• 대한청각학회지
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• 제25권4호
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• pp.235-240
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• 2021

Sudden sensorineural hearing loss (SSNHL) is a common disorder; however, sequential, bilateral presentation of the disease is rarer than unilateral presentation. Clinical otologists usually focus on treating the side with impaired hearing when patients first present with unilateral SSNHL, and therefore, may not warn patients of the possibility of subsequent hearing impairment in the contralateral ear. Furthermore, it is professionally discouraging when a patient presents with profound, sequential SSNHL after initial treatment. This may adversely impact the doctor-patient relationship, even if the patient is offered the best possible care from their first visit. Herein, we report the case of a patient with profound, idiopathic, bilateral SSNHL with a time interval of 37 days between involvement of both ears. Even though high-dose steroids were administered intraorally and intratympanically, the patient's hearing was not restored, and the patient eventually required bilateral cochlear implant surgery. Our report demonstrates that sequential, profound, bilateral SSNHL may manifest without any specific signs.

• Journal of Audiology & Otology
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• 제25권4호
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• pp.235-240
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• 2021

Sudden sensorineural hearing loss (SSNHL) is a common disorder; however, sequential, bilateral presentation of the disease is rarer than unilateral presentation. Clinical otologists usually focus on treating the side with impaired hearing when patients first present with unilateral SSNHL, and therefore, may not warn patients of the possibility of subsequent hearing impairment in the contralateral ear. Furthermore, it is professionally discouraging when a patient presents with profound, sequential SSNHL after initial treatment. This may adversely impact the doctor-patient relationship, even if the patient is offered the best possible care from their first visit. Herein, we report the case of a patient with profound, idiopathic, bilateral SSNHL with a time interval of 37 days between involvement of both ears. Even though high-dose steroids were administered intraorally and intratympanically, the patient's hearing was not restored, and the patient eventually required bilateral cochlear implant surgery. Our report demonstrates that sequential, profound, bilateral SSNHL may manifest without any specific signs.

• 대한후두음성언어의학회지
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• 제12권2호
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• pp.140-144
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• 2001

Backgrounds & Objectives : Prolonged hearing loss was considered as one of the factors which have the potential to cause vocal changes. However, the analysis of quality of phonation in hearing loss patients has not been achieved enough. The purpose of the study was to evaluate the difference in objective acoustic parameters between long-term hearing impaired patients and normal control group. Material & Methods : The material of this investigation comprised a group of 20 patients (M : F=10 : 10) with moderate or profound hearing loss(over 50dB). The duration of all hearing loss was over 1 year. All of them underwent the acoustic examinations comprising electroglottography, multidimensional voice program and formant analysis during phonation of the bowels /a/ with free confortable tone and /i/ with voluntary high tone. The results of the acoustic examinations were compared with those of a control group, composed of 20 sex- and age-matched normal hearing subjects. Results : In the male hearing loss subjects, the significant increase was detected in pitch and shimmer during phonation of /a/ and in pitch during phonation of /i/. In addition, this group was characterized by decreased fundamental frequency during phonation of /i/. In female, there was no difference between hearing loss group and normal control group except a decreased formant 1 frequency. Conclusion : Long-term moderate and profound sensorineural hearing loss could affect the objective voice parameters.

• Genomics & Informatics
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• 제16권4호
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• pp.20.1-20.20
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• 2018

Genetic hearing loss crosses almost all the categories of hearing loss which includes the following: conductive, sensory, and neural; syndromic and nonsyndromic; congenital, progressive, and adult onset; high-frequency, low-frequency, or mixed frequency; mild or profound; and recessive, dominant, or sex-linked. Genes play a role in almost half of all cases of hearing loss but effective treatment options are very limited. Genetic hearing loss is considered to be extremely genetically heterogeneous. The advancements in genomics have been instrumental to the identification of more than 6,000 causative variants in more than 150 genes causing hearing loss. Identification of genes for hearing impairment provides an increased insight into the normal development and function of cells in the auditory system. These defective genes will ultimately be important therapeutic targets. However, the auditory system is extremely complex which requires tremendous advances in gene therapy including gene vectors, routes of administration, and therapeutic approaches. This review summarizes and discusses recent advances in elucidating the genomics of genetic hearing loss and technologies aimed at developing a gene therapy that may become a treatment option for in the near future.

• 말소리와 음성과학
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• 제2권4호
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• pp.229-238
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• 2010

This study investigates the differences in acoustic parameters in vowel space across hearing loss, gender and vowels. The parameters include F1, F2, Euclidean Distance between vowels, and vowel triangular area comprised of /i/, /a/ and /u/. For this study, 20 hearing-impaired and normal hearing adults as a control group were asked to read 7 Korean vowels (/a, $\wedge$, o, u, w, i, $\varepsilon$/). Subjects' readings were recorded by NasalView and analyzed by Praat. Results showed that F1 were significantly higher in the hearing impaired group than in the normal hearing group, higher in the female group than in male group, and higher in low vowels than in high vowels. And the means of F2 was significantly higher in the hearing impaired group than in normal hearing group, higher in high vowels than in low vowels, and there was no difference between male and female group. Secondly, Euclidean distance between vowels was significantly shorter in the hearing-impaired group than in the normal group. Finally, acoustic vowel space area was significantly smaller in the hearing-impaired group than in the normal hearing group. The hearing-impaired group showed that front vowels tended to be backed and back vowels to be fronted.

• Journal of Genetic Medicine
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• 제6권1호
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• pp.8-24
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• 2009

난청의 약 50%는 유전적 요소가 원인이기 때문에 난청의 유전적 배경을 이해하는 것은 중요하다. 현재까지 150개 이상의 원인유전자들이 밝혀져 있다. 이번 종설에서는 유전성 난청의 분류, 유전성 난청 원인규명의 난점, 유전성 난청과 관련된 내이의 구조와 기능, 증후군성난청, 비증후군성난청, 미토콘드리아 유전성 난청, 그리고 다인자성 난청에 관해 논하고자 한다. 그리고 유전성 난청을 가진 환자의 치료적 접근과 유전적 상담을 간략하게 설명하고, 마지막으로 유전성 난청에 대한 앞으로의 연구 방향을 제시하고자 한다.

• Clinical and Experimental Pediatrics
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• 제50권1호
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• pp.7-13
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• 2007

Hearing loss in newborns is the most frequently occurring birth defect. If hearing impaired children are not identified and managed early, it is difficult for many of them to acquire the fundamental language, social and cognitive skills that provide the foundation for later schooling and success in society. All newborns, both high and low risk, should be screened for hearing loss in the birth hospital prior discharge (Universal Newborn Heaing Screening, UNHS). Objective physiologic measures must be used to detect newborns and very young infants with hearing loss. Recent technological developments have produced screening methods and both evoked otoacoustic emission (EOAE) and auditory brainstem response (ABR) have been successfully implemented for UNHS. Audiologic evaluation should be carried out before 3 months of age and infants with confirmed hearing loss should receive intervention before 6 months of age. All infants who pass newborn hearing screening but who have risk indicators for other auditory disorders and/or speech and language delay receive ongoing audiologic surveillance and monitoring for communication development. Infants with sensorineural hearing loss are managed with hearing aids and receive auditory and speech-language rehabilitation therapies. Cochlear implants can be an outstanding option for certain children aged 12 months and older with severe to profound hearing loss who show limited benefit from conventional amplifications.

• 말소리와 음성과학
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• 제1권3호
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• pp.123-132
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• 2009

This study investigates the differences in nasalance across handicap, gender, and vowels and the correlation between nasal energy and oral energy both of which are used to compute nasalance. For this study, 20 hearing-impaired adults and 20 normal hearing adults as a control group were asked to read 7 Korean vowels (/$\alpha$, $\Lambda$, o, u, ɯ, i, $\varepsilon$/). Subjects' readings were recorded by NasalView and analyzed by Praat. Results showed that the hearing impaired group (HL) has a significantly higher nasalance than the normal hearing group(NH), and that there was a significant positive correlation between nasal energy and oral energy. A higher nasalance of the hearing impaired group seems to be due to an improper velopharyngeal control which is caused by lack of a proper auditory feedback.

• Neonatal Medicine
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• 제18권2호
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• pp.328-336
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• 2011

목적: 양측 고도 난청은 신생아 집중치료실에서 치료를 하였던 신생아나 미숙아인 경우 위험률이 증가한다. 이에 저자들은 1,500 g 미만으로 출생한 미숙아를 대상으로 난청을 유발할 수 있는 인자들을 알아보고자 하였다. 방법: 2008년 12월부터 2011년 2월까지 부산대학교 어린이병원 신생아 중환자실에 입원한 1,500 g 미만의 미숙아 65명을 대상으로 난청의 위험 인자들의 의무 기록을 후향적으로 조사하였다. 청력 검사는 교정 연령 37주 이상이 되었을 때 ABR로 시행하였으며, 처음 검사에서 정상 청력인 환아와 난청인 환아로 분류하였다. 결과: 65명의 환아 중에서 34명은 정상으로 나왔고, 31명은 난청을 보였다(47.7%). 난청을 보인 환아 중에서 일상 생활에 지장을 주는 중도 이상의 난청을 가진 환아는 10명(15.4%) 이었다. 이들의 위험인자를 비교해본 결과 비정상군에서 청력 검사를 시행했던 시기가 늦었고, 출생 체중이 적었으며, 기계 환기를 했던 기간이 길었고, 생후 1주일간 동맥혈 가스 분석 결과 최저 산소 분압이 낮았고, 이뇨제의 누적 용량이 의미 있게 많았다 (P<0.05). 이 위험 인자를 경도 난청과 중/고도 난청/농으로 나누고 출생 체중으로 보정한 다인자 분석을 하니 5분 Apgar 점수가 증가할 때마다 중/고도 난청/농의 가능성은 낮아지고 (OR=0.34, 95% CI: 0.13-0.89), 기계 환기의 기간이 길어질 때마다 중/고도 난청/농의 가능성은 증가하였다(OR=1.06, 95% CI: 1.01-1.12). 31명의 난청 환아 중에 추적 검사를 시행한 17명 중 16명이 호전을 보였고(94.1%), ABR로 재검을 한 8명 중 7명은 호전이 될 때까지 평균 5.6개월이 소요되었으며, 1명만이 양측 농으로 나와 보청기를 착용하였다. 결론: 1,500 g 미만의 미숙아에게 처음 시행한 청력 선별 검사에서 중도 이상의 난청에 영향을 미치는 인자들은 낮은 5분 Apgar점수와 장기간의 인공 호흡기 사용이었고, 대부분이 5-6개월 이내에 청력에 호전을 보여 수개월간은 추적 검사를 시행하면서 회복되기를 기다려도 될 것으로 생각된다.

• Journal of Audiology & Otology
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• 제24권3호
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• pp.127-132
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• 2020

Background and Objectives: In this study, we compared the outcomes of patients with idiopathic sudden sensorineural hearing loss who underwent steroid treatment with or without hyperbaric oxygen (HBO) therapy and were followed-up in our clinic. Subjects and Methods: Patients were divided into two groups according to their treatment regimen. Steroid group received intravenous 1 mg/kg methylprednisolone which was due to be completed in 2-3 weeks with decreasing doses, and five doses of 0.5 mL intratympanic dexamethasone. Steroid+HBO group received the same steroid treatment with the addition of HBO therapy. The audiologic results of both treatment groups were compared after considering the patients' risk factors. Results: There was no significant difference between the steroid and Steroid+HBO groups in terms of hearing gain and degree of recovery, both at all degrees of hearing loss, and in severe and profound hearing loss. Hearing gain was similar when evaluated by audiogram type and admission time in both treatment groups. Conclusions: We found that the addition of HBO therapy to systemic plus intratympanic steroid treatment did not affect hearing gain at all degrees of hearing loss in this study. Furthermore, audiogram type and admission time did not affect hearing gain between the two groups.