Severe Hyperacusis Research Articles

중두개와 접근법을 통한 상반고리관 피열 증후군의 수술적 치료 1예

Superior semicircular canal dehiscence (SSCD) syndrome is a rare disease characterized by sound and pressure induced vertigo accompanying vertical-torsional nystagmus, caused by dehiscence or absence of a bone overlying the superior semicircular canal. Patients show various extent of symptoms related to hearing and balance impairment, according to the degrees of bony dehiscence of superior semicircular canal. The authors have experienced a 41-year-old male patient with the chief complaint of severe hyperacusis and dizziness. The patient was finally diagnosed as left SSCD, and the symtoms were successfully treated surgically by repairing SSCD via middle cranial fossa approach. We thus report a rare case of SSCD syndrome accompanying not only dizziness but also severe hyperacusis and successfully managed by surgical method via middle cranial fossa approach.

Audiological biomarkers of tinnitus in an older Portuguese population.

Tinnitus is a phantom sound perceived in the absence of external acoustic stimulation. It is described in a variety of ways (e.g., buzzing, ringing, and roaring) and can be a single sound or a combination of different sounds. Our study evaluated associations between audiological parameters and the presence or severity of tinnitus, to improve tinnitus diagnosis, treatment, and prognosis. Our sample included 122 older participants (63 women and 59 men), aged 55–75 years from the Portuguese population, with or without sensory presbycusis and with or without tinnitus. All participants underwent a clinical evaluation through a structured interview, Ear, Nose, and Throat observation, and audiological evaluation (standard and extended audiometry, psychoacoustic tinnitus evaluation, auditory brainstem responses, and distortion product otoacoustic emissions). The Tinnitus Handicap Inventory was used to measure tinnitus symptom severity. Our data confirmed that the odds of developing tinnitus were significantly higher in the presence of noise exposure and hearing loss. Also, participants who had abrupt tinnitus onset and moderate or severe hyperacusis featured higher odds of at least moderate tinnitus. However, it was in the ABR that we obtained the most exciting and promising results, namely, in wave I, which was the common denominator in all findings. The increase in wave I amplitude is a protective factor to the odds of having tinnitus. Concerning the severity of tinnitus, the logistic regression model showed that for each unit of increase in the mean ratio V/I of ABR, the likelihood of having at least moderate tinnitus was 10% higher. Advancing knowledge concerning potential tinnitus audiological biomarkers can be crucial for the adequate diagnosis and treatment of tinnitus.

Association between Hyperacusis and Tinnitus.

Many individuals with tinnitus report experiencing hyperacusis (enhanced sensitivity to sounds). However, estimates of the association between hyperacusis and tinnitus is lacking. Here, we investigate this relationship in a Swedish study. A total of 3645 participants (1984 with tinnitus and 1661 without tinnitus) were enrolled via LifeGene, a study from the general Swedish population, aged 18–90 years, and provided information on socio-demographic characteristics, as well as presence of hyperacusis and its severity. Tinnitus presence and severity were self-reported or assessed using the Tinnitus Handicap Inventory (THI). Phenotypes of tinnitus with (n = 1388) or without (n = 1044) hyperacusis were also compared. Of 1661 participants without tinnitus, 1098 (66.1%) were women and 563 were men (33.9%), and the mean (SD) age was 45.1 (12.9). Of 1984 participants with tinnitus, 1034 (52.1%) were women and 950 (47.9%) were men, and the mean (SD) age was 47.7 (14.0) years. Hyperacusis was associated with any tinnitus [Odds ratio (OR) 3.51, 95% confidence interval (CI) 2.99–4.13], self-reported severe tinnitus (OR 7.43, 95% CI 5.06–10.9), and THI ≥ 58 (OR 12.1, 95% CI 7.06–20.6). The association with THI ≥ 58 was greater with increasing severity of hyperacusis, the ORs being 8.15 (95% CI 4.68–14.2) for moderate and 77.4 (95% CI 35.0–171.3) for severe hyperacusis. No difference between sexes was observed in the association between hyperacusis and tinnitus. The occurrence of hyperacusis in severe tinnitus is as high as 80%, showing a very tight relationship. Discriminating the pathophysiological mechanisms between the two conditions in cases of severe tinnitus will be challenging, and optimized study designs are necessary to better understand the mechanisms behind the strong relationship between hyperacusis and tinnitus.

Stapes hypermobility as a possible cause of hyperacusis.

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. A prospective, repeated-measure single-arm design was used for this study. All patients were evaluated and treated at a tertiary level otologic referral center. 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. 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. 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.

Prevalence and Characteristics of Patients with Severe Hyperacusis among Patients Seen in a Tinnitus and Hyperacusis Clinic.

The purpose was to assess the proportion of patients seeking help for tinnitus and/or hyperacusis who have severe hyperacusis and to examine factors associated with severe hyperacusis. This was a retrospective cross-sectional study based on 362 consecutive patients who attended a National Health Service audiology clinic for tinnitus and/or hyperacusis rehabilitation and for whom uncomfortable loudness levels (ULLs) had been measured. The criterion for severe hyperacusis was taken as a ULL of 30 dB HL or less for at least one of the measured frequencies for at least one ear. Thirteen patients had severe hyperacusis, and eight of those had normal hearing. The lowest average ULL across frequencies was 28 dB HL. The difference in average ULLs between ears was 5 dB or less for nine patients. The range of ULLs across frequencies was between 5 and 60 dB, ULLs often being lowest at 8 kHz. Eleven patients had tinnitus, eight had otological abnormalities, twelve had mental health problems, and six were taking antidepressants. Severe hyperacusis is characterized by low ULLs for specific frequencies and no or mild hearing loss. Given the high incidence of tinnitus, otological abnormalities, and mental health problems, the management of patients with severe hyperacusis should involve otologists and psychiatrists in addition to audiologists.

Uncomfortable loudness levels among children and adolescents seeking help for tinnitus and/or hyperacusis

Objective: To assess the prevalence of hyperacusis and severe hyperacusis among children and adolescents seen at an audiology outpatient tinnitus and hyperacusis service.Design: This was a retrospective study. Hyperacusis was considered as present if the average uncomfortable loudness level (ULL) at 0.25, 0.5, 1, 2, 4 and 8 kHz for the ear with the lower average ULL, which is denoted as ULLmin, was ≤77 dB HL. Severe hyperacusis was considered as present if the ULL was 30 dB HL or less for at least one of the measured frequencies for at least one ear.Study sample: There were 62 young patients with an average age of 12 years (SD = 4.1 years, range 4–18 years).Results: Eighty-five percent of patients had hyperacusis and 17% had severe hyperacusis. On average, ULLs at 8 kHz were 9.3 dB lower than ULLs at 0.25 kHz. For 33% of patients, ULLs were at least 20 dB lower at 8 than at 0.25 kHz.Conclusions: Among children and adolescents seen at an audiology outpatient clinic for tinnitus and hyperacusis, hyperacusis diagnosed on the basis of ULLs is very prevalent and it is often characterised by lower ULLs at 8 than at 0.25 kHz.

Effect of sound generator on tinnitus and hyperacusis

Objective: Sound generator (SG) plays a role as effective sound therapy of tinnitus retaining therapy (TRT) in patients with severe tinnitus (Category 1) or hyperacusis (Category 3). This study was performed to evaluate the therapeutic effect of SGs.Methods: A total of 120 tinnitus patients who visited our tinnitus clinic and were treated with SG along with TRT from January 2008 to December 2016 were included. The patients were divided into two groups by tinnitus category; 78 patients of category 1 and 42 patients of category 3. Their medical records including questionnaires regarding tinnitus severity were retrospectively reviewed to evaluate the therapeutic effect of SGs on tinnitus and hyperacusis.Results: Category 3 patients included more female patients, were younger than category 1 patients (p = .001), and were prescribed SG earlier due to their severe symptom of hyperacusis. (p = .004) All patients showed significant improvements on all categories of tinnitus visual analogue scale (VAS) scores and tinnitus handicap inventory (THI) scores after six months use of SG (p < .05). Loudness discomfort levels measured by pure tone audiometry were significantly improved in category 3 group after six months use of SGs.Conclusion: SG with TRT seems to be an effective treatment modality for all tinnitus patients, especially those with comorbid 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.

Severe Hyperacusis, Photophobia, and Skin Hypersensitivity.

We report a case of a patient with severe hyperacusis, photophobia, and skin hypersensitivity. The patient was initially treated with sound therapy and medical therapy for 4 months and successfully with a selective serotonin reuptake inhibitor (SSRI) and cognitive behavioral therapy which improved her mood and the tolerance for sounds and light.

Tonic tensor tympani syndrome in tinnitus and hyperacusis patients: A multi-clinic prevalence study

Tonic tensor tympani syndrome (TTTS) is an involuntary, anxiety-based condition where the reflex threshold for tensor tympani muscle activity is reduced, causing a frequent spasm. This can trigger aural symptoms from tympanic membrane tension, middle ear ventilation alterations and trigeminal nerve irritability. TTTS is considered to cause the distinctive symptoms of acoustic shock (AS), which can develop after exposure to an unexpected loud sound perceived as highly threatening. Hyperacusis is a dominant AS symptom. Aural pain/blockage without underlying pathology has been noted in tinnitus and hyperacusis patients, without wide acknowledgment. This multiclinic study investigated the prevalence of TTTS symptoms and AS in tinnitus and hyperacusis patients. This study included consecutive patients with tinnitus and/or hyperacusis seen in multiple clinics. Symptoms consistent with TTTS (pain/numbness/burning in and around the ear; aural "blockage"; mild vertigo/nausea; "muffled" hearing; tympanic flutter; headache); onset or exacerbation from exposure to loud/intolerable sounds; tinnitus/hyperacusis severity. All patients were medically cleared of underlying pathology, which could cause these symptoms. 60.0% of the total sample (345 patients), 40.6% of tinnitus only patients, 81.1% of hyperacusis patients had ≥ 1 symptoms (P < 0.001). 68% of severe tinnitus patients, 91.3% of severe hyperacusis patients had ≥ 1 symptoms (P < 0.001). 19.7% (68/345) of patients in the total sample had AS. 83.8% of AS patients had hyperacusis, 41.2% of non-AS patients had hyperacusis (P < 0.001). The high prevalence of TTTS symptoms suggests they readily develop in tinnitus patients, more particularly with hyperacusis. Along with AS, they should be routinely investigated in history-taking.

Assessment and amelioration of hyperacusis in tinnitus patients

ConclusionsTinnitus can be bothersome even without hyperacusis. The good correlation found in this study between the multiple-activity scale for hyperacusis (MASH) score and the overall annoyance of hyperacusis score supports the reliability of self-rating of hyperacusis.ObjectivesA prospective study was undertaken to investigate the relationships between hyperacusis and tinnitus and to determine whether hyperacusis can be rapidly controlled over time.Material and methodsAll tinnitus patients seen over a period of 16 months with surnames beginning with the letters A–L (n=249) were assessed during a structured interview using (i) a scale ranging from 0 to 10 for assessing the annoyance of tinnitus and the overall annoyance of hyperacusis and (ii) the newly introduced MASH.ResultsThe annoyance of tinnitus was greater in females. The prevalence of hyperacusis was high (79%). The annoyance of hyperacusis varied. Patients were categorized into five groups according to the hyperacusis annoyance score, as follows: no hyperacusis; mild hyperacusis (≤3); moderate hyperacusis (3.1–5.0); substantial hyperacusis (5.1–7.0); and severe hyperacusis (≥7.1). Individuals with severe hyperacusis were younger than those in the other groups. The correlation between the annoyance of tinnitus and the annoyance of hyperacusis was poor (r=0.35). No audiometric difference was found between categories, whereas self-rated hearing deficiency increased with the annoyance of hyperacusis. A good correlation (r=0.89) was found between the overall annoyance of hyperacusis and the MASH score. Changes over time, assessed in 32 patients investigated at least 3 times, showed more frequent improvement in hyperacusis (63%) than tinnitus (47%).