Gonca Sennaroglu

Intratympanic dexamethasone, intratympanic gentamicin, and endolymphatic sac surgery for intractable vertigo in Meniere's disease.

OBJECTIVE: To compare the efficacy of intratympanic dexamethasone (ID), intratympanic gentamicin (IG), and decompression of the endolymphatic sac (ESD) for intractable vertigo in Menieres disease. STUDY DESIGN AND SETTING: This prospective study was conducted at Hacettepe University Medical Faculty, a tertiary care center. Dexamethasone was applied through a ventilation tube in 24 patients, intratympanic gentamicin (also through a ventilation tube) to 16 patients, and 25 patients underwent ESD. RESULTS: Satisfactory control of vertigo was 72%, 75%, and 52%, respectively for the ID, IG, and ESD. Two patients in the gentamicine group had total hearing loss. In the dexamethasone group, hearing level remained the same in 46% of the patients with 16% increase and 38% decrease (30% 10dB and 8% 20 dB). CONCLUSION: If the vertiginous symptoms still persist after 6 months of medical treatment, ID can be started. If there is no further improvement after 3 months with ID, patients with profound sensorineural hearing loss undergo treatment with IG, ESD is reserved for patients with good hearing. If ESD also fails, patients with good hearing may undergo vestibular nerve section; patients with nonservicable hearing become candidates for labyrinthectomy.

Preliminary results of auditory brainstem implantation in prelingually deaf children with inner ear malformations including severe stenosis of the cochlear aperture and aplasia of the cochlear nerve.

Objective: The aim of our study is to present the results of 11 children where auditory brainstem implantation (ABI) was successfully performed to restore hearing. Study Design: Case presentation. This study was conducted at the departments of Otolaryngology and Neurosurgery at Hacettepe University Ankara, Turkey. Patients: Between July 2006 and April 2008, 11 prelingual (30-56 mo) deaf children with several cochlear malformations had ABI. Intervention: All patients were programmed and were enrolled in auditory verbal therapy sessions and family counseling programs at Hacettepe Auditory Verbal Center. The evaluation was performed at preimplant and again 1, 3, 6, 9, and 12 months post-switch on. Main Outcome Measures: The main test components composing this test battery were Ling 6 Sound Detection-Identification Test, Word Identification Test in Turkish, Meaningful Auditory Integration Scale, and Meaningful Use of Speech Scale. Results: Successful brainstem implantations were performed in all patients with retrosigmoid approach. Six children gained basic audiologic functions and were able to recognize and discriminate sounds, and many could identify environmental sounds such as a doorbell and telephone ring by the third month of ABI. Improvement in mean performance on Meaningful Auditory Integration Scale is apparent for all ABI children. Improvement in Meaningful Use of Speech Scale scores in 2 patients, demonstrating that the child using its own voice for speech performance, was observed between the baseline and 12th month. First, 5 children were able to identify Lings 6 sound by the end of 2 to 6 months, and 2 of them also started to identify words due to their pattern differences and multisyllabic word identification by 6 to 9 months. Two children with Attention Deficit Hyperactivity Disorder have made slower progress than the other children with ABIs. Conclusion: Our preliminary results show that there is adequate contribution of brainstem implants in the development of auditory-verbal skills. Additional handicaps slow the progress of the prelingually deaf children.

Auditory brainstem implantation in children and non-neurofibromatosis type 2 patients: a consensus statement.

On the 18th of September 2009, a group of health care professionals and scientists involved in implantation of the auditory system attended a meeting convened by the Hacettepe Cochlear Implant Group. The aim of the meeting was to have a detailed discussion on the pressing and pertinent issues around

Audiological findings in pregnancy

This investigation was carried out in the Audiology and Speech Pathology Section of the Department of Otorhinolaryngology of Hacettepe University. The pregnant group comprised of 20 women followed by the Department of Gynaecology and Obstetrics of the same university; 18 non-pregnant women comprised the control group. The aim of this investigation was to study the relationship between hormonal changes in pregnancy and cochlear functions. All subjects underwent ENT examination, audiologic and acoustic immitance measurements and auditory brain stem response (ABR) tests. Results from each trimester and post-partum period of the pregnant group were compared. These results demonstrated that there was a decrease in hearing levels for 125 Hz, beginning in the first trimester and increasing in the second and third trimesters. Hearing returned to normal in the post-partum period. Similar findings were also obtained for 250 and 500 Hz; however, frequencies higher than 500 Hz demonstrated no significant correlation. Uncomfortable loudness was statistically significant between the third trimester and post-partum period. ABR tests did not reveal any differences. In conclusion, there is a low-frequency hearing loss and tolerance problem in pregnancy mimicking cochlear pathology. However, this hearing loss did not reach pathologic levels in any case and returned to normal in the post-partum period.

Comprehensive analysis via exome sequencing uncovers genetic etiology in autosomal recessive nonsyndromic deafness in a large multiethnic cohort

Purpose:Autosomal recessive nonsyndromic deafness (ARNSD) is characterized by a high degree of genetic heterogeneity, with reported mutations in 58 different genes. This study was designed to detect deafness-causing variants in a multiethnic cohort with ARNSD by using whole-exome sequencing (WES).Methods:After excluding mutations in the most common gene, GJB2, we performed WES in 160 multiplex families with ARNSD from Turkey, Iran, Mexico, Ecuador, and Puerto Rico to screen for mutations in all known ARNSD genes.Results:We detected ARNSD-causing variants in 90 (56%) families, 54% of which had not been previously reported. Identified mutations were located in 31 known ARNSD genes. The most common genes with mutations were MYO15A (13%), MYO7A (11%), SLC26A4 (10%), TMPRSS3 (9%), TMC1 (8%), ILDR1 (6%), and CDH23 (4%). Nine mutations were detected in multiple families with shared haplotypes, suggesting founder effects.Conclusion:We report on a large multiethnic cohort with ARNSD in which comprehensive analysis of all known ARNSD genes identifies causative DNA variants in 56% of the families. In the remaining families, WES allows us to search for causative variants in novel genes, thus improving our ability to explain the underlying etiology in more families.Genet Med 18 4, 364–371.

Transtympanic dexamethasone application in Ménière's disease : an alternative treatment for intractable vertigo

The aetiology of Ménières disease still remains unknown and its therapy is therefore empirical. As a result of immunological abnormalities demonstrated, steroids are commonly used in Ménières disease. The place of topical steroids is still controversial. In this investigation topical dexamethasone is applied for three months through a ventilation tube in patients with intractable vertigo. The results showed that this treatment controls vertigo in 72 per cent of cases. No patient was worse than before treatment. Only in 17 per cent of the patients was there an increase in hearing level. When compared to the reports which used only tympanostomy tubes, this procedure seems to have a placebo effect with minimal harmful effects. It appears that transtympanic dexamethasone application is a good alternative to vestibular nerve section. Topical treatment may be sufficient in most patients. Systemic treatment may be used in patients where topical treatment fails.

Effect of Teflon Piston Diameter On Hearing Result After Stapedotomy

This study compares hearing results after stapedotomy by 0.6 mm and 0.8 mm teflon pistons. Retrospective analysis studied 100 patients randomly selected who underwent stapedotomy for otosclerosis with insertion of 0.8 mm teflon piston prosthesis and 100 patients with 0.6 mm teflon piston prosthesis. Air-conduction hearing level in both groups were measured before and after the procedure and the gain of the air conduction between the 2 groups at different frequencies were compared statistically. The group with the 0.8 mm prosthesis had better results that were statistically significant and more pronounced at lower frequencies.

Multicentre investigation on electrically evoked compound action potential and stapedius reflex: how do these objective measures relate to implant programming parameters?

Abstract Objectives The aims of this study were to collect data on electrically evoked compound action potential (eCAP) and electrically evoked stapedius reflex thresholds (eSRT) in HiResolutionTM cochlear implant (CI) users, and to explore the relationships between these objective measures and behavioural measures of comfort levels (M-levels). Methods A prospective study on newly implanted subjects was designed. The eCAP was measured intra-operatively and at first fitting through neural response imaging (NRI), using the SoundWaveTM fitting software. The eSRT was measured intra-operatively by visual monitoring of the stapes, using both single-electrode stimulation and speech bursts (four electrodes stimulated at the same time). Measures of M-levels were performed according to standard clinical practice and collected at first fitting, 3 and 6 months of CI use. Results One hundred seventeen subjects from 14 centres, all implanted unilaterally with a HiResolution CII Bionic Ear® or HiRes 90K®, were included in the study. Speech burst stimulation elicited a significantly higher eSRT success rate than single-electrode stimulation, 84 vs. 64% respectively. The NRI success rate was 81% intra-operatively, significantly increasing to 96% after 6 months. Fitting guidelines were defined on the basis of a single NRI measurement. Correlations, analysis of variance, and multiple regression analysis were applied to generate a predictive model for the M-levels. Discussion Useful insights were produced into the behaviour of objective measures according to time, electrode location, and fitting parameters. They may usefully assist in programming the CI when no reliable feedback is obtained through standard behavioural procedures.

Vestibular stimulation after cochlear implantation in common cavity deformity

Congenital malformations of the inner ear are very rare and constitute approximately 20% of children with congenital sensorineural hearing loss.1 According to Jackler and Luxford,2 common cavity deformity is the second most common malformation after Mondini deformity. Dizziness and vertigo after cochlear implantation have been reported in the literature.3 We present a case with abnormal vestibular stimulation during the hook-up of cochlear implant in a patient with common cavity malformation.

Relationship of Vestibular Aqueduct and Inner Ear Pressure in Ménière's Disease and the Normal Population

Objective Etiopathogenesis of Ménières disease has not been resolved. The principal histopathologic finding in this disease is endolymphatic hydrops. The majority of radiologic and histopathologic studies demonstrated a narrow vestibular aqueduct in Ménières disease. There is no study in the literature investigating the relationship between inner ear pressure and vestibular aqueduct dimensions. Static acoustic compliance is a noninvasive procedure that is thought to measure perilymphatic pressure at the footplate. An increase in mechanical fluid pressure in the inner ear is transmitted to the footplate of the stapes. This causes a reduction in the compliance at the drum. The aim of this study is to investigate the relationship between vestibular aqueduct dimensions and static acoustic compliance in Ménières disease and the normal population.