• Korean Journal of Audiology
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• v.25 no.4
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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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• v.25 no.4
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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 the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.12 no.2
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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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• v.16 no.4
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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.

• Phonetics and Speech Sciences
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• v.2 no.4
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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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• v.6 no.1
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• pp.8-24
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• 2009

Understanding the genetic background of hearing loss is important since almost 50% of the cases of profound hearing loss are caused by genetic factors. Until now, more than 150 causative genes have been identified. In this review, classification of genetic hearing loss (syndromic versus non-syndromic, recessive versus dominant, X-linked and mitochondrial), pitfalls in elucidating causative genes, anatomy of the inner ear, introduction of the most common syndromic hearing loss, introduction of the most common non-syndromic hearing loss-causing genes, mitochondrial and multifactorial hearing losses were discussed. Moreover, clinical approaches to the patients with hereditary hearing loss and genetic counseling were also explained briefly. Finally, future directions of the hereditary hearing loss research in Korean population were presented.

• Clinical and Experimental Pediatrics
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• v.50 no.1
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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.

• Phonetics and Speech Sciences
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• v.1 no.3
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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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• v.18 no.2
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• pp.328-336
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• 2011

Purpose: An association between very low birth weight infants(VLBWI) and hearing loss has long been recognized. Early identification and intervention for hearing loss benefits language and speech/cognitive development. We investigated the risk factors and clinical outcomes of hearing loss among VLBWI. Methods: We analyzed the results of auditory brainstem response (ABR) testing of VLBWI. These infants were admitted to the neonatal intensive care unit (NICU) of Pusan National University Yangsan Hospital between December 2008 and February 2011. A follow-up was conducted subsequently. Results: ABR evaluations were performed on 65 infants, and 31 showed abnormal results (47.7%). Among the 31 infants, 10 were classified with moderate/severe/profound hearing loss (15.4%). The infants with abnormal ABR had a higher incidence of low birth weight, prolonged ventilator care, cumulative dose of furosemide, and the lowest $PaO_2$ (P<0.05). Those with moderate/severe/profound hearing loss had a higher incidence of low Apgar scores at 5 minutes (odds ratio[OR],0.34; 95% confidence interval[CI],0.13-0.89), prolonged ventilator care (OR,1.06; 95% CI,1.01-1.12), and mild hearing loss compared to those without profound hearing loss. Follow-up evaluations on eight infants with ABR reveled improvements 5.6${\pm}$3.9 months later. One infant, who had profound hearing loss in both ears, used a hearing aid. Conclusion: Factors influencing hearing loss at the first VLBWI hearing screening test included lower Apgar scores at 5 min and prolonged use of a ventilator. Most VLBWI with hearing losses were expected to recover after several months of follow-up.

• Journal of Audiology & Otology
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• v.24 no.3
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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.