A 4-year-old girl with acquired aphasia was diagnosed with Landau-Kleffner syndrome. She had shown no developmental problems before the onset of acquired aphasia. Auditory examinations yielded abnormal findings suggesting retrocochlear hearing loss. Electroencephalographic examination presented abnormal findings such as multifocal spike. Steroid pulse therapy was effective, and frequent abnormal brain waves disappeared. She showed difficulty with spoken language, but good understanding of visual language and is receiving education in a school for the deaf. In the case of children who show disappearance of spoken language, we frequently suggest hearing loss at first; however, it is important to coordinate with pediatricians and reach a diagnosis carefully, because such cases can result from neurologic disorders such as Landau-Kleffner syndrome.

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 Subannular tube (SAT) method is a simple method in which T-tube is inserted tympanomeatal flap from external auditory canal wall to the middle ear subannularly, leaving tympanic membrane intact. We have used SAT method in intractable otitis media with effusion and atelectatic ear since February 2013. Seven SATs were placed in total of 4 patients: 2 males and 2 females. Patients' mean age at surgery was 7.2 (median: 8) years. Average of the disease duration before SAT was about 56.7 months, and 2.0 times of transtympanic tube techniques were performed during the disease duration as average. The mean improvement of hearing was 22.7 dB. Three SATs underwent spontaneous extrusion and one of three tympanic membrane perforations has closed spontaneously. All 7 ears have been going well without complications, recurrence of middle-ear effusion and atelectasis. SAT method provides a safe and effective option for management of intractable otitis media with effusion and atelectatic ear.

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 Although dysplasia of inner ear structures in Down syndrome (DS) has been reported in many studies, the association with sensorineural hearing loss (SNHL) has not been identified. This is because it is difficult to assess the hearing acuity of pediatric patients with DS. The aims were to evaluate the prevalence of inner ear anomalies and the association with SNHL.
 Temporal bone computed tomography (CT) was performed for assessment of the inner ear structures in 33 pediatric DS patients (62 ears) and 26 controls (50 ears). Twenty-seven parameters of the inner ear structures were measured. ASSR, ABR, OAE, COR, BOA, and pure-tone audiometry were performed on each side according to the examinee's age and ability. In addition, the development quotient (DQ) of 16 DS patients was assessed with a questionnaire.
 Thirteen of the 62 ears in the DS patients were associated with severe-profound hearing disability. Although the bony island widths in superior and lateral semicircular canals were significantly smaller, their canals and ampulla lumens were not smaller than the controls. The long diameter of the vestibules, cochlea basal turn, and upper turns were shorter than in controls, whereas their width was not significantly different from controls significantly except for the cochlea upper turn. The widths of the cochlea nerve canal (CNC) and mid internal auditory canal (IAC) in DS patients were significantly narrower than in controls and also in hearing-impaired compared with normal-hearing DS patients. DS patients under DQ40 had a significantly higher rate of hearing impairment related to stenotic CNC and the IAC mid-width than in tose over DQ41
 Although the inner ear dimensions of DS patients were significantly different from controls, most parameters were not important as causes of SNHL. CNC and IAC widths should be measured in DS patients with SNHL.

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 Objective: With an incidence of 0.2–2.5%, congenital cytomegalovirus infection (CCMVI) is one of the most frequently encountered prenatal infections. It has been reported that approximately 90% of CCMVI infants exhibit no apparent manifestations, which is termed asymptomatic CCMVI, while the remaining 10% of CCMVI infants are symptomatic and display abnormal test findings. Recently, late-onset symptomatic CCMVI has been described as hearing loss or developmental abnormalities not in the newborn period, but instead during infancy or young childhood. Therefore, early diagnosis and prompt treatment of this condition is important. We herein present our ongoing prospective study on screening tests for CCMVI. Methods: A total of 3072 infants who were delivered at one of our associated institutes between February 2013 and March 2015 were enrolled. Along with newborn mass screening, several drops of blood were collected using FTA cards from which DNA was extracted and examined for CMV DNA load by real-time PCR. In cases where CMV DNA was serologically detected, we confirmed the diagnosis of CCMVI by testing for CMV DNA in preserved dried umbilical cord samples. Results: Eight infants were diagnosed as having CCMVI (0.26% incidence). Of the four who received detailed examination, two were asymptomatic, one had unilateral hearing loss and microcephaly, and one had ventricular dilatation as detected by magnetic resonance imaging (MRI). Discussion: We observed the overall incidence of CCMVI to be comparable with that in previous reports. However, the presence of symptomatic cases was relatively higher, and one patient had the silent symptom of abnormal MRI findings. Thus, newborn screening for CCMVI and subsequent detailed examination may be useful for early CMV detection and intervention.

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 Arteriovenous malformation (AVM) is a congenital vascular abnormality characterized by a direct connection between an artery and vein without normal capillary beds, which is called a nidus, and which is reported to be rare in the head and neck region. We report a case of infantile AVM of the masseter muscle in a 12-year-old male with a complaint of a soft and painless mass measuring 3.5×3.5 cm, in the left mandibular region, which becoming erect on clenching and in which vascular bruit-like sound could be heard. AVM of the masseter muscle was diagnosed based on the imaging findings of ultrasonography, enhanced CT and MRI. Notably, the 3D–CT angiography findings showed that the feeding artery was a left transverse facial artery followed by a nidus with venous drainage into the left masseteric vein in the left masseter muscle. Surgical removal was performed via an extraoral approach. The postoperative course was without complications and there has been no sign of recurrence until now. AVM generally requires treatment in the early stage due to its lack of natural regression. The preoperative evaluation of the related vessels by 3D–CT angiography is necessary for surgery which is considered to be the treatment of choice when possible.

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 As a consequence of rubella outbreaks from 2012 through 2013, 45 cases of congenital rubella syndrome were reported in Japan. We report the case of a boy who suffered from late-onset bilateral hearing loss due to congenital rubella infection. His unvaccinated mother had been infected with rubella in the early stage of pregnancy. He was born healthy without any symptoms of congenital rubella syndrome, so congenital rubella infection was diagnosed. Hearing was examined in the newborn period by auto-auditory brainstem response (AABR) and both ears passed. When he was 13 months old, his parents suspected hearing loss because his reactions to sound seemed to have deteriorated. He was treated for otitis media with effusion for a few months, and then diagnosed with sensorineural hearing loss at 18 months old. The threshold for ABR was 60 dBnHL in the right ear but was scaled out in the left ear. The diagnosis was finally changed from congenital rubella infection to congenital rubella syndrome.

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 We describe rare pediatric sleep apnea caused by Hashimoto's thyroiditis. A girl aged 4 years and 8 months was referred to our hospital due to nocturnal awakening due to stifling and nasal congestion. The primary care doctor identified sleep apnea. A physical examination revealed adenoidal hypertrophy and allergic rhinitis, but tonsillar hypertrophy was not significant. She was obese, short for her age and appeared tired with low activity levels. Hormonal assessment and blood testing for the short stature revealed Hashimoto's thyroiditis. Levothyroxine sodium was administered, which improved the nocturnal awakening after two weeks, and snoring, nasal congestion and thyroid function after three months. Sixteen months of medication resulted in a decrease in the Apnea Hypopnea index (AHI) from 50.8 to 8.5 and an improved desaturation rate (rate of O2 saturation ≤90%) from 20% to 1.28%. A medical history of a growth disorder accompanied by other signs and symptoms are important for a diagnosis of hypothyroidism in pediatric sleep apnea because pediatric patients are usually unable to effectively describe their symptoms.

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 We report a case of autism spectrum disorder (ASD) with repetitive insertion of foreign bodies into the external auditory canals. The patient was a 6-year-old boy and he first visited our department because he repeatedly inserted foreign bodies into the external auditory canals. We introduced him to the Department of Psychosomatic Medicine, and he was diagnosed with ASD. He had other symptoms such as communication deficits and strong impulsivity, but they improved gradually after medication and intervention. Repetitive insertion of foreign bodies into the external auditory canals is a repetitive and stereotyped behavior. These repetitive and stereotyped behaviors become compulsive due to external factors such as changes in the social environment and family relationships. Sensory abnormality is a characteristic of most people with ASD, and it was considered that this patient was insensitive to the pain. It is well known that a diagnosis of ASD secondarily complicates other mental disorders at a high rate because of adjustment disorder. Thus, it is necessary to diagnose and intervene early if a patient has a special type of ASD. A patient not yet diagnosed with ASD may visit an otolaryngology department for the first time. A cooperative approach with specialists of psychosomatic medicine, child psychiatry, or neurology is recommended.

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 The aim of this study was to evaluate the feasibility and outcomes of video-assisted thyroidectomy for a pediatric case. Methods: Between May 2009 and February 2016, a total of 210 patients with thyroid diseases underwent thyroid surgery by video-assisted neck surgery (VANS method). Among these patients, three pediatric patients (10–14 years-old girls, minimum height was 143 cm) underwent thyroidectomy by VANS method for the benign thyroid nodule. We had modified VANS method mainly by using originally developed skin-flap retractor. In our modified method 2.5 cm the main incision was made on the diseased side of the chest wall below the clavicle, then newly developed retractor was inserted under the subplatysmal layer for creating working space. Results: The operation was successfully completed endoscopically in all pediatric patients without any complications. But one patient, whose histopathological result of the dissected thyroid was follicular adenoma, developed neck lymph node metastasis six months after the surgery. Because the diagnosis altered to follicular carcinoma, she underwent neck dissection and completion thyroidectomy. Conclusion: Surgery would be a reasonable option for a thyroid nodule >3 cm with children and our modified VANS method could be applicable to children.

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 After updating the guidelines in 2014, the bilateral use of cochlear implants for prelingually deaf children is expected to be more common in Japan. The usefulness and effectiveness of the bilateral cochlear implant should be verified among Japanese language users, although the number of bilateral cases is still limited in Japan. Fifty consecutive bilateral pediatric cochlear implant cases in our institute were retrospectively reviewed in this study. Forty-five of them revealed prelingual severe to profound deafness, and all of them underwent sequential surgery. All cases demonstrated remarkable improvement in the hearing threshold (38.0 dB–32.1 dB) and speech perception (72.5%–87.8%) after second implant usage. Because most children were too young for language evaluation, the result of language development is ambiguous because of the limited number of suitable cases. Longer follow-up data are needed to draw the final conclusion of the usefulness of the bilateral cochlear implant.

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 Tympanoplasty (myringoplasty) for pediatric chronic otitis media remains controversial. Clinicians must be aware of the frequency of upper respiratory tract infections, Eustachian tube dysfunction, and the narrowness of the ear canal in such cases.
 Between January 2004 and August 2014, 20 pediatric tympanoplasties (myringoplasties) were performed for chronic otitis media at Nara Medical University Hospital. The aim of this study was to analyze the factors associated with the surgical closure of perforated tympanic membranes. The following factors were analyzed: age at surgery (<10 years, ≥10 years); the size of the perforation; the presence/absence of an active infection (otorrhea) at the time of surgery; the state of the mucosa of the middle ear; and postoperative myringitis.
 Tympanic membrane closure was achieved in 16 out of 20 ears (80%). According to the Guidelines of the Japan Otological Society, the audiological success rate was 94.7% at the 6-month follow-up. Active infection (otorrhea) at the time of surgery (p=0.032) and postoperative myringitis (p=0.032) were significantly correlated with postoperative re-perforation of the tympanic membrane (Fisher's exact probability test). Age at surgery, the size of the perforation, and the state of the mucosa of the middle ear were not correlated with re-perforation of the tympanic membrane.
 In cases of pediatric tympanoplasty (myringoplasty), it is crucial to control pre- and postoperative infections in order to prevent re-perforation of the tympanic membrane.

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 A 2-year-old girl presented to our department with left hearing impairment and visited. Her newborn hearing screening was normal, and she had no past history of otolaryngological disease. When she was 1 year old, she had been treated with chemotherapy and proton therapy for a cerebellar tumor. COR and OAE were normal, but ABR showed no response in the left ear. From this history and her examination results, she was diagnosed with delayed retrolabyrinthine deafness after photon therapy. She was prescribed oral steroids but her deafness did not improve. The case highlights the need to pay attention to delayed deafness caused by radiation therapy for a brain tumor and to conduct hearing examinations such as COR, OAE, and ABR and detailed interviews for obtaining medical histories in children.

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