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Objective: The aim of this study is to describe the rehabilitative outcomes of pediatric auditory brainstem implant (ABI) users in the Department of Otolaryngology in the Hacettepe University. It was a retrospective study, and all patients' files were reviewed., Materials and Methods: The data was collected from 41 children who were fitted with ABI between 2005 and 2013. Inclusion criteria for children in our study are profound, congenital bilateral sensory-neural hearing loss with anomalies (such as cochlear, labyrinthine, and cochlear nerve aplasia) and more than one year of auditory experience with ABI. Post-meningitis patients and neurofibromatosis type 2 (NF2) patients were excluded. Auditory perception was evaluated using the Meaningful Auditory Integration Scale (MAIS), Functioning after Pediatric Cochlear Implantation (FAPCI) instrument, Categories of Auditory Performance (CAP), and Children's Auditory Perception Skills Test in Turkish (CIAT). Speech intelligibility was categorized with speech intelligibility rating (SIR), and language development was assessed using the Test of Early Language Development-Third Edition (TELD-3) and Manchester Spoken Language Development Scale (MSLD)., Results: All patients gained basic audiological functions and were able to recognize and discriminate sounds by the third month of ABI surgery. According to the duration of ABI use and learning skills, patients revealed development from word identification to sentence recognition level in a wide spectrum., Conclusion: Preliminary results indicate that all children have gained basic auditory perception skills. On the other hand, language and speech development data were varying among children. Additional handicaps seemed to slow down progression. Secondary improvement was seen at psychosocial areas with respect to behavioral and social adjustment as well as eagerness to start communication.

Objective: Gusher in inner ear malformations is common in patients with incomplete partition type I and type III. It is also common in less severe form as oozing in incomplete partition type II and large vestibular aqueduct. It is important to prevent cerebrospinal fluid (CSF) escape around the electrode to prevent meningitis., Methods: The custom-made device was produced by Med-El Company. It has a "cork"-like stopper instead of the usual silicon ring to prevent gusher. There are two types of electrodes of different lengths. The standard one is 25mm (contact space 1.7mm) and the short one is 20mm (contact space 1.3mm). It was used in 50 patients with different inner ear malformations., Results: Thirteen patients had gusher, and 11 patients oozing during cochleostomy. One patient with initial prototype of the cork electrode had to be revised because of persistent oozing around the electrode. Another patient had slow extrusion of the electrode most probably due to CSF pulsation and had to be revised. Both patients had no more CSF fistula., Conclusion: CSF fistula in inner ear malformations is a serious situation which may lead to recurrent meningitis. The new electrode with "cork" stopper looks promising in preventing the postoperative CSF leak around the electrode., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

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.)

Objectives: This study aims to investigate the incidence of otitis media with effusion and to report long-term clinical findings in children with cleft lip and palate types., Patients and Methods: Between March 2000 - October 2010, medical data of 175 patients (84 females, 95 males; mean age 8 years; range, 4 to 20 years) who were operated due to otitis media with effusion with long-term follow-up data were retrospectively analyzed., Results: A total of 105 patients had a complete cleft lip and palate. In the first examination, 134 patients had ear effusion, the eustachian tube were not patent in 146 patients, and middle ear pressure was measured as -200 and below in 121 patients. After the operations, the eardrums had ear effusion in 89 patients, in 93 patients middle ear pressure was lower than -200, and 11 patients had chronic otitis media in the final examination., Conclusion: Our study results suggested that there was a statistically significant relationship among the middle ear disease, function of eustachian tube, treatment outcomes, middle ear pressure, cleft palate type and bilaterality of the disease. We believe that it is of utmost importance as it may affect the treatment process and the patient follow-up in such patients.

Objective: Pneumolabyrinth resulting from temporal bone trauma and stapes luxation has been associated with sensorineural hearing loss (SNHL). The principal purpose of this study was to determine the incidence and volume of pneumolabyrinth after stapedotomy in which iatrogenic perilymphatic fistula is created and to also correlate this with possible hearing loss and vertigo., Study Design: Prospective study., Setting: Tertiary referral center., Patients and Methods: Fifty stapedotomy patients were operated on for otosclerosis, and of those 50, 20 underwent high-resolution computed tomography (CT) on the first day, 10 on the third day, and 20 on the seventh day. The patients followed up regarding SNHL and vertigo that could develop postoperatively, and the correlation of such complications with HRCT findings was examined., Results: The 20 patients who had high-resolution CT (HRCT) on the first day all presented with pneumolabyrinth, and none of the 20 patients who underwent HRCT on the seventh day had pneumolabyrinth. Postoperatively, 92% of the patients had less than 20 dB and 62% had less than 10 dB air-bone gap. None of the patients had SNHL or persistent vertigo. There was no correlation between pneumolabyrinth and hearing loss or vertigo., Conclusion: Pneumolabyrinth is a radiological sign of perilymphatic fistula and has no effect on sensorineural hearing loss and vertigo. Observing pneumolabyrinth during the early postoperative stage should not necessarily implicate a complication; however, pneumolabyrinth after the first week supported with the clinical symptoms of perilymphatic fistula would be a meaningful finding.

We present a 4-year-old girl with congenital profound sensorineural deafness associated with inner ear malformation (incomplete partition type II, enlarged vestibule, and enlarged vestibular aqueduct). The proposita also had pseudocleft lips, skin defects, auricle abnormalities, and unilateral multicystic dysplastic kidney, leading to the diagnosis of branchio-oculo-facial (BOF) syndrome. Mutation analysis of the TFAP2A gene showed a de novo deletion of 18 and insertion of 6 nucleotides, resulting in deletion of amino acids LPGARR and insertion of RI between amino acids 276 and 281. Altered amino acids are located within the basic DNA binding and dimerization domains of TFAP2A. Previously reported amino acid substitutions in TFAP2A involved only DNA binding domain in four patients with BOF syndrome who were not reported to have profound sensorineural deafness. Our report implies that the localization of mutations in TFAP2A might be responsible with the phenotypic findings in BOF syndrome., (2009 Wiley-Liss, Inc.)

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.

Patients with diffuse obliterative otosclerosis have more extensive footplate pathology than annular cases. As a result of this more skill is required for diffuse otosclerosis cases, and postoperative hearing results are usually worse than annular cases. In this retrospective study we compared the preoperative audiological features of annular and diffuse otosclerosis patients. The subjects were 60 patients with conductive hearing loss who had undergone stapedectomy. Annular and diffuse groups were comprised of 30 patients each. Annular otosclerosis was defined as the footplate pathology involving the annular ligament only, where the footplate of the stapes is very thin and retains its bluish color. On the other hand diffuse, obliterative otosclerosis was defined as the pathology involving the whole footplate and also in some cases extending beyond the confines of the annular ligament. In each group preoperative air- and bone-conduction levels at 125-6000 Hz and 500-4000 Hz were noted respectively. Average air-bone gap for the obliterative otosclerosis group was 37.5 dB; the same value for the annular group was 23.8 dB (p<0.05). The gap characteristics of the audiogram were different for the two groups. The annular group had an air-bone gap which was nearly constant for all the frequencies. In the diffuse otosclerosis group, the air-bone gap was more prominent in the low frequencies and it decreased at higher frequencies. No difference was noted in bone-conduction thresholds, and Carhart notch between the two groups. This study demonstrated that a large air-bone gap in patients with conductive hearing loss may be a sign of diffuse obliterative otosclerosis. This may warn the surgeon that a more challenging surgery is possible, and the patient may have a less favorable hearing result. Therefore, in the presence of a large air-bone gap, it may be appropriate to inform the patient of the strong possibility of diffuse otosclerosis.

Children with symptoms of profound biotinidase deficiency with null mutations are more likely to have hearing loss develop than those with missense mutations, even if not treated for a period of time. Hearing loss appears to be preventable in children with null mutations if treatment is initiated soon after birth.

Background and Aim: Inner ear anomalies have been reported in approximately 30% of children with early onset deafness. Identification of causative genetic factors in a large proportion of these patients was not successful. Mutations in the SLC26A4 gene have been detected in individuals with enlarged vestibular aqueduct (EVA) or Mondini dysplasia. We aimed to characterize the inner ear anomalies associated with SLC26A4 mutations., Methods: The SLC26A4 gene has been screened for mutations in 16 subjects from 14 unrelated Turkish families with a variety of inner ear anomalies ranging from Michel aplasia to incomplete partition-II and EVA. None of the patients was diagnosed to have a recognizable genetic syndrome. Additional four patients with Pendred syndrome from three families were included., Results: Only one patient with EVA was found to have a heterozygous mutation (c.1586delT) in SLC26A4. All patients with Pendred syndrome had homozygous mutations and were noted to have either EVA or EVA associated with incomplete partition-II on the computed tomography of the temporal bone., Conclusion: SLC26A4 mutations are not associated with a large spectrum of inner ear anomalies. They, instead, result in a specific morphological appearance consistent with EVA or incomplete partition-II.

Tumors arising from the ceruminous glands are very rare. Herein, we report a case of ceruminous adenoma mimicking furunculosis in the external auditory canal (EAC), along with a discussion of the various pathological types and a review of their management.

Objectives: This study was designed to evaluate the relationship between swimming and benign paroxysmal positional vertigo (BPPV)., Patients and Methods: This prospective study was comprised of 30 subjects (15 females, 15 males, mean age 15.6 years; range 11 to 23 years) who had been swimming regularly for at least two years. Twenty subjects (9 females, 11 males; mean age 15.4 years; range 11 to 21 years) with no vertigo and ear complaints comprised the control group. The Dix-Hallpike maneuver was used in all the swimmers and controls to diagnose BPPV., Results: Four swimmers (13%) exhibited characteristic findings of BPPV, being unilateral in three, and bilateral in one. Interestingly, no swimmer experienced vertigo during swimming. The characteristics of nystagmus were typical of posterior semicircular canal BPPV in all cases. None of the subjects in the control group exhibited symptoms or findings of BPPV. Patients with BPPV underwent the Epley maneuver for therapy. All were free of vertigo after one month. There was no relationship between swimming and BPPV with respect to the frequency and duration of swimming., Conclusion: Swimming may be one of the etiological factors of BPPV. It appears that rapid head movements during swimming cause otoconia to be dislodged from the macula and enter the semicircular canals.

Objective: The pathogenesis of otitic hydrocephalus remains unclear. The use of radiological examination is very important to clarify both the diagnosis and the pathogenesis. In this article the role of magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) is discussed., Patients: Two patients with the diagnosis of otitic hydrocephalus and their MRI and MRV findings are presented., Results: Both of the patients presented had thrombus in their transverse sinuses pre-operatively demonstrated by MRI causing decreased flow on MRV. After the treatment blood flow through the sinus increased significantly as the symptoms subsided., Conclusion: MRI and MRV are valuable tools for diagnosis and follow-up of otitic hydrocephalus.

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".

Purpose: To evaluate the contribution of cervical spine radiograms, Doppler US, cervical and cranial MRI, MRA and two dimensional quantification flow in the diagnosis of vertebrobasilar insufficiency., Materials and Methods: We prospectively examined 25 patients having signs of vertebrobasilar insufficiency (16 females and 9 males, aged 34 to 74 years) and 25 controls of the same age and sex with MRI, MRA, Doppler US and cervical radiograms. Cervical spine radiograms were evaluated for the presence of degenerative changes, osteophytes and loss of height. The flow velocities of the basilar artery and vertebral arteries were measured with two dimensional quantification flow, using time-velocity graphics achieved by means of a region of interest drawn on transverse sections of the vessels. Posterior circulation in farcts and slow flow in vertebrobasilar system arteries were investigated on brain MR images and the presence of cervical osteophytes, spinal cord compression and the effect of the presence of degenerative changes on vertebral arteries were evaluated on cervical MR images. Paired samples T-test, independent samples T-test, Wilcoxon W, Mann-Whitney U and chi-square tests were used for statistical analysis. The significance level was taken at p below 0.05., Results: From the statistical analysis, we found that there were more pathologic findings on MRA within the patient group compared with the controls (p = 0.002). The velocities measured by MRI were significantly affected by the presence of unknown bright objects on brain MR images, osteophytes on cervical MR images, loss of height and the presence of osteophytes on radiograms in patients having VBI signs, when compared to the controls. In both groups, the measurements of flow velocity in vertebral arteries by Doppler US did not correlate with the measurements by two dimensional quantification flow., Conclusion: Among the methods we used in our study, MRA was the most specific in the diagnosis of patients with vertebrobasilar insufficiency. However, two dimensional quantification flow can be used as a noninvasive but indirect method to investigate the influence of several factors on the hemodynamics.

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.

Purpose: The "adequate" therapy of tongue cancer has not yet been determined. The authors report their experience with 58 N(0) patients to elucidate the role of elective neck dissection in surgical treatment of cancer of the tongue., Materials and Methods: The files of 58 N(0) patients with tongue cancer were evaluated retrospectively. In every patient, partial glossectomy continuous with neck dissection was the mainstay of the treatment. TNM staging, intraoperative N staging, pathologically confirmed cervical lymph node metastases and their levels, and clinical outcomes (local and regional recurrences) were recorded. The sensitivity and specificity of intraoperative staging was determined., Results: Fifty-four percent (31/58) of the patients presented as T(1), and 26% (15/58) as T(2). The overall occult metastasis rate was 29.3% (17/58). The occult metastasis rate for T(1) and T(2) lesions was 19.4% (6/31) and 26.7% (4/15), respectively. The sensitivity of intraoperative staging was 76.5%, and the specificity was 51.2%., Conclusions: The rate of occult metastasis to the neck is too high in all tongue cancer cases to take the risk of regional recurrence, and the surgeon can not solely depend on neck palpation for determination of neck metastasis. Radiologic investigations and fine-needle aspiration decrease, but never reduce to zero the rate of false-negative examination. There is an obvious indication for neck dissection, even in early cases.

Aneurysmal bone cyst is a cystic vascular osseous tumor. It is rare in the craniofacial area; it is even rarer in the zygoma. A case of aneurysmal bone cyst of the zygoma was presented. Its pathogenesis, clinical and radiological features and treatment options were discussed under the view of the literature.

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.

A 17-year-old male patient was admitted because of progressive hearing loss since the age of six. His former blood and radiology investigation had revealed idiopathic hyperphosphatasia. On ENT examination bilateral thickened tympanic membranes with severe mixed-type hearing loss was diagnosed. Computerized tomography (CT) demonstrated expansion of the calvarial bones, including the temporal bones, except for the otic capsule. Middle-ear exploration revealed thickened middle-ear mucosa and a stone hard, immobile bony mass instead of the normal ossicular chain at the posterior superior part of the mesotympanum. No ossicular reconstruction could be attempted and the patient was rehabilitated with a hearing aid.

The pathogenesis of otitic hydrocephalus still remains doubtful. Magnetic resonance imaging (MRI) demonstrated sigmoid and transverse sinus thrombosis in two consecutive patients. The role of MRI in the diagnosis and pathogenesis of this complication, together with literature findings, is discussed. Until now, lumbar puncture, which is a minimally invasive technique, was the only diagnostic measure. MRI, however, can be used to establish the diagnosis of otitic hydrocephalus by showing the thrombosis in the sigmoid and transverse sinus.

Neoplasms of the trachea are rare. The fifth case of malignant fibrous histiocytoma that nearly obliterated the lumen is reported. It is one of the most frequent soft tissue sarcomas particularly in the extremities. It is very rare in the trachea. Treatment is mainly based on local resection. Postoperative radiotherapy seems of little value.

Cervicomediastinal abscess developed in a 14-year-old girl after temporomandibular joint surgery. No such complication could be found in the literature. The complications of temporomandibular joint surgery and the possible ways this abscess may have developed are discussed.

Neutrophil random motility and chemotactic responsiveness were determined in 17 healthy adults and 19 adult patients with chronic tonsillitis before and 10 days after tonsillectomy, using a modified Boyden chamber method. The mean chemotactic index values of the healthy controls were significantly higher when compared with the chronic tonsillitis patients before tonsillectomy. The postoperative mean chemotactic values were increased, and there was a statistically significant difference between the mean chemotactic index of neutrophils in preoperative and postoperative patients. The authors conclude that this change in host defense mechanism may be the reason for frequent attacks of acute tonsillitis in these patients.