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This study has been performed on show the incidence of sensory neural hearing loss (SNHL) in patients with chronic Otitis Media (COM) and it’s different parameters. 207 patients with persistent or previous otorrhea have been examined. 70 patients had bilateral otorrhea and 137 patients had unilateral problem so overall 277 ears are evaluated. The BC greater than 15 dB without carhartnotch was the basis for diagnosis of SNHL. 66 patients had SNHL and of them 26 cases were bilateral so total number of ears with SNHL were 92 (The incidence of SNHL was 32.2%). 3 cases were due to other causes like TB (1 case) RM fistula (1 case) and squamous cell carcinoma (SCC) of middle ear (1 case). About 90.3% of cases had down slooping high frequency SNHL in their audiogram. In 47.6% of the ears with granulation tissue and cholesteatoma had SNHL while 28.7% of cases with chronic perforation revealed SNHL. Analysis of this study showed that there is a probability of increased SNHL during old ages the cases of prolonged and persisted otorrhea also revealed a higher incidence of SNHL. We didn’t disclose correlation between age and severity of SNHL.

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Background & Objective: Major ?-Thalassemia is the most common form of anemia, which has a relatively high prevalency especially in the northern part of the country one of this disease side effect the hearing abnormality. This study has been carried out in Gorgan for the determination of the hearing level of ?-Thalassemic patients, and its relation with the level of serum Ferritin, the rate and the duration of blood transussion and dyspheral. Materials & Methods: In this study 95 patients with major ?-Thalassemia have been studied for the rate of hearing level. The variation parameter include age, gender, Ferritin level, the rate and duration of disferal consumption. Audiometry, tempanometry and physical examination carried out on all the patients. The findings from this research gathered and were analyzed using the SPSS statistical software. Results: 95 patients (190 ears) with age 3-29 year of old were gone under this study and only 72 ears had the threshold over 15 decibel, from this 43.9% were from sensorineural type of hearing. The 80% of ears’s thempograms were type A. The results from this study showed that there is a meaningful statistical correlation between the hearing loss and serum Ferritin level. The rate of dyspheral consumption, in each time and its duration (P<0.05). Conclusion: This study showed that high serum Ferritin level and the increased length of dyspheral consumption lead to the hearing loss in major ?-Thalassemia, therefore clinical examination of hearing interrally has to be carried out.

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Background and Objective: Aircraft as a safe means of transportation may cause occupational diseases and hearing loss. Prevention should be implemented for airport employee. This study was done to detemine the scale of hearing loss among Isfahan airport employees in 2005. Materials and Methods: This historical cohort study was conducted among Isfahan International airport employees. 80 employees were selected in four different case groups, the control group consist of two sub-gourps. The sub-group I (18 subjects) including the adminstrative airport employees (low noise exposed) and the sub-group II (32 subjects) including non-airport employees (non-noise exposed). The sound pressure level was measured and equivalent level (Leq) was calculated for all groups. Also the history of participants about past noise exposure and other confounding variables was detrmined by a questionnaire. Pure tone audiometry was carried out on conventional frequencies (0.5-8 KHz). Results: The mean age of subjects was 40.03±9.75 and 37.85±8.15 for exposed and non-exposed groups respectively. Leq (noise equivalent level) for Ramp and traffic workers was estimated more than 95 dBA. 35.7% (n=10) of Ramp workers were suffering in their right ear from noise induced hearing loss (26-40dB) and 32.1% (n=9) of them in their left ear. Relative risks of noise induced hearing loss in Ramp workers in comparison with control group were 9.4 and 7.5 for right and left ears. A siginificant difference was found between the hearing thresholds of exposed and non-exposed groups (P<0.05). Conclusion: This study showed that occupational exposure to noise cause hearing loss among airport employee. It is suggested strategies of noise assessment and prevention should be implemented for airport employees.

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Background and Objective: Multiple sclerosis is one of demyelinating disorder of CNS that is an uncommon cause of the sensorineural hearing loss. This study was done to determine the hearing loss in multiple sclerosis patients.

Materials and Methods: This case-control study was performed on 60 (44 women, 16 men) multiple sclerosis patients and 38 (27 women, 11 men) normal subjects by pure tone audiometery, otoacustic emissions and auditory brainstem responses in Gilan provine, Iran during 2010-11. Data was analyzed by using SPSS-17, Chi-Square and Fischer tests.

Results: 12.5% of case and 3.9% of the control ears had abnormal pure tone audiometery (P<0.05). The frequencies of abnormal HF-pure tone audiometery and two modalities of otoacustic emission did not show any significant differences in two groups. Abnormal autidory brainstem response of ears were observed in 20% and 9.2% of cases and controls, respectively (P<0.05). 20% of case and 9.2% of the control ears had abnormal auditory brainstem response (P<0.05). The absolute latencies of waves I, II and V had not significant differences between two groups. Inter peak latencies of I-III and III-V waves were observed in 10% and 11.7% in cases ears and 1.3% and zero percent in controls, respecticely. 6.7% of cases and 2.6% of control ears had retrocochlear abnormality.

Conclusion: Hearing loss detected by pure tone audiometery and auditory brainstem response is more common in multiple sclerosis compared to normal population.

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Cogan's syndrome is a chronic inflammatory disorder of unknown cause affecting mostly young adults. Two main observation of the disease are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between Cogan's syndrome and systemic vasculitis as well as aortitis are exist. The diagnosis of the disease is based upon the presence of inflammatory eye disease and vestibuloauditory dysfunction . In this article, the classic Cogan's syndrome has been reported in a 47-year-old woman. Two months prior to admission, the patient had been suffering from headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss and blindness. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally and on slit lamp examination, there was a conjunctival hyperemia, bilateral cataract and interstitial keratitis. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The patient was initially treated with pulse intravenous methylprednisolone and was followed by oral prednisolone and cyclophosphamide, which in follow-up showed partial improvement.

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Background and Objective: SLC26A4 gene mutations after GJB2 mutations are the second currently identifiable genetic cause of autosomal recessive non syndromic hearing loss (ARNSHL) which currently is used in molecular diagnosis of ARNSHL. Several potential STR markers related to this region have been reported .This study was carried out to identity the informativeness of D7S2456 CA repeat STR marker in SLC26A4 gene region in five ethnic groups of the Iranian population. Methods: In this descriptive study, The locus was genotyped in 165 unrelated healthy individuals of five different ethnics including Fars, Azari, Turkmen, Gilaki and Arabs ethnic groups using polymerase chain reaction (PCR) followed by polyacrylamide gel electrophoresis (PAGE) and fluorescent capillary electrophoresis. Data was analyzed by Gene Marker HID Human STR Identity software, Gene Pop program and Microsatellite Tools software. Results: Analysis of the allelic frequency revealed the presence of 9 alleles for D7S2456 marker in the Iranian population, which allele 5 at the D7S2456 locus with 55% frequency was the most frequent. The most frequent heterozygosity with rate of 81.8% belongs to Azari ethnic group. Analysis of deviations from Hardy-Weinberg equilibrium demonstrated that all the ethnics except Fars were in equilibrium for D7S2456 locus. D7S2456 marker is a moderately informative marker in Iranian ethnic population (PIC value within 0.44 and 0.7). Conclusion: D7S2456 is a moderately informative marker in diagnosis of SLC26A4 based autosomal recessive non syndromic hearing loss in Iranian population by linkage analysis.

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According to the US Centers for Disease Control and Prevention (CDC) definition, genetic counseling is a process in which information is presented about how genetic conditions affect a patient or his/her family. A genetic counselor collects a patient’s personal and family health history to promote the family’s awareness and perception of specific genetic diseases, testing risks and advantages, disease management, and assessment of available therapeutic options. Intellectual disability (ID) and deafness are two common disabilities with considerable impacts on the quality of life of patients and their families. The present research has investigated the role of genetic counseling in the screening and prevention of deafness and ID based on the studies published in the Web of Science, PubMed, and Google Scholar databases between 2015 and 2023. Genetic counseling can be employed as an influential tool in screening, early diagnosis, and prevention of ID and deafness. Considering that many cases of ID and deafness are rooted in individual genetics, genetic counseling can help lessen the risk factors of developing these disabilities and improve the quality of individual and family life. The effect of genetic counseling, as an influential tool, on screening, early diagnosis, and prevention of ID and hearing loss is also assessed.