Publications by authors named "Levent Sennaroglu"

75 Publications

Children with Auditory Brainstem Implant: How Do They Perform in Motor and Language Skills?

Audiol Neurootol 2021 Jan 26:1-9. Epub 2021 Jan 26.

Department of Otorhinolaryngology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Background: Young children are able to explore new objects and practice language through the acquisition of motor skills that lead to their overall development. Congenital hearing loss and total vestibular loss may contribute to the delay in speech and motor skill development.

Objectives: To investigate the relationship between motor development performance, speech perception, and language performance in children with auditory brainstem implant (ABI).

Method: Ten children, aged 4-17 years (mean age 9.76 ± 4.03), fitted with unilateral ABI for at least 2 years due to the presence of labyrinthine aplasia and rudimentary otocyst at least 1 side were included in the study. Several standardized tests, such as Bruininks-Oseretsky Motor Proficiency Test-2 (BOT-2), Children's Auditory Perception Test Battery, Meaningful Auditory Integration Scale (MAIS), and Test of Early Language Development-3, were performed to evaluate their skills of fine motor control, balance, manual dexterity, language, and auditory perception.

Results: A significant correlation was established between the BOT-2 manual dexterity and MAIS scores (r = 0.827, p < 0.05) and between the manual dexterity and language skills (for expressive language, r = 0.762, p < 0.05; for receptive language, r = 0.650, p < 0.05). Some of the BOT-2 balance tasks, such as standing on 1 leg on a line with eyes closed, standing on 1 leg on a balance beam with eyes open, standing heel-to-toe on a balance beam, and walking forward heel-to-toe on a line, showed a strong correlation with their receptive and expressive language performance (p < 0.05).

Conclusion: The current study has indicated that significantly poor manual and balance performances are associated with poor speech perception and language skills in children with ABI. The authors recommend performing a vestibular assessment before and after ABI surgery and the use of a holistic rehabilitation approach, including auditory and vestibular rehabilitation, to support development of the children with ABI.
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http://dx.doi.org/10.1159/000510584DOI Listing
January 2021

Cerebrospinal fluid gusher in incomplete partition type I anomaly.

Eur Arch Otorhinolaryngol 2021 Jan 13. Epub 2021 Jan 13.

Department of Otolaryngology, Hacettepe University Medical Faculty, Ankara, Turkey.

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http://dx.doi.org/10.1007/s00405-020-06592-6DOI Listing
January 2021

Management of stapes footplate fistula in inner ear malformations.

Int J Pediatr Otorhinolaryngol 2021 Jan 26;140:110525. Epub 2020 Nov 26.

Department of Otolaryngology, Hacettepe University Medical Faculty, Turkey.

Background: Certain inner ear malformations have stapes footplate fistula which may cause meningitis during otitis media. This may result in fatality. It is the responsibility of the otolaryngologist to diagnose and treat the condition to prevent further attacks of meningitis.

Materials And Methods: Surgical findings of the 17 patients who have inner ear malformations with oval window fistula were retrospectively analyzed. Inner ear malformations were classified according to Sennaroglu classification. Different stages of stapes footplate fistula are classified.

Findings: Seventeen patients had spontaneous stapes footplate fistula at the oval window. No patient had spontaneous leakage at the round window site. Proper sealing of the leakage area with fascia in a dumbbell fashion is mandatory. Keeping the stapes in place and lumbar drainage are the two most important factors in the successful management of fistula. Particularly important is the simultaneous fistula repair and cochlear implantation where combined postauricular-transcanal approach provides the best method. Vaccination is important but not sufficient to prevent meningitis in inner ear malformations unless repair of the fistula is performed.

Conclusion: If the patient has a history of meningitis in the presence of inner ear malformation, particular attention should be given to oval window area to look for an opacity, cyst or a leaking lesion at the stapes footplate. Immediate surgical exploration and repair of the leak is mandatory to prevent further attacks of meningitis. Surgeon should not leave the operation without fully controlling the leak.
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http://dx.doi.org/10.1016/j.ijporl.2020.110525DOI Listing
January 2021

Postural instability in children with severe inner ear malformations: Characteristics of vestibular and balance function.

Int J Audiol 2021 Feb 4;60(2):115-122. Epub 2020 Sep 4.

Department of Otorhinolaryngology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Objective: To investigate the postural instability and vestibular functions in children with severe inner ear malformations (IEMs).

Design: A prospective case-control study.

Study Sample: The study group consisted of 10 children using unilateral auditory brainstem implant (ABI) with labyrinthine aplasia or rudimentary otocyst. The age-matched control groups consisted of 10 unilateral cochlear implant (CI) users with normal inner ear structures and 10 healthy peers. All tests were performed to implant users when the implants were off.

Results: All median VOR gains in the ABI group (median anterior, lateral, and posterior canal 0.15, 0.05, and 0.05, respectively, for the non-implanted sides) were significantly lower than those of the control groups (median VOR gains ≥ 0.90 in both control groups). There were no oVEMP and cVEMP responses in the study group. The mean BOT-2 balance scores of the ABI (3.70 ± 1.34) group was dramatically lower than those of the CI (9.40 ± 2.88) and healthy control (16.20 ± 4.16,  < 0.001).

Conclusions: The postural instability in children with severe IEMs was higher than those in CI users with normal inner ear structures and healthy peers. The level of deficiency in the labyrinthine was more important for postural stability in children.
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http://dx.doi.org/10.1080/14992027.2020.1808250DOI Listing
February 2021

Audiological Performance in Children with Inner Ear Malformations Before and After Cochlear Implantation: A Cohort Study of 274 Patients.

Clin Otolaryngol 2021 Jan 22;46(1):154-160. Epub 2020 Sep 22.

Department of Otorhinolaryngology, Hacettepe University, Ankara, Turkey.

Background And Objective: Inner ear malformations (IEMs) are common in children with hearing loss. The different types of IEMs form a unique subgroup of cochlear implant (CI) candidates. We aimed to evaluate the auditory perception outcomes of CI in children with different types of IEMs and compare them with CI users without IEMs.

Methods: The study included 274 CI users with and without IEMs as two groups (n = 137, each). Both groups' chronological age at implantation and duration of CI usage was matched (± 8 months). All subjects were evaluated pre-operatively and post-operatively by the Ling's sound test and the auditory perception test battery, which includes the Meaningful Auditory Integration Scale (MAIS), closed-set Pattern Perception Test (PPT) and open-set Sentence Recognition Test (SRT). Besides, children with IEMs were assessed for language development.

Results: Progress in the IEMs' group differed according to the type of ear anomaly. CI users with enlarged vestibular aqueduct had the highest scores, while users with common cavity had the lowest. Children with IEMs performed well on the closed-set test while having difficulty with the open-set test.

Conclusion: Cochlear implantation outcomes are favourable in IEMs' patients with a cochlear nerve visible on magnetic resonance imaging. Our results indicate that it is critical to take the anatomical differences into account during follow-up and rehabilitation programmes. Each CI user should be evaluated according to his or her individual needs.
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http://dx.doi.org/10.1111/coa.13625DOI Listing
January 2021

Successful Use of a Cochlear Implant in a Patient with Bony Cochlear Nerve Canal Atresia.

J Int Adv Otol 2020 Aug;16(2):271-273

Department of Otolaryngology Head and Neck Surgery, Hacettepe University School of Medicine, Ankara Turkey.

The anatomical cause of congenital sensorineural hearing loss can be atresia of the bony cochlear nerve canal (BCNC). It has been reported that the cochlear nerve (CN) can be either hypoplastic or aplastic when the BCNC width is <1.5 mm radioanatomically. It is difficult to estimate the auditory-verbal abilities after cochlear implantation (CI) in patients with a hypoplastic CN. In such cases, it is also challenging to decide on the best treatment modality: CI or auditory brainstem implantation. In this case report, we present a 4-year-old male patient with BCNC atresia and the successful use of a cochlear implant; we also discussed the importance of audiological evaluation. A detailed radiological evaluation must be performed in every case following electrophysiological studies prior to CI. To accurately diagnose the pathology and select the surgical side, both computed tomography and magnetic resonance imaging scans should be used as complementary imaging methods in all CI candidates.
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http://dx.doi.org/10.5152/iao.2020.3766DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7419081PMC
August 2020

Evaluation of temporal and suprasegmental auditory processing in patients with unilateral hearing loss.

Auris Nasus Larynx 2020 Oct 5;47(5):785-792. Epub 2020 May 5.

Department of Audiology, Faculty of Health Science, Hacettepe University, 06100 Ankara, Turkey.

Objectives: To determine the temporal order, resolution, and perception of prosody skills in Single-Sided Deafness (SSD) compared to an age- and sex-matched normal hearing group's same side ear and both ears.

Methods: This was a Case-Control study including 30 subjects with SSD, and age- and sex-matched 30 subjects with bilateral normal hearing (total of 60 subjects- mean age: 38.7 ± 11.6 years). The Montreal Cognitive Assessment (MoCA), Frequency Pattern Test (FPT), Duration Pattern Test (DPT), Random Gap Detection Test (RGDT), Evaluation of Motor Response Time and Emotional Prosody Assessment were performed on the clinically normal ear in the SSD group, the same side ear in the normal hearing group, and both ears of the normal hearing group (the SSD, MNH, and BNH groups, respectively).

Results: Individuals with SSD had worse results in DPT (p < .001), gap detection at 0.5 kHz (p < .001), gap detection at 4 kHz (p < .001), and composite score (p < .001) than the BNH group. For reaction time measurements, the SSD group had slower performance scores than the BNH group for DPT (p < .001) and FPT (p < .001).

Conclusions: Poor temporal processing ability and reduced reaction times may help explain the difficulties in those with SSD in performing daily living activities such as speech understanding in noise and requires more processing efforts. However, there were no significant differences between the groups in frequency pattern performance and emotional prosody skills, supporting the claim that fundamental frequency is one of the most important measures of perception in emotional prosody. We demonstrated that unilateral hearing is adequate to analyze frequency patterns to aid in prosody perception. Analysis of reaction times in temporal processing and emotional prosody could provide different perspectives of auditory processing. Slower reaction time of SSD should be considered for habilitation purposes.
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http://dx.doi.org/10.1016/j.anl.2020.04.009DOI Listing
October 2020

Management of Far-Advanced Otosclerosis: Stapes Surgery or Cochlear Implant.

Turk Arch Otorhinolaryngol 2020 Mar 6;58(1):35-40. Epub 2019 Jul 6.

Department of Otorhinolaryngology-Head and Neck Surgery, Hacettepe University School of Medicine, Ankara, Turkey.

Objective: The aim of this report is to share our experience and treatment outcomes with far-advanced otosclerosis (FAO) patients.

Methods: Patients that underwent surgery from 2003 through 2014 at a tertiary referral center were retrospectively reviewed. Nineteen FAO patients were included in the study. Audiological results and the ability to communicate face to face and over telephone were considered as the main outcome measures.

Results: Six FAO patients benefited well from stapedotomy with an average of 5.9-decibel (dB) air-bone gap and 86% median speech discrimination. Cochlear implantation (CI) was performed in 13 patients; two had disease progression after stapedotomy, five had failed stapes surgeries elsewhere, and six preferred CI as primary treatment. Median speech discrimination score of CI patients was 78.4%. Overall, all patients had satisfactory face-to-face communication and 90% could use telephone.

Conclusion: Bilateral stapedotomy and wearing hearing aid is an effective and cost-effective solution for restoring natural binaural hearing and requires no specific training. Should stapedotomy fail, cochlear implantation is always a successful back-up option.
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http://dx.doi.org/10.5152/tao.2020.4600DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7162605PMC
March 2020

Simultaneous Cochlear and Auditory Brainstem Implantation in Children With Severe Inner Ear Malformations: Initial Surgical and Audiological Results.

Otol Neurotol 2020 Jun;41(5):625-630

House Clinic, Los Angeles, CA, USA.

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.
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http://dx.doi.org/10.1097/MAO.0000000000002595DOI Listing
June 2020

A Novel Classification: Anomalous Routes of the Facial Nerve in Relation to Inner Ear Malformations.

Laryngoscope 2020 11 5;130(11):E696-E703. Epub 2020 Mar 5.

Department of Otolaryngology, Ondokuz Mayıs University School of Medicine, Samsun, Turkey.

Objectives/hypothesis: The objective of this study was to classify anomalous facial nerve (FN) routes and to determine their association with inner ear malformations (IEMs).

Study Design: Retrospective cross sectional study.

Methods: The computed tomography images of 519 patients (796 ears) with IEMs were retrospectively evaluated, and the abnormal routes of the FN were classified as: Meatal segment: type 1, normal internal auditory canal (IAC); type 2, narrow IAC; type 3, facial canal (FC) only; type 4: separate FC/duplicated IAC. Labyrinthine segment (LS): type 1, normal; type 2a/b/c, mild/moderate/severe anterior displacement; type 3, superior displacement; type 4: straight LS. Tympanic segment (TS): type 1, normal; type 2, superiorly displaced TS; type 3, TS at the oval window; type 4: TS inferior to the oval window; type 5: unclassified. Mastoid segment: type 1, normal facial recess (FR)/normal mastoid segment; type 2: narrow FR; type 3, unclassified.

Results: In meatal segment classification, a narrow IAC was common in ears with cochlear hypoplasia (CH) (76.1%), and only FC was common in ears with severe IEMs (62.7%) such as Michel deformity, common cavity, and cochlear aplasia. Incomplete partition-III has its unique superiorly displaced LS (100%). CH-IV also has its unique mild anterosuperior displacement. Ears with a superiorly displaced TS usually (93.1%) had aplastic or hypoplastic semicircular canals. The FR is likely to be narrow in CH and severe IEMs.

Conclusions: The FN route is affected in IEMs, which must be kept in mind when operating on ears with IEMs. Especially in CH cases, all segments of the FN can be abnormal.

Level Of Evidence: 4 Laryngoscope, 130:E696-E703, 2020.
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http://dx.doi.org/10.1002/lary.28596DOI Listing
November 2020

Bimodal stimulation in children with inner ear malformation: One side cochlear implant and contralateral auditory brainstem implant.

Clin Otolaryngol 2020 03 22;45(2):231-238. Epub 2020 Jan 22.

Department of Otorhinolaryngology, Hacettepe University, Ankara, Turkey.

Objective: To determine audiological outcomes of children who use a cochlear implant (CI) in one ear and an auditory brainstem implant (ABI) in the contralateral ear.

Design: Retrospective case review.

Setting: Tertiary referral hospital.

Participants: Twelve children followed with CI and contralateral auditory brainstem implant (ABI) by Hacettepe University Department of Otorhinolaryngology and Audiology in Turkey. All children were diagnosed with different inner ear malformations with cochlear nerve aplasia/hypoplasia. CI was planned in the ear with better sound detection during behavioural testing with inserted ear phones and with better CN as seen on MRI. Due to the limited auditory and speech progress with the cochlear implant, ABI was performed on the contralateral ear in all subjects.

Main Outcome Measures: Audiological performance and auditory perception skills of children with cochlear nerve deficiency (CND) who use bimodal electrical stimulation with CI and contralateral ABI.

Results: Mean age of the subjects was 84.00 ± 33.94 months. Age at CI surgery and ABI surgery was 25.00 ± 10.98 months and 41.50 ± 16.14 months, respectively. However, hearing thresholds only with CI and only with ABI did not reveal significant difference, and auditory perception scores improved with bimodal stimulation. The MAIS scores were significantly improved from unilateral CI to bimodal stimulation (P = .002). Pattern perception and word recognition scores were significantly higher with the bimodal condition when compared to CI only and ABI only conditions.

Conclusion: Children with CND showed better performance with CI and contralateral ABI combined. Depending on the audiological and radiological results, bimodal stimulation should be advised for children with CND.
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http://dx.doi.org/10.1111/coa.13499DOI Listing
March 2020

Effects of Age at Auditory Brainstem Implantation: Impact on Auditory Perception, Language Development, Speech Intelligibility.

Otol Neurotol 2020 01;41(1):11-20

Otorhinolaryngology Department, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

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.
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http://dx.doi.org/10.1097/MAO.0000000000002455DOI Listing
January 2020

Is Early Cochlear Implant Device Activation Safe for All on-the-Ear and off-the-Ear Sound Processors?

Audiol Neurootol 2019 30;24(6):279-284. Epub 2019 Oct 30.

Department of Otorhinolarygology, Hacettepe University, Ankara, Turkey.

Background: Cochlear implantation (CI) is an effective treatment option for patients with severe-to-profound hearing loss. When CI first started, it was recommended to wait until at least 4 weeks after the CI surgery for the initial activation because of possible complications. Advances in the surgical techniques and experiences in fitting have made initial activation possible within 24 h.

Objectives: To compare the complaints and complications after early activation between behind-the-ear (BTE) and off-the-ear (OTE) sound processors and to show the impact of early activation on the electrode impedance values.

Method: CI surgeries performed between March 2013 and July 2018 were retrospectively analyzed from the database. In total, 294 CI users were included in the present study. The impedance measurements were analyzed postoperatively at the initial activation prior to the stimulation, and 4 weeks after the initial activation in the first-month follow-up visit. A customized questionnaire was administered in the first-month follow-up fitting session to caregivers and/or patients who were using CI at least for 6 months. Medical records were also reviewed to identify any postoperative complications.

Results: In the early activation group, impedance values were significantly lower than in the control group (p < 0.05) at first fitting. At the first-month follow-up, no significant difference was found between the groups (p > 0.05). The most common side effects were reported to be edema (6.1%) and pain (5.7%) in the early activation group. In patients with OTE sound processors, the rate of side effects such as skin infection, wound swelling, skin hyperemia, and pain was higher than in patients with BTE sound processors; however, a statistical significance was only observed in wound swelling (p = 0.005). Selecting the appropriate magnet was defined as a problem for the OTE sound processors during the initial activation.

Conclusion: This study revealed that early activation of CI was clinically safe and feasible in patients with BTE sound processors. When using OTE sound processors, the audiologists should be careful during the activation period and inform patients of possible side effects. The first fitting should be delayed for 4 weeks after CI for OTE sound processors. This current study is the first to report this finding with 5 years of experience in a large cohort.
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http://dx.doi.org/10.1159/000503378DOI Listing
June 2020

Wideband tympanometry findings in inner ear malformations.

Auris Nasus Larynx 2020 Apr 17;47(2):220-226. Epub 2019 Sep 17.

Hacettepe University, Medical Faculty, ENT Department, Ankara, Turkey.

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.
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http://dx.doi.org/10.1016/j.anl.2019.09.001DOI Listing
April 2020

Histopathology of Inner Ear Malformations: Potential Pitfalls for Cochlear Implantation.

Otol Neurotol 2019 09;40(8):e839-e846

Department of Otolaryngology, Head and Neck Surgery, University of Minnesota - Minneapolis, Minnesota, USA.

Hypothesis: The presence of bony inner ear malformations may associate with a number of anatomical abnormalities affecting the middle ear structures. Those malformations may create pitfalls and complications for cochlear implantation.

Background: Inner ear malformations associate with varying degrees of hearing loss, and frequently require cochlear implantation for hearing rehabilitation. Therefore, the abnormalities affecting the middle- and inner-ear structures may increase the risk of surgical complications.

Methods: We examined 38 human temporal bones from donors with bony inner ear malformations. Using light microscopy, we analyzed the presence of abnormalities in the structures of the middle- and inner-ear.

Results: Our collection comprises of 38 specimens with inner-ear malformations (cochlear aplasia, n = 3; cochlear hypoplasia, n = 30; incomplete partition, n = 3; isolated vestibular malformation, n = 2). The anatomy of the middle ear was abnormal in most temporal bones with cochlear aplasia, cochlear hypoplasia, and incomplete partition type I (40%-100%). Some of those abnormalities (hypoplastic or obliterated mastoid, 55.2%; aplastic or obliterated round window, 71.0%; aberrant course of the facial nerve, 36.8%) may hinder the access to the round window using the conventional facial recess approach for cochlear implantation. The cochlear nerve and associated bony structures (internal auditory canal and bony canal for cochlear nerve) were normal in 71.0% of all temporal bones with inner ear malformations.

Conclusion: Each different type of malformation may create specific surgical challenges to surgeons. Comprehensive preoperative imaging is fundamental toward the surgical success of cochlear implants in patients with malformations. Alternatives to circumvent those middle- and inner-ear abnormalities and potential complications are further discussed.
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http://dx.doi.org/10.1097/MAO.0000000000002356DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7377297PMC
September 2019

Cochlear Nerve Hypoplasia: Audiological Characteristics in Children and Adults.

Audiol Neurootol 2019 15;24(3):147-153. Epub 2019 Jul 15.

Department of Ear, Nose and Throat, Hacettepe University, Ankara, Turkey.

Background: Cochlear nerve deficiency is a general term used to describe both cochlear nerve hypoplasia (CNH) and cochlear nerve aplasia. Although these two conditions can have similar results on audiological evaluation, CNH yields more variation in audiological tests.

Objectives: To describe the audiological characteristics of the CNH cases in our series in relation to radiological findings.

Methods: We reviewed the medical charts, audiological findings, and radiological findings on cases with CNH. We included cases with CNH in one ear or both ears. Out of 90 subjects with CNH, we included a total of 40 individuals (21 women and 19 men; 49 ears) in the current study. We reviewed and analyzed the participants' audiological test results according to the radiological findings.

Results: Cases with CNH showed variations according to the cochlear structure. There were 13 normal cochleae, 4 with incomplete partition type I, and 32 with cochlear hypoplasia. The accompanying cochlear apertures also showed variation: 17 were normal, 28 stenotic, and 4 aplastic cochlear apertures. The subjects displayed hearing loss ranging from moderate to profound; furthermore, 4 subjects had no response to sound whatsoever. The degree of hearing loss was not statistically significantly different with regard to the presence or absence of cochlear malformation with CNH (p > 0.005). We observed both sensorineural hearing loss and mixed-type hearing loss among the CNH cases.

Conclusions: CNH is the presence of a cochlear nerve that is smaller in diameter than the facial nerve. It can be accompanied with other associated inner ear malformations of different degrees of severity. We observed degrees of hearing loss ranging from moderate to profound.
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http://dx.doi.org/10.1159/000500938DOI Listing
April 2020

Contralateral non-auditory stimulation in auditory brainstem implantation: A case report.

Int J Pediatr Otorhinolaryngol 2019 10 27;125:71-78. Epub 2019 Jun 27.

Department of Otolaryngology, Hacettepe University, Ankara, Turkey.

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http://dx.doi.org/10.1016/j.ijporl.2019.06.026DOI Listing
October 2019

The Effect of National Pneumococcal Vaccination Program on Incidence of Postmeningitis Sensorineural Hearing Loss and Current Treatment Modalities.

J Int Adv Otol 2018 Dec;14(3):443-446

Department of Otolaryngology, Hacettepe University School of Medicine, Ankara, Turkey.

Objectives: The aim of the present study was to investigate the effect of the national pneumococcal vaccination program on postmeningitis sensorineural hearing loss (SNHL).

Materials And Methods: Overall, 2751 patients (2615 cochlear implantation and 136 auditory brainstem implantation) who underwent cochlear implantation (CI) and auditory brainstem implantation (ABI) at a tertiary referral hospital otolaryngology clinic were retrospectively analyzed. One hundred sixteen patients with a history of meningitis were included in the study. Patients were evaluated for their age at the time of surgery, gender, computerized tomography (CT) and magnetic resonance imaging (MRI) findings, implant type, side, and incidence before and after the vaccination program.

Results: When patients with cochlear implants or ABI were examined, the incidence of meningitis-induced hearing loss was 6.2% in the pre-vaccination period and 0.6% in the post-vaccination period. There is a significant difference between them when compared by chi-square test (p<0.001).

Conclusion: The most important finding of the present study is the dramatic decrease in the number of CI and ABI surgeries performed in patients with SNHL due to meningitis. This shows the effectivity of pneumococcal vaccination in this special group of patients. If total ossification is detected on CT of patients with postmeningitis, ABI should be preferred to CI.
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http://dx.doi.org/10.5152/iao.2018.6169DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6354544PMC
December 2018

Traumatic Facial and Vestibulocochlear Nerve Injury in The Internal Acoustic Canal in The Absence of A Temporal Bone Fracture.

J Int Adv Otol 2018 Aug;14(2):330-333

Department of Otorhinolaryngology, Hacettepe University School of Medicine, Ankara, Turkey.

We present a rare case of traumatic facial and vestibulocochlear nerve injury in the internal acoustic canal in the absence of a temporal bone fracture. A 2.5-year-old female presented with sudden-onset left-sided facial paralysis and ipsilateral total hearing loss after being hit by a falling television. High-resolution computed tomography revealed an occipital fracture line that spared the temporal bone and otic capsule. Diagnostic auditory brainstem response testing showed that wave V at 90-db normal hearing level was absent in the left ear. Needle electromyography revealed severe axonal injury. Facial paralysis regressed to House-Brackmann grade IV 9 months after the trauma, and no surgical intervention was scheduled. Traumatic facial and vestibulocochlear nerve injury can occur in the absence of a temporal bone fracture. Thus, careful evaluation of the internal acoustic canal is mandatory if concurrent 7th and 8th cranial nerve paralyses exist with no visible fracture line.
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http://dx.doi.org/10.5152/iao.2018.4782DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6354463PMC
August 2018

The Association Between Modiolar Base Anomalies and Intraoperative Cerebrospinal Fluid Leakage in Patients With Incomplete Partition Type-II Anomaly: A Classification System and Presentation of 73 Cases.

Otol Neurotol 2018 08;39(7):e538-e542

Department of Otorhinolaryngology, Faculty of Medicine, Hacettepe University.

Objective: Modiolus and modiolar base abnormalities in patients with incomplete partition-II anomaly (IP-II) increase the risk of intraoperative cerebrospinal fluid (CSF) leakage. This study aimed to classify modiolar abnormalities and define objective radiological measures for preoperatively evaluating intraoperative CSF leakage risk.

Study Design: Retrospective case series.

Setting: Tertiary referral center.

Patients: The study included 73 patients with IP-II that underwent cochlear implant surgery between 2002 and 2017.

Intervention: Analysis of preoperative temporal bone computed tomography (CT) scans and surgical records.

Main Outcome Measures: Preoperative CT modiolar anomalies and intraoperative CSF leakage status.

Results: Among the 73 patients (41 men and 32 women), mean age at cochlear implant surgery was 11.4 (range, 0-42) years. Preoperative CT-based modiolar base anomaly classification was as follows: grade 1 (complete modiolus, n = 1), grade 2 (thin plate of bone in the modiolar base + partial modiolus, n = 14); grade 3 (thin plate of bone in the modiolar base, n = 53); grade 4 (total modiolar base defect, n = 5). The gusher rate was 8%. All patients with grade 4 anomaly had an intraoperative gusher. Patients with grade 3 anomaly accounted for 86% of oozing cases. Oozing, pulsation, and no CSF leakage rates were similar in those with grade 2 anomaly.

Conclusions: The modiolus and modiolar base must be carefully evaluated in patients with IP-II. Gushers primarily occur in IP-II patients with grade 4 anomaly. A thin plate of bone in the modiolar base most commonly prevents gushers.
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http://dx.doi.org/10.1097/MAO.0000000000001871DOI Listing
August 2018

Another evidence for pressure transfer mechanism in incomplete partition two anomaly via enlarged vestibular aqueduct.

Cochlear Implants Int 2018 11 3;19(6):355-357. Epub 2018 Jul 3.

a Department of Otolaryngology , Hacettepe University Medical Faculty , Ankara , Turkey.

A female patient with unilateral enlarged vestibular aqueduct (EVA) demonstrated scala vestibuli dilatation on that side while on the contralateral side both vestibular aqueduct and scala vestibuli were normal. This important radiological finding demonstrates that modiolar defects (hence 'cystic apex') observed in Incomplete partition-II is due to pressure transfer via EVA during embryological development. Therefore, it supports the previous histopathological ideas radiologically. Depending on the patency of EVA, variety of modiolar defects may arise.
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http://dx.doi.org/10.1080/14670100.2018.1489938DOI Listing
November 2018

Management of Cochlear Nerve Hypoplasia and Aplasia.

Adv Otorhinolaryngol 2018 6;81:81-92. Epub 2018 Apr 6.

Approximately 2% of congenital profound deafness cases are due to cochlear nerve (CN) deficiency. MRI is essential to confirm if the nerve is deficient, but because of limitations with resolution, especially when the internal auditory canal is narrowed, it is often unable to distinguish between hypoplasia and aplasia. A full audiometric test battery should always be performed, even if the MRI suggests CN aplasia, as there will sometimes be evidence of audition. Electrically evoked auditory brainstem response testing can be carried out transtympanically via the round window or using an intracochlear test electrode to help determine the status of the CN. If any test suggests the presence of a CN, then cochlear implantation (CI) should be considered. Children should be followed up closely with audiometric, electrophysiological and language assessments to determine the benefits. They may initially show benefit but fail to progress. CI results are variable and often result in poor outcomes with Categories of Auditory Perception scores of <5. Auditory brainstem implantation (ABI) can be considered when CI is contraindicated or fails to provide adequate benefit. This may provide better outcomes, but this form of surgery has greater risks and future device replacement (in case of device failure) may be complicated. Careful patient selection is required when considering ABI as significant learning difficulties make programming extremely challenging. Patients should be given the option of CI first and then ABI. A small minority of patients presenting late (around 2-3 years of age) may be candidates for simultaneous CI and ABI.
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http://dx.doi.org/10.1159/000485542DOI Listing
October 2018

Novel pathogenic variants underlie SLC26A4-related hearing loss in a multiethnic cohort.

Int J Pediatr Otorhinolaryngol 2017 Oct 8;101:167-171. Epub 2017 Aug 8.

John P. Hussman Institute for Human Genomics, University of Miami, Miller School of Medicine, Miami, FL, USA; Dr. John T. Macdonald Foundation, Department of Human Genetics, University of Miami, Miller School of Medicine, Miami, FL, USA; Department of Otorhinolaryngology, University of Miami, Miller School of Medicine, Miami, FL, USA. Electronic address:

Objectives: The genetics of sensorineural hearing loss is characterized by a high degree of heterogeneity. Despite this heterogeneity, DNA variants found within SLC26A4 have been reported to be the second most common contributor after those of GJB2 in many populations.

Methods: Whole exome sequencing and/or Sanger sequencing of SLC26A4 in 117 individuals with sensorineural hearing loss with or without inner ear anomalies but not with goiter from Turkey, Iran, and Mexico were performed.

Results: We identified 27 unique SLC26A4 variants in 31 probands. The variants c.1673A > G (p.N558S), c.1708-1G > A, c.1952C > T (p.P651L), and c.2090-1G > A have not been previously reported. The p.N558S variant was detected in two unrelated Mexican families.

Conclusion: A range of SLC26A4 variants without a common recurrent mutation underlies SLC26A4-related hearing loss in Turkey, Iran, and Mexico.
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http://dx.doi.org/10.1016/j.ijporl.2017.08.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5679420PMC
October 2017

Classification and Current Management of Inner Ear Malformations.

Balkan Med J 2017 Sep 25;34(5):397-411. Epub 2017 Aug 25.

Department of Otolaryngology, Hacettepe University School of Medicine, Ankara, Turkey.

Morphologically congenital sensorineural hearing loss can be investigated under two categories. The majority of congenital hearing loss causes (80%) are membranous malformations. Here, the pathology involves inner ear hair cells. There is no gross bony abnormality and, therefore, in these cases high-resolution computerized tomography and magnetic resonance imaging of the temporal bone reveal normal findings. The remaining 20% have various malformations involving the bony labyrinth and, therefore, can be radiologically demonstrated by computerized tomography and magnetic resonance imaging. The latter group involves surgical challenges as well as problems in decision-making. Some cases may be managed by a hearing aid, others need cochlear implantation, and some cases are candidates for an auditory brainstem implantation (ABI). During cochlear implantation, there may be facial nerve abnormalities, cerebrospinal fluid leakage, electrode misplacement or difficulty in finding the cochlea itself. During surgery for inner ear malformations, the surgeon must be ready to modify the surgical approach or choose special electrodes for surgery. In the present review article, inner ear malformations are classified according to the differences observed in the cochlea. Hearing and language outcomes after various implantation methods are closely related to the status of the cochlear nerve, and a practical classification of the cochlear nerve deficiency is also provided.
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http://dx.doi.org/10.4274/balkanmedj.2017.0367DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5635626PMC
September 2017

Audiological and Radiological Characteristics in Incomplete Partition Malformations.

J Int Adv Otol 2017 Aug;13(2):233-238

Department of Audiology, Hacettepe University School of Medicine, Ankara, Turkey.

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.
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http://dx.doi.org/10.5152/iao.2017.3030DOI Listing
August 2017

The role of eABR with intracochlear test electrode in decision making between cochlear and brainstem implants: preliminary results.

Eur Arch Otorhinolaryngol 2017 Sep 19;274(9):3315-3326. Epub 2017 Jun 19.

Department of Otolaryngology, Head and Neck Surgery, Hacettepe University, Ankara, Turkey.

The objective of the study was to discuss the findings of intraoperative electrically evoked auditory brainstem response (eABR) test results with a recently designed intracochlear test electrode (ITE) in terms of their relation to decisions of cochlear or auditory brainstem implantation. This clinical study was conducted in Hacettepe University, Department of Otolaryngology, Head and Neck Surgery and Department of Audiology. Subjects were selected from inner ear malformation (IEM) database. Eleven subjects with profound sensorineural hearing loss were included in the current study with age range from 1 year 3 months to 4 years 3 months for children with prelingual hearing loss. There was only one 42-year-old post-lingual subject. eABR was recorded with an ITE and intraoperatively with an original cochlear implant (CI) electrode in 11 cases with different IEMs. Findings of eABR with ITE and their relation to the decision for CI or auditory brainstem implant (ABI) are discussed. Positive eABR test results were found to be dependent on close to normal cochlear structures and auditory nerve. The probability of positive result decreases with increasing degree of malformation severity. The prediction value of eABR via ITE on decision for hearing restoration was found to be questionable in this study. The results of eABR with ITE have predictive value on what we will get with the actual CI electrode. ITE appears to stimulate the cochlea like an actual CI. If the eABR is positive, the results are reliable. However, if eABR is negative, the results should be evaluated with preoperative audiological testing and MRI findings.
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http://dx.doi.org/10.1007/s00405-017-4643-3DOI Listing
September 2017

Accuracy of turbo spin-echo diffusion-weighted imaging signal intensity measurements for the diagnosis of cholesteatoma.

Diagn Interv Radiol 2017 Jul-Aug;23(4):300-306

Department of Radiology, Hacettepe University School of Medicine, Ankara, Turkey.

Purpose: We aimed to evaluate the diagnostic accuracy of turbo spin-echo diffusion-weighted imaging (TSE-DWI) at 3 T, for cholesteatoma (CS) diagnosis, using qualitative and quantitative methods with numerical assessment of signal intensity (SI), signal intensity ratios (SIR), and apparent diffusion coefficient (ADC) values.

Methods: In this retrospective study, two blinded observers independently evaluated the preoperative TSE-DWI images of 57 patients who were imaged with a presumed diagnosis of CS. Qualitative assessment with respect to the SI of the adjacent cortex and quantitative measurements of SI, SIR, and ADC values were performed.

Results: Surgery with histopathologic examination revealed 30 CS patients and 27 patients with non-cholesteatoma (NCS) lesions including chronic inflammation and cholesterol granuloma. On TSE-DWI, 96.7% of the CS lesions and none of the NCS lesions appeared hyperintense compared with the cortex. The mean SI and SIR indices of the CS group were significantly higher and the mean ADC values significantly lower compared with those of the NCS group (P < 0.001). Using specific cutoff values for SI (92.5) and SIR (0.9), CS could be diagnosed with 100% sensitivity and specificity. The use of quantitative imaging further increased the sensitivity of the TSE-DWI technique.

Conclusion: The quantitative indices of SI, SIR, and ADC of TSE-DWI appear to be highly accurate parameters that can be used to confirm the diagnosis of CS.
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http://dx.doi.org/10.5152/dir.2017.16024DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5508954PMC
February 2018

Incomplete partition type III: A rare and difficult cochlear implant surgical indication.

Auris Nasus Larynx 2018 Feb 16;45(1):26-32. Epub 2017 Mar 16.

Department of Otolaryngology, Head and Neck Surgery, Hacettepe University, Faculty of Medicine, Turkey. Electronic address:

Objective: Presenting the clinical features and treatment options for incomplete partition type-III.

Methods: Nine primary and 1 revision incomplete partition type-III cochlear implant cases treated between 2004 and 2015 in Hacettepe University Department of Otolaryngology were included in the study. Treatment options and particularly cochlear implantation tecnique were described.

Results: Nine primary and 1 revison cases were all succesfully implanted. Eight cases were standart cases with no secondary intervention. Case #9 has to be revised intraoperatively and case #10 were operated four times in another center and revised in our department.

Conclusion: Incomplete partition type-III is one of the rarest inner ear anomaly and the rarest among incomplete partition group. Treatment options may differ depending on the hearing loss level of the patient. Stapes surgery should be avoided because it will lead to gusher and further hearing loss. Preoperative imaging is mandatory in order to avoid unnecessary stapes surgery. Incochlear implantation surgery a gusher and misplacement into the IAC may complicate the surgery. Gusher should be controlled intraoperatively and the position of the electrode should be controlled via intraoperative imaging.
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http://dx.doi.org/10.1016/j.anl.2017.02.006DOI Listing
February 2018