Endoscopic trans-canal facial nerve decompression in Melkersson-Rosenthal syndrome: A novel approach.

Key Clinical Message: Melkersson-Rosenthal syndrome can cause recurring bilateral facial paralysis. When steroids fail, surgical decompression of facial nerve is recommended, with endoscopic trans-canal decompression as a safe, minimally invasive, and effective option. Abstract: Melkersson-Rosenthal syndrome (MRS) is a rare neuro-mucocutaneous disorder, clinically diagnosed by a triad of orofacial swelling, recurrent facial palsy, and fissured tongue. Due to the lack of a comprehensive understanding of MRS, there is no accepted standard of care. In this study we report a 30-year-old female patient, who was referred to the otolaryngology clinic of Rasool Akram Hospital, with classical triad of MRS that was managed by endoscopic trans-canal facial nerve decompression. Bilateral endoscopic trans-canal facial nerve decompression was done when we did not find any improvement with systemic steroids. Endoscopic trans-canal facial nerve decompression could be a safe, reliable minimal invasive treatment of facial paralysis in MRS patients. It needs no external incision or temporal bone drilling which makes this method more convenient for patients with shorter recovery time.

Role of Electrodiagnostic Modalities in Detection of Nasal Septal Deviation.

Nasal Septal Deviation (NSD) is a common sign in otorhinolaryngology that can lead to facial asymmetry. In this case-control observational study, we assessed the role of EMG and NCS in the diagnosis of NSD and its effect on neuromuscular function. Participants were divided into two groups based on paranasal sinus computed tomography scan (PNS CT) results: NSD cases (n = 21) and controls without NSD (n = 13). EMG and NCS were performed on both groups to assess nasal alar muscles at the root of the zygomatic nerve. Our findings showed a significant correlation between NSD and EMG/NCS tests (P-value = 000) and a significant association between septal deviation and nasal alar lateralization (P-value = 000). EMG/NCS can be useful in assessing NSD by providing a better understanding of related neuromuscular structures and neuromuscular function of the nasal alar dilator muscles and aid in the diagnosis of NSD. Nasal Septal Deviation, EMG (electromyography), NCS (nerve conduction studies), Neuromuscular function, Facial asymmetry, Otorhinolaryngology, Paranasal sinus, Computed tomography, Nasal alar muscles, Zygomatic nerve, Nasal Obstruction, Nasal alar lateralization, Diagnosis.

Cochlear Implantation in Children with Meningitis: A Multicenter Study on Auditory Performance and Speech Production Outcomes.

BACKGROUND: This study aims to evaluate speech production outcomes and auditory performance in children with post-meningitis deafness who were treated with cochlear implants. Additionally, the study assesses the impact of electrode insertion depth on surgical outcomes.". METHODS: We conducted a study on 66 pediatric patients with bilateral postmeningitis hearing loss who were being prepared for cochlear implantation at four tertiary referral academic institutions. The speech intelligibility rating (SIR) and categories of auditory performance (CAP) were evaluated after the first and second years following implantation. The patients were divided into two groups based on electrode insertion depth: one group had full electrode insertion (more than two-thirds), while the other had partial electrode insertion (less than two-thirds). We compared the SIR and CAP scores between the two groups to assess the impact of electrode insertion depth on outcomes. RESULTS: Before implantation, the median CAP score was one, but it improved significantly to six within two years after the procedure (P-value < 0.001). Similarly, the median SIR score before implantation was one, but it improved significantly to three within two years after surgery (P-value < 0.001). However, there was no significant difference between the partial and full electrode insertion groups in terms of CAP and SIR scores during the follow-up evaluations conducted after the first and second years. CONCLUSION: The study found that cochlear implantation significantly improved speech production skills and auditory performance in children with postmeningitis deafness. Importantly, the amount of electrode insertion at the time of implantation did not have a significant impact on the outcomes.

Genetic insights into PHARC syndrome: identification of a novel frameshift mutation in ABHD12.

BACKGROUND: Mutations in ABHD12 (OMIM: 613,599) are associated with polyneuropathy, hearing loss, ataxia, retinitis pigmentosa, and cataract (PHARC) syndrome (OMIM: 612674), which is a rare autosomal recessive neurodegenerative disease. PHARC syndrome is easily misdiagnosed as other neurologic disorders, such as retinitis pigmentosa, Charcot-Marie-Tooth disease, and Refsum disease, due to phenotype variability and slow progression. This paper presents a novel mutation in ABHD12 in two affected siblings with PHARC syndrome phenotypes. In addition, we summarize genotype-phenotype information of the previously reported patients with ABHD12 mutation. METHODS: Following a thorough medical evaluation, whole-exome sequencing was done on the proband to look for potential genetic causes. This was followed by confirmation of identified variant in the proband and segregation analysis in the family by Sanger sequencing. The variants were interpreted based on the American College of Medical Genetics and Genomics (ACMG) guidelines. RESULTS: A novel pathogenic homozygous frameshift variant, NM_001042472.3:c.601dup, p.(Val201GlyfsTer4), was identified in exon 6 of ABHD12 (ACMG criteria: PVS1 and PM2, PM1, PM4, PP3, and PP4). Through Sanger sequencing, we showed that this variant is co-segregated with the disease in the family. Further medical evaluations confirmed the compatibility of the patients' phenotype with PHARC syndrome. CONCLUSIONS: Our findings expand the spectrum of mutations in the ABHD12 and emphasize the significance of multidisciplinary diagnostic collaboration among clinicians and geneticists to solve the differential diagnosis of related disorders. Moreover, a summary based on mutations found so far in the ABHD12 gene did not suggest a clear genotype-phenotype correlation for PHARC syndrome.

The Therapeutic Outcome of Sialendoscopy in Patients with Sialoadenitis.

Introduction: Recent advances have led to the development of sialendoscopy, an accurate, minimally invasive procedure with high diagnostic and therapeutic capabilities in treating sialolithiasis. This study aimed to evaluate the results and complications of sialendoscopy in patients suffering from sialoadenitis. Materials and Methods: This study was a prospective interventional case series study on patients with sialoadenitis due to sludge or stone formation preoperatively confirmed by sonography or computed tomography (CT) scanning. Diagnostic sialendoscopy was performed, and the presence of stenosis, sludge, or stones inside the gland or duct was examined, and surgery was done. During follow-up time (18.8 ± 7.4 months), recurrence of symptoms, the need for reoperation, and postoperative complications were also assessed. Results: The sialendoscopy was performed in 51 patients, including 55 glands. Forty-five Patients (88.2%) reported pain relief, and 46 patients (90.2%) reported that the treatment using sialendoscopy was better than conservative methods. The duct restenosis also occurred in one patient requiring open surgery. In assessing the main factors predicting the need for reoperation, the site of involvement (parotid versus submandibular glands) and the size of the stone were identified as the main determinants. The best cut-off value for stone size in predicting reoperation requirement was 7.0mm, with a sensitivity of 100% and a specificity of 85.7%. Conclusion: Intraoperative sialendoscopy is a successful diagnostic and therapeutic tool with minimal postoperative complications in salivary gland duct involvement patients.

Complications and outcomes of cochlear implantation in children younger than 12 months: A multicenter study.

OBJECTIVES: Evidence suggests that Cochlear Implantation (CI) is a beneficial approach for auditory and speech skills improvement in children with severe to profound hearing loss. However, it remains controversial if implantation in children <12 months is safe and effective compared to older children. The present study aimed to determine whether children's ages affect surgical complications and auditory and speech development. METHODS: The current multicenter study enrolled 86 children who underwent CI surgery at <12 months of age (group A) and 362 children who underwent implantation between 12 and 24 months of age (group B). The Categories of Auditory Performance (CAP) and Speech Intelligibility Rating (SIR) scores were determined pre-impanation, and "one-year" and "two-year" post-implantation. RESULTS: All children had full insertions of the electrode array. Four complications (overall rate: 4.65%; three minor) occurred in group A and 12 complications (overall rate: 4.41%; nine minor) occurred in group B. We found no statistically significant difference in the complication rates between the groups (p > 0.05). The mean SIR and CAP scores improved over time following CI activation in both groups. However, we did not find significant differences in CAP and SIR scores between the groups across different time points. CONCLUSION: Cochlear implantation in children younger than 12 months is a safe and efficient procedure, providing substantial auditory and speech benefits. Furthermore, rates and nature of minor and major complications in infants are similar to those of children undergoing the CI at an older age.

The Outcome of Cochlear Implantations in Deaf-Blind Patients: A Multicenter Observational Study.

OBJECTIVE: This multicenter study aimed to evaluate the auditory and speech outcomes of cochlear implantation (CI) in deaf-blind patients compared with deaf-only patients. STUDY DESIGN: Retrospective cohort study. SETTING: Multiple cochlear implant centers. PATIENTS: The current study was conducted on 17 prelingual deaf-blind children and 12 postlingual deaf-blind adults who underwent CI surgery. As a control group, 17 prelingual deaf children and 12 postlingual deaf adults were selected. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Auditory and linguistic performances in children were assessed using the categories of auditory performance (CAP) and Speech Intelligibility Rating (SIR) scales, respectively. The word recognition score (WRS) was also used to measure speech perception ability in adults. The mean CAP, SIR, and WRS cores were compared between the deaf-only and deaf-blind groups before CI surgery and at "12 months" and "24 months" after device activation. Cohen's d was used for effect size estimation. RESULTS: We found no significant differences in the mean CAP and SIR scores between the deaf-blind and deaf-only children before the CI surgery. For both groups, SIR and CAP scores improved with increasing time after the device activation. The mean CAP scores in the deaf-only children were either equivalent or slightly higher than those of the deaf-blind children at "12 months post-CI" (3.94 ± 0.74 vs 3.24 ± 1.25; mean difference score, 0.706) and "24 months post-CI" (6.01 ± 0.79 vs 5.47 ± 1.06; mean difference score, 0.529) time intervals, but these differences were not statistically significant. The SIR scores in deaf-only implanted children were, on average, 0.870 scores greater than the deaf-blind children at "12 months post-CI" (2.94 ± 0.55 vs 2.07 ± 1.4; p = 0.01, d = 0.97) and, on average, 1.067 scores greater than deaf-blind children at "24 months post-CI" (4.35 ± 0.49 vs 3.29 ± 1.20; p = 0.002; d = 1.15) time intervals. We also found an improvement in WRS scores from the "preimplantation" to the "12-month post-CI" and "24-month post-CI" time intervals in both groups. Pairwise comparisons indicated that the mean WRS in the deaf-only adults was, on average, 10.61% better than deaf-blind implanted adults at "12 months post-CI" (62.33 ± 9.09% vs 51.71 ± 10.73%, p = 0.034, d = 1.06) and, on average, 15.81% better than deaf-blind adults at "24-months post-CI" (72.67 ± 8.66% vs 56.8 ± 9.78%, p = 0.002, d = 1.61) follow-ups. CONCLUSION: Cochlear implantation is a beneficial method for the rehabilitation of deaf-blind patients. Both deaf-blind and deaf-only implanted children revealed similar auditory performances. However, speech perception ability in deaf-blind patients was slightly lower than the deaf-only patients in both children and adults.

Evaluation of Serum Immunoreactivity to Common Indigenous Iranian Inhalation and Food Allergens in Patients with Meniere's Disease.

BACKGROUND: A few studies investigated the relationship between allergy and Meniere disease considering complete allergen panel. We aimed to evaluate the serum immunoreactivity in patients with Meniere's disease (MD) compared with healthy people according to common indigenous Iranian inhalation and food allergens. METHODS: Thirty-nine patients with MD referred to Rasoul Akram Hospital (Tehran, Iran) were evaluated and compared with a 41 membered control group. A panel of common inhalation and food allergens (using an immunoblotting method), as well as total immunoglobulin E (IgE) level (using the sandwich enzyme-linked immunosorbent assay method), were checked on the patients' serum. RESULTS: The mean total IgE level was 193.85 ± 175.43 IU/ml in the patients with MD and 117.61 ± 138.05 IU/ml in the control group, which was significantly higher than the other subjects in the control group (P = .016). There was a significant difference between the two groups regarding inhalation allergens such as; sweet vernal grass, cultivated rye, cultivated oat, Russian thistle, goosefoot, and rough pigweed (P = .01-0.038). Patients with MD reported more reactive to food allergens such as; rye flour, hazelnut, pepper, citrus mix 2, potato, strawberry, and celery allergens. There was a significant relationship between Meniere and serum immunoreactivity to inhalation and food allergens (both P = .001).Conclusion: Serum total IgE level in patients with MD (in both inhalation and food allergens groups) was higher than the control group, and there was a relationship between MD and immunoreactivity to common indigenous inhalation and food allergens of Iran.

Hyperbilirobinemia and Its Effect on Auditory Perception and Speech Intelligibility in Cochlear Implanted Children (Cochlear Implantation Outcomes in Children Who Have Experienced Hyperbilirobinemia).

The goal of this study was to investigate the probable difference in auditory perception and speech intelligibility performance amongst cochlear implanted children who experienced hyperbilirubinemia or auditory neuropathy in comparison to the cochlear implanted children with unknown etiology for hearing loss. This case-control study was carried out on 106 cochlear implanted children with mean age of 32.36 ± 11.98 months who were purposively selected and allocated into four groups. Out of the total, 30 had no specific etiology for hearing loss, while the others had experienced auditory neuropathy or hyperbilirubinemia with/without blood exchange. The auditory perception and speech intelligibility performance of all the participants who had received auditory verbal therapy were assessed after 6 and 12 months of rehabilitation. Then, the data was analyzed, using the Statistical Package for Social Sciences-version 21(SPSS-21). Results indicated poor auditory perception and speech intelligibility performance of the cochlear implanted children with hyperbilirubinemia and blood exchange (P ≤ 0.05), while the participants in the control group with no specific etiology for hearing loss, the children with hyperbilirubinemia with no blood exchange, and those who suffered from auditory neuropathy performed better, respectively. Also, a significant correlation between auditory neuropathy and hyperbilirubinemia was observed. Despite lower improvement of auditory perception and speech intelligibility of the hearing impaired children who were experiencing moderate to severe degrees of hyperbilirubinemia or auditory neuropathy, cochlear implantation is highly recommended not only for children with unknown etiology for severe hearing loss but also for this group of hearing impaired children.

Simulation and In Vitro Experimental Studies on Targeted Photothermal Therapy of Cancer using Folate-PEG-Gold Nanorods.

BACKGROUND: Selective targeting of malignant cells is the ultimate goal of anticancer studies around the world. There are some modalities for cancer therapy devastating tumor size and growth rate, meanwhile attacking normal cells. Utilizing appropriate ligands, like folate, allow the delivery of therapeutic molecules to cancer cells selectively. There are a variety of photosensitizers, like gold nanorods (GNRs), capable of absorbing the energy of light and converting it to heat, evidently build a photothermal procedure for cancer therapy. OBJECTIVE: To develop a one-step approach for calculating the temperature distribution by solving the heat transfer equation with multiple heat sources originating from NIR laser-exposed GNRs. MATERIAL AND METHODS: In this experimental study, we simulated NIR laser heating process in a single cancer cell, with and without incubation with folate conjugated PEG-GNRs. This simulation was based on a real TEM image from an experiment with the same setup. An in vitro experiment based on aforesaid scenario was performed to validate the simulated model in practice. RESULTS: According to the simplifications due to computational resource limits, the resulting outcome of simulation showed significant compatibility to the supporting experiment. Both simulation and experimental studies showed a similar trend for heating and cooling of the cells incubated with GNRs and irradiated by NIR laser (5 min, 1.8 W/cm2). It was observed that temperature of the cells in microplate reached 53.6 °C when irradiated by laser. CONCLUSION: This new method can be of great application in developing a planning technique for treating tumors utilizing GNP-mediated thermal therapy.

Endoscopic Versus Microscopic Cartilage Myringoplasty in Chronic Otitis Media.

INTRODUCTION: Operations on the tympanic membrane of the middle ear, myringoplasty, and tympanoplasty are now widely accepted, and attempts are underway all over the world to standardize the surgical techniques. This study aimed to compare postoperative outcomes of endoscopic and microscopic cartilage myringoplasty in patients suffering from chronic otitis media (COM). MATERIALS AND METHODS: This clinical trial study compared 130 patients with COM who underwent transcanal endoscopic myringoplasty by repairing perforation using auricular concha cartilage under general anesthesia (n=75) and conventional repairing method by postauricular incision and tympanomeatal flap elevation under microscopic surgery (n=55). RESULTS: According to the results, there was no significant difference between the two groups in terms of hearing gain 1, 6, and 12 months after surgery (P=0.063); however, higher hearing gain scores were observed in the endoscopic group. Moreover, lower recovery time and post-operative pain were reported in patients who underwent the endoscopic approach, compared to those who treated with the conventional repairing method (P<0.001). CONCLUSION: Endoscopic myringoplasty technique is a safe and effective way to improve hearing loss as much as the conventional method. However, due to the lower recovery time and post-operative pain, it seems to be the method of choice in myringoplasty surgery.

Balance function after cochlear implant and inner ear anomaly: Comparison of dynamic posturography.

INTRODUCTION: Patients with sensorineural hearing loss suffer concomitant vestibular dysfunction that is more prevalent in patients with inner ear anomaly and could be aggravated with cochlear implantation. To assess the vestibular dysfunction in patients with cochlear implantation, we compared their results with those of patients with and without inner ear anomaly. MATERIALS AND METHODS: This is a historical cohort study lasting for 20 years on 50 patients with cochlear implantation. All patients underwent dynamic posturography and Bruininks-Oseretsky Test. RESULTS: Twenty-two (44%) of the participants showed some types of inner ear anomaly. The frequency of abnormal Bruininks-Oseretsky Test was 45.5% and 10.7% (P = .005, odds ratio [OR] = 6.9). Abnormal composite was seen in 77.3% and 21.4%, respectively (P < .001; OR = 12.5). The mean strategy score in the fifth condition of the sensory organization test was 25.0 ± 20.4 in patients with inner ear anomaly, whereas it was 44.1 ± 18.9 in those without it (P = .001). CONCLUSION: Balance capability in cochlear implantation patients with inner ear anomaly compared to those without inner ear anomaly was worse. More vestibular rehabilitation treatment plans are suggested for these patients.

New marsupialization technique in endolymphatic sac surgery.

OBJECTIVES: The aim of the present study was to describe and evaluate the results of a new technique in endolymphatic sac decompression surgery. METHODS: Forty-three patients with intractable unilateral Meniere's disease were selected. Endolymphatic sac was identified after simple mastoidectomy, and its lateral layer was incised, using a sickle knife. Outer layer of the sac was turned around and placed under the anterior bony border. RESULTS: Mean duration of the follow-up was 24 months. Mean tinnitus handicap index, pure tone average (PTA) on thresholds at 500, 1000, 2000, and 4000 Hz, mean speech reception threshold, mean speech discrimination score, hearing stage, and mean vertigo score before and after surgery were evaluated. CONCLUSION: The new marsupialization technique with anterior bony border is a safe and effective way to improve tinnitus, vertigo, and ear fullness among these patients. According to PTA and hearing stage, this surgery can control progressive hearing loss. LEVEL OF EVIDENCE: 3.

Impact of Acamprosate on Chronic Tinnitus: A Randomized-Controlled Trial.

OBJECTIVES: Tinnitus is a common and distressing otologic symptom, with various probable pathophysiologic mechanisms, such as an imbalance between excitatory and inhibitory mechanisms. Acamprosate, generally used to treat alcoholism, is a glutaminergic antagonist and GABA agonist suggested for treating tinnitus. Thus, we aimed to evaluate the efficacy and safety of acamprosate in the treatment of tinnitus. METHODS: The current randomized-controlled trial study included 20 subjects with chronic tinnitus. After performing psycho-acoustic, psychometric and electrophysiological evaluations, all studied tinnitus subjects were randomly divided into two groups of acamprosate and placebo. The first group received oral acamprosate (two tablets of 333 mg/d, three times a day), whereas the second group was given placebo treatment (two tablets, three times a day). After the first 30 days, all evaluations were repeated for the studied groups just in the same manner before the study. Subsequently, the final results of each evaluation were compared together with the baseline values. RESULTS: Nine studied subjects randomly received acamprosate, whereas eleven others received a placebo. There was no significant improvement in the psycho-acoustic tests, except a decrease was observed in the pitch match of tinnitus (P = .039). For those subjects who were receiving acamprosate, a significant reduction was observed in tinnitus handicap inventory (P = .006), tinnitus questionnaire scores (P = .007), and the visual analog scores (P = .007) compared to the placebo group. There was a significant reduction in Action Potential latency (P = .048) as well as an increase in the amplitude of distortion product otoacoustic emissions at 4 kHz (P = .048). CONCLUSIONS: The study results indicated a subjective relief of tinnitus as well as some degree of the electrophysiological improvement at the level of the cochlear and the distal portion of the auditory nerve among the subjects who received the acamprosate. CLINICAL TRIAL REGISTRATION CODE: IRCT2013121115751N1.

Limbic System Associated Membrane Protein Mutation in an Iranian Family Diagnosed with Ménière's Disease.

BACKGROUND: Ménière's disease (MD) is a common inner ear disorder which is characterized by recurrent attacks of vertigo, fluctuating sensorineural hearing loss (SNHL), tinnitus, and a sense of fullness in the affected ear. MD is a complex disorder; although six genes have been linked to familial autosomal dominant form of the disease, in many cases, the exact genetic etiology remains elusive. METHODS: To elucidate the genetic causes of MD in an Iranian family, we performed exome sequencing on all members of the family: consanguineous parents and four children (two affected and two unaffected). Variant filtering was completed using a customized workflow keeping variants based on segregation with MD in autosomal recessive (AR) inheritance pattern, minor allele frequency (MAF), and in-silico prediction of pathogenicity. RESULTS: Analysis revealed that in this family, 970 variants co-segregated with MD in AR pattern, out of which eight variants (one intergenic, four intronic, and three exonic) were extremely rare. The exonic variants included a synonymous substitution in USP3 gene, an in-frame deletion in ZBED2 gene, and a rare, highly conserved deleterious missense alteration in LSAMP gene. CONCLUSION: The phenotype observed in the proband described here, i.e. vertigo, poor sense of smell, tinnitus, and borderline hearing ability, may originate from aberrant changes in the cerebellum and limbic system due to a deleterious mutation in the LSAMP gene; hence, LSAMP mutation is a possible candidate for the etiology of MD in this family.

Titanium Snoreplasty- A New Surgical Technique.

INTRODUCTION: Based on the previous data, among the general population aged between 30 and 60 years, snoring is observed in 44% and 28% of males and females, respectively. Therefore, it is important to treat snoring to reduce the disruption of the bed partner's sleep and the patients' own problems. This study aimed to present a minimally invasive procedure which is easy to perform with less tissue damage. MATERIALS AND METHODS: This study included 13 patients suffering from primary snoring with soft palate length of 2.5cm or more. All of the patients were examined and their partners were asked to fill-out the relevant questionnaires at baselines, 90 days, 6 months and 1 year after the surgery in order to assess snoring. A crescent strip of oral mucosa along with the underlying muscle were removed under general anesthesia followed by the insertion of a piece of oval-shaped titanium mesh. Moreover, two subjective methods were employed to assess the snoring of all patients. RESULTS: 11 patients were male, and the mean age and the mean body mass index of the patients were 48.69 years and 28.34 kg/m2, respectively. The scores obtained from the Visual Analog Scale for snoring loudness before surgery reduced from 7.63 to 3.54, which was statistically significant (P<0.05). None of the patients experienced major complications after surgery; however, there was a partial extrusion of the implant in one case which was managed conservatively with spontaneous healing. CONCLUSIONS: Titanium snoreplasty was successful in the reduction of snoring in this study. This method is a single-stage treatment for simple snoring with the multiple effects of palatal shortening, space increasing, and palatal stiffening.

Cochlear implantation in children with inner ear malformation: A multicenter study on auditory performance and speech production outcomes.

OBJECTIVES: This multicenter study evaluated the auditory performance and speech production outcomes of cochlear implantation in children with inner ear anomaly and compared the outcomes of patients with different kinds of malformation. METHODS: Cochlear implantation was performed in 107 children with inner ear malformation at four tertiary academic centers. The categories of auditory performance (CAP) and speech intelligibility rating (SIR) scores were evaluated preoperatively and one year and two years postoperatively. RESULTS: Types of inner ear malformation and their frequencies were: incomplete partition type-I, 19 (17.8%) patients; incomplete partition type-II, 31 (29%), common cavity, 17 (15.9%), cochlear hypoplasia, 17 (15.9%), and isolated enlarged vestibular aqueduct (isolated EVA), 23 (21.5%) patients. EVA was the coexisting anomaly in 27(25.2%) subjects. The median CAP and SIR scores improved significantly during the first two years after cochlear implantation in all groups (p-values <0.001 and < 0.001, respectively). No significant difference was seen in CAP and SIR scores of children with different inner ear malformations (p-value = 0.147 and 0.570, respectively) or in patients with isolated EVA compared to coexisting EVA (p-value = 0.538 and 0.075, respectively). CONCLUSION: The median CAP and SIR scores two years after surgery were 5 (Understanding of common phrases without lip-reading) IQR: 4-6, and 3 (Connected speech is intelligible to a listener who concentrates and lip-reads within a known context) IQR: 3-4, respectively. Auditory performance and speech production were significantly improved in all inner ear malformation patient groups, and no significant difference was observed between the scores of patients with different types of anomaly.

Toward An Exploration of Habituating to Tinnitus: Perspectives on Sensory Gating.

BACKGROUND: Some tinnitus participants habituate to their tinnitus, but some others do not and complain of its annoyance so much. It has been suggested that tinnitus is a habituation deficit. Habituation and the ability to ignore a sensory input depend on the normal function of filtering mechanism of sensory gating. PURPOSE: The purpose of this study was to compare behavioral aspects of sensory gating in normal and tinnitus participants to search for the reason why some tinnitus participants habituate to their tinnitus but some others do not. RESEARCH DESIGN: This investigation was an observational case-control study. STUDY SAMPLE: There were 60 tinnitus participants who were categorized into two tinnitus groups (30 compensated tinnitus participants and 30 decompensated tinnitus participants) based on the complaint of tinnitus annoyance, visual analog scale (VAS) for tinnitus loudness, annoyance, and awareness, scores on Tinnitus Questionnaire (TQ), and Tinnitus Handicap Inventory (THI). Also, there were 30 normal hearing participants without tinnitus as the normal control group. DATA COLLECTION AND ANALYSIS: Sensory Gating Inventory (SGI), TQ, THI, and VAS was obtained from all participants. THI, TQ, VAS, and SGI total scores and their factors were compared among the groups. Correlations between SGI scores with THI, TQ, and VAS score were calculated. RESULTS: The results showed that SGI total score and the scores of its four factors were significantly higher in decompensated tinnitus participants compared with compensated tinnitus participants and normal controls. Also, there was a positive correlation between SGI perceptual modulation factor and TQ emotional distress factor and with the VAS for loudness in decompensated tinnitus group. CONCLUSIONS: These results suggested that tinnitus associated with behavioral aspects of sensory gating and decompensated tinnitus may be a result of deficient sensory gating.

The potential role of auditory prediction error in decompensated tinnitus: An auditory mismatch negativity study.

INTRODUCTION: Some tinnitus subjects habituate to their tinnitus but some others do not and complain of its annoyance tremendously. Normal sensory memory and change detection processes are needed for detecting the tinnitus signal as a prediction error and habituation to tinnitus. The purpose of this study was to compare auditory mismatch negativity as the index of sensory memory and change detection among the studied groups to search for the factors involving in the perception of tinnitus and preventing habituation in decompensated tinnitus subjects. METHODS: Electroencephalography was recorded from scalp electrodes in compensated tinnitus, decompensated tinnitus, and no tinnitus control subjects. Mismatch negativity was obtained using the oddball paradigm with frequency, duration, and silent gap deviants. Amplitude, latency, and area under the curve of mismatch negativities were compared among the three studied groups. RESULTS: The results showed lower mismatch negativity amplitude and area under the curve for the higher frequency deviant and for the silent gap deviant in decompensated tinnitus group compared to normal control and compensated tinnitus group. CONCLUSIONS: This study revealed a deficit in sensory memory and change detection processing in decompensated tinnitus subjects. This causes persistent prediction errors; tinnitus signal is consistently detected as a new signal and activates the brain salience network and consequently prevents habituation to tinnitus. Mismatch negativity is proposed as an index for monitoring tinnitus rehabilitation.

Developing the Persian Version of Sensory Gating Inventory and Assessing Its Validity and Reliability.

INTRODUCTION: Sensory Gating Inventory (SGI) measures behavioral aspects of Sensory Gating (SG), which filters irrelevant sensory inputs into the higher cortex. It modifies sensitivity to sensory stimuli. Abnormal SG leads to overloading of information in the brain and its subsequent dysfunction. Electrophysiological techniques cannot assess the behavioral aspects of SG. We aimed to design the Persian version of SGI with high validity and reliability. METHODS: After a forward and then backward translation of the original SGI, we assessed the content validity and construct validity of the Persian version. A total of 405 participants filled the Persian version of SGI. To assess test-retest reliability, 100 participants filled the inventory again 7-10 days later. The content validity ratio and index, as well as confirmatory factor analysis, were computed, too. Finally, the Cronbach's alpha, Cohen's kappa, and intraclass correlation coefficients were calculated. RESULTS: The content validity ratios of all items of the inventory were more than 60%, which means that they were necessary according to the experts' opinions. Confirmatory factor analysis confirmed the fitness of the 4-factor structure of the original Inventory. The test-retest reliability using the intraclass correlation coefficient and Cronbach's alpha coefficients were also high for the four subscales. The Cohen's kappa coefficients revealed moderate to substantial level of agreement between the first and second scores for all items. CONCLUSION: The Persian version of SGI has good and acceptable psychometric properties. It can be used as a valid and reliable tool for studying behavioral aspects of SG in Persian speaking population.