Potential diagnostic and prognostic biomarkers of pediatric Burkitt lymphoma identified through miRNA expression profiling.

BACKGROUND: Pediatric Burkitt lymphoma (pBL) is the most common non-Hodgkin lymphoma in children. These patients require prompt diagnosis and initiation of therapy due to rapid tumor growth. The roles of tumor tissue and circulating microRNAs (miRNAs) in the diagnosis or prognostication have not been fully elucidated in pBLs. METHODS: Differentially expressed (DE) miRNAs were identified with microRNA sequencing (miRNA-Seq) in tumor tissues and plasma of diagnostic pBLs. The diagnostic potential of total miRNA concentrations and overexpressed miRNAs were evaluated through receiver operating characteristic (ROC) analyses. Log-rank test was employed to evaluate survival differences associated with DE miRNAs. Selected miRNA expressions were cross-validated with quantitative reverse transcription PCR (qRT-PCR). RESULTS: Total circulating cell-free miRNAs were higher in pBL cases compared to controls. Cancer-associated pathways were enriched among miRNAs differentially expressed in pBL tumor tissues. Several upregulated miRNAs in pBL tumors demonstrated high diagnostic potential. Similarly, ROC analysis of overexpressed plasma miRNAs revealed circulating cell-free or exosomal miRNAs that can distinguish pBLs from control cases. Indeed, integrative analysis of overexpressed circulating exosomal miRNAs showed an enhanced diagnostic potential for certain triple combinations. Kaplan-Meier analyses of DE miRNAs in tumor tissues identified miRNAs predicting overall survival. CONCLUSIONS: Differentially expressed miRNAs in tumor tissue and plasma of pBL have the potential to improve diagnosis and prognosis. IMPACT: Differentially expressed miRNAs in treatment-naive pediatric Burkitt lymphoma cases have diagnostic or prognostic biomarker potential. This is the first study that applied miRNA-Seq on treatment-naive pediatric Burkitt lymphoma cases for identification of differentially expressed miRNAs both in tumor tissue and plasma samples with diagnostic potential. Through systematic analysis of differentially expressed miRNAs, tumor tissue miRNAs associated with the overall survival of pBLs have been discovered. The clinically significant, differentially expressed miRNAs identified in pediatric Burkitt lymphoma cases can potentially improve the current tissue-based or non-invasive clinical practice in terms of diagnosis or prognostication.

Luc abscess: an extraordinary complication of acute otitis media.

Luc abscess is an uncommon suppurative complication of otitis media. Unfamiliarity of this complication leads to delayed diagnosis and treatment. This abscess is usually benign. Infection in the middle ear spreads via anatomic preexisting pathways, and this process results with subperiosteal pus collection. Conservative treatment with drainage under empirical wide spectrum antibiotic is efficient. Here,we present a 9-year-old boy who had left facial swelling after a period of otalgia, diagnosed as Luc abscess without mastoiditis.

[Analysis and publication rate of the presentations at the Turkish National Otorhinolaryngology and Head and Neck Surgery meetings]. / Türk Ulusal Kulak Burun Bogaz ve Bas Boyun Cerrahisi Kongre bildirilerinin analizi ve yayimlanma oranlari

OBJECTIVES: This study aims to evaluate the analysis and publication rates of presentations presented at the Turkish National Otorhinolaryngology and Head & Neck Surgery (TNORL and HNS) meetings. MATERIALS AND METHODS: The TNORL and HNS meetings of 2008, 2009 and 2010 were included in the study. The number, subjects, types (clinical study, experimental study, case report) and institutions of the oral presentations and posters were documented using the abstract CD's of the meetings. The conversion rate of presentations into the full-text articles in the peer-reviewed journals were reviewed through the search engines Pubmed for the international indexes and Türk Medline and Ulakbim for the national ones. The time from presentation in the meetings to publication was determined. The distribution of journals according to the publication dates of the articles were evaluated in terms of the Science citation index (SCI), SCI expanded (SCI-E), PubMed and Turkish citation index. RESULTS: The total number of presentations submitted in the three TNORL and HNS meetings was 1,454 and posters accounted for 75.4% of all presentations. While case reports were 53.2% of the total presentations, the ratios were found to be 43% and 3.8% for the research and experimental studies, respectively. Of the oral presentations, 88% included research studies, whereas 70.3% of the posters were case reports. The origin of the presentations was university hospitals, education hospitals, other national institutions, and international institutions with ratios of 51.6%, 44.3%, 3% and 1.1%, respectively. The conversion rate of presentations into the full-text articles was found as 21.9%. The rate was 37.3% for oral presentations and 17% for the posters (p=0.00). For all of the 319 published papers, the overall mean time from presentation to publication was 18.6 months. While 62.7% of the articles were published in international journals, 37.3% were published in national journals. The conversion rate of oral presentations into publications was higher than the posters (p=0.00). CONCLUSION: The first study related to TNORL and HNS meetings in Turkey revealed that, although the quantity of presentations was high, the rate of conversion into the full-text journal articles was lower compared to the similar international annual meetings held by otorhinolaryngology or other disciplines. The quality and success of our scientific meetings can be enhanced with some particular precautions.

[Primary intraosseous squamous cell carcinoma of the mandible]. / Alt çenenin primer intraosseöz yassi hücreli karsinomu.

Primary intraosseous squamous cell carcinoma is an extremely rare tumor of the mandible. The diagnosis is based on the evidences showing that the tumor is neither originated from a distant metastasis of another primary tumor nor an invasive oral cancer. In this article, we report a 59-year-old female case with a medical history of ductal carcinoma of the breast, lichen planus, and rheumatoid arthritis who was admitted with complaints of a painful and swollen left lower jaw, and was surgically treated for primary intraosseous carcinoma of the mandible and with postoperative adjuvant radiotherapy.

Epistaxis management: is medical intervention required for inactive epistaxis?

OBJECTIVES: This study aims to discuss the management and the follow-up approach in patients with epistaxis. PATIENTS AND METHODS: A total of 367 patients with epistaxis (209 males, 158 females; mean age 52.6±18.3 years; range 18 to 85 years) admitted to the Adult Emergency Department of a university hospital between January 2000 and December 2004 were retrospectively analyzed. RESULTS: Of patients, 56.7% had an idiopathic bleeding. A significantly higher number of patients aged >50 years had high blood pressure on admission. Of 141 patients (38.49%) presenting without bleeding on admission, 20 required medical intervention for recurrent epistaxis. Conservative approaches were effective in stopping bleeding in 97.8% patients. The hospitalization ratio was 5.7%. CONCLUSION: Our study result show that endonasal endoscopic mucosal cauterization is an effective method for resistant-to-treatment cases and inactive bleeding on admission is not a restraint for further examination.

The effect of percutaneous and transcutaneous BAHA on hearing and subjective auditory abilities: A comparative study.

Hearing loss significantly affects communication, social interactions, and the overall quality of life. The bone-anchored hearing aid (BAHA) is an implantable system that bypasses the outer and middle ear to directly stimulate the cochlea through bone conduction. This study aimed to compare hearing performance and subjective auditory ability improvements between transcutaneous and percutaneous BAHA devices using audiological assessments and Speech, Spatial and Qualities of Hearing Scale. This cross-sectional prospective study included 29 participants aged 14 to 69 years who had used BAHA for at least 6 months. Both Cochlear Baha System's percutaneous (connect) and transcutaneous (attract) implants were evaluated. Audiological assessments involved pure-tone audiometry, speech recognition threshold, and free-field (FF) audiometry, while subjective auditory ability was measured using the Turkish Speech, Spatial and Qualities of Hearing scale (Tr-SSQ). Significant improvements in FF audiometry averages and speech recognition thresholds were observed with BAHA compared to without BAHA (P < .001). Both implant types provided similar FF averages, speech audiometry results, and Tr-SSQ outcomes, with no significant differences between them. Tr-SSQ scores showed substantial satisfaction, indicating significant improvements in speech perception, spatial perception, and hearing quality with BAHA (P < .001). The findings align with previous research, demonstrating that BAHA is a reliable and effective solution for hearing rehabilitation. The study also emphasized the importance of using both audiological test results and daily hearing function scales to comprehensively evaluate the benefits of hearing rehabilitation in real-world environments. In conclusion, BAHA, regardless of the implant type, can provide predictable and lasting improvements in hearing thresholds and daily hearing abilities, making it a valuable option for patients with conductive hearing loss.

Intramasseteric solitary fibrous tumor.

Solitary fibrous tumor (SFT) is a rare spindle cell neoplasm that usually arises from the pleura. SFTs occurring within the head and neck region are uncommon. Recently, it has been described in various head and neck sites such as oral cavity, nasal cavity, paranasal sinuses, salivary glands, thyroid, buccal space, and larynx. Here, we report a case of SFT originating in the masseter muscle of a 27-year-old woman. To our knowledge, this is the first description of a SFT of the head and neck region, arising within the masseter muscle. We present the clinical history, radiologic and histopathologic findings as well as immunoreactivity of this tumor.

[Publication rates of presentations which were presented at Turkish National Rhinology Congresses]. / Türk Ulusal Rinoloji Kongrelerinde sunulan bildirilerin yayinlanma oranlari

OBJECTIVES: This study aims to determine the publication rates of oral presentations and posters presented at Turkish National Rhinology Congresses in the medical journals and to make a comparison with related literature data. MATERIALS AND METHODS: The name and surname of the first authors and the title of presentation presented at 2007, 2008, 2009 and 2010 were searched using PubMed, Turkmedline and Ulakbim national search engines. RESULTS: Of the 218 papers presented, 61 (28%) were published. The mean time from presentation at congress to publication was 21.8 months. The mean time to publication for oral presentations was 23.2 months, while it was 20.8 months for poster presentations. The publication rates of oral and poster presentations were 35.8% and 24.5%, respectively, indicating no statistically significant difference between two groups (p=0.08). The publication rate of research articles was statistically significantly higher than that of case reports (20.7% vs. 33.3%; p=0.04). CONCLUSION: The publication rate of presentations presented at four Turkish National Rhinology Congresses is lower compared to the previously reported publication rates in otorhinolaryngology. Giving wider publicity to research presentations and being more selective for case reports will provide inclusion of more qualified papers with high possibility of publication and also enhance the scientific value of such congresses.

[Quantitative and qualitative analysis of four national otorhinolaryngology journals between 2002 and 2010]. / Dört ulusal KBB dergisinin 2002/2010 yillari arasindaki niceliksel ve niteliksel analizi.

OBJECTIVES: This study aims to perform a quantitative and qualitative analysis of four national peer-reviewed otorhinolaryngology journals between 2002 and 2010 and compare various parameters in 1990-1994 period. MATERIALS AND METHODS: The number of authors and female authors, gender of first author, city and the institution where the article was submitted, number of references and national references were noted separately for each article in all issues of four national peer-reviewed journals in years 2002, 2005 and 2010. Language of articles was noted and they were grouped under six main headings based on their subjects. Quantitative analysis was performed considering evidence-based medicine principle and evidence levels of articles were noted between 1 and 5. Statistical analysis was performed using Pearson chi-square and one-way ANOVA tests. RESULTS: A total of 424 articles including 143 in 2002, 147 in 2005 and 134 in 2010 in four national otorhinolaryngology journals were evaluated. The number of authors per article was found to be 4.49, indicating no statistically significant difference between the years (p>0.05). The mean number of female authors per article was 0.85. When the institutions submitted articles were assessed, number of publications from university hospitals was higher than the education hospitals and other health care services; however, this difference was reduced in 2010. In the evaluation of cited references, the mean number of references and national references per article increased from 16.90 to 18.12 and from 1.54 to 1.68 in 2002 and 2010, respectively. According to the articles categorized to their main subjects, it was found that most of the publications were related to upper respiratory/digestive tract and neck and the least was related to facial plastic surgery. The qualitative analysis in terms of evidence-based medicine revealed no articles with level 1 evidence through three years studied. CONCLUSION: It will be useful to make similar periodical studies to improve the quality of otorhinolaryngology journals and related articles in Turkey.

Exploring the immunological basis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: immunohistochemical staining features of palatine tonsils.

OBJECTIVES: Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common periodic fever syndrome during early childhood period with regular febrile attacks of sterile upper airway inflammation. The cessation of attacks following tonsillectomy points to fundamental role of tonsil tissue on etiopathogenesis of disease, which is not clarified satisfactorily. The aim of this study is to explore the immunological basis of PFAPA by evaluating the cellular properties of tonsils, and microbial exposition such as Helicobacter pylori on tonsillectomy materials. METHODS: The paraffinized tonsil samples of 26 PFAPA and 29 control patients with obstructive upper airway disorder were compared in terms of immunohistochemical staining features including CD4, CD8, CD123, CD1a, CD20, and H. pylori. RESULTS: The median number of CD8+ cells was 1485 (1218-1287) in PFAPA while it was 1003 (852-1261.5) in control group and the difference was statistically significant (p=0.001). Similarly, CD4+ cell counts were statistically higher in PFAPA group than control (833.5 vs 622). The ratio of CD4/CD8 did not differ between two groups; also, there was no statistically difference in terms of the other immunohistochemical staining results, such as CD20, CD1a, CD123 and H. pylori. CONCLUSION: This is the largest number of pediatric tonsillar tissue study of PFAPA patients in current literature and we emphasized the triggering effects of CD8+ and CD4+ T-cells on PFAPA tonsils. KEY POINTS: • The cessation of attacks following tonsillectomy points to fundamental role of tonsil tissue on etiopathogenesis of disease, which is not clarified satisfactorily. • In current study, 92.3% of our patients did not experience any attacks following operation similarly with literature. • We observed the increased number of CD4+ and CD8+ T cell counts on PFAPA tonsils compared to control group and emphasized the active role of both CD4+ and CD8+ cells localized on PFAPA tonsils in immune dysregulation. • Some other cell types evaluated in this study such as CD19+ (B cells), CD1a (dendritic cells), and CD123 (IL-3 receptors, for pluripotent stem cells) and H. pylori did not differ in PFAPA patients compared to the control group.

Comparison of clinical and pathological staging in oral cavity cancers.

OBJECTIVES: This study aims to compare the clinical and pathological TN stages of patients with oral cavity cancer and to identify the factors leading to staging discrepancies. PATIENTS AND METHODS: The medical records of 125 patients (77 males, 48 females; mean age 57 years; range 19 to 82 years) who underwent primary tumor resection and neck dissection simultaneously for oral cavity cancer were retrospectively analyzed. Clinical and pathological TN stages of all patients were compared. Sensitivity, specificity and predictive values of clinical staging were calculated. RESULTS: Computed tomography (CT) with contrast which was used to examine the cervical lymph node metastasis showed a sensitivity of 71.9%, a specificity of 75%, a positive predictive value of 70.6%, and a negative predictive value of 76.1%. The diagnostic accuracy of CT for detecting mandibular invasion was as follows: sensitivity, 92.6%; specificity, 97%; positive predictive value, 96.1%; and negative predictive value, 94.3%. CONCLUSION: High correlation between clinical and pathological stages for assessment of mandibular invasion and neck metastasis supports the reliability of CT in our study. Diagnostic contribution of magnetic resonance imaging is necessary for assessment of extrinsic tongue muscle involvement; in cases of tongue cancer which are surrounded by induration on palpation and extending to the floor of the mouth.

Whole Transcriptome Sequencing Reveals Cancer-Related, Prognostically Significant Transcripts and Tumor-Infiltrating Immunocytes in Mantle Cell Lymphoma.

Mantle cell lymphoma (MCL) is an aggressive B-cell non-Hodgkin lymphoma (NHL) subtype characterized by overexpression of CCND1 and SOX11 genes. It is generally associated with clinically poor outcomes despite recent improvements in therapeutic approaches. The genes associated with the development and prognosis of MCL are still largely unknown. Through whole transcriptome sequencing (WTS), we identified mRNAs, lncRNAs, and alternative transcripts differentially expressed in MCL cases compared with reactive tonsil B-cell subsets. CCND1, VCAM1, and VWF mRNAs, as well as MIR100HG and ROR1-AS1 lncRNAs, were among the top 10 most significantly overexpressed, oncogenesis-related transcripts. Survival analyses with each of the top upregulated transcripts showed that MCL cases with high expression of VWF mRNA and low expression of FTX lncRNA were associated with poor overall survival. Similarly, high expression of MSTRG.153013.3, an overexpressed alternative transcript, was associated with shortened MCL survival. Known tumor suppressor candidates (e.g., PI3KIP1, UBXN) were significantly downregulated in MCL cases. Top differentially expressed protein-coding genes were enriched in signaling pathways related to invasion and metastasis. Survival analyses based on the abundance of tumor-infiltrating immunocytes estimated with CIBERSORTx showed that high ratios of CD8+ T-cells or resting NK cells and low ratios of eosinophils are associated with poor overall survival in diagnostic MCL cases. Integrative analysis of tumor-infiltrating CD8+ T-cell abundance and overexpressed oncogene candidates showed that MCL cases with high ratio CD8+ T-cells and low expression of FTX or PCA3 can potentially predict high-risk MCL patients. WTS results were cross-validated with qRT-PCR of selected transcripts as well as linear correlation analyses. In conclusion, expression levels of oncogenesis-associated transcripts and/or the ratios of microenvironmental immunocytes in MCL tumors may be used to improve prognostication, thereby leading to better patient management and outcomes.

A randomized clinical study: outcome of submucous resection of compensatory inferior turbinate during septoplasty.

BACKGROUND: Septoplasty and/or turbinate surgery are commonly used surgical techniques for the treatment of mechanical nasal obstruction. The aim of this study was to define the effectiveness of submucous resection of a hypertrophied turbinate together with simultaneous septoplasty for the treatment of nasal obstruction. METHODS: Forty-two patients with septum deviation and compensatory contralateral inferior turbinate hypertrophy were recruited in this study. The inferior turbinate hypertrophy was diagnosed based on examination. The patients were randomly divided into two groups. In group A, a submucous resection was performed to treat a hypertrophied inferior turbinate, together with a septoplasty. In group B, only a septoplasty was performed. Acoustic rhinometry and rhinomanometry tests were conducted for an objective evaluation of nasal patency. A visual analog scale (VAS) was applied to the patients for the subjective evaluation of nasal obstruction complaints. RESULTS: The application of submucous resection intended to reduce a hypertrophied inferior turbinate led to a distinctive increase in cross-sectional area of nasal patency; however, when the two groups were compared, it was statistically significant only at the post-operative sixth month. There was no difference between the results of rhinomanometry. The subjective symptom scores were better in group A than in group B between the post-operative first to sixth month. CONCLUSION: Submucous resection of a hypertrophied inferior turbinate is necessary for the treatment of nasal obstruction.

Metastatic adenocarcinomas of the temporal bone: a report of three cases.

We reported three cases with adenocarcinomas of the lung, prostate and stomach, which metastasized to the temporal bone. Two of them had proven adenocarcinoma of the lung and stomach, respectively at the time of the diagnosis of the temporal bone tumor. But the other patient had no known primary malignancy when the tumor in the petrous apex was demonstrated radiologically. First he underwent biopsy of the petrous apex lesion and the pathology was reported as malignant; then a probable distant malignancy spreading to the temporal bone was suspected and searched for. Subsequently, his prostate adenocarcinoma was proved with prostate biopsy.

Comparison of Cortical Auditory Evoked Potential Findings in Presbycusis with Low and High Word Recognition Score.

BACKGROUND: Deteriorated speech understanding is a common complaint in elderly people, and behavioral tests are used for routine clinical assessment of this problem. Cortical auditory evoked potentials (CAEPs) are frequently used for assessing speech detection and discrimination abilities of the elderly, and give promise for differential diagnosis of speech understanding problems. PURPOSE: The aim of the study was to compare the P1, N1, and P2 CAEP latencies and amplitudes in presbycusis with low and high word recognition score (WRS). RESEARCH DESIGN: A cross-sectional study design was used forthe study. Two groups were formed from the patients with presbycusis based on their scores on the speech recognition test. STUDY SAMPLE: Fifty-seven elderly volunteers participated in the study. The first group composed of 27 participants with high WRS, the other group composed of 30 participants with low WRS. DATA COLLECTION AND ANALYSIS: The CAEP waves were recorded from these participants using speech signals. Latencies and amplitudes of P1 -N1-P2 waves of the two groups were compared with the f-test statistic. RESULTS: There were significant prolongation of P1 and N1 latencies in presbycusis with low WRS when compared with presbycusis with a relatively high word score (p < 0.05). CONCLUSION: According to the result of the research, P1 and N1 latencies of presbycusis with low WRS were longer than the participants with high WRS. Factors affecting peripheral auditory system, such as stimulus sensation level, might be responsible for P1 and N1 latency prolongation of the low WRS group.

Giant Congenital Sialolipoma of Parotid Gland with Parapharyngeal Extension.

INTRODUCTION: Sialolipoma is an extremely rare salivary gland tumor characterized by a well circumscribed mass composed of glandular tissue and mature adipose elements. Herein our aim was to present the fifth case of congenital sialolipoma, which was firstly followed up as a parotid gland hemangioma, and underline the fact that sialolipomas should be kept in mind in the differential diagnosis of congenital parotid gland masses. CASE REPORT: A 10-month old male presented with a left-sided huge neck mass which progressed after birth. Radiologic examination revealed a tumor originating from the parotid gland filling the parapharyngeal space. Histopathologic examination of an incisional biopsy was consistent with sialolipoma. A total parotidectomy with preservation of the facial nerve was performed at the age of 1 year. The postoperative recovery was uneventful with normal facial nerve function. There was no recurrence at the 24-month follow-up. CONCLUSION: Although it is a very rare benign tumor, congenital sialolipoma should be kept in mind in the differential diagnosis of congenital parotid mass.

Comparison of Cortical Auditory Evoked Potential Findings in Presbycusis with Low and High Word Recognition Score.

BACKGROUND: Deteriorated speech understanding is a common complaint in elderly people, and behavioral tests are used for routine clinical assessment of this problem. Cortical auditory evoked potentials (CAEPs) are frequently used for assessing speech detection and discrimination abilities of the elderly, and give promise for differential diagnosis of speech understanding problems. PURPOSE: The aim of the study was to compare the P1, N1, and P2 CAEP latencies and amplitudes in presbycusis with low and high word recognition score (WRS). RESEARCH DESIGN: A cross-sectional study design was used for the study. Two groups were formed from the patients with presbycusis based on their scores on the speech recognition test. STUDY SAMPLE: Fifty-seven elderly volunteers participated in the study. The first group composed of 27 participants with high WRS, the other group composed of 30 participants with low WRS. DATA COLLECTION AND ANALYSIS: The CAEP waves were recorded from these participants using speech signals. Latencies and amplitudes of P1-N1-P2 waves of the two groups were compared with the t-test statistic. RESULTS: There were significant prolongation of P1 and N1 latencies in presbycusis with low WRS when compared with presbycusis with a relatively high word score (p < 0.05). CONCLUSION: According to the result of the research, P1 and N1 latencies of presbycusis with low WRS were longer than the participants with high WRS. Factors affecting peripheral auditory system, such as stimulus sensation level, might be responsible for P1 and N1 latency prolongation of the low WRS group.

The frequency of auditory neuropathy detected by universal newborn hearing screening program.

OBJECTIVE: Auditory neuropathy/auditory dyssynchrony (AN/AD) has become a well-accepted clinical entity. The combined use of oto-acoustic emissions (OAEs) and auditory brainstem response (ABR) testing in the universal newborn hearing screening (UNHS) has led to the easy recognition of this disorder. Although, we are now able to diagnose AN/AD reliably, little is known about its epidemiology, etiology, and especially the frequency of its occurrence. The primary goal of this study was to determine the frequency of AN/AD in the Western Anatolian region of Turkey. The secondary goal was to compare the detection rate of AN/AD before and after the implementation of the UNHS in the audiology department of Dokuz Eylul University Hospital. METHOD: Between 2005 and 2007, among the 23,786 newborns who were screened by automated click evoked oto-acoustic emissions (a-CEOAE) and automated auditory brainstem responses (a-ABRs), 2236 were referred to our department. All necessary audiological tests were performed for all the referred newborns. Among them, babies with deficient or abnormal ABR in combination with normal OAEs were considered as having AN/AD. These babies were evaluated with additional diagnostic audiological tests. Furthermore, comparison of the incidence of children diagnosed with AN/AD before and after the implementation of UNHS in our audiology department was also performed. RESULTS: Among the referred newborns, 65 had abnormal or deficient ABR test results. Ten of these 65 newborn babies (mean diagnostic age: 5.7 months) with hearing impairment showed electrophysiological test results that were consistent with AN/AD. The frequency of AN/AD in these 65 children with hearing loss was 15.38%. Moreover, the frequency of AN/AD within UNHS was found to be 0.044%. Seven of the 10 babies with AN/AD had hyperbilirubinemia as a risk factor, which is a high rate to be emphasized. On the other hand, the retrospective investigation of children diagnosed with AN/AD in the same audiology department between 1999 and 2005 (i.e. before the implementation of UNHS) revealed only 7 children, with an average diagnostic age of 34 months. CONCLUSION: After implementing the UNHS, the incidence of AN/AD in the audiology department increased from 1.16 to 4.13. Furthermore, the age of diagnosis of AN/AD decreased from 34 months to 5.7 months. This study shows that AN/AD, when screened, is a comparatively common disorder in the population of hearing-impaired infants. While newborn hearing screening provides early detection of babies with hearing loss, it also helps to differentiate AN/AD cases when the screening is performed with both a-ABR and automated oto-acoustic emission (a-OAE) tests. Thus, the routine combined use of a-ABR and a-OAE tests in UNHS programs, especially for the high-risk infants, can provide better detection of newborns with AN/AD. Furthermore, hyperbilirubinemia is merely an association and maybe etiologically linked.

[Audiological evaluation of a child with Klippel-Feil syndrome]. / Klippel-Feil sendromlu bir çocukta odyolojik degerlendirme.

A 6-year-old boy with formerly diagnosed Klippel-Feil syndrome (KFS) presented with complaints of hearing loss, speech disorder, and problems related to his hearing aid. Clinical examination and computed tomography showed bilateral external and middle ear anomalies and audiological examination revealed bilateral severe conductive hearing impairment. The patient was prescribed a bone conduction hearing aid and, with necessary consultations, was examined for additional physical anomalies. All children with a diagnosis of KFS should be evaluated with audiologic and otologic examinations, consultations should be implemented with other disciplines concerning the presenting problems, a regular follow-up should be scheduled, and the parents should be informed on possible complications.

Hearing loss and middle ear involvement in patients with juvenile idiopathic arthritis.

OBJECTIVE: Evaluation of the hearing status and middle ear function of patients with juvenile idiopathic arthritis. METHODS: The study group was comprised of 38 ears of 19 patients (6 males, 13 females) aged between 5 and 23 years. The control group was comprised of 30 ears of 15 healthy subjects (5 males, 10 females) aged between 5 and 22 years. All subjects were examined audiologically using tympanometry, stapedial reflex, acoustic reflex decay, pure-tone audiometry, high frequency audiometry and transient evoked otoacoustic emission tests. RESULTS: There were statistically significant (p<0.05) number of ears (32%) with abnormal tympanograms in the patient population while all tympanograms were normal, type A in the control group. Seven type As, 2 type Ad, and 3 type C tympanograms were seen in the patient population. In pure tone audiometry tests there was no subject having neither a conductive nor sensorineural hearing loss individually in both groups. But as a group, patients with juvenile idiopathic arthritis showed statistically significant elevation of air conduction thresholds at frequencies of 250, 500, 6000, 14,000 and 16,000 Hz for right ears; and at 500, 2000, 12,500 and 16,000 Hz for left ears; and larger air-bone gaps at 500 and 2000 Hz for right ears; and at 500 Hz for left ears (p<0.05). Comparison of bone conduction thresholds and otoacoustic emission tests between both groups did not reveal any statistically significant difference (p>0.05). CONCLUSION: This study suggests a dual effect of disease on both the middle and inner ear of patients with juvenile idiopathic arthritis. Presence of abnormal tympanograms together with worse air conduction thresholds at lower frequencies as well as larger air bone gaps at frequencies of 500 and 2000 Hz suggest subclinical middle ear involvement; while hearing losses at 6000 Hz and very high frequencies of 12,500, 14,000 and 16,000 Hz suggest inner involvement at an early stage.