Profound Unilateral Sensorineural Hearing Loss Research Articles

Early Audiometric Intervention in Bacterial Meningitis: Cochlear Implantation in a 10-Week-Old Child.

Sensorineural hearing loss is a well-known complication of Streptococcus pneumoniae meningitis. Given the propensity for fibrosis and ossification of the cochlea in bacterial meningitis, implantation must be performed in a timely fashion because a delayed attempt at implantation can frustrate obtaining an optimal technical result or lead to an inability to implant. Obtaining optimal audiometric outcomes is reliant on early hearing screening in patients with streptococcal meningitis. In the absence of standardized protocols, audiometric testing is often overlooked or delayed in the workup and management of meningitis. Our institution implemented a meningitis protocol with a particular focus on timing of audiometric testing in patients with meningitis. We present a patient diagnosed with streptococcal meningitis in the first week of life. Early hearing screening allowed the diagnosis of profound unilateral sensorineural hearing loss and subsequent cochlear implantation at 10 weeks of age, the youngest described in the medical literature. Despite early implantation, there was cochlear fibrosis at the time of implantation. Fortunately, the majority of electrodes were implanted to achieve a serviceable hearing outcome. Serial magnetic resonance imaging scans were obtained because of her contralateral ventriculoperitoneal shunt that allowed unique visualization of the progression of cochlear fibrosis over time. This case demonstrates the importance of including audiometric testing in a standardized meningitis protocol to diagnose hearing loss in a timely and accurate way and to achieve optimal long-term hearing outcomes.

A Randomized Crossover Study in Single-Sided Deafness Comparing a Cartilage Conduction CROS System and an Air-Conduction CROS System.

To investigate if cartilage conduction (CC) rerouting devices are noninferior to air-conduction (AC) rerouting devices for single-sided deafness (SSD) patients by measuring objective and subjective performance using speech-in-noise tests that resemble a realistic hearing environment, sound localization tests, and standardized questionnaires. Prospective, single-subject randomized, crossover study. Anechoic room inside a university. Nine adults between 21 and 58 years of age with severe or profound unilateral sensorineural hearing loss. Patients' baseline hearing was assessed; they then used both the cartilage conduction contralateral routing of signals device (CC-CROS) and an air-conduction CROS hearing aid (AC-CROS). Patients wore each device for 2 weeks in a randomly assigned order. Three main outcome measures were 1) speech-in-noise tests, measuring speech reception thresholds; 2) proportion of correct sound localization responses; and 3) scores on the questionnaires, "Abbreviated Profile of Hearing Aid Benefit" (APHAB) and "Speech, Spatial, and Qualities of Hearing Scale" with 12 questions (SSQ-12). Speech reception threshold improved significantly when noise was ambient, and speech was presented from the front or the poor-ear side with both CC-CROS and AC-CROS. When speech was delivered from the better-ear side, AC-CROS significantly improved performance, whereas CC-CROS had no significant effect. Both devices mainly worsened sound localization, whereas the APHAB and SSQ-12 scores showed benefits. CC-CROS has noninferior hearing-in-noise performance except when the speech was presented to the better ear under ambient noise. Subjective measures showed that the patients realized the effectiveness of both devices.

Prospective study of sound localisation in patients with single sided deafness and asymmetric hearing loss treated with cochlear implant

Background and objectiveSound localization plays a crucial role in our daily lives, enabling us to recognize voices, respond to alarming situations, avoid dangers, and navigate towards specific signals. However, this ability is compromised in patients with Single-Sided Deafness (SSD) and Asymmetric Hearing Loss (AHL), negatively impacting their daily functioning. The main objective of the study was to quantify the degree of sound source localization in patients with single-sided deafness or asymmetric hearing loss using a Cochlear Implant (CI) and to compare between the two subgroups. Materials and methodsThis was a prospective, longitudinal, observational, single-center study involving adult patients diagnosed with profound unilateral or asymmetric sensorineural hearing loss who underwent cochlear implantation. Sound localization was assessed in a chamber equipped with seven speakers evenly distributed from −90º to 90º. Stimuli were presented at 1000 Hz and intensities of 65 dB, 70 dB, and 75 dB. Each stimulus was presented only once per speaker, totaling 21 presentations. The number of correct responses at different intensities was recorded, and angular error in degrees was calculated to determine the mean angular distance between the patient-indicated speaker and the speaker presenting the stimulus. Both assessments were conducted preoperatively without a cochlear implant and two years post-implantation. ResultsThe total sample comprised 20 patients, with 9 assigned to the SSD group and 11 to the AHL group. The Preoperative Pure Tone Average (PTA) in free field was 31.7 dB in the SSD group and 41.8 dB in the AHL group. There was a statistically significant improvement in sound localization ability and angular error with the use of the cochlear implant at all intensities in both SSD and AHL subgroups. ConclusionsCochlear implantation in patients with SSD and AHL enhances sound localization, reducing mean angular error and increasing the number of correct sound localization responses.

Bone Conduction Implants: Comparative of Audiometric Results and Quality-of-Life Bonebridge® versus Osia®

Introduction: Bone conduction implants have been indicated for patients with conductive hearing loss, mixed hearing loss, and even profound unilateral sensorineural hearing loss. With the introduction of Bonebridge®, new transcutaneous implant options emerged. The latest is Osia®, a direct-drive variant or active systems, where the implant directly generates and applies vibration to the bone. Materials and Methods: Retrospective study of two cohorts of patients treated with active bone conduction implants at a single center, one with the Bonebridge® device and the other with Osia®. Outcomes: Fourteen patients were included, seven in each group (n = 14). The Bonebridge® group showed an average hearing gain in tonal intelligibility thresholds of 32.43 ± 21.39 dB and a gain in the average intelligibility threshold (with 50% discrimination) of 26.29 ± 19.10 dB. In the Osia® group, there was a gain in average tonal thresholds of 41.49 ± 14.16 dB and 23.72 ± 6.98 dB in average intelligibility thresholds. Both devices contributed to improvements in patients’ quality of life, as assessed with APHAB in all the variables studied in the test. Both devices offer rehabilitation for hearing loss as an alternative to hearing aids. The Osia® system shows statistically significant (p < 0.05) improvements in mid and high frequencies, but Bonebridge® slightly outperforms in speech understanding at 50%. Differences in average tonal thresholds and quality of life are not statistically significant. Conclusions: While auditory improvement is observed postimplantation, other aspects, such as intelligibility thresholds and quality of life, lack statistical significance. Given the limited experience with Osia® and the small sample size, the choice of the device should be personalized. Although the literature is inconsistent due to small sample sizes and variable approaches, some studies suggest potential advantages of the Osia® system, especially in speech comprehension in different environments and greater hearing gain compared to Bonebridge®.

Genetic Underpinnings and Audiological Characteristics in Children With Unilateral Sensorineural Hearing Loss.

Unilateral sensorineural hearing loss (USNHL) is a condition commonly encountered in otolaryngology clinics. However, its molecular pathogenesis remains unclear. This study aimed to investigate the genetic underpinnings of childhood USNHL and analyze the associated audiological features. Retrospective analysis of a prospectively recruited cohort. Tertiary referral center. We enrolled 38 children with USNHL between January 1, 2018, and December 31, 2021, and performed physical, audiological, imaging, and congenital cytomegalovirus (cCMV) examinations as well as genetic testing using next-generation sequencing (NGS) targeting 30 deafness genes. The audiological results were compared across different etiologies. Causative genetic variants were identified in 8 (21.1%) patients, including 5 with GJB2 variants, 2 with PAX3 variants, and 1 with the EDNRB variant. GJB2 variants were found to be associated with mild-to-moderate USNHL in various audiogram configurations, whereas PAX3 and EDNRB variants were associated with profound USNHL in flat audiogram configurations. In addition, whole-genome sequencing and extended NGS targeting 213 deafness genes were performed in 2 multiplex families compatible with autosomal recessive inheritance; yet no definite causative variants were identified. Cochlear nerve deficiency and cCMV infection were observed in 9 and 2, respectively, patients without definite genetic diagnoses. Genetic underpinnings can contribute to approximately 20% of childhood USNHL, and different genotypes are associated with various audiological features. These findings highlight the utility of genetic examinations in guiding the diagnosis, counseling, and treatment of USNHL in children.

Multicenter Results With an Active Transcutaneous Bone Conduction Implant in Patients With Single-sided Deafness.

Objective:To evaluate the audiological and subjective benefit from hearing rehabilitation with an active bone conduction implant in subjects with single-sided sensorineural deafness (SSD).Study Design:Prospective, multicenter, single-subject repeated measures.Setting:Tertiary referral center, five clinics in Germany and Switzerland.Patients:Seventeen subjects aged 18 years and older with severe to profound unilateral sensorineural hearing loss and contralateral normal hearing were followed up for 24 months.Intervention:Active bone conduction implant.Main Outcome Measures:Speech understanding in noise was assessed in three situations: with signal from front, deaf, or normal hearing side (with noise from front in all set-ups). Subjective benefit was evaluated using the Speech, Spatial, and Qualities of Hearing (SSQ-B) and Bern Benefit in Single-Sided Deafness (BBSS) questionnaire.Results:When the signal was coming from the deaf side the mean improvement of the speech reception threshold in noise ranged from 1.5 up to 2.2 dB with the device and was statistically and clinically significant at all tested timepoints. No significant difference between the aided and unaided situation was found when signal and noise were coming from the front. With the signal from the normal hearing side no clinically significant difference, that is, greater than 1 dB between the aided and unaided situation was found. The SSQ-B and BBSS questionnaire showed an overall improvement with no significant difference between time points.Conclusions:The study demonstrates long-term efficacy and benefit of the device in adults with SSD. Patients reported substantial and persistent subjective benefit from the active bone conduction implant.

Evaluation of hematological parameters of patients with profound unilateral and mild to profound bilateral sensorineural hearing loss

OBJECTIVE: This study aimed to investigate whether changes in blood cells have an effect on sensorineural hearing loss (SNHL) and compare the hematological parameters of profound unilateral and mild to profound bilateral SNHL patients and the normal population. METHODS: This retrospective study included 100 SNHL patients (81 males, 19 females; mean age: 37.8±16.8 years; range, 18 to 83 years) and 32 healthy volunteers (22 males, 10 females; mean age: 32.8±5.7 years; range 22 to 46 years) between January 2018 and February 2019. Patients who underwent pure tone audiometry and auditory brainstem response (ABR) tests for any reason and were found to have SNHL with no accompanying disease, and those that are non-smokers and without medication were included. Hematological parameters were evaluated in all three groups. RESULTS: Of the 100 SNHL patients, 59 had bilateral and 41 had profound unilateral SNHL. Among the evaluated hematological parameters, the difference in red blood cell count, hematocrit, hemoglobin, platelet distribution width, and neutrophil to lymphocyte ratio (NLR) between the groups was statistically significant (p&lt;0.001). CONCLUSION: Erythrocyte values were high in patients with profound unilateral SNHL, whereas platelet-related values [platelet count, PDW, and platelet to lymphocyte ratio) were higher in patients with bilateral SNHL. In addition, the NLR was found to be high in these patient groups. These findings may provide a treatment target, particularly for profound unilateral SNHL, and may shed light on further studies on SNHL.

Contralateral Masking in the Measurement of Auditory Brainstem Responses with Air-Conducted Tone Burst Stimuli in Individuals with Unilateral Hearing Loss.

Contralateral noise masking is an important aspect of auditory brainstem response (ABR) measurements. The primary aim of this study is to determine how contralateral white noise (WN) masking influences the amplitude and the latency of V wave generated during ABR measurements, using tone burst (TB), in adult ears with normal hearing (NH). The secondary aim of this study is to ascertain the need of contralateral masking in ABR measurements with the TB stimuli using a 3A insertion earphone, and to propose the applicability of WN masking in unilateral sensorineural hearing loss (USNHL). It is a cross-sectional observational and descriptive study. Experiment 1: Thirty individuals, without any otologic, psychological, or neurological dysfunction, were selected. Experiment 2: Fifteen individuals with previous audiological diagnoses of severe and profound USNHL were considered. The study involves ABR TB at specific frequencies of 0.5, 1, 2, and 4 kHz. Experiment 1: The evaluation was performed at the fixed intensity of 80 dB nHL (decibel normalized hearing level) on the tested ear, followed by the application of simultaneous masking to the nontested ear, intensity ranged from 0 to 80 dB. Experiment 2: ABR threshold measurements were first performed on the ear with hearing loss (HL) at the frequencies of 1, 2, and 4 kHz. The results were subsequently confirmed using contralateral masking. Experiment 1: At any given frequency, there were no statistically significant differences in the amplitude and latency of V wave with increase in the intensities of WN masking. Experiment 2: Cross-hearing was observed at least once in all frequencies analyzed through the occurrence of V wave. In conclusion, the contralateral WN masking at the maximum intensity of 80 dB does not affect the amplitude and latency of V wave of the ABR TB at 1, 2, and 4 kHz. Contralateral masking for the ABR TB presented using 3A insertion earphones is necessary at 1, 2, and 4 kHz in individuals with severe or profound degrees of USNHL and at intensities of 15, 20, and 10 dB above the ABR threshold of the nontested ear.

Audiometric and Radiological Evaluation of Patients with Single-Sided Sensorineural Hearing Loss; Single Center Experience

Background: In this study, It was aimed to make audiological and radiological evaluations of patients in the pediatric age group with unilateral sensorineural hearing loss, to reveal the degree of hearing loss, frequency of inner ear anomalies and the 8th cranial nerve anomaly according to age groups.Materials and Methods: Forty-four pediatric patients who applied to Harran University Medical Faculty Otorhinolaryngology outpatient clinic between June 2014 and June 2015 and diagnosed with unilateral sensorineural hearing loss were included in the study. Patient’s age, gender, lateralization of hearing loss, otoscopic examination findings, tympanometric and audiological findings, and radiological inner ear pathologies were evaluated.Results: Nineteen patients were female (43.2%) and 25 were male (56.8%). The age range was between 0 to 16 years. The mean age of male patients was 9.88 years, while the mean age of female patients was 9.84 years. Twenty (45.5%) of the patients had sensorineural hearing loss in the right ear and 24 (54.5%) in the left ear. In the patients audiometric examination, 2 (4.5%) patients had unilateral moderate; five patients had (11.4%) unilateral moderate-severe, 11 (25%) patients had unilateral severe, and 26 (59.1%) patients had unilateral profound sensorineural hearing loss. In the radiological examinations, 33 (75%) patients had no pathological findings, while 11 (25%) patients had internal ear pathology. Four patients (9.1%) had bilateral internal inner ear pathology, 4 (9.1%) had a right inner ear, and 3 (6.8%) had left inner ear pathology.Conclusions: Audiological and radiological imaging should be performed in patients with unilateral sensorineural hearing loss. High resolution computed tomography, and magnetic resonance imaging should be performed for the possible presence of an inner ear anomaly and evaluation of the 8th cranial nerve.

Single-Sided Deafness-Outcomes of Three Interventions for Profound Unilateral Sensorineural Hearing Loss: A Randomized Clinical Trial.

A comparison of three interventions for profound unilateral sensorineural hearing loss. Prospective, crossover randomized clinical trial. Fifteen participants with profound unilateral sensorineural hearing loss. Three potential technical interventions were compared: Bone Conduction Device on softband, Contralateral Routing of Signal (CROS), and Remote Microphone . Each intervention was randomly trialed for a period of 3 weeks, separated by a 1 week washout period. Speech in noise recognition test performed under four conditions (lateral noise poorer ear, lateral noise better ear, speech poorer ear, speech better ear). Standardized questionnaires (Abbreviated Profile of Hearing Aid Benefit, Bern Benefit in Single Sided Deafness Questionnaire, and Speech, Spatial, and Other Qualities 12) were used to evaluate amplification benefit at baseline and following each intervention. The use of remote microphone provided the best results in the speech recognition in noise test. A benefit in some signal-to-noise ratios was presented of the CROS over bone conduction device on softband in the Speech Poor Ear condition. On questionnaires of benefit, participants did not rate a particular intervention as significantly better than any other. Following the study, CROS was the intervention preferred by the 8 of 15 participants (53%). The majority of participants (80%) chose to continue with an intervention rather than no treatment. The use of all interventions resulted in increased performance in speech recognition in noise and rated higher on subjective benefits in comparison with baseline. People with SSD are a heterogeneous population when considering perceived difficulties. Future research should focus on segmenting the population of SSD depending on factors such as etiology, high frequency loss in the better ear, and age of acquired loss for the poorer ear. This stratification may possibly increase the benefit for the patient in terms of more individual-based clinical routines.

Effects of Simulated and Profound Unilateral Sensorineural Hearing Loss on Recognition of Speech in Competing Speech

Unilateral hearing loss (UHL) is a condition as common as bilateral hearing loss in adults. Because of the unilaterally reduced audibility associated with UHL, binaural processing of sounds may be disrupted. As a consequence, daily tasks such as listening to speech in a background of spatially distinct competing sounds may be challenging. A growing body of subjective and objective data suggests that spatial hearing is negatively affected by UHL. However, the type and degree of UHL vary considerably in previous studies. The aim here was to determine the effect of a profound sensorineural UHL, and of a simulated UHL, on recognition of speech in competing speech, and the binaural and monaural contributions to spatial release from masking, in a demanding multisource listening environment. Nine subjects (25 to 61 years) with profound sensorineural UHL [mean pure-tone average (PTA) across 0.5, 1, 2, and 4 kHz = 105 dB HL] and normal contralateral hearing (mean PTA = 7.2 dB HL) were included based on the criterion that the target and competing speech were inaudible in the ear with hearing loss. Thirteen subjects with normal hearing (19 to 60 years; mean left PTA = 4.1 dB HL; mean right PTA = 5.5 dB HL) contributed data in normal and simulated "mild-to-moderate" UHL conditions (PTA = 38.6 dB HL). The main outcome measure was the threshold for 40% correct speech recognition in colocated (0°) and spatially and symmetrically separated (±30° and ±150°) competing speech conditions. Spatial release from masking was quantified as the threshold difference between colocated and separated conditions. Thresholds in profound UHL were higher (worse) than normal hearing in separated and colocated conditions, and comparable to simulated UHL. Monaural spatial release from masking, that is, the spatial release achieved by subjects with profound UHL, was significantly different from zero and 49% of the magnitude of the spatial release from masking achieved by subjects with normal hearing. There were subjects with profound UHL who showed negative spatial release, whereas subjects with normal hearing consistently showed positive spatial release from masking in the normal condition. The simulated UHL had a larger effect on the speech recognition threshold for separated than for colocated conditions, resulting in decreased spatial release from masking. The difference in spatial release between normal-hearing and simulated UHL conditions increased with age. The results demonstrate that while recognition of speech in colocated and separated competing speech is impaired for profound sensorineural UHL, spatial release from masking may be possible when competing speech is symmetrically distributed around the listener. A "mild-to-moderate" simulated UHL decreases spatial release from masking compared with normal-hearing conditions and interacts with age, indicating that small amounts of residual hearing in the UHL ear may be more beneficial for separated than for colocated interferer conditions for young listeners.

Untreated primary hypothyroidism with simultaneous rhabdomyolysis, pericardial effusion, and sudden sensorineural hearing loss: a case report

BackgroundHypothyroidism, one of the prevalent endocrine disorders worldwide, has a broad spectrum of clinical manifestations, from an asymptomatic condition to myxedema coma. Although the majority of patients with hypothyroidism have minor clinical symptoms, which are recovered with levothyroxine treatment, some patients occasionally do experience fatal complications. Here we report, for the first time, the case of a patient who had hypothyroidism with simultaneous occurrence of rhabdomyolysis with acute kidney injury, moderate pericardial effusion, and sudden sensorineural hearing loss.Case presentationA 57-year-old man with a previous history of dyslipidemia and untreated hypothyroidism was admitted to the hospital due to shortness of breath, lethargy, lower extremity discomfort, and unilateral hearing loss. Laboratory results revealed rhabdomyolysis with acute kidney injury and severe hypothyroidism. We detected cardiomegaly without lung parenchymal infiltration on chest radiography and moderate pericardial effusion on transthoracic echocardiography. We performed pure tone audiometry and identified profound unilateral sensorineural hearing loss. Aggressive fluid resuscitation, levothyroxine treatment, and systemic and intratympanic steroid therapy alleviated the patient’s severe hypothyroidism, rhabdomyolysis, and pericardial effusion; however, sensorineural hearing loss was not fully recovered.ConclusionsEarly recognition of life-threatening complications is important in patients with severe hypothyroidism to prevent adverse outcomes. This case suggests that hypothyroidism should be considered in patients who have rhabdomyolysis with acute kidney disease and pericardial effusion. Moreover, sudden sensorineural hearing loss should be kept in mind as a rare complication of hypothyroidism.

Labyrinthectomy: Our Experience in a Tertiary Care Centre.

Labyrinthectomy is an effective surgical procedure for the management of poorly compensated unilateral peripheral vestibular dysfunction in the presence of a nonserviceable hearing. It involves removal of labyrinthine portion of the inner ear and exenteration of the neuroepithelium. In our institution, 8 cases underwent surgical labyrinthectomy from a period of 2013-2018 for various extensive disease manifestations, age ranges from 2 to 48years. Includes, a child of 2years age presented with bilateral foul smelling otorrhoea with external auditory canal cartilaginous stenosis, 5 cases of extensive cholesteatoma with labrynthitis and 2 cases of purulent labrynthitis among them 1 were suffering from Tuberculosis and was on Category 1 ATT and other one suffering from extensive granulation at the tympanomastoid area which was inconclusive of the diagnosis even after histopathological examination, so treated as tuberculosis and started on prophylactic antitubercular treatment in addition to surgery. All patients except the child gave past history of giddiness, but at the time of presentation they were not having giddiness or noticeable nystagmus and all had profound unilateral sensorineural hearing loss. Thus all the patients underwent a radical mastoidectomy with total labyrinthectomy and blind sac closure in 2 patients.

Vestibular and balance function is often impaired in children with profound unilateral sensorineural hearing loss

RationaleChildren with unilateral deafness could have concurrent vestibular dysfunction which would be associated with balance deficits and potentially impair overall development. The prevalence of vestibular and balance deficits remains to be defined in these children. MethodsTwenty children with unilateral deafness underwent comprehensive vestibular and balance evaluation. ResultsRetrospective review revealed that more than half of the cohort demonstrated some abnormality of the vestibular end organs (otoliths and horizontal canal), with the prevalence of end organ specific dysfunction ranging from 17 to 48% depending on organ tested and method used. In most children, impairment occurred only on the deaf side. Children with unilateral deafness also displayed significantly poorer balance function than their normal hearing peers. ConclusionsThe prevalence of vestibular dysfunction in children with unilateral deafness is high and similar to that of children with bilateral deafness. Vestibular and balance evaluation should be routine and the functional impact of combined vestibulo-cochlear sensory deficits considered.

The clinical features and prognosis of mumps-associated hearing loss: a retrospective, multi-institutional investigation in Japan

Conclusions: The majority of hearing loss due to mumps presents as unilateral profound sensorineural hearing loss, which is refractory to treatment. In rare cases of bilateral total deafness, cochlear implants were beneficial for speech perception. Vaccination against mumps is recommended to prevent mumps-associated hearing loss.Objective: The objective of this study is to investigate the clinical characteristics of hearing loss due to mumps and to evaluate hearing outcomes.Subjects and methods: The clinical parameters were analyzed under a retrospective multi-institutional study design in patients diagnosed with hearing loss due to mumps at the Otolaryngology departments of 19 hospitals between 1987 and 2016.Results: Sixty-seven patients with hearing loss due to mumps were enrolled. The study population consisted of 35 males and 32 females, ranging in age from 1 to 54, with a median age of 9.5 years. Sixty-three patients presented with unilateral, and 4 with bilateral hearing loss. Profound hearing loss was observed in 65 ears. Only one ear with severe hearing loss showed complete recovery. Four patients with bilateral hearing loss received cochlear implant surgery. Most of the patients with hearing loss due to mumps had no history of vaccination.

Evaluation of Long-Term Cochlear Implant Use in Subjects With Acquired Unilateral Profound Hearing Loss: Focus on Binaural Auditory Outcomes

Cochlear implantation (CI) in subjects with unilateral profound sensorineural hearing loss was investigated. The authors of the present study demonstrated the binaural auditory outcomes in a 12- and 36-month prospective cohort outcome study. The present study aimed to do a long-term (LT) evaluation of the auditory outcomes in an analogous study group. LT evaluation was derived from 12 single-sided deaf (SSD) CI recipients and from 11 CI recipients with asymmetric hearing loss (AHL). A structured interview was conducted with each subjects. Speech perception in noise and sound localization were assessed in a CIOFF and in a CION condition. Four binaural effects were calculated: summation effect (S0N0), squelch effect (S0NCI), combined head shadow effect (SCIN0), and spatial release from masking (SRM). At the LT evaluation, the contribution of a CI or a bone conduction device on speech perception in noise was investigated in two challenging spatial configurations in the SSD group. All (23/23) subjects wore their CI 7 days a week at LT follow-up evaluation, which ranged from 3 to 10 years after implantation. In the SSD group, a significant combined head shadow effect of 3.17 dB and an SRM benefit of 4.33 dB were found. In the AHL group, on the other hand, the summation effect (2.00 dB), the squelch effect (2.67 dB), the combined head shadow effect (3.67 dB), and SRM benefit (2.00 dB) were significant at LT testing. In both the spatial challenging configurations, the speech in noise results was significantly worse in the condition with the bone conduction device compared with the unaided condition. No negative effect was found for the CION condition. A significant benefit in the CION condition was found for sound localization compared with the CIOFF condition in the SSD group and in the AHL group. All subjects wore their CI 7 days a week at LT follow-up evaluation. The presence of binaural effects has been demonstrated with speech in noise testing, sound localization, and subjective evaluation. In the AHL group, all investigated binaural effects were found to be significant. In the SSD group on the other hand, only SRM and the head shadow, the two most robust binaural effects, were significantly present. However, it took 12M before the SSD and the AHL subjects significantly benefit from the head shadow effect. These reported results could guide counseling of future CI candidates with SSD and AHL in general.

Tinnitus suppression after cochlear implantation in patients with single-sided deafness

BackgroundTinnitus associated with single-sided deafness (SSD) is frequent and often incapacitating, and is difficult to treat. Numerous studies have reported the suppression of tinnitus by electrical stimulation of the acoustic pathway through a cochlear implant (CI), with a low risk of worsening of tinnitus after implantation.ObjectiveThe main aim of this study was to demonstrate the effectiveness of CI as a treatment option in patients with SSD and incapacitating tinnitus.Patients and methodsWe studied the tinnitus-suppression effect of CI in a series of 13 patients with unilateral profound sensorineural hearing loss (SSD), associated with incapacitating tinnitus with normal hearing in the contralateral ear. Tinnitus impact was measured with the Tinnitus Handicap Inventory (THI), and tinnitus severity was measured with the Tinnitus Rating Scale (TRS) before and after CI.ResultsThirteen patients were enrolled in this study, eight men and five women, ranging in age from 24 to 60 years with a mean±SD of 40±10 years. Mean scores for THI and TRS were obtained preoperatively and at 1 and 3 months postoperatively after activation of the CI. Mean scores for the THI total scores ranged from 79.6±7 preoperatively to 12±13.5 at 3 months postoperatively. Mean scores for the TRS ranged from 4.53±0.5 preoperatively to 1.46±0.5 at 3 months postoperatively. The postoperative THI and TRS improved significantly as compared with the baseline preoperative scores (P<0.005).ConclusionThe outcome of the current study supports the belief that CI is not only a treatment option for hearing loss in SSD but also a treatment option to suppress tinnitus.

Transcranial Attenuation in Patients with Single-Sided Deafness

Transcranial attenuation (TA) of bone-conducted sound has a high degree of variability by frequency and subject, which may play a role in the objective benefit of individuals with single-sided deafness (SSD) treated with a bone-anchored implant (BAI). This study sought to determine whether TA is predictive of benefit in individuals with SSD who receive a BAI. Adult, English-speaking patients with unilateral profound sensorineural hearing loss who underwent a BAI evaluation were included for study. Absolute TA values were consistent with previously published reports. Regression analysis indicated no correlation between TA values and aided speech-in-noise performance for any combined or individual frequencies. Measures of TA do not provide predictive value in determining behavioral outcomes in the SSD population. Specifically, low TA does not suggest improved outcomes with a BAI.

Ipsilateral single stage conversion from BAHA to cochlear implant

Profound unilateral sensorineural hearing loss is an indication for the placement of a bone anchored hearing aid. In a few unfortunate patients who later develop contralateral hearing loss, a cochlear implant becomes a good option. We present our experience in these cases and discuss our technique for single stage conversion from a bone anchored hearing aid to a cochlear implant.

Vestibular dysfunction in patients with post-mumps sensorineural hearing loss.

To study the possible damage to the vestibular system in patients with post-mumps sensorineural hearing loss. Nineteen patients with recent mumps infection participated in the study. All patients had unilateral profound sensorineural hearing loss or total hearing loss. Patients were subjected to video-nystagmography and vestibular-evoked myogenic potential testing. Eight patients (42.1 per cent) had normal video-nystagmography results and intact vestibular-evoked myogenic potentials on both sides, whereas the other 11 patients (57.9 per cent) had vestibular lesions in the form of marked canal weakness and absent vestibular-evoked myogenic potential responses on the same side as hearing loss. The overall findings indicated a peripheral site for the lesions. The majority of patients with post-mumps sensorineural hearing loss had peripheral vestibular pathology in the same ear as hearing loss. Further research should be directed to saving the inner ear following mumps infection.