In Osaka Prefecture, the detailed examination system after newborn hearing screening has been a two-stage system from beginning. 1966 children who visited detailed inspection institutions from January 2013 to December 2017 were investigated and analyzed for their hearing level and the status of hearing aid intervention. There were 58 bilateral hearing loss, 429 unilateral hearing loss, 734 bilateral normal and 45 unknown. There were 352 children with hearing loss of 50 dB or more, for which hearing aid intervention was indicated, representing 0.1% of births. The two-stage system was underutilized but it had merits. The rate of deafness in the detailed examination was higher in referrals through ABR hospitals than in direct referrals from screening institutions. On the other hand, there were more than a few cases in which hearing aid intervention was not performed despite the diagnosis of bilateral hearing loss. Comparing the proportion of patients who were NHS referees but were judged to be normal by detailed examination, there was a significant difference, with 45% for referrals directly from screening institutions and 21% for referrals through primary laboratory hospitals.

The aim of the NHS is to detect hearing loss early so that appropriate intervention can be carried out at the appropriate time. Since Osaka Prefecture has a large population and medical institutions, it is thought that sufficient functioning of the two-stage system will lead to examinations and interventions at appropriate times. It is necessary to build cooperative relationships not only with otolaryngology but also with obstetrics, pediatrics, and local governments.

Hearing level of new-born babies with unilateral congenital aural atresia should be evaluated within the first few months of birth. We mostly classify the patients as follows; conductive/mixed hearing loss in affected ears, and normal hearing in unaffected ears. Regular evaluations for hearing level and treatments for otitis media in unaffected ears are important in speech and language development. In addition, it is necessary to peal an eye for the risk of cholesteatoma. Children with unilateral hearing loss (UHL) due to congenital aural atresia (CAA) suffer from a wide range of auditory difficulties, but until recently we have had only meatoplasty and tympanoplasty to deal with hearing impairment, with low success rate. However, new devices to improve hearing impairment regarding UHL due to CAA have recently appeared, such as cartilage conduction hearing aids, bone conductive implants (BAHA and Bonebridge), and active middle ear implants (Vibrant Soundbridge: VSB). These devices have been utilized to improve hearing impairment with UHL due to CAA, but it remains unclear when to use these devices, also which optimal devices to use for individuals.

We normally introduce cartilage conduction hearing aids without operation to children with UHL due to CAA. We have 51 patients (aged 0–15) with UHL due to CAA in our hospital. 10 patients used cartilage conduction hearing aids and 1 patient used air conductive hearing aid. Almost all of these 11 patients recognized improvement of auditory difficulties. Only 4 out of them have received educational supports especially for audibility. As a whole, we have to more inform patients and their parents of their auditory difficulties, which facilitate more effective use of hearing aids and auditory implants.

Purpose: Puberty is the period of physical change from a child’s body to an adult’s body, during which male and female hormones are secreted. The appearance of secondary sex characteristics and rapid physical changes occur. The voice change is one of the secondary sex characteristics, voice changes so called voice mutation, which investigated perceptively were 12 years in boys and earlier in girls.

In this study, we regarded voice change as physical changes, and considered it as change from the lengths of the vocal folds in children to that of adults.

Methods: We calculated the lengths of the vocal folds in children, which increases with age [0.84×age (years)+4.02 mm], and the average lengths of the vocal fold in adults, 14.8 mm for males and 12.7 mm for females.

Results: The calculated period at the voice change was 12.83 years for males and 10.33 years for females, which occurred earlier in females than males.

Conclusion: The calculated boys’ age was very similar to the age at peak growth rate (12.89 years), and the girls’ age was similar to the age at pubertal onset (10.1 years) in Ogi growth study. These ages estimated from the lengths of the vocal folds of children and adults were considered to correspond to the present age of voice change in Japanese people.

Schwannoma is a benign tumor that originates from the Schwann cells. It is rare in the oral cavity. In this paper, we present a case of a 10-year-old girl with lingual schwannoma. She had noticed a tumor in the right posterior edge of the tongue, but left it for two years. Recently, she complained of a growth of the tumor and pain. A spherical tumor 2 cm in diameter in the right tongue edge was identified. Cervical MRI revealed a well-demarcated mass with a diameter of 19 mm. The tumor was shown to have low intensity in T1-weighted images, faint high intensity in T2, and marginal enhancement with gadolinium. With a preoperative diagnosis of neurinoma or salivary gland tumor, the tumor was excised trans-orally using a CO₂ laser. Histopathological examination revealed the tumor was an Antoni type A schwannoma. The nerve of origin of the tumor was unknown, but no neurologic deficit was observed postoperatively. The postoperative course was uneventful, and no recurrence has been observed one year and ten months after the operation.

Pyriform sinus fistula is a congenital fistula arising from the pyriform sinus of the hypopharynx to the dorsal thyroid. It often presented in childhood with acute thyroiditis or neck infection.

We report a case of the patient with a 3-cm mass on the left neck at birth. Six days after birth, the patient was referred from the NICU. Cervical ultrasound scan and CT revealed a monocystic lesion on the left neck. We punctured the mass and aspirated the yellow fluid. The mass was reduced, but it grew again after feeding. Laryngoscopy revealed that fluid similar to the aspirated fluid drained from the left pyriform sinus, and we diagnosed pyriform sinus fistula. At 20 days after birth, we attempted laryngoscopy cauterization to close the fistula, but could not identify the fistula intraoperatively. We only made a cervical incision to drain the fistula. At 48 days after birth, we attempted cauterization again. We found a fistula in the left pyriform sinus of the hypopharynx and cauterized with 10% silver nitrate. Postoperatively, the cervical mass did not increase in size.

We considered 10% silver nitrate to be an effective option for fistula closure in neonatal and infantile.

Langerhans cell histiocytosis (LCH) is a rare inflammatory neoplasia characterized by infiltration of monoclonal LCH cells; it is more common in children than in adults. Even after achievement of complete remission, there is concern that this disease can cause permanent consequences or secondary malignancies, thereby reducing quality of life. Herein, we report a patient with multifocal bone LCH who developed papillary thyroid carcinoma (PTC) after complete remission of LCH. The patient, a 15-year-old male, had LCH lesions on the right temporal bone and left femur at 9-year-old. He received chemotherapy according to Japan LCH Study Group (JLSG)-02 protocol, and obtained complete remission. However, five years later, he developed PTC with bilateral lymph node metastases; he underwent a total thyroidectomy and bilateral neck dissection. Both LCH and PTC lesions were positive for BRAF V600E mutation. To date, 13 years after the onset of LCH, he is alive in remission of both LCH and PTC. Because ear lesions are often observed in LCH and a close relationship between LCH and papillary thyroid cancer has been noted, otolaryngologists should be familiar to diagnosis and long-term complications of LCH.

Some children suffer from severe vertigo and dizziness. The symptoms of these include vestibular migraine and recurrent vertigo. However, a few cases arise due to vestibular issues. There are no reports on the treatment of pediatric vertigo using transtympanic positive-pressure therapy. Herein, we report a case that was effectively treated with this new approach. The child had severe vertigo, tinnitus and low-tone hearing loss after acute febrile mucocutaneous lymph node syndrome. She resisted the conservative treatment, and her cVEMP tuning property test, one of the tests that detect endolymphatic hydrops in the inner ear, was positive. We speculated that her vertigo occurred from endolymphatic hydrops and started this treatment. Following the initiation of this new therapy, her vertigo disappeared soon, and she did not need to take any medications and was even able to resume going to school. To us, transtympanic positive-pressure therapy is helpful for severe vertigo with endolymphatic hydrops in childhood.

We report two cases of hamartomas developed in infants. First patient was a 3-year-old girl. She had occasional wheezing since around one month of birth and presented to our hospital. The endoscopy revealed a 15 mm-sized mass with a smooth surface on the left side of the tongue roof. Since she had no wheezing and the patient was feeding well when we first examined her, we followed up until she was three years old and performed complete resection of the mass. The mass had a stalk at the base of the left tongue and was histopathologically diagnosed as a hamartoma. The other patient, the 4-year-old girl, presented to our hospital with nasal obstruction and snoring since around one year of age. A red mass with smooth surface was observed on the fiberscope from the left posterior nostril to the nasopharynx, and its base was located at the posterior end of the nasal septum. Complete resection of the mass was made by nasal endoscopic surgery. The mass was 20 mm in size and histopathologically diagnosed as respiratory epithelial adenomatous hamartoma (REAH). Hamartoma is defined as a tumor-like malformation composed of a disordered mixture of mature tissues indigenous to the region. It can develop at any site in the body, but few hamartomas occur in the oral area. When hamartomas occur in infants’ nasopharynx, infants present with dyspnea due to upper airway obstruction. REAH is hamartoma occurring in the nasal sinuses and pharynx, a new disease concept proposed in 1995. Although REAH was previously considered as a rare entity, reports on REAH have increased in recent years. REAH typically arises in the third to ninth decades of life, and a pediatric case such as the second case is extremely rare.

We report the case of a 17-month-old child who developed hypoglycemic encephalopathy associated with secondary carnitine deficiency after long-term administration of pivalate conjugated antibiotics (PCAB) for otitis media. The patient had been intermittently prescribed cefcapene pivoxil for otitis media for more than 100 days in a 6-month period at a local otorhinolaryngology clinic. She presented with generalized seizure, impaired consciousness, and spontaneous respiratory arrest triggered by poor eating and fever. The plasma glucose level was found to be 1 mg/dL, and spontaneous respiration resumed after the administration of glucose solution. Based on the patient’s medical history, hypocarnitinemia was suspected; levocarnitine was administered, and plasma glucose levels remained within the normal range. After treatment for hypoglycemic encephalopathy, her consciousness improved, and she has shown no evidence of developmental delay.

Even short-term administration of PCAB causes secondary carnitine deficiency and subsequent hypoglycemia and, in some cases, encephalopathy and sequelae. It is also necessary to raise awareness about the clinical diagnosis of acute otitis media, selection of antibiotics, administration period, etc., and to spread the contents of the Clinical Practice Guidelines for the Diagnosis and Management of Acute Otitis Media in Children in Japan.