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Mutations in the OTOF gene are a common cause of hereditary hearing loss and the main cause of auditory neuropathy spectrum disorder (ANSD). Although it is reported that most of the patients with OTOF mutations have stable, congenital or prelingual onset severe-to-profound hearing loss, some patients show atypical clinical phenotypes, and the genotype–phenotype correlation in patients with OTOF mutations is not yet fully understood. In this study, we aimed to reveal detailed clinical characteristics of OTOF-related hearing loss patients and the genotype–phenotype correlation. Detailed clinical information was available for 64 patients in our database who were diagnosed with OTOF-related hearing loss. As reported previously, most of the patients (90.6%) showed a “typical” phenotype; prelingual and severe-to-profound hearing loss. Forty-seven patients (73.4%) underwent cochlear implantation surgery and showed successful outcomes; approximately 85–90% of the patients showed a hearing level of 20–39 dB with cochlear implant and a Categories of Auditory Performance (CAP) scale level 6 or better. Although truncating mutations and p.Arg1939Gln were clearly related to severe phenotype, almost half of the patients with one or more non-truncating mutations showed mild-to-moderate hearing loss. Notably, patients with p.His513Arg, p.Ile1573Thr and p.Glu1910Lys showed “true” auditory neuropathy-like clinical characteristics. In this study, we have clarified genotype–phenotype correlation and efficacy of cochlear implantation for OTOF-related hearing loss patients in the biggest cohort studied to date. We believe that the clinical characteristics and genotype–phenotype correlation found in this study will support preoperative counseling and appropriate intervention for OTOF-related hearing loss patients.

Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), a novel coronavirus, causes coronavirus disease 2019 (COVID-19). Otologic surgeries with drilling by powered instruments induce significant aerosols, which may induce SARS-CoV-2 transmission to medical staff if SARS-CoV-2 exists in the middle ear and mastoid cavity. During a COVID-19 pandemic, therefore, confirming a negative COVID-19 test prior to otologic surgery is recommended. However, previous coronavirus studies demonstrated that coronavirus was detected in the middle ear in some patients even though the polymerase chain reaction (PCR) test using their nasopharyngeal swab was negative. This study aimed to elucidate the probability of a positive SARS-CoV-2 PCR test in the middle ear or mastoid specimens from otologic surgery patients in whom SARS-CoV-2 was not detected by preoperative PCR test using a nasopharyngeal swab.We conducted a prospective, multicenter clinical study. Between April 2020 and December 2021, during the COVID-19 pandemic, 251 ears of the 228 participants who underwent otologic surgery were included in this study. All participants had no symptoms suggesting COVID-19 or close contact with a confirmed COVID-19 patient two weeks prior to the surgery. They were also negative in the SARS-CoV-2 PCR tests using a nasopharyngeal swab before surgery. We collected mucosa, granulation, bone dust with mucosa or fluid from the middle ear or mastoid for the SARS-CoV-2 PCR tests during each otologic surgery.The median age of the participants at surgery was 31.5 years old. Mastoidectomy using a powered instrument was conducted in 180 of 251 otologic surgeries (71.8%). According to intraoperative findings, active inflammation in the middle ear or mastoid cavities was evident in 20 otologic surgeries (8.0%), while minor inflammation was observed in 77 (30.7%). All SARS-CoV-2 PCR tests of otologic specimens showed a negative result. No patient suffered from COVID-19 within two months after otologic surgery. Furthermore, no hospital-acquired infections associated with otologic surgery occurred in our institutions CONCLUSIONS: Our results showed that PCR testing did not detect SARS-CoV-2 in middle ear and mastoid specimens, suggesting that the risk of transmission of SARS-CoV-2 is not high in otologic surgeries even using powered instruments when both clinical and laboratory tests are confirmed to be negative for COVID-19.

Clinical practice guidelines in Japan for tinnitus were published in May 2019. Effective treatment of tinnitus contributes to quality of life and may improve depression, anxiety, and cognitive function. The highest priority of treatment recommended by this guideline involves educational counseling, including an explanation of the mechanisms of tinnitus. Understanding tinnitus pathology has also been reported to be a therapeutically effective educational counseling strategy. Further, explaining how sound therapy such as tinnitus retraining therapy (TRT) works is effective. Psychological and cognitive behavioral therapies may be an option. Here, the hearing aid is coupled with a sound generator in order to administer sound therapy for patients with more-severe symptoms. In Japan, it can be difficult to perform psychotherapy alongside otorhinolaryngology, and exemplary sound therapy is more likely to be carried out. In any case, the purpose of treatment is not to eliminate tinnitus, but rather reduce distress that manifests in response to tinnitus by promoting sensory adaptation. Clinically, the most important subject is not the loudness of tinnitus, but the severity of tinnitus distress. TRT consists of educational counseling and sound therapy. The tinnitus handicap inventory (THI) is used to measure the distress level for tinnitus and to determine treatment priorities. Rehabilitation of tinnitus is important because anxiety and depression are often observed in patients with severe tinnitus. Additionally, drug administration should be avoided. As a surgical treatment, a cochlear implant can affect tinnitus symptoms. It is essential to establish a treatment policy according to the disease condition and degree of distress.

Vertebral artery (VA) injuries following trauma to the neck are uncommon. We treated a case of VA injury in an 82-year-old man presented to a hospital with penetrating neck injuries caused by broken glass and underwent surgery for retrieval of the pieces. After the surgery, he complained about hoarseness, computed tomography scan showed glass remnants in his neck, damaging the right VA and forming a pseudoaneurysm. Thereafter, he came to our hospital seeking treatment for the injured VA. Severe bleeding may have occurred during exploration around the pseudoaneurysm; hence, we performed interventional angiography and occluded the right VA, followed by neck surgery. Five days after embolization, we performed the surgery to remove the glasses. There was no bleeding during and after the operation, and the patient was discharged without any complication. In this case report, we discuss the management of VA injury and review the relevant medical literature.

Background High riding jugular bulb (HRJB) develops after 2 years and is rare at younger ages. High riding jugular bulb sometimes protrudes into the tympanic cavity, which can cause hemorrhagic complications during otologic surgery. Case Presentation We describe a congenitally deaf child with bilateral inner ear malformations and a right-sided HRJB on CT at 9 months. This child had undergone left cochlear implantation (CI) at 19 months, and right CI was planned at 6 years. However, we decided not to perform the right CI because preoperative CT images revealed that the right jugular bulb (JB) was enlarged and protruded into the tympanic cavity, completely covering the round window (RW). Conclusion This is the first pediatric case in which a longitudinal CT scan proves that HRJB develops and protrudes over time. These findings suggest that the earlier the HRJB forms, the larger it may grow. We must keep in mind the possibility of the enlargement and protrusion of HRJB.

Introduction Papillary thyroid carcinoma has a relatively good prognosis among malignant tumors of the thyroid. Therefore, a “wait and see” strategy is often adopted for patients with micropapillary thyroid carcinoma. On the other hand, patients with papillary thyroid carcinoma with N1b or M1 disease are known to show a poor prognosis. Here, we investigated the differences in the characteristics between patients with papillary thyroid microcarcinoma with and without N1b metastatic nodes/distant metastases to identify the risk factors for metastasis. Methods The retrospective study was performed in patients with thyroid microcarcinoma who were treated at the Kobe City Medical Center General Hospital from 2007 to 2017. The characteristics of the patients with thyroid microcarcinoma who were classified as having N1b or M1 disease (N1b/M1 group) were compared with those of patients with the same cancer classified as having N0, N1a, or M0 disease (N0/N1a group). Results A total of 65 patients were enrolled in this study; 12 were classified into the N1b/M1 group and 53 were classified into the N0/N1a group. The proportion of males was significantly higher in the N1b/M1 group than in the N0/N1a group. There were no statistically significant differences in the distribution of the tumor sites, the ultrasonographic findings, or the frequency of the presence of multiple carcinomas. Although three patients died due to other causes, there were no patients who died due to papillary thyroid carcinoma. Conclusion No significant predictors were identified for classifying patients with papillary thyroid microcarcinoma into the N1b/M1 group in this study. However, our findings suggested that male patients with papillary thyroid microcarcinoma require more careful follow-up in comparison to female patients with this cancer.

Objective The objective of this study was to evaluate safety and efficacy of regenerative treatment using gelatin sponge with basic fibroblast growth factor (bFGF) in patients with tympanic membrane perforation (TMP). Methods The current study was a prospective, multicenter, open-label, single-arm, and exploratory clinical trial to evaluate the safety and efficacy of the TM regeneration procedure (TMRP). Myringotomy was used to mechanically disrupt the edge of the TMP, and a gelatin sponge immersed in bFGF was then placed over the perforation. Fibrin glue was dripped over the sponge as a sealant. TMP closure was examined 4 weeks later and, if insufficient, TMRP was repeated a maximum of three more times. TMP closure and hearing improvement 12 weeks after the final TMRP as well as safety were evaluated. Results Of the 11 patients with TMP who participated in this study, one who fulfilled the exclusion criteria and did not undergo TMRP and one with cholesteatoma were excluded from the efficacy analysis. TMP closure and hearing improvement 12 weeks after the final TMRP were achieved in eight out of nine patients (88.9%). Mean bone conduction threshold significantly improved 12 weeks after the TMRP compared with baseline (35.7 ± 20.3 vs 29.4 ± 21.0 dB, P = 0.015). Six out of ten patients receiving TMRP experienced temporary adverse events: appendicitis (serious, severe), otorrhea (mild), otitis media (mild), and sudden hearing loss (mild). However, none were related to the protocol treatment. Conclusion TMP closure and hearing improvement were frequently confirmed following the TMRPs which were safely performed. These favorable outcomes were accompanied with significant improvement of the bone conduction threshold. These promising outcomes would encourage a large-scaled, randomized and pivotal clinical trial in the future. This trial is registered at http://www.umin.ac.jp/ctr/index.htm (identifier: UMIN000006585).

Background In Japan, there has been a lot of reports showing an association between facial flushing after light alcohol consumption and heterozygosity for inactive aldehyde dehydrogenase-2 (ALDH2). Persons with inactive ALDH2 may have a higher risk of alcohol-related oral, pharyngeal and esophageal cancers, compared with those with wild-type ALDH2. The purpose of this study was to examine whether flushers with oral or pharyngeal squamous cell carcinoma have an increased risk of synchronous or metachronous cancer of the upper gastrointestinal (UGI) tract. Methods A retrospective study was performed by medical chart review and through a questionnaire sent to 285 patients treated for oral and pharyngeal cancer. Responses were obtained from 150 patients (52.6%), who were classified as flushers or non-flushers, smokers (≥20 pack-year; 1 pack-years = number of cigarettes/20 per day) or non-smokers, and drinkers (≥14 units of alcohol consumption per week; 1 unit = 22 g) or non-drinkers. Relationships of these factors with occurrence of second primary cancers (SPCs) in the UGI tract were investigated. Results In Kaplan-Meier analysis, there was a significantly higher rate of SPC at 5 years in flushers and drinkers, but no relationship with smoking. In multivariate analyses, a history of flushing was significantly associated with SPC in the UGI tract (HR 2.64, 95% CI 1.25-5.52, P = 0.0109), but not with smoking or alcohol consumption. Conclusions A simple interview on history of facial flushing after alcohol intake can be useful for identifying patients at high risk for synchronous or metachronous cancers of the UGI tract.

Objectives An effect of audio-visual (AV) integration is observed when the auditory and visual stimuli are incongruent (the McGurk effect). In general, AV integration is helpful especially in subjects wearing hearing aids or cochlear implants (CIs). However, the influence of AV integration on spoken word recognition in individuals with bilateral CIs (Bi-CIs) has not been fully investigated so far. In this study, we investigated AV integration in children with Bi-CIs. Methods The study sample included thirty one prelingually deafened children who underwent sequential bilateral cochlear implantation. We assessed their responses to congruent and incongruent AV stimuli with three CI-listening modes: only the 1st CI, only the 2nd CI, and Bi-CIs. The responses were assessed in the whole group as well as in two sub-groups: a proficient group (syllable intelligibility ≥80% with the 1st CI) and a non-proficient group (syllable intelligibility Results We found evidence of the McGurk effect in each of the three CI-listening modes. AV integration responses were observed in a subset of incongruent AV stimuli, and the patterns observed with the 1st CI and with Bi-CIs were similar. In the proficient group, the responses with the 2nd CI were not significantly different from those with the 1st CI whereas in the non-proficient group the responses with the 2nd CI were driven by visual stimuli more than those with the 1st CI. Conclusion Our results suggested that prelingually deafened Japanese children who underwent sequential bilateral cochlear implantation exhibit AV integration abilities, both in monaural listening as well as in binaural listening. We also observed a higher influence of visual stimuli on speech perception with the 2nd CI in the non-proficient group, suggesting that Bi-CIs listeners with poorer speech recognition rely on visual information more compared to the proficient subjects to compensate for poorer auditory input. Nevertheless, poorer quality auditory input with the 2nd CI did not interfere with AV integration with binaural listening (with Bi-CIs). Overall, the findings of this study might be used to inform future research to identify the best strategies for speech training using AV integration effectively in prelingually deafened children.

Objective: To report on the safety and efficacy of an investigational active middle ear implant (AMEI) in Japan, and to compare results to preoperative results with a hearing aid. Design: Prospective study conducted in Japan in which 23 Japanese-speaking adults suffering from conductive or mixed hearing loss received a VIBRANT SOUNDBRIDGE with implantation at the round window. Postoperative thresholds, speech perception results (word recognition scores, speech reception thresholds, signal-to-noise ratio [SNR]), and quality of life questionnaires at 20 weeks were compared with preoperative results with all patients receiving the same, best available hearing aid (HA). Results: Statistically significant improvements in postoperative AMEI-aided thresholds (1, 2, 4, and 8 kHz) and on the speech reception thresholds and word recognition scores tests, compared with preoperative HA-aided results, were observed. On the SNR, the subjects' mean values showed statistically significant improvement, with-5.7 dB SNR for the AMEI-aided mean and-2.1 dB SNR for the preoperative HA-assisted mean. The APHAB quality of life questionnaire also showed statistically significant improvement with the AMEI. Conclusion: Results with the AMEI applied to the round window exceeded those of the best available hearing aid in speech perception as well as quality of life questionnaires. There were minimal adverse events or changes to patients' residual hearing., Otology & Neurotology, 38(6), e145-e151; 2017

The majority of hearing loss due to mumps presents as unilateral profound sensorineural hearing loss, which is refractory to treatment. In rare cases of bilateral total deafness, cochlear implants were beneficial for speech perception. Vaccination against mumps is recommended to prevent mumps-associated hearing loss.The objective of this study is to investigate the clinical characteristics of hearing loss due to mumps and to evaluate hearing outcomes.The clinical parameters were analyzed under a retrospective multi-institutional study design in patients diagnosed with hearing loss due to mumps at the Otolaryngology departments of 19 hospitals between 1987 and 2016.Sixty-seven patients with hearing loss due to mumps were enrolled. The study population consisted of 35 males and 32 females, ranging in age from 1 to 54, with a median age of 9.5 years. Sixty-three patients presented with unilateral, and 4 with bilateral hearing loss. Profound hearing loss was observed in 65 ears. Only one ear with severe hearing loss showed complete recovery. Four patients with bilateral hearing loss received cochlear implant surgery. Most of the patients with hearing loss due to mumps had no history of vaccination.

Objectives: A nationwide epidemiological survey involving 23 hospitals in Japan was conducted and the predictive values of demographic data were examined statistically. Methods: A total of 642 patients from 23 hospitals, including 20 university hospitals, in Japan were enrolled in the study. Age ranged from 8 to 87 years, and all were diagnosed with acute low-tone sensorineural hearing loss (ALHL) between 1994 and 2016. Demographic data for the patients, such as symptoms, gender, mean age, and distribution of ALHL grading, were collected and analyzed in relation to prognosis using Student’s t-test, χ2 test and logistic regression. Results: Female gender (p?.013), younger age (p?

Objectives: The aim of this study was to investigate the differences between idiopathic sudden sensorineural hearing loss (SSNHL), and acute low-tone sensorineural hearing loss (ALHL) using the results of a nationwide survey database in Japan and to analyze the variables associated with their clinical features and the severity of hearing impairment, treatment, and prognosis. Methods: Participants were patients registered between April 2014 and March 2016 in a multicenter epidemiological survey database involving 30 university hospitals and medical centers across Japan. Statistical analysis was performed to clarify the factors associated with their clinical characteristics and the severity of hearing impairment, treatment, and prognosis. Results: Idiopathic SSNHL and ALHL differed significantly in terms of male-to-female ratio, age distribution, and time from onset to start of treatment. The treatment methods and hearing prognosis also differed markedly between the two diseases. A majority (92%) of idiopathic SSNHL patients were administered some type of corticosteroid, while half of the ALHL patients received corticosteroids and a diuretic agent. Conclusion: The results suggested that idiopathic SSNHL and ALHL belonged to different categories of inner ear disease.

The aging Japanese population has led to an increase in the number of accidentally swallowed dentures. Therefore, we report the case of an elderly Japanese woman with dementia who accidentally and sequentially swallowed two dentures. The patient presented to our hospital with a diagnosis of a single impacted denture in her hypopharynx although we subsequently identified a second impacted denture in her rectum after taking a careful clinical history. Open surgery was required to remove the second denture from her rectum. This case highlights the importance of carefully considering the patient’s history if there is a possibility of latent swallowed dentures.

Objectives: To present the cardiovascular risk factors in idiopathic sudden sensorineural hearing loss (SSNHL) patients enrolled in a nationwide epidemiological survey of hearing disorders in Japan. Materials and methods: We compiled the cardiovascular risk factors in 3073 idiopathic SSNHL subjects (1621 men and 1452 women) and compared their proportions with controls as part of the National Health and Nutrition Survey in Japan, 2014. The cardiovascular risk factors consisted of drinking and smoking habits, a history of five conditions related to cardiovascular disease and body mass index. Results: The proportion of current smokers was significantly higher among men aged 50?59, 60?69 and 70+ and among women aged 30?39, 40?49 and 60?69. The proportion of patients with a history of diabetes mellitus was significantly higher among men aged 50?59, 60?69 and 70+, but not in women. In addition, male and female SSNHL subjects aged 60?69 showed lower proportions of current drinking; and female SSNHL subjects aged 60?69 showed higher proportions of overweight (BMI ?25?kg/m2). Conclusions: The present cross-sectional study revealed showed significantly higher proportions of current smokers among both men and women as well as those with a history of diabetes mellitus among men across many age groups in patients with idiopathic SSNHL compared with the controls.

Intersinus septal cells (ISSC) are defined as cells confined to the thin septum separating the frontal sinuses. In 2008, Som et al. first described four cases with a mucocele proved to have arisen in ISSC. Here, we report two cases with a mucocele in ISSC, representing the second report to appear in the English literature. Case 1 involved a mucocele in the center of the frontal lesion with rudimentary bilateral frontal sinuses, which suggested that the ISSC mucocele occurred during very early development of the frontal sinuses. Case 2 concerned a mucocele in the unilocular frontal sinus invading the left orbit. The frontal sinus septum was destroyed with partial ‘Y’-shaped remnants, which implied the ballooning and bursting of the ISSC mucocele into both sides of the frontal sinus. The two different mechanisms of development of the ISSC mucoceles may vary in relation to the time of onset.

Objectives: To evaluate the differences between acoustic trauma (AT) and other types of acute noise-induced hearing loss (ANIHL), we performed a literature search and case reviews. Methods: The literature search based on online databases was completed in September 2016. Articles on ANIHL and steroid treatment for human subjects were reviewed. The source sounds and treatment sequelae of our accumulated cases were also reviewed. Hearing loss caused by gun-shots and explosions was categorized into the AT group, while hearing loss caused by concerts and other noises was categorized into the ANIHL group. Results: Systemic steroid treatment did not appear to be effective, at least in the AT group, based on both the literature and our case reviews. However, effective recovery after treatment including steroids was observed in the ANIHL group. The difference in hearing recovery between the AT and ANIHL groups was statistically significant (p?=?.030), although differences in age, days from the onset to treatment and pretreatment hearing levels were not significant. Conclusions: Hearing recovery from AT is very poor, whereas, ANIHL is recoverable to some extent. Therefore, it is essential to differentiate between these two groups for accurate prediction of the hearing prognosis and evaluation of treatment effects.

Objectives: To investigate the hearing prognosis of idiopathic sudden sensorineural hearing loss (SSNHL) treated with different initial therapies. Methods: Subjects consisted of patients diagnosed with idiopathic SSNHL within 7 days from onset and showing severe hearing loss (?60?dB), who were registered in a Japanese multicenter database between April 2014 and March 2016. Subjects were divided into four groups according to initial therapy: (1) steroids, (2) steroids?+?Prostaglandins (PGs), (3) intratympanic steroids (ITS), and (4) no steroids. Hearing outcomes were compared among the groups. Results: In total, 1305 patients were enrolled. The final hearing level and hearing gain of patients treated with steroids?+?PGs were significantly higher than those of patients treated with steroids alone or no steroids. The ratio of good prognosis (complete recovery or marked improvement) in patients treated with steroids?+?PGs was higher than that in patients treated with steroids alone or no steroids. There was no difference in the prognosis of patients treated with steroids alone or no steroids. Conclusion: A large number of patients with idiopathic SSNHL were registered in a multicenter database. PG use in combination with steroid administration was associated with a good hearing prognosis in patients with severe hearing loss.

The OTOF gene (Locus: DFNB9), encoding otoferlin, is reported to be one of the major causes of non-syndromic recessive sensorineural hearing loss, and is also reported to be the most common cause of non-syndromic recessive auditory neuropathy spectrum disorder (ANSD). In the present study, we performed OTOF mutation analysis using massively parallel DNA sequencing (MPS). The purpose of this study was to reveal the frequency and precise genetic and clinical background of OTOF-related hearing loss in a large hearing loss population. A total of 2,265 Japanese sensorineural hearing loss (SNHL) patients compatible with autosomal recessive inheritance (including sporadic cases) from 53 otorhinolaryngology departments nationwide participated in this study. The mutation analysis of 68 genes, including the OTOF gene, reported to cause non-syndromic hearing loss was performed using MPS. Thirty-nine out of the 2,265 patients (1.72%) carried homozygous or compound heterozygous mutations in the OTOF gene. It is assumed that the frequency of hearing loss associated with OTOF mutations is about 1.72% of autosomal recessive or sporadic SNHL cases. Hearing level information was available for 32 of 39 patients with biallelic OTOF mutations; 24 of them (75.0%) showed profound hearing loss, 7 (21.9%) showed severe hearing loss and 1 (3.1%) showed mild hearing loss. The hearing level of patients with biallelic OTOF mutations in this study was mostly severe to profound, which is consistent with the results of past reports. Eleven of the 39 patients with biallelic OTOF mutations had been diagnosed with ANSD. The genetic diagnosis of OTOF mutations has significant benefits in terms of clinical decision-making. Patients with OTOF mutations would be good candidates for cochlear implantation; therefore, the detection of OTOF mutations is quite beneficial for patients, especially for those with ANSD.

When sizing the femoral component or determining its placement in total knee arthroplasty (TKA), if the anterior–posterior diameter of the femoral condyle is between component sizes, the selected size will differ depending on whether anterior referencing (AR) or posterior referencing (PR) is used. As a result, the amount of resected bone will also vary. In the present prospective study, we compared the two referencing methods to determine which is more suitable for individual patients. We recruited 58 patients (92 joints) who received TKA using the standard technique with intermediate-size components. AR was used in 26 joints, and PR in 23 joints. Seventeen of the patients underwent same-day bilateral TKA in which components of different sizes were used for the left and right joints. AR resulted in significantly smaller anterior and posterior offsets than PR. Preoperative clinical evaluation revealed no significant differences among cases in which intermediate-size components were indicated, or those in which components of different sizes were indicated. When an intermediate-sized component was indicated using the AR method, moving the sizer forward resulted in a larger posterior gap, but this technique was nevertheless considered acceptable. AR is likely to be more suitable than PR as it achieves more physiological anterior clearance.

To investigate the McGurk effect in profoundly deafened Japanese children with cochlear implants (CI) and in normal-hearing children. This was done to identify how children with profound deafness using CI established audiovisual integration during the speech acquisition period.Twenty-four prelingually deafened children with CI and 12 age-matched normal-hearing children participated in this study. Responses to audiovisual stimuli were compared between deafened and normal-hearing controls. Additionally, responses of the children with CI younger than 6 years of age were compared with those of the children with CI at least 6 years of age at the time of the test.Responses to stimuli combining auditory labials and visual non-labials were significantly different between deafened children with CI and normal-hearing controls (p0.05). Additionally, the McGurk effect tended to be more induced in deafened children older than 6 years of age than in their younger counterparts.The McGurk effect was more significantly induced in prelingually deafened Japanese children with CI than in normal-hearing, age-matched Japanese children. Despite having good speech-perception skills and auditory input through their CI, from early childhood, deafened children may use more visual information in speech perception than normal-hearing children. As children using CI need to communicate based on insufficient speech signals coded by CI, additional activities of higher-order brain function may be necessary to compensate for the incomplete auditory input. This study provided information on the influence of deafness on the development of audiovisual integration related to speech, which could contribute to our further understanding of the strategies used in spoken language communication by prelingually deafened children.