Background: Thyroidal hormones influence the inner ear development. Hearing is one of the most important senses in human. Normal hearing in early stage of life, especially in first six months, is essential for speech and education and communication. So diagnosis and treatment of hearing loss in young children is very critical and important. Therefore, high risk finding and hearing loss screening in these groups is essential. Method and Material: This study was performed upon 50 patients who referred to Imam Reza Hospital with congenital hypothyroidism, The patient was studied according their sex, age at diagnosis, familial history, etiology, clinical presentation, and other ENT abnormalities. Hearing threshold was evaluated by ABR. Result: 38% of patients were male and 62% were female. Mean of age at initial management was 10.96 months. 32% had positive familial history. Etiology was dysgenesy in 64%, dyshormonogenes in 26%, unknown in 24% and transient in 4%. The most common presentation was prolonged jaundice 18% had purulent PND and Rhinorrhea. Prevalence of hearing loss was 22% and speech abnormality 16% There was no relationship between hearing loss and sex, age at the time of diagnosis, and thyroidal hormone level. Conclusion: prevalence of hearing loss was 22% in this study, and it was fairly high. Most of our patients were detected before 6 months age. when it was most important for diagnosis and rehabilitation of hearing loss. We believe that it is essential to screen all congenital hypothyroid patients for hearing loss and treat them as soon as diagnosed.

Introduction: Diabetes Mellitus is the most prevalent endocrine disease. It causes several complications, such as retinopathy and nephropathy. The aim of this study is to evaluate the effect of diabetes mellitus on sensory-neuron auditory impairment.       Materials and Methods: We conducted this case - control study during the years of 2006-2008 with the collaboration of Mashhad Diabetes Center and the Audiometric Clinic at Ghaem Hospital. Considering the inclusion and exclusion criteria, 300 diabetic patients, as the case group and 300 persons as the control group entered this study. These two groups were matched by sex and age. Audiometric examinations, consisting of Pure Tone Audiometric Test, Diapason Tests and Autoscopy were administered on the participants in the present study.Results: There was a significant difference between control and case groups in sensory-neuron auditory impairment at high and low-median frequencies. Also, there was not any association between the duration of the disease and the level of sensory-neuron auditory impairment (P>0.05). In addition, the findings indicate a significant relationship between the complicated and uncomplicated groups in terms of the level of sensory-neuron auditory impairment at high frequencies. Patients with type II Diabetes Mellitus showed prominent sensory-neuron auditory impairment at high and low-median frequencies, as compared to the control group (P<0.05). There was not such association in Type I Diabetes Mellitus (P>0.05). Furthermore, there was a significant difference between two types of Diabetes Mellitus in the level of sensory-neuron auditory impairment. which was prominent in high frequencies (P<0.05). Also, this study showed that there was not any sex difference in the sensory-neuron auditory impairment (P>0.05).Conclusion: We propose the necessity of administrating regular audiometric examinations for the patients with diabetes mellitus. Early detection of sensory-neuron auditory impairment can decrease its progression due to better glycemic control.

Objective: Use of hearing aids is a common and an important programming of auditory rehabilitation. Hearing aid is an important device especially in children with hearing loss, because good hearing is effective for development of speech. The aim of this study was to assess the usefulness and disadvantages associated with use of this device among patients with hearing loss.Subjects and Methods: This cross-sectional retrospective and descriptive study included 100 patients, visiting Social Support Organization (behzisty center) of Ahvaz, with sensory-neural hearing loss on hearing aids. Demographic charteristics, medical records history, options of hearing aids, advantages and disadvantages of those were analyzed.Results: Of the 100 cases of sensory neural hearing loss, 68 % of patients were under 20 years old. Eighty three % of cases were psychologically satisfied with their hearing aid, and 55% had good progress in their education. Eighty four % of cases used post-auricular hearing aids. The disadvantages reported of using hearing aid were: not enough power (18%), ear mold impression (24%) and allergy to the mold (12%).Conclusion: Hearing aids are useful in patients with sensory neural hearing loss and can be used with comfort in majority of patients who need them. Good selection of hearing aid, suitable mold and teaching the patients can reduce the disadvantages associated with use of hearing aids.

Introduction: Regarding the high incidence rate of hyperlipidemia in the society and the adverse effects of its complications including the occurrence of hearing loss in some of the patients, we decided to do this research.Materials and Methods: This is a cohort study, in cooperation with ENT units of Islamic Azad University Mashhad Hospitals, which was carried out in 2008-2009 in Mashhad to assess the effect of hyperlipidemia on hearing. Ninety hyperlipidemic cases and 90 controls that were matched in terms of both age (two groups 30-50 years and 50-70 years) and sex entered the study. All the cases answered the questionnaire before the tests of audiometry. Then, those who were diagnosed with impaired hearing due to other factors rather than hyperlipidemia were excluded from the study. Audiologic exams on 180 cases (including both the case and control groups) included pure tone audiometry, diapasonic tests and otoscopy.Results: This study revealed that there was a significant difference in hearing loss in 5 frequencies (250, 1000, 2000, 4000, 8000 HZ) between two groups of case and control (P<0.05).Hypertriglyceridemic patients showed a distinct hearing loss in 4 frequencies (250, 2000, 4000, 8000 HZ) comparing the control group and hypercholesterolemic patients (P<0.05). Hypercholesterolemic patients were found to have hearing loss just in 1000 HZ of frequency, as compared to the control group and hypertriglyceridemic patients. Also, the findings of this study suggest that the sex of the patients had no influence on hearing loss (P>0.05).Conclusion: According to our results, regular and periodic tests of audiometry are recommended for the hyperlipidemic patients to find out a history of audiologic problems and ultimately prevent the subsequent complications.

Objective: The thalassemias are among the most common genetic disorders worldwide, occurring more frequently in the Mediterranean region. The aim of this study was to determined frequency of sensory-neural hearing loss in major b- thalassemias transfusion dependent patients in south of Iran.Methods: A cross sectional study on 308 cases of major beta-thalassemia patients referring to Thalassemia Center of Shiraz University of Medical Sciences between 2006-2007 years. The diagnosis of b- thalassemia major was based on clinical history, complete blood count and hemoglobine electrophoresis. Clinical data such as serum ferritin level, deferoxamine (DFO) dose, mean daily doses of DFO (mg/kg) and audiometric variables was recorded. Findings: Out of 308 cases, 283 (96.5%) had normal hearing and 10 (3.5%) sensorineural hearing loss. There was no statically significant difference between two groups regarding mean age, weight, age at the first blood transfusion, age at the first DFO infusion.Conclusion: We found the lowest incidence of sensorineural hearing loss in a large population of patients suffered from major thalassemia who received DFO. We show that DFO is not ototoxic at a low dose. When considering all related literature, as a whole there has been much critical misrepresentation about DFO ototoxicity.

Introduction: Multiple sclerosis (MS) is a lifelong disease of the brain and spinal cord. Fingolimod is an oral drug which modulates the S1P receptor and is used for relapsing remitting form of MS and can causes rebound activity if it is ceased even in a short period of washout time. Case Report: Here, we introduce a young girl, a known case of MS, who developed reversible isolated unilateral sensory-neural hearing loss along imaging activity two weeks after stopping fingolimod. The patient responded well to the intravenous corticosteroid therapy which is the first line treatment of new MS attack. Conclusion: fingolimod cessation can cause rebound activity in a short period of the time. It is important to consider any new neurological sign and symptom as a rebound activity during washout time. Although SNHL is not common in MS, it could be presented as an unusual manifestation of rebound relapse after stopping fingolimod.

Background & Aim: Consanguineous marriage is strongly favored in many large human populations. In most parts of South Asia, consanguineous marriage accounts for 20-50% of the total present generation. The effect of consanguinity on hereditary deafness has been well studied and documented. Many authors have suggested that approximately one half of sensory neural hearing loss in children can be attributed to hereditary causes. The aim of this research is to evaluate the prevalence of consanguineous marriage in children with SNHL.Patients & Method: This research was carried out in rehabilitation centers of welfare organization in seven provinces of Iran. 1352 infants and preschool children with SNHL participated in this study. To collect data, participants were asked to fill out questionnaires including personal history, medical history, and hearing loss history. Results: Consanguinity was found among 45.7% of children whose parents were first cousins and 17.2% of children whose parents were second cousins. Hereditary factors were thought to be the cause of bilateral SNHL in 863 children (62.9%). Conclusion: The incidence of hereditary hearing impairment is very high in developing countries compared to developed ones. Prevention seems essential to reduce the incidence of genetic hearing loss. Consanguinity should be discouraged and genetic counseling is to be applied at least for those at risk of developing genetic diseases including hearing impairment.

Background and Aim: Auditory rehabilitation is an essential step after hearing aid fitting for children with hearing loss. The aim of this study was to evaluate the auditory rehabilitation results based on auditory verbal therapy approach in children with severe to profound sensory-neural hearing loss. Auditory verbal therapy is a popular approach for rehabilitation children with hearing loss. Methods: This is a retrospective study that eva-luates the results of auditory rehabilitation of 19 children with severe to profound hearing loss. Most children were about three years old at the beginning of the rehabilitation process. They were received bilateral hearing aid and auditory rehabilitation based on auditory verbal therapy (AVT) approach. Each case progress was meas-ured by Newsha developmental scale. Results: All of the children showed progress during the AVT program (p < 0. 05). The Newsha developmental scale showed that hearing and receptive language categories had more progress. The progress was similar between male and female participants and there was no difference in all of categories (p > 0. 05). Conclusion: The AVT approach is beneficial for rehabilitation of older children with bilateral sev-ere to profound sensory-neural hearing loss. The progress was observed in all categories of Newsha developmental scale.

Objective: Mumps infection is endemic in Iran and mumps parotiditis is a common disease in Iranian children. There has been a dramatic decrease in the worldwide incidence of mumps since the introduction and use in 1968 of the very effective and inexpensive mumps vaccine. In Iran probably due to a higher percentage of unvaccinated young persons <15yr, the incidence rate of mumps infection and its sequel are higher in comparison to corresponding data from developed countries prior to comprehensive vaccination programs. The aim of the study was to investigate the effects of the mumps virus on cochlear function and to determine the frequency of related Sensory Neural Hearing Loss (SNHL) in children. Material& Methods: This descriptive case-series study was conducted in 94children, aged less than 14 years, hospitalized between 1999 and 2001, in the pediatric ward of the Hazrat Rasool Hospital in Tehran. All patients with documented mumps infection (specific mumps-IgM antibody) were evaluated twice for audiometeric function on the basis of diagnostic parameters for sensory neural hearing loss; the first evaluation was done on admission and the second three weeks later. 54 patients (age range 1-14y, mean age 4.83±3.93, male: female ratio30:24) were studied in two years. Results: The highest incidence of mumps was seen in winter (37%) and spring (28%) and the lowest in summer (13%). Specific IgM antibody for mumps virus was detected in 74 children. Comprehensive audiologic evaluation was done in 54 patients at admission and again 3 weeks later. SNHL was detected in 7.2% of patients; the 4.4% incidence of SNHL in this study was higher than in other studies in developed countries prior to comprehensive vaccination programs. Conclusion: Implementation of comprehensive vaccination programs in young Iranians could dramatically reduce the burden and costs imposed by the infection and its sequelae.

Background and Aim: Hearing loss is one of the most common disorders in the elderly people. Sensory neural hearing loss mostly results from outer and inner hair cell damages in cochlea. The area wherein inner hair cells or auditory nerve fibers are inactive is named Dead Region (DR) or Cochlear dead Region (CDR). Many studies have investigated DR at high frequencies in sloping or sharply sloping audiograms, but few studies have investigated flat audiograms. Hence, the aim of the present study was to investigate the prevalence of DR in hearing impaired adults with mild to severe flat sensory neural hearing loss. Materials and Methods: In the current descriptive-analytical study, 30 elderly people (60 ears) aged 65 years and above, with bilateral mild to severe flat sensory neural hearing loss were tested for DR at frequencies from 0. 5 to 4 kHz using the threshold equalizing noise (HL) test. Based on the hearing thresholds, participants were divided into three groups of mild, moderate, and severe hearing loss. Results: The prevalence of DR at 2 kHz, 3 kHz, and 4 kHz were significantly higher than DR at frequencies below 2 kHz (p<0. 05). With increasing the amount of hearing loss, the number of DR increased. As a result, the difference between prevalence of DR in severe hearing loss and the other two groups was statistically significant (p<0. 05). Conclusion: The results indicated a relatively high prevalence of dead regions in the elderly with severe degree of hearing loss (60-80dBHL) and at high frequencies. Furthermore, with increasing frequency, the number of dead regions increased correspondingly.