We studied the prevalence and characteristics of coughing and nasal and pharyngo-laryngeal symptoms in 42 children 15 years or younger and 42 adults with Japanese cedar pollinosis via questionnaires in the pollen season in 2009. The prevalence of coughing in children in past pollen seasons was the same as in adults, although pharyngo-laryngeal symptoms were significantly less prevalent in children than in adults. When seen at our clinic, 31.0% of children and 43.6% of adults had coughs, and the difference was not significant. The prevalence of pharyngo-laryngeal symptoms was significantly lower in children than in adults. There was no significant difference between the nasal symptom scores of children and adults. In adults, the nasal obstruction score and the prevalence of postnasal drip were significantly higher in patients with cough than in those without coughs. Although coughing occurs in more than 30% of children with Japanese cedar pollinosis, the prevalence of pharyngo-laryngeal symptoms was low. There is a possibility that the laryngeal allergy is less prevalent in children than in adults.

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  We report a case of Floyd's type II tracheal agenesis. A male infant weighing 3146 g was born at 39 weeks of gestation. He did not cry but instead showed cyanosis and severe respiratory distress after birth. Bag-mask ventilation was briefly effective, but tracheal intubation was never successful. His trachea was not detected by neck palpation. An attempt to examine his trachea by fiberoptic laryngoscopy failed because of complete closure of the airway just under the glottis. His respiratory condition improved slightly after esophageal intubation. He underwent esophageal banding and a gastrostomy on day 0. A bronchoesophageal fistula 1.5 mm in diameter was found at the middle esophagus by endoscopy after the surgery. A CT scan revealed that his trachea was absent but both main bronchi appeared to be normally bifurcated from the carina just below the bronchoesophageal fistula. The clinical diagnosis was tracheal agenesis (Floyd's type II). The respiratory distress was progressive, and he died on the 38^th day after birth. Tracheotomy is one of the major initial treatment options for respiratory failure caused by congenital upper airway blockage such as laryngeal atresia or laryngo-tracheo-esophageal cleft. However, if the trachea is absent, such as in this case, the only method to support respiration is esophageal intubation. Pediatric otolaryngologists who perform emergency tracheotomies on newborns should therefore be familiar with this disease.

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  Infantile myofibromatosis is a rare disease characterized by single or multiple mesenchymal tumors. The tumors are often found in the head and neck, and 88% of the patients are diagnosed before the age of two. The prognosis is considered poor when the tumor is multiple or involves internal organs. We report a case of infantile myofibromatosis with the chief complaint of glossoncus. The case was a two-month-old female; a decrease in nursing and defective weight gain were noticed three weeks after she was born. At the first visit to the hospital, a hard 10×4 mm tumor at the left base of the tongue was found. According to pathological study and close examination of the whole body, we diagnosed her with infantile myofibromatosis with metastasis in the cerebrum, cerebellum, cervix, skin, bilateral lungs, and bones. Chemotherapy with vinblastine (VBL) and methotrexate (MTX) produced complete regression, and no new tumor appeared. Therefore, the treatment was considered effective.

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  We treated two cases of parapharyngeal space infections in children. Case 1 was a 6-year-old female and case 2 was a 7-year-old female. In both cases, CT scan showed ring-enhanced mass lesions in the parapharyngeal space, which indicated abscess formation. Case 1 underwent tonsillectomy and needle aspiration of the parapharyngeal space but no pus was obtained. Case 2 underwent needle aspiration of the peri-tonsillar space, but no pus was obtained. In case 1, ASO and ASK titers were high. In case 2, a rapid antigen detection test for group A streptococcus was positive. However, no beta-hemolytic streptococci were isolated in either case. With 16–18 days of high-dose intravenous antibiotic therapies, the patients recovered completely.

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  A lipoblastoma is a benign tumor composed of embryonic adipose tissue that usually occurs before the age of three. We had a case with a rapidly growing lipoblastoma in a 2-year-old boy. The patient's mother noticed a mass in his posterior neck. We found a 35-mm hard mass in his posterior neck, and because we could not find any evidence of malignancy by FNA, we diagnosed it as a lipoma. One year later, the mass size was 65 mm. MRI showed a 68×52×36 mm mass with clear edges. The growth was so quick that we couldn't rule out malignant tumor. We performed an operation under general anesthesia on Dec. 8, 2009. The tumor was located right under the back muscles of neck, and the color was white. It had no adhesions so we removed it completely. The pathological diagnosis identified a lipoblastoma. Surgical resection is appropriate treatment for lipoblastoma. There have been reports of local relapse of lipoma, so follow-up is required.

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  [Objective] Children with mild and moderate hearing impairment have been identified at a very early stage by the neonatal hearing screening that has been widely performed in recent years. The 80 Hz auditory steady-state response (ASSR) is useful to estimate frequency-specific thresholds in sleeping infants. However, a dissociation between the ASSR and behavioral audiogram is sometimes observed, especially in cases of multiply-handicapped or underdeveloped children.
  [Subjects and Methods] Eighty-one children (116 ears) identified as having mild or moderate hearing loss (35–70 dB) using GSI Audera^® were investigated in this study. Subjects consisted of 46 boys and 35 girls aged one month to nine years old. The ASSR–threshold was assessed under sedation in all cases. In addition, auditory brainstem response (ABR), otoacoustic emissions (OAE), and behavioral audiograms were obtained depending on the developmental age.
  [Results] Of the 81 cases, 33 (39.8%) were referred for appropriate specialized diagnostic intervention by the neonatal screening center. Thirty patients (37.0%) had multiple handicaps. The causes of impaired hearing were otitis media (50 ears, 43.1%), malformation of the middle ear (5 ears), auditory neuropathy (3 ears), and unknown (31 ears). In the remaining 27 ears (19.4%), hearing levels were ultimately determined to be within normal limits after considering findings of other hearing tests. Of these 27 ears, 13 ears (48.1%) were in multiply-handicapped children. In 11 ears (40.7%), ASSR had been performed less than 6 months after birth.
  [Conclusion] It was reconfirmed that the hearing level of children with mildly or moderately impaired hearing should be repeatedly assessed not only by ASSR but also by ABR, OAE, and behavioral audiogram.

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  Thyroglossal duct cyst carcinoma is rarely seen in children. Only 23 cases have been reported in the English literature so far. We report here a case of thyroglossal duct cyst carcinoma in a 12-year-old boy. He complained of a 3-cm diameter tumor in the right submental region. CT examination revealed a low density mass with calcification. Initially, we diagnosed this condition as papillary carcinoma of the thyroid gland with lymph node metastasis. The resected tumor was close to the hyoid bone and looked like a papillary carcinoma. However, because no tumor was observed in the thyroid gland, we considered this case a thyroglossal duct cyst carcinoma.

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  To determine whether otitis media will be severe or not and its sequelae, I studied the clinical course of severe otitis media and character of the tympanic membrane of 2,000 children under the age of 15 with acute otitis media (AOM) or otitis media with effusion (OME) who visited Kato Ear Nose Throat Clinic from September 2005 to March 2008. The tympanic membrane was photographed using a rigid otoscope with a CCD camera. In September 2010, for a clinical course, I retrospectively investigated the age at the first incidence of otitis media, characteristics of the tympanic membrane, the side of the diseased ear, the age at the recurrence of final otitis media, and the number of recurrences. I found that 30% of all cases had a single episode of AOM, 30% of all cases with an incidence of AOM after follow-up had severe otitis media, 50% with an incidence of AOM at 0-years-old had severe otitis media, and 40% with an incidence of AOM at 1-year-old had severe otitis media. The findings of the tympanic membrane observed most often in severe otitis media were effusion in the tympanic cavity not lasting more than one month. The next was bulla formation on the tympanic membrane, otorrhea, crust, spots under the tympanic membrane, and throbbing of the tympanic membrane.

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  To investigate the extent of involvement of pathological gastroesophageal reflux in the development of childhood otitis media, the pH of the fluid in the middle ear at various stages of otitis media of 39 children aged 0 to 13 years old (median age, 2 years and 5 months) who had undergone tympanostomy between June 2009 and January 2010 was measured, and the amount of pepsinogen 1 (PG1) in the fluid was also quantitated. The pH was measured using a Horiba compact pH meter (B-211). PG1 was measured at 450 nm using an ELISA kit with a human PG1-specific monoclonal antibody. Measurement of the middle ear fluid pH revealed an average pH of 7.86 (SD±0.56). When a PG1 concentration of 100 μg/l in the retained fluid in the middle ear was defined as the cutoff value and lower levels were defined as negative, positive results (values of 100 μg/l or over) were obtained in 41% of the patients (16/39 cases). The average pH level in the negative group was 8.04 (SD±0.43), while that in the positive group was 7.54 (SD±0.50), and the difference in pH between the two groups was significant. There was a significantly higher number of children aged 2 years or over in the positive group. The results suggested pathological gastroesophageal reflux in 41% of the subjects with childhood otitis media. Measurement of the middle ear fluid pH levels may be useful for determining the presence of pathological gastroesophageal reflux.

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  Adenotomy and tonsillectomy are commonly performed in pediatric patients, with obstructive sleep apnea being the most common indication. Because these surgeries are still associated with a relatively high risk of postoperative morbidity, surgeons tend to avoid cases when they are combined with other high risk factors, such as heart or pulmonary failure or infancy. Surgeries performed on nine children diagnosed with obstructive sleep apnea syndrome combined with high risk were reported. We suggest that careful diagnosis with polysomnography and precise informed consent before surgery, an operation using the newer technologies, and cooperation of the pediatrician and veteran anesthesiologists during the surgery lead to surgeries without incident.

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  In general, the younger the age at cochlear implantation, the better the development of spoken language. Factors affecting the status of language use after cochlear implant surgery were investigated in 12 children (3–9 years old) at Hyogo College of Medicine. The use of the cochlear implant by patients under 5 years old was assessed using IT-MAIS (Infant-Toddler Meaningful Auditory Integration Scale), and that by patients over 5 years old was assessed using MAIS. Active participation of the parents was the key to better use of the cochlear implant. Active use of the cochlear implant improved the hearing ability and speech intelligibility of the patients in this series.

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  We studied the relationships between the sleep-induction time from the administration of hypnotics to the test time and between the test time and the extent of hearing loss, aiming at more efficient air-conduction ASSR (auditory steady-state responses) testing. We tested 144 patients with an average age of 4.1±3.4 for whom the sleep-induction and testing times could be obtained and measurements could be made for all carrier frequencies (250, 500, 1 K, 2 K, and 4 kHz) for both ears. Navigator Pro was used for ASSR testing with a limitation of using sleep-inducing drugs up to twice the normal dose. The rate for completing the testing with a single dose of a sleep-inducing drug was 60.7% (62.8% for Tricloryl syrup, 41.7% for Rabona, and 63.6% for Escre). The average sleep-induction time was approximately 40 minutes, and the testing time was 84±26 minutes. There was a positive relationship between an originally created hearing-loss measure and the testing time. In order to shorten the testing time, it is necessary to determine the initial stimulation intensity, considering other hearing tests and previous values.

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  We report a 3-year-old boy with severe bilateral hearing loss caused by congenital cytomegalovirus infection. He initially consulted the pediatrics department of another hospital for common cold-like symptoms. Speech delay was initially suspected because he spoke only a few words; however, when referred to our department we found he in fact had deterioration in speech and language caused by late-onset, progressive hearing loss due to congenital cytomegalovirus infection. One of the most important features of this infection is that it is difficult to diagnose. This relates to the wide variation in clinical features (in particular, onset age, progression, hearing level, and asymmetry), tendency to be asymptomatic, and limited methods for retrospective diagnosis after infancy. Due to decreasing levels of immunity to cytomegalovirus in pregnant women, concern is rising regarding the possibility of increasing numbers of children with hearing loss attributed to congenital cytomegalovirus. Indeed, congenital cytomegalovirus infection is extremely important as a cause of hearing loss in children.

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  A two-month-old boy was sent to our clinic for difficulty in breathing. He had been experiencing stridor for one month before referral. He was put on a respirator for a month without any obvious causes to account for the symptoms. Computer tomography (CT) and magnetic resonance imaging (MRI) suggested a cystic lesion of the pharyngeal and tongue base. We diagnosed a cyst that obstructed the upper airway. Resection of the cyst by a transoral approach was successfully performed using an endoscope under general anesthesia. The cyst was located lateral to the right side of the vallecula epiglottica. The histopathological examination confirmed the diagnosis of a cyst, but the histological origin of the cyst was unclear. Stridor was resolved after the surgery, but he needed respiratory and nutritional support for another month after surgery. He has been free of symptoms and recurrence for a year since the surgery. Although an cyst at the base of the tongue is rare in infants, accurate diagnosis and treatment is of paramount importance for a good prognosis.

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  We aggregated test results for 30 children complaining of hyperacusis. Of these 30 children, the percentage with autism spectrum disorders (ASD) was highest among the preschool children. Only five of the 19 elementary school children had ASD. The WISC–III was given to 17 children for whom consent was obtained. Three children were thought to have visual information processing dominance because the performance intelligence quotient (PIQ) was significantly higher than the verbal intelligence quotient (VIQ), and the perceptual reasoning index (PRI) was significantly higher than the verbal comprehension index (VCI). Conversely, eight children showed auditory information processing dominance. Auditory brainstem response (ABR) was measured in 11 children who became dizzy or crouched down when they heard a loud sound. Large amplitude waves following ABR were seen in eight children. These waves resembled vestibular-evoked myogenic potentials (VEMP), but VEMP could not always be detected with simultaneously measured myogenic potentials. Children in whom these large waves were seen on one side showed improved symptoms when the ipsilateral ear was plugged. Functional magnetic resonance imaging was conducted on 13 children who felt sensations other than auditory sensations, such as seeing objects distorted when they heard a particular sound. Colored-hearing synesthesia was suspected in three children. Efforts to ascertain the information-processing and sensory features of children complaining of hyperacusis are important in understanding these children and determining suitable approaches.

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  The present study retrospectively examined the incidence, treatment, and outcome of otitis media with effusion (OME) in 233 children with cleft palate who underwent palatoplasty from 1994 to 2004 at Kanagawa Children's Medical Center, Yokohama. Data on the presence of otitis media with effusion at the first visit and last follow-up and grommet insertion was collected from clinical notes. At the first visit (mean age 10.4 months), OME was found in 298/466 ears (64%) of all cleft palate children, but was found in only 6/30 ears (20%) of submucous cleft palate children. Grommet insertion was performed in 220 ears (47.3%). At the last follow-up (mean age of 80.4 months), patients did not have OME in 365 ears (78.3%), and had OME in 38 ears (8.2%), perforations in 24 ears (5.2%), and grommets in 38 ears (8.2%). OME was not found in 138/220 ears (62.7%) with grommet insertion and 227/245 ears (92.7%) without grommet insertion. Thus, OME was found in 298/466 ears (64.0%) of cleft palate children at the first visit, but it was not found in 365 ears (78.3%) at the last follow-up. At the last follow-up, the incidence of ears without OME was significantly lower in the ears with grommet insertion than in the ears without it. It is not recommended that all children with cleft palate undergo grommet insertion at palatoplasty, but we think that grommet insertion should be performed in cleft palate children in whom OME is persistent.

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  Pervasive developmental disorders (PDD) including autism and profound deafness are both particularly difficult to diagnose in infancy and early childhood. Communication in PDD children is so strikingly impaired that it may resemble communication in deaf children, but the specific intervention methods are different. Our objective is to determine the characteristics in cases of comorbid profound deafness and PDD. We conducted a retrospective review of five children with comorbid bilateral sensorineural hearing loss and PDD. An early intervention group (n=3) was found in a newborn hearing screening program, and a late intervention group (n=2) was identified by language development delay. PDD was recognized in children of both groups by analysis of behavior profiles in a group setting. They had the same inappropriate interpersonal communication characteristics. The late intervention group tended to be less likely to respond to speech and to acquire language. These overlap cases need early intervention based on their characteristics.

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  This was a retrospective study of sleep structures of seven pediatric patients diagnosed with obstructive sleep apnea syndrome based on the diagnostic criteria of the International Classification of Sleep Disorders. All patients underwent pre-and post-operative polysomnography and sleep stages were scored based on the Rechtschaffen and Kales classification. We identified significant polysomnographic changes in the sleep structures in children with obstructive sleep apnea syndrome after adenotonsillectomy. Both sleep stage 1 and arousals at night decreased significantly. Changes in slow wave stages were not statistically significant. Further research is needed to evaluate whether these changes might affect the post-operative mental and physical health.

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  We report two cases of cholesteatoma that disappeared or decreased in size in pediatric patients. Patient 1 was a male aged 1 year and 6 months. At the first consultation, a white mass was observed in the postero-superior quadrant of the right tympanic membrane. CT revealed a round soft tissue shadow in the tympanum. Congenital cholesteatoma was suspected. Ten months later, CT showed an increase in the size of the soft tissue shadow. In September 2008, exploratory right tympanotomy was performed. When the tympanic membrane was opened, a large amount of viscous exudate was aspirated. No clear cholesteatoma or debris was observed.
  Patient 2 was a male aged 1 year and 7 months. At the age of 1 year ventilation tubes were inserted. In June 2008, a white mass in the right ear was detected. The tube was present in the antero-inferior quadrant, and a white mass above the tube was visible. CT revealed a soft round tissue shadow in contact with the tube. Cholesteatoma was suspected. In November 2009, the tube in the right ear was removed, when no debris from the perforation edge was observed. In January 2010, the size of the soft tissue shadow decreased on CT images.
  Some patients show spontaneous disappearance of congenital cholesteatoma. Reasons for the spontaneous disappearance of a cholesteatoma include absorption and evacuation of cholesteatoma due to destruction of the capsule by inflammation or presumptive apoptosis. In patients with suspected cholesteatoma, observation of the course is one option when the mass is localized in the tympanum, is not in contact with the ossicle, and neither ossicle destruction nor complications are observed.

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  We report a case of inner ear malformation with three episodes of bacterial meningitis. A 3-year-old girl suffered from recurrent meningitis. CT scanning revealed bilateral inner ear malformation (incomplete partition type). Profound hearing loss in both ears was found by audiometric examination, and fluid from the puncture of the right tympanic membrane contained glucose, resulting from cerebrospinal fluid (CSF) leakage. To prevent the recurrent meningitis, we tried a tympanoplasty to block the CSF leakage. Before the operation, intermittent spinal fluid drainage was performed to control of the CSF pressure. The bony foot plate of the stapes was defective and replaced the membranous plate there. There was no anomaly in other auditory ossicles. When the superstructure of the stapes was removed, a severe gusher of CSF occurred promptly, requiring extra-intermittent spinal drainage and an intravenous drip infusion of d-mannitol. We packed the fascia of the temporal muscle into the oval window tightly with the addition of adhesive fibrin glue. She has remained free from recurrent meningitis during 12 months follow-up since surgery.

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  The quality of life after tracheal separation surgery was evaluated by analysis of a questionnaire answered by the families of pediatric patients with severe physical and mental disabilities. Operations were performed on 23 pediatric patients between February 2000 and July 2008, which included laryngotracheal separation (12 cases), tracheoesophageal diversion (one case), and tracheoesophageal diversion with tracheal closure (tracheal flap method) (10 cases). In four out of 17 cases, the tracheal separation was preceded by tracheotomy. Questionnaires were sent to 17 patients excluding six cases (three deaths, three lost to follow-up), and answers from all 17 patient families were analyzed. The frequency of oral and tracheal treatment for aspiration of saliva was markedly reduced after tracheal separation in all four cases that underwent preceding tracheotomy, and was also reduced in seven of 13 cases without preceding tracheotomy. Aspiration pneumonia and other respiratory disorders were well controlled after operation in almost all cases, which resulted in the reduction of hospitalizations in 11 out of 15 cases. Major complications after operation were granulations around the tracheal stoma, narrowing of the tracheal stoma, and tracheo-cutaneous fistula. In only one case of tracheo-cutaneous fistula, surgical reconstruction was needed, while in the other cases, all complications were recovered by conservative treatments. Tracheal separation improved the QOL of patients due to decrease in incidence of aspiration pneumonia and other respiratory disorders, and the QOL of patient families was improved by the reduced burden of nursing care. As a result, patient families were almost all satisfied with the result of the operation. These results suggest that, if the surgical adaptation is strictly selected, tracheal separation is one of the most effective operations for pediatric patients with severe physical and mental disabilities.

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