The effect of cochlear implantation on tinnitus in patients with post lingual bilateral profound hearing loss.

INTRODUCTION: In this study we investigated the effect of cochlear implantation on tinnitus in patients with post lingual bilateral profound hearing loss. METHODS: In this retrospective study, 66 post-lingual deaf patients over 18 years old who referred for cochlear implantation and complained of tinnitus were included. Patients were divided into two groups with residual hearing (in frequency 250-500 Hz) and without residual hearing. All data including age, gender, type of implants, type of surgery, cause of the disease, and information on the THI score before cochlear implantation, one month after cochlear implantation and one year after cochlear implantation was recorded and analyzed. RESULTS: Nine patients had residual hearing, while 57 patients did not. After one month and one year, in both groups, the mean THI score has decreased significantly (p-value = 0.001), but there was no significant difference in the mean THI score before surgery and one month and one year after surgery between two groups with and without residual hearing. There was no significant difference in tinnitus severity according to THI-grade between two groups. Also there was no significant difference in THI score and the cause of the hearing loss, type and insertion method of implantation, one month and one year after the surgery between two groups. CONCLUSION: According to our results, the was no significant correlation between residual hearing and tinnitus score before surgery, one month and one year after the surgery.

Silastic Electrode Positioner Extrusion as a Late Complication of Cochlear Implantation Surgery.

It was the aim of this study to report a rare case of electrode positioner extrusion in cochlear-implanted patients as a late complication and introduce safe management. A retrospective case report in a tertiary referral center. Extrusion of Silastic electrode positioner through tympanic membrane occurred 17 years after cochlear implantation surgery. Although electrode positioner cochlear implants are not being used anymore, otologists and surgeons should be aware of their possible related complications and provide a proper management.

Correlation of semi-quantitative findings of endolymphatic hydrops in MRI with the audiometric findings in patients with Meniere's disease.

Purpose: To investigate the correlation between vestibular hydrops (VH), cochlearhydrops (CH), vestibular aqueduct non-visibility (VANV), and visually increased perilymphatic enhancement (VIPE) with the findings of pure-tone audiometry (PTA) in Meniere's disease (MD) patients. Methods: In this cross-sectional study, 53 ears belonging to 48 patients were divided into two groups and evaluated. In group "MD patients," there were 24 ears of 19 patients diagnosed with the definite MD (14 patients with unilateral and 5 patients withbilateral involvements). The "control group" consisted of 29 non-symptomatic ears belonging to patients diagnosed with unilateral sudden sensory-neural hearing loss or unilateral schwannoma. All the patients underwent 2 sessions of temporal bone MRI using the same 3T system: an unenhanced axial T1, T2, and 3D-FLAIR MRI, an intravenous gadolinium-enhanced axial T1 fat-sat, and 4 h after the injection, an axial 3D-T2 cube and 3D-FLAIR session. VH, CH, VANV, and VIPE were assessed. Subsequently, the correlation between EH indices and PTA findings (in three frequency domains of low, middle, and high) were evaluated, and the predictive value of MRI was calculated. Results: VH was significantly correlated with the hearing threshold in the low, middle, and high-frequency domains. CH was also correlated with the hearing threshold in the low and middle domains. Contrarily, VIPE was not associated with hearing thresholds, and VANV was only correlated with the hearing threshold in low frequencies. Conclusion: The grade of VH, CH, and VANV were significantly correlated with the hearing thresholds in PTA.

Sound-Field Speech Evoked Auditory Brainstem Response in Cochlear-Implant Recipients.

BACKGROUND AND OBJECTIVES: Currently limited information is available on speech stimuli processing at the subcortical level in the recipients of cochlear implant (CI). Speech processing in the brainstem level is measured using speech-auditory brainstem response (S-ABR). The purpose of the present study was to measure the S-ABR components in the sound-field presentation in CI recipients, and compare with normal hearing (NH) children. Subjects and. METHODS: In this descriptive-analytical study, participants were divided in two groups: patients with CIs; and NH group. The CI group consisted of 20 prelingual hearing impairment children (mean age=8.90 ± 0.79 years), with ipsilateral CIs (right side). The control group consisted of 20 healthy NH children, with comparable age and sex distribution. The S-ABR was evoked by the 40-ms synthesized /da/ syllable stimulus that was indicated in the sound-field presentation. RESULTS: Sound-field S-ABR measured in the CI recipients indicated statistically significant delayed latencies, than in the NH group. In addition, these results demonstrated that the frequency following response peak amplitude was significantly higher in CI recipients, than in the NH counterparts (p<0.05). Finally, the neural phase locking were significantly lower in CI recipients (p<0.05). CONCLUSIONS: The findings of sound-field S-ABR demonstrated that CI recipients have neural encoding deficits in temporal and spectral domains at the brainstem level; therefore, the sound-field S-ABR can be considered an efficient clinical procedure to assess the speech process in CI recipients.

G130V de novo mutation in an Iranian pedigree with nonsyndromic hearing loss without palmoplantar keratoderma.

Mutations in the GJB2 gene encoding connexin 26 (Cx26) cause autosomal recessive and rarely dominant nonsyndromic sensorineural hearing loss as well as asyndromic hearing impairment with skin problems. A dominant GJB2 mutation, c.389G > T (p.G130V), has been reported previously in association with hearing impairment and palmoplantar keratoderm. Here we report the first de novo G130V mutation of GJB2 gene in a sporadic case of hearing loss in a consanguineous Iranian family which is not associated with skin disorder.

The clinical utility of search coil horizontal vestibulo-ocular reflex testing.

CONCLUSION: Testing of the horizontal vestibulo-ocular reflex (VOR) with head rotations (including head impulses) using the magnetic scleral search coil technique (SCT HHI) provides valuable additional diagnostic information in patients with persistent dizziness, oscillopsia or imbalance. It identifies high and low frequency/acceleration vestibular abnormalities that are frequently missed using other methods. OBJECTIVES: To evaluate the diagnostic utility of SCT measurement of the horizontal VOR in the multidisciplinary neurotology clinic of a tertiary referral centre. PATIENTS AND METHODS: The records of 127 consecutive patients referred for persistent dizziness, oscillopsia, imbalance, or with clinical findings suggestive of high frequency/acceleration vestibular dysfunction were reviewed. All had been tested with clinical head impulses, bithermal calorics and vestibular-evoked myogenic potentials. VOR gain (peak eye velocity/peak head velocity) had been measured both in response to sinusoidal oscillations in a rotating chair (0.1-11 Hz) and to manually delivered horizontal head rotations (peak head velocities 50-500 degrees/s) using SCT. RESULTS: Agreement between the different test modalities of horizontal semicircular canal function was moderate. Relative to SCT HHI, clinical HHI showed the highest sensitivity and the lowest specificity (both 70%). SCT HHI appeared to have the greatest diagnostic yield, when compared with calorics and SCT ROT (23% of all abnormalities shown were detected only by SCT HHI) and also allowed detection of significant asymmetries in patients with bilateral vestibular dysfunction.

The effects of narrative-based language intervention (NBLI) on spoken narrative structures in Persian-speaking cochlear implanted children: A prospective randomized control trial.

BACKGROUND: Previous research has shown that narrative-based language intervention (NBLI) is a feasible approach increasing the narrative skills of hearing-impaired children. OBJECTIVE: In the present study, the efficacy of NBLI on the macrostructure and microstructure components of the spoken narrative of children with cochlear implants (CI) was evaluated in an experimental study. MATERIALS AND METHODS: Thirty-six CI children between the ages of 5.5 and 7 years were randomly divided in three groups of equal size. Group 1 attended 24 1-h sessions of NBLI group therapy. Group 2 participants each attended 24 1-h private sessions of NBLI instruction. Group 3 attended 24 1-h conventional speech therapy (CST) sessions. The subjects' storytelling skills were prompted using pictures from the Persian version of the Language Sample Analysis test. The results were assessed before treatment (T0), after treatment (T1) and two months after treatment (T2) as follow-up. RESULTS: Groups 1 and 2 showed significantly better results over Group 3 on all microstructure components of spoken narrative at the T1 (p ≤ 0.04) and T2 (p ≤ 0.04) levels in comparison with T0, but no differences were observed between the NBLI approaches (p > 0.05). All three intervention programs significantly improved the macrostructure of the spoken narrative in CI children. CONCLUSION: Improvements in spoken narrative structures were observed in CI children that support the efficacy of NBLI over CST for the hearing-impaired population.

Prognosticating hearing outcome in patients with idiopathic sudden sensorineural hearing loss by means of otoacoustic emissions and auditory brainstem response.

This is an analytic-descriptive study, parallel with a randomized, controlled trial performed at Amir'Alam Hospital, a tertiary referral center, with the aim of evaluating the correlation between otoacoustic emission (OAE) and auditory brainstem response (ABR) findings with hearing outcome after treatment of idiopathic sudden sensorineural hearing loss (SSNHL). Sixty patients with idiopathic SSNHL who presented to the emergency services and otology clinics between 2012 and 2014, and whose symptoms had begun <10 days previously, enrolled in this study. Before commencing treatment, distortion-product OAE (DP-OAE) and ABR were performed for all patients. They also underwent magnetic resonance imaging ± gadolinium. Therapeutic intervention was done in a parallel randomized, controlled trial, and responders to the medical therapy were selected for our final analysis. There was no significant correlation between the OAE record and responsiveness to treatment, but there was a correlation between ABR presence and the probability of responsiveness in patients with profound hearing loss who responded to medical therapy and had at least wave V ABR. However, in those who had no recorded wave, the response to treatment was variable. In conclusion, in patients with profound hearing loss, studying the waves of ABR could be a factor in predicting hearing loss resolution after treatment.

Language sampling for children with and without cochlear implant: MLU, NDW, and NTW.

OBJECTIVES: Cochlear implants (CIs) can considerably improve the oral language of prelingual hearing-impaired children. However, because most studies have been performed with English speaking children, available information regarding Persian-speaking children is scarce. Therefore, this study compared measures of lexical diversity (numbers of different words and total words), and syntactic complexity (mean length of utterance) in Persian-speaking children with and without CIs. METHODS: A cross-sectional study with 20 children with CIs and 20 typically developing children was conducted. To collect the data, the children's language samples were gathered via picture descriptions. The first 50 utterances were analyzed. RESULTS: All measures were significantly different between children with CIs and their typically developing age-matched peers, whereas no differences between children with CIs and their typically developing hearing age-matched peers were detected (p<0.05). CONCLUSIONS: CIs have been recognized to be one of the most beneficial rehabilitation prostheses because they help children to acquire speech and language abilities similar to their typically developing hearing age-matched peers. After implantation, the performance of children with CIs is similar to the performance of normal children with the same hearing experience. The duration of the hearing experience after the implantation is an important factor for determining the development of speech and language abilities.

What is expected of the facial nerve in michel aplasia? Anatomic variation.

We sought better understanding about the facial nerve anatomy in the rare inner ear Michel anomaly to help better define this aplasia and prevent potential complications in surgery on these patients. The data from computed tomography scans and magnetic resonance images of six Michel aplastic ears (three patients) were evaluated for a facial nerve course. Facial nerve course and anatomic landmarks were noted. Based on data obtained from this group of very rare patients, three different facial nerve anatomies were encountered. The first patient had normal-looking mastoid cells, normal middle ear ossicles, and a completely formed facial nerve canal through the middle ear. The second patient had pneumatized mastoid air cells despite an anomalous ossicular chain. This patient also had a facial nerve canal but not through the middle ear. In the third patient, although mastoid cells were present, neither ossicles nor a definite facial nerve canal could be detected. With guidance provided by the anatomy of the other parts of the ear, such as air cells and the ossicular chain, the danger zones posing a high probability of facial nerve injury can be predicted. Although all Michel aplasias may have aplastic petrous bone in common, there are some degrees of variation.

Nasal mucosal involvement in Behçet disease: a study of its incidence and characteristics in 400 patients.

Behçet disease is a multisystem vasculitis characterized by mucosal aphthosis, primarily in the oral and genital mucosa. Only a few cases of involvement of the nasal mucosa have been reported in the literature, and its true prevalence is not known. We conducted a cross-sectional study of 400 consecutively presenting patients with confirmed Behçet disease (according to classification-tree criteria) to determine the incidence of nasal mucosal involvement, to identify its particular characteristics, and to determine if there are any statistically significant differences in clinical and paraclinical parameters between Behçet disease patients with nasal involvement and those without. To the best of our knowledge, this is the first systematic evaluation of nasal involvement in patients with Behçet disease to be published in the literature. Data analysis was performed with Statistical Package for the Social Sciences software (SPSS), and a confidence interval (CI) at 95% was calculated for each finding. Involvement of the nasal mucosa was seen in 31 of the 400 patients (7.8%; CI: 5.1 to 10.5). Among those 31 patients, the most common nasal symptom was dysosmia, which was seen in 15 patients (3.8% of the total population; CI: 1.9 to 5.7); other nasal symptoms included obstruction in 10 patients (unilateral in 9), ulcers in 2, pain in 2, and a burning sensation and discharge in 1 each. No patient reported a history of epistaxis or nasal itching. Abnormal signs were present in only 16 patients (4.0%; CI: 2.1 to 5.9); they included nasal cartilage deformity in 6, unilateral nasal obstruction in 4, postnasal discharge in 3, nonaphthous ulcer in 3, and crusted ulcer in 2 (2 patients each had 2 abnormal signs). No case of aphthous ulcer, nasal discharge, nasal scar or deformity, septal perforation, or nodular or granulomatous cartilage lesion was found. There were no statistically significant differences in clinical and paraclinical disease manifestations between those patients who had nasal mucosal involvement and those who did not.

Clinical predictors for germline mutations in head and neck paraganglioma patients: cost reduction strategy in genetic diagnostic process as fall-out.

Multiple genes and their variants that lend susceptibility to many diseases will play a major role in clinical routine. Genetics-based cost reduction strategies in diagnostic processes are important in the setting of multiple susceptibility genes for a single disease. Head and neck paraganglioma (HNP) is caused by germline mutations of at least three succinate dehydrogenase subunit genes (SDHx). Mutation analysis for all 3 costs approximately US$2,700 per patient. Genetic classification is essential for downstream management of the patient and preemptive management of family members. Utilizing HNP as a model, we wanted to determine predictors to prioritize the most heritable clinical presentations and which gene to begin testing in HNP presentations, to reduce costs of genetic screening. Patients were tested for SDHB, SDHC, and SDHD intragenic mutations and large deletions. Clinical parameters were analyzed as potential predictors for finding germline mutations. Cost reduction was calculated between prioritized gene testing compared with that for all genes. Of 598 patients, 30.6% had SDHx germline mutations: 34.4% in SDHB, 14.2% SDHC, and 51.4% SDHD. Predictors for an SDHx mutation are family history [odds ratio (OR), 37.9], previous pheochromocytoma (OR, 10.9), multiple HNP (OR, 10.6), age

Transmastoid labyrinthotomy approach for cochlear implantation in a common cavity malformation: a case report.

The standard transmastoid facial recess approach has become the popular technique for cochlear implantation. Although this approach has been used for implantation in patients with common cavity malformations, in patients with this anomaly and severe abnormalities of the middle ear, this approach may be technically difficult and makes the facial nerve vulnerable to injury. Using a direct approach to the common cavity through the mastoid cavity, we have successfully performed cochlear implantation in 1 patient with a common cavity malformation. This transmastoid labyrinthotomy approach to the common cavity is an effective and simple technique for placing the electrode array. It minimizes the risk to the facial nerve and may decrease postoperative cerebrospinal fluid leaks.

A familial occurrence of lip anomaly.

Lip pits are among the rarest congenital deformities recorded. Initially reported in 1845, its familial occurrence has been reported just once. These developmental anomalies occur either as an isolated defect or in association with other developmental deformities, including cleft lip, cleft palate, or both. They may be located at the commissures of the lips or at the midline of the lower lip. Lip pits are often inherited as an autosomal dominant trait with variable penetrance. Our report of a family in which all of the 3 children (2 girls and 1 boy) and their father were involved is in concert with the latter statement.