Prediction of hearing outcomes by auditory steady-state response in patients with sudden sensorineural hearing loss.

BACKGROUND: There has been no report on the predictive value of auditory steady-state response (ASSR) in the hearing prognosis of sudden sensorineural hearing loss (SSNHL). AIMS/OBJECTIVES: To investigate whether ASSR can be a prognostic indicator of hearing outcome in patients with SSNHL after systemic steroid treatment. MATERIAL AND METHODS: Fifty-three patients with unilateral mild to severe SSNHL (≤90 dB HL at 0.5k, 1k, 2k, and 4 kHz, 4FA) were included. All patients received systemic high dose steroid therapy within one month after onset. The difference between the threshold levels measured by ASSR and PTA on the same day [ASSR - PTA] was calculated. The hearing recovery (HR) was defined as a < 30 dB HL of final degree of hearing loss and a > 15 dB HL of hearing gain. RESULTS: The HR (+) group showed significantly worse ASSR predicted threshold than pure-tone threshold in univariate (t(51) = 2.412, p = .020) and multivariate analysis (OR 0.910, p = .012). The [ASSR - PTA] threshold showed significantly moderate correlation with hearing gain (r = -0.303, p = .028). CONCLUSIONS: Worse ASSR predicted threshold than pure-tone threshold predicted poor hearing outcome after systemic steroid treatment in mild to severe unilateral SSNHL.

Efficacy and Safety of AM-111 in the Treatment of Acute Unilateral Sudden Deafness-A Double-blind, Randomized, Placebo-controlled Phase 3 Study.

OBJECTIVE: To confirm the efficacy and safety of AM-111 (brimapitide), a cell-penetrating c-Jun N-terminal Kinase (JNK) inhibitor, in patients suffering from severe to profound acute unilateral idiopathic sudden sensorineural hearing loss (ISSNHL). STUDY DESIGN: Prospective, double-blind, randomized, placebo-controlled phase 3 study with follow-up visits on Days 3, 7, 28, and 91. SETTING: Fifty-one European and Asian sites (tertiary referral centers, private ENT practices). PATIENTS: Two hundred fifty-six patients aged 18 to 65 years presenting within 72 hours following ISSNHL onset with mean hearing loss ≥ 40 dB and mean threshold ≥ 60 dB at the 3 worst affected contiguous test frequencies. INTERVENTIONS: Single-dose intratympanic injection of AM-111 (0.4 or 0.8 mg/ml) or placebo; oral prednisolone as reserve therapy if hearing improvement < 10 dB at Day 7. MAIN OUTCOME MEASURES: Hearing improvement to Day 28 was the primary efficacy endpoint; complete hearing recovery, frequency of reserve therapy used, complete tinnitus remission, improvement in word recognition were secondary endpoints. Safety was evaluated by the frequency of clinically relevant hearing deterioration and adverse events. RESULTS: While the primary efficacy endpoint was not met in the overall study population, post-hoc analysis showed a clinically relevant and nominally significant treatment effect for AM-111 0.4 mg/ml in patients with profound ISSNHL. The study drug and the administration procedure were well tolerated. CONCLUSIONS: AM-111 provides effective otoprotection in case of profound ISSNHL. Activation of the JNK stress kinase, AM-111's pharmacologic target, seems to set in only following pronounced acute cochlear injury associated with large hearing threshold shifts.

Successful continual intratympanic steroid injection therapy in a patient with refractory sensorineural hearing loss accompanied by relapsing polychondritis.

OBJECTIVE: To report the treatment efficacy of continual intratympanic steroid injection (ITSI) therapy in a patient with refractory sensorineural hearing loss accompanied by relapsing polychondritis. PATIENT: A 49-year-old female diagnosed with relapsing polychondritis at the age of 45 years and who had been treated with corticosteroids and immunosuppressants developed sensorineural hearing loss in the left ear. INTERVENTION: Her unilateral hearing loss did not recover despite receiving one cyclophosphamide pulse treatment, one methylprednisolone pulse treatment, and weekly leukapheresis. Thus, we decided to initiate weekly ITSI therapy. MAIN OUTCOME MEASURE: Pure tone audiometry. RESULTS: A week after the first ITSI treatment, the patient's hearing improved. We continued ITSI therapy and attempted to extend the interval between treatments, but her hearing worsened when ITSI therapy was delivered at 2- to 3-week intervals. Thus, we returned ITSI therapy to once per week for 21 months (total of 71 treatments). She experienced no adverse events, like tympanic perforation, and 1 year after terminating the therapy, her hearing remained stable and did not worsen. CONCLUSIONS: Continual, weekly ITSI therapy was effective in treating sensorineural hearing loss accompanied by relapsing polychondritis. ITSI therapy may be a promising treatment option for sensorineural hearing loss caused by autoimmune disease.

Pathologic erythrocyte deformability in patients with sudden sensorineural hearing loss.

OBJECTIVE: To evaluate if viscoelastic properties of blood influence suffering sudden sensorineural hearing loss and the capacity to respond after a specific therapy. PATIENTS AND METHODS: A longitudinal prospective study included 85 ears bearing sudden deafness. In them, the mean hearing loss compared to the healthy ear and the recovery ratio were measured at the onset and 6 months after a treatment with corticoids and piracetam. In addition, tinnitus or vestibular symptoms, whole blood filterability (WBF) and erythrocyte deformability -by means of the erythrocyte rigidity index (ERI)- were determined and noted at the beginning and the end of the study. RESULTS: Mean hearing loss was 30.3±19.7% at the onset, and 25.8±39% at the end. Forty-one ears showed a recovery of more than 75%. In these (48% of the entire study group), an increase in WBF and a decrease in ERI were observed (P<.001). Ears without tinnitus or vestibular crisis recovered more hearing at 6 months and showed a significant improvement in WBF and ERI, not detected among patients with these clinical findings. There were good correlations between mean hearing loss at onset and WBF, and between recovery and ERI at 6 months, but without statistical significance. Patients with arterial hypertension, cardiopathy and hypercholesterolemia were the most frequently detected, while hypertension and hyperuricaemia showed a better hearing recovery ratio. CONCLUSIONS: The blood viscosity parameters WBF and ERI offer useful information about the risk of suffering sudden deafness and the capacity to recover hearing with reactive therapies.

Idiopathic bilateral auricular petrification.

Auricular petrification is a rare diagnosis and presents an interesting therapeutic challenge. Fewer than 160 cases have been reported in the literature since the first description by Bochdalek in 1866. The most common etiology is ectopic calcification. It may also result from injurious processes including frostbite, physical trauma, inflammatory conditions, and various endocrinopathies. We report an incidental finding of idiopathic bilateral auricular petrification in a 40-year-old man presenting with idiopathic unilateral sensorineural deafness.

[Sound therapy in sudden deafness]. / Terapia sonora en sordera súbita.

INTRODUCTION AND GOALS: Idiopathic sudden sensorineural hearing loss is a hearing disorder of unknown cause. The spontaneous recovery rate ranges from 50 to 75% of the patients. Scientific experiments on animals support the present study in patients with sudden deafness treated with sounds. PATIENTS AND METHODS: During the period 2003-2009, patients with idiopathic sudden sensorineural hearing loss were administered steroids, piracetam and antioxidants, together with the addition of sounds by means of music and words. RESULTS: Comparing the results of patients treated with medication (n=65) and those treated with medication and sounds (n=67), it was observed that patients treated with medication and sounds had higher recovery. Within the group of patients treated with medication and sounds, 25 (37%) experienced complete recovery, 28 (42%) good recovery, 11 (16%) slight recovery and 3 (5%) poor or no recovery. CONCLUSION: The patients who recovered more than half of their audition accounted for 54% in the group treated with medication and for 79% in the group of patients receiving medication and sounds. Auditory recuperation showed no alterations, at least up to 12 months after therapy.

How long should the sudden hearing loss patients be followed after early steroid combination therapy?

To evaluate the beginning time and the completion time of hearing improvement in patients with sudden hearing loss who were treated with combination therapy including oral steroid. From September 2006 to December 2007, 102 idiopathic sudden hearing loss patients who showed any 'recovery' in hearing according to the Siegel's criteria after treatment were analyzed. Pure tone audiometries were performed on the pretreatment day and on the third, seventh, fourteenth post-treatment day, and on the first, second, third, sixth post-treatment month. The time of initial hearing improvement and the completion time of hearing improvement were analyzed. Of 102 patients who showed any improvement, cumulatively, 93.1% showed beginning of hearing improvement within 14 days after treatment. Complete recovery or an end of change was achieved in cumulatively 80.4% of the patients within 1 month after treatment and in 92.2% of the patients within 2 months after treatment. Prognosis can be predicted approximately 2 weeks after start of treatment because time of commencement shows plateau after 2 weeks in improved cases. Hearing should be followed-up for at least 2 months after treatment in patients who show incomplete or delayed hearing improvement.

Fluctuating corticosteroid-responsive auditory neuropathy/dyssynchrony is suggestive of central nervous system pathology.

OBJECTIVE: To highlight diagnostic and management features of auditory neuropathy/auditory dyssynchrony (AN/DS) due to central demyelinating disorder. PATIENTS: A child with AN/DS due to central nervous system pathologic findings. INTERVENTIONS: Audiometry, auditory brainstem response (ABR) test, otoacoustic emission test, magnetic resonance imaging (MRI) with gadolinium contrast, intravenous corticosteroid treatment, antiviral treatment, stereotactic biopsy, and cyclophosphamide immunomodulation. MAIN OUTCOME MEASURES: Pure-tone audiometry, speech discrimination testing, ABR, and MRI. RESULTS: A 12-year-old girl presented with acute sensorineural hearing loss, abnormal ABR, and normal otoacoustic emissions consistent with AN/DS. The hearing loss demonstrated fluctuation and corticosteroid responsiveness. Magnetic resonance imaging and stereotactic biopsy revealed brainstem demyelination consistent with multiple sclerosis. Definitive treatment consisted of cyclophosphamide immunomodulation. CONCLUSION: Although recent focus on pathophysiology of AN/DS has shifted from auditory nerve abnormalities to dyssynchrony within the cochlea, cases associated with fluctuating sensorineural hearing loss and responsiveness to corticosteroid therapy should raise the suspicion of central nervous system pathologic findings. Therefore, it is crucial to obtain brain MRI with contrast enhancement in all patients with AN/DS. This is critical in patients undergoing cochlear implantation because MRI may be contraindicated postoperatively.

Hemodilution therapy with hydroxyethyl starch solution (130/0.4) in unilateral idiopathic sudden sensorineural hearing loss: a dose-finding, double-blind, placebo-controlled, international multicenter trial with 210 patients.

OBJECTIVE: Obtain and analyze first data on hydroxyethyl starch (HES 130/0.4) as monotherapy in acute idiopathic sudden sensorineural hearing loss (ISSNHL). DESIGN: Randomized, double-blind, Phase-II, dose-finding study. SETTING: Twenty-five ENT centers in Germany, the Czech Republic, Romania, and Austria. PATIENTS: Two hundred and ten inpatients with first-time ISSNHL of at least 20 dB at two or more frequencies and 95 dB or less at all of the speech frequencies (0.5, 1.0, 2.0, 3.0, and 4.0 kHz) with respect to the other (normal) ear for up to 7 days (d). INTERVENTION: Infusion of 750 mL/d with 45 (Group H), 30 (Group M), or 15 g/d HES (Group L), or glucose 5% (Group G) acting as "placebo" control during 6 days. MAIN ENDPOINT: Gain in average auditory threshold (in dB) from baseline to Day 7. RESULTS (MEDIANS): Average hearing loss at baseline was 24 dB, and infusions started 2 days after ISSNHL onset. No relevant group difference was observed in hearing gain or adverse treatment events, including pruritus. Half of all patients recovered completely by Day 7. SECONDARY ANALYSIS: In patients who started treatment within 2 days after the onset of symptoms and who had systolic blood'pressure (RRsyst) of less than 140 mm Hg, hearing at Day 90 had improved in all 28 cases under glucose 5%; for those who started treatment later and/or had RRsyst of 140 mm Hg or more, the risk for failing to recover under placebo was 29.2% (7/24). Comparing all 118 (51.9%) of 208 patients at such risk, outcome at Day 7 was markedly better in all HES subgroups than in the G'subgroup'(nH:nM:nL:nG = 32:29:32:24, Kruskal-Wallis, p = 0.0221). CONCLUSION: All treatment groups were equivalent, including adverse treatment events. The secondary analysis showed that ISSNHL patients at risk for not improving under placebo (i.e., patients who started treatment more than 48 h after ISSNHL onset and/or with elevated RRsyst) recovered markedly better under infusions of HES 130/0.4.

[Susac's syndrome. A rare microangiopathy of cochlea, retina, and brain]. / Susac-Syndrom. Eine sehr seltene Mikroangiopathie von Kochlea, Retina und Gehirn.

Susac's syndrome is a rare disease characterized by encephalopathy, retinal artery occlusion, and a sensorineural hearing loss. Diagnosis may be difficult since most specialists are not familiar with this angiopathy. However, the typical symptom complex can mimic different pathologies, therefore requiring the attention of radiologists, ENT specialists, and ophthalmologists. We present a rare case of Susac's syndrome unveiled by audiometry results, MR imaging of the brain, and the ophthalmological findings..