnosis of CSF effusion. Gauze packing of the external auditory meatus closed the perforation of the TM, the route of leakage of the CSF. Two months later, the hearing recovered, with no CSF leak, and four months later, the soft tissue density on the CT disappeared. In contrast, the second case showed just a small amount of otorrhea, which was glucose-negative. After removal of the ventilation tube, the perforation of the TM closed. However, pulsation of the TM and conductive hearing loss persisted. We performed a diagnostic/therapeutic operation seven months after the onset. During surgery, we found intermittent pulsatile liquid outflow from the middle cranial fossa, on the basis of which we made the diagnosis of spontaneous CSF effusion of the temporal bone. The leakage area confirmed by staining was covered by fascia, bone pate, and bone chips. Until now, 12 months after the surgery, there has been no recurrence. Adult-onset spontaneous CSF leakage may be cured by conservative treatments, as observed in case 1. As an initial treatment of spontaneous CSF effusion of the temporal bone, we propose removal of the ventilation tube, if in place, and/or the packing of the ear canal to close the CSF outflow route.

Glomangiopericytoma is a rare tumor entity that arises from the sinonasal tract, and accounts for less than 0.5% of all sinonasal tumors. This tumor originates from the capillary pericytes, and generally exhibits borderline to low-grade malignant biological characteristics. Herein, we report a case of glomangiopericytoma of the nasal cavity that showed positive accumulation on ¹⁸F-fluorodeoxyglucose-positron emission tomography (FDG-PET).

An 85-year-old male patient presented with a 1-month history of repeated right-sided epistaxis. Physical and endoscopic examinations revealed a soft, reddish, hemorrhagic mass in the olfactory cleft of the right nasal cavity. Computed tomography visualized a well-defined, moderately-enhanced mass in the upper posterior part of the right nasal cavity. The lesion showed positive accumulation on FDG-PET, with an SUVmax of 6.12. Biopsy of the mass was performed, and the histopathological diagnosis was glomangiopericytoma with borderline malignancy. The patient was treated by transnasal endoscopic resection of the tumor. The intraoperative blood loss was 50 ml. The postoperative clinical course was uneventful, and the patient was discharged 6 days after surgery. He has been free from disease for 10 months since the surgery.

Positive accumulation on FDG-PET has been documented in several previous case reports of glomangiopericytoma. Physicians should be aware that glomangiopericytoma could exhibit accumulation on FDG-PET despite showing only borderline to low-grade malignant biological properties.

Tonsilloliths are often observed in general clinical practice, and cases of giant tonsilloliths have been reported. To the best of our knowledge, however, cases with the formation of a giant tonsillolith after having undergone tonsillectomy are very rare.

A dentist detected a calcific lesion in the X-ray of a 65-year old female patient. The patient was referred to the Department of Otolaryngology of a nearby hospital. A cervical CT at this hospital revealed a relatively giant tonsillolith, measuring 20 mm in maximum diameter. A lithectomy to remove the left tonsillar stone was performed under general anesthesia. The tonsillar stone was removed in two pieces. Over 95% of the stone was calcium phosphate. Grocott’s staining led to the suspicion of fungal involvement. We theorize that the core of the stone was formed due to inflammation of the residual tonsil after tonsillectomy; thereafter, smoking and other chronic stimuli let to the growth of the stone into a giant tonsillolith.

Between July 2017 and June 2019, we attempted observation of the larynx with a flexible laryngeal endoscope inserted transorally, and classified the visualizable range of the larynx by the modified Cormack classification (Observation score). We also evaluated the visualizable range of the pharynx by the modified Mallampati score (Spatial score) and the strength of the pharyngeal reflex by the modified Kisa score (Reflex score). The number of cases in which the vocal cords could be visualized in their entirety was 216 (64.7%), with the posterior part of the vocal cords alone visualized in 19 cases (5.7%) and the epiglottis alone visualized in 51 cases (15.3%). The relationships between the Spatial score and Observation score were as follows: the entire vocal cords could be visualized in 73 of the 96 cases (76.0%) with a Spatial score of 1, 63 of the 92 cases with a Spatial score of 2 (68.5%), 54 of the 99 cases (54.5%) with a Spatial score of 3, and 26 of the 47 cases with a Spatial score of 4; the differences were not significant (P>0.01). The relationships between the Reflex score and the Observation score were as follows: the entire vocal cords could be visualized in 164 of the 190 cases (86.3%) with a Reflex score of 1, 42 of the 64 cases (65.6%) with a Reflex score of 2, 6 of the 26 cases (23.1%) with a Reflex score of 3, and 4 of the 54 cases (7.4%) with a Reflex score of 4; the differences were significant (P<0.01). By using the Spatial scores and Reflex scores, it would seem possible to predict extent of visualization of the larynx with a flexible laryngeal endoscope inserted transorally.

Peripheral nerve sheath tumors (PNSTs) can arise from almost any nerve in the cervix, and these tumors are mostly benign. In clinical practice, diagnosis of PNSTs at the outpatient department is difficult, therefore, surgical excision, including tumor enucleation, is the first-line treatment. However, surgical excision itself can also cause neurological complications by damaging the nerve from which the PNSTs arise. Smouha has proposed a conservative treatment algorithm for schwannomas of the acoustic nerve. However, there are no reports of such an algorithm for cervical PNSTs.

8 patients suspected by MRI as having cervical PNSTs were followed-up without surgical treatment at our institution between October 1, 2018, and September 30, 2019. Among 23 patients with cervical PNSTs who had finally undergone tumor resection since 2000, only one patient had been initially followed up for more than 12 months. There were 5 females and 4 males, and the average age was 50 years (range, 36–74 years). All of them presented at first with a neck mass, and none showed any neurological manifestations. The median follow-up duration was 30 months (range, 6–224 months). The median estimated tumor volume was 12.1 cm³ (range 5.0–54.9 cm³). In 8 patients, the tumors were located in the parapharyngeal space and suspected as arising from the vagus nerve or the sympathetic trunk, and in the remaining 1 patient, it was suspected as arising from the C5 trunk. The latest MRI showed an increase of the estimated tumor volume in 2 cases (by more than 120% of the volume in the initial image), a stable tumor volume in 5 cases (80%–120%), and a reduction of the tumor volume in 2 cases (<80%). Of the 9 patients, 8 remained asymptomatic. These results, in spite of the limited number of patients, suggest that a wait-and-see approach could be adopted for some patients with suspected cervical PNSTs. A treatment algorithm for cervical PNSTs including conservative management is expected to be established.

Metastatic carcinoma of the thyroid gland is unusual and is very rarely associated with thyroid dysfunction. We encountered a 51-year-old male lung adenocarcinoma patient with thyroid metastasis who presented with the features of hyperthyroidism. The patient presented with a swelling in the anterior neck and difficulty in swallowing. Clinical examination revealed a diffuse painless swelling of the thyroid gland, and blood tests showed evidence of hyperthyroidism; tests for thyroid autoantibodies were negative. Computed tomography (CT) revealed the diffuse swelling of the thyroid gland, bilateral neck lymphadenopathy, multiple nodules in the lungs, and infiltrative opacities in the right lower lobe, with right pleural effusion. Magnetic resonance imaging (MRI) showed multiple metastatic lesions in the brain. Fine needle aspiration cytology from the thyroid gland and cervical lymph nodes revealed Class V cytology, suspicious for adenocarcinoma. Transbronchial biopsy of a lesion in the right lung revealed lung adenocarcinoma (cT4N3M1c stage IV). Genetic analysis revealed an exon 19 deletion mutation of the epidermal growth factor receptor (EGFR) gene. Treatment with oral osimertinib (80 mg/day), a third-generation EGFR-tyrosine kinase inhibitor (TKI), was very effective. The primary and metastatic lesions were well-controlled until the end of the one-year follow-up. The hyperthyroidism was reversed within a month after the start of osimertinib treatment, but the patient eventually developed hypothyroidism, needing treatment with oral levothyroxine, 100 μg/day.

We present a case of metastatic carcinoma of the thyroid gland in a patient with lung adenocarcinoma, who presented with features of hyperthyroidism. Treatment with an EGFR-TKI was effective, eliciting partial remission in the patient. Thyroid metastasis is often associated with a poor prognosis, therefore, continued strict follow-up of the patient is necessary.

Descending necrotizing mediastinitis (DNM) can occasionally complicate a deep neck infection, and has a high mortality rate. Thus, rapid diagnosis and prompt surgical intervention are essential. We encountered a rare case of DNM with the formation of a tracheomediastinal fistula. Tracheomediastinal fistula is a potentially life-threatening complication. To the best of our knowledge, only four reported cases have been successfully saved. Our patient was a 79-year-old male patient who presented with the chief complaint of dyspnea. CT imaging showed a retropharyngeal abscess with gas extending widely over the mediastinum. We performed transcervical and transthoracic drainage, combined with antimicrobial therapy. After the drainage operation, bronchoscopy revealed a tracheomediastinal fistula. Due to the relatively good respiratory condition, we selected conservative treatments, such as fibrin glue sealing and saline irrigation via a drain. As the patient developed the complication of methicillin-resistant Staphylococcus aureus (MRSA) bacteremia and pyogenic discitis between the fourth and fifth thoracic vertebrae during the course of treatment, we administered anti-MRSA antibiotics and performed another drainage surgery. The patient responded to these treatments, with eventual cure and closure of the tracheomediastinal fistula.

Magnetic resonance (MR)-based volumetric measurement of the endolymphatic space (ELS) was conducted in patients with acute low-tone sensorineural hearing loss (ALHL), sudden deafness (SD), cochlear Meniere’s disease (cMD), and unilateral MD (uMD), and the results were compared with those in control subjects (CS). Seventy-nine patients with ALHL, 99 with SD, 65 with cMD, 77 with uMD, and 47 CS participated in this study. With the exception of the patients with uMD, none of the subjects had vertigo. Images of the inner ear fluid space, positive perilymph and positive endolymph signal were acquired using a 3-T MR imaging scanner. Three-dimensional images were reconstructed semi-automatically by using anatomical and tissue information to fuse the inner ear fluid space images with the ELS images. The cochlear and vestibular ELS/total fluid space (TFS) volume ratios were 10.2%±6.8% and 17.7%±10.2%, respectively. The cochlear ELS/TFS volume ratio in patients with uMD was significantly higher than that in the CS, and patients with ALHL, SD, and cMD (CS=ALHL<SD<cMD<uMD). The vestibular ELS/TFS volume ratio in patients with uMD was significantly higher than that in the CS and other patients groups (CS=ALHL=SD<cMD<uMD). The cochlear and vestibular ELS volume ratio in patients with uMD and other endolymphatic hydrops-related diseases differed from that in the CS. These results suggest that assessment of the prognosis is possible by MR evaluation in patients with endolymphatic hydrops-related inner ear diseases.