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A, Middle ear exposure. Incudostapedial joint (IS), chorda tympani nerve ( Ã ), facial nerve (VII). B, Left ear. Transcanal approach. Tragal perichondrim covering the round window niche (big arrow) and Gelfoam to hold the fascia in place (small arrow). Incudostapedial joint (IS).
Source publication
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 te...
Contexts in source publication
Context 1
... trans-canal round window and oval window reinforcement under general anesthesia was performed similar to an approach for middle ear procedures. For hemostasis, a four-quadrant injection in the ear canal and tragus was made with 1% Lidocaine with 1:100,000 Epinephrine. Vertical canal incisions were made at 6 o'clock and 12 o'clock position and a standard tympanomeatal flap was elevated. The middle ear was entered and chorda tympani was preserved in all cases. If additional exposure was needed, the bony posterior external canal was drilled down with a 1.5 mm diamond burr or curetted to allow adequate visualization of the ossicular chain, round window niche, chorda tympani, facial nerve, and hypotympanum ( Fig. 2A). When necessary, additional drilling of the bony round window niche was performed with a 1.0 mm diamond burr for exposure of the round window membrane. In the first two cases in this series, temporalis fascia graft was used. However, because of incision discomfort, tragal perichondrium graft was used in remaining cases. Through a separate 1 cm incision, a small piece of the perichondrium was taken leaving the tragal cartilage intact. The graft was flattened with the fascia press; 4 and 2 mm round grafts were obtained with a biopsy punch. The mucosa of the round window niche and the stapes footplate was scraped with a micro pick to facilitate tissue welding. The 4 mm graft was used for the round window and the 2 mm graft was placed between the stapes crura on the stapes footplate (Fig. 2B). A small piece of Gelfoam was placed over the round window graft to hold it in place against the round window. The tympanomeatal flap was re-approximated along the posterior canal wall and the canal was filled with mupirocin ointment. Fast absorbing suture was used to close tragal ...
Context 2
... trans-canal round window and oval window reinforcement under general anesthesia was performed similar to an approach for middle ear procedures. For hemostasis, a four-quadrant injection in the ear canal and tragus was made with 1% Lidocaine with 1:100,000 Epinephrine. Vertical canal incisions were made at 6 o'clock and 12 o'clock position and a standard tympanomeatal flap was elevated. The middle ear was entered and chorda tympani was preserved in all cases. If additional exposure was needed, the bony posterior external canal was drilled down with a 1.5 mm diamond burr or curetted to allow adequate visualization of the ossicular chain, round window niche, chorda tympani, facial nerve, and hypotympanum ( Fig. 2A). When necessary, additional drilling of the bony round window niche was performed with a 1.0 mm diamond burr for exposure of the round window membrane. In the first two cases in this series, temporalis fascia graft was used. However, because of incision discomfort, tragal perichondrium graft was used in remaining cases. Through a separate 1 cm incision, a small piece of the perichondrium was taken leaving the tragal cartilage intact. The graft was flattened with the fascia press; 4 and 2 mm round grafts were obtained with a biopsy punch. The mucosa of the round window niche and the stapes footplate was scraped with a micro pick to facilitate tissue welding. The 4 mm graft was used for the round window and the 2 mm graft was placed between the stapes crura on the stapes footplate (Fig. 2B). A small piece of Gelfoam was placed over the round window graft to hold it in place against the round window. The tympanomeatal flap was re-approximated along the posterior canal wall and the canal was filled with mupirocin ointment. Fast absorbing suture was used to close tragal ...
Citations
... Furthermore, in cases of advanced otosclerosis, the RW membrane can become covered by remodeled bone on its middle ear side (Nadol, 2001). Finally, reinforcement of the RW has been used as a treatment for hyperacusis, both in isolated cases (Silverstein et al., 2016) and within the context of superior semicircular canal dehiscence (Ahmed et al., 2019;Silverstein et al., 2014). ...
... Furthermore, the results from the present study are relevant for the relatively recent practice of treating patients with severe hyperacusis with RW and OW reinforcement (Silverstein et al., 2016;H. 2015). ...
... A limitation of designing an effective therapy for hyperacusis is the finite knowledge of the underlying mechanism of hyperacusis (Wong et al., 2020). Although numerous studies were performed to find a therapy for hyperacusis (Attri & Nagarkar, 2010;Hawley et al., 2008;Jastreboff & Jastreboff, 2014;Jüris et al., 2014;Miani et al., 2001;Noreña & Chery-Croze, 2007;Silverstein et al., 2016;Valente et al., 2000), there is no universally accepted therapy (Assi et al., 2018;Fackrell et al., 2017;Jüris et al., 2014). Therapies to reduce hyperacusis may consist of cognitive behavioral therapy (CBT), tinnitus retraining therapy (TRT), counseling, hearing devices, pharmacological therapy, and surgery (Attri & Nagarkar, 2010;Dauman & Bouscau-Faure, 2005;Fackrell et al., 2019;Jüris et al., 2014;Miani et al., 2001;Noreña & Chery-Croze, 2007;Silverstein et al., 2016;Valente et al., 2000). ...
... Although numerous studies were performed to find a therapy for hyperacusis (Attri & Nagarkar, 2010;Hawley et al., 2008;Jastreboff & Jastreboff, 2014;Jüris et al., 2014;Miani et al., 2001;Noreña & Chery-Croze, 2007;Silverstein et al., 2016;Valente et al., 2000), there is no universally accepted therapy (Assi et al., 2018;Fackrell et al., 2017;Jüris et al., 2014). Therapies to reduce hyperacusis may consist of cognitive behavioral therapy (CBT), tinnitus retraining therapy (TRT), counseling, hearing devices, pharmacological therapy, and surgery (Attri & Nagarkar, 2010;Dauman & Bouscau-Faure, 2005;Fackrell et al., 2019;Jüris et al., 2014;Miani et al., 2001;Noreña & Chery-Croze, 2007;Silverstein et al., 2016;Valente et al., 2000). ...
... Until now, multiple attempts have been made to implement therapies aimed at reducing the distress caused by hyperacusis, including CBT, TRT, counseling, use of hearing devices, pharmacological therapy, and surgery (Attri & Nagarkar, 2010;Dauman & Bouscau-Faure, 2005;Fackrell et al., 2019;Jüris et al., 2014;Miani et al., 2001;Noreña & Chery-Croze, 2007;Silverstein et al., 2016;Valente et al., 2000). Formby et al. (2015) expanded the auditory dynamic range for loudness for persons with sensorineural hearing loss. ...
Purpose
The aim of this study was to investigate the short- and long-term effects of a new cognitive sound exposure therapy (CSET) in patients with hyperacusis.
Method
A new therapy was developed to reduce hyperacusis using sound exposure combined with breathing and relaxation strategies from both acceptance and commitment therapy and cognitive behavioral therapy. Patients who were referred to the Speech and Hearing Centers located in Hengelo and Zwolle in the Netherlands and aged ≥ 18 years with hyperacusis as main complaint and no or mild hearing loss were included in this study. Patients were seen for CSET between June 2020 and August 2022. The sessions took place biweekly. Sessions ended when exposure reached a level with a maximum of 70–80 dB SPL. Short-term effects between the start and the end of therapy were based on tolerable level of sound exposure (dB SPL), subjective-level hinderance of hyperacusis, and sensitivity to sound using the Hyperacusis Questionnaire (HQ). The long-term effect was based on HQ 6 months after the end of therapy. Linear mixed-effects and regression models were applied to study outcomes over time.
Results
In total, 30 patients, 15 men and 15 women, aged between 24 and 76 years were included in this study. The mean number of sessions during therapy was 6 and ranged between 4 and 8. Results showed an increase of exposure level (mean change was +23.7 dB with an SD of 7.9, p < .001), a decrease in sensitivity to daily sounds (mean [ SD ] change was −1.6 [2.1], p < .001), and a decrease in HQ (mean [ SD ] change was −9.8 [4.9], p < .001), between the start and the end of therapy. There was no significant change in HQ after the end of therapy and 6 months later; mean ( SD ) change was 0.2 (4.3), p = .81.
Conclusions
The evaluation of CSET indicated a decrease in short- and long-term sensitivity to sound in patients with hyperacusis. Additionally, CSET has shown a positive impact, not only for the sounds used in the therapy sessions but also in transferring benefits to everyday sounds. The results of combining psychoeducation, sound exposure, and counseling are promising and warrant further evaluation.
... Individuals were assessed for postural control using linear and nonlinear parameters calculated in the anterior-posterior (AP) and mediolateral (ML) directions across each test. Additionally, participants provided subjective ratings of sound annoyance on a scale from 1 to 10 [19] and completed the Hearing Hypersensitivity Questionnaire [13,20]. ...
... . The hyperacusis questionnaire [13,20]. Figure A1. ...
... Figure A1. The hyperacusis questionnaire [13,20]. ...
Objectives: This study aimed to explore the impact of irritating sounds on the postural control of healthy adults, considering both linear and nonlinear parameters, subjective assessments, and gender differences. Methods: Thirty-four young participants (17 females, 17 males) completed three 30 s bipedal standing stability tests on a balance platform: one with visual control (EO), another without visual control (EC), and a third without visual control but accompanied by irritating sounds (ECS). Additionally, participants filled out a questionnaire evaluating their sound sensitivity. Linear and nonlinear parameters from each balance test were considered for statistical analysis. Results: The findings reveal significant gender-based variations in sensitivity to sound, with women exhibiting higher sensitivity. No statistically significant differences in postural control were observed between males and females, except for a notable increase in irregularity (SampEn values) in the anterior–posterior direction for females in the ECS trial. Correlation analyses revealed a moderate and statistically significant correlation between SampEn values in the AP direction and SE scores. Conclusions: This study highlights the intricate relationship between sensory stimuli, attention, and the body’s ability to maintain balance. The presence of irritating sounds led to increased irregularity in postural control, particularly in the absence of visual control.
... However, some therapeutic procedures have been implemented and have centered upon the avoidance of provocative stimuli, desensitization by gradual sound exposure [22,23], acoustic training [24], the use of sound generators in Tinnitus Retraining Therapy [25,26], and Cognitive Behavioral Therapy. Minimally invasive surgery, involving reinforcing the oval and round windows and stapes superstructure, has been developed as a potential treatment for hyperacusis patients [27,28]. ...
Hyperacusis, a kind of decreased sound tolerance, is difficult to measure objectively. It often co-occurs with tinnitus. There is a need for valid and reliable patient-reported outcome measures to capture this subjective phenomenon. The aim of the study was to create a questionnaire capturing hyperacusis in terms of loudness, fear, and pain and to evaluate its psychometric properties. The study sample consisted of 106 adult patients with hyperacusis and tinnitus with a mean age of 45.2 years. A medical interview, an audiological examination, and several questionnaires (the Tinnitus Handicap Inventory, the Hyperacusis Questionnaire, the State–Trait Anxiety Inventory, and Visual Analog Scales) were applied. The final 14-item Hyperacusis Assessment Questionnaire showed an appropriate three-factor structure with 70.5% of the variance explained. Convergent and divergent validity were confirmed by correlations with other measures of hyperacusis, anxiety, tinnitus severity, misophonia, and hearing thresholds. The internal consistency assessed with Cronbach’s alpha was excellent (α = 0.91), as was reproducibility (intraclass correlation, ICC = 0.96). The new Hyperacusis Assessment Questionnaire is a psychometrically sound and brief tool assessing the severity of hyperacusis in terms of loudness, fear, and pain. It can be used in clinical practice and scientific research for patients with hyperacusis and tinnitus.
... Запропонована раніше часткова шліфовка цього місця давала нестійкий результат [60,79]. Були спроби проводити ощадливі втручання по оклюзії круглого вікна, але отохірурги зіткнулись з пробле-мою реверсії процесу і втрати слуху, за даними різних авторів -різного ступеню і різного характеру [8, 28,80]. ...
The report presents modern views on the “third mobile window syndrome”, starting with the anatomical and physiological features of the inner ear function, both in normal conditions and in the presence of an additional opening in the bony labyrinth, which changes the hydrodynamic resistance of fluid flow in the perilymphatic space. Characteristic symptoms and modern views (sometimes hypothesis) on the causes of its occurrence are described in detail. Attention focused on diagnostic methods at the outpatient and clinical conditions of a highly specialized department. The latest data on the modern classification of this syndrome and the conclusions of the consensus of the International Barani Society in 2021 are presented. Own series with a detailed description of the features of each case, illustration of the audiometric and tomographic images and successful results of surgical treatment are presented. In the conclusions, the attention of practicing doctors is focused on the signs of the “third mobile window syndrome” which can be detected at the outpatient service – pressure or sound-induced vertigo, bone conduction hyperacusis, and pulsatile tinnitus.
... [6][7][8][9][10] Treatment options for hyperacusis include avoidance of provocative stimuli, cognitive behavioral therapy, tinnitus retraining therapy, hearing amplification, and surgical reinforcement of the round and oval windows. [11][12][13][14] Hyperacusis as it relates to chronic migraine is well described and is typically called "phonophobia" in the migraine literature. It is a symptom commonly found in patients with migraine and is strongly associated with the severity of headache. ...
... The authors cautioned that the test-retest reliability of the LDL measurement can depend on the consistency of instructions provided by the tester, however other studies have reported that the LDL was a reliable tool for follow-up. 12 Recently, sound therapy has been used to improve the LDL of patients with tinnitus and co-morbid hyperacusis and recent reviews have confirmed modest benefit. 11,21 The present study is the first to describe treatment of hyperacusis with a multi-modal migraine prophylactic regimen. ...
Objectives
To evaluate the efficacy of a multi-modal migraine prophylaxis therapy for patients with hyperacusis.
Methods
In a prospective cohort, patients with hyperacusis were treated with a multi-modal step-wise migraine prophylactic regimen (nortriptyline, verapamil, topiramate, or a combination thereof) as well as lifestyle and dietary modifications. Pre- and post-treatment average loudness discomfort level (LDL), hyperacusis discomfort level measured by a visual analogue scale (VAS), and scores on the modified Khalfa questionnaire for severity of hyperacusis were compared.
Results
Twenty-two of the 25 patients (88%) reported subjective resolution of their symptoms following treatment. Post-treatment audiograms showed significant improvement in average LDL from 81.3 ± 3.2 dB to 86.4 ± 2.6 dB ( P < .001), indicating increased sound tolerability. The VAS discomfort level also showed significant improvement from a pre-treatment average of 7.7 ± 1.1 to 3.7 ± 1.6 post-treatment ( P < .001). There was also significant improvement in the average total score on modified Khalfa questionnaire (32.2 ± 3.6 vs 22.0 ± 5.7, P < .001).
Conclusions
The majority of patients with hyperacusis demonstrated symptomatic improvement from migraine prophylaxis therapy, as indicated by self-reported and audiometric measures. Our findings indicate that, for some patients, hyperacusis may share a pathophysiologic basis with migraine disorder and may be successfully managed with multimodal migraine prophylaxis therapy.
... More recently, a surgical intervention has been developed as a potential treatment for hyperacusis patients. This minimally invasive surgery involving reinforcing the oval and round windows and stapes superstructure with temporalis fascia or tragal perichondrium may reduce sound intolerance [7,9,10]. ...
... All patients were enrolled in an ongoing clinical trial to evaluate the effectiveness of round and oval window reinforcement for treatment of hyperacusis. Inclusion criteria have been previously described in a previous publication from our study [10]. Prior to enrollment, subjects underwent a thorough history and physical examination, high resolution CT scan of the temporal bones and an audiometric workup involving an audiogram with pure tone air and bone conduction thresholds, speech discrimination, tympanometry and loudness discomfort level (LDL) testing. ...
... In 2015, Silverstein et al. has reported patients with hyperacusis who underwent surgical treatment including oval and round window reinforcement with tissue [9]. Results show that most patients experienced improvement in sound tolerance after surgery with minimal changes to hearing [6,9,10]. In 2017, the surgical technique was modified to place additional reinforcement of tissue around the oval window and stapes. ...
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.
... Since the patient was agreeable for a less invasive surgical approach for SCDS recently proposed by Silverstein et al [14,15], round window reinforcement (RWR) was performed under general anesthesia by an endoscope-assisted transcanal approach. Round window (RW) niche was denuded of mucosa, and the RW was gently packed with a piece of tragal perichondrium, a piece of cartilage then the area was covered with a layer of fascia (Fig. 5). ...
... This relatively new method presents a lower risk compared to other surgical methods including manipulation of the superior semicircular canal itself and can be offered as a first procedure in patients with mild symptoms [14], elderly patients and the ear with better hearing. Complete occlusion of the RW is not recommended because symptoms may worsen in the late postoperative period [15]. ...
A 71-year-old Japanese male patient presented with a rare case of Glomangiopericytoma (GPC) of the left nasal with obstruction. Complete resection with endoscopic surgery was performed. Immunohistochemical staining for smooth muscle actin, β catenin, cyclin D1, vimentin, and factor 13 were helpful in establishing a definitive diagnosis. Extranasal treatment has been traditionally performed for successful management. However, recent advances in endoscopic treatment have enabled complete endoscopic resection of GPC, minimizing morbidity and facilitating subsequent surveillance for recurrence. Endoscopic management should be considered in suitable cases.
... Since the patient was agreeable for a less invasive surgical approach for SCDS recently proposed by Silverstein et al [14,15], round window reinforcement (RWR) was performed under general anesthesia by an endoscope-assisted transcanal approach. Round window (RW) niche was denuded of mucosa, and the RW was gently packed with a piece of tragal perichondrium, a piece of cartilage then the area was covered with a layer of fascia (Fig. 5). ...
... This relatively new method presents a lower risk compared to other surgical methods including manipulation of the superior semicircular canal itself and can be offered as a first procedure in patients with mild symptoms [14], elderly patients and the ear with better hearing. Complete occlusion of the RW is not recommended because symptoms may worsen in the late postoperative period [15]. ...
Objectives:
The patulous Eustachian tube (PET) and superior semicircular canal dehiscence syndrome (SCDS) have similarity in their symptoms and similar effects caused by positional changes, causing difficulty in the differentiation between the two disorders. This report describes a case of both SCDS and PET that was eventually successfully treated.
Methods:
A 68-year-old man presented with hyperacusis to his own footsteps and gait disturbance. He had been diagnosed as PET two years before and had been treated by insertion of a silicone plug (Kobayashi plug) at the other hospital. Clinical case records, audiological data, cervical vestibular-evoked myogenic potential (cVEMP), Eustachian tube function tests and computed tomography (CT) were taken in the sitting position.
Results:
While the CT confirmed superior semicircular canal dehiscence, the results of cVEMP was not typical of SCD likely due to preexisting hearing impairment in the right ear with a history of middle ear surgeries for the treatment of PET. He received round window reinforcement (RWR) and achieved relief from his symptoms but six months after the surgery, he visited again with complaints of autophony of his own voice and breathing. The tympanic membrane was found to move synchronous with respiration, and Eustachian tube function tests and the sitting CT confirmed the recurrence of severe PET. He had his silicone plug exchanged (increase in size of the Kobayashi plug) and achieved relief from symptoms.
Conclusions:
The present case was a rare instance showing that PET and SCDS can occur simultaneously in a patient. The patient achieved relief from symptoms after treatment with RWR and insertion of the Kobayashi plug.
... Unfortunately, there is limited published data on the benefit of CBT in patients with hyperacusis and further investigation is warranted [4]. In our institute, we have investigated and published a series of patients with clinically diagnosed hyperacusis treated with a minimally invasive surgical procedure [10,11]. The procedure involves reinforcement of the round and oval window and patients showed improvement of objective measures of hyperacusis including loudness discomfort level (LDL) and a non-validated hyperacusis questionnaire. ...
... All patients were enrolled in an ongoing clinical trial to evaluate the effectiveness of round and oval window reinforcement for treatment of hyperacusis. Inclusion criteria have been previously described [10]. ...
... A standard trans-canal approach under general anesthesia was performed similar to an approach for middle ear procedures. This has been previously described [10]. Both temporalis fascia and perichondria grafts have been used to reinforce the oval and round windows. ...
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.
