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Objective: To determine the prevalence and distribution of inner-ear malformations in congenital single-sided deafness cases, as details of malformation type are crucial for disease prognosis and management. Methods: A retrospective study was conducted of 90 patients aged under 16 years with congenital single-sided deafness. Radiological findings were evaluated using computed tomography and magnetic resonance imaging. Inner-ear malformations were identified and cochlear nerve status was determined in affected ears. Results: Out of 90 ears, 42 (46.7 per cent) were found to have inner-ear malformation. Isolated cochlear aperture stenosis was the most common anomaly (n = 18, 20 per cent), followed by isolated cochlear aperture atresia (n = 11, 12.2 per cent) and cochlear hypoplasia (n = 7, 7.8 per cent). Cochlear nerve deficiency was encountered in 41 ears (45.6 per cent). The internal auditory canal was also stenotic in 49 ears (54.4 per cent). Conclusion: Inner-ear malformations, especially cochlear aperture anomalies, are involved in the aetiology of single-sided deafness more than expected. The cause of single-sided deafness differs greatly between congenital and adult-onset cases. All children with single-sided deafness should undergo radiological evaluation, as the prognosis and management, as well as the aetiology, may be significantly influenced by inner-ear malformation type. [ABSTRACT FROM AUTHOR]

Cochlear implant is a electronic device that can help to provide a sense of sound to a person who is profoundly deaf or severely hard-of-hearing. Inner ear malformations constitute about %20 of conjenital sensorineural hearing loss. Cochlear hypoplasia represents a group of cochlear malformations where external dimensions are less than those of a normal cochlea with various internal architecture deformities. In Cochlear Hypoplasia-II, the cochlea has smaller dimensions with defective modiolus and interscalar septa, but with normal external outline; The vestibular aqueduct may be enlarged and the vestibule may be dilated (Sennaroglu,2010). A woman aged 33 was diagnosed with hearing loss at the age of 5. She has used hearing aid regularly in her right ear since she was 6 years old. She hasn't ever used hearing aid in her left ear. She applied to our clinic for cochlear implant operation. According to her audiological assessment; right ear hearing thresholds were increasing towards high frequencies,the pure tone average of right ear thresholds was 62 dB HL; left ear hearing thresholds couldn't obtain (total hearing loss). Her free field thresholds average with heariNg aid was 30 dB HL. Also the previous audiological assessment results were similar with our assessment. In the sentence test, the auditory-visual score was %87, and the only auditory score was %56. The findings from MRI and CT screening were bilateral cochlear hypoplasia-II and dilated vestibule. As a result; It was decided that the patient should be followed up with a hearing aid for a while;because she had benefited from the hearing aid and there was residual hearing in the right ear, especially at low frequencies. In addition; FM system was recommended to this patient to understand speech in noise. [ABSTRACT FROM AUTHOR]

Introduction: For a comprehensive approach in children with hearing loss (HL), some cognitive and language skills should also be considered, along with auditory skills. The main aim of this study was to evaluate the working memory and language skills in children with mild to moderate HL., Methods: Forty children with mild to moderate HL between the ages of 4 and 9 years were included in this study. The children with mild and moderate HL were evaluated in two groups. The Meaningless Word Repetition test, Test of Language Development-Primary test, and Meaningful Auditory Integration Scale were administered to assess working memory, language skills, and auditory perception, respectively. Also, the relationship between language and memory skills was evaluated., Results: The study found statistically significant correlations between language skills and working memory test scores. Additionally, statistically significant differences were found between children with mild and moderate HL in terms of language and memory skills (p < 0.001)., Conclusion: The children with moderate HL demonstrated poorer performance compared to those with mild HL. Even if it is mild, the degree of HL affects children's language and memory skills. It is emphasized that the relationship between language and working memory should be taken into consideration in auditory rehabilitation programs for these children., (© 2024 S. Karger AG, Basel.)

Purpose: This study aims to evaluate school-age language skills and auditory performance in different listening situations in children with cochlear implants and auditory brainstem implants., Method: The study included 60 children between the ages of 5 and 9 years with cochlear implants (CI) and auditory brainstem implants (ABI). The volunteer children were divided into two groups: bimodal CI-ABI and bilateral CI users. Test of Language Development: Primary (TOLD-P:4), which assesses components of language such as phonology, morphology, syntax and semantics, was used to evaluate school-age language skills. Children's Auditory Performance Scale (CHAPS) was used to measure their listening performance in quiet, noisy, multi-stimulus environments and their auditory attention and memory skills in daily life. The correlations between language and auditory performance were analyzed and compared between the two groups., Results: Children with ABI showed poorer performance in school-age language skills and auditory performance in different listening environments (p < 0.05). Significant correlations were between school-age language skills and auditory performance (p < 0.05)., Conclusion: Improved auditory performance is crucial for the development of school-age language skills. To improve auditory performance in children with ABI in different listening environments, assistive listening devices, acoustic environmental arrangements, informative activities, etc., should be used., (© 2024. The Author(s).)

Background:  Incomplete partition type II (IP-II) malformation is often accompanied by a large vestibular aqueduct (LVA). In IP anomalies, the patient's auditory rehabilitation requirements are decided according to the presence of inner ear structures and the degree of hearing loss (HL). There has been limited research on auditory rehabilitation (AR) requirement selection in patients diagnosed with IP-II and LVA. This study investigated the typical characteristics of HL and AR choices in patients diagnosed with IP-II and LVA., Methods:  Patients with IP-II and LVA (n=55; 25 women and 30 men) were identified, and audiological evaluations were performed. The patient's demographic characteristics, the type and degree of HL, the AR method, age at diagnosis, and educational status were retrospectively compared., Results:  The distribution of our 55 patients according to cochlear implants, hearing aids (HA), and bimodal applications was 29.1% (n=16), 43.6% (n=24), and 27.3% (n=15), respectively. Statistical analyses using chi-square tests found no significant differences in the incidence of dizziness/imbalance, tinnitus, HL progression, or the degree and onset of HL among the patients., Conclusion:  The data revealed different audiological characteristics among patients with IP-II and LVA, as well as different AR solutions. The most widely used AR modality was found to be HA. Prediction of sudden versus progressive HL development among patients is challenging, and the characteristics of IP-II vary. Therefore, they should be interpreted with caution.

The just-noticeable differences (JNDs) of the voice cues of voice pitch (F0) and vocal-tract length (VTL) were measured in school-aged children with bilateral hearing aids and children and adults with normal hearing. The JNDs were larger for hearing-aided than normal-hearing children up to the age of 12 for F0 and into adulthood for all ages for VTL. Age was a significant factor for both groups for F0 JNDs, but only for the hearing-aided group for VTL JNDs. Age of maturation was later for F0 than VTL. Individual JNDs of the two groups largely overlapped for F0, but little for VTL. Hearing thresholds (unaided or aided, 500-400 Hz, overlapping with mid-range speech frequencies) did not correlate with the JNDs. However, extended low-frequency hearing thresholds (unaided, 125-250 Hz, overlapping with voice F0 ranges) correlated with the F0 JNDs. Hence, age and hearing status differentially interact with F0 and VTL perception, and VTL perception seems challenging for hearing-aided children. On the other hand, even children with profound hearing loss could do the task, indicating a hearing aid benefit for voice perception. Given the significant age effect and that for F0 the hearing-aided children seem to be catching up with age-typical development, voice cue perception may continue developing in hearing-aided children., (© 2024 Author(s). All article content, except where otherwise noted, is licensed under a Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).)

Purpose: The purpose of this study was to (a) investigate which speech material is most appropriate as stimulus in head shadow effect (HSE) and binaural squelch (SQ) tests, (b) obtain normative values of both tests using the material decided to be optimal, and (c) explore the results in bilateral cochlear implant (CI) users., Method: Study participants consisted of 30 normal-hearing (NH) persons and 34 bilateral CI users. This study consisted of three phases. In the first phase, three different speech materials (1) monosyllabic words, (2) spondee words, and (3) sentences were compared in terms of (a) effect size, (b) test-retest reliability, and (c) interindividual variability. In the second phase, the speech material selected in the first phase was used to test a further 24 NHs to obtain normative values for both tests. In the third phase, tests were administered to a further 23 bilateral CI users, together with localization test and the Speech, Spatial, and Qualities of Hearing scale., Results: The results of the first phase indicated that spondees and sentences were more robust materials compared with monosyllables. Although the effect size and interindividual variability were comparable for spondees and sentences, sentences had higher test-retest reliability in this sample of CI users. With sentences, the mean (± standard deviation) HSE and SQ in the NH group were 58 ± 14% and 22 ± 11%, respectively. In the CI group, the mean HSE and SQ were 49 ± 13% and 13 ± 14%, respectively. There were no statistically significant correlations between the test results and the interval between the implantations, the length of binaural listening experience, or the asymmetry between the ears., Conclusions: Sentences are preferred as stimulus material in the binaural HSE and SQ tests. Normative data are given for HSE and SQ with the LiCoS (linguistically controlled sentences) test. HSE is present for all bilateral CI users, whereas SQ is present in approximately seven out of 10 cases.

Objectives: After auditory brainstem implant (ABI) surgery, stimulation of certain cranial nerves may result in a non-auditory response, and the electrodes that stimulate these nerves may be deactivated. The goals of this study are to compare the number of active electrodes in the initial activation and the last fitting, to investigate non-auditory response types and their frequency as a result of non-auditory stimulation, to compare the placements of deactivated electrodes as a result of non-auditory stimulation in the initial activation and the last fitting., Methods: The computer software system was used to perform a retrospective analysis of the fitting data of 69 ABI users who underwent auditory brainstem implant surgery between January 1997 and January 2019. The non-auditory response types, deactive electrodes, and the positioning of the deactive electrodes horizontally and vertically were recorded in these users during the initial activation and the last fitting., Results: There was no statistically significant difference between the number of active electrodes in the initial activation and the last fitting. The proportion of the users with deactive electrodes in the initial activation and the last fitting was not statistically significant different. In the horizontal and vertical placement classification, the placement of the deactive electrodes was not statistically different between initial activation and last fitting. The most common type of non-auditory response was facial nerve stimulation at the initial activation and no auditory perception at the last fitting. According to the difference between the number of active and deactive electrodes in the initial activation and the last fitting, as well as the auditory and non-auditory responses, it was found that the ABI users were statistically different between the initial activation and the last fitting., Conclusion: The results of this study show that not only auditory but also non-auditory responses occur in most ABI users. In addition, to the best of our knowledge, this study is the first to examine the frequencies of non-auditory response types, and the placement of the electrodes that cause these responses according to horizontal and vertical classifications., Competing Interests: Declaration of competing interest The authors declare that they have no conflict of interest., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Mothers' awareness of hearing loss, its risk factors, and available detection and intervention choices have been well known to play an essential role in the early detection and intervention of hearing loss., Objective: To investigate the knowledge and attitude of Syrian mothers toward infant hearing loss, early identification, and intervention., Methods: The "Maternal Views on Infant Hearing Loss" questionnaire was adapted and translated into Arabic and then administered to 100 Syrian mothers living in different neighborhoods in Ankara within the age range of 18-68 years old. The internal consistency for the main domains of the questionnaire was tested by Cronbach's alpha coefficient. Descriptive statistics and Spearman's rank correlation coefficient were used to evaluate the responses., Results: The highest level of knowledge about the risk factors was about noise exposure (76%), while the lowest level of knowledge was about jaundice (25%). While 98% of the mothers had a positive attitude toward early detection, 97% of them did not mind the early intervention., Conclusions: The present findings showed the need to improve mothers' awareness about infant hearing loss risk factors, available detection, and intervention. Such results may help in performing programs that aim to increase awareness about hearing loss., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

This study aimed to evaluate audiological findings among patients with glutaric aciduria type 1 (GA-1). We used a large test battery for the audiological evaluation of 17 individuals with GA-1 (the study group) and 20 healthy individuals (the control group). Conventional audiometry (0.125-8 kHz), distortion product otoacoustic emissions (DPOAEs) (1, 1.5, 2, 3, 4, 6, and 8 kHz), contralateral suppression of otoacoustic emissions, and auditory brainstem response (ABR) ( 30, 50, 70 and 90 dB nHL) were measured for all participants (n = 37). Mild sensorineural hearing loss was found in 77.47% (n = 13) of the patients with GA-1, and normal hearing thresholds were seen in 23.53% (n = 4). There were three asymptomatic patients at the time of diagnosis [two developed mild mental motor retardation (MMR) and one developed severe MMR during the follow-up], one with a normal hearing threshold and two with mild hearing loss), and 14 symptomatic patients (three with normal hearing thresholds and 11 with mild hearing loss). Seven of the symptomatic patients diagnosed following an encephalopathic crisis required intensive care and showed significantly worse hearing thresholds than those without symptoms [20.86 ± 4.47 vs. 15.44 ± 3.96 decibel hearing level (dB HL), p = 0.039*], while five had mild-to-moderate hearing loss. Acute encephalopathic crisis had a negative effect on hearing function in the symptomatic patients. The emission and contralateral suppression amplitude values of the study group were significantly lower compared to the control group (p < 0.05). The I-V interpeak latency and absolute latencies of ABR waves I, III, and V of the study group were observed to be significantly prolonged and morphologically distorted compared to those of the control group (p < 0.05). Five patients had MMR, and three had moderate MMR; all eight had mild-to-moderate hearing loss. In addition, of the eight patients with mild MMR, four had mild hearing loss. In particular, the morphological findings of ABR waves were significantly worse in the patients with severe and moderate MMR (p < 0.05). There was a significant correlation between a macrocephaly history (12 patients) and hearing loss (p = 0.041*). Magnetic resonance imaging findings were evaluated in all the 17 patients with GA-1, and typical fronto-temporal atrophy and sylvian fissure enlargement were observed. Our findings support that GA-1 is associated with auditory impairment, primarily in symptomatic patients. Adequate audiological test battery evaluation is essential in this context, particularly for symptomatic patients with a history of encephalopathic crises., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: To report the audiological, rehabilitative, and surgical outcomes of revision surgery for pediatric auditory brainstem implant (ABI) users., Study Design: Retrospective cohort., Setting: Tertiary referral center., Patients: Five pediatric ABI users who had revision surgery for device malfunctions., Interventions: Revision surgery for ABI malfunctions., Main Outcome Measures: The findings of free-field audiometry with the device, the Meaningful Auditory Integration Scale, and the pattern discrimination, word identification, sentence recognition, and expressive and receptive language tests before the device failure and after revision surgery were obtained from the patient records and compared., Results: The revision rate for pediatric ABI was 6.45%. The Meaningful Auditory Integration Scale and expressive-receptive language scores showed improvements following revision surgery, while the aided thresholds, pattern perception, and word identification scores did not change. Individual differences in performance for these measures were observed., Conclusion: Equal or improved performance after the revision surgeries in the current study showed that revision surgery is successful and important for pediatric ABI users. It is essential to consider remedying the loss of auditory input in sensitive periods of pediatric development., Competing Interests: The author discloses no funding and conflicts of interest., (Copyright © 2021, Otology & Neurotology, Inc.)

Background: This study was planned (1) to evaluate long-term outcome after cochlear implantation in patients with post-meningitic deafness and (2) to compare the outcome measures with patients implanted for deafness due to other causes., Methods: Records of 54 patients deafened as a sequel of bacterial meningitis and implanted at the largest university-based cochlear implant program in Turkey were retrospectively reviewed. Fifty-four age- and sex-matched patients with a similar interval of implant use were selected for controls. Surgical and long-term audiological outcome (in terms of categories of auditory performance-II scores) was assessed and compared., Results: Twenty-seven (52%) patients had some degree of labyrinthitis ossificans and 19 of them had full electrode insertion via basal turn cochleostomy. Patients with and without labyrinthitis ossificans in the post-meningitic group had no difference in final categories of auditory performance-II score (P=.559). Median categories of auditory performance-II scores were 6 for post-meningitic group and 7 for controls, with a significant statistical difference (P < .001). Partial or full insertions did not differ in outcome (P=.938). Mean time to implantation was not correlated with the final categories of auditory performance-II score for the post-meningitic group (P=.695)., Conclusion: Cochlear implant recipients deafened due to meningitis have a worse long-term hearing and speech performance as measured by categories of auditory performance-II than patients implanted for congenital deafness. The presence of labyrinthitis ossificans or the limited extent of electrode insertion produced overall results that were comparable with other cases.

Objective: To report the initial surgical and audiological outcomes of three pediatric patients with severe inner ear malformations who were simultaneously implanted with cochlear and brainstem implants in the same surgical session., Study Design: Retrospective case review., Setting: Tertiary referral center., Patients: Three pediatric patients with severe inner ear malformations between ages of 1.9 to 2.5 years, who were simultaneously implanted with cochlear implant in one ear and auditory brainstem implant in the other ear., Intervention(s): Simultaneous application of cochlar implant in one ear, auditory brainstem implant in the other ear., Main Outcome Measures: Free field thresholds with cochlear and brainstem implants. Surgical issues are also discussed., Results: The study is descriptive in nature. Free field thresholds with each device alone and together showed good progress. One of the patients had slower progress possibly due to comorbid CHARGE syndrome., Conclusions: The results showed good progress in terms of audition with both devices. Simultaneous cochlear and brainstem application serves as a remedy for pediatric patients who are candidates for cochlear implant on one side and brainstem implant on the other side. With this simultaneous application precious time for auditory development is not lost.

Objective: The objective of this study was to determine the fastest and the most effective auditory brainstem response (ABR) measurement protocol for audiological diagnosis in babies up to three months of age., Methods: Twenty-two newborns (aged 0 to 63 days) who passed the newborn screening test in at least one ear were evaluated in the study. The ABR were recorded with click stimulus using two different electrode montages (1 st montage: ipsilateral mastoid, contralateral mastoid, vertex. 2 nd montage: nape of the neck, vertex, cheek). Latencies of waves I, III, V and duration of the test were recorded and analyzed., Results: Wave V latencies from both electrode montages were statistically shortest at the level of 70 dBnHL and longest at the level of 20 dBnHL (p=0.00). When the duration of the test at three different intensity levels were compared between the two electrode montages, only the test durations at 50 dBnHL were significantly different (p=0.017). The test times at 70 dBnHL in the first montage were observed to be significantly different in babies aged 1 to 30 days and aged 31 to 63 days (p=0.005)., Conclusion: In protocols to evaluate the hearing of pediatric groups, it is very important to complete the ABR, which has significant value in early diagnosis, in a short time and reliably. It is concluded that in terms of practicality, the second montage is more advantageous and comfortable for both audiologists and newborns in single channel ABR systems., Competing Interests: Conflict of Interest: The authors have no conflicts of interest to declare., (© Copyright 2020 by Official Journal of the Turkish Society of Otorhinolaryngology and Head and Neck Surgery.)

Objective: The deficits in the cochlea which is at the one end of the ear sound transfer system, may effect middle ear functions. Wideband typanometry (WBT) is frequently used to evaluate these transfer functions which play a crucial role in setting the impedance matching between the external ear and the cochlea. To this end, the aim of this study was to investigate the ear transfer functions in inner ear malformations via WBT, and to question whether these functions change depending on the types of inner ear malformation., Methods: This prospective case-control study was conducted in a university hospital. One hundered-fifty-seven ears (aged 3-37 years) under the groups of cochlear hypoplasia, incomplete partition I, incomplete partition II, cochlear aplasia and complete labyrinthine aplasia were evaluated. In the control group, 30 ears with normal hearing were enrolled and WBT was carried out. Tympanometric peak pressure, equivalent middle ear volume, static admittance, tympanogram width, resonance frequency, average wideband tympanometry and absorbance measurements were analyzed., Results: The inner ear malformation groups demonstrated statistically significant differences than the control group and from each other in terms of traditional tympanometric parameters and WBT test parameters (p<0.05). The most remarkable difference was between the group of complete labyrinthine aplasia and the control group, most probably because of complete labyrinthine aplasia's structural effects. However, on some parameters, incomplete partition II and the control group showed similarities. In absorbance measurements, there was significant difference between all patient groups and the control group, especially at high frequencies (p<0.05). The largest difference was between the control group and the group of complete labyrinthine aplasia which has revealed the lowest absorbance values (p<0.05). In averaged-wideband tympanogram analysis, all patient groups obtained a lower amplitude peak than the control group; complete labyrinthine aplasia group had the flattest peaked amplitude, while the incomplete partition II group had a near-normal curve., Conclusion: The results of the study revealed the distinctive effects of inner ear malformations in middle ear transfer functions. It is concluded that the absence of inner ear structures causes negative effects on energy absorbance and the other transfer functions of the middle ear. WBT may provide additional information on diagnosis of patients with inner ear malformations., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Purpose: The present study aimed to identify temporal processing abilities in users of bone-anchored hearing aid (BAHA)., Methods: Seventeen subjects with BAHA (SWB) users and 29 subjects with normal-hearing were compared using the frequency pattern test, duration pattern test, and speech-in-noise test. Besides behavioral temporal processing tests, a multi-feature mismatch negativity (MMN) test was performed in BAHA users to assess the accuracy of objective auditory discrimination at the cortical level, irrespective of the subjects' attention or behavioral task., Results: The results of the multi-feature MMN test showed that BAHA users could perceive differences in frequency, duration, intensity. However, no response was elicited regarding localization of stimulus and gaps within tones. Word recognition performance in noise was significantly poorer compared with normal hearing groups. All age groups showed no differences in terms of temporal processing abilities except 30-39 years duration pattern ability weaker in SWB group., Conclusion: The present study was the first to indicate that the use of unilateral BAHA has no effect on the localization of auditory stimuli at the cortical level according to electrophysiological test. Although Baha users have near-normal levels of hearing thresholds and temporal ordering abilities with their devices, since they benefit from the unilateral device, they still have some difficulties in resolution, recognizing and distinguishing the spatial aspects of speech, especially in multiple and noisy listening environments.

Objective: To study the effect of age at auditory brainstem implant (ABI) surgery on auditory perception, language, and speech intelligibility., Study Design: Retrospective single cohort design., Setting: Tertiary referral center., Patients: In this study, 30 pediatric ABI users with no significant developmental issues were included. Participants were divided into two groups, according to age at surgery (Early Group: < 3 yr old [n = 15], Late Group: ≥ 3 yr old [n = 15]). Groups were matched by duration of ABI use and participants were evaluated after 5 years (±1 yr) experience with their device. The mean age at ABI surgery was 22.27 (ranged ± 6.5) months in the early group, 45.53 (ranged ± 7.9) months in the late group., Intervention(s): Retrosigmoid craniotomy and ABI placement., Main Outcome Measure(s): Auditory perception skills were evaluated using the Meaningful Auditory Integration Scale and Categories of Auditory Performance from the Children's Auditory Perception Test Battery. We used a closed-set pattern perception subtest, a closed-set word identification subtest, and an open-set sentence recognition subtest. Language performance was assessed with the Test of Early Language Development and Speech Intelligibility Rating, which was administered in a quiet room., Results: In this study, the results demonstrated that the Early Group's auditory perception performance was better than the Late Group after 5 years of ABI use, when children had no additional needs (U = 12, p < 0.001). Speech intelligibility was the most challenging skill to develop, in both groups. Due to multiple regression analysis, we found that auditory perception categories can be estimated with speech intelligibility scores, pattern perception scores, receptive language scores, and age at ABI surgery variables in ABI users with no additional handicaps., Conclusions: ABI is a viable option to provide auditory sensations for children with cochlear anomalies. ABI surgery under age 3 is associated with improved auditory perception and language development compared with older users.

Objective: To compare the audiological and radiological findings of patients with incomplete partition malformations (IPs) and analyze the relationship between the audiological and radiological findings., Materials and Methods: The study included 84 patients (168 ears) with IPs as follows: 26 patients with Type I;IP-I (41 ears), 54 patients with Type II;IP-II (108 ears), and 4 patients with Type III;IP-III (8 ears). Remaining 11 ears were diagnosed with other inner ear malformations. Air and bone conduction thresholds were determined with pure tone audiometry, and the air bone gap was recorded in all patients with IPs. Magnetic resonance imaging studies and computerized tomography scans of the temporal bones were analyzed using the PACS system of our university., Results: It was found that all the ears with IP-I were diagnosed with severe to profound hearing loss. The degree of the hearing loss varied from mild to severe/profound in patients with IP-II. Severe to profound mixed hearing loss (MHL) was determined in all ears with IP-III. The air bone gap was larger in the lower frequencies in the IP-II cases diagnosed with MHL. There was not a significant difference between the air bone gap and the size of the vestibular aqueduct in ears with IP-II (p>0.05)., Conclusion: Each type of IP has different audiological findings. Depending on the type and degree of the hearing loss, it is possible to choose the appropriate audiological intervention. Patients with IP should be evaluated according to the type of malformation.

Objective: To report the long-term outcomes of children who received auditory brainstem implant (ABI) because of severe inner ear malformations., Study Design: Retrospective chart review., Setting: Tertiary referral otolaryngology clinic., Subjects and Methods: Between July 2006 and October 2014, 60 children received ABI at Hacettepe University. Preoperative work up included otolaryngologic examination, audiological assessment, radiological evaluation together with assessment of language development and psychological status. The surgeries were performed via retrosigmoid approach with a pediatric neurosurgeon. Intraoperatively, electrical auditory brainstem response was utilized. Initial stimulation was done 4 to 5 weeks postoperatively. Outcomes were evaluated with Categories of Auditory Performance (CAP), speech intelligibility rate (SIR), functional auditory performance of cochlear implant (FAPCI) and Manchester Spoken Language Development Scale scores; receptive and expressive language ages were determined., Results: Sixty children who received ABI were between ages of 12 and 64 months. Thirty-five patients with follow up period of at least 1 year, were reported in means of long-term audiological and language results. The most prevelant inner ear malformation was cochlear hypoplasia (n = 19). No major complication was encountered. Majority of the patients were in CAP 5 category, which implies that they can understand common phrases without lip reading. SIR was found out to be better with improving hearing thresholds. Children with ABI were performing worse than average cochlear implantation (CI) users when FAPCI scores were compared. Patients with the best hearing thresholds have expressive vocabulary of 50 to 200 words when evaluated with Manchester Spoken Language Development Scale. There was no relationship between the number of active electrodes and hearing thresholds. The type of inner ear anomaly with the best and the worst hearing thresholds were common cavity and cochlear aperture aplasia, respectively. Patients with additional handicaps had worse outcomes. Among 35 children, 29 had closed set discrimination and 12 developed open set discrimination above 50%. It was determined that, progress of the patients is faster in the initial 2 years when compared with further use of ABI., Conclusion: ABI is an acceptable and effective treatment modality for pediatric population with severe inner ear malformations. Bilateral stimulation together with CI and contralateral ABI should be utilized in suitable cases.

The aim of this study was to investigate the amount of binaural squelch effect (BSE) and head shadow effect (HSE) in children who use unilateral cochlear implants (CI) and contralateral hearing aids (HA). The study group consisted of 19 CI recipient children who consistently wore a contralateral HA. Speech sounds were used to evaluate speech perception performance in noise. Testing was performed in three listening conditions: (1) bimodal listening with noise source on HA side; (2) CI only with noise source contralaterally (HA off); (3) CI only with noise source on the CI side. Statistical analysis revealed a significant difference between the three listening conditions and post hoc tests indicated significant differences for all pairwise comparisons (p < 0.001). The average BSE and HSE were 11.8% and 17.1% respectively. The majority of bimodal CI users showed BSE and HSE with significant speech perception improvement in the presence of noise.

Objectives: The Turkish matrix sentence test, TURMatrix, was developed for precise, internationally comparable speech intelligibility testing., Design: The TURMatrix comprises a base matrix of ten well-known Turkish names, numbers, adjectives, objects, verbs, from which syntactically fixed sentences were randomly composed. Test conduction may be in an open-set (standard), or closed-set response format. Homogeneity in intelligibility of the test material was optimized by applying level adaptations (maximal ± 3 dB) based on word-specific speech reception thresholds (SRTs). Test list equivalence was verified and reference values were determined., Study Sample: Thirty-eight native listeners of Turkish with normal hearing., Results: After training, mean SRT and slope of the final test lists were -8.3 ± 0.2 dB SNR and 14.1 ± 1.0%/dB, respectively (fixed SNR measurements; inter-list variability). For adaptive measurements, average across listeners was -7.2 ± 0.7 dB SNR in the open-set and -7.9 ± 0.7 dB SNR in the closed-set response format. Mean SRT for adaptive measurements in the open-set response format in quiet was 20.3 ± 4.1 dB. Individual SRTs in quiet correlated more closely with audiograms than with SRTs in noise., Conclusions: The TURMatrix was developed according to European standards and provides reliable speech intelligibility measurements in noise and quiet.

Objectives: The facial nerve usually occupies the oval window area in patients with oval window atresia. During exploration, if the facial nerve is discovered to lie in the oval window area, this is usually regarded as a contraindication for further surgical intervention. The aim of the present paper is to demonstrate the preoperative pathognomonic radiological sign and describe a new surgical approach for this difficult situation., Methods: 3 patients and 4 ears were operated due to conductive hearing loss by the same surgeon in a tertiary referral center. Their clinical presentation, radiological findings, surgical findings and final outcomes were evaluated and correlated., Results: Surgical findings were identical in all 4 ears: facial nerve was running over the oval window and tympanic portion was completely dehiscent. Incus long arm was medially displaced due to abnormal development of the stapes suprastructure. In each ear a successful vestibulotomy and teflon piston placement was achieved. Preoperative mean air-bone gap of 47.5dB was improved to 21.5dB. There were no complications., Conclusion: Oval window atresia is a rare middle ear anomaly usually regarded as a contraindication for surgical intervention. In this study we present a novel surgical approach with succesful results. However the best approach is to inform the family by showing the nerve on tomography, showing the operation video, informing the family about the sensorineural hearing loss and letting the family choose the treatment option., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

The aim of the study was to present the hearing and speech-language findings of preschool children. The children in this study were aged 3-5 years. Sixtyseven of 239 children (28.0%) had been referred to a physician because of possible middle ear problems, and 25 of the 67 children had slight and mild conduction type hearing loss with air-bone gaps. One of 239 children had profound sensorineural hearing loss. Speech-language problems were found in 70 of 239 children (29.3%). Necessary attention should be paid to the evaluation of hearing and speech-language skills in preschool-aged children to avoid delayed detection and to give these children the opportunity for timely intervention for hearing and speech-language problems.

The aim of the study was to assess the audiological findings of a 4-year-old child with a homozygous COL11A2 mutation and to point out the role of continuous follow-ups in children with craniofacial syndromes after the newborn hearing screening. A 4-year-old boy with otospondylomegaepiphyseal dysplasia (OSMED) was followed up after birth for hearing loss. Transient Otoacoustic Emissions (TEOAEs), Distortion Product Otoacoustic Emissions (DPOAEs), Automated and Clinical Auditory Brainstem Response (AABR and ABR) measurements, Visual Reinforcement Audiometry, immitansmetric measurements and hearing threshold measurements were performed for audiological evaluation. The patient developed sensorineural hearing loss at 11 months of age while his hearing was normal at birth. Because of auditory-verbal training with hearing aids started at 20 months of age, he now has normal verbal communication with his peers. This study clearly demonstrates that hearing loss develops in infancy in patients with OSMED and underscores the importance of continued hearing screening beyond newborn period for early intervention of hearing impairment and communication problems., (Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.)

Introduction: We aimed to study the influence of age, in normal hearing individuals, on: the masking level difference test, the speech recognition in noise test, the transient evoked otoacoustic emissions test, and the contralateral transient evoked otoacoustic emission suppression test. We also aimed to research the effect of age when using these tests to evaluate the central auditory nervous system., Methods: Transient evoked otoacoustic emissions and contralateral transient evoked otoacoustic emission suppression were measured in all subjects. Subjects also underwent masking level difference and speech recognition in noise tests., Results: We found a decrease in transient evoked otoacoustic emission amplitudes, speech recognition in noise scores and hearing thresholds with age. We also found that higher masking level difference values were associated with lower speech recognition in noise scores and contralateral transient evoked otoacoustic emission suppression values., Conclusion: We conclude that decreasing speech recognition in noise scores are associated with decreasing contralateral transient evoked otoacoustic emission supression values. This effect may be related to medial efferent system dysfunction.

Mutations in the GJB2 gene have been shown to be the major cause of autosomal recessively inherited, prelingual, non-syndromic hearing loss. 35delG was found to be the most frequent mutation among Caucasians. In this study, we performed haplotype analysis of two large families with autosomal recessive non-syndromic hearing loss (totally 33 affected, 37 unaffected) from Trabzon (a city from the Eastern Black Sea region) by using polymorphic markers close to the 35delG mutation region, and identified a common haplotype, "2-6-4". The frequency of the mutant chromosomes having the 2-6-4 haplotype was compared between the Eastern Black Sea region and the other regions of Turkey and the difference was found to be significant (chi squared = 5.13/df = 1/p = 0.023). Also, when the frequency of mutant and wild type chromosomes having the 2-6-4 haplotype was compared in the Eastern Black Sea region, a statistically significant difference was observed in the mutant chromosomes (chi squared = 7.46/df = 1/p < or = 0.01). The results of this study demonstrate that the ancestral haplotype of the chromosomes bearing 35delG mutation in the Eastern Black Sea region is "2-6-4".

Objective/hypothesis: To determine the acute and subacute cochlear effects of sublethal total body irradiation., Study Design: The study was designed prospectively as a Sprague-Dawley (SD) rat model with otoacoustic emission measurement., Methods and Materials: Ten rats and 20 ears were included in the study. Distortion product otoacoustic emission (DPOAE) measurements were performed under ketamine anesthesia before and 1 to 8 weeks after sublethal dose total body irradiation. Eight and six rats survived at the first and eighth week measurements, respectively. The frequency bandwidth was set to 1,067 Hz (referenced to f1) - 9,512 Hz (referenced to f2) (2f1 - f2 = 832 - 6,076 Hz) and eight points were sampled per octave, but low-frequency (<2,549 Hz [referenced to 2f1 - f2]) results were not included in the analysis. Averages were obtained at each frequency, and statistical analysis was performed to compare the pre-and postradiation values. Then, three of the rats were killed for transient electron microscopy (TEM) of the temporal bones., Results: The posttreatment DPOAEs at almost all frequencies showed a trend toward decline at both the first and eighth weeks, but significant reduction was only detected at 3,600 and 3,961 Hz (referenced to 2f1 - f2) in the first week. TEM of the cochlea revealed ultrastructural changes in the outer hair cells (OHCs) and stria vascularis of the basal turn. The cochlear ganglion nuclei and cochlear nerve axons were unaffected., Conclusion: Radiation can affect the cochlea in the acute phase at the mid to high frequencies; further prediction for long-term effects requires longer follow-ups.

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.

The aetiology of Ménière's disease still remains unknown and its therapy is therefore empirical. As a result of immunological abnormalities demonstrated, steroids are commonly used in Ménière's 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.