Long term results of glass ionomer ossiculoplasty.

OBJECTIVE: To analyze and compare the early and late post-operative results of glass ionomer bone cement (GIBC) used in ossiculoplasty. METHODS: The pre-operative, early post-operative (3 months) and late post-operative (> 2 years) audiometric findings, namely the pure-tone average (PTA), bone conduction threshold (BCT) and air-bone gap (ABG) of 40 patients who underwent GIBC ossiculoplasty for different etiologies were analyzed. Early and late results were compared. Also, the patients were grouped in terms of prognostic factors and applied ossiculoplasty techniques, and the results were compared between the groups. RESULTS: There were statistically significant improvements in the pure-tone average and air-bone gap of the patients in the early post-operative period (PTA from 59.60 ± 15.95 to 40.37 ± 17.83 and ABG from 37.12 ± 11.18 to 19.78 ± 10.41, p < 0.001 for both). There were no statistically significant changes in any of the audiometric parameters in the late post-operative period (PTA from 40.37 ± 17.83 to 39.79 ± 17.91, ABG from 19.78 ± 10.41 to 19.32 ± 9.60, BCT from 17.99 ± 12.71 to 18.31 ± 13.99, p > 0.05 for all). Presence of tympanosclerosis was found to be the only prognostic factor to affect the outcome. CONCLUSION: GIBC is a safe and reliable material for ossiculoplasty, which maintains its ability to conduct sound in the long-term follow-up.

Transcanal Endoscopic Management of Middle Ear Paragangliomas.

OBJECTIVE: To evaluate the clinical and audiological outcomes of transcanal endoscopic resection of middle ear paragangliomas. STUDY DESIGN: Retrospective multicenter study. SETTING: Tertiary referral center and private otology clinic. PATIENTS: Patients who underwent transcanal endoscopic surgery between January 2015 and September 2020. INTERVENTIONS: Transcanal endocope-assisted resection of middle ear paragangliomas. MAIN OUTCOME MEASURES: Demographic data. RESULTS: Twenty-three patients (2 men, 21 women) with a mean (standard deviation [SD]) age of 50.5 (11.8) years and stage 1 or 2 disease were included in the study. The mean follow-up time was 2.7 years (range, 1-5 yr). Preoperatively, the mean (SD) air-conduction threshold was 33.8 (17.9) dB, and the mean (SD) air-bone gap was 13.1 (13.9) dB. Postoperatively, the mean (SD) air-conduction threshold was 25.7 (10.2) dB, the mean (SD) air-bone gap was 6.3 (6.1) dB. The mean (SD) hospital stay was 27.7 (9.9) hours. No tumor regrowth was detected on magnetic resonance imaging during postoperative follow-up. CONCLUSIONS: Endoscopic transcanal tumor resection is effective and feasible in the treatment of stage 1 and 2 tumors and is associated with short operative time, low risk of perioperative and postoperative complications, and rapid discharge.

Hearing Loss Related to Gene Mutations in Distal Renal Tubular Acidosis.

INTRODUCTION: Distal renal tubular acidosis (dRTA) is a disease that may develop either primarily or secondarily, resulting from urinary acidification defects in distal tubules. Hearing loss may accompany primary forms of dRTA. This study aims to determine the characteristics of hearing loss due to different gene mutations in patients with dRTA. METHODS: Behavioral and electrophysiological audiological evaluations were performed after otolaryngology examination in 21 patients with clinically diagnosed dRTA. Radiological imaging of the inner ear (n = 9) was conducted and results of genetic analyses using next-generation sequencing method (n = 16) were included. RESULTS: Twenty-one patients with dRTA from 20 unrelated families, aged between 8 months and 33 years (median = 12, interquartile range = 20), participated. All patients with ATP6V1B1 mutations (n = 9) had different degrees of hearing loss. There was one patient with hearing loss in patients with ATP6V0A4 mutations (n = 6). One patient with the WDR72 mutation had normal hearing. Large vestibular aqueduct syndrome (LVAS) was detected in 6 (67%) of 9 patients whose radiological evaluation results were available. CONCLUSIONS: LVAS is common in patients with dRTA and may influence the type and severity of hearing loss in these patients. The possibility of both congenital and late-onset and progressive hearing loss should be considered in dRTA patients. A regular audiological follow-up is essential for the early detection of a possible late-onset or progressive hearing loss in these patients.

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.

Evidence of visual crossmodal reorganization positively relates to speech outcomes in cochlear implant users.

Deaf individuals who use a cochlear implant (CI) have remarkably different outcomes for auditory speech communication ability. One factor assumed to affect CI outcomes is visual crossmodal plasticity in auditory cortex, where deprived auditory regions begin to support non-auditory functions such as vision. Previous research has viewed crossmodal plasticity as harmful for speech outcomes for CI users if it interferes with sound processing, while others have demonstrated that plasticity related to visual language may be beneficial for speech recovery. To clarify, we used electroencephalography (EEG) to measure brain responses to a partial face speaking a silent single-syllable word (visual language) in 15 CI users and 13 age-matched typical-hearing controls. We used source analysis on EEG activity to measure crossmodal visual responses in auditory cortex and then compared them to CI users' speech-in-noise listening ability. CI users' brain response to the onset of the video stimulus (face) was larger than controls in left auditory cortex, consistent with crossmodal activation after deafness. CI users also produced a mixture of alpha (8-12 Hz) synchronization and desynchronization in auditory cortex while watching lip movement while controls instead showed desynchronization. CI users with higher speech scores had stronger crossmodal responses in auditory cortex to the onset of the video, but those with lower speech scores had increases in alpha power during lip movement in auditory areas. Therefore, evidence of crossmodal reorganization in CI users does not necessarily predict poor speech outcomes, and differences in crossmodal activation during lip reading may instead relate to strategies or differences that CI users use in audiovisual speech communication.

Hidden hearing loss: current concepts.

PURPOSE OF REVIEW: The purpose of this review is to offer a concise summary of current knowledge regarding hidden hearing loss (HHL) and to describe the variety of mechanisms that contribute to its development. We will also discuss the various diagnostic tools that are available as well as future directions. RECENT FINDINGS: Hidden hearing loss often also called cochlear synaptopathy affects afferent synapses of the inner hair cells. This description is in contrast to traditional models of hearing loss, which predominantly affects auditory hair cells. In HHL, the synapses of nerve fibres with a slow spontaneous firing rate, which are crucial for locating sound in background noise, are severely impaired. In addition, recent research suggests that HHL may also be related to cochlear nerve demyelination. Noise exposure causes loss of myelin sheath thickness. Auditory brainstem response, envelope-following response and middle-ear muscle reflex are promising diagnostic tests, but they have yet to be validated in humans. SUMMARY: Establishing diagnostic tools for cochlear synaptopathy in humans is important to better understand this patient population, predict the long-term outcomes and allow patients to take the necessary protective precautions.

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.

Unilateral Facial Paralysis and Ophthalmoplegia Caused by Lower Lip Carcinoma: A Case Report.

We discuss a case of lower lip carcinoma which presented with atypical symptoms; facial paralysis, conductive type hearing loss, and ophthalmoplegia. Due to an earlier resection, no mass was evident on the primary examination. Diagnostic imaging revealed a mass originating from the lower lip, the perineural spread of the tumor along the left inferior alveolar nerve to the left infratemporal fossa and the left foramen ovale. Through a retrograde course from the foramen ovale, the tumor extended the ipsilateral cavernous sinus, Meckel's cave, and cisternal portion of the CN V. This atypical spread pattern of the tumor caused symptoms that may be attributed to a diagnosis related to the ear. The biopsy confirmed squamous cell carcinoma, and the patient was referred for chemotherapy and radiotherapy.

Endonasal choanal atresia repair; evaluating the surgical results of 58 cases.

BACKGROUND: Choanal atresia is the most common congenital nasal anomaly, with an incidence of 1:5000-1:8000 live births. Atresia can be seen as membraneous, bony or mixed type. When it is bilateral, it is accepted lifethreatening, therefore bilateral atresia necessitates immediate intervention. Diagnosis is confirmed by endoscopic examination and computed tomography. The absolute treatment is surgical, and different approaches have been proposed. METHODS: Herein, we describe our 15-year experience in the treatment of 58 patients of congenital choanal atresia with transnasal endoscopic approach, and we compare the efficacy of placement of an intranasal stent and applying mitomycin while endoscopic microsurgical repair. RESULTS: The study included 41 female patients (71%) and 17 male patients (29%) with congenital CA. The mean age was 3 years ranging from 10 days to 16 years. The atretic plate was bilateral in 24 patients (41%) and unilateral in 34 (59%). The most common atresia type was the mixed type with 29 patients (50%). A total of 17 patients (29%) required postoperative revision(s). Postoperative revisions were more frequent among patients with bilateral CA (50%), and with mixed CA (31%). Stenting was used additionally by surgical correction for 10 patients. After stenting, fibrosis and restenosis was seen in 7 patients (79%). Mitomycin C was applied peroperatively in 8 patients. Restenosis after mitomycin application was seen in 4 patients (50%). CONCLUSIONS: By our experience, endoscopic microsurgical repair of atresia proved to be an effective and safe procedure, results compared with adjuvant treatment modalities like stent or mitomycin C use, was not better. Restenosis was the major problem seen after surgical correction.

Management of stapes footplate fistula in inner ear malformations.

BACKGROUND: Certain inner ear malformations have stapes footplate fistula which may cause meningitis during otitis media. This may result in fatality. It is the responsibility of the otolaryngologist to diagnose and treat the condition to prevent further attacks of meningitis. MATERIALS AND METHODS: Surgical findings of the 17 patients who have inner ear malformations with oval window fistula were retrospectively analyzed. Inner ear malformations were classified according to Sennaroglu classification. Different stages of stapes footplate fistula are classified. FINDINGS: Seventeen patients had spontaneous stapes footplate fistula at the oval window. No patient had spontaneous leakage at the round window site. Proper sealing of the leakage area with fascia in a dumbbell fashion is mandatory. Keeping the stapes in place and lumbar drainage are the two most important factors in the successful management of fistula. Particularly important is the simultaneous fistula repair and cochlear implantation where combined postauricular-transcanal approach provides the best method. Vaccination is important but not sufficient to prevent meningitis in inner ear malformations unless repair of the fistula is performed. CONCLUSION: If the patient has a history of meningitis in the presence of inner ear malformation, particular attention should be given to oval window area to look for an opacity, cyst or a leaking lesion at the stapes footplate. Immediate surgical exploration and repair of the leak is mandatory to prevent further attacks of meningitis. Surgeon should not leave the operation without fully controlling the leak.

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

OBJECTIVES: To determine the temporal order, resolution, and perception of prosody skills in Single-Sided Deafness (SSD) compared to an age- and sex-matched normal hearing group's same side ear and both ears. METHODS: This was a Case-Control study including 30 subjects with SSD, and age- and sex-matched 30 subjects with bilateral normal hearing (total of 60 subjects- mean age: 38.7 ± 11.6 years). The Montreal Cognitive Assessment (MoCA), Frequency Pattern Test (FPT), Duration Pattern Test (DPT), Random Gap Detection Test (RGDT), Evaluation of Motor Response Time and Emotional Prosody Assessment were performed on the clinically normal ear in the SSD group, the same side ear in the normal hearing group, and both ears of the normal hearing group (the SSD, MNH, and BNH groups, respectively). RESULTS: Individuals with SSD had worse results in DPT (p < .001), gap detection at 0.5 kHz (p < .001), gap detection at 4 kHz (p < .001), and composite score (p < .001) than the BNH group. For reaction time measurements, the SSD group had slower performance scores than the BNH group for DPT (p < .001) and FPT (p < .001). CONCLUSIONS: Poor temporal processing ability and reduced reaction times may help explain the difficulties in those with SSD in performing daily living activities such as speech understanding in noise and requires more processing efforts. However, there were no significant differences between the groups in frequency pattern performance and emotional prosody skills, supporting the claim that fundamental frequency is one of the most important measures of perception in emotional prosody. We demonstrated that unilateral hearing is adequate to analyze frequency patterns to aid in prosody perception. Analysis of reaction times in temporal processing and emotional prosody could provide different perspectives of auditory processing. Slower reaction time of SSD should be considered for habilitation purposes.

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.

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

OBJECTIVES: The aim of the present study was to investigate the effect of the national pneumococcal vaccination program on postmeningitis sensorineural hearing loss (SNHL). MATERIALS AND METHODS: Overall, 2751 patients (2615 cochlear implantation and 136 auditory brainstem implantation) who underwent cochlear implantation (CI) and auditory brainstem implantation (ABI) at a tertiary referral hospital otolaryngology clinic were retrospectively analyzed. One hundred sixteen patients with a history of meningitis were included in the study. Patients were evaluated for their age at the time of surgery, gender, computerized tomography (CT) and magnetic resonance imaging (MRI) findings, implant type, side, and incidence before and after the vaccination program. RESULTS: When patients with cochlear implants or ABI were examined, the incidence of meningitis-induced hearing loss was 6.2% in the pre-vaccination period and 0.6% in the post-vaccination period. There is a significant difference between them when compared by chi-square test (p<0.001). CONCLUSION: The most important finding of the present study is the dramatic decrease in the number of CI and ABI surgeries performed in patients with SNHL due to meningitis. This shows the effectivity of pneumococcal vaccination in this special group of patients. If total ossification is detected on CT of patients with postmeningitis, ABI should be preferred to CI.

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

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