Evaluation of Auditory Temporal Discrimination Thresholds in Migraine Patients.

INTRODUCTION: Although vestibular migraine is well defined, the effects of migraine on the auditory system have not been clearly identified yet. The aim of this study was to determine the effect of migraine on the auditory system. METHODS: Migraine patients without hearing loss were included in the study. Group 1 consisted of patients with migraine pain, group 2 consisted of patients with migraine in the interictal period, and group 3 consisted of healthy volunteers with similar demographic characteristics to groups 1 and 2. Random gap detection test was applied to all 3 groups. Additionally, group 2 and group 3 patients were evaluated with the auditory cortical potentials and the mismatch negativity test. RESULTS: There was a statistically significant difference between the 3 groups in the random gap detection test. There was no statistically significant difference in auditory cortical potentials between group 2 and group 3; however, a statistically significant difference was found between the groups in terms of mismatch negativity test latency. CONCLUSION: An auditory pathway may be affected in migraine patients, although hearing tests are normal. This interaction continues between attacks, being more evident during the pain period. Therefore, disorders of hearing or speech perception in migraine patients should be evaluated by further audiological tests.

The Effect of An Indirect Voice Therapy Approach on the Voice of Children With Vocal Fold Nodules: A Prospective Cohort Study.

PURPOSE: To investigate the effect of a combination indirect voice therapy approach on the management of vocal nodules in the pediatric population. METHODS: A prospective cohort study with thirty children (24 males and 6 females; mean age 8.8 ± 2.2) with a diagnosis of vocal fold nodule (VFN) was conducted. All participants were included in an 8-week voice therapy program consisting of vocal hygiene education and parental rule-setting. Videolaryngoscopic examination, auditory-perceptual evaluation, and acoustic voice analysis were performed immediately before and after the intervention. RESULTS: The comparison of videolaryngoscopic findings before and after the therapy revealed that the size of the nodules decreased significantly (P < 0.001). Findings from the laryngeal examination showed that the vast majority of children 73.3% (n = 22) had no VFN anymore after the intervention. The auditory-perceptual evaluation showed that overall severity of dysphonia decreased significantly [from 50 (40-70) to 38 (30-45), P < 0.001]. Jitter local (P = 0.031) and Jitter local abs (P = 0.043) parameters statistically differed after the therapy. There were not any statistically significant differences for mean F0, shimmer local (%), shimmer local (dB), and mean HNR. CONCLUSIONS: The voice therapy method in this study has a clear benefit on the voice of children with VFN and can be used in clinical practice. Specifically, clinicians should consider the parents as a core component of voice therapies and pay attention to vocal hygiene education for optimal management of VFN related childhood dysphonia.

Validating the Turkish version of the Weinstein noise sensitivity scale: effects of age, sex, and education level

Background/aim: Subjective noise sensitivity (SNS) is not only one of the most important predictors of perceived noise annoyance for the subjects exposed to the noise, but also strongly related with some psychological conditions and poor health perception of the subjects. Weinstein's Noise Sensitivity Scale (WNSS) is widely used for measuring of SNS. Materials and methods: In this study we adapted WNSS to Turkish (Tr-WNSS) and evaluated its psychometric properties and effect of age, sex and education level on SNS. Results: The study included 105 males and 105 female subjects between 18 and 55 years of age. Conclusion: Our data demonstrated that Tr-WNSS was a reliable, valid, and invariant scale for the Turkish population, and not affected by sex, age and education level.

The Effects of Riluzole on Cisplatin-induced Ototoxicity.

Introduction Riluzole (2-amino-6-trifluoromethoxy benzothiazole) is known as a neuroprotective, antioxidant, antiapoptotic agent. It may have beneficial effects on neuronal cell death due to cisplatin-induced ototoxicity. Objective To evaluate the effect of riluzole on cisplatin-induced ototoxicity in guinea pigs. Methods Twenty-four guinea pigs, studied in three groups, underwent auditory brainstem response evaluation using click and 8 kHz tone burst stimuli. Subsequently, 5 mg/kg of cisplatin were administered to all animals for 3 days intraperitoneally (i.p.) to induce ototoxicity. Half an hour prior to cisplatin, groups 1, 2 and 3 received 2 ml of saline i.p., 6 mg/kg of riluzole hydrochloride i.p., and 8 mg/kg of riluzole hydrochloride i.p., respectively, for 3 days. The auditory brainstem responses were repeated 24 hours after the last drug administration. The cochleae were analyzed by transmission electron microscopy (TEM). Results After drug administiration, for 8,000 Hz stimulus, group 1 had significantly higher threshold shifts when compared with groups 2 ( p < 0.05) and 3 ( p < 0.05), and there was no significant difference in threshold shifts between groups 2 and 3 ( p > 0.05). Transmission electron microscopy findings demonstrated the protective effect of riluzole on the hair cells and the stria vascularis, especially in the group treated with 8 mg/kg of riluzole hydrochloride. Conclusion We can say that riluzole may have a protective effect on cisplatin- induced ototoxicity. However, additional studies are needed to confirm these results and the mechanisms of action of riluzole.

Turkish Adaptation of the Pediatric Voice Related Quality of Life Survey: A validity and reliability study.

OBJECTIVES: Childhood voice disorders have increased in recent years reduce not only communication of the child, but also; the quality of life by affecting the socio-emotional state. METHODS: In the study, it is aimed to test validity and reliability of Pediatric Voice Related Quality of Life Survey (PVRQOL)'s Turkish adaptation. A total of 223 individuals aged between 2 years and 18 years, with and without voice problems were involved in this study. RESULTS: Statistically significant differences were found between study and control groups for total score and sub domain scores for PVRQOL (p < 0,001). Total score of quality of life was higher in the control group. Cronbach alpha coefficient for overall PVRQOL was 0,922; dimension of physical function was 0,894 and socio-emotional domain was 0,804. In the test-retest reliability test, overall PVRQOL was found to be 0,732; physical functional sub domain was 0,734; socio-emotional sub domain was 0,721. The validity of the questionnaire was determined by factor analysis. CONCLUSION: The results suggest that the Turkish version of the PVRQOL has reliability and validity, and may play a crucial role in evaluating children with voice disorders.

Evaluation of Effect of Garlic Aged Extracts and Vitamin B12 on Noise-Induced Hearing Loss.

OBJECTIVE: This study investigated effects of S-allylmercaptocysteine (SAMC), diallyl disulfide (DADS), and vitamin B12 on inner ear functions and morphology after long-period high-level broadband noise exposure. MATERIALS AND METHODS: Twenty-four healthy rats were randomly divided into four groups. First group was chosen as the control group. Vitamin B12, SAMC, and DADS were applied to other groups for 4 weeks. On the 14th day, each group was exposed to broadband noise. Auditory brainstem response test was performed before and immediately after noise exposure and repeated on the 2nd and 14th day. RESULTS: Permanent threshold shifts were significantly lower in groups treated with vitamin B12, SAMC, and DADS. Histologically, cochleae of SAMC and DADS groups were found to be better preserved than the cochleae of vitamin B12 and control groups. CONCLUSION: Physiologically and histologically, SAMC and DADS reduced the long-term effects of noise. However, physiological recovery was not consistent with the morphological findings in vitamin B12 group.

Determination of the level of noise in nurseries and pre-schools and the teachers' level of annoyance.

OBJECTIVE: The aim of this article is to determine the level of noise in nurseries and pre-schools and also to compare measured levels with standard levels and evaluate the teachers' level of annoyance. MATERIALS AND METHODS: The level of noise was measured in three different schools. A total of 162 students, whose ages were between 3 and 6 years, and 12 teachers were included the study. Every age groups' level of noise was measured during sleeping, gaming, and eating activity. In addition, teachers' annoyance was assessed in different age groups. RESULTS: The 4- to 6-year-old groups were found to have higher level of sounds than 3-year-old group. Eating period was found to be the highest level of sound whereas sleeping was found the lowest. Furthermore, teachers' annoyance was found higher as the age decreased. CONCLUSION: Nurseries and pre-schools have noisy environment both for the students and the teachers. High level of noise, which has bad effects on health, is a public health problem. Both the students' families and teachers must be aware of this annoying situation.

Management of children with auditory neuropathy spectrum disorder (ANSD) / Tratamento de crianças com espectro da neuropatia auditiva (ENA)

ABSTRACT INTRODUCTION: ANSD is a challenging problem. OBJECTIVE: To present our experience on management of the children with ANSD with respect to clinical data. METHODS: This retrospective study included all children younger than 16 years of age who applied to the department between 2005 and 2013 (with the exception of newborn hearing screening NHS referrals). The data were derived from pure tone, OAEs and ABR tests, and further medical risk factors of the subjects were evaluated. RESULTS: ANSD was recognized in 74 ears of 40 children (B/U: 34/6) among 1952 children with SNHL (2.04%) detected among 9520 applicants to the department (0.42%). The clinical tests revealed that hearing loss greater than 15 dB was present in both ears of 38 cases. The degree of hearing loss was profound in 48% children, severe in 12% children, moderate in 28% children, mild in 10% children and normal in 5% children. ABRs were absent/abnormal in 37/3 ears and CMs were detected in all. Acoustic reflexes were absent in all ears. Rehabilitation was managed by CI and hearing aids in 15 and 23 cases, respectively. FM system was given to two cases displaying normal hearing but poor speech discrimination in noisy environments. CONCLUSION: ANSD is a relatively challenging problem for the audiology departments because of its various clinical features and difficulties in management. Our patients with ANSD most commonly displayed profound hearing loss. The number of overlooked cases may be minimized by performing ABR and OAE in every case referred with the suspicion of hearing loss.

Resumo Introdução: Espectro da neuropatia auditiva ainda é uma condição clínica desafiadora. Objetivo: Apresentar nossa experiência no tratamento de crianças com espectro da neuropatia auditiva em relação aos dados clínicos. Método: Este estudo retrospectivo incluiu crianças menores de 16 anos de idade que deram entrada no departamento entre 2005 e 2013 (com exceção de encaminhamentos para triagem auditiva neonatal). Foram avaliados os dados obtidos a partir dos exames de audiometria tonal, emissões otoacústicas (EOA), potencial evocado auditivo de tronco encefálico (ABR) e outros fatores de risco. Resultados: Das 1.952 crianças com perda auditiva neurossensorial (2,04%) detectadas dentre os 9.520 candidatos que deram entrada no departamento (0,42%), espectro da neuropatia auditiva foi reconhecida em 74 orelhas de 40 crianças (B/U: 34/6). Os testes clínicos revelaram que uma perda auditiva superior a 15 dB estava presente em ambas as orelhas em 38 casos. O grau de perda auditiva das crianças era profundo em 48%, grave em 12%, moderado em 28%, leve em 10%, e normal em 5%. ABR estava ausente/anormal em 37/3 orelhas e microfonia coclear foi detectado em todas as crianças. Reflexos acústicos estavam ausentes em todas as orelhas. A reabilitação foi tratada com implante coclear e aparelhos auditivos em 15 e 23 casos, respectivamente. Um sistema FM foi utilizado em dois casos que apresentavam audição normal, mas discriminação deficiente da fala em ambientes ruidosos. Conclusão: Espectro da neuropatia auditiva é um problema desafiador para os departamentos de audiologia, devido às suas várias características clínicas e dificuldades no tratamento. Em nossos pacientes a perda auditiva profunda foi a mais frequente. O número de casos negligenciados pode ser diminuído com a realização dos exames ABR e EOA em todos os casos encaminhados com suspeita de perda auditiva.

Auditory evoked potentials in a newborn Wistar rat model of hyperbilirubinemia / Potenciais evocados auditivos em um modelo de rato Wistar neonato com hiperbilirrubinemia

ABSTRACT INTRODUCTION: Hyperbilirubinemia is a common health problem in newborns. Its effects can be different according to the level and duration of the hyperbilirubinemia. The toxic effect of bilirubin on the auditory system can be seen as a sensory neural hearing loss or auditory neuropathy spectrum disorder (ANSD). OBJECTIVE: The purpose of our study was to determine the effects of toxic bilirubin level on the auditory system by using Auditory Brainstem Response audiometry. METHODS: Rats are used as animal models due to their low cost and easy attainability. Auditory Brainstem Response was used for auditory assessment. In this study, three groups were established: experimental, control and placebo groups. RESULTS: In the experimental group, which consists of rats with hyperbilirubinemia, sensory neural hearing loss was found bilaterally in 4 rats (66.67%) and unilaterally in 2 rats (16.67%) and auditory neuropathy spectrum disorder was found unilaterally in 1 rat (8.33%). Auditory Brainstem Response thresholds were significantly elevated compared to control and placebo groups (p < 0.05). CONCLUSION: Hyperbilirubinemia of newborn rats may result both in sensory neural hearing loss and auditory neuropathy spectrum disorder.

RESUMO INTRODUÇÃO: A hiperbilirrubinemia é um problema de saúde comum em neonatos. Seus efeitos podem variar, dependendo do nível e da duração da hiperbilirrubinemia. O efeito tóxico da bilirrubina no sistema auditivo pode ser observado na forma de deficiência auditiva sensorioneural ou de distúrbio do espectro da neuropatia auditiva. OBJETIVO: A finalidade de nosso estudo foi determinar os efeitos de nível tóxico de bilirrubina no sistema auditivo, com o uso da audiometria da resposta auditiva evocada de tronco cerebral. MÉTODO: Os ratos são empregados como modelos animais graças a seu baixo custo e fácil obtenção. Utilizamos a resposta auditiva evocada de tronco cerebral para avaliação da audição. No estudo, foram estabelecidos três grupos: experimental, controle e placebo. RESULTADOS: No grupo experimental, constituído de ratos com hiperbilirrubinemia, disacusia auditiva neurosensorial foi diagnosticada bilateralmente em quatro ratos (66,67%), e unilateralmente em dois (16,67%); e distúrbio do espectro da neuropatia auditiva foi observado unilateralmente em um rato (8,33%). Os limiares da resposta evocada de tronco cerebral estavam significantemente elevados, em comparação com os grupos controle e placebo (p < 0,05). CONCLUSÃO: A hiperbilirrubinemia de ratos neonatos pode resultar tanto em disacusia auditiva neurosensorial como em distúrbio do espectro da neuropatia auditiva.

Auditory evoked potentials in a newborn Wistar rat model of hyperbilirubinemia.

INTRODUCTION: Hyperbilirubinemia is a common health problem in newborns. Its effects can be different according to the level and duration of the hyperbilirubinemia. The toxic effect of bilirubin on the auditory system can be seen as a sensory neural hearing loss or auditory neuropathy spectrum disorder (ANSD). OBJECTIVE: The purpose of our study was to determine the effects of toxic bilirubin level on the auditory system by using Auditory Brainstem Response audiometry. METHODS: Rats are used as animal models due to their low cost and easy attainability. Auditory Brainstem Response was used for auditory assessment. In this study, three groups were established: experimental, control and placebo groups. RESULTS: In the experimental group, which consists of rats with hyperbilirubinemia, sensory neural hearing loss was found bilaterally in 4 rats (66.67%) and unilaterally in 2 rats (16.67%) and auditory neuropathy spectrum disorder was found unilaterally in 1 rat (8.33%). Auditory Brainstem Response thresholds were significantly elevated compared to control and placebo groups (p<0.05). CONCLUSION: Hyperbilirubinemia of newborn rats may result both in sensory neural hearing loss and auditory neuropathy spectrum disorder.

Management of children with auditory neuropathy spectrum disorder (ANSD).

INTRODUCTION: ANSD is a challenging problem. OBJECTIVE: To present our experience on management of the children with ANSD with respect to clinical data. METHODS: This retrospective study included all children younger than 16 years of age who applied to the department between 2005 and 2013 (with the exception of newborn hearing screening NHS referrals). The data were derived from pure tone, OAEs and ABR tests, and further medical risk factors of the subjects were evaluated. RESULTS: ANSD was recognized in 74 ears of 40 children (B/U: 34/6) among 1952 children with SNHL (2.04%) detected among 9520 applicants to the department (0.42%). The clinical tests revealed that hearing loss greater than 15dB was present in both ears of 38 cases. The degree of hearing loss was profound in 48% children, severe in 12% children, moderate in 28% children, mild in 10% children and normal in 5% children. ABRs were absent/abnormal in 37/3 ears and CMs were detected in all. Acoustic reflexes were absent in all ears. Rehabilitation was managed by CI and hearing aids in 15 and 23 cases, respectively. FM system was given to two cases displaying normal hearing but poor speech discrimination in noisy environments. CONCLUSION: ANSD is a relatively challenging problem for the audiology departments because of its various clinical features and difficulties in management. Our patients with ANSD most commonly displayed profound hearing loss. The number of overlooked cases may be minimized by performing ABR and OAE in every case referred with the suspicion of hearing loss.

Newborn hearing screening outcomes during the first decade of the program in a reference hospital from Turkey.

In this study, the authors report the results of a three-stage newborn hearing screening (NHS) program for well babies at the Gazi University Hospital (GUH) in Ankara between 2003 and 2013. GUH-NHS was performed by automated transient evoked otoacoustic emission (a-TEOAE) at the first and second steps and by automated brainstem audiometry (a-ABR) at the third step. The data were analysed to assess not only rate of congenital permanent hearing loss (CPHL), but also the effectiveness of the program during the years. A total of 18,470 well babies were tested. The data showed that coverage ratio for the GUH-born babies was increased and more outside-born babies (OBB) were admitted by time (means 84.31 and 11.28 %, respectively). Mean CPHL was found to be 0.26 %. Mean referral rate was decreased to 0.81 % by a-ABR from 2.16 % by a-TEOAE. Mean of missed cases in any stage of GUH-NHS was 4.88 %. It was seen that neither CPHL nor referral rate, but only ratio of missed ones presented increase in parallel to increment in OBB. This paper first presents that clinically acceptable screening procedures developed in GUH by time, and secondly higher rate of CPHL in Turkey than in the Western countries, and benefits of third stage screening by a-ABR because it prevented referral of 251 children (1.29 %) to the clinical tests. We think that this number is reasonably important regarding not only economical point of view, but also waiting lists in the audiology departments in a developing country, in which audiological service is still limited.

Speech Range Profile (SRP) Findings Before and After Mutational Falsetto (Puberphonia).

INTRODUCTION: Mutational falsetto is a temporary adolescent voice. Voice therapy support in mutational falsetto helps the individual to regain a healthy voice. Speech Range Profile (SRP) is considered to be a method requiring less time in the pretherapy and posttherapy evaluations of voice patients. OBJECTIVE: The purpose of this study was to determine the differences between the SRP results of male subjects experiencing mutational falsetto before and after therapy. METHODS: Sixteen male participants have been included in this study. F0, MinF0, MaxF0, F0Range, F0Range (st), MindB SPL, and MaxdB SPL have been taken to evaluation. SRP recordings of all subjects have been performed. All the recordings have been repeated before and after therapy. RESULTS: A statistically significant difference has been observed between the F0 and MinF0 values before and after therapy (P < 0.001). CONCLUSIONS: SRP provides important information about frequency and intensity. In mutational falsetto, SRP is a useful technique in terms of evaluating success in therapy.

Audiologic evaluations of children with mucopolysaccharidosis.

INTRODUCTION: Mucopolysaccharidosis is a hereditary lysosomal storage disease, which develops due to a deficiency in the enzymes that play a role in the metabolism of glycosaminoglycans (GAG). The incidence of mucopolysaccharidosis is 1/25,000, with autosomal recessive inheritance (except for MPS II). Mucopolysaccharidosis occurs in seven different types, each with a different congenital deficiency of lysosomal enzymes. In mucopolysaccharidosis patients, even though progression of clinical findings is not prominent, the disease advances and causes death at early ages. Facial dysmorphism, growth retardation, mental retardation, and skeletal or joint dysplasia are the most frequently found symptoms in these patients. OBJECTIVE: The purpose of our study is to present the types of hearing loss types and tympanometric findings of patients with mucopolysaccharidosis referred to our clinic with suspicion of hearing loss. METHODS: After otorhinolaryngological examination, 9 patients with different types of mucopolysaccharidosis, underwent to immittance and audiometric evaluations, performed according to their physical and mental abilities, and ages, in order to determine their hearing thresholds. RESULTS: The audiometric findings of the 9 patients followed with mucopolysaccharidosis were reported separately for each case. CONCLUSION: Based on the high frequency of hearing loss in mucopolysaccharidosis patients, early and detailed audiological evaluations are highly desirable. Therefore, regular and systematic multidisciplinary evaluations are very important.

Effects of polylactic acid film on middle ear mucosa and cochlear function in Guinea pigs.

Our aim was to assess the effects of polylactic acid (PLA) on middle ear mucosa and cochlea, to be used as a film barrier for postoperative adhesion prevention in the middle ear. Twenty-one albino Guinea pigs were included in the study. A window was opened on both tympanic bulla and on one side PLA material was placed in the middle ear and on the other side only fenestration was performed and used as a control. All Guinea pigs underwent evaluation of tympanic membranes microscopically; functional hearing was analyzed by auditory brainstem responses preoperatively, in the first and the sixth month. All Guinea pigs were killed on the sixth month for histopathologic evaluation of their temporal bones. There was no statistical difference between both groups regarding hearing thresholds, interpeak wave latencies preoperatively and on first and the sixth months postoperatively. Histopathological evaluation revealed no specific changes. There was a mild local inflammation both in the PLA implanted and control ears. PLA film barrier most likely has no toxic effects on Guinea pig middle ear and does not show any ototoxic side effects.

[Evaluation of music department students who passed the entrance exam with phonetogram (Voice Range Profile)]. / Müzik bölümü giris sinavinda basarili olan ögrencilerin fonotogram (Voice Range Profile) ile degerlendirilmesi.

OBJECTIVES: This study aims to evaluate phonetogram data of the students in the department of music who passed the entrance exam. PATIENTS AND METHODS: The phonetogram data of 44 individuals with a good voice quality in the department of music and age-matched individuals who were not trained in the field of music or not involved in music amateurish as the control group were compared. The voice of both groups were recorded using the voice range profile within the scope of Kay Elemetrics CSL (Model 4300 B) programmed. RESULTS: There was a significant difference in the voice range profile parameters including max Fo, Fo range, Fo range (St), min dB SPL, and max dB sound pressure level (p<0.05). CONCLUSION: Our study results suggest that the voice interval of the department of music is higher than the control group and that plays a major role in their acceptance to the department of music.

Methods and preliminary outcomes of pediatric auditory brainstem implantation.

OBJECTIVE: The objective was to provide information about methods used and preliminary outcomes for pediatric ABI (auditory brainstem implant). STUDY DESIGN: An analysis of outcome was performed in children who received an ABI. METHODS: Twelve children received a MED-EL ABI system. Progress in audition and language was monitored through parental reports, questionnaires, profiles, and closed-set tests. RESULTS: The median number of active electrodes was 9 of 12. Seven of 12 users consistently respond to sound, and 5 of 12 do not. Highest performers can recognize words in small sets and have begun to use some words. CONCLUSION: Auditory brainstem implants appear to be beneficial for some pediatric patients who cannot benefit from traditional cochlear implant surgery. Benefits in the short-term can be recognition of environmental sounds, recognition of some words and very commonly used phrases, and the beginning use of words. Although some of our ABI users demonstrate no response to sound, they do want to wear their sound processors all waking hours. The cause of lack of response may be related to the second intervention, which might have led to displacement of the electrode array, or presence of additional handicaps or syndromes. However, the results are less than optimal. The relatively short postoperative follow-up duration is a considered weakness of this study.

Functional outcomes of Vibrant Soundbridge applied on the middle ear windows in comparison with conventional hearing aids.

CONCLUSION: Vibrant Soundbridge (VSB) application to the middle ear windows yields better functional outcomes than conventional hearing aids. However, speech discrimination scores obtained with VSB and conventional hearing aids are similar. OBJECTIVE: To assess audiological outcomes of round and oval window applications of VSB in comparison with conventional hearing aids. METHODS: Nineteen adult patients were included in the study. The patients had mild to moderate, moderate or moderate to profound conductive or mixed hearing loss. During surgery the floating mass transducer (FMT) was placed on the round (n = 14) or oval (n = 5) window. After the surgery, audiometric evaluation and free field audiometric evaluation of both ears was carried out. RESULTS: The hearing thresholds in the low frequencies were not significantly different between the conventional hearing aids and VSB. The functional gains obtained with oval and round window approaches were similar except for 500 Hz. The hearing thresholds in the mid and high frequencies were significantly better with VSB than the conventional hearing aids. The functional gain in the low frequencies was not significantly different between VSB and conventional hearing aids. The functional gain in the other frequencies was significantly better with VSB than conventional hearing aids.

Association of GJB2 gene mutation with cochlear implant performance in genetic non-syndromic hearing loss.

OBJECTIVE: To analyze the association of GJB2 gene mutations with cochlear implant performance in children. METHODS: Sixty-five consecutive children who underwent cochlear implantation due to congenital profound senseurineural hearing between 2006 and 2008 were included in the study. In children, GJB2 gene mutation analysis was performed. Their auditory performance was assessed using MAIS, MUSS and LittlEARS tests. RESULTS: Twenty-two of sixty-five patients GJB2 mutations, and 35delG was the most frequent mutation. No significant difference was found between the auditory performance of mutation positive and negative children after one year follow up (p>0.05). CONCLUSION: GJB2 gene mutations do not impact on the outcome of cochlear implantation.