Identifying non-otologic risk factors for tinnitus: A systematic review.

BACKGROUND: The origin of tinnitus has been attributed to a peripheral auditory lesion, inducing bottom-up changes and resulting in the perception of a "phantom sound." However, non-auditory factors can co-exist as well, and can even lie at the origin of tinnitus development. An increasing body of literature focuses on psychological, (neuro)muscular, cardiovascular and many other influences and their respective associations with tinnitus prevalence. OBJECTIVE OF REVIEW: The purpose of this study was to provide a comprehensive description of these non-otologic risk factors, and to summarise the evidence in literature about their link with tinnitus. TYPE OF REVIEW: A narrative systematic review was conducted, following the Preferred Reporting Items for Systematic reviews and Meta-Analyses statement. SEARCH STRATEGY: The MEDLINE, Embase and Web of Science databases were systematically searched for eligible articles, supplemented with manual search methods and grey literature search. Epidemiological studies reporting on the relationship between various non-otologic risk factors and tinnitus were included. EVALUATION METHOD: Quality assessment was performed using the Hoy & Brooks tool. RESULTS: Fifty-five studies were included. Studies were of variable quality, with poor tinnitus definitions and evaluations or questionable sampling of the study population as main contributing factors for high risk of bias. Multiple associated factors have been identified, including cardiovascular, psychological, neurological, musculoskeletal and dietary factors. CONCLUSIONS: The current literature review identified multiple risk factors that could be of significant importance for tinnitus development, maintenance or aggravation. While causality remains uncertain, this systematic elaboration of possible tinnitus comorbidities/risk factors can help provide direction for future research, and can direct clinicians to identify patients at risk and treat relevant symptoms accordingly.

Invasive Neuromodulation as a Treatment for Tinnitus: A Systematic Review.

OBJECTIVE: Although the prevalence and burden of tinnitus is high, none of the available tinnitus treatments has been proven to be effective for the majority of tinnitus patients so far. Neuromodulation is currently gaining more interest to explore as tinnitus treatment. Because noninvasive neuromodulation has been shown to be effective in some tinnitus patients in the short term, more invasive techniques have been applied with variable success and without clear clinical applicability. As new insights into the neuropathophysiology of tinnitus arise, it seems essential to recapitulate the current evidence of invasive neuromodulation for tinnitus, to assess the quality of the available studies and identify gaps in this research domain. DATA SOURCES: MEDLINE, Embase, Web of Science and Clinical Trial Register. MATERIALS AND METHODS: We conducted a systematic review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. Studies since 2005 that reported on adult human subjects with chronic subjective tinnitus, who underwent some form of invasive neuromodulation, were included. Quality evaluation was performed using the modified Downs and Black checklist. RESULTS AND CONCLUSION: Twenty-one studies were included. Studies were often of low quality due to low sample sizes, lack of controlled designs, or investigating tinnitus as a secondary indication of neuromodulation. Current research results provide insufficient evidence to generally recommend invasive neuromodulation as an alternative treatment alternative for intractable tinnitus, although some promising effects are mentioned. Further research must be encouraged to gain more insight in this treatment including optimization of the technique, and standardization of tinnitus evaluation in subgroups.

The P300 auditory event-related potential as a method to assess the benefit of contralateral hearing aid use in bimodal listeners: a proof-of-concept.

Objective: Bimodal listeners vary in the amount of benefit they receive from wearing the contralateral hearing aid. This may partially depend on the listener's auditory processing capacities. The current study explores whether the P300 event-related potential can provide insight into the mechanisms underlying the benefits of wearing a contralateral hearing aid.Design: P300s were recorded using an oddball paradigm with 500 and 250 Hz tone-bursts as standard and deviant stimuli, respectively. Subjects counted the number of deviants - a measure of performance. N2b latencies, P300 latencies, N2b-P300 amplitudes, and performance were assessed during CI-only and bimodal listening.Study sample: Five bimodal listeners.Results: P300s were present in four subjects. Amplitudes were larger during bimodal listening (bimodal: 22.3 ± 4.83 µV, CI-only: 13.1 ± 3.86 µV). Both N2b and P300 latencies were shorter during bimodal (N2b: 265 ± 20.0 ms, P300: 551 ± 129.4 ms) than CI-only listening (N2b: 326 ± 42.2 ms, P300: 402 ± 38.4 ms). While performance often reached ceiling level, the difference between the standard and deviant was generally more salient during bimodal listening.Conclusions: This study provides a proof-of-concept, suggesting that P300s may provide insight into benefits that are not always measurable with behavioural tasks.

Test-Retest Reproducibility of Response Duration in Tinnitus Patients With Positive Residual Inhibition.

Purpose Functional imaging is often used to try to elucidate the pathophysiological mechanism of tinnitus. Residual inhibition, the temporary suppression of tinnitus after application of a masking noise, could be an interesting technique to modulate tinnitus perception in functional imaging paradigms. The purposes of this study were to primarily assess reproducibility of the (partial) positive residual inhibition response duration in patients with tinnitus and to explore its utility in experimental designs. Method Patients with tinnitus exhibiting a (partial) positive residual inhibition response or tinnitus reduction after a 1-min white noise presentation were selected from a broad consulting tinnitus population. In 27 patients, this response was tested 4 times: twice during initial testing and twice during a retest of the psychoacoustic tinnitus measures, 4-8 weeks after initial consultation. In 17 patients with stable residual inhibition responses, reproducibility of response duration, the duration of tinnitus reduction up to pretesting state, was analyzed. Results Initial testing showed a residual inhibition duration of 29.5 s on average. Test-retest reproducibility of response duration was shown to be reliable with an ICC(3, 4) of .871 (95% CI [0.733, 0.948]) and a standard error of measurement of 6.64 s. Conclusions This study indicates the good test-retest reproducibility of residual inhibition duration in our subset of 17 patients with stable (partial) positive residual inhibition. Residual inhibition is, therefore, a technique that can potentially be used for temporary tinnitus manipulation in experimental paradigms to unravel tinnitus pathophysiology.

Bilateral cochlear implantation or bimodal listening in the paediatric population: Retrospective analysis of decisive criteria.

INTRODUCTION: In children with bilateral severe to profound hearing loss, bilateral hearing can be achieved by either bimodal stimulation (CIHA) or bilateral cochlear implantation (BICI). The aim of this study was to analyse the audiologic test protocol that is currently applied to make decisions regarding the bilateral hearing modality in the paediatric population. METHODS: Pre- and postoperative audiologic test results of 21 CIHA, 19 sequential BICI and 12 simultaneous BICI children were examined retrospectively. RESULTS: Deciding between either simultaneous BICI or unilateral implantation was mainly based on the infant's preoperative Auditory Brainstem Response thresholds. Evolution from CIHA to sequential BICI was mainly based on the audiometric test results in the contralateral (hearing aid) ear after unilateral cochlear implantation. Preoperative audiometric thresholds in the hearing aid ear were significantly better in CIHA versus sequential BICI children (p < 0.001 and p = 0.001 in unaided and aided condition, respectively). Decisive values obtained in the hearing aid ear in favour of BICI were: An average hearing threshold measured at 0.5, 1, 2 and 4 kHz of at least 93 dB HL without, and at least 52 dB HL with hearing aid together with a 40% aided speech recognition score and a 70% aided score on the phoneme discrimination subtest of the Auditory Speech Sounds Evaluation test battery. CONCLUSIONS: Although pure tone audiometry offers no information about bimodal benefit, it remains the most obvious audiometric evaluation in the decision process on the mode of bilateral stimulation in the paediatric population. A theoretical test protocol for adequate evaluation of bimodal benefit in the paediatric population is proposed.

Phenotype of a Belgian Family With 6p25 Deletion Syndrome.

BACKGROUND: The 6p25 deletion syndrome is one of the many syndromes with both hearing impairment as well as vision impairment. However, the audiometric characteristics and radiological findings of patients with 6p25 deletions are only scarcely described in literature. This study focused on characterizing the audiometric and radiological features of a Belgian family with a chromosome 6p25 deletion. OBJECTIVE: To evaluate the hearing impairment, audiometric testing and radiological examination of the temporal bones in 3 family members with a 3.4 Mb deletion in chromosome band 6p25. RESULTS: All 3 family members demonstrated slowly progressive sensorineural or mixed hearing impairment. Radiologic examination revealed thickened and sclerotic stapes in all patients and a minor internal partition type II of the cochlea in 2 patients. CONCLUSION: There is a significant phenotypic variability within and among families with the 6p25 deletion syndrome. A thorough genotype-phenotype correlation is difficult because of the small number of affected patients and the limited clinical data available. More clinical data of families with 6p25 deletions need to be published in order to create a reliable and precise phenotypic characterization. However, our findings can facilitate counseling of hearing impairment caused by 6p25 deletions.

Cephalometrics in Stickler syndrome: Objectification of the typical facial appearance.

INTRODUCTION: Stickler syndrome is a connective tissue disorder characterized by orofacial, ocular, skeletal and auditory symptoms. The orofacial phenotype mainly consists of midfacial hypoplasia, micrognathia and cleft palate. Large phenotypic variability is evident though. Few studies have tried to substantiate the typical facial appearance in Stickler syndrome patients. METHODS: Molecularly confirmed Stickler patients were invited to undergo cephalometric analysis based on a lateral radiograph in standardized conditions. Angular and linear measurements were performed according to Steiner's and Sassouni's analysis and compared with age- and gender-matched reference values. RESULTS: Thirteen patients aged 10-62y were included, twelve of whom had type 1 Stickler syndrome (COL2A1 mutation) and one type 2 Stickler syndrome (COL11A1 mutation). The position of maxilla and mandible relative to the cranial base was not significantly different from the reference population (S-N-A: p = 0.73, S-N-B: p = 0.43). The mandibular plane and y-axis showed an elevated angle with the cranial base in most patients, although not significant for the total group (S-N to Go-Me: p = 0.20, S-N to S-Gn: p = 0.18). Dental analysis was normal, except for a higher overjet value (p = 0.006) and a higher angle between occlusal plane and Frankfort plane (p = 0.022). CONCLUSION: Cephalometric analysis was not able to thoroughly prove the abnormal facial appearance in Stickler syndrome. The majority of patients had normal dentofacial proportions. The most frequently observed anomaly in our series is a rather short and posteriorly rotated mandible, but clinical variability is high.

Age-Related Changes in Binaural Interaction at Brainstem Level.

OBJECTIVES: Age-related hearing loss hampers the ability to understand speech in adverse listening conditions. This is attributed to a complex interaction of changes in the peripheral and central auditory system. One aspect that may deteriorate across the lifespan is binaural interaction. The present study investigates binaural interaction at the level of the auditory brainstem. It is hypothesized that brainstem binaural interaction deteriorates with advancing age. DESIGN: Forty-two subjects of various age participated in the study. Auditory brainstem responses (ABRs) were recorded using clicks and 500 Hz tone-bursts. ABRs were elicited by monaural right, monaural left, and binaural stimulation. Binaural interaction was investigated in two ways. First, grand averages of the binaural interaction component were computed for each age group. Second, wave V characteristics of the binaural ABR were compared with those of the summed left and right ABRs. RESULTS: Binaural interaction in the click ABR was demonstrated by shorter latencies and smaller amplitudes in the binaural compared with the summed monaural responses. For 500 Hz tone-burst ABR, no latency differences were found. However, amplitudes were significantly smaller in the binaural than summed monaural condition. An age-effect was found for 500 Hz tone-burst, but not for click ABR. CONCLUSIONS: Brainstem binaural interaction seems to decline with age. Interestingly, these changes seem to be stimulus-dependent.

Auditory phenotype in Stickler syndrome: results of audiometric analysis in 20 patients.

Hearing loss in Stickler syndrome has received little attention due to the often more disabling ocular, orofacial and skeletal manifestations. Estimates suggest a global prevalence of sensorineural hearing loss (SNHL) ranging from 50 % to about 100 % though, depending on the underlying Stickler genotype. By performing extensive audiometric analysis in Stickler patients, we aimed to further elucidate the auditory phenotype. Twenty molecularly confirmed Stickler patients (age 10-62 year), of whom sixteen with type 1 Stickler syndrome (COL2A1 mutation) and four with type 2 Stickler syndrome (COL11A1 mutation) underwent an otological questionnaire, clinical examination, pure tone and speech audiometry, tympanometry and otoacoustic emission testing. Cross-sectional and longitudinal regression analysis of the audiograms was performed to assess progression. In type 1 Stickler syndrome, 75 % demonstrated hearing loss, predominantly in the high frequencies. No significant progression beyond presbyacusis was observed. All type 2 Stickler patients exhibited mild-to-moderate low- and mid-frequency SNHL and moderate-to-severe high-frequency SNHL. In both types, hearing loss was observed in childhood. Otoacoustic emissions were only detectable in 7/40 ears and had very low amplitudes, even in frequency bands with normal hearing on pure tone audiometry. Type 1 Stickler syndrome is characterized by a mild high-frequency SNHL, emerging in childhood and non-progressive. Absent otoacoustic emissions are a frequent finding. Patients with type 2 Stickler syndrome exhibit early-onset moderate SNHL affecting all frequencies with a sloping audiogram. Taking into account the visual impairment in many patients, we recommend regular auditory follow-up in patients with Stickler syndrome, especially in childhood.

Binaural interaction in the auditory brainstem response: a normative study.

OBJECTIVE: Binaural interaction can be investigated using auditory evoked potentials. A binaural interaction component can be derived from the auditory brainstem response (ABR-BIC) and is considered evidence for binaural interaction at the level of the brainstem. Although click ABR-BIC has been investigated thoroughly, data on 500 Hz tone-burst (TB) ABR-BICs are scarce. In this study, characteristics of click and 500 Hz TB ABR-BICs are described. Furthermore, reliability of both click and 500 Hz TB ABR-BIC are investigated. METHODS: Eighteen normal hearing young adults (eight women, ten men) were included. ABRs were recorded in response to clicks and 500 Hz TBs. ABR-BICs were derived by subtracting the binaural response from the sum of the monaural responses measured in opposite ears. RESULTS: Good inter-rater reliability is obtained for both click and 500 Hz TB ABR-BICs. The most reliable peak in click ABR-BIC occurs at a mean latency of 6.06 ms (SD 0.354 ms). Reliable 500 Hz TB ABR-BIC are obtained with a mean latency of 9.47 ms (SD 0.678 ms). Amplitudes are larger for 500 Hz TB ABR-BIC than for clicks. CONCLUSION: The most reliable peak in click ABR-BIC occurs at the downslope of wave V. Five hundred Hertz TB ABR-BIC is characterized by a broad positivity occurring at the level of wave V. SIGNIFICANCE: The ABR-BIC is a useful technique to investigate binaural interaction in certain populations. Examples are bilateral hearing aid users, bilateral cochlear implant users and bimodal listeners. The latter refers to the combination of unilateral cochlear implantation and contralateral residual hearing. The majority of these patients have residual hearing in the low frequencies. The current study suggests that 500 Hz TB ABR-BIC may be a suitable technique to assess binaural interaction in this specific population of cochlear implant users.

Temporal bone imaging in osteogenesis imperfecta patients with hearing loss.

OBJECTIVES/HYPOTHESIS: Osteogenesis imperfecta (OI) is an autosomal-dominant connective-tissue disorder, predominantly characterized by bone fragility. Conductive hearing loss develops in half of the OI patients and often progresses to mixed loss. Findings of computed tomography (CT) and magnetic resonance (MR) imaging of the temporal bone in the largest series of OI patients to date are presented and correlated with the audiograms. STUDY DESIGN: Retrospective case series. METHODS: CT images and audiograms of 17 hearing-impaired OI patients, aged 9 to 67 years, were analyzed retrospectively. In four patients, MR imaging was performed as well. Imaging abnormalities were correlated with type and severity of hearing loss deduced from the audiograms. RESULTS: CT revealed fenestral hypodense foci in the fissula ante fenestram (25 of 33 ears), oval window (23 of 33 ears), and round window (20 of 33 ears). Retrofenestral hypodensities were observed, affecting the cochlear turns (16 of 33 ears), facial nerve canal (10 of 33 ears), or semicircular canals (6 of 33 ears), or appearing like the fourth turn of the cochlea (11 of 33 ears). The site of hypodensities corresponded to the type of hearing loss in 72.2% of the OI ears. The air-bone gap and bone-conduction thresholds showed significant positive associations with the number of affected fenestral (P < .05) and retrofenestral structures (P < .01), respectively. Gadolinium-enhanced MR images demonstrated active lesions in three patients with mixed hearing loss or deafness. CONCLUSIONS: The site of hypodensities on temporal bone CT images in OI corresponds to presence and type of hearing loss determined by audiometry. The more severe the hearing loss, the more affected temporal bone structures in OI.

Hearing impairment in Stickler syndrome: a systematic review.

BACKGROUND: Stickler syndrome is a connective tissue disorder characterized by ocular, skeletal, orofacial and auditory defects. It is caused by mutations in different collagen genes, namely COL2A1, COL11A1 and COL11A2 (autosomal dominant inheritance), and COL9A1 and COL9A2 (autosomal recessive inheritance). The auditory phenotype in Stickler syndrome is inconsistently reported. Therefore we performed a systematic review of the literature to give an up-to-date overview of hearing loss in Stickler syndrome, and correlated it with the genotype. METHODS: English-language literature was reviewed through searches of PubMed and Web of Science, in order to find relevant articles describing auditory features in Stickler patients, along with genotype. Prevalences of hearing loss are calculated and correlated with the different affected genes and type of mutation. RESULTS: 313 patients (102 families) individually described in 46 articles were included. Hearing loss was found in 62.9%, mostly mild to moderate when reported. Hearing impairment was predominantly sensorineural (67.8%). Conductive (14.1%) and mixed (18.1%) hearing loss was primarily found in young patients or patients with a palatal defect. Overall, mutations in COL11A1 (82.5%) and COL11A2 (94.1%) seem to be more frequently associated with hearing impairment than mutations in COL2A1 (52.2%). CONCLUSIONS: Hearing impairment in patients with Stickler syndrome is common. Sensorineural hearing loss predominates, but also conductive hearing loss, especially in children and patients with a palatal defect, may occur. The distinct disease-causing collagen genes are associated with a different prevalence of hearing impairment, but still large phenotypic variation exists. Regular auditory follow-up is strongly advised, particularly because many Stickler patients are visually impaired.

Stapes surgery in osteogenesis imperfecta: retrospective analysis of 34 operated ears.

Intraoperative findings of stapes surgery in 34 ears from 22 patients with genetically confirmed osteogenesis imperfecta (OI) are reported, as well as the audiometric results after the longest postoperative follow-up published to date. Twenty-nine out of 34 ears underwent primary stapes surgery and 5 ears revision surgery. Postoperative audiometric follow-up ranged from 6 months to 37 years. Stapes footplates were fixed in all ears. Additionally, footplates were thickened or fragile, stapes crura atrophic or fractured, and middle ear mucosae thickened or hypervascularized. Short-term postoperative audiometry revealed improved hearing and reduced air-bone gaps in 28/29 primary operated ears and in all revision cases. In the 22 ears with long-term postoperative follow-up (mean duration: 16 years), hearing gain was still significant at the latest audiometric evaluation. Independently of the patients being diagnosed with OI type I or IV and independently of the underlying OI genotype, beneficial results are obtained in the majority of OI patients undergoing primary or revision stapes surgery for reduction of conductive hearing loss components caused by stapes footplate fixation. Despite the progressive course of the concomitant sensorineural component, hearing gain remains beneficial over several decades.

Association between bone mineral density and hearing loss in osteogenesis imperfecta.

OBJECTIVES/HYPOTHESIS: Osteogenesis imperfecta (OI) is a heritable connective tissue disorder, predominantly characterized by bone fragility. In half of the patients, progressive hearing loss develops, which is associated with abnormal bony changes involving the middle ear ossicles and stapes footplate. In the present study, we investigated whether the development of hearing loss in OI may be related to the overall aberrant bone quality. STUDY DESIGN: Observational study. METHODS: Following audiologic evaluation, 56 adult OI patients were classified as presenting normal hearing or conductive/mixed or pure sensorineural hearing loss. Areal bone mineral density (BMD) (aBMD) was measured using lumbar spine (LS) and whole body (WB) dual X-ray absorptiometry. By means of peripheral computed tomography, volumetric BMD (vBMD) and morphometric bone parameters were determined at distal and proximal radius, providing separate results for trabecular and cortical bone. The obtained bone parameters were compared between normal-hearing OI patients and those with either conductive/mixed or pure sensorineural hearing loss. RESULTS: Z scores demonstrated decreased LS aBMD, WB aBMD, and trabecular vBMD in OI adults compared to the healthy population. Patients with conductive/mixed hearing loss had lower trabecular vBMD compared to those with normal hearing or pure sensorineural loss at both whole-group and between-relatives comparisons. CONCLUSIONS: It is hypothesized that OI patients with lower BMD might be more susceptible to accumulating microfractures, which may interfere with the bone remodeling inhibition pathways in the temporal bone and, therefore, contribute to stapes footplate fixation and a conductive hearing loss component.

Audiologic phenotype of osteogenesis imperfecta: use in clinical differentiation.

OBJECTIVES: To describe the audiologic phenotype in osteogenesis imperfecta (OI). STUDY DESIGN: Observational study. SETTING: Tertiary referral center. PATIENTS: One hundred eighty-two patients with genetically confirmed OI, aged 3 to 89 years. INTERVENTION: Diagnostic hearing evaluation through otoadmittance and acoustic stapedius reflex measurements, pure tone, and speech audiometry. MAIN OUTCOME MEASURE(S): Prevalence, type, severity, symmetry, and audiometric configuration of the hearing loss in OI. Progression of hearing thresholds was determined by constructing age-related typical audiograms. RESULTS: Approximately 52.2% of all OI patients demonstrated hearing loss unilaterally (7.7%) or bilaterally (44.5%). Pure conductive, mixed, and pure sensorineural hearing losses were observed in 8.5%, 37.8%, and 11.6% of OI ears, respectively. Multiple linear regression revealed that thresholds progressed by 0.5 dB/yr at 0.25 kHz to 0.8 dB/yr at 0.8 kHz in the ears with conductive or mixed hearing loss. Pure sensorineural hearing loss progressed by less than 0.1 dB/yr at 0.25 kHz to 1.2 dB/yr at 8.0 kHz. Audiometric configuration was predominantly flat (70.5%) in the ears with conductive/mixed loss and sloping (50.0%) in those with pure sensorineural loss. CONCLUSION: Patients with OI are at risk for hearing loss. The hearing loss in OI may initiate at a young age and is progressive. However, the rate of progression, as well as the hearing loss severity, onset, and configuration depend on the type of hearing loss, which may be conductive/mixed or pure sensorineural. For both types, age-related threshold audiograms are constructed and may help the clinician to estimate the course of the hearing loss in patients with OI. In addition, they may be valuable to distinguish between hearing loss associated with OI and other similar forms of hearing loss, such as in otosclerosis.

Osteogenesis Imperfecta: the audiological phenotype lacks correlation with the genotype.

BACKGROUND: Osteogenesis Imperfecta (OI) is a heritable connective tissue disorder mainly caused by mutations in the genes COL1A1 and COL1A2 and is associated with hearing loss in approximately half of the cases. The hearing impairment usually starts between the second and fourth decade of life as a conductive hearing loss, frequently evolving to mixed hearing loss thereafter. A minority of patients develop pure sensorineural hearing loss. The interindividual variability in the audiological characteristics of the hearing loss is unexplained. METHODS: With the purpose of evaluating inter- and intrafamilial variability, hearing was thorougly examined in 184 OI patients (type I: 154; type III: 4; type IV: 26), aged 3-89 years, with a mutation in either COL1A1 or COL1A2 and originating from 89 different families. Due to the adult onset of hearing loss in OI, correlations between the presence and/or characteristics of the hearing loss and the underlying mutation were investigated in a subsample of 114 OI patients from 64 different families who were older than 40 years of age or had developed hearing loss before the age of 40. RESULTS: Hearing loss was diagnosed in 48.4% of the total sample of OI ears with increasing prevalence in the older age groups. The predominant type was a mixed hearing loss (27.5%). A minority presented a pure conductive (8.4%) or pure sensorineural (12.5%) loss. In the subsample of 114 OI subjects, no association was found between the nature of the mutation in COL1A1 or COL1A2 genes and the occurrence, type or severity of hearing loss. Relatives originating from the same family differed in audiological features, which may partially be attributed to their dissimilar age. CONCLUSIONS: Our study confirms that hearing loss in OI shows a strong intrafamilial variability. Additional modifications in other genes are assumed to be responsible for the expression of hearing loss in OI.

Earlier intervention leads to better sound localization in children with bilateral cochlear implants.

We present sound localization results from 30 children with bilateral cochlear implants. All children received their implants sequentially, at ages from 6 months to 9 years for the first implant and 1.5-12 years for the second implant, with delays of 10 months to 9 years. Localization was measured in the sound field, with a broadband bell-ring presented from 1 of 9 loudspeakers positioned in the frontal horizontal plane. The majority of the children (63%) were able to localize this signal significantly better than chance level. Mean absolute error scores varied from 9 to 51 degrees (root mean square error scores from 13 to 63 degrees ). The best scores were obtained by children who received their first implant before the age of 2 years and by children who used hearing aids prior to implantation for a period of 18 months or longer. Age at second implantation was important in the group of children who did not use a contralateral hearing aid during the unilateral implant period. Additionally, children who attended a mainstream school had significantly better localization scores than children who attended a school for the deaf. No other child or implantation variables were related to localization performance. Data of parent questionnaires derived from the Speech, Spatial and Qualities of Hearing Scale were significantly correlated with localization performance. This study shows that the sound localization ability of children with bilateral cochlear implants varies across subjects, from near-normal to chance performance, and that stimulation early in life, acoustically or electrically, is important for the development of this capacity.

Audiometric, surgical, and genetic findings in 15 ears of patients with osteogenesis imperfecta.

OBJECTIVES/HYPOTHESIS: To provide data on the outcome of stapes surgery in patients with osteogenesis imperfecta (OI). The audiometric results of 15 ears (12 patients), in which a stapes operation was performed, are presented and compared with results from literature. STUDY DESIGN: Retrospective study. METHODS: In 12 patients with genetically confirmed OI, intraoperative findings and audiometric evaluations were recorded. RESULTS: In all patients the genetic mutation was located in the COL1A1 gene. Surgical findings in OI may be particular like mobile, atrophic stapes crura combined with a fixation of the stapes footplate, which may be thickened, and a hypervascularized or thickened middle-ear mucosa. Outcome for hearing in 13 primary surgered ears was good because at short-term follow-up the air-bone gap was reduced in all cases. These results were maintained in the long-term, with exception of one ear, in which progression of the sensorineural component occurred shortly after the operation. Although initial success was noted in two ears with revision surgery, in the long term this was only maintained in one of them. CONCLUSIONS: In general, stapes surgery is successful in resolving the conductive hearing loss in OI patients, even in the long term. Hearing loss in OI is mostly of the mixed type, and the sensorineural component is reported to be progressive. Stapedotomy, by improving the hearing level, may facilitate the rehabilitation with a hearing aid. Because the identified mutation could be located in the COL1A1 gene in all patients, conductive hearing loss in OI caused by stapes fixation is possibly linked to a mutation in this gene. Laryngoscope, 2009.

Sound localization, sound lateralization, and binaural masking level differences in young children with normal hearing.

OBJECTIVES: In this study, procedures for measuring sound localization, sound lateralization, and binaural masking level differences (BMLDs) in young children were developed. Sensitivity for these tasks was assessed in large groups of children between 4 and 9 yr of age to investigate potential developmental trends. DESIGN: Sound localization was measured in the sound field, with a broadband bell-ring presented from one of nine loudspeakers positioned in the frontal horizontal field. A group of 33 children between 4 and 6 yr of age and 5 adults took part in this experiment. Sound lateralization based on interaural time differences was measured with headphones in 49 children between 4 and 9 yr of age and 10 adults. A low-frequency stimulus containing harmonics 2 to 5 from a click train with a rate of 160 Hz was used. In the BMLD test, the same filtered click train was presented diotically or dichotically (phase reversed or time delayed) in a broadband (200 to 1000 Hz) frozen noise to 23 children between 4 and 6 yr of age and 10 adults. For comparison with literature, additional measurements with a 500-Hz sinusoid were administered to adults. All tasks were adapted to the interest and attention span of young children. RESULTS: Children of 5 yr of age did not perform significantly different from adults on the sound localization task, but mean absolute errors were larger for the 4-yr-olds. Also on the BMLD task, 5-yr-old children performed at the adult level, whereas the 4-yr-old children obtained significantly less binaural unmasking compared with the adults. Concerning sound lateralization, a small but significant difference between adults and children existed, but no age effects were apparent in the 4- to 9-yr-old group. Overall, the variation was relatively large in the 4-yr-old group, with some of the children performing at adult level, in all three tasks. CONCLUSIONS: The results of this study show that the modified procedures are suitable for testing children from the age of 4 to 5 yr. Furthermore, it seems that binaural hearing capacities of the 5-yr-olds are similar to those of adults. Several observations led to the hypothesis that the observed age differences between 4-yr-olds and older subjects on localization and BMLD or between those 4- to 9-yr old and adults on lateralization, were attributable to both a development in binaural hearing and to nonauditory factors, such as task comprehension, attention, and testing conditions. It is possible that the developmental process is more obvious and prolonged in other aspects of binaural hearing, which require more dynamic or more central processing.

Bilateral cochlear implants in children: binaural unmasking.

Bilateral cochlear implants (CIs) may offer deaf children a range of advantages compared to unilateral CIs. However, speech perception in noise is mainly facilitated by better-ear effects and much less by interaural comparisons or true 'binaural' hearing. Little is known about the development of the binaural auditory system with CIs provided at a young age. It is possible that, as with adults, binaural sensitivity exists but is not accessed due to technical limitations in electrical stimulation methods. In this paper, we present results on binaural hearing in children with bilateral CIs. Binaural masking level differences (BMLDs) were measured for a 180-degree phase shift in a 125-Hz sinusoid, presented in a 50-Hz-wide noise band and modulating a 1000-pps carrier pulse train. Stimuli were presented to a single electrode in the middle of the electrode array at both ears. Eight children between 6 and 15 years of age participated in this study. Six children had a significantly better detection threshold when the signal was out of phase (dichotic) between two ears than when it was in phase (diotic), with a mean difference (BMLD) of 6.4 dB. The present results show that children with bilateral CIs are sensitive to binaural cues in electrical stimuli, similar to adults, even when implants are provided at a later age and with a longer delay between implantations.