Oval and round window reinforcement surgery leads to improvements in sound tolerance and quality of life for hyperacusis patients.

PURPOSE: Hyperacusis is an audiological disorder in which patients become persistently sensitive and intolerant to everyday environmental sounds. For those patients that fail conservative options, a minimally invasive surgical procedure has been developed. MATERIALS & METHODS: Retrospective case series of 73 adult patients with hyperacusis who underwent oval and round window reinforcement surgery between 1/2017-6/2023. Small pieces of temporalis fascia were used to reinforce the round and oval windows. Patients were separated into two groups based on their preoperative speech Loudness Discomfort Level (LDL). Patients with a preoperative speech LDL ≤ 70 dB were placed in the "low LDL group" whereas patients with a preoperative speech LDL >70 dB were placed in the "high LDL group." Preoperative and one-week postoperative audiogram and speech LDLs were compared. Quality of life was assessed using the Glasgow Benefit Inventory (GBI) survey. RESULTS: 73 patients met inclusion criteria - 21 patients in the low LDL group and 52 in the high LDL group. Patients in the high LDL group significantly improved their LDLs by an average of 3.5 dB (P < 0.0001). 42 patients (80.8 %) in the high LDL group had improvement and would recommend the surgery for hyperacusis. Patients in the low LDL group significantly improved their LDL by an average of 12.9 dB (P = 0.032). Ten patients (47.6 %) from the low LDL group experienced improvement and would recommend hyperacusis surgery. CONCLUSION: Many patients with hyperacusis who undergo oval and round window reinforcement can receive significant improvement in sound tolerance and quality of life. Patients with a pre-op speech LDL > 70 dB have the greatest potential for improvement with surgery (80.8 %), probably because their hyperacusis was less severe. In the high LDL group(>70dB) the improvement in 1-10 scale went from 8.6 pre-op to 2.4 post op. In the low LDL group(<70dB) went from 9.2 pre-op to 6.8 post-op. These findings were consistent with the GBI results.

Outcomes of transtympanic dexamethasone perfusion using the MicroWickTM in patients with Ménière's disease: A cross-sectional study.

PURPOSE: To assess the efficacy of a 4-week transtympanic dexamethasone perfusion using the Silverstein MicroWickTM in patients with Ménière's disease. MATERIALS AND METHODS: A self-reported questionnaire was designed and sent to patients who underwent transtympanic dexamethasone perfusion using the Silverstein MicroWickTM from January 2017 to December 2020. A retrospective chart review was conducted to gather demographic and audiological data of those who responded. RESULTS: Forty respondents were separated into Group 1 (n = 34), who required no further procedure, and Group 2 (n = 6), who required additional procedure for Ménière's disease. In Group 1, 50% reported subjective improvement in tinnitus, 59% in aural fullness, 79% in vertigo, and 21% in hearing loss after the MicroWickTM treatment. A statistical analysis of the scores revealed that the improvement in aural fullness and vertigo met significance (p = 0.03 and p = 0.002, respectively). In Group 2, no significant change was seen in their symptoms. Audiological data showed no significant change in the pure tone average or the word recognition score after the treatment. CONCLUSION: Transtympanic dexamethasone perfusion using the Silverstein MicroWickTM is a well-tolerated treatment option for patients with Ménière's disease. Our survey data suggest its significant efficacy in reducing aural fullness and vertigo attacks in these patients. Prospective studies will be conducted to further establish its potential role in successfully managing patients with Ménière's disease.

Minimally invasive surgery for the treatment of hyperacusis: New technique and long term results.

OBJECTIVE: A minimally invasive surgery developed by the senior author has previously been reported to significantly improve sound tolerance after surgery. This report compares the new versus original surgical technique used and long-term results of all patients who have undergone minimally invasive surgery for hyperacusis. STUDY DESIGN: A prospective, IRB approved clinical research trial at a single institution with surgery performed by the author (HS). SETTING: All patients were evaluated and treated at a tertiary level otologic referral center. SUBJECTS AND METHODS: 47 subjects were enrolled from 2014 through 2019, 40 met inclusion criteria including adequate follow-up in the analysis. All subjects underwent oval and round window reinforcement. 20 subjects underwent surgery before 2017 with the original technique of round window reinforcement. 20 subjects underwent new technique with additional oval window and stapes reinforcement. RESULTS: 80% of subjects who underwent the new surgical technique had improvement in hyperacusis symptoms after surgery compared to 60% of subjects who underwent the original technique. Long term follow-up showed sustained results with both techniques with a mean follow-up of 2 years after surgery. CONCLUSIONS: The most recent, newer technique employed appears to have an 80% success rate in improving sound tolerance with small changes to hearing. The improvement in hyperacusis symptoms after surgery is significant and now found to be sustainable with a mean follow-up of 2 years after initial surgery. Psychological measures of anxiety and depression also were found to be significantly improved after surgery in the newer technique group.

Stapes hypermobility as a possible cause of hyperacusis.

OBJECTIVE: Hyperacusis is a reduction of normal tolerances for everyday sounds. Although several publications have been produced demonstrating that minimally invasive surgical procedures may improve patient symptoms, the precise etiology of hyperacusis often remains elusive. This study describes 21 patients, 7 of whom stapes hypermobility is believed to be a mechanical genesis of their hyperacusis symptoms. STUDY DESIGN: A prospective, repeated-measure single-arm design was used for this study. SETTING: All patients were evaluated and treated at a tertiary level otologic referral center. SUBJECTS AND METHODS: 21 patients (Cohort A) with severe hyperacusis underwent oval and round window reinforcement. Seven patients (Cohort B) intraoperatively appeared to have subjective hypermobility of the stapes. Additional reinforcement of the stapes superstructure was performed in these patients. RESULTS: In Cohort A, loudness discomfort level (LDL) values improved on average from 72.7 dB to 81.9 dB. Hyperacusis questionnaire (HQ) scores improved from 30.1 to 14.7. Numeric Rating Scale scores (0-10) decreased from 8.5 to 4.0. In Cohort B, values similarly improved from an average of 72.4 dB to 88.2 dB. HQ scores improved from 35.8 to 18.9. Numeric Rating Scale scores fell from 10.0 to 3.7. Postoperatively there were no complaints of hearing loss. Sixteen out of 21(76%) reported improved quality of life and diminished symptoms of hyperacusis. CONCLUSION: It is possible that patients suffering from hyperacusis may have a mechanical cause for their symptoms. Further research is necessary to clarify stapes mobility in patients with these symptoms. Excess temporalis tissue reinforcement of the stapes along with round window reinforcement shows promise as a minimally invasive surgical option for patients suffering from hyperacusis.

Minimally Invasive Surgery for the Treatment of Hyperacusis.

OBJECTIVE: To evaluate the efficacy of a minimally invasive surgical procedure in patients with severe hyperacusis. STUDY DESIGN: Prospective, longitudinal design. SETTING: Tertiary referral center. PATIENTS: Adult patients with history of severe hyperacusis. INTERVENTION: Using a transcanal approach, the round and oval window was reinforced with temporalis fascia or tragal perichondrium in six subjects (nine ears) and was subdivided into two groups (unilateral or bilateral reinforcement procedure). MAIN OUTCOME MEASURES: Pre- and postoperative noise tolerance was measured using uncomfortable loudness level (ULL) test scores. In addition, a self-report hyperacusis questionnaire (HQ) was used to assess hypersensitivity to sound before and after the intervention. RESULTS: Analysis of the data reveals improved postoperative mean ULL test scores of 14 dB (confidence interval [CI], 70-98 dB) in the unilateral group. For the bilateral group, improved mean scores were 13 dB (CI, 63-88 dB) in the first ear and 8 dB (CI, 71-86 dB) for the second ear. Further, a negative linear trend was observed in the mean subjective scores for the HQ when both groups measures were analyzed together decreasing from a mean score of 32.0 (standard deviation [SD] = 3.32) preoperative to a mean score of 11.5 (SD = 7.42) after surgery. Postoperatively, the patients reported no change in hearing and improved quality of life after the procedure. CONCLUSION: The results suggest that reinforcement of the round and oval window with temporalis fascia or tragal perichondrium may offer significant benefit for individuals with severe hyperacusis that has not responded to traditional therapy. ULL scores and self-report measures postoperatively demonstrate improved noise tolerance, high patient satisfaction, and enhanced quality of life.

Round and oval window reinforcement for the treatment of hyperacusis.

PURPOSE: To present the outcomes of two patients (three ears) with hyperacusis treated with round and oval window reinforcement. MATERIALS AND METHODS: Transcanal placement of temporalis fascia on the round window membrane and stapes footplate was performed. Loudness discomfort level testing was performed. Results of pre and post-operative hyperacusis questionnaires and audiometric testing were reviewed. RESULTS: Two patients (three ears) underwent surgery. Results from the hyperacusis questionnaire improved by 21 and 13 points, respectively. Except for a mild loss in the high frequencies, no change in hearing was noted post-operatively. Both patients reported no negative effects from surgery, marked improvement in ability to tolerate noise, and would recommend the procedure to others. There were no complications. CONCLUSIONS: Round and oval window reinforcement is a minimally invasive option for treating hyperacusis when usual medical therapies fail. Further studies are needed to evaluate the effectiveness of the procedure in reducing noise intolerance.

Round window reinforcement for superior semicircular canal dehiscence: a retrospective multi-center case series.

PURPOSE: To evaluate the outcome of round window (RW) tissue reinforcement in the management of superior semicircular canal dehiscence (SSCD). MATERIALS AND METHODS: Twenty-two patients with confirmed diagnosis of SSCD by clinical presentation, imaging, and/or testing were included in the study. Six surgeons at four institutions conducted a multicenter chart review of patients treated for symptomatic superior canal dehiscence using RW tissue reinforcement or complete RW occlusion. A transcanal approach was used to reinforce the RW with various types of tissue. Patients completed a novel postoperative survey, grading preoperative and postoperative symptom severity. RESULTS: Analysis revealed statistically significant improvement in all symptoms with the exception of hearing loss in 19 patients who underwent RW reinforcement. In contrast, 2 of 3 participants who underwent the alternate treatment of RW niche occlusion experienced worsened symptoms requiring revision surgery. CONCLUSION: RW tissue reinforcement may reduce the symptoms associated with SSCD. The reinforcement technique may benefit SSCD patients by reducing the "third window" effect created by a dehiscent semicircular canal. Given its low risks compared to middle cranial fossa or transmastoid canal occlusion, RW reinforcement may prove to be a suitable initial procedure for intractable SSCD. In contrast, complete RW occlusion is not advised.

A prospective study to evaluate the efficacy of isopropyl alcohol irrigations to prevent cerumen impaction.

We conducted a prospective crossover study to assess the safety and efficacy of 70% isopropyl alcohol delivered from a squeezable bottle with a specially designed tip as a weekly irrigant to reduce cerumen accumulation. Twenty patients were divided into 2 groups of 10 (20 ears in each group). The patients in group 1 instilled 70% isopropyl alcohol once a week for 2 months; this was followed by 2 months of no ear cleaning. The patients in group 2 performed the opposite routine. At each visit (0, 2, and 4 mo), cerumen accumulation was graded on a scale of 0 to 4, indicating 0, 25, 50, 75, and 100% occlusion, respectively. After the accumulation was graded, a cerumenectomy was performed. At the initial evaluation, the mean occlusion scores were 3.1 for group 1 and 3.3 for group 2-not a statistically significant difference. After the first 2 months of the study, there was a significant difference in occlusion scores between groups 1 and 2 (0.75 and 1.55, respectively; p < 0.0002). At 4 months, after the crossover, the occlusion scores were 1.15 and 0.95, respectively, not a significant difference (p = 0.38). At study's end, there were also significant differences within each group between occlusion scores obtained during the treatment and nontreatment periods (group 1: p < 0.02; group 2: p < 0.01). All patients tolerated the alcohol rinse well, and there were no cases of external otitis or other complications. We conclude that weekly irrigation with 70% isopropyl alcohol is safe and reduces the accumulation of cerumen in the external auditory canal. Routine use should decrease the number of office visits for cerumen removal and hearing aid cleaning.

Mixed hearing loss in iatrogenic tympanic membrane perforations over the round window niche and the immediate effect of paper patch myringoplasty.

OBJECTIVES: Report the immediate audiologic effect of paper patch myringoplasty to repair iatrogenic tympanic membrane perforations directly over the round window. METHODS: Retrospective case-control study of 15 patients treated for inner ear disease with a MicroWick and dexamethasone for 1 month, resulting in 2-mm perforations over the round window. Paper patch myringoplasties were performed to repair the perforations. Audiograms were performed before and immediately after the paper patch myringoplasty. RESULTS: After paper patch placement, there was a significant improvement in air-bone gap at 250 (p < 0.001), 500 (p = 0.003), and 1000 Hz (p = 0.004) and a significant improvement in bone conduction (BC) threshold at 250 (p = 0.002), 500 (p < 0.001), 1000 (p = 0.002), 2000 (p = 0.003), and 3000 Hz (p = 0.02). CONCLUSIONS: Paper patch myringoplasty improves both air conduction and BC hearing from small perforations over the round window. The decrease in BC hearing is a result of middle ear mechanics and is not a true sensorineural hearing loss.

Laser stapedotomy minus prosthesis: long-term follow-up.

OBJECTIVE: To evaluate long-term hearing results following laser stapedotomy minus prosthesis (laser STAMP). STUDY DESIGN: Case series with chart review. SETTING: Otologic referral center. SUBJECTS AND METHODS: From April 1995 to April 2009, 74 adult patients (77 ears; consecutive cases) underwent primary laser STAMP for otosclerosis limited to the fissula ante fenestram. Pure-tone air, bone conduction thresholds, and word discrimination scores were obtained preoperatively, 1 month postoperatively, and yearly thereafter. RESULTS: One-year minimum follow-up data were available for 43 patients (44 ears; nonconsecutive cases). The average air-bone gap (ABG) was closed from a mean of 23 dB to 6 dB at 6 weeks postoperatively. At a median of 33 (lower quartile, 20; upper quartile 46; range, 12-140) months postoperatively,the mean ABG was 7 dB, indicating little evidence of refixation. Seven of 77 patients (9%) underwent revision surgery with stapedotomy and placement of prosthesis with good results. Four patients (5%) were revised within 1 year for other problems unrelated to stapes refixation. Refixation was noted in 3 patients (4%) undergoing revision surgery. Two of these patients (3%) were revised within 1 year, and 1 patient (1%)was revised after 12 years. CONCLUSIONS: The laser STAMP is a minimally invasive procedure resulting in stable long-term hearing improvement in those patients available for long-term follow-up. Success depends on proper candidate selection of patients with accessible anatomy and minimal otosclerosis confined to the fissula ante fenestram. Advantages of this procedure include preservation of stapes anatomy and stable long-term, high-frequency hearing improvement.

A pilot study of rituximab in immune-mediated inner ear disease.

Immune-mediated inner ear disease (IMED) is a cause of rapidly progressive auditory dysfunction. Patients are often responsive to high-dose corticosteroids and the disease is believed to be mediated by an antibody to inner ear proteins. To date, no therapies have proven effective as corticosteroid-sparing agents. Rituximab is a monoclonal antibody that depletes B cells, resulting in a reduction in autoantibody production. For that reason, rituximab was evaluated in a small pilot study in patients with IMED to see if there was a signal suggesting benefit. In all, 5/7 patients met the primary endpoint of an improvement in pure tone average (500-3000 Hz) by 10 dB in at least one ear, or an improvement in word identification score by at least 12% at 24 weeks, both relative to screening precorticosteroid values after 1 course of treatment. No significant adverse events were reported. The results of this study suggest further evaluation of rituximab as a treatment for IMED is indicated.

Intratympanic gentamicin treatment of patients with Ménière's disease with normal hearing.

OBJECTIVE: Understand the safety and outcomes of intratympanic gentamicin treatment in patients with Ménière's disease with normal hearing. STUDY DESIGN: Case series with chart review. SETTING: Tertiary referral center. SUBJECTS AND METHODS: A total of 224 patients with disabling Ménière's disease treated between May 1996 and June 2007 were grouped according to the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) 1995 Committee on Hearing and Equilibrium staging guidelines: stage 1 (<25 dB pure-tone average [PTA]); stage 2 through 4 (>25 dB PTA). Patients underwent self-treatment with intratympanic gentamicin (10 mg/mL) three times daily for one to eight weeks. Outcome measures included pre- and post-treatment speech discrimination score (SDS), PTA, electronystagmography, vertigo relief, and statistical analysis utilizing the Pearson chi(2) test. RESULTS: Twenty-two (88%) of 24 patients with stage 1 Ménière's disease showed unchanged or improved SDS. All 24 patients showed a mean PTA loss of 8 dB. Seventeen (71%) patients reported complete or improved vertigo control. One hundred sixteen (59%) of 200 patients with stage 2 through 4 Ménière's disease showed unchanged or improved SDS. All 200 patients showed a mean PTA loss of 11 dB. One hundred forty-eight (74%) patients reported complete or improved vertigo control. CONCLUSIONS: Patients with stage 1 Ménière's disease appear to have similar vertigo control with better hearing preservation than patients with advanced disease when treated with low-dose intratympanic gentamicin (10 mg/mL).

Complete round window niche occlusion for superior semicircular canal dehiscence syndrome: a minimally invasive approach.

Superior semicircular canal dehiscence (SCD) syndrome is a recognized condition associated with varying degrees of vestibular and auditory dysfunction. The authors present a case study of disabling SCD syndrome in a 37-year-old man who was successfully treated with a complete round window niche occlusion via a transcanal approach. This case represents the first report of a transcanal complete round window niche occlusion for the treatment of SCD syndrome. A brief literature review and discussion of surgical techniques follow.

Dexamethasone inner ear perfusion for subclinical endolymphatic hydrops.

We suggest that the term subclinical cochlear hydrops be used to describe patients presenting with aural fullness, stuffiness, or ear pressure when all other diagnoses have been excluded. We reviewed the charts of 20 patients who had presented with a chief complaint of aural fullness, stuffiness, or pressure and who underwent perfusion of the inner ear for 1 month with dexamethasone 4 or 10 mg/ml. The symptoms improved in 13 (68.4%) of 19 patients after treatment. None of these patients reported a worsening of symptoms, and no patient had worse hearing after perfusion. Complications were infrequent, and patients tolerated the treatment well. Perfusion of the inner ear appears to be a safe procedure with little risk.

Long-term follow-up after gentamicin application via the Silverstein MicroWick in the treatment of Ménière's disease.

We conducted a retrospective study to evaluate the long-term effectiveness of gentamicin applied directly to the round window membrane via the Silverstein MicroWick system in patients with definite or probable Ménière's disease. The study population was made up of 69 patients who had received 3 drops of gentamicin 10 mg/ml three times daily; the duration of treatment was guided by objective data obtained from weekly electronystagmography and audiometry. Relief of vertigo was assessed by chart review and telephone interviews. After a minimum follow-up of 24 months, we found that vertiginous symptoms remained controlled in 53 patients (76.8%) after one or more courses of drug therapy. A single course of treatmentwas successful in 41 patients (59.4%). We conclude that long-term control of vertigo can be achieved inpatients with Ménère's disease by direct application of gentamicin to the round window membrane via the Silverstein MicroWick. This minimally invasive self-treatment technique was well tolerated and free of long-term complications.

Assessment of patient satisfaction with various configurations of digital CROS and BiCROS hearing aids.

We conducted a study of 91 patients with severe-to-profound asymmetric hearing loss to assess their satisfaction with digital contralateral routing of signal (CROS) or bilateral contralateral routing of signal (BiCROS) hearing aids, Satisfaction was evaluated on the basis of the number of patients who elected to purchase their hearing aid following a free 30-day trial and on the results of a subsequent 8-question survey. We found that overall patient satisfaction was generally high. At the end of the 30-day trial, 66 of the 91 patients (72.5%) elected to keep their CROS or BiCROS device, a percentage that is far greater than the acceptance rates of 10 to 20% that had been previously reported with older models of the CROS and BiCROS devices. According to the survey responses, those who kept their devices gave them an overall rating of 3.4 on a scale of 1 (very dissatisfied) to 5 (very satisfied); those who returned their devices gave them an overall rating of 1.9.

Experience with the SOUNDTEC implantable hearing aid.

OBJECTIVE: To assess the efficacy, morbidity, and patient satisfaction of the SOUNDTEC semi-implantable hearing aid. STUDY DESIGN: Retrospective case review. SETTING: Two tertiary referral centers (the Silverstein Institute and the Atkins Institute). PATIENTS: Sixty-four (four bilateral placements) patients with bilateral moderately severe sensorineural hearing loss: 47 patients had previously worn hearing aids. INTERVENTIONS: After separating the incudostapedial joint, a magnet encased in a titanium canister with a ring was introduced onto the stapes neck. Gelfoam or adipose tissue was used to stabilize the magnet. After 3 months, the external processor was fitted. MAIN OUTCOME MEASURES: At 1 month, audiometric testing was performed and functional gain was assessed. Patient acceptance and implant performance were measured by a visual analogue questionnaire. RESULTS: The device produced an average functional gain of 26 dB. Fifty-five percent of patients complained of magnet movement; this was eliminated in 80% of Silverstein Institute patients when the external processor was worn and fat was used to stabilize the magnet. CONCLUSION: The SOUNDTEC direct device is well tolerated in the majority of patients, with a significant increase in functional gain. Magnet instability and noise were the most frequent complaints and improved with processor placement and anchoring the magnet with fat. This electromagnetic semi-implantable hearing aid confers greater functional gain over conventional hearing aids and reduces occlusion effect and feedback.

Silverstein MicroWick.

Using the MicroWick to deliver otic medication to the inner ear fluids isa new, unique, effective, safe, efficient, and inexpensive method to treat inner ear disease. This self-treatment method using otic medication represents a major breakthrough in the treatment of inner ear disease. It allows the otologist to treat inner ear disease much as the ophthalmologist treats eye disease using eye drops. In the future, new drugs and medications will likely be developed that will relieve vertigo attacks, improve certain types of sensorineural hearing loss, and reduce tinnitus. The MicroWick will be available for patients to self-administer these medications to the inner ear through the round window membrane.

Transtympanic perfusion: indications and limitations.

PURPOSE OF REVIEW: To review the most recent literature regarding the application of transtympanic inner ear perfusion in the treatment of inner ear disorders including Meniere disease, sudden sensorineural hearing loss, and autoimmune inner ear disease. RECENT FINDINGS: The use of gentamicin perfusion in the management of Meniere disease with intractable vertigo has been demonstrated to have a very high rate of success, and is much less invasive than alternative surgical procedures such as vestibular nerve section or labyrinthectomy. The technique for achieving the highest rate of success while still minimizing the risk of cochleotoxicity continues to be investigated. Sustained delivery techniques such as the Silverstein MicroWick appear to achieve the best pharmacokinetic profile within the inner ear fluids. The end point of treatment does not necessarily require complete vestibular ablation to cure the patient, and shorter courses of treatment may help to reduce the risk of hearing loss. Cochlear Meniere disease can be treated with dexamethasone 4 mg/cc perfusion of the inner ear, which may improve the hearing, tinnitus, and pressure in the ear. Sudden sensorineural hearing loss has been managed with transtympanic steroid delivery, and this appears to be beneficial for some patients who have failed to respond to oral steroids, or have medical contraindications to systemic steroids. SUMMARY: Inner ear perfusion via transtympanic delivery is an emerging technique in the management of inner ear disease. Improved results are expected over time as research in this area answers questions about dosage and delivery techniques, as well as identifying new applications and pharmaceuticals.