Cochlear Function in Adults Exposed to Severe Acute Respiratory Syndrome Coronavirus 2.

INTRODUCTION: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is a viral infection with a wide variety of symptoms, including fever, coughing, sneezing, fatigue, and a loss of taste and smell. Moreover, there are some recent studies that investigate the effects of SARS-CoV-2 on hearing and auditory performance. With this current study, we investigate the early effects of the coronavirus disease on hair cells in the cochlea. METHODS: In the current study, there were 25 subjects (17 females, 8 males) who tested positive for polymerase chain reaction on nasopharyngeal swabs. They had reported normal auditory functions and no history of otology before SARS-CoV-2. To determine auditory functions, pure-tone audiometry, otoacoustic emissions (OAE) tests, and threshold equalizing noise (TEN) tests were used. RESULTS: Although the hearing thresholds increased at higher frequencies, they were within normal limits according to four-frequency pure-tone averages. All participants had normal OAE, and there were no detected dead regions for any of the subjects. Even so, there were significant increases in hearing thresholds in TEN. CONCLUSION: There is no cochlear dysfunction discovered by OAE and TEN in SARS-CoV-2-affected individuals. Nonetheless, the increase in hearing thresholds at higher frequencies, other than the pure-tone average frequencies detected by TEN, and the decrease in the presence of detected OAE could be related to deterioration in the basal part of the cochlea.

Evaluation of the non-auditory responses in individuals with auditory brainstem implant.

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

Pediatric cochlear implant fitting parameters in inner ear malformation: Is it same with normal cochlea?

OBJECTIVES: The aim was to evaluate the cochlear implant (CI) mapping parameters of CI users with inner ear malformation (IEM) and to reveal the changes in parameters over time. METHODS: In total, 118 CI users were included with 127 ears (68-IEM; 59-normal cochlear anatomy) in present retrospective study. The impedance measurements, thresholds levels-THR, most comfortable levels- MCL, pulse width-PW and rate values were analyzed in both IEM and control group at the initial activation, 6th,12th and 24th months postoperatively. RESULTS: There were statistically significant differences in impedance measurements in several time points. And also, there was a remarkable difference in THR & MCL and PW values between IEM and control groups in all time points (p < 0.05). THR & MCL levels and PW values increased significantly between all time periods in both groups (p < 0,008) and values of parameters in IEM-group were higher than those of control group. When comparing rates, statistically significant difference was observed only at the initial activation in both within (p < 0.001) and between groups (p = 0.03). CONCLUSION: Pediatric CI users with IEM need individual changes in fitting parameters. More frequent map sessions should be planned as they require more PW, THR and MCL increase over time. The increase rate differs between IEM subgroups depending on the deviation of malformation from the normal cochlear anatomy. This study is the first to in its attempt to reveal the mapping characteristics and long-term changes in pediatric CI users with different IEM subgroups.

Revision Surgery for Pediatric Nontumor Auditory Brainstem Implant Users: Audiological, Rehabilitative, and Surgical Outcomes.

OBJECTIVE: To report the audiological, rehabilitative, and surgical outcomes of revision surgery for pediatric auditory brainstem implant (ABI) users. STUDY DESIGN: Retrospective cohort. SETTING: Tertiary referral center. PATIENTS: Five pediatric ABI users who had revision surgery for device malfunctions. INTERVENTIONS: Revision surgery for ABI malfunctions. MAIN OUTCOME MEASURES: The findings of free-field audiometry with the device, the Meaningful Auditory Integration Scale, and the pattern discrimination, word identification, sentence recognition, and expressive and receptive language tests before the device failure and after revision surgery were obtained from the patient records and compared. RESULTS: The revision rate for pediatric ABI was 6.45%. The Meaningful Auditory Integration Scale and expressive-receptive language scores showed improvements following revision surgery, while the aided thresholds, pattern perception, and word identification scores did not change. Individual differences in performance for these measures were observed. CONCLUSION: Equal or improved performance after the revision surgeries in the current study showed that revision surgery is successful and important for pediatric ABI users. It is essential to consider remedying the loss of auditory input in sensitive periods of pediatric development.

Stapedotomy in Congenital Fixation with Cochlear Hypoplasia: A New Concept Among the Treatment Options for Cochlear Hypoplasia.

OBJECTIVES: To introduce the concept of stapedotomy as a new treatment alternative in cochlear hypoplasia (CH) and propose a new guideline for its management. METHODS: Forty-two primary cases out of 355 presented with congenital stapes fixation between January 2003 and September 2015 were included in the study. Computed tomography scans of all cases with congenital stapes fixation were reviewed, and cases with inner ear anomalies were taken into account. Eleven cases had various inner ear anomalies, and 9 cases had various types of CH. In the present paper, only the CH cases with stapes fixation, all of whom underwent stapedotomy, are reviewed regarding preoperative audiological and radiological characteristics as well as surgical findings and postoperative audiological results. RESULTS: The patients were aged between 4 and 22. There were 2 males (3 ears) and 4 females (6 ears). Three cases had bilateral stapedotomy. The remaining 3 cases had unilateral surgery. The average preoperative air-bone gap (ABG) was 50.3 dB. Postoperative hearing: preoperative ABG was 50.3 dB. Postoperative ABG was calculated as 20.1 dB hearing. CONCLUSION: Hearing loss (HL) in hypoplastic cochlea demonstrates the full spectrum of HL types. CH is a unique inner ear anomaly that can be treated with all of the available rehabilitation modalities. As a result of current findings, a new treatment algorithm for CH is proposed.

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

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.

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

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.

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

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.

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

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.

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

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.

Cochlear Nerve Hypoplasia: Audiological Characteristics in Children and Adults.

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.

Effects of distractors on upright balance performance in school-aged children with attention deficit hyperactivity disorder, preliminary study / Efeitos de distrações sobre o desempenho do equilíbrio vertical em crianças em idade escolar com transtorno de déficit de atenção e hiperatividade - estudo preliminar

Abstract Introduction: Attention deficit hyperactivity disorder is a common impairing neuropsychiatric disorder with onset in early childhood. Almost half of the children with attention deficit hyperactivity disorder also experience a variety of motor-related dysfunctions ranging from fine/gross motor control problems to difficulties in maintaining balance. Objectives: The main purpose of this study was to investigate the effects of distractors two different auditory distractors namely, relaxing music and white noise on upright balance performance in children with attention deficit hyperactivity disorder. Methods: We compared upright balance performance and the involvement of different sensory systems in the presence of auditory distractors between school-aged children with attention deficit hyperactivity disorder (n = 26) and typically developing controls (n = 20). Neurocom SMART Balance Master Dynamic Posturography device was used for the sensory organization test. Sensory organization test was repeated three times for each participant in three different test environments. Results: The balance scores in the silence environment were lower in the attention deficit hyperactivity disorder group but the differences were not statistically significant. In addition to lower balance scores the visual and vestibular ratios were also lower. Auditory distractors affected the general balance performance positively for both groups. More challenging conditions, using an unstable platform with distorted somatosensory signals were more affected. Relaxing music was more effective in the control group, and white noise was more effective in the attention deficit hyperactivity disorder group and the positive effects of white noise became more apparent in challenging conditions. Conclusion: To the best of our knowledge, this is the first study evaluating balance performance in children with attention deficit hyperactivity disorder under the effects of auditory distractors. Although more studies are needed, our results indicate that auditory distractors may have enhancing effects on upright balance performance in children with attention deficit hyperactivity disorder.

Resumo Introdução: O transtorno do déficit de atenção e hiperatividade é um distúrbio neuropsiquiátrico comum que causa comprometimentos, com início na primeira infância. Quase metade das crianças com transtorno do déficit de atenção e hiperatividade também experimenta uma variedade de distúrbios relacionados às habilidades motoras, desde problemas de controle de habilidades motoras finas/grossas até dificuldades na manutenção do equilíbrio. Objetivos: O principal objetivo deste estudo foi investigar os efeitos de distrações, especificamente duas distrações auditivas diferentes, música relaxante e ruído branco, sobre o desempenho do equilíbrio vertical em crianças com transtorno de déficit de atenção e hiperatividade. Método: Comparamos o desempenho do equilíbrio vertical e o envolvimento de diferentes sistemas sensoriais na presença de distração auditiva entre crianças em idade escolar com transtorno do déficit de atenção e hiperatividade (n = 26) e controles com desenvolvimento típico (n = 20). O dispositivo Neurocom Smart Balance Master Dynamic Posturography foi utilizado para o teste de organização sensorial. O teste de organização sensorial foi repetido três vezes para cada participante em três ambientes de teste diferentes. Resultados: Os escores de equilíbrio no ambiente em silêncio foram menores no grupo com transtorno do déficit de atenção e hiperatividade, mas as diferenças não foram estatisticamente significativas. Além dos escores de equilíbrio mais baixos, as razões vestibulares e visuais também foram menores. As distrações auditivas afetaram positivamente o desempenho do equilíbrio geral para ambos os grupos. Condições mais desafiadoras, usando uma plataforma instável com sinais somatossensoriais distorcidos, foram mais afetados. Música relaxante foi mais eficaz no grupo de controle, e ruído branco foi mais eficaz no grupo de hiperatividade com déficit de atenção e os efeitos positivos do ruído branco se tornaram mais evidentes em condições desafiadoras. Conclusão: Que seja de nosso conhecimento, este é o primeiro estudo que avalia o desempenho do equilíbrio em crianças com transtorno do déficit de atenção e hiperatividade sob os efeitos de distrações auditivas. Embora mais estudos sejam necessários, os nossos resultados indicam que as distrações auditivas podem ter efeitos de aumento no desempenho do equilíbrio vertical em crianças com transtorno de déficit de atenção e hiperatividade.

Effects of distractors on upright balance performance in school-aged children with attention deficit hyperactivity disorder, preliminary study.

INTRODUCTION: Attention deficit hyperactivity disorder is a common impairing neuropsychiatric disorder with onset in early childhood. Almost half of the children with attention deficit hyperactivity disorder also experience a variety of motor-related dysfunctions ranging from fine/gross motor control problems to difficulties in maintaining balance. OBJECTIVES: The main purpose of this study was to investigate the effects of distractors two different auditory distractors namely, relaxing music and white noise on upright balance performance in children with attention deficit hyperactivity disorder. METHODS: We compared upright balance performance and the involvement of different sensory systems in the presence of auditory distractors between school-aged children with attention deficit hyperactivity disorder (n=26) and typically developing controls (n=20). Neurocom SMART Balance Master Dynamic Posturography device was used for the sensory organization test. Sensory organization test was repeated three times for each participant in three different test environments. RESULTS: The balance scores in the silence environment were lower in the attention deficit hyperactivity disorder group but the differences were not statistically significant. In addition to lower balance scores the visual and vestibular ratios were also lower. Auditory distractors affected the general balance performance positively for both groups. More challenging conditions, using an unstable platform with distorted somatosensory signals were more affected. Relaxing music was more effective in the control group, and white noise was more effective in the attention deficit hyperactivity disorder group and the positive effects of white noise became more apparent in challenging conditions. CONCLUSION: To the best of our knowledge, this is the first study evaluating balance performance in children with attention deficit hyperactivity disorder under the effects of auditory distractors. Although more studies are needed, our results indicate that auditory distractors may have enhancing effects on upright balance performance in children with attention deficit hyperactivity disorder.

Audiological and Radiological Characteristics in Incomplete Partition Malformations.

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.

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

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.

Audiologic and radiologic findings in cochlear hypoplasia.

OBJECTIVE: The aim of the current study is to evaluate audiologic and radiologic findings of cochlear hypoplasia which is a subgroup of inner ear malformations. METHODS: This study was a prospective clinical study and based on voluntary participation from cases with cochlear hypoplasia diagnosis. The study was conducted at Hacettepe University, Department of Otolaryngology, Head and Neck Surgery and Department of Audiology. Subjects were selected from an inner ear malformations database. Inclusion criteria were having cochlear hypoplasia for at least one ear. There were 66 subjects with an age range of 12 months and 60 years 5 months. For each subject, pure tone audiometry and tympanometry were applied according to chronological and cognitive age. And also, auditory brainstem response test was applied to when it is need. Subjects' radiologic results were reevaluated to confirm cochlear hypoplasia, cochlear nerve and cochlear aperture. RESULTS: Cochlear hypoplasia types were statistically significantly different in terms of HL degree. This difference was caused by cochlear hypoplasia type IV group being was statistically different from the other three groups. Like with degree of HL, cochlear hypoplasia groups were statistically different from other three groups in terms of type of hearing loss. Cochlear aperture and cochlear nerve status showed variation according to cochlear hypoplasia type but these differences were not statistically approved. CONCLUSIONS: In the current study, incidence of cochlear hypoplasia was 23.5% in all inner ear malformation. With this study, it was seen that subtypes of cochlear hypoplasia showed variability in terms of degree and type of hearing loss and also cochlear aperture and cochlear nerve status. Especially cochlear hypoplasia type IV differs from other three cochlear hypoplasia types.

Prognostic Factors in Sudden Sensorineural Hearing Loss.

BACKGROUND: Sudden sensorineural hearing loss (SSNHL) is still a complex and challenging process which requires clinical evidence regarding its etiology, treatment and prognostic factors. Therefore, determination of prognostic factors might aid in the selection of proper treatment modality. AIMS: The aim of this study is to analyze whether there is correlation between SSNHL outcomes and (1) systemic steroid therapy, (2) time gap between onset of symptoms and initiation of therapy and (3) audiological pattern of hearing loss. STUDY DESIGN: Retrospective chart review. METHODS: Patients diagnosed at our clinic with SSNHL between May 2005 and December 2011 were reviewed. A detailed history of demographic features, side of hearing loss, previous SSNHL and/or ear surgery, recent upper respiratory tract infection, season of admission, duration of symptoms before admission and the presence of co-morbid diseases was obtained. Radiological and audiological evaluations were recorded and treatment protocol was assessed to determine whether systemic steroids were administered or not. Treatment started ≤5 days was regarded as "early" and >5 days as "delayed". Initial audiological configurations were grouped as "upward sloping", "downward sloping", "flat" and "profound" hearing loss. Significant recovery was defined as thresholds improved to the same level with the unaffected ear or improved ≥30 dB on average. Slight recovery was hearing improvement between 10-30dB on average. Hearing recovery less than 10 dB was accepted as unchanged. RESULTS: Among the 181 patients who met the inclusion criteria, systemic steroid was administered to 122 patients (67.4%), whereas 59 (32.6%) patients did not have steroids. It was found that steroid administration did not have any statistically significant effect in either recovered or unchanged hearing groups. Early treatment was achieved in 105 patients (58%) and 76 patients (42%) had delayed treatment. Recovery rates were no different in these two groups; however, when unchanged hearing rates were compared, it was statistically significantly lower in the early treatment group (p<0.05). When hearing outcomes were compared according to initial audiological pattern, significant recovery and unchanged hearing rates did not differ between groups; however, slight recovery rate was highest in the "flat" type audiological configuration (p<0.05). CONCLUSION: According to this patient series, oral steroid therapy does not have any influence on the outcomes of SSNHL. However, mid-frequency hearing loss of flat type and initiation of treatment earlier than 5 days from the onset of symptoms, seem to have positive prognostic effects. Further randomized controlled subject groups might contribute to determine prognostic factors of SSNHL.

Methods and preliminary outcomes of pediatric auditory brainstem implantation.

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