Deep Brain Stimulation for Primary Refractory Tinnitus: A Systematic Review.

BACKGROUND: tinnitus is a common and often debilitating condition with limited evidence-based treatment options. Deep brain stimulation (DBS) is an approved treatment modality for certain neurological conditions; its experimental use as a treatment modality for severe tinnitus is novel and beginning to show promise. This systematic review focuses on the current evidence for the safety and efficacy of DBS for treatment of refractory tinnitus. METHODS: a systematic search in PubMed and EMBASE was performed to identify peer-reviewed studies on DBS of non-cortical structures for the primary indication of tinnitus treatment. Three studies were identified as meeting these criteria, one of which had two related sub-studies. RESULTS: seven patients with available data who underwent DBS for tinnitus were identified. DBS targets included nucleus accumbens (NAc), ventral anterior limb of the internal capsule (vALIC), caudate nucleus, and the medial geniculate body (MGB) of the thalamus. All studies used the Tinnitus Functional Index (TFI) as a primary outcome measure. DBS of the caudate was most commonly reported (n = 5), with a mean TFI improvement of 23.3 points. Only one subject underwent DBS targeting the NAc/vALIC (extrapolated TFI improvement 46.8) and one subject underwent DBS targeting the MGB (TFI improvement 59 points). CONCLUSIONS: DBS is a promising treatment option for refractory subjective tinnitus, with early data, from small patient cohorts in multiple studies, suggesting its safety and efficacy. Further studies with a larger patient population are needed to support this safety and efficacy before implementing this treatment to daily practice.

Auditory Outcomes Following Cochlear Implantation in Children with Unilateral Hearing Loss.

OBJECTIVE: Unilateral hearing loss (UHL) in children is associated with speech and language delays. Cochlear implantation (CI) is currently the only rehabilitative option that restores binaural hearing. This study aims to describe auditory outcomes in children who underwent CI for UHL and to determine the association between duration of hearing loss and auditory outcomes. STUDY DESIGN: Retrospective case series. SETTING: Three tertiary-level, academic institutions. PATIENTS: Children <18 years with UHL who underwent CI between 2018 and 2021. INTERVENTION: Cochlear implantation. MAIN OUTCOME MEASURES: Speech perception and Speech, Spatial and Qualities of Hearing Scale (SSQ) were assessed postimplantation. Scores >50% on speech perception and SSQ scores >8 points were considered satisfactory. Associations between duration of UHL and implantation age and outcomes were assessed using Spearman's rank correlation. RESULTS: Of the 38 children included, mean age at CI was 7.9 ± 3.2 years and mean UHL duration was 5.0 ± 2.8 years. Mean datalogging was 8.1 ± 3.1 hours/day. Mean auditory testing scores were SSQ, 7.9 ± 1.2; BABY BIO, 68.1 ± 30.2%; CNC, 38.4 ± 28.4%; WIPI, 52.5 ± 23.1%. Scores >50% on CNC testing were achieved by 40% of patients. SSQ scores >8 points were reported by 78% (7/9) of patients. There were no significant correlations between UHL duration and auditory outcomes. CONCLUSION: Overall, children with UHL who undergo CI can achieve satisfactory speech perception scores and SSQ scores. There were no associations between duration of hearing loss and age at implantation with auditory outcomes. Multiple variables may impact auditory outcomes, including motivation, family support, access to technology, and consistent isolated auditory training postactivation and should be taken into consideration in addition to age at implantation and duration of UHL in determination of CI candidacy.

Cochlear Implantation Outcomes in Pediatric Unilateral Hearing Loss: Impact of Device Use and Direct Input.

OBJECTIVE: To investigate the impact of daily cochlear implant (CI) use on speech perception outcomes among children with unilateral hearing loss (UHL). STUDY DESIGN: Multi-institutional retrospective case series of pediatric patients with UHL who underwent CI between 2018 to 2022. SETTING: Three tertiary children's hospitals. METHODS: Demographics were obtained including duration of deafness and age at CI. Best consonant-nucleus-consonant (CNC) word scores and data logs describing hours of CI usage were assessed postimplantation. Use of direct audio input (DAI) during rehabilitation was recorded. RESULTS: Twenty-seven children were included, with a mean age at CI of 7.8 years. Mean datalogging time was 7.8 ± 3.0 hours/day. 40.7% of children utilized daily DAI. The mean CNC score using the best score during the study period was 34.9%. There was no significant correlation between hours of CI usage and CNC score. There was a significant improvement in CNC score associated with whether the child used DAI during rehabilitation (CNC 50.91% [yes] vs 23.81% [no]), which remained significant when adjusting for age at CI, duration of deafness, and data log hours. CONCLUSION: Unlike children with bilateral hearing loss and CI, children with UHL and CI demonstrate no significant correlation between hours of daily CI usage and CNC scores. However, children who used DAI during postoperative rehabilitation achieved significantly higher CNC scores than those who did not. This suggests that rehabilitation focused on isolated listening with the implanted ear maybe critical in optimizing outcomes with CI in UHL patients.

American Cochlear Implant Alliance Task Force: Recommendations for Determining Cochlear Implant Candidacy in Adults.

The indications for cochlear implantation have expanded over time due to evidence demonstrating identification and implantation of appropriate cochlear implant (CI) candidates lead to significant improvements in speech recognition and quality of life (QoL). However, clinical practice is variable, with some providers using outdated criteria and others exceeding current labeled indications. As a results, only a fraction of those persons who could benefit from CI technology receive it. This document summarizes the current evidence for determining appropriate referrals for adults with bilateral hearing loss into CI centers for formal evaluation by stressing the importance of treating each ear individually and a "revised 60/60 rule". By mirroring contemporary clinical practice and available evidence, these recommendations will also provide a standardized testing protocol for CI candidates using a team-based approach that prioritizes individualized patient care. This manuscript was developed by the Adult Cochlear Implantation Candidacy Task Force of the American Cochlear Implant Alliance using review of the existing literature and clinical consensus. LEVEL OF EVIDENCE: N/A Laryngoscope, 134:S1-S14, 2024.

Predicting Acoustic Hearing Preservation Following Cochlear Implant Surgery Using Machine Learning.

OBJECTIVES: The aim of the study was to train and test supervised machine-learning classifiers to predict acoustic hearing preservation after CI using preoperative clinical data. STUDY DESIGN: Retrospective predictive modeling study of prospectively collected single-institution CI dataset. METHODS: One hundred and seventy-five patients from a REDCap database including 761 patients >18 years who underwent CI and had audiometric testing preoperatively and one month after surgery were included. The primary outcome variable was the lowest quartile change in acoustic hearing at one month after CI using various formulae (standard pure tone average, SPTA; low-frequency PTA, LFPTA). Analysis involved applying multivariate logistic regression to detect statistical associations and training and testing supervised learning classifiers. Classifier performance was assessed with numerous metrics including area under the receiver operating characteristic curve (AUC) and Matthews correlation coefficient (MCC). RESULTS: Lowest quartile change (indicating hearing preservation) in SPTA was positively associated with a history of meningitis, preoperative LFPTA, and preoperative SPTA. Lowest quartile change in SPTA was negatively associated with sudden hearing loss, noise exposure, aural fullness, and abnormal anatomy. Lowest quartile change in LFPTA was positively associated with preoperative LFPTA. Lowest quartile change in LFPTA was negatively associated with tobacco use. Random forest demonstrated the highest mean classification performance on the validation dataset when predicting each of the outcome variables. CONCLUSIONS: Machine learning demonstrated utility for predicting preservation of residual acoustic hearing in patients undergoing CI surgery, and the detected associations facilitated the interpretation of our machine-learning models. The models and statistical associations together may be used to facilitate improvements in shared clinical decision-making and patient outcomes. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:926-936, 2024.

Benefits of unilateral cochlear implantation in adults with asymmetric hearing loss: Audiologic and patient-related outcome measures.

PURPOSE: To investigate the benefits of cochlear implantation in adults with single-sided deafness (SSD) and asymmetric hearing loss (AHL). STUDY DESIGN: Prospective within-subjects repeated-measures. SETTING: Two tertiary cochlear implant centers. PATIENTS: Fourteen adults with severe-to-profound sensorineural hearing loss in the worse hearing ear and up to moderate SNHL in the better hearing ear. INTERVENTION: Cochlear implantation in the worse hearing ear. MAIN OUTCOME MEASURES: Consonant-nucleus-consonant (CNC) test, AzBio sentence test in noise, and lateralization testing were conducted preoperatively and at 3-, 6-, and 12-months post-activation. Patient-related outcomes were measured using the Speech, Spatial, and Qualities of Hearing Scale and Glasgow Benefit Inventory. Tinnitus Handicap Inventory was administered to subjects with tinnitus. RESULTS: Mean length of hearing loss in the worse hearing ear was 3.5 years. The mean CNC change scores from baseline were 54.8, 55.9, and 58.9 percentage points at 3-, 6-, and 12-months (p < 0.001). AzBio sentence test in noise demonstrated improved scores in all spatial configurations, although statistically significant in S0N0 (speech front, noise front) only. Lateralization testing showed significant improvement of 22.9, 24.5, and 24.0 percentage points at 3-, 6-, and 12 months post-activation (p = 0.002). All patient-related outcome measures revealed significant improvement. CONCLUSION: This study demonstrates improved speech perception in noise, sound lateralization, quality of life, and reduction in tinnitus perception in adults with SSD/AHL who undergo cochlear implantation. Our results add to the growing body of evidence that cochlear implant should be offered to this population.

Health-Related Quality of Life in Children With Unilateral Sensorineural Hearing Loss Following Cochlear Implantation.

OBJECTIVE: Evaluate health-related quality of life (HR-QOL) benefits with cochlear implantation (CI) in children with unilateral sensorineural hearing loss (USNHL) versus bilateral sensorineural hearing loss (BSNHL). STUDY DESIGN: A cross-sectional survey of parents of children who underwent CI for USNHL and BSNHL. SETTING: Tertiary care academic centers. METHODS: The "Children with cochlear implants: parental perspectives" survey was administered. Parents rated responses on a 5-point Likert scale. Scores greater than 3.0 were considered favorable. Responses were recorded within 8 domains and groups were compared with respect to domain scores. Analysis of covariance models was used to compare groups while adjusting for age at implantation and duration of implant use. RESULTS: There were 31 patients with USNHL and 27 patients with BSNHL. The average age of implantation in BSNHL patients was 1.9 and 6.7 years for USNHL. Parents of all children answered favorably in all domains. When adjusted for age at implantation and duration of implant use, parents of BSNHL children responded significantly more favorably only in 2 domains. When comparing patients with older age or prolonged duration of hearing loss in the USNHL cohort, there were favorable responses in all domains with no significant differences between groups. CONCLUSION: There are HR-QOL benefits of CI in USNHL children; less pronounced favorable results were noted only in 2 domains when compared to BSNHL children. Benefits were noted with CI in USNHL children at an older age at implantation or prolonged duration of hearing loss. Therefore, these factors should not be absolute contraindications for CI in USNHL.

Creating an Extraordinary Experience for Women Undergoing Cystoscopy: A Patient-Centered Approach to Process Improvement.

OBJECTIVE: To re-examine and improve the cystoscopy process for women based on patient input. While cystoscopy is a common urological procedure, women perceive it as invasive, personal, and fear-inducing. Patients want to be treated as individuals and not just another "procedure." METHODS: Women's perspectives on cystoscopy were collected using experience-based design. Observations and timings, emotion word lists, debrief forms, patient surveys, simulation, and interviews were used. A structured 2-day quality improvement event included both in-person and virtual patient participation to gain a deeper understanding of patients' perspectives. Ideas for process improvements were generated using brainstorming, creativity exercises, and prioritization. These changes were implemented and refined using an iterative process based on feedback. RESULTS: Patients who reported feeling grateful for the positive impact of their care tended to minimize procedure-associated wait times, inconvenience, and discomfort. Women in the evaluation phase of their treatment and those who were unhappy with their symptoms tended to magnify the negative emotions associated with their procedure. Patient feedback and areas for improvement specific to women's needs were identified. Actionable changes were implemented including engaging clinic staff, updating the cystoscopy workflow, and physical changes to enhance patient privacy. CONCLUSION: Identifying and addressing the needs of women undergoing cystoscopy improves satisfaction as their emotional, physical, and knowledge-based needs are addressed. Active participation in the health care process empowers patients to have a voice in their care. An extraordinary experience with cystoscopy may decrease anxiety of the unknown and help patients have control over the experience.

Speech perception abilities of adult cochlear implant listeners with single-sided deafness vs. bilateral hearing loss.

OBJECTIVES: The purpose of this study was to compare the speech perception abilities in adult cochlear implant recipients implanted for bilateral sensorineural hearing loss (BSNHL) with those implanted for single-sided deafness (SSD). DESIGN: A total of 12 adults with BSNHL and 12 adults with SSD participated. Each participant completed a battery of speech perception measures including monosyllabic words, sentences, and consonant recognition. RESULTS: Cochlear implant users with BSNHL performed higher on word and sentence recognition. Consonant recognition scores showed higher performance for CI listeners with BSNHL for voicing and manner, but not for place or articulation. CONCLUSIONS: Results of this study suggest that adults with SSD may have lower speech perception abilities with their cochlear implant when compared to adults implanted for BSNHL.

Upward Shifts in the Internal Representation of Frequency Can Persist Over a 3-Year Period for Cochlear Implant Patients Fit With a Relatively Short Electrode Array.

Patients fit with cochlear implants (CIs) commonly indicate at the time of device fitting and for some time after, that the speech signal sounds abnormal. A high pitch or timbre is one component of the abnormal percept. In this project, our aim was to determine whether a number of years of CI use reduced perceived upshifts in frequency spectrum and/or voice fundamental frequency. The participants were five individuals who were deaf in one ear and who had normal hearing in the other ear. The deafened ears had been implanted with a 18.5 mm electrode array which resulted in signal input frequencies being directed to locations in the spiral ganglion (SG) that were between one and two octaves higher than the input frequencies. The patients judged the similarity of a clean signal (a male-voice sentence) presented to their implanted ear and candidate, implant-like, signals presented to their normal-hearing (NH) ear. Matches to implant sound quality were obtained, on average, at 8 months after device activation (see section "Time 1") and at 35 months after activation (see section "Time 2"). At Time 1, the matches to CI sound quality were characterized, most generally, by upshifts in the frequency spectrum and in voice pitch. At Time 2, for four of the five patients, frequency spectrum values remained elevated. For all five patients F0 values remained elevated. Overall, the data offer little support for the proposition that, for patients fit with shorter electrode arrays, cortical plasticity nudges the cortical representation of the CI voice toward more normal, or less upshifted, frequency values between 8 and 35 months after device activation. Cortical plasticity may be limited when there are large differences between frequencies in the input signal and the locations in the SG stimulated by those frequencies.

Tumor-Related and Patient-Related Variables Affecting Length of Hospital Stay Following Vestibular Schwannoma Microsurgery.

OBJECTIVE: Review a single institution's vestibular schwannoma (VS) microsurgery experience to determine (1) correlations between demographics, comorbidities, and/or surgical approach on hospital length of stay (LOS) and discharge disposition and (2) trends in surgical approach over time. METHODS: Retrospective case series from a multidisciplinary skull base program at a tertiary care, academic hospital. All adult (>18 years) patients undergoing primary microsurgery for VS between 2008 and 2018 were included. RESULTS: A total of 147 subjects were identified. Surgical approach was split between middle fossa (MF) (16%), retrosigmoid (RS) (35%), and translabyrinthine (TL) (49%) craniotomies. For the 8% of patients had other than routine (OTR) discharge. Mean LOS was significantly longer for patients undergoing RS than either MF or TL. Brainstem compression by the tumor was associated with longer LOS as were diagnoses of chronic obstructive pulmonary disease (COPD) and peripheral vascular disease (PVD). For all discharges, the 40 to 50- and 50 to 60-year-old subgroups had significantly shorter LOS than the 70-years-and-older patients. For the 92% of patients routinely discharged, there was a significantly shorter LOS in the 40 to 50-year-olds compared to the 70-years-and-older patients. There was a significant shift in surgical approach from RS to TL over the study period. CONCLUSION: Over 90% of VS microsurgery patients were routinely discharged with a median hospital LOS of 3.2 days, both of which are consistent with published data. There is an inverse relationship between age and LOS with patients older than 70 years having significantly longer LOS. Brainstem compression, COPD, PVD, and the RS approach negatively affect LOS. LEVEL OF EVIDENCE: 4.

Comparing the Precision and Reliability Between Three Radiographic Techniques for Measuring Sporadic Vestibular Schwannomas.

RATIONALE AND OBJECTIVES: Several methods exist for measuring vestibular schwannoma (VS) size radiographically. Our aim was to compare the precision and reproducibility of three different radiographic measurement techniques for assessing VS tumor size. MATERIAL AND METHODS: Twenty patients with unilateral, sporadic VS previously untreated were identified. All patients had thin-slice T1 weighted, postcontrasted magnetization prepared rapid acquisition gradient echo images. Three measurement techniques were performed using within-subject and between-subject comparison. Experimental comparison of interobserver agreement between techniques was calculated. Interobserver intraclass correlation coefficients, repeatability coefficients, and relative smallest detectable difference were calculated and compared. RESULTS: Mean tumor measurements were: 10.3 mm (maximum linear dimension, [MLD]), 495.9 mm3 (orthogonal volumetric analysis, [OVA]), and 572.1 mm3 (segmented volumetric analysis, [SVA]). Interobserver correlation coefficient was excellent for all measurement techniques, but highest for segmented volumetric analysis. Repeatability coefficient was 1.44 mm for MLD, 298.9 mm3 for OVA, and 174.8 mm3 for SVA. The smallest detectable difference was 13.9% for MLD, 60.2% for OVA, and 30.6% for SVA. A subgroup analysis was performed for small tumors (<14 mm) and large tumors (>14 mm) and demonstrated increased precision of segmented volumetric analysis for larger tumors. CONCLUSION: Semi-automated segmented volumetric analysis appears more precise than either linear measurement or orthogonal volumetric analysis for reporting VS tumor size, and becomes increasingly precise for larger tumors. Tumor volume and tumor volume change over time using SVA may be more sensitive in surveilling VS than current measurement techniques.

Intratympanic Steroid Injection Complicated by Iatrogenic Perilymphatic Fistula: A Cautionary Tale.

Intratympanic (IT) steroid therapy is a mainstay treatment for sudden sensorineural hearing loss (SSNHL) for both initial therapy and salvage therapy. We report a rare case of iatrogenic perilymphatic fistula that resulted from trauma during an IT steroid injection for SSNHL. We discuss the diagnosis and treatment in the current case and compare it with previous reports from the literature. Laryngoscope, 131:2088-2090, 2021.

Testing Speech Perception with Cochlear Implants Through Digital Audio Streaming in a Virtual Sound Booth: A Feasibility Study.

OBJECTIVE: For patients who have received cochlear implants (CIs), speech-perception testing requires specialized equipment. This limits locations where these services can be provided, which can introduce barriers for provision of care. Providing speech test stimuli directly to the CI via wireless digital audio streaming (DAS) or wired direct audio input (DAI) allows for testing without the need for a sound booth (SB). A few studies have investigated the use of DAI for testing speech perception in CIs, but none have evaluated DAS. The goal of this study was to compare speech perception testing in CI users via DAS versus a traditional SB to determine if differences exist between the two presentation modes. We also sought to determine whether pre-processing the DAS signal with room acoustics (reverberation and noise floor) to emulate the SB environment would affect performance differences between the SB and DAS. DESIGN: In Experiment 1, speech perception was measured for monosyllabic words in quiet and sentences in quiet and in noise. Scores were obtained in a SB and compared to those obtained via DAS with unprocessed speech (DAS-U) for 11 adult CI users (12 ears). In Experiment 2, speech perception was measured for sentences in noise, where both the speech and noise stimuli were pre-processed to emulate the SB environment. Scores were obtained for 11 adult CI users (12 ears) in the SB, via DAS-U, and via DAS with the processed speech (DAS-P). RESULTS: For Experiment 1, there was no significant difference between SB and DAS-U conditions for words or sentences in quiet. However, DAS-U scores were significantly better than SB scores for sentences in noise. For Experiment 2, there was no significant difference between the SB and DAS-P conditions. Similar to Experiment 1, DAS-U scores were significantly better than SB or DAS-P scores. CONCLUSIONS: By pre-processing the test materials to emulate the noise and reverberation characteristics of a traditional SB, we can account for differences in speech-perception scores between those obtained via DAS and in a SB.

Cochlear Place of Stimulation Is One Determinant of Cochlear Implant Sound Quality.

OBJECTIVE: Our aim was to determine the effect of acute changes in cochlear place of stimulation on cochlear implant (CI) sound quality. DESIGN: In Experiment 1, 5 single-sided deaf (SSD) listeners fitted with a long (28-mm) electrode array were tested. Basal shifts in place of stimulation were implemented by turning off the most apical electrodes and reassigning the filters to more basal electrodes. In Experiment 2, 2 SSD patients fitted with a shorter (16.5-mm) electrode array were tested. Both basal and apical shifts in place of stimulation were implemented. The apical shifts were accomplished by current steering and creating a virtual place of stimulation more apical that that of the most apical electrode. RESULTS: Listeners matched basal shifts by shifting, in the normal-hearing ear, the overall spectrum up in frequency and/or increasing voice pitch (F0). Listeners matched apical shifts by shifting down the overall frequency spectrum in the normal-hearing ear. CONCLUSION: One factor determining CI voice quality is the location of stimulation along the cochlear partition.

Looking for Mickey Mouse™ But Finding a Munchkin: The Perceptual Effects of Frequency Upshifts for Single-Sided Deaf, Cochlear Implant Patients.

Purpose Our aim was to make audible for normal-hearing listeners the Mickey Mouse™ sound quality of cochlear implants (CIs) often found following device activation. Method The listeners were 3 single-sided deaf patients fit with a CI and who had 6 months or less of CI experience. Computed tomography imaging established the location of each electrode contact in the cochlea and allowed an estimate of the place frequency of the tissue nearest each electrode. For the most apical electrodes, this estimate ranged from 650 to 780 Hz. To determine CI sound quality, a clean signal (a sentence) was presented to the CI ear via a direct connect cable and candidate, and CI-like signals were presented to the ear with normal hearing via an insert receiver. The listeners rated the similarity of the candidate signals to the sound of the CI on a 1- to 10-point scale, with 10 being a complete match. Results To make the match to CI sound quality, all 3 patients need an upshift in formant frequencies (300-800 Hz) and a metallic sound quality. Two of the 3 patients also needed an upshift in voice pitch (10-80 Hz) and a muffling of sound quality. Similarity scores ranged from 8 to 9.7. Conclusion The formant frequency upshifts, fundamental frequency upshifts, and metallic sound quality experienced by the listeners can be linked to the relatively basal locations of the electrode contacts and short duration experience with their devices. The perceptual consequence was not the voice quality of Mickey Mouse™ but rather that of Munchkins in The Wizard of Oz for whom both formant frequencies and voice pitch were upshifted. Supplemental Material https://doi.org/10.23641/asha.9341651.

Clinical Practice Guideline: Sudden Hearing Loss (Update) Executive Summary.

OBJECTIVE: Sudden hearing loss is a frightening symptom that often prompts an urgent or emergent visit to a health care provider. It is frequently, but not universally, accompanied by tinnitus and/or vertigo. Sudden sensorineural hearing loss affects 5 to 27 per 100,000 people annually, with about 66,000 new cases per year in the United States. This guideline update provides evidence-based recommendations for the diagnosis, management, and follow-up of patients who present with sudden hearing loss. It focuses on sudden sensorineural hearing loss in adult patients aged 18 and over and primarily on those with idiopathic sudden sensorineural hearing loss. Prompt recognition and management of sudden sensorineural hearing loss may improve hearing recovery and patient quality of life. The guideline update is intended for all clinicians who diagnose or manage adult patients who present with sudden hearing loss. PURPOSE: The purpose of this guideline update is to provide clinicians with evidence-based recommendations in evaluating patients with sudden hearing loss and sudden sensorineural hearing loss, with particular emphasis on managing idiopathic sudden sensorineural hearing loss. The guideline update group recognized that patients enter the health care system with sudden hearing loss as a nonspecific primary complaint. Therefore, the initial recommendations of this guideline update address distinguishing sensorineural hearing loss from conductive hearing loss at the time of presentation with hearing loss. They also clarify the need to identify rare, nonidiopathic sudden sensorineural hearing loss to help separate those patients from those with idiopathic sudden sensorineural hearing loss, who are the target population for the therapeutic interventions that make up the bulk of the guideline update. By focusing on opportunities for quality improvement, this guideline should improve diagnostic accuracy, facilitate prompt intervention, decrease variations in management, reduce unnecessary tests and imaging procedures, and improve hearing and rehabilitative outcomes for affected patients. METHODS: Consistent with the American Academy of Otolaryngology-Head and Neck Surgery Foundation's Clinical Practice Guideline Development Manual, Third Edition, the guideline update group was convened with representation from the disciplines of otolaryngology-head and neck surgery, otology, neurotology, family medicine, audiology, emergency medicine, neurology, radiology, advanced practice nursing, and consumer advocacy. A systematic review of the literature was performed, and the prior clinical practice guideline on sudden hearing loss was reviewed in detail. Key action statements (KASs) were updated with new literature, and evidence profiles were brought up to the current standard. Research needs identified in the original clinical practice guideline and data addressing them were reviewed. Current research needs were identified and delineated. RESULTS: The guideline update group made strong recommendations for the following: clinicians should distinguish sensorineural hearing loss from conductive hearing loss when a patient first presents with sudden hearing loss (KAS 1); clinicians should educate patients with sudden sensorineural hearing loss about the natural history of the condition, the benefits and risks of medical interventions, and the limitations of existing evidence regarding efficacy (KAS 7); and clinicians should counsel patients with sudden sensorineural hearing loss who have residual hearing loss and/or tinnitus about the possible benefits of audiological rehabilitation and other supportive measures (KAS 13). These strong recommendations were modified from the initial clinical practice guideline for clarity and timing of intervention. The guideline update group made strong recommendation against the following: clinicians should not order routine computed tomography of the head in the initial evaluation of a patient with presumptive sudden sensorineural hearing loss (KAS 3); clinicians should not obtain routine laboratory tests in patients with sudden sensorineural hearing loss (KAS 5); and clinicians should not routinely prescribe antivirals, thrombolytics, vasodilators, or vasoactive substances to patients with sudden sensorineural hearing loss (KAS 11). The guideline update group made recommendations for the following: clinicians should assess patients with presumptive sudden sensorineural hearing loss through history and physical examination for bilateral sudden hearing loss, recurrent episodes of sudden hearing loss, and/or focal neurologic findings (KAS 2); in patients with sudden hearing loss, clinicians should obtain, or refer to a clinician who can obtain, audiometry as soon as possible (within 14 days of symptom onset) to confirm the diagnosis of sudden sensorineural hearing loss (KAS 4); clinicians should evaluate patients with sudden sensorineural hearing loss for retrocochlear pathology by obtaining a magnetic resonance imaging or auditory brainstem response (KAS 6); clinicians should offer, or refer to a clinician who can offer, intratympanic steroid therapy when patients have incomplete recovery from sudden sensorineural hearing loss 2 to 6 weeks after onset of symptoms (KAS 10); and clinicians should obtain follow-up audiometric evaluation for patients with sudden sensorineural hearing loss at the conclusion of treatment and within 6 months of completion of treatment (KAS 12). These recommendations were clarified in terms of timing of intervention and audiometry, as well as method of retrocochlear workup. The guideline update group offered the following KASs as options: clinicians may offer corticosteroids as initial therapy to patients with sudden sensorineural hearing loss within 2 weeks of symptom onset (KAS 8); clinicians may offer, or refer to a clinician who can offer, hyperbaric oxygen therapy combined with steroid therapy within 2 weeks of onset of sudden sensorineural hearing loss (KAS 9a); and clinicians may offer, or refer to a clinician who can offer, hyperbaric oxygen therapy combined with steroid therapy as salvage therapy within 1 month of onset of sudden sensorineural hearing loss (KAS 9b). DIFFERENCES FROM PRIOR GUIDELINE: Incorporation of new evidence profiles to include quality improvement opportunities, confidence in the evidence, and differences of opinion Included 10 clinical practice guidelines, 29 new systematic reviews, and 36 new randomized controlled trials Highlights the urgency of evaluation and initiation of treatment, if treatment is offered, by emphasizing the time from symptom occurrence Clarification of terminology by changing potentially unclear statements; use of the term sudden sensorineural hearing loss to mean idiopathic sudden sensorineural hearing loss to emphasize that over 90% of sudden sensorineural hearing loss is idiopathic sudden sensorineural hearing loss and to avoid confusion in nomenclature for the reader Changes to the key action statements (KASs) from the original guideline: KAS 1: When a patient first presents with sudden hearing loss, conductive hearing loss should be distinguished from sensorineural. KAS 2: The utility of history and physical examination when assessing for modifying factors is emphasized. KAS 3: The word routine is added to clarify that this statement addresses a nontargeted head computed tomography scan that is often ordered in the emergency room setting for patients presenting with sudden hearing loss. It does not refer to targeted scans such as a temporal bone computed tomography scan to assess for temporal bone pathology. KAS 4: The importance of audiometric confirmation of hearing status as soon as possible and within 14 days of symptom onset is emphasized. KAS 5: New studies were added to confirm the lack of benefit of nontargeted laboratory testing in sudden sensorineural hearing loss. KAS 6: Audiometric follow-up is excluded as a reasonable workup for retrocochlear pathology. Magnetic resonance imaging, computed tomography scan if magnetic resonance imaging cannot be done, or, secondarily, auditory brainstem response evaluation are the modalities recommended. A time frame for such testing is not specified, nor is it specified which clinician should be ordering this workup; however, it is implied that it would be the general or subspecialty otolaryngologist. KAS 7: The importance of shared decision making is highlighted, and salient points are emphasized. KAS 8: The option for corticosteroid intervention within 2 weeks of symptom onset is emphasized. KAS 9: Changed to KAS 9a and 9b; hyperbaric oxygen therapy remains an option but only when combined with steroid therapy for either initial treatment (9a) or for salvage therapy (9b). The timing is within 2 weeks of onset for initial therapy and within 1 month of onset of sudden sensorineural hearing loss for salvage therapy. KAS 10: Intratympanic steroid therapy for salvage is recommended within 2 to 6 weeks following onset of sudden sensorineural hearing loss. The time to treatment is defined and emphasized. KAS 11: Antioxidants were removed from the list of interventions that the clinical practice guideline recommends against using. KAS 12: Follow-up audiometry at conclusion of treatment and also within 6 months posttreatment is added. KAS 13: This statement on audiologic rehabilitation includes patients who have residual hearing loss and/or tinnitus who may benefit from treatment. Addition of an algorithm outlining KASs Enhanced emphasis on patient education and shared decision making with tools provided to assist in the same.

Clinical Practice Guideline: Sudden Hearing Loss (Update).

OBJECTIVE: Sudden hearing loss is a frightening symptom that often prompts an urgent or emergent visit to a health care provider. It is frequently but not universally accompanied by tinnitus and/or vertigo. Sudden sensorineural hearing loss affects 5 to 27 per 100,000 people annually, with about 66,000 new cases per year in the United States. This guideline update provides evidence-based recommendations for the diagnosis, management, and follow-up of patients who present with sudden hearing loss. It focuses on sudden sensorineural hearing loss in adult patients aged ≥18 years and primarily on those with idiopathic sudden sensorineural hearing loss. Prompt recognition and management of sudden sensorineural hearing loss may improve hearing recovery and patient quality of life. The guideline update is intended for all clinicians who diagnose or manage adult patients who present with sudden hearing loss. PURPOSE: The purpose of this guideline update is to provide clinicians with evidence-based recommendations in evaluating patients with sudden hearing loss and sudden sensorineural hearing loss, with particular emphasis on managing idiopathic sudden sensorineural hearing loss. The guideline update group recognized that patients enter the health care system with sudden hearing loss as a nonspecific primary complaint. Therefore, the initial recommendations of this guideline update address distinguishing sensorineural hearing loss from conductive hearing loss at the time of presentation with hearing loss. They also clarify the need to identify rare, nonidiopathic sudden sensorineural hearing loss to help separate those patients from those with idiopathic sudden sensorineural hearing loss, who are the target population for the therapeutic interventions that make up the bulk of the guideline update. By focusing on opportunities for quality improvement, this guideline should improve diagnostic accuracy, facilitate prompt intervention, decrease variations in management, reduce unnecessary tests and imaging procedures, and improve hearing and rehabilitative outcomes for affected patients. METHODS: Consistent with the American Academy of Otolaryngology-Head and Neck Surgery Foundation's "Clinical Practice Guideline Development Manual, Third Edition" (Rosenfeld et al. Otolaryngol Head Neck Surg. 2013;148[1]:S1-S55), the guideline update group was convened with representation from the disciplines of otolaryngology-head and neck surgery, otology, neurotology, family medicine, audiology, emergency medicine, neurology, radiology, advanced practice nursing, and consumer advocacy. A systematic review of the literature was performed, and the prior clinical practice guideline on sudden hearing loss was reviewed in detail. Key Action Statements (KASs) were updated with new literature, and evidence profiles were brought up to the current standard. Research needs identified in the original clinical practice guideline and data addressing them were reviewed. Current research needs were identified and delineated. RESULTS: The guideline update group made strong recommendations for the following: (KAS 1) Clinicians should distinguish sensorineural hearing loss from conductive hearing loss when a patient first presents with sudden hearing loss. (KAS 7) Clinicians should educate patients with sudden sensorineural hearing loss about the natural history of the condition, the benefits and risks of medical interventions, and the limitations of existing evidence regarding efficacy. (KAS 13) Clinicians should counsel patients with sudden sensorineural hearing loss who have residual hearing loss and/or tinnitus about the possible benefits of audiologic rehabilitation and other supportive measures. These strong recommendations were modified from the initial clinical practice guideline for clarity and timing of intervention. The guideline update group made strong recommendations against the following: (KAS 3) Clinicians should not order routine computed tomography of the head in the initial evaluation of a patient with presumptive sudden sensorineural hearing loss. (KAS 5) Clinicians should not obtain routine laboratory tests in patients with sudden sensorineural hearing loss. (KAS 11) Clinicians should not routinely prescribe antivirals, thrombolytics, vasodilators, or vasoactive substances to patients with sudden sensorineural hearing loss. The guideline update group made recommendations for the following: (KAS 2) Clinicians should assess patients with presumptive sudden sensorineural hearing loss through history and physical examination for bilateral sudden hearing loss, recurrent episodes of sudden hearing loss, and/or focal neurologic findings. (KAS 4) In patients with sudden hearing loss, clinicians should obtain, or refer to a clinician who can obtain, audiometry as soon as possible (within 14 days of symptom onset) to confirm the diagnosis of sudden sensorineural hearing loss. (KAS 6) Clinicians should evaluate patients with sudden sensorineural hearing loss for retrocochlear pathology by obtaining magnetic resonance imaging or auditory brainstem response. (KAS 10) Clinicians should offer, or refer to a clinician who can offer, intratympanic steroid therapy when patients have incomplete recovery from sudden sensorineural hearing loss 2 to 6 weeks after onset of symptoms. (KAS 12) Clinicians should obtain follow-up audiometric evaluation for patients with sudden sensorineural hearing loss at the conclusion of treatment and within 6 months of completion of treatment. These recommendations were clarified in terms of timing of intervention and audiometry and method of retrocochlear workup. The guideline update group offered the following KASs as options: (KAS 8) Clinicians may offer corticosteroids as initial therapy to patients with sudden sensorineural hearing loss within 2 weeks of symptom onset. (KAS 9a) Clinicians may offer, or refer to a clinician who can offer, hyperbaric oxygen therapy combined with steroid therapy within 2 weeks of onset of sudden sensorineural hearing loss. (KAS 9b) Clinicians may offer, or refer to a clinician who can offer, hyperbaric oxygen therapy combined with steroid therapy as salvage therapy within 1 month of onset of sudden sensorineural hearing loss. DIFFERENCES FROM PRIOR GUIDELINE: Incorporation of new evidence profiles to include quality improvement opportunities, confidence in the evidence, and differences of opinion Included 10 clinical practice guidelines, 29 new systematic reviews, and 36 new randomized controlled trials Highlights the urgency of evaluation and initiation of treatment, if treatment is offered, by emphasizing the time from symptom occurrence Clarification of terminology by changing potentially unclear statements; use of the term sudden sensorineural hearing loss to mean idiopathic sudden sensorineural hearing loss to emphasize that >90% of sudden sensorineural hearing loss is idiopathic sudden sensorineural hearing loss and to avoid confusion in nomenclature for the reader Changes to the KASs from the original guideline: KAS 1-When a patient first presents with sudden hearing loss, conductive hearing loss should be distinguished from sensorineural. KAS 2-The utility of history and physical examination when assessing for modifying factors is emphasized. KAS 3-The word "routine" is added to clarify that this statement addresses nontargeted head computerized tomography scan that is often ordered in the emergency room setting for patients presenting with sudden hearing loss. It does not refer to targeted scans, such as temporal bone computerized tomography scan, to assess for temporal bone pathology. KAS 4-The importance of audiometric confirmation of hearing status as soon as possible and within 14 days of symptom onset is emphasized. KAS 5-New studies were added to confirm the lack of benefit of nontargeted laboratory testing in sudden sensorineural hearing loss. KAS 6-Audiometric follow-up is excluded as a reasonable workup for retrocochlear pathology. Magnetic resonance imaging, computerized tomography scan if magnetic resonance imaging cannot be done, and, secondarily, auditory brainstem response evaluation are the modalities recommended. A time frame for such testing is not specified, nor is it specified which clinician should be ordering this workup; however, it is implied that it would be the general or subspecialty otolaryngologist. KAS 7-The importance of shared decision making is highlighted, and salient points are emphasized. KAS 8-The option for corticosteroid intervention within 2 weeks of symptom onset is emphasized. KAS 9-Changed to KAS 9A and 9B. Hyperbaric oxygen therapy remains an option but only when combined with steroid therapy for either initial treatment (9A) or salvage therapy (9B). The timing of initial therapy is within 2 weeks of onset, and that of salvage therapy is within 1 month of onset of sudden sensorineural hearing loss. KAS 10-Intratympanic steroid therapy for salvage is recommended within 2 to 6 weeks following onset of sudden sensorineural hearing loss. The time to treatment is defined and emphasized. KAS 11-Antioxidants were removed from the list of interventions that the clinical practice guideline recommends against using. KAS 12-Follow-up audiometry at conclusion of treatment and also within 6 months posttreatment is added. KAS 13-This statement on audiologic rehabilitation includes patients who have residual hearing loss and/or tinnitus who may benefit from treatment. Addition of an algorithm outlining KASs Enhanced emphasis on patient education and shared decision making with tools provided to assist in same.

Cochlear Implantation for Single-Sided Deafness: A New Treatment Paradigm.

Unilateral severe-to-profound sensorineural hearing loss (SNHL), also known as single sided deafness (SSD), is a problem that affects both children and adults, and can have severe and detrimental effects on multiple aspects of life including music appreciation, speech understanding in noise, speech and language acquisition, performance in the classroom and/or the workplace, and quality of life. Additionally, the loss of binaural hearing in SSD patients affects those processes that rely on two functional ears including sound localization, binaural squelch and summation, and the head shadow effect. Over the last decade, there has been increasing interest in cochlear implantation for SSD to restore binaural hearing. Early data are promising that cochlear implantation for SSD can help to restore binaural functionality, improve quality of life, and may faciliate reversal of neuroplasticity related to auditory deprivation in the pediatric population. Additionally, this new patient population has allowed researchers the opportunity to investigate the age-old question "what does a cochlear implant (CI) sound like?."