Effective Hearing Loss Screening in Primary Care: The Early Auditory Referral-Primary Care Study.

PURPOSE: Hearing loss, the second most common disability in the United States, is under-diagnosed and under-treated. Identifying it in early stages could prevent its known substantial adverse outcomes. METHODS: A multiple baseline design was implemented to assess a screening paradigm for identifying and referring patients aged ≥55 years with hearing loss at 10 family medicine clinics in 2 health systems. Patients completed a consent form and the Hearing Handicap Inventory for the Elderly (HHI). An electronic alert prompted clinicians to screen for hearing loss during visits. RESULTS: The 14,877 eligible patients during the study period had 36,701 encounters. Referral rates in the family medicine clinics increased from a baseline rate of 3.2% to 14.4% in 1 health system and from a baseline rate of 0.7% to 4.7% in the other. A general medicine comparison group showed referral rate increase from the 3.0% baseline rate to 3.3%. Of the 5,883 study patients who completed the HHI 25.2% (n=1,484) had HHI scores suggestive of hearing loss; those patients had higher referral rates, 28% vs 9.2% (P <.001). Of 1,660 patients referred for hearing testing, 717 had audiology data available for analysis: 669 (93.3%) were rated appropriately referred and 421 (58.7%) were considered hearing aid candidates. Overall, 71.5% of patients contacted felt their referral was appropriate. CONCLUSION: An electronic alert used to remind clinicians to ask patients aged ≥55 years about hearing loss significantly increased audiology referrals for at-risk patients. Audiologic and audiogram data support the effectiveness of the prompt. Clinicians should consider adopting this method to identify patients with hearing loss to reduce its known and adverse sequelae.

Assessment of Intraoperative Nerve Monitoring Parameters Associated With Facial Nerve Outcome in Parotidectomy for Benign Disease.

Importance: Prior studies suggest that the use of facial nerve monitoring decreases the rate of immediate postoperative facial nerve weakness in parotid surgery, but published data are lacking on normative values for these parameters or cutoff values to prognosticate facial nerve outcomes. Objective: To identify intraoperative facial nerve monitoring parameters associated with postoperative weakness and to evaluate cutoff values for these parameters under which normal nerve function is more likely. Design, Setting, and Participants: This retrospective case series of 222 adult patients undergoing parotid surgery for benign disease performed with intraoperative nerve monitoring was conducted at an academic medical institution from September 13, 2004, to October 30, 2014. The data analysis was conducted from May 2018 to January 2019. Main Outcomes and Measures: The main outcome measure was facial nerve weakness. Receiver operating characteristic curves were generated to define optimal cut point to maximize the sensitivity and specificity of the stimulation threshold, mechanical events, and spasm events associated with facial nerve weakness. Results: Of 222 participants, 121 were women and 101 were men, with a mean (SD) age of 51 (16) years. The rate of temporary facial nerve paresis of any nerve branch was 45%, and the rate of permanent paralysis was 1.3%. The mean predissection threshold was 0.22 milliamperes (mA) (range, 0.1-0.6 mA) and the mean postdissection threshold was 0.24 mA (range, 0.08-1.0 mA). The average number of mechanical events was 9 (range, 0-66), and mean number of spontaneous spasm events was 1 (range, 0-12). Both the postdissection threshold (area under the curve [AUC], 0.69; 95% CI, 0.62-0.77) and the number of mechanical events (AUC, 0.58; 95% CI, 0.50-0.66) were associated with early postoperative facial nerve outcome. The number of spasm events was not associated with facial nerve outcome. The optimal cutoff value for the threshold was 0.25 mA, and the optimal cutoff for number of mechanical events was 8. If a threshold of greater than 0.25 mA was paired with more than 8 mechanical events, there was a 77% chance of postoperative nerve weakness. Conversely, if a threshold was 0.25 mA or less and there were 8 mechanical events or less, there was 69% chance of normal postoperative nerve function. No parameters were associated with permanent facial nerve injury. Conclusions and Relevance: Postdissection threshold and the number of mechanical events are associated with immediate postoperative facial nerve function. Accurate prediction of facial nerve function may provide anticipatory guidance to patients and may provide surgeons with intraoperative feedback allowing adjustment in operative techniques and perioperative management.

Electrocochleography: New Uses for an Old Test and Normative Values.

Purpose This article combines the results of 3 studies that were presented at the HeAL 2018 Conference in Lake Como, Italy, in June 2018. Each study involved electrocochleography (ECochG), a neurodiagnostic evaluation that has been used clinically for over 80 years but whose applications continue to expand. The 1st study describes recent research wherein ECochG was recorded from asymptomatic subjects who were siblings or offspring of patients with a confirmed diagnosis of Ménière's disease (MD). Our results provide evidence that ECochG may be helpful in not only diagnosing MD but also predicting it as well. Second, case studies are described where ECochG was important in both diagnosing superior semicircular canal dehiscence and monitoring the repair of this condition during surgery. Finally, although ECochG has been practiced clinically for over 8 decades, the protocols for recording, measuring, and interpreting the electrocochleogram continue to lack standardization among clinicians and scientists. We thus present normative data for some of these features based on noninvasive recordings made from the tympanic membrane from 100 normal hearing subjects. Conclusions Although the primary use of ECochG continues to be in the diagnosis of MD, we report on 2 additional clinical applications for this important test of inner ear/auditory nerve function. First, a preliminary study on a small sample of subjects indicates that ECochG may also be useful in predicting MD prior to the onset of symptoms in individuals who may be genetically predisposed to developing it. Second, through a series of case studies, we demonstrate how ECochG is used to help diagnose superior semicircular canal dehiscence and monitor the status of the inner ear during the surgical repair of this condition. Finally, normative values for clinically important components of the electrocochleogram based on tympanic membrane recordings have been established from a large sample of subjects.

Initial Results of the Early Auditory Referral-Primary Care (EAR-PC) Study.

INTRODUCTION: Hearing loss (HL) is the second most common disability in the U.S., yet is clinically underdiagnosed. To manage its common adverse psychosocial and cognitive outcomes, early identification of HL must be improved. METHODS: A feasibility study conducted to increase screening for HL and referral of patients aged ≥55 years arriving at two family medicine clinics. Eligible patients were asked to complete a self-administered consent form and the Hearing Handicap Inventory (HHI). Independently, clinicians received a brief educational program after which an electronic clinical prompt (intervention) alerted them (blinded to HHI results) to screen for HL during applicable patient visits. Pre- and post-intervention differences were analyzed to assess the proportion of patients referred to audiology and those diagnosed with HL (primary outcomes) and the audiology referral appropriateness (secondary outcome). Referral rates for those who screened positive for HL on the HHI were compared with those who scored negatively. RESULTS: There were 5,520 eligible patients during the study period, of which 1,236 (22.4%) consented. After the intervention's implementation, audiology referral rates increased from 1.2% to 7.1% (p<0.001). Overall, 293 consented patients (24%) completed the HHI and scored >10, indicating probable HL. Of these 293 patients, 28.0% were referred to audiology versus only 7.4% with scores <10 (p<0.001). Forty-two of the 54 referred patients seen by audiology were diagnosed with HL (78%). Overall, the diagnosis of HL on problem lists increased from 90 of 4,815 patients (1.9%) at baseline to 163 of 5,520 patients (3.0%, p<0.001) over only 8 months. CONCLUSIONS: The electronic clinical prompt significantly increased audiology referrals for at-risk patients for HL in two family medicine clinics. Larger-scale studies are needed to address the U.S. Preventive Services Task Force call to assess the long-term impact of HL screening in community populations.

Superior Canal Dehiscence Syndrome Affecting 3 Families.

Importance: Superior canal dehiscence syndrome (SCDS) is an increasingly recognized cause of hearing loss and vestibular symptoms, but the etiology of this condition remains unknown. Objective: To describe 7 cases of SCDS across 3 families. Design, Setting, and Participants: This retrospective case series included 7 patients from 3 different families treated at a neurotology clinic at a tertiary academic medical center from 2010 to 2014. Patients were referred by other otolaryngologists or were self-referred. Each patient demonstrated unilateral or bilateral SCDS or near dehiscence. Interventions: Clinical evaluation involved body mass index calculation, audiometry, cervical vestibular evoked myogenic potential testing, electrocochleography, and multiplanar computed tomographic (CT) scan of the temporal bones. Zygosity testing was performed on twin siblings. Main Outcomes and Measures: The diagnosis of SCDS was made if bone was absent over the superior semicircular canal on 2 consecutive CT images, in addition to 1 physiologic sign consistent with labyrinthine dehiscence. Near dehiscence was defined as absent bone on only 1 CT image but with symptoms and at least 1 physiologic sign of labyrinthine dehiscence. Results: A total of 7 patients (5 female and 2 male; age range, 8-49 years) from 3 families underwent evaluation. Family A consisted of 3 adult first-degree relatives, of whom 2 were diagnosed with SCDS and 1 with near dehiscence. Family B included a mother and her child, both of whom were diagnosed with unilateral SCDS. Family C consisted of adult monozygotic twins, each of whom was diagnosed with unilateral SCDS. For all cases, dehiscence was located at the arcuate eminence. Obesity alone did not explain the occurrence of SCDS because 5 of the 7 cases had a body mass index (calculated as weight in kilograms divided by height in meters squared) less than 30.0. Conclusions and Relevance: Superior canal dehiscence syndrome is a rare, often unrecognized condition. This report of 3 multiplex families with SCDS provides evidence in support of a potential genetic contribution to the etiology. Symptomatic first-degree relatives of patients diagnosed with SCDS should be offered evaluation to improve detection of this disorder.

Surgical treatment of hearing loss when otosclerosis coexists with superior semicircular canal dehiscence syndrome.

OBJECTIVE: Document a case of bilateral otosclerosis with coexisting bilateral superior semicircular canal dehiscence syndrome and the treatment of hearing loss in this setting. PATIENT: A 33-year-old woman presented with bilateral mixed hearing loss; worse in the left ear. This was gradual in onset, and she denied dizziness. Computerized tomographic scan revealed fenestral otosclerosis and a large dehiscence of the superior semicircular canal bilaterally. She declined amplification. INTERVENTION: Sequential laser-assisted stapedotomy with insertion of a Kurz titanium CliP Piston prosthesis. MAIN OUTCOME MEASURE: Comparison of audiovestibular symptoms, hearing thresholds, and neurodiagnostic testing results preoperatively and postoperatively. RESULTS: Hearing improved bilaterally with closure of the air-bone gaps at most frequencies, and she has not had permanent vestibular symptoms. Postoperative follow-up time is 37 months for the left ear and 13 months for the right ear. CONCLUSION: When otosclerosis and superior semicircular canal dehiscence syndrome coexist and hearing loss is the dominant symptom, stapes surgery can be effective for improving hearing without permanent vestibular symptoms.

Incomplete electrode extraction during cochlear implant revision.

OBJECTIVES: To report 3 occurrences of incomplete electrode extraction during cochlear implant revision, resulting in partial intracochlear retention of an implant electrode array. STUDY DESIGN: Retrospective case series. SETTING: Three tertiary care academic medical centers. PATIENTS: Three patients with cochlear implant failure necessitating revision cochlear implantation. INTERVENTION: Diagnosis and surgical revision of failed cochlear implants. RESULTS: A rare intraoperative complication was encountered in 3 pediatric patients undergoing revision cochlear implantation. During attempted explantation of the indwelling electrode, the array fractured and resulted in intracochlear retention of a distal portion of the electrode. Each case was managed differently by the operative team. CONCLUSION: Incomplete electrode extraction is a rare but significant complication of revision cochlear implantation that is generally not anticipated when counseling patients and families. Preoperative discussion of this potential complication is appropriate.

A psychophysical study of auditory and pressure sensitivity in patients with fibromyalgia and healthy controls.

UNLABELLED: Fibromyalgia (FM) is characterized by widespread tenderness. Studies have also reported that persons with FM are sensitive to other stimuli, such as auditory tones. We hypothesized that subjects with FM would display greater sensitivity to both pressure and auditory tones and report greater sensitivity to sounds encountered in daily activities. FM subjects (n = 30) and healthy control subjects (n = 28) were administered auditory tones and pressure using the same psychophysical methods to deliver the stimuli and a common way of scaling responses. Subjects were also administered a self-report questionnaire regarding sensitivity to everyday sounds. Participants with FM displayed significantly greater sensitivity to all levels of auditory stimulation (Ps < .05). The magnitude of difference between FM patients' lowered auditory sensitivity (relative to control subjects) was similar to that seen with pressure, and pressure and auditory ratings were significantly correlated in both control subjects and subjects with FM. FM patients also were more sensitive to everyday sounds (t = 8.65, P < .001). These findings support that FM is associated with a global central nervous system augmentation in sensory processing. Further research is needed to examine the neural substrates associated with this abnormality and its role in the etiology and maintenance of FM. PERSPECTIVE: Muscle tenderness is the hallmark of FM, but the findings of this study and others suggest that persons with FM display sensitivity to a number of sensory stimuli. These findings suggest that FM is associated with a global central nervous system augmentation of sensory information. These findings may also help to explain why persons with FM display a number of comorbid physical symptoms other than pain.

Transient evoked otoacoustic emissions pattern as a prognostic indicator for hearing preservation in acoustic neuroma surgery.

OBJECTIVE: To determine whether pre-operative transient otoacoustic emission (TEOAE) patterns are predictive of successful hearing preservation in acoustic neuroma surgery. STUDY DESIGN: Retrospective observational study. SETTING: Tertiary referral medical center. PATIENTS: A convenience sample was identified in whom pre-operative TEOAE data were available in patients undergoing acoustic neuroma surgery from 1993-2004. Ninety-three patients were identified who met this inclusion criterion. INTERVENTIONS: Subjects underwent attempted hearing preservation surgery via middle cranial fossa or retrosigmoid approaches. Routine audiometry, ABR, and TEOAE. MAIN OUTCOME MEASURES: Pre- and post-operative pure tone and speech results were categorized into hearing classes A, B, C, and D as described in the American Academy of Otolaryngology guidelines (1995). Hearing preservation was defined by maintenance of the pre-operative hearing class or downgrade to within one hearing class post-op. Pre-operative TEOAE results were divided into five frequency bands and described as positive in each band if there was a response above the noise floor with >50% reproducibility. RESULTS: Hearing was preserved in 51 patients (55%). Of these, 11 (22%) had positive TEOAE response in all five frequency bands measured (1, 1.5, 2, 3, 4 kHz), whereas 40 (78%) had TEOAE responses ranging from 0 to 4 frequency bands. 42 patients failed to preserve their hearing. Of these, only three (7%) had positive TEOAE in all five frequency bands, and 39 (93%) had TEOAE responses ranging from 0 to 4 frequency bands (p<0.05). Other variables of prognostic significance to hearing preservation in our series included smaller tumor size, tumor location within the IAC, better pre-operative hearing, and shorter latencies on ABR. Logistic regression was then used to compare the prognostic value of TEOAE against these variables. In our series, ABR latencies and 5 frequency band response on TEOAE showed the highest significant correlation to hearing preservation (p<0.05). CONCLUSION: A robust pre-operative TEOAE frequency band pattern may be used as a favorable prognostic indicator for potential hearing preservation in acoustic neuroma surgery. The prognostic value may be enhanced when combined with other prognostic factors such as tumor size, tumor location, pre-operative ABR and audiometric results.

Intraoperative neurophysiologic monitoring: indications and techniques for common procedures in otolaryngology-head and neck surgery.

Intraoperative cranial nerve monitoring can be an effective adjunct in otolaryngology-head and neck surgery. Monitoring is not considered standard of care, despite indications of cost effectiveness and improved functional outcomes. Lessons learned performing facial nerve monitoring are applicable to upper and lower cranial motor nerves. Auditory nerve monitoring can be modified accord-ing to need for selected otologic and neurotologic surgery. Process standardization and effective communication can lead to improved patient outcomes.

Prospective study of inner ear radiation dose and hearing loss in head-and-neck cancer patients.

PURPOSE: To determine the relationship between the radiation dose to the inner ear and long-term hearing loss. METHODS AND MATERIALS: Eligible patients included those receiving curative radiotherapy (RT) for head-and-neck cancer. After enrollment, patients underwent three-dimensional conformal RT planning and delivery (180-200 cGy/fraction) appropriate for their disease site and stage. The inner ear was contoured on axial CT planning images. Dose-volume histograms, as well as the mean and maximal dose for each structure, were calculated. Patients underwent pure tone audiometry at baseline (before treatment) and 1, 6, 12, 24, and 36 months after RT. The threshold level (the greater the value, the more hearing loss) in decibels was recorded for 250, 500, 1000, 2000, 4000, and 8000 Hz. For patients receiving predominantly unilateral RT, the contralateral ear served as the de facto control. The differences in threshold level between the ipsilateral and contralateral ears were calculated, and the temporal pattern and dose-response relation of hearing loss were analyzed using statistical methods that take into account the correlation between two ears in the same subject and repeated, sequential measurements of each subject. RESULTS: Of the 40 patients enrolled in this study, 35 qualified for analysis. Four patients who received concurrent chemotherapy and RT were analyzed separately. The 31 unilaterally treated patients received a median dose of 47.4 Gy (range, 14.1-68.8 Gy) to the ipsilateral inner ear and 4.2 Gy (range, 0.5-31.3 Gy) to the contralateral inner ear. Hearing loss was associated with the radiation dose received by the inner ear (loss of 210dB was observed in ears receiving >/=45 Gy) and was most appreciable in the higher frequencies (>/=2000 Hz). For a 60-year-old patient with no previous hearing loss in either ear, after receiving 45 Gy, the ipsilateral ear, according to our clinical model, would have a 19.3-dB (95% confidence interval [CI], 15.5-23.0) and 5.4-dB (95% CI, 3.5-7.5) hearing decrement compared with the contralateral ear for 8000 Hz and 1000 Hz, respectively. Age and an initial hearing difference within an ear pair also affected hearing loss. The baseline hearing threshold was inversely related to radiation-induced hearing loss. The degree of hearing loss was dependent on the frequency tested, age, baseline hearing, and baseline difference in hearing between a patient's two ears. CONCLUSION: High-frequency (>/=2000 Hz) hearing acuity worsens significantly after RT in a dose-dependent fashion. A larger number of patients needs to be studied to validate these results. This knowledge can be applied to create guidelines regarding future dose limits to the auditory apparatus for patients undergoing head-and-neck RT.

Prediction of long-term facial nerve outcomes with intraoperative nerve monitoring.

OBJECTIVE: Evaluate the utility of a statistical equation using two independent intraoperative monitoring parameters in predicting long-term facial nerve function. STUDY DESIGN: Retrospective case review. SETTING: Tertiary care hospital. PATIENTS: Sixty patients undergoing resection of vestibular schwannomas with intraoperative facial nerve monitoring at a single institution. INTERVENTION: All patients underwent microsurgical resection of vestibular schwannomas with the use of intraoperative cranial nerve monitoring. MAIN OUTCOME MEASURE: Final facial nerve outcome measured using the House-Brackmann scale at least 6 months after microsurgical resection. RESULTS: Five out of 60 (8.3%) patients demonstrated significant long-term weakness (i.e., House-Brackmann grade III or worse). Intraoperative monitoring parameters (proximal stimulation threshold, proximal-to-distal response amplitude ratio) were accurate in predicting increased risk of long-term facial nerve dysfunction when used in a logistic regression model. A Student's t test confirmed the equation result was statistically significant in differentiating long-term facial nerve outcomes. CONCLUSION: Patients with immediate weakness are at higher risk of having long-term poor facial nerve function. The use of intraoperative monitoring parameters was reliable in predicting facial nerve outcomes. Patients with permanent facial nerve dysfunction often require rehabilitative procedures. The ability to predict facial nerve outcomes with intraoperative monitoring may allow early rehabilitative procedures to improve quality of life and prevent ocular complications.

Longitudinal effects of Botox injections on voice-related quality of life (V-RQOL) for patients with adductory spasmodic dysphonia: part II.

OBJECTIVE: To investigate the longitudinal effects of botulinum toxin type A (Botox) injections on voice-related quality of life (V-RQOL) for patients with adductory spasmodic dysphonia. DESIGN: Prospective study. SETTING: Academic tertiary care referral center. PARTICIPANTS: Forty-two patients who presented to our institution with dysphonia and were diagnosed as having adductory spasmodic dysphonia during a 38-month period. INTERVENTION: Patients received Botox injections into both thyroarytenoid muscles via the cricothyroid membrane. The typical starting dose was 1.0 U per vocal fold. If necessary, the dosage was adjusted in subsequent injections to reduce adverse effects or to enhance duration of benefit. MAIN OUTCOME MEASURES: Patients filled out questionnaires, including the V-RQOL Measure and a self-assessed overall voice rating, before each injection. Postinjection questionnaires were completed 6 to 8 weeks after each treatment. Mean pretreatment and posttreatment scores were calculated for each treatment. RESULTS: The number of treatments per patient ranged from 1 to 7. Statistically significant improvements in mean total and domain V-RQOL scores were calculated for every injection (P<.01) (no postinjection questionnaires were available for the seventh injections). The magnitude of the effect remained constant for later injections. Eighty-two percent of the population recorded at least 1 category of improvement in overall self-assessed voice rating with each injection. CONCLUSIONS: Botox has a significant beneficial effect on V-RQOL for at least 6 injection cycles. This study demonstrates the efficacy of Botox for treating patients with adductory spasmodic dysphonia and further illustrates the usefulness and validity of the V-RQOL Measure in evaluating patients with dysphonia.

Intraoperative monitoring and facial nerve outcomes after vestibular schwannoma resection.

OBJECTIVE: To determine the predictive value of proximal facial nerve electrical threshold and proximal-to-distal facial muscle compound action potential amplitude ratio on facial nerve outcomes after resection of vestibular schwannomas. STUDY DESIGN: Retrospective case review. SETTING: Tertiary care hospital. PATIENTS: Two hundred twenty-nine patients undergoing resection of vestibular schwannomas with intraoperative facial nerve monitoring at a single institution. INTERVENTION: All patients underwent resection of vestibular schwannomas with the use of intraoperative monitoring. MAIN OUTCOME MEASURE: Facial nerve function was classified according to the House-Brackmann scale at the patient's last office follow-up. Last follow-up was at least 6 months after surgery. RESULTS: Good facial nerve function (House-Brackmann Grade I or II) was observed in 87% of the patients at their last office follow-up. Proximal-to-distal amplitude ratio and proximal electric threshold were statistically significant in predicting facial nerve outcome. A mathematical model predicting the probability of good outcome on the basis of the intraoperative parameters is presented. CONCLUSION: Intraoperative monitoring has significantly decreased facial nerve morbidity in vestibular schwannoma surgery. Despite the advances in surgery and monitoring, a group of patients still have poor facial nerve outcomes. The use of intraoperative nerve monitoring may be able to predict poor long-term facial nerve outcomes and thus modify the timing of rehabilitation.