Assessment of Peripheral and Central Auditory Processing after Treatment for Idiopathic Sudden Sensorineural Hearing Loss.

Introduction When cases of idiopathic sudden sensorineural hearing loss (SSNHL) are treated successfully, most clinicians assume the normality and symmetry of the auditory processing. This assumption is based on the recovery of the detection ability on the part of the patients, but the auditory processing involves much more than detection alone. Since certain studies have suggested a possible involvement of the central auditory system during the acute phase of sudden hearing loss, the present study hypothesized that auditory processing would be asymmetric in people who have experienced sudden hearing loss. Objective To assess the physiologic and electrophysiological conditions of the cochlea and central auditory system, as well as behavioral discrimination, of three primary aspects of sound (intensity, frequency, and time) in subjects with normal ears and ears treated successfully for SSNHL. Methods The study included 19 SSNHL patients whose normal and treated ears were assessed for otoacoustic emissions, speech auditory brainstem response, intensity and pitch discrimination, and temporal resolution in a within-subject design. Results The otoacoustic emissions were poorer in the treated ears compared to the normal ears. Ear- and sex-dependent differences were observed regarding otoacoustic emissions and pitch discrimination. Conclusion The asymmetrical processing observed in the present study was not consistent with the hearing threshold values, which might suggest that the central auditory system would be affected regardless of the status of the peripheral hearing. Further experiments with larger samples, different recovery scenarios after treatment, and other assessments are required.

Features and evaluation of mucormycosis in COVID-19 patients from two referral hospitals in Iran.

OBJECTIVES: The present study aimed to assess the features, clinical characteristics, and species diversity among patients admitted to referral Hospitals for SARS-CoV-2 pneumonia and mucormycosis in Tehran, Iran, and the relationship between seasonal and species diversity was considered. METHODS: Confirmed COVID-19 patients with a positive reverse-transcriptase real-time (rRT-PCR) test for SARS-CoV2 were primarily included based on clinically suspected mucormycosis infection and confirmed by histopathology and mycology examination of biopsy specimens. The PCR technique was performed by the amplification of the high-affinity iron permease 1 (FTR1) gene for identification and discrimination between Rhizopus arrhizus and non- Rhizopus arrhizus isolates. In contrast, species identification of non-Rhizopus arrhizus was performed by sequencing of ITS rDNA region. RESULTS: Rhino-sino-orbital mucormycosis was identified in the majority of cases (n = 33), with 66 % and 34 % of the cases involving male and female patients, respectively. Rhizopus arrhizus was found to be the most prevalent (84.6 %), followed by Mucor circinelloides (7.6 %). Rhizopus arrhizus was the most prevalent species and present in all the seasons; however, Mucor circinelloides was only present in the autumn. The overall mortality of the total population was 24.6 % (16/ 65); the mortality rates occurring in patients diagnosed with rhino-sino-orbital infection and rhino-sinusal form were 21.4 % and 25 %, respectively. CONCLUSION: CAM can be a serious complication of severe COVID-19, especially in patients with uncontrolled diabetes. It is important to monitor the epidemiology of mucormycosis to raise awareness of the disease and improve diagnosis, treatment and prognosis, particularly in the setting of pandemic.

Predictive role of facial nerve palsy improvement in malignant external otitis.

PURPOSE: To evaluate the effects of different factors on facial nerve palsy improvement in patients with malignant external otitis (MEO) and the predictive role of improvement on MEO. METHODS: Data were collected from all MEO patients with facial paralysis who were hospitalized between 2012 and 2017 at a tertiary referral center. We contacted patients at least 6 months after their admission to evaluate their facial nerve function and survival rate. RESULTS: In a study of 19 samples with a mean age of 69.1 years, 9 patients (47.7%) had some or complete improvement, while 10 (52.6%) had no or very minimal improvement. In this study, there was no statistically significant difference between patients with and without facial nerve palsy improvement in terms of age, sex, usage of antifungal treatment alongside antibiotics, duration of hospital stays, HbA1c level, presentation of hearing loss and vertigo, the severity of facial palsy, comorbidity score, mean of fasting blood sugar, leukocytosis, first ESR and ESR drop, CRP and physiotherapy. We found a positive correlation between improving facial palsy and patients' survival rates. CONCLUSION: Considering the possible influence of facial paralysis improvement prognosis on MEO patients' survival, it could affect our approach to the disease.

Omega-3 Adjunctive Therapy in Idiopathic SSNHL: A Randomised, Triple-Blind, Placebo-Controlled Trial.

Introduction: Idiopathic Sudden Sensorineural Hearing Loss as a subset of sensorineural hearing loss will be confirmed by a progressive hearing loss of at least 30 dB at three contiguous frequencies over 72 hours or less. A sudden or abrupt hearing loss correlates with the time course, and a vascular event is presumptive aetiology. There is an inverse association between Omega-3 consumption and hearing loss. This study aimed to evaluate the efficacy of Omega-3 adjunctive therapy in Idiopathic Sudden Sensorineural Hearing Loss by audiometric assessments. Materials and Methods: In this randomised, triple-blind, placebo-controlled trial, all participants aged 18-70 with a history of sudden deafness (within 12 hours and ≤ 30 days) were eligible for enrollment. They were included if audiology diagnostic tests confirmed the SSNHL. Ultimately, they were randomised to the Omega-3 group and the placebo group. Results: Thirty-three patients were randomly allocated to the Omega-3 group and thirty-two to the placebo group. Vertigo (32.3% of all patients) and underlying conditions had significant relationships with complete response (C.R.)-final hearing level ≤of 25 dB in pure-tone average (P < 0.05). There was no significant difference between both groups before and after treatment. Although it was not statistically significant, patients in the Omega-3 group had faster recovery than placebo. Conclusions: Omega-3 adjunctive therapy did not have a therapeutic effect on SSNHL patients. Moreover, C.R. happened in half the patients. Vertigo and underlying conditions considerably worsen the recovery from SSNHL.

Intratympanic Triamcinolone or Dexamethasone in Sudden Sensory Neural Hearing Loss: A Randomized Clinical Trial.

Sudden sensorineural hearing loss (SSNHL) is a condition in which a person experiences a rapid loss of hearing, often in one ear. The cause of SSNHL is not always clear, but it is thought to be related to issues with the inner ear, auditory nerve, or other parts of the hearing pathway. Intratympanic injection is a treatment method for SSNHL in which a medication (corticosteroids) is injected directly into the middle ear space through the tympanic membrane. This randomized clinical trial was conducted in two hospitals in Tehran, Iran. Patients with SSNHL were randomized (using the 4-block method) and included in this study. 0.5 cc of dexamethasone or triamcinolone was injected via a 25-gauge spinal needle into the cone of light of tympanic membrane. This procedure was repeated six times. In the present study, the rate of response to treatment was compared in the two groups of triamcinolone and dexamethasone, which showed that in the group where intratympanic injection of dexamethasone was performed, 70.9% of patients responded to treatment (48.4% had a complete response and 22.5% had a partial response), and in the group receiving intratympanic triamcinolone, 72.5% responded to the treatment and improved, 50% showed a complete response and 22.5% showed a partial response, and no significant difference was found in the statistical analysis of the two groups. Also, the comparison of audiometric parameters (SDS and SRT) did not show any significant difference before and after the treatment based on the treatment group, and the treatment response was found in the same group. No significant differences were noted in terms of outcome. Intratympanic dexamethasone injection was associated with a 70.9% response and triamcinolone injection with a 72.5% response.

The Use of Herbal Medicine in Sudden Sensorineural Hearing Loss in Diabetic Patients.

Introduction: This study was conducted to evaluate the effect of Doluperine® capsule (curcumin, piperine, and gingerol) on hearing recovery in diabetic patients with Sudden Sensorineural Hearing Loss (SSNHL). Materials and Methods: Fifty-one diabetic patients with SSNHL were randomized to receive two placebo capsules (group 1), a Doluperine® plus one placebo capsule (group 2), or two Doluperine® capsules (group 3). Moreover, all patients had an injection of dexamethasone in the middle ear. Results: The proportion of significant positive changes in PTA, SDS, and SRT was 45.4%, 45.4%, and 36.37% in group1, 84.6%, 84.6%, and 76.92% in group 2, and 70%, 50.0%, and 80.0% in group 3, respectively. Many patients in group 3 did not respond to treatment in the first month, while they recovered at the end of the second month. The chance of recovery reduced with increased time between the onset of symptoms and treatment (delayed visitation) in group 1; however, this finding was not seen in groups 2 and 3. Conclusion: Doluperine® is recommended as a complementary medicine along with steroid therapy for hearing loss improvement in diabetic patients; moreover, this herbal medicine seems to play an important role in recovery in patients with delayed visitation.

Enhancing Medical Students' Knowledge and Performance in Otolaryngology Rotation through Combining Microlearning and Task-Based Learning Strategies.

Microlearning is recommended to be implemented within the context of a wider teaching-learning ecosystem, especially in real working environments. Task-based learning is used in clinical education setting. This study aims at assessing the effect of an integrated approach of microlearning with task-based learning on medical students' knowledge and performance in Ear, Nose and Throat clerkship rotation. A total of 59 final-year medical students participated in this quasi-experimental study which included two control groups (routine teaching and task-based learning methods) and one intervention group (combined microlearning and task-based learning method). Pre- and post-tests of students' knowledge and performance were assessed through a multiple-choice question test and a Direct Observation Procedural Skills (DOPS) instrument, respectively. Performing Analysis of Covariance for knowledge post-test scores among three groups revealed significant differences (F = 3.423, p-value = 0.040) and the intervention group had the highest score. Analyzing DOPS results showed that the intervention group achieved significantly higher scores compared to the control ones for all the expected tasks (p-values = 0.001). The findings of the present study show that the combined strategy of microlearning with task-based learning is an effective clinical teaching method for enhancing medical students' knowledge and performance in a real working environment.

Predictive factors in treatment response of malignant external otitis.

PURPOSE: To evaluate the prevalence and impact of various predictive factors including diabetes control in malignant external otitis (MEO) treatment response. METHODS: In a cross-sectional study on MEO patients, we defined treatment response with three indices; ESR level decrease, hospitalization period, and systemic antifungal drug usage. The impact of diabetes control and other predictive factors on these indices have been evaluated. RESULTS: Overall, 164 patients with a mean age of 67.8 ± 9.7 years were included. Cranial nerve involvement was present in 56 patients. Nine patients had immunodeficiency. 19.5% of cases had leukocytosis. Diabetes mellitus was present in 156 patients, suffering for an average of 13.9 ± 8.6 years. The overall mean hemoglobin A1C (HbA1c) level was 8.3% (4.4-12.8%), and the mean fasting blood sugar was 146.4 mg/dl (63-292 mg/dl). 29.3% of patients had good diabetes control before admission (HbA1c < 7%), 54.9% had poor control (7% < HbA1c < 10%) and 15.9% had very poor glycemic control (HbA1c > 10%). The predictive role for the following factors were not statistically significant: age, gender, comorbidities, diabetes, diabetes management method used before and during hospitalization, diabetes duration, leukocytosis, immunodeficiency, fasting blood sugar level, HbA1c level, glycemic control index, and insulin amount. However, CRP level with a mean value of 34.3 mg/L showed a significant correlation with ESR decrease, hospitalization period, and antifungal drug usage. CONCLUSION: CRP level could be used as a predictor for the hospitalization period, the need for systemic antifungal and ESR level decrease. It would be helpful to check the CRP level at the time of diagnosis to predict the hospitalization period and the necessity of systemic antifungal management to adjust the treatment strategy.

Inaccuracy of Standard 2D Photography for the Diagnosis of Cephalic Malposition of Lateral Crural Cartilages.

Lateral crural cephalic malposition (LCCM) is a well-known deformity of the nasal tip which contributes to functional disturbances of the external nasal valve. Accurate diagnosis of this deformity helps surgeons plan for better outcomes. A total of 176 candidate patients for primary rhinoplasty underwent standard 2D medical photography of the face. Senior authors analyzed photography results and differentiated the patients with LCCM. In addition, we measured the angle between the dorsal septum and lateral end of the long axis of the alar cartilage in the operation room. Ninety-five patients were diagnosed with LCCM on photography. As much as 31.3% (55) of all the patients had LCCM in intraoperative measurements. The sensitivity and specificity of 2D photography for diagnosing LCCM were 0.7924 and 0.5391, respectively. The main surgical techniques for correction of LCCM were alar repositioning (34.3% in total, 56% in LCCM patients) and lateral crural strut graft (43.8% in total, 69% in LCCM patients). LCCM is overdiagnosed via 2D photography, and this method lacks sensitivity. The overall frequency of LCCM seems to be lower than the previously reported frequency. For optimal results in rhinoplasty, surgeons must focus on the best contouring and function rather than solely correcting angles and rotations.

The Effect of Desmopressin Intraoperatively on Hemorrhage During the Rhinoplasty Surgery.

This clinical trial discusses the efficacy of premedication with desmopressin in the management of bleeding and clears the surgical field during rhinoplasty surgery. This study is a randomized, double-blinded placebo-control clinical trial. Seventy patients were enrolled in this study and divided into two equal intervention-control groups. Thirty minutes before surgery, the intervention group received 500 ml of normal saline containing 0.1 µg/kg desmopressin and, the control group received 500 ml of normal saline. According to the surgeon's opinion, the local distribution of bleeding was dramatically different in both groups. While DDAVP receivers had grade 1 or 2 bleeding (according to the FROMME-BOEZAART grading score), the control group had grade 3 or 4 bleeding, and this difference was statistically meaningful. It seems that intravenous DDAVP can reduce bleeding and clear the surgical field during rhinoplasty surgery, but further studies are needed to determine the exact role and dose of the DDAVP.

First molecular report of causative agent of otomycosis due to Aspergillus luchuensis.

Otomycosis is a fungal infection of the external auditory canal caused mainly by the genus Aspergillus. Aspergillus luchuensis, an industrially important fungus, is a member of Aspergillus section Nigri. In this report, we present a case of otomycosis due to Aspergillus luchuensis in a 43-year-old female patient. We performed a partial PCR-sequencing of ß-tubulin and calmodulin genes to identify the isolate to the species level. Further, we determined the in vitro susceptibility of the isolate to nystatin, clotrimazole and itraconazole according to the Clinical & Laboratory Standards Institute (CLSI) M38-A2 protocol. Accordingly, the minimum inhibitory concentrations of clotrimazole, nystatin and itraconazole were 0.25µg/mL, 0.5µg/mL and 1µg/mL, respectively. This is the first report of clinically relevant isolation of Aspergillus luchuensis identified by a molecular technique as a causative agent of otomycosis.

Desmopressin effects on bleeding during functional endoscopic sinus surgery on patients with chronic rhinosinusitis.

OBJECTIVE: In this study we aimed to determine whether Desmopressin (DDAVP) can alter bleeding and improves surgeon visual field and decrease operation time or lessen use of anesthesiology medication in a clinical trial study. METHOD: This study is a randomized clinical trial using the permuted block randomization method. 44 patients were enrolled in study and divided into two equal intervention-control groups. The intervention group received maximum dose of 0/2 micrograms per kg of DDAVP. In the control group, 30 min before the surgery, 100 ml of normal saline will be injected. RESULTS: The amount of bleeding was 517/17 cc in control group during surgery while it was 387/72 cc in group receiving DDAVP which is significantly lower. The satisfaction of surgeon regarding suitable visual field was 6/45 in control group while it was 3/77 in DDAVP receivers which is lower. CONCLUSION: It seems that intravenous DDAVP can reduce bleeding during surgery and offer an enhanced vision for surgeon during surgery but it has no potential efficacy on reduction of period of surgery and need for anesthesiology medication like remifentanil and isoflurane.

Subacute Thyroiditis in COVID-19 Patients.

INTRODUCTION: Since December 2019, novel coronavirus (COVID-19) infection has been identified as the cause of an outbreak of respiratory illness in Wuhan, China. The classic presentation of COVID-19 infection was described as fever, myalgia, cough, and fatigue. Whether coronavirus can directly attack the endocrine glands is unclear. OBJECTIVE: Post-viral subacute thyroiditis (SAT, de Quervain thyroiditis) has been reported following other viral infection. A limited number of SAT after COVID-19 infection have been reported up to now. METHODS: Here, we reported 6 patients with SAT and positive COVID-19 serology tests. Demographic, clinical, biochemical, and imaging data were presented. RESULTS: In this study, 6 patients (4 women and 2 men) with clinician manifestations and physical examination in favor of SAT were described. Cervical ultrasonography showed bilateral hypoechoic areas in the thyroid gland which was suggestive of SAT. Elevated C-reactive protein, erythrocyte sedimentation rate, free thyroxine, free tri-iodothyronine, and undetectable thyrotropin were found in laboratory evaluations. Both IgM and IgG were positive for COVID-19 infection, but the PCR tests were negative in all patients. Patients had history of working in a COVID center and/or family member hospitalized due to COVID-19 pneumonia. Patients were followed up for 1 month and were treated effectively with steroids. CONCLUSION: This report may help physicians to identify lesser-known manifestations and complications of COVID-19. Early diagnosis of COVID-19 infection results in the prevention of further transmission.

Evaluation of Patients with Delayed Endolymphatic Hydrops by Videonystagmography, Vestibular-Evoked Myogenic Potentials, and Electrocochleography.

INTRODUCTION: The categorization of delayed endolymphatic hydrops (DEH) based on the ear which produces vertigo may sometimes cause misdiagnosis. OBJECTIVES: The aim of this study was investigating the vestibular-evoked myogenic potentials (VEMPs), electrocochleography (ECoG), and videonystagmography (VNG) in cases with DEH to determine the ear that originates symptoms. METHODS: In this cross-sectional study, 34 patients - 20 males and 14 females - with profound unilateral sensorineural deafness and vertigo attacks were recruited and evaluated by the ECoG, VNG, and VEMPs tests. RESULTS: The average age was around 43; the summating potential/action potential was abnormal in 29.4% of patients in their normal auditive ear. In 32.4, 17.6, and 50% of cases with a deaf ear, absent, normal, and abnormal VEMPs results were sequentially observed, respectively. In normal-hearing ears, absent, normal, and abnormal VEMPs were observed in 23.5, 50, and 26.5%, respectively. In the normal-hearing ear, the distribution of abnormal VEMPs was 26.5%, and in the deaf ear, this parameter was abnormal in 50% of the opposite ear (p value = 0.00021). In the VNG test, among patients with a normal-hearing ear, results in 27 and 7 patients were sequentially normal and hypofunction. CONCLUSION AND SIGNIFICANCE: The probability of a hypofunction VNG test in a normal-hearing ear might be greater when the VEMPs results of the contralateral deaf ear are normal. In patients with a normal-hearing ear, the distribution of abnormal VEMPs in the contralateral deaf ear is greater, although the intact side may also manifest abnormality in VEMPs tests. The initial evaluation should begin in a deaf ear as well as for the normal-hearing ear ere utilizing ablation surgery.

Evaluation and comparison of the efficacy of long-acting betamethasone and dexamethasone as injections in the treatment of idiopathic sudden hearing loss.

INTRODUCTION & OBJECTIVE: The aim of the present study was to determine the efficacy of long-acting betamethasone, and its comparison with Dexamethasone as an intratympanic injection in the treatment of Sudden Sensorineural Hearing Loss (SSNHL). MATERIALS AND METHODS: Thirty-one patients who do not respond to systemic steroids and poor prognosis patients were enrolled in this study. The patients divided randomly into two groups: 1- Dexamethasone and 2- Long acting betamethasone. Dexamethasone (0.4 ml/mg) or long-acting betamethasone (0.1 ml/mg) was slowly injected (0.4 to 0.6 cc) into the superior-anterior area of the tympanic membrane as 6 injections twice a week for a total of 3 weeks. Right after the treatment and one, two and six months after completion of treatment, an audiometry was performed and compared with the pre-injection values. RESULTS: Speech Reception Threshold (SRT) showed improvements in both groups immediately after treatment and in the follow-up period, compared to baseline. Speech Discrimination Score (SDS) also improved in both groups directly after treatment and at one-month follow-up. The hearing improvement in the Dexamethasone group was clinically better than in the Beta group, but due to the non-parametric data, it was not possible to analyze the hearing improvement process in the variable group. CONCLUSION: According to the results obtained in this study, intratympanic corticosteroid injection in the treatment of patients with SSNHL has positive and promising results on improving hearing level.

Investigation of Etiologic Agents and Clinical Presentations of Otomycosis at a Tertiary Referral Center in Tehran, Iran.

BACKGROUND: Otomycosis is a superficial infection of the ear caused by a spectrum of various fungal agents and its epidemiology depends on geographical region and climatic condition. The aim of this study was to investigate the causal agents and clinical manifestations of otomycosis at a tertiary referral center in Tehran, Iran. METHODS: From Apr 2016 to Jan 2017 a set of 412 subjects with suspicion of external otitis were included. Clinical examination and specimen collection were performed by an otorhinolaryngologist. Subsequently, direct examination and culture were performed on specimens and isolated molds were identified morphologically. Yeast isolates were identified using CHROMagar Candida medium and PCR-RFLP of ribosomal DNA whenever needed. Data were analyzed using SPSS. RESULTS: Otomycosis was confirmed in 117 cases (28.39%) including 64 (54.7%) males and 53 (45.3%) females. Patients were within the age range of 10-75 yr and the highest prevalence was found in the age group of 46-55 yr (30.77%). Pruritus (89.74%) and auditory manipulation and trauma (83.76%) were the predominant symptom and predisposing factor, respectively. Among 133 isolates from 117 patients, Aspergillus niger (n=50, 37.59%) was the most common etiologic agent and Candida glabrata (n=25, 18.8%) was the predominantly isolated yeast. Furthermore, 16 cases of mixed infection were identified and coinfection due to A. niger and C. glabrata (seven cases) was the predominant pattern. CONCLUSION: Our results revealed the high prevalence of C. glabrata and mixed infections in otomycosis patients. Therefore, mycological examinations should be considered for proper treatment.

MTHFR AND ApoE genetic variants association with sudden sensorineural hearing loss.

HYPOTHESIS: Although the pathogenesis of sudden sensorineural hearing loss (SSNHL) is not clear, however several causes including genetic factors seems to be implicated. We hypothesized that common genetic variants might be involved in SSNHL. BACKGROUND: SSNHL is known to be an idiopathic disease because the causative factors have not been identified. Several causes including genetic and viral infection besides immune system reaction, neurological disorders, medications, etc. have been previously reported. We examined the association between ApoE and MTHFR gene variants in SSNHL. METHODS: This study includes case-control scheme encompassing a total of 177 individuals, include patients inflicted with SSNHL and healthy subjects as control group. Genotyping of MTHFR and ApoE variants was conducted by PCR - RFLP method. RESULT: Our study showed that MTHFR rs1801133 allele frequency is significantly different between cases and controls. Also genotype distribution of ApoE was significantly different between patients and healthy controls. CONCLUSIONS: MTHFR C677T and ApoE gene variant may be associated with sudden sensorineural hearing loss in an Iranian population.

eNOS gene Glu298Asp variant confer risk in sudden sensorineural hearing loss.

BACKGROUND: Sudden sensorineural hearing loss (SSNHL) causes the loss of hearing of 30 dB or greater on at least three contiguous frequencies. It is known to be a multifactorial disease which the exact cause is unknown, rendering it as an idiopathic disorder of patients. AIMS/OBJECTIVES: This study aims to shed further light on pathogenesis of this disease by studying the association between eNOS gene Glu298Asp polymorphism and VDR gene FokI polymorphism with SSNHL in Iranian population. MATERIAL AND METHODS: This study involves a total of 77 cases and 100 controls, with patients inflicted with SSNHL categorized in case group and healthy subjects as control group. Genotyping of the VDR and eNOS genes was conducted by the polymerase chain reaction and restriction fragment length polymorphism (PCR-RFLP) method. RESULTS: Our results showed a statistically significant association between genotype frequencies of eNOS gene Glu298Asp polymorphism in case group compared to healthy individuals in the control group (p = .01). Also, TT genotype was significantly the most prevalent genotype in case group in comparison to control group (TT vs GT + GG, OR = 3.5; 95% CI = 1.18-11.79). On the other hand, analysis of VDR gene FokI polymorphism frequencies showed no statistically significant association with SSNHL. CONCLUSIONS AND SIGNIFICANCE: Our findings showed a significant association between the eNOS gene Glu298Asp polymorphism and SSNHL in the Iranian population; and "TT" genotype might be considered as a risk factor for SSNHL.

The first successful application of preimplantation genetic diagnosis for hearing loss in Iran.

Hearing impairment (HI) caused by mutations in the connexin-26 gene (GJB2) accounts for the majority of cases with inherited, nonsyndromic sensorineural hearing loss. Due to the illegality of the abortion of deaf fetuses in Islamic countries, preimplantation genetic diagnosis (PGD) is a possible solution for afflicted families to have a healthy offspring. This study describes the first use of PGD for GJB2 associated non-syndromic deafness in Iran. GJB2 donor splicing site IVS1+1G>A mutation analysis was performed using Sanger sequencing for a total of 71 Iranian families with at least 1 deaf child diagnosed with non-syndromic deafness. In Vitro Fertilization (IVF) was performed, followed by PGD for a cousin couple with a 50% chance of having an affected child. Bi-allelic pathogenic mutations were found in a total of 12 families (~17 %); of which a couple was a PGD volunteer. The deaf woman in this family was homozygous and her husband was a carrier of the IVS1+1G>A gene mutation. Among 8 biopsied embryos, two healthy embryos were implanted which resulted in a single pregnancy and subsequent birth of a healthy baby boy. This is the first report of a successful application of PGD for hearing loss in Iran. Having a baby with a severe hearing impairment often imposes families with long-term disease burden and heavy therapy costs. Today PGD has provided an opportunity for high-risk individuals to avoid the birth of a deaf child.

Laryngeal paraganglioma: a case report.

Paragangliomas in the head and neck are found typically in the region of the carotid body, jugular body, and along the 9th and 10th cranial nerves. They can occur in multicenteric forms, but generally, laryngeal paragangliomas are not found in these patients. Only two cases of laryngeal paraganglioma have been reported with a synchronous lesion elsewhere. We report an additional case of a 34-year-old female with multiple paragangliomas beginning with laryngeal involvement.