dB Hearing Loss Research Articles

Isolated Sensorineural Hearing Loss as a Sequela after Lightning Strike.

In most of the surviving patients after a lightning strike, audiovestibular abnormalities have been reported. The most frequently reported type of abnormalities is a tympanic membrane perforation with hearing loss and external ear canal burn. However a sensor neural hearing loss and mixed type hearing loss can also occur, but these occur rarely. A nineteen-year-old female patient had, after a lightning strike, serious burns on the left ear, behind the ear, and on the chest and neck. She also had in her left ear 108 dB hearing loss with irregular central perforation and in her right ear 52 dB sensorineural hearing loss. There was no hearing loss before the strike. A hearing aid was recommended for the right ear and good care and follow-up were recommended for the left ear. A lightning strike can cause serious audiological damage. Therefore, it is necessary to make a careful audiovestibular evaluation of the patients. Although there exist rarely healed cases from sensorineural hearing loss after lightning strike in literature, in our case hearing loss occurred bilaterally and then it healed unilaterally. This condition is quite rare in literature.

Delayed auditory pathway maturation and prematurity.

Hearing loss is the most common sensory disorder in developed countries and leads to a severe reduction in quality of life. In this uncontrolled case series, we evaluated the auditory development in patients suffering from congenital nonsyndromic hearing impairment related to preterm birth. Six patients delivered preterm (25th-35th gestational weeks) suffering from mild to profound congenital nonsyndromic hearing impairment, descending from healthy, nonconsanguineous parents and were evaluated by otoacoustic emissions, tympanometry, brainstem-evoked response audiometry, and genetic testing. All patients were treated with hearing aids, and one patient required cochlear implantation. One preterm infant (32nd gestational week) initially presented with a 70 dB hearing loss, accompanied by negative otoacoustic emissions and normal tympanometric findings. The patient was treated with hearing aids and displayed a gradual improvement in bilateral hearing that completely normalized by 14 months of age accompanied by the development of otoacoustic emission responses. Conclusions We present here for the first time a fully documented preterm patient with delayed auditory pathway maturation and normalization of hearing within 14 months of birth. Although rare, postpartum development of the auditory system should, therefore, be considered in the initial stages for treating preterm hearing impaired patients.

The ICF core sets for hearing loss project: Functioning and disability from the patient perspective

Objective: To explore areas of functioning, disability, and environmental factors of adults with hearing loss (HL) by using the ICF classification as a tool to determine and document each element. Design: A qualitative study applying mainly focus-group methodology was applied. Study sample: Thirty-six Dutch and South African adults (≥ 18 years of age) with HL (20–95 dB HL) who used oral communication as first communication. Summative content analysis was performed on the transcripts by linkage to appropriate ICF categories. Results: 143 ICF categories were identified, most of which belonged to the Activities & Participation (d) component, closely followed by the Environmental factors component. Participants specifically mentioned categories related to oral communication and interaction. Assistive technology (such as hearing aids), noise, and support by and attitudes of others in the environment of the participants were considered highly influential for functioning and disability. Conclusions: The present study illustrates the complex and encompassing nature of aspects involved in functioning and disability of adults with HL. Findings highlight the necessity of using a multidimensional tool, such as the ICF, to map functioning and disability with hearing loss, allowing consideration and evaluation of aspects that are both internal and external.

Indication criteria and outcomes with the Bonebridge transcutaneous bone-conduction implant.

The aim of this study was to evaluate functional hearing gain, speech understanding, and preoperative bone-conduction thresholds with the bone-conduction implant Bonebridge. Retrospective study at a tertiary referral center. Twenty-four consecutive Bonebridge patients were identified. Nine patients suffered from combined hearing loss (HL), 12 from atresia of the external auditory canal and three from single-sided deafness. One patient was lost to follow-up. Twenty-three patients were therefore analyzed. The overall average functional hearing gain of all patients (n = 23) was 28.8 dB (±16.1 standard deviation [SD]). Monosyllabic word scores at 65 dB sound pressure level in quiet increased statistically significantly from 4.6 (±7.4 SD) percentage points to 53.7 (±23.0 SD) percentage points. Evaluation of preoperative bone-conduction thresholds revealed three patients with thresholds higher than 45 dB HL in the high frequencies starting at 2 kHz. These three patients had a very limited benefit of their bone-conduction implants. The Bonebridge bone-conduction implant provides satisfactory results concerning functional gain and speech perception if preoperative bone conduction lies within 45 dB HL. 4.

PRIEŪMINIO IDIOPATINIO NEUROSENSORINIO KLAUSOS PAŽEIDIMO PROGNOSTINIAI VEIKSNIAI

Objectives. We investigated prognostic factors in sudden sensorineural hearing loss.Patients and methods. Patients were recruited into the trial between August 2011 and June 2013 with complains : an abrupt onset of more than 30 dB hearing loss at three consecutive frequencies during period 72 hours, unilateral. 202 patients were randomized to 1 of 2 groups and administered treatment. Group I : patients were given 12 capsules (60 mg) of prednisolon for 7 days, then 2 less capsule per two days until finished. Group II : patients were given systemic prednisolon and additionally, three times a week were administered a transtympanic injection 1,0 ml - 4 mg/ml dexamethasone solution. Audiograms were performed at first visit and 30 days after treatment. Pure tone averages (PTAs) were measured by taking the 6-frequency average of the threshold value at 250, 500, 1,000, 2,000, 4,000 and 8,000 Hz.Results. Our analysis suggests the presence of the following82prognostic factors for sudden sensorineural hearing loss : method of sudden sensorineural hearing loss treatment (better results in combined treatment group); time delay before start of treatment (better results when treatment started within 2 days of the first symptoms of SSNHL); age; seasonal occurrence of the disease in the winter and spring; stress condition, viral infection and trauma(acoustic) before SSNHL, tinnitus, fullness of the ear.Conclusions. Method of sudden sensorineural hearing loss treatment (better results in combined treatment group); time delay before start of treatment ( better results when treatment started within 2 days of the first symptoms of SSNHL); age; seasonal occurrence of the disease in the winter and spring; stress condition, viral infection and trauma (acoustic) before SSNHL, tinnitus, fullness of the ear are prognostic factors for hearing recovery in SSNHL. Gender, proffesional skills, smoking habits, otological and family history, osteochondrosis are not confirmed as prognostic factors for hearing recovery in SSNHL in our study. Knowing of prognostic factors for hearing recovery in SSNHL leads as predict course of the illness and proper prognosis for hearing improvement.

Does the accuracy of blood pressure measurement correlate with hearing loss of the observer?

The auscultatory method is influenced by the hearing level of the observers. If the observer has hearing loss, it is possible to measure blood pressure inaccurately by misreading the Korotkoff sounds at systolic blood pressure (SBP) and diastolic blood pressure (DBP). Because of the potential clinical problems this discrepancy may cause, we used a hearing loss simulator to determine how hearing level affects the accuracy of blood pressure measurements. Two data sets (data set A, 32 Korotkoff sound video clips recorded by the British Hypertension Society; data set B, 28 Korotkoff sound data acquired from the Korotkoff sound recording system developed by Hanyang University) were used and all the data were attenuated to simulate a hearing loss of 5, 10, 15, 20, and 25 dB using the hearing loss simulator. Five observers with normal hearing assessed the blood pressures from these data sets and the differences between the values measured from the original recordings (no attenuation) and the attenuated versions were analyzed. Greater attenuation of the Korotkoff sounds, or greater hearing loss, resulted in larger blood pressure measurement differences when compared with the original data. When measuring blood pressure with hearing loss, the SBP tended to be underestimated and the DBP was overestimated. The mean differences between the original data and the 25 dB hearing loss data for the two data sets combined were 1.55±2.71 and -4.32±4.21 mmHg for SBP and DBP, respectively. This experiment showed that the accuracy of blood pressure measurements using the auscultatory method is affected by observer hearing level. Therefore, to reduce possible error using the auscultatory method, observers' hearing should be tested.

Steroids in aminoglycoside‐containing ear drops: Do they reduce cochlear toxicity?

To determine whether betamethasone (BM) reduces the cochlear toxicity of otic gentamicin (GM) if given together. Controlled animal study. Thirty-four mice were assigned at random to receive intratympanic injections of either 0.1 % BM (11 mice), 0.3% GM (13 mice), or a combination of both (GM/BM) with benzalkonium chloride (10 mice) in the left ear (treated) and saline on the right (untreated). Six injections were given on alternate days. Auditory brainstem response thresholds were assessed at 1 month, 2 months, and >2 months. There was a significantly greater degree of hearing loss in the BM-treated ears compared to the untreated ears (6.48 dB hearing loss, P = .007) and in the GM-treated ears compared to untreated ears (6.59 dB hearing loss, P = .010,). However, otic GM/BM and benzalkonium chloride did not cause significant additional hearing loss compared with the untreated ears (3.56 dB hearing loss, P = .242). Our data suggest that hearing loss caused by GM otic drops may be reduced by the inclusion of BM and benzalkonium chloride. Our finding that BM alone was associated with hearing loss suggests that the benzalkonium chloride may be the protective agent in combination otic drops.

Typical consonant cluster acquisition in auditory-verbal children with early-identified severe/profound hearing loss

Early-identified severe/profound hearing loss (HL) following universal newborn hearing screening (UNHS) has been associated with improved speech and language outcomes. However, speech outcome reports have typically been based on broad measures of speech intelligibility and/or singleton consonant accuracy, with little known about production of consonant clusters. Using a prospective design, the range and accuracy of consonant clusters produced by a homogenous cohort of 12 children early-identified with severe/profound HL aged 3- and 4-years were examined. All children demonstrated bilateral aided thresholds within a range of 15–25 dB HL across all frequencies, were optimally amplified with cochlear implants (11/12) or hearing aids (1/12), and attended auditory-verbal (AV) early intervention. Standardized speech and language assessments were administered. Consonant clusters were strategically sampled in single-word and conversational speech contexts. All standard scores for speech, receptive, and expressive language were within normal limits. All children produced consonant clusters commensurate with expectations for typically-developing hearing peers at 3- and 4- years-of-age. Children's production of phonetically complex morphophonemes (final consonant clusters marking grammatical morphemes) was also in keeping with developmental expectations. Factors which contributed to these encouraging outcomes require further investigation.

Traumatic Hearing Loss in the Context of Blast‐Related Tympanic Membrane Perforation

Objectives: Identify associated clinical factors and characterize the various forms of hearing loss in the context of blast related traumatic tympanic membrane perforation sustained in the combat setting. Methods: A prospective study of 41 patients who sustained a perforation secondary to blast injury between 2010-12 while serving in combat. Clinical exams and audiograms were obtained for all patients by a single institution ear, nose, and throat (ENT) service with comparative data generated from review of pre- and post-trauma hearing screens. Follow-up clinical and audiogram data were obtained on all available subjects. Results: Fifty-nine tympanic membrane (TM) perforations were documented with audiograms. A conductive hearing loss of 14.8dB, 22.4dB, 20.3dB, and 18.9dB and sensorineural hearing loss of 18.0dB, 13.5dB, 19.9dB, and 22.0dB at 500, 1000, 3000, and 4000 Hz respectively was observed. Forty-nine percent of all hearing loss was SNHL. Clinical factors did not correlate to hearing loss ( P > 0.05). Follow-up data were available in 33 patients at an average of 11.8 months. Eighty-eight percent of patients still had tinnitus, and 11% had continued vertigo. Forty-nine percent of perforations healed spontaneously, and 42% had surgical correction. On average, patients demonstrated a 51% improvement in SNHL at time of follow-up. Conclusions: In the subacute setting, patients with traumatic TM perforations secondary to combat blast injury demonstrate conductive and sensorineural hearing loss, with 49% of all loss being sensorineural. An average sensorineural hearing loss improvement of 51% was seen at follow-up. Clinical factors did not correlate with hearing loss. 49% of perforations healed spontaneously, and 42% were surgically corrected.

How Well Can Centenarians Hear?

With advancements in modern medicine and significant improvements in life conditions in the past four decades, the elderly population is rapidly expanding. There is a growing number of those aged 100 years and older. While many changes in the human body occur with physiological aging, as many as 35% to 50% of the population aged 65 to 75 years have presbycusis. Presbycusis is a progressive sensorineural hearing loss that occurs as people get older. There are many studies of the prevalence of age-related hearing loss in the United States, Europe, and Asia. However, no audiological assessment of the population aged 100 years and older has been done. Therefore, it is not clear how well centenarians can hear. We measured middle ear impedance, pure-tone behavioral thresholds, and distortion-product otoacoustic emission from 74 centenarians living in the city of Shaoxing, China, to evaluate their middle and inner ear functions. We show that most centenarian listeners had an “As” type tympanogram, suggesting reduced static compliance of the tympanic membrane. Hearing threshold tests using pure-tone audiometry show that all centenarian subjects had varying degrees of hearing loss. More than 90% suffered from moderate to severe (41 to 80 dB) hearing loss below 2,000 Hz, and profound (>81 dB) hearing loss at 4,000 and 8,000 Hz. Otoacoustic emission, which is generated by the active process of cochlear outer hair cells, was undetectable in the majority of listeners. Our study shows the extent and severity of hearing loss in the centenarian population and represents the first audiological assessment of their middle and inner ear functions.

Diaphanous homolog 3 (Diap3) Overexpression Causes Progressive Hearing Loss and Inner Hair Cell Defects in a Transgenic Mouse Model of Human Deafness

We previously demonstrated that a mutation in the 5′ untranslated region of Diaphanous homolog 3 (DIAPH3) results in 2 to 3-fold overexpression of the gene, leading to a form of delayed onset, progressive human deafness known as AUNA1 (auditory neuropathy, nonsyndromic, autosomal dominant, 1). To investigate the mechanism of deafness, we generated two lines of transgenic mice overexpressing Diap3, the murine ortholog of DIAPH3, on an FVB/NJ background. Line 771 exhibits a relatively mild 20 dB hearing loss at 12 kHz at 4 and 8 weeks of age, progressing to 40 dB and 60 dB losses at 16 and 24 weeks, respectively, at 12 and 24 kHz. Line 924 shows no hearing loss at 4 or 8 weeks, but manifests 35 and 50 dB threshold shifts at 16 and 24 weeks, respectively, at both 12 and 24 kHz. Notably, mice from the two transgenic lines retain distortion product otoacoustic emissions, indicative of normal cochlear outer hair cell (OHC) function despite elevation of auditory thresholds. Scanning electron microscopy of the organ of Corti demonstrates striking anomalies of the inner hair cell (IHC) stereocilia, while OHCs are essentially intact. Over time, IHCs of both lines develop elongated stereocilia that appear fused with neighboring stereocilia, in parallel to the time course of hearing loss in each line. Furthermore, we observe significant reduction in the number of IHC ribbon synapses over 24 weeks in both lines, although this reduction does not correlate temporally with onset and progression of hearing loss or stereociliary anomalies. In summary, overexpression of wild-type Diap3 in two lines of transgenic mice results in hearing loss that recapitulates human AUNA1 deafness. These findings suggest an essential role of Diap3 in regulating assembly and/or maintenance of actin filaments in IHC stereocilia, as well as a potential role at the IHC ribbon synapse.

Auditory analysis of xeroderma pigmentosum 1971–2012: hearing function, sun sensitivity and DNA repair predict neurological degeneration

To assess the role of DNA repair in maintenance of hearing function and neurological integrity, we examined hearing status, neurological function, DNA repair complementation group and history of acute burning on minimal sun exposure in all patients with xeroderma pigmentosum, who had at least one complete audiogram, examined at the National Institutes of Health from 1971 to 2012. Seventy-nine patients, aged 1-61 years, were diagnosed with xeroderma pigmentosum (n = 77) or xeroderma pigmentosum/Cockayne syndrome (n = 2). A total of 178 audiograms were included. Clinically significant hearing loss (>20 dB) was present in 23 (29%) of 79 patients. Of the 17 patients with xeroderma pigmentosum-type neurological degeneration, 13 (76%) developed hearing loss, and all 17 were in complementation groups xeroderma pigmentosum type A or type D and reported acute burning on minimal sun exposure. Acute burning on minimal sun exposure without xeroderma pigmentosum-type neurological degeneration was present in 18% of the patients (10/55). Temporal bone histology in a patient with severe xeroderma pigmentosum-type neurological degeneration revealed marked atrophy of the cochlear sensory epithelium and neurons. The 19-year mean age of detection of clinically significant hearing loss in the patients with xeroderma pigmentosum with xeroderma pigmentosum-type neurological degeneration was 54 years younger than that predicted by international norms. The four frequency (0.5/1/2/4 kHz) pure-tone average correlated with degree of neurodegeneration (P < 0.001). In patients with xeroderma pigmentosum, aged 4-30 years, a four-frequency pure-tone average ≥10 dB hearing loss was associated with a 39-fold increased risk (P = 0.002) of having xeroderma pigmentosum-type neurological degeneration. Severity of hearing loss parallels neurological decline in patients with xeroderma pigmentosum-type neurological degeneration. Audiometric findings, complementation group, acute burning on minimal sun exposure and age were important predictors of xeroderma pigmentosum-type neurological degeneration. These results provide evidence that DNA repair is critical in maintaining neurological integrity of the auditory system.

Assessing Special Needs of Students with Hearing Impairment in Jordan and Its Relation to Some Variables

The main purpose of the present study was to assess special needs for students with hearing impairment. The sample of the study consisted of 231 students enrolled at schools for deaf children in Jordan, 113 males and 118 females. A special needs scale was developed and used in assessing special needs for students. The reliability and validity of the scale were established. The investigator used the t-test and ANOVA to answer the research questions. The results indicated that the differences between all pairs of means for both male and female respondents were statistically non-significant. Also, findings showed that the hearing-impaired students who have 55-69 dB hearing loss were more affected by communication needs, audiological rehabilitation needs, and learning style needs than hearing-impaired students who have 90 dB hearing loss and beyond hearing loss. In addition to, findings revealed that the grade groups of 2-5 and 6-8 were more affected than grade group of 9-11 for the following dependent variables: communication needs, social and emotional needs, personal needs, behavioral needs, and academic needs. Also, the grade group of 6-8 was more affected than the grade groups of 2-5 and 9-11 with respect to the dependent variable of learning style needs. Differences between mean scores of students who used sign language and total communication were in favor of students who used total communication on the dimensions of communication needs, audiological rehabilitation needs, behavioral needs, and learning style needs. The study recommended on meeting the needs of students with hearing impairment throughout planned programs.

Interaural Differences of Distortion Product Otoacoustic Emission Amplitudes in Patients with Unilateral Tinnitus

We aimed to determine whether abnormalities in outer hair cell (OHC) function were related to tinnitus through interaural comparison of distortion product otoacoustic emissions (DPOAEs). Cross-sectional study. Tertiary care university teaching hospital. Twenty-seven patients with unilateral tinnitus and pure-tone average of both ears ≤ 25 dB hearing loss (HL) at 500, 1000, 2000, and 4000 Hz were included. Pure-tone thresholds observed at 500 to 16,000 Hz and DPOAE amplitudes at f2 frequencies of 1001 to 6348 Hz were compared between the tinnitus ears and nontinnitus ears in patients with unilateral tinnitus. The pure-tone averages (13 ± 6 dB HL) in the nontinnitus ears were similar to those (15 ± 6 dB HL) in the tinnitus ears. There were no differences in pure-tone averages at all frequencies tested. While the DPOAE amplitudes measured at f2 frequencies of 1001 to 3174 Hz in tinnitus ears were not different from those in the nontinnitus ears, the tinnitus ears showed significantly reduced DPOAE amplitudes when compared with the nontinnitus ears at frequencies of 4004 to 6348 Hz. OHC dysfunction was correlated with tinnitus at high frequencies, and DPOAE amplitudes can provide additional information about cochlear dysfunction, which is complementary to pure-tone audiometry.

Hearing Outcomes After Surgical Plugging of the Superior Semicircular Canal by a Middle Cranial Fossa Approach

To determine postoperative hearing outcomes after surgical plugging via middle cranial fossa approach for superior semicircular canal dehiscence syndrome (SCDS). Clinical review. Tertiary care medical center. Forty-three cases of SCDS based on history, physical examination, vestibular function testing, and computed tomography imaging confirming the presence of a dehiscence. All patients underwent surgical plugging of the superior semicircular canal via middle cranial fossa approach. Pure tone audiometry was performed preoperatively and at 7 days and at least 1 month postoperatively. Change in air-bone gap (ABG) and pure tone average (PTA). Preoperative average ABG across 0.25, 0.5, 1, and 2 kHz was 16.0 dB (standard deviation [SD], 7.5 dB). At 7 days postoperatively, average ABG was 16.5 dB (SD, 11.1; p = 0.42), and at greater than 1 month was 8.1 dB (SD, 8.4; p < 0.001). 53% (95% confidence interval, 33-69) of affected ears had greater than 10 dB increase in their 4-frequency (0.5, 1, 2, and 4 kHz) PTA measured by bone-conduction (BC) threshold 7 days postoperatively and 25% (95% confidence interval, 8-39) at greater than 1 month postoperatively. Mean BC PTA of affected ears was 8.4 dB hearing loss (HL) (SD, 10.4) preoperatively. Compared with baseline, this declined to 19.2 dB HL (SD, 12.6; p < 0.001) at 7 days postoperatively and 16.4 dB HL (SD, 18.8; p = 0.01) at greater than 1 month. No significant differences in speech discrimination score were noted (F = 0.17). Low-frequency air-bone gap decreases after surgical plugging and seems to be due to both increased BC thresholds and decreased AC thresholds. Surgical plugging via a middle cranial fossa approach in SCDS is associated with mild high-frequency sensorineural hearing loss that persists in 25% but no change in speech discrimination.

Regeneration of Stereocilia of Hair Cells by Forced Atoh1 Expression in the Adult Mammalian Cochlea

The hallmark of mechanosensory hair cells is the stereocilia, where mechanical stimuli are converted into electrical signals. These delicate stereocilia are susceptible to acoustic trauma and ototoxic drugs. While hair cells in lower vertebrates and the mammalian vestibular system can spontaneously regenerate lost stereocilia, mammalian cochlear hair cells no longer retain this capability. We explored the possibility of regenerating stereocilia in the noise-deafened guinea pig cochlea by cochlear inoculation of a viral vector carrying Atoh1, a gene critical for hair cell differentiation. Exposure to simulated gunfire resulted in a 60–70 dB hearing loss and extensive damage and loss of stereocilia bundles of both inner and outer hair cells along the entire cochlear length. However, most injured hair cells remained in the organ of Corti for up to 10 days after the trauma. A viral vector carrying an EGFP-labeled Atoh1 gene was inoculated into the cochlea through the round window on the seventh day after noise exposure. Auditory brainstem response measured one month after inoculation showed that hearing thresholds were substantially improved. Scanning electron microscopy revealed that the damaged/lost stereocilia bundles were repaired or regenerated after Atoh1 treatment, suggesting that Atoh1 was able to induce repair/regeneration of the damaged or lost stereocilia. Therefore, our studies revealed a new role of Atoh1 as a gene critical for promoting repair/regeneration of stereocilia and maintaining injured hair cells in the adult mammal cochlea. Atoh1-based gene therapy, therefore, has the potential to treat noise-induced hearing loss if the treatment is carried out before hair cells die.

Endoscopic Myringotomy and Ventilation Tube Insertion under Topical Anesthesia

Endoscopic Myringotomy and Ventilation Tube Insertion under Topical Anesthesia

The Challenge of Detecting Minimal Hearing Loss in Audiometric Surveys

This tutorial provides framework and context for understanding the complex interaction of hearing measurement methodology and cohort social factors, as well as their relation to approaches to data interpretation and identification of minimal hearing loss (HL) in audiometric surveys. Pertinent archival studies were reviewed, and an original analysis on U.S. Centers for Disease Control and Prevention (CDC) audiometric survey data from children (ages 6-19) was performed. The definition of an otologically normal individual, the pass-fail criterion representing the upper limit of the range of normal hearing, and the quality of the audiometry affect the percentage of persons who are falsely identified as having a minimal HL. An upper limit of normal hearing of 15 dB HL yields an unacceptably high false-positive rate, particularly when the more variable higher audiometric frequencies are examined. When air-conduction thresholds are assessed in isolation to estimate potential noise damage, the failure to exclude persons who have possible middle and external ear problems, including earwax, results in high false-positive rates. When these factors and other limitations are considered, audiograms from teens from a recent CDC survey do not show evidence consistent with widespread noise-induced HL. Suggestions are made to improve the effectiveness of pure-tone audiometry and the identification of minimal HL.

Speech Recognition and Communication Outcomes With Cochlear Implantation in Usher Syndrome Type 3

Usher syndrome Type 3 (USH3) is an autosomal recessive disorder characterized by variable type and degree of progressive sensorineural hearing loss and retinitis pigmentosa. Cochlear implants are widely used among these patients. To evaluate the results and benefits of cochlear implantation in patients with USH3. A nationwide multicenter retrospective review. During the years 1995-2005, in 5 Finnish university hospitals, 19 patients with USH3 received a cochlear implant. Saliva samples were collected to verify the USH3 genotype. Patients answered to 3 questionnaires: Glasgow Benefit Inventory, Glasgow Health Status Inventory, and a self-made questionnaire. Audiological data were collected from patient records. All the patients with USH3 in the study were homozygous for the Finnish major mutation (p.Y176X). Either they had severe sensorineural hearing loss or they were profoundly deaf. The mean preoperative hearing level (pure-tone average, 0.5-4 kHz) was 110 ± 8 dB hearing loss (HL) and the mean aided hearing level was 58 ± 11 dB HL. The postoperative hearing level (34 ± 9 dB HL) and word recognition scores were significantly better than before surgery. According to the Glasgow Benefit Inventory scores and Glasgow Health Status Inventory data related to hearing, the cochlear implantation was beneficial to patients with USH3. Cochlear implantation is beneficial to patients with USH3, and patients learn to use the implant without assistance.

Counteracting tinnitus by acoustic coordinated reset neuromodulation

Subjective tinnitus is associated with pathologic enhanced neuronal synchronization. We used a model based desynchronization technique, acoustic coordinated reset (CR) neuromodulation, to specifically counteract tinnitus-related neuronal synchrony thereby inducing an unlearning of pathological synaptic connectivity and neuronal synchrony. In a prospective, randomized, single blind, placebo-controlled trial in 63 patients with chronic tonal tinnitus and up to 50 dB hearing loss we studied safety and efficacy of different doses of acoustic CR neuromodulation. We measured visual analogue scale and tinnitus questionnaire (TQ) scores and spontaneous EEG. CR treatment was safe, well-tolerated and caused a significant decrease of tinnitus loudness and symptoms. Placebo treatment did not lead to any significant changes. Effects gained in 12 weeks of treatment persisted through a preplanned 4-week therapy pause and showed sustained long-term effects after 10 months of therapy: response, i.e. a reduction of at least 6 TQ points, was obtained in 75% of patients with a mean TQ reduction of 50% among responders. CR therapy significantly lowered tinnitus frequency and reversed the tinnitus related EEG alterations. The CR-induced reduction of tinnitus and underlying neuronal characteristics indicates a new non-invasive therapy which might also be applicable to other conditions with neuronal hypersynchrony.