Evaluation of Digits-in-Noise Test and Hearing Handicap Inventory for Adults Screening in Patients with Occupational Noise-Induced Hearing Loss

Objective:To explore the clinical evaluation role of the Digits-in-Noise(DIN)test and Hearing Handicap Inventory for Adults Screening(HHIA-S)for patients with occupational noise-induced hearing loss and to observe and analyze their application values.Methods:Fifty patients with suspected occupational noise-induced hearing loss were randomly selected from the Department of Otolaryngology at the hospital as the research target.The collection period for the research cases spanned from January 2022 to November 2023,and all patients had a history of noise exposure.The DIN test and HHIA-S were used for hearing examinations,with clinical,comprehensive diagnosis serving as the gold standard to study their diagnostic performance.Results:The compliance rate of the DIN test was 88.00%,the HHIA-S’s compliance rate was 80.00%,and the combined compliance rate was 94.00%.The compliance rate of the DIN test and the combined compliance rates of the patients were statistically significant compared to the clinical gold standard data(P<0.05),while there was no difference between the compliance rate of the HHIA-S and the gold standard(P>0.05).The data shows that the sensitivity of the combined diagnosis is significantly higher than the sensitivity data of the DIN test and HHIA-S examination alone(P<0.05).Its specificity is 100.00%,and the accuracy data of the joint diagnosis in the degree were higher than those of the DIN test alone(P>0.05)and the HHIA-S alone(P<0.05).Conclusion:For patients with occupational noise-induced hearing loss,the joint evaluation of the DIN test and HHIA-S can significantly improve their diagnostic value with high sensitivity and accuracy.

Effects of Environmental Noise on School Performance among Hearing-Impaired Students

Background and Purpose: Our study examined the effect of ambient noise on hearing and school performance in children with hearing loss, and the relationship between hearing loss and cognition. Method: A pilot study involving 728 people (aged 11 to 16 years) was conducted in Kinshasa, Democratic Republic of the Congo. All participants completed the ENAFEP test, the SIFTER short scale, pure tone audiometry, and a three-digit test. Teachers and investigators completed a 6-day listening training course. Results: Correlation and structural equation modeling indicated that ambient noise and five cognitive domains were associated with hearing in noise, but only unmeasured cognition was associated with hearing loss and poor academic performance. Cognitive compensation significantly reduces communication problems and improves concentration and academic performance. Noise and deafness had significant and moderate effects on academic performance (r = -0.13). Conclusion: The sample size and relatively high participation rate meant the study was representative. However, the results showed an association between ambient noise, hearing loss and cognition, and reduced school performance. Large-scale randomized interventions for hearing loss and long-term noise exposure studies are needed to measure cognitive outcomes after short- and multi-year noise exposure.

Educational Assistance Through Modeling in Tsukuba University of Technology-Professional Education for the Hearing or Visually Impaired

The National University Corporation Tsukuba University of Technology（NTUT） is the only institute of higher education for the hearing and the visually impaired in Japan. In our university, hearing or visually impaired students are studying to be technicians after they graduate, toward social independence. From previous experience of higher education for students with disabilities, effects are increased when modeling is used by the teachers involved in professional education. In the Mechanical Engineering Course, we are using modeling, to match the drawing and shape for beginning students. It includes support for enhancing one＇s view, and how to draw out the ability of mechanical engineering students for the basics. For students to study Mechanical Design and Drawing, Modeling of Gear Pump, Jack and Globe Valve are easily shown through drawings and the operation of each mechanism through sample drawings in the textbook. It is possible to make an opportunity to think about the machine mechanism. It will be shown by students＇ works. The assembling of the model triggers the need for form accuracy by making a function, and improves the quality of learning. It is possible that a three-dimensional molding machine can be produced through experiential learning by the model, and modeling with the dimension numerical data. Moreover, it is also embodied in a three-dimensional modeling which results in the image processing programming created. Confirming the improvement of the program through the shape with the quality. In the Department of Synthetic Design, students have chances to realize and self-evaluate from the design of the lamp shade with a complicated shape. In the Faculty of Health Science from Department of Health, high quality teaching of visually-impaired students through the use of bone model teaching materials has become possible in the medical-related courses.

Current trends in outcome studies for children with hearing loss and the need to establish a comprehensive framework of measuring outcomes in children with hearing loss in China

Since the 1970s, outcome studies for children with hearing loss expanded from focusing on assessing auditory awareness and speech perception skills to evaluating language and speech development. Since the early 2000s, the multi-center large scale research systematically studied outcomes in the areas of auditory awareness, speech-perception, language development, speech development, educational achievements, cognitive development, and psychosocial development. These studies advocated the establishment of baseline and regular follow-up evaluations with a comprehensive framework centered on language development. Recent research interests also include understanding the vast differences in outcomes for children with hearing loss, understanding the relationships between neurocognitive development and language acquisition in children with hearing loss, and using outcome studies to guide evidence-based clinical practice. After the establishment of standardized Mandarin language assessments, outcomes research in China's Mainland has the potential to expand beyond auditory awareness and speech perception studies.

A comparison of the quality of life of parents of children using hearing aids and those using cochlear implants

Objective:The goal of this study was to evaluate the quality of life of parents of children who use hearing aids(HA)with those who use cochlear implants(CI)in the Indian context and document any differences found.Methods:The Kannada version of the AQoL-4D was administered in a modified fashion to 131 parents(87 HA and 44 CI).Sociodemographic details were collected for supplemental information on the intervention strategy used.Results:A total of 49 parents(29 HA and 20 CI)responded to the questionnaire sent.The mean total scores for both the groups were similar(HA group=17.9(SD=5.5),CI group=17.2(SD=3.4)),as was the score for the first subscale(HA group=8.6(SD=2.9);CI group=8.5(SD=2.6))of the AQoL-4D.No significant differences were found between the two groups on either scores[Total Score:U(N_(HA)=29,NCI=20)=280.5,z=0.194,p>0.05;Subscale 1 Score:U(N_(HA)=29,NCI=20)=281.5,z=-0.176,p>0.05].The degree of hearing loss in the hearing aid group was equivalent to that of the cochlear implant group but this did not appear to influence parental quality of life.Conclusion:Parents of children with hearing aids and cochlear implants appear to be similar on several psychosocial factors in the realms of functional,social,and psychological well-being.In terms of parental quality of life,hearing aids and cochlear implants appear to be equally effective intervention techniques.

Speech Intelligibility Enhancement Algorithm Based on Multi-Resolution Power-Normalized Cepstral Coefficients(MRPNCC)for Digital Hearing Aids

Speech intelligibility enhancement in noisy environments is still one of the major challenges for hearing impaired in everyday life.Recently,Machine-learning based approaches to speech enhancement have shown great promise for improving speech intelligibility.Two key issues of these approaches are acoustic features extracted from noisy signals and classifiers used for supervised learning.In this paper,features are focused.Multi-resolution power-normalized cepstral coefficients(MRPNCC)are proposed as a new feature to enhance the speech intelligibility for hearing impaired.The new feature is constructed by combining four cepstrum at different time–frequency(T–F)resolutions in order to capture both the local and contextual information.MRPNCC vectors and binary masking labels calculated by signals passed through gammatone filterbank are used to train support vector machine(SVM)classifier,which aim to identify the binary masking values of the T–F units in the enhancement stage.The enhanced speech is synthesized by using the estimated masking values and wiener filtered T–F unit.Objective experimental results demonstrate that the proposed feature is superior to other comparing features in terms of HIT-FA,STOI,HASPI and PESQ,and that the proposed algorithm not only improves speech intelligibility but also improves speech quality slightly.Subjective tests validate the effectiveness of the proposed algorithm for hearing impaired.

The impact of hearing impairment and noise-induced hearing injury on quality of life in the active-duty military population:challenges to the study of this issue

The objectives of this review were to 1)summarize the available evidence on the impact of hearing loss on quality of life(QOL)among U.S.active-duty service members,2)describe the QOL instruments that have been used to quantify the impact of hearing loss on quality of life,3)examine national population-level secondary databases and report on their utility for studying the impact of hearing loss on QOL among active-duty service members,and 4)provide recommendations for future studies that seek to quantify the impact of hearing loss in this population.There is a lack of literature that addresses the intersection of hearing impairment,the military population,and quality of life measures.For audiological research,U.S.military personnel offer a unique research population,as they are exposed to noise levels and blast environments that are highly unusual in civilian work settings and can serve as a model population for studying the impact on QOL associated with these conditions.This review recommends conducting a study on the active-duty service member population using a measurement instrument suitable for determining decreases in QOL specifically due to hearing loss.

Acceptable noise level as a deciding factor for prescribing hearing aids for older adults with cochlear hearing loss-A scoping review

Older adults often find it difficult to perceive speech, especially in noisy conditions. Though hearing aid is one of the rehabilitative devices available to older adults to alleviate hearing loss, some of them may experience annoyance through hearing aid and hence reject it, may be due to circuitry noise and/or background noise. Acceptable noise level is a direct behavioural measure to estimate the extent of how much a person is able to put up with noise while simultaneously listening to speech. Acceptable noise level is a central auditory measure and it is not influenced by age, gender, presentation level or speaker. Using this measure, we can quantify the annoyance level experienced by an individual. This in-formation is of utmost importance and caution should be paid before setting the parameters in hearing aid, especially for those who are unable to accept noise. In this review article, an attempt has been made to document how to optimize the hearing aid program by setting parameters such as noise reduction circuit, microphone sensitivity and gain. These adjustments of parameters might help to reduce rejection rate of hearing aids, especially in those individuals who are annoyed by background noise. Copyright ? 2015 The Authors. Production & hosting by Elsevier (Singapore) Pte Ltd On behalf of PLA General Hospital Department of Otolaryngology Head and Neck Surgery. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Myosin-7A Mutation Screening in Patients with Prelingual Non-Syndromic Hearing Impairment

Objective To screen for mutations in myosin-7A gene(MYO7A) in patients with prelingual nonsyndromic hearing impairment. Methods 31 sporadic patients with congenital hearing impairment and 65 patients from 34 families with prelingual hereditary hearing impairment in China were tested in this study, and 100 hearing normal individuals were used as control. Genomic DNA isolated from whole blood of all subjects was subjected to polymerase chain reaction(PCR) to amplify selected exons of MYO7A gene. The PCR products were subsequently screened using single strand conformational polymorphism analysis(SSCP) and direct sequencing when the fragments showed an abnormal electrophoretic pattern. Results GgA transition at position 617 in exon 7, which would produce an A206G amino acid substitution, was detected in two patients but in none of the unaffected members in the families. This heterozygous missense mutation happened within a highly conserved heptapeptide sequence of MYO7A protein, and is closely relevant to preligual nonsyndromic deafness. Conclusions The A206G substitution is possibly a new mutation to cause preligual nonsyndromic hearing impairment. Our results provide evidence that exon 7 of MYO7A is a mutational hotspot in genetic hearing impairment.

Hearing Loss in Lassa Fever: A Systematic Review

Background: Lassa fever is a viral haemorrhagic fever associated with several complications which include hearing loss. Hearing loss in Lassa fever may become residual with its negative impact on the quality of life. Purpose: This paper is a review of various studies on Lassa fever related hearing loss. Methods: Systematic literature review was done on articles on Lassa fever patients and Lassa fever survivors. Relevant articles published in English between 1969 and 2021 were searched for using Google Scholar and PubMed databases. Eight studies which met the inclusion criteria were finally selected and included in the review. The variables of interest were extracted from the full-text articles. Results: Lassa fever related hearing loss occurs in both sexes and in all age groups. The incidence of self-reported hearing loss ranged from 12.5% to 30% with a mean of 17.6%. The incidence of audiometrically determined sensorineural hearing loss ranged from 13.5% to 29% with a mean of 22.1%. Hearing loss persisted in 64% of those that were followed up. Cases of hearing loss varied in severity, ranging from mild to profound. No successful specific treatment for Lassa fever related hearing loss was reported apart from hearing rehabilitation. Conclusion: Hearing loss among Lassa fever patients remains a major challenge. Lack of effective treatment for Lassa fever related hearing loss is a serious gap in the management of Lassa fever patients which should be addressed in future research.

Does Hearing Impairment Have an Impact on the Children’s Education in Casamance, South of Senegal?

Goal: To determine the impact of deafness on the schooling of children in Casamance and the factors that characterize these repercussions. Patients and Methods: This was a retrospective, cross-sectional and multicenter study carried out in the ENT departments of the peace and regional hospitals of Ziguinchor over a period of 7 years from January 1, 2012 to December 31, 2019. It included hearing impaired children aged 1 to 18. Results: 178 hearing impaired children were collected during the study period. One hundred and forty-seven children were in school (82, 58%), the majority (85.03%) attending formal schools. Fifty-seven children had a speech disorder (32.02%). The average age of screening for deafness was 9 years. Deafness was mild in 53 patients. Thirteen patients had severe deafness. Two patients presented with cophosis. Conclusion: Our study shows that deafness does indeed have an impact on a child’s education in Casamance, but this impact is hardly taken into account.

Auditory Clinical Profile of Nightclub Employees in the City of Parakou in 2021

Introduction: Hearing impairments are common among nightclub workers. The present work aims to study the hearing clinical profile of these employees in the city of Parakou in 2021. Methods: This was a cross-sectional, descriptive analytical study from March 14 to June 15, 2021. It focused on employees of 13 nightclubs in the city of Parakou. Were included in the study, employees with at least 03 months of practice and aged at least 18 to 50 years at most on the date of the survey and who had given their informed consent to participate in the study. Each employee received a questionnaire, an otoscopy and an acoumetry in tune. Noise pollution was measured at each workstation using a sound level meter. Results: A total of 114 employees were collected. The mean age was 26.38 years ± 4.81 and the sex ratio 0.87. The server position was occupied by 65 employees (57%) followed by managers, 19 or 16.70%- and 17-disc jockeys, 14.90%. Among the employees 31.60% exercised between 03 and 09 months. 76.32% were exposed to noise for more than 8 hours per day and 81.58% were exposed to noise for more than 40 hours per week. The sound level at the workstation was for 59 employees (51.75%) between 90 and 100 dB. Tinnitus was the most expressed complaint, respectively 21 (18.40%) on the right and 22 (19.30%) on the left. Rinne’s test was positive in 85.10% of employees. Weber’s test was indifferent to all frequencies in the majority of respondents. Conclusion: Hearing damage among nightclub employees in the city of Parakou is a reality. It is important to put preventive measures in place.

GJB2 mutation spectrum in Inner Mongolia and its comparison with other Asian populations

Mutations in the GJB2 gene are the most frequently found mutations in patients with nonsyndromic hearing impairment. However, the mutation spectrum and prevalence of mutations vary among different ethnic groups. Every year, 30, 000 babies are born with congenital hearing impairment in China. In order to provide appropriate genetic testing and counseling to the family, we investigated the molecular etiology of nonsyndromic deafness in 135 unrelated school children attending Chifeng Municipal Special Education School in Inner Mongolia, China. The coding exon of the GJB2 gene was PCR amplified and sequenced. In addition, the 12S rRNA gene and tRNAser(UCN) of mitochondrial genome were screened for mutations responsible for hearing impairment. Sixty four GJB2 mutant alleles, including 60 confirmed pathogenic alleles and 4 unclassified variants, were identified in 31.1% (42/135) of the subjects. Twenty two subjects carried two pathogenic mutations and 20 subjects carried one mutant allele, including one subject with one autosomal dominant mutation. The 235delC was the most common mutation accounting for 65.6%(42/64) GJB2 mutant alleles. When compared to other Asian populations, our subject cohort had higher frequency of 235delC mutation than the Japanese population. The GJB2 mutant alleles account for 23.7% (64/270) of all chromosomes responsible for nonsyndromic hearing impairment. Testing of the 4 most prevalent deleterious frame shift mutations(235delC, 29900delAT, 17691del16, and 56005ins46) in this cohort detected 90% of all GJB2 mutant alleles. These results demonstrate that effective genetic testing of the GJB2 gene for patients and families with nonsyndromic hearing impairment is possible in the Chinese population. Since the most common 309kb GJB6 deletion is not detected and only one 1555 A>G mutation in mitochondrial DNA is detected in our patients, investigation of mutations in other nuclear genes and/or environmental factors responsible for nonsyndromic hearing impairment in the Chinese population is necessary.

Inner Ear Disorders in the Elderly with Carotid Artery Disease Requiring Revascularization:Prevalence,Characteristics,and Association

Background:Aging is almost assocaited with inner ear disorders(InEarDs)by means of age-related hearing impairment(ARHI)or vertigo-anddizziness as well as the carotid artery disease requiring revascularization(CAD-R).Objective:The present study aimed to study the prevalence and characteristics of InEarDs in older adults diagnosed with CAD-R.The other aim was to determine if InEarDs in CAD-R patients is age-related or might be explained by a concomitant CAD-R.Method:A retrospective,case-control study was conducted at the Mayo Clinic,Florida.The study cohort includes 919 patients who had CAD-R.The control group consisted of 244 age-and gender-matched patients presenting with cardiac or peripheral artery disease.The InEarDs were assessed based on the diagnosis upon presentation to the Audiology Clinic and follow-up.Results:Of the 919,348 had ARHI that includes significant peripheral signs and central symptoms(24.9%),vertigo-and-dizziness events that are recurrent and persistent with normal objective vestibular testing(12.9%),or a combination of both(11.0%).These percentages were significantly higher in the study group relative to the control group.After adjustment for the vascular risk factors,the study group had significantly higher odds of ARHI(OR=1.94;95%CI:1.09-3.44;P<0.05).Conclusion:CAD-R patients had significantly higher InEarDs than the control group.CAD-R is more likely to be associated with ARHI rather than the vertigo-and-dizziness even after adjusting for the vascular risk factors.

Medical Therapy of Hearing Impairment and Tinnitus with Chinese Medicine:An Overview

The current review gives a comprehensive overview of the recent development in Chinese medicine(CM)for treating several kinds of acquired nerve deafness and tinnitus,as well as links the traditional principle to well-established pharmacological mechanisms for future research.To date,about 24 herbal species and 40 related ingredients used in CM to treat hearing loss and tinnitus are reported for the treatment of endocochlear potential,endolymph growth,lowering toxic and provocative substance aggregation,inhibiting sensory cell death,and retaining sensory transfer.However,there are a few herbal species that can be used for medicinal purposes.Nevertheless,clinical studies have been hampered by a limited population sample,a deficiency of a suitable control research group,or contradictory results.Enhanced cochlear blood flow,antiinflammatory antioxidant,neuroprotective effects,and anti-apoptotic,as well as multi-target approach on different auditory sections of the inner ear,are all possible benefits of CM medications.There are numerous unknown natural products for aural ailment and tinnitus identified in CM that are expected to be examined in the future utilizing various aural ailment models and processes.

A CASE STUDY —A SUCCESSFUL STUDENT OF ENGLISH WITH IMPAIRED HEARING

In China,there is not one school that does nottake English teaching seriously.There is not one stu-dent who does not want to learn English well.How-ever,not many learners who succeed in Englishlearning,communicate fluently in both oral or writ-ten English,and much fewer reach a high level inhigh school years.Therefore,when we encountereda high school English lover who reached a very highlevel of English,our interest in knowing her natural-ly grew.

Identification of Two Disease-causing Genes TJP2 and GJB2 in a Chinese Family with Unconditional Autosomal Dominant Nonsyndromic Hereditary Hearing Impairment

Background： There are more than 300 genetic loci that have been found to be related to hereditary hearing impairment （HHI）, including 92 causative genes for nonsyndromic hearing loss, among which 34 genes are related to autosomal dominant nonsyndromic HHI （ADNSHH 1）. Traditional linkage analysis and candidate gene sequencing are not effective at detecting the ADNSHHI, especially for the unconditional families that may have more than one pathogenic cause. This study identified two disease-causing genes TJP2 and GJB2 in a Chinese family with unconditional ADNSHHI. Methods： To decipher the genetic code of a Chinese family （family 686） with ADNSHHI, different gene screening techniques have been performed, including linkage analysis, candidate genes screening, high-throughput sequencing and Sanger sequencing. These techniques were done on samples obtained from this family over a period of 10 years. Results： We identified a pathogenic missense mutation, c. 2081G〉A （p.G694E）, in TJP2, a gene that plays a crucial role in apoptosis and age-related hearing loss （ARHL）. The mutation was co-segregated in this pedigree in all, but not in the two patients who presented with different phenotypes from the other affected family members. In one of the two patients, we confirmed that the compound heterozygosity for p.Y136^＊ and p.G45E in the GJB2 gene may account for the phenotype shown in this patient. Conclusions： We identified the co-occurrence of two genetic causes in family 686. The possible disease-causing missense mutation of TJP2 in family 686 presents an opportunity for further investigation into ARHL. It is necessary to combine various genes screening methods, especially for some unconventional cases.

Etiology of newborn hearing impairment in Guangdong province: 10-year experience with screening, diagnosis, and follow-up

Background Hearing impairment is one of the most common birth defects in children.Universal newborn hearing screenings have been performed for 19 years in Guangdong province,China.A screening/diagnosis/intervention system has gradually been put in place.Over the past 10 years,a relatively complete data management system had been established.In the present study,an etiological analysis of newborn cases that failed the initial and follow-up screenings was performed.Methods The nature and degree of hearing impairment in newborns were confirmed by a set of procedures performed at the time of initial hearing screening,rescreening and final hearing diagnosis.Then,multiple examinations were performed to explore the associated etiology.Results Over a period of 10 years,720 children were diagnosed with newborn hearing loss.Among these children,445 (61.81%) children had a clearly identified cause,which included genetic factor(s) (30.56%),secretory otitis media (13.30%),maternal rubella virus infection during pregnancy (5.83%),inner ear malformations (4.86%),maternal human cytomegalovirus infection during pregnancy (2.92%),malformation of the middle ear ossicular chain (2.50%) and auditory neuropathy (1.81%).In addition,275 cases of sensorineural hearing loss of unknown etiology accounted for 38.19% of the children surveyed.Conclusions Long-term follow-up is needed to detect delayed hearing impairment and auditory development in children.The need for long-term follow-up should be taken into account when designing an intervention strategy.Furthermore,the use of the deafness gene chip should further elucidate the etiology of neonatal hearing impairment.

Single nucleotide polymorphisms and haplotypes analysis of DFNB1 locus in Chinese sporadic hearing impairment population

Background The DFNB1 locus, which contains the gap junction beta-2 （GJB2） and gap junction beta-6 （GJB6） genes, plays a key role in the nonsyndromic and sporadic hearing impairment. Mutations of DFNB1 result in autosomaJ recessive nonsyndromic hearing impairment （ARNSHI）. Previous researches have identified mutations in GJB2 and GJB6, but single nucleotide polymorphisms （SNPs） of DFNB1 locus have not been studied. So we chose five SNPs to evaluate whether there is difference between deafness people and normal-hearing people in Han Chinese. Methods Five SNPs in the DFNB1 region were examined using a case-control association study between cases with sporadic hearing impairment and controls with normal hearing. The HWEsoft and SHEsis softwares were used to analyze the results. Results Single-locus association analysis showed a positive association for three SNPs： rs9315400, rs2274084 and 235delC. When we compared the distributions of the haplotypes, we also found significant differences between cases and controls in the haplotype combination of rs2274084 and rs2274083 （Х^2=12.978, df=3, global P=0.004719）. Conclusions The haplotypes composed of rs2274084 and rs2274083 suggested that C-C may be a risk haplotype for the sporadic hearing impairment while T-T may be protective against hearing impairment. From that point of view, we can conclude that the SNPs of DFNB1 locus also plays an important role in sporadic hearing impairment cases.

Phenotype and genotype analysis of a Chinese family with prelingual X-linked hereditary hearing impairment

Background X-linked hearing impairment is clinically and genetically a heterogeneous disease. Although many disorders manifest with hearing loss, a limited number of sex-linked loci and only one gene （POU3F4） have been shown to be implicated in X-linked non-syndromic hearing impairment. In the present study, we have performed a clinical and genetic analysis of a Chinese family with X-linked non-syndromic hearing loss, with emphasis on audiological findings and genomic mapping. Methods The clinical features of Family JX01 were evaluated by physical and audiometric examination in eighteen family members. Mutation screening of POU3F4 was identified by polymerase chain reaction （PCR） amplification and sequencing. Molecular evaluation consisted of X-chromosome wide genotyping by microsatellite makers （STR）, followed by analyzing using MLINK computer program. Results Five affected males demonstrated bilateral, symmetrical sensorineural and profound hearing loss. The hearing impairment started prelingual. The female carriers did not have any complain of hearing loss, however, two of them were tested with milder loss with high frequency. No causative mutations in POU3F4 gene were detected by DNA sequencing. Linkage analysis indicated that the responsible gene was linked to locus DXS1227 （maximum Iod score=2.04 at θ=0）. Conclusions The affected males in Family JX01 have profound prelingual sensorineural hearing impairment. In addition, two female carriers showed mild to moderate hearing losses. However, none of females complained of any hearing loss. Analysis of hereditary deafness in this family mapped most compatibly to the Xq27.2.