Objective: Main Objectives of research were: 1) developing a lip-reading test for Persian adults, 2) Standardization of the test in a group with normal hearing and 3) Evaluation of it in a group of adults with acquires hearing impairment.

Materials & Methods: First according to "Denver Quick Test of lip-reading" (Alpiner et al, 1970), a lip-reading test, composed of 20 simple and common sentences was developed. Then the test was performed on 105 normally hearing and vision adults with male and female talkers. Also 48 adults with acquired hearing impairment were evaluated twice, lip-reading alone and lip-reading + residual hearing.

Results: According to the results, "Sara Lip-reading Test" is a valid and reliable test for assessing lip-reading ability of Persian adults.

Objective: A “hearing-impaired is defined as abnormal or reduced function in hearing resulting from auditory disorder” (Stach,1997). The goal of any preschool and school screening program should be to accurately identify those children whose hearing has impaired resulting from either conductive and / or sensory - neural pathology. Although some ear pathologies (e.g., middle ear effusion) might not produce a significant hearing loss, screening for these pathologies should be a part of the screening program because they may influence the potential to learn.

Materials & Methods: This research was carried out in a cross-sectional descriptive study on 577 children (278 girls and 299 boys) among the 3-6 years old children of kindergartens in Welfare Organization of the Tehran Province from March to June 2001. The otoscopy examination, pure-tone screening and impedance screening was conducted after completion the parents awareness form of the hearing loss existence.

Results: In this study, there were 12.58% abnormal conditions of external ear canal, 23.7% abnormal tympanic membrane, 34.3% abnormal tympanograms, 35% no acoustic reflex, 12.6% hearing loss including 9.7% bilateral and 2.9% unilateral hearing loss (12.0% conductive hearing loss, 0.64% sensory-neural hearing loss and 0.36% mixed hearing loss), higher prevalence of left ear disorders and statistically meaning difference between two ears (P<0.05) in all of studied items, except of acoustic reflex condition.

Conclusion: With respect to high prevalence of need to medical care and the negative effects of middle ear disorders in learning of preschool children, and also due to the importance of early identification and intervention of hearing loss in aural rehabilitation programs, awaring and teaching the people about the effects of hearing disorders and it's prevention and identification are very important.

Objective: Multiple sclerosis (MS) is a neurological disease that causes sudden deaf. In this research auditory disorders were studied in the patients with multiple sclerosis (MS).

Materials & Methods: This cross sectional descriptive-analytical study was performed on 107 patients with multiple sclerosis in the range of 20-45 yrs. There were not history of trauma and middle ear disease in all cases. Sampling was randomized. A complete auditory evaluation including pure tone audiometry, speech audiometry, imittance audiometry and brainstem Auditory Evoked Potential were performed on patients.

Results: In pure tone audiometry 62.19% of cases had sensory neural high tone loss. In speech audiometry and imittance audiometry 18 and 31 cases were abnormal, respectively. 14.55% of cases had abnormality in BAEP. The most of abnormalities were prolonged latency of V, decrease of V/I amplitude ratio and poor reproducibility, respectively. With high rate of stimulation 57.77% of cases were abnormal. Statistical analysis showed significant difference between latency of V and stimulation rate.

Conclusion: According to findings of this research it seems that hearing evaluation is very important for follow-up and early rehabilitation of auditory disorder in patients with MS. Also auditory tests battery especially brain-stem auditory evoked potentials (BAEP) with high stimulation rate are useful in the diagnosis of Multiple Sclerosis.

Hereditary Hearing loss (HHL) affects one in 2000 netborns and more than 50% of these cases, the loss has a genetic basis. About 70% of HHL is non-syndromic with autosomal recessive mode of inheritance accounting for ~85% of the genetic load and more than hundred loci have been estimated for this kind of deafness. A 4–year-old boy and her sixteen-year- old sister, both suffering from congenital deafness without any other clinical symptoms were referred to Genetics Research Center to be screened for molecular basis of their phenotype. Bases on clinical observation, autosomal recessive non-syndromic hearing loss was dignosed for both of them. Using linkage analysis their phenotype localized to 6p21-3 chromosome in which COL11A2 has been located Several mutations have been reported in this gene associated with stickler syndrome type3 and non-syndromic hearing loss, both with autosomal dominant form of inheritance.

In this study linkage between COL11A2 gene in DFNA13 locus, causative for autosomal dominant hearing loss, and phenotype of autosomal recessive non-syndromic hearing loss in and Iranian family was analyzed and Pro621Thr mutation was found in this family. Pro 621 Thr mutation found in this study is the first report of COL11A2 mutation associatied with autosomal recessive non-syndromic hearing loss.

Objective: This study investigated the level of hearing disorder children’s acceptance by their peers in inlusive and non-inclusive schools in the city of Tehran.

Materials & Methods: The sample in this study was 323 elementary school students in grades 3-5 from the schools in different regions of Tehran. These students were studying alongside their peers with mild hearing disability. Also for comparison this study used a sample of 65 students from non-inclusive (regular) schools. Level of acceptance was measured through the use of Acceptance Scale developed by Voeltz (1980). The major questions in this study were based on the impact of the following factors, gender and the type of schools attended by students (inclusive or non-inclusive).

Results: The results indicated that in inclusive schools, boys level of acceptance were higher than that of the girls. Also a significantly lower level of acceptance was found in regular school students as compared with that found in inclusive schools.

Conclusion: This finding led the investigators to conclude that the experience of inclusive education can lead to higher level of regular students’ acceptance towards special need students. Suggestions for future research are given in light of limitations of present research and the need for further understanding of the factors contributing to student acceptance in inclusive settings is emphasized.

Objective: Hereditary Hearing loss (HHL) affects one in 1000-2000 newborns and more than 50% of these cases, the loss has a genetic basis. About 70% of HHL is non-syndromic with autosomal recessive forms accounting for ~85% of the genetic load. To date, more than 100 locus estimated for this kind of deafness. Different genes have been reported to be involved, but mutations in the connexin 26 gene (Cx26) have been established as the basis of autosomal recessive non-syndromic hearing loss. The aim of this project is to study the prevalence of connexin 26 mutations.

Materials & Methods: In this descriptive study, the prevalence of connexin 26 mutations was evaluated by using amplification refractory mutation system (ARMS)-PCR for detection of 35delG and then analyzed all samples excluding 35delG homozygote by DHPLC and direct sequencing. 38 patients with autosomal recessive non-syndromic hearing loss were selected simply and participated in this research. 

Results:  76 chromosomes (38 patient) were screened for GJB2 mutations. Thirty two (42%) carry GJB2 mutations including 35 delG, W24X, R32H, R127H, -3170G>A. Among them, 35 delG has the highest frequency (84%). Polymorphism V153I was found in three chromosomes.

Conclusion: According to these results, other loci and genes may be the major responsible for nonsyndromic deafness in this population.

The incidence of profound congenital hearing loss is about 1 in 1,000 live birth. There are more than 50 distinct genetic loci (known as DFNB loci) at which mutations can cause recessive hearing loss. DFNB4, one recessive locus for deafness, also maps to 7q31 considerably for nonsyndromic hearing loss and Pendred Syndrome which has been named PDS gene. Pendred syndrome (PS, MIM 274600) with an estimated frequency 1-8 per 100,000, is an autosomal recessive disorder and classically characterized by sensor neural hearing loss and goiter. Here, we reported a family with Pendred syndrome that in which a new mutation, T420I, in the homozygous state caused the condition. Also there was a 9-year-old boy in the related family with hearing loss and with no signs of thyroid dysfunction or goiter, whom was compound heterozygous for PDS mutations including 1197delT and T420I. Both of these mutations result to PDS syndrome. However we are not sure if the 9-year old boy show the goiter appearance in near future, but the results could be used as the prognostic factor. Consequently we should follow up the patient. Besides all, characterization of mutations in PDS gene can guide us to early diagnosis of Pendred syndrome and consequently early treatment of the patients maybe show the clinical features of hypothyroidism in later onset.

It has been estimated that approximately one in 1000 live births suffer from profound deafness, and greater than 50% of this group is genetic etiology. So far more than 300 different genetic conditions responsible for deafness have been reported that among them 70% are non-syndromic and the rest are syndromic. Non-syndromic and syndromic hearing loss may be divided into Autosomal dominant (DFNA), Autosomal recessive (DFNB), X-linked (DFN), and mitochondrial. Approximately 75-80% are autosomal recessive, 10-20% autosomal dominant, 1-5% X-linked, and 0-2% mitochondrial. To date, 51 DFNA loci, 61 DFNB loci, and 7 DFN loci have been described. Non-syndromic hearing loss is divided into two postlingual and perlingual groups. As a general rule, most autosomal dominant non-syndromic hereditary hearing impaired is postlingual, while autosomal recessive non-syndromic hereditary hearing impaired is prelingual.

Objective: Hearing loss (HL) is the most prevalent sensorineural defect in human. Mild to severly Profound HL occurs in about l.0 per 1000 births. Many previous studies have shown that about 50% of deafness is due to genetic factor and 50% is due to environmental and etiologically unknown. In this study, we are searching the causes of deafness at these families and introduce strategies for diagnosis and prevention.

Materials & Methods: Questionaires were distributed in deaf schools, hearing loss and rehabilitation centers in Qom and Markazi provinces collecting done after filling by the parents. The criterias such as age, sex, number of deaf individuals in families their close relatives, the marriage type of the parents and etiology of disease (if it is genetic or environmental) were extracted from questionnaires and were analysed by SPSS software.

Results: Three hundred and fifty four (354) Questionnaires contained complete information from hearing loss families were collected. Age mean of proband individuals is 16.1±8.1. Sex distribution of 48.4% and 51.6% girls and boys, respectively. Parents were 59.3% and 36.7% of consanguineous and unfamilial marriage, respectively. The marriage type of were not determined in 4%. Mean of deaf individuals among these families was 1.8±1.4.By assessing the filled questionnaires and pedigrees. The deafness etiology in the studies population was categorized as genetic cause (70.9 %), environment factor (9%) and unknown etiology (20.1%).

Conclusion: Genetic factor with and autosomal recessive inheritance pattern was the most common cause of hearing loss due to the high prevalence of consanguineous marriage which resulted and increased genetic causes in more than 50%. Multiplicity of offspring also shawn an increased in frequency of hearing loss in families that have deaf with genetic background of this disorder. In this study, environmental and unknown factors are second cause of HL. We may interestingly reduce frequency of HL in Iran with discouraging consanguineous marriage, health education, population regeneration control also genetic counseling especially for high risk families.

Objective: Congenital hearing loss occurs in 1 out of 1000 births and about 50% of all cases are estimated to be of genetic origin. About 70% of hereditary hearing loss is non-syndromic with autosomal recessive inheritance accounting for 80% of the genetic load. To assess the importance of other loci in the Iranian population, we screened 50 consanguineous families with ARNSHL for DFNB21, which was linked to ARNSHL in Middle East countries. 

Materials & Methods: 50 consanguineous families with at least three affected children, previously excluded by mutational analysis from GJB2 and GJB6 genes, were included in this study. We used three polymorphic markers including D11S1998, D11S4464, and D11S1299 in this study.

Results: Two families were linked to DFNB21 and two novel mutations have been detected so far. In two families a 266 Del T mutation and a large 9611bp deletion that starts from intron 9 and includes exon 10 in TECTA gene were detected.

Conclusion: This study showed that mutations in DFNB21 locus are the most common cause of ARNSHL in Iranian population. It seems that DFNB21 may play an important role in genetic load of ARNSHL in Iran. This will be confirmed by screening more families for this locus in Iranian deaf population.

Objective: There is an assumption that pragmatic competence and performance in deaf loss students is less than normal students , so this research notice to this pragmatic aspects in two groups of deaf students of Baghcheban schools and normal students in Tehran.

Materials & Methods: Subjects were 70 girls and boys from deaf students with severe impaired hearing (70 - 90 dB) and mean of education 15 - 18 in 4 levels high school. Then those comparied with 70 normal students that was matched in terms of sex, educational level, course of education and area of live. Method of research was comparative and cross - sectional. Instruments include questionnaire and pragmatic test (reception and expression of metaphors, reception and expression of proverbs, reception of indirect speech acts, reception of inferential meaning, select of best topic and reception of humour sentences). Then the data was analyzed with the use of Kruskal Wallis test, Mann - Whitney U, t - test and paired t – test.

Results: Analysis of data demonstrated significantly different (P < 0.05) between average scale of pragmatic competence and performance in two groups. There was significantly difference between average scale of pragmatic competence and performance with the increase of educational level in hearing loss students also. But there weren’t significantly difference between average scales of pragmatic with the increase of educational level in normal students.

Conclusion: The deaf students understand pragmatic competence less then normal students and they are unable to use them appropriative in different contexts and situations.

Objective: The negative effects of hearing loss rendered to the processes of fast motor skills of articulators, speed and intelligibility of speech have been proved scientifically. The main question is how different thresholds are, can effect in such and try to find relations between mentioned three speech skills specially if there are relations between tests in evaluating speed articulators such as oral diadochokinesis and speed of speech.

Materials & Methods: This cross-sectional–analyzical study was performed on 46 hearing-impaired (profound, sever, moderately sever) and 15 normal -hearing high-school students that were selected randomized. Subjects were measured by oral diadochokinesis test (The records were analyzed by speech analyzer of Dr. Speech Software), 100 words text to evaluate speed of speech according read words per minute), and use of rate scale method to define the rate of intelligibility of speech in reading text. Data were analyzed by SPSS software.

Results: The general pattern suggest that the high threshold hearing subjects spend more time to repeat oral diadochokinesis syllables and have decreased speed and intelligibility of speech. In respect to speed of speech and oral diadochokinesis ability, there were only significant differences between profound hearing-impaired subjects and both sever and moderately sever subjects (P<0.05). There was significantly negative relationship between speed of speech and spent time to repeat the oral diadochokinesis syllables in hearing-impaired groups (P=0.02). There was not any relationship in normal-hearing group (P>0/05). General findings suggest some significant relationship between intelligibility and speed of speech (P>0.05).

Conclusion: Respect to the negative effects of hearing loss on fast oral motor skills, speed of speech and intelligibility of speech and such relationships between these three variables, suggested interventions on each of the above variables may be useful in other comparative subjects.

Objective: Hereditary hearing loss (HHL) is a very common disorder. When inherited in an autosomal recessive manner, it typically presents as an isolated finding. Interestingly and unexpectedly, in spite of extreme heterogeneity, mutations in one gene, GJB2, are the most common cause of congenital severe-to-profound deafness in many different populations. In this study, we assessed the contributions made by GJB2 mutations and deletion in a portion of GJB6 to the autosomal recessive non-syndromic deafness genetic load in Iran.

Materials & Methods: In this descriptive and cross – sectional study1605 probands from 1605 different nuclear families with autosomal recessive non-syndromic hearing loss were investigated. Hearing loss tests and clinical examination were done and 10 ml blood was drawn as DNA source. After study of 35delG mutation by ARMs PCR, negative or heterozygote individuals were sent to IOWA University for detection of other GJB2 mutations. 

Results: GJB2-related deafness was found in 243 families (15.1%).

Conclusion: Varient geographic pattern for GJB2-related deafness has considerable results in Iran in comparable with other study in Europe and our neighboring countries and deletion in GJB6. [∆ (GJB6-D13S1830)] hasn't been detected in our studied population.

Objective: Hearing loss can affect on physical, mental and social health of deaf adults and lead to depression, anxiety, isolation, suspicion and stress of them. Cochlear implantation has positive effects on behavioral and emotional status of postlingually hearing impaired adults. This study is aimed to compare dizziness, depression, anxiety and mental health in adult cochlear implant recipients and candidates.

Materials & Methods: This case- control and comparative study was conducted on 49 patients, 24 cochlear implant recipients (as case group) and 25 severe-profound hearing impaired adults (as control groups) whom were selected by simple and convenient sampling. Beck Depression Inventory, Dizziness Handicap Inventory, Beck Anxiety Inventory and General Health Questionnaire were completed to determine and compare cochlear implant effects. Data were analyzed by MANOVA.

Results: Mean depression and anxiety scores in cochlear implant candidates were more than cochlear implant recipients. This difference was significant in depression (P=0.001). There was no significant difference between two groups in general health (P=0.415). The results of this study also showed that dizziness is more in cochlear implanted group (P=0.004).

Conclusion: It seems that cochlear implant use leads to decrease of depression and anxiety. It leads to increase of dizziness.

Objective: Pragmatics is the study of speech acts and social aspects of communicative interactions in context. Hearing-impaired children suffer from language impairments due to lack of hearing sense which has important role in developing language skills. The purpose of this study is comparison of some of pragmatic skills including topic maintenance, turn-taking and repairs between 4 to 6 year-old Farsi speaking hard of hearing children with normal hearing peers.

Materials & Methods: In this cross-sectional and case-control research, twenty one normal hearing children were chosen by simple random sampling method from ordinary kindergartens and twenty one hard of hearing children (71-95 db) were selected from deaf rehabilitation centers and speech therapy clinics of Tehran and Karaj by simple sampling method. The children of both groups were assessed by nonverbal intelligence subtests. If they did not show any difficulties, the pragmatic skills of them (topic maintenance, turn-taking and repair) were evaluated by researcher made tasks which their content validity was confirmed by a linguistic and two speech and language pathologists and reliability of them was determined by test-retest (0.70)

Results: There were significant differences between two groups in duration of topic maintenance (P<0.001), scores of turn-talking (P<0.001), repairs in form of reply to request for clarification (P=0.003) and request for clarification (P=0.002).

Conclusion: The pragmatic abilities of hearing-impaired children were Significantly lower than those of normal hearing.
 

Objective: Hearing impairment is the most common disability in birth. Many hearing impaired people suffer from language impairment such as metaphor comprehension. The purpose of this study is comparison of comprehension of metaphoric expressions in normal students with hearing impaired of junior high school sever.
Materials & Methods: In this descriptive–analytic and comparative research, fifty hearing students and twenty five hearing impaired students were selected in a simple random sampling from normal and exceptional schools. Two researcher- made tasks have been used. At first the content validity and then with test re test reliability of non metaphoric words (r=0.724) and metaphoric expression comprehension task were determined. Then task of non metaphoric words was performed. If the subjects answered more than 75 percent of words correctly and they had all of inclusion criteria, task of metaphoric expression comprehension task was performed. Data were analyzed by statistical test such as independent sample T test and one way ANOVA.
Results: There was significant difference in score of comprehension of metaphoric expressions between two groups (P<0. 001). The most number of incorrect answers of hearing impaired students were literal. In both groups, there was no significant difference in mean score of comprehension of metaphoric expressions between three grades of junior high school.
Conclusion: Hearing impaired students had no development in comprehension of metaphoric expressions in first, second and third grade of junior high school and they understand metaphoric expressions literally according to their incorrect answers. They need especial training because of their significant different with hearing students.

Objective: Hearing impairment is the most frequent sensorineural defect in 2 forms, syndromic and non–syndromic. The aim of this study is haplotype analysis of seven loci of non–syndromic autosomal recessive hearing loss in Iranian families.

Materials & Methods: In this descriptive study, forty one Iranian families with 2 or more affected individuals segregating as an autosomal recessive non–syndromic hearing loss were selected simply and conveniently. The patients have been tested negative for the following loci, DFNB1, DFNB2, DFNB3, DFNB4, DFNB6, DFNB7/11, DFNB8/10, DFNB9, DFNB12, DFNB16, DFNB18, DFNB21, DFNB23, DFNB29 and DFNB4 previously. The subjects have been investigated additional 7 loci (DFNB22, DFNB28, DFNB30, DFNB31, DFNB36, DFNB37 and DFNB67) , to determine the prevalence of these genes involve in these loci. Homozygosity mapping was applied using number of STR (Short Tandem Repeat) markers.

Results: Three families linked to the following loci DFNB28, DFNB30 and DFNB 31.

Conclusion: In this research, the cause of additional 7% of non–syndromic hearing loss was determined in Iranian population.

Objective: Hearing Impairment during childhood affects all aspects of speech production and comprehension. It seems that hearing impaired people suffer from language and speech impairments such as comprehension of structures derived by syntactic movement. The purpose of this study is to compare the comprehension of structures derived by syntactic movement in children with hearing impairment and normal children.

Materials & Methods: In this case-control research, twenty normal children, aged 6-7 years and twenty children with severe to profound hearing impairment, aged 8-12 years were selected in a simple random sampling from normal kindergartens and schools, and exceptional schools for hearing impaired people. The children didn’t have any confirmed diseases or neurological disorders, except hearing loss in students with hearing impairment. The children's aural records and also the confirmation of the audiologist in the exceptional schools for hearing impaired people were considered in order to determine the kind and degree of hearing loss. The comprehension of sentences was tested by using a researcher-made task called sentence-picture matching task. At first the content validity was determined and then the reliability was confirmed with Cronbach Alpha Test. Data were analyzed by statistical test such as Independent Samples T-Test and Mann-Whitney U Test and using SPSS software.

Results: Comprehension of the hearing-impaired group was significantly different from that of the hearing control group (P<0/05). The children with hearing impairment failed to understand structures derived by syntactic movement. Comprehension of the hearing –impaired group on structures with canonical word order was better than on structures derived by syntactic movement.

Conclusion: Incorrect answers of the children with hearing impairment to the sentence - picture matching task alludes to the incomplete comprehension of structures derived by syntactic movement.

Objective: The goal of this research was to study progress of reading literacy in hearing impaired students by comparing them in three educational degrees.

Materials & Methods: In this analytical and applied research that was a cross–sectional and comparative study, from fourth grade, guidance and high school students of the exceptional schools in Karaj, Shahriar, Shahre Ghods, Hashtgerd and Robat Karim, 119 hearing impaired students were selected conveniently and their reading literacy evaluated by PIRLS (Progress in International Reading Literacy Study) booklets in three components including: reading ability, Informational comprehension and literal comprehension. Data were analyzed by independent t-test.

Results: There were no significant differences between elementary fourth grade and guidance school third grade students with moderate and sever hearing impairment in reading literacy level and informational and literal comprehension texts (P>0.05). Reading literacy level and literal comprehension texts were significantly different between guidance school third grade and high school third grade students with moderate (P=0.004 & P=0.032 respectively) and sever (P=0.011 & P=0.005 respectively) hearing impairment, but informational texts comprehension between these two groups was significantly different only in moderate hearing impaired students (P=0.008).

Conclusion: The reading literacy development in hearing impaired students has a slow progress which is beyond educational degrees and amount of hearing loss. It is necessary to consider the literacy and reading promotion and direction towards the learning and comprehension deep layers in different educational degrees in exceptional educational system.

Objective: Many researchers believe hearing impaired people suffer from special disability in metaphor comprehension. This study was aimed to compare the comprehension of metaphoric expressions in mainstreaming sever hearing impaired and normal students of junior school in order to determine the source of metaphor comprehension impairment and find whether is this impairment due to their educational environment?

Materials & Methods: In this cross-sectional and comparative study, 50 hearing student and 25 hearing impaired student were selected by simple randomized method and tested by two researcher made tasks. All examinee did non metaphoric words task. If they answered more than 75 percent of words correctly and they had all of inclusion criteria, metaphoric expression comprehension task was performed. Then the data were analyzed by statistical tests such as Independent sample T test and one way ANOVA.

Results: Mainstreaming hearing impaired students showed difference significantly in score of comprehension of metaphoric expressions (P<0/001). The numbers of literal incorrect answers were more than meaning incorrect answers in mainstreaming hearing impaired students. There were no significant differences between score of comprehension of metaphoric expressions task in first, second and third grade of junior high school in normal hearing students (P=0.401) and mainstreaming hearing impaired students (P=0.120).

Conclusion: Incorrect answers of mainstreaming hearing impaired students to test showed their incomplete comprehension from metaphoric expressions and significant difference between hearing impaired students and normal peers in comprehension of metaphoric expressions showed the educational environment of mainstreaming schools is not enough for understanding metaphoric expressions.