From October 2017 to March 2020, we reviewed 29 suspected cases of hearing loss or speech and language disorders as a result of the pre-school otorhinolaryngological checkup, including the diagnoses, treatments and managements provided for school attendance. Eight cases were suspected of bilateral hearing loss; the final diagnoses in five of the eight cases were not particular, two cases had conductive hearing loss due to otitis media with effusion, and one case had bilateral sensorineural hearing loss in high frequency. Seventeen cases were suspected of unilateral hearing loss, three of the 17 were not particular, three had unilateral conductive hearing loss due to otitis media with effusion, seven had unilateral sensorineural hearing loss, and four had bilateral sensorineural hearing loss. Three of the 7 cases with unilateral hearing loss had inner ear malformations. Two of the 4 bilateral hearing loss cases who were suspected of having unilateral hearing loss required hearing aids.

Two of the five suspected speech-language disorders were developmental dysarthria, and the others were lateralized dysarthria, pediatric vocal cord nodules, and delayed language.

As a result of this study, the hearing test at the pre-school otorhinolaryngological checkup was considered to be significant in screening for diseases that may interfere with school life and in preparing support for schooling. However, since the period between the pre-school checkup and the start of school is only about half a year, it was considered important to transfer and collaborate with the language class at the elementary school.

Cartilage conduction (CC) hearing aids are the innovated devices launched commercially in Japan since 2017, and have become a beneficial option of hearing intervention, particularly to the atretic ear. Unfortunately, their price is not reasonable, which is the same price as middle-range to high-end models of conventional air conduction hearing aids, and public supports are desirable in the introduction of them. This study surveyed the situation of public support to purchase of CC hearing aids in the patients aged 20 years or younger. Sixty-six patients were enrolled in this study, and purchase rate was totally 85%. Twenty-seven patients suffered bilateral hearing deficits. Sixteen patients of them had been officially recognized as severe auditory handicapped people, and CC hearing aids were publicly provided as an exception in almost all of them. In the other patients, approximately half of them obtained partial support through the subsidies for the children with mild to moderate hearing deficit. In 39 patients with unilateral hearing deficit, most of them purchased CC hearing aids without any subsidy. Some patients without any subsidies gave up the purchase despite good performance of hearing aids. These public supports involved regional disparities. Further public supports and the reduction of the disparities are needed in the future.

Pediatric larynx represents smaller size compared with adult larynx, furthermore, its size changes along with the growth and maturation of the body. Thus, precise understanding of laryngeal size is important for the proper management of pediatric airway. In this study, for the better understanding of laryngeal morphology in children, the length of the vocal folds and the membranous vocal folds were calculated based on the outer diameter of the endotracheal tubes by using the intraoperative laryngeal images taken during microlaryngeal surgery under general anesthesia. Furthermore, values of the length of the vocal folds and the membranous vocal folds were compared with the age, height and weight of the patients to evaluate the relationships between these parameters.

Images of 6 patients during 8 surgeries were incorporated in this study. The age of the patients ranged from 1 month to 66 months old. Values of the length of the vocal folds and membranous vocal folds were 3.88 mm to 8.83 mm, and 3.32 mm to 6.07 mm, respectively. The simple regression analyses and further multiple regression analyses revealed that the age was an explanatory variable for estimating the length of the vocal folds and membranous vocal folds in pediatric larynges.

Our study supported the idea that the age of the pediatric patient could be the significant parameter to estimate the size of the pediatric larynx, and to suggest the appropriate size of endotracheal tube to manage the pediatric airway.

Accidents in which children swallow foreign bodies happen every year, including deaths by suffocation. We administered a questionnaire survey to all 62 nursery schools/kindergartens in Tsu City, Mie Prefecture, to investigate the recognition of accidents caused by airway foreign bodies and to present measures to prevent such accidents. We obtained responses from 53 nursery schools/kindergartens (85.5%). Many of these facilities sufficiently understood the possibilities of death in infants due to airway foreign bodies, the symptoms and countermeasures when such an accident occurs, and foods that were at increased risk of becoming lodged in the airway. On the other hand, while all of the facilities understood that dry beans might be dangerous, dry beans were given to the children for the bean-scattering ceremony on the day before the beginning of spring at 17 facilities (32.1%). Furthermore, small tomatoes were cultivated at 8 facilities (15.1%). Only one facility held an awareness workshop for parents. Based on these findings, it was found that knowledge about the risk of airway foreign bodies was not helpful for the prevention of such accidents, and that guidance for the parents was insufficient. In educational activities, information should be provided, and the danger of such accidents should be reinforced, while proposing concrete measures to prevent such accidents.

Otitis media with effusion in children with Down’s syndrome is often difficult to cure and carries a high risk of recurrence. Herein, we report on children with Down’s syndrome who presented with resistant otitis media at the Japanese Red Cross Medical Center between 2006 and 2018. Twenty patients underwent ventilation tube insertion. Ten patients presented with subsequent complications or sequelae (7 ears of otorrhea, 8 ears of tympanic membrane perforation, 1 ear of atelectatic ear, 4 cases of mild conductive hearing loss). After 2015, the hearing aids were selected in accordance with the 2015 Japan guidelines set forth in the directive for children with otitis media to mitigate the side effects of hearing loss and to optimize hearing rehabilitation. From the above findings, we conclude that it is imperative to deftly select cases that carefully need surgical intervention to prevent the occurrence of further complications or sequelae.

The Support Center for Hearing Impaired Children in Ishikawa was established in the Ishikawa Prefecture by volunteers in 2010. The center that provides three one-on-one consultations to parents of children with hearing loss serves as a bridge to connect such children to proper nursing care. The center was established 11 years ago, when 96 children with hearing loss were treated therein. To determine how to best improve the center, I distributed a questionnaire among parents who were introduced to our center between 2010 and 2018 to determine what aspects should be improved in the future. Overall, 50% of the respondents reported that they were satisfied, and 24% were very satisfied. To be precise, the feedback was very good: 11% reported that the center had solved all their problems, and for 49%, the center had solved most of their problems. However, there were a few instances of detachment found between the services provided to parents and their children and the problems that they faced. Recommendations for future improvement include the following. (1) System aspect: to thoroughly report the precise medical availability and request the children’s cooperation. If their cooperation can be obtained, the parents of those who are undergoing medical treatment should also participate in the one-on-one discussion. (2) Medical explanation: to provide an extensive explanation of genetic investigation, cochlear implants, and hearing aids. An explanation template should be provided, and explanations should be given systematically and comprehensively. (3) Description of rehabilitation: if the children’s cooperation can be obtained, the person responsible for nursing should participate in the respective discussion. These issues deserve future consideration.

The periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a non-hereditary autoinflammatory disease usually affecting children below 5 years of age. However, its etiology remains unclear. We present the case of a child with PFAPA syndrome where palatal tonsillectomy successfully managed the symptoms. We searched PubMed for the keywords “PFAPA syndrome” and “tonsillectomy,” and found that 41 out of 49 articles showed the effectiveness of tonsillectomy in such cases.

According to the pediatric tonsillectomy practice guidelines from the United States, some conditions, including PFAPA syndrome, are not included in the watchful waiting criteria.

Based on the literature review, tonsillectomy seems effective in avoiding frequent steroid administration and reducing parental work and life restrictions due to outpatient visits.

External auditory canal cholesteatoma (EACC) is relatively rare disease, especially in children, and it is mostly following the congenital canal stenosis. Primary (idiopathic) EACC without ear canal malformation is considered extremely rare, and therefore very few reports on primary EACC can be seen in the literature. We here report those 3 cases, in one of which differential diagnosis as EACC is difficult from squamous cell carcinoma.

Case 1 was 13-year-old girl presenting with prolonged ear ache and discharge over 2 weeks even after treatment with oral intake and ear solution of antibiotics. Case 2 was 11-year-old girl with mild mental retardation. She had been suffering from ear pruritus in the past 3 months after sea bathing. Case 3 was 6-year-old girl with ear discharge lasting over 2 weeks even though she had been treated with antibiotics.

Every case presented with ear polyp or pathological granulation in their ear canals, so tympanic membranes were hard to be identified. In all 3 case, temporal bone CT scan revealed bone erosion or destruction in ear canals, and lead to a diagnosis as EACC. In all of 3 cases, surgical debridement of pathological skin, smoothing of eroded bone in the osseous canal, and covering with collagen sponge were performed under general anesthesia, and resulted in complete dry ear canal without recurrence during a long follow-up.

However, atypical cells infiltration with severe inflammation made it difficult to obtain differential diagnosis as ear canal cholesteatoma from squamous cell carcinoma histopathologically using intraoperative frozen sections, and 2 stages operation was thereby needed in case 3. Squamous cell carcinoma might be misdiagnosed as EACC, and therefore an earlier surgical exploration of all the pathological skin should be considered for differential diagnosis between those 2 entities.

Myeloid sarcoma (MS) is a myeloid malignant disease characterized by the formation of extramedullary tumors. Tumors can develop at any site, but tumor formation in the nasal sinuses is relatively rare. For MS cases wherein tumors form in the nasal sinuses, the clinical symptoms and endoscopic findings resemble those of nonmalignant diseases (allergic rhinitis and sinusitis), which may delay diagnosis. Delayed diagnosis is associated with a risk of organ damage because of tumor formation, so early diagnosis is critical. In this study, we present a case of pediatric MS with tumor formation in the nasal sinuses. At the time of initial diagnosis, the patient was a five-year-old boy who had been treated for rhinosinusitis by his local physician. As the patient was refractory to treatment and gradually developed exophthalmos, he was referred to our hospital. Endoscopy showed only swelling of the nasal mucosa, but we suspected an early-stage malignant disease based on imaging findings and performed a tissue biopsy in cooperation with a hematologist. We were able to diagnose MS by properly processing the sample. We report our case of MS with a literature review.

Burkitt lymphoma is a high-grade B-cell lymphoma. In children, the symptoms of this disease vary depending on the primary site and include a few systemic symptoms, including fever and weight loss. Malignant lymphoma of the nasal cavity and paranasal sinuses is very rare. Herein, we report two cases of Burkitt lymphoma in the paranasal sinuses in children with rapid onset of visual impairment.

Both cases developed visual impairment and were admitted to the pediatric department and then referred to our department. Computed tomography and magnetic resonance imaging revealed mass lesions in the paranasal sinuses. Biopsies led to diagnosis of Burkitt lymphoma. Chemotherapy was administered and complete remission has been maintained since the end of treatment. However, blindness developed as a complication in case 1. These cases emphasize the need to be aware of the rapid progression of symptoms of Burkitt lymphoma.

Primary ciliary dyskinesia (PCD) causes respiratory distress during the neonatal period. Here we report 2 cases in which PCD was suspected and diagnosed before age 1 year. Case 1 was a 2-month-old girl who had been born at term. Situs inversus and extensive atelectasis were noted on the first day after birth and she was managed in the neonatal intensive care unit. Echocardiography showed no obvious congenital heart disease and there were no signs of rhinitis. At age 2 months, she was referred to our department for genetic testing, which revealed compound heterozygous variants in DNAH5. Case 2 was a 3-month-old girl. She had been born without asphyxia but developed respiratory distress requiring intubation. After extubation on the second day after birth, nasal discharge and sputum gradually increased. Inspiratory wheezing and exacerbation of respiratory depression were observed, and she was reintubated. PCD was suspected because of airway hypersecretion and persistent atelectasis after birth. The PICADAR score was 7, and genetic testing revealed a 27,748-bp deletion in both alleles of DRC1. The diagnosis was made in these 2 cases because the pediatricians in charge suspected PCD due to respiratory distress that persisted from the early neonatal period.

PORD (Cytochrome P450 oxidoreductase deficiency) is a type of congenital adrenal enzyme deficiency. Patients with PORD have skeletal malformation, genital abnormalities, pubertal failure, adrenal dysfunction, and maternal virilization during pregnancy, which can be ascribed to impaired activities of POR-dependent microsomal enzymes.

We previously reported the pediatric case of a 2-year-old boy with PORD who had ossicular malformation and whose hearing level improved after surgery. He visited our hospital because his mother was concerned about delayed language development.

ASSR (Auditory steady-state response) assessment revealed that his hearing levels were 56.3 dBHL on the right side and 82.5 dBHL on the left side. The wave V threshold of the ABR (auditory brainstem response) was 50.0 dBnHL on the right side and 40.0 dBnHL on the left side. The thin deformed long process of the incus on both sides was observed on temporal CT. He started to receive AVT (auditory verbal therapy) because of delayed language development. In addition, right bone conduction hearing was started and his hearing level with a hearing aid was 20 dB. He underwent stapes surgery on the left ear and tympanoplasty on the right ear when he was 5 years old. His hearing level by the right and left ears improved to 30.0 and 31.7 dB, respectively, after ear surgery. Bone conduction hearing, AVT, and surgical treatment were effective in a pediatric case of ossicular malformation with PORD.

We report a case of bilateral orbital complications caused by acute sinusitis. A 12-year-old boy presented with headache, fever, general fatigue, bilateral periorbital swelling, and decreased visual acuity. Computed tomography (CT) and magnetic resonance imaging (MRI) showed a subperiosteal orbital abscess of the left orbit and bilateral orbital cellulitis. The patient was treated with intravenous antibiotics (Sulbactam Sodium, Ampicillin Sodium) for one day. But the fever persisted and the periorbital swelling worsened. We performed bilateral endoscopic sinus surgery and drained the subperiosteal abscess of the left orbit. The fever fell on the next day, bilateral periorbital swelling and visual acuity were improved, and the patient was discharged on postoperative day 14. Follow up CT showed repair of the medial wall of the left orbit and clear paranasal sinuses. Bilateral orbital complications caused by sinusitis is very rare, and the prompt surgical treatment is necessary when conservative antibiotics therapy in not effective.

Epithelioid hemangioma (EH) is classified as a benign vascular tumor that usually presents as a slowly growing nodule on the face or digit of a middle-aged woman. EH is a benign neoplasm and surgical excision is sufficient for its treatment. We report a case of EH in the left nasal cavity of a five-year-old boy who was admitted with recurrent unilateral epistaxis. CT scan showed a mass with a diameter of 15 mm in the left nasal cavity. The lesion was excised endoscopically under general anesthesia. Histopathologic examination showed proliferation of epithelioid cells and immunohistochemical studies with CD34 was strongly positive, thus confirming a vascular origin and diagnosed as EH. No recurrence was seen 2 years after surgery. Complete excision and intensive observation is necessary owing to the risk of recurrence.

Our patient was a 6-years-old girl. She was prenatally diagnosed as congenital high airway obstruction syndrome (CHAOS) by MRI. At 34 weeks’ gestation, ex utero intrapartum treatment (EXIT) procedure with tracheostomy was performed. She was diagnosed as congenital subglottic stenosis Grade III (99% stenosis) by rigid bronchoscopy. We performed double stage partial cricotracheal resection (PCTR). The cricoid cartilage was elliptically shaped. The dome-shaped silicon T-tube was used to maintain the airway expanded after surgery. We changed the dome-shaped T-tube to tracheostomy tube on the 35^th postoperative day and we could successfully decannulate on the 52^nd postoperative day. She has a patent airway and normal development and can speak at 7 years old. PCTR with the dome-shaped T-tube stenting is one of effective and safe procedure for the children with congenital subglottic stenosis.