Tensor Tympani and Stapedial Tendon Reconstruction for Postoperative Hyperacusis after Tendon Lysis for Middle Ear Myoclonus.

OBJECTIVE: To present a method for repair of the stapedial and tensor tympani tendons in a patient with hyperacusis after a tendon lysis procedure. PATIENTS: A 71-year-old professional musician who presented to clinic with debilitating hyperacusis following a tensor tympani and stapedial tendon lysis procedure to treat middle ear myoclonus. INTERVENTIONS: A novel procedure for reapproximation of the tensor tympani and stapedial tendons into their native insertion points using periosteal grafts and nitinol wire. MAIN OUTCOMES MEASURES: Stapedial reflex measurements, uncomfortable loudness level, and subjective patient experience. RESULTS: Postoperatively, the patient had objective improvement in hyperacusis with return of acoustic reflexes in the affected ear and durable improvements in their frequency-specific uncomfortable loudness levels. CONCLUSIONS: This case describes the debilitating complication of hyperacusis following tendon lysis and highlights the importance of maximizing behavioral and medical measures prior to undergoing surgical intervention for middle ear myoclonus.

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.

Postmastoidectomy Hyperacusis Syndrome: Clinical Features and Treatment.

OBJECTIVE: We report a novel postmastoidectomy hyperacusis syndrome (PMHS) in patients who have had cortical mastoidectomies and experience hyperacusis to stimuli involving touch of the pinna and periauricular area. This report aims to describe the clinical characteristics of patients predisposed to this disabling complication after mastoid surgery and describes surgical treatment with mastoid cortex resurfacing with hydroxyapatite bone cement. PATIENTS: Three patients who have undergone intact canal wall mastoidectomies for nonchronic middle ear-related pathologies all reported a similar constellation of postoperative symptoms. None of the patients had any ossicular chain or middle ear abnormalities, and none had preoperative conductive hearing loss. All patients reported disabling hyperacusis related to light touch stimuli in the periauricular area. On examination, all three patients demonstrated synchronous movement of the tympanic membrane when the postauricular area was palpated. INTERVENTIONS: After a period of observation, none of the patients noted any improvement to their symptoms. Resurfacing of the mastoid cortex with hydroxyapatite bone cement was performed in all patients. MAIN OUTCOME MEASURES: Presence of touch-induced hyperacusis and audiometry was assessed postoperatively. Patients were also examined for synchronous movement of the tympanic membrane with palpation of the postauricular area. RESULTS: All patients experienced complete resolution of touch-induced hyperacusis postoperatively. Pure-tone audiometric hearing thresholds remained unchanged after mastoid cortex resurfacing, and there was no longer tympanic membrane movement with palpation of the postauricular area. CONCLUSIONS: PMHS can occur in patients after cortical mastoidectomy when there is no history of ossicular chain or history of chronic middle ear disease or middle ear abnormalities. PMHS can cause significant distress to patients and remain underrecognized unless synchronous tympanic membrane movement is specifically examined for. Treatment via mastoid cortex surfacing with hydroxyapatite bone cement is safe and effective.

Outcomes of Stapedotomy in Patients With Concomitant Otosclerosis and Superior Semicircular Canal Dehiscence: Should a Radiographic Third-Window Be a Contraindication to Stapes Surgery?

OBJECTIVE: Review surgical outcomes of stapedotomy in patients with concomitant otosclerosis and superior semicircular canal dehiscence. STUDY DESIGN: Retrospective case review. SETTING: Tertiary referral center. PATIENTS: Patients with otosclerosis and radiographic superior semicircular canal dehiscence undergoing stapedotomy between 2008 and 2020. INTERVENTION: Stapedotomy. MAIN OUTCOME MEASURES: Pre- and postoperative hearing and unmasking of third-window symptoms. Hearing was measured by air conduction (AC) and bone conduction (BC) pure-tone averages (PTA), air-bone gap (ABG), and word recognition scores (WRS). Third-window symptoms included hyperacusis, autophony, sound- or pressure-induced vertigo, imbalance, or oscillopsia. RESULTS: Twenty patients with otosclerosis and radiographic superior semicircular canal dehiscence underwent stapedotomy, 13 primary and 7 revision. Mean AC PTA was 49.3 dB preoperatively and 35.6 dB postoperatively (p = 0.0077), while the ABG improved on average from 23.9 to 9.68 dB (p < 0.0001). The ABG improved to ≤10 dB in 12/20 patients (60%), and ≤20 dB in 18/20 patients (90%). There was no significant difference in BC PTA or WRS postoperatively. Two patients (10%) experienced potential transient unmasking of third-window symptoms-hyperacusis and prolonged imbalance-which both resolved. There were no other complications. There was no significant difference in audiologic outcomes or unmasking of third window symptoms between primary or revision cases. CONCLUSIONS: Persistent conductive hearing loss is common following stapedotomy for otosclerosis in patients with concomitant superior semicircular canal dehiscence. However, a majority of patients can achieve excellent hearing outcomes, while unmasking of third window symptoms appears to be rare. Radiographic superior semicircular canal dehiscence may not be an absolute contraindication to stapes surgery for otosclerosis.

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.

Simultaneous, unilateral plugging of superior and posterior semicircular canal dehiscences to treat debilitating hyperacusis.

OBJECTIVE: To describe a case of bilateral superior and posterior semicircular canal dehiscences, and the use of a unilateral transmastoid approach to address both right-sided defects simultaneously. CASE REPORT: In a patient with right-sided hyperacusis, bilateral dehiscence of both the superior and the posterior semicircular canals was identified, located adjacent to the common crus, together with a right-sided, anterosuperiorly positioned sigmoid sinus and a high-riding jugular bulb. Results for audiography and cervical vestibular evoked myogenic potential testing were consistent with right-sided semicircular canal dehiscence. At surgery, a right-sided transmastoid approach provided access to plug both defects simultaneously, following posterior mobilisation of the sigmoid sinus. The patient's hyperacusis was completely resolved, with a 10-30 dB improvement in his right ear air conduction hearing, without decrement in bone conduction. CONCLUSION: In properly selected patients, a transmastoid approach can be used to effectively manage superior semicircular canal dehiscence and posterior semicircular canal dehiscence simultaneously. Pre-operative computed tomography is recommended to evaluate the dehiscence sites and to identify complicating vascular anatomy.

Surgical capping of superior semicircular canal dehiscence.

Surgical plugging and resurfacing are well established treatments of superior semicircular canal dehiscence, while capping with hydroxyapatite cement has been little discussed in literature. The aim of this study was to prove the efficacy of the capping technique. Charts of patients diagnosed with superior semicircular canal dehiscence were reviewed retrospectively. All patients answered the dizziness handicap inventory, a survey analyzing the impact of their symptoms on their quality of life. Capping of the dehiscent canal was performed via the middle fossa approach in all cases. Ten out of 22 patients diagnosed with superior semicircular canal dehiscence were treated with surgical capping, nine of which were included in this study. No major perioperative complications occurred. In 8 out of 9 (89%) patients, capping led to a satisfying reduction of the main symptoms. One patient underwent revision surgery 1 year after the initial intervention. Scores in the dizziness handicap inventory were lower in the surgically treated group than in the non-surgically treated group, but results were not statistically significant (P = 0.45). Overall, capping is a safe and efficient alternative to plugging and resurfacing of superior semicircular canal dehiscence.

Occlusion of the round window: a novel way to treat hyperacusis symptoms in superior semicircular canal dehiscence syndrome.

BACKGROUND: Conductive hyperacusis in superior semicircular canal dehiscence syndrome occurs due to the presence of a 'third window' created by the dehiscence. Reversible blocking of the round window can, in theory, cause a reduction in the compression-related volume displacement, and thereby minimise symptoms of conductive hyperacusis. This study describes a technique of permeatal blocking of the round window. METHOD: The tympanomeatal flap is elevated and the round window niche is identified. The round window membrane is subsequently identified and occluded with bone wax, muscle and fascia, in three separate layers. Finally, the tympanomeatal flap is reflected, and an ear wick is inserted. RESULTS: Two patients who underwent the procedure reported a reduction in symptoms. Importantly, no Tullio phenomenon was reported post-operation. CONCLUSION: Blocking of the round window can be used to control symptoms of superior semicircular canal dehiscence syndrome in patients who present solely with symptoms of conductive hyperacusis. This technique provides an alternative to resurfacing techniques. The procedure is simple to perform, reversible and can be undertaken as day-case surgery.

Endoscopic ligation of the patulous eustachian tube as treatment for autophony.

OBJECTIVES/HYPOTHESIS: Patulous eustachian tube remains a challenging management problem in otolaryngology. The autophony experienced by this patient population can be severe, and as yet no reliable surgical method exists to reduce or eliminate this annoying symptom. Our objective was to develop a novel endoscopic technique to assist these patients. STUDY DESIGN: Longitudinal case series. METHODS: A prospective longitudinal study was conducted of a series of consecutive patients undergoing surgery for patulous eustachian tube. Under transnasal endoscopic guidance, using a combination of fat plugging, endoluminal cauterization, and suture ligation, 14 ears underwent surgical treatment during a 4-year period. The main outcome measure assessed was the level of autophony present after surgery as compared to baseline. Audiometric outcomes, surgical time, and complications were also recorded. RESULTS: There was sustained satisfactory subjective improvement in the autophony in 12 of the 14 ears (85.7%) at primary surgery, with nine of these 12 ears (75%) demonstrating full autophony cessation. Wilcoxon signed rank test showed a significant improvement in autophony in the study population compared to baseline (z = 3.16, P < .001). The mean operating length of time per ear over the full duration of our series was approximately 45 minutes. Postoperative audiometry showed that surgery had no impact on hearing. CONCLUSIONS: Multilayer endoscopic ligation of the patulous eustachian tube resulted in a high autophony improvement rate. The procedure is technically straightforward, and results were sustained at 6-month follow-up.

Vestibular-evoked myogenic potentials after superior semicircular canal obliteration.

OBJECTIVE: To analyse vestibular evoked myogenic potentials (VEMPs) characteristics in patients with superior semicircular canal dehiscence syndrome (SCDS) after surgical plugging. MATERIALS AND METHODS: Five surgical plugging of SCD were performed on 4 patients presenting uni- or bilateral SCD. VEMPs were recorded before and after surgery. RESULTS: Postoperative VEMP testing revealed in all cases a normalization of the response on the operated side. DISCUSSION: Patients with SCDS typically have VEMPs with pathologically low thresholds and large amplitude potentials. VEMPs represent a screening investigation for SSCD and a valuable tool in the postoperative follow-up to confirm the successful repair of the dehiscence. In bilateral SSCD cases, VEMPs help to localize the worst side which is operated first. CONCLUSION: VEMPs are essential in both preoperative planning and postoperative monitoring of patients with SCDS.

Relief of severe hyperacusis and diplacusis in a deafened ear by cochlear labyrinthectomy.

A professional musician with intolerable hyperacusis and dysharmonic diplacusis in a severely deafened ear was successfully relieved of his symptoms by deliberate destruction of the cochlea.