Audiology and Neurotology 2020-09-09

Analysis of Click and Swept-Tone Auditory Brainstem Response Results for Moderate and Severe Sensorineural Hearing Loss

J Tan,J Luo,X Wang,Y Jiang,X Zeng,S Chen,P Li

Publicatie 09-09-2020

Introduction: Auditory brainstem response (ABR) is one of the commonly used methods in clinical settings to evaluate the hearing sensitivity and auditory function. The current ABR measurement usually adopts click sound as the stimuli. However, there may be partial ABR amplitude attenuation due to the delay characteristics of the cochlear traveling wave along the basilar membrane. To solve that problem, a swept-tone method was proposed, in which the show-up time of different frequency components was adjusted to compensate the delay characteristics of the cochlear basilar membrane; therefore, different ABR subcomponents of different frequencies were synchronized. Methods: The normal hearing group, moderate sensorineural hearing loss group, and severe sensorineural hearing loss group underwent click ABR and swept-tone ABR with different stimulus intensities. The latencies and amplitudes of waves I, III, and V in 2 detections were recorded. Results: It was found that the latency of each of the recorded I, III, and V waves detected by swept-tone ABR was shorter than that by click ABR in both the control group and experimental groups. In addition, the amplitude of each of the recorded I, III, and V waves, except V waves under 60 dB nHL in the moderate sensorineural hearing loss group, detected by swept-tone ABR was larger than that by click ABR. The results also showed that the swept-tone ABR could measure the visible V waves at lower stimulus levels in the severe sensorineural hearing loss group. Conclusion: Swept-tone improves the ABR waveforms and helps to obtain more accurate threshold to some extent. Therefore, the proposed swept-tone ABR may provide a new solution for better morphology of ABR waveform, which can help to make more accurate diagnosis about the hearing functionality in the clinic. Audiol Neurotol

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Changes in Tinnitus by Cochlear Implantation: A Parametric Study of the Effect of Single-Electrode Stimulation

FJJ Kloostra,E de Kleine,RH Free,R Hofman,P Van Dijk

Publicatie 07-09-2020

Introduction: While cochlear implantation may have a positive effect on tinnitus, it is not effective in reducing tinnitus in all patients. This may be due to different patients requiring different strategies of electrical stimulation in order to obtain a positive effect on tinnitus. It is, therefore, important to identify the most effective stimulation strategies to reduce tinnitus. The simplest possible strategy is stimulation by only one electrode. In this study, we investigated tinnitus suppression by electrical stimulation via a single electrode of the cochlear implant. Methods: We performed a listening experiment in 19 adult participants, who had received a unilateral cochlear implant (CI) because of severe bilateral hearing loss. All of these patients had indicated that they suffered from tinnitus. During a 300-s interval, patients listened to blocks of single-electrode stimulation and rated the loudness of the stimulus and any effects on their tinnitus. The 300-s interval included a block of single-electrode stimulation (duration 120 s). In consecutive intervals, the stimulus differed in its cochlear location (basal or apical), its pulse rate (720 or 725 Hz, 1,200 Hz, and 2,400 or 2,320 Hz), and amplitude (just above threshold or equivalent to moderate loudness). Thus, 2 × 3 × 2 = 12 stimulus conditions were tested in each participant, and each condition was presented only once. During the experiment, the participants promptly rated the loudness of the stimuli and the loudness of their tinnitus on a Visual Analogue Scale (10-point VAS). Results: Significantly more tinnitus reduction was observed with stimuli at a moderate intensity level (30%) compared to stimuli at near-threshold level (18%) (χ2 1, N = 222 = 14.115, p #x3c; 0.01). No significant differences in tinnitus levels resulted from the different pulse rates and stimulation sites. Eight participants reported an increase of tinnitus loudness under at least one stimulus condition. Changes in tinnitus loudness were generally minor, and never exceeded 3 points on the VAS. The overall effect of cochlear implantation on tinnitus, that is, the effect with full-array stimulation, was not correlated with the effectiveness of the single-electrode stimulation on tinnitus. Conclusion: In conclusion, the effect of single-electrode stimulation on tinnitus is relatively insignificant in comparison to the effect of full-array stimulation. However, in some individual cases, sustained single-electrode stimulation may be beneficial for tinnitus management. Audiol Neurotol

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How Long Is Otosclerosis Surgery Effective? Hearing Results after a 22-Year Follow-Up

D Lucidi,G Paludetti,S Settimi,E De Corso,PM Picciotti,B Sergi

Publicatie 03-09-2020

Introduction: Stapes surgery is a safe procedure, with favourable hearing outcome. The objective of the study is to assess the long-term hearing results, addressing the bone conduction (BC) decay and the need for hearing aids in otosclerosis patients. Methods: We enrolled patients who underwent stapes surgery by means of stapedectomy or stapedotomy between 1991 and 2001. All enrolled patients underwent pure-tone audiometry (PTA) between September 2017 and June 2018. A set of questions was administered to record the prevalence of subjective symptoms and the need for hearing aids. Results: Seventy patients were enrolled for a long-term evaluation; 37 patients underwent bilateral surgery; therefore, 107 ears were included in the analysis. The average follow-up period was 22 years. No statistically significant difference was found between early and late post-operative air conduction (AC) PTA (41 vs. 49 dB; p #x3e; 0.05) nor between early and late post-operative BC-PTA (29 vs. 37 dB; p #x3e; 0.05). A significant difference was observed for AC at 8 kHz (65 vs. 78 dB; p #x3c; 0.05) and BC at 2 and 4 kHz (28 vs. 40 dB and 45 vs. 58 dB, respectively; p #x3c; 0.05). Conclusions: This is, to our knowledge, the longest mean follow-up time in the literature. A mild decrease in both AC and BC threshold can be expected and the sensorineural decay is more pronounced on the high frequencies. The subjective hearing symptoms and overall sound perception are satisfactory. Audiol Neurotol

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Influence of Semicircular Canal Dehiscence on Cochlear Implant Outcome

J Matic,S Winklhofer,F Pfiffner,C Roosli,D Veraguth,A Huber,A Dalbert

Publicatie 02-09-2020

Introduction: Semicircular canal dehiscence (SCD) is defined as a defect of the bone overlying the semicircular canal. It has a relatively high prevalence of 3% in the general population, which makes it likely that a certain number of patients receiving a cochlear implant (CI) would have it. However, little is known about the influence of SCD on the CI outcome. Therefore, the aim of this study was to determine the influence of SCD on CI outcome with regard to short- and long-term word perception and hearing preservation. Methods: This study was a retrospective analysis of postoperative word perception ability in the electric-only condition after 6, 12, and ≥18 months and of hearing preservation 4 weeks after surgery in CI recipients with and without SCD. All patients received a preoperative 1.5- or 3-tesla magnetic resonance imaging. Results: Fifty-five patients were included. Forty-eight patients (87%) had no SCD, and 7 patients (13%) had SCD. Mean postoperative word perception scores were 66% in the non-SCD group versus 50% in the SCD group (p = 0.17) after 6 months, 74 versus 64% (p = 0.28) after 12 months, and 77 versus 73% (p = 0.62) after 18 or more months. The mean postoperative hearing loss in patients with functional residual hearing before surgery (n = 34) was 22 dB in the non-SCD group versus 31 dB in the SCD group (p = 0.15). Conclusions: CI outcome is comparable between recipients without and with SCD. Specifically, hearing preservation rate and word perception ability in the electric-only condition seem not affected by SCD. The rate of progress of word perception ability in the first 12 months after cochlear implantation is not influenced by SCD. Audiol Neurotol

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Effect of Cochlear Implantation on Hearing Fluctuation in Patients with Biallelic SLC26A4 Variants

G Na,JM Lee,HJ Lee,Y Jeong,J Jung,JY Choi

Publicatie 02-09-2020

Introduction: Fluctuating hearing loss is a distinctive feature caused by SLC26A4 variants. We investigated whether cochlear implantation had protective or deleterious effect on hearing fluctuation in patients with biallelic SLC26A4 variants. Methods: Patients with biallelic SLC26A4 variants (N = 16; age = 10.24 ± 9.20 years) who had unilateral cochlear implantation and consecutive postsurgical, bilateral pure-tone audiograms more than 3 times were selected. We retrospectively reviewed the patients’ medical records from 2008 to 2019 obtained from a tertiary medical center and used the auditory threshold change (Shift) over time as a marker of hearing fluctuation. Fluctuation events were counted, and the Shift of the implanted and contralateral ears was compared using logistic regression with a generalized estimating equation and linear mixed model. A total of 178 values were included. Results: The odds of fluctuating hearing frequency were 11.185-fold higher in the unimplanted ears than in the implanted ears postoperatively (p = 0.001). The extent of fluctuation at 250 and 500 Hz was also significantly lower in the implanted ears than in the unimplanted ears after adjusting for every other effect (p = 0.003 and p #x3c; 0.001, respectively). Notably, higher residual hearing was rather associated with lesser fluctuation in frequency and the extent of fluctuation at 500 Hz, indicating residual hearing function is not the positive predictor for hearing fluctuation. Conclusion: In patients with biallelic SLC26A4 variants, cochlear implantation may reduce the frequency and extent of hearing fluctuations. Audiol Neurotol

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A New Pathogenic Variant in the TRIOBP Associated with Profound Deafness Is Remediable with Cochlear Implantation

AM Tekin,G de Ceulaer,P Govaerts,Y Bayazit,W Wuyts,P Van de Heyning,V Topsakal

Publicatie 02-09-2020

Background and Objectives: A rare type of nonsyndromic autosomal recessive hereditary hearing loss is caused by pathogenic mutations in the TRIOBP gene mostly involving exons 6 and 7. These mutations cause hearing loss originating from dysfunction of sensory inner ear hair cells. Of all the affected siblings, 2 brothers and 1 sister, part of an Afghan family, were referred to our clinic for diagnostic workup and candidacy selection for cochlear implantation (CI). Methods: Molecular analysis showed a homozygous c.1342C #x3e; T p. (Arg448*) pathogenic variant in exon 7 of the TRIOBP gene (reference sequence NM_001039141.2) in all 3 affected siblings. Clinical audiometry demonstrated profound sensorineural hearing loss in all 3 affected siblings (2 males and 1 female), and they were implanted unilaterally. Results: One month after activation, the pure-tone averages with the CI processor were between 30 and 23 dBHL. Ten months after the first activation of the implant, open-set speech audiometry test could be performed for the first time in the 2 younger CI recipients (S5 and S9), and they could identify up to a maximum 77% phonemes correctly. The oldest brother (S12) could not yet perform open-set speech audiometry at that moment. Conclusions: Implant outcomes are better with normal inner ear anatomy in general. The earlier congenital patients are implanted, the better their outcomes. Here, we demonstrate both statements are true in a homozygous c.1342C #x3e; T p. (Arg448*) pathogenic variant in the TRIOBP gene in all 3 affected siblings. Audiol Neurotol

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Acceptance and Benefits of Electro-Acoustic Stimulation for Conventional-Length Electrode Arrays

ER Spitzer,SB Waltzman,DM Landsberger,DR Friedmann

Publicatie 28-07-2020

Background: Prior studies have shown an advantage for electro-acoustic stimulation (EAS) in cochlear implant (CI) patients with residual hearing, but the degree of benefit can vary. The objective was to explore which factors relate to performance with and acceptance of EAS for CI users with conventional-length electrodes. Methods: A retrospective chart review was conducted for adults with an average threshold of 75 dB hearing loss or better across 250 and 500 Hz preoperatively (n = 83). All patients underwent cochlear implantation with a conventional-length electrode. Low-frequency audiometric thresholds were measured at initial activation as well as 3 and 12 months postoperatively to determine who met the criteria for EAS. Speech perception for CNC words and AzBio sentences in quiet and +10 dB SNR noise was evaluated 3 and 12 months after activation. Results: Speech perception in quiet and noise was similar regardless of whether or not the patient was eligible for EAS. Less than half of the patients who met the EAS criteria chose to use it, citing reasons such as physical discomfort or lack of perceived benefit. EAS users performed better on CNC words but not sentence recognition than EAS nonusers. Conclusions: EAS use is dependent on audiologic and nonaudiologic issues. Hearing preservation is possible with conventional electrodes, but hearing preservation alone does not guarantee superior speech perception. Audiol Neurotol

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Efficacy of Various Corticosteroid Treatment Modalities for the Initial Treatment of Idiopathic Sudden Hearing Loss: A Prospective Randomized Controlled Trial

B Tong,Q Wang,Q Dai,S Hellstrom,M Duan

Publicatie 15-07-2020

Objective: To evaluate the efficacy of systemic and intratympanic steroid treatment in patients with idiopathic sudden sensorineural hearing loss (ISSNHL). Methods: Ninety patients who met the inclusion criteria were randomly divided into 3 groups (n = 30 per group). Group I patients were given oral methylprednisolone (MEPD) tablets, group II received intravenous MEPD injection, and group III received intratympanic MEPD injection. Pure-tone average (PTA) hearing threshold was recorded before and after treatment as well as 1 month later. The general efficacy rate was the ratio of patients whose hearing improved by #x3e;10 dB in each group. Results: Thirty-two of the 60 patients (53.3%) in groups I and II together showed a hearing improvement of #x3e;10 dB hearing level (HL) in the pure-tone audiogram, compared to 21 patients (70%) in group III, respectively. The mean improvements were 16.1, 14.3, and 21.6 dB HL measured in Groups I, II, and III, respectively. Both the general efficacy rate and hearing improvement were significantly greater in group III than in groups I and II. Conclusions: Our study showed that intratympanic injection rendered better treatment efficacy than systemic administration. Audiol Neurotol

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Systematic Review of Quality of Life Assessments after Cochlear Implantation in Older Adults

E Andries,A Gilles,V Topsakal,OM Vanderveken,P Van de Heyning,V Van Rompaey,G Mertens

Publicatie 10-07-2020

Cochlear implants (CI) have increasingly been adopted in older adults with severe to profound sensorineural hearing loss as a result of the growing and aging world population. Consequently, researchers have recently shown great interest in the cost-effectiveness of cochlear implantation and its effect on quality of life (QoL) in older CI users. Therefore, a systematic review and critical evaluation of the available literature on QoL in older adult CI users was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies were selected by searching MEDLINE (PubMed) and the Cochrane Library and by checking the reference lists of relevant articles. Inclusion criteria were as follows: (1) the study sample were adults aged 50 years and older with postlingual onset of bilateral severe to profound hearing loss, (2) all subjects received a multi-electrode CI, and (3) QoL was assessed before and after implantation. Out of 1,093 records, 18 articles were accepted for review. Several studies demonstrated significant positive effects of cochlear implantation on QoL in older adults, but high-level evidence-based medicine is lacking. An improvement of QoL was generally reported when using disease-specific instruments, which are designed to detect treatment-specific changes, whereas the outcomes of generic QoL questionnaires, assessing general health states, were rather ambiguous. However, only generic questionnaires would be able to provide calculations of the cost-effectiveness of CI and comparisons across patient populations, diseases, or interventions. Hence, generic and disease-specific QoL instruments are complementary rather than contradictory. In general, older CI users’ QoL was assessed using a variety of methods and instruments, which complicated comparisons between studies. There is a need for a standardized, multidimensional, and comprehensive QoL study protocol including all relevant generic and disease-specific instruments to measure and compare QoL, utility, and/or daily life performance in CI users. Audiol Neurotol

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Fibrovascular Tumor-Like Lesions of the Facial Nerve

L Lassaletta,E Ruiz-Bravo,P García-Raya,A Fernández-Zubillaga,T González-Otero,J Gavilán

Publicatie 29-06-2020

Background: Facial nerve tumors (FNTs) are relatively rare benign lesions that arise from any segment of the facial nerve (FN). About half of all patients present with FN dysfunction, mainly long-standing or progressive facial paralysis. Diagnosis of an FNT is usually based on radiological imaging and confirmed by histological study. Most reported cases of FNTs are schwannomas and hemangiomas. Objectives: The aim of this study was to review 4 cases of lesions with clinical, radiological, and surgical findings that suggested an FNT, the pathology revealing a fibrovascular proliferation with no clear signs of a specific tumor. Method: Medical records of patients who had surgery due to an FN lesion were reviewed. Cases with known tumoral lesions were excluded. Four patients with tumor-like lesions were identified. Their imaging studies were re-evaluated. The pathological study included hematoxylin-eosin, Masson’s trichrome, and immunohistochemistry for S100 protein, neurofilaments, CD31, Wilms’ tumor 1 (WT1), and D240. Results: The 4 cases revealed tumor-like fibrovascular lesions that could not be classified as typical pathological entities. All cases had a complete facial palsy preoperatively. Computed tomography and magnetic resonance imaging (MRI) suggested schwannoma or hemangioma. A complete excision was achieved, and a facial reconstruction was performed immediately after interruption. Postoperative FN function was improved in all cases. The histological study showed nervous tracts of normal morphology, with fibrous and vascular tissue interspersed in variable proportions. All cases showed areas of fibrosis with Masson’s stain. In all cases, nervous tissue and Schwann cells tested positively for neurofilaments and S100, respectively. In vascular areas, endothelial cells stained positively for CD31, and negatively for D240 and WT1. Conclusions: Fibrovascular lesions of the FN may mimic primary FNTs, especially schwannomas and hemangiomas. Surgical excision with grafting or nerve transfer is the procedure of choice if a complete facial paralysis is found. This unusual condition should be considered when counseling patients with FN lesions. The lack of hyperintensity on MRI T2-weighted images may suggest the presence of fibrous tissue. Audiol Neurotol

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