Genetic testing and diagnosis are becoming important in medical practice. Hearing loss is the most frequent hereditary disease among monogenic genetic disorders in the field of otolaryngology. Genetic testing for congenital hearing loss and young adult-onset bilateral sensorineural hearing loss is reimbursable by the social health insurance system in Japan. Therefore, it is important to have a thorough knowledge about hereditary hearing loss and current medical practice for patients with hereditary hearing loss.

　In more than half of the cases, congenital hearing loss is of hereditary causation; hereditary hearing loss is divided into nonsyndromic and syndromic types. So far, over 120 causative genes of nonsyndromic hereditary hearing loss have been discovered. Pathogenic variants of the GJB2 gene, which encodes connexin 26, are the most common cause of congenital hearing loss. Identification of the genetic etiology is useful for hearing loss/genetic counseling, as well as for predicting the prognosis estimating the potential usefulness of cochlear implantation, etc.

　Young adult-onset bilateral sensorineural hearing loss is a designated intractable disease, and genetic testing is essential for its diagnosis. So far, only the following seven causative genes have been confirmed: ACTG1, CDH23, COCH, KCNQ4, TECTA, TMPRSS3, and WFS1; however, EYA4, MYO6, MYO15A, and POU4F3 are also expected to be added in the near future.

　 We examined the relationship between speech perception in noise and the auditory attention ability in preschool children. A total of 47 preschool children aged 3 years 6 months to 6 years 5 months (mean: 5 years 1 months, SD=0.88 years) participated in this study. Speech perception in noise was measured by the single digit number repetition task in real-life classroom noise. Combinations of two signal-to-noise (SN) ratio conditions (0dB, -6dB) and two stimulus presentation timings (i.e., constant, random) were used in the task. The auditory attention ability was assessed using the Auditory Continuous Performance Test for Preschoolers (ACPT-P). In the number repetition task, the task performance in the -6dB condition was lower than that in the 0dB condition. Furthermore, the task performance increased with age. However, there was no significant difference in the task performance depending on the stimulus presentation timing condition. In the ACPT-P, the response latency and its variability were significantly shortened and decreased, respectively with age. There was a significant negative correlation between the task performance in the number repetition task and the reaction time in the ACPT-P under a low SN ratio and random stimulus presentation timing condition. These results suggest that auditory attention might be related to the listening ability in noise.

　Using the results of a questionnaire survey on the work life and issues of hearing-impaired teachers, we constructed an evaluation scale consisting of 40 items and confirmed the reliability, such as the content validity, construct validity, and internal consistency of this scale. From the item contents of this scale, it was interpreted that its score corresponds to the level of participation restriction, response behavior, and mental health according to the International Classification of Functioning, Disability and Health (ICF, WHO).

　Using this scale, we conducted a multiple regression path analysis to examine the factors determining participation restrictions in educational settings in hearing-impaired teachers working at schools for students with special hearing needs and at regular schools. The results identified limited auditory information, other staff's level of understanding of disabilities, the educational relationships with children and students, relationships with their parents, and educational effect as significant factors; they confirmed that resolution of issues should involve the formulation of codes of conduct to respond to disabilities by fostering awareness of the significance of the educational profession. Under the current circumstances, with the development of a more inclusive educational environment, this scale was suggested as being effective to assess the functioning and environmental development of hearing-impaired teachers.

　We examined the hearing management status in early childhood and the hearing level of good-side ears at age 3 and older in 65 children who were detected to have unilateral hearing loss at the first examination after newborn hearing screening.

　Of the 65 children, hearing management had been continued until the age of 3 years or older in 42 cases (65%), hearing management had been completed before the age of 3 years due to normalization of hearing bilaterally in 14 cases (22%), and hearing management had been interrupted because the children stopped visiting our center despite the absence of any change in the status of the unilateral hearing loss in 9 cases (14%).

　The average hearing level at the final visit (average age 63.6±15.7 months) in the children who had received continuous hearing management up to 3 years of age or later was less than 25 dB in 90% (38/42) of cases. There were 4 cases of hearing loss (1 case of mild hearing loss, 1 case of moderate hearing loss, 2 cases of severe hearing loss), of which the three cases with moderate or more severe hearing loss required hearing aids (even though two of the parents were unaware that the hearing loss in their child had changed from unilateral to bilateral).

　Hearing on the normal side of children with unilateral hearing loss rarely deteriorates in early childhood, but when it does, it is not always easy for parents to notice. Therefore, it was reconfirmed that it is important for the detailed inspection organization to follow-up regularly.

Objective: To examine the frequency of comorbid headache in patients with tinnitus, frequency of headache associated with tinnitus, and the relationship between the presence of headache and the severity of tinnitus.

Methods: This retrospective study was conducted in new patients who visited our clinic with the chief complaint of tinnitus. Patients were asked whether they had recurring headache, and to complete two questionnaires: the Tinnitus Handicap Inventory (THI) to assess the severity of tinnitus, and the Hospital Anxiety and Depression Scale (HADS) to assess depression and anxiety.

Results: Of the 126 patients with tinnitus enrolled, 38 (30.2%) had comorbid headache. In 16 patients (42.1%), the tinnitus was associated with headache. There was no significant difference in the THI or HADS score between patients with and without headache, (p=0.501 and 0.317, respectively). On the other hand, the THI and HADS scores were significantly higher (p=0.046 and 0.032, respectively) in patients in whom the tinnitus was associated with headache that in the other patients.

Conclusion: It is not uncommon for patients with tinnitus to have headache. Patients in whom the tinnitus is associated with headache are more likely to have severe tinnitus and depression.

　We developed a listening comprehension test for long sentences based on a speech corpus and evaluated an automatic scoring system. A 100 sentences of 5, 6, and 7 syllables were extracted from a speech corpus based on the ATR (Advanced Telecommunications Research Institute International) phoneme-balanced sentences in the speech corpus of the Acoustical Society of Japan built from newspaper articles. A total of 34 patients admitted for orthopedic surgery with no cognitive impairment were requested to take a dictation task of 10 sentences per day for 10 days. After the dictation, the handwritten data were converted to text data, and the percentages of correct sentences, syllables, phonemes, vowels, and consonants were automatically scored using programs based on Python, CaboCha, MeCab, KyTea, and KAKASI. The correct rates of sentences, syllables, phonemes, vowels, and consonants for the total of 100 sentences were 0.66±0.22, 0.78±0.17, 0.81±0.14, and 0.81±0.14, respectively. This system could be a useful basic tool for the development and evaluation of listening comprehension tests based on new speech corpuses.