Adults With Cochlear Implants: Residual Hearing, Musical Perception, and Temporal Skills.

PURPOSE: The main purpose of the study is to investigate whether the presence of residual hearing before cochlear implantation has an effect on temporal pattern recognition skills and musical perception in adults. METHOD: The study included adults with cochlear implants who were between the ages of 20 and 45 years. Adults with cochlear implants were divided into two groups: absent (n = 20) and available (n = 20) groups according to residual hearing before implantation. Frequency Pattern and Duration Pattern tests were applied to evaluate temporal pattern recognition, and the "Music-Related Quality of Life Questionnaire" was applied to evaluate musical perceptions. RESULTS: There were no statistically significant differences between the groups in terms of cochlear-implanted hearing thresholds and speech recognition scores, musical perception, and musical activities, according to their residual hearing. On the other hand, the frequency and duration pattern recognition skills were statistically significantly better in adults with residual hearing. Also, statistically significant correlations were obtained in terms of temporal skills and musical perception subskills. CONCLUSIONS: The presence of residual hearing before cochlear implantation may affect temporal auditory processing skills. It is concluded that the integration of cochlear implants may affect temporal processing skills in adults as well as quality of life. It is recommended that experts be aware of this issue and pay attention to residual hearing.

Evaluation of Verbal Working Memory and Phonemic Analysis Skills in Adolescents with Cochlear Implant.

Introduction Adolescents with cochlear implants may have difficulty developing language and memory abilities. Objective The primary objectives of this study are (1) to evaluate the language skills and verbal working memory performance of early and late diagnosed-intervened cochlear implanted adolescents and (2) to investigate the relationship between the verbal working memory and phonemic analysis skills in adolescents with cochlear implant. Methods This study included 72 participants with cochlear implant aged between 10 and 18 years. The participants were divided into two groups; those who had first hearing aid at the age of 6 months at the latest and had a first cochlear implant in at least one ear at the latest at 24 months were included in the early group, all the others were in the late group. The phonemic analysis test, a subtest of the test of language development: Primary, 4th edition (TOLD: P-4) - Turkish version, was used to assess language-based abilities, and the meaningless word repetition (MWR) test was utilized to assess verbal working memory. Results The adolescents with cochlear implants who received early diagnosis and intervention performed statistically significantly better in phonemic analysis scores and verbal working memory tests ( p < 0.001). A statistically significant relationship was found between phonemic analysis and verbal working memory skills (Pearson, r = 0.567 and r = 0.659, p < 0.001). Conclusions Rehabilitation of phonological skills can contribute to the development of verbal working memory in adolescents with cochlear implants. There is a need for further studies on this issue with more detailed tests.

The Challenges Encountered by Conventional Hearing Aid and/or Cochlear Implant Users During the COVID-19 Pandemic.

PURPOSE: The purpose of this study was to evaluate the stress, quality of life, and access to hearing health services experienced by adults and children who use hearing aid and/or cochlear implant and their families during the COVID-19 pandemic. METHOD: Perceived Stress Scale, Personal Wellbeing Index, and World Health Organization Quality of Life questionnaires were applied online to 30 adults with hearing loss and parents of 61 children with hearing loss. Also, a survey was conducted to acquire more about the demographics of people who use cochlear implants and conventional hearing aids, as well as the difficulties they encountered during the COVID-19 period. RESULTS: Although child user families experienced more problems than adults during the COVID-19 period, the stress levels of child user families were higher than that of adult user families (p = .05). Even though there was no statistically significant difference, the families of pediatric users had lower quality of life than adult users. CONCLUSIONS: The stress/anxiety problems that arise as a result of this are coming to the forefront of both patients and their relatives. The findings indicate that creative strategies should be developed to give individuals with hearing loss efficient access to hearing health services.

Evaluation of Verbal Working Memory and Phonemic Analysis Skills in Adolescents with Cochlear Implant

Abstract Introduction Adolescents with cochlear implants may have difficulty developing language and memory abilities. Objective The primary objectives of this study are (1) to evaluate the language skills and verbal working memory performance of early and late diagnosed-intervened cochlear implanted adolescents and (2) to investigate the relationship between the verbal working memory and phonemic analysis skills in adolescents with cochlear implant. Methods This study included 72 participants with cochlear implant aged between 10 and 18 years. The participants were divided into two groups; those who had first hearing aid at the age of 6 months at the latest and had a first cochlear implant in at least one ear at the latest at 24 months were included in the early group, all the others were in the late group. The phonemic analysis test, a subtest of the test of language development: Primary, 4th edition (TOLD: P-4) - Turkish version, was used to assess language-based abilities, and the meaningless word repetition (MWR) test was utilized to assess verbal working memory. Results The adolescents with cochlear implants who received early diagnosis and intervention performed statistically significantly better in phonemic analysis scores and verbal working memory tests (p < 0.001). A statistically significant relationship was found between phonemic analysis and verbal working memory skills (Pearson, r = 0.567 and r = 0.659, p < 0.001). Conclusions Rehabilitation of phonological skills can contribute to the development of verbal working memory in adolescents with cochlear implants. There is a need for further studies on this issue with more detailed tests.

Concordance of vestibular test batteries in patients with vestibular neuritis.

BACKGROUND: A growing number of vestibular function tests are utilized to differentiate and verify the diagnosis of vestibular neuritis. The aim of this study is to retrospectively investigate the consistency of the results of the objective vestibular test batteries in patients with a preliminary diagnosis of vestibular neuritis. METHODS: We reviewed a total of 37 adult patients (mean age: 39.03 ± 11.67, 19 females, 18 males) who met the inclusion criteria with a prediagnosis of vestibular neuritis from 379 patients suffering vestibular symptoms. Caloric test (CVT), video head impulse test (vHIT), and ocular and cervical VEMP tests were compared with Cohen's kappa (Κ) analysis according to the likely affected part of the vestibular nerve. RESULTS: The highest statistically significant K value was found between horizontal vHIT and ocular VEMP (K = 0.707; good grade, p < 0.05). All the tests compared with CVT were poorly in agreement (K = 0.288; 0.262; 0.256 for HvHIT, oVEMP, AvHIT, respectively, p < 0.05). DISCUSSION: VEMP and vHIT tests have prominent diagnostic value and agree with each other for detecting and differentiating the types of vestibular neuritis. Further studies should aim to include cutting-edge technologies such as functional HIT and ocular counter roll test.

Assessment of auditory functions in patients with hepatic glycogen storage diseases.

BACKGROUND: Hepatic glycogen storage diseases are a group of diseases manifesting mainly with hypoglycemia and hepatomegaly. The patients require frequent daytime and nocturnal feedings. Hypoglycemia may cause sensorineural hearing loss and nocturnal feeding is a risk factor for the development of gastroesophageal reflux that may cause chronic otitis media and hearing loss consequently. We aimed to determine the prevalence and characteristics of hearing loss in hepatic glycogen storage diseases. METHODS: A total of 24 patients with hepatic glycogen storage disease (15 glycogen storage disease type I and 9 non type I) and 24 age/sex matched healthy controls were enrolled in the study. Pure tone audiometer, immitansmetry, acoustic reflex measurement, otoacoustic emission test (OAE) and auditory brainstem response (ABR) tests were applied to all participants. RESULTS: Hearing loss was determined in 17/24 patients (12 glycogen storage disease type I and 5 non type I) with pure tone audiometer. Interpretation of all the findings revealed a total of 8 patients had conductive and 9 had mixed hearing loss. All parameters were significantly different than the control group. CONCLUSIONS: This is the first study to comprehensively assess the auditory functions of patients with hepatic glycogen storage disease. Audiological findings determined a significantly increased prevalence of conductive/ mixed type hearing loss in the patient group which is a new finding in the literature. Further studies with extended patient numbers are required to enlighten the underlying pathophysiology.

Vocal outcomes after COVID-19 infection: acoustic voice analyses, durational measurements, self-reported findings, and auditory-perceptual evaluations.

PURPOSE: The ongoing literature suggests that COVID-19 may have a potential impact on voice characteristics during the infection period. In the current study, we explored how the disease deteriorates different vocal parameters in patients who recovered from COVID-19. METHODS: A total of 80 participants, 40 patients with a prior history of COVID-19 (20 male, 20 female) with a mean age of 39.9 ± 8.8 (range, 21-53) and 40 gender and age-matched healthy individuals (mean age, 37.3 ± 8.8; range, 21-54) were included to this study. The data of acoustic voice analyses, durational measurements, patient-reported outcomes, and auditory-perceptual evaluations were compared between the study group and the control group. Correlation analyses were conducted to examine the association between the clinical characteristics of the recovering patients and measured outcomes. RESULTS: Maximum phonation time (MPT) and the scores of both Voice Handicap Index-10 (VHI-10) and Voice-Related Quality of Life (V-RQOL) questionnaires significantly differed between the groups, which was more evident in female participants. The overall severity score of dysphonia was found to be higher in the study group than the control group (p = 0.023), but gender-based comparisons reached significance only in males (p = 0.032). VHI-10 and V-RQOL revealed significant correlations with the symptom scores of the disease. CONCLUSIONS: Patients with a prior history of COVID-19 had significantly lower MPT, increased VHI-10 scores, decreased voice-related quality of life based on the V-RQOL questionnaire, and higher overall severity scores in the auditory-perceptual evaluation. Self-reported voice complaints disclosed close relationships with the symptom scores of COVID-19 disease.

Instantaneous Gain in Video Head Impulse Test: A Reliability Study.

Objective: Vestibulo-ocular reflex gain at 40, 60, and 80 ms following the head movement start is calculated as the instantaneous gain. The purpose of this study was to investigate the reliability of instantaneous gain values at 40, 60, and 80 ms with testing and retesting in healthy adults. Methods: The study was conducted with Interacoustics EyeSeeCam vHIT (Interacoustics, Denmark), and 42 healthy adults were evaluated twice at half-hour intervals (test and retest) by the same practitioner. Agreement of mean gain, gain asymmetry, and instantaneous gain was evaluated using a paired samples t-test. Results: Mean age of the participants was 33.62±11.17; 38.1% were male and 61.9% were female. In the degree of the agreement, paired sample correlation (r) between test and retest results of the horizontal semicircular canals was found to be higher than those of the vertical semicircular canals. Moreover, the highest correlation between test and retest for instantaneous gain, calculated for only horizontal semicircular canals, was found at 80 ms on each side (0.791; 0.838, right and left, respectively), while the lowest correlation between these parameters was found between the gain asymmetry values. Conclusion: The video head impulse test used in studies calculates the mean gain in approximately at 60 ms. However, the higher correlation between mean gain values at 80 ms in our findings indicates that gain calculation strategies and techniques for latencies should be discussed. Additionally, the low correlation of vertical semicircular canals for mean gain and gain asymmetry between semicircular canal pairs, which clearly shows that more standard and more reliable methods should be developed.

The comprehensive audiological evaluation in young violinists: the medial olivocochlear system, high frequency thresholds, and the auditory figure ground test.

PURPOSE: The aim of this study was to examine whether the medial olivocochlear hearing system functions, the high frequency hearing thresholds and speech discrimination in noise performance can guide us in assessing the risk of hearing loss among violinists. It is aimed to investigate possible hearing damage that is not reflected in pure tone hearing thresholds in violinists. METHODS: The participants (n = 50) who have normal hearing and the ages of 18-30 were included in this study in two groups: violinists and controls who are unrelated to music. High frequency audiometer, auditory figure ground test (AFG) for speech discrimination in noise performance, Distortion Product Otoacoustic Emission (DPOAE) and contralateral suppression on DPOAE for medial olivocochlear system function tests were applied to all participants as well as routine audiological tests. RESULTS: The high frequency hearing thresholds were obtained higher in violinists compared to the controls. In violinists, the AFG test scores and the suppression amount at 1 kHz were lower than the controls. In addition, DPOAE responses at 4-6 kHz were obtained lower in violinists (p < 0.05). CONCLUSION: The reason for high frequency hearing loss, decreased DPOAE response amplitudes, and poor medial olivocochlear function in violinists can be explained by the long-term exposure to high-level noise caused by the violin, one of the closest musical instruments. Routine and comprehensive audiological follow-up is crucial for musicians.

Methyl donor supplementation alters serum leptin levels and increases appetite but not body weight in cross-fostered male Syrian hamster offspring (Mesocricetus auratus).

A pregnant hamster's exposure to changes in environmental factors, such as light, temperature and nutrition, may influence behavioural and physiological changes in offspring. In this study, dietary methyl donor supplementation was employed to examine the role of maternal diet on appetite, body weight, serum leptin levels and locomotor activity in male Syrian hamster offspring. Dams were fed a standard control (SC) or methyl donor-supplemented (MDSD) diet through pregnancy and lactation. At birth, offspring were cross-fostered to dams fed an SC or MDSD diet (SC-MDSD and MDSD-SC) or remained with their birth mothers (SC-SC and MDSD-MDSD). At weaning, offspring were fed a SC or MDSD diet until 60 days of age. Food intake, serum leptin levels and locomotor activity were measured from 30-60 days of age. Offspring fed a MDSD diet post-weaning (MDSD-MDSD and SC-MDSD) consumed more than double the amount of food daily compared with offspring fed a SC diet post-weaning (SC-SC, MDSD-SC). Interestingly, there were no observed differences in body weight among all four groups. Serum leptin levels at 60 days of age were depressed in offspring fed a MDSD diet post-weaning (MDSD-MDSD and SC-MDSD). There were no observed differences in wheel running activity between the SC-SC and MDSC-SC groups. Wheel running activity was at least twice the amount in offspring fed a MDSD diet post-weaning (SC-MDSD and MDSD-MDSD). Taken together, these results indicate that the timing of methyl donor supplementation appears to be an important factor during the development of offspring.

Turkish Validity and Reliability Study of the Speech, Spatial and Qualities of Hearing Scale.

OBJECTIVE: The Speech, Spatial and Qualities of Hearing Scale (SSQ) is a self-report scale that evaluates hearing in complex daily life situations in the areas of hearing quality, speech perception, and spatial perception. It is also frequently used in the follow-up of hearing-impaired people, hearing aid and cochlear implant users. It is aimed to translate and adapt SSQ into Turkish, and to investigate its test-retest reliability, and construct validity and reliability, and further to present associations of SSQ scores with the pure tone averages (PTA). METHODS: The Turkish SSQ (Tr-SSQ) scale was administered on 114 adults including those with and without hearing loss. Cronbach's alpha was used to assess its reliability. The reliability coefficient of the scale was calculated by test-retest method. Associations of SSQ scores with PTAs in better and worse hearing ears (BHE and WHE) were evaluated. RESULTS: Tr-SSQ presented high internal consistency (Cronbach's alpha = 0.984) and test-retest reliability (r=0.994). Tr-SSQ scores were lower in the subjects with hearing loss and correlated with PTAs. Age was found to be correlated with PTAs; regression analysis demonstrated that only WHE-PTA was extracted as explanatory variable for average Tr-SSQ, speech perception and spatial perception scores while both BHE-PTA and WHE-PTA were found to be predictors of hearing quality, but not age for any of Tr-SSQ scores. CONCLUSION: Tr-SSQ is a convenient tool for assessing the hearing abilities of individuals with hearing impaired.

Turkish stimulability treatment program for children with speech sound disorders: a preliminary study.

PURPOSE: The objective of this study was to create a stimulability treatment program in Turkish language that targets Turkish consonants and vowels and to present the preliminary findings of the effectiveness of the program in a small group of Turkish children with speech sound disorder (SSD). METHOD: Twenty-two children with SSD participated in the study. The Turkish Articulation and Phonology Test (SST) was used in the assessment of children. Intelligibility ratings were completed by their caregivers through the Turkish version of Intelligibility in Context Scale (ICS), and the stimulability assessments were completed. Stimulability intervention were given for a total of 12 sessions. Comparisons were made between the pre- and post-test results of children, and the preliminary data of treatment efficacy were collected. RESULTS: The data analysis showed a significant difference in the total number of articulation errors of children between Time 1 and Time 2 (p < .0001), a statistically significant difference in auditory discrimination scores between the two measurements, (p < .05), and an increase in stimulability ratings from Time 1 to Time 2. CONCLUSION: These findings provide initial data on the positive effect of the Turkish stimulability treatment program for children with SSD. This preliminary study would contribute toward designing the main trial for an evidence-based study for the Turkish stimulability treatment program, thereby leading to better practices in SSD.

Negative Effects of Noise on NICU Graduates' Cochlear Functions.

AIM: To evaluate the adverse effects of noise on hearing. Methods: Thirty-two infants that had been admitted to neonatal intensive care unit (NICU) and 25 healthy controls were included in this study. Noise levels were recorded continously during the hospitalization period. Results: All healthy controls passed the hearing screening tests before discharge and on the sixth-month follow up. Hospitalized infants had lower "Distortion Product Auto Acoustic Emission Signal Noise Ratio" (DPOAE SNR) amplitudes (dB) at five frequencies (1001, 1501, 3003, 4004, 6006 Hz in both ears). DPOAE fail rates at 1001 Hz and 1501 Hz were higher than in hospitalized infants (81.8% and 50.0% vs 20.0% and 4.0%). Infants who failed the test at 1001 and 1501 Hz were exposed to noise above the recommended maximum level for longer periods of time. Conclusion: Hearing tests performed at sixth-months of life were adversely affected in NICU graduates.

Evaluation of Efferent Auditory System and Hearing Quality in Parkinson's Disease: Is the Difficulty in Speech Understanding in Complex Listening Conditions Related to Neural Degeneration or Aging?

Purpose The effects of neurological diseases on the auditory system have been a notable issue for investigators because the auditory pathway is closely associated with neural systems. The purposes of this study are to evaluate the efferent auditory system function and hearing quality in Parkinson's disease (PD) and to compare the findings with age-matched individuals without PD to present a perspective on aging. Method The study included 35 individuals with PD (mean age of 48.50 ± 8.00 years) and 35 normal-hearing peers (mean age of 49 ± 10 years). The following tests were administered for all participants: the first section of the Speech, Spatial and Qualities of Hearing Scale; pure-tone audiometry, speech audiometry, tympanometry, and acoustic reflexes; and distortion product otoacoustic emissions (DPOAEs) and contralateral suppression of DPOAEs. SPSS Version 25 was used for statistical analyses, and values of p < .05 were considered statistically significant. Results There were no statistically significant differences in the pure-tone audiometry thresholds and DPOAE responses between the individuals with PD and their normal-hearing peers (p = .732). However, statistically significant differences were found between the groups in suppression levels of DPOAEs and hearing quality (p < .05). In addition, a statistically significant and positive correlation was found between the amount of suppression at some frequencies and the Speech, Spatial and Qualities of Hearing Scale scores. Conclusions This study indicates that medial olivocochlear efferent system function and the hearing quality of individuals with PD were affected adversely due to the results of PD pathophysiology on the hearing system. For optimal intervention and follow-up, tasks related to hearing quality in daily life can also be added to therapies for PD.

Reliability and validity of the Turkish version of the vestibular rehabilitation benefit questionnaire

Background/aims: Vestibular rehabilitation has an important role in the reduction of symptoms and in the recovery of patients in peripheral vestibular pathologies. Objective and subjective vestibular assessment tools are needed to assess vestibular rehabilitation effectiveness. The aims of the study were to develop the Turkish version of the internationally used Vestibular Rehabilitation Benefit Questionnaire (VRBQ) measure and to demonstrate the reliability and validity properties of the Turkish version in patients with peripheral vestibular hypofunction (PVH). Materials and methods: 110 patients with unilateral PVH were included. For the analysis of test-retest reliability, Turkish version of VRBQ developed by translation-back translation method was applied to patients on the day of admission and the day after admission. To assess validity, patients were also evaluated with the VRBQ, Dizziness Handicap Inventory (DHI), Vertigo Symptom Scale-Short Form (VSS-SF), Vertigo Dizziness Imbalance (VDI) Questionnaire. Results: The VRBQ showed moderate to excellent internal consistency in total score and subscales scores (VRBQ-total Cronbach's α = 0.91; dizziness α = 0.81; anxiety α = 0.68; motion-provoked dizziness α = 0.89; aypmtoms α = 0.88; health-related quality of life α = 0.87). In the test-retest reliability of VRBQ-total score was excellent (ICC = 0.94). The dizziness, the anxiety, the motion-provoked dizziness, symptoms and the health-related quality of life domains' ICC were found respectively 0.90, 0.89, 0.84, 0.90, and 0.92. The construct validity of the VRBQ was determined. The VRBQ total was correlated with all parameters (r: 0.308 to ­0.699, P < 0.05). The highest positive correlation was found between VRBQ total and DHI-functional (r: 0.680). The highest negative correlation was found between VRBQ total and VDI-total (r: ­0.699). Conclusion: The results suggest that the Turkish version of the VRBQ is reliable and valid for evaluating the vestibular rehabilitation results.

Evaluation of medial olivocochlear efferent system and hearing loss in patients with primary Sjögren's syndrome

Background/aim: Autoimmune diseases are a remarkable issue for researchers due to their adverse effects on the auditory system, but for primary Sjögren's syndrome (pSS) there is little research on the comprehensive audiological findings in literature. The main objective of this study was to investigate the medial olivocochlear efferent functions of subjects with pSS and to examine the audiological findings. Materials and methods: The study included 36 subjects with pSS and 36 healthy subjects. All the subjects underwent testing including pure tone, speech, and high frequency audiometry; tympanometry and acoustic reflexes; distortion product otoacoustic emissions (DPOAE); and suppression of DPOAE. Results: The hearing thresholds of the pSS group were higher than those of the control at all frequencies (P < 0.001). Minimal to mild sensorineural hearing loss was observed in 52.77% of all the subjects with pSS. Additionally, all of the subjects had type A curve tympanograms, but the static compliances of the pSS group were lower and the acoustic reflex thresholds were higher than in the control (P < 0.001). In suppression levels of DPOAE, no statistically significant difference was found between the groups (P > 0.05). Conclusion: The study indicates that because of obtaining normal suppression levels in DPOAE, the medial olivocochlear efferent system is functional in pSS. However, there is a need for more tests, including auditory brainstem response, to evaluate the afferent auditory system in pSS.

Long-term Average Speech Spectra of Postlingual Cochlear Implant Users.

Long term average speech spectra (LTASS) is a commonly used voice analysis method for different purposes. This method offers an acoustic representation of the language in daily conservations. Results of that method can be altered by the deteriorations in the auditory feedback loop. Hearing losses occurred in the post lingual stage of life have some serious negative effects on the auditory feedback loop. Cochlear implantation may help these patients with regards to auditory feedback loop. Therefore, we aimed to evaluate the LTASS of cochlear implant users whose have a post lingual hearing loss. We assessed the LTASS of 24 cochlear implant users and compared our findings with normal hearing subjects. Our findings revealed that cochlear implant users have similar LTASS findings with normal hearing subjects. We conclude that cochlear implantation helps to the recovery of auditory feedback loop in patients with post lingual hearing losses.

Long term average speech spectra of Turkish.

Long-term average speech spectra (LTASS) are a voice analysis method that is recommended to be used in various areas, such as hearing aid fittings, automatic speaker recognition and voice disorders. Evaluations of LTASS in different languages have been published; however, this analysis has not been conducted for the Turkish. Therefore, the first aim of this study was to conduct this analysis, generate normative values and compare the obtained LTASS curve for the Turkish with those obtained for other languages reported in the literature. To achieve this objective, 40 adults with healthy hearing and speech characteristics were evaluated. Long-term spectral shape is presented in a graphical form, with visual comparisons to other research findings. Spectral differences by gender were analyzed, with statistically significant differences observed between gender groups and overall LTASS. The LTASS curve for the Turkish is similar to those reported for other languages, but there are also observable differences. Use of generated speech signals in hearing aid fitting procedures is recommended.

Management of children with auditory neuropathy spectrum disorder (ANSD) / Tratamento de crianças com espectro da neuropatia auditiva (ENA)

ABSTRACT INTRODUCTION: ANSD is a challenging problem. OBJECTIVE: To present our experience on management of the children with ANSD with respect to clinical data. METHODS: This retrospective study included all children younger than 16 years of age who applied to the department between 2005 and 2013 (with the exception of newborn hearing screening NHS referrals). The data were derived from pure tone, OAEs and ABR tests, and further medical risk factors of the subjects were evaluated. RESULTS: ANSD was recognized in 74 ears of 40 children (B/U: 34/6) among 1952 children with SNHL (2.04%) detected among 9520 applicants to the department (0.42%). The clinical tests revealed that hearing loss greater than 15 dB was present in both ears of 38 cases. The degree of hearing loss was profound in 48% children, severe in 12% children, moderate in 28% children, mild in 10% children and normal in 5% children. ABRs were absent/abnormal in 37/3 ears and CMs were detected in all. Acoustic reflexes were absent in all ears. Rehabilitation was managed by CI and hearing aids in 15 and 23 cases, respectively. FM system was given to two cases displaying normal hearing but poor speech discrimination in noisy environments. CONCLUSION: ANSD is a relatively challenging problem for the audiology departments because of its various clinical features and difficulties in management. Our patients with ANSD most commonly displayed profound hearing loss. The number of overlooked cases may be minimized by performing ABR and OAE in every case referred with the suspicion of hearing loss.

Resumo Introdução: Espectro da neuropatia auditiva ainda é uma condição clínica desafiadora. Objetivo: Apresentar nossa experiência no tratamento de crianças com espectro da neuropatia auditiva em relação aos dados clínicos. Método: Este estudo retrospectivo incluiu crianças menores de 16 anos de idade que deram entrada no departamento entre 2005 e 2013 (com exceção de encaminhamentos para triagem auditiva neonatal). Foram avaliados os dados obtidos a partir dos exames de audiometria tonal, emissões otoacústicas (EOA), potencial evocado auditivo de tronco encefálico (ABR) e outros fatores de risco. Resultados: Das 1.952 crianças com perda auditiva neurossensorial (2,04%) detectadas dentre os 9.520 candidatos que deram entrada no departamento (0,42%), espectro da neuropatia auditiva foi reconhecida em 74 orelhas de 40 crianças (B/U: 34/6). Os testes clínicos revelaram que uma perda auditiva superior a 15 dB estava presente em ambas as orelhas em 38 casos. O grau de perda auditiva das crianças era profundo em 48%, grave em 12%, moderado em 28%, leve em 10%, e normal em 5%. ABR estava ausente/anormal em 37/3 orelhas e microfonia coclear foi detectado em todas as crianças. Reflexos acústicos estavam ausentes em todas as orelhas. A reabilitação foi tratada com implante coclear e aparelhos auditivos em 15 e 23 casos, respectivamente. Um sistema FM foi utilizado em dois casos que apresentavam audição normal, mas discriminação deficiente da fala em ambientes ruidosos. Conclusão: Espectro da neuropatia auditiva é um problema desafiador para os departamentos de audiologia, devido às suas várias características clínicas e dificuldades no tratamento. Em nossos pacientes a perda auditiva profunda foi a mais frequente. O número de casos negligenciados pode ser diminuído com a realização dos exames ABR e EOA em todos os casos encaminhados com suspeita de perda auditiva.

Audiologic evaluations of children with mucopolysaccharidosis / Avaliação audiológica em crianças com mucopolissacaridose

ABSTRACT INTRODUCTION: Mucopolysaccharidosis is a hereditary lysosomal storage disease, which develops due to a deficiency in the enzymes that play a role in the metabolism of glycosaminoglycans (GAG). The incidence of mucopolysaccharidosis is 1/25,000, with autosomal recessive inheritance (except for MPS II). Mucopolysaccharidosis occurs in seven different types, each with a different congenital deficiency of lysosomal enzymes. In mucopolysaccharidosis patients, even though progression of clinical findings is not prominent, the disease advances and causes death at early ages. Facial dysmorphism, growth retardation, mental retardation, and skeletal or joint dysplasia are the most frequently found symptoms in these patients. OBJECTIVE: The purpose of our study is to present the types of hearing loss types and tympanometric findings of patients with mucopolysaccharidosis referred to our clinic with suspicion of hearing loss. METHODS: After otorhinolaryngological examination, 9 patients with different types of mucopolysaccharidosis, underwent to immittance and audiometric evaluations, performed according to their physical and mental abilities, and ages, in order to determine their hearing thresholds. RESULTS: The audiometric findings of the 9 patients followed with mucopolysaccharidosis were reported separately for each case. CONCLUSION: Based on the high frequency of hearing loss in mucopolysaccharidosis patients, early and detailed audiological evaluations are highly desirable. Therefore, regular and systematic multidisciplinary evaluations are very important.

Resumo Introdução: Mucopolissacaridose (MPS) é uma doença hereditária de depósito lisossômico, decorrente da deficiência das enzimas que influenciam o metabolismo dos glicosaminoglicanos (GAGs). A incidência de MPS é de 1/25.000 habitantes, resultante de herança autossômica recessiva (exceto no caso de MPS II). MPS se apresenta na forma de sete tipos diferentes e, em cada tipo, ocorre uma deficiência congênita distinta de enzimas lisossômicas. Embora em pacientes com MPS os achados clínicos não sejam geralmente observados, a doença progride em seu curso natural e costuma levar a óbito pacientes muito jovens. Dismorfismo facial, retardo de crescimento, retardo mental e displasia esquelética ou articular são os sinais e sintomas mais frequentemente observados nesses pacientes. Objetivo: A finalidade do presente estudo foi apresentar os tipos de perda auditiva e os achados timpanométricos de pacientes com MPS encaminhados para nossa clínica com suspeita de perda auditiva. Método: Em seguida ao exame otorrinolaringológico, nove pacientes com diferentes tipos de MPS, foram submetidos a avaliações imitanciométricas e testes audiométricos de acordo com sua faixa etária eficiência física e mental, no intuito de identificar seus limiares auditivos. Resultados: Os achados audiométricos dos nove pacientes acompanhados por MPS foram descritos separadamente para cada caso. Conclusão: Baseado na alta frequência de perda auditiva em pacientes com MPS, avaliação audiológica precoce e detalhada é altamente desejável. Para tanto, é importante que sejam realizados avaliações multidisciplinares periódicas e sistemáticas.