Congenital Single-sided Deafness Research Articles

Dissociated Representation of Binaural Cues in Single-Sided Deafness: Implications for Cochlear Implantation.

Congenital single-sided deafness (SSD) leads to an aural preference syndrome that is characterized by overrepresentation of the hearing ear in the auditory system. Cochlear implantation (CI) of the deaf ear is an effective treatment for SSD. However, the newly introduced auditory input in congenital SSD often does not reach expectations in late-implanted CI recipients with respect to binaural hearing and speech perception. In a previous study, a reduction of interaural time difference (ITD) sensitivity has been shown in unilaterally congenitally deaf cats (uCDC). In the present study, we focused on the interaural level difference (ILD) processing in the primary auditory cortex. The uCDC group was compared with hearing cats (HC) and bilaterally congenitally deaf cats (CDC). The ILD representation was reorganized, replacing the preference for the contralateral ear with a preference for the hearing ear, regardless of the cortical hemisphere. In accordance with the previous study, uCDCs were less sensitive to interaural time differences than HCs, resulting in unmodulated ITD responses, thus lacking directional information. Such incongruent ITDs and ILDs cannot be integrated for binaural sound source localization. In normal hearing the predominant effect of each ear is excitation of auditory cortex in the contralateral cortical hemisphere and inhibition in the ipsilateral hemisphere. In SSD, however, auditory pathways reorganized such that the hearing ear produced greater excitation in both cortical hemispheres and the deaf ear produced weaker excitation and preserved inhibition in both cortical hemispheres.Significance statement Congenital single-sided deafness is a clinically relevant form of hearing loss. We studied this phenomenon in a unique animal model with congenital deafness of one ear and normal hearing of the other ear with electrical stimulation on both ears. Differential effects of single-sided deafness on interaural time and level difference sensitivity were observed, consistent with the aural preference syndrome. The influence of the previously hearing ear became dominant and the deaf ear mildly suppressive in the majority of units in both hemispheres, resulting in an inconsistent representation of binaural cues. These changes in neuronal representation of the two ears explain why late restoration with cochlear implants in single-sided deafness often results in insufficient benefit of the previously deaf ear.

Clinical experience of an adhesive bone conduction hearing system in children with congenital single-sided deafness

ObjectivesThis study aimed to investigate the effects of an adhesive bone conduction device (aBCD) in children with congenital single-sided deafness (SSD). Specifically, we examined whether the aBCD elicits improvement in the speech perception ability of children with congenital SSD and whether using this device would adversely affect the horizontal localisation abilities of these children. MethodsThirteen school-aged children with SSD and seven children with Normal Hearing (NH) were included in this study. Speech perception in noise was measured using the Mandarin Speech Test Materials and sound localisation performance was evaluated using broadband noise stimuli (0.5–20 kHz), randomly played from seven loudspeakers at different stimulus levels (65-, 70-, and 75-dB SPL). ResultsAll children with SSD showed inferior speech perception and sound localisation performance compared with children with NH. The aBCD use remarkably improved the speech perception abilities of these children under quiet and noise conditions; however, their sound localisation abilities neither improved nor deteriorated. ConclusionThis study reveals the effectiveness and safety of a non-surgical aBCD in paediatric patients with SSD. Our results provide a theoretical basis for early hearing intervention with an aBCD in children with congenital SSD who are temporarily unable to undergo ear surgery. Level of evidenceLevel 3.

Long-Term Outcome of Cochlear Implantation in Children With Congenital, Perilingual, and Postlingual Single-Sided Deafness.

We investigated the long-term outcomes of children with single-sided deafness (SSD) after cochlear implant (CI) surgery, during and after rehabilitation, and compared the results of children with congenital, perilingual, and postlingual SSD. We evaluated the impact of SSD at age at onset and duration of deafness on their performance. Thirty-six children with SSD treated with CI participated in the study: 20 had congenital, seven perilingual (defined: >0 to 4 years), and nine had postlingual deafness (defined as >4 years of age). Their outcome with CI were measured on both subjective and objective scales: duration of device use, speech intelligibility in noise and in quiet, bilateral hearing and localization ability, quality of life and hearing, presence and loudness of tinnitus, and hearing ability of the better hearing ear. After a mean follow-up time of 4.75 years, 32 of the 36 children used their CI on a regular basis. The remaining four children were nonusers. These children had congenital SSD and were older than three years at the time of CI surgery. Overall, for congenital/perilingual and postlingual SSD, speech intelligibility in noise and the Speech, Spatial and Qualities of Hearing Scale (SSQ) speech subscore were significantly improved, as were their subjective and objective localization ability and hearing-related quality of life. Children with postlingual SSD benefited from the CI with regard to speech intelligibility, SSQ speech/spatial/total score, and localization error, and children with congenital SSD showed better results with a short duration of deafness of less than 3 years compared with those with a longer deafness period. Cochlear implantation is a successful treatment for children with congenital/perilingual or postlingual SSD. Results largely differed with respect to the onset and duration of deafness, and better outcomes were achieved by children with postlingual SSD and with a short duration of deafness. Our data also confirmed that children with congenital SSD should be implanted with a CI within three years of age.

A Porcine Congenital Single-Sided Deafness Model, Its Population Statistics and Degenerative Changes.

ObjectiveTo describe and study the population statistics, hearing phenotype, and pathological changes of a porcine congenital single-sided deafness (CSSD) pedigree.MethodsClick auditory brainstem response (ABR), full-frequency ABR, and distortion product otoacoustic emission (DPOAE) were used to assess the hearing phenotype of the strain. Tympanogram was used to assess the middle ear function since birth. Celloidin embedding–hematoxylin–eosin (CE-HE) stain and scanning electron microscopy (SEM) were used to study the pathological changes of cochlear microstructures. Chi-square analysis was used to analyze the relation between hearing loss and other phenotypes.ResultsThe mating mood of CSSD with CSSD was most efficient in breeding-targeted CSSD phenotype (47.62%), and the prevalence of CSSD reached 46.67% till the fifth generation, where 42.22% were bilateral hearing loss (BHL) and 9.00% were normal hearing (NH) individuals. Hearing loss was proved to have no relation with coat color (P = 0.0841 > 0.05) and gender (P = 0.4621 > 0.05) by chi-square analysis. The deaf side of CSSD offspring in the fifth generation had no relation with that of their maternal parent (P = 0.2387 > 0.05). All individuals in this strain exhibited congenital severe to profound sensorineural hearing loss with no malformation and dysfunction of the middle ear. The good hearing ear of CSSD stayed stable over age. The deaf side of CSSD and BHL presented cochlear and saccular degeneration, and the hair cell exhibited malformation since birth and degenerated from the apex to base turn through time. The pathology in BHL cochlea progressed more rapidly than CSSD and till P30, the hair cell had been totally gone. The stria vascularis (SV) was normal since birth and degenerated through time and finally exhibited disorganization of three layers of cells.ConclusionThis inbred porcine strain exhibited high and stable prevalence of CSSD, which highly resembled human non-syndromic CSSD disease. This porcine model could be used to further explore the etiology of CSSD and serve as an ideal tool for the studies of the effects of single-sided hearing deprivation on neural, cognitive, and behavioral developments and the benefits brought by CI in CSSD individuals.

Cochlear Implantation in Children With Single-Sided Deafness

In 2019, the US Food and Drug Administration approved cochlear implantation for children with single-sided deafness (SSD). The absence of robust clinical data specific to pediatric patients to guide shared decision-making and to identify potential advantages is a challenge in family counseling. To evaluate the audiological and patient-reported outcomes in children who underwent cochlear implantation for SSD and to assess the association between time of implantation, subjective outcomes, and cochlear implant device use rates. MEDLINE, Embase, Scopus, Cochrane, and PubMed were searched for English-language articles that were published in a peer-reviewed journal from database inception to February 18, 2020. Inclusion criteria were designed to capture studies that evaluated pediatric patients (1) younger than 18 years, (2) with a diagnosis of SSD for which they underwent a cochlear implantation, and (3) with at least 1 outcome of interest measured numerically: speech perception, sound localization, device use, and patient-reported outcomes. Of the 526 articles reviewed, 12 (2.3%) met the selection criteria. The Meta-analyses Of Observational Studies in Epidemiology (MOOSE) reporting guidelines were followed. Data were pooled using fixed-effect and random-effect models. The following information was obtained from each article: study characteristics, patient characteristics, hearing loss and intervention characteristics, and outcomes. Outcomes were (1) postoperative changes in speech perception (in quiet was measured as a proportion of correct responses, and in noise was measured as decibel signal to noise ratio for speech reception threshold) and sound localization (measured in degree of localization error), (2) patient-reported audiological outcomes (measured by the speech, spatial, and qualities of hearing scale), and (3) device use rates among children who received cochlear implantation for SSD. Twelve observational studies that evaluated 119 children (mean [SD] age, 6.6 [4.0] years) with SSD who received a cochlear implant were included. Most children showed clinically meaningful improvement in speech perception in noise (39 of 49 children [79.6%]) and in quiet (34 of 42 children [81.0%]). Long duration of deafness (>4 years in congenital SSD and >7 years in perilingual SSD) was the most commonly proposed reason for lack of improvement. Sound localization as measured by degrees of error from true location (mean difference [MD], -24.78°; 95% CI, -34.16° to -15.40°; I2 = 10%) improved statistically significantly after cochlear implantation. Patients with acquired SSD and shorter duration of deafness compared with those with congenital SSD reported greater improvements in speech (MD, 2.27; 95% CI, 1.89-2.65 vs 1.58; 95% CI, 1.00-2.16) and spatial (MD, 2.95; 95% CI, 2.66-3.24 vs 1.68; 95% CI, 0.96-2.39) hearing qualities. The duration of deafness among device nonusers was statistically significantly longer than the duration of deafness among regular device users (median difference, 6.84; 95% CI, 4.02-9.58). This systematic review and meta-analysis found that cochlear implantation for children with SSD was associated with clinically meaningful improvements in audiological and patient-reported outcomes; shorter duration of deafness may lead to better outcomes. These findings can guide future research efforts, refine cochlear implantation candidacy criteria, and aid in family counseling and shared decision-making.

Computer tomography demonstrations of single-sided deafness

Objective:To investigate the distribution of common inner ear and internal auditory canal malformations in children with single-sided deafness（SSD） ,and to explore the imaging etiology of SSD by comparing the quantitative parameters of key bone structures between deaf and normal ears in children with congenital SSD. Method:Forty children with SSD diagnosed in the Second Hospital of Lanzhou University from September 2016 to March 2019 were collected. All of them underwent HRCT examinations of temporal bone . The area of bone island, the width of vestibular, the width of internal auditory canal, the height of cochlear and the width of cochlear basal axis were measured. Paired t test was used to compare the difference between the hearing abnormality and normal hearing in children with SSD. Result:The rate of inner ear deformity was 62.5% in SSD group,the most common deformity was cochlear nerve canal deformity, 20 cases （50.0%） of cochlear canal stenosis and 3 cases （7.5%） of cochlear canal atresia.The second most common deformity was internal auditory canal deformity, including 5 cases （12.5%） of internal auditory canal stenosis and 1 case （2.5%） of internal auditory canal atresia. Other malformations included 1 case（2.5%） of RO, 2 cases （5.0%） of incomplete partition （IP） type II and 1 case （2.5%） of enlargement of vestibular aqueduct （EVA）. There are no significant difference in the measured results of the key structures of the inner ear between two groups except the width of cochlear nerve canal, internal auditory canal and the area of bone island. Conclusion:The main inner ear deformities in children with SSD are cochlear nerve canal stenosis and inner auditory canal stenosis. HRCT of temporal bone has high diagnostic value for inner ear deformities in children with SSD.

Long-term results of cochlear implantation in children with congenital single-sided deafness

PurposeThe purpose of this retrospective study was to investigate the outcome and critical age of cochlear implantation in congenital single-sided deafness (SSD).Methods11 children with congenital SSD were implanted with a cochlear implant (CI). Auditory performance was measured through the results of speech discrimination, subjective assessment by the Categories of auditory performance (CAP) score, the Speech, Spatial and Qualities scale questionnaire (SSQ) and the German version of the IOI-HA [Internationales Inventar zur Evaluation von Hörgeräten (IIEH, version for CI)].ResultsLong-term follow-up [median: 3 years and 5 months (3;5 years)] revealed that nine children use their CI (> 8 h/day) and two became nonusers. In children aged below 3;2 years at surgery, there was a substantial long-term increase in speech discrimination and subjective benefit. Children over 4;4 years of age at CI surgery improved partially in audiological/subjective measurements. Among children above 5 years, the SSQ score did not improve despite further slight improvement in speech discrimination long-term.ConclusionOur data suggest a critical age for CI surgery below 3 years in children with congenital SSD for successful hearing rehabilitation. It is mandatory to identify children with SSD as early as bilaterally deaf children.

Auditory experience, for a certain duration, is a prerequisite for tinnitus: lessons from subjects with unilateral tinnitus in the better-hearing ear.

Tinnitus has traditionally been considered an otologic disorder; however, recent advances in auditory neuroscience have shifted investigations toward the brain. The Bayesian brain model explains tinnitus as an auditory phantom percept. According to the model, the brain works to reduce environmental uncertainty, and thus the absence of auditory information due to hearing loss may cause auditory phantom percepts, i.e., tinnitus. As in animal studies, our recent human observational study revealed the absence of ipsilesional tinnitus in subjects with congenital single-sided deafness, suggesting that auditory experience is a prerequisite for the generation of tinnitus. Prompted by anecdotal cases, we hypothesized that subjects with acquired hearing loss would not develop tinnitus if their duration of auditory experience was not sufficiently long. We retrospectively enrolled 22 subjects with acquired asymmetric hearing loss and unilateral tinnitus in better ear (TBE). Twenty-two hearing threshold-matched controls with tinnitus in worse ear (TWE) were selected from our database of tinnitus patients. All 22 TBE subjects reported that their acquired hearing loss developed before the age of 20, and the reported duration of auditory deprivation in the ear without tinnitus in the TBE group was significantly longer than that of the TWE group. In other words, the TBE group with limited auditory experience in the worse ear did not develop tinnitus in the worse ear while subjects with enough auditory experiences in the worse ear developed ipsilesional tinnitus in the TWE group. These preliminary results support our hypothesis that both auditory experience itself, and an individually variable critical duration of auditory deprivation, are prerequisites for the generation of tinnitus.

Inner-ear malformations as a cause of single-sided deafness.

To determine the prevalence and distribution of inner-ear malformations in congenital single-sided deafness cases, as details of malformation type are crucial for disease prognosis and management. A retrospective study was conducted of 90 patients aged under 16 years with congenital single-sided deafness. Radiological findings were evaluated using computed tomography and magnetic resonance imaging. Inner-ear malformations were identified and cochlear nerve status was determined in affected ears. Out of 90 ears, 42 (46.7 per cent) were found to have inner-ear malformation. Isolated cochlear aperture stenosis was the most common anomaly (n = 18, 20 per cent), followed by isolated cochlear aperture atresia (n = 11, 12.2 per cent) and cochlear hypoplasia (n = 7, 7.8 per cent). Cochlear nerve deficiency was encountered in 41 ears (45.6 per cent). The internal auditory canal was also stenotic in 49 ears (54.4 per cent). Inner-ear malformations, especially cochlear aperture anomalies, are involved in the aetiology of single-sided deafness more than expected. The cause of single-sided deafness differs greatly between congenital and adult-onset cases. All children with single-sided deafness should undergo radiological evaluation, as the prognosis and management, as well as the aetiology, may be significantly influenced by inner-ear malformation type.

Therapeutic challenges and clinical characteristics of single-sided deafness in children

IntroductionThe clinical implications of single-sided deafness (SSD) in children has historically been underappreciated by patients and providers alike, despite a large body of literature on the wide-ranging neurocognitive, language, scholastic, and functional impairments that occur. Conventional amplification options are marked by variable results and frequent loss of follow-up. MethodsRetrospective case series for pediatric SSD from 2008 to 2018. Results88 children with congenital SSD were identified. Seventeen (N = 17/88, 23.9%) passed their newborn hearing screen. Median age at first otolaryngology evaluation was 0.65 years (range 0.1–16.9 years). Most common etiologies included cochlear nerve deficiency (N = 39, CND, 44.3%), unknown (N = 30, 35.2%), inner ear malformation (N = 7, 8.0%), and congenital cytomegalovirus (N = 6, 6.8%). 32.5% of patients elected for continued observation only, followed by bone conduction hearing aid (27.7%), contralateral routing of sound aid (20.5%), conventional hearing aid (13.3%), or cochlear implant (6%). Lack of follow-up at ≥1 year was common (39.8%). Of those with device use data (N = 39), 84.7% reported either discontinued or <6 h of daily use. ConclusionsDespite early diagnosis and evaluation, the pediatric SSD cohort is characterized by high rates of loss of follow-up and amplification discontinuation. Cochlear nerve deficiency is commonly seen in congenital SSD. Early specialist referral is critical for habilitation evaluation. Patients and caregivers should be educated on the significant implications of unilateral hearing loss.

Speech Perception and Gap Detection Performance of Single-Sided Deafness under Noisy Conditions

Background and ObjectivesMany studies have reported no benefit of sound localization, but improved speech understanding in noise after treating patients with single-sided deafness (SSD). Furthermore, their performances provided a large individual difference. The present study aimed to measure the ability of speech perception and gap detection in noise for the SSD patients to better understand their hearing nature.Subjects and MethodsNine SSD patients with different onset and period of hearing deprivation and 20 young adults with normal hearing and simulated conductive hearing loss as the control groups conducted speech perception in noise (SPIN) and Gap-In-Noise (GIN) tests. The SPIN test asked how many presented sentences were understood at the +5 and -5 dB signal-to-noise ratio. The GIN test was asked to find the shortest gap in white noise with different lengths in the gap.ResultsCompared to the groups with normal hearing and simulated instant hearing loss, the SSD group showed much poor performance in both SPIN and GIN tests while supporting central auditory plasticity of the SSD patients. Rather than a longer period of deafness, the large individual variance indicated that the congenital SSD patients showed better performance than the acquired SSD patients in two measurements.ConclusionsThe results suggested that comprehensive assessments should be implemented before any treatment of the SSD patient considering their onset time and etiology, although these findings need to be generalized with a large sample size.

Appropriate Imaging Modality for the Etiologic Diagnosis of Congenital Single-Sided Deafness in Children.

We aimed to compare the diagnostic yield between temporal bone computed tomography (TBCT) and internal auditory canal MRI (IAC MRI) for the etiologic diagnosis of children with congenital single-sided deafness (SSD) and the evaluation of cochlear implant (CI) candidacy. In the original cohort, 24 subjects with congenital SSD were enrolled and underwent both TBCT and IAC MRI. We recruited an additional 22 consecutive infants with congenital SSD (the supplementary cohort) and evaluated in particular the cochlear nerve (CN) integrity using IAC MRI. Cochlear nerve deficiency (CND) was classified as ‘absent’, ‘small’, and ‘indeterminate’ via mutual comparison between optical and parameters based on the MRI results. The most common etiologies were CND in the original cohort (19 out of 24). Notably, accurate evaluations of CN status (‘small CN’ = 2, ‘indeterminate CN’ = 2), inner ear malformations, and brain abnormalities were possible only with MRI. The ‘indeterminate CN’ tended to be more frequently detected in SSD ears than in unaffected ears. MRI appeared to be more accurate than TBCT in a meticulous differentiation of CN, which is crucial for the selection of appropriate CI candidacy among congenital SSD children. Additionally, we introduced the novel concept of ‘indeterminate CN’, of which the causal relationship with SSD awaits confirmation.

No auditory experience, no tinnitus: Lessons from subjects with congenital- and acquired single-sided deafness

Recent studies have adopted the Bayesian brain model to explain the generation of tinnitus in subjects with auditory deafferentation. That is, as the human brain works in a Bayesian manner to reduce environmental uncertainty, missing auditory information due to hearing loss may cause auditory phantom percepts, i.e., tinnitus. This type of deafferentation-induced auditory phantom percept should be preceded by auditory experience because the fill-in phenomenon, namely tinnitus, is based upon auditory prediction and the resultant prediction error. For example, a recent animal study observed the absence of tinnitus in cats with congenital single-sided deafness (SSD; Eggermont and Kral, Hear Res 2016). However, no human studies have investigated the presence and characteristics of tinnitus in subjects with congenital SSD. Thus, the present study sought to reveal differences in the generation of tinnitus between subjects with congenital SSD and those with acquired SSD to evaluate the replicability of previous animal studies. This study enrolled 20 subjects with congenital SSD and 44 subjects with acquired SSD and examined the presence and characteristics of tinnitus in the groups. None of the 20 subjects with congenital SSD perceived tinnitus on the affected side, whereas 30 of 44 subjects with acquired SSD experienced tinnitus on the affected side. Additionally, there were significant positive correlations between tinnitus characteristics and the audiometric characteristics of the SSD. In accordance with the findings of the recent animal study, tinnitus was absent in subjects with congenital SSD, but relatively frequent in subjects with acquired SSD, which suggests that the development of tinnitus should be preceded by auditory experience. In other words, subjects with profound congenital peripheral deafferentation do not develop auditory phantom percepts because no auditory predictions are available from the Bayesian brain.

Cochlear Implantation in Children With Congenital Single-Sided Deafness.

To determine audiological and clinical results of cochlear implantation in children with congenital single sided deafness (SSD), with an emphasis on children implanted before and after 6 years of age. Retrospective study. Tertiary referral center. Twenty one children with congenital SSD who were implanted aged 10 months to 11;3 years. Unilateral cochlear implantation. Speech recognition in noise via the German Oldenburg Sentence Test for Children (OlKiSa), lateralization ability, and subjective evaluation of hearing results using self- and third-party assessment questionnaires. Significant improvements of all three aspects of true binaural hearing were found. The most striking improvement was the combined head shadow effect by 2.11 dB (squelch effect: 0.95 dB, summation effect 0.98 dB). An improvement of lateralization ability was also demonstrated. Parents had a high overall level of satisfaction with their children's cochlear implantation. Subjective benefit was verified in all three subscales of the Speech, Spatial, and Qualities of Hearing Questionnaire. No significant difference was found between subjects implanted before the age of 6 with those implanted later. Three of the five subjects with a follow-up of greater than 3 years were limited users or nonusers. Cochlear implant (CI) provision provides children with congenital SSD with significant audiological and subjective benefits which can be seen even in children implanted after the age of 3;6. The problem of limited use and nonuser, however, should not be ignored and has to be considered for further studies.

Cochlear Implantation in Children with Single-Sided Deafness: Does Aetiology and Duration of Deafness Matter?

For adult patients with single-sided deafness (SSD), treatment with a cochlear implant (CI) is well established as an acceptable and beneficial hearing rehabilitation method administered routinely in clinical practice. In contrast, for children with SSD, CI has been applied less often to date, with the rationale to decide either on a case-by-case basis or under the realm of clinical research. The aim of our clinical study was to evaluate the longitudinal benefits of CI for a group of children diagnosed with SSD and to compare their outcomes with respect to patient characteristics. Evaluating a pool of paediatric SSD patients presenting for possible CI surgery revealed that the primary aetiology of deafness was congenital cochlear nerve deficiency. A subgroup of children meeting the CI candidacy criteria for the affected ear (the majority with acquired hearing loss) were enrolled in the study. Preliminary group results suggest substantial improvements in speech comprehension in noise and in the ability to localise sound, which was demonstrated through objective and subjective assessments after CI treatment for the group, with results varying from patient to patient. Our study shows a trend towards superior outcomes for children with acquired hearing loss and a shorter duration of hearing loss compared to congenitally deafened children who had a longer duration of SSD. This indicates an interactive influence of the age at onset, aetiology and duration of deafness upon the restoration of binaural integration and the overall benefits of sound stimulation to two ears after CI treatment. Continued longitudinal investigation of these children and further studies in larger groups may provide more guidance on the optimal timing of treatment for paediatric patients with acquired and congenital SSD.