We studied 17 ears in 14 patients who underwent stapes surgery for congenital ossicular mal­formations at the Department of Otolaryngology, Head and Neck Surgery, Osaka University Hospital from April 2012 to December 2020. The ages ranged from 3 to 13 years, and there were 8 males and 10 ears, and 6 females and 7 ears (both ears: 3 cases). According to Funasaka’s classification, multifocal malformations accounted for about half of the cases. The pistons which we used were Teflon Wire Pistons (TWP) in 8 ears, Teflon Pistons (TP) in 5 ears, and Malleus Attachment Pistons (MAP) in 4 ears. At 6 months after surgery, 94% of the patients were successful according to the criteria of the Oto-Rhino-Laryngological Society of Japan, and 48% were within 10 dB of the air-bone gap. The success rates are good, but the hearing results were inferior to those of otosclerosis in our department. The air-bone gap was significantly greater for multifocal than monofocal, and for MAP than TWP/TP, suggesting that a higher percentage of patients who had other malformations, such as mal­formation of the incus, fixation of the malleus and incus, and that a higher rate of MAP use.

We examined 105 children who were diagnosed as functional hearing loss during from 2017 to 2020. Seventy percent of the subjects were girls, with a peak in 8-year-olds. In 2020, unlike the other years, the most children visited us from July to September. This change was thought to be due to SARS-CoV-2 pandemic. While 58% of the children visited us with symptoms (53%; hearing loss, 5%; miscellaneous). Forty two percent were found incidentally to have hearing loss by school health examinations. 21% of the children had some psychological problems. At the first visit, 21% were accompanied by developmental, neurological, or psychosomatic disorders.

Audiological examination revealed bilateral and unilateral functional hearing loss in 73 cases (70%) and 32 cases (30%), respectively.

Children who had been referred from school health examinations were significantly younger than children with symptoms, however, there was no difference in the positive ratio of psychological factors, the side of hearing loss, or the prevalence of profound hearing loss in pure-tone audiogram between the two groups.

After the diagnosis of functional hearing loss, 49% of the children were considered to require the consultation with pediatric neurologists, psychiatrists, or psychotherapists. Cases with symptoms or profound hearing loss were significantly more likely to require consultation with these specialists. In the managements of functional hearing, it is important to collaborate with specialists in child development and psychosomatic disorders.

The target age for pediatric cochlear implant surgery has been decreasing over the years, but a certain percentage of cochlear implant surgeries are still performed in children older than 4 years; it is unclear why the surgeries were delayed. We investigated the background in children who underwent cochlear implant surgery at the age of 4 years or older at our hospital between 2000 and 2019. The backgrounds leading to the delayed surgery were categorized into “early surgery was possible,” “the indication of surgery was difficult to determine,” and “surgery was delayed due to etiology.” Comparing the backgrounds in the recent 10 years with those in previous 10 years, the percentage of cases whose surgery was delayed due to etiology has remained almost unchanged, while the percentage of cases in which the surgical indication was difficult to determine has decreased and that of cases in which early surgery was possible has increased. In order to promote appropriate early cochlear implantation, it will be necessary to continue to actively educate the public about the importance of early diagnosis of hearing loss and the subsequent decision on the indications of cochlear implants.

Introduction

In 2016, the Japanese government introduced the National Action Plan on antimicrobial resistance (AMR) to reduce the number of antimicrobial prescriptions. Although antimicrobial agents tend to be prescribed more frequently for children in primary medical emergency centers, reports are lacking on prescription trends in otorhinolaryngology departments.

Method

This single-center descriptive epidemiology study retrospectively investigated oral antimicrobial prescriptions for patients aged 15 years or younger who visited the department of otorhinolaryngology at the Himeji City Emergency Medical Center in 2015–2019. We then calculated the total antimicrobial prescription rate and the number of each antimicrobial prescription per 1,000 patients.

Result

The total antimicrobial prescription rate remained around 60％, and the number of prescriptions per 1,000 patients decreased from 442 to 218 and 59.7 to 4.7 for third-generation cephalosporins and carbapenems, respectively, whereas it increased from 128 to 386 for amoxicillin.

Conclusion

We observed a change in prescription trends of third-generation cephalosporins, carbapenems, and amoxicillin, suggesting the possibility of the effectiveness of the guidelines and action plan developed to tackle AMR. We plan to continue a prospective survey in collaboration with otorhinolaryngologists and pediatricians.

Kawasaki disease is a systemic vasculitis of unknown etiology that often causes various symptoms such as fever, rash, and mucositis, especially in children under four years of age. Hearing loss frequently occurs along with coronary aneurysms and is an important complication in Kawasaki disease. Detection of hearing loss may be difficult, and effective treatment is often delayed because the awareness of hearing loss associated with Kawasaki disease is low and children who suffer from hearing loss rarely complain to another person. As a delay of adequate treatment for deafness may cause residual hearing loss, there is a need for greater awareness of hearing loss in Kawasaki disease.

We experienced a case of a 3-year-old girl with bilateral combined hearing loss discovered because of a poor response to her parents’ voices after the onset of Kawasaki disease. Hearing loss was diagnosed promptly after visiting our department using objective tests, and improvement of hearing levels was observed immediately after systemic administration of steroids. The patient can now live without a hearing aid and language disorder. Follow-up is ongoing.

In this case report, we describe the course of treatment of this case, the pathogenesis of hearing loss in Kawasaki disease, and the necessity of therapeutic intervention with reference to past literature.

Laryngomalacia is one of the common causes of inspiratory stridor in newborns and infants. In contrast, there are few reports of acquired laryngomalacia in school-age children and adolescents. We report a case of a school-age boy with aryepiglottic fold type laryngomalacia that responded well to surgical treatment. Endoscopic examination revealed an inward prolapse of the bilateral excess arytenoid mucosa and shortened aryepiglottic folds. When conservative treatment fails, surgical treatment is recommended for acquired laryngomalacia. In our patient, the symptoms improved after supraglottoplasty, namely resection of the excess mucosa and dissection of the hemilateral aryepiglottic fold. Postoperatively, he recovered from inspiratory stridor, but still experienced intermittent cough. He was diagnosed with voice tics and treated by a psychiatrist. His inspiratory dyspnea has been successfully relieved so far. Since there are very few reports of laryngomalacia in school-age children in Japan, this condition may be misdiagnosed. However, otorhinolaryngologists should recognize laryngomalacia as a differential disease causing inspiratory stridor, accurately diagnose it using endoscopic examination, and provide appropriate treatment.

We experienced two cases of esophageal foreign bodies that were removed with pharyngolaryngeal surgery instruments. Case 1: an 11-month-old boy who accidentally swallowed a button battery, and Case 2: an esophageal foreign body (large amount of hair) in a 5-year-old girl with a history of 21 trisomy and congenital esophageal stricture. Both cases were removed by a pediatric surgeon under general anesthesia using an upper gastrointestinal endoscope, but the removal was difficult due to difficulty in grasping with the endoscopic forceps. Therefore, an otolaryngology/head and neck surgeon was called and used a laryngoscope and Laryngoforce II^® grasping forceps for laryngeal microsurgery, which resulted in complete removal. In both cases, the foreign body was present in the cervical esophagus. In case 1, a high-voltage lithium battery was suspended in the esophagus and came into contact with the esophageal wall, producing hydroxide by discharge and adhering to the mucosa, and in case 2, the amount of hair was so large that it was difficult to remove it with a balloon catheter or forceps from the upper gastrointestinal endoscope. Laryngoscopes and forceps in pharyngolaryngeal surgery may be useful for removal of cervical esophageal foreign bodies in children.

Dominant deafness-onychodystrophy (DDOD) syndrome is an autosomal dominant genetic disorder characterized by sensorineural hearing loss and nail dysplasia. DDOD syndrome has been reported due to c.1516C>T[p.(Arg506*)] mutation in ATP6V1B2 gene. Here we report a female case of DDOD syndrome. She was diagnosed as a congenital bilateral severe sensorineural hearing by auditory steady-state response at the age of 3 months. A genetic testing (exosome analysis) revealed ATP6V1B2 c.1516C>T[p.(Arg506*)] gene mutation. She started using bilateral hearing aids at the age of 5 months, but no obvious change in auditory behavior was observed. She underwent bilateral cochlear implantation at the age of 11 months. Postoperative hearing threshold reached the steady state of 30–40 dB one month after the implantation. The auditory and speech behavior assessments also improved postoperatively. Because of few reports on DDOD syndrome, the usefulness of cochlear implantation is not yet elucidated. Further accumulation of the cases with cochlear implantation for the sensorineural hearing loss in DDOD syndrome should be warranted.

We present two pediatric patients of progressive hearing loss with heterozygous p.A684V mutation in WFS1 gene from independent two Japanese families.

One patient is a 4-year-old girl. She had passed the Newborn Hearing Screening (NHS), however, delayed speech was pointed out at the age of 18 months. Auditory brainstem response (ABR) thresholds of bilateral ears were both over 100 dBnHL at 23 months of age. Heterozygous mutation in the WFS1 gene (c.2051C>T: p.A684V) was detected by genetic testing at the age of 2 years and 6 months. Bilateral cochlear implantations were performed at the age of 2 years and 6 months, and 3 years and 5 months, respectively, because of insufficient benefits of hearing aids. The conditioned orientation response audiometry (COR) under wearing cochlear implants demonstrated threshold ranging from 35–40 dB at the age of 3 years and 9 months, and the speech and language development has been improving now.

The other patient is an 18-month-old boy. Although NHS at 3 weeks of age demonstrated bilateral refer result, the ABR threshold was 40 dBnHL on the right ear and 30 dBnHL on the left ear. However, the thresholds had increased to 75 dBnHL and 70 dBnHL on respective ears at the age of 4 months, and hearing aids were begun to be used in both ears. Heterozygous mutation in the WFS1 gene (c.2051C>T: p.A684V) was detected by genetic testing at the age of 13 months. No symptoms other than hearing loss have not been found in both patients, and they have been observed by the department of otolaryngology, ophthalmology, and pediatrics.

Here we describe a pediatric case of cheilitis granulomatosa (CG) manifesting as swollen lips that mimicked oral allergy syndrome. A 13-year old girl with a chief complaint of painless swelling of the lips was referred to our outpatient ear, nose, and throat clinic. Histopathology of the first biopsy showed inflammatory changes with infiltration of lymphocytes and plasma cells but no evidence of granuloma. Additional clinical findings from imaging and laboratory examinations led to the diagnosis of CG. Initial conservative treatment reduced the swelling, but it soon recurred. Histopathological findings of granuloma were then found and surgical resection was performed. However, swelling of the lips has repeatedly recurred. CG is a manifestation of orofacial granulomatosis and has features similar to other granulomatous diseases such as Melkersson-Rosenthal syndrome, Crohn’s disease, and sarcoidosis, including histopathologic findings, prognosis, and unclear etiology. CG in children may develop into Crohn’s disease with oral involvement, and therefore long-term systemic follow-up of this condition is recommended.

We report two cases of early pediatric dysphagia that were successfully treated by a novel swallowing rehabilitation procedure using interferential current stimulation (IFC) of the neck.

These two cases developed aspiration during suckling and were managed with tube feeding.

Videofluoroscopy showed aspiration during and after the pharyngeal stage of swallowing, and showed the instability of swallowing-respiratory coordination.

Since the application of IFC stimulation to the neck provided an immediate improvement of the swallowing function, swallowing rehabilitation therapy using concurrent IFC stimulation to the neck was performed. At one month after the initiation of IFC therapy, they were capable of taking a whole feeding orally, without aspiration or penetration. We therefore propose that the combination of the environmental adjustments and the surface IFC stimulation to the neck has a potential role in rehabilitation therapy for pediatric dysphagia.