Cochlear implantation in incomplete partition type I.

OBJECTIVES: To share our experience in cochlear implanted patients with incomplete partition type I, to compare it with the literature results and to disclose difficulties facing cochlear implant teams dealing with these patients. MATERIALS AND METHODS: Clinical records of 1089 cochlear implant procedures in a cochlear implant center were reviewed and data of patients who had incomplete partition type I were enrolled in this study. Their auditory and speech performances were evaluated 3 years after the implantation. RESULTS: Eighteen cases (1.65%) had incomplete partition type I. Cerebrospinal fluid gusher was encountered during opening the cochlea in 15 patients (83.3%). There were no cases of persistent CSF leak or postoperative meningitis. In 61.1% of patients, some additional anomalies were found during the operation. Although in 55.6% of cases no electrically evoked compound action potential was detected even in long-term follow-up, all patients had satisfactory auditory and speech outcome. CONCLUSION: Cochlear implantation is a relatively safe and effective treatment for patients who have incomplete partition type I, even if the procedure may be somehow challenging.

A National Iranian Cochlear Implant Registry (ICIR): cochlear implanted recipient observational study.

BACKGROUND AND OBJECTIVE: Patients who receive cochlear implants (CIs) constitutes a significant population in Iran. This population needs regular monitor on long-term outcomes, educational placement and quality of life. Currently, there is no national or regional registry on the long term outcomes of CI users in Iran. The present study aims to introduce the design and implementation of a national patient-outcomes registry on CI recipients for Iran. This Iranian CI registry (ICIR) provides an integrated framework for data collection and sharing, scientific communication and collaboration inCI research. METHODS: The national ICIR is a prospective patient-outcomes registry for patients who are implanted in one of Iranian centers. The registry is based on an integrated database that utilizes a secure web-based platform to collect response data from clinicians and patient's proxy via electronic case report forms (e-CRFs) at predefined intervals. The CI candidates are evaluated with a set of standardized and non-standardized questionnaires prior to initial device activation(as baseline variables) and at three-monthly interval follow-up intervals up to 24 months and annually thereafter. RESULTS: The software application of the ICIR registry is designed in a user-friendly graphical interface with different entry fields. The collected data are categorized into four subsets including personal information, clinical data, surgery data and commission results. The main parameters include audiometric performance of patient, device use, patient comorbidities, device use, quality of life and health-related utilities, across different types of CI devices from different manufacturers. CONCLUSION: The ICIR database could be used by the increasingly growing network of CI centers in Iran. Clinicians, academic and industrial researchers as well as healthcare policy makers could use this database to develop more effective CI devices and better management of the recipients as well as to develop national guidelines.

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.

Effect of depth of general anesthesia on the threshold of electrically evoked compound action potential in cochlear implantation.

The purpose of the present study was to evaluate effect of depth of general anesthesia on the threshold of electrically evoked compound action potential in cochlear implantation. A prospective clinical study in a single-subject design was conducted in the cochlear implant center of a tertiary care University-based hospital. Sixty-one cochlear-implanted children with bilateral, severe to profound sensory neural hearing loss were enrolled in the study. During the operation electrically evoked compound action potentials (e-ECAP) were measured in two phase of general anesthesia; in deep and in light anesthesia. Thresholds of e-ECAP in these two phases of anesthesia were compared. Thirty-one children received HiRes90k1j prosthesis and 30 children received CI24RE prosthesis. Thresholds difference of electrically evoked compound action potential between light and deep anesthesia in all tested electrodes in either group were statistically significant (P < 0.001). Non-measurable e-ECAP in some electrodes at deep anesthesia was measurable in light phase of anesthesia. Depth of anesthesia can have significant influence on e-ECAP threshold and it is important to reduce the depth of anesthesia to achieve better results.

Comparison Between the Effect of Intra-Scalar Methylprednisolone and Sodium Hyaluronate in Impedance and Electrically Evoked Compound Action Potentials in Cochlear Implant Patients.

To evaluate and compare the effect of intra-scalar methylprednisolone and sodium hyaluronate on cochlear implants' impedance and electrically evoked compound action potentials thresholds. In a prospective randomized clinical trial, 103 children with pre-lingual hearing loss candidates for cochlear implantation at a tertiary hospital were divided into three groups based on intervention. Intraoperatively, one group received intra-scalar methylprednisolone, the second sodium hyaluronate, and the third group was the control group. Impedance and electrically evoked compound action potentials (e-ECAP) thresholds on long-term follow-up were evaluated and compared in these three groups. Significant decrease in impedance and e-ECAP thresholds were observed in all groups in a 4-year follow-up. No statistically significant difference was observed among all mentioned groups. Impedance and e-ECAP thresholds decrease in the long term, and using topical intra-scalar Healon or methylprednisolone may not significantly affect these parameters.

Hepatitis C virus in patients with squamous cell carcinoma of the head and neck in Iran: is there any relation?

Recently, some studies have noticed a large number of patients with squamous cell carcinoma of the head and neck (SCCHN), who are infected with hepatitis C virus (HCV). The aim of this study is to determine the prevalence of HCV in these patients in our population. Patients with SCCHN in four tertiary centers in different regions of Iran were checked for HCV. The prevalence of HCV in 107 patients diagnosed with head and neck squamous cell carcinoma was 0.9%, which has no significant difference with its prevalence in normal population. It seems that HCV at least in Iran could not be considered as a risk factor for SCCHN.

Contralateral tinnitus and hearing loss due to a tumor at the region of inferior colliculus: illustrative case.

BACKGROUND: Subjective hearing loss resulting from unilateral lesions of the quadrigeminal cistern region or inferior colliculus is a rare entity. OBSERVATIONS: The authors presented the case of a 32-year-old man with a history of 4 months of nonpulsatile tinnitus and 1 month of unilateral sensorineural hearing loss. He was otherwise healthy with no significant previous medical history. Intravenous gadolinium contrast magnetic resonance imaging of the head revealed a lesion at the region of the left inferior colliculus suggestive of a lipoma. The tumor was removed, and the patient had no significant hearing change afterward, with an improvement in tinnitus. The histological examination demonstrated the tumor as a fibrolipoma. LESSONS: Contralateral subjective hearing loss is an uncommon presentation for a lesion in the region of the inferior colliculus.

Deep neck infections: a retrospective review of 112 cases.

The purpose of this study was to review our recent experience with deep neck infections and compare it to the experiences in the available literature. A total number of 112 charts of patients treated for deep neck infections at the department of otolaryngology of our hospital between August 1996 and September 2007 were retrospectively reviewed. All the relevant clinical charts including presentation, origin and site of deep neck infection, radiological, and bacteriological studies and interventions along with demographic profile, details of hospital stay, and outcomes were evaluated. The findings were compared to those in the available literature. Until 2002, we had nearly constant number of patients annually but since then we have found an increasing number of them. The most common presenting symptom was neck swelling (88.4%). Fever was present in 63.4 and 78% had leukocytosis. The most common known cause was dental infection (31.3%) and in 32.1% of them origin remained unknown. The most commonly encountered site was the submandibular space and 23.2% of patients had two or more involved spaces. Exactly 20.5% were treated with intravenous antibiotic therapy alone whereas 79.5% had surgical procedures with successful results in 98.2% of them. Diabetes mellitus was diagnosed in 20.5% of cases. There was no case of known primary or acquired immunodeficiency. We had two deaths from septic shock but there was not any other complication. Mean hospitalization time was 7 days. Deep neck infections remain potentially lethal infections if they are not diagnosed early and treated promptly. Widespread diffusion of empirical broad-spectrum oral antibiotic and anti-inflammatory treatments may cause masked presentations of deep neck infections without swelling, fever, or leukocytosis. Our tailored approach (medical or medical and surgical) based on clinical and radiological evidence was successful in 98.2% of the patients with a short mean hospitalization time.

Delay Posttraumatic Paradoxical Cerebrospinal Fluid Leak with Recurrent Meningitis.

Cerebrospinal fluid (CSF) rhinorrhea complicates 2% of all head traumas, and 12%-30% of all basilar skull fractures. Posttraumatic CSF rhinorrhea usually occurs within the first 48 h, and majority of them occur in the first 3 months, whereas delayed CSF leak beyond 3 months is rare. On the other hand, CSF usually leaks through dural tearing associated with fracture of the anterior skull base. CSF leak through fractures of middle cranial fossa to the nose through the eustachian tube is very rare. We present a 52-year-old woman with delayed posttraumatic paradoxical CSF rhinorrhea and recurrent meningitis.

Bruxism and adenotonsillectomy.

OBJECTIVE: The main goal of this study is to assess the effect of adenotonsillectomy on bruxism in children with obstructive symptoms due to adenotonsillar hypertrophy. PATIENTS AND METHODS: In a prospective study, 140 children aged between 4 and 12 years with obstructive symptoms due to adenotonsillar hypertrophy were evaluated. With a questionnaire existence of bruxism was evaluated before and after adenotonsillectomy and the results were compared with each other. RESULTS: The prevalence of bruxism was 25.7% before surgery and 7.1% after it. The difference was significant by p value of 0.02. There was not any significant difference between male and female dominancy. CONCLUSION: This study suggests that adenotonsillectomy could improve bruxism significantly in children who have obstructive symptoms due to adenotonsillar hypertrophy.

Plasticity after pediatric cochlear implantation: Implication from changes in peripheral plasma level of BDNF and auditory nerve responses.

INTRODUCTION: Sensory neural hearing loss could lead to some structural and physiological changes in the auditory pathways, such as alteration in the expression of neurotrophins. These factors, especially Brain-Derived Neurotrophic Factor (BDNF), play an important role in synaptic functions and experience-related plasticity. Restoring cochlear function after hearing loss is possible through cochlear implantation (CI). Evaluation of the blood concentration changes of neurotrophins as prerequisites of plasticity could help scientists to determine the prognosis of CI as in the candidacy procedure or enhancing prosthesis function by adding the exact needed amount of BDNF to the electrode array. METHODS: Here we have studied the plasma BDNF concentration before CI surgery and 6 months after using CI device in 15 pediatric CI recipients and compared this level with changes of BDNF concentration in 10 children who were using hearing aid (H.A). In addition, we searched for a possible correlation between post-surgery plasma BDNF concentration and electrical compound action potential (ECAP) and comfort-level (C-level) thresholds. RESULTS: Plasma BDNF concentration in children with CI increased significantly after CI surgery, while this difference in H.A group was not significant. Analysis of repeated measures of ECAP and C-level thresholds in CI group showed that there were some kinds of steadiness during follow- up sessions for ECAP thresholds in basal and E16 of middle electrodes, whereas C-level thresholds for all selected electrodes increased significantly up to six months follow-up. Interestingly, we did not find any significant correlation between post-surgery plasma BDNF concentration and ECAP or C-level threshold changes. CONCLUSION: It is concluded that changes in C-level threshold and steady state of ECAP thresholds and significant changes in BDNF concentration could be regarded as an indicator of experienced-related plasticity after CI stimulation.

A Case-Control Study of Comparison of Plasma Levels of Matrix Metalloproteinase-9 in Inhaled Opium Addicts and Clinically Healthy Persons.

BACKGROUND: Metalloproteinase-9 exists in the basement membrane of normal tissues and is a major factor in cancer invasion and lymphatic metastasis. Smoking has been reported to increase the metalloproteinase level, but the role of opium consumption in metalloproteinase level has not yet been examined. The current research intended to examine the impacts of opium consumption on the serum levels of metalloproteinase. METHODS: This case-control research was conducted in Kerman (in the southeast of Iran), after getting medical approve by the ethics committee. Case group of 33 non-smokers with no active inflammatory diseases who had the experience of inhaled opium and its derivatives were compared with a control group of 40 non-smokers with no active inflammatory disease and no experience of inhaled opium and its derivatives. Student's t-test, mean, and chi-square test were employed to determine the correlation between the variables. FINDINGS: No statistically meaningful variation was detected in plasma metalloproteinase concentration between the case and control groups (P = 0.160). Also, there was no significant relation between the plasma metalloproteinase concentration and urinary morphine in case groups (P = 0.410), but a statistically significant correlation was found between gender and metalloproteinase in both the case and control groups (P = 0.003). CONCLUSION: According to the possible role of opioid drugs in cancer and its prognosis both directly and through their impact on serum levels of metalloproteinase, further studies are suggested.

Pulse steroid therapy in idiopathic sudden sensorineural hearing loss: A randomized controlled clinical trial.

OBJECTIVES/HYPOTHESIS: To evaluate in patients with idiopathic sensorineural hearing loss whether pulse therapy with methylprednisolone leads to better recovery of hearing than traditional oral prednisolone therapy. STUDY DESIGN: Randomized controlled trial. METHODS: Sixty-seven patients diagnosed with idiopathic sensorineural hearing loss were randomly divided into two groups based on therapy. The study group received 500-mg daily intravenous methylprednisolone for 3 consecutive days, followed by 1 mg/kg (maximum 60 mg) oral prednisolone for 11 days (total treatment: 14 days). The control group received 1 mg/kg (maximum 60 mg) oral prednisolone for 14 days. Hearing change was evaluated by comparing initial hearing tests and the third-month hearing tests in three ways: 1) pure tone improvement in each individual tone (0.5, 1, 2, 3, and 4 kHz); 2) word-recognition score improvement; and 3) complete, partial, and no recovery of hearing calculated (as defined by American Academy of Otolaryngology-Head and Neck Surgery Clinical Practice Guidelines). RESULTS: Sixty of 67 patients, 29 of 34 patients in the study group and 31 of 33 patients in the control group, completed the study. Frequency-specific hearing improvement did not differ significantly among the groups. Word-recognition score improvement was 20.34% ± 27.35% for the study group and 13.41% ± 23.48% for the control group, which had no statistically significant difference. There was also no significant difference in hearing recovery rates for the two groups. CONCLUSION: Pulse therapy with methylprednisolone and traditional oral prednisolone therapy resulted in similar hearing improvement.

Jervell and Lange-Nielsen syndrome in cochlear implanted patients: our experience and a review of literature.

OBJECTIVES: To share our experience in cochlear implanted patients with Jervell and Lange-Nielsen syndrome (JLNS), to review the literature results and to disclose precautions which have to be taken dealing with these patients. MATERIALS AND METHODS: Electrocardiograms (ECG) of 503 children with congenital bilateral profound hearing loss which were cochlear implanted in cochlear implant center of a tertiary hospital were evaluated for long QT syndrome. Clinical reports of the patients with JLNS were evaluated and a review of literature performed. RESULTS: The prevalence of disease was 0.79% (four cases) in our center which is in the range of literature reports (0-2.6%). None of our patients had a history of syncopal attack. Two patients (50%) were born from parents with consanguineous marriage. Considering all precautions their cochlear implant surgeries were done uneventfully. A review of the literature has identified sixteen reports on cochlear implantation in a total of 38 children with JLNS. Similar to our cases none of the authors reported cardiac events during device switch-on. Nine available reports about the outcome of cochlear implantation in these patients indicated good auditory outcome. CONCLUSION: It is recommended that all congenitally deaf patients have an ECG taken as a part of the evaluation. As auditory stimuli is reported to be a specific trigger, it is prudent to activate the processor with continuous heart monitoring even though there is no reported cardiac event during device switch-on. Cochlear implantation can be performed relatively safely in these patients if necessary precautions have been taken appropriately and their auditory outcome is good. Triggers of the cardiac events should be avoided throughout their life.

The distance between stapedial footplate and incus in otosclerosis surgery.

BACKGROUND: The measurement of the piston length during stapedotomy is important and it may have significant effects on the surgical outcome. OBJECTIVE: To determine the piston length in a group of otosclerosis patients who underwent primary stapedotomy. MATERIAL AND METHODS: Between Sep 2013 and Sep 2014, 85 patients with diagnosed otosclerosis underwent primary stapedotomy. Teflon prosthesis of 0.6 mm of diameter was used in all patients. The distance between medial surface of the long process of incus and center point of the stapedial footplate measured. The piston length calculated by adding 0.25 mm for the thickness of footplate and 0.50 mm for placement of the prosthesis into the vestibule. RESULTS: The distance between stapedial foot plate and incus ranged from 3.50 to 4.50 mm. The mean distance was 3.95 ± 0.16 mm. In majority of cases (74.1%) piston length was 4.75 mm followed by 4.50 mm in 15.3% and 5.00 mm in 5.9%. Piston length was 4.25 mm in 3.5% and 5.25 mm in only 1.2% of patients. There was no significant post-operative complication and air bone gap closure obtained in all patients. CONCLUSION: The most common distance between foot plate and incus is 4.00 mm. No case needed a piston longer than 5.25 mm or shorter than 4.25 mm.

Cerebrospinal fluid gusher in cochlear implantation.

OBJECTIVES: To share our experience of cerebrospinal fluid (CSF) gusher in cochlear implantation. METHODS: Demographic, radiological, and surgical results of patients with CSF gusher in 523 consecutive cochlear implant recipients including children and adults as well as our management technique were evaluated and a review of the literature has been included. RESULTS: Fifteen (2.87%) cases had CSF gusher. Two patients (13.3%) were adults with post-lingual hearing loss and the rest 12 (86.7%) were children with congenital hearing loss. Twelve patients (80%) had various types of inner ear malformation. Three patients (20%) had no predictable risk of CSF gusher from history or pre-operative imaging. In all patients, CSF gushers were controlled with our technique of packing the electrode entrance site with no additional measures. CONCLUSION: CSF gusher may occur with post-lingual hearing loss and in children with apparently unremarkable imaging and history. Thus, surgeons should always be ready to manage it. Management of CSF gusher can be mainly performed during the initial surgery by precise tight packing of the electrode entrance site. Furthermore, non-surgical or surgical measures are rarely required to stop a persistent leak. Our results show that our management technique may be recommended as well.

Use of intravenous steroids at induction of anesthesia for septoplasty to reduce post-operative nausea and vomiting and pain: a double-blind randomized controlled trial.

To determine the effectiveness of dexamethasone to reduce pain, nausea and vomiting after septoplasty. Study is a prospective double-blind randomized controlled trial. A total of 90 patients were enrolled and received an either of dexamethasone or placebo in the induction of anesthesia. The patients were asked to note the level of pain on the visual analogue scale in the first 24 h after surgery. They also had to record their severity of nausea and number of vomiting attacks. There were statistically significant differences between the two groups for the level of pain noted on the visual analogue scale. The severity of nausea was lower after treatment with dexamethasone but the difference between two groups was not significant. There was also a significant decrease in the number of vomiting attacks in patients treated with dexamethasone. A single dose of 8 mg of dexamethasone, given intravenously, at induction of anesthesia for septoplasty significantly decreased the pain, post-operative nausea and vomiting for the day of operation.

Light microscopic histopathology of adenoid tissue in otitis media with effusion: is there any relation?

OBJECTIVE: The adenoid has long been recognized as an important factor in the pathogenesis of otitis media with effusion. The aim of this study was to determine if there is any relationship between light microscopic histopathologic parameters of adenoid tissue and otitis media with effusion. METHODS: The study group consists of 30 children aged from 2 to 8 years old with bilateral otitis media with effusion who were selected also for adenoidectomy due to obstructive symptoms of hypertrophied adenoid. The control group included 30 age-matched patients who undergone adenoidectomy due to same indications but without even unilateral otitis media with effusion. Adenoid specimens were examined under light microscopy by an expert pathologist who not aware of the specimens were belongs to which group. Nine histopathologic parameters of the specimens were compared between the two groups. RESULTS: There was no statistically significant difference between these groups (p values>0.05). CONCLUSIONS: The role of the adenoids in pathogenesis of otitis media with effusion is beyond its histopathologic changes at least in the level of light microscopic findings.