Unilateral Sensorineural Deafness Research Articles

Unilateral Sensorineural Deafness Contemporary Aspects of Etiopathogenesis, Diagnosis and Treatment

Unilateral Sensorineural Deafness Contemporary Aspects of Etiopathogenesis, Diagnosis and Treatment

Evaluation of Patients with Delayed Endolymphatic Hydrops by Videonystagmography, Vestibular-Evoked Myogenic Potentials, and Electrocochleography

Introduction: The categorization of delayed endolymphatic hydrops (DEH) based on the ear which produces vertigo may sometimes cause misdiagnosis. Objectives: The aim of this study was investigating the vestibular-evoked myogenic potentials (VEMPs), electrocochleography (ECoG), and videonystagmography (VNG) in cases with DEH to determine the ear that originates symptoms. Methods: In this cross-sectional study, 34 patients – 20 males and 14 females – with profound unilateral sensorineural deafness and vertigo attacks were recruited and evaluated by the ECoG, VNG, and VEMPs tests. Results: The average age was around 43; the summating potential/action potential was abnormal in 29.4% of patients in their normal auditive ear. In 32.4, 17.6, and 50% of cases with a deaf ear, absent, normal, and abnormal VEMPs results were sequentially observed, respectively. In normal-hearing ears, absent, normal, and abnormal VEMPs were observed in 23.5, 50, and 26.5%, respectively. In the normal-hearing ear, the distribution of abnormal VEMPs was 26.5%, and in the deaf ear, this parameter was abnormal in 50% of the opposite ear (p value = 0.00021). In the VNG test, among patients with a normal-hearing ear, results in 27 and 7 patients were sequentially normal and hypofunction. Conclusion and Significance: The probability of a hypofunction VNG test in a normal-hearing ear might be greater when the VEMPs results of the contralateral deaf ear are normal. In patients with a normal-hearing ear, the distribution of abnormal VEMPs in the contralateral deaf ear is greater, although the intact side may also manifest abnormality in VEMPs tests. The initial evaluation should begin in a deaf ear as well as for the normal-hearing ear ere utilizing ablation surgery.

Feasibility of constructing a unilateral sudden sensorineural hearing loss machine learning classification model based on diffusion tensor imaging

Objective To explore the feasibility of constructing a machine learning classification model for unilateral sudden sensorineural hearing loss (SSHL) patients and normal controls based on diffusion tensor imaging. Methods Prospective collection of 84 patients with untreated SSHL were recruited from the otolaryngology department of the Union Hospital of Tongji Medical College of Huazhong University of Science and Technology between June 2013 to May 2015 as the SSHL group. Meanwhile, a total of 63 healthy volunteers who were no any ear disease history, and the hearing function were confirmed with pure tone audiometry, were collected as the control group. All subjects underwent a brain DTI scan. The data were divided into the training set and validation set according to the ratio of 7 to 3, that was, the training set contained 58 cases of SSHL patients and 44 control groups, and the validation set included 26 cases of SSHL patients and 19 control groups. A vector which included the DTI parameters such as fractional anisotropy, mean diffusivity, axial diffusivity and radial diffusivity was constructed with the software R. The LASSO regression of machine learning method was used to perform feature dimensionality reduction and construct a classification model. The training set samples were used to map the nomogram based on the multivariate logistic analysis method, the validation set and the AUC were used to evaluate the prediction ability of the nomogram, and the calibration curve was used to evaluate the model. Results From the 200 feature vectors including the fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) values of each brain region, after each dimension reduction process, a total of six features were retained, which were the MD of left superior corona radiate and right superior fronto-occipital fasciculus, the AD of the body of corpus callosum, and the RD of left inferior cerebellar peduncle, left superior corona radiate and right posterior limb of internal capsule. The six features of patients with unilateral SSHL were higher than the control group, and the difference was statistically significant (P<0.05). Based on this, a two-class model is constructed and a nomogram is drawn. The sensitivity, specificity, accuracy and AUC of the training set were 93.1% (54/58), 72.7% (32/44), 84.3% (86/102) and 0.854, respectively; the sensitivity, specificity, accuracy and AUC of validation set were 80.8% (21/26), 84.2% (16/19), 82.2% (37/45), 0.870, respectively. Nomogram could significantly improve the classification efficiency of the control group and patients, and the model with the LASSO method showed a higher prediction curve than other models. Conclusions The machine learning classification model based on DTI metrics can effectively distinguish patients with unilateral sudden sensorineural deafness from healthy control people. Key words: Hearing loss, sensorineural; Diffusion tensor imaging; Machine learning; Classification; Nomogram; Decision curve analysis

Head Shadow and Binaural Squelch for Unilaterally Deaf Cochlear Implantees.

Cochlear implants (CIs) can improve speech-in-noise performance for listeners with unilateral sensorineural deafness. But these benefits are modest and in most cases are limited to head-shadow advantages, with little evidence of binaural squelch. The goal of the investigation was to determine whether CI listeners with normal hearing or moderate hearing loss in the contralateral ear would receive a larger head-shadow benefit for target speech and noise originating from opposite sides of the head, and whether listeners would experience binaural squelch in the free field in a test involving interfering talkers. Eleven CI listeners performed a speech-identification task in the presence of interfering noise or speech. Six listeners had single-sided deafness (normal or near-normal audiometric thresholds in the acoustic ear) and five had asymmetric hearing loss (hearing loss in the acoustic ear treated with a hearing aid). Listeners were tested with the acoustic ear only and bilaterally with the CI turned on. One set of conditions examined head-shadow effects with target speech and masking noise presented from azimuths of 0 or ±108 degrees. A second set of conditions examined binaural squelch, with target speech presented from the front and interfering talkers symmetrically placed on both sides. On average, the largest head-shadow benefit (5 dB) occurred when the target and masking noise were presented on opposite sides of the head. Listeners also showed an average of 2 dB of squelch, but only when the target speech was masked by interfering talkers of the same sex as the target. CIs provide listeners with unilateral deafness important benefits for speech perception in complex spatial environments, including a larger head-shadow benefit when speech and noise originate on opposite sides of the head, and an improved ability to perceptually organize an auditory scene with multiple competing voices.The views expressed in this abstract are those of the authors and do not reflect the official policy of the Department of Army/Navy/Air Force, Department of Defense, or US Government.

Mechanism of neurodevelopment of children with hearing deprivation

100 children with sensorineural hearing loss, who had confirmed hearing impairment, were examined by routine somatoneurological and surdological survey, as well as standard psychomotor development scales (Alberta Infant Motor Scale (AIMS), Denver Developmental Screening Test (DDST), Griffiths Mental Development Scales (GMDS, GMDS-ER) in the city rehabilitation center for children with hearing and speech pathology. Criterion for inclusion in the study were the following: post-conceptual age of the child had to be not more than 36 months at the time of observation; bilateral or unilateral chronic sensorineural hearing loss or deafness and/or the presence of auditory neuropathy, confirmed by objective methods with modern audiological examinations for the core group; the absence of hearing loss, set by the modern objective methods of audiological examination for the comparison group; assessment of psychomotor and speech development of a child with at least two scales from the selection below. The survey results were analyzed using nonparametric statistical methods. Next Bayesian networks were used as efficient, compact, and intuitive way to represent knowledge related to uncertainty. Patterns of mental development, motor skills, speech and social functions in children with hearing impairment were obtained. The technique, which, depending on the factors affecting the trait under study, can help to predict with certainty the likelihood of various normal and deviant development of the child.

Bone-anchored hearing aids and unilateral sensorineural hearing loss: why do patients reject them?

This study aimed to report the bone-anchored hearing aid uptake and the reasons for their rejection by unilateral sensorineural deafness patients. A retrospective review of 90 consecutive unilateral sensorineural deafness patients referred to the Greater Manchester Bone-Anchored Hearing Aid Programme between September 2008 and August 2011 was performed. In all, 79 (87.8 per cent) were deemed audiologically suitable: 24 (30.3 per cent) eventually had a bone-anchored hearing aid implanted and 55 (69.6 per cent) patients declined. Of those who declined, 26 (47.3 per cent) cited perceived limited benefits, 18 (32.7 per cent) cited reservations regarding surgery, 13 (23.6 per cent) preferred a wireless contralateral routing of sound device and 12 (21.8 per cent) cited cosmetic reasons. In all, 32 (40.5 per cent) suitable patients eventually chose the wireless contralateral routing of sound device. The uptake rate was 30 per cent for audiologically suitable patients. Almost half of suitable patients did not perceive a sufficient benefit to proceed to device implantation and a significant proportion rejected it. It is therefore important that clinicians do not to rush to implant all unilateral sensorineural hearing loss patients with a bone-anchored hearing aid.

Jervell and Lange-Nielsen syndrome in a father and daughter from a large highly inbred family: a 16-year follow-up of 59 living members

To report the autosomal dominant inheritance of the Jervell and Lange-Nielsen syndrome in a highly inbred family, the initiation of Torsades de Pointes, and the natural history of the syndrome based on a 16-year follow-up of the kindred. A family tree was constructed that included 66 blood relatives from three successive generations. Electrocardiograms were obtained from 59 living members including the proband, four members from a nuclear family, and 54 from the extended family. Evoked response audiometry was recorded for the proband and the nuclear family. All 59 family members were followed up regularly for 16 years. A total of 24 living members were affected--QTc: 480-680 ms. The proband had long QTc, bilateral high-tone sensorineural deafness, recurrent syncope, and Torsades de Pointes. The asymptomatic father had long QTc and unilateral high-tone sensorineural deafness that involved specifically the left ear. One asymptomatic sibling of the proband had long QTc and normal hearing. The mother and another sibling were asymptomatic; QTc and hearing were normal in both. A total of 21 affected members from the extended family had only long QTc, and all were asymptomatic. There were three congenitally deaf first cousins who had recurrent syncope and adrenergic-triggered sudden death. In all, seven of 10 parents had consanguineous marriage to a first cousin. Each affected offspring had at least one affected parent. The severely symptomatic proband who received only β-blocker therapy and the 23 affected members without antiadrenergic therapy, all remained asymptomatic throughout the 16-year follow-up period. Jervell and Lange-Nielsen syndrome was inherited as autosomal dominant in this kindred. The majority of the affected members had a mild phenotype. The severity of auditory and cardiac phenotypes corresponded.

Genotype and Phenotype Heterogeneity in Perrault Syndrome

BackgroundThe hallmarks of Perrault syndrome are progressive sensorineural hearing loss and ovarian dysgenesis, but the disorder is both clinically and genetically heterogenous. CaseWe report a 15-year-old girl with gonadal dysgenesis, unilateral sensorineural deafness, cataracts in both eyes, and Marfanoid body proportions diagnosed Perrault syndrome. We detected 14 single nucleotide variations including 2 homozygous missense change of c.317G>A (p.Arg106His) and c.1675A>G (p.Ile559Val) in HSD17B4. No significant mutation in HARS2 and PSMC3IP, and gene copy number variant were found as the cause of Perrault syndrome. Summary and ConclusionMutations in HARS2, HSD17B4, and PSMC3IP genes do not explain Perrault syndrome in our patient, indicating that other critical genes remain to be identified.

A rare association of not so rare symptoms

A 13 year old boy presented to us with history of vomiting, dizziness, red eyes and lethargy. Within the next few days he was found to have unilateral sensori neural deafness. He was also found to have anterior uveitis which as the disease progressed turned out to be interstitial keratitis. He was extensively investigated for infectious, inflammatory, rheumatological and autoimmune diseases, but all came back as negative. He was started on steroids after discussion with a tertiary rheumatology centre, to which he responded well. He was steroid dependant, so was commenced on methotrexate. His deafness completely resolved and his vision was better whilst on methotrexate. He was diagnosed to have Cogan syndrome based on the association of audio vestibular symptoms and interstitial keratitis and after excluding other conditions. Cogan syndrome is a rare disorder characterised by association of audio vestibular and ocular symptoms particularly interstitial keratitis. There is no diagnostic test as a result of which the diagnosis may be delayed. The disease may also be associated with non-specific systemic symptoms. The ocular symptoms typically may present as interstitial keratitis but in some cases it may present as uveitis or iritis and eventually progress to interstitial keratitis. Steroids and methotrexate are used in the treatment of the disease. If the condition is not diagnosed early and treated promptly it can cause permanent damage such as hearing loss and vision loss. Key message: Cogan syndrome is characterised by audio vestibular and ocular symptoms. One has to think of Cogan syndrome as a possibility when there is such an association. As there is no diagnostic test, there may be delay in diagnosing the disease. But early detection and treatment may prevent the permanent damage such as hearing loss or loss of vision.

Idiopathic Bilateral Auricular Petrification

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

Unilateral Sensorineural Deafness: BAHA vs TransEar Device

Objective: Compare the audiological and quality of life assessment in 2 groups of patients: users of BAHA and users of TransEar devices. Method: Retrospective study of 20 patients with unilateral sensorineural hearing loss. Group number 1, 10 patients using TransEar. Group number 2, 10 patients using BAHA. Prefitting Pure Tone Average (PTA) 0.25 to 4kHz compared with postfitting PTA 0.25- 3kHz between groups. Survey results of the Hearing Device Satisfaction Scale tool. Results: Functional hearing gain was observed in both groups. The BAHA system showed higher hearing benefit than the TransEar system. When mean thresholds of each system were compared, mean functional response was 41dBSPL with the TransEar and 25dBSPL with the BAHA. The satisfaction level of sound category was slightly better for the BAHA device. TransEar is preferred over BAHA in fitting, cosmesis, and management issues. BAHA showed better scores for quality of life aspects. Conclusion: In this group of patients, higher hearing benefit of osseointegrated implant regarding the effectiveness over the intracanal system was observed. When satisfaction level is analyzed related to fitting, management, and quality of life, an intracanal device is preferred.

Combined Acoustic and Electric Stimulation

Combined Acoustic and Electric Stimulation

SARCOIDOSIS PRESENTING WITH MASSIVE SPLENOMEGALY IN A CHILD WITH A HISTORY OF IRIDOCYCLITIS AND SENSORINEURAL DEAFNESS

Childhood sarcoidosis is a rare multisystemic disorder that can have variable clinical presentations. A triad of skin, eye, and joint involvement is common in children younger than 5 years; however, pulmonary disease is more common in older children, similar to adults. The authors report the case of a 10-year-old girl who presented solely with massive splenomegaly. Her history was significant for iridocyclitis and unilateral sensorineural deafness at 6 and 7 years of age, respectively. A gallium scan showed diffuse splenic uptake, and the pathology of the spleen was consistent with a noncaseating granuloma, with no evidence of malignancy. A work-up for infectious etiology was unremarkable. This case demonstrates that the challenge in diagnosing sarcoidosis in young children stems from its ability to present in several unique clinical scenarios. It also reinforces the importance of tissue evaluation and the exclusion of other differential diagnoses, such as lymphoma, to confirm the diagnosis of sarcoidosis.

Unilateral and Bilateral Congenital Sensorineural Deafness in Client‐Owned Pure‐Breed White Cats

Congenital sensorineural deafness has been reported frequently in experimental mixed-breed white cats but there is a paucity of data on occurrence of deafness in client-owned pure-breed white cats. To describe hearing status in client-owned pure-breed white cats. Eighty-four pure-breed client-owned cats with white coat color of 10 registered breeds presented for routine hearing evaluation before breeding (1995-2008). Hearing was assessed by click-evoked brainstem auditory evoked response. Overall deafness prevalence was 20.2%; 9 cats (10.7%) were bilaterally deaf and 8 cats (9.5%) were unilaterally deaf. There was no association between sex and deafness status (P= .85). Deafness status was associated with iris color (P= .04). Congenital sensorineural deafness frequently occurs in pure-breed cats with white coat color. Unilateral sensorineural deafness was as common as bilateral deafness.

Binaural Hearing after Cochlear Implantation in Subjects with Unilateral Sensorineural Deafness and Tinnitus

The aim of this clinical study was to assess speech recognition in noise after cochlear implantation in subjects with single-sided deafness and incapacitating tinnitus. 20 subjects complaining of severe intractable tinnitus unresponsive to treatment received a MED-EL cochlear implant (CI). 11 subjects had normal hearing (NH group) on the contralateral side, while 9 used a hearing aid (HA group). The subjects were tested in noise in two listening conditions, i.e. with their acoustic hearing only and with adding the CI to the acoustic hearing (binaural). Subjective improvement in daily life was evaluated using the Speech Spatial and Qualities Hearing Scale (SSQ). The summation effect (3.3 dB for the HA group and 0.6 dB for the NH group) is not significant in both groups. A significant squelch effect of adding the CI was seen for the HA users (3.8 dB), but not for the NH group (1.2 dB). Additionally, a significant effect of adding the CI was found for the spatial configuration where noise is presented in front and speech on the CI side for both the HA group (6.5 dB) and the NH group (1.7 dB). Results of the SSQ show a significant overall benefit of wearing the CI for both groups. The preliminary results of these 20 subjects suggest that cochlear implantation can improve hearing in people suffering from single-sided deafness combined with tinnitus.

Plasma antidiuretic hormone in cases with the early onset of profound unilateral deafness

Objective The p-ADH level in cases of juvenile unilateral profound deafness (JUPD) and the timecourse of the level were examined to investigate whether or not an increase of p-ADH is involved in the development of delayed endolymphatic hydrops (DEH) in JUPD. Materials and methods In 90 consecutive patients with unilateral profound or total sensorineural deafness with the onset in early childhood, pure-tone audiometric examination and the measurement of p-ADH and plasma osmolality (p-OSM) were followed up once or twice a year as far as possible. At every testing, we performed careful history-taking about episodic vertigo/dizziness, fluctuant hearing loss, and tinnitus in order to find out whether patients had experienced these clinical signs of the development of DEH. Results Means and standard deviation (S.D.) of p-ADH level and osmolality in all samples tested ( n = 368) were 7.3 ± 7.0 pg/mL (0.7–52.0 pg/mL), and 288.6 ± 4.4 mOsm/L (273–306 mOsm/L), respectively. The mean of p-ADH level was much higher than those previously reported in children and adolescents. High levels of p-ADH (over 5.0 pg/mL) were often observed in subjects between 6 and 19 years of age, but not so frequently in subjects of 20 years of age or older. Long-term follow-up of p-ADH levels revealed that DEH frequently developed in cases with persistent elevation of p-ADH. Conclusions The elevation of p-ADH is likely to promote the development of DEH in cases of JUPD, although the underlying mechanism remains to be elucidated.

Coexistence of unilateral iridocorneal endothelial syndrome and sensorineural deafness

Coexistence of unilateral iridocorneal endothelial syndrome and sensorineural deafness

Barttin mutations in antenatal Bartter syndrome with sensorineural deafness

Case 1 The male patient had been born at 30 weeks of gestation (weight 1,360 g) to consanguineous parents. Pregnancy was complicated by severe polyhydramnios. Findings at his physical examination were normal except for a triangular face, large eyes, protruding ears, tachypnea and retractions. He rapidly developed renal salt wasting, hyper-reninemic hyperaldosteronism, hypokalemic metabolic alkalosis, and impaired renal function [creatinine (Crea) 1.5 mg/dl]. The diagnosis of antenatal Bartter syndrome (aBS) was suspected. On day 49 oral administration of indomethacin was started (2–3 mg/kg per day), which could not help to regulate serum potassium levels. Instead, upon indomethacin treatment, renal function further deteriorated (Crea 2.5 mg/dl). Therefore, indomethacin was stopped, and he was treated with spironolactone. Potassium need declined to 27 mEq/kg, and he was discharged after 140 days. He has unilateral sensorineural deafness detected by impaired brain stem evoked potentials and growth retardation. A homozygous mutation in the Barttin gene (BSND) leading to a loss of start codon was detected (Table 1). A similar mutation has been previously described [1].

Oto‐spondylo‐megaepiphyseal dysplasia (OSMED): Clinical and radiological findings in sibs homozygous for premature stop codon mutation in the COL11A2 gene

Oto-spondylo-megaepiphyseal dysplasia (OSMED) is a very rare disorder due to mutation of type XI collagen. Less than 30 patients have been reported in the literature so far. It could be either of autosomal dominant (OMIM 154780) or recessive (OMIM 215150) etiology. Two sibs with OSMED are presented. They had disproportionate short stature and short limbs, distinct face with midface hypoplasia, short nose, depressed nasal bridge, long philtrum, and non-progressive sensorineural deafness. Radiological findings showed short long bones and large epiphyses with metaphyseal flaring and mild platyspondyly and coronal clefting. Homozygosity of a single nucleotide deletion in exon 55 causing a premature stop codon in exon 56 of COL11A2 was detected in the affected sibs. Parents were heterozygotes for the same mutation and interestingly, the father had mild unilateral non-progressive sensorineural deafness. This finding adds more weight that the type of mutation and location in COL11A2 are crucial in determining the phenotype. The purpose of this study is to report clinical and radiological findings in two molecularly proven Egyptian sibs with autosomal recessive OSMED.

Nonsyndromic Isolated Unilateral Cochlear Nerve Aplasia without Narrow Internal Auditory Meatus: A Previously Overlooked Cause of Unilateral Profound Deafness in Childhood

Juvenile or adolescent unilateral profound sensorineural deafness (worldwide prevalence, 0.1% to 0.2%) has been attributed to postnatal viral infection, sudden deafness, prenatal and perinatal problems including maternal rubella and viral infections, congenital innerear anomalies, and other factors. Herein, 2 cases are reported and another potentially important cause of unilateral profound hearing loss is proposed. Two nonsyndromic cases of a presently "very rare" cause of pediatric unilateral deafness are presented as a retrospective case study. The 2 patients showed isolated aplasia of the cochlear nerve; other branches of the eighth cranial nerve, the seventh nerve, and the inner ear were spared,and there was no anomaly of the internal auditory meatus. Both functional and imaging studies confirmed the isolated lesion (absence) of the cochlear nerve. Because of the absence of bony abnormalities, such cases may have been overlooked. The authors would like to advocate this isolated anomaly of thecochlear nerve as an important cause of juvenile or adolescent unilateral profound deafness.