Speech Discrimination Ability Research Articles

Relationship of Hearing Loss to Parkinson's Disease, Dementia, and APOE Genotype in Adults.

Background: Hearing loss has been recognized as a risk factor for dementia and non-motor features of Parkinson's disease (PD). The apolipoprotein E (APOE) protein contributes to maintenance and repair of neuronal cell membranes, causing age-related disorders. This study aimed to analyze the impact of hearing loss on cognitive impairment, PD severity, and APOE gene expression in these patients. Methods: A total of 72 out-patients diagnosed with either PD or hearing loss were enrolled in this study. The hearing assessment included pure-tone audiometry, speech reception thresholds, and speech discrimination ability. Dementia was assessed by filling out the Clinical Dementia Rating and Mini-Mental State Examination questionnaires. The severity of PD was assessed using the Modified Hoehn and Yahr scale. Blood samples were tested for the gene expression of APOE. Results: Out of the 72 cases, there were 44 males and 28 females, with an average age of 64.4 ± 9.1 years. A total of 41 out of 72 cases had dementia and had a worse hearing threshold than those without dementia (47.1 ± 24.4 vs. 31.7 ± 22.1 dB, p = 0.006). A total of 58 patients were diagnosed with PD, with 14 of them classified as having severe symptoms (Modified Hoehn and Yahr scale > 2). Patients with severe PD were found to have a worse hearing threshold (49.6 ± 28.3 vs. 30.3 ± 17.8 dB, p = 0.028) and higher prevalence of dementia (12/14 vs. 18/44, p = 0.006). Among 10 individuals with the APOE ε4 gene, the prevalence of dementia was higher than those without the ε4 allele (9/10 vs. 32/62, p = 0.036). Conclusions: Hearing loss is common in severe PD and in dementia patients. Severe PD has a negative impact on the hearing threshold and cognitive dysfunction. Patients with APOE ε4 have a higher prevalence of dementia.

Cochlear Implantation in Children Affected by Single-Sided Deafness: A Comprehensive Review.

Children with single-sided deafness (SSD) may experience delays in language and speech development. Reduced speech discrimination and poor sound localization abilities in young SSD patients may result in greater cognitive efforts required to focus and process auditory information, as well as increased listening-related fatigue. Consequently, these children can have a higher risk of academic failure and are often in need of extra help at school. Recently, cochlear implants (CIs) have been introduced as a rehabilitative option for these children, but their effectiveness is still a topic of debate. A literature review was performed according to PRISMA guidelines, searching the Medline database from inception to October 2023. The research identified nine papers that met the inclusion criteria. Data extracted from the selected studies included 311 children affected by SSD and cochlear implants. The reported audiological outcomes were further analyzed. Overall, a high level of satisfaction was described by parents of children with SSD and CI, and those who received a CI under the age of 3 presented better results. However, a proportion of patients did not use the device daily. Our review highlights the possible, and still controversial, role of CI for the hearing rehabilitation of children with unilateral deafness, underlining the need for further research in this field. To date, careful and comprehensive counseling with the child and the family is necessary before considering this option.

Identifying Developmental Language Disorder in Deaf Children with Cochlear Implants: A Case Study of Three Children.

(1) Background: While spoken language learning delays are assumed for deaf and hard of hearing (DHH) children after cochlear implant (CI), many catch up with their hearing peers. Some DHH children with CIs, however, show persistent delays in language, despite protective factors being in place. This suggests a developmental language disorder (DLD). However, at present there is little consensus on how to diagnose DLD in DHH children. (2) Methods: Given the lack of consensus in this area, a set of case studies provides an appropriate first step. The goal of this paper is to show the plausibility of a DLD diagnosis, following careful analysis of protective and risk factors. A retrospective case study review was conducted for three children. Their long-term language outcomes up to four years after CI were considered in the context of access to sound, speech sound discrimination, social skills and non-verbal cognition. (3) Results: It was possible to posit DLD in one child who had experienced good access to sound, alongside good speech discrimination abilities and social development, and normal non-verbal cognition, but who presented with severe language learning difficulties. (4) Conclusions: Finding markers for DLD in DHH children is important for diagnosis and intervention. The implications for clinical practice are discussed.

Exploring the impact hearing differences have on the development of speech perception during infancy

Early periods of perceptual skill development are driven by the dynamic interplay between language experiences and maturation of auditory sensory pathways. Among infants with normal hearing (INH), the first year of life is a seminal period for refining speech perception abilities shaped by exposure to language. Research suggests that refinement of speech discrimination abilities depends on an infant’s exposure to speech sounds, thus infants who are hard-of-hearing (IHH) are susceptible to atypical development during this period. Currently, the impact of inconsistent auditory cue access on development of speech perception is unknown among IHH. To investigate this, our lab has employed both electroencephalography (EEG) and a conditioned head turn (CHT) paradigm to examine auditory cue access and speech perception abilities, respectively, over the first year of life. We will provide a broad overview of our recent and continuing work among IHH and INH using EEG and CHT findings which suggest: (1) early EEG measures of cue access predict later behavioral speech perception abilities, (2) hearing age (duration of time between hearing aid fitting and testing) is related to speech perception abilities measured by CHT, and (3) a significant relationship between infant speech perception and both spoken expressive and receptive language abilities.

The Use of Heart Rate Responses Extracted From Functional Near-Infrared Spectroscopy Data as a Measure of Speech Discrimination Ability in Sleeping Infants.

Cardiac responses (e.g., heart rate changes) due to an autonomous response to sensory stimuli have been reported in several studies. This study investigated whether heart rate information extracted from functional near-infrared spectroscopy (fNIRS) data can be used to assess the discrimination of speech sounds in sleeping infants. This study also investigated the adaptation of the heart rate response over multiple, sequential stimulus presentations. fNIRS data were recorded from 23 infants with no known hearing loss, aged 2 to 10 months. Speech syllables were presented using a habituation/dishabituation test paradigm: the infant's heart rate response was first habituated by repeating blocks of one speech sound; then, the heart rate response was dishabituated with the contrasting (novel) speech sound. This stimulus presentation sequence was repeated for as long as the infants were asleep. The group-level average heart rate response to the novel stimulus was greater than that to the habituated first sound, indicating that sleeping infants were able to discriminate the speech sound contrast. A significant adaptation of the heart rate responses was seen over the session duration. The dishabituation response could be a valuable marker for speech discrimination, especially when used in conjunction with the fNIRS hemodynamic response.

Comparison and combination of gamified neurofeedback training and general behavioral training.

With the rapid development of the international community, foreign language learning has become increasingly important. Listening training is a particularly important component of foreign language learning. The most difficult aspect of listening training is the development of speech discrimination ability, which is crucial to speech perception. General behavioral training requires a substantial amount of time and attention. To address this, we previously developed a neurofeedback (NF) training system that enables unconscious learning of auditory discrimination. However, to our knowledge, no studies have compared NF training and general behavioral training. In the present study, we compared the learning effects of NF training, general behavioral training, and a combination of both strategies. Specifically, we developed a gamified and adapted NF training of auditory discrimination. We found that both NF training and general behavioral training enhanced behavioral performance, whereas only NF training elicited significant changes in brain activity. Furthermore, the participants that used both training methods exhibited the largest improvement in behavioral performance. This indicates that the combined use of NF and general behavioral training methods may be optimal for enhancing auditory discrimination ability when learning foreign languages.

Effect of Aging on Speech Discrimination

The aim of this study was to investigate the factors associated with decrease in speech discrimination ability seen in patients with presbycusis and whether aging has a significant effect on the observed decline. We retrospectively analyzed the medical records of patients aged >40 years who had undergone pure-tone audiometry, speech audiometry, and auditory brainstem response for hearing loss at our hospital from January 2019 to June 2021, and investigated the factors that correlated with speech discrimination score. We enrolled 103 patients with 120 ears, with a mean age of 65.8±11.9 years. The pure-tone average of the patients' thresholds at 0.5, 1, 2, and 4 kHz was 37.2±27.7 dB HL, and their mean speech discrimination score was 82.5%± 22.3%. A correlation analysis revealed a significant negative correlation between the patients' speech discrimination scores and age. In addition, all variables of pure-tone audiometry and the patients' auditory brainstem responses were significantly correlated with the speech discrimination scores. The pure-tone average had the strongest negative correlation. On analyzing the significant predictors of lower speech discrimination scores, using a multiple linear regression analysis, pure-tone average and age showed significant results. The speech discrimination ability of older patients with hearing loss significantly decreases with increasing pure-tone average and age.

Speech token detection and discrimination in individual infants using functional near-infrared spectroscopy

Speech detection and discrimination ability are important measures of hearing ability that may inform crucial audiological intervention decisions for individuals with a hearing impairment. However, behavioral assessment of speech discrimination can be difficult and inaccurate in infants, prompting the need for an objective measure of speech detection and discrimination ability. In this study, the authors used functional near-infrared spectroscopy (fNIRS) as the objective measure. Twenty-three infants, 2 to 10 months of age participated, all of whom had passed newborn hearing screening or diagnostic audiology testing. They were presented with speech tokens at a comfortable listening level in a natural sleep state using a habituation/dishabituation paradigm. The authors hypothesized that fNIRS responses to speech token detection as well as speech token contrast discrimination could be measured in individual infants. The authors found significant fNIRS responses to speech detection in 87% of tested infants (false positive rate 0%), as well as to speech discrimination in 35% of tested infants (false positive rate 9%). The results show initial promise for the use of fNIRS as an objective clinical tool for measuring infant speech detection and discrimination ability; the authors highlight the further optimizations of test procedures and analysis techniques that would be required to improve accuracy and reliability to levels needed for clinical decision-making.

Evaluation of working memory in relation to cochlear implant consonant speech discrimination

BackgroundSpeech perception in cochlear implants (CI) is affected by frequency resolution, exposure time, and working memory. Frequency discrimination is especially difficult in CI. Working memory is important for speech and language development and is expected to contribute to the vast variability in CI speech reception and expression outcome. The aim of this study is to evaluate CI patients’ consonants discrimination that varies in voicing, manner, and place of articulation imparting differences in pitch, time, and intensity, and also to evaluate working memory status and its possible effect on consonant discrimination.ResultsFifty-five CI patients were included in this study. Their aided thresholds were less than 40 dBHL. Consonant speech discrimination was assessed using Arabic consonant discrimination words. Working memory was assessed using Test of Memory and Learning-2 (TOMAL-2). Subjects were divided according to the onset of hearing loss into prelingual children and postlingual adults and teenagers. Consonant classes studied were fricatives, stops, nasals, and laterals. Performance on the high frequency CVC words was 64.23% ± 17.41 for prelinguals and 61.70% ± 14.47 for postlinguals. These scores were significantly lower than scores on phonetically balanced word list (PBWL) of 79.94% ± 12.69 for prelinguals and 80.80% ± 11.36 for postlinguals. The lowest scores were for the fricatives. Working memory scores were strongly and positively correlated with speech discrimination scores.ConclusionsConsonant discrimination using high frequency weighted words can provide a realistic tool for assessment of CI speech perception. Working memory skills showed a strong positive relationship with speech discrimination abilities in CI.

Auditory perception skills in children receiving simultaneous bilateral cochlear implants: early speech-discrimination results.

The objective of the present study is to evaluate early speech-perception abilities using VRISD in toddlers who received simultaneous bilateral CIs between 12 and 36months of age and to compare them with the findings in NH infants and to monitor the development of speech-perception skills within 6months after cochlear implantation. The VRISD test was performed using video visual reinforcement to assess speech-discrimination ability in the CI and NH groups. Four stimuli were used for testing in the present study: /a/, /i/, /ba/, and /da/. The two contrasts used for the research were /a-i/ and /ba-da/. Auditory and listening skills in the CI group were assessed using the IT-MAIS. The responses to the /a-i/ and /ba-da/ phoneme contrast were found to be similar in the NH and CI groups. No statistically significant difference was found between the groups (p > 0.05). VRISD test result and the IT-MAIS score were highly correlated in CI group (p = 0.001, r = 0.822). The VRISD test can be effectively used to evaluate the development of speech-discrimination skills in hearing-impaired babies before and after CI. This research suggests that the development of speech-perception ability with CI is seriously influenced by environmental exposure and sound access. To the best of our knowledge, this is the first study to evaluate the speech-perception skills in toddlers with simultaneous bilateral CI.

Asymmetry of Auditory-Motor Speech Processing is Determined by Language Experience.

Speech processing relies on interactions between auditory and motor systems and is asymmetrically organized in the human brain. The left auditory system is specialized for processing of phonemes, whereas the right is specialized for processing of pitch changes in speech affecting prosody. In speakers of tonal languages, however, processing of pitch (i.e., tone) changes that alter word meaning is left-lateralized indicating that linguistic function and language experience shape speech processing asymmetries. Here, we investigated the asymmetry of motor contributions to auditory speech processing in male and female speakers of tonal and non-tonal languages. We temporarily disrupted the right or left speech motor cortex using transcranial magnetic stimulation (TMS) and measured the impact of these disruptions on auditory discrimination (mismatch negativity; MMN) responses to phoneme and tone changes in sequences of syllables using electroencephalography (EEG). We found that the effect of motor disruptions on processing of tone changes differed between language groups: disruption of the right speech motor cortex suppressed responses to tone changes in non-tonal language speakers, whereas disruption of the left speech motor cortex suppressed responses to tone changes in tonal language speakers. In non-tonal language speakers, the effects of disruption of left speech motor cortex on responses to tone changes were inconclusive. For phoneme changes, disruption of left but not right speech motor cortex suppressed responses in both language groups. We conclude that the contributions of the right and left speech motor cortex to auditory speech processing are determined by the functional roles of acoustic cues in the listener's native language.SIGNIFICANCE STATEMENT The principles underlying hemispheric asymmetries of auditory speech processing remain debated. The asymmetry of processing of speech sounds is affected by low-level acoustic cues, but also by their linguistic function. By combining transcranial magnetic stimulation (TMS) and electroencephalography (EEG), we investigated the asymmetry of motor contributions to auditory speech processing in tonal and non-tonal language speakers. We provide causal evidence that the functional role of the acoustic cues in the listener's native language affects the asymmetry of motor influences on auditory speech discrimination ability [indexed by mismatch negativity (MMN) responses]. Lateralized top-down motor influences can affect asymmetry of speech processing in the auditory system.

Effect of Auditory Training and Speech Reading on Speech Discrimination Ability of Children with Hearing Loss in Ibadan, Oyo State, Nigeria

Effect of Auditory Training and Speech Reading on Speech Discrimination Ability of Children with Hearing Loss in Ibadan, Oyo State, Nigeria

Effects of the Conductive Component of Hearing Loss on Speech Discrimination Ability

Effects of decreasing auditory activity on speech discrimination ability are not fully understood. To investigate influence of decrease in auditory activity due to conductive and sensorineural components of hearing loss (HL) on speech discrimination ability. We retrospectively reviewed medical records of patients with suspected HL at Kitasato University Hospital in 2017 and 2018. Patients were divided according to pure-tone audiometry findings: no HL (N-HL), conductive HL (C-HL), sensorineural HL (S-HL), and mixed HL (M-HL) groups. In total, 149 patients (224 ears) were eligible. The maximum speech discrimination score (SDSmax) for all ears significantly negatively correlated with age (r = -0.29, p<0.0001) and bone conduction (BC) threshold (r = -0.55, p<0.0001). For patients aged <50 years in N-HL and C-HL groups, SDSmax was nearly 100%, with no significant difference. SDSmax was significantly lower for older patients (≥50 years) in the M-HL group than in the S-HL group, even though there were no significant differences in age and BC thresholds between groups. Decrease of auditory activity due to the conductive component of M-HL may worsen speech discrimination ability. Early treatment of M-HL would be desirable for the preservation of auditory function.

TIMELY APPLICATION OF HEARING AIDS HELPS PRESERVE SPEECH DISCRIMINATION ABILITY

Background Hearing damage has been related to certain brain conditions, particularly cognitive impairment. Hearing aids are commonly prescribed to compensate for hearing loss. In the current study, the time from when a hearing aid was first used by a patient was studied in terms of their speech discrimination ability in comparison with matched individuals who used no hearing aids. Material and methods Some 204 patients were enrolled and classified into two groups: 102 patients willing to use a hearing aid as soon as their hearing loss was diagnosed (Aided Group) and 102 patients who were also diagnosed with hearing loss but did not adopt hearing aids (Unaided group). The mean hearing threshold (HT) at octave intervals from 125 to 8000 Hz, speech reception threshold (SRT), and speech discrimination score (SDS) were compared between the two groups of patients at 18 and 36 months. Results According to measurements 36 months after a patient’s first attendance, patients who did not use a hearing aid immediately had significantly lower SDS scores compared to patients who decided to use hearing aids early on. This clearly demonstrates that timely application of hearing aids can prevent the loss of speech perception capabilities. Conclusions It is suggested that the golden time for hearing aid application is important. Hearing aids worn during the early stages of hearing loss can lead to improved speech discrimination ability. If there is hearing loss while discrimination is good (80% or higher), a hearing aid will be very useful. However, if discrimination is poor the results will not be as good.

An acoustic perspective on 45 years of infant speech perception. II. Vowels and suprasegmentals

AbstractIn this two‐part review we examine the major results from infant consonant (Part 1), vowel, and suprasegmental (Part 2) discrimination research over the past 45 years from an acoustic perspective—an exegesis of the developmental perception literature that appeals to both acoustic aspects of speech contrasts and historically relevant typological facts about sound systems of the world's languages. We argue that infants’ speech discrimination abilities are best viewed through a lens that considers both synchronic and diachronic aspects of the particular speech contrast. The key to this approach is the notion that acoustic–perceptual salience, or the relative separation of speech categories along perceptually relevant acoustic dimensions and corresponding discrimination performance in adults, is reflected in both infant's perceptual performance and patterns observed in phonological typology and history. The present review highlights challenges offered by four decades of literature, identifies broad patterns in infant vowel perception according to the acoustic properties of speech contrasts, and offers linguistically motivated explanations and directions for future research into the nature of young infants’ discrimination abilities.

Comparison of Cortical Auditory Evoked Potential Findings in Presbycusis with Low and High Word Recognition Score.

Deteriorated speech understanding is a common complaint in elderly people, and behavioral tests are used for routine clinical assessment of this problem. Cortical auditory evoked potentials (CAEPs) are frequently used for assessing speech detection and discrimination abilities of the elderly, and give promise for differential diagnosis of speech understanding problems. The aim of the study was to compare the P1, N1, and P2 CAEP latencies and amplitudes in presbycusis with low and high word recognition score (WRS). A cross-sectional study design was used for the study. Two groups were formed from the patients with presbycusis based on their scores on the speech recognition test. Fifty-seven elderly volunteers participated in the study. The first group composed of 27 participants with high WRS, the other group composed of 30 participants with low WRS. The CAEP waves were recorded from these participants using speech signals. Latencies and amplitudes of P1-N1-P2 waves of the two groups were compared with the t-test statistic. There were significant prolongation of P1 and N1 latencies in presbycusis with low WRS when compared with presbycusis with a relatively high word score (p < 0.05). According to the result of the research, P1 and N1 latencies of presbycusis with low WRS were longer than the participants with high WRS. Factors affecting peripheral auditory system, such as stimulus sensation level, might be responsible for P1 and N1 latency prolongation of the low WRS group.

An acoustic perspective on 45 years of infant speech perception, Part 1: Consonants

AbstractIn this two‐part review, we examine major results from infant consonant (Part 1), vowel, and suprasegmental (Part 2) discrimination research over the past 45 years from an acoustic perspective—an exegesis of the developmental speech perception literature that appeals to both acoustic aspects of speech contrasts and historically relevant typological facts about the sound systems of the world's languages. We argue that infants' speech discrimination abilities are best viewed through a lens that considers both synchronic and diachronic aspects of the particular speech contrast. The key to this approach is the notion that acoustic–perceptual salience, or the relative separation of speech categories along perceptually relevant acoustic dimensions and corresponding discrimination performance in adults, is reflected in both infants perceptual performance and patterns observed in phonological typology and history. The review highlights challenges presented by four decades of literature, identifies broad patterns in infant consonant perception according to the acoustic properties of speech contrasts, and offers linguistically motivated explanations and directions for future research into the nature of young infants' discrimination abilities.

Comparison of Cortical Auditory Evoked Potential Findings in Presbycusis with Low and High Word Recognition Score

Background: Deteriorated speech understanding is a common complaint in elderly people, and behavioraltests are used for routine clinical assessment of this problem. Cortical auditory evoked potentials(CAEPs) are frequently used for assessing speech detection and discrimination abilities of the elderly,and give promise for differential diagnosis of speech understanding problems.&lt;br /&gt;Purpose: The aim of the study was to compare the P1, N1, and P2 CAEP latencies and amplitudes inpresbycusis with low and high word recognition score (WRS).&lt;br /&gt;Research Design: A cross-sectional study design was used for the study. Two groups were formed fromthe patients with presbycusis based on their scores on the speech recognition test.&lt;br /&gt;Study Sample: Fifty-seven elderly volunteers participated in the study. The first group composed of 27participants with high WRS, the other group composed of 30 participants with low WRS.&lt;br /&gt;Data Collection and Analysis: The CAEP waves were recorded from these participants using speech signals.Latencies and amplitudes of P1-N1-P2 waves of the two groups were compared with the t-test statistic.&lt;br /&gt;Results: There were significant prolongation of P1 and N1 latencies in presbycusis with low WRS whencompared with presbycusis with a relatively high word score (p &lt; 0.05).&lt;br /&gt;Conclusion: According to the result of the research, P1 and N1 latencies of presbycusis with low WRSwere longer than the participants with high WRS. Factors affecting peripheral auditory system, such asstimulus sensation level, might be responsible for P1 and N1 latency prolongation of the low WRS group.&lt;br /&gt;

Comparison of Cortical Auditory Evoked Potential Findings in Presbycusis with Low and High Word-Recognition Score

Background: Deteriorated speech understanding is a common complaint in elderly people, and behavioraltests are used for routine clinical assessment of this problem. Cortical auditory evoked potentials(CAEPs) are frequently used for assessing speech detection and discrimination abilities of the elderly,and give promise for differential diagnosis of speech-understanding problems.&lt;br /&gt;Purpose: The aim of the study was to compare the P1, N1, and P2 CAEP latencies and amplitudes inpresbycusis with low and high word recognition score (WRS).&lt;br /&gt;Research Design: A cross-sectional study design was used for the study. Two groups were formed fromthe patients with presbycusis based on their scores on the speech-recognition test.&lt;br /&gt;Study Sample: Fifty-seven elderly volunteers participated in the study. The first group composed of 27participants with high WRS, the other group composed of 30 participants with low WRS.&lt;br /&gt;Data Collection and Analysis: The CAEP waves were recorded from these participants using speech signals.Latencies and amplitudes of P1-N1-P2 waves of the two groups were compared with the t-test statistic.&lt;br /&gt;Results: There were significant prolongation of P1 and N1 latencies in presbycusis with low WRS whencompared with presbycusis with a relatively high word score (p &lt; 0.05).&lt;br /&gt;Conclusion: According to the result of the research, P1 and N1 latencies of presbycusis with low WRSwere longer than the participants with high WRS. Factors affecting peripheral auditory system, such asstimulus sensation level, might be responsible for P1 and N1 latency prolongation of the low WRS group.&lt;br /&gt;

Audiological outcomes in sudden sensorineural hearing loss with presumed inner ear hemorrhage.

To explore the hearing outcomes and prognostic factors in patients with sudden sensorineural hearing loss resulting from inner ear hemorrhage. 42 patients (22 male and 20 female) were recruited from January 2016 to December 2017. Intravenous methylprednisolone and/or intratympanic corticosteroid were used as salvage therapy. The main measures included systemic risk factors and audiometric outcomes as proposed by American Academy of Otolaryngology-Head and Neck Surgery Hearing Loss Scale. All individuals were assessed at baseline, discharge (2 weeks post-treatment) and at 1, 3 and 6 months. The mean ages of patients were 39.3 ± 14.8 yrs. Cardiovascular disorders were seen in 19.0-33.3% of cases. Restoration of hearing and speech discrimination abilities were assessed at the first month post-treatment versus initial levels (95.5 ± 15.5 vs. 109.2 ± 9.6 dB, p = 0.000; and 17.6 ± 24.4 vs. 1.3 ± 4.0%, p = 0.003, respectively). Word recognition scores continued to recover at month 6 (38.7 ± 35.4%, p = 0.000), whereas puretone ceased to change (90.8 ± 16.2 dB, p = 0.139). The final percentages of complete, partial and no recovery were 0%, 57.1% and 42.9% respectively. The prognosis was independent of accompanying systemic risk factors as analyzed in this study. Intratympanic intervention was associated with improved word recognition scores, although intravenous corticosteroid was not. Profound sudden sensorineural hearing loss caused by inner ear hemorrhage often has an unsatisfactory prognosis. However, this cohort did experience partial audiological recovery with delayed onset. Immediate and effective intratympanic corticosteroid may have therapeutic potential for this intractable disease.