Delayed restoration of maximum speech discrimination scores in patients with idiopathic sudden sensorineural hearing loss.

PubMed

Noguchi, Yoshihiro; Takahashi, Masatoki; Ito, Taku; Fujikawa, Taro; Kawashima, Yoshiyuki; Kitamura, Ken

2016-10-01

To assess possible delayed recovery of the maximum speech discrimination score (SDS) when the audiometric threshold ceases to change. We retrospectively examined 20 patients with idiopathic sudden sensorineural hearing loss (ISSNHL) (gender: 9 males and 11 females, age: 24-71 years). The findings of pure-tone average (PTA), maximum SDS, auditory brainstem responses (ABRs), and tinnitus handicap inventory (THI) were compared among the three periods of 1-3 months, 6-8 months, and 11-13 months after ISSNHL onset. No significant differences were noted in PTA, whereas an increase of greater than or equal to 10% in maximum SDS was recognized in 9 patients (45%) from the period of 1-3 months to the period of 11-13 months. Four of the 9 patients showed 20% or more recovery of maximum SDS. No significant differences were observed in the interpeak latency difference between waves I and V and the interaural latency difference of wave V in ABRs, whereas an improvement in the THI grade was recognized in 11 patients (55%) from the period of 1-3 months to the period of 11-13 months. The present study suggested the incidence of maximum SDS restoration over 1 year after ISSNHL onset. These findings may be because of the effects of auditory plasticity via the central auditory pathway. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Neuro-rehabilitation Approach for Sudden Sensorineural Hearing Loss

PubMed Central

Sekiya, Kenichi; Fukushima, Munehisa; Teismann, Henning; Lagemann, Lothar; Kakigi, Ryusuke; Pantev, Christo; Okamoto, Hidehiko

2016-01-01

Sudden sensorineural hearing loss (SSHL) is characterized by acute, idiopathic hearing loss. The estimated incidence rate is 5-30 cases per 100,000 people per year. The causes of SSHL and the mechanisms underlying SSHL currently remain unknown. Based on several hypotheses such as a circulatory disturbance to the cochlea, viral infection, and autoimmune disease, pharmaco-therapeutic approaches have been applied to treat SSHL patients; however, the efficacy of the standard treatment, corticosteroid therapy, is still under debate. Exposure to intense sounds has been shown to cause permanent damage to the auditory system; however, exposure to a moderate level enriched acoustic environment after noise trauma may reduce hearing impairments. Several neuroimaging studies recently suggested that the onset of SSHL induced maladaptive cortical reorganization in the human auditory cortex, and that the degree of cortical reorganization in the acute SSHL phase negatively correlated with the recovery rate from hearing loss. This article reports the development of a novel neuro-rehabilitation approach for SSHL, "constraint-induced sound therapy (CIST)". The aim of the CIST protocol is to prevent or reduce maladaptive cortical reorganization by using an enriched acoustic environment. The canal of the intact ear of SSHL patients is plugged in order to motivate them to actively use the affected ear and thereby prevent progress of maladaptive cortical reorganization. The affected ear is also exposed to music via a headphone for 6 hr per day during hospitalization. The CIST protocol appears to be a safe, easy, inexpensive, and effective treatment for SSHL. PMID:26863274

Healthy-side dominance of middle- and long-latency neuromagnetic fields in idiopathic sudden sensorineural hearing loss.

PubMed

Li, L P H; Shiao, A S; Chen, L F; Niddam, D M; Chang, S Y; Lien, C F; Lee, S K; Hsieh, J C

2006-08-01

Any lesion along the neural axis may induce a subsequent functional reorganization at the level above. The present study used magnetoencephalography to investigate auditory-evoked magnetic fields [a component of the middle-latency auditory evoked fields peaking at approximately 50 ms (P50m) and a component of the long-latency auditory evoked fields peaking at approximately 100 ms (N100m)] on stimulation of both healthy and affected ears in patients with acute unilateral idiopathic sudden sensorineural hearing loss (ISSNHL) of moderate degree in order to elucidate the functional plasticity of the auditory system. Sixteen right-handed, previously untreated adult patients with acute unilateral left (n = 8) or right (n = 8) ISSNHL of moderate degree were studied. Sixteen right-handed healthy volunteers with normal hearing served as control. Auditory neuromagnetic responses, measured by a whole-head 306-channel neuromagnetometer, were detected by monaural tone stimulation applied to affected and healthy ears, respectively, in different sessions. Intragroup and intergroup interhemispheric differences of peak dipole strengths and latencies of P50m and N100m, respectively, to monaural tones were evaluated. Healthy-side amplitude dominance of both P50m and N100m was found in ISSNHL, i.e. contralateral dominance was preserved on affected-ear stimulation but ipsilateral dominance was seen on healthy-ear stimulation. The phenomena could be attributed to the combined contralateral attenuation and ipsilateral enhancement of P50m and N100m activity in response to healthy-ear stimulation. Our findings confirmed that functional modulation can occur within the first few tens of milliseconds of evoked response at the auditory cortex in ISSNHL. The mechanisms of healthy-side dominance might be ascribed to a functional retune of auditory pathways, i.e. conjoined contralateral inhibition and ipsilateral excitation of the auditory pathway in response to healthy-ear stimulation. The

The relationship between neonatal hyperbilirubinemia and sensorineural hearing loss.

PubMed

Corujo-Santana, Cándido; Falcón-González, Juan Carlos; Borkoski-Barreiro, Silvia Andrea; Pérez-Plasencia, Daniel; Ramos-Macías, Ángel

2015-01-01

Severe jaundice that requires exchange transfusion has become a relatively rare situation today. About 60% of full term neonates and 80% of premature ones will suffer from jaundice within the first week of life. Hyperbilirubinemia at birth is a risk factor associated with hearing loss that is usually further linked to other factors that might have an effect on hearing synergistically. This study aimed to identify the relationship between hyperbilirubinemia at birth as a risk factor for sensorineural hearing loss in children born at Complejo Hospitalario Universitario Insular Materno-Infantil de Gran Canaria, in the 2007-2011 period. This was a retrospective study of 796 newborns that had hyperbilirubinemia at birth, using transient evoked otoacoustic emissions and evoked auditory brainstem response. Hundred eighty-five newborns (23.24%) were referred for evoked auditory brainstem response. Hearing loss was diagnosed for 35 (4.39%): 18 neonates (51.43%) with conductive hearing loss and 17 (48.57%) with sensorineural hearing loss, 3 of which were diagnosed as bilateral profound hearing loss. Half of the children had other risk factors associated, the most frequent being exposure to ototoxic medications. The percentage of children diagnosed with sensorineural hearing loss that suffered hyperbilirubinemia at birth is higher than for the general population. Of those diagnosed, none had levels of indirect bilirubin≥20mg/dl, only 47% had hyperbilirubinemia at birth as a risk factor and 53% had another auditory risk factor associated. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

Sudden onset unilateral sensorineural hearing loss after rabies vaccination.

PubMed

Okhovat, Saleh; Fox, Richard; Magill, Jennifer; Narula, Antony

2015-12-15

A 33-year-old man developed profound sudden onset right-sided hearing loss with tinnitus and vertigo, within 24 h of pretravel rabies vaccination. There was no history of upper respiratory tract infection, systemic illness, ototoxic medication or trauma, and normal otoscopic examination. Pure tone audiograms (PTA) demonstrated right-sided sensorineural hearing loss (thresholds 90-100 dB) and normal left-sided hearing. MRI internal acoustic meatus, viral serology (hepatitis B, C, HIV and cytomegalovirus) and syphilis screen were normal. Positive Epstein-Barr virus IgG, viral capsid IgG and anticochlear antibodies (anti-HSP-70) were noted. Initial treatment involved a course of high-dose oral prednisolone and acyclovir. Repeat PTAs after 12 days of treatment showed a small improvement in hearing thresholds. Salvage intratympanic steroid injections were attempted but failed to improve hearing further. Sudden onset sensorineural hearing loss (SSNHL) is an uncommon but frightening experience for patients. This is the first report of SSNHL following rabies immunisation in an adult. 2015 BMJ Publishing Group Ltd.

Bilateral sudden sensorineural hearing loss as a presenting feature of systemic lupus erythematosus

PubMed Central

Chawki, Sylvain; Aouizerate, Jessie; Trad, Selim; Prinseau, Jacques; Hanslik, Thomas

2016-01-01

Abstract Introduction: Sudden sensorineural hearing loss is an unusual presenting clinical feature of systemic lupus erythematosus. Case report: We report the case of a young woman who was admitted to hospital for sudden sensorineural hearing loss and hemophagocytic syndrome which was attributed to systemic lupus erythematosus on the basis of specific renal involvement, thrombocytopenia, and consistent autoantibodies. Favorable outcome was obtained on high-dose corticosteroids, and the hearing fully recovered. Discussion: Sudden sensorineural hearing loss in systemic lupus erythematosus is seemingly more frequently associated with severe systemic involvement and antiphospholipid antibodies may be present. Although management remains empirical, the high risk of permanent hearing impairment seems to justify emergency treatment with high-dose corticosteroids. When the clinical and laboratory criteria of antiphospholipid syndrome are met, antiplatelets agents or anticoagulation therapy shall be considered. PMID:27603334

Noonan Syndrome: An Underestimated Cause of Severe to Profound Sensorineural Hearing Impairment. Which Clues to Suspect the Diagnosis?

PubMed

Ziegler, Alban; Loundon, Natalie; Jonard, Laurence; Cavé, Hélène; Baujat, Geneviève; Gherbi, Souad; Couloigner, Vincent; Marlin, Sandrine

2017-09-01

To highlight Noonan syndrome as a clinically recognizable cause of severe to profound sensorineural hearing impairment. New clinical cases and review. Patients evaluated for etiological diagnosis by a medical geneticist in a reference center for hearing impairment. Five patients presenting with confirmed Noonan syndrome and profound sensorineural hearing impairment. Diagnostic and review of the literature. Five patients presented with profound sensorineural hearing impairment and molecularly confirmed Noonan syndrome. Sensorineural hearing impairment has been progressive for three patients. Cardiac echography identified pulmonary stenosis in two patients and was normal for the three other patients. Short stature was found in two patients. Mild intellectual disability was found in one patient. Inconspicuous clinical features as facial dysmorphism, cryptorchidism, or easy bruising were of peculiar interest to reach the diagnosis of Noonan syndrome. Profound sensorineural hearing impairment can be the main feature of Noonan syndrome. Associated features are highly variable; thus, detailed medical history and careful physical examination are mandatory to consider the diagnosis in case of a sensorineural hearing impairment.

Early cortical metabolic rearrangement related to clinical data in idiopathic sudden sensorineural hearing loss.

PubMed

Micarelli, Alessandro; Chiaravalloti, Agostino; Viziano, Andrea; Danieli, Roberta; Schillaci, Orazio; Alessandrini, Marco

2017-07-01

Results in studies concerning cortical changes in idiopathic sudden sensorineural hearing loss (ISSNHL) are not homogeneous, in particular due to the different neuroimaging techniques implemented and the diverse stages of ISSNHL studied. Considering the recent advances in state-of-the-art positron emission tomography (PET) cameras, the aim of this study was to gain more insight into the neuroanatomical differences associated with the earliest stages of unilateral ISSNHL and clinical-perceptual performance changes. After an audiological examination including the mean auditory threshold (mean AT), mean speech discrimination score (mean SDS) and Tinnitus Handicap Inventory (THI), 14 right-handed ISSNHL patients underwent brain [ 18 F]fluorodeoxyglucose (FDG)-PET within 72 h of the onset of symptoms. When compared to an homogeneous group of 35 healthy subjects by means of statistical parametric mapping, a relative increase in FDG uptake was found in the right superior and medial frontal gyrus as well as in the right anterior cingulate cortex in ISSNHL patients. Conversely, the same group showed a significant relative decrease in FDG uptake in the right middle temporal, precentral and postcentral gyrus as well as in the left posterior cingulate cortex, left lingual, superior, middle temporal and middle frontal gyrus and in the left insula. Regression analysis showed a positive correlation between mean THI and glucose consumption in the right anterior cingulate cortex and a positive correlation between mean SDS and glucose consumption in the left precentral gyrus. The relative changes in FDG uptake found in these brain regions and the positive correlation with mean SDS and THI scores in ISSNHL could possibly highlight new aspects of cerebral rearrangement, contributing to further explain changes in those functions that support speech recognition during the sudden impairment of unilateral auditory input. Copyright © 2017 Elsevier B.V. All rights reserved.

Acute unilateral sensorineural hearing loss associated with anabolic steroids and polycythaemia: case report.

PubMed

Tikka, T; Mistry, N; Janjua, A

2016-03-01

Unilateral sudden sensorineural hearing loss due to an infarct in the vertebrobasilar system has been widely reported. Most patients have a background of traditional coronary risk factors related to these cerebrovascular episodes. A 32-year-old male, a regular user of anabolic steroids, presented to the emergency department with unilateral sensorineural hearing loss and symptoms suggestive of an infarct of the anterior inferior cerebellar artery but in the absence of risk factors for ischaemic stroke. Magnetic resonance imaging confirmed the presence of infarction in the region supplied by the anterior inferior cerebellar artery. Polycythaemia was found on haematological analysis, which we believe was secondary to the use of anabolic steroids. The patient was commenced on aspirin as per the stroke management protocol. There was resolution of neurological symptomatology six weeks after the episode, but no improvement in hearing. To our knowledge, this is the first case report of unilateral sensorineural hearing loss secondary to the use of anabolic steroids causing polycythaemia. This cause should be considered in the differential diagnosis of patients presenting with sensorineural hearing loss, especially in young males, when no other risk factors can be identified.

Isolated Sensorineural Hearing Loss as a Sequela after Lightning Strike

PubMed Central

Turan, Mahfuz; Kalkan, Ferhat; Bozan, Nazım; Özçalimli, İsa; Zeki Erdem, Mehmet; Yalınkılıç, Abdülaziz; Garca, Mehmet Fatih

2015-01-01

In most of the surviving patients after a lightning strike, audiovestibular abnormalities have been reported. The most frequently reported type of abnormalities is a tympanic membrane perforation with hearing loss and external ear canal burn. However a sensor neural hearing loss and mixed type hearing loss can also occur, but these occur rarely. A nineteen-year-old female patient had, after a lightning strike, serious burns on the left ear, behind the ear, and on the chest and neck. She also had in her left ear 108 dB hearing loss with irregular central perforation and in her right ear 52 dB sensorineural hearing loss. There was no hearing loss before the strike. A hearing aid was recommended for the right ear and good care and follow-up were recommended for the left ear. A lightning strike can cause serious audiological damage. Therefore, it is necessary to make a careful audiovestibular evaluation of the patients. Although there exist rarely healed cases from sensorineural hearing loss after lightning strike in literature, in our case hearing loss occurred bilaterally and then it healed unilaterally. This condition is quite rare in literature. PMID:26161278

Asymmetric hearing loss in a random population of patients with mild to moderate sensorineural hearing loss.

PubMed

Segal, Nili; Shkolnik, Mark; Kochba, Anat; Segal, Avichai; Kraus, Mordechai

2007-01-01

We evaluated the correlation of asymmetric hearing loss, in a random population of patients with mild to moderate sensorineural hearing loss, to several clinical factors such as age, sex, handedness, and noise exposure. We randomly selected, from 8 hearing institutes in Israel, 429 patients with sensorineural hearing loss of at least 30 dB at one frequency and a speech reception threshold not exceeding 30 dB. Patients with middle ear disease or retrocochlear disorders were excluded. The results of audiometric examinations were compared binaurally and in relation to the selected factors. The left ear's hearing threshold level was significantly higher than that of the right ear at all frequencies except 1.0 kHz (p < .05). One hundred fifty patients (35%) had asymmetric hearing loss (more than 10 dB difference between ears). In most of the patients (85%) the binaural difference in hearing threshold level, at any frequency, was less than 20 dB. Age, handedness, and sex were not found to be correlated to asymmetric hearing loss. Noise exposure was found to be correlated to asymmetric hearing loss.

[Subclinical sensorineural hearing loss in female patients with rheumatoid arthritis].

PubMed

Treviño-González, José Luis; Villegas-González, Mario Jesús; Muñoz-Maldonado, Gerardo Enrique; Montero-Cantu, Carlos Alberto; Nava-Zavala, Arnulfo Hernán; Garza-Elizondo, Mario Alberto

2015-01-01

The rheumatoid arthritis is a clinical entity capable to cause hearing impairment that can be diagnosed promptly with high frequencies audiometry. To detect subclinical sensorineural hearing loss in patients with rheumatoid arthritis. Cross-sectional study on patients with rheumatoid arthritis performing high frequency audiometry 125Hz to 16,000Hz and tympanometry. The results were correlated with markers of disease activity and response to therapy. High frequency audiometry was performed in 117 female patients aged from 19 to 65 years. Sensorineural hearing loss was observed at a sensitivity of pure tones from 125 to 8,000 Hz in 43.59%, a tone threshold of 10,000 to 16,000Hz in 94.02% patients in the right ear and in 95.73% in the left ear. Hearing was normal in 8 (6.84%) patients. Hearing loss was observed in 109 (93.16%), and was asymmetric in 36 (30.77%), symmetric in 73 (62.37%), bilateral in 107 (91.45%), unilateral in 2 (1.71%), and no conduction and/or mixed hearing loss was encountered. Eight (6.83%) patients presented vertigo, 24 (20.51%) tinnitus. Tympanogram type A presented in 88.90% in the right ear and 91.46% in the left ear, with 5.98 to 10.25% type As. Stapedius reflex was present in 75.3 to 85.2%. Speech discrimination in the left ear was significantly different (p = 0.02)in the group older than 50 years. No association was found regarding markers of disease activity, but there was an association with the onset of rheumatoid arthritis disease. Patients with rheumatoid arthritis had a high prevalence of sensorineural hearing loss for high and very high frequencies. Copyright © 2015 Academia Mexicana de Cirugía A.C. Published by Masson Doyma México S.A. All rights reserved.

Sensorineural Hearing Impairment and Subclinical Atherosclerosis in Rheumatoid Arthritis Patients Without Traditional Cardiovascular Risk Factors

PubMed Central

MACIAS-REYES, Hector; DURAN-BARRAGAN, Sergio; CARDENAS-CONTRERAS, Cynthia R.; CHAVEZ-MARTIN, Cesar G.; GOMEZ-BAÑUELOS, Eduardo; NAVARRO-HERNANDEZ, Rosa E.; YANOWSKY-GONZALEZ, Carlos O.; GONZALEZ-LOPEZ, Laura; GAMEZ-NAVA, Jorge I.

2016-01-01

Objectives This study aims to evaluate the association of hearing impairment with carotid intima-media thickness and subclinical atherosclerosis in rheumatoid arthritis (RA) patients. Patients and methods A total of 41 RA patients (2 males, 39 females; mean age 46.5±10.2 years; range 20 to 63 years) with no known traditional cardiovascular risk factors were included. Routine clinical and laboratory assessments for RA patients were performed. Pure tone air (250-8000 Hz) and bone conduction (250-6000 Hz) thresholds were obtained, tympanograms and impedance audiometry were conducted. Sensorineural hearing impairment was defined if the average thresholds were ≥25 decibels. Carotid intima-media thickness was assessed and classified with a cut-off point of 0.6 mm. Results Thirteen patients (31.7%) had normal audition, while 28 (68.3%) had hearing impairment. Of these, 22 had bilateral sensorineural hearing impairment. Four patients had conductive hearing impairment (right in three patients and left in one patient). Patients with sensorineural hearing impairment had increased carotid intima-media thickness in the media segment of carotid common artery compared to patients with normal hearing (right ear p=0.007; left ear p=0.075). Thickening of the carotid intima-media thickness was associated with sensorineural hearing impairment in RA patients. Conclusion Rheumatoid arthritis patients should be evaluated by carotid intima-media thickness as a possible contributing factor of hearing impairment in patients without cardiovascular risk factors. PMID:29900940

A new disease: pregnancy-induced sudden sensorineural hearing loss?

PubMed

Hou, Zhi-Qiang; Wang, Qiu-Ju

2011-07-01

Sudden sensorineural hearing loss (SSNHL) may occur during pregnancy, but its prevalence is very low. It is conjectured that SSNHL is closely related to the changes in the cardiovascular system, hematological system, endocrine system, and/or some other systems due to pregnancy. These changes possibly evoke disorders of cochlear circulation or cochlear fluid homeostasis leading to SSNHL. Two SSNHL cases were observed in our clinic, and their clinical features were analyzed. In one patient the SSNHL was likely to be related to the disturbance of cochlear fluid homestasis and in the other it might be induced by some disorders in cochlear circulation. Based on their distinct clinic profiles, we defined a new disease, called "pregnancy-induced sudden sensorineural hearing loss," similar to the definition of "pregnancy-induced hypertension." This study also deepened our understanding of the etiology of SSNHL.

Sudden sensorineural hearing loss: results of intratympanic steroids as salvage treatment.

PubMed

Dispenza, Francesco; De Stefano, Alessandro; Costantino, Claudio; Marchese, Donatella; Riggio, Francesco

2013-01-01

The aim of the present study was to verify the efficacy and the safety of intratympanic dexamethasone to treat sudden sensorineural hearing loss as salvage therapy. A prospective study was conducted on patients affected by idiopathic sudden hearing loss who were treated before with some systemic therapy, but without recovery of the hearing The patients able to undergo the study, but who refused salvage treatment were considered as control group. A solution of Dexamethasone 4 mg/ml was then injected through the posterior-inferior quadrant filling completely the middle ear. The follow-up in the following 6 months included an audiogram every month. The number of patients treated with salvage therapy was 36. The patients who refused treatment were further 10. The salvage treatment was done with a mean delay of 24.3 days from the onset of symptoms. Mean hearing threshold after the onset of sudden hearing loss at PTA was 66.5 dB. After the failed treatment the mean PTA was 59.6 dB. The mean PTA after the intratympanic steroid administration was 46.8 dB, with a mean improvement of 12.8 dB. No hearing change was noted in the 10 patients who refused salvage therapy. The patients that assumed systemic steroid as first therapy showed a better PTA threshold after the salvage intratympanic treatment (p<0.01). A significant difference (p<0.05) of hearing recovery was evidenced between non-smoker patients and those with smoking habit. Our data showed that a salvage treatment with intratympanic dexamethasone should be suggested to all patients who failed the first systemic treatment. The systemic steroid therapy done before the salvage treatment seems to exert a protective role for the inner ear, as shown by our series. On the contrary the smoke habit is a negative prognostic factor in the hearing recovery. Copyright © 2013 Elsevier Inc. All rights reserved.

Risk factors of sensorineural hearing loss in preterm infants.

PubMed

Borradori, C; Fawer, C L; Buclin, T; Calame, A

1997-01-01

Among 547 preterm infants of < or = 34 weeks gestation born between 1987 and 1991, 8 children (1.46%) developed severe progressive and bilateral sensorineural hearing loss. Perinatal risk factors of infants with hearing loss were compared with those of two control groups matched for gestation and birth weight and for perinatal complications. Our observations demonstrated an association of hearing loss with a higher incidence of perinatal complications. Ototoxicity appeared closely related to a prolonged administration and higher total dose of ototoxic drugs, particularly aminoglycosides and furosemide. Finally, we strongly recommend to prospectively and regularly perform audiologic assessment in sick preterm children as hearing loss is of delayed onset and in most cases bilateral and severe.

Speech recognition in individuals with sensorineural hearing loss.

PubMed

de Andrade, Adriana Neves; Iorio, Maria Cecilia Martinelli; Gil, Daniela

2016-01-01

Hearing loss can negatively influence the communication performance of individuals, who should be evaluated with suitable material and in situations of listening close to those found in everyday life. To analyze and compare the performance of patients with mild-to-moderate sensorineural hearing loss in speech recognition tests carried out in silence and with noise, according to the variables ear (right and left) and type of stimulus presentation. The study included 19 right-handed individuals with mild-to-moderate symmetrical bilateral sensorineural hearing loss, submitted to the speech recognition test with words in different modalities and speech test with white noise and pictures. There was no significant difference between right and left ears in any of the tests. The mean number of correct responses in the speech recognition test with pictures, live voice, and recorded monosyllables was 97.1%, 85.9%, and 76.1%, respectively, whereas after the introduction of noise, the performance decreased to 72.6% accuracy. The best performances in the Speech Recognition Percentage Index were obtained using monosyllabic stimuli, represented by pictures presented in silence, with no significant differences between the right and left ears. After the introduction of competitive noise, there was a decrease in individuals' performance. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

[The algorithm for the medical maintenance of the aircraft personnel suffering from chronic sensorineural impairment of hearing].

PubMed

Pankova, V B; Skryabina, L Yu; Barkhatova, O A

2016-01-01

The present study was designed to systematize the causes underlying the development of chronic sensorineural impairment of hearing in the aircraft personnel engaged in commercial aviation of the Russian Federation. A detailed clinical and audiological picture of chronic sensorineural loss of hearing in the aircraft personnel is presented with special reference to the criteria accepted in the civil aviationfor the evaluation of professional suitability and occupational selection in terms of hearing conditions. The study has demonstrated the paramount importance of the aviation medical expertise for the flight safety control in civil aviation. We analyzed the results of the audiological examination of the aircraft personnel suffering from chronic sensorineural impairment of hearing and proposed the algorithm for the rehabilitation of such subjects taking into consideration the stage of the chronic process.

Using Standardized Psychometric Tests to Identify Learning Disabilities in Students with Sensorineural Hearing Impairments.

ERIC Educational Resources Information Center

Sikora, Darryn M.; Plapinger, Donald S.

1994-01-01

The use of standardized psychoeducational diagnostic instruments to identify learning disabilities was evaluated with 19 students (ages 7 to 13) with sensorineural hearing impairments. Students with hearing impairment were found to demonstrate learning disabilities with a frequency similar to that found in students with normal hearing, suggesting…

Profound Bilateral Sensorineural Hearing Loss in Nigerian Children: Any Shift in Etiology?

ERIC Educational Resources Information Center

Dunmade, A. D.; Segun-Busari, S.; Olajide, T. G.; Ologe, F. E.

2007-01-01

Deafness, profound hearing loss, is a global problem. However, the causes of, attitudes toward, and management options for deafness differ considerably from region to region. This study seeks to identify the present causes of profound sensorineural hearing loss in Nigeria, which in our environment is almost synonymous to a life sentence of silence…

The Use of Standardized Test Batteries in Assessing the Skill Development of Children with Mild-to-Moderate Sensorineural Hearing Loss.

ERIC Educational Resources Information Center

Plapinger, Donald S.; Sikora, Darryn M.

1995-01-01

This study of 12 children (ages 7-13) with mild to moderate bilateral sensorineural hearing loss found that psychoeducational diagnostic tests standardized on students with normal hearing may be used with confidence to assess both cognitive and academic levels of functioning in students with sensorineural hearing loss. (Author/JDD)

High-frequency toneburst-evoked ABR latency-intensity functions in sensorineural hearing-impaired humans.

PubMed

Fausti, S A; Olson, D J; Frey, R H; Henry, J A; Schaffer, H I; Phillips, D S

1995-01-01

The latency-intensity functions (LIFs) of ABRs elicited by high-frequency (8, 10, 12, and 14 kHz) toneburst stimuli were evaluated in 20 subjects with confirmed 'moderate' high-frequency sensorineural hearing loss. Wave V results from clicks and tonebursts revealed all intra- and intersession data to be reliable (p > 0.05). Linear regression curves were highly significant (p < or = 0.0001), indicating linear relationships for all stimuli analyzed. Comparisons between the linear regression curves from a previously reported normal-hearing subject group and this sensorineural hearing-impaired group showed no significant differences. This study demonstrated that tonebursts at 8, 10, and 12 kHz evoked ABRs which decreased in latency as a function of increasing intensity and that these LIFs were consistent and orderly (14 kHz was not determinable). These results will contribute information to facilitate the establishment of change criteria used to predict change in hearing during treatment with ototoxic medications.

Acute otitis media associated bilateral sudden hearing loss: case report and literature review.

PubMed

Smith, A; Gutteridge, I; Elliott, D; Cronin, M

2017-07-01

Sudden sensorineural hearing loss is a rare otological condition with potential for dire outcomes including permanent hearing loss. Although the majority of cases are deemed idiopathic, bilateral sudden sensorineural hearing loss represents a rare subset typically related to systemic conditions, with higher morbidity and mortality. A controversial association with acute otitis media has been reported, with few bilateral cases published in the literature. A very rare case of bilateral sudden sensorineural hearing loss associated with acute otitis media is described, with a review of the literature. The limited evidence available suggests that acute otitis media with tinnitus and/or bacterial pathology may have an increased risk of sudden sensorineural hearing loss, which is consistent with the case described. Although there is no sufficiently powered published evidence to provide definitive treatment guidelines, the literature reviewed suggests that early myringotomy and antibiotics may greatly improve treatment outcomes.

Improvements in speech understanding with wireless binaural broadband digital hearing instruments in adults with sensorineural hearing loss.

PubMed

Kreisman, Brian M; Mazevski, Annette G; Schum, Donald J; Sockalingam, Ravichandran

2010-03-01

This investigation examined whether speech intelligibility in noise can be improved using a new, binaural broadband hearing instrument system. Participants were 36 adults with symmetrical, sensorineural hearing loss (18 experienced hearing instrument users and 18 without prior experience). Participants were fit binaurally in a planned comparison, randomized crossover design study with binaural broadband hearing instruments and advanced digital hearing instruments. Following an adjustment period with each device, participants underwent two speech-in-noise tests: the QuickSIN and the Hearing in Noise Test (HINT). Results suggested significantly better performance on the QuickSIN and the HINT measures with the binaural broadband hearing instruments, when compared with the advanced digital hearing instruments and unaided, across and within all noise conditions.

Distribution of HLA-A, -B and -DRB1 alleles in patients with sudden sensorineural hearing loss.

PubMed

Yeo, S W; Chang, K H; Suh, B D; Kim, T G; Han, H

2000-09-01

This study was performed to investigate the association between human leukocyte antigen (HLA) and susceptibility to sudden sensorineural hearing loss in the Korean population. HLA-A and HLA-B typing using a standard microlymphocytotoxicity technique and HLA-DRB1 genotyping were performed in 35 patients with sudden sensorineural hearing loss and in 206 healthy controls. Prednisone (usual dose 60 mg/day) was administered for 6 days and tapered for an additional 4-6 days. Both initial hearing levels at the onset of deafness and final hearing levels after treatment were examined and evaluated for association with HLA alleles. The frequency of HLA-DRB1*14 was increased in patients with sudden sensorineural hearing loss compared with controls (relative risk [RR] = 2.7, p = 0.016). The frequencies of HLA-A2, -A31, -B52, -B61, -DRB1*04, -DRB1*11 and -DRB1*12 were slightly higher than in the controls, but did not reach statistical significance. When an association between the treatment results and HLA alleles was also evaluated, the frequency of HLA-DRB1*04 was found to be increased in the patients who did not respond to steroid treatment compared with both patients who responded well to steroid (50%, vs 16%, p = 0.034) and controls (RR = 3.0, p = 0.046). These results suggest that there is an association between HLA-DRB1*14 and disease susceptibility and that the presence of HLA-DRB1*04 may be an useful marker for predicting a poor prognosis in Korean patients with sudden sensorineural hearing loss.

The evidence base for the application of contralateral bone anchored hearing aids in acquired unilateral sensorineural hearing loss in adults.

PubMed

Baguley, D M; Bird, J; Humphriss, R L; Prevost, A T

2006-02-01

. Acquired unilateral sensorineural hearing loss reduces the ability to localize sounds and to discriminate in background noise. . Four controlled trials attempt to determine the benefit of contralateral bone anchored hearing aids over contralateral routing of signal (CROS) hearing aids and over the unaided condition. All found no significant improvement in auditory localization with either aid. Speech discrimination in noise and subjective questionnaire measures of auditory abilities showed an advantage for bone anchored hearing aid (BAHA) > CROS > unaided conditions. . All four studies have material shortfalls: (i) the BAHA was always trialled after the CROS aid; (ii) CROS aids were only trialled for 4 weeks; (iii) none used any measure of hearing handicap when selecting subjects; (iv) two studies have a bias in terms of patient selection; (v) all studies were underpowered (vi) double reporting of patients occurred. . There is a paucity of evidence to support the efficacy of BAHA in the treatment of acquired unilateral sensorineural hearing loss. Clinicians should proceed with caution and perhaps await a larger randomized trial. . It is perhaps only appropriate to insert a BAHA peg at the time of vestibular schwanoma tumour excision in patients with good preoperative hearing, as their hearing handicap increases most.

Randomised, double-blinded, placebo-controlled, clinical trial of ozone therapy as treatment of sudden sensorineural hearing loss.

PubMed

Ragab, A; Shreef, E; Behiry, E; Zalat, S; Noaman, M

2009-01-01

To investigate the safety and efficacy of ozone therapy in adult patients with sudden sensorineural hearing loss. Prospective, randomised, double-blinded, placebo-controlled, parallel group, clinical trial. Forty-five adult patients presented with sudden sensorineural hearing loss, and were randomly allocated to receive either placebo (15 patients) or ozone therapy (auto-haemotherapy; 30 patients). For the latter treatment, 100 ml of the patient's blood was treated immediately with a 1:1 volume, gaseous mixture of oxygen and ozone (from an ozone generator) and re-injected into the patient by intravenous infusion. Treatments were administered twice weekly for 10 sessions. The following data were recorded: pre- and post-treatment mean hearing gains; air and bone pure tone averages; speech reception thresholds; speech discrimination scores; and subjective recovery rates. Significant recovery was observed in 23 patients (77 per cent) receiving ozone treatment, compared with six (40 per cent) patients receiving placebo (p < 0.05). Mean hearing gains, pure tone averages, speech reception thresholds and subjective recovery rates were significantly better in ozone-treated patients compared with placebo-treated patients (p < 0.05). Ozone therapy is a significant modality for treatment of sudden sensorineural hearing loss; no complications were observed.

Evaluation of very low birth weight (≤ 1,500 g) as a risk indicator for sensorineural hearing loss.

PubMed

Borkoski-Barreiro, Silvia A; Falcón-González, Juan C; Limiñana-Cañal, José M; Ramos-Macías, Angel

2013-01-01

Hearing plays an essential role in the acquisition, development and maintenance of the properties of the speech and language. Birth weight is an indicator of biological maturation of the newborn. Premature newborns with very low birth weight (VLBW<1,500 g) constitute a group with the highest risk of sensorineural hearing loss. Our objective was to ascertain the degree of hearing loss, sensorineural hearing loss and presence of the association to other risk factors for hearing loss in VLBW infants included in the Universal Hearing Loss Screening Programme at the University Mother-Child Hospital of Gran Canaria (Spain) in the 2007-2010 period. This was a retrospective study of 364 infants with VLBW, measured by transient evoked otoacoustic emissions and auditory brainstem response. There were 112 newborn (30.8%) referred for auditory brainstem response. A diagnosis of hearing loss was given to 22 newborns (2.2%), 14 had conductive hearing loss and 8, sensorineural hearing loss (SNHL), of which 2 had bilateral profound hearing loss. The VLBW newborn presented the association to another risk factor in more than a quarter of the sample studied. All those diagnosed with SNHL were premature. The percentage of VLBW newborns diagnosed with hearing loss is higher than expected in the general population. All those diagnosed with SNHL were premature and presented one or 2 hearing risk factors associated with VLBW. Copyright © 2013 Elsevier España, S.L. All rights reserved.

Effects of sensorineural hearing loss on temporal coding of narrowband and broadband signals in the auditory periphery

PubMed Central

Henry, Kenneth S.; Heinz, Michael G.

2013-01-01

People with sensorineural hearing loss have substantial difficulty understanding speech under degraded listening conditions. Behavioral studies suggest that this difficulty may be caused by changes in auditory processing of the rapidly-varying temporal fine structure (TFS) of acoustic signals. In this paper, we review the presently known effects of sensorineural hearing loss on processing of TFS and slower envelope modulations in the peripheral auditory system of mammals. Cochlear damage has relatively subtle effects on phase locking by auditory-nerve fibers to the temporal structure of narrowband signals under quiet conditions. In background noise, however, sensorineural loss does substantially reduce phase locking to the TFS of pure-tone stimuli. For auditory processing of broadband stimuli, sensorineural hearing loss has been shown to severely alter the neural representation of temporal information along the tonotopic axis of the cochlea. Notably, auditory-nerve fibers innervating the high-frequency part of the cochlea grow increasingly responsive to low-frequency TFS information and less responsive to temporal information near their characteristic frequency (CF). Cochlear damage also increases the correlation of the response to TFS across fibers of varying CF, decreases the traveling-wave delay between TFS responses of fibers with different CFs, and can increase the range of temporal modulation frequencies encoded in the periphery for broadband sounds. Weaker neural coding of temporal structure in background noise and degraded coding of broadband signals along the tonotopic axis of the cochlea are expected to contribute considerably to speech perception problems in people with sensorineural hearing loss. PMID:23376018

[Combined treatment supported by piracetam and/or acyclovir in idiopathic sudden sensorineural hearing loss: experience with 81 cases].

PubMed

Karakurt, Süleyman Emre; Ozkul, Mehmet Doğan; Cukurova, Ibrahim; Demirhan, Erhan; Yiğitbaşi, Orhan Gazi

2009-01-01

To investigate the efficiency of piracetam and acyclovir in treating sudden hearing loss. Eightyone patients (44 males, 37 females; mean age 40.4 year; range 18 to 72 years) who had treatment between January 2002 and December 2006 with diagnosis of idiopathic sudden hearing loss were evaluated retrospectively. These patients were divided into four groups according to the treatment they received. The patients who had combined treatment constituted the first group; those who had combined treatment and piracetam the second; those who had combined treatment and acyclovir the third; those who had combined treatment, acyclovir, and piracetam the fourth group. For the four treatment groups, in the pre-and post-treatment (10th day) evaluation of the treatment efficiency made by calculation of the hearing thresholds in 250-8000 Hz frequencies, no significant difference between the groups was determined (p>0.05). No additional benefit was obtained with acyclovir and piracetam in treatment.

Static and dynamic balance of children and adolescents with sensorineural hearing loss.

PubMed

Melo, Renato de Souza; Marinho, Sônia Elvira Dos Santos; Freire, Maryelly Evelly Araújo; Souza, Robson Arruda; Damasceno, Hélio Anderson Melo; Raposo, Maria Cristina Falcão

2017-01-01

To assess the static and dynamic balance performance of students with normal hearing and with sensorineural hearing loss. A cross-sectional study assessing 96 students, 48 with normal hearing and 48 with sensorineural hearing loss of both sexes, aged 7 and 18 years. To evaluate static balance, Romberg, Romberg-Barré and Fournier tests were used; and for the dynamic balance, we applied the Unterberger test. Hearing loss students showed more changes in static and dynamic balance as compared to normal hearing, in all tests used (p<0.001). The same difference was found when subjects were grouped by sex. For females, Romberg, Romberg-Barré, Fournier and Unterberger test p values were, respectively, p=0.004, p<0.001, p<0.001 and p=0.023; for males, the p values were p=0.009, p<0.001, p<0.001 and p=0.002, respectively. The same difference was observed when students were classified by age. For 7 to 10 years old students, the p values for Romberg, Romberg-Barré and Fournier tests were, respectively, p=0.007, p<0.001 and p=0.001; for those aged 11 and 14 years, the p values for Romberg, Romberg-Barré, Fournier and Unterberger tests were p=0.002, p<0.001, p<0.001 and p=0.015, respectively; and for those aged 15 and 18 years, the p values for Romberg-Barré, Fournier and Unterberger tests were, respectively, p=0.037, p<0.001 and p=0.037. Hearing-loss students showed more changes in static and dynamic balance comparing to normal hearing of same sex and age groups.

Cochlear implantation for severe sensorineural hearing loss caused by lightning.

PubMed

Myung, Nam-Suk; Lee, Il-Woo; Goh, Eui-Kyung; Kong, Soo-Keun

2012-01-01

Lightning strike can produce an array of clinical symptoms and injuries. It may damage multiple organs and cause auditory injuries ranging from transient hearing loss and vertigo to complete disruption of the auditory system. Tympanic-membrane rupture is relatively common in patients with lightning injury. The exact pathogenetic mechanisms of auditory lesions in lightning survivors have not been fully elucidated. We report the case of a 45-year-old woman with bilateral profound sensorineural hearing loss caused by a lightning strike, who was successfully rehabilitated after a cochlear implantation. Copyright © 2012 Elsevier Inc. All rights reserved.

In vivo imaging of saccular hydrops in humans reflects sensorineural hearing loss rather than Meniere's disease symptoms.

PubMed

Attyé, Arnaud; Eliezer, Michael; Medici, Maud; Tropres, Irène; Dumas, Georges; Krainik, Alexandre; Schmerber, Sébastien

2018-07-01

A case-controlled imaging study demonstrated that saccular hydrops was specific to Meniere's disease (MD), but only present in a subset of patients. Here, we compared patients with definite MD, vertigo and sensorineural hearing loss (SNHL) to elucidate the relationship between saccular hydrops and extent of SNHL. In this prospective study, we performed 3D-FLAIR sequences between 4.5 and 5.5 h after contrast media injection in patients with MD (n=20), SNHL (n=20), vertigo (n=20) and 30 healthy subjects. Two radiologists independently graded saccular hydrops. ROC analysis was performed to determine the hearing loss threshold to differentiate patients with saccular hydrops. Saccular hydrops was found in 11 of 20 MD patients, 10 of 20 SNHL patients and in none of the vertigo patients and healthy subjects. In SNHL patients, 45 dB was the threshold above which there was a significant association with saccular hydrops, with sensitivity of 100 % and specificity of 90 %. In MD patients, 40 dB was the threshold above which there was a significant association with saccular hydrops, with sensitivity of 100 % and specificity of 44 %. Our results indicate saccular hydrops as a feature of worse than moderate SNHL rather than MD itself. • MRI helps clinicians to assess patients with isolated low-tone sensorineural hearing loss. • Saccular hydrops correlates with sensorineural hearing loss at levels above 40 dB. • Vertigo patients without sensorineural hearing loss do not have saccular hydrops. • Saccular hydrops is described in patients without clinical diagnosis of Meniere's disease.

Diffusion Tensor Imaging of Central Auditory Pathways in Patients with Sensorineural Hearing Loss: A Systematic Review.

PubMed

Tarabichi, Osama; Kozin, Elliott D; Kanumuri, Vivek V; Barber, Samuel; Ghosh, Satra; Sitek, Kevin R; Reinshagen, Katherine; Herrmann, Barbara; Remenschneider, Aaron K; Lee, Daniel J

2018-03-01

Objective The radiologic evaluation of patients with hearing loss includes computed tomography and magnetic resonance imaging (MRI) to highlight temporal bone and cochlear nerve anatomy. The central auditory pathways are often not studied for routine clinical evaluation. Diffusion tensor imaging (DTI) is an emerging MRI-based modality that can reveal microstructural changes in white matter. In this systematic review, we summarize the value of DTI in the detection of structural changes of the central auditory pathways in patients with sensorineural hearing loss. Data Sources PubMed, Embase, and Cochrane. Review Methods We used the Preferred Reporting Items for Systematic Reviews and Meta-Analysis statement checklist for study design. All studies that included at least 1 sensorineural hearing loss patient with DTI outcome data were included. Results After inclusion and exclusion criteria were met, 20 articles were analyzed. Patients with bilateral hearing loss comprised 60.8% of all subjects. Patients with unilateral or progressive hearing loss and tinnitus made up the remaining studies. The auditory cortex and inferior colliculus (IC) were the most commonly studied regions using DTI, and most cases were found to have changes in diffusion metrics, such as fractional anisotropy, compared to normal hearing controls. Detectable changes in other auditory regions were reported, but there was a higher degree of variability. Conclusion White matter changes based on DTI metrics can be seen in patients with sensorineural hearing loss, but studies are few in number with modest sample sizes. Further standardization of DTI using a prospective study design with larger sample sizes is needed.

The efficacy and safety of systemic injection of Ginkgo biloba extract, EGb761, in idiopathic sudden sensorineural hearing loss: a randomized placebo-controlled clinical trial.

PubMed

Koo, Ja-Won; Chang, Mun Young; Yun, Sung-Cheol; Kim, Tae Su; Kong, Soo-Keun; Chung, Jong Woo; Goh, Eui-Kyung

2016-09-01

Steroids are currently the most frequently accepted agents for idiopathic sudden sensorineural hearing loss (ISSNHL). However, the therapeutic effect of steroids is not always satisfactory. In this pilot study, we evaluated whether systemic treatment with Ginkgo biloba extract (EGb761) has an additive therapeutic effect in patients receiving a systemic steroid due to ISSNHL. A multicenter, randomized, double-blind clinical trial was performed. Fifty-six patients with ISSNHL were allocated to either EGb761 or placebo. In both groups, methylprednisolone was administered for 14 days. EGb761 was infused intravenously for 5 days in the EGb761 group, while the same amount of normal saline was infused in the placebo group. For the efficacy evaluation, pure-tone audiometry, speech audiometry, tinnitus handicap inventory (THI) and short form-36 health (SF-36) survey outcomes were obtained before administration and on days 3, 5, 14 and 28 of administration. Twenty-four patients in each group completed the study protocol. There was no difference in hearing loss between the two groups before treatment. At day 28, air conduction threshold values in the placebo and EGb761 groups were 34.63 ± 28.90 and 23.84 ± 25.42 dB, respectively (p = 0.082). Speech discrimination scores in the placebo and EGb761 groups were 69.17 ± 40.89 and 87.48 ± 28.65 %, respectively (p = 0.050). THI and SF-36 scores in the placebo and EGb761 groups were similar. Although a combination of steroid and EGb761 for initial treatment did not show better pure tone threshold, compared with steroid alone, speech discrimination was significantly improved in combination therapy. Further studies will be needed to know if addition of EGb761 actually improves the outcome of ISSNHL treatment.

Streptococcus suis meningitis with bilateral sensorineural hearing loss.

PubMed

Huh, Hee Jae; Park, Kyoung-Jin; Jang, Ja-Hyun; Lee, Mina; Lee, Jang Ho; Ahn, Yoon Hee; Kang, Cheol-In; Ki, Chang-Seok; Lee, Nam Yong

2011-07-01

Streptococcus suis infection is an emerging zoonosis in Asia. The most common disease manifestation is meningitis, which is often associated with hearing loss and cochleovestibular signs. S. suis infection in humans mainly occurs among risk groups that have frequent exposure to pigs or raw pork. Here, we report a case of S. suis meningitis in a 67-yr-old pig carcass handler, who presented with dizziness and sensorineural hearing loss followed by headaches. Gram-positive diplococci were isolated from cerebrospinal fluid (CSF) and blood cultures and showed gray-white colonies with α-hemolysis. S. suis was identified from CSF and blood cultures by using a Vitek 2 system (bioMérieux, France), API 20 STREP (bioMérieux), and performing 16S rRNA and tuf gene sequencing. Even after receiving antibiotic treatment, patients with S. suis infection frequently show complications such as hearing impairment and vestibular dysfunction. To the best of our knowledge, this is the first case of S. suis meningitis in Korea. Prevention through public health surveillance is recommended, especially for individuals who have occupational exposures to swine and raw pork.

Perception of dissonance by people with normal hearing and sensorineural hearing loss

NASA Astrophysics Data System (ADS)

Tufts, Jennifer B.; Molis, Michelle R.; Leek, Marjorie R.

2005-08-01

The purpose of this study was to determine whether the perceived sensory dissonance of pairs of pure tones (PT dyads) or pairs of harmonic complex tones (HC dyads) is altered due to sensorineural hearing loss. Four normal-hearing (NH) and four hearing-impaired (HI) listeners judged the sensory dissonance of PT dyads geometrically centered at 500 and 2000 Hz, and of HC dyads with fundamental frequencies geometrically centered at 500 Hz. The frequency separation of the members of the dyads varied from 0 Hz to just over an octave. In addition, frequency selectivity was assessed at 500 and 2000 Hz for each listener. Maximum dissonance was perceived at frequency separations smaller than the auditory filter bandwidth for both groups of listners, but maximum dissonance for HI listeners occurred at a greater proportion of their bandwidths at 500 Hz than at 2000 Hz. Further, their auditory filter bandwidths at 500 Hz were significantly wider than those of the NH listeners. For both the PT and HC dyads, curves displaying dissonance as a function of frequency separation were more compressed for the HI listeners, possibly reflecting less contrast between their perceptions of consonance and dissonance compared with the NH listeners.

Language Development and Impairment in Children with Mild to Moderate Sensorineural Hearing Loss

ERIC Educational Resources Information Center

Halliday, Lorna F.; Tuomainen, Outi; Rosen, Stuart

2017-01-01

Purpose: The goal of this study was to examine language development and factors related to language impairments in children with mild to moderate sensorineural hearing loss (MMHL). Method: Ninety children, aged 8-16 years (46 children with MMHL; 44 aged-matched controls), were administered a battery of standardized language assessments, including…

The place of hyperbaric oxygen therapy and ozone therapy in sudden hearing loss.

PubMed

Ergun Taşdöven, Gülin; Derin, Alper Tunga; Yaprak, Neslihan; Özçağlar, Hasan Ümit

It is difficult to evaluate the effect of drugs clinically used for idiopathic sudden sensorineural hearing loss, mainly because its underlying mechanism remains unknown. This study assessed the efficacy of hyperbaric oxygen therapy or ozone therapy in the treatment of idiopathic sudden sensorineural hearing loss, when either therapy was included with steroid treatment. A retrospective analysis examined 106 patients with idiopathic sudden sensorineural hearing loss seen between January 2010 and June 2012. Those with an identified etiology were excluded. The patients were divided into three treatment groups: oral steroid only (n=65), oral steroid+hyperbaric oxygen (n=26), and oral steroid+ozone (n=17). Treatment success was assessed using Siegel criteria and mean gains using pre- and post-treatment audiograms. The highest response rate to treatment was observed in the oral steroid+ozone therapy group (82.4%), followed by the oral steroid+hyperbaric oxygen (61.5%), and oral steroid groups (50.8%). There were no significant differences in the response to treatment between the oral steroid and oral steroid+hyperbaric oxygen groups (p<0.355). The oral steroid+ozone group showed a significantly higher response rate to treatment than the oral steroid group (p=0.019). There were no significant differences between the oral steroid+hyperbaric oxygen and oral steroid+ozone groups (p=0.146). The efficiency of steroid treatment in patients with severe hearing loss was low. It was statistically ascertained that adding hyperbaric oxygen or ozone therapy to the treatment contributed significantly to treatment success. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

Secondhand smoke and sensorineural hearing loss in adolescents.

PubMed

Lalwani, Anil K; Liu, Ying-Hua; Weitzman, Michael

2011-07-01

To investigate the hypothesis that second-hand smoke (SHS) exposure is associated with sensorineural hearing loss (SNHL) in adolescents. A complex, multistage, stratified geographic area design for collecting representative data from the noninstitutionalized US population. Cross-sectional data from National Health and Nutrition Examination Survey (2005-2006) were available for 1533 participants 12 to 19 years of age who underwent audiometric testing, had serum cotinine levels available, and were not actively smoking. SNHL was defined as an average pure-tone level greater than 15 dB for 0.5, 1, and 2 kHz (low frequency) and 3, 4, 6, and 8 kHz (high frequency). Secondhand smoke exposure, as assessed by serum cotinine levels, was associated with elevated pure-tone hearing thresholds at 2, 3, and 4 kHz, a higher rate of unilateral low-frequency SNHL (11.8% vs 7.5%; P < .04), and a 1.83-fold increased risk of unilateral low-frequency SNHL in multivariate analyses (95% confidence interval, 1.08-3.41). The prevalence of SNHL was directly related to level of SHS exposure as reflected by serum cotinine levels. In addition, nearly 82% of adolescents with SNHL did not recognize hearing difficulties. Secondhand smoke is associated with elevated pure-tone thresholds and an increased prevalence of low-frequency SNHL that is directly related to level of exposure, and most affected individuals are unaware of the hearing loss. Thus, adolescents exposed to SHS may need to be closely monitored for early hearing loss with periodic audiologic testing.

Effects of sensorineural hearing loss on visually guided attention in a multitalker environment.

PubMed

Best, Virginia; Marrone, Nicole; Mason, Christine R; Kidd, Gerald; Shinn-Cunningham, Barbara G

2009-03-01

This study asked whether or not listeners with sensorineural hearing loss have an impaired ability to use top-down attention to enhance speech intelligibility in the presence of interfering talkers. Listeners were presented with a target string of spoken digits embedded in a mixture of five spatially separated speech streams. The benefit of providing simple visual cues indicating when and/or where the target would occur was measured in listeners with hearing loss, listeners with normal hearing, and a control group of listeners with normal hearing who were tested at a lower target-to-masker ratio to equate their baseline (no cue) performance with the hearing-loss group. All groups received robust benefits from the visual cues. The magnitude of the spatial-cue benefit, however, was significantly smaller in listeners with hearing loss. Results suggest that reduced utility of selective attention for resolving competition between simultaneous sounds contributes to the communication difficulties experienced by listeners with hearing loss in everyday listening situations.

Perspectives for the treatment of sensorineural hearing loss by cellular regeneration of the inner ear.

PubMed

Almeida-Branco, Mario S; Cabrera, Sonia; Lopez-Escamez, Jose A

2015-01-01

Sensorineural hearing loss is a caused by the loss of the cochlear hair cells with the consequent deafferentation of spiral ganglion neurons. Humans do not show endogenous cellular regeneration in the inner ear and there is no exogenous therapy that allows the replacement of the damaged hair cells. Currently, treatment is based on the use of hearing aids and cochlear implants that present different outcomes, some difficulties in auditory discrimination and a limited useful life. More advanced technology is hindered by the functional capacity of the remaining spiral ganglion neurons. The latest advances with stem cell therapy and cellular reprogramming have developed several possibilities to induce endogenous regeneration or stem cell transplantation to replace damaged inner ear hair cells and restore hearing function. With further knowledge of the cellular and molecular biology of the inner ear and its embryonic development, it will be possible to use induced stem cells as in vitro models of disease and as replacement cellular therapy. Investigation in this area is focused on generating cellular therapy with clinical use for the treatment of profound sensorineural hearing loss. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

Cochlear Homocysteine Metabolism at the Crossroad of Nutrition and Sensorineural Hearing Loss.

PubMed

Partearroyo, Teresa; Vallecillo, Néstor; Pajares, María A; Varela-Moreiras, Gregorio; Varela-Nieto, Isabel

2017-01-01

Hearing loss (HL) is one of the most common causes of disability, affecting 360 million people according to the World Health Organization (WHO). HL is most frequently of sensorineural origin, being caused by the irreversible loss of hair cells and/or spiral ganglion neurons. The etiology of sensorineural HL (SNHL) is multifactorial, with genetic and environmental factors such as noise, ototoxic substances and aging playing a role. The nutritional status is central in aging disability, but the interplay between nutrition and SNHL has only recently gained attention. Dietary supplementation could therefore constitute the first step for the prevention and potential repair of hearing damage before it reaches irreversibility. In this context, different epidemiological studies have shown correlations among the nutritional condition, increased total plasma homocysteine (tHcy) and SNHL. Several human genetic rare diseases are also associated with homocysteine (Hcy) metabolism and SNHL confirming this potential link. Accordingly, rodent experimental models have provided the molecular basis to understand the observed effects. Thus, increased tHcy levels and vitamin deficiencies, such as folic acid (FA), have been linked with SNHL, whereas long-term dietary supplementation with omega-3 fatty acids improved Hcy metabolism, cell survival and hearing acuity. Furthermore, pharmacological supplementations with the anti-oxidant fumaric acid that targets Hcy metabolism also improved SNHL. Overall these results strongly suggest that cochlear Hcy metabolism is a key player in the onset and progression of SNHL, opening the way for the design of prospective nutritional therapies.

Cochlear Homocysteine Metabolism at the Crossroad of Nutrition and Sensorineural Hearing Loss

PubMed Central

Partearroyo, Teresa; Vallecillo, Néstor; Pajares, María A.; Varela-Moreiras, Gregorio; Varela-Nieto, Isabel

2017-01-01

Hearing loss (HL) is one of the most common causes of disability, affecting 360 million people according to the World Health Organization (WHO). HL is most frequently of sensorineural origin, being caused by the irreversible loss of hair cells and/or spiral ganglion neurons. The etiology of sensorineural HL (SNHL) is multifactorial, with genetic and environmental factors such as noise, ototoxic substances and aging playing a role. The nutritional status is central in aging disability, but the interplay between nutrition and SNHL has only recently gained attention. Dietary supplementation could therefore constitute the first step for the prevention and potential repair of hearing damage before it reaches irreversibility. In this context, different epidemiological studies have shown correlations among the nutritional condition, increased total plasma homocysteine (tHcy) and SNHL. Several human genetic rare diseases are also associated with homocysteine (Hcy) metabolism and SNHL confirming this potential link. Accordingly, rodent experimental models have provided the molecular basis to understand the observed effects. Thus, increased tHcy levels and vitamin deficiencies, such as folic acid (FA), have been linked with SNHL, whereas long-term dietary supplementation with omega-3 fatty acids improved Hcy metabolism, cell survival and hearing acuity. Furthermore, pharmacological supplementations with the anti-oxidant fumaric acid that targets Hcy metabolism also improved SNHL. Overall these results strongly suggest that cochlear Hcy metabolism is a key player in the onset and progression of SNHL, opening the way for the design of prospective nutritional therapies. PMID:28487633

Sensorineural hearing loss and prematurity.

PubMed

Marlow, E S; Hunt, L P; Marlow, N

2000-03-01

To elucidate clinical antecedents of sensorineural hearing loss (SNHL) in very preterm infants. Case-control study. Fifteen children < 33 weeks' gestation with significant SNHL born between 1 January 1990 and 31 December 1994, detected within 9 months of birth, and 30 matched control children. Perinatal variables in the two groups were compared using non-parametric tests and conditional logistic regression (EGRET). Median birth weight for the index group was 960 g (range 600-2914 g) compared with 1026 g (range 410-2814 g) for controls. Children with SNHL had longer periods of intubation, ventilation, oxygen treatment, and acidosis, and more frequent treatment with dopamine or frusemide. Neither peak nor trough aminoglycoside levels, nor duration of jaundice or level of bilirubin varied between groups. However, SNHL was more likely if peak bilirubin levels coexisted with netilmicin use (odds ratio (95% confidence interval) 14.2 (1.8 to 113.6)) or if acidosis occurred when bilirubin levels were over 200 micromol/l (OR 8.0 (0.9 to 71.6). Frusemide use in the face of high serum creatinine levels (OR 8.9 (1.1 to 74.5)) or netilmicin treatment (OR 5.0 (0.99 to 24.8)) was also associated with SNHL. At 12 months of age, seven of 15 children with SNHL had evidence of cerebral palsy compared with two of 30 controls (OR 12.3 (2.1 to 71)). Preterm children with SNHL required more intensive care in the perinatal period and developed more neurological complications than controls. Among very preterm babies, the coexistence of risk factors for hearing loss may be more important than the individual factors themselves.

Imported Case of Lassa Fever in Sweden With Encephalopathy and Sensorineural Hearing Deficit.

PubMed

Grahn, Anna; Bråve, Andreas; Lagging, Martin; Dotevall, Leif; Ekqvist, David; Hammarström, Helena; Karlberg, Helen; Lagerqvist, Nina; Sansone, Martina; Tegnell, Anders; Ulleryd, Peter; Studahl, Marie

2016-10-01

We describe an imported case of Lassa fever with both encephalopathy and bilateral sensorineural hearing deficit. Absence of fever during hospitalization, initially nonspecific symptoms, and onset of hearing deficit in a late stage of disease probably contributed to delayed diagnosis (14 days after admittance to hospital). The pathogenesis of neurological manifestations of Lassa fever is poorly understood and no specific treatment was given. A total of 118 personnel had close contact with the patient, but no secondary cases occurred. This case highlights the importance of considering Lassa fever as a differential diagnosis in patients with recent travel to endemic areas.

Neurodevelopmental Disorders in Children with Severe to Profound Sensorineural Hearing Loss: A Clinical Study

ERIC Educational Resources Information Center

Chilosi, Anna M.; Comparini, Alessandro; Scusa, Maria F.; Berrettini, Stefano; Forli, Francesca; Battini, Roberta; Cipriani, Paola; Cioni, Giovanni

2010-01-01

Aim: The effects of sensorineural hearing loss (SNHL) are often complicated by additional disabilities, but the epidemiology of associated disorders is not clearly defined. The aim of this study was to evaluate the frequency and type of additional neurodevelopmental disabilities in a sample of children with SNHL and to investigate the relation…

The current status of audiologic rehabilitation for profound unilateral sensorineural hearing loss.

PubMed

Bishop, Charles E; Eby, Thomas L

2010-03-01

Audiologic rehabilitation of individuals with profound unilateral sensorineural hearing loss (USNHL) has traditionally been limited to the use of air-conduction contralateral routing of sound (CROS) hearing aids. Treatment for these individuals has expanded with new applications of the bone-anchored hearing aid (BAHA), transcranial hearing aid (t-CROS), and the cochlear implant. In this article, the authors review the literature that addresses these various treatment options. Contemporary review Historical information is available that describes the limited efficacy of air-conduction CROS hearing aids in lifting hearing handicap associated with USNHL. Current investigations on providing cross hearing are generally focused on use of the BAHA. Little is known at present whether new developments in hearing aid technology can improve on conventional air-conduction CROS or t-CROS approaches. Interestingly, the cochlear implant seems to be a viable option for individuals with USNHL and tinnitus who also have intact auditory nerve pathways. There is indication in the literature that BAHA provides greater relief of hearing handicap associated with USNHL than CROS hearing aids; however, both have been found to provide limited patient satisfaction and seemingly fall short of restoring true sound localization. Adequate trials have not been performed comparing BAHA with the best CROS hearing aid technology. Transcranial hearing aids and cochlear implants are experimental methods to treat USNHL and hold promise, although there remains a lack of studies available to fully support this.

Sensorineural hearing loss and celiac disease: A coincidental finding

PubMed Central

Volta, Umberto; Ferri, Gian Gaetano; De Giorgio, Roberto; Fabbri, Angela; Parisi, Claudia; Sciajno, Laura; Castellari, Alessandra; Fiorini, Erica; Piscaglia, Maria; Barbara, Giovanni; Granito, Alessandro; Pirodda, Antonio

2009-01-01

BACKGROUND Celiac disease (CD) can be associated with a variety of extraintestinal manifestations, including neurological diseases. A new neurological correlation has been found between CD and sensorineural hearing loss (SNHL). OBJECTIVE To verify the association between SNHL and CD, and to establish whether the neurological hearing impairment in CD is related to nonorgan-specific and antineuronal antibodies, as well as the presence of autoimmune disorders. METHODS A sample of 59 consecutive biopsy- and serologically proven CD patients were studied. Among CD patients, 11 were newly diagnosed and 48 were on a gluten-free diet. Hearing function was assessed by audiometric analysis in all CD patients as well as in 59 age- and sex-matched controls. Patients were tested for a panel of immune markers including nonorgan-specific autoantibodies and antineuronal antibodies. RESULTS SNHL was detected in five CD patients (8.5%) and in two controls (3.4%). In one patient, the SNHL was bilateral, whereas the remaining four had a monolateral impairment. The prevalence of SNHL was not significantly different between CD patients and controls. At least one of the antibodies tested for was positive in two of the five CD patients with SNHL and in 12 of the 54 CD patients without SNHL. Antineuronal antibodies to central nervous system antigens were consistently negative in the five CD patients with SNHL. Only one of the five CD patients with SNHL had Hashimoto thyroiditis. CONCLUSIONS SNHL and CD occur coincidentally. Hearing function should be assessed only in CD patients with clinical signs of hearing deficiency. PMID:19668795

Prevalence of otitis media and risk-factors for sensorineural hearing loss among infants attending Child Welfare Clinics in the Solomon Islands.

PubMed

Kaspar, Annette; Newton, Obiga; Kei, Joseph; Driscoll, Carlie; Swanepoel, De Wet; Goulios, Helen

2018-08-01

The present study aimed to assess the prevalence of otitis media and risk-factors for sensorineural hearing loss among infants in the Solomon Islands, in order to recommend an Infant Ear and Hearing Program that would be suitable to the Pacific Island context. Ear examinations and the JCIH Risk-Factor Questionnaire were administered to 288 infants attending Child Welfare Clinics in the Solomon Islands. Overall, 150 infants (52.084%) presented with bilateral normal ear examinations and no risk-factors for SNHL. There were 73 infants (25.34%) with ear pathology in at least one ear, 13 (4.5%) of whom required referral to the ENT Clinic for medical management. The most common pathology was otitis media with effusion (OME) (21.87%). Infants aged 7-12 months were significantly more likely to present with OME (p<0.001) and a history of otitis media (p=0.017) than infants aged 0-6 months. There were 71 infants (24.65%) with at least one risk-factor for sensorineural hearing loss. The most common risk-factors were ototoxicity (8.3%), non-elective caesarean delivery (6.59%), and possible in-utero syphilis infection (5.55%). The prevalence of otitis media and risk-factors for sensorineural hearing loss indicate the importance of initiating Infant Ear and Hearing Programs in the Solomon Islands. Program should facilitate early education on prevention of ear disease, as well as early diagnosis and management of children with hearing loss. Copyright © 2018. Published by Elsevier B.V.

Gait performance of children and adolescents with sensorineural hearing loss.

PubMed

Melo, Renato de Souza

2017-09-01

Several studies have demonstrated that children with sensorineural hearing loss (SNHL) may exhibit balance disorders, which can compromise the gait performance of this population. Compare the gait performance of normal hearing (NH) children and those with SNHL, considering the sex and age range of the sample, and analyze gait performance according to degrees of hearing loss and etiological factors in the latter group. This is a cross-sectional study that assessed 96 students, 48 NH and 48 with SNHL, aged between 7 and 18 years. The Brazilian version of the Dynamic Gait Index (DGI) was used to analyze gait and the Mann-Whitney test for statistical analysis. The group with SNHL obtained lower average gait performance compared to NH subjects (p=0.000). This was also observed when the children were grouped by sex female and male (p=0.000). The same difference occurred when the children were stratified by age group: 7-18 years (p=0.000). The group with severe and profound hearing loss exhibited worse gait performance than those with mild and moderate loss (p=0.048) and children with prematurity as an etiological factor demonstrated the worst gait performance. The children with SNHL showed worse gait performance compared to NH of the same sex and age group. Those with severe and profound hearing loss and prematurity as an etiological factor demonstrated the worst gait performances. Copyright © 2017 Elsevier B.V. All rights reserved.

Critical evaluation of round window membrane sealing in the treatment of idiopathic sudden unilateral hearing loss.

PubMed

Hoch, Stephan; Vomhof, Thomas; Teymoortash, Afshin

2015-03-01

Rupture of the round window membrane with consecutive development of a perilymphatic fistula (PLF) is still a matter of controversial debate in the pathogenesis of idiopathic sudden sensorineural hearing loss (SSHL). Until now no consensus exists about whether these patients benefit from performing an exploratory tympanotomy with sealing of the round window. The aim of the present study was to analyze critically the effectiveness of sealing the round window membrane in patients with SSHL. The clinical data of 51 patients with SSHL and a mean hearing decline of at least 60 dB over 5 frequencies who were treated with tympanotomy and sealing of the round window membrane were retrospectively analyzed. The results have been compared to the current state of the literature. Intraoperatively a round window membrane rupture or fluid leak was observed in none of the patients. After performing tympanotomy the mean improvement of hearing level was 32.7 dB. Twenty of 51 examined patients (39.2%) showed a mean improvement of the hearing level of more than 30 dB and a complete remission could be detected in 12 patients (23.5%). Reviewing the literature revealed no standard guidelines for definition or treatment of SSHL as well as for evaluation of hearing loss and its recovery. The results of the present study and the literature should be discussed critically. It is unclear whether tympanotomy and sealing of the round window membrane may be a meaningful treatment for SSHL. Therefore this procedure should be discussed as a therapeutic option only in selected patients with sudden deafness or profound hearing loss in which PLF is strongly suspicious or conservative treatment failed.

Strategies for Maximizing Speech-Recognition Performance: Adults with Mild to Moderately Severe Sensorineural Hearing Loss. EBP Briefs. Volume 11, Issue 4

ERIC Educational Resources Information Center

Donai, Jeremy J.; Schwartz, Jeremy C.

2016-01-01

Clinical Question: What high-frequency amplification strategy maximizes speechrecognition performance among adult hearing-impaired listeners with mild sloping to moderately severe sensorineural hearing loss? Method: Quick response review. Study Sources: EBSCO, PubMed, Google Scholar, as well as journals from the American Speech-Language-Hearing…

Sudden sensorineural hearing loss with positional vertigo: Initial findings of positional nystagmus and hearing outcomes.

PubMed

Kim, Chang-Hee; Shin, Jung Eun; Yang, Young Soo; Im, Donghyuk

2016-10-01

To investigate the initial findings of positional nystagmus in patients with sudden sensorineural hearing loss (SSNHL) and positional vertigo, and to compare hearing improvement among patients with different types of positional nystagmus. The characteristics of positional nystagmus upon initial examination were analysed, and the initial mean pure-tone audiometry (PTA) threshold was compared with that at three months after treatment. Forty-four SSNHL patients with concomitant positional vertigo were included. Positional nystagmus was classified into five subgroups; persistent geotropic direction-changing positional nystagmus (DCPN) in head-roll test (HRT) and negative Dix-Hallpike test (DHT), persistent apogeotropic DCPN in HRT and negative DHT, positive DHT and negative HRT, persistent geotropic DCPN in HRT and positive DHT, and persistent apogeotropic DCPN in HRT and positive DHT. PTA threshold improvement was significantly greater in SSNHL patients with negative DHT than with positive DHT (p = 0.027). When geotropic DCPN was elicited by HRT, the nystagmus was persistent, which suggests that alteration of specific gravity of the endolymph, rather than the lateral canal canalolithiasis, may be a cause of this characteristic positional nystagmus. Positive DTH may be a prognostic factor for worse hearing recovery among patients with SSNHL and positional vertigo.

[The experimental research of inner ear metabolism and electrical physiology of autoimmune sensorineural hearing loss].

PubMed

Tan, C; Cao, Y; Hu, P

1998-09-01

Inquire into the mechanism of inner ear pathological physiology in autoimmune sensorineural hearing loss (ASHL). With the auditory electric-physiological techniques and enzyme-histochemical method, the change of inner ear hearing function and enzyme activity were observed. These animals, which threshold of auditory nerve compound active potential (CAP) and cochlear microphonic potential(CM) heightening evidently, showed that the amplitude of endolymphatic potential(EP) (include-EP) bring down in various degrees, which was related to the change of the active of Na(+)-K(+)-ATPase and SDH in vascularis stria and endolymphatic sac. The abnormality of enzymes metabolism in inner ear tissues, which following autoimmune inflammation damage, is the pathological foundation of hearing dysfunction.

The Bjornstad syndrome (sensorineural hearing loss and pili torti) disease gene maps to chromosome 2q34-36.

PubMed Central

Lubianca Neto, J F; Lu, L; Eavey, R D; Flores, M A; Caldera, R M; Sangwatanaroj, S; Schott, J J; McDonough, B; Santos, J I; Seidman, C E; Seidman, J G

1998-01-01

We report that the Bjornstad syndrome gene maps to chromosome 2q34-36. The clinical association of sensorineural hearing loss with pili torti (broken, twisted hairs) was described >30 years ago by Bjornstad; subsequently, several small families have been studied. We evaluated a large kindred with Bjornstad syndrome in which eight members inherited pili torti and prelingual sensorineural hearing loss as autosomal recessive traits. A genomewide search using polymorphic loci demonstrated linkage between the disease gene segregating in this kindred and D2S434 (maximum two-point LOD score = 4.98 at theta = 0). Haplotype analysis of recombination events located the disease gene in a 3-cM region between loci D2S1371 and D2S163. We speculate that intermediate filament and intermediate filament-associated proteins are good candidate genes for causing Bjornstad syndrome. PMID:9545407

[Acute sensorineural hearing loss at the Otorhinolaryngology Department of the General Hospital in Subotica 1991-1996].

PubMed

Rakić, N

1999-01-01

Sudden sensorineural hearing loss is an acute hearing loss of a perceptive type, of unknown etiology, in most cases one-sided, or, a every sudden sensorineural deafness that occurs in the period of three days or less. Modern scientific literature points to three possible etiological factors: viral cochleitis, vascular lesion of the labyrinth and unrecognized rupture of the membranous labyrinth. The therapy for this disease is extremely various. There are dozens of reported different (or similar) healing protocols, including vasodilators, Hydroxyethyl starch, low molecular dextran, diuretics, defibrinogenation, fibrmolytic therapy, steroids, prostacyclin, oxygen therapy, hyperbaric oxygen therapy, vitamins, etc. The aim of the study was to recapitulate in brief modern views of etiology and therapy of the sudden sensorineural hearing loss, to analyze cases of this illness within the period of 1991-1996 in our Otorhinolaryngology Department and to compare these data with the same or similar data of other authors. In this study, medical records of patients admitted in the period of 1991-1996 in the Otorhinolaryngology Department of the Health Center in Subotica with a diagnosis of sudden sensorineural hearing loss were used. There were 53 patients with this diagnosis. All patients were treated in the hospital with infusions of rheoactive drugs. During the diagnostic procedure, all patients underwent a complete clinical otorhinolaryngologic examination, detailed anamnesis was taken, acoustic impedance tests (tympanometry) and tonal liminal audiometry were performed. Routine blood test was made (including complete blood picture, sedimentation rate, blood sugar) and a complete urine examination. Sometimes we demanded x-ray of the temporal bones (Schnller and Stenwers). A short increment sensitivity index (SISI) test and Carhart test were also made, if necessary. Function of the vestibular system was tested in cases when the vestibular symptoms were more strongly expressed

Clinical Study to Evaluate the Association Between Sensorineural Hearing Loss and Diabetes Mellitus in Poorly Controlled Patients Whose HbA1c >8.

PubMed

Srinivas, C V; Shyamala, V; Shiva Kumar, B R

2016-06-01

The relationship between sensorineural hearing loss (SNHL) and Diabetes mellitus has been known since more than 150 years. The pathophysiology of diabetes related hearing loss is speculative. Hearing loss is usually, bilateral, gradual onset, affecting higher frequencies. This study aims at knowing the prevalence of SNHL in DM and its relation to age, sex, duration of DM and control of DM. A total of 50 type 2 diabetics of age group 30-65 years were involved in the study. FBS, PPBS, HbA1c of all the subjects were done and later subjected to PTA. The type and severity of hearing loss was noted. Occurrence of SNHL was later compared with age, sex, duration, and control of DM. Sensorineural hearing loss was found in 66 % of type II diabetic patients and 34 % were found normal. Out of 50 diabetes mellitus patients, 33 patients had SNHL. All cases of SNHL detected were of gradual in onset and no one had hearing loss of sudden onset. Normal hearing was found in 34 % of patients, whereas 54 % of patients had mild hearing loss and 12 % of patients had moderate hearing loss. Association of hearing loss of DM patients with sex of the patient is insignificant. However there is significant association between older age group, longer duration and uncontrolled DM with that of SNHL. In subjects with HbA1c more than 8 and duration of diabetes mellitus more than 10 years prevalence of SNHL is more than 85 %, which is statistically significant. Sensorineural hearing loss in diabetes mellitus is gradually progressive involving high frequency thresholds. Hearing threshold increases with increasing age duration of diabetes and also high level of HbA1c greater than 8 %.

The role of alternative GJB2 transcription in screening for neonatal sensorineural deafness in Austria.

PubMed

Parzefall, Thomas; Lucas, Trevor; Koenighofer, Martin; Ramsebner, Reinhard; Frohne, Alexandra; Czeiger, Shelly; Baumgartner, Wolf-Dieter; Schoefer, Christian; Gstoettner, Wolfgang; Frei, Klemens

2017-04-01

Alterations within a novel putative Exon 1a within the gap junction beta 2 (GJB2) gene may play a role in the development of genetic hearing impairment in Austria. Mutations in the GJB2 gene are the most common cause of hereditary sensorineural deafness. Genome-wide screening for alternative transcriptional start sites in the human genome has revealed the presence of an additional GJB2 exon (E1a). This study tested the hypothesis of whether alternative GJB2 transcription involving E1a may play a role in the development of congenital sensorineural deafness in Austria. GJB2 E1a and flanking regions were sequenced in randomized normal hearing control subjects and three different patient groups with non-syndromic hearing impairment (NSHI), and bioinformatic analysis was performed. Statistical analysis of disease association was carried out using the Cochran-Armitage test for trend. A single change 2410 bp proximal to the translational start site (c.-2410T > C, rs7994748, NM_004004.5:c.-23 + 792T > C) was found to be significantly associated with the common c.35delG GJB2 mutation (p = .009). c.35delG in combination with c.-2410CC occurred at a 6.9-fold increased frequency compared to the control group. Additionally, one patient with idiopathic congenital hearing loss was found to be homozygous c.-2410CC.

Altered Brain Functional Activity in Infants with Congenital Bilateral Severe Sensorineural Hearing Loss: A Resting-State Functional MRI Study under Sedation.

PubMed

Xia, Shuang; Song, TianBin; Che, Jing; Li, Qiang; Chai, Chao; Zheng, Meizhu; Shen, Wen

2017-01-01

Early hearing deprivation could affect the development of auditory, language, and vision ability. Insufficient or no stimulation of the auditory cortex during the sensitive periods of plasticity could affect the function of hearing, language, and vision development. Twenty-three infants with congenital severe sensorineural hearing loss (CSSHL) and 17 age and sex matched normal hearing subjects were recruited. The amplitude of low frequency fluctuations (ALFF) and regional homogeneity (ReHo) of the auditory, language, and vision related brain areas were compared between deaf infants and normal subjects. Compared with normal hearing subjects, decreased ALFF and ReHo were observed in auditory and language-related cortex. Increased ALFF and ReHo were observed in vision related cortex, which suggest that hearing and language function were impaired and vision function was enhanced due to the loss of hearing. ALFF of left Brodmann area 45 (BA45) was negatively correlated with deaf duration in infants with CSSHL. ALFF of right BA39 was positively correlated with deaf duration in infants with CSSHL. In conclusion, ALFF and ReHo can reflect the abnormal brain function in language, auditory, and visual information processing in infants with CSSHL. This demonstrates that the development of auditory, language, and vision processing function has been affected by congenital severe sensorineural hearing loss before 4 years of age.

Sensorineural hearing loss among cerebellopontine-angle tumor patients examined with pure tone audiometry and brainstem-evoked response audiometry

NASA Astrophysics Data System (ADS)

Rinindra, A. M.; Zizlavsky, S.; Bashiruddin, J.; Aman, R. A.; Wulani, V.; Bardosono, S.

2017-08-01

Tumor in the cerebellopontine angle (CPA) accurs for approximately 5-10% of all intracranial tumors, where unilateral hearing loss and tinnitus are the most frequent symptoms. This study aimed to collect data on sensorineural hearing loss in CPA tumor patients in Dr. Cipto Mangunkusumo Hospital (CMH) using pure tone audiometry and brainstem-evoked response audiometry (BERA). It also aimed to obtaine data on CPA-tumor imaging through magnetic resonance imaging (MRI). This was a descriptive, analytic, and cross-sectional study. The subjects of this study were gathered using a total sampling method from secondary data between July 2012 and November 2016. From 104 patients, 30 matched the inclusion criteria. The CPA-tumor patients in the ENT CMH outpatient clinic were mostly female, middle-aged patients (41-60 years) whose clinical presentation was mostly tinnitus and severe, asymmetric sensorineural hearing loss in 10 subjects. From 30 subjects, 29 showed ipsilaterally impaired BERA results, and 17 subjects showed contralaterally impaired BERA results. There were 24 subjects who with large-sized tumors and 19 subjects who had intracanal tumors that had spread until they were extracanal in 19 subjects.

Hearing Loss in Adults.

ERIC Educational Resources Information Center

House, John W.

1997-01-01

This article discusses hearing loss in adults. It begins with an explanation of the anatomy of the ear and then explains the three types of hearing loss: conductive hearing loss, sensorineural hearing loss, and mixed conductive-sensorineural hearing loss. Tinnitus, hearing aids, and cochlear implants are also addressed. (CR)

Diabetes mellitus and sensorineural hearing loss: is there an association? Baseline of the Brazilian Longitudinal Study of Adult Health (ELSA-Brasil).

PubMed

Samelli, Alessandra G; Santos, Itamar S; Moreira, Renata R; Rabelo, Camila M; Rolim, Laurie P; Bensenõr, Isabela J; Lotufo, Paulo A

2017-01-01

Although several studies have investigated the effects of diabetes on hearing loss, the relationship between these two conditions remains unclear. Some studies have suggested that diabetes may cause sensorineural hearing loss, whereas others have failed to find an association. The biggest challenge in investigating the association between diabetes and hearing loss is the presence of confounding variables and the complexity of the auditory system. Our study investigated the association between diabetes and sensorineural hearing loss. We evaluated the influence of time from diabetes diagnosis on this association after controlling for age, gender, and hypertension diagnosis and excluding those subjects with exposure to noise. This cross-sectional study evaluated 901 adult and elderly Brazilian Longitudinal Study of Adult Health (ELSA-Brasil) participants from São Paulo, Brazil who underwent audiometry testing as part of ELSA-Brasil's baseline assessment. Hearing thresholds and speech test results were significantly worse in the group with diabetes than in the group without diabetes. However, no significant differences were found between participants with and without diabetes after adjusting for age, gender, and the presence of hypertension. Hearing thresholds were not affected by occupational noise exposure in the groups with and without diabetes. In addition, no association between the duration of diabetes and hearing thresholds was observed after adjusting for age, gender, and hypertension. We found no association between the duration of diabetes and worse hearing thresholds after models were adjusted for age, gender, and the presence of hypertension.

[Bilateral sensorineural hearing impairment due to Streptococcus suis meningitis 20 days after swine bite].

PubMed

Mori, Kousuke; Ishii, Nobuyuki; Mochizuki, Hitoshi; Taniguchi, Akitoshi; Shiomi, Kazutaka; Nakazato, Masamitsu

2013-01-01

Streptococcus suis (S. suis) is a zoonotic pathogen in pigs, which can be transmitted to humans by close contact. Meningitis is the most common clinical manifestations of S. suis infection and hearing impairment is a frequent complication. The risk of S. suis meningitis is higher in people who work in the swine industry. The patient was a 53-year-old woman working in the swine industry, who developed headache and fever 20 days after a swine bite. She was diagnosed as meningitis and S. suis was detected in the cerebrospinal fluid. We treated her with ceftriaxone, vancomycin, and dexamethasone, and signs of meningeal irritation diminished three days after admission. However, bilateral sensorineural hearing impairment occurred on the ninth day after admission. We added methylprednisolone (500 mg, 2 days) but moderate hearing impairment remained on the left. Antibiotic therapy should be considered for wounds of people involved in the swine industry for preventing S. suis infection.When S. suis meningitis occurs, symptoms of hearing impairment must be monitored carefully.

A comparison of the effects of filtering and sensorineural hearing loss on patients of consonant confusions.

PubMed

Wang, M D; Reed, C M; Bilger, R C

1978-03-01

It has been found that listeners with sensorineural hearing loss who show similar patterns of consonant confusions also tend to have similar audiometric profiles. The present study determined whether normal listeners, presented with filtered speech, would produce consonant confusions similar to those previously reported for the hearing-impaired listener. Consonant confusion matrices were obtained from eight normal-hearing subjects for four sets of CV and VC nonsense syllables presented under six high-pass and six-low pass filtering conditions. Patterns of consonant confusion for each condition were described using phonological features in sequential information analysis. Severe low-pass filtering produced consonant confusions comparable to those of listeners with high-frequency hearing loss. Severe high-pass filtering gave a result comparable to that of patients with flat or rising audiograms. And, mild filtering resulted in confusion patterns comparable to those of listeners with essentially normal hearing. An explanation in terms of the spectrum, the level of speech, and the configuration of this individual listener's audiogram is given.

Sensorineural hearing loss in hemodialysis patients.

PubMed

Jakić, Marko; Mihaljević, Dubravka; Zibar, Lada; Jakić, Marijana; Kotromanović, Zeljko; Roguljić, Hrvoje

2010-03-01

Chronic renal failure affects all organ systems. Senses are not exception and hearing impairment is common, particularly sensorineural hearing loss (SNHL). The term SNOS of unknown origin or uremic deafness is related to only a smaller part of the cases with unclear etiology of the impairment. The study searched for SNOS in 66 chronic hemodialysis (HD) patients, mean age 51.50 +/- 12.70 years. They were treated by HD for 69.70 +/- 53.80 months. The relation between the severity of the impairment and the patients' age, duration of HD treatment (months) and a set of laboratory parameters typical for chronic HD patients was examined. The aim of the study was to detect potential causes of the impairment. The increased hearing threshold (HT) of above 20 dB for all frequencies was found in 42 patients (mean HT 26 +/- 10.50 dB), for speaking area frequencies in 22 patients (mean HT 19.70 +/- 8.80 dB), and in 56 patients for high frequencies (mean HT 41.70 +/- 19.70 dB). The significant positive correlation of HT was found only with the patients' age (r = 0.49, p < 0.01). The patients older than 45 years had higher mean HT than those younger, and those older than 65 also had higher HT than the younger ones. Patients with pathological value of HT were significantly more common among the older subgroup of patients, when divided according to the age at both cutoff values of 45 and of 60 years. Mean HT did not differ significantly according to the duration of HD treatment (subgroups A- no longer than 60 months, B- from 61 to 120 months, and C- longer than 120 months). The patients with pathological HT did not differ significantly in frequency among those subgroups, and the subgroups were not different according to the mean age (A--50.30 +/- 13.20 years; B--51.40 +/- 12.75 years; C--55.80 +/- 10.55 years). In conclusion, our results along with other authors'published data report on SNHL as very frequent finding among chronic HD patients and suggest multifactorial etiology

Sensorineural hearing loss in very low birth weight infants with histological chorioamnionitis.

PubMed

Vedovato, Stefania; Lo Iacono, Angela; Morando, Carla; Suppiej, Agnese; Orzan, Eva; Trevisanuto, Daniele; Visentin, Silvia; Cavallin, Francesco; Chiarelli, Silvia; Zanardo, Vincenzo

2015-05-01

Histological chorioamnionitis (HCAM) has been associated with inflammatory diseases of preterm infants. Recently we have observed that it increased the risk of speech delay and hearing loss. So the aim of this study was to evaluate the relationship between sensorineural hearing loss (SNHL) of VLBW infants and HCAM. We performed an observational study on VLBW infants admitted to the NICU of Padua. Each patient with HCAM was matched with one control without HCAM. All infants underwent hearing screening before discharge by means of automated transient-evoked otoacustic emissions and automated auditory brainstem responses, which were repeated at 3 and 6 months of age with tympanometry measurement. Incidence of SNHL at 6 months of age was compared in the 2 groups and risk factors for hearing loss were studied. Two of 77 (2.6%) newborns with HCAM e 6/73 (8.2%) without it presented SNHL at 6 months of corrected age (p = 0.16). Multivariable logistic regression analysis identified surgical ligation of patent ductus arteriosus (PDA) as independent predictors of SNHL (OR: 5.75, 95% CI 1.34-24.84, p = 0.02), whereas the effect of HCAM on SNHL was only near to statistical significance level. Surgical ligation of PDA is associated with an increased risk of SNHL in VLBW infants, regardless of HCAM.

[Effect of chronic sensorineural hearing loss on several indicators of immune and endocrine systems of 7-11 year-old children].

PubMed

Beschasnyĭ, S P

2013-01-01

We investigated the effects of chronic bilateral sensorineural hearing loss of III-IV degree on the performance of interleukins, immunoglobulins serum and saliva, the functional activity of granulocyte-monocyte cell immunity, evaluated the activity of the hypothalamic-pituitary-adrenal system in children aged 7-11 years. It was found that due to stress activation of the sympathetic-adrenal system the function of granulocytes and monocytes is suppressed, with a predominance of production of anti-inflammatory interleukins. This leads to the dominance of T-helper type 2. Products granulocytes and T-helper type-2 anti-inflammatory interleukins IL-4, IL-5, IL-10, IL-13 leads to the activation of B-cells. Thus, in children 7-11 years of age with congenital bilateral sensorineural hearing loss is a decrease of non-specific humoral immunity dominated type of immune response to increased levels of IgG.

Computed tomography demonstrates abnormalities of contralateral ear in subjects with unilateral sensorineural hearing loss.

PubMed

Marcus, Sonya; Whitlow, Christopher T; Koonce, James; Zapadka, Michael E; Chen, Michael Y; Williams, Daniel W; Lewis, Meagan; Evans, Adele K

2014-02-01

Prior studies have associated gross inner ear abnormalities with pediatric sensorineural hearing loss (SNHL) using computed tomography (CT). No studies to date have specifically investigated morphologic inner ear abnormalities involving the contralateral unaffected ear in patients with unilateral SNHL. The purpose of this study is to evaluate contralateral inner ear structures of subjects with unilateral SNHL but no grossly abnormal findings on CT. IRB-approved retrospective analysis of pediatric temporal bone CT scans. 97 temporal bone CT scans, previously interpreted as "normal" based upon previously accepted guidelines by board certified neuroradiologists, were assessed using 12 measurements of the semicircular canals, cochlea and vestibule. The control-group consisted of 72 "normal" temporal bone CTs with underlying SNHL in the subject excluded. The study-group consisted of 25 normal-hearing contralateral temporal bones in subjects with unilateral SNHL. Multivariate analysis of covariance (MANCOVA) was then conducted to evaluate for differences between the study and control group. Cochlea basal turn lumen width was significantly greater in magnitude and central lucency of the lateral semicircular canal bony island was significantly lower in density for audiometrically normal ears of subjects with unilateral SNHL compared to controls. Abnormalities of the inner ear were present in the contralateral audiometrically normal ears of subjects with unilateral SNHL. These data suggest that patients with unilateral SNHL may have a more pervasive disease process that results in abnormalities of both ears. The findings of a cochlea basal turn lumen width disparity >5% from "normal" and/or a lateral semicircular canal bony island central lucency disparity of >5% from "normal" may indicate inherent risk to the contralateral unaffected ear in pediatric patients with unilateral sensorineural hearing loss. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Hearing evaluation of intratympanic methylprednisolone perfusion for refractory sudden sensorineural hearing loss.

PubMed

She, Wandong; Dai, Yanhong; Du, Xiaoping; Yu, Chenjie; Chen, Feng; Wang, Junguo; Qin, Xiaoming

2010-02-01

To investigate the effectiveness and safety of intratympanic methylprednisolone perfusion (IMP) through a microcatheter in patients with sudden sensorineural hearing loss (SSNHL) who failed a conventional treatment. Prospective clinical study. This study was conducted in Nanjing Drum Tower Hospital, Nanjing University Medical School. Patients who had failed a minimum 10-day conventional treatment were included. Twenty-six patients in the study group (SG) received methylprednisolone perfusion through a microcatheter placed into the tympanum once a day for 10 days and the conventional treatment. Twenty-three patients who received a second conventional treatment (no steroid) served as the comparison group (CG). All patients were followed up for three months after the end of treatment. The effective rates for SG and CG were 50 percent (61.9% when only patients with an interval from onset to IMP < or = 60 days were included) and 21.7 percent, respectively (chi(2) = 4.194, P = 0.041). The pure-tone average improvement was 20.2 +/- 15.6 dB in SG, and 9.2 +/- 13.7 dB in CG (z = 2.51, P = 0.011). In SG, hearing improvement at low frequencies was better than that at high frequencies. The interval from onset to IMP affected the efficacy of IMP. IMP through a microcatheter is a promising treatment for refractory SSNHL. The data suggest that the treatment may be more effective when administered at the earlier stages of SSNHL when the conventional treatment has failed. Copyright 2010 American Academy of Otolaryngology-Head and Neck Surgery Foundation. Published by Mosby, Inc. All rights reserved.

Hearing Instruments for Unilateral Severe-to-Profound Sensorineural Hearing Loss in Adults: A Systematic Review and Meta-Analysis

PubMed Central

Smith, Sandra Nelson; Lucas, Laura

2016-01-01

Objectives: A systematic review of the literature and meta-analysis was conducted to assess the nature and quality of the evidence for the use of hearing instruments in adults with a unilateral severe to profound sensorineural hearing loss. Design: The PubMed, EMBASE, MEDLINE, Cochrane, CINAHL, and DARE databases were searched with no restrictions on language. The search included articles from the start of each database until February 11, 2015. Studies were included that (a) assessed the impact of any form of hearing instrument, including devices that reroute signals between the ears or restore aspects of hearing to a deaf ear, in adults with a sensorineural severe to profound loss in one ear and normal or near-normal hearing in the other ear; (b) compared different devices or compared a device with placebo or the unaided condition; (c) measured outcomes in terms of speech perception, spatial listening, or quality of life; (d) were prospective controlled or observational studies. Studies that met prospectively defined criteria were subjected to random effects meta-analyses. Results: Twenty-seven studies reported in 30 articles were included. The evidence was graded as low-to-moderate quality having been obtained primarily from observational before-after comparisons. The meta-analysis identified statistically significant benefits to speech perception in noise for devices that rerouted the speech signals of interest from the worse ear to the better ear using either air or bone conduction (mean benefit, 2.5 dB). However, these devices also degraded speech understanding significantly and to a similar extent (mean deficit, 3.1 dB) when noise was rerouted to the better ear. Data on the effects of cochlear implantation on speech perception could not be pooled as the prospectively defined criteria for meta-analysis were not met. Inconsistency in the assessment of outcomes relating to sound localization also precluded the synthesis of evidence across studies. Evidence for

A Sound Therapy-Based Intervention to Expand the Auditory Dynamic Range for Loudness among Persons with Sensorineural Hearing Losses: Case Evidence Showcasing Treatment Efficacy

PubMed Central

Formby, Craig; Sherlock, LaGuinn P.; Hawley, Monica L.; Gold, Susan L.

2017-01-01

Case evidence is presented that highlights the clinical relevance and significance of a novel sound therapy-based treatment. This intervention has been shown to be efficacious in a randomized controlled trial for promoting expansion of the dynamic range for loudness and increased sound tolerance among persons with sensorineural hearing losses. Prior to treatment, these individuals were unable to use aided sound effectively because of their limited dynamic ranges. These promising treatment effects are shown in this article to be functionally significant, giving rise to improved speech understanding and enhanced hearing aid benefit and satisfaction, and, in turn, to enhanced quality of life posttreatment. These posttreatment sound therapy effects also are shown to be sustained, in whole or part, with aided environmental sound and to be dependent on specialized counseling to maximize treatment benefit. Importantly, the treatment appears to be efficacious for hearing-impaired persons with primary hyperacusis (i.e., abnormally reduced loudness discomfort levels [LDLs]) and for persons with loudness recruitment (i.e., LDLs within the typical range), which suggests the intervention should generalize across most individuals with reduced dynamic ranges owing to sensorineural hearing loss. An exception presented in this article is for a person describing the perceptual experience of pronounced loudness adaptation, which apparently rendered the sound therapy inaudible and ineffectual for this individual. Ultimately, these case examples showcase the enormous potential of a surprisingly simple sound therapy intervention, which has utility for virtually all audiologists to master and empower the adaptive plasticity of the auditory system to achieve remarkable treatment benefits for large numbers of individuals with sensorineural hearing losses. PMID:28286368

Sensorineural hearing loss degrades behavioral and physiological measures of human spatial selective auditory attention

PubMed Central

Dai, Lengshi; Best, Virginia; Shinn-Cunningham, Barbara G.

2018-01-01

Listeners with sensorineural hearing loss often have trouble understanding speech amid other voices. While poor spatial hearing is often implicated, direct evidence is weak; moreover, studies suggest that reduced audibility and degraded spectrotemporal coding may explain such problems. We hypothesized that poor spatial acuity leads to difficulty deploying selective attention, which normally filters out distracting sounds. In listeners with normal hearing, selective attention causes changes in the neural responses evoked by competing sounds, which can be used to quantify the effectiveness of attentional control. Here, we used behavior and electroencephalography to explore whether control of selective auditory attention is degraded in hearing-impaired (HI) listeners. Normal-hearing (NH) and HI listeners identified a simple melody presented simultaneously with two competing melodies, each simulated from different lateral angles. We quantified performance and attentional modulation of cortical responses evoked by these competing streams. Compared with NH listeners, HI listeners had poorer sensitivity to spatial cues, performed more poorly on the selective attention task, and showed less robust attentional modulation of cortical responses. Moreover, across NH and HI individuals, these measures were correlated. While both groups showed cortical suppression of distracting streams, this modulation was weaker in HI listeners, especially when attending to a target at midline, surrounded by competing streams. These findings suggest that hearing loss interferes with the ability to filter out sound sources based on location, contributing to communication difficulties in social situations. These findings also have implications for technologies aiming to use neural signals to guide hearing aid processing. PMID:29555752

Sensorineural hearing loss in patients with chronic suppurative otitis media: Is there a significant correlation?

PubMed

Amali, Amin; Hosseinzadeh, Nima; Samadi, Shahram; Nasiri, Shirin; Zebardast, Jayran

2017-02-01

Hearing loss as a sequel of chronic suppurative otitis media (CSOM) is often conductive, but recent studies have found an additional sensorineural component in these patients, thus demonstrating inner ear damage. The aim of the study was to determine the association between CSOM and sensorineural hearing loss (SNHL) and to assess the influence of patient's age, duration of disease, and presence of cholesteatoma and ossicular erosion on the degree of SNHL. In a retrospective study, the medical records of 119 patients who underwent surgery was reviewed. Seventy patients met the inclusion criteria of unilateral otorrhea, normal contralateral ear on otoscopy, and age between 10-65 years with no history of head trauma or ear surgery or familial hearing loss. Bone conduction (BC) thresholds for affected and contralateral ear were measured at frequencies of 500, 1000, 2000, and 4000 Hz. Data analysis was performed using SPSS 13 with independent-samples t-test, Pearson correlation test, and two-tailed analysis. A p ≤ 0.05 was considered statistically significant. Significant higher BC thresholds were found in the affected ear than in the normal ear for each frequency (p < 0.001), which increased with increasing frequency (7.00 dB at the 500 Hz and 9.71 dB at the 4000 Hz). There was a significant correlation between age and degree of SNHL (r = 0.422, p < 0.001) but no significant correlation was in duration of the disease (r = 0.119, p > 0.05). There was no relationship between presence of cholesteatoma and ossicular erosion with SNHL (p > 0.05). These findings demonstrate that CSOM is associated with some degree of SNHL and cochlear damage, and higher frequencies are more affected. Aging can act as a precipitating factor in this pathological process.

[A comparative study on efficacy of glucocorticoids, mineralocorticoids and vasoactive drugs on reversing hearing loss in patients suffering idiopathic sensorineural cochlear hypoacusis. A preliminary clinical trial].

PubMed

Campos-Bañales, Eugenia María; López-Campos, Daniel; de Serdio-Arias, José Luis; Esteban-Rodriguez, J; García-Sáinz, Mar; Muñoz-Cortés, Álvaro; López-Aguado, Daniel

2015-01-01

Sensory neural hearing loss (SNHL) is a disorder characterised by an important deterioration of the auditory function. Re-establishing normal ion homeostasis of the endolymph could be related to hearing recovery and it might be mediated by mineralocorticoids. The main purpose of this preliminary, randomized controlled clinical trial was assessing the recovery of idiopathic sensory neural cochlear hearing loss (SNHL) by comparing the efficacy of 2 types of steroids versus vasodilators. The 3-month intervention involved 70 patients, allocated into 4 different groups: a control with no medication, consisting of 14 patients (8 men and 6 women); a vasodilator group of 21 patients (11 men and 10 women); a glucocorticoid group with 16 patients (10 men and 6 women); and a mineralocorticoid therapy group, consisting of 19 patients (11 men and 8 women). The level of hearing loss and its topography were estimated using Liminal Tone Audiometry (LTA) and Auditory Brainstem Response (ABR). Our research found overall greater efficacy of mineralocorticoids versus glucocorticoids and vasodilators. There was better response in women than in men and it was higher from the left ear, regardless of patient gender. The hearing gain was significantly superior in the mineralocorticoid group, followed by the glucocorticoid group. However, the responses to vasodilators were lesser and of low statistical significance. Copyright © 2014 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Patología Cérvico-Facial. All rights reserved.

TMTC2 variant associated with sensorineural hearing loss and auditory neuropathy spectrum disorder in a family dyad.

PubMed

Guillen-Ahlers, Hector; Erbe, Christy B; Chevalier, Frédéric D; Montoya, Maria J; Zimmerman, Kip D; Langefeld, Carl D; Olivier, Michael; Runge, Christina L

2018-04-19

Sensorineural hearing loss (SNHL) is a common form of hearing loss that can be inherited or triggered by environmental insults; auditory neuropathy spectrum disorder (ANSD) is a SNHL subtype with unique diagnostic criteria. The genetic factors associated with these impairments are vast and diverse, but causal genetic factors are rarely characterized. A family dyad, both cochlear implant recipients, presented with a hearing history of bilateral, progressive SNHL, and ANSD. Whole-exome sequencing was performed to identify coding sequence variants shared by both family members, and screened against genes relevant to hearing loss and variants known to be associated with SNHL and ANSD. Both family members are successful cochlear implant users, demonstrating effective auditory nerve stimulation with their devices. Genetic analyses revealed a mutation (rs35725509) in the TMTC2 gene, which has been reported previously as a likely genetic cause of SNHL in another family of Northern European descent. This study represents the first confirmation of the rs35725509 variant in an independent family as a likely cause for the complex hearing loss phenotype (SNHL and ANSD) observed in this family dyad. © 2018 The Authors. Molecular Genetics & Genomic Medicine published by Wiley Periodicals, Inc.

Sudden sensorineural hearing loss: is there a relationship between routine haematological parameters and audiogram shapes?

PubMed

Salvago, Pietro; Rizzo, Serena; Bianco, Antonino; Martines, Francesco

2017-03-01

To investigate the relationship between haematological routine parameters and audiogram shapes in patients affected by sudden sensorineural hearing loss (SSNHL). A retrospective study. All patients were divided into four groups according to the audiometric curve and mean values of haematological parameters (haemoglobin, white blood cell, neutrophils and lymphocytes relative count, platelet count, haematocrit, prothrombin time, activated partial thromboplastin time, fibrinogen and neutrophil-to-lymphocite ratio) of each group were statistically compared. The prognostic role of blood profile and coagulation test was also examined. A cohort of 183 SSNHL patients without comorbidities. With a 48.78% of complete hearing recovery, individuals affected by upsloping hearing loss presented a better prognosis instead of flat (18.36%), downsloping (19.23%) and anacusis (2.45%) groups (p = 0.0001). The multivariate analysis of complete blood count values revealed lower mean percentage of lymphocytes (p = 0.041) and higher platelet levels (p = 0.015) in case of downsloping hearing loss; with the exception of fibrinogen (p = 0.041), none of the main haematological parameters studied resulted associated with poorer prognosis. Our work suggested a lack of association between haematological parameters and a defined audiometric picture in SSNHL patients; furthermore, only fibrinogen seems to influence the prognosis of this disease.

Sudden Bilateral Sensorineural Hearing Loss Following Postpartum Hemorrhage: A Case Report

PubMed Central

Mirzaeian, Sara; Ayati, Sedigheh; Maleki, Asieh

2017-01-01

The prevalence of bilateral sudden sensorineural hearing loss (SSNHL) is less than 5% and the etiology of most cases is unknown. Due to many structural and functional similarities between the kidney and inner ear, many conditions, diseases, and drugs have both renal and cochlear effects and toxicities. There are several reports of SSNHL in patients with CRF, uraemic patient, hemodialysis treatment, and ARF. Here, we report a rare manifestation of SSNHL following severe postpartum hemorrhage that has simultaneous renal failure and cochlear impairment. The patient was a 22-year-old primigravida woman with term pregnancy who after delivery and episiotomy hematoma and postpartum hemorrhage subsequently suffered from kidney failure, oliguria, and SSNHL that occurred after 3 days of delivery. In conditions such as severe postpartum bleeding leading to acute renal involvement, the possibility of simultaneous involvement of cochlea due to hypoxia or received drugs should be considered. PMID:28761208

Lassa fever-induced sensorineural hearing loss: A neglected public health and social burden.

PubMed

Mateer, Elizabeth J; Huang, Cheng; Shehu, Nathan Y; Paessler, Slobodan

2018-02-01

Although an association between Lassa fever (LF) and sudden-onset sensorineural hearing loss (SNHL) was confirmed clinically in 1990, the prevalence of LF-induced SNHL in endemic countries is still underestimated. LF, a viral hemorrhagic fever disease caused by Lassa virus (LASV), is endemic in West Africa, causing an estimated 500,000 cases and 5,000 deaths per year. Sudden-onset SNHL, one complication of LF, occurs in approximately one-third of survivors and constitutes a neglected public health and social burden. In the endemic countries, where access to hearing aids is limited, SNHL results in a decline of the quality of life for those affected. In addition, hearing loss costs Nigeria approximately 43 million dollars per year. The epidemiology of LF-induced SNHL has not been characterized well. The complication of LF induced by SNHL is also an important consideration for vaccine development and treatments. However, research into the mechanism has been hindered by the lack of autopsy samples and relevant small animal models. Recently, the first animal model that mimics the symptoms of SNHL associated with LF was developed. Preliminary data from the new animal model as well as the clinical case studies support the mechanism of immune-mediated injury that causes SNHL in LF patients. This article summarizes clinical findings of hearing loss in LF patients highlighting the association between LASV infection and SNHL as well as the potential mechanism(s) for LF-induced SNHL. Further research is necessary to identify the mechanism and the epidemiology of LF-induced SNHL.

Electroretinogram assessment of children with sensorineural hearing loss: implications for screening.

PubMed

West, Stephanie K; Hindocha, Maya; Hogg, Chris R; Holder, Graham E; Moore, Anthony T; Reddy, M Ashwin

2015-10-01

The guidelines of the National Deaf Children's Society recommend that children with sensorineural hearing loss (SNHL) be routinely screened for ophthalmological problems and suggest electroretinography (ERG) to exclude Usher syndrome. The present study reports the nature and prevalence of abnormal ERG findings in a cohort of children with SNHL undergoing ERG with the aim of identifying risk factors for the diagnosis of Usher syndrome. The medical records of children (<18 years of age) with SNHL referred for ERG at Moorfields Eye Hospital, London, between January 2009 and December 2011 were retrospectively reviewed. Patients were included if they had been referred with SNHL by an audiological medicine consultant and the primary indication for electrodiagnostic testing was possible Usher syndrome. A total of 84 cases met inclusion criteria of which 13 (15%) had ERG findings showing rod-cone dysfunction consistent with a diagnosis of Usher syndrome. Two patients with retinal pigmentary changes had normal ERGs and were diagnosed with rubella retinopathy based on the clinical findings. Risk factor analysis showed that age of ≥8 years at the time of ERG, sex, and bilateral hearing loss were not predictive of a diagnosis of Usher syndrome. However, the presence of or referral for cochlear implants, having relevant symptoms and/or clinical signs consistent with a retinal dystrophy, and profound hearing loss were all highly predictive. ERG is a useful diagnostic tool in children with SNHL and should be performed in children with SNHL who have cochlear implants and/or have signs or symptoms of retinal dystrophy. A focused approach could have potential cost-saving benefit. Copyright © 2015 American Association for Pediatric Ophthalmology and Strabismus. Published by Elsevier Inc. All rights reserved.

Hearing status in patients with rheumatoid arthritis.

PubMed

Ahmadzadeh, A; Daraei, M; Jalessi, M; Peyvandi, A A; Amini, E; Ranjbar, L A; Daneshi, A

2017-10-01

Rheumatoid arthritis is thought to induce conductive hearing loss and/or sensorineural hearing loss. This study evaluated the function of the middle ear and cochlea, and the related factors. Pure tone audiometry, speech reception thresholds, speech discrimination scores, tympanometry, acoustic reflexes, and distortion product otoacoustic emissions were assessed in rheumatoid arthritis patients and healthy volunteers. Pure tone audiometry results revealed a higher bone conduction threshold in the rheumatoid arthritis group, but there was no significant difference when evaluated according to the sensorineural hearing loss definition. Distortion product otoacoustic emissions related prevalence of conductive or mixed hearing loss, tympanometry values, acoustic reflexes, and speech discrimination scores were not significantly different between the two groups. Sensorineural hearing loss was significantly more prevalent in patients who used azathioprine, cyclosporine and etanercept. Higher bone conduction thresholds in some frequencies were detected in rheumatoid arthritis patients that were not clinically significant. Sensorineural hearing loss is significantly more prevalent in refractory rheumatoid arthritis patients.

Children with minimal sensorineural hearing loss: prevalence, educational performance, and functional status.

PubMed

Bess, F H; Dodd-Murphy, J; Parker, R A

1998-10-01

This study was designed to determine the prevalence of minimal sensorineural hearing loss (MSHL) in school-age children and to assess the relationship of MSHL to educational performance and functional status. To determine prevalence, a single-staged sampling frame of all schools in the district was created for 3rd, 6th, and 9th grades. Schools were selected with probability proportional to size in each grade group. The final study sample was 1218 children. To assess the association of MSHL with educational performance, children identified with MSHL were assigned as cases into a subsequent case-control study. Scores of the Comprehensive Test of Basic Skills (4th Edition) (CTBS/4) then were compared between children with MSHL and children with normal hearing. School teachers completed the Screening Instrument for Targeting Education Risk (SIFTER) and the Revised Behavior Problem Checklist for a subsample of children with MSHL and their normally hearing counterparts. Finally, data on grade retention for a sample of children with MSHL were obtained from school records and compared with school district norm data. To assess the relationship between MSHL and functional status, test scores of all children with MSHL and all children with normal hearing in grades 6 and 9 were compared on the COOP Adolescent Chart Method (COOP), a screening tool for functional status. MSHL was exhibited by 5.4% of the study sample. The prevalence of all types of hearing impairment was 11.3%. Third grade children with MSHL exhibited significantly lower scores than normally hearing controls on a series of subtests of the CTBS/4; however, no differences were noted at the 6th and 9th grade levels. The SIFTER results revealed that children with MSHL scored poorer on the communication subtest than normal-hearing controls. Thirty-seven percent of the children with MSHL failed at least one grade. Finally, children with MSHL exhibited significantly greater dysfunction than children with normal hearing

Cytokine gene polymorphism associations with congenital cytomegalovirus infection and sensorineural hearing loss.

PubMed

Kasztelewicz, B; Czech-Kowalska, J; Lipka, B; Milewska-Bobula, B; Borszewska-Kornacka, M K; Romańska, J; Dzierżanowska-Fangrat, K

2017-10-01

Cytomegalovirus (CMV) is the most common viral agent of congenital infections and a leading nongenetic cause of sensorineural hearing loss (SNHL). The host immunologic factors that render a developing foetus prone to intrauterine CMV infection and development of hearing loss are unknown. The aim of this study was to assess the potential associations between the polymorphisms within cytokine and cytokine receptors genes, and the risk of congenital CMV infection, and the hearing outcome. A panel of 11 candidate single nucleotide polymorphisms (SNPs): TNF rs1799964, TNF rs1800629, TNFRSF1A rs4149570, IL1B rs16944, IL1B rs1143634, IL10 rs1800896, IL10RA rs4252279, IL12B rs3212227, CCL2 rs1024611, CCL2 rs13900, CCR5 rs333 was genotyped in 470 infants (72 with confirmed intrauterine CMV infection and 398 uninfected controls), and related to congenital CMV infection, and the outcome. In multivariate analysis, the IL1B rs16944 TT and TNF rs1799964 TC genotypes were significantly associated with intrauterine CMV infection (aOR = 2.32; 95% CI, 1.11-4.89; p = 0.032, and aOR = 2.17, 95% CI, 1.25-3.77; p = 0.007, respectively). Twenty-two out of 72 congenitally infected newborns had confirmed SNHL. Carriers of CT or TT genotype of CCL2 rs13900 had increased risk of hearing loss at birth and at 6 months of age (aOR = 3.59; p = 0.028 and aOR = 4.10; p = 0.039, respectively). This is the first study to report an association between SNPs in IL1B, TNF, and CCL2, and susceptibility to congenital CMV infection (IL1B and TNF) and SNHL (CCL2).

Epidemiological and clinical aspects of ear nose and throat sensorineural emergencies in the Yaoundé reference hospital.

PubMed

Djomou, François; Nkouo, Yves Christian Andjock; Mindja, Eko David; Nchinda, Choffor; Meka, Luc; Mbamyah-Lyonga, Emilia; Ndjolo, Alexis

2016-01-01

Sensorineural emergencies (SNE) are rare clinical situations. Few patients consult early explaining subsequent difficulty in having accurate data and management. Three clinical conditions are considered SNE in otolaryngology; they include sudden sensorineural hearing loss (SSHL), Bell's palsy and acute vertigo. There is very little data available on sensorineural emergencies in our setting. The aim of this study was to provide preliminary data on the management of Ear Nose and Throat (ENT) sensorineural emergency cases in Yaoundé Reference Hospital. A descriptive retrospective study was carried out based on data collected over a period of 5 years, January 2010 to July 2014 at the Yaoundé Reference Hospital. Information was obtained from patients' files collected from the archives of the institution. Patients presenting with SSHL, Bell's palsy, acute vertigo who consulted during the study period were included in the study. A total of 22 patients were included in the study out of 6406 patients who consulted at the ENT Unit. The prevalence of SNE in ENT consultations was 0.003, distributed as follows; 13 patients (59.1%) of SNE had Bell's palsy, seven (31.8%) had vestibular neuritis and two (9.1%) had SSHL. The prevalence of SNE was low with idiopathic Bell's palsy being the most frequent. There was a general delay in arrival of patients hence delay in diagnosis. This delay could equally be a factor for treatment failure and poor prognosis. More effort should be made in terms of population sensitization about the necessity of getting early medical attention.

EEG activity as an objective measure of cognitive load during effortful listening: A study on pediatric subjects with bilateral, asymmetric sensorineural hearing loss.

PubMed

Marsella, Pasquale; Scorpecci, Alessandro; Cartocci, Giulia; Giannantonio, Sara; Maglione, Anton Giulio; Venuti, Isotta; Brizi, Ambra; Babiloni, Fabio

2017-08-01

Deaf subjects with hearing aids or cochlear implants generally find it challenging to understand speech in noisy environments where a great deal of listening effort and cognitive load are invested. In prelingually deaf children, such difficulties may have detrimental consequences on the learning process and, later in life, on academic performance. Despite the importance of such a topic, currently, there is no validated test for the assessment of cognitive load during audiological tasks. Recently, alpha and theta EEG rhythm variations in the parietal and frontal areas, respectively, have been used as indicators of cognitive load in adult subjects. The aim of the present study was to investigate, by means of EEG, the cognitive load of pediatric subjects affected by asymmetric sensorineural hearing loss as they were engaged in a speech-in-noise identification task. Seven children (4F and 3M, age range = 8-16 years) affected by asymmetric sensorineural hearing loss (i.e. profound degree on one side, mild-to-severe degree on the other side) and using a hearing aid only in their better ear, were included in the study. All of them underwent EEG recording during a speech-in-noise identification task: the experimental conditions were quiet, binaural noise, noise to the better hearing ear and noise to the poorer hearing ear. The subjects' Speech Recognition Thresholds (SRT) were also measured in each test condition. The primary outcome measures were: frontal EEG Power Spectral Density (PSD) in the theta band and parietal EEG PSD in the alpha band, as assessed before stimulus (word) onset. No statistically significant differences were noted among frontal theta power levels in the four test conditions. However, parietal alpha power levels were significantly higher in the "binaural noise" and in the "noise to worse hearing ear" conditions than in the "quiet" and "noise to better hearing ear" conditions (p < 0.001). SRT scores were consistent with task difficulty, but did

Sensorineural hearing loss amplifies neural coding of envelope information in the central auditory system of chinchillas

PubMed Central

Zhong, Ziwei; Henry, Kenneth S.; Heinz, Michael G.

2014-01-01

People with sensorineural hearing loss often have substantial difficulty understanding speech under challenging listening conditions. Behavioral studies suggest that reduced sensitivity to the temporal structure of sound may be responsible, but underlying neurophysiological pathologies are incompletely understood. Here, we investigate the effects of noise-induced hearing loss on coding of envelope (ENV) structure in the central auditory system of anesthetized chinchillas. ENV coding was evaluated noninvasively using auditory evoked potentials recorded from the scalp surface in response to sinusoidally amplitude modulated tones with carrier frequencies of 1, 2, 4, and 8 kHz and a modulation frequency of 140 Hz. Stimuli were presented in quiet and in three levels of white background noise. The latency of scalp-recorded ENV responses was consistent with generation in the auditory midbrain. Hearing loss amplified neural coding of ENV at carrier frequencies of 2 kHz and above. This result may reflect enhanced ENV coding from the periphery and/or an increase in the gain of central auditory neurons. In contrast to expectations, hearing loss was not associated with a stronger adverse effect of increasing masker intensity on ENV coding. The exaggerated neural representation of ENV information shown here at the level of the auditory midbrain helps to explain previous findings of enhanced sensitivity to amplitude modulation in people with hearing loss under some conditions. Furthermore, amplified ENV coding may potentially contribute to speech perception problems in people with cochlear hearing loss by acting as a distraction from more salient acoustic cues, particularly in fluctuating backgrounds. PMID:24315815

Plasticity in the Developing Auditory Cortex: Evidence from Children with Sensorineural Hearing Loss and Auditory Neuropathy Spectrum Disorder

PubMed Central

Cardon, Garrett; Campbell, Julia; Sharma, Anu

2013-01-01

The developing auditory cortex is highly plastic. As such, the cortex is both primed to mature normally and at risk for re-organizing abnormally, depending upon numerous factors that determine central maturation. From a clinical perspective, at least two major components of development can be manipulated: 1) input to the cortex and 2) the timing of cortical input. Children with sensorineural hearing loss (SNHL) and auditory neuropathy spectrum disorder (ANSD) have provided a model of early deprivation of sensory input to the cortex, and demonstrated the resulting plasticity and development that can occur upon introduction of stimulation. In this article, we review several fundamental principles of cortical development and plasticity and discuss the clinical applications in children with SNHL and ANSD who receive intervention with hearing aids and/or cochlear implants. PMID:22668761

Vestibular-evoked myogenic potential in the prediction of recovery from acute low-tone sensorineural hearing loss.

PubMed

Wang, Chi-Te; Fang, Kai-Min; Young, Yi-Ho; Cheng, Po-Wen

2010-04-01

Click and galvanic stimulations of vestibular-evoked myogenic potential (c-VEMP and g-VEMP) were applied to measure the interaural difference (IAD) of saccular responses in patients with acute low-tone sensorineural hearing loss (ALHL). This study intended to explore the relationship between saccular asymmetry and final hearing recovery. We hypothesize that greater extent of saccular dysfunction may be associated with lesser hearing recovery. Twenty-one patients with unilateral ALHL were prospectively enrolled to receive c-VEMP and g-VEMP tests in a random sequence. The IAD of the saccular responses for each patient was measured using three parameters-the raw and corrected amplitudes of c-VEMP, and corrected c-VEMP to g-VEMP amplitude ratio (C/G ratio). The IAD for each parameter was classified as depressed, normal, or augmented by calculating the difference between the affected and unaffected ears and dividing by its sum for both ears. After 3 consecutive months of oral medication and follow-up, 19 patients displayed a hearing recovery of >50%; only two had a recovery of <50%. The significant correlation between the IAD of corrected C/G ratios and hearing recovery demonstrated that subjects with depressed responses had a worse hearing outcome (percent recovery: 51% [45-80%], median [minimum-maximum]), compared with those with normal responses, who exhibited the best recovery (87% [56-100%]), whereas patients with augmented response showed an intermediate recovery (67% [54-100%]; p = 0.02, Kruskal-Wallis test). On the contrary, the raw and corrected amplitudes of c-VEMP did not reveal a significantly different hearing recovery among the three groups of saccular responses. The extent of saccular dysfunction in ALHL might be better explored by combining the results of c-VEMP and g-VEMP. Outcome analysis indicated that the corrected C/G ratio might be a promising prognostic factor for hearing recovery in ALHL.

[The very severe sensorineural deafness patients caused by rubella virus infection: two cases report].

PubMed

Ma, Jing; Wan, Lang; Xu, Fen

2015-09-01

To explore the audiological features in children who were sever sensorineural hearing loss infected with rubella virus. There were two cases of rubella virus infection in children who were deaf, they conducted the distortion product otoacoustic emission, ABR and auditory steady-state evoked response (ASSR) examination, then analyzed the results comprehensively. Two patients' mothers were prompted to have infected rubella virus during the early three months pregnant period by history and laboratory tests. The two patients were not detected deafness gene mutation. Audiology results implied the two patients were very severe binaural sensorineural deafness, so they were recommended to equipped with hearing aids and cochlear implant surgery. Early pregnancy women infected with rubella virus can cause very severe offspring sensorineural deafness. The crowd whose mother were suspected to infect with rubella virus in early pregnancy, that should be tracked and detected hearing in order to achieve early detection, early intervention and early treatment.

Progressive sensorineural hearing loss, subjective tinnitus and vertigo caused by elevated blood lipids.

PubMed

Pulec, J L; Pulec, M B; Mendoza, I

1997-10-01

The otologist frequently sees patients with progressive sensorineural hearing loss, subjective aural tinnitus and vertigo with no apparent cause. Elevated blood lipids may be a cause of inner ear malfunction on a biochemical basis. To establish the true incidence of this condition, all new patients (4,251) seen during an eight-year period were evaluated; of these, 2,332 patients had complaints of inner ear disease. All had a complete neurotologic examination, appropriate audiometric and vestibular studies and imaging, and blood tests including lipid phenotype studies. Hyperlipoproteinemia was found in 120 patients (5.1%). Most patients were found to be overweight and had additional coexisting conditions such as diabetes mellitus. Treatment with vasodilators and a 500-calorie, high-protein, low-carbohydrate diet yielded improvement of symptoms in 83% of patients within five months of initiation of treatment.

Genetic studies in a patient with X-linked retinoschisis coexisting with developmental delay and sensorineural hearing loss.

PubMed

Sudha, Dhandayuthapani; Patric, Irene Rosita Pia; Ganapathy, Aparna; Agarwal, Smitha; Krishna, Shuba; Neriyanuri, Srividya; Sripriya, Sarangapani; Sen, Parveen; Chidambaram, Subbulakshmi; Arunachalam, Jayamuruga Pandian

2017-01-01

In this study, we present a juvenile retinoschisis patient with developmental delay, sensorineural hearing loss, and reduced axial tone. X-linked juvenile retinoschisis (XLRS) is a retinal dystrophy, most often not associated with systemic anomalies and also not showing any locus heterogeneity. Therefore it was of interest to understand the genetic basis of the condition in this patient. RS1 gene screening for XLRS was performed by Sanger sequencing. Whole genome SNP 6.0 array analysis was carried out to investigate gross chromosomal aberrations that could result in systemic phenotype. In addition, targeted next generation sequencing (NGS) was employed to determine any possible involvement of X-linked syndromic and non-syndromic mental retardation genes. This NGS panel consisted of 550 genes implicated in several other rare inherited diseases. RS1 gene screening revealed a pathogenic hemizygous splice site mutation (c.78+1G>T), inherited from the mother. SNP 6.0 array analysis did not indicate any significant chromosomal aberrations that could be disease-associated. Targeted resequencing did not identify any mutations in the X-linked mental retardation genes. However, variations in three other genes (NSD1, LARGE, and POLG) were detected, which were all inherited from the patient's unaffected father. Taken together, RS1 mutation was found to segregate with retinoschisis phenotype while none of the other identified variations were co-segregating with the systemic defects. Hereby, we infer that the multisystemic defects harbored by the patient are a rare coexistence of XLRS, developmental delay, sensorineural hearing loss, and reduced axial tone reported for the first time in the literature.

The effect of sensorineural hearing loss and tinnitus on speech recognition over air and bone conduction military communications headsets.

PubMed

Manning, Candice; Mermagen, Timothy; Scharine, Angelique

2017-06-01

Military personnel are at risk for hearing loss due to noise exposure during deployment (USACHPPM, 2008). Despite mandated use of hearing protection, hearing loss and tinnitus are prevalent due to reluctance to use hearing protection. Bone conduction headsets can offer good speech intelligibility for normal hearing (NH) listeners while allowing the ears to remain open in quiet environments and the use of hearing protection when needed. Those who suffer from tinnitus, the experience of perceiving a sound not produced by an external source, often show degraded speech recognition; however, it is unclear whether this is a result of decreased hearing sensitivity or increased distractibility (Moon et al., 2015). It has been suggested that the vibratory stimulation of a bone conduction headset might ameliorate the effects of tinnitus on speech perception; however, there is currently no research to support or refute this claim (Hoare et al., 2014). Speech recognition of words presented over air conduction and bone conduction headsets was measured for three groups of listeners: NH, sensorineural hearing impaired, and/or tinnitus sufferers. Three levels of speech-to-noise (SNR = 0, -6, -12 dB) were created by embedding speech items in pink noise. Better speech recognition performance was observed with the bone conduction headset regardless of hearing profile, and speech intelligibility was a function of SNR. Discussion will include study limitations and the implications of these findings for those serving in the military. Published by Elsevier B.V.

Cytomegalovirus-induced sensorineural hearing loss with persistent cochlear inflammation in neonatal mice.

PubMed

Schachtele, Scott J; Mutnal, Manohar B; Schleiss, Mark R; Lokensgard, James R

2011-06-01

Congenital cytomegalovirus (CMV) infection is the leading cause of sensorineural hearing loss (SNHL) in children. During murine (M)CMV-induced encephalitis, the immune response is important for both the control of viral dissemination and the clearance of virus from the brain. While the importance of CMV-induced SNHL has been described, the mechanisms surrounding its pathogenesis and the role of inflammatory responses remain unclear. This study presents a neonatal mouse model of profound SNHL in which MCMV preferentially infected both cochlear perilymphatic epithelial cells and spiral ganglion neurons. Interestingly, MCMV infection induced cochlear hair cell death by 21 days post-infection, despite a clear lack of direct infection of hair cells and the complete clearance of the virus from the cochlea by 14 dpi. Flow cytometric, immunohistochemical, and quantitative PCR analysis of MCMV-infected cochlea revealed a robust and chronic inflammatory response, including a prolonged increase in reactive oxygen species production by infiltrating macrophages. These data support a pivotal role for inflammation during MCMV-induced SNHL.

An Evaluation of the BKB-SIN, HINT, QuickSIN, and WIN Materials on Listeners with Normal Hearing and Listeners with Hearing Loss

ERIC Educational Resources Information Center

Wilson, Richard H.; McArdle, Rachel A.; Smith, Sherri L.

2007-01-01

Purpose: The purpose of this study was to examine in listeners with normal hearing and listeners with sensorineural hearing loss the within- and between-group differences obtained with 4 commonly available speech-in-noise protocols. Method: Recognition performances by 24 listeners with normal hearing and 72 listeners with sensorineural hearing…

External ear anomalies and hearing impairment in Noonan Syndrome.

PubMed

van Trier, Dorothée C; van Nierop, Josephine; Draaisma, Jos M Th; van der Burgt, Ineke; Kunst, Henricus; Croonen, Ellen A; Admiraal, Ronald J C

2015-06-01

This is the first cohort in which hearing impairment and external ear anomalies in Noonan Syndrome are described extensively. Retrospective analysis of the otorhinolaryngological and clinical genetic data from 97 Noonan Syndrome (NS) patients. Forty-four NS patients were seen by an otorhinolaryngologist for the analysis of hearing impairment. In our cohort 80 of the 97 patients were genetically tested. In 71 of these mutations were found: in 48 patients a mutation in PTPN11, in 10 patients in SOS1, in 5 patients in SHOC2, in 5 patients in RAF1, in 1 patient in MAP2K2, in 1 patient in KRAS and in 1 patient in A2ML1. External ear anomalies were reported in 75 NS patients (77%). In 69 patients the ears were low-set, 28 patients had posteriorly rotated ears, 14 patients showed protruding ears and 18 had thickened helices. Hearing impairment was detected in 34 NS patients. Nine patients had sensorineural hearing impairment, two a permanent conductive hearing impairment, two other patients had mixed hearing impairment and 20 patients had conductive hearing impairment in the past, caused by otitis media with effusion. Their temporary conductive hearing impairment resolved between the ages of 2 and 18 years. Sensorineural hearing impairment varied between mild high-frequency hearing impairment and profound (uni- and bilateral) hearing impairment and was progressive in three patients. Four NS patients received cochlear implants for their severe sensorineural hearing impairment. The cohort is small for genotype-phenotype correlations, but sensorineural hearing impairment, especially the bilateral severe hearing impairment, was only seen in patients with a PTPN11 mutation. NS is characterized by dysmorphic external ear anomalies and both sensorineural and conductive hearing impairment. Audiological examinations are recommended in all patients with Noonan Syndrome. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

Gentamicin Exposure and Sensorineural Hearing Loss in Preterm Infants.

PubMed

Fuchs, Aline; Zimmermann, Lara; Bickle Graz, Myriam; Cherpillod, Jacques; Tolsa, Jean-François; Buclin, Thierry; Giannoni, Eric

2016-01-01

To evaluate the impact of gentamicin exposure on sensorineural hearing loss (SNHL) in very low birth weight (VLBW) infants. Exposure to gentamicin was determined in infants born between 1993 and 2010 at a gestational age < 32 weeks and/or with a birthweight < 1500 g, who presented with SNHL during the first 5 years of life. For each case, we selected two controls matched for gender, gestational age, birthweight, and year of birth. We identified 25 infants affected by SNHL, leading to an incidence of SNHL of 1.58% in our population of VLBW infants. The proportion of infants treated with gentamicin was 76% in the study group and 70% in controls (p = 0.78). The total cumulated dose of gentamicin administered did not differ between the study group (median 10.2 mg/kg, Q1-Q3 1.6-13.2) and the control group (median 7.9 mg/kg, Q1-Q3 0-12.8, p = 0.47). The median duration of gentamicin treatment was 3 days both in the study group and the control group (p = 0.58). Maximum predicted trough serum levels of gentamicin, cumulative area under the curve and gentamicin clearance were not different between cases and controls. The impact of gentamicin on SNHL can be minimized with treatments of short duration, monitoring of blood levels and dose adjustment.

Clinical and audiologic characteristics of patients with sensorineural tinnitus and its association with psychological aspects: an analytic retrospective study.

PubMed

Al-Swiahb, Jamil Nasser; Hwang, Eul Seung; Kong, Ji Sun; Kim, Woo Jin; Yeo, Sang Won; Park, Shi Nae

2016-12-01

This study was performed to analyze clinical and audiologic characteristics of sensorineural tinnitus and to investigate the associating factors reflecting psychological aspects of stress and depression of the patients. This is a retrospective analytical study conducted in a tinnitus clinic of a tertiary referral center of a university hospital. The medical records of 216 patients suffering from sensorineural tinnitus were thoroughly evaluated to determine correlations between clinical and audiological characteristics, including age, sex, predisposing or etiologic factors, hearing levels up to extended high frequencies, and tinnitus severity. Psychological aspects of stress and depression were also evaluated and analyzed to seek the associations with tinnitus severity. All data were stored in our database bank and were statistically analyzed. Our study subjects showed a slight male predominance. The highest percentage of tinnitus was found in patients of 60-80 years old. Only 32.5 % of tinnitus patients were subjectively aware of their hearing loss, whereas 73 % of subjects had hearing deficits in some frequencies in their audiogram. Hearing impairments were of the low-frequency sensorineural type in 18.2 % of patients and were limited to the high frequencies in 77.9 % of patients. Tinnitus was unilateral in 51 % of patients and had a tonal nature in 45 % of patients. In total, 45.8 % of patients with high-frequency sensorineural hearing loss had high-pitched tinnitus. There were significant correlations between tinnitus severity, loudness and annoyance. Correlations with THI (Tinnitus Handicap Inventory) and Beck depression index scores were also found. Sensorineural tinnitus was related with hearing loss in some frequencies nevertheless of patients' own awareness of hearing loss. Loudness and annoyance of tinnitus seems to be two important factors reflecting psychological problems of patients' stress and depression.

A multidisciplinary approach to paediatric hearing loss: programme at the centre for hearing intervention and language development, National University Hospital, Singapore.

PubMed

Lim, Lynne H Y

2008-12-01

The objective is to describe the multidisciplinary management programme at the National University Hospital (NUH) in Singapore for children with hearing impairment (HI). Over 99.95% of babies born at NUH have hearing tested with both otoacoustic emission and automated auditory brainstem response tests by 6 weeks of age. The referral rate to Otolaryngology is 0.5%. Acquired causes of congenital HI are decreasing. Thirty percent of patients at NUH with idiopathic congenital sensorineural HI have DFNB1/ GJB6 Connexin 26 HI. CT scan or MRI imaging has a higher diagnostic yield when there is unilateral, fluctuating or non-Connexin 26 related HI. Routine electrocardiogram and Opthalmology evaluations will exclude associations of fatal cardiac rhythm anomaly and retinopathy. Other investigations are directed by history and clinical examination. There is now a very wide range of increasingly sophisticated medication, neuro-otologic external, middle and inner ear surgery, hearing aids, middle ear implants and cochlear implants available to improve hearing. A multidisciplinary team from neonatology, paediatrics, otolaryngology, audiology, auditory verbal and speech therapy, ophthalmology, radiology, and psychology working closely with the child, family and schools is needed to develop a cost-effective and comprehensive management programme for paediatric HI.

"Gun-shooting hearing loss": A pilot study.

PubMed

Sataloff, Joseph; Hawkshaw, Mary J; Sataloff, Robert T

2010-01-01

Gun-shooting deafness is the common terminology applied to sensorineural hearing loss caused by shooting firearms. Many characteristics of gun-shooting hearing loss have been proposed, but they have not been defined clearly or established conclusively. We studied 37 users of recreational firearms to obtain pilot data to help determine if it is true that right-handed gun shooters develop more hearing loss in the left ear and vice versa, whether everyone who frequently shoots guns develops sensorineural hearing loss, and whether significant hearing loss is typically prevented by wearing commercially available ear protectors while shooting.

Cytomegalovirus induced sensorineural hearing loss with persistent cochlear inflammation in neonatal mice

PubMed Central

Schachtele, Scott J.; Mutnal, Manohar B.; Schleiss, Mark R.; Lokensgard, James R.

2011-01-01

Congenital cytomegalovirus (CMV) infection is the leading cause of sensorineural hearing loss (SNHL) in children. During murine (M)CMV-induced encephalitis, the immune response is important for both the control of viral dissemination and the clearance of virus from the brain. While the importance of CMV-induced SNHL has been described, the mechanisms surrounding its pathogenesis and the role of inflammatory responses remain unclear. This study presents a neonatal mouse model of profound SNHL in which MCMV preferentially infected both cochlear perilymphatic epithelial cells and spiral ganglion neurons. Interestingly, MCMV infection induced cochlear hair cell death by 21 days post-infection, despite a clear lack of direct infection of hair cells and the complete clearance of the virus from the cochlea by 14 dpi. Flow cytometric, immunohistochemical, and quantitative PCR analysis of MCMV-infected cochlea revealed a robust and chronic inflammatory response, including a prolonged increase in reactive oxygen species production by infiltrating macrophages. These data support a pivotal role for inflammation during MCMV-induced SNHL. PMID:21416394

Prelingual sensorineural hearing loss and infants at risk: Western Sicily report.

PubMed

Martines, Francesco; Martines, Enrico; Mucia, Marianna; Sciacca, Vincenzo; Salvago, Pietro

2013-04-01

To evaluate independent etiologic factor associated with sensorineural hearing loss (SNHL) in newborn at risk; to study the role of their interaction especially in NICU infants who present often multiple risk factors for SNHL. The main risk factors for SNHL reported by JCIH 2007 were evaluated on 508 infant at risk ranging from 4 to 20 weeks of life, transferred to the Audiology Department of Palermo from the main births centers of Western Sicily. After a global audiological assessment, performed with TEOAE, tympanometry and ABR, the prevalence and the effect of risk factors was statistically studied through univariate and multivariate analysis on the total population (normal and deaf subjects). Fifty-one infants (10.03%) were diagnosed with SNHL (45 bilateral and 6 monolateral) with a mean hearing threshold of 87.39 ± 28.25 dB HL; from logistic regression analysis family history of hearing impairment (HI) and TORCH infections resulted independent significant risk factors (P<0.00001 and P=0.024 respectively). High SNHL percentages were evidenced also in NICU babies, due to the various pathologies and risk factors presented by these infants, and among newborns who suffered from hyperbilirubinemia requiring exchange transfusion (11.97% and 9.52% respectively). Craniofacial abnormalities (CFA) and syndromes associated to HI showed an important relationship (P<0.00001) with conductive hearing loss (CHL). Multiple regression analysis of the variation in SNHL among NICU infants evidenced an increased risk for SNHL of 21.24% and of 19.33% respectively in preterm infants and in case of hyperbilirubinemia if respiratory distress is concomitant with these risk factors. It was also observed an higher risk of SNHL (99.66%) in case of coexistence of prematurity and hyperbilirubinemia. Finally among infants with very low birth weight (VLBW) it was evidenced a statistically difference between the mean weight of SNHL infants respect to NHL newborns (P=0.048). The high SNHL

Sensorineural deafness in congenital hypopituitarism with severe hypothyroidism.

PubMed

De Luca, F; Arrigo, T; Mangione, O A; Maiolino, M G; Muritano, M

1985-01-01

Marked sensorineural hearing loss was documented in an 18-year-old boy with untreated congenital anterior panhypopituitarism. The clinical manifestations of the hypothalamic thyroid failure were unusually severe in this patient. Seemingly this is the first case report of perceptive-cochlear deafness in a subject with non primary congenital hypothyroidism.

Gentamicin Exposure and Sensorineural Hearing Loss in Preterm Infants

PubMed Central

Fuchs, Aline; Zimmermann, Lara; Bickle Graz, Myriam; Cherpillod, Jacques; Tolsa, Jean-François; Buclin, Thierry; Giannoni, Eric

2016-01-01

Objective To evaluate the impact of gentamicin exposure on sensorineural hearing loss (SNHL) in very low birth weight (VLBW) infants. Methods Exposure to gentamicin was determined in infants born between 1993 and 2010 at a gestational age < 32 weeks and/or with a birthweight < 1500 g, who presented with SNHL during the first 5 years of life. For each case, we selected two controls matched for gender, gestational age, birthweight, and year of birth. Results We identified 25 infants affected by SNHL, leading to an incidence of SNHL of 1.58% in our population of VLBW infants. The proportion of infants treated with gentamicin was 76% in the study group and 70% in controls (p = 0.78). The total cumulated dose of gentamicin administered did not differ between the study group (median 10.2 mg/kg, Q1-Q3 1.6–13.2) and the control group (median 7.9 mg/kg, Q1-Q3 0–12.8, p = 0.47). The median duration of gentamicin treatment was 3 days both in the study group and the control group (p = 0.58). Maximum predicted trough serum levels of gentamicin, cumulative area under the curve and gentamicin clearance were not different between cases and controls. Conclusion The impact of gentamicin on SNHL can be minimized with treatments of short duration, monitoring of blood levels and dose adjustment. PMID:27390846

Congenital cytomegalovirus infection as a cause of sensorineural hearing loss in a highly immune population.

PubMed

Yamamoto, Aparecida Y; Mussi-Pinhata, Marisa Marcia; Isaac, Myriam de Lima; Amaral, Fabiana R; Carvalheiro, Cristina G; Aragon, Davi C; Manfredi, Alessandra K da Silva; Boppana, Suresh B; Britt, William J

2011-12-01

The burden of congenital cytomegalovirus (CMV)-associated sensorineural hearing loss (SNHL) in populations with CMV seroprevalence approaching 100% is unknown. The purpose of this study was to assess the rate, associated factors, and predictors of SNHL in CMV-infected infants identified by newborn screening in a highly seropositive maternal population. Newborns with positive saliva CMV-DNA that was confirmed by virus isolation in the first 2 weeks of life were enrolled in a prospective follow-up study to monitor hearing outcome. Of 12,195 infants screened, 121 (1%) were infected with CMV and 12 (10%) had symptomatic infection at birth. Hearing function could be assessed in 102/121 children who underwent at least one auditory brainstem evoked response testing at a median age of 12 months. SNHL was observed in 10/102 (9.8%; 95% confidence interval: 5.1-16.7) children. Median age at the latest hearing evaluation was 47 months (12-84 months). Profound loss (>90 dB) was found in 4/5 children with bilateral SNHL while all 5 children with unilateral loss had moderate to severe deficit. The presence of symptomatic infection at birth (odds ratio, 38.1; 95% confidence interval: 1.6-916.7) was independently associated with SNHL after adjusting for intrauterine growth restriction, gestational age, gravidity, and maternal age. Among 10 infants with SNHL, 6 (60%) were born to mothers with nonprimary CMV infection. Even in populations with near universal immunity to CMV, congenital CMV infection is a significant cause of SNHL demonstrating the importance of CMV as a major cause of SNHL in children worldwide. As in other populations, SNHL is more frequently observed in symptomatic CMV infection.

Sensorineural hearing loss in a pediatric population: association of congenital cytomegalovirus infection with intracranial abnormalities.

PubMed

Kimani, Jane W; Buchman, Craig A; Booker, Jessica K; Huang, Benjamin Y; Castillo, Mauricio; Powell, Cynthia M; Weck, Karen E

2010-10-01

To examine the incidence of congenital cytomegalovirus (CMV) infection relative to common genetic etiologies of hearing loss in a pediatric population with sensorineural hearing loss (SNHL), and to characterize intracranial radiological abnormalities in patients with CMV-associated hearing loss. Retrospective study. Academic tertiary care center. A total of 112 pediatric patients with confirmed SNHL. The association of congenital CMV infection status with abnormal brain magnetic resonance imaging (MRI) scans and the frequencies of congenital CMV infection, gap junction β-2 (GJB2) mutations, and the mitochondrial DNA (mtDNA) 1555A>G mutation in children with SNHL. Of 109 patients, 11 (10%) had positive results for CMV DNA; 10 of the 11 had normal GJB2 sequence and had negative test results for the mtDNA 1555A>G mutation. Brain MRI scans for 97 patients demonstrated a higher proportion of abnormalities in patients with positive CMV test results (80%) compared with those with no detectable CMV DNA (33%) (P = .006). GJB2 mutations and the mtDNA 1555A>G mutation were seen in 10 of 88 patients (11%) and 1 of 97 patients (1%) with SNHL, respectively. The presence of brain abnormalities in most patients with congenital CMV infection suggests that neurological damage in otherwise asymptomatic patients may not be limited to SNHL. Congenital CMV infection accounted for a significant proportion of patients with SNHL, with an incidence rate comparable with that of GJB2-related SNHL.

Sudden sensorineural hearing loss: subclinical viral and toxoplasmosis infections as aetiology and how they alter the clinical course.

PubMed

Kikidis, Dimitrios; Nikolopoulos, Thomas P; Kampessis, Georgios; Stamatiou, Georgios; Chrysovergis, Aristeidis

2011-01-01

To explore in a prospective study the evidence of certain viral and toxoplasmosis infections in sudden sensorineural hearing loss (SSHL). 84 consecutive patients with SSHL meeting certain criteria. All patients were assessed for specific IgM antibodies against cytomegalovirus, herpes simplex virus, toxoplasma and Epstein-Barr virus. All were treated with intravenous steroids and assigned to two groups: 76 IgM negative (NV group) and 8 IgM positive (no history of acute infection - V group). The mean hearing level at presentation was 86.5 dB HL (median, 100) in the V group and 60.7 dB HL (median, 61) in the NV group. The difference was statistically significant (p = 0.003). The mean hearing level following treatment was 81.8 dB HL (median, 88) in the V group and 48.7 dB HL (median, 39) in the NV group. The difference was statistically significant (p = 0.004). There was a considerable improvement in hearing after treatment only in the NV group (p < 0.000001). Recent subclinical viral or toxoplasmosis infections may be involved in the pathogenesis of SSHL (in approx. 10% of cases), suggesting that SSHL is not a single disease. When certain viruses or toxoplasmoses are involved, the hearing is much worse in comparison to patients with no such indication of infection. An alteration in treatment dosage or method of steroid administration may be needed in such cases. Copyright © 2011 S. Karger AG, Basel.

Brain activity in patients with unilateral sensorineural hearing loss during auditory perception in noisy environments.

PubMed

Yamamoto, Katsura; Tabei, Kenichi; Katsuyama, Narumi; Taira, Masato; Kitamura, Ken

2017-01-01

Patients with unilateral sensorineural hearing loss (UHL) often complain of hearing difficulties in noisy environments. To clarify this, we compared brain activation in patients with UHL with that of healthy participants during speech perception in a noisy environment, using functional magnetic resonance imaging (fMRI). A pure tone of 1 kHz, or 14 monosyllabic speech sounds at 65‒70 dB accompanied by MRI scan noise at 75 dB, were presented to both ears for 1 second each and participants were instructed to press a button when they could hear the pure tone or speech sound. Based on the activation areas of healthy participants, the primary auditory cortex, the anterior auditory association areas, and the posterior auditory association areas were set as regions of interest (ROI). In each of these regions, we compared brain activity between healthy participants and patients with UHL. The results revealed that patients with right-side UHL showed different brain activity in the right posterior auditory area during perception of pure tones versus monosyllables. Clinically, left-side and right-side UHL are not presently differentiated and are similarly diagnosed and treated; however, the results of this study suggest that a lateralityspecific treatment should be chosen.

Motivation to Address Self-Reported Hearing Problems in Adults with Normal Hearing Thresholds

ERIC Educational Resources Information Center

Alicea, Carly C. M.; Doherty, Karen A.

2017-01-01

Purpose: The purpose of this study was to compare the motivation to change in relation to hearing problems in adults with normal hearing thresholds but who report hearing problems and that of adults with a mild-to-moderate sensorineural hearing loss. Factors related to their motivation were also assessed. Method: The motivation to change in…

Autoimmune Associated Systemic Vasculitis as the Cause of Sudden onset Bilateral Sensorineural Hearing Loss Following Lassa Virus Exposure in a Cynomolgus Macaque Deafness Model

DTIC Science & Technology

2017-01-10

and IL-15 has been considered as a potential therapeutic target for autoimmune diseases such as rheumatoid arthritis and GPA (36, 41, 42...22(1):19-33. 41. Baslund B, et al. (2005) Targeting interleukin-15 in patients with rheumatoid arthritis : a proof-of-concept study. Arthritis Rheum...Berrettini S, et al. (1998) Progressive sensorineural hearing impairment in systemic vasculitides. Semin Arthritis Rheum 27(5):301-318. 27. Webb AA

A novel mutation of LHX3 is associated with combined pituitary hormone deficiency including ACTH deficiency, sensorineural hearing loss, and short neck-a case report and review of the literature.

PubMed

Bonfig, Walter; Krude, Heiko; Schmidt, Heinrich

2011-08-01

The LHX3 LIM-homeodomain transcription factor gene is required for normal pituitary and motoneuron development. LHX3 mutations are associated with growth hormone, prolactin, gonadotropin, and TSH deficiency; abnormal pituitary morphology; and may be accompanied with limited neck rotation and sensorineural hearing loss. We report on a boy, who presented with hypoglycemia in the newborn period. He is the second child of healthy unrelated parents. Short neck, growth hormone deficiency, and central hypothyroidism were diagnosed at a general pediatric hospital. Growth hormone and levothyroxine treatment were started, and blood sugar normalized with this treatment. On cerebral MRI, the anterior pituitary gland was hypoplastic. Sensorineural hearing loss was diagnosed by auditory testing. During follow-up, six repeatedly low morning cortisol levels (<1 μg/dl) and low ACTH levels (<10 pg/ml) were documented, so ACTH deficiency had developed over time and therefore hydrocortisone replacement was started at 1.5 years of age. Mutation analysis of the LHX3 gene revealed a homozygous stop mutation in exon 2: c.229C>T (CGA > TGA), Arg77stop (R77X). A complete loss of function is assumed with this homozygous stop mutation. We report a novel LHX3 mutation, which is associated with combined pituitary hormone deficiency including ACTH deficiency, short neck, and sensorineural hearing loss. All patients with LHX3 defects should undergo longitudinal screening for ACTH deficiency, since corticotrope function may decline over time. All patients should have auditory testing to allow for regular speech development.

Safety and otoprotection of metformin in radiation-induced sensorineural hearing loss in the guinea pig.

PubMed

Mujica-Mota, Mario A; Salehi, Pezhman; Devic, Slobodan; Daniel, Sam J

2014-05-01

There is currently no treatment available to prevent radiation-induced sensorineural hearing loss. Metformin has antineoplastic effects and is able to regulate the mitochondrial production of reactive oxygen species after cellular stress, which is one of the mechanisms involved in apoptosis after radiation damage. The objective of this study was to determine the safety and radioprotective properties of metformin against radiation-induced cochlear damage both in vitro and in vivo. In vitro and prospective animal study. Animal Care Facilities of the Montreal Children's Hospital Research Institute. Cultured auditory hair cells (HEI-OC1) were exposed to different concentrations of metformin to determine its safety. Cells were incubated with different metformin concentrations and subjected to radiation. Cell viability after experiments was determined with the 3-(4,5-dimethylthiazol-2-yl)-5-(3-carboxymethoxyphenyl)-2-(4-sulfophenyl)-2H-tetrazolium (MTS) assay. Sixteen guinea pigs were divided in 2 groups: drinking tap water (n = 8) and drinking water containing metformin (n = 8). The animals were unilaterally irradiated for 20 days (total dose 70 Gy), and the ears were divided in 4 groups: control (n = 8), irradiated (n = 8), metformin (n = 8), and experimental (n = 8). Auditory brainstem responses were assessed before and 1, 6, and 16 weeks after completion of radiotherapy. Metformin was not cytotoxic or radioprotective in cultured auditory hair cells. Experimental ears had less hearing loss than radiated ones; however, differences were not statistically significant (P > .05). Metformin is not ototoxic or radioprotective in vitro or in vivo. Ears solely subjected to metformin had better hearing thresholds than the rest of the groups.

Hearing loss in very preterm and very low birthweight infants at the age of 5 years in a nationwide cohort.

PubMed

Veen, S; Sassen, M L; Schreuder, A M; Ens-Dokkum, M H; Verloove-Vanhorick, S P; Brand, R; Grote, J J; Ruys, J H

1993-02-01

In a geographically defined population of very preterm and very low birthweight infants (gestational age < 32 weeks and/or birthweight < 1500 g) hearing was evaluated in 890 children by pure-tone audiometry at the age of 5 years. Hearing loss was conductive/unspecified in 123 (13.8%) and sensorineural in 13 (1.5%) children. The prevalence of sensorineural hearing loss was 15 times as high as in 5-7 year old children in the Dutch population at large. The sensorineural hearing loss prevalence in very low birthweight and extremely low birthweight infants was similar. On account of communication disorders 10 (1.1%) children were classified as disabled and 6 (0.7%) as handicapped, following the definitions of the International Classification of Impairments, Disabilities, and Handicaps of the World Health Organisation. Children with conductive hearing loss had a higher risk of impairments, disabilities and handicaps of language and speech development, than children with normal hearing, the difference being statistically significant. The same holds for children with sensorineural hearing loss; moreover they had a significantly higher risk of impairments, disabilities and handicaps of mental development. Overall comparison of children with and without sensorineural hearing loss proved that the children with sensorineural hearing loss had a significantly less favourable outcome, based on 15 perinatal factors simultaneously. The age at which sensorineural hearing loss in very preterm and/or very low birthweight infants is detected has to be improved.

The clinical analysis of bilateral successive sudden sensorineural hearing loss.

PubMed

Wang, Yaowen; Zhang, Litao; Zhang, Jianhua; Zhang, Xuqun; Zhang, Weimin; Chen, Xing; Tang, Shixiong

2016-11-01

The objective of this study is to investigate the clinical characteristics of successive bilateral sudden sensorineural hearing loss (SSNHL) with an interval of more than 1 year to aid the evaluation and management of successive bilateral SSNHL (BSSNHL). 14 successive BSSNHL patients and 118 unilateral SSNHL patients with severe and profound hearing loss were reviewed retrospectively. Information about successive BSSNHL was collected included demographics, the intervals between the attacks of bilateral ears, the past medical history, inducing factors, accompanying symptoms, pure-tone tests, blood tests, b-ultrasound examinations of vertebral artery and carotid artery, and medical interventions. And the comparison of improvement rate was made between successive BSSNHL and unilateral SSNHL. SPSS 15.0 was used to analyze the data. In successive BSSNHL, there were six males and eight females; the average aged was 49.86 ± 15.45 years (20-73 years). The interval of the two attacks was 11.43 ± 12.07 years (1-50 years) on average. The onset of treatment was 18.86 ± 12.71 days. Tinnitus was seen in 100 % of the patients, followed by vertigo in 42.85 %, and ear fullness in 21.43 %. 3 of 14 patients described obvious inducing factor: tiredness. Five patients (35.71 %) had hypertension histories, three (21.43 %) had diabetes histories, two (14.29 %) had surgery histories, one (7.14 %) was with depression history, one (7.14 %) was with coronary heart disease history. 30 % (3/10) patients were with atherosclerotic plaque in carotid artery. 4 (28.57 %) patients were with high blood sugar. 8 patients (57.14 %) were with high blood lipids. Thyroid function tests were positive in 27.27 % (3/11) patients. No abnormality was found in antinuclear antibodies titer. The percentage of profound and severe hearing loss were 71.43 and 78.57 % in the recently affected ear and contralateral ear separately. The PTAs of the recently affected ear were 77.14 ± 27.12�

Iloprost-induced sudden hearing loss.

PubMed

Dursun, E; Dogru, S; Cincik, H; Cekin, E; Gungor, A; Poyrazoglu, E

2007-06-01

We report a patient who developed sudden, bilateral, sensorineural hearing loss during therapeutic use of iloprost for Raynaud's phenomenon. The sudden hearing loss was attributed to iloprost use and completely reversed in eight days with conservative therapy. Iloprost may be a potentially ototoxic drug, causing sudden hearing loss.

Active Duty - U.S. Army Noise Induced Hearing Injury Surveillance Calendar Years 2009-2013

DTIC Science & Technology

2014-06-01

rates for sensorineural hearing loss, significant threshold shift, tinnitus , and Noise-Induced Hearing Loss. The intention is to monitor the morbidity...surveillance. These code groups include sensorineural hearing loss (SNHL), significant threshold shift (STS), noise-induced hearing loss (NIHL) and tinnitus ... Tinnitus ) was analyzed using a regression model to determine the trend of incidence rates from 2007 to the current year. Statistical significance of a

The Relationship between Serum Lipids and Sudden Sensorineural Hearing Loss: A Systematic Review and Meta-Analysis

PubMed Central

Chang, I Jen; Kang, Chung Jan; Yueh, Chen Yu; Fang, Ku Hao; Yeh, Re Ming; Tsai, Yao Te

2015-01-01

Background Sudden sensorineural hearing loss (SSNHL) is a relatively common condition that is usually of unknown etiology. A number of individual studies have investigated the association between various serum lipids and SSNHL; however, the findings have been inconsistent. In an attempt to obtain more definitive information on the relationship between serum lipids and SSNHL, we carried out a systematic review and meta-analysis. Methods Medline, the Cochrane Library, and EMBASE were searched using the following key words: lipid, cholesterol, triglyceride, fat, serum, blood, sudden hearing loss, hearing loss, hearing disorders. Randomized controlled trials, prospective cohort studies, and retrospective case-control studies involving patients with SSNHL and healthy controls that examined the relationship (reported as odds ratios [OR]) between lipid profiles and SSNHL were included. Primary outcomes were total cholesterol and low-density lipoprotein cholesterol (LDL-C) concentrations. Secondary outcomes were triglyceride, high-density lipoprotein cholesterol, and lipoprotein(a) concentrations. Results A total of 6 case-control studies were included in this systematic review/meta-analysis. The total number of participants ranged from 30 to 250 in the case group and from 43 to 271 in the control group. Meta-analysis revealed no significant difference in total cholesterol levels between the case and control groups (pooled OR = 1.79, 95% confidence interval [CI] = 0.98 to 3.26, P = 0.057). Likewise, meta-analysis revealed no significant difference in LDL-C concentrations between the case and control groups (pooled OR = 1.15, 95% CI = 0.64 to 2.07, P = 0.639). Since there were an insufficient number of studies reporting data for the secondary outcomes, meta-analysis was not possible. Conclusions Our results do not provide evidence for serum lipids being associated with SSNHL, nor do they definitively rule out such an association. Additional studies are needed to ascertain

Vestibular Evoked Myogenic Potential Produced by Bone-Conducted Stimuli: A Study on its Basics and Clinical Applications in Patients with Conductive and Sensorineural Hearing Loss and a Group with Vestibular Schawannoma.

PubMed

Mahdi, Parvane; Amali, Amin; Pourbakht, Akram; Karimi Yazdi, Alireza; Bassam, Ali

2013-06-01

Vestibular evoked myogenic potential (VEMP) has recently been broadly studied in vestibular disorders. As it is evoked by loud sound stimulation, even mild conductive hearing loss may affect VEMP results. Bone-conducted (BC) stimulus is an alternative stimulation for evoking this response. This study aims to assess the characteristics of BC-VEMP in different groups of patients. We performed a cross sectional analysis on 20 healthy volunteers with normal pure-tone audiometry as a control group; and on a group of patients consisted of 20 participants with conductive hearing loss, five with bilateral sensorineural hearing loss and four with vestibular schawannoma. AC and BC-VEMP were performed in all participants. In control group the VEMP responses to both kinds of stimuli had an acceptable morphology and consisted of p13 and n23 waves. Latency value of these main components in each type of stimulus was not significantly different (P>0.05). However, the mean amplitude was larger in BC modality than AC stimulation (P=0.025). In the group with conductive hearing loss, the VEMP response was absent in fifteen (46.87%) of the 32 ears using the AC method, whereas all (100%) displayed positive elicitability of VEMP by BC method. Normal VEMP responses in both stimuli were evoked in all patients with sensorineural hearing loss. In patients with unilateral vestibular schwannomas (VS), 2 (50.00%) had neither AC-VEMP nor BC-VEMP. Auditory stimuli delivered by bone conduction can evoke VEMP response. These responses are of vestibular origin and can be used in vestibular evaluation of patients with conductive hearing loss.

Managing Hearing Loss | NIH MedlinePlus the Magazine

MedlinePlus

... certain medications, or long-term exposure to loud noises. Sensorineural hearing loss occurs when there is damage ... Many people may have a combination of both noise-induced hearing loss and hearing loss from aging. ...

Longitudinal Development of Phonology and Morphology in Children with Late-Identified Mild-Moderate Sensorineural Hearing Loss

PubMed Central

Moeller, Mary Pat; McCleary, Elizabeth; Putman, Coille; Tyler-Krings, Amy; Hoover, Brenda; Stelmachowicz, Patricia

2010-01-01

Objective Studies of language development in children with mild-moderate hearing loss are relatively rare. Longitudinal studies of children with late-identified hearing loss have not been conducted, and they are relevant for determining how a period of unaided mild-moderate hearing loss impacts development. In recent years, newborn hearing screening programs have effectively reduced the ages of identification for most children with permanent hearing loss. However, some children continue to be identified late and research is needed to guide management decisions. Further, studies of this group may help to discern if language normalizes following intervention, and/or if certain aspects of language might be vulnerable to persistent delays. The current study examines the impact of late identification and reduced audibility on speech and language outcomes via a longitudinal study of four children with mild-moderate sensorineural hearing loss. Design Longitudinal outcomes of four children with late-identified mild-moderate sensorinueral hearing loss were studied using standardized measures and language sampling procedures, from at or near the point of identification (28 – 41 months) through 84 months of age. The children with hearing loss were compared to ten age-matched children with normal hearing on a majority of the measures through 60 months of age. Spontaneous language samples were collected from mother-child interaction sessions, recorded at consistent intervals in a laboratory-based play setting. Transcripts were analyzed using computer-based procedures (Systematic Analysis of Language Transcripts) and the Index of Productive Syntax. Possible influences of audibility were explored by examining the onset and productive use of a set of verb tense markers, and by monitoring the children’s accuracy in use of morphological endings. Phonological samples at baseline were transcribed and analyzed using Computerized Profiling. Results At entry to the study, the four

Ranking Hearing Aid Input-Output Functions for Understanding Low-, Conversational-, and High-Level Speech in Multitalker Babble

ERIC Educational Resources Information Center

Chung, King; Killion, Mead C.; Christensen, Laurel A.

2007-01-01

Purpose: To determine the rankings of 6 input-output functions for understanding low-level, conversational, and high-level speech in multitalker babble without manipulating volume control for listeners with normal hearing, flat sensorineural hearing loss, and mildly sloping sensorineural hearing loss. Method: Peak clipping, compression limiting,…

Sudden Sensorineural Hearing Loss in the Department of Defense.

PubMed

Hughes, Charlotte K; Fischer, Jakob; Esquivel, Carlos R; Laury, Adrienne M

2018-04-01

Objective The American Academy of Otolaryngology-Head and Neck Surgery Foundation clinical practice guideline (CPG) proposes recommendations regarding sudden sensorineural hearing loss (SSNHL). SSNHL is managed by primary care, emergency medicine, and otolaryngology providers in the Department of Defense (DoD). However, their adherence to this CPG is unknown. We sought to determine provider compliance and identify areas for improvement. Study Design Case series with chart review. Setting DoD's electronic medical record. Subjects and Methods Patients with SSNHL (N = 204) were treated between March 1, 2012, and September 30, 2015. Time from onset of symptoms to evaluation by primary care, emergency department, audiology, and otolaryngology providers and treatments were analyzed. Results The average interval from onset of symptoms to evaluation by a primary care or emergency department provider was 4.86 days (95% CI, 3.46-6.26). Time from presentation to ear, nose, and throat and audiologic evaluation was 15.26 days (95% CI, 12.34-18.20) and 14.16 days (95% CI, 11.31-17.01), respectively. Diagnostic workup included magnetic resonance imaging (n = 150, 73.5%), computed tomography (n = 28, 13.7%), and laboratory testing (n = 50, 24.5%). Oral steroids were used in 137 (67.2%) patients, with 78.8% treated with the recommended dose. Intratympanic steroids were utilized in 65 (31.9%) patients, with variable dosing. Conclusion The DoD is uniquely positioned to evaluate adherence to CPGs on national and international levels given the robust and standardized electronic medical record. Areas of improvement include timely identification of SSNHL with rapid referral to ear, nose, and throat and audiology providers; minimizing unnecessary imaging, laboratory testing, and medications; and correct dosing of oral and intratympanic steroids.

[Aberrant topological properties of whole-brain functional network in chronic right-sided sensorineural hearing loss: a resting-state functional MRI study].

PubMed

Zhang, Lingling; Liu, Bin; Xu, Yangwen; Yang, Ming; Feng, Yuan; Huang, Yaqing; Huan, Zhichun; Hou, Zhaorui

2015-02-03

To investigate the topological properties of the functional brain network in unilateral sensorineural hearing loss patients. In this study, we acquired resting-state BOLD- fMRI data from 19 right-sided SNHL patients and 31 healthy controls with normal hearing and constructed their whole brain functional networks. Two-sample two-tailed t-tests were performed to investigate group differences in topological parameters between the USNHL patients and the controls. Partial correlation analysis was conducted to determine the relationships between the network metrics and USNHL-related variables. Both USNHL patients and controls exhibited small-word architecture in their brain functional networks within the range 0. 1 - 0. 2 of sparsity. Compared to the controls, USNHL patients showed significant increase in characteristic path length and normalized characteristic path length, but significant decrease in global efficiency. Clustering coefficient, local efficiency and normalized clustering coefficient demonstrated no significant difference. Furthermore, USNHL patients exhibited no significant association between the altered network metrics and the duration of USNHL or the severity of hearing loss. Our results indicated the altered topological properties of whole brain functional networks in USNHL patients, which may help us to understand pathophysiologic mechanism of USNHL patients.

Speech-evoked auditory brainstem responses in children with hearing loss.

PubMed

Koravand, Amineh; Al Osman, Rida; Rivest, Véronique; Poulin, Catherine

2017-08-01

The main objective of the present study was to investigate subcortical auditory processing in children with sensorineural hearing loss. Auditory Brainstem Responses (ABRs) were recorded using click and speech/da/stimuli. Twenty-five children, aged 6-14 years old, participated in the study: 13 with normal hearing acuity and 12 with sensorineural hearing loss. No significant differences were observed for the click-evoked ABRs between normal hearing and hearing-impaired groups. For the speech-evoked ABRs, no significant differences were found for the latencies of the following responses between the two groups: onset (V and A), transition (C), one of the steady-state wave (F), and offset (O). However, the latency of the steady-state waves (D and E) was significantly longer for the hearing-impaired compared to the normal hearing group. Furthermore, the amplitude of the offset wave O and of the envelope frequency response (EFR) of the speech-evoked ABRs was significantly larger for the hearing-impaired compared to the normal hearing group. Results obtained from the speech-evoked ABRs suggest that children with a mild to moderately-severe sensorineural hearing loss have a specific pattern of subcortical auditory processing. Our results show differences for the speech-evoked ABRs in normal hearing children compared to hearing-impaired children. These results add to the body of the literature on how children with hearing loss process speech at the brainstem level. Copyright © 2017 Elsevier B.V. All rights reserved.

Reports of sensorineural deafness after measles, mumps, and rubella immunisation.

PubMed Central

Stewart, B J; Prabhu, P U

1993-01-01

There have been nine reports of sensorineural hearing loss after measles, mumps, and rubella (MMR) immunisation. In three cases the deafness was unrelated to MMR immunisation. In six cases the cause was unknown and MMR remained a possible aetiology. Any risk associated with attenuated viruses must be weighed against the risks of the natural diseases. PMID:8024302

Vestibular Evoked Myogenic Potential Produced by Bone-Conducted Stimuli: A Study on its Basics and Clinical Applications in Patients with Conductive and Sensorineural Hearing Loss and a Group with Vestibular Schawannoma

PubMed Central

Mahdi, Parvane; Amali, Amin; Pourbakht, Akram; Karimi Yazdi, Alireza; Bassam, Ali

2013-01-01

Introduction: Vestibular evoked myogenic potential (VEMP) has recently been broadly studied in vestibular disorders. As it is evoked by loud sound stimulation, even mild conductive hearing loss may affect VEMP results. Bone-conducted (BC) stimulus is an alternative stimulation for evoking this response. This study aims to assess the characteristics of BC-VEMP in different groups of patients. Materials and Methods: We performed a cross sectional analysis on 20 healthy volunteers with normal pure-tone audiometry as a control group; and on a group of patients consisted of 20 participants with conductive hearing loss, five with bilateral sensorineural hearing loss and four with vestibular schawannoma. AC and BC-VEMP were performed in all participants. Results: In control group the VEMP responses to both kinds of stimuli had an acceptable morphology and consisted of p13 and n23 waves. Latency value of these main components in each type of stimulus was not significantly different (P>0.05). However, the mean amplitude was larger in BC modality than AC stimulation (P=0.025). In the group with conductive hearing loss, the VEMP response was absent in fifteen (46.87%) of the 32 ears using the AC method, whereas all (100%) displayed positive elicitability of VEMP by BC method. Normal VEMP responses in both stimuli were evoked in all patients with sensorineural hearing loss. In patients with unilateral vestibular schwannomas (VS), 2 (50.00%) had neither AC-VEMP nor BC-VEMP. Conclusion: Auditory stimuli delivered by bone conduction can evoke VEMP response. These responses are of vestibular origin and can be used in vestibular evaluation of patients with conductive hearing loss. PMID:24303434

The Prevalence of Gap Junction Protein Beta 2 (GJB2) Mutations in Non Syndromic Sensorineural Hearing Loss in Çukurova Region.

PubMed

Bozdoğan, Sevcan Tuğ; Kuran, Gökhan; Yüregir, Özge Özalp; Aslan, Hüseyin; Haytoğlu, Süheyl; Ayaz, Akif; Arıkan, Osman Kürşat

2015-08-01

To date, studies in all populations showed that mutations in the gene of Gap junction protein beta 2 (GJB2) play an important role in non-syndromic autosomal recessive congenital hearing loss. The aim of this study was to evaluate GJB2 gene of patients with hearing loss in our region using deoxyribonucleic acid (DNA) sequencing method and to demonstrate region-specific mutation and polymorphism distribution. Patients who had bilateral severe sensorineural non-syndromic hearing loss identified by audiologic evaluation were included. Peripheral blood samples were collected and the GJB2 gene exon1 and exon 2 regions were amplified by polymerase chain reaction (PCR). Obtained PCR products were sequenced by the DNA sequence analysis method (SeqFinder Sequencing System; ABI 3130; Foster City, CA, USA) and analyzed using the SeqScape software. Of the 77 patients, 16 had homozygous or heterozygous mutation. The mutation of 35delG, which is known as the most frequent mutation of GJB2 gene, was also the most frequently seen mutation at a ratio of 5.5% in patients with hearing loss in our region; this was followed by the V27I mutation. As this is the first study conducted by sequence analysis in our region, it was worth to be presented in terms of showing the distribution of mutation.

Direct Reprogramming of Spiral Ganglion Non-neuronal Cells into Neurons: Toward Ameliorating Sensorineural Hearing Loss by Gene Therapy

PubMed Central

Noda, Teppei; Meas, Steven J.; Nogami, Jumpei; Amemiya, Yutaka; Uchi, Ryutaro; Ohkawa, Yasuyuki; Nishimura, Koji; Dabdoub, Alain

2018-01-01

Primary auditory neurons (PANs) play a critical role in hearing by transmitting sound information from the inner ear to the brain. Their progressive degeneration is associated with excessive noise, disease and aging. The loss of PANs leads to permanent hearing impairment since they are incapable of regenerating. Spiral ganglion non-neuronal cells (SGNNCs), comprised mainly of glia, are resident within the modiolus and continue to survive after PAN loss. These attributes make SGNNCs an excellent target for replacing damaged PANs through cellular reprogramming. We used the neurogenic pioneer transcription factor Ascl1 and the auditory neuron differentiation factor NeuroD1 to reprogram SGNNCs into induced neurons (iNs). The overexpression of both Ascl1 and NeuroD1 in vitro generated iNs at high efficiency. Transcriptome analyses revealed that iNs displayed a transcriptome profile resembling that of endogenous PANs, including expression of several key markers of neuronal identity: Tubb3, Map2, Prph, Snap25, and Prox1. Pathway analyses indicated that essential pathways in neuronal growth and maturation were activated in cells upon neuronal induction. Furthermore, iNs extended projections toward cochlear hair cells and cochlear nucleus neurons when cultured with each respective tissue. Taken together, our study demonstrates that PAN-like neurons can be generated from endogenous SGNNCs. This work suggests that gene therapy can be a viable strategy to treat sensorineural hearing loss caused by degeneration of PANs. PMID:29492404

Genes and Syndromic Hearing Loss.

ERIC Educational Resources Information Center

Keats, Bronya J. B.

2002-01-01

This article provides a description of the human genome and patterns of inheritance and discusses genes that are associated with some of the syndromes for which hearing loss is a common finding, including: Waardenburg, Stickler, Jervell and Lange-Neilsen, Usher, Alport, mitochondrial encephalomyopathy, and sensorineural hearing loss. (Contains…

Progressive Hearing Impairment in Children with Congenital Cytomegalovirus Infection.

ERIC Educational Resources Information Center

Dahle, Arthur J.; And Others

1979-01-01

Audiological assessment of 86 children (mean age 38 months at last evaluation time) with congenital cytomegalovirus infection revealed progressive hearing loss in four of 12 Ss with sensorineural hearing impairments. Case descriptions documented the progression of the hearing loss. (Author)

Risk factors for sensorineural hearing loss in children.

PubMed

Núñez-Batalla, Faustino; Trinidad-Ramos, Germán; Sequí-Canet, José Miguel; Alzina De Aguilar, Valentín; Jáudenes-Casaubón, Carmen

2012-01-01

In the last decade, tremendous progress has been made very rapidly in the development of Early Hearing Detection and Intervention (EHDI) systems as a major public health initiative. The percentage of infants screened annually in Spain has increased significantly since the EHDI systems have expanded to all autonomic regions. Historically, high risk indicators have been used for the identification of infants who should receive audiological evaluation but who live in geographic locations where universal hearing screening is not yet available, to help identify infants who pass neonatal screening but are at risk of developing delayed-onset hearing loss and to identify infants who may have passed neonatal screening but have mild forms of permanent hearing loss. In this review, the standard risk factors for hearing loss are analysed and the risk factors known to be associated with late onset or progressive hearing loss are identified. The recommendation for infants with a risk factor that may be considered as low risk is to perform at least one audiology assessment by 24-30 months. In contrast, for an infant with risk factors known to be associated with late onset or progressive hearing loss (such as cytomegalovirus infection or family history), early and more frequent assessment is appropriate. All infants should have an objective standardised screening of global development with a validated assessment tool at 9, 18 and 24-30 months of age or at any time if the health care professional or the family is concerned. Copyright © 2011 Elsevier España, S.L. All rights reserved.

Novel compound heterozygous mutations in MYO7A gene associated with autosomal recessive sensorineural hearing loss in a Chinese family.

PubMed

Ma, Yalin; Xiao, Yun; Zhang, Fengguo; Han, Yuechen; Li, Jianfeng; Xu, Lei; Bai, Xiaohui; Wang, Haibo

2016-04-01

Mutations in MYO7A gene have been reported to be associated with Usher Syndrome type 1B (USH1B) and nonsyndromic hearing loss (DFNB2, DFNA11). Most mutations in MYO7A gene caused USH1B, whereas only a few reported mutations led to DFNB2 and DFNA11. The current study was designed to investigate the mutations among a Chinese family with autosomal recessive hearing loss. In this study, we present the clinical, genetic and molecular characteristics of a Chinese family. Targeted capture of 127 known deafness genes and next-generation sequencing were employed to study the genetic causes of two siblings in the Chinese family. Sanger sequencing was employed to examine those variant mutations in the members of this family and other ethnicity-matched controls. We identified the novel compound heterozygous mutant alleles of MYO7A gene: a novel missense mutation c.3671C>A (p.A1224D) and a reported insert mutation c.390_391insC (p.P131PfsX9). Variants were further confirmed by Sanger sequencing. These two compound heterozygous variants were co-segregated with autosomal recessive hearing loss phenotype. The gene mutation analysis and protein sequence alignment further supported that the novel compound heterozygous mutations were pathogenic. The novel compound heterozygous mutations (c.3671C>A and c.390_391insC) in MYO7A gene identified in this study were responsible for the autosomal recessive sensorineural hearing loss of this Chinese family. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

Hearing Status in Pediatric Renal Transplant Recipients.

PubMed

Gulleroglu, Kaan; Baskin, Esra; Aydin, Erdinc; Ozluoglu, Levent; Moray, Gokhan; Haberal, Mehmet

2015-08-01

Renal transplant provides a long-term survival. Hearing impairment is a major factor in subjective health status. Status of hearing and the cause of hearing impairment in the pediatric renal transplant group have not been evaluated. Here, we studied to evaluate hearing status in pediatric renal transplant patients and to determine the factors that cause hearing impairment. Twenty-seven pediatric renal transplant recipients were investigated. All patients underwent audiologic assessment by means of pure-tone audiometry. The factors on hearing impairment were performed. Sensorineural hearing impairment was found in 17 patients. There was marked hearing impairment for the higher frequencies between 4000 and 8000 Hz. Sudden hearing loss developed in 2 patients, 1 of them had tinnitus. Decrease of speech understanding was found in 8 patients. The cyclosporine level was significantly high in patients with hearing impairment compared with group without hearing impairment. Cyclosporine levels also were found to be statistically significantly high when compared with the group with decrease of speech understanding and the group without decrease of speech understanding. Similar relations cannot be found between tacrolimus levels and hearing impairment and speech understanding. Sensorineural hearing impairment prevalence was high in pediatric renal transplant recipients when compared with the general population of children. Cyclosporine may be responsible for causing hearing impairment after renal transplant. We suggest that this effect is a dose-dependent toxicity.

Investigating the Impact of Hearing Aid Use and Auditory Training on Cognition, Depressive Symptoms, and Social Interaction in Adults With Hearing Loss: Protocol for a Crossover Trial.

PubMed

Nkyekyer, Joanna; Meyer, Denny; Blamey, Peter J; Pipingas, Andrew; Bhar, Sunil

2018-03-23

Sensorineural hearing loss is the most common sensory deficit among older adults. Some of the psychosocial consequences of this condition include difficulty in understanding speech, depression, and social isolation. Studies have shown that older adults with hearing loss show some age-related cognitive decline. Hearing aids have been proven as successful interventions to alleviate sensorineural hearing loss. In addition to hearing aid use, the positive effects of auditory training-formal listening activities designed to optimize speech perception-are now being documented among adults with hearing loss who use hearing aids, especially new hearing aid users. Auditory training has also been shown to produce prolonged cognitive performance improvements. However, there is still little evidence to support the benefits of simultaneous hearing aid use and individualized face-to-face auditory training on cognitive performance in adults with hearing loss. This study will investigate whether using hearing aids for the first time will improve the impact of individualized face-to-face auditory training on cognition, depression, and social interaction for adults with sensorineural hearing loss. The rationale for this study is based on the hypothesis that, in adults with sensorineural hearing loss, using hearing aids for the first time in combination with individualized face-to-face auditory training will be more effective for improving cognition, depressive symptoms, and social interaction rather than auditory training on its own. This is a crossover trial targeting 40 men and women between 50 and 90 years of age with either mild or moderate symmetric sensorineural hearing loss. Consented, willing participants will be recruited from either an independent living accommodation or via a community database to undergo a 6-month intensive face-to-face auditory training program (active control). Participants will be assigned in random order to receive hearing aid (intervention) for

Investigating the Impact of Hearing Aid Use and Auditory Training on Cognition, Depressive Symptoms, and Social Interaction in Adults With Hearing Loss: Protocol for a Crossover Trial

PubMed Central

Meyer, Denny; Blamey, Peter J; Pipingas, Andrew; Bhar, Sunil

2018-01-01

Background Sensorineural hearing loss is the most common sensory deficit among older adults. Some of the psychosocial consequences of this condition include difficulty in understanding speech, depression, and social isolation. Studies have shown that older adults with hearing loss show some age-related cognitive decline. Hearing aids have been proven as successful interventions to alleviate sensorineural hearing loss. In addition to hearing aid use, the positive effects of auditory training—formal listening activities designed to optimize speech perception—are now being documented among adults with hearing loss who use hearing aids, especially new hearing aid users. Auditory training has also been shown to produce prolonged cognitive performance improvements. However, there is still little evidence to support the benefits of simultaneous hearing aid use and individualized face-to-face auditory training on cognitive performance in adults with hearing loss. Objective This study will investigate whether using hearing aids for the first time will improve the impact of individualized face-to-face auditory training on cognition, depression, and social interaction for adults with sensorineural hearing loss. The rationale for this study is based on the hypothesis that, in adults with sensorineural hearing loss, using hearing aids for the first time in combination with individualized face-to-face auditory training will be more effective for improving cognition, depressive symptoms, and social interaction rather than auditory training on its own. Methods This is a crossover trial targeting 40 men and women between 50 and 90 years of age with either mild or moderate symmetric sensorineural hearing loss. Consented, willing participants will be recruited from either an independent living accommodation or via a community database to undergo a 6-month intensive face-to-face auditory training program (active control). Participants will be assigned in random order to receive

Hearing impairment in children with congenital cytomegalovirus (CMV) infection based on distortion product otoacoustic emissions (DPOAE) and brain evoked response audiometry stimulus click (BERA Click) examinations

NASA Astrophysics Data System (ADS)

Airlangga, T. J.; Mangunatmadja, I.; Prihartono, J.; Zizlavsky, S.

2017-08-01

Congenital cytomegalovirus (congenital CMV) infection is a leading factor of nongenetic sensorineural hearing loss in children. Hearing loss caused by CMV infection does not have a pathognomonic configuration hence further research is needed. The development of knowledge on hearing loss caused by congenital CMV infection is progressing in many countries. Due to a lack of research in the context of Indonesia, this study assesses hearing impairment in children with congenital CMV infection in Indonesia, more specifically in the Cipto Mangunkusumo Hospital. Our objective was to profile hearing impairment in children 0-5 years of age with congenital CMV infection using Distortion Product Otoacoustic Emissions (DPOAE) and Brain Evoked Response Audiometry Stimulus Click (BERA Click) examinations. This cross-sectional study was conducted in the Cipto Mangunkusum Hospital from November, 2015 to May 2016 with 27 children 0-5 years of age with congenital CMV infection. Of individual ears studied, 58.0% exhibited sensorineural hearing loss. There was a significant relationship between developmental delay and incidence of sensorineural hearing loss. Subjects with a developmental delay were 6.57 times more likely (CI 95%; 1.88-22.87) to experience sensorineural hearing loss. Congenital CMV infection has an important role in causing sensorineural hearing loss in children.

Autocorrelation factors and intelligibility of Japanese monosyllables in individuals with sensorineural hearing loss.

PubMed

Shimokura, Ryota; Akasaka, Sakie; Nishimura, Tadashi; Hosoi, Hiroshi; Matsui, Toshie

2017-02-01

Some Japanese monosyllables contain consonants that are not easily discernible for individuals with sensorineural hearing loss. However, the acoustic features that make these monosyllables difficult to discern have not been clearly identified. Here, this study used the autocorrelation function (ACF), which can capture temporal features of signals, to clarify the factors influencing speech intelligibility. For each monosyllable, five factors extracted from the ACF [Φ(0): total energy; τ 1 and ϕ 1 : delay time and amplitude of the maximum peak; τ e : effective duration; W ϕ (0) : spectral centroid], voice onset time, speech intelligibility index, and loudness level were compared with the percentage of correctly perceived articulations (144 ears) obtained by 50 Japanese vowel and consonant-vowel monosyllables produced by one female speaker. Results showed that median effective duration [(τ e ) med ] was strongly correlated with the percentage of correctly perceived articulations of the consonants (r = 0.87, p < 0.01). (τ e ) med values were computed by running ACFs with the time lag at which the magnitude of the logarithmic-ACF envelope had decayed to -10 dB. Effective duration is a measure of temporal pattern persistence, i.e., the duration over which the waveform maintains a stable pattern. The authors postulate that low recognition ability is related to degraded perception of temporal fluctuation patterns.

Radiation Therapy and Hearing Loss

DOE Office of Scientific and Technical Information (OSTI.GOV)

Bhandare, Niranjan; Jackson, Andrew; Eisbruch, Avraham

2010-03-01

A review of literature on the development of sensorineural hearing loss after high-dose radiation therapy for head-and-neck tumors and stereotactic radiosurgery or fractionated stereotactic radiotherapy for the treatment of vestibular schwannoma is presented. Because of the small volume of the cochlea a dose-volume analysis is not feasible. Instead, the current literature on the effect of the mean dose received by the cochlea and other treatment- and patient-related factors on outcome are evaluated. Based on the data, a specific threshold dose to cochlea for sensorineural hearing loss cannot be determined; therefore, dose-prescription limits are suggested. A standard for evaluating radiation therapy-associatedmore » ototoxicity as well as a detailed approach for scoring toxicity is presented.« less

[Analysis of mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes in patients with nonsyndromic sensorineural hearing loss from various regions of Russia].

PubMed

Dzhemileva, L U; Posukh, O L; Tazetdinov, A M; Barashkov, N A; Zhuravskiĭ, S G; Ponidelko, S N; Markova, T G; Tadinova, V N; Fedorova, S A; Maksimova, N R; Khusnutdinova, E K

2009-07-01

Mitochondrial DNA (mtDNA) mutations play an important role in etiology of hereditary hearing loss. In various regions of the world, patients suffer from nonsyndromic sensorineural hearing loss initiated by aminoglycoside antibiotics. Mutations that had been shown as pathogenetically important for hearing function disturbance were identified in mitochondrial 12S rRNA and tRNA(Ser(UCN)) genes while pathogenic role of several DNA sequences requires additional studies. This work presents the results of studying the spectrum of mutations and polymorphic variations in mtDNA genes 12S rRNA and tRNA(Ser(UGN)) in 410 patients with nonsyndromal sensoneural hearing impairment/loss from the Volga Ural region, St Petersburg, Yakutia, and Altai and in 520 individuals with normal hearing, which represent several ethnic groups (Russians, Tatars, Bashkirs, Yakuts, Altaians) residing in the Russian Federation. Pathogenetically significant mutation A1555G (12S rRNA) was found in two families (from Yakutia and St Peresburg) with hearing loss, probably caused by treatment with aminoglucosides, and in the population sample of Yakuts with a frequency of 0.83%. Further research is needed to confirm the role in hearing impairment of mutations 961insC, 961insC(n), 961delTinsC(n), T961G, T1095C (12S rRNA) and G7444A, A7445C (tRNA(Ser(UGN revealed in the patients. In addition, in the patients and the population groups, polymorphic mt DNA variants were detected, which are characteristic also of other Eurasian populations both in spectrum and frequency.

The combination of vestibular impairment and congenital sensorineural hearing loss predisposes patients to ocular anomalies, including Usher syndrome.

PubMed

Kletke, S; Batmanabane, V; Dai, T; Vincent, A; Li, S; Gordon, K A; Papsin, B C; Cushing, S L; Héon, E

2017-07-01

The co-occurrence of hearing impairment and visual dysfunction is devastating. Most deaf-blind etiologies are genetically determined, the commonest being Usher syndrome (USH). While studies of the congenitally deaf population reveal a variable degree of visual problems, there are no effective ophthalmic screening guidelines. We hypothesized that children with congenital sensorineural hearing loss (SNHL) and vestibular impairment were at an increased risk of having USH. A retrospective chart review of 33 cochlear implants recipients for severe to profound SNHL and measured vestibular dysfunction was performed to determine the ocular phenotype. All the cases had undergone ocular examination and electroretinogram (ERG). Patients with an abnormal ERG underwent genetic testing for USH. We found an underlying ocular abnormality in 81.81% (27/33) of cases; of which 75% had refractive errors, and 50% of those patients showed visual improvement with refractive correction. A total of 14 cases (42.42%; 14/33) had generalized rod-cone dysfunction on ERG suggestive of Usher syndrome type 1, confirmed by mutational analysis. This work shows that adding vestibular impairment as a criterion for requesting an eye exam and adding the ERG to detect USH increases the chances of detecting ocular anomalies, when compared with previous literature focusing only on congenital SNHL. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

Changes of the directional brain networks related with brain plasticity in patients with long-term unilateral sensorineural hearing loss.

PubMed

Zhang, G-Y; Yang, M; Liu, B; Huang, Z-C; Li, J; Chen, J-Y; Chen, H; Zhang, P-P; Liu, L-J; Wang, J; Teng, G-J

2016-01-28

Previous studies often report that early auditory deprivation or congenital deafness contributes to cross-modal reorganization in the auditory-deprived cortex, and this cross-modal reorganization limits clinical benefit from cochlear prosthetics. However, there are inconsistencies among study results on cortical reorganization in those subjects with long-term unilateral sensorineural hearing loss (USNHL). It is also unclear whether there exists a similar cross-modal plasticity of the auditory cortex for acquired monaural deafness and early or congenital deafness. To address this issue, we constructed the directional brain functional networks based on entropy connectivity of resting-state functional MRI and researched changes of the networks. Thirty-four long-term USNHL individuals and seventeen normally hearing individuals participated in the test, and all USNHL patients had acquired deafness. We found that certain brain regions of the sensorimotor and visual networks presented enhanced synchronous output entropy connectivity with the left primary auditory cortex in the left long-term USNHL individuals as compared with normally hearing individuals. Especially, the left USNHL showed more significant changes of entropy connectivity than the right USNHL. No significant plastic changes were observed in the right USNHL. Our results indicate that the left primary auditory cortex (non-auditory-deprived cortex) in patients with left USNHL has been reorganized by visual and sensorimotor modalities through cross-modal plasticity. Furthermore, the cross-modal reorganization also alters the directional brain functional networks. The auditory deprivation from the left or right side generates different influences on the human brain. Copyright © 2015 IBRO. Published by Elsevier Ltd. All rights reserved.

[Newborn hearing screening program: association between hearing loss and risk factors].

PubMed

Pereira, Priscila Karla Santana; Martins, Adriana de Souza; Vieira, Márcia Ribeiro; Azevedo, Marisa Frasson de

2007-01-01

Hearing loss in newborns. To verify the prevalence of auditory alterations in newborns of Hospital São Paulo (hospital), observing if there are any correlations with the following variables: birth weight, gestational age, relation weight/gestational age and risk factors for hearing loss. A retrospective analysis of the hospital records of 1696 newborns; 648 records of preterm infants and 1048 records of infants born at term. All of the infants had been submitted to an auditory evaluation consisting of: Transient Otoacoustic Emissions, investigation of the cochleal-palpebral reflexes and acoustic imittance tests, identifying the type and level of hearing loss. Sensorineural hearing loss was identified in .82% of the infants who were born at term and in 3.1% of the preterm infants -- with a statistically significant difference. Conductive hearing loss was the most frequent type of hearing loss in both groups, occurring in 14.6% of the term infants and in 16.3% of the preterm infants. Alteration of the central auditory system was considered as a possible diagnosis for 5.8% of the preterm infants and for 3.3% of the term infants. For the group of infants who were born at term, a significant correlation was observed between failure in the hearing screening test and the presence of risk factors such as family history and presence of a syndrome -- the child who presented a syndrome had 37 times more chances of failing in the hearing screening test and seven times more chances of failing in the right ear when there was a family history for hearing loss. The lower the gestational age (< 30 weeks) and birth weight (< 1500 g), the higher the chances of failing in the hearing screening test (3 times more). Hearing loss had a higher occurrence in preterm infants who remained in the ICU. Gestational age and birth weight were important variables related to the possibility of failure in the hearing screening test. A correlation was observed between the presence of a syndrome and

Binaural Loudness Summation in the Hearing Impaired.

ERIC Educational Resources Information Center

Hawkins, David B.; And Others

1987-01-01

Binaural loudness summation was measured using three different paradigms with 10 normally hearing and 20 bilaterally symmetrical high-frequency sensorineural hearing loss subjects. Binaural summation increased with presentation level using the loudness matching procedure, with values in the 6-10 dB range. Summation decreased with level using the…

Delayed diagnosis of a patient with Usher syndrome 1C in a Louisiana Acadian family highlights the necessity of timely genetic testing for the diagnosis and management of congenital hearing loss.

PubMed

Umrigar, Ayesha; Musso, Amanda; Mercer, Danielle; Hurley, Annette; Glausier, Cassondra; Bakeer, Mona; Marble, Michael; Hicks, Chindo; Tsien, Fern

2017-01-01

Advances in sequencing technologies and increased understanding of the contribution of genetics to congenital sensorineural hearing loss have led to vastly improved outcomes for patients and their families. Next-generation sequencing and diagnostic panels have become increasingly reliable and less expensive for clinical use. Despite these developments, the diagnosis of genetic sensorineural hearing loss still presents challenges for healthcare providers. Inherited sensorineural hearing loss has high levels of genetic heterogeneity and variable expressivity. Additionally, syndromic hearing loss (hearing loss and additional clinical abnormalities) should be distinguished from non-syndromic (hearing loss is the only clinical symptom). Although the diagnosis of genetic sensorineural hearing loss can be challenging, the patient's family history and ethnicity may provide critical information, as certain genetic mutations are more common in specific ethnic populations. The early identification of the cause of deafness can benefit patients and their families by estimating recurrence risks for future family planning and offering the proper interventions to improve their quality of life. Collaboration between pediatricians, audiologists, otolaryngologists, geneticists, and other specialists are essential in the diagnosis and management of patients with hearing disorders. An early diagnosis is vital for proper management and care, as some clinical manifestations of syndromic sensorineural hearing loss are not apparent at birth and have a delayed age of onset. We present a case of Usher syndrome (congenital deafness and childhood-onset blindness) illustrating the challenges encountered in the diagnosis and management of children presenting with congenital genetic sensorineural hearing loss, along with helpful resources for clinicians and families.

[Intratympanic injection therapy for therapy refractory acute hearing loss: A safe option for secondary treatment].

PubMed

Mühlmeier, G; Maier, S; Maier, M; Maier, H

2015-10-01

High-dose corticosteroids are currently recommended for idiopathic sudden sensorineural hearing loss (ISSNHL) treatment. Intratympanic injections (ITI) are of growing importance, especially in cases of therapy resistance. The selection of patients for this procedure in SSNHL has not been adequately examined so far. A total of 77 patients with ISSNHL after ineffective systemic pretreatment underwent intratympanic administration of dexamethasone and hyaluronic acid. Improvement after treatment was determined by pure tone audiometry for both ears before and of the treated ear after ITI. In this study 34 female and 43 male patients with mean age of 57 years showed a pre-ITI hearing loss of 35 dB in the lower frequencies and 69 dB in the higher frequencies. The mean hearing gain was 10 dB and the response rate was 62%. Absolute hearing gain revealed significant improvements at 500 Hz, 1 kHz and 2 kHz. Under inclusion of contralateral thresholds there were hardly any differences up to 4 kHz. In a detailed analysis of responders moderate improvements could be observed even in higher frequencies. Overall, no relevant adverse events occurred. Treatment of ISSNHL resistant to systemic regimens by ITI of steroids provides an option that offers additional prospects of auditory improvement for affected patients. The presented results indicate that these modalities are also valid for patients with pancochlear ISSNHL.

Interrupted Monosyllabic Words: The Effects of Ten Interruption Locations on Recognition Performance by Older Listeners with Sensorineural Hearing Loss.

PubMed

Wilson, Richard H; Sharrett, Kadie C

2017-01-01

Two previous experiments from our laboratory with 70 interrupted monosyllabic words demonstrated that recognition performance was influenced by the temporal location of the interruption pattern. The interruption pattern (10 interruptions/sec, 50% duty cycle) was always the same and referenced word onset; the only difference between the patterns was the temporal location of the on- and off-segments of the interruption cycle. In the first study, both young and older listeners obtained better recognition performances when the initial on-segment coincided with word onset than when the initial on-segment was delayed by 50 msec. The second experiment with 24 young listeners detailed recognition performance as the interruption pattern was incremented in 10-msec steps through the 0- to 90-msec onset range. Across the onset conditions, 95% of the functions were either flat or U-shaped. To define the effects that interruption pattern locations had on word recognition by older listeners with sensorineural hearing loss as the interruption pattern incremented, re: word onset, from 0 to 90 msec in 10-msec steps. A repeated-measures design with ten interruption patterns (onset conditions) and one uninterruption condition. Twenty-four older males (mean = 69.6 yr) with sensorineural hearing loss participated in two 1-hour sessions. The three-frequency pure-tone average was 24.0 dB HL and word recognition was ≥80% correct. Seventy consonant-vowel nucleus-consonant words formed the corpus of materials with 25 additional words used for practice. For each participant, the 700 interrupted stimuli (70 words by 10 onset conditions), the 70 words uninterrupted, and two practice lists each were randomized and recorded on compact disc in 33 tracks of 25 words each. The data were analyzed at the participant and word levels and compared to the results obtained earlier on 24 young listeners with normal hearing. The mean recognition performance on the 70 words uninterrupted was 91.0% with an

Hyponatremia and sensorineural hearing loss in preterm infants.

PubMed

Ertl, T; Hadzsiev, K; Vincze, O; Pytel, J; Szabo, I; Sulyok, E

2001-02-01

In a case-control study the role of hyponatremia in the hearing loss of preterm infants was investigated. One hundred and sixty-four premature infants treated at the neonatal intensive care unit were screened with transient evoked otoacoustic emission (TEAOE). In 32 infants TEAOE results indicated the need for further investigations. Auditory brainstem response was performed and 22 of 32 cases had bilateral hearing impairment (HI). The birth weight and gestational age in the HI group were 1,425 +/- 528 g and 30.4 +/- 3.7 weeks. The matched control group consisted of 25 infants with a mean birth weight and gestational age of 1,410 +/- 280 g and 31.1 +/- 2.1 weeks. Significant differences were found between the HI and control groups: Apgar score (p < 0.05), pH value (p < 0.01) and pO(2) level (p < 0.05) were lower; the total dose of aminoglycosides (p < 0.01), furosemide usage (p < 0.01), the maximum pCO(2) level (p < 0.01), incubator stay (p < 0.05) and hyponatremia (p < 0.01) were higher, and the duration of hyponatremia (p < 0.05) was longer in the HI group. Multivariate logistic regression revealed that aminoglycoside treatment and hyponatremia were the two most significant factors in the development of hearing impairment. These results suggest that hyponatraemia is an additional risk factor for hearing loss in preterm infants. Copyright 2001 S. Karger AG, Basel

Sensorineural deafness following routine transurethral resection of the prostate

PubMed Central

Bowsher, Benjamin

2015-01-01

A man in his 50s presented to a rural Australian emergency department with complete unilateral hearing loss following transurethral resection of the prostate. His hearing impairment progressed from ‘muffled hearing’ with tinnitus on emergence from anaesthesia, to total sensorineural deafness by day three. His surgery and anaesthesia were uncomplicated and he had remained normotensive throughout. He had no pre-existing auditory disease. He had received 240 mg of intravenous gentamicin intraoperatively for surgical prophylaxis. Renal function was normal. Brain imaging was negative for structural pathology, stroke and circulatory insufficiency. Ear nose and throat advised 7 days of oral corticosteroids, transtympanic dexamethasone and hyperbaric oxygen therapy. A working diagnosis of gentamicin-induced ototoxicity was applied. Intervention has proven unsuccessful and there is no possibility for rehabilitation. The patient is permanently disabled. PMID:26564118

Systematic review to evaluate the safety, efficacy and economical outcomes of the Vibrant Soundbridge for the treatment of sensorineural hearing loss.

PubMed

Bruchhage, Karl-Ludwig; Leichtle, Anke; Schönweiler, Rainer; Todt, Ingo; Baumgartner, Wolf-Dieter; Frenzel, Henning; Wollenberg, Barbara

2017-04-01

Introduced in the late 90s, the active middle ear implant Vibrant Soundbridge (VSB) is nowadays used for hearing rehabilitation in patients with mild to severe sensorineural hearing loss (SNHL) unable to tolerate conventional hearing aids. In experienced hands, the surgical implantation is fast done, safe and highly standardized. Here, we present a systematic review, after more than 15 years of application, to determine the efficacy/effectiveness and cost-effectiveness, as well as patient satisfaction with the VSB active middle ear implant in the treatment of mild to severe SNHL. A systematic search of electronic databases, investigating the safety and effectiveness of the VSB in SNHL plus medical condition resulted in a total of 1640 papers. After removing duplicates, unrelated articles, screening against inclusion criteria and after in-depth screening, the number decreased to 37 articles. 13 articles were further excluded due to insufficient outcome data. 24 studies remained to be systematically reviewed. Data was searched on safety, efficacy and economical outcomes with the VSB. Safety-oriented outcomes included complication/adverse event rates, damage to the middle/inner ear, revision surgery/explant rate/device failure and mortality. Efficacy outcomes were divided into audiological outcomes, including hearing thresholds, functional gain, speech perception in quiet and noise, speech recognition thresholds, real ear insertion gain and subjective outcomes determined by questionnaires and patient-oriented scales. Data related to quality of life (QALY, ICER) were considered under economical outcomes. The VSB turns out to be a highly reliable and a safe device which significantly improves perception of speech in noisy situations with a high sound quality. In addition, the subjective benefit of the VSB was found to be mostly significant in all studies. Finally, implantation with the VSB proved to be a cost-effective and justified health care intervention.

Profound hearing loss associated with hydrocodone/acetaminophen abuse.

PubMed

Friedman, R A; House, J W; Luxford, W M; Gherini, S; Mills, D

2000-03-01

To describe profound hearing loss associated with hydrocodone overuse and the successful rehabilitation of these patients with cochlear implantation. Retrospective review. A tertiary otologic referral center. Twelve patients with rapidly progressive hearing loss and a concurrent history of hydrocodone overuse. Comprehensive medical histories, physical findings, audiometric tests, and, in those patients undergoing cochlear implantation, postimplantation performance data were reviewed. Clinical characteristics of hydrocodone-related hearing loss and open set word and sentence performance in those patients undergoing cochlear implantation. Hydrocodone overuse was associated with rapidly progressive sensorineural hearing loss in 12 patients. In four patients the initial presentation was unilateral, and two of the patients experienced vestibular symptoms. None of the 12 patients experienced improved thresholds after high-dose prednisone. Seven of the eight patients undergoing cochlear implantation have demonstrated early success with their devices. Hydrocodone is frequently prescribed in combination with acetaminophen for the relief of pain and has a side effects profile similar to other medications in its class. Although not described previously, overuse or abuse can be associated with a rapidly progressive sensorineural hearing loss. These patients can be successfully rehabilitated with cochlear implantation.

Development of the stria vascularis and potassium regulation in the human fetal cochlea: Insights into hereditary sensorineural hearing loss

PubMed Central

de Groot, John C.M.J.; van Iperen, Liesbeth; Huisman, Margriet A.; Frijns, Johan H.M.

2015-01-01

ABSTRACT Sensorineural hearing loss (SNHL) is one of the most common congenital disorders in humans, afflicting one in every thousand newborns. The majority is of heritable origin and can be divided in syndromic and nonsyndromic forms. Knowledge of the expression profile of affected genes in the human fetal cochlea is limited, and as many of the gene mutations causing SNHL likely affect the stria vascularis or cochlear potassium homeostasis (both essential to hearing), a better insight into the embryological development of this organ is needed to understand SNHL etiologies. We present an investigation on the development of the stria vascularis in the human fetal cochlea between 9 and 18 weeks of gestation (W9–W18) and show the cochlear expression dynamics of key potassium‐regulating proteins. At W12, MITF+/SOX10+/KIT+ neural‐crest‐derived melanocytes migrated into the cochlea and penetrated the basement membrane of the lateral wall epithelium, developing into the intermediate cells of the stria vascularis. These melanocytes tightly integrated with Na+/K+‐ATPase‐positive marginal cells, which started to express KCNQ1 in their apical membrane at W16. At W18, KCNJ10 and gap junction proteins GJB2/CX26 and GJB6/CX30 were expressed in the cells in the outer sulcus, but not in the spiral ligament. Finally, we investigated GJA1/CX43 and GJE1/CX23 expression, and suggest that GJE1 presents a potential new SNHL associated locus. Our study helps to better understand human cochlear development, provides more insight into multiple forms of hereditary SNHL, and suggests that human hearing does not commence before the third trimester of pregnancy. © 2015 Wiley Periodicals, Inc. Develop Neurobiol 75: 1219–1240, 2015 PMID:25663387

Neurotrophins and electrical stimulation for protection and repair of spiral ganglion neurons following sensorineural hearing loss

PubMed Central

Shepherd, Robert K.; Coco, Anne; Epp, Stephanie B.

2008-01-01

Exogenous neurotrophins (NTs) have been shown to rescue spiral ganglion neurons (SGNs) from degeneration following a sensorineural hearing loss (SNHL). Furthermore, chronic electrical stimulation (ES) has been shown to retard SGN degeneration in some studies but not others. Since there is evidence of even greater SGN rescue when NT administration is combined with ES, we examined whether chronic ES can maintain SGN survival long after cessation of NT delivery. Young adult guinea pigs were profoundly deafened using ototoxic drugs; five days later they were unilaterally implanted with an electrode array and drug delivery system. Brain derived neurotrophic factor (BDNF) was continuously delivered to the scala tympani over a four week period while the animal simultaneously received ES via bipolar electrodes in the basal turn (i.e. turn 1) scala tympani. One cohort (n=5) received ES for six weeks (i.e. including a two week period after the cessation of BDNF delivery; ES6); a second cohort (n=5) received ES for 10 weeks (i.e. a six week period following cessation of BDNF delivery; ES10). The cochleae were harvested for histology and SGN density determined for each cochlear turn for comparison with normal hearing controls (n=4). The withdrawal of BDNF resulted in a rapid loss of SGNs in turns 2–4 of the deafened/BDNF-treated cochleae; this was significant as early as two weeks following removal of the NT when compared with normal controls (p<0.05). Importantly, there was not a significant reduction in SGNs in turn 1 (i.e. adjacent to the electrode array) two and six weeks after NT removal, as compared with normal controls. This result suggests that chronic ES can prevent the rapid loss of SGNs that occurs after the withdrawal of exogenous NTs. Implications for the clinical delivery of NTs are discussed. PMID:18243608

Effect of Antioxidant Vitamins as Adjuvant Therapy for Sudden Sensorineural Hearing Loss: Systematic Review Study.

PubMed

Ibrahim, Iman; Zeitouni, Anthony; da Silva, Sabrina Daniela

2018-06-22

Sudden sensorineural hearing loss (SSNHL) is an otological emergency of unknown etiology. Recent reports showed that antioxidant drugs can benefit patients with SSNHL. This study attempted to evaluate the effect of adding antioxidant vitamins as an adjuvant therapy alongside with corticosteroids. To evaluate the effects of the 3 major antioxidant vitamins (A, C, and E) as an adjuvant therapy, administered with corticosteroids, for the treatment of SSNHL in adult patients (≥18 years). MEDLINE, EMBASE, PubMed, Web of Science and Cochrane electronic databases from January 1, 1995, through September 25, 2017. Published studies of adult patients who received antioxidant vitamins (A, C, E, or any combination of these vitamins) as an adjuvant therapy in addition to the regular treatment (corticosteroids) for SSNHL. Quality assessment was performed using the Cochrane Collaboration Tool for Assessing Risk of Bias. Each study had a control group (conventional treatment + placebo) and a trial group (antioxidant vitamin(s) + conventional treatment). From 446 manuscripts identified in the literature, 3 studies were included in the review with 279 patients. The most common vitamins used to treat SSNHL were the 3 major antioxidant vitamins A, C, and E, combined sometimes with other antioxidants such as selenium. The success of the treatment is increased in patients who received antioxidant vitamins in combination with conventional therapy. © 2018 S. Karger AG, Basel.

Extended high frequency audiometry can diagnose sub-clinic involvement in a seemingly normal hearing systemic lupus erythematosus population.

PubMed

Lasso de la Vega, Mar; Villarreal, Ithzel María; López Moya, Julio; García-Berrocal, José Ramón

2017-02-01

Sensorineural hearing loss must be considered within the clinical picture of systemic lupus erythematosus. The results confirm the usefulness of extended high-frequency audiometry in the audiologic testing of these patients, enabling the possibility of modifying or applying a preventive treatment for a possible hearing loss. Hearing involvement is usually under-diagnosed with routine auditory examination. This study proposes the use of extended high-frequency audiometry to achieve a correct detection of a possible asymptomatic hypoacusis in early stages of the disease. The aim of this study is to analyze the hearing levels in extended high-frequencies in these patients and to correlate the hearing loss with the severity of the disease and the immunological parameters. A descriptive cross-sectional study was performed. Fifty-five patients with systemic lupus erythematosus were included in the study. The control group consisted of 71 patients paired by age and sex with the study population. Both a pure tone audiometry and an extended high-frequency audiometry (8-18 KHz) were performed. In total, 70% were diagnosed with sensorineural hearing loss with extended high-frequency audiometry, overcoming the results obtained with pure tone audiometry (30.9%). Statistically significant correlations were found within the patients regarding sensorineural hearing loss related with age, disease activity and cryoglobulinemia.

Effects of sensorineural hearing loss on temporal coding of harmonic and inharmonic tone complexes in the auditory nerve

PubMed Central

Kale, Sushrut; Micheyl, Christophe; Heinz, Michael G.

2013-01-01

Listeners with sensorineural hearing loss (SNHL) often show poorer thresholds for fundamental-frequency (F0) discrimination, and poorer discrimination between harmonic and frequency-shifted (inharmonic) complex tones, than normal-hearing (NH) listeners—especially when these tones contain resolved or partially resolved components. It has been suggested that these perceptual deficits reflect reduced access to temporal-fine-structure (TFS) information, and could be due to degraded phase-locking in the auditory nerve (AN) with SNHL. In the present study, TFS and temporal-envelope (ENV) cues in single AN-fiber responses to bandpass-filtered harmonic and inharmonic complex tones were measured in chinchillas with either normal hearing or noise-induced SNHL. The stimuli were comparable to those used in recent psychophysical studies of F0 and harmonic/inharmonic discrimination. As in those studies, the rank of the center component was manipulated to produce different resolvability conditions, different phase relationships (cosine and random phase) were tested, and background noise was present. Neural TFS and ENV cues were quantified using cross-correlation coefficients computed using shuffled cross-correlograms between neural responses to REF (harmonic) and TEST (F0- or frequency-shifted) stimuli. In animals with SNHL, AN-fiber tuning curves showed elevated thresholds, broadened tuning, best-frequency shifts, and downward shifts in the dominant TFS response component; however, no significant degradation in the ability of AN fibers to encode TFS or ENV cues was found. Consistent with optimal-observer analyses, the results indicate that TFS and ENV cues depended only on the relevant frequency shift in Hz and thus were not degraded because phase-locking remained intact. These results suggest that perceptual “TFS-processing” deficits do not simply reflect degraded phase-locking at the level of the AN. To the extent that performance in F0 and harmonic

A software tool for pure‑tone audiometry. Classification of audiograms for inclusion of patients in clinical trials. English version.

PubMed

Rahne, T; Buthut, F; Plößl, S; Plontke, S K

2016-03-01

Selecting subjects for clinical trials on hearing loss therapies relies on the patient meeting the audiological inclusion criteria. In studies on the treatment of idiopathic sudden sensorineural hearing loss, the patient's acute audiogram is usually compared with a previous audiogram, the audiogram of the non-affected ear, or a normal audiogram according to an ISO standard. Generally, many more patients are screened than actually fulfill the particular inclusion criteria. The inclusion criteria often require a calculation of pure-tone averages, selection of the most affected frequencies, and calculation of hearing loss differences. A software tool was developed to simplify and accelerate this inclusion procedure for investigators to estimate the possible recruitment rate during the planning phase of a clinical trial and during the actual study. This tool is Microsoft Excel-based and easy to modify to meet the particular inclusion criteria of a specific clinical trial. The tool was retrospectively evaluated on 100 patients with acute hearing loss comparing the times for classifying automatically and manually. The study sample comprised 100 patients with idiopathic sudden sensorineural hearing loss. The age- and sex-related normative audiogram was calculated automatically by the tool and the hearing impairment was graded. The estimated recruitment rate of our sample was quickly calculated. Information about meeting the inclusion criteria was provided instantaneously. A significant reduction of 30 % in the time required for classifying (30 s per patient) was observed.

Acquisition of who-question comprehension in German children with hearing loss.

PubMed

Wimmer, Eva; Rothweiler, Monika; Penke, Martina

2017-05-01

For children with sensorineural hearing loss the ability to understand wh-questions might be particularly challenging because they often have only restricted access to spoken language input during optimal periods of language acquisition. In previous research it has been suggested that this restricted input during critical stages in language acquisition might lead to syntactic deficits that persist into adolescence. In this study we want to pursue this issue by investigating the comprehension of wh-questions in German children with bilateral sensorineural hearing loss. We report results of a who-question comprehension task in a group of 21 3- to 4-year-old German hard-of-hearing children compared to a group of age-matched children with normal hearing. The group data and individual performance patterns suggest that the syntactic comprehension difficulties observed in some, but not all, of the children with hearing loss reflect a delay in the acquisition of who-question comprehension rather than a persistent syntactic deficit. Follow-up data elicited from a subgroup of children confirm this supposition. Copyright © 2017 Elsevier Inc. All rights reserved.

Perception of Suprasegmental Features of Speech by Children with Cochlear Implants and Children with Hearing Aids

ERIC Educational Resources Information Center

Most, Tova; Peled, Miriam

2007-01-01

This study assessed perception of suprasegmental features of speech by 30 prelingual children with sensorineural hearing loss. Ten children had cochlear implants (CIs), and 20 children wore hearing aids (HA): 10 with severe hearing loss and 10 with profound hearing loss. Perception of intonation, syllable stress, word emphasis, and word pattern…

Electromagnetic semi-implantable hearing device: phase I. Clinical trials.

PubMed

McGee, T M; Kartush, J M; Heide, J C; Bojrab, D I; Clemis, J D; Kulick, K C

1991-04-01

Conventional hearing aids have improved significantly in recent years; however, amplification of sound within the external auditory canal creates a number of intrinsic problems, including acoustic feedback and the need for a tight ear mold to increase usable gain. Nonacoustic alternatives which could obviate these encumbrances have not become practical due to inefficient coupling (piezoelectric techniques) or unfeasible power requirements (electromagnetic techniques). Recent technical advances, however, prompted a major clinical investigation of a new electromagnetic, semi-implantable hearing device. This study presents the details of clinical phase I, in which an electromagnetic driver was coupled with a target magnet temporarily affixed onto the lateral surface of the malleus of six hearing aid users with sensorineural losses. The results indicate that the electromagnetic hearing device provides sufficient gain and output characteristics to benefit individuals with sensorineural hearing loss. Significant improvements compared to conventional hearing aids were noted in pure-tone testing and, to a lesser degree, in speech discrimination. Subjective responses were quite favorable, indicating that the electromagnetic hearing device 1. produces no acoustic feedback; 2. works well in noisy environments; and 3. provides a more quiet, natural sound than patients' conventional hearing aids. These favorable results led to phase II of the project, in which patients with surgically amendable mixed hearing losses were implanted with the target magnet incorporated within a hydroxyapatite ossicular prosthesis. The results of this second-stage investigation were also encouraging and will be reported separately.

Temporal Intraspeech Masking of Plosive Bursts: Effects of Hearing Loss and Frequency Shaping

ERIC Educational Resources Information Center

Mackersie, Carol L.

2007-01-01

Purpose: The purposes were (a) to compare masking of consonant bursts by adjacent vowels for listeners with and without hearing loss and (b) to determine the extent to which the temporal intraspeech masking can be reduced by a simulated hearing-aid frequency-response shaping. Method: Fourteen adults with sensorineural hearing loss and 10 with…

Role of worry in patients with chronic tinnitus and sensorineural hearing loss: a preliminary study.

PubMed

Caldirola, Daniela; Teggi, Roberto; Daccò, Silvia; Sangiorgio, Erika; Bussi, Mario; Perna, Giampaolo

2016-12-01

Tinnitus-related distress appears to be more strongly associated with multiple psychological factors than with any perceptual properties of tinnitus. Prior studies have not investigated the role of worry in tinnitus sufferers. Worry is a dispositional cognitive trait that involves a pervasive, non-specific, future-oriented proneness to fretting, which can foster negative affective states and catastrophic thinking about a specific trouble when the trouble is actual and present. We examined the relationship between worry and self-perceived anxiety and depressive symptoms and handicap in 54 outpatients with chronic tinnitus and sensorineural hearing loss who had been previously recruited for a randomized double-blind study on the efficacy of transmeatal low-level laser therapy for tinnitus. We measured the current anxiety and depressive symptoms with the State-Trait Anxiety Inventory Form Y-1/Self-evaluation Depression Scale, the handicap with the Tinnitus Handicap Inventory, and the proneness to worry with the Penn State Worry Questionnaire. For the psychoacoustic tinnitus measures, we considered the loudness match and the minimum masking level. We found that tinnitus-related anxiety and depressive symptoms and handicap were significantly associated with proneness to worry (linear regression models, p < 0.01), whereas no associations were found with the psychoacoustic measures. This suggests the usefulness of worry assessment when managing chronic tinnitus in clinical practice. Early therapeutic interventions for reducing proneness to worry may facilitate better adaptation to tinnitus.

Mutational screening in patients with profound sensorineural hearing loss and neurodevelopmental delay: Description of a novel m.3861A > C mitochondrial mutation in the MT-ND1 gene.

PubMed

Ammar, Marwa; Tabebi, Mouna; Sfaihi, Lamia; Alila-Fersi, Olfa; Maalej, Marwa; Felhi, Rahma; Chabchoub, Imen; Keskes, Leila; Hachicha, Mongia; Fakhfakh, Faiza; Mkaouar-Rebai, Emna

2016-06-10

Mitochondrial diseases caused by mitochondrial dysfunction are a clinically and genetically, heterogeneous group of disorders involving multiple organs, particularly tissues with high-energy demand. Hearing loss is a recognized symptom of a number of mitochondrial diseases and can result from neuronal or cochlear dysfunction. The tissue affected in this pathology is most probably the cochlear hair cells, which are essential for hearing function since they are responsible for maintaining the ionic gradients necessary for sound signal transduction. Several mitochondrial DNA mutations have been associated with hearing loss and since mitochondria are crucial for the cellular energy supply in many tissues, most of these mtDNA mutations affect several tissues and will cause syndromic hearing loss. In the present study, we described 2 patients with sensorineural hearing loss and neurodevelopmental delay in whom we tested mitochondrial genes described to be associated with syndromic hearing loss. One of these patients showed a novel heteroplasmic mitochondrial mutation m.3861A > C (W185C) which lead to a loss of stability of the ND1 protein since it created a new hydrogen bund between the unique created cystein C185 and the A182 residue. In the second patient, we detected two novel heteroplasmic variations m.12350C > A (T5N) and m.14351T > C (E108G) respectively in the MT-ND5 and the MT-ND6 genes. The TopPred II prediction for the E108G variation revealed a decrease of the hydrophobicity in the mutated MT-ND6. Copyright © 2016 Elsevier Inc. All rights reserved.

Munchausen Syndrome by Proxy: Mother Fabricates Infant's Hearing Impairment.

ERIC Educational Resources Information Center

Kahn, Gerri; Goldman, Ellen

1991-01-01

Case study reports a case of Munchausen Syndrome by Proxy, a form of child abuse in which the mother presents a child for treatment for a condition she herself has invented or created. This case study describes the ways in which a mother obtained a diagnosis of sensorineural hearing loss as well as amplification for her normally hearing infant.…

Working Memory, Sleep, and Hearing Problems in Patients with Tinnitus and Hearing Loss Fitted with Hearing Aids.

PubMed

Zarenoe, Reza; Hällgren, Mathias; Andersson, Gerhard; Ledin, Torbjörn

2017-02-01

Tinnitus is a common condition and there is a need to evaluate effects of tinnitus management in relation to moderating factors such as degree of hearing loss. As it is possible that tinnitus influences concentration, and thus is likely to disturb cognitive processing, the role of cognitive functioning also needs to be investigated. To compare a group of patients with sensorineural hearing loss and tinnitus to a control group with only sensorineural hearing loss (and no tinnitus). To investigate working memory, sleep, and hearing problems measured before and after hearing rehabilitation. A prospective study. The sample consisted of 100 patients, 50 with hearing loss and tinnitus, and 50 controls with hearing loss but no tinnitus. All patients were between 40 and 82 yr old and had a pure-tone average (PTA; average of 0.5, 1, 2, and 4 kHz) <70 dB HL. Patients were tested before and after rehabilitation with hearing aids with regard to their working memory capacity, sleep quality, hearing problems, speech recognition, and tinnitus annoyance. Eight patients dropped out of the study. Thus, a total of 92 patients were included for analysis, with 46 in each group. As a consequence of unplanned age and PTA differences between the groups, an age-matched subsample (n = 30 + 30) was selected for further analysis. Tests including the Reading Span, Hearing-in-Noise Test (HINT), Tinnitus Handicap Inventory (THI), Hearing Handicap Inventory for the Elderly (HHIE), and Pittsburgh Sleep Quality Index (PSQI) were administered before and after hearing aid rehabilitation. There were no between-group differences at baseline in the full sample (n = 92), with the exception of the THI (p < 0.001) and the PSQI (p < 0.002), on which the hearing loss and tinnitus group had significantly higher scores. Pre/post changes were significant for both groups on the Reading Span, and HHIE. However, these improvements were significantly larger for the patients in the hearing loss and tinnitus group on

Evaluation of Extended-Wear Hearing Technology for Children with Hearing Loss.

PubMed

Wolfe, Jace; Schafer, Erin; Martella, Natalie; Morais, Mila; Mann, Misty

2015-01-01

Research shows that many older children and teenagers who have mild to moderately severe sensorineural hearing loss do not use their hearing instruments during all waking hours. A variety of reasons may contribute toward this problem, including concerns about cosmetics associated with hearing aid use and the inconvenience of daily maintenance associated with hearing instruments. Extended-wear hearing instruments are inserted into the wearer's ear canal by an audiologist and are essentially invisible to outside observers. The goal of this study was to evaluate the potential benefits and limitations associated with use of extended-wear hearing instruments in a group of children with hearing loss. A two-way repeated measures design was used to examine performance differences obtained with the participants' daily-wear hearing instruments versus that obtained with extended-wear hearing instruments. Sixteen children, ages 10-17 yr old, with sensorineural hearing loss ranging from mild to moderately severe. Probe microphone measures were completed to evaluate the aided output of device. Behavioral test measures included word recognition in quiet, sentence recognition in noise, aided warble-tone thresholds, and psychophysical loudness scaling. Questionnaires were also administered to evaluate subjective performance with each hearing technology. Data logging suggested that many participants were not using their daily-wear hearing instruments during all waking hours (mean use was less than 6 h/day). Real ear probe microphone measurements indicated that a closer fit to the Desired Sensation Level Version 5 prescriptive targets was achieved with the children's daily-wear instruments when compared to the extended-wear instruments. There was no statistically significant difference in monosyllabic word recognition at 50 or 60 dBA obtained with the two hearing technologies. Sentence recognition in noise obtained with use of the extended-wear devices was, however, significantly

Auditory Sequential Organization among Children with and without a Hearing Loss.

ERIC Educational Resources Information Center

Jutras, Benoit; Gagne, Jean-Pierre

1999-01-01

Forty-eight children, either with or without a sensorineural hearing loss and either young (6 and 7 years old) or older (9 and 10 years old) reproduced sequences of acoustic stimuli that varied in number, temporal spacing, and type. Results suggested that the poorer performance of the hearing-impaired children was due to auditory processing…

Experimental autoimmune hearing loss

PubMed Central

Billings, Peter

2004-01-01

Understanding of autoimmune sensorineural hearing loss (ASNHL) has been hindered by the inaccessibility of the inner ear to biopsy and the lack of workable animal models. A report in this issue of the JCI describes a mouse model of CD4+ T cell–mediated ASNHL induced by immunization with peptides from the inner ear–specific proteins cochlin and β-tectorin. PMID:15085190

Effects of Smoking on Eustachian Tube and Hearing.

PubMed

Pezzoli, Matteo; Lofaro, Denise; Oliva, Alessandro; Orione, Monica; Cupi, Daniela; Albera, Andrea; Bongioannini, Guido; Albera, Roberto

2017-12-01

The purpose of this study was to evaluate the effect of tobacco use on the Eustachian tube and inner ear function. Case-control study. Thirty-one nonsmoking volunteers and 34 smoking subjects recruited in an University Hospital, submitted to an audiological evaluation including pure tone audiometry, basal tympanogram, stapedial reflexes analysis, and nine-step eustachian tube (ET) function test. Pure Tone Average (PTA) threshold at all frequencies tested was 12.5 dB in smokers and 3.7 in nonsmoking subjects. Nine smokers (27%) presented some degree of hearing loss versus none in the nonsmoker group. Linear regression analysis showed a higher degree of sensorineural hearing loss with age in smokers. Among the smokers, 20 subjects (59%) presented an impaired tubal function for the nine-step inflation/deflation tympanometric test, while only 6 (19%) subjects in the group of nonsmokers showed a tubal dysfunction. Tobacco use may reduce the ability to hear, mainly causing a sensorineural hearing loss for higher frequencies. We also found the presence of a high number of smokers suffering from tubal dysfunction. This has an important clinical relevance, not only because smoking increases the incidence of middle ear diseases, but also because tubal dysfunction may cause nonspecific symptoms characterised by ear fullness and difficulties in middle ear equalisation.

Hearing loss in a glue sniffer.

PubMed

Williams, D M

1988-10-01

A case is presented of a 27-year-old glue sniffing woman with sensorineural hearing loss, optic atrophy and global brain damage. This form of addiction has not received much attention as a cause of otologic catastrophes, and should be borne in mind where similar cases come to the otolaryngologist.

Association Between Childhood Hearing Disorders and Tinnitus in Adulthood.

PubMed

Aarhus, Lisa; Engdahl, Bo; Tambs, Kristian; Kvestad, Ellen; Hoffman, Howard J

2015-11-01

The association between childhood hearing disorders and adult tinnitus has not been examined in longitudinal cohort studies. To determine the association between different types of childhood hearing loss and tinnitus in adulthood and evaluate whether tinnitus risk is mediated by adult hearing loss. Population-based cohort study of 32 430 adults (aged 20-56 years) who underwent pure-tone audiometry and completed a tinnitus questionnaire in the Nord-Trøndelag Hearing Loss Study, which was a part of the Nord-Trøndelag Health Study 2 (HUNT2). The study was conducted from January 1, 2014, to April 1, 2015. Data analysis was performed from April 1, 2014, to April 1, 2015. As children, the same individuals had undergone screening audiometry in a longitudinal primary school hearing investigation, including ear, nose, and throat examinations when indicated. Pure-tone audiometry, questionnaires, and ear, nose, and throat examinations. Self-reported tinnitus (yes or no) in adulthood measured by questionnaires. Adults who had hearing loss at the time of the school investigation (n = 3026) reported more tinnitus, measured as odds ratio (95% CI), than did adults with normal childhood hearing (n = 29 404) (1.4 [1.3-1.6]). Childhood hearing disorders associated with tinnitus in adulthood included sensorineural hearing loss, chronic suppurative otitis media, and hearing loss associated with a history of recurrent acute otitis media (2.4 [1.9-3.0], 2.4 [1.5-3.9], and 1.6 [1.3-2.0], respectively). These estimates were adjusted for age, sex, and noise exposure in adulthood. After further analyses that included adjustment for adult hearing threshold, none of these childhood hearing disorders remained positively associated with tinnitus. Childhood hearing disorders associated with tinnitus in adulthood include sensorineural hearing loss, chronic suppurative otitis media, and hearing loss associated with a history of recurrent acute otitis media. After adjustment for the

Human Frequency Following Response: Neural Representation of Envelope and Temporal Fine Structure in Listeners with Normal Hearing and Sensorineural Hearing Loss

PubMed Central

Ananthakrishnan, Saradha; Krishnan, Ananthanarayan; Bartlett, Edward

2015-01-01

Objective Listeners with sensorineural hearing loss (SNHL) typically experience reduced speech perception, which is not completely restored with amplification. This likely occurs because cochlear damage, in addition to elevating audiometric thresholds, alters the neural representation of speech transmitted to higher centers along the auditory neuroaxis. While the deleterious effects of SNHL on speech perception in humans have been well-documented using behavioral paradigms, our understanding of the neural correlates underlying these perceptual deficits remains limited. Using the scalp-recorded Frequency Following Response (FFR), the authors examine the effects of SNHL and aging on subcortical neural representation of acoustic features important for pitch and speech perception, namely the periodicity envelope (F0) and temporal fine structure (TFS) (formant structure), as reflected in the phase-locked neural activity generating the FFR. Design FFRs were obtained from 10 listeners with normal hearing (NH) and 9 listeners with mild-moderate SNHL in response to a steady-state English back vowel /u/ presented at multiple intensity levels. Use of multiple presentation levels facilitated comparisons at equal sound pressure level (SPL) and equal sensation level (SL). In a second follow-up experiment to address the effect of age on envelope and TFS representation, FFRs were obtained from 25 NH and 19 listeners with mild to moderately-severe SNHL to the same vowel stimulus presented at 80 dB SPL. Temporal waveforms, Fast Fourier Transform (FFT) and spectrograms were used to evaluate the magnitude of the phase-locked activity at F0 (periodicity envelope) and F1 (TFS). Results Neural representation of both envelope (F0) and TFS (F1) at equal SPLs was stronger in NH listeners compared to listeners with SNHL. Also, comparison of neural representation of F0 and F1 across stimulus levels expressed in SPL and SL (accounting for audibility) revealed that level-related changes in F0

Hyperbaric Oxygen Therapy Registry

ClinicalTrials.gov

2018-04-30

Air or Gas Embolism; Carbon Monoxide Poisoning; Clostridial Myositis and Myonecrosis (Gas Gangrene); Crush Injury, Compartment Syndrome & Other Acute Traumatic Ischemias; Decompression Sickness; Peripheral Arterial Insufficiency and Central Retinal Artery Occlusion; Severe Anemia; Intracranial Abscess; Necrotizing Soft Tissue Infections; Osteomyelitis (Refractory); Delayed Radiation Injury (Soft Tissue and Bony Necrosis); Compromised Grafts and Flaps; Acute Thermal Burn Injury; Idiopathic Sudden Sensorineural Hearing Loss

[Idiopathic sudden deafness: a report of 96 patients].

PubMed

Gabanou, F; Bera, G; Vincent, C

2012-01-01

Evaluation of the management of idiopathic sudden deafness indicating the usefulness of biological assessments and the pronostic factors of hearing recovery. This is a retrospective study of 96 patients with idiopathic sudden deafness referred to a tertiary centre between 2005 and 2009 treated with corticosteroids intravenously at a daily dose of 1 mg/kg. Mean tonal thresholds were assessed (PTA = [500 Hz + 1000 Hz + 2000 Hz + 4000 Hz]/4). Each audiogram was classified as five classes according to its frequency profile. The hearing recovery is significant between D0-D5 and D5-M1 for the frequencies 0.5, 1 and 2 kHz. For 4 kHz, the recovery is significant between 0 and J5. There is no statistically significant correlation between the presence of associated signs (tinnitus, vertigo) and hearing recovery. Hearing recovery according to the five types of audiograms has the same evolution in the follow-up time but with audiograms type E (cophosis or subcophosis) often associated with an hyporeactivity at the videonystagmography. The presence of cardiovascular disease is a predictor of poor hearing recovery. The usefullness of systematic extensive blood tests is low. In sudden deafness, the maximum hearing recovery takes place in the month following the onset of symptoms. The predictors of poor hearing recovery are an initial mean threshold > 70 dB, the existence of an associated cardiovascular disease.

Neuromagnetic Index of Hemispheric Asymmetry Prognosticating the Outcome of Sudden Hearing Loss

PubMed Central

Li, Lieber Po-Hung; Shiao, An-Suey; Chen, Kuang-Chao; Lee, Po-Lei; Niddam, David M.; Chang, Shyue-Yih; Hsieh, Jen-Chuen

2012-01-01

The longitudinal relationship between central plastic changes and clinical presentations of peripheral hearing impairment remains unknown. Previously, we reported a unique plastic pattern of “healthy-side dominance” in acute unilateral idiopathic sudden sensorineural hearing loss (ISSNHL). This study aimed to explore whether such hemispheric asymmetry bears any prognostic relevance to ISSNHL along the disease course. Using magnetoencephalography (MEG), inter-hemispheric differences in peak dipole amplitude and latency of N100m to monaural tones were evaluated in 21 controls and 21 ISSNHL patients at two stages: initial and fixed stage (1 month later). Dynamics/Prognostication of hemispheric asymmetry were assessed by the interplay between hearing level/hearing gain and ipsilateral/contralateral ratio (I/C) of N100m latency and amplitude. Healthy-side dominance of N100m amplitude was observed in ISSNHL initially. The pattern changed with disease process. There is a strong correlation between the hearing level at the fixed stage and initial I/Camplitude on affected-ear stimulation in ISSNHL. The optimal cut-off value with the best prognostication effect for the hearing improvement at the fixed stage was an initial I/Clatency on affected-ear stimulation of 1.34 (between subgroups of complete and partial recovery) and an initial I/Clatency on healthy-ear stimulation of 0.76 (between subgroups of partial and no recovery), respectively. This study suggested that a dynamic process of central auditory plasticity can be induced by peripheral lesions. The hemispheric asymmetry at the initial stage bears an excellent prognostic potential for the treatment outcomes and hearing level at the fixed stage in ISSNHL. Our study demonstrated that such brain signature of central auditory plasticity in terms of both N100m latency and amplitude at defined time can serve as a prognostication predictor for ISSNHL. Further studies are needed to explore the long-term temporal scenario

Bilateral versus unilateral hearing aids for bilateral hearing impairment in adults.

PubMed

Schilder, Anne Gm; Chong, Lee Yee; Ftouh, Saoussen; Burton, Martin J

2017-12-19

Acquired hearing loss is common and its incidence increases markedly with age. In most people, 'age-related' hearing loss is sensorineural (due to the loss of cochlear hair cells) and bilateral, affecting both ears to the same degree. Hearing loss categorised as mild, moderate or severe is primarily managed with hearing aids. People with bilateral hearing loss may be offered one aid, fitted to one specific ear, or two aids fitted to both ears. There is uncertainty about the relative benefits to people with hearing loss of these different strategies. To assess the effects of bilateral versus unilateral hearing aids in adults with a bilateral hearing impairment. The Cochrane ENT Information Specialist searched the ENT Trials Register; Cochrane Register of Studies Online; PubMed; Ovid Embase; CINAHL; Web of Science; ClinicalTrials.gov; ICTRP and additional sources for published and unpublished trials. The date of the search was 8 June 2017. Randomised controlled trials (RCTs) comparing the fitting of two versus one ear-level acoustic hearing aids in adults (over 18 years) with a bilateral hearing impairment, both ears being eligible for hearing aids. We used the standard methodological procedures expected by Cochrane. Our primary outcomes were patient preference for bilateral or unilateral aids, hearing-specific health-related quality of life and adverse effects (pain or discomfort in the ear, initiation or exacerbation of middle or outer ear infection). Secondary outcomes included: usage of hearing aids (as measured by, for example, data logging or battery consumption), generic health-related quality of life, listening ability and audiometric benefit measured as binaural loudness summation. We used GRADE to assess the quality of the evidence for each outcome; this is indicated in italics. We included four cross-over RCTs with a total of 209 participants, ranging in age from 23 to 85 and with a preponderance of men. All the studies allowed the use of hearing aids for

38 CFR 17.149 - Sensori-neural aids.

Code of Federal Regulations, 2014 CFR

2014-07-01

... 38 Pensions, Bonuses, and Veterans' Relief 1 2014-07-01 2014-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact lenses...

38 CFR 17.149 - Sensori-neural aids.

Code of Federal Regulations, 2012 CFR

2012-07-01

... 38 Pensions, Bonuses, and Veterans' Relief 1 2012-07-01 2012-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact lenses...

38 CFR 17.149 - Sensori-neural aids.

Code of Federal Regulations, 2013 CFR

2013-07-01

... 38 Pensions, Bonuses, and Veterans' Relief 1 2013-07-01 2013-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact lenses...

38 CFR 17.149 - Sensori-neural aids.

Code of Federal Regulations, 2011 CFR

2011-07-01

... 38 Pensions, Bonuses, and Veterans' Relief 1 2011-07-01 2011-07-01 false Sensori-neural aids. 17... Prosthetic, Sensory, and Rehabilitative Aids § 17.149 Sensori-neural aids. (a) Notwithstanding any other provision of this part, VA will furnish needed sensori-neural aids (i.e., eyeglasses, contact lenses...

Newborn Hearing Screening and Early Diagnostic in the NICU

PubMed Central

Colella-Santos, Maria Francisca; Hein, Thaís Antonelli Diniz; de Souza, Gabriele Libano; do Amaral, Maria Isabel Ramos; Casali, Raquel Leme

2014-01-01

The aim was to describe the outcome of neonatal hearing screening (NHS) and audiological diagnosis in neonates in the NICU. The sample was divided into Group I: neonates who underwent NHS in one step and Group II: neonates who underwent a test and retest NHS. NHS procedure was automated auditory brainstem response. NHS was performed in 82.1% of surviving neonates. For GI, referral rate was 18.6% and false-positive was 62.2% (normal hearing in the diagnostic stage). In GII, with retest, referral rate dropped to 4.1% and false-positive to 12.5%. Sensorineural hearing loss was found in 13.2% of infants and conductive in 26.4% of cases. There was one case of auditory neuropathy spectrum (1.9%). Dropout rate in whole process was 21.7% for GI and 24.03% for GII. We concluded that it was not possible to perform universal NHS in the studied sample or, in many cases, to apply it within the first month of life. Retest reduced failure and false-positive rate and did not increase evasion, indicating that it is a recommendable step in NHS programs in the NICU. The incidence of hearing loss was 2.9%, considering sensorineural hearing loss (0.91%), conductive (1.83%) and auditory neuropathy spectrum (0.19%). PMID:24999481

Risk Factors for Sensorineural Hearing Loss Among High-Risk Infants in Golestan Province, Iran in 2010 - 2011

PubMed Central

Alaee, Ehsan; Sirati, Mohsen; Taziki, Mohammad Hossein; Fouladinejad, Mahnaz

2015-01-01

Background: Hearing impairment, as one of the most common birth defects, is a hidden disability with negative impacts on speech and cognitive development. Objectives: The aim of this study was to assess the prevalence of sensorineural hearing loss (SNHL) and determine the associated risk factors among infants admitted to neonatal intensive care units (NICUs) and neonatal wards of teaching hospitals, affiliated to Golestan University of Medical Sciences, Gorgan, Iran. Patients and Methods: In this cross-sectional study, 791 infants were recruited via non-random sampling. Demographic and clinical characteristics of the subjects were gathered, and the Automated Auditory Brainstem Response (AABR) test was performed upon admission. Afterwards, the subjects were followed-up and re-assessed, using the AABR test. For infants with abnormal AABR results, the Auditory Brainstem Response (ABR) test was performed on the day of discharge. Results: The mean age of the infants was 3.75 ± 4.86 days upon admission, and 56.4% of the subjects were female. The mean length of hospital stay was 9.63 ± 1.1 days; the subjects were hospitalized for 3.50 ± 10.21 days in the NICUs and 6.1 ± 5.27 days in the neonatal wards. In total, 3.4% of the infants presented with SNHL. No significant difference was found between SNHL and neonates’ age (P = 0.52), sex (P = 0.5), or sepsis (P = 0.94). However, SNHL was significantly associated with gestational age (P = 0.045), birth weight (P < 0.001), length of hospital stay (P < 0.001), pathological jaundice (P=0.033), antibiotic treatments (P = 0.007), and total serum bilirubin level (P = 0.01). Additionally, binary logistic regression analysis demonstrated the association between SNHL and these factors. Conclusions: In this study, the prevalence of SNHL among hospitalized neonates was similar to previous reports in Iran and other countries. Based on the findings, administration of ototoxic drugs during the neonatal period can lead to SNHL

Acupuncture therapy for sudden sensorineural hearing loss: a systematic review and meta-analysis of randomized controlled trials.

PubMed

Zhang, Xin-chang; Xu, Xiu-ping; Xu, Wen-tao; Hou, Wen-zhen; Cheng, Ying-ying; Li, Chang-xi; Ni, Guang-xia

2015-01-01

Acupuncture has commonly been used in China, either alone or in combination with Western medicine, to treat sudden sensorineural hearing loss (SSHL). The purpose of this systematic review is to assess the efficacy and safety of acupuncture therapy for patients with SSHL. We searched PubMed, the Cochrane Library, Embase, China National Knowledge Internet (CNKI), Database for Chinese Technical Periodicals (VIP), and Chinese Biomedical literature service system (SinoMed) to collect randomized controlled trials of acupuncture for SSHL published before July 2014. A meta-analysis was conducted according to the Cochrane systematic review method using RevMan 5.2 software. The evidence level for each outcome was assessed using the GRADE methodology. Twelve trials involving 863 patients were included. A meta-analysis showed that the effect of manual acupuncture combined with Western medicine comprehensive treatment (WMCT) was better than WMCT alone (RR 1.33, 95%CI 1.19-1.49) and the same as the effect of electroacupuncture combined with WMCT (RR 1.33, 95%CI 1.19-1.50). One study showed a better effect of electroacupuncture than of WMCT (RR 1.34, 95%CI 1.24-1.45). For mean changes in hearing over all frequencies, the meta-analysis showed a better effect with the combination of acupuncture and WMCT than with WMCT alone (MD 10.85, 95%CI 6.84-14.86). However, the evidence levels for these interventions were low or very low due to a high risk of bias and small sample sizes in the included studies. There was not sufficient evidence showing that acupuncture therapy alone was beneficial for treating SSHL. However, interventions combining acupuncture with WMCT had more efficacious results in the treatment of SSHL than WMCT alone. Electroacupuncture alone might be a viable alternative treatment besides WMCT for SSHL. However, given that there were fewer eligible RCTs and limitations in the included trials, such as methodological drawbacks and small sample sizes, large-scale RCTs are

Cochlear synaptopathy in acquired sensorineural hearing loss: Manifestations and mechanisms.

PubMed

Liberman, M Charles; Kujawa, Sharon G

2017-06-01

Common causes of hearing loss in humans - exposure to loud noise or ototoxic drugs and aging - often damage sensory hair cells, reflected as elevated thresholds on the clinical audiogram. Recent studies in animal models suggest, however, that well before this overt hearing loss can be seen, a more insidious, but likely more common, process is taking place that permanently interrupts synaptic communication between sensory inner hair cells and subsets of cochlear nerve fibers. The silencing of affected neurons alters auditory information processing, whether accompanied by threshold elevations or not, and is a likely contributor to a variety of perceptual abnormalities, including speech-in-noise difficulties, tinnitus and hyperacusis. Work described here will review structural and functional manifestations of this cochlear synaptopathy and will consider possible mechanisms underlying its appearance and progression in ears with and without traditional 'hearing loss' arising from several common causes in humans. Copyright © 2017 Elsevier B.V. All rights reserved.

The right not to hear: the ethics of parental refusal of hearing rehabilitation.

PubMed

Byrd, Serena; Shuman, Andrew G; Kileny, Sharon; Kileny, Paul R

2011-08-01

To explore the ethics of parental refusal of auditory-oral hearing rehabilitation. Case study with medical ethical discussion and review. Two young brothers present with severe-to-profound congenital sensorineural hearing loss. The parents, both of whom have normal hearing and work as sign language interpreters, have decided to raise their children with American Sign Language as their only form of communication. They have chosen not to pursue cochlear implantation nor support the use of hearing aids. This case raises significant questions concerning whether hearing rehabilitation should be mandated, and if there are circumstances in which parental preferences should be questioned or overridden with regard to this issue. In addition, legal concerns may be raised regarding the possible need to file a report with Child Protective Services. Although similar cases involving the Deaf community have historically favored parental rights to forego hearing rehabilitation with either cochlear implantation or hearing aids, we explore whether conclusions should be different because the parents in this case are not hearing impaired. The ethics of parental rights to refuse hearing rehabilitation are complex and strikingly context-dependent. A comprehensive appreciation of the medical, practical, and legal issues is crucial prior to intervening in such challenging situations. Copyright © 2011 The American Laryngological, Rhinological, and Otological Society, Inc.

The Right Not To Hear: The Ethics of Parental Refusal of Hearing Rehabilitation

PubMed Central

Byrd, Serena; Shuman, Andrew G.; Kileny, Sharon; Kileny, Paul R.

2015-01-01

Objective To explore the ethics of parental refusal of auditory-oral hearing rehabilitation. Study Design Case study with medical ethical discussion and review. Methods Two young brothers present with severe-to-profound congenital sensorineural hearing loss. The parents, both of whom have normal hearing and work as sign language interpreters, have decided to raise their children with American Sign Language as their only form of communication. They have chosen not to pursue cochlear implantation nor support the use of hearing aids. Discussion This case raises significant questions concerning whether hearing rehabilitation should be mandated, and if there are circumstances in which parental preferences should be questioned or overridden with regard to this issue. In addition, legal concerns may be raised regarding the possible need to file a report with child protective services. Although similar cases involving the Deaf community have historically favored parental rights to forego hearing rehabilitation with either cochlear implantation or hearing aids, we explore whether conclusions should be different because the parents in this case are not hearing impaired. Conclusions The ethics of parental rights to refuse hearing augmentation are complex and strikingly context-dependent. A comprehensive appreciation of the medical, practical and legal issues is crucial prior to intervening in such challenging situations. PMID:21792972

Total serum bilirubin levels and sensorineural hearing loss in the US adolescents: NHANES 2007-2010.

PubMed

Zhou, Guoli; Fu, Wenjiang

2018-02-01

We aimed to investigate whether current levels of total serum bilirubin are associated with different subtypes of sensorineural hearing loss (SNHL) in adolescents. A set of cross-sectional data from the National Health and Nutrition Examination Survey (NHANES) (2007-2010) was used. A subset of 1404 adolescents was sampled for measurements of total serum bilirubin, tympanometry, and average pure tone threshold at low-frequencies (LPTA: 500, 1000, 2000 Hz) or high-frequencies (HPTA: 3000, 4000, 6000, and 8000 Hz). SNHL was defined as the hearing loss that had type A tympanograms with a peak admittance of 0.3 ml or greater. Associations between serum bilirubin (square-root transformed) and different subtypes of SNHL were evaluated using binary or multinomial logistic regression models with 4-year sampling weights. The bootstrap method was used for estimation of variance and 10-fold cross-validation for assessment of overfitting issue. Total serum bilirubin levels were found to be associated with any high-frequency (HPTA>15 dB in at least one ear, adjusted odds-ratio (OR a )(bootstrap 95% confidence interval) = 3.29(1.31-8.19), p = 0.011), but not with any low-frequency (LPTA>15 dB in at least one ear), SNHL in the US adolescents. Furthermore, high-frequency SNHL with HPTA>15 dB in both ears (bilateral) or HPTA≥25 dB in at least one ear, compared to that with HPTA>15 dB in one ear only (unilateral) or HPTA = 15-25 dB in at least one ear, had a stronger association with total serum bilirubin levels (OR a  = 5.37(1.27-22.65), p = 0.022 for bilateral; OR a  = 2.64(0.84-8.25), p = 0.094 for unilateral; OR a  = 5.00(0.95-26.58), p = 0.058 for HPTA≥25 dB in at least one ear; as well as OR a  = 3.06(1.15-8.25), p = 0.025 for HPTA = 15-25 dB in at least one ear). No severe overfitting problems were found. Our findings suggest that current levels of total serum bilirubin may be informative in predicting and/or targeting high-frequency SNHL

Hearing loss in Behçet syndrome.

PubMed

Bakhshaee, Mehdi; Mahdi, Bakhshaee; Ghasemi, Mohammad Mehdi; Mehdi, Ghasemi Mohammad; Hatef, Mohammad Reza; Reza, Hatef Mohammad; Talebmehr, Mahdieh; Mahdieh, Talebmehr; Shakeri, Mohammad Taghi; Taghi, Shakeri Mohammad

2007-09-01

To determine the prevalence and characteristics of hearing loss in Behçet syndrome. This study included 27 patients with Behçet syndrome and 35 sex-and age-matched controls. A complete audiological evaluation was performed. The average pure-tone audiograms from both groups showed a statistically significant hearing loss in the Behçet group. Sixteen patients (59.26%) showed some degrees of sensorineural hearing loss (SNHL), with the high-frequency type (4, 8, 10, and 12 kHz) being the most common pattern (93.75%). Hearing loss was the fourth most common manifestation. Although the patient's age, sex, and the duration of the disease were not related to hearing loss, there was a significant correlation between a negative pathergy test and hearing loss in patients with Behçet syndrome. We should consider audiovestibular involvement in Behçet syndrome as a common finding.

Hearing Impairment Among Children Referred to a Public Audiology Clinic in Gaborone, Botswana.

PubMed

Banda, Francis M; Powis, Kathleen M; Mokoka, Agnes B; Mmapetla, Moalosi; Westmoreland, Katherine D; David, Thuso; Steenhoff, Andrew P

2018-01-01

Objective . To describe and quantify hearing impairment among children referred to the audiology clinic in Princess Marina Hospital, a public referral hospital in Botswana. Methods . In a retrospective case series, we reviewed medical records of children aged 10 years and younger whose hearing was assessed between January 2006 and December 2015 at the audiology clinic of Princess Marina Hospital in Gaborone, Botswana. Results . Of 622 children, 50% were male, and median age was 6.7 years (interquartile range = 5.0-8.3). Hearing impairment was diagnosed in 32% of clinic attendees, comprising sensorineural (23%), conductive (25%), and mixed (11%) hearing loss, while 41% of children with diagnosed hearing impairment did not have a classification type. Hearing impairment was mild in 22.9%, moderate in 22.4%, severe in 19.4%, profound in 16.9%, and of undocumented severity in 18.4%. Children younger than 5 years were 2.7 times (95% confidence interval = 1.29-5.49; P = .008) more likely to be diagnosed with sensorineural hearing impairment compared with those older than 5 years. By contrast, children older than 5 years were 9.6 times (95% confidence interval = 2.22-41.0; P = .002) more likely to be diagnosed with conductive hearing loss compared with those under 5 years. Conclusion . Hearing impairment was common among children referred to this audiology clinic in Botswana. Of those with hearing impairment, more than a third had moderate or severe deficits, suggesting that referrals for hearing assessments are not occurring early enough. Hearing awareness programs individually tailored to parents, educators, and health care workers are needed. Neonatal and school hearing screening programs would also be beneficial.

Auditory Performance and Electrical Stimulation Measures in Cochlear Implant Recipients With Auditory Neuropathy Compared With Severe to Profound Sensorineural Hearing Loss.

PubMed

Attias, Joseph; Greenstein, Tally; Peled, Miriam; Ulanovski, David; Wohlgelernter, Jay; Raveh, Eyal

The aim of the study was to compare auditory and speech outcomes and electrical parameters on average 8 years after cochlear implantation between children with isolated auditory neuropathy (AN) and children with sensorineural hearing loss (SNHL). The study was conducted at a tertiary, university-affiliated pediatric medical center. The cohort included 16 patients with isolated AN with current age of 5 to 12.2 years who had been using a cochlear implant for at least 3.4 years and 16 control patients with SNHL matched for duration of deafness, age at implantation, type of implant, and unilateral/bilateral implant placement. All participants had had extensive auditory rehabilitation before and after implantation, including the use of conventional hearing aids. Most patients received Cochlear Nucleus devices, and the remainder either Med-El or Advanced Bionics devices. Unaided pure-tone audiograms were evaluated before and after implantation. Implantation outcomes were assessed by auditory and speech recognition tests in quiet and in noise. Data were also collected on the educational setting at 1 year after implantation and at school age. The electrical stimulation measures were evaluated only in the Cochlear Nucleus implant recipients in the two groups. Similar mapping and electrical measurement techniques were used in the two groups. Electrical thresholds, comfortable level, dynamic range, and objective neural response telemetry threshold were measured across the 22-electrode array in each patient. Main outcome measures were between-group differences in the following parameters: (1) Auditory and speech tests. (2) Residual hearing. (3) Electrical stimulation parameters. (4) Correlations of residual hearing at low frequencies with electrical thresholds at the basal, middle, and apical electrodes. The children with isolated AN performed equally well to the children with SNHL on auditory and speech recognition tests in both quiet and noise. More children in the AN group

The round window diameter in congenital aural atresia and comparison with sensorineural hearing loss and control group.

PubMed

Saylisoy, Suzan; Incesulu, Armagan; Kaya, Ercan; Pinarbasli, Ozgur; Adapinar, Baki

2014-01-01

The aim of this study was to measure round window (RW) diameters in patients with congenital aural atresia (CAA) or sensorineural hearing loss (SNHL) and a normal control group and to analyze whether differences exist between these groups. Temporal bone computed tomographic scans of 12 patients with CAA (5 males, 7 females) aged 1 to 50 years (median age, 6 years), 12 patients with SNHL (8 males, 4 females) aged 2 to 32 years (median age, 5 years), and 11 patients (3 males, 7 females) aged 2 months to 53 years (median age, 8 years) randomly selected from a pool of patients with unilateral chronic otitis media or cholesteatoma were reviewed. We measured RW diameter on oblique reconstruction planes. To prevent possible individual differences, skull width was measured. There were no statistically significant differences between all groups for skull width. Both RW diameter and RW membrane width were found smaller in the CAA group than both SNHL group and control group with statistical significance, whereas there were no statistically significant differences between the SNHL group and the control group. We found that both the RW diameter and RW membrane width in CAA were smaller than those in the control group. If this finding is supported in future studies, the production of floating mass transducer with different sizes may be useful. We suggest that RW diameter should be measured in each patient before operation and thus a floating mass transducer with the appropriate caliber should be chosen.

State of the art in perceptual design of hearing aids

NASA Astrophysics Data System (ADS)

Edwards, Brent W.; van Tasell, Dianne J.

2002-05-01

Hearing aid capabilities have increased dramatically over the past six years, in large part due to the development of small, low-power digital signal processing chips suitable for hearing aid applications. As hearing aid signal processing capabilities increase, there will be new opportunities to apply perceptually based knowledge to technological development. Most hearing loss compensation techniques in today's hearing aids are based on simple estimates of audibility and loudness. As our understanding of the psychoacoustical and physiological characteristics of sensorineural hearing loss improves, the result should be improved design of hearing aids and fitting methods. The state of the art in hearing aids will be reviewed, including form factors, user requirements, and technology that improves speech intelligibility, sound quality, and functionality. General areas of auditory perception that remain unaddressed by current hearing aid technology will be discussed.

Occupational hearing loss of market mill workers in the city of Accra, Ghana.

PubMed

Kitcher, Emmanuel D; Ocansey, Grace; Abaidoo, Benjamin; Atule, Alidu

2014-01-01

Noise induced hearing loss (NIHL) is an irreversible sensorineural hearing loss associated with exposure to high levels of excessive noise. Prevention measures are not well established in developing countries. This comparative cross sectional study aims to determine the prevalence of hearing loss in both a group of high risk workers and a control group and to assess their knowledge of the effects of noise on hearing health. A total of 101 market mill workers and 103 controls employed within markets in the city of Accra, Ghana, were evaluated using a structured questionnaire and pure tone audiometry. The questionnaire assessed factors including self-reported hearing loss, tinnitus, knowledge on the effects of noise on hearing health and the use of hearing protective devices. Pure tone audiometric testing was conducted for both mill workers and controls. Noise levels at the work premises of the mill workers and controls were measured. Symptoms of hearing loss were reported by 24 (23.76%) and 8 (7.7%) mill workers and controls respectively. Fifty-five (54.5%) and fifty-four (52.37%) mill workers and controls exhibited knowledge of the effects of noise on hearing health. Five (5.0%) mill workers used hearing protective devices. There was significant sensorineural hearing loss and the presence of a 4 kHz audiometric notch among mill workers when compared with controls for the mean thresholds of 2 kHz, 3 kHz and 4 kHz (P = 0. 001). The prevalence of hearing loss in the better hearing ears of the mill workers and controls was 24.8% and 4.8% respectively (P < 0.5). The prevalence of hearing loss, which may be characteristic of NIHL in the better hearing ears of the mill workers and controls was 24.8% and 4.8% respectively. The majority of mill workers did not use hearing protection.

Hearing Loss in Infants with Microcephaly and Evidence of Congenital Zika Virus Infection - Brazil, November 2015-May 2016.

PubMed

Leal, Mariana C; Muniz, Lilian F; Ferreira, Tamires S A; Santos, Cristiane M; Almeida, Luciana C; Van Der Linden, Vanessa; Ramos, Regina C F; Rodrigues, Laura C; Neto, Silvio S Caldas

2016-09-02

Congenital infection with Zika virus causes microcephaly and other brain abnormalities (1). Hearing loss associated with other congenital viral infections is well described; however, little is known about hearing loss in infants with congenital Zika virus infection. A retrospective assessment of a series of 70 infants aged 0-10 months with microcephaly and laboratory evidence of Zika virus infection was conducted by the Hospital Agamenon Magalhães in Brazil and partners. The infants were enrolled during November 2015-May 2016 and had screening and diagnostic hearing tests. Five (7%) infants had sensorineural hearing loss, all of whom had severe microcephaly; however, one child was tested after receiving treatment with an ototoxic antibiotic. If this child is excluded, the prevalence of sensorineural hearing loss was 5.8% (four of 69), which is similar to that seen in association with other congenital viral infections. Additional information is needed to understand the prevalence and spectrum of hearing loss in children with congenital Zika virus infection; all infants born to women with evidence of Zika virus infection during pregnancy should have their hearing tested, including infants who appear normal at birth.

Early investigational drugs for hearing loss

PubMed Central

Mukherjea, Debashree; Ghosh, Sumana; Bhatta, Puspanjali; Sheth, Sandeep; Tupal, Srinivasan; Borse, Vikrant; Brozoski, Thomas; Sheehan, Kelly E; Rybak, Leonard P; Ramkumar, Vickram

2017-01-01

Introduction Sensorineural hearing loss (HL) is becoming a global phenomenon at an alarming rate. Nearly 600 million people have been estimated to have significant HL in at least one ear. There are several different causes of sensorineural HL included in this review of new investigational drugs for HL. They are noise-induced, drug-induced, sudden sensorineural HL, presbycusis and HL due to cytomegalovirus infections. Areas covered This review presents trends in research for new investigational drugs encompassing a variety of causes of HL. The studies presented here are the latest developments either in the research laboratories or in preclinical, Phase 0, Phase I or Phase II clinical trials for drugs targeting HL. Expert opinion While it is important that prophylactic measures are developed, it is extremely crucial that rescue strategies for unexpected or unavoidable cochlear insult be established. To achieve this goal for the development of drugs for HL, innovative strategies and extensive testing are required for progress from the bench to bedside. However, although a great deal of research needs to be done to achieve the ultimate goal of protecting the ear against acquired sensorineural HL, we are likely to see exciting breakthroughs in the near future. PMID:25243609

[Hearing screening at nursery schools: results of an evaluation study].

PubMed

Weichbold, Viktor; Rohrer, Monika; Winkler, Cornelia; Welzl-Müller, Kunigunde

2004-07-31

This study aimed to evaluate the hearing screening of pre-school children at nursery schools in Tyrol, Austria. 47 nursery schools with a total of 2199 enrolled children participated in the study. At the screening, the children were presented a series of tones at frequencies 0.5 kHz (25dB), 1 kHz, 2 kHz, 3 kHz, and 4 kHz (20 dB each) from portable audiometers. The tones were presented over headphones for each ear separately and at irregular intervals. Failure to respond to any of the frequencies was considered failure of the screening. Parents were then advised in written form to have the child examined by an ENT-specialist. 1832 individuals were screened (coverage: 83% of nursery school children; corresponding to at least 63% of all Tyrolean children aged 3 to 5 years). Of these, 390 failed the test (referral rate: 21% of all screened). Examination through an ENT-specialist occurred with 217 children, and this confirmed the positive test in 139 children (hit rate: 64%). In most cases, a temporary conductive hearing loss due to external or middle ear problems (glue ear, tube dysfunction, cerumen, otitis media) was diagnosed. A sensorineural hearing loss was found in 4 children (in 3 of them bilateral). The need for therapy was recognized in 81 children (4% of all screened). Pre-school hearing screening identifies children with ear and hearing problems that need therapeutical intervention. Although the hearing problems are mostly of a temporary nature, some may require monitoring over some period. Also some children with permanent sensorineural hearing loss may be detected through this measure. Hearing screening is an efficient means of assessing ear and hearing problems in pre-school children. However, the follow-up rate needs to be improved for optimizing the efficacy.

[Functional hearing examinations in patients suffering from diabetes mellitus type 1 in regard to disease duration].

PubMed

Pudar, Goran; Vlaski, Ljiljana; Filipović, Danka; Tanackov, Ilija

2010-01-01

Problems of hearing disturbances in persons suffering from diabetes have been attracting great attention for many decades. In this study we examined the auditory function of 50 patients suffering from diabetes mellitus type 1 of different duration by analyzing results of pure-tone audiometry and brainstem auditory evoked potentials. The obtained results of measuring were compared to 30 healthy subjects from the corresponding age and gender group. The group of diabetic patients was divided according to the disease duration (I group 0-5 years; II group 6-10 years, III group over 10 years). A statistically significant increase of sensorineural hearing loss was found in the diabetics according to the duration of their disease (I group = 14.09%, II group = 21.39%, III group = 104.89%). The results of the brain stem auditory evoked potentials, the significance threshold being p = 0.05 between the controls and the diabetics at all levels of absolute latency of right and left sides, did not show significant differences in the mean values. In the case of interwave latencies, the diabetic patients were found to have a significant qualitative difference of intervals I-III and I-V on both ears in the sense of internal distribution of response. In cases of sensorineural hearing loss we found a significant connection with prolonged latencies of I wave on the right ear and of I and V waves on the left ear. In all probability, the cause of these results could be found in distinctive individuality of the organism reactions to the consequences of this disease (disturbance in the distal part of N. cochlearis). The results of research have shown the existence of a significant sensorineural hearing loss in the patients with diabetes mellitus type 1 in accordance to the disease duration. We also found qualitative changes of brainstem auditory evoked potentials in the diabetic patients in comparison to the controls as well as significant quantitative changes in regard to the presence of

Imaging of post-traumatic hearing loss.

PubMed

Mazón, M; Pont, E; Albertz, N; Carreres-Polo, J; Más-Estellés, F

Hearing loss is the most frequent complication of temporal bone trauma. The role of the radiologist is of great importance; the adequacy and selection of the imaging technique, as well as its correct interpretation, are crucial to establish the diagnosis, prognosis and enable the selection of appropriate treatment. With the aim of systematizing the most relevant concepts in the evaluation of image studies in this scenario, this review will be outlined according to the hearing loss type. The potential lesions of its components will be assessed; In each case the most appropriate imaging technique will be suggested and the findings will be described and depicted. In postraumatic hearing loss, computed tomography is the initial technique of choice and will allow the detection of alterations that cause conductive hearing loss; magnetic resonance imaging will be useful in the evaluation of sensorineural hearing loss. Copyright © 2017 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.

Active Duty-U.S. Army Noise Induced Hearing Injury Quarterly Surveillance Q3 2011 thru Q4 2013

DTIC Science & Technology

2014-06-30

incident case rates for sensorineural hearing loss significant threshold shift, tinnitus , and Noise-Induced Hearing Loss. RECOMMENDATIONS: Commanders...2013 A-1 APPENDIX A REFERENCES Humes LE, Jollenbeck LM, Durch JS: Noise and military service: Implications for hearing loss and tinnitus . Washington...FUNCTION STUDIES TINN Tinnitus 38830 TINNITUS UNSPECIFIED TINN Tinnitus 38831 SUBJECTIVE TINNITUS TINN Tinnitus 38832 OBJECTIVE TINNITUS CPT Codes

Identifying hearing loss by means of iridology.

PubMed

Stearn, Natalie; Swanepoel, De Wet

2006-11-13

Isolated reports of hearing loss presenting as markings on the iris exist, but to date the effectiveness of iridology to identify hearing loss has not been investigated. This study therefore aimed to determine the efficacy of iridological analysis in the identification of moderate to profound sensorineural hearing loss in adolescents. A controlled trial was conducted with an iridologist, blind to the actual hearing status of participants, analyzing the irises of participants with and without hearing loss. Fifty hearing impaired and fifty normal hearing subjects, between the ages of 15 and 19 years, controlled for gender, participated in the study. An experienced iridologist analyzed the randomised set of participants' irises. A 70% correct identification of hearing status was obtained by iridological analyses with a false negative rate of 41% compared to a 19% false positive rate. The respective sensitivity and specificity rates therefore came to 59% and 81%. Iridological analysis of hearing status indicated a statistically significant relationship to actual hearing status (P < 0.05). Although statistically significant sensitivity and specificity rates for identifying hearing loss by iridology were not comparable to those of traditional audiological screening procedures.

Electrocardiogram Screening in Children with Congenital Sensorineural Hearing Loss: Prevalence and Follow-up of Abnormalities.

PubMed

Farzal, Zainab; Walsh, Jonathan; Ahmad, Faisal I; Roberts, Jason; Ferns, Sunita J; Zdanski, Carlton J

2018-03-01

Objective The purpose is to determine the prevalence of electrocardiogram (ECG) abnormalities, including borderline and prolonged QT, among screened children with sensorineural hearing loss (SNHL) and to analyze their subsequent medical workup. Study Design Institutional Review Board-approved case series with chart review. Setting Tertiary academic center. Subjects and Methods Cases from 1996 to 2014 involving pediatric patients (N = 1994) with SNHL were analyzed. Abnormal ECGs were categorized as borderline/prolonged QT or other. A board-certified pediatric cardiologist retrospectively determined the clinical significance of ECG changes. For follow-up analysis, children with heart disease, known syndromes, or inaccessible records were excluded. Results Among 772 children who had ECGs, 215 (27.8%) had abnormal results: 35 (4.5%) with QT abnormalities and 180 (23.3%) with other abnormalities. For children with QT abnormalities meeting inclusion criteria (n = 30), follow-up measures included cardiology referral (46.6%), repeat ECG by ear, nose, and throat (ENT) specialist (20%), clearance by ENT specialist with clinical correlation and/or comparison with old ECGs (20%), and pediatrician follow-up (6.7%). Documentation of further workup by ENT or referral was absent for 6.7%. For children with other ECG changes meeting inclusion criteria (n = 136), abnormalities were documented for 57 (41.9%); normal QT without other abnormality was documented for 18 (13.2%). The most common follow-up referrals were to pediatricians (16.9%) and cardiologists (10.3%). Among patients with clinically significant non-QT abnormalities mandating further evaluation (n = 122), 38 (31.1%) had documented follow-up in medical records. Conclusion There is a high prevalence of ECG abnormalities among children with congenital SNHL. If findings are confirmed by future studies, screening should be considered for congenital unilateral or bilateral SNHL, regardless of severity. We describe a

Sudden Sensorineural Hearing Loss: The Question of Perilymph Fistula.

ERIC Educational Resources Information Center

Backous, Douglas D.; Niparko, John K.

1997-01-01

Perilymph fistula (PLF) is an abnormal communication between the fluid-containing spaces of the inner ear and the air-containing spaces of the temporal bone that can cause hearing loss, tinnitus, aural fullness, vertigo, and postural instability. Diagnosis of PLF and management of those with presumed PLF are discussed. (Contains extensive…

The audiological characteristics of a hereditary Y-linked hearing loss in a Chinese ethnic Tujia pedigree.

PubMed

Fu, Siqing; Yan, Ju; Wang, Xiyin; Dong, Jiashu; Chen, Peiwei; Wang, Chunfang; Chen, Guanming

2011-02-01

To investigate audiometric characteristics of hearing loss in a large Chinese ethnic Tujia family and determine its hereditary type. Total 76 live individuals were investigated in the notable 84 members of this family. The detailed audiometric evaluations were undertaken for the proband and his 47 family members. The degrees of sensorineural hearing impairment were defined as an air/bone gap <15dB hearing loss averaged over 0.5, 1 and 2kHz. The severity of hearing loss was established based on the hearing ability of the better ear, averaged over 0.5, 1, 2 and 4kHz, and classified into four categories: mild, moderate, severe and profound. Nineteen patrilineal relatives of the 76 live members had hearing impairment. The age of onset ranged from 7 to 21 years old with the average of 13.2 years. The audiometric defect was described by auditory curves of a high frequency in 47% of the patients. Affected members in this family demonstrated a non-syndromic, late onset, bilateral, symmetrical, postlingual and sensorineural hearing loss. The audiometric configuration in males of the pedigree is consistent with the hereditary Y-linked hearing loss. Thus we speculate that a putative gene on the Y chromosome could contribute to the cause of the disease. Copyright © 2010 Elsevier Ireland Ltd. All rights reserved.

Hearing loss in Usher syndrome type II is nonprogressive.

PubMed

Reisser, Christoph F V; Kimberling, William J; Otterstedde, Christian R

2002-12-01

Usher syndrome is an autosomal recessive disorder characterized by sensorineural hearing loss and progressive visual loss secondary to retinitis pigmentosa. In the literature, a possible progression of the moderate to severe hearing loss in Usher syndrome type II (Usher II) is controversial. We studied the development of the hearing loss of 125 patients with a clinical diagnosis of Usher syndrome type II intraindividually and interindividually by repeatedly performing complete audiological and neuro-otologic examinations. Our data show a very characteristic slope of the hearing curve in all Usher II patients and no clinically relevant progression of the hearing loss over up to 17 years. The subjective impression of a deterioration of the communicative abilities of Usher II patients must therefore be attributed to the progressive visual loss. The patients should be reassured that changes in their hearing abilities are unlikely and should be provided with optimally fitted modern hearing aids.

The Prevalence and Characteristics of Tinnitus with Profound Sensori-Neural Hearing Impairment.

ERIC Educational Resources Information Center

Drukier, Gale S.

1989-01-01

Of 331 children (aged 6-18) with profound hearing impairment, 96 were found to have tinnitus. More females than males reported tinnitus. Most of the children with tinnitus were bothered to some degree by it and indicated that the noises adversely affected their ability to hear voices. (JDD)

Incidence of tympanic membrane perforation after intratympanic steroid treatment through myringotomy tubes.

PubMed

Rutt, Amy L; Hawkshaw, Mary J; Sataloff, Robert T

2011-04-01

Intratympanic (IT) steroids are often used to treat inner ear disorders such as sudden idiopathic sensorineural hearing loss, autoimmune inner ear disease, and Ménière disease. Administration of corticosteroids via IT injection, via application with a pledget to the round window, or via catheter has been used for this purpose. The frequency of adverse events related to the IT injection of steroids is low, with pain, short-lasting vertigo, otitis media, and tympanic perforations being the most common complications. However, the safety of IT steroid therapy has not yet been established in a randomized clinical trial. In this article, we discuss a group of 11 patients with sensorineural hearing loss who underwent myringotomy and tube placement for home-based dexamethasone instillation and subsequently developed the complication of tympanic membrane perforation. It appears that there is a significantly increased incidence of tympanic membrane perforations in this population.

A Sound Therapy-Based Intervention to Expand the Auditory Dynamic Range for Loudness among Persons with Sensorineural Hearing Losses: A Randomized Placebo-Controlled Clinical Trial

PubMed Central

Formby, Craig; Hawley, Monica L.; Sherlock, LaGuinn P.; Gold, Susan; Payne, JoAnne; Brooks, Rebecca; Parton, Jason M.; Juneau, Roger; Desporte, Edward J.; Siegle, Gregory R.

2015-01-01

The primary aim of this research was to evaluate the validity, efficacy, and generalization of principles underlying a sound therapy–based treatment for promoting expansion of the auditory dynamic range (DR) for loudness. The basic sound therapy principles, originally devised for treatment of hyperacusis among patients with tinnitus, were evaluated in this study in a target sample of unsuccessfully fit and/or problematic prospective hearing aid users with diminished DRs (owing to their elevated audiometric thresholds and reduced sound tolerance). Secondary aims included: (1) delineation of the treatment contributions from the counseling and sound therapy components to the full-treatment protocol and, in turn, the isolated treatment effects from each of these individual components to intervention success; and (2) characterization of the respective dynamics for full, partial, and control treatments. Thirty-six participants with bilateral sensorineural hearing losses and reduced DRs, which affected their actual or perceived ability to use hearing aids, were enrolled in and completed a placebo-controlled (for sound therapy) randomized clinical trial. The 2 × 2 factorial trial design was implemented with or without various assignments of counseling and sound therapy. Specifically, participants were assigned randomly to one of four treatment groups (nine participants per group), including: (1) group 1—full treatment achieved with scripted counseling plus sound therapy implemented with binaural sound generators; (2) group 2—partial treatment achieved with counseling and placebo sound generators (PSGs); (3) group 3—partial treatment achieved with binaural sound generators alone; and (4) group 4—a neutral control treatment implemented with the PSGs alone. Repeated measurements of categorical loudness judgments served as the primary outcome measure. The full-treatment categorical-loudness judgments for group 1, measured at treatment termination, were

A Sound Therapy-Based Intervention to Expand the Auditory Dynamic Range for Loudness among Persons with Sensorineural Hearing Losses: A Randomized Placebo-Controlled Clinical Trial.

PubMed

Formby, Craig; Hawley, Monica L; Sherlock, LaGuinn P; Gold, Susan; Payne, JoAnne; Brooks, Rebecca; Parton, Jason M; Juneau, Roger; Desporte, Edward J; Siegle, Gregory R

2015-05-01

The primary aim of this research was to evaluate the validity, efficacy, and generalization of principles underlying a sound therapy-based treatment for promoting expansion of the auditory dynamic range (DR) for loudness. The basic sound therapy principles, originally devised for treatment of hyperacusis among patients with tinnitus, were evaluated in this study in a target sample of unsuccessfully fit and/or problematic prospective hearing aid users with diminished DRs (owing to their elevated audiometric thresholds and reduced sound tolerance). Secondary aims included: (1) delineation of the treatment contributions from the counseling and sound therapy components to the full-treatment protocol and, in turn, the isolated treatment effects from each of these individual components to intervention success; and (2) characterization of the respective dynamics for full, partial, and control treatments. Thirty-six participants with bilateral sensorineural hearing losses and reduced DRs, which affected their actual or perceived ability to use hearing aids, were enrolled in and completed a placebo-controlled (for sound therapy) randomized clinical trial. The 2 × 2 factorial trial design was implemented with or without various assignments of counseling and sound therapy. Specifically, participants were assigned randomly to one of four treatment groups (nine participants per group), including: (1) group 1-full treatment achieved with scripted counseling plus sound therapy implemented with binaural sound generators; (2) group 2-partial treatment achieved with counseling and placebo sound generators (PSGs); (3) group 3-partial treatment achieved with binaural sound generators alone; and (4) group 4-a neutral control treatment implemented with the PSGs alone. Repeated measurements of categorical loudness judgments served as the primary outcome measure. The full-treatment categorical-loudness judgments for group 1, measured at treatment termination, were significantly

The Natural History and Rehabilitative Outcomes of Hearing Loss in Congenital Cytomegalovirus: A Systematic Review.

PubMed

Fletcher, Kyle T; Horrell, Erin M Wolf; Ayugi, John; Irungu, Catherine; Muthoka, Maria; Creel, Liza M; Lester, Cathy; Bush, Matthew L

2018-06-15

The purpose of this study was to examine the literature regarding the natural history and rehabilitative outcomes of sensorineural hearing loss from congenital cytomegalovirus infections. A systematic search was performed in PubMed, PsychINFO, CINAHL, and Web of Science to identify peer-reviewed research. Eligible studies were those containing original peer-reviewed research in English addressing either the natural history or rehabilitative outcomes of sensorineural hearing loss (SNHL) in congenital cytomegalovirus (cCMV). Two investigators independently reviewed all articles and extracted data. Bias was assessed using the Cochrane Collaboration's tool and the Newcastle-Ottawa Assessment Scale. Thirty-six articles were reviewed. Universal screening identifies 0.2 to 1% of newborns with cCMV infection. SNHL ranged from 8 to 32% of infants and was more prevalent in symptomatic versus asymptomatic cases. Nine to 68% of hearing loss occurs in a late or delayed fashion. In 7 to 71% of cases hearing loss is progressive. Cochlear implantation (CI) is a viable option for patients with cCMV associated hearing loss and leads to improvements in hearing and language. There is limited literature comparing rehabilitation outcomes in cCMV and non-cCMV CI recipients. Late onset and progressive hearing loss is seen in children who develop hearing loss from cCMV. Frequent audiologic follow-up is necessary considering the natural history of cCMV hearing loss. Universal screening should be pursued due to the number of asymptomatic children, at birth, who develop late onset/delayed hearing loss. CI is an effective means of improving speech and language in this population.

No Association Between Time of Onset of Hearing Loss (Childhood Versus Adulthood) and Self-Reported Hearing Handicap in Adults.

PubMed

Aarhus, Lisa; Tambs, Kristian; Engdahl, Bo

2015-12-01

This study examined the association between time of onset of hearing loss (childhood vs. adulthood) and self-reported hearing handicap in adults. This is a population-based cohort study of 2,024 adults (mean = 48 years) with hearing loss (binaural pure-tone average 0.5-4 kHz ≥ 20 dB HL) who completed a hearing handicap questionnaire. In childhood, the same persons (N = 2,024) underwent audiometry in a school investigation (at ages 7, 10, and 13 years), in which 129 were diagnosed with sensorineural hearing loss (binaural pure-tone average 0.5-4 kHz ≥ 20 dB HL), whereas 1,895 had normal hearing thresholds. Hearing handicap was measured in adulthood as the sum-score of various speech perception and social impairment items (15 items). The sum-score increased with adult hearing threshold level (p < .001). After adjustment for adult hearing threshold level, hearing aid use, adult age, sex, and socioeconomic status, there was no significant difference in hearing handicap sum-score between the group with childhood-onset hearing loss (n = 129) and the group with adult-onset hearing loss (n = 1,895; p = .882). Self-reported hearing handicap in adults increased with hearing threshold level. After adjustment for adult hearing threshold level, this cohort study revealed no significant association between time of onset of hearing loss (childhood vs. adulthood) and self-reported hearing handicap.

Validity of hearing impairment calculation methods for prediction of self-reported hearing handicap.

PubMed

John, Andrew B; Kreisman, Brian M; Pallett, Stephen

2012-01-01

Worker's compensation for hearing loss caused by occupational noise exposure is calculated by varying methods, from state to state within the United States (US), with many employing arithmetic formulas based on the pure-tone audiogram, to quantify hearing loss. Several assumptions unsupported or weakly supported by empirical data underlie these formulas. The present study evaluated the ability of various arithmetic hearing impairment calculations to predict a self-reported hearing handicap in a sample of presenting with sensorineural hearing loss. 204 adults (127 male, 77 female) ranging in age from 18 to 94 served as participants. The sample was selected to exclude patients who had been referred for hearing testing for a medicolegal examination or a hearing conservation appointment. A hearing handicap was measured by the Hearing Handicap Inventory for Adults/for the Elderly (HHIA/E). The covariance analysis of linear structural equations was used to assess the relative strength of correlation with the HHIA/E score among the six formulas and various forms of pure-tone average. The results revealed that all the hearing impairment calculations examined were significantly, but weakly, correlated with the self-reported hearing impairment scores. No significant differences among the predictive abilities of the impairment calculations were evident; however, the average binaural impairment assigned differed significantly among the six calculations examined. Individuals who demonstrated 0% impairment had significantly lower (i.e., better) HHIA/E scores compared to those with non-zero impairment for each formula. These results supported the idea that audiometric data provided an insufficient explanation for real-world hearing difficulties.

Genetic and Pharmacological Intervention for Treatment/Prevention of Hearing Loss

ERIC Educational Resources Information Center

Cotanche, Douglas A.

2008-01-01

Twenty years ago it was first demonstrated that birds could regenerate their cochlear hair cells following noise damage or aminoglycoside treatment. An understanding of how this structural and functional regeneration occurred might lead to the development of therapies for treatment of sensorineural hearing loss in humans. Recent experiments have…

Endocrine Glands and Hearing: Auditory Manifestations of Various Endocrine and Metabolic Conditions

PubMed Central

Cherian, Kripa Elizabeth; Kapoor, Nitin; Mathews, Suma Susan; Paul, Thomas Vizhalil

2017-01-01

The aetiology of hearing loss in humans is multifactorial. Besides genetic, environmental and infectious causes, several endocrine and metabolic abnormalities are associated with varying degrees of hearing impairment. The pattern of hearing loss may be conductive, sensori-neural or mixed. The neurophysiology of hearing as well as the anatomical structure of the auditory system may be influenced by changes in the hormonal and metabolic milieu. Optimal management of these conditions requires the integrated efforts of the otolaryngologist and the endocrinologist. The presence of hearing loss especially in the young age group should prompt the clinician to explore the possibility of an associated endocrine or metabolic disorder for timely referral and early initiation of treatment. PMID:28553606

A novel WFS1 mutation in a family with dominant low frequency sensorineural hearing loss with normal VEMP and EcochG findings

PubMed Central

Bramhall, Naomi F; Kallman, Jeremy C; Verrall, Aimee M; Street, Valerie A

2008-01-01

Background Low frequency sensorineural hearing loss (LFSNHL) is an uncommon clinical finding. Mutations within three different identified genes (DIAPH1, MYO7A, and WFS1) are known to cause LFSNHL. The majority of hereditary LFSNHL is associated with heterozygous mutations in the WFS1 gene (wolframin protein). The goal of this study was to use genetic analysis to determine if a small American family's hereditary LFSNHL is linked to a mutation in the WFS1 gene and to use VEMP and EcochG testing to further characterize the family's audiovestibular phenotype. Methods The clinical phenotype of the American family was characterized by audiologic testing, vestibular evoked myogenic potentials (VEMP), and electrocochleography (EcochG) evaluation. Genetic characterization was performed by microsatellite analysis and direct sequencing of WFS1 for mutation detection. Results Sequence analysis of the WFS1 gene revealed a novel heterozygous mutation at c.2054G>C predicting a p.R685P amino acid substitution in wolframin. The c.2054G>C mutation segregates faithfully with hearing loss in the family and is absent in 230 control chromosomes. The p.R685 residue is located within the hydrophilic C-terminus of wolframin and is conserved across species. The VEMP and EcochG findings were normal in individuals segregating the WFS1 c.2054G>C mutation. Conclusion We discovered a novel heterozygous missense mutation in exon 8 of WFS1 predicting a p.R685P amino acid substitution that is likely to underlie the LFSNHL phenotype in the American family. For the first time, we describe VEMP and EcochG findings for individuals segregating a heterozygous WFS1 mutation. PMID:18518985

A novel WFS1 mutation in a family with dominant low frequency sensorineural hearing loss with normal VEMP and EcochG findings.

PubMed

Bramhall, Naomi F; Kallman, Jeremy C; Verrall, Aimee M; Street, Valerie A

2008-06-02

Low frequency sensorineural hearing loss (LFSNHL) is an uncommon clinical finding. Mutations within three different identified genes (DIAPH1, MYO7A, and WFS1) are known to cause LFSNHL. The majority of hereditary LFSNHL is associated with heterozygous mutations in the WFS1 gene (wolframin protein). The goal of this study was to use genetic analysis to determine if a small American family's hereditary LFSNHL is linked to a mutation in the WFS1 gene and to use VEMP and EcochG testing to further characterize the family's audiovestibular phenotype. The clinical phenotype of the American family was characterized by audiologic testing, vestibular evoked myogenic potentials (VEMP), and electrocochleography (EcochG) evaluation. Genetic characterization was performed by microsatellite analysis and direct sequencing of WFS1 for mutation detection. Sequence analysis of the WFS1 gene revealed a novel heterozygous mutation at c.2054G>C predicting a p.R685P amino acid substitution in wolframin. The c.2054G>C mutation segregates faithfully with hearing loss in the family and is absent in 230 control chromosomes. The p.R685 residue is located within the hydrophilic C-terminus of wolframin and is conserved across species. The VEMP and EcochG findings were normal in individuals segregating the WFS1 c.2054G>C mutation. We discovered a novel heterozygous missense mutation in exon 8 of WFS1 predicting a p.R685P amino acid substitution that is likely to underlie the LFSNHL phenotype in the American family. For the first time, we describe VEMP and EcochG findings for individuals segregating a heterozygous WFS1 mutation.

Consonant-recognition patterns and self-assessment of hearing handicap.

PubMed

Hustedde, C G; Wiley, T L

1991-12-01

Two companion experiments were conducted with normal-hearing subjects and subjects with high-frequency, sensorineural hearing loss. In Experiment 1, the validity of a self-assessment device of hearing handicap was evaluated in two groups of hearing-impaired listeners with significantly different consonant-recognition ability. Data for the Hearing Performance Inventory--Revised (Lamb, Owens, & Schubert, 1983) did not reveal differences in self-perceived handicap for the two groups of hearing-impaired listeners; it was sensitive to perceived differences in hearing abilities for listeners who did and did not have a hearing loss. Experiment 2 was aimed at evaluation of consonant error patterns that accounted for observed group differences in consonant-recognition ability. Error patterns on the Nonsense-Syllable Test (NST) across the two subject groups differed in both degree and type of error. Listeners in the group with poorer NST performance always demonstrated greater difficulty with selected low-frequency and high-frequency syllables than did listeners in the group with better NST performance. Overall, the NST was sensitive to differences in consonant-recognition ability for normal-hearing and hearing-impaired listeners.

The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome.

PubMed

Koyama, Hajime; Kashio, Akinori; Sakata, Aki; Tsutsumiuchi, Katsuhiro; Matsumoto, Yu; Karino, Shotaro; Kakigi, Akinobu; Iwasaki, Shinichi; Yamasoba, Tatsuya

2016-01-01

Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months). Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome.

The Hearing Outcomes of Cochlear Implantation in Waardenburg Syndrome

PubMed Central

Koyama, Hajime; Kashio, Akinori; Sakata, Aki; Tsutsumiuchi, Katsuhiro; Matsumoto, Yu; Karino, Shotaro; Kakigi, Akinobu; Iwasaki, Shinichi; Yamasoba, Tatsuya

2016-01-01

Objectives. This study aimed to determine the feasibility of cochlear implantation for sensorineural hearing loss in patients with Waardenburg syndrome. Method. A retrospective chart review was performed on patients who underwent cochlear implantation at the University of Tokyo Hospital. Clinical classification, genetic mutation, clinical course, preoperative hearing threshold, high-resolution computed tomography of the temporal bone, and postoperative hearing outcome were assessed. Result. Five children with Waardenburg syndrome underwent cochlear implantation. The average age at implantation was 2 years 11 months (ranging from 1 year 9 months to 6 years 3 months). Four patients had congenital profound hearing loss and one patient had progressive hearing loss. Two patients had an inner ear malformation of cochlear incomplete partition type 2. No surgical complication or difficulty was seen in any patient. All patients showed good hearing outcome postoperatively. Conclusion. Cochlear implantation could be a good treatment option for Waardenburg syndrome. PMID:27376080

Hearing Loss After Radiotherapy for Pediatric Brain Tumors: Effect of Cochlear Dose

DOE Office of Scientific and Technical Information (OSTI.GOV)

Hua, Chiaho; Bass, Johnnie K.; Khan, Raja

Purpose: To determine the effect of cochlear dose on sensorineural hearing loss in pediatric patients with brain tumor treated by using conformal radiation therapy (CRT). Patients and Methods: We studied 78 pediatric patients (155 ears) with localized brain tumors treated in 1997-2001 who had not received platinum-based chemotherapy and were followed up for at least 48 months. They were evaluated prospectively by means of serial pure-tone audiograms (250 Hz-8 kHz) and/or auditory brainstem response before and every 6 months after CRT. Results: Hearing loss occurred in 14% (11 of 78) of patients and 11% (17 of 155) of cochleae, withmore » onset most often at 3-5 years after CRT. The incidence of hearing loss was low for a cochlear mean dose of 30 Gy or less and increased at greater than 40-45 Gy. Risk was greater at high frequencies (6-8 kHz). In children who tested abnormal for hearing, average hearing thresholds increased from a less than 25 decibel (dB) hearing level (HL) at baseline to a mean of 46 {+-} 13 (SD) dB HL for high frequencies, 41 {+-} 7 dB HL for low frequencies, and 38 {+-} 6 dB HL for intermediate frequencies. Conclusions: Sensorineural hearing loss is a late effect of CRT. In the absence of other factors, including ototoxic chemotherapy, increase in cochlear dose correlates positively with hearing loss in pediatric patients with brain tumor. To minimize the risk of hearing loss for children treated with radiation therapy, a cumulative cochlear dose less than 35 Gy is recommended for patients planned to receive 54-59.4 Gy in 30-33 treatment fractions.« less

Vocabulary and Working Memory in Children Fit with Hearing Aids

ERIC Educational Resources Information Center

Stiles, Derek J.; McGregor, Karla K.; Bentler, Ruth A.

2012-01-01

Purpose: To determine whether children with mild-to-moderately severe sensorineural hearing loss (CHL) present with disturbances in working memory and whether these disturbances relate to the size of their receptive vocabularies. Method: Children 6 to 9 years of age participated. Aspects of working memory were tapped by articulation rate, forward…

Acute auditory agnosia as the presenting hearing disorder in MELAS.

PubMed

Miceli, Gabriele; Conti, Guido; Cianfoni, Alessandro; Di Giacopo, Raffaella; Zampetti, Patrizia; Servidei, Serenella

2008-12-01

MELAS is commonly associated with peripheral hearing loss. Auditory agnosia is a rare cortical auditory impairment, usually due to bilateral temporal damage. We document, for the first time, auditory agnosia as the presenting hearing disorder in MELAS. A young woman with MELAS (A3243G mtDNA mutation) suffered from acute cortical hearing damage following a single stroke-like episode, in the absence of previous hearing deficits. Audiometric testing showed marked central hearing impairment and very mild sensorineural hearing loss. MRI documented bilateral, acute lesions to superior temporal regions. Neuropsychological tests demonstrated auditory agnosia without aphasia. Our data and a review of published reports show that cortical auditory disorders are relatively frequent in MELAS, probably due to the strikingly high incidence of bilateral and symmetric damage following stroke-like episodes. Acute auditory agnosia can be the presenting hearing deficit in MELAS and, conversely, MELAS should be suspected in young adults with sudden hearing loss.

[The problems of hearing impairment in the flying staff of commercial aviation in Russia].

PubMed

Pankova, V B; Bushmanov, A Iu

2014-01-01

The authors discuss the problems pertaining to the growing incidence of hearing impairment in the members of the flying staff employed in commercial aviation of Russia and the main criteria used to elucidate the causes behind occupational diseases of the organs of hearing. Special attention is given to the principal normative documents regulating the methodological basis on which the acoustic factor in the aircraft cockpit is evaluated, peculiarities of occupational sensorineural hearing impairment and the methods for its detection. The main errors in the determination of the relationship between the working conditions and the diseases of the organs of hearing are discussed.

Pseudoexfoliation Syndrome and Sensorineural Hearing Loss

PubMed Central

Zojaji, Ramin; Alesheykh, Ali; Sedaghat, Mohammad Reza; Navia, Kiamarz; Mazloom Farsi Baf, Morteza; Khaki, Masoud; Raouf, Aliasghar

2011-01-01

Introduction: Pseudoexfoliation syndrome (PXS) occurs due to the deposition of extracellular fibrillar materials on the anterior chamber of the eye. This syndrome has been considered to be part of a systemic disease with the potential involvement of the inner ear called sensoroneural hearing loss (SNHL). In this study, we aimed on evaluating SNHL within PXS patients in Iran to compare them with other international reports. Materials and Methods: In total, 33 patients with PXS and 33 age and sex matched controls were enrolled prospectively in a case-control study. Both groups underwent complete ophthalmologic and otorhinolaryngologic examinations and pure tone audiometry (PTA) testing. Six frequencies (0.25, 0.5, 1, 2, 3, 4 and 6 KHz) were evaluated for PTA in the same ethnic group in order to select the case and control individuals. Data were analyzed using t-test and chi-square test. Results: Forty-nine out of 66 ears (75.2%) in the PXS group and 27 ears (40.9%) in the control group had SNHL (P<0.001). No significant difference was found between the existence of exfoliative glaucoma (EXG) and SNHL in the PXS patients (P=0.768). Conclusion: Our results indicate a significant association between PXS and SNHL and may support the systemic nature of this disease. PMID:24303375

Hearing parameters in noise exposed industrial workers.

PubMed

Celik, O; Yalçin, S; Oztürk, A

1998-12-01

This paper presents the results of a study carried out in a group of noise-exposed workers in a hydro-electric power plant. Thus, the main focus of the study is on 130 industrial workers who were exposed to high level of noise. The control group was consisted of 33 subjects with normal hearing. Hearing and acoustic reflex thresholds were obtained from all subjects and the results from age-matched subgroups were compared. The sensorineural hearing loss which were detected in 71 workers were bilateral, symmetrical and affected mainly frequencies of 4-6 kHz. In essence, the hearing losses were developed within the first 10 years of noise exposure and associated with slight progress in the following years. When acoustic reflex thresholds derived from the study and control groups were compared, statistically significant difference was determined only for the thresholds obtained at 4 kHz (p < 0.0005).

Estrogen and hearing from a clinical point of view; characteristics of auditory function in women with Turner syndrome.

PubMed

Hederstierna, Christina; Hultcrantz, Malou; Rosenhall, Ulf

2009-06-01

Turner syndrome is a chromosomal aberration affecting 1:2000 newborn girls, in which all or part of one X chromosome is absent. This leads to ovarial dysgenesis and little or no endogenous estrogen production. These women have, among many other syndromal features, a high occurrence of ear and hearing problems, and neurocognitive dysfunctions, including reduced visual-spatial abilities; it is assumed that estrogen deficiency is at least partially responsible for these problems. In this, study 30 Turner women aged 40-67, with mild to moderate hearing loss, performed a battery of hearing tests aimed at localizing the lesion causing the sensorineural hearing impairment and assessing central auditory function, primarily sound localization. The results of TEOAE, ABR and speech recognition scores in noise were all indicative of cochlear dysfunction as the cause of the sensorineural impairment. Phase audiometry, a test for sound localization, showed mild disturbances in the Turner women compared to the reference group, suggesting that auditory-spatial dysfunction is another facet of the recognized neurocognitive phenotype in Turner women.

Using Speech Recall in Hearing Aid Fitting and Outcome Evaluation Under Ecological Test Conditions.

PubMed

Lunner, Thomas; Rudner, Mary; Rosenbom, Tove; Ågren, Jessica; Ng, Elaine Hoi Ning

2016-01-01

In adaptive Speech Reception Threshold (SRT) tests used in the audiological clinic, speech is presented at signal to noise ratios (SNRs) that are lower than those generally encountered in real-life communication situations. At higher, ecologically valid SNRs, however, SRTs are insensitive to changes in hearing aid signal processing that may be of benefit to listeners who are hard of hearing. Previous studies conducted in Swedish using the Sentence-final Word Identification and Recall test (SWIR) have indicated that at such SNRs, the ability to recall spoken words may be a more informative measure. In the present study, a Danish version of SWIR, known as the Sentence-final Word Identification and Recall Test in a New Language (SWIRL) was introduced and evaluated in two experiments. The objective of experiment 1 was to determine if the Swedish results demonstrating benefit from noise reduction signal processing for hearing aid wearers could be replicated in 25 Danish participants with mild to moderate symmetrical sensorineural hearing loss. The objective of experiment 2 was to compare direct-drive and skin-drive transmission in 16 Danish users of bone-anchored hearing aids with conductive hearing loss or mixed sensorineural and conductive hearing loss. In experiment 1, performance on SWIRL improved when hearing aid noise reduction was used, replicating the Swedish results and generalizing them across languages. In experiment 2, performance on SWIRL was better for direct-drive compared with skin-drive transmission conditions. These findings indicate that spoken word recall can be used to identify benefits from hearing aid signal processing at ecologically valid, positive SNRs where SRTs are insensitive.

Working Memory and Speech Comprehension in Older Adults with Hearing Impairment

ERIC Educational Resources Information Center

Nagaraj, Naveen K.

2017-01-01

Purpose: This study examined the relationship between working memory (WM) and speech comprehension in older adults with hearing impairment (HI). It was hypothesized that WM would explain significant variance in speech comprehension measured in multitalker babble (MTB). Method: Twenty-four older (59-73 years) adults with sensorineural HI…

Otoacoustic Emissions in an Adult with Severe Hearing Loss.

ERIC Educational Resources Information Center

Prieve, Beth A.; And Others

1991-01-01

The paper describes the unexpected finding of evoked otoacoustic emissions from one ear of a subject with severe-to-profound bilateral sensorineural hearing loss. It is suggested that the subject may have a group of surviving outer hair cells in some regions of the left cochlea with corresponding inner hair cell or neural damage. (Author/DB)

Sensorineural hearing loss--a common finding in early-onset type 2 diabetes mellitus.

PubMed

Lerman-Garber, Israel; Cuevas-Ramos, Daniel; Valdés, Samantha; Enríquez, Lorena; Lobato, Marlette; Osornio, Melannie; Escobedo, Ana Rosa; Pascual-Ramos, Virginia; Mehta, Roopa; Ramírez-Anguiano, Jacqueline; Gómez-Pérez, Francisco J

2012-01-01

To evaluate the prevalence and potential associations of hearing impairment in patients 30 to 50 years old with diabetes diagnosed before age 40 years-early-onset type 2 diabetes mellitus (T2DM). The study cohorts consisted of 46 consecutive patients with early-onset T2DM and 47 age-matched control subjects with rheumatoid arthritis. All study subjects completed clinical, serologic, and auditory assessments. The patients with T2DM had a mean age of 42 ± 6 years and a mean disease duration of 11 ± 6 years. Microalbuminuria was present in 26.1%, proliferative retinopathy in 26.1%, and symptomatic peripheral neuropathy in 23.9%. The prevalence of unilateral or bilateral hearing loss was significantly higher in the patients with T2DM than in the patients with rheumatoid arthritis (21.7% versus 6.4%, respectively; P = .01). Most cases of hearing loss were mild and involved high or acute tones. After multivariate analysis with adjustment for age, there was a significant association between hearing loss and hemoglobin A1c (odds ratio, 1.3; 95% confidence interval, 1.02 to 1.81; P = .035). In the patients with T2DM, the lengthening of the brainstem response was not significantly increased; however, the wave morphologic features were abnormal and the reproducibility was poor in both ears in 11 patients (24%). Patients with early-onset T2DM and poor glycemic control have an increased prevalence of subclinical hearing loss and impaired auditory brainstem responses. Hearing impairment may be an underrecognized complication of diabetes.

No Association Between Time of Onset of Hearing Loss (Childhood Versus Adulthood) and Self-Reported Hearing Handicap in Adults

PubMed Central

Tambs, Kristian; Engdahl, Bo

2015-01-01

Purpose This study examined the association between time of onset of hearing loss (childhood vs. adulthood) and self-reported hearing handicap in adults. Methods This is a population-based cohort study of 2,024 adults (mean = 48 years) with hearing loss (binaural pure-tone average 0.5–4 kHz ≥ 20 dB HL) who completed a hearing handicap questionnaire. In childhood, the same persons (N = 2,024) underwent audiometry in a school investigation (at ages 7, 10, and 13 years), in which 129 were diagnosed with sensorineural hearing loss (binaural pure-tone average 0.5–4 kHz ≥ 20 dB HL), whereas 1,895 had normal hearing thresholds. Results Hearing handicap was measured in adulthood as the sum-score of various speech perception and social impairment items (15 items). The sum-score increased with adult hearing threshold level (p < .001). After adjustment for adult hearing threshold level, hearing aid use, adult age, sex, and socioeconomic status, there was no significant difference in hearing handicap sum-score between the group with childhood-onset hearing loss (n = 129) and the group with adult-onset hearing loss (n = 1,895; p = .882). Conclusion Self-reported hearing handicap in adults increased with hearing threshold level. After adjustment for adult hearing threshold level, this cohort study revealed no significant association between time of onset of hearing loss (childhood vs. adulthood) and self-reported hearing handicap. PMID:26649831

Congenital stapes malformation: Rare conductive hearing loss in a patient with Waardenburg syndrome.

PubMed

Melzer, Jonathan M; Eliason, Michael; Conley, George S

2016-04-01

Waardenburg syndrome is a known autosomal dominant cause of congenital hearing loss. It is characterized by a distinctive phenotypic appearance and often involves sensorineural hearing loss. Temporal bone abnormalities and inner ear dysmorphisms have been described in association with the disease. However, middle ear abnormalities as causes of conductive hearing loss are not typically seen in Waardenburg syndrome. We discuss a case of an 8-year-old female who meets diagnostic criteria for Waardenburg syndrome type 3 and who presented with a bilateral conductive hearing loss associated with congenital stapes fixation. We discuss management strategy in this previously unreported phenotype. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

CEP250 mutations associated with mild cone-rod dystrophy and sensorineural hearing loss in a Japanese family.

PubMed

Kubota, Daiki; Gocho, Kiyoko; Kikuchi, Sachiko; Akeo, Keiichiro; Miura, Masahiro; Yamaki, Kunihiko; Takahashi, Hiroshi; Kameya, Shuhei

2018-05-02

CEP250 encodes the C-Nap1 protein which belongs to the CEP family of proteins. C-Nap1 has been reported to be expressed in the photoreceptor cilia and is known to interact with other ciliary proteins. Mutations of CEP250 cause atypical Usher syndrome which is characterized by early-onset sensorineural hearing loss (SNHL) and a relatively mild retinitis pigmentosa. This study tested the hypothesis that the mild cone-rod dystrophy (CRD) and SNHL in a non-consanguineous Japanese family was caused by CEP250 mutations. Detailed ophthalmic and auditory examinations were performed on the proband and her family members. Whole exome sequencing (WES) was used on the DNA obtained from the proband. Electrophysiological analysis revealed a mild CRD in two family members. Adaptive optics (AO) imaging showed reduced cone density around the fovea. Auditory examinations showed a slight SNHL in both patients. WES of the proband identified compound heterozygous variants c.361C>T, p.R121*, and c.562C>T, p.R188* in CEP250. The variants were found to co-segregate with the disease in five members of the family. The variants of CEP250 are both null variants and according to American College of Medical Genetics and Genomics (ACMG) standards and guideline, these variants are classified into the very strong category (PVS1). The criteria for both alleles will be pathogenic. Our data indicate that mutations of CEP250 can cause mild CRD and SNHL in Japanese patients. Because the ophthalmological phenotypes were very mild, high-resolution retinal imaging analysis, such as AO, will be helpful in diagnosing CEP250-associated disease.

Predicting hearing thresholds and occupational hearing loss with multiple-frequency auditory steady-state responses.

PubMed

Hsu, Ruey-Fen; Ho, Chi-Kung; Lu, Sheng-Nan; Chen, Shun-Sheng

2010-10-01

An objective investigation is needed to verify the existence and severity of hearing impairments resulting from work-related, noise-induced hearing loss in arbitration of medicolegal aspects. We investigated the accuracy of multiple-frequency auditory steady-state responses (Mf-ASSRs) between subjects with sensorineural hearing loss (SNHL) with and without occupational noise exposure. Cross-sectional study. Tertiary referral medical centre. Pure-tone audiometry and Mf-ASSRs were recorded in 88 subjects (34 patients had occupational noise-induced hearing loss [NIHL], 36 patients had SNHL without noise exposure, and 18 volunteers were normal controls). Inter- and intragroup comparisons were made. A predicting equation was derived using multiple linear regression analysis. ASSRs and pure-tone thresholds (PTTs) showed a strong correlation for all subjects (r = .77 ≈ .94). The relationship is demonstrated by the equationThe differences between the ASSR and PTT were significantly higher for the NIHL group than for the subjects with non-noise-induced SNHL (p < .001). Mf-ASSR is a promising tool for objectively evaluating hearing thresholds. Predictive value may be lower in subjects with occupational hearing loss. Regardless of carrier frequencies, the severity of hearing loss affects the steady-state response. Moreover, the ASSR may assist in detecting noise-induced injury of the auditory pathway. A multiple linear regression equation to accurately predict thresholds was shown that takes into consideration all effect factors.

Speech perception in noise in unilateral hearing loss.

PubMed

Mondelli, Maria Fernanda Capoani Garcia; Dos Santos, Marina de Marchi; José, Maria Renata

2016-01-01

Unilateral hearing loss is characterized by a decrease of hearing in one ear only. In the presence of ambient noise, individuals with unilateral hearing loss are faced with greater difficulties understanding speech than normal listeners. To evaluate the speech perception of individuals with unilateral hearing loss in speech perception with and without competitive noise, before and after the hearing aid fitting process. The study included 30 adults of both genders diagnosed with moderate or severe sensorineural unilateral hearing loss using the Hearing In Noise Test - Hearing In Noise Test-Brazil, in the following scenarios: silence, frontal noise, noise to the right, and noise to the left, before and after the hearing aid fitting process. The study participants had a mean age of 41.9 years and most of them presented right unilateral hearing loss. In all cases evaluated with Hearing In Noise Test, a better performance in speech perception was observed with the use of hearing aids. Using the Hearing In Noise Test-Brazil test evaluation, individuals with unilateral hearing loss demonstrated better performance in speech perception when using hearing aids, both in silence and in situations with a competing noise, with use of hearing aids. Copyright © 2015 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

The effectiveness of the directional microphone in the Oticon Medical Ponto Pro in participants with unilateral sensorineural hearing loss.

PubMed

Oeding, Kristi; Valente, Michael

2013-09-01

Current bone anchored hearing solutions (BAHSs) have incorporated automatic adaptive multichannel directional microphones (DMs). Previous fixed single-channel hypercardioid DMs in BAHSs have provided benefit in a diffuse listening environment, but little data are available on the performance of adaptive multichannel DMs in BAHSs for persons with unilateral sensorineural hearing loss (USNHL). The primary goal was to determine if statistically significant differences existed in the mean Reception Threshold for Sentences (RTS in dB) in diffuse uncorrelated restaurant noise between unaided, an omnidirectional microphone (OM), split DM (SDM), and full DM (FDM) in the Oticon Medical Ponto Pro. A second goal was to assess subjective benefit using the Abbreviated Profile of Hearing Aid Benefit (APHAB) comparing the Ponto Pro to the participant's current BAHS, and the Ponto Pro and participant's own BAHS to unaided. The third goal was to compare RTS data of the Ponto Pro to data from an identical study examining Cochlear Americas' Divino. A randomized repeated measures, single blind design was used to measure an RTS for each participant for unaided, OM, SDM, and FDM. Fifteen BAHS users with USNHL were recruited from Washington University in St. Louis and the surrounding area. The Ponto Pro was fit by measuring in-situ bone conduction thresholds and was worn for 4 wk. An RTS was obtained utilizing Hearing in Noise Test (HINT) sentences in uncorrelated restaurant noise from an eight loudspeaker array, and subjective benefit was determined utilizing the APHAB. Analysis of variance (ANOVA) was used to analyze the results of the Ponto Pro HINT and APHAB data, and comparisons between the Ponto Pro and previous Divino data. No statistically significant differences existed in mean RTS between unaided, the Ponto Pro's OM, SDM, or FDM (p = 0.10). The Ponto Pro provided statistically significant benefit for the Background Noise (BN) (p < 0.01) and Reverberation (RV) (p < 0

Successful bone-anchored hearing aid implantation in a patient with osteogenesis imperfecta.

PubMed

Coutinho, M B; Marques, C; Mendes, G J; Gonçalves, C

2015-11-01

To report a case of successful bone-anchored hearing aid implantation in an adult patient with type III osteogenesis imperfecta, which is commonly regarded as a contraindication to this procedure. A 45-year-old man with type III osteogenesis imperfecta presented with mixed hearing loss. There was a mild sensorineural component in both ears, with an air-bone gap between 45 and 50 dB HL. He was implanted with a bone-anchored hearing aid. The audiological outcome was good, with no complications and good implant stability (as measured by resonance frequency analysis). To our knowledge, this is the first recorded case of bone-anchored hearing aid implantation in a patient with osteogenesis imperfecta.

Delayed diagnosis of childhood deafness: the value of false negatives in the Programme for Early Detection of Neonatal Hearing Loss.

PubMed

Martínez-Pacheco, María C; Ferrán de la Cierva, Luis; García-Purriños, Francisco J

Despite its importance, the existence of false negatives (patients who are told they hear well, but they have some degree of hipacusia) is rarely evaluated in programs for early detection of hearing loss. The aim of this study is to determine the variables that can lead to a delayed diagnosis, especially the existence of false negatives and the lack of registration of risk factors. A retrospective study of prevalence has been carried out, in which the medical records of children diagnosed with sensorineural hearing loss born within 2005 and 2012 in the health centers of study have been analyzed. Of the 32 children with sensorineural hearing loss, 16 passed the OAE, 12 did not passed the OAE, and in four they were not carried out. Of the children who passed the OAE, 57% have severe hearing loss. 66% of children with hearing loss presented a risk factor for hearing loss at birth, being the most frecuent family history of hearing loss, but only 7% of those with family history of hearing loss were included in the risk group. The results of the study indicate that the late diagnosis of hearing loss is related to the presence of false negatives to the OAE and the non-registration of risk factors. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. All rights reserved.

Enhancing Auditory Selective Attention Using a Visually Guided Hearing Aid.

PubMed

Kidd, Gerald

2017-10-17

Listeners with hearing loss, as well as many listeners with clinically normal hearing, often experience great difficulty segregating talkers in a multiple-talker sound field and selectively attending to the desired "target" talker while ignoring the speech from unwanted "masker" talkers and other sources of sound. This listening situation forms the classic "cocktail party problem" described by Cherry (1953) that has received a great deal of study over the past few decades. In this article, a new approach to improving sound source segregation and enhancing auditory selective attention is described. The conceptual design, current implementation, and results obtained to date are reviewed and discussed in this article. This approach, embodied in a prototype "visually guided hearing aid" (VGHA) currently used for research, employs acoustic beamforming steered by eye gaze as a means for improving the ability of listeners to segregate and attend to one sound source in the presence of competing sound sources. The results from several studies demonstrate that listeners with normal hearing are able to use an attention-based "spatial filter" operating primarily on binaural cues to selectively attend to one source among competing spatially distributed sources. Furthermore, listeners with sensorineural hearing loss generally are less able to use this spatial filter as effectively as are listeners with normal hearing especially in conditions high in "informational masking." The VGHA enhances auditory spatial attention for speech-on-speech masking and improves signal-to-noise ratio for conditions high in "energetic masking." Visual steering of the beamformer supports the coordinated actions of vision and audition in selective attention and facilitates following sound source transitions in complex listening situations. Both listeners with normal hearing and with sensorineural hearing loss may benefit from the acoustic beamforming implemented by the VGHA, especially for nearby

Hearing aids: Do they help and, if so, how does one know?

NASA Astrophysics Data System (ADS)

Humes, Larry E.

2003-04-01

For those individuals with sensorineural hearing loss, ranging from mild to severe in degree, the conventional hearing aid is the most appropriate rehabilitative device available. Despite the fact that such devices have been available commercially for over 60 years, until recently, relatively little research has been directed at evaluating the effectiveness of these rehabilitative devices. How does one evaluate the effectiveness of a hearing aid as a rehabilitative device? Should effectiveness be based on the relative improvement in communication with and without the hearing aid, typically referred to as hearing-aid benefit, the satisfaction of the consumer with the device, or simply whether and how much the hearing aid is used? How are these aspects of hearing-aid effectiveness or outcome measured? Are the measures of hearing-aid outcome related to one another? What evidence is there regarding the effectiveness of contemporary hearing aids? Recent research regarding these and other related questions will be reviewed in this presentation. [Work supported, in part, by NIA.

Simultaneous versus Sequential Intratympanic Steroid Treatment for Severe-to-Profound Sudden Sensorineural Hearing Loss.

PubMed

Yoo, Myung Hoon; Lim, Won Sub; Park, Joo Hyun; Kwon, Joong Keun; Lee, Tae-Hoon; An, Yong-Hwi; Kim, Young-Jin; Kim, Jong Yang; Lim, Hyun Woo; Park, Hong Ju

2016-01-01

Severe-to-profound sudden sensorineural hearing loss (SSNHL) has a poor prognosis. We aimed to compare the efficacy of simultaneous and sequential oral and intratympanic steroids for this condition. Fifty patients with severe-to-profound SSNHL (>70 dB HL) were included from 7 centers. The simultaneous group (27 patients) received oral and intratympanic steroid injections for 2 weeks. The sequential group (23 patients) was treated with oral steroids for 2 weeks and intratympanic steroids for the subsequent 2 weeks. Pure-tone averages (PTA) and word discrimination scores (WDS) were compared before treatment and 2 weeks and 1 and 2 months after treatment. Treatment outcomes according to the modified American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) criteria were also analyzed. The improvement in PTA and WDS at the 2-week follow-up was 23 ± 21 dB HL and 20 ± 39% in the simultaneous group and 31 ± 29 dB HL and 37 ± 42% in the sequential group; this was not statistically significant. Complete or partial recovery at the 2-week follow-up was observed in 26% of the simultaneous group and 30% of the sequential group; this was also not significant. The improvement in PTA and WDS at the 2-month follow-up was 40 ± 20 dB HL and 37 ± 35% in the simultaneous group and 41 ± 25 dB HL and 48 ± 41% in the sequential group; this was not statistically significant. Complete or partial recovery at the 2-month follow-up was observed in 33% of the simultaneous group and 35% of the sequential group; this was also not significant. Seven patients in the sequential group did not need intratympanic steroid injections for sufficient improvement after oral steroids alone. Simultaneous oral/intratympanic steroid treatment yielded a recovery similar to that produced by sequential treatment. Because the addition of intratympanic steroids can be decided upon based on the improvement after an oral steroid, the sequential regimen can be recommended to avoid unnecessary

Incidence and relative risk of hearing disorders in professional musicians

PubMed Central

Schink, Tania; Kreutz, Gunter; Busch, Veronika; Pigeot, Iris; Ahrens, Wolfgang

2014-01-01

Background Hearing disorders have been associated with occupational exposure to music. Musicians may benefit from non-amplified and low-intensity music, but may also have high risks of music-induced hearing loss. Aims To compare the incidence of hearing loss (HL) and its subentities in professional musicians with that in the general population. Methods We performed a historical cohort study among insurants between 19 and 66 years who were employed subject to social insurance contributions. The study was conducted with data from three German statutory health insurance providers covering the years 2004–2008 with about 7 million insurants. Incidence rates with 95% CIs of HL and the subentities noise-induced hearing loss (NIHL), conductive HL, sensorineural HL, conductive and sensorineural HL, as well as tinnitus were estimated stratified by age, sex and federal state. A Cox regression analysis was conducted to estimate adjusted HRs and two-sided 95% CIs for HL and its subentities. Results More than 3 million insurants were eligible, of whom 2227 were identified as professional musicians (0.07%). During the 4-year observation period, 283 697cases of HL were seen, 238 of them among professional musicians (0.08%), leading to an unadjusted incidence rate ratio of 1.27. The adjusted hazard ratio of musicians was 1.45 (95% CI 1.28 to 1.65) for HL and 3.61 (95% CI 1.81 to 7.20) for NIHL. Conclusions Professional musicians have a high risk of contracting hearing disorders. Use of already available prevention measures should reduce the incidence of HL in professional musicians. PMID:24790053

Detection of Unilateral Hearing Loss by Stationary Wavelet Entropy.

PubMed

Zhang, Yudong; Nayak, Deepak Ranjan; Yang, Ming; Yuan, Ti-Fei; Liu, Bin; Lu, Huimin; Wang, Shuihua

2017-01-01

Sensorineural hearing loss is correlated to massive neurological or psychiatric disease. T1-weighted volumetric images were acquired from fourteen subjects with right-sided hearing loss (RHL), fifteen subjects with left-sided hearing loss (LHL), and twenty healthy controls (HC). We treated a three-class classification problem: HC, LHL, and RHL. Stationary wavelet entropy was employed to extract global features from magnetic resonance images of each subject. Those stationary wavelet entropy features were used as input to a single-hidden layer feedforward neuralnetwork classifier. The 10 repetition results of 10-fold cross validation show that the accuracies of HC, LHL, and RHL are 96.94%, 97.14%, and 97.35%, respectively. Our developed system is promising and effective in detecting hearing loss. Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.org.

Cochlear implants as a treatment option for unilateral hearing loss, severe tinnitus and hyperacusis.

PubMed

Ramos Macías, Angel; Falcón González, Juan Carlos; Manrique, Manuel; Morera, Constantino; García-Ibáñez, Luis; Cenjor, Carlos; Coudert-Koall, Chrystellel; Killian, Matthijs

2015-01-01

Tinnitus is an incapacitating condition commonly affecting cochlear implant (CI) candidates. The aim of this clinical study is to assess the long-term effects of CI treatment in patients with severe-to-profound, sensorineural, unilateral hearing loss (UHL) and incapacitating tinnitus. We performed a prospective Cochlear™ company-sponsored multicentre study in five Spanish centres. Sixteen patients with UHL and incapacitating tinnitus, which was indicated by a Tinnitus Handicap Inventory (THI) score >58%, received a Nucleus® CI in their deaf ear. The study design includes repeated within-subject measures on hearing, tinnitus, hyperacusis and quality of life up to 12 months after initial CI fitting. In addition to hearing loss and tinnitus, all patients suffered from hyperacusis. Most patients had a sudden hearing loss and received a CI within 2 years after their hearing loss. Preliminary 6-month, post-CI activation data of 13 subjects showed that the majority of patients perceived a subjective benefit from CI treatment, which was assessed using the THI, a Visual Analogue Scale of tinnitus loudness/annoyance and the Speech, Spatial and Qualities of Hearing Scale. Preliminary 12-month data of 7 subjects showed that most patients also perceived a degree of relief from their hyperacusis. One patient showed no improvements in any of the applied scales, which could be explained by partial insertion of the electrode due to obstruction of the cochlea by otosclerosis. In conclusion, CI can successfully be used in the treatment of UHL patients with accompanying severe tinnitus and hyperacusis. Implantation resulted in hearing benefits and a durable relief from tinnitus and hyperacusis in the majority of patients. These findings support the hypothesis that pathophysiological mechanisms after peripheral sensorineural hearing loss are at least partly reversible when hearing is restored with a CI. © 2015 S. Karger AG, Basel.

Hearing Loss in Osteogenesis Imperfecta: Characteristics and Treatment Considerations

PubMed Central

Pillion, Joseph P.; Vernick, David; Shapiro, Jay

2011-01-01

Osteogenesis imperfecta (OI) is the most common heritable disorder of connective tissue. It is associated with fractures following relatively minor injury, blue sclerae, dentinogenesis imperfecta, increased joint mobility, short stature, and hearing loss. Structures in the otic capsule and inner ear share in the histologic features common to other skeletal tissues. OI is due to mutations involving several genes, the most commonly involved are the COL1A1 or COL1A2 genes which are responsible for the synthesis of the proalpha-1 and proalpha-2 polypeptide chains that form the type I collagen triple helix. A genotype/phenotype relationship to hearing loss has not been established in OI. Hearing loss is commonly found in OI with prevalence rates ranging from 50 to 92% in some studies. Hearing loss in OI may be conductive, mixed, or sensorineural and is more common by the second or third decade. Treatment options such as hearing aids, stapes surgery, and cochlear implants are discussed. PMID:22567374

Subjective and Objective Effects of Fast and Slow Compression on the Perception of Reverberant Speech in Listeners with Hearing Loss

ERIC Educational Resources Information Center

Shi, Lu-Feng; Doherty, Karen A.

2008-01-01

Purpose: The purpose of the current study was to assess the effect of fast and slow attack/release times (ATs/RTs) on aided perception of reverberant speech in quiet. Method: Thirty listeners with mild-to-moderate sensorineural hearing loss were tested monaurally with a commercial hearing aid programmed in 3 AT/RT settings: linear, fast (AT = 9…

Hearing loss in the shaken baby syndrome.

PubMed

Alzahrani, Musaed; Ratelle, Justine; Cavel, Oren; Laberge-Malo, Marie; Saliba, Issam

2014-05-01

To evaluate hearing in children diagnosed with shaken baby syndrome. A retrospective study conducted in a pediatric tertiary care center between 2006 and 2012. Children diagnosed with shaken baby syndrome were included for hearing evaluation by conventional audiometry, distortion product otoacoustic emissions and auditory brainstem responses. Twenty-eight children were included (22 boys and 6 girls). The mean age of children at presentation was 8 months (range 1-26 months) and the mean delay before audiometric evaluation was 30 months (range 1-87 months). One child was diagnosed as having a moderate sensorineural hearing loss. The tympanic membrane mobility was normal (type A) for both ears in 22 children, one child had a reduced tympanic mobility in one ear, two children had a negative pressure, one child had a functional trans-tympanic tube and test was not performed in 2 patients. This is the first study reporting hearing loss as a possible result of shaken baby syndrome. However, further studies with larger number of children would be preferable. We recommend hearing evaluation for these children to rule out hearing loss. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

Monitoring auditory cortical plasticity in hearing aid users with long latency auditory evoked potentials: a longitudinal study.

PubMed

Leite, Renata Aparecida; Magliaro, Fernanda Cristina Leite; Raimundo, Jeziela Cristina; Bento, Ricardo Ferreira; Matas, Carla Gentile

2018-02-19

The objective of this study was to compare long-latency auditory evoked potentials before and after hearing aid fittings in children with sensorineural hearing loss compared with age-matched children with normal hearing. Thirty-two subjects of both genders aged 7 to 12 years participated in this study and were divided into two groups as follows: 14 children with normal hearing were assigned to the control group (mean age 9 years and 8 months), and 18 children with mild to moderate symmetrical bilateral sensorineural hearing loss were assigned to the study group (mean age 9 years and 2 months). The children underwent tympanometry, pure tone and speech audiometry and long-latency auditory evoked potential testing with speech and tone burst stimuli. The groups were assessed at three time points. The study group had a lower percentage of positive responses, lower P1-N1 and P2-N2 amplitudes (speech and tone burst), and increased latencies for the P1 and P300 components following the tone burst stimuli. They also showed improvements in long-latency auditory evoked potentials (with regard to both the amplitude and presence of responses) after hearing aid use. Alterations in the central auditory pathways can be identified using P1-N1 and P2-N2 amplitude components, and the presence of these components increases after a short period of auditory stimulation (hearing aid use). These findings emphasize the importance of using these amplitude components to monitor the neuroplasticity of the central auditory nervous system in hearing aid users.

Monitoring auditory cortical plasticity in hearing aid users with long latency auditory evoked potentials: a longitudinal study

PubMed Central

Leite, Renata Aparecida; Magliaro, Fernanda Cristina Leite; Raimundo, Jeziela Cristina; Bento, Ricardo Ferreira; Matas, Carla Gentile

2018-01-01

OBJECTIVE: The objective of this study was to compare long-latency auditory evoked potentials before and after hearing aid fittings in children with sensorineural hearing loss compared with age-matched children with normal hearing. METHODS: Thirty-two subjects of both genders aged 7 to 12 years participated in this study and were divided into two groups as follows: 14 children with normal hearing were assigned to the control group (mean age 9 years and 8 months), and 18 children with mild to moderate symmetrical bilateral sensorineural hearing loss were assigned to the study group (mean age 9 years and 2 months). The children underwent tympanometry, pure tone and speech audiometry and long-latency auditory evoked potential testing with speech and tone burst stimuli. The groups were assessed at three time points. RESULTS: The study group had a lower percentage of positive responses, lower P1-N1 and P2-N2 amplitudes (speech and tone burst), and increased latencies for the P1 and P300 components following the tone burst stimuli. They also showed improvements in long-latency auditory evoked potentials (with regard to both the amplitude and presence of responses) after hearing aid use. CONCLUSIONS: Alterations in the central auditory pathways can be identified using P1-N1 and P2-N2 amplitude components, and the presence of these components increases after a short period of auditory stimulation (hearing aid use). These findings emphasize the importance of using these amplitude components to monitor the neuroplasticity of the central auditory nervous system in hearing aid users. PMID:29466495

Hearing and neurodevelopmental outcomes for children with asymptomatic congenital cytomegalovirus infection: A systematic review.

PubMed

Bartlett, Adam W; McMullan, Brendan; Rawlinson, William D; Palasanthiran, Pamela

2017-09-06

Congenital CMV is one of the commonest congenital infections and a recognised cause of sensorineural hearing loss and neurodevelopmental impairment. Ninety percent are clinically inapparent at birth but are reported to be at risk of developing such abnormalities throughout childhood, the extent of which requires further elucidation. A systematic literature review was conducted using Medline and Embase databases, manual citation review, and personal libraries for articles reporting primary data on hearing and neurodevelopmental outcomes for children with asymptomatic congenital CMV. PROSPERO registration number CRD42015025407. Thirty-seven of 480 articles identified between 1969 and 2016 met the eligibility criteria. Twenty-nine of these contributed primary data on hearing outcomes and 20 on neurodevelopmental outcomes (12 of the 37 studies contributed data on both). Cumulative incidence of sensorineural hearing loss with follow-up to at least 5 years was 7% to 11%, which is more than healthy controls but less than children with symptomatic congenital CMV (34%-41%). The onset, course, and severity of hearing loss was variable with no reliable virological prognostic marker. In comparison to controls, children with asymptomatic congenital CMV did not perform worse than controls in neurodevelopmental assessments and performed better than children with symptomatic congenital CMV. Studies show children with asymptomatic congenital CMV are at increased risk of developing hearing loss but perform equally well on neurodevelopmental assessments when compared with healthy controls. There is no reliable virological marker to determine which infants will develop sequelae. Regular follow-up until school entry is supported by the literature. Copyright © 2017 John Wiley & Sons, Ltd.

A case-control analysis on the association between erectile dysfunction and sudden sensorineural hearing loss in Taiwan.

PubMed

Keller, Joseph J; Chen, Yi-Kuang; Lin, Herng-Ching

2012-05-01

Although the cause of sudden sensorineural hearing loss (SSNHL) is yet to be elucidated, many theories have been proposed regarding potentially contributory etiologies. One increasingly well-supported theory purports an underlying vascular pathomechanism. If this is the case, SSNHL may also associate with conditions comorbid with vascular diseases, such as erectile dysfunction (ED). However, no studies to date have investigated the association between ED and SSNHL. This study set out to estimate a putative association between ED and having been previously diagnosed with SSNHL using a population-based dataset with a case-control design. This study used administrative claim data from the Taiwan National Health Insurance program. We identified 4,504 patients with ED as the study group and randomly selected 22,520 patients as the comparison group. Conditional logistic regression was used to examine the association between ED and having previously received a diagnosis of SSNHL. The prevalence and risk of SSNHL between cases and controls were calculated. Of the sampled patients, 41 (0.15%) had been diagnosed with SSNHL before the index date; 22 (0.49% of the cases) were from the study group and 19 (0.08% of controls) were from the control group. Conditional logistic regression analysis revealed that after adjusting for the patient's monthly income, geographic location, hypertension, diabetes, hyperlipidemia, coronary heart disease, obesity, and alcohol abuse/alcohol dependence syndrome status, patients with ED were more likely than controls to have been diagnosed with SSNHL before the index date (odds ratio = 6.06, 95% confidence interval = 3.25-11.29). There was an association between ED and prior SSNHL. The results of this study add to the evidence supporting an underlying vascular pathomechanism regarding the development of SSNHL and highlight a need for clinicians dealing with SSNHL patients to be alert to the development of ED. © 2012 International Society for

Binaural speech discrimination under noise in hearing-impaired listeners.

PubMed

Kumar, K V; Rao, A B

1988-10-01

This study was undertaken to assess speech discrimination under binaural listening with background noise in hearing-impaired subjects. Subjects (58 sensori-neural, 23 conductive, and 19 mixed) were administered an indigenous version of W-22 PB words under: Condition I--Quiet--chamber noise below 28 dB with speech at 60 dB; and at a constant signal-to-noise (S/N) ratio of +10 dB with background white noise at 70 dB in Condition II and 80 dB in Condition III. The scores were a) 81 +/- 16%, b) 77 +/- 9%, and c) 79 +/- 13%. Mean scores decreased significantly (p less than 0.001) with noise in all groups while the score was more (p less than 0.001) at the higher noise level only in the sensori-neural group. The decrease in scores with advancing hearing impairment was less in noise than in quiet, probably due to binaural and satisfactory S/N ratio. The scores did not fall below 70% unless the handicap was marked. The need for suitable standards of binaural speech discrimination under noise in aircrew assessment is emphasized.

Hearing impairment in premature newborns—Analysis based on the national hearing screening database in Poland

PubMed Central

Greczka, Grazyna; Dabrowski, Piotr; Szyfter-Harris, Joanna; Mazela, Jan

2017-01-01

Objectives The incidence of sensorineural hearing loss is between 1 and 3 per 1000 in healthy neonates and 2–4 per 100 in high-risk infants. The national universal neonatal hearing screening carried out in Poland since 2002 enables selection of infants with suspicion and/or risk factors of hearing loss. In this study, we assessed the incidence and risk factors of hearing impairment in infants ≤33 weeks’ gestational age (wga). Methods We analyzed the database of the Polish Universal Newborns Hearing Screening Program from 2010 to 2013. The study group involved 11438 infants born before 33 wga, the control group—1487730 infants. Screening was performed by means of transient evoked otoacoustic emissions. The risk factors of hearing loss were recorded. Infants who failed the screening test and/or had risk factors were referred for further audiological evaluation. Results Hearing deficit was diagnosed in 11% of infants ≤25 wga, 5% at 26–27 wga, 3.46% at 28 wga and 2–3% at 29–32 wga. In the control group the incidence of hearing deficit was 0.2% (2.87% with risk factors). The most important risk factors were craniofacial malformations, very low birth weight, low Apgar score and mechanical ventilation. Hearing screening was positive in 22.42% newborns ≤28 wga and 10% at 29–32 wga and in the control group. Conclusions Hearing impairment is a severe consequence of prematurity. Its prevalence is inversely related to the maturity of the baby. Premature infants have many concomitant risk factors which influence the occurrence of hearing deficit. PMID:28910311

Systematic review of middle ear implants: do they improve hearing as much as conventional hearing AIDS?

PubMed

Tysome, James R; Moorthy, Ram; Lee, Ambrose; Jiang, Dan; O'Connor, Alec Fitzgerald

2010-12-01

A systematic review to determine whether middle ear implants (MEIs) improve hearing as much as hearing aids. Databases included MEDLINE, EMBASE, DARE, and Cochrane searched with no language restrictions from 1950 or the start date of each database. Initial search found 644 articles, of which 17 met the inclusion criteria of MEI in adults with a sensorineural hearing loss, where hearing outcomes and patient-reported outcome measures (PROMs) compared MEI with conventional hearing aids (CHAs). Study quality assessment included whether ethical approval was gained, the study was prospective, eligibility criteria specified, a power calculation made and appropriate controls, outcome measures, and analysis performed. Middle ear implant outcome analysis included residual hearing, complications, and comparison to CHA in terms of functional gain, speech perception in quiet and in noise, and validated PROM questionnaires. Because of heterogeneity of outcome measures, comparisons were made by structured review. The quality of studies was moderate to poor with short follow-up. The evidence supports the use of MEI because, overall, they do not decrease residual hearing, result in a functional gain in hearing comparable to CHA, and may improve perception of speech in noise and sound quality. We recommend the publication of long-term results comparing MEI with CHA, reporting a minimum of functional gain, speech perception in quiet and in noise, complications, and a validated PROM to guide the engineering of the new generation of MEI in the future.

Should patients with asymmetrical noise-induced hearing loss be screened for vestibular schwannomas?

PubMed

Baker, R; Stevens-King, A; Bhat, N; Leong, P

2003-08-01

The Peterborough ENT department receives many referrals for MoD personnel who have suffered hearing loss from occupational noise exposure. Those patients with asymmetrical sensorineural hearing loss are routinely screened for vestibular schwannomas by MRI scanning. Scan reports from the past 5 years have been reviewed and out of 152 scans, four revealed vestibular schwannomas giving a pick-up rate of 2.5%, which compares favourably with other published pick-up rates. Review of the audiograms in these cases suggests that they can be misleading in this context. The conclusion is that patients with noise-induced asymmetrical hearing loss should be screened for acoustic neuromas.

Evaluation of hearing and speech-language in preschool children: how important, why we should perform?

PubMed

Tokgöz-Yılmaz, Suna; Özcebe, Esra; Türkyılmaz, Meral Didem; Köse, Aysen; Sennaroğlu, Gonca; Orhon, Filiz; Ulukol, Betül

2013-01-01

The aim of the study was to present the hearing and speech-language findings of preschool children. The children in this study were aged 3-5 years. Sixtyseven of 239 children (28.0%) had been referred to a physician because of possible middle ear problems, and 25 of the 67 children had slight and mild conduction type hearing loss with air-bone gaps. One of 239 children had profound sensorineural hearing loss. Speech-language problems were found in 70 of 239 children (29.3%). Necessary attention should be paid to the evaluation of hearing and speech-language skills in preschool-aged children to avoid delayed detection and to give these children the opportunity for timely intervention for hearing and speech-language problems.

Active Duty-U.S. Army Noise Induced Hearing Injury Quarterly Surveillance Q3 2007 thru Q4 2009

DTIC Science & Technology

2014-05-11

years (CY) Q3 2007-Q4 2009 shows incident case rates for sensorineural hearing loss (SNHL), significant threshold shift (STS), tinnitus , and Noise-Induced...Prev Med. 2010;38(1S):S71-S77. Humes LE, Jollenbeck LM, Durch JS. Noise and military service: Implications for hearing loss and tinnitus . Washington...threshold shift 79415 NONSPECIFIC ABNORMAL AUDITORY FUNCTION STUDIES TINN Tinnitus 38830 TINNITUS UNSPECIFIED TINN Tinnitus 38831 SUBJECTIVE TINNITUS

Enhancing Auditory Selective Attention Using a Visually Guided Hearing Aid

PubMed Central

2017-01-01

Purpose Listeners with hearing loss, as well as many listeners with clinically normal hearing, often experience great difficulty segregating talkers in a multiple-talker sound field and selectively attending to the desired “target” talker while ignoring the speech from unwanted “masker” talkers and other sources of sound. This listening situation forms the classic “cocktail party problem” described by Cherry (1953) that has received a great deal of study over the past few decades. In this article, a new approach to improving sound source segregation and enhancing auditory selective attention is described. The conceptual design, current implementation, and results obtained to date are reviewed and discussed in this article. Method This approach, embodied in a prototype “visually guided hearing aid” (VGHA) currently used for research, employs acoustic beamforming steered by eye gaze as a means for improving the ability of listeners to segregate and attend to one sound source in the presence of competing sound sources. Results The results from several studies demonstrate that listeners with normal hearing are able to use an attention-based “spatial filter” operating primarily on binaural cues to selectively attend to one source among competing spatially distributed sources. Furthermore, listeners with sensorineural hearing loss generally are less able to use this spatial filter as effectively as are listeners with normal hearing especially in conditions high in “informational masking.” The VGHA enhances auditory spatial attention for speech-on-speech masking and improves signal-to-noise ratio for conditions high in “energetic masking.” Visual steering of the beamformer supports the coordinated actions of vision and audition in selective attention and facilitates following sound source transitions in complex listening situations. Conclusions Both listeners with normal hearing and with sensorineural hearing loss may benefit from the acoustic

Musical Experience, Sensorineural Auditory Processing, and Reading Subskills in Adults.

PubMed

Tichko, Parker; Skoe, Erika

2018-04-27

Developmental research suggests that sensorineural auditory processing, reading subskills (e.g., phonological awareness and rapid naming), and musical experience are related during early periods of reading development. Interestingly, recent work suggests that these relations may extend into adulthood, with indices of sensorineural auditory processing relating to global reading ability. However, it is largely unknown whether sensorineural auditory processing relates to specific reading subskills, such as phonological awareness and rapid naming, as well as musical experience in mature readers. To address this question, we recorded electrophysiological responses to a repeating click (auditory stimulus) in a sample of adult readers. We then investigated relations between electrophysiological responses to sound, reading subskills, and musical experience in this same set of adult readers. Analyses suggest that sensorineural auditory processing, reading subskills, and musical experience are related in adulthood, with faster neural conduction times and greater musical experience associated with stronger rapid-naming skills. These results are similar to the developmental findings that suggest reading subskills are related to sensorineural auditory processing and musical experience in children.

Musical Experience, Sensorineural Auditory Processing, and Reading Subskills in Adults

PubMed Central

Tichko, Parker; Skoe, Erika

2018-01-01

Developmental research suggests that sensorineural auditory processing, reading subskills (e.g., phonological awareness and rapid naming), and musical experience are related during early periods of reading development. Interestingly, recent work suggests that these relations may extend into adulthood, with indices of sensorineural auditory processing relating to global reading ability. However, it is largely unknown whether sensorineural auditory processing relates to specific reading subskills, such as phonological awareness and rapid naming, as well as musical experience in mature readers. To address this question, we recorded electrophysiological responses to a repeating click (auditory stimulus) in a sample of adult readers. We then investigated relations between electrophysiological responses to sound, reading subskills, and musical experience in this same set of adult readers. Analyses suggest that sensorineural auditory processing, reading subskills, and musical experience are related in adulthood, with faster neural conduction times and greater musical experience associated with stronger rapid-naming skills. These results are similar to the developmental findings that suggest reading subskills are related to sensorineural auditory processing and musical experience in children. PMID:29702572

Development of a digital hearing aid to meet the Brazilian Government's Ordinance 587 (APAC).

PubMed

Penteado, Silvio Pires; Bento, Ricardo Ferreira

2010-01-01

The treatment of sensorineural hearing loss is based on hearing aids, also known as individual sound amplification devices. The hearing aids purchased by the Brazilian Government, aiming at fulfilling public policies, are based on dedicated components, which bring about benefits, but also render them expensive and may impair repair services after manufacture's warranty expires. to design digital behind-the-ear hearing aids built from standardized components coming from the very supply chain of these manufacturers. experimental. to identify the supply chain of these manufacturers, request samples and set up hearing aids in the laboratory. The developed hearing aids did not show lesser electroacoustic characteristics when compared to those acquired by the Government, also being tested by the same reference international technical standard. It is possible to develop digital behind-the-ear hearing aids based on off-the-shelf components from hearing aid manufacturers' supply chain. Their advantages include low operational costs - for acquisition (with clear advantages for the Government) and service (advantage for the patient).

Examination of the neighborhood activation theory in normal and hearing-impaired listeners.

PubMed

Dirks, D D; Takayanagi, S; Moshfegh, A; Noffsinger, P D; Fausti, S A

2001-02-01

Experiments were conducted to examine the effects of lexical information on word recognition among normal hearing listeners and individuals with sensorineural hearing loss. The lexical factors of interest were incorporated in the Neighborhood Activation Model (NAM). Central to this model is the concept that words are recognized relationally in the context of other phonemically similar words. NAM suggests that words in the mental lexicon are organized into similarity neighborhoods and the listener is required to select the target word from competing lexical items. Two structural characteristics of similarity neighborhoods that influence word recognition have been identified; "neighborhood density" or the number of phonemically similar words (neighbors) for a particular target item and "neighborhood frequency" or the average frequency of occurrence of all the items within a neighborhood. A third lexical factor, "word frequency" or the frequency of occurrence of a target word in the language, is assumed to optimize the word recognition process by biasing the system toward choosing a high frequency over a low frequency word. Three experiments were performed. In the initial experiments, word recognition for consonant-vowel-consonant (CVC) monosyllables was assessed in young normal hearing listeners by systematically partitioning the items into the eight possible lexical conditions that could be created by two levels of the three lexical factors, word frequency (high and low), neighborhood density (high and low), and average neighborhood frequency (high and low). Neighborhood structure and word frequency were estimated computationally using a large, on-line lexicon-based Webster's Pocket Dictionary. From this program 400 highly familiar, monosyllables were selected and partitioned into eight orthogonal lexical groups (50 words/group). The 400 words were presented randomly to normal hearing listeners in speech-shaped noise (Experiment 1) and "in quiet" (Experiment 2) as

Characterization of Hearing Thresholds from 500 to 16,000 Hz in Dentists: A Comparative Study

PubMed Central

Gonçalves, Claudia Giglio de Oliveira; Santos, Luciana; Lobato, Diolen; Ribas, Angela; Lacerda, Adriana Bender Moreira; Marques, Jair

2014-01-01

Introduction High-level noise exposure in dentists' workplaces may cause damages to the auditory systems. High-frequency audiometry is an important tool in the investigation in the early diagnosis of hearing loss. Objectives To analyze the auditory thresholds at frequencies from 500 to 16,000 Hz of dentists in the city of Curitiba. Methods This historic cohort study retrospectively tested hearing thresholds from 500 to 16,000 Hz with a group of dentists from Curitiba, in the state of Paraná, Brazil. Eighty subjects participated in the study, separated into a dentist group and a control group, with the same age range and gender across groups but with no history of occupational exposure to high levels of sound pressure in the control group. Subjects were tested with conventional audiometry and high-frequency audiometry and answered a questionnaire about exposure to noise. Results Results showed that 81% of dentists did not receive any information regarding noise at university; 6 (15%) dentists had sensorineural hearing impairment; significant differences were observed between the groups only at frequencies of 500 Hz and 1,000, 6,000 and 8,000 Hz in the right ear. There was no significant difference between the groups after analysis of mean hearing thresholds of high frequencies with the average hearing thresholds in conventional frequencies; subjects who had been working as dentists for longer than 10 years had worse tonal hearing thresholds at high frequencies. Conclusions In this study, we observed that dentists are at risk for the development of sensorineural hearing loss especially after 10 years of service. PMID:25992172

Effects of hearing aids in the balance, quality of life and fear to fall in elderly people with sensorineural hearing loss

PubMed Central

Lacerda, Clara Fonseca; Silva, Luciana Oliveira e; de Tavares Canto, Roberto Sérgio; Cheik, Nadia Carla

2012-01-01

Summary Introduction: The aging process provokes structural modifications and functional to it greets, compromising the postural control and central processing. Studies have boarded the necessity to identify to the harmful factors of risk to aged the auditory health and security in stricken aged by auditory deficits and with alterations of balance. Objective: To evaluate the effect of auditory prosthesis in the quality of life, the balance and the fear of fall in aged with bilateral auditory loss. Method: Carried through clinical and experimental study with 56 aged ones with sensorineural auditory loss, submitted to the use of auditory prosthesis of individual sonorous amplification (AASI). The aged ones had answered to the questionnaires of quality of life Short Form Health Survey (SF-36), Falls Efficacy International Scale- (FES-I) and the test of Berg Balance Scale (BBS). After 4 months, the aged ones that they adapted to the use of the AASI had been reevaluated. Results: It had 50% of adaptation of the aged ones to the AASI. It was observed that the masculine sex had greater difficulty in adapting to the auditory device and that the variable age, degree of loss, presence of humming and vertigo had not intervened with the adaptation to auditory prosthesis. It had improvement of the quality of life in the dominance of the State General Health (EGS) and Functional Capacity (CF) and of the humming, as well as the increase of the auto-confidence after adaptation of auditory prosthesis. Conclusion: The use of auditory prosthesis provided the improvement of the domains of the quality of life, what it reflected consequently in one better auto-confidence and in the long run in the reduction of the fear of fall in aged with sensorineural auditory loss. PMID:25991930

Comparing Specific Language Impairment and Hearing Impairment: Different Profiles in German Verbal Agreement Morphology

ERIC Educational Resources Information Center

Penke, Martina; Rothweiler, Monika

2018-01-01

The study aims at identifying characteristic phenotypes for children with SLI and children with sensorineural hearing impairment (HI) in language and in domains associated with language. We focus on verbal agreement inflection and phonological short-term memory, phenomena that have been repeatedly found to be impaired in both groups of children. A…

Rehabilitation for disabling hearing loss: evaluating the need relative to provision of hearing aids in the public health care system.

PubMed

Hlayisi, Vera-Genevey; Ramma, Lebogang

2018-05-20

To determine the demand relative to supply of hearing rehabilitation through hearing aids for those with disabling hearing loss in a public health care setting in South Africa. Retrospective cross-sectional survey of medical records of all patients were seen at a public hospital (Polokwane Provincial Academic) during 2012-2014, was conducted. Audiological data from 3894 medical folders were accessed and reviewed; thereafter, results were analyzed using descriptive statistics. Of the 3894 medical folders reviewed, two-third (62%, n = 2402) were diagnosed with hearing loss, mostly bilaterally (81%). More than 30% of all patients diagnosed with hearing loss were ≤10 years old. Sensorineural (permanent) hearing loss was diagnosed most often (38%, n = 913) and 74% (n = 1778) of hearing losses diagnosed were of moderate or worse severity (i.e., disabling loss). Hearing aids were fitted to only 15% (n = 272) of those diagnosed with disabling hearing loss and most hearing aid fittings were to low-income adult patients (≥25 years old) with more severe-profound hearing losses. This study showed that the need for hearing aids to provide hearing rehabilitation far exceeds the supply. Therefore, a multi-pronged approach that includes increased budget allocation and exploring low-cost interventions for developing countries to meet the demand for hearing aids. Furthermore, study highlighted a high prevalence of hearing loss in those younger than 10 years of age, and thus highlights the need for early intervention as well as intensifying efforts to reduce preventable causes of hearing loss. Implications for Rehabilitation Audiologists need to advocate for an increase in budget allocation for hearing rehabilitation devices. Study indicates need to explore low-cost hearing devices/rehabilitation interventions for developing countries. Health professionals should consider preventative measures to reduce prevalence of preventable hearing loss.

How well can centenarians hear?

PubMed

Mao, Zhongping; Zhao, Lijun; Pu, Lichun; Wang, Mingxiao; Zhang, Qian; He, David Z Z

2013-01-01

With advancements in modern medicine and significant improvements in life conditions in the past four decades, the elderly population is rapidly expanding. There is a growing number of those aged 100 years and older. While many changes in the human body occur with physiological aging, as many as 35% to 50% of the population aged 65 to 75 years have presbycusis. Presbycusis is a progressive sensorineural hearing loss that occurs as people get older. There are many studies of the prevalence of age-related hearing loss in the United States, Europe, and Asia. However, no audiological assessment of the population aged 100 years and older has been done. Therefore, it is not clear how well centenarians can hear. We measured middle ear impedance, pure-tone behavioral thresholds, and distortion-product otoacoustic emission from 74 centenarians living in the city of Shaoxing, China, to evaluate their middle and inner ear functions. We show that most centenarian listeners had an "As" type tympanogram, suggesting reduced static compliance of the tympanic membrane. Hearing threshold tests using pure-tone audiometry show that all centenarian subjects had varying degrees of hearing loss. More than 90% suffered from moderate to severe (41 to 80 dB) hearing loss below 2,000 Hz, and profound (>81 dB) hearing loss at 4,000 and 8,000 Hz. Otoacoustic emission, which is generated by the active process of cochlear outer hair cells, was undetectable in the majority of listeners. Our study shows the extent and severity of hearing loss in the centenarian population and represents the first audiological assessment of their middle and inner ear functions.

Temporal modulation transfer functions for listeners with real and simulated hearing loss

PubMed Central

Desloge, Joseph G.; Reed, Charlotte M.; Braida, Louis D.; Perez, Zachary D.; Delhorne, Lorraine A.

2011-01-01

A functional simulation of hearing loss was evaluated in its ability to reproduce the temporal modulation transfer functions (TMTFs) for nine listeners with mild to profound sensorineural hearing loss. Each hearing loss was simulated in a group of three age-matched normal-hearing listeners through spectrally shaped masking noise or a combination of masking noise and multiband expansion. TMTFs were measured for both groups of listeners using a broadband noise carrier as a function of modulation rate in the range 2 to 1024 Hz. The TMTFs were fit with a lowpass filter function that provided estimates of overall modulation-depth sensitivity and modulation cutoff frequency. Although the simulations were capable of accurately reproducing the threshold elevations of the hearing-impaired listeners, they were not successful in reproducing the TMTFs. On average, the simulations resulted in lower sensitivity and higher cutoff frequency than were observed in the TMTFs of the hearing-impaired listeners. Discrepancies in performance between listeners with real and simulated hearing loss are possibly related to inaccuracies in the simulation of recruitment. PMID:21682411

Correlational Study of Diabetic Retinopathy and Hearing Loss.

PubMed

Ooley, Caroline; Jun, Weon; Le, Kim; Kim, Allen; Rock, Nathan; Cardenal, Molly; Kline, Rebecca; Aldrich, Drew; Hayes, John

2017-03-01

Our research goal was to complete a retrospective chart review to determine if there is a correlation between the level of diabetic retinopathy and diabetic neurosensory hearing loss. A retrospective analysis of 175 Department of Veterans Affairs Computerized Patient Record System charts was completed at the VA Portland Health Care System. Subjects were classified by degree of diabetic retinopathy as follows: no diabetic retinopathy (n = 80), mild nonproliferative diabetic retinopathy (n = 51), moderate nonproliferative diabetic retinopathy (n = 25), and combined severe nonproliferative diabetic retinopathy and proliferative diabetic retinopathy (PDR) (n = 17). Degree of sensorineural hearing was collected for each ear. Additionally, measures of diabetic control, including hemoglobin A1C, and creatinine, were recorded. After controlling for diabetic control, as measured by HbA1C and creatinine, level of diabetic retinopathy was significantly associated with hearing loss severity in both ears (right ear, P = .018 and left ear, P = .007). When adjusted to include diabetes control, the severity of diabetic retinopathy showed a correlation with degree of hearing loss at most levels. Because of this association, recommendation for hearing evaluations may be considered for those with mild, moderate, or severe nonproliferative or proliferative diabetic retinopathy.

Consumer Preferences for Hearing Aid Attributes

PubMed Central

Lataille, Angela T.; Buttorff, Christine; White, Sharon; Niparko, John K.

2012-01-01

Low utilization of hearing aids has drawn increased attention to the study of consumer preferences using both simple ratings (e.g., Likert scale) and conjoint analyses, but these two approaches often produce inconsistent results. The study aims to directly compare Likert scales and conjoint analysis in identifying important attributes associated with hearing aids among those with hearing loss. Seven attributes of hearing aids were identified through qualitative research: performance in quiet settings, comfort, feedback, frequency of battery replacement, purchase price, water and sweat resistance, and performance in noisy settings. The preferences of 75 outpatients with hearing loss were measured with both a 5-point Likert scale and with 8 paired-comparison conjoint tasks (the latter being analyzed using OLS [ordinary least squares] and logistic regression). Results were compared by examining implied willingness-to-pay and Pearson’s Rho. A total of 56 respondents (75%) provided complete responses. Two thirds of respondents were male, most had sensorineural hearing loss, and most were older than 50; 44% of respondents had never used a hearing aid. Both methods identified improved performance in noisy settings as the most valued attribute. Respondents were twice as likely to buy a hearing aid with better functionality in noisy environments (p < .001), and willingness to pay for this attribute ranged from US$2674 on the Likert to US$9000 in the conjoint analysis. The authors find a high level of concordance between the methods—a result that is in stark contrast with previous research. The authors conclude that their result stems from constraining the levels on the Likert scale. PMID:22514094

Utility of anti-HSP 70, TNF-alpha, ESR, antinuclear antibody, and antiphospholipid antibodies in the diagnosis and treatment of sudden sensorineural hearing loss.

PubMed

Süslü, Nilda; Yilmaz, Taner; Gürsel, Bülent

2009-02-01

To investigate the performance of various laboratory tests used for patients with sudden sensorineural hearing loss (SSNHL). Prospective clinical trial. Thirty patients who presented with SSNHL and 30 healthy people with no cochleovestibular disorders were selected as study and control groups. The laboratory panel includes the following tests: anti-HSP 70 antibody immunoassay, tumor necrosis factor-alpha (TNF-alpha), erythrocyte sedimentation rate (ESR), antinuclear antibody (ANA), and antiphospholipid antibodies. The study group was given corticosteroid therapy and separated into two groups: the corticosteroid responders and the corticosteroid nonresponders. In the follow-up, repeat audiograms were evaluated to determine the response to treatment. TNF-alpha was found at lower titers in the study group when compared with the control group in contrast to other studies. Also, anti-HSP 70 was not found in high titers in the study group. ANA and ESR were the two parameters that were significantly more positive in the study group compared with the control group. Because of the lack of association between a positive test and response to corticosteroid treatment, detection of the anti-HSP 70 antibody, TNF-alpha, ESR, and ANA, at present, do not offer clinically useful information in the treatment of SSNHL. Also, because of the lower titers of TNF-alpha documented in patients with SSNHL, we do not recommend the use of specific TNF-alpha inhibitors in SSNHL.

Hearing aids: indications, technology, adaptation, and quality control

PubMed Central

Hoppe, Ulrich; Hesse, Gerhard

2017-01-01

Hearing loss can be caused by a number of different pathological conditions. Some of them can be successfully treated, mainly by surgery, depending on the individual’s disease process. However, the treatment of chronic sensorineural hearing loss with damaged cochlear structures usually needs hearing rehabilitation by means of technical amplification. During the last two decades tremendous improvements in hearing aid technology led to a higher quality of the hearing rehabilitation process. For example, due to sophisticated signal processing acoustic feedback could be reduced and hence open fitting options are available even for more subjects with higher degrees of hearing loss. In particular for high-frequency hearing loss, the use of open fitting is an option. Both the users’ acceptance and the perceived sound quality were significantly increased by open fittings. However, we are still faced with a low level of readiness in many hearing impaired subjects to accept acoustic amplification. Since ENT specialists play a key-role in hearing aid provision, they should promote early hearing aid rehabilitation and include this in the counselling even in subjects with mild and moderate hearing loss. Recent investigations demonstrated the benefit of early hearing aid use in this group of patients since this may help to reduce subsequent damages as auditory deprivation, social isolation, development of dementia, and cognitive decline. For subjects with tinnitus, hearing aids may also support masking by environmental sounds and enhance cortical inhibition. The present paper describes the latest developments of hearing aid technology and the current state of the art for amplification modalities. Implications for both hearing aid indication and provision are discussed. PMID:29279726

[Digital vs. analog hearing aids for children. Is there a method for making an objective comparison possible?].

PubMed

Prinz, I; Nubel, K; Gross, M

2002-09-01

Until now, the assumed benefits of digital hearing aids are reflected only in subjective descriptions by patients with hearing aids, but cannot be documented adequately by routine diagnostic methods. Seventeen schoolchildren with moderate severe bilateral symmetrical sensorineural hearing loss were examined in a double-blinded crossover study. Differences in performance between a fully digital hearing aid (DigiFocus compact/Oticon) and an analogous digitally programmable two-channel hearing aid were evaluated. Of the 17 children, 13 choose the digital and 4 the analogous hearing aid. In contrast to the clear subjective preferences for the fully digital hearing aid, we could not obtain any significant results with routine diagnostic methods. Using the "virtual hearing aid," a subjective comparison and speech recognition performance task yielded significant differences. The virtual hearing aid proved to be suitable for a direct comparison of different hearing aids and can be used for double-blind testing in a pediatric population.

The novel c.247_249delTTC (p.F83del) GJB2 mutation in a family with prelingual sensorineural deafness.

PubMed

Petersen, Michael B; Grigoriadou, Maria; Koutroumpe, Maria; Kokotas, Haris

2012-07-01

Non-syndromic hearing loss is one of the most common hereditary determined diseases in human, and the disease is a genetically heterogeneous disorder. Mutations in the GJB2 gene, encoding connexin 26 (Cx26), are a major cause of non-syndromic recessive hearing impairment in many countries and are largely dependent on ethnic groups. Due to the high frequency of the c.35delG GJB2 mutation in the Greek population, we have previously suggested that Greek patients with sensorineural, non-syndromic deafness should be tested for the c.35delG mutation and the coding region of the GJB2 gene should be sequenced in c.35delG heterozygotes. Here we present on the clinical and molecular genetic evaluation of a family suffering from prelingual, sensorineural, non-syndromic deafness. A novel c.247_249delTTC (p.F83del) GJB2 mutation was detected in compound heterozygosity with the c.35delG GJB2 mutation in the proband and was later confirmed in the father, while the mother was homozygous for the c.35delG GJB2 mutation. We conclude that compound heterozygosity of the novel c.247_249delTTC (p.F83del) and the c.35delG mutations in the GJB2 gene was the cause of deafness in the proband and his father. Copyright © 2012 Elsevier Ireland Ltd. All rights reserved.

The time course of learning during a vowel discrimination task by hearing-impaired and masked normal-hearing listeners

NASA Astrophysics Data System (ADS)

Davis, Carrie; Kewley-Port, Diane; Coughlin, Maureen

2002-05-01

Vowel discrimination was compared between a group of young, well-trained listeners with mild-to-moderate sensorineural hearing impairment (YHI), and a matched group of normal hearing, noise-masked listeners (YNH). Unexpectedly, discrimination of F1 and F2 in the YHI listeners was equal to or better than that observed in YNH listeners in three conditions of similar audibility [Davis et al., J. Acoust. Soc. Am. 109, 2501 (2001)]. However, in the same time interval, the YHI subjects completed an average of 55% more blocks of testing than the YNH group. New analyses were undertaken to examine the time course of learning during the vowel discrimination task, to determine whether performance was affected by number of trials. Learning curves for a set of vowels in the F1 and F2 regions showed no significant differences between the YHI and YNH listeners. Thus while the YHI subjects completed more trials overall, they achieved a level of discrimination similar to that of their normal-hearing peers within the same number of blocks. Implications of discrimination performance in relation to hearing status and listening strategies will be discussed. [Work supported by NIHDCD-02229.

Identifying congenital hearing impairment. Personal experience based on selective hearing screening.

PubMed

Molini, E; Ricci, G; Baroni, S; Ciorba, A; Bellocci, A; Simoncelli, C

2004-06-01

If all degrees of permanent uni- or bilateral hypoacusis are taken into consideration, hearing impairment is the most common congenital disease. Early detection of permanent infantile hearing impairment has become extremely important in preventive medicine, since steps can be taken with hearing aids and rehabilitation to ensure better development of language and higher cognitive functions. Aim of this study is to provide a critical review of the time of diagnosis of hypoacusis at our audiology laboratory, where two methods were used to screen hearing of children with/without risk indicators. Results of approximately 10 years' work were re-examined during which time outpatient screening was conducted on children referred by colleagues in neonatology and paediatrics. All were carriers of congenital risk indicators associated with sensorineural and/or conductive hearing loss, based on the Joint Committee on Infant Hearing findings, or were suspected of being hypoacusic even if they had no known congenital risk factors. Hearing screening was conducted in hospital on newborns with no risk factors, within the first few days of birth. Results of the present study showed that when selective hearing screening was performed, the mean age of high-risk patients diagnosed with hypoacusis was slightly higher than that in international guidelines. Moreover, these patients represent approximately half the hypoacusic population identified in the study period. The other half of congenital hypoacusic subjects identified had no risk indicators and there was a significant delay in diagnosis due to later manifestation of symptoms indicating hypoacusis, and thus, in turn, delayed referral for hearing tests. In contrast, subjects without risk indicators who underwent in-hospital hearing screening and proved to be hypoacusic, were diagnosed early. In our experience, however, universal screening has considerable disadvantages, such as difficulty in covering the entire population, difficulty

Hearing thresholds in patients with drug-resistant tuberculosis: baseline audiogram configurations and associations

PubMed Central

Sogebi, Olusola Ayodele; Fadeyi, Muse Olatunbosun; Adefuye, Bolanle Olufunlola; Soyinka, Festus Olukayode

2017-01-01

ABSTRACT Objective: To use baseline audiogram parameters in order to ascertain whether drug-resistant tuberculosis (DR-TB) has effects on hearing, as well as to describe the configurations of the audiograms and to determine whether there are parameters that can be associated with those configurations. Methods: This was a prospective study involving patients diagnosed with DR-TB at a tuberculosis treatment center in the state of Ogun, in Nigeria. The patients included in the study were submitted to pure tone audiometry at baseline (within two weeks after treatment initiation). For comparative analyses, data regarding demographic and clinical characteristics were collected from the medical records of the patients. Results: The final sample comprised 132 patients. The mean age of the patients was 34.5 ± 12.6 years (range, 8-82 years), and the male:female ratio was 2:1. Of the 132 patients, 103 (78.0%) resided in neighboring states, 125 (94.7%) had previously experienced antituberculosis treatment failure, and 18 (13.6%) were retroviral-positive. Normal audiograms were found in 12 patients (9.1%), whereas sensorineural hearing loss was identified in 104 (78.8%), the two most common configurations being ascending, in 54 (40.9%), and sloping, in 26 (19.7%). Pure-tone averages at low frequencies (0.25-1.0 kHz) and high frequencies (2.0-8.0 kHz) were 33.0 dB and 40.0 dB, respectively. Regarding the degree of hearing loss in the better ear, 36 patients (27.3%) were classified as having normal hearing and 67 (50.8%) were classified as having mild hearing loss (26-40 dB), whereas 29 (21.9%) showed moderate or severe hearing loss. Among the variables studied (age, gender, retroviral status, previous treatment outcome, and weight at admission), only male gender was associated with audiometric configurations. Conclusions: In this sample of patients with DR-TB, most presented with bilateral, mild, suboptimal sensorineural hearing loss, and ascending/sloping audiometric

Three novel GJB2 (connexin 26) variants associated with autosomal dominant syndromic and nonsyndromic hearing loss.

PubMed

DeMille, Desiree; Carlston, Colleen M; Tam, Oliver H; Palumbos, Janice C; Stalker, Heather J; Mao, Rong; Zori, Roberto T; Viskochil, David H; Park, Albert H; Carey, John C

2018-04-01

Connexin 26 (Cx26), encoded by the GJB2 gene, is a key protein involved in the formation of gap junctions in epithelial organs including the inner ear and palmoplantar epidermis. Pathogenic variants in GJB2 are responsible for approximately 50% of inherited sensorineural deafness. The majority of these variants are associated with autosomal recessive inheritance; however, rare reports of dominantly co-segregating variants have been published. Since we began offering GJB2 testing in 2003, only about 2% of detected GJB2 variants from our laboratory have been classified as dominant. Here we report three novel dominant GJB2 variants (p.Thr55Ala, p.Gln57_Pro58delinsHisSer, and p.Trp44Gly); two associated with syndromic sensorineural hearing loss and one with nonsyndromic hearing loss. In the kindred with the p.Thr55Ala variant, the proband and his father present with only leukonychia as a cutaneous finding of their syndromic hearing loss. This phenotype has been previously documented in conjunction with palmoplantar hyperkeratosis, but isolated leukonychia is a novel finding likely associated with the unique threonine to alanine change at codon 55 (other variants at this codon have been reported in cases of nonsyndromic hearing loss). This report contributes to the short list of GJB2 variants associated with autosomal dominant hearing loss, highlights the variability of skin and nail findings associated with such cases, and illustrates the occurrence of both syndromic and nonsyndromic presentations with changes in the same gene. © 2018 Wiley Periodicals, Inc.

Two novel LHX3 mutations in patients with combined pituitary hormone deficiency including cervical rigidity and sensorineural hearing loss.

PubMed

Ramzan, Khushnooda; Bin-Abbas, Bassam; Al-Jomaa, Lolwa; Allam, Rabab; Al-Owain, Mohammed; Imtiaz, Faiqa

2017-03-16

Congenital combined pituitary hormone deficiency (CPHD) is a rare heterogeneous group of conditions. CPHD-type 3 (CPHD3; MIM# 221750) is caused by recessive mutations in LHX3, a LIM-homeodomain transcription factor gene. The isoforms of LHX3 are critical for pituitary gland formation and specification of the anterior pituitary hormone-secreting cell types. They also play distinct roles in the development of neuroendocrine and auditory systems. Here, we summarize the clinical, endocrinological, radiological and molecular features of three patients from two unrelated families. Clinical evaluation revealed severe CPHD coupled with cervical vertebral malformations (rigid neck, scoliosis), mild developmental delay and moderate sensorineural hearing loss (SNHL). The patients were diagnosed with CPHD3 based on the array of hormone deficiencies and other associated syndromic symptoms, suggestive of targeted LHX3 gene sequencing. A novel missense mutation c.437G > T (p. Cys146Phe) and a novel nonsense mutation c.466C > T (p. Arg156Ter), both in homozygous forms, were found. The altered Cys146 resides in the LIM2 domain of the encoded protein and is a phylogenetically conserved residue, which mediates LHX3 transcription factor binding with a zinc cation. The p. Arg156Ter is predicted to result in a severely truncated protein, lacking the DNA binding homeodomain. Considering genotype/phenotype correlation, we suggest that the presence of SNHL and limited neck rotation should be considered in the differential diagnosis of CPHD3 to facilitate molecular diagnosis. This report describes the first LHX3 mutations from Saudi patients and highlights the importance of combining molecular diagnosis with the clinical findings. In addition, it also expands the knowledge of LHX3-related CPHD3 phenotype and the allelic spectrum for this gene.

Prevalence, heritability and genetic correlations of congenital sensorineural deafness and pigmentation phenotypes in the Border Collie.

PubMed

De Risio, Luisa; Lewis, Tom; Freeman, Julia; de Stefani, Alberta; Matiasek, Lara; Blott, Sarah

2011-06-01

The objectives of this study were to estimate prevalence, heritability and genetic correlations of congenital sensorineural deafness (CSD) and pigmentation phenotypes in the Border Collie. Entire litters of Border Collies that presented to the Animal Health Trust (1994-2008) for assessment of hearing status by brain stem auditory evoked response (BAER) at 4-10 weeks of age were included. Heritability and genetic correlations were estimated using residual maximum likelihood (REML). Of 4143 puppies that met the inclusion criteria, 97.6% had normal hearing status, 2.0% were unilaterally deaf and 0.4% were bilaterally deaf. Heritability of deafness as a trichotomous trait (normal/unilaterally deaf/bilaterally deaf) was estimated at 0.42 using multivariate analysis. Genetic correlations of deafness with iris colour and merle coat colour were 0.58 and 0.26, respectively. These results indicate that there is a significant genetic effect on CSD in Border Collies and that some of the genes determining deafness also influence pigmentation phenotypes. Copyright © 2010 Elsevier Ltd. All rights reserved.

Vowel perception by noise masked normal-hearing young adults

NASA Astrophysics Data System (ADS)

Richie, Carolyn; Kewley-Port, Diane; Coughlin, Maureen

2005-08-01

This study examined vowel perception by young normal-hearing (YNH) adults, in various listening conditions designed to simulate mild-to-moderate sloping sensorineural hearing loss. YNH listeners were individually age- and gender-matched to young hearing-impaired (YHI) listeners tested in a previous study [Richie et al., J. Acoust. Soc. Am. 114, 2923-2933 (2003)]. YNH listeners were tested in three conditions designed to create equal audibility with the YHI listeners; a low signal level with and without a simulated hearing loss, and a high signal level with a simulated hearing loss. Listeners discriminated changes in synthetic vowel tokens /smcapi e ɛ invv æ/ when F1 or F2 varied in frequency. Comparison of YNH with YHI results failed to reveal significant differences between groups in terms of performance on vowel discrimination, in conditions of similar audibility by using both noise masking to elevate the hearing thresholds of the YNH and applying frequency-specific gain to the YHI listeners. Further, analysis of learning curves suggests that while the YHI listeners completed an average of 46% more test blocks than YNH listeners, the YHI achieved a level of discrimination similar to that of the YNH within the same number of blocks. Apparently, when age and gender are closely matched between young hearing-impaired and normal-hearing adults, performance on vowel tasks may be explained by audibility alone.

Frequency of auditory involvement and of associated factors in patients with juvenile idiopathic arthritis.

PubMed

Céspedes Cruz, Adriana Ivonne; Méndez Núñez, Myriam; Solís Vallejo, Eunice; Zeferino Cruz, Maritza; Torres Jiménez, Alfonso Ragnar; Ocampo Sánchez, Verónica; Flores Meza, Beatriz; Quintana Ruiz, Norma

2017-09-08

Juvenile idiopathic arthritis (JIA) is a chronic autoimmune disease characterized by the presence of arthritis in children under 16 years of age for more than 6 weeks in the absence of any other known cause. The extra-articular manifestations, especially in the audiovestibular system, are related to the involvement of the joints of the ossicular chain as a result of the inflammatory process in the synovium. Previous clinical studies in pediatric patients have shown conductive or sensorineural hearing loss. The aim of this study was to assess the frequency of hearing impairment and of associated factors in patients with JIA. A prospective, analytical study was conducted from January 2013 to August 2014 in 62 patients with JIA aged between 5 and 15 years. The study was approved by the local ethics committee and parents signed their informed consent. All subjects underwent audiological examination involving otomicroscopy, audiometry, tympanometry, stapedius reflex and test for transient otoacoustic emissions (TOAE); rheumatologic evaluation included joint examination and the application of a measure of functional ability (disability) using the Childhood Health Assessment Questionnaire (CHAQ). Measures of central tendency and of dispersion were used (chi-square for associations and P<.05 for statistical significance). Sixty-two patients were included: 56 girls and 6 boys, mean age 11.9 years and mean disease duration of 3.4 years; 46% had rheumatoid factor (RF)- positive polyarticular JIA, 40% had RF-negative polyarticular JIA, 15% had disease of systemic onset and 3% had oligoarthritis. Active disease was found in 29 patients and 33 were in remission with medication. Of the total of 124 ears evaluated according to the Jerger classification for tympanometry, abnormal findings were observed in 78 that were type As and in 1 that was type Ad, whereas there were 45 type A ears. Hearing loss was disclosed by speech audiometry, rather than by pure tone audiometry. The TOAE

The Neural Representation of Consonant-Vowel Transitions in Adults Who Wear Hearing Aids

PubMed Central

Tremblay, Kelly L.; Kalstein, Laura; Billings, Cuttis J.; Souza, Pamela E.

2006-01-01

Hearing aids help compensate for disorders of the ear by amplifying sound; however, their effectiveness also depends on the central auditory system's ability to represent and integrate spectral and temporal information delivered by the hearing aid. The authors report that the neural detection of time-varying acoustic cues contained in speech can be recorded in adult hearing aid users using the acoustic change complex (ACC). Seven adults (50–76 years) with mild to severe sensorineural hearing participated in the study. When presented with 2 identifiable consonant-vowel (CV) syllables (“shee” and “see”), the neural detection of CV transitions (as indicated by the presence of a P1-N1-P2 response) was different for each speech sound. More specifically, the latency of the evoked neural response coincided in time with the onset of the vowel, similar to the latency patterns the authors previously reported in normal-hearing listeners. PMID:16959736

Sound localization in noise in hearing-impaired listeners.

PubMed

Lorenzi, C; Gatehouse, S; Lever, C

1999-06-01

The present study assesses the ability of four listeners with high-frequency, bilateral symmetrical sensorineural hearing loss to localize and detect a broadband click train in the frontal-horizontal plane, in quiet and in the presence of a white noise. The speaker array and stimuli are identical to those described by Lorenzi et al. (in press). The results show that: (1) localization performance is only slightly poorer in hearing-impaired listeners than in normal-hearing listeners when noise is at 0 deg azimuth, (2) localization performance begins to decrease at higher signal-to-noise ratios for hearing-impaired listeners than for normal-hearing listeners when noise is at +/- 90 deg azimuth, and (3) the performance of hearing-impaired listeners is less consistent when noise is at +/- 90 deg azimuth than at 0 deg azimuth. The effects of a high-frequency hearing loss were also studied by measuring the ability of normal-hearing listeners to localize the low-pass filtered version of the clicks. The data reproduce the effects of noise on three out of the four hearing-impaired listeners when noise is at 0 deg azimuth. They reproduce the effects of noise on only two out of the four hearing-impaired listeners when noise is at +/- 90 deg azimuth. The additional effects of a low-frequency hearing loss were investigated by attenuating the low-pass filtered clicks and the noise by 20 dB. The results show that attenuation does not strongly affect localization accuracy for normal-hearing listeners. Measurements of the clicks' detectability indicate that the hearing-impaired listeners who show the poorest localization accuracy also show the poorest ability to detect the clicks. The inaudibility of high frequencies, "distortions," and reduced detectability of the signal are assumed to have caused the poorer-than-normal localization accuracy for hearing-impaired listeners.

NRF2 Is a Key Target for Prevention of Noise-Induced Hearing Loss by Reducing Oxidative Damage of Cochlea

PubMed Central

Honkura, Yohei; Matsuo, Hirotaka; Murakami, Shohei; Sakiyama, Masayuki; Mizutari, Kunio; Shiotani, Akihiro; Yamamoto, Masayuki; Morita, Ichiro; Shinomiya, Nariyoshi; Kawase, Tetsuaki; Katori, Yukio; Motohashi, Hozumi

2016-01-01

Noise-induced hearing loss (NIHL) is one of the most common sensorineural hearing deficits. Recent studies have demonstrated that the pathogenesis of NIHL is closely related to ischemia-reperfusion injury of cochlea, which is caused by blood flow decrease and free radical production due to excessive noise. This suggests that protecting the cochlea from oxidative stress is an effective therapeutic approach for NIHL. NRF2 is a transcriptional activator playing an essential role in the defense mechanism against oxidative stress. To clarify the contribution of NRF2 to cochlear protection, we examined Nrf2–/– mice for susceptibility to NIHL. Threshold shifts of the auditory brainstem response at 7 days post-exposure were significantly larger in Nrf2–/– mice than wild-type mice. Treatment with CDDO-Im, a potent NRF2-activating drug, before but not after the noise exposure preserved the integrity of hair cells and improved post-exposure hearing levels in wild-type mice, but not in Nrf2–/– mice. Therefore, NRF2 activation is effective for NIHL prevention. Consistently, a human NRF2 SNP was significantly associated with impaired sensorineural hearing levels in a cohort subjected to occupational noise exposure. Thus, high NRF2 activity is advantageous for cochlear protection from noise-induced injury, and NRF2 is a promising target for NIHL prevention. PMID:26776972

NRF2 Is a Key Target for Prevention of Noise-Induced Hearing Loss by Reducing Oxidative Damage of Cochlea.

PubMed

Honkura, Yohei; Matsuo, Hirotaka; Murakami, Shohei; Sakiyama, Masayuki; Mizutari, Kunio; Shiotani, Akihiro; Yamamoto, Masayuki; Morita, Ichiro; Shinomiya, Nariyoshi; Kawase, Tetsuaki; Katori, Yukio; Motohashi, Hozumi

2016-01-18

Noise-induced hearing loss (NIHL) is one of the most common sensorineural hearing deficits. Recent studies have demonstrated that the pathogenesis of NIHL is closely related to ischemia-reperfusion injury of cochlea, which is caused by blood flow decrease and free radical production due to excessive noise. This suggests that protecting the cochlea from oxidative stress is an effective therapeutic approach for NIHL. NRF2 is a transcriptional activator playing an essential role in the defense mechanism against oxidative stress. To clarify the contribution of NRF2 to cochlear protection, we examined Nrf2(-/-) mice for susceptibility to NIHL. Threshold shifts of the auditory brainstem response at 7 days post-exposure were significantly larger in Nrf2(-/-) mice than wild-type mice. Treatment with CDDO-Im, a potent NRF2-activating drug, before but not after the noise exposure preserved the integrity of hair cells and improved post-exposure hearing levels in wild-type mice, but not in Nrf2(-/-) mice. Therefore, NRF2 activation is effective for NIHL prevention. Consistently, a human NRF2 SNP was significantly associated with impaired sensorineural hearing levels in a cohort subjected to occupational noise exposure. Thus, high NRF2 activity is advantageous for cochlear protection from noise-induced injury, and NRF2 is a promising target for NIHL prevention.

A comparison of CIC and BTE hearing aids for three-dimensional localization of speech.

PubMed

Best, Virginia; Kalluri, Sridhar; McLachlan, Sara; Valentine, Susie; Edwards, Brent; Carlile, Simon

2010-10-01

Three-dimensional sound localization of speech in anechoic space was examined for eleven listeners with sensorineural hearing loss. The listeners were fitted bilaterally with CIC and BTE hearing aids having similar bandwidth capabilities. The goal was to determine whether differences in microphone placement for these two styles (CICs at the ear canal entrance; BTEs above the pinna) would influence the availability of pinna-related spectral cues and hence localization performance. While lateral and polar angle localization was unaffected by the hearing aid style, the rate of front-back reversals was lower with CICs. This pattern persisted after listeners accommodated to each set of aids for a six week period, although the overall rate of reversals declined. Performance on all measures in all conditions was considerably poorer than in a control group of listeners with normal hearing.

Auditory brainstem response screening for hearing loss in high risk neonates.

PubMed

Watson, D R; McClelland, R J; Adams, D A

1996-07-01

The present paper reports the findings of a 7 year study evaluating the use of the auditory brainstem response (ABR) as the basis of a hearing screening procedure in a group of newborns at increased risk of hearing impairment. A Special Care Baby Unit (SCBU) population of 417 infants with diverse clinical backgrounds and treatment histories was tested for hearing impairment at birth using ABR audiometry. Some 332 passed the original screen at 30 dBnHL test level in both ears. Of the failure group, 18 did not survive and 32 had some degree of hearing impairment confirmed, nine of which were sensorineural in origin. An increased incidence of persistent middle ear disease was also noted in the failure group. A detailed operational analysis demonstrates that provided appropriate pass/fail criteria are adopted, the ABR technique offers excellent sensitivity and specificity for the detection of significant hearing loss in the test population. Furthermore, the study establishes that implementation of an ABR-based screening programme could reduce the average age at detection of permanent hearing loss by 7 months. A cost assessment shows that the introduction of such a targetted screening procedure could be done at a reasonable outlay.

The benefits of hearing aids and closed captioning for television viewing by older adults with hearing loss

PubMed Central

Gordon-Salant, Sandra; Callahan, Julia S.

2010-01-01

Objectives Although watching television is a common leisure activity of older adults, the ability to understand televised speech may be compromised by age-related hearing loss. Two potential assistive devices for improving television viewing are hearing aids and closed captioning, but their use and benefit by older adults with hearing loss are unknown. The primary purpose of this initial investigation was to determine if older hearing-impaired adults show improvements in understanding televised speech with the use of these two assistive devices (hearing aids and closed captioning) compared to conditions without these devices. A secondary purpose was to examine the frequency of hearing aid use and closed captioning use among a sample of older hearing aid wearers. Design The investigation entailed a randomized, repeated-measures design of 15 older adults (59–82 years) with bilateral sensorineural hearing losses who wore hearing aids. Participants viewed three types of televised programs (news, drama, game show) that were each edited into lists of speech segments, and provided an identification response. Each participant was tested in four conditions: baseline (no hearing aids or closed captioning), hearing aids only, closed captioning only, and hearing aids + closed captioning. Pilot testing with young normal-hearing listeners was conducted also to establish list equivalence and stimulus intelligibility with a control group. All testing was conducted in a quiet room to simulate a living room, using a 19-in flat screen television. Questionnaires were also administered to participants to determine frequency of hearing aid use and closed captioning use while watching television. Results A significant effect of viewing condition was observed for all programs. Participants exhibited significantly better speech recognition scores in conditions with closed captioning than those without closed captioning (p<.01). Use of personal hearing aids did not significantly improve

The effect of symmetrical and asymmetrical hearing impairment on music quality perception.

PubMed

Cai, Yuexin; Zhao, Fei; Chen, Yuebo; Liang, Maojin; Chen, Ling; Yang, Haidi; Xiong, Hao; Zhang, Xueyuan; Zheng, Yiqing

2016-09-01

The purpose of this study was to investigate the effect of symmetrical, asymmetrical and unilateral hearing impairment on music quality perception. Six validated music pieces in the categories of classical music, folk music and pop music were used to assess music quality in terms of its 'pleasantness', 'naturalness', 'fullness', 'roughness' and 'sharpness'. 58 participants with sensorineural hearing loss [20 with unilateral hearing loss (UHL), 20 with bilateral symmetrical hearing loss (BSHL) and 18 with bilateral asymmetrical hearing loss (BAHL)] and 29 normal hearing (NH) subjects participated in the present study. Hearing impaired (HI) participants had greater difficulty in overall music quality perception than NH participants. Participants with BSHL rated music pleasantness and naturalness to be higher than participants with BAHL. Moreover, the hearing thresholds of the better ears from BSHL and BAHL participants as well as the hearing thresholds of the worse ears from BSHL participants were negatively correlated to the pleasantness and naturalness perception. HI participants rated the familiar music pieces higher than unfamiliar music pieces in the three music categories. Music quality perception in participants with hearing impairment appeared to be affected by symmetry of hearing loss, degree of hearing loss and music familiarity when they were assessed using the music quality rating test (MQRT). This indicates that binaural symmetrical hearing is important to achieve a high level of music quality perception in HI listeners. This emphasizes the importance of provision of bilateral hearing assistive devices for people with asymmetrical hearing impairment.

Pilot study of cognition in children with unilateral hearing loss.

PubMed

Ead, Banan; Hale, Sandra; DeAlwis, Duneesha; Lieu, Judith E C

2013-11-01

The objective of this study was to obtain preliminary data on the cognitive function of children with unilateral hearing loss in order to identify, quantify, and interpret differences in cognitive and language functions between children with unilateral hearing loss and with normal hearing. Fourteen children ages 9-14 years old (7 with severe-to-profound sensorineural unilateral hearing loss and 7 sibling controls with normal hearing) were administered five tests that assessed cognitive functions of working memory, processing speed, attention, and phonological processing. Mean composite scores for phonological processing were significantly lower for the group with unilateral hearing loss than for controls on one composite and four subtests. The unilateral hearing loss group trended toward worse performance on one additional composite and on two additional phonological processing subtests. The unilateral hearing loss group also performed worse than the control group on the complex letter span task. Analysis examining performance on the two levels of task difficulty revealed a significant main effect of task difficulty and an interaction between task difficulty and group. Cognitive function and phonological processing test results suggest two related deficits associated with unilateral hearing loss: (1) reduced accuracy and efficiency associated with phonological processing, and (2) impaired executive control function when engaged in maintaining verbal information in the face of processing incoming, irrelevant verbal information. These results provide a possible explanation for the educational difficulties experienced by children with unilateral hearing loss. Copyright © 2013 Elsevier Ireland Ltd. All rights reserved.

Fitting of hearing aids with different technical parameters to a patient with dead regions

NASA Astrophysics Data System (ADS)

Hojan-Jezierska, Dorota; Skrodzka, Ewa

2009-01-01

The purpose of the study was to determine an optimal hearing aid fitting procedure for a patient with well diagnosed high-frequency ‘dead regions’ in both cochleas. The patient reported non-symmetrical hearing problems of sensorineural origin. For binaural amplification two similar independent hearing aids were used as well as a pair of dependent devices with an ear-to-ear function. Two fitting methods were used: DSLi/o and NAL-NL1, and four different strategies of fitting were tested: the initial fitting based on the DSLi/o or NAL-NL1 method with necessary loudness corrections, the second fitting taking into account all the available functions of hearing instruments, the third fitting (based on the second one) but with significantly reduced amplification well above one octave of frequency inside dead region, and the final fitting with significantly reduced gain slightly below one octave inside dead regions. The results of hearing aids fitting were assessed using an APHAB procedure.

[Hearing and balance in metabolic bone diseases].

PubMed

Zatoński, Tomasz; Temporale, Hanna; Krecicki, Tomasz

2012-03-01

There are reports that hearing loss is one of the clinical manifestations of metabolic bone diseases. Demineralization can lead to a reduction in ossicular mass. Paget's disease can reveal loss of mineral density of the cochlear bone. Ear bone remodeling in osteoporosis is similar to the changes in otosclerosis. Moreover, osteoporosis, osteogenesis imperfecta and otosclerosis have a similar genetic mechanism. According to some researchers osteopenia and osteoporosis may well be associated with idiopathic benign positional vertigo (BPV). Dysfunction of the organ of hearing and balance in patients with renal insufficiency may be due to disturbances in calcium phosphate balance and renal osteodystrophy in the course of the disease. Proving the presence of hearing loss in patients with metabolic bone diseases may lead to determining the new indications for bone densitometry in some patients with hearing impairment. Furthermore, audiological examination in patients with osteoporosis may be important because of the impact of hearing loss on prognosis for patients with metabolic bone diseases.

Electromagnetic interference of bone-anchored hearing aids by cellular phones.

PubMed

Kompis, M; Negri, S; Häusler, R

2000-10-01

We report a case of electromagnetic interference between a bone-anchored hearing aid (BAHA) and a cellular phone. A 54-year-old women was successfully treated for severe mixed conductive and sensorineural hearing loss with a BAHA. Five years after implantation, the patient experienced a sudden feeling of dizziness, accompanied by a loud buzzing sound and by a sensation of head pressure while examining a digital mobile phone. During a subsequent experiment, the buzzing sound could be reproduced and was identified as electromagnetic interference between the BAHA and digital cellular phones. Seventeen adult BAHA users from our clinic participated in a subsequent survey. Of the 13 patients with some experience of digital cellular phones, 11 reported hearing annoying noises elicited by these devices. However, no other sensation, such as dizziness, was described. Owing to the increasing number of users of both hearing aids and cellular phones, the incidence of electromagnetic interference must be expected to increase as well. Although to date there is no evidence that such interference may be harmful or dangerous to users of conventional or bone-anchored hearing aids, unexpected interference can be a frightening experience.

[Severe sensorineural impairment in very premature infants: epidemiological aspects].

PubMed

Ancel, P-Y

2004-10-01

Advances in perinatal care have resulted in a sharply increasing survival rate among very preterm infants. However, there is some concern about the later neurodevelopmental outcome of those infants who survive. In this paper, we review the prevalence estimates of motor (cerebral palsy), sensorineural and cognitive impairments and their recent time-trends in very preterm infants. A review of studies describing neurodevelopmental outcome of very preterm infants in Europe, Australia and America North. The gestational age-specific prevalences of cerebral palsy (CP) were 72-86 for extremely preterm children (<28 weeks), 32-60 for very preterm (28-31 weeks) and 5-6 for moderate preterm (32-36 weeks), and 1.3-1.5 for term children per 1000. The live birth prevalence for CP remained unchanged in extremely and very preterm infants since 1990. The prevalence estimates of moderate and severe cognitive impairments are 15 to 25% in very preterm children. Less than 4% of very preterm infants develop severe hearing or visual loss. This review indicates that very preterm infants have high risk of disability. Most studies have been conducted between 1985 and 1995. Thus, these results should be interpreted with caution before generalisation to recent cohorts.

The Envoy® Totally Implantable Hearing System, St. Croix Medical

PubMed Central

Kroll, Kai; Grant, Iain L.; Javel, Eric

2002-01-01

The Totally Implantable Envoy® System is currently undergoing clinical trials in both the United States and Europe. The fully implantable hearing device is intended for use in patients with sensorineural hearing loss. The device employs piezoelectric transducers to sense ossicle motion and drive the stapes. Programmable signal processing parameters include amplification, compression, and variable frequency response. The fully implantable attribute allows users to take advantage of normal external ear resonances and head-related transfer functions, while avoiding undesirable earmold effects. The high sensitivity, low power consumption, and high fidelity attributes of piezoelectric transducers minimize acoustic feedback and maximize battery life (Gyo, 1996; Yanagihara, (1987) and 2001). The surgical procedure to install the device has been accurately defined and implantation is reversible. PMID:25425915

Hearing loss in children with growth hormone deficiency.

PubMed

Muus, John S; Weir, Forest W; Kreicher, Kathryn L; Bowlby, Deborah A; Discolo, Christopher M; Meyer, Ted A

2017-09-01

Although insulin-like growth factor 1 (IGF-1) has been shown to be important for inner-ear development in animal models, little is known about the otologic and audiologic findings of children with growth hormone deficiency (GHD). The goal of this study is to evaluate the prevalence, type, and severity of hearing impairment in children with GHD. Audiologic, otologic, and demographic data were recorded for children with a diagnosis of GHD in the AudGen database. Data for each patient were selected based on the first encounter with available complete audiometric data or the first encounter with a type of hearing loss documented. The patients were then stratified by type and severity of hearing loss, and otologic issues were documented. A separate cohort comprised of children with GHD without hearing loss was compared as a control. 209 children with GHD met inclusion criteria. 173 (83%) of these patients had hearing loss. 79% of losses were bilateral and 21% were unilateral (309 total ears with hearing loss). 293 of the 309 ears with hearing loss had audiograms with ear-specific thresholds; 47 had conductive, 24 had sensorineural, 65 had mixed and 157 had undefined hearing loss with incomplete audiograms. Pure-tone averages (PTA) were higher among patients with mixed hearing loss compared to patients with all other loss types. Hearing loss is prevalent in children with GHD with a predisposition to be bilateral. These findings suggest the need for increased awareness and routine hearing screening for patients with GHD. Further studies may elucidate the etiology of the hearing impairment in children with GHD to better aid pediatricians, endocrinologists, otolaryngologists and audiologists when assessing and managing these children. Copyright © 2017 Elsevier B.V. All rights reserved.

Open-Fit Domes and Children with Bilateral High-Frequency Sensorineural Hearing Loss: Benefits and Outcomes.

PubMed

Johnstone, Patti M; Yeager, Kelly R; Pomeroy, Marnie L; Hawk, Nicole

2018-04-01

Open-fit domes (OFDs) coupled with behind-the-ear (BTE) hearing aids were designed for adult listeners with moderate-to-severe bilateral high-frequency hearing loss (BHFL) with little to no concurrent loss in the lower frequencies. Adult research shows that BHFL degrades sound localization accuracy (SLA) and that BTE hearing aids with conventional earmolds (CEs) make matters worse. In contrast, research has shown that OFDs enhance spatial hearing percepts in adults with BHFL. Although the benefits of OFDs have been studied in adults with BHFL, no published studies to date have investigated the use of OFDs in children with the same hearing loss configuration. This study seeks to use SLA measurements to assess efficacy of bilateral OFDs in children with BHFL. To measure SLA in children with BHFL to determine the extent to which hearing loss, age, duration of CE use, and OFDs affect localization accuracy. A within-participant experimental design using repeated measures was used to determine the effect of OFDs on localization accuracy in children with BHFL. A between-participant experimental design was used to compare localization accuracy between children with BHFL and age-matched controls with normal hearing (NH). Eighteen children with BHFL who used CE and 18 age-matched NH controls. Children in both groups were divided into two age groups: older children (10-16 yr) and younger children (6-9 yr). All testing was done in a sound-treated booth with a horizontal array of 15 loudspeakers (radius of 1 m). The stimulus was a spondee word, "baseball": the level averaged 60 dB SPL and randomly roved (±8 dB). Each child was asked to identify the location of a sound source. Localization error was calculated across the loudspeaker array for each listening condition. A significant interaction was found between immediate benefit from OFD and duration of CE usage. Longer CE usage was associated with degraded localization accuracy using OFDs. Regardless of chronological age

Auditory, speech and language development in young children with cochlear implants compared with children with normal hearing.

PubMed

Schramm, Bianka; Bohnert, Andrea; Keilmann, Annerose

2010-07-01

This study had two aims: (1) to document the auditory and lexical development of children who are deaf and received the first cochlear implant (CI) by the age of 16 months and the second CI by the age of 31 months and (2) to compare these children's results with those of children with normal hearing (NH). This longitudinal study included five children with NH and five with sensorineural deafness. All children of the second group were observed for 36 months after the first fitting of the device (cochlear implant). The auditory development of the CI group was documented every 3 months up to the age of two years in hearing age and chronological age and for the NH group in chronological age. The language development of each NH child was assessed at 12, 18, 24 and 36 months of chronological age. Children with CIs were examined at the same age intervals at chronological and hearing age. In both groups, children showed individual patterns of auditory and language development. The children with CIs developed differently in the amount of receptive and expressive vocabulary compared with the NH control group. Three children in the CI group needed almost 6 months to make gains in speech development that were consistent with what would be expected for their chronological age. Overall, the receptive and expressive development in all children of the implanted group increased with their hearing age. These results indicate that early identification and early implantation is advisable to give children with sensorineural hearing loss a realistic chance to develop satisfactory expressive and receptive vocabulary and also to develop stable phonological, morphological and syntactical skills for school life. On the basis of these longitudinal data, we will be able to develop new diagnostic tools that enable clinicians to assess child's progress in hearing and speech development. Copyright 2010 Elsevier Ireland Ltd. All rights reserved.

[Audiophonological evaluation of 16 children fitted with cochlear implants for sensorineural hearing loss induced by bacterial meningitis].

PubMed

Teissier, N; Doehring, I; Noel-Petroff, N; Elmaleh-Bergès, M; Viala, P; François, M; Faye, A; Van Den Abbeele, T; Lorrot, M

2013-06-01

Bacterial meningitis (BM) is the primary etiology of acquired sensorineural hearing loss (SNHL) in children and may compromise language development. Since the 1990 s, cochlear implants (CIs) have become part of the management of children with profound SNHL with encouraging results. The aim of this study was to analyze the audiophonological performance of children before and after cochlear implantation for SNHL following bacterial meningitis. Retrospective study of all children fitted with CIs for bilateral severe to profound SNHL after bacterial meningitis in the Robert-Debré pediatric ENT department between August 1990 and March 2009. Audiophonological performance was assessed using the APCEI profile. Of the 283 children receiving implants during that period, 16 children (6%; 6 boys, 10 girls) underwent CI implantation after bacterial meningitis (Streptococcus pneumoniae in 8 cases, Neisseria meningitidis in 2 cases, and Haemophilus influenzae in 4 cases). The mean time from meningitis to SNHL was 8.3 months (median, 1.5 months; range, 1 day to 13 years). The mean time from meningitis to cochlear implantation was 2 years and 3 months (median, 7 months; range, 1 month to 13 years 3 months). Twelve children (75%) presented partial cochlear and/or vestibular ossification on presurgical CT scan. Three children received bilateral implants. Thirteen children (81%) developed early SNHL in the first 3 months, whereas 3 children developed SNHL more than 10 months after meningitis. As for the benefits of cochlear implantation, 11 children presented near to normal intelligibility and optimal use of their cochlear implant; 5 children presented partial benefits due to neurological sequelae (1), a long delay before implantation (1), technical problems (2), or a social problem in relation to low socioeconomic status (1). After bacterial meningitis, audiological evaluation must be made carefully during the first 3 months to detect early SNHL, but SNHL may also develop several

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

PubMed

Morita, Shinya; Fujiwara, Keishi; Fukuda, Atsushi; Fukuda, Satoshi; Nishio, Shin-Ya; Kitoh, Ryosuke; Hato, Naohito; Ikezono, Tetsuo; Ishikawa, Kotaro; Kaga, Kimitaka; Matsubara, Atsushi; Matsunaga, Tatsuo; Murata, Takaaki; Naito, Yasushi; Nishizaki, Kazunori; Ogawa, Kaoru; Sano, Hajime; Sato, Hiroaki; Sone, Michihiko; Suzuki, Mikio; Takahashi, Haruo; Tono, Tetsuya; Yamashita, Hiroshi; Yamasoba, Tatsuya; Usami, Shin-Ichi

2017-01-01

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. The objective of this study is to investigate the clinical characteristics of hearing loss due to mumps and to evaluate hearing outcomes. 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. 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.

Newborn hearing screening in Queensland 2009-2011: Comparison of hearing screening and diagnostic audiological assessment between term and preterm infants.

PubMed

Calcutt, Trent L; Dornan, Dimity; Beswick, Rachael; Tudehope, David I

2016-08-13

This study compares rates and timing of newborn hearing screening outcomes, audiological assessment and hearing loss diagnosis between infants of different gestational age groups. Early identification and management of sensorineural hearing loss (SNHL), ideally by 3-6 months of age, facilitates speech and language optimisation. Literature stratifying hearing screening and diagnostic audiology assessment by gestational age groups is lacking. Subjects were infants with recorded gestational ages receiving newborn hearing screening in Queensland between 2009 and 2011. Data were provided through the Queensland Healthy Hearing database. Infants were analysed in <34 weeks, 34-36 +6 weeks, 37-38 +6 weeks and ≥39 weeks gestational age groups. Infants (175 911) were eligible for analysis, 7.9% being preterm. Per 1000 infants analysed, bilateral SNHL of >40 dB occurred in 2.4 for <34, 1.4 for 34-36 +6 , 0.7 for 37-38 +6 and 0.7 for ≥39 weeks gestation. Diagnoses attributable to newborn hearing screening direct referral were 93.1% for bilateral >40 dB SNHL and 88.2% for other hearing loss. Relative to term, preterm infants had a higher incidence of direct and targeted surveillance referrals, audiology assessment and hearing loss diagnosis. Preterm infants were screened later after birth. Specific hearing screening and diagnosis characteristics differed between preterm infants <34 and 34-36 +6 weeks gestation, and term infants. Consideration of unique gestational age strata characteristics supports care individualisation. Preterm infants represent a diagnostic challenge, with higher rates of bilateral >40 dB SNHL than term but correspondingly higher false positive results on screening, justifying vigilant monitoring. Focused research into specific risk factors in preterm infants is warranted. © 2016 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).

Hearing outcomes of the active bone conduction system Bonebridge® in conductive or mixed hearing loss.

PubMed

Carnevale, Claudio; Til-Pérez, Guillermo; Arancibia-Tagle, Diego J; Tomás-Barberán, Manuel D; Sarría-Echegaray, Pedro L

2018-05-18

The active transcutaneous bone conduction implant Bonebridge ® , is indicated for patients affected by bilateral conductive/mixed hearing loss or unilateral sensorineural hearing loss, showing hearing outcomes similar to other percutaneous bone conduction implants, but with a lower rate of complications. The aim of this study was to analyze the hearing outcomes in a series of 26 patients affected by conductive or mixed hearing loss and treated with Bonebridge ® . 26 of 30 patients implanted with Bonebridge ® between October 2012 and May 2017, were included in the study. We compared the air conduction thresholds at the frequencies 500, 1000, 2000, 3000, 4000Hz, the SRT50% and the percentage of correct answers at an intensity of 50dB with and without the implant. "Pure tone average" with the implant was 34.91dB showing an average gain of 33.46dB. Average SRT 50% with the implant was 34.33dB, whereas before the surgery no patient achieved 50% of correct answers at a sound intensity of 50dB. The percentage of correct answers at 50dB changed from 11% without the implant to 85% with it. We only observed one complication consisting of an extrusion of the implant in a patient with a history of 2 previous rhytidectomies. The hearing outcomes obtained in our study are similar to those published in the literature. Bonebridge ® represents an excellent alternative in the treatment of conductive or mixed hearing loss, and with a lower rate of complications. Copyright © 2018 Sociedad Española de Otorrinolaringología y Cirugía de Cabeza y Cuello. Publicado por Elsevier España, S.L.U. All rights reserved.

Effect of a Bluetooth-implemented hearing aid on speech recognition performance: subjective and objective measurement.

PubMed

Kim, Min-Beom; Chung, Won-Ho; Choi, Jeesun; Hong, Sung Hwa; Cho, Yang-Sun; Park, Gyuseok; Lee, Sangmin

2014-06-01

The object was to evaluate speech perception improvement through Bluetooth-implemented hearing aids in hearing-impaired adults. Thirty subjects with bilateral symmetric moderate sensorineural hearing loss participated in this study. A Bluetooth-implemented hearing aid was fitted unilaterally in all study subjects. Objective speech recognition score and subjective satisfaction were measured with a Bluetooth-implemented hearing aid to replace the acoustic connection from either a cellular phone or a loudspeaker system. In each system, participants were assigned to 4 conditions: wireless speech signal transmission into hearing aid (wireless mode) in quiet or noisy environment and conventional speech signal transmission using external microphone of hearing aid (conventional mode) in quiet or noisy environment. Also, participants completed questionnaires to investigate subjective satisfaction. Both cellular phone and loudspeaker system situation, participants showed improvements in sentence and word recognition scores with wireless mode compared to conventional mode in both quiet and noise conditions (P < .001). Participants also reported subjective improvements, including better sound quality, less noise interference, and better accuracy naturalness, when using the wireless mode (P < .001). Bluetooth-implemented hearing aids helped to improve subjective and objective speech recognition performances in quiet and noisy environments during the use of electronic audio devices.

Radiotherapy-induced hearing loss in patients with laryngeal and hypopharyngeal carcinomas.

PubMed

Gudelj, Goran; Trotić, Robert; Herceg, Tonko; Parazajder, Domagoj; Vagić, Davor; Geber, Goran

2014-09-01

The purpose of this study was to investigate a hypothesized correlation of development of a sensorineural hearing loss and radiotherapy in patients with laryngeal and hypopharyngeal carcinoma. This prospective study included a total of 50 patients, which after strict exclusion critera (audiologic problems before RT primary tumors of the auditory system, spread of the primary tumor to any part of the auditory system) resulted in 23 analyzed patients, ranging between 50 and 76 years of age, with a mean age of 60. Audiometry measuring frequency-specific thresholds was performed in three time points: one month before radiotherapy, one and six months after radiotherapy. A significant statistical difference in hearing tresholds after radiotherapy was found in 6 out of 23 patients. An obvious tendency towards hearing loss without statistical significance at 250 and 4000 Hz was found for a whole tested population (p < or = 0.3 with Bonferroni correction). Observed tendency towards hearing loss after radiotherapy of laryngeal carcinoma was related to side of the tumor and less severe when chemotherapy was not added as adjuvant therapy. These results should help to decrease a rate of hearing loss by careful planing of ear protection, by using observed frequencies as relevant markers of hearing loss and by reconsidering adjuvant chemoterapy during radiotherapy of laryngeal carcinoma.

Polish universal neonatal hearing screening program-4-year experience (2003-2006).

PubMed

Szyfter, Witold; Wróbel, Maciej; Radziszewska-Konopka, Marzanna; Szyfter-Harris, Joanna; Karlik, Michał

2008-12-01

The aim of this paper is to share our experience and observations in running the Universal Neonatal Hearing Screening Program on a national level, present results and indicate some problems that have arisen during these 4 years. Polish Universal Neonatal Hearing Screening Program started back in 2002 in all neonatal units in Poland. Implemented testing methods consisted of test of transient evoked otoacoustic emission (TEOAE) performed in all new born children in their first 2-3 days of life and auditory brainstem response testing (ABR) conducted on children, who did not meet the TEOAE pass criteria. Additional questionnaire registered information on ototoxic drugs and family history of hearing impairment in every newborn. Diagnosed children were further referred for treatment and rehabilitation. After 4 years of running the program (between 2003 and 2006) a total number of 1,392,427 children were screened for hearing impairment, what stands for 96.3% of all delivered babies, registered in Poland. The screening program enabled to identify and refer for further treatment 2485 children with various types of hearing loss, 312 with profound (0.02% of population) and 145 with severe sensorineural hearing loss (0.11% of population). Our results indicate the accuracy of newborn hearing screening which remain an issue. Although improvement is needed in both intervention systems and diagnostic follow-up of hospitals, the Polish Universal Neonatal Hearing Program fully has achieved the main goal, the identification and treatment of hearing impaired children.

A longitudinal study on postoperative hearing thresholds with the Vibrant Soundbridge device.

PubMed

Vincent, C; Fraysse, B; Lavieille, J-P; Truy, E; Sterkers, O; Vaneecloo, F-M

2004-10-01

The Vibrant Soundbridge is a semi-implantable middle ear hearing device used in the rehabilitation of adults with sensorineural hearing loss. In order to evaluate the long-term effects of the implanted part of the device, audiological data from 39 patients implanted over several implant sites across France were collected and analyzed retrospectively. The mean follow-up time was 16 months; 25 patients had a follow-up period of over 1 year. Surgery was uneventful in all cases. The present study of the 39 implanted patients with a mid- to long-term follow-up found a statistically significant modification of hearing thresholds (pre- versus postoperative) for frequencies of 0.5 and 4 kHz. However, the shift of threshold was rather limited (2.79 and 3.34 dB, respectively), and this variation was not statistically different from the evolution of the opposite non-operated ear.

Evaluation of Speech Intelligibility and Sound Localization Abilities with Hearing Aids Using Binaural Wireless Technology.

PubMed

Ibrahim, Iman; Parsa, Vijay; Macpherson, Ewan; Cheesman, Margaret

2013-01-02

Wireless synchronization of the digital signal processing (DSP) features between two hearing aids in a bilateral hearing aid fitting is a fairly new technology. This technology is expected to preserve the differences in time and intensity between the two ears by co-ordinating the bilateral DSP features such as multichannel compression, noise reduction, and adaptive directionality. The purpose of this study was to evaluate the benefits of wireless communication as implemented in two commercially available hearing aids. More specifically, this study measured speech intelligibility and sound localization abilities of normal hearing and hearing impaired listeners using bilateral hearing aids with wireless synchronization of multichannel Wide Dynamic Range Compression (WDRC). Twenty subjects participated; 8 had normal hearing and 12 had bilaterally symmetrical sensorineural hearing loss. Each individual completed the Hearing in Noise Test (HINT) and a sound localization test with two types of stimuli. No specific benefit from wireless WDRC synchronization was observed for the HINT; however, hearing impaired listeners had better localization with the wireless synchronization. Binaural wireless technology in hearing aids may improve localization abilities although the possible effect appears to be small at the initial fitting. With adaptation, the hearing aids with synchronized signal processing may lead to an improvement in localization and speech intelligibility. Further research is required to demonstrate the effect of adaptation to the hearing aids with synchronized signal processing on different aspects of auditory performance.

Congenital malformations of the inner ear and the vestibulocochlear nerve in children with sensorineural hearing loss: evaluation with CT and MRI.

PubMed

Westerhof, J P; Rademaker, J; Weber, B P; Becker, H

2001-01-01

The purpose of this work was to study the diagnostic value of CT and MRI in children with sensorineural hearing loss and to analyze anatomic abnormalities of the inner ear and the vestibulocochlear nerve in this patient group. We evaluated 42 inner ears in 21 children with congenital deafness who had congenital inner ear malformations and who were candidates for cochlear implants. All patients were studied with high resolution MR and helical CT examinations. The MR study included a T2-weighted 3D fast SE sequence. We describe and tabulate the anatomic abnormalities. Special attention was given to abnormalities of the vestibulocochlear nerve. The field of view in the plane according to the length axis of the internal auditory canal (IAC) was 4 cm. Additional continuous parasagittal reformations perpendicular to the length axis of the IAC were studied with a field of view of 3 cm. CT and MRI allowed accurate identification of malformations of the inner ear in children with congenital deafness. We identified 99 malformations, with a majority of patients demonstrating multiple abnormalities. Common imaging findings were Mondini abnormality and Mondini variants (12/42) and fusion of the lateral or superior semicircular canal with the vestibule (12/42). MRI demonstrated in 9 of 21 patients a rudimentary or absent vestibulocochlear nerve in the auditory canal. CT and MRI are important modalities to analyze the inner ear in children who are candidates for cochlear implants. MRI with an extremely small field of view should be used to study possible abnormalities of the vestibulocochlear nerves. This may alter clinical care and allow cochlear implant placement in patients whose electrodiagnostic studies suggest that the implant should not be performed. The detailed analysis of abnormalities of the inner ear might establish prognostic factors.

Cisplatin and Aminoglycoside Antibiotics: Hearing Loss and Its Prevention

PubMed Central

Schacht, Jochen; Talaska, Andra E.; Rybak, Leonard P.

2013-01-01

This review introduces the pathology of aminoglycoside antibiotic and the cisplatin chemotherapy classes of drugs, discusses oxidative stress in the inner ear as a primary trigger for cell damage, and delineates the ensuing cell death pathways. Among potentially ototoxic (damaging the inner ear) therapeutics, the platinum-based anti-cancer drugs and the aminoglycoside antibiotics are of critical clinical importance. Both drugs cause sensorineural hearing loss in patients, a side effect that can be reproduced in experimental animals. Hearing loss is reflected primarily in damage to outer hair cells, beginning in the basal turn of the cochlea. In addition, aminoglycosides might affect the vestibular system while cisplatin seems to have a much lower likelihood to do so. Finally, based on an understanding the mechanisms of ototoxicity pharmaceutical ways of protection of the cochlea are presented PMID:23045231

An Auditory-Masking-Threshold-Based Noise Suppression Algorithm GMMSE-AMT[ERB] for Listeners with Sensorineural Hearing Loss

NASA Astrophysics Data System (ADS)

Natarajan, Ajay; Hansen, John H. L.; Arehart, Kathryn Hoberg; Rossi-Katz, Jessica

2005-12-01

This study describes a new noise suppression scheme for hearing aid applications based on the auditory masking threshold (AMT) in conjunction with a modified generalized minimum mean square error estimator (GMMSE) for individual subjects with hearing loss. The representation of cochlear frequency resolution is achieved in terms of auditory filter equivalent rectangular bandwidths (ERBs). Estimation of AMT and spreading functions for masking are implemented in two ways: with normal auditory thresholds and normal auditory filter bandwidths (GMMSE-AMT[ERB]-NH) and with elevated thresholds and broader auditory filters characteristic of cochlear hearing loss (GMMSE-AMT[ERB]-HI). Evaluation is performed using speech corpora with objective quality measures (segmental SNR, Itakura-Saito), along with formal listener evaluations of speech quality rating and intelligibility. While no measurable changes in intelligibility occurred, evaluations showed quality improvement with both algorithm implementations. However, the customized formulation based on individual hearing losses was similar in performance to the formulation based on the normal auditory system.

Analysis of Performance on Cognitive Test Measures Before, During, and After 6 Months of Hearing Aid Use: A Single-Subject Experimental Design.

PubMed

Desjardins, Jamie L

2016-06-01

The present study examined the effect of hearing aid use on cognitive test performance using a single-subject treatment design. Six participants 54 to 64 years old with sensorineural hearing loss were fitted with hearing aids. Participants used the hearing aids for approximately 8 hr each day for the duration of the study. A battery of cognitive tests was administered to participants during baseline (pre-hearing aid fitting), treatment (hearing aid use), and withdrawal (post-hearing aid use) study phases over a period of 6 months of hearing aid use. All participants showed significant improvements in performance on the cognitive test measures with hearing aid use. The most significant treatment effects were evidenced at 2 to 4 weeks of hearing aid use on the Listening Span Test and an auditory selective attention task. In many cases, cognitive performance scores returned to baseline levels after the participant stopped using the hearing aids. The findings from this study are consistent with the hypothesis that hearing aid use may improve cognitive performance by improving audibility and decreasing the cognitive load of the listening task.

[The criteria for the relationship between hearing impairment and professional occupation and the criteria for professional suitability in terms of hearing among the aeronautical personnel engaged in civilian aviation].

PubMed

Pankova, V B

This article describes the main clinical features associated with the development and manifestations of disturbed sound perception in the members of the aeronautical personnel engaged in the Russian civilian aviation. The main expert criteria for the relationship between the diseases of the organs of hearing (as exemplified by chronic sensorineural hearing impairment) and professional occupation have been developed based on the results of the clinical and diagnostic examination with the use of whispered and loud speech acumetry, tonal threshold audiometry, speech audiometry, impedancometry and evoked optoacoustic emission. Civilian aviation sectoral approaches to the evaluation of professional suitability in terms of hearing among the aeronautical personnel are considered in accordance with the criteria adopted by the International Civil Aviation Organization (ICAO), a specialized UN agency that sets the international standards for the civilian aviation and co-ordinates its development with the purpose of enhancing the safety and effectiveness of flights. The criteria are formulated for the solution of the expert problems arising from the relationship between the professional occupation and the diseases of the organs of hearing and for the evaluation of the professional suitability in terms of hearing among the aeronautical personnel engaged in civilian aviation.

Perception of suprasegmental features of speech by children with cochlear implants and children with hearing AIDS.

PubMed

Most, Tova; Peled, Miriam

2007-01-01

This study assessed perception of suprasegmental features of speech by 30 prelingual children with sensorineural hearing loss. Ten children had cochlear implants (CIs), and 20 children wore hearing aids (HA): 10 with severe hearing loss and 10 with profound hearing loss. Perception of intonation, syllable stress, word emphasis, and word pattern was assessed. Results revealed that the two HA groups significantly outperformed the CI group in perceiving both intonation and stress. Within each group, word pattern was perceived best, and then intonation and emphasis, with syllable stress perceived poorest. No significant correlation emerged between age at implantation and perception of the various suprasegmental features, possibly due to participants' relatively late age at implantation. Results indicated that CI use did not show an advantage over HA use in the perception of suprasegmental features of speech. Future research should continue to explore variables that might improve this perception.

Musicians change their tune: how hearing loss alters the neural code.

PubMed

Parbery-Clark, Alexandra; Anderson, Samira; Kraus, Nina

2013-08-01

Individuals with sensorineural hearing loss have difficulty understanding speech, especially in background noise. This deficit remains even when audibility is restored through amplification, suggesting that mechanisms beyond a reduction in peripheral sensitivity contribute to the perceptual difficulties associated with hearing loss. Given that normal-hearing musicians have enhanced auditory perceptual skills, including speech-in-noise perception, coupled with heightened subcortical responses to speech, we aimed to determine whether similar advantages could be observed in middle-aged adults with hearing loss. Results indicate that musicians with hearing loss, despite self-perceptions of average performance for understanding speech in noise, have a greater ability to hear in noise relative to nonmusicians. This is accompanied by more robust subcortical encoding of sound (e.g., stimulus-to-response correlations and response consistency) as well as more resilient neural responses to speech in the presence of background noise (e.g., neural timing). Musicians with hearing loss also demonstrate unique neural signatures of spectral encoding relative to nonmusicians: enhanced neural encoding of the speech-sound's fundamental frequency but not of its upper harmonics. This stands in contrast to previous outcomes in normal-hearing musicians, who have enhanced encoding of the harmonics but not the fundamental frequency. Taken together, our data suggest that although hearing loss modifies a musician's spectral encoding of speech, the musician advantage for perceiving speech in noise persists in a hearing-impaired population by adaptively strengthening underlying neural mechanisms for speech-in-noise perception. Copyright © 2013 Elsevier B.V. All rights reserved.

Vestibular (dys)function in children with sensorineural hearing loss: a systematic review.

PubMed

Verbecque, Evi; Marijnissen, Tessa; De Belder, Niels; Van Rompaey, Vincent; Boudewyns, An; Van de Heyning, Paul; Vereeck, Luc; Hallemans, Ann

2017-06-01

The objective of this study is to provide an overview of the prevalence of vestibular dysfunction in children with SNHL classified according to the applied test and its corresponding sensitivity and specificity. Data were gathered using a systematic search query including reference screening. Pubmed, Web of Science and Embase were searched. Strategy and reporting of this review was based on the Meta-analysis of Observational Studies in Epidemiology (MOOSE) guidelines. Methodological quality was assessed with the COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) checklist. All studies, regardless the applied vestibular test, showed that vestibular function differs significantly between children with hearing loss and normal hearing (p < 0.05). Compared with caloric testing, the sensitivity of the Rotational Chair Test (RCT) varies between 61 and 80% and specificity between 21 and 80%, whereas this was, respectively, 71-100% and 30-100% for collic Vestibular Evoked Myogenic Potentials (cVEMP). Compared with RCT, the sensitivity was 88-100% and the specificity was 69-100% for the Dynamic Visual Acuity test, respectively, 67-100% and 71-100% for the (video) Head Impulse Test and 83% and 86% for the ocular VEMP. Currently, due to methodological shortcoming, evidence on sensitivity and specificity of vestibular tests is unknown to moderate. Future research should focus on adequate sample sizes (subgroups >30).

Comparison of muzzle suppression and ear-level hearing protection in firearm use.

PubMed

Branch, Matthew Parker

2011-06-01

To compare noise reduction of commercially available ear-level hearing protection (muffs/inserts) to that of firearm muzzle suppressors. Experimental sound measurements under consistent environmental conditions. None. Muzzle suppressors for 2 pistol and 2 rifle calibers were tested using the Bruel & Kjaer 2209 sound meter and Bruel & Kjaer 4136 microphone calibrated with the Bruel & Kjaer Pistonphone using Military-Standard 1474D placement protocol. Five shots were recorded unsuppressed and 10 shots suppressed under consistent environmental conditions. Sound reduction was then compared with the real-world noise reduction rate of the best available ear-level protectors. All suppressors offered significantly greater noise reduction than ear-level protection, usually greater than 50% better. Noise reduction of all ear-level protectors is unable to reduce the impulse pressure below 140 dB for certain common firearms, an international standard for prevention of sensorineural hearing loss. Modern muzzle-level suppression is vastly superior to ear-level protection and the only available form of suppression capable of making certain sporting arms safe for hearing. The inadequacy of standard hearing protectors with certain common firearms is not recognized by most hearing professionals or their patients and should affect the way hearing professionals counsel patients and the public.

[Hearing capacity and speech production in 417 children with facial cleft abnormalities].

PubMed

Schönweiler, R; Schönweiler, B; Schmelzeisen, R

1994-11-01

Children with cleft palates often suffer from chronic conductive hearing losses, delayed language acquisition and speech disorders. This study presents results of speech and language outcomes in relation to hearing function and types of palatal malformations found. 417 children with cleft palates were examined during followup evaluations that extended over several years. Disorders were studied as they affected the ears, nose and throat, audiometry and speech and language pathology. Children with isolated cleft lips were excluded. Among the total group, 8% had normal speech and language development while 92% had speech or language disorders. 80% of these latter children had hearing problems that predominantly consisted of fluctuating conductive hearing losses caused by otitis media with effusion. 5% had sensorineural hearing losses. Fifty-eight children (14%) with rhinolalia aperta were not improved by speech therapy and required velopharyngoplasties, using a cranial-based pharyngeal flap. Language skills did not depend on the type of cleft palate presents but on the frequency and amount of hearing loss found. Otomicroscopy and audiometric follow-ups with insertions of ventilation tubes were considered to be most important for language development in those children with repeated middle ear infections. Speech or language therapy was necessary in 49% of the children.

Hearing loss and speech perception in noise difficulties in Fanconi anemia.

PubMed

Verheij, Emmy; Oomen, Karin P Q; Smetsers, Stephanie E; van Zanten, Gijsbert A; Speleman, Lucienne

2017-10-01

Fanconi anemia is a hereditary chromosomal instability disorder. Hearing loss and ear abnormalities are among the many manifestations reported in this disorder. In addition, Fanconi anemia patients often complain about hearing difficulties in situations with background noise (speech perception in noise difficulties). Our study aimed to describe the prevalence of hearing loss and speech perception in noise difficulties in Dutch Fanconi anemia patients. Retrospective chart review. A retrospective chart review was conducted at a Dutch tertiary care center. All patients with Fanconi anemia at clinical follow-up in our hospital were included. Medical files were reviewed to collect data on hearing loss and speech perception in noise difficulties. In total, 49 Fanconi anemia patients were included. Audiograms were available in 29 patients and showed hearing loss in 16 patients (55%). Conductive hearing loss was present in 24.1%, sensorineural in 20.7%, and mixed in 10.3%. A speech in noise test was performed in 17 patients; speech perception in noise was subnormal in nine patients (52.9%) and abnormal in two patients (11.7%). Hearing loss and speech perception in noise abnormalities are common in Fanconi anemia. Therefore, pure tone audiograms and speech in noise tests should be performed, preferably already at a young age, because hearing aids or assistive listening devices could be very valuable in developing language and communication skills. 4. Laryngoscope, 127:2358-2361, 2017. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

Acute hyperfibrinogenemia impairs cochlear blood flow and hearing function in guinea pigs in vivo.

PubMed

Ihler, Fritz; Strieth, Sebastian; Pieri, Nicos; Göhring, Peter; Canis, Martin

2012-03-01

Impairment of microcirculation is a possible cause of sudden sensorineural hearing loss (SSNHL). Fibrinogen is known as a risk factor for both microvascular dysfunction and SSNHL. Therefore, the aim of this study was to investigate the effect of elevated serum levels of fibrinogen on cochlear blood flow and hearing function in vivo. One group of guinea pigs received two consecutive injections of 100 mg fibrinogen while a control group received equimolar doses of albumin. Measurements of cochlear microcirculation by intravital microscopy and of hearing thresholds by auditory brainstem response (ABR) recordings were carried out before, after first and after second injection. Ten healthy guinea pigs were randomly assigned to a treatment group or a control group of five animals each. Serum fibrinogen levels were elevated after the first and second injections of fibrinogen compared to basal values and control group respectively. Increasing levels of fibrinogen were paralleled by decreasing cochlear blood flow as well as increasing hearing thresholds. Hearing threshold correlated negatively with cochlear blood flow. The effect of microcirculatory impairment on hearing function could be explained by a malfunction of the cochlear amplifier. Further investigation is needed to quantify cochlear potentials under elevated serum fibrinogen levels.

The BEACH protein LRBA is required for hair bundle maintenance in cochlear hair cells and for hearing.

PubMed

Vogl, Christian; Butola, Tanvi; Haag, Natja; Hausrat, Torben J; Leitner, Michael G; Moutschen, Michel; Lefèbvre, Philippe P; Speckmann, Carsten; Garrett, Lillian; Becker, Lore; Fuchs, Helmut; Hrabe de Angelis, Martin; Nietzsche, Sandor; Kessels, Michael M; Oliver, Dominik; Kneussel, Matthias; Kilimann, Manfred W; Strenzke, Nicola

2017-11-01

Lipopolysaccharide-responsive beige-like anchor protein (LRBA) belongs to the enigmatic class of BEACH domain-containing proteins, which have been attributed various cellular functions, typically involving intracellular protein and membrane transport processes. Here, we show that LRBA deficiency in mice leads to progressive sensorineural hearing loss. In LRBA knockout mice, inner and outer hair cell stereociliary bundles initially develop normally, but then partially degenerate during the second postnatal week. LRBA deficiency is associated with a reduced abundance of radixin and Nherf2, two adaptor proteins, which are important for the mechanical stability of the basal taper region of stereocilia. Our data suggest that due to the loss of structural integrity of the central parts of the hair bundle, the hair cell receptor potential is reduced, resulting in a loss of cochlear sensitivity and functional loss of the fraction of spiral ganglion neurons with low spontaneous firing rates. Clinical data obtained from two human patients with protein-truncating nonsense or frameshift mutations suggest that LRBA deficiency may likewise cause syndromic sensorineural hearing impairment in humans, albeit less severe than in our mouse model. © 2017 The Authors.

TAUROURSODEOXYCHOLIC ACID PREVENTS HEARING LOSS AND HAIR CELL DEATH IN Cdh23erl/erl MICE

PubMed Central

HU, J.; XU, M.; YUAN, J.; LI, B.; Entenman, S.; YU, H.; ZHENG, Q.Y.

2016-01-01

Sensorineural hearing loss has long been the subject of experimental and clinical research for many years. The recently identified novel mutation of the Cdh23 gene, Cdh23erl/erl, was proven to be a mouse model of human autosomal recessive nonsyndromic deafness (DFNB12). Tauroursodeoxycholic acid (TUDCA), a taurine-conjugated bile acid, has been used in experimental research and clinical applications related to liver disease, diabetes, neurodegenerative diseases, and other diseases associated with apoptosis. Because hair cell apoptosis was implied to be the cellular mechanism leading to hearing loss in Cdh23erl/erl mice (erl mice), this study investigated TUDCA’s otoprotective effects in erl mice: preventing hearing impairment and protecting against hair cell death. Our results showed that systemic treatment with TUDCA significantly alleviated hearing loss and suppressed hair cell death in erl mice. Additionally, TUDCA inhibited apoptotic genes and caspase-3 activation in erl mouse cochleae. The data suggest that TUDCA could be a potential therapeutic agent for human DFNB12. PMID:26748055

Hearing loss and enlarged internal auditory canal in children.

PubMed

Santos, Saturnino; Domínguez, M Jesús; Cervera, Javier; Suárez, Alicia; Bueno, Antonio; Bartolomé, Margarita; López, Rafael

2014-01-01

Among the temporal bone abnormalities that can be found in the etiological study of paediatric sensorineural hearing loss (SNHL) by imaging techniques, those related to the internal auditory canal (IAC) are the least frequent. The most prevalent of these abnormalities that is associated with SNHL is stenotic IAC due to its association with cochlear nerve deficiencies. Less frequent and less concomitant with SNHL is the finding of an enlarged IAC (>8mm). Retrospective and descriptive review of clinical associations, imaging, audiological patterns and treatment of 9 children with hearing loss and enlarged IAC in the period 1999 to 2012. Two groups of patients are described. The first, without association with vestibulocochlear dysplasias, consisted of: 2 patients with SNHL without other temporal bone or systemic abnormalities, one with bilateral mixed HL from chromosome 18q deletion, one with a genetic X-linked DFN3 hearing loss, one with unilateral hearing loss in neurofibromatosis type 2 with bilateral acoustic neuroma, and one with unilateral hearing loss with cochlear nerve deficiency. The second group, with association with vestibulocochlear dysplasias, was comprised of: one patient with moderate bilateral mixed hearing loss in branchio-oto-renal syndrome, one with profound unilateral SNHL with recurrent meningitis, and another with profound bilateral SNHL with congenital hypothyroidism. The presence of an enlarged IAC in children can be found in different clinical and audiological settings with relevancies that can range from life-threatening situations, such as recurrent meningitis, to isolated hearing loss with no other associations. Copyright © 2013 Elsevier España, S.L. All rights reserved.

Variations in otological presentation of lightning strike victims: Clinical report of 3 patients.

PubMed

Kılıç, Erbil; Genç, Hakan; Aydın, Ümit; Aşık, Burak; Satar, Bülent

2017-03-01

Lightning strike can cause fatal or nonfatal injuries. Some nonfatal injuries are associated with otological symptoms and findings. Conductive hearing loss due to rupture of the tympanic membrane is the most common audiovestibular lesion of lightning strike. Various forms of sensorineural hearing loss and dizziness have also been reported. Presently described are 3 cases of lightning strike injury. First patient had mid-frequency hearing loss in right ear and high frequency sensorineural hearing loss in left ear. Second patient had high frequency sensorineural hearing loss in left ear, and the third had peripheral facial palsy with perilymphatic fistula on same side. This is the first documented case of mid-frequency hearing loss occurring after lightning strike.

Hearing, speech, language, and vestibular disorders in the fetal alcohol syndrome: a literature review.

PubMed

Church, M W; Kaltenbach, J A

1997-05-01

Fetal alcohol syndrome (FAS) is characterized in part by mental impairment, as well as craniofacial and ocular anomalies. These conditions are traditionally associated with childhood hearing disorders, because they all have a common embryonic origin in malformations of the first and second branchial arches, and have similar critical periods of vulnerability to toxic insult. A review of human and animal research indicates that there are four types of hearing disorders associated with FAS. These are: (1) a developmental delay in auditory maturation, (2) sensorineural hearing loss, (3) intermittent conductive hearing loss due to recurrent serous otitis media, and (4) central hearing loss. The auditory and vestibular systems share the same peripheral apparatuses (the inner ear and eighth cranial nerve) and are embryologically and structurally similar. Consequently, vestibular disorders in FAS children might be expected. The evidence for vestibular dysfunction in FAS is ambiguous, however. Like other syndromes associated with craniofacial anomalies, hearing disorders, and mental impairment, FAS is also characterized by a high prevalence of speech and language pathology. Hearing disorders are a form of sensory deprivation. If present during early childhood, they can result in permanent hearing, language, and mental impairment. Early identification and intervention to treat hearing, language, and speech disorders could therefore result in improved outcome for the FAS child. Specific recommendations are made for intervention and future research.

The effects of repetitive vibration on sensorineural function: biomarkers of sensorineural injury in an animal model of metabolic syndrome

PubMed Central

Kiedrowski, Megan; Waugh, Stacey; Miller, Roger; Johnson, Claud; Krajnak, Kristine

2016-01-01

Exposure to hand-transmitted vibration in the work-place can result in the loss of sensation and pain in workers. These effects may be exacerbated by pre-existing conditions such as diabetes or the presence of primary Raynaud's phenomena. The goal of these studies was to use an established model of vibration-induced injury in Zucker rats. Lean Zucker rats have a normal metabolic profile, while obese Zucker rats display symptoms of metabolic disorder or Type II diabetes. This study examined the effects of vibration in obese and lean rats. Zucker rats were exposed to 4 h of vibration for 10 consecutive days at a frequency of 125 Hz and acceleration of 49 m/s2 for 10 consecutive days. Sensory function was checked using transcutaneous electrical stimulation on days 1, 5 and 9 of the exposure. Once the study was complete the ventral tail nerves, dorsal root ganglia and spinal cord were dissected, and levels of various transcripts involved in sensorineural dysfunction were measured. Sensorineural dysfunction was assessed using transcutaneous electrical stimulation. Obese Zucker rats displayed very few changes in sensorineural function. However they did display significant changes in transcript levels for factors involved in synapse formation, peripheral nerve remodeling, and inflammation. The changes in transcript levels suggested that obese Zucker rats had some level of sensory nerve injury prior to exposure, and that exposure to vibration activated pathways involved in injury and re-innervation. PMID:26433044

Prevalence of Tinnitus and Noise-induced Hearing Loss in Dentists

PubMed Central

Myers, Jamie; John, Andrew B.; Kimball, Suzanne; Fruits, Terry

2016-01-01

Introduction: The purpose of this study was to evaluate noise levels in dental offices and to estimate the risk and prevalence of tinnitus and noise-induced hearing loss (NIHL) in practicing dentists. Materials and Methods: First, measures were collected of sound pressure levels produced by dental handpieces and dental suction in the University of Oklahoma Health Sciences Center (OUHSC) College of Dentistry. Second, a survey was distributed to members of the Oklahoma Dental Association (ODA). Results: Measurements made in the dental operatory revealed dangerous levels when high-volume suction was in use alone and in conjunction with a dental handpiece. Questionnaire results suggested that practicing dentists report sensorineural hearing loss at a rate broadly in line with national averages. However, dentists reported a higher prevalence of tinnitus symptoms than would be expected based on sample demographics. Conclusion: Results from sound level measurements and questionnaire responses indicate that dentists are a population that could be placing their hearing health at risk in a typical daily work environment. PMID:27991466

Current aspects of hearing loss from occupational and leisure noise

PubMed Central

Plontke, S.; Zenner, H.-P.

2004-01-01

Hearing loss from occupational and leisure noise numbers amongst the most frequent causes of an acquired sensorineural hearing loss. Here we present a review of up-to-date findings on the pathophysiology of acoustic injury to the inner ear, with special attention being paid to its molecular-biological and genetic aspects. Epidemiological aspects shall also be dealt with, as shall the roles of lacking recovery from occupational noise due to additional exposure by leisure noise and the combined exposure of noise and chemicals. Based on the epidemiological and pathophysiological findings and against the background of published animal-experimental, pre-clinical and clinical findings, the various approaches for prevention, protection and therapeutic intervention with acoustic trauma are discussed. Pharmacological strategies involving anti-oxidative, anti-excitotoxic and anti-apoptotic substances as well as non-pharmacological strategies like "sound conditioning" are given attention. Furthermore, systemic and local substance application as well as the therapy of acute acoustic trauma and chronic hearing problems (including modern therapy forms for comorbidities such as tinnitus) shall be delved into. PMID:22073048

Temporal masking functions for listeners with real and simulated hearing loss

PubMed Central

Desloge, Joseph G.; Reed, Charlotte M.; Braida, Louis D.; Perez, Zachary D.; Delhorne, Lorraine A.

2011-01-01

A functional simulation of hearing loss was evaluated in its ability to reproduce the temporal masking functions for eight listeners with mild to severe sensorineural hearing loss. Each audiometric loss was simulated in a group of age-matched normal-hearing listeners through a combination of spectrally-shaped masking noise and multi-band expansion. Temporal-masking functions were obtained in both groups of listeners using a forward-masking paradigm in which the level of a 110-ms masker required to just mask a 10-ms fixed-level probe (5-10 dB SL) was measured as a function of the time delay between the masker offset and probe onset. At each of four probe frequencies (500, 1000, 2000, and 4000 Hz), temporal-masking functions were obtained using maskers that were 0.55, 1.0, and 1.15 times the probe frequency. The slopes and y-intercepts of the masking functions were not significantly different for listeners with real and simulated hearing loss. The y-intercepts were positively correlated with level of hearing loss while the slopes were negatively correlated. The ratio of the slopes obtained with the low-frequency maskers relative to the on-frequency maskers was similar for both groups of listeners and indicated a smaller compressive effect than that observed in normal-hearing listeners. PMID:21877806

Evaluation of Speech Intelligibility and Sound Localization Abilities with Hearing Aids Using Binaural Wireless Technology

PubMed Central

Ibrahim, Iman; Parsa, Vijay; Macpherson, Ewan; Cheesman, Margaret

2012-01-01

Wireless synchronization of the digital signal processing (DSP) features between two hearing aids in a bilateral hearing aid fitting is a fairly new technology. This technology is expected to preserve the differences in time and intensity between the two ears by co-ordinating the bilateral DSP features such as multichannel compression, noise reduction, and adaptive directionality. The purpose of this study was to evaluate the benefits of wireless communication as implemented in two commercially available hearing aids. More specifically, this study measured speech intelligibility and sound localization abilities of normal hearing and hearing impaired listeners using bilateral hearing aids with wireless synchronization of multichannel Wide Dynamic Range Compression (WDRC). Twenty subjects participated; 8 had normal hearing and 12 had bilaterally symmetrical sensorineural hearing loss. Each individual completed the Hearing in Noise Test (HINT) and a sound localization test with two types of stimuli. No specific benefit from wireless WDRC synchronization was observed for the HINT; however, hearing impaired listeners had better localization with the wireless synchronization. Binaural wireless technology in hearing aids may improve localization abilities although the possible effect appears to be small at the initial fitting. With adaptation, the hearing aids with synchronized signal processing may lead to an improvement in localization and speech intelligibility. Further research is required to demonstrate the effect of adaptation to the hearing aids with synchronized signal processing on different aspects of auditory performance. PMID:26557339

The relationship of speech intelligibility with hearing sensitivity, cognition, and perceived hearing difficulties varies for different speech perception tests

PubMed Central

Heinrich, Antje; Henshaw, Helen; Ferguson, Melanie A.

2015-01-01

Listeners vary in their ability to understand speech in noisy environments. Hearing sensitivity, as measured by pure-tone audiometry, can only partly explain these results, and cognition has emerged as another key concept. Although cognition relates to speech perception, the exact nature of the relationship remains to be fully understood. This study investigates how different aspects of cognition, particularly working memory and attention, relate to speech intelligibility for various tests. Perceptual accuracy of speech perception represents just one aspect of functioning in a listening environment. Activity and participation limits imposed by hearing loss, in addition to the demands of a listening environment, are also important and may be better captured by self-report questionnaires. Understanding how speech perception relates to self-reported aspects of listening forms the second focus of the study. Forty-four listeners aged between 50 and 74 years with mild sensorineural hearing loss were tested on speech perception tests differing in complexity from low (phoneme discrimination in quiet), to medium (digit triplet perception in speech-shaped noise) to high (sentence perception in modulated noise); cognitive tests of attention, memory, and non-verbal intelligence quotient; and self-report questionnaires of general health-related and hearing-specific quality of life. Hearing sensitivity and cognition related to intelligibility differently depending on the speech test: neither was important for phoneme discrimination, hearing sensitivity alone was important for digit triplet perception, and hearing and cognition together played a role in sentence perception. Self-reported aspects of auditory functioning were correlated with speech intelligibility to different degrees, with digit triplets in noise showing the richest pattern. The results suggest that intelligibility tests can vary in their auditory and cognitive demands and their sensitivity to the challenges that

Hearing and Mortality Outcomes following Temporal Bone Fractures.

PubMed

Honeybrook, Adam; Patki, Aniruddha; Chapurin, Nikita; Woodard, Charles

2017-12-01

The aim of this article is to determine hearing and mortality outcomes following temporal bone fractures. Retrospective chart review was performed of 152 patients diagnosed with a temporal bone fracture presenting to the emergency room at a tertiary care referral center over a 10-year period. Utilizing Patients' previously obtained temporal bone computed tomographic scans and audiograms, fractures were classified based on several classification schemes. Correlations between fracture patterns, mortality, and hearing outcomes were analyzed using χ 2 tests. Ossicular chain disruption was seen in 11.8% of patients, and otic capsule violation was seen in 5.9%; 22.7% of patients presented for audiologic follow-up. Seventeen patients with conductive hearing loss had air-bone gaps of 26 ± 7.5 dB (500 Hz), 27 ± 6.8 dB (1,000 Hz), 18 ± 6.2 dB (2,000 Hz), and 32 ± 7.7 dB (4,000 Hz). Two cases of profound sensorineural hearing loss were associated with otic capsule violation. No fracture classification scheme was predictive of hearing loss, although longitudinal fractures were statistically associated with ossicular chain disruption ( p  < 0.01). Temporal bone fractures in patients older than 60 years carried a relative risk of death of 3.15 compared with those younger than 60 years. The average magnitude of conductive hearing loss resulting from temporal bone fracture ranged from 18 to 32 dB in this cohort. Classification of fracture type was not predictive of hearing loss, despite the statistical association between ossicular chain disruption and longitudinal fractures. This finding may be due to the low follow-up rates of this patient population. Physicians should make a concerted effort to ensure that audiological monitoring is executed to prevent and manage long-term hearing impairment.

Auditory processing deficits are sometimes necessary and sometimes sufficient for language difficulties in children: Evidence from mild to moderate sensorineural hearing loss.

PubMed

Halliday, Lorna F; Tuomainen, Outi; Rosen, Stuart

2017-09-01

There is a general consensus that many children and adults with dyslexia and/or specific language impairment display deficits in auditory processing. However, how these deficits are related to developmental disorders of language is uncertain, and at least four categories of model have been proposed: single distal cause models, risk factor models, association models, and consequence models. This study used children with mild to moderate sensorineural hearing loss (MMHL) to investigate the link between auditory processing deficits and language disorders. We examined the auditory processing and language skills of 46, 8-16year-old children with MMHL and 44 age-matched typically developing controls. Auditory processing abilities were assessed using child-friendly psychophysical techniques in order to obtain discrimination thresholds. Stimuli incorporated three different timescales (µs, ms, s) and three different levels of complexity (simple nonspeech tones, complex nonspeech sounds, speech sounds), and tasks required discrimination of frequency or amplitude cues. Language abilities were assessed using a battery of standardised assessments of phonological processing, reading, vocabulary, and grammar. We found evidence that three different auditory processing abilities showed different relationships with language: Deficits in a general auditory processing component were necessary but not sufficient for language difficulties, and were consistent with a risk factor model; Deficits in slow-rate amplitude modulation (envelope) detection were sufficient but not necessary for language difficulties, and were consistent with either a single distal cause or a consequence model; And deficits in the discrimination of a single speech contrast (/bɑ/ vs /dɑ/) were neither necessary nor sufficient for language difficulties, and were consistent with an association model. Our findings suggest that different auditory processing deficits may constitute distinct and independent routes to

Postnatally acquired cytomegalovirus infection via breast milk: effects on hearing and development in preterm infants.

PubMed

Vollmer, Brigitte; Seibold-Weiger, Karin; Schmitz-Salue, Christine; Hamprecht, Klaus; Goelz, Rangmar; Krageloh-Mann, Ingeborg; Speer, Christian P

2004-04-01

In preterm infants there is a high risk of transmission of cytomegalovirus (CMV) via breast milk from seropositive mothers with reactivation of the virus during lactation. There is little information about the long term sequel of early postnatally acquired CMV infection in pre-term infants. This study aimed to investigate whether there was an increased frequency of impaired neurodevelopmental outcome and sensorineural hearing loss in preterm infants with postnatally acquired CMV infection through transmission by CMV-positive breast milk. Twenty-two preterm infants [median birth weight, 1020 g (range, 600 to 1870 g); median gestational age, 27.6 weeks (range, 23.6 to 32 weeks] with early postnatally acquired CMV infection by breast-feeding (onset of viruria between Days 23 and 190 postnatally) were compared with 22 CMV-negative preterm infants individually matched for gestational age, birth weight, gender, intracranial hemorrhage and duration of ventilation. At 2 to 4.5 years of age, follow-up assessments were conducted consisting of neurologic examination, neurodevelopmental assessment and detailed audiologic tests. None of the children had sensorineural hearing loss. There was no difference between the groups with regard to neurologic, speech and language or motor development. The results of this study suggest that early postnatally acquired CMV infection via CMV-positive breast milk does not have a negative effect on neurodevelopment and hearing in this group of patients. Because we studied a small number of infants, further follow-up studies are warranted in preterm infants with early postnatally acquired CMV infection.

Peripheral hearing loss reduces the ability of children to direct selective attention during multi-talker listening.

PubMed

Holmes, Emma; Kitterick, Padraig T; Summerfield, A Quentin

2017-07-01

Restoring normal hearing requires knowledge of how peripheral and central auditory processes are affected by hearing loss. Previous research has focussed primarily on peripheral changes following sensorineural hearing loss, whereas consequences for central auditory processing have received less attention. We examined the ability of hearing-impaired children to direct auditory attention to a voice of interest (based on the talker's spatial location or gender) in the presence of a common form of background noise: the voices of competing talkers (i.e. during multi-talker, or "Cocktail Party" listening). We measured brain activity using electro-encephalography (EEG) when children prepared to direct attention to the spatial location or gender of an upcoming target talker who spoke in a mixture of three talkers. Compared to normally-hearing children, hearing-impaired children showed significantly less evidence of preparatory brain activity when required to direct spatial attention. This finding is consistent with the idea that hearing-impaired children have a reduced ability to prepare spatial attention for an upcoming talker. Moreover, preparatory brain activity was not restored when hearing-impaired children listened with their acoustic hearing aids. An implication of these findings is that steps to improve auditory attention alongside acoustic hearing aids may be required to improve the ability of hearing-impaired children to understand speech in the presence of competing talkers. Copyright © 2017 Elsevier B.V. All rights reserved.

[Characteristics of audiology and clinical genetics of a Chinese family with the DFNA5 genetic hearing loss].

PubMed

Jin, Zhanguo; Cheng, Jing; Han, Bing; Li, Hongbo; Lu, Yu; Li, Zhengyue; Han, Dongyi

2011-05-01

To analysis the characteristics of audiology and clinical genetics of a Chinese family with the DFNA5 genetic hearing loss in detail. A detailed family history and clinical data were collected. The Chinese pedigree is an autosomal-dominant inherited hearing loss. The data of audiological examination about genetic characteristics was analysed. The relationship between the hearing-impaired of this family and age was contrasted. This Chinese family spanned five generations and comprised 42 members. The mode of inheritance of the families should be autosomal dominant according to the pedigree. Pure-tone audiograms showed a so-called Z shape curve. The hearing loss is sensorineural, progressive and beginning at the high frequencies. The audiograms were fairly symmetric. Whole frequencies became involved with increasing age. The Chinese family with the DFNA5 mutation was an autosomal dominant pedigree. In this family, non-syndromic symmetric hearing impairment was severest at the high frequencies early, and gradually accumulated all frequencies of hearing. A mutation in DFNA5 leads to a type of hearing loss that closely resembles the frequently observed age-related hearing impairment. It should take into account DFNA5 mutation which the audiogram of a genetic hearing impaired has the same feature.

Alteration of frequency range for binaural beats in acute low-tone hearing loss.

PubMed

Karino, Shotaro; Yamasoba, Tatsuya; Ito, Ken; Kaga, Kimitaka

2005-01-01

The effect of acute low-tone sensorineural hearing loss (ALHL) on the interaural frequency difference (IFD) required for perception of binaural beats (BBs) was investigated in 12 patients with unilateral ALHL and 7 patients in whom ALHL had lessened. A continuous pure tone of 30 dB sensation level at 250 Hz was presented to the contralateral, normal-hearing ear. The presence of BBs was determined by a subjective yes-no procedure as the frequency of a loudness-balanced test tone was gradually adjusted around 250 Hz in the affected ear. The frequency range in which no BBs were perceived (FRNB) was significantly wider in the patients with ALHL than in the controls, and FRNBs became narrower in the recovered ALHL group. Specifically, detection of slow BBs with a small IFD was impaired in this limited (10 s) observation period. The significant correlation between the hearing level at 250 Hz and FRNBs suggests that FRNBs represent the degree of cochlear damage caused by ALHL.

Unique autosomal recessive variant of palmoplantar keratoderma associated with hearing loss not caused by known mutations*

PubMed Central

Hegazi, Moustafa Abdelaal; Manou, Sommen; Sakr, Hazem; Camp, Guy Van

2017-01-01

Inherited Palmoplantar Keratodermas are rare disorders of genodermatosis that are conventionally regarded as autosomal dominant in inheritance with extensive clinical and genetic heterogeneity. This is the first report of a unique autosomal recessive Inherited Palmoplantar keratoderma - sensorineural hearing loss syndrome which has not been reported before in 3 siblings of a large consanguineous family. The patients presented unique clinical features that were different from other known Inherited Palmoplantar Keratodermas - hearing loss syndromes. Mutations in GJB2 or GJB6 and the mitochondrial A7445G mutation, known to be the major causes of diverse Inherited Palmoplantar Keratodermas -hearing loss syndromes were not detected by Sanger sequencing. Moreover, the pathogenic mutation could not be identified using whole exome sequencing. Other known Inherited Palmoplantar keratoderma syndromes were excluded based on both clinical criteria and genetic analysis. PMID:29267478

Working Memory and Speech Recognition in Noise under Ecologically Relevant Listening Conditions: Effects of Visual Cues and Noise Type among Adults with Hearing Loss

ERIC Educational Resources Information Center

Miller, Christi W.; Stewart, Erin K.; Wu, Yu-Hsiang; Bishop, Christopher; Bentler, Ruth A.; Tremblay, Kelly

2017-01-01

Purpose: This study evaluated the relationship between working memory (WM) and speech recognition in noise with different noise types as well as in the presence of visual cues. Method: Seventy-six adults with bilateral, mild to moderately severe sensorineural hearing loss (mean age: 69 years) participated. Using a cross-sectional design, 2…

Noise-induced hearing loss alters the temporal dynamics of auditory-nerve responses

PubMed Central

Scheidt, Ryan E.; Kale, Sushrut; Heinz, Michael G.

2010-01-01

Auditory-nerve fibers demonstrate dynamic response properties in that they adapt to rapid changes in sound level, both at the onset and offset of a sound. These dynamic response properties affect temporal coding of stimulus modulations that are perceptually relevant for many sounds such as speech and music. Temporal dynamics have been well characterized in auditory-nerve fibers from normal-hearing animals, but little is known about the effects of sensorineural hearing loss on these dynamics. This study examined the effects of noise-induced hearing loss on the temporal dynamics in auditory-nerve fiber responses from anesthetized chinchillas. Post-stimulus time histograms were computed from responses to 50-ms tones presented at characteristic frequency and 30 dB above fiber threshold. Several response metrics related to temporal dynamics were computed from post-stimulus-time histograms and were compared between normal-hearing and noise-exposed animals. Results indicate that noise-exposed auditory-nerve fibers show significantly reduced response latency, increased onset response and percent adaptation, faster adaptation after onset, and slower recovery after offset. The decrease in response latency only occurred in noise-exposed fibers with significantly reduced frequency selectivity. These changes in temporal dynamics have important implications for temporal envelope coding in hearing-impaired ears, as well as for the design of dynamic compression algorithms for hearing aids. PMID:20696230

Paired comparisons of nonlinear frequency compression, extended bandwidth, and restricted bandwidth hearing-aid processing for children and adults with hearing loss

PubMed Central

Brennan, Marc A.; McCreery, Ryan; Kopun, Judy; Hoover, Brenda; Alexander, Joshua; Lewis, Dawna; Stelmachowicz, Patricia G.

2014-01-01

Background Preference for speech and music processed with nonlinear frequency compression and two controls (restricted and extended bandwidth hearing-aid processing) was examined in adults and children with hearing loss. Purpose Determine if stimulus type (music, sentences), age (children, adults) and degree of hearing loss influence listener preference for nonlinear frequency compression, restricted bandwidth and extended bandwidth. Research Design Within-subject, quasi-experimental study. Using a round-robin procedure, participants listened to amplified stimuli that were 1) frequency-lowered using nonlinear frequency compression, 2) low-pass filtered at 5 kHz to simulate the restricted bandwidth of conventional hearing aid processing, or 3) low-pass filtered at 11 kHz to simulate extended bandwidth amplification. The examiner and participants were blinded to the type of processing. Using a two-alternative forced-choice task, participants selected the preferred music or sentence passage. Study Sample Sixteen children (8–16 years) and 16 adults (19–65 years) with mild-to-severe sensorineural hearing loss. Intervention All subjects listened to speech and music processed using a hearing-aid simulator fit to the Desired Sensation Level algorithm v.5.0a (Scollie et al, 2005). Results Children and adults did not differ in their preferences. For speech, participants preferred extended bandwidth to both nonlinear frequency compression and restricted bandwidth. Participants also preferred nonlinear frequency compression to restricted bandwidth. Preference was not related to degree of hearing loss. For music, listeners did not show a preference. However, participants with greater hearing loss preferred nonlinear frequency compression to restricted bandwidth more than participants with less hearing loss. Conversely, participants with greater hearing loss were less likely to prefer extended bandwidth to restricted bandwidth. Conclusion Both age groups preferred access to

Interactions between amplitude modulation and frequency modulation processing: Effects of age and hearing loss.

PubMed

Paraouty, Nihaad; Ewert, Stephan D; Wallaert, Nicolas; Lorenzi, Christian

2016-07-01

Frequency modulation (FM) and amplitude modulation (AM) detection thresholds were measured for a 500-Hz carrier frequency and a 5-Hz modulation rate. For AM detection, FM at the same rate as the AM was superimposed with varying FM depth. For FM detection, AM at the same rate was superimposed with varying AM depth. The target stimuli always contained both amplitude and frequency modulations, while the standard stimuli only contained the interfering modulation. Young and older normal-hearing listeners, as well as older listeners with mild-to-moderate sensorineural hearing loss were tested. For all groups, AM and FM detection thresholds were degraded in the presence of the interfering modulation. AM detection with and without interfering FM was hardly affected by either age or hearing loss. While aging had an overall detrimental effect on FM detection with and without interfering AM, there was a trend that hearing loss further impaired FM detection in the presence of AM. Several models using optimal combination of temporal-envelope cues at the outputs of off-frequency filters were tested. The interfering effects could only be predicted for hearing-impaired listeners. This indirectly supports the idea that, in addition to envelope cues resulting from FM-to-AM conversion, normal-hearing listeners use temporal fine-structure cues for FM detection.

Hearing rehabilitation with single-stage bilateral vibroplasty in a child with Franceschetti syndrome.

PubMed

Sargsyan, Sona; Rahne, Torsten; Kösling, Sabrina; Eichler, Gerburg; Plontke, Stefan K

2014-05-01

Hearing is of utmost importance for normal speech and social development. Even children who have mild or unilateral permanent hearing loss may experience difficulties with understanding speech, as well as problems with educational and psycho-social development. The increasing advantages of middle-ear implant technologies are opening new perspectives for restoring hearing. Active middle-ear implants can be used in children and adolescents with hearing loss. In addition to the well-documented results for improving speech intelligibility and quality of hearing in sensorineural hearing loss active middle-ear implants are now successfully used in patients with conductive and mixed hearing loss. In this article we present a case of successful, single-stage vibroplasty, on the right side with the fixation of the FMT on the stapes and PORP CLiP vibroplasty on the left side in a 6-year-old girl with bilateral mixed hearing loss and multiple dyslalia associated with Franceschetti syndrome (mandibulofacial dysostosis). CT revealed bilateral middle-ear malformations as well as an atretic right and stenotic left external auditory canal. Due to craniofacial dysmorphia airway and (post)operative, management is significantly more difficult in patients with a Franceschetti syndrome which in this case favoured a single-stage bilateral procedure. No intra- or postoperative surgical complications were reported. The middle-ear implants were activated 4 weeks after surgery. In the audiological examination 6 months after surgery, the child showed 100% speech intelligibility with activated implants on each side.

Binaural speech discrimination under noise in hearing-impaired listeners

NASA Technical Reports Server (NTRS)

Kumar, K. V.; Rao, A. B.

1988-01-01

This paper presents the results of an assessment of speech discrimination by hearing-impaired listeners (sensori-neural, conductive, and mixed groups) under binaural free-field listening in the presence of background noise. Subjects with pure-tone thresholds greater than 20 dB in 0.5, 1.0 and 2.0 kHz were presented with a version of the W-22 list of phonetically balanced words under three conditions: (1) 'quiet', with the chamber noise below 28 dB and speech at 60 dB; (2) at a constant S/N ratio of +10 dB, and with a background white noise at 70 dB; and (3) same as condition (2), but with the background noise at 80 dB. The mean speech discrimination scores decreased significantly with noise in all groups. However, the decrease in binaural speech discrimination scores with an increase in hearing impairment was less for material presented under the noise conditions than for the material presented in quiet.

The Effects of Hearing Aid Directional Microphone and Noise Reduction Processing on Listening Effort in Older Adults with Hearing Loss.

PubMed

Desjardins, Jamie L

2016-01-01

Older listeners with hearing loss may exert more cognitive resources to maintain a level of listening performance similar to that of younger listeners with normal hearing. Unfortunately, this increase in cognitive load, which is often conceptualized as increased listening effort, may come at the cost of cognitive processing resources that might otherwise be available for other tasks. The purpose of this study was to evaluate the independent and combined effects of a hearing aid directional microphone and a noise reduction (NR) algorithm on reducing the listening effort older listeners with hearing loss expend on a speech-in-noise task. Participants were fitted with study worn commercially available behind-the-ear hearing aids. Listening effort on a sentence recognition in noise task was measured using an objective auditory-visual dual-task paradigm. The primary task required participants to repeat sentences presented in quiet and in a four-talker babble. The secondary task was a digital visual pursuit rotor-tracking test, for which participants were instructed to use a computer mouse to track a moving target around an ellipse that was displayed on a computer screen. Each of the two tasks was presented separately and concurrently at a fixed overall speech recognition performance level of 50% correct with and without the directional microphone and/or the NR algorithm activated in the hearing aids. In addition, participants reported how effortful it was to listen to the sentences in quiet and in background noise in the different hearing aid listening conditions. Fifteen older listeners with mild sloping to severe sensorineural hearing loss participated in this study. Listening effort in background noise was significantly reduced with the directional microphones activated in the hearing aids. However, there was no significant change in listening effort with the hearing aid NR algorithm compared to no noise processing. Correlation analysis between objective and self

Visual Cues Contribute Differentially to Audiovisual Perception of Consonants and Vowels in Improving Recognition and Reducing Cognitive Demands in Listeners With Hearing Impairment Using Hearing Aids.

PubMed

Moradi, Shahram; Lidestam, Björn; Danielsson, Henrik; Ng, Elaine Hoi Ning; Rönnberg, Jerker

2017-09-18

We sought to examine the contribution of visual cues in audiovisual identification of consonants and vowels-in terms of isolation points (the shortest time required for correct identification of a speech stimulus), accuracy, and cognitive demands-in listeners with hearing impairment using hearing aids. The study comprised 199 participants with hearing impairment (mean age = 61.1 years) with bilateral, symmetrical, mild-to-severe sensorineural hearing loss. Gated Swedish consonants and vowels were presented aurally and audiovisually to participants. Linear amplification was adjusted for each participant to assure audibility. The reading span test was used to measure participants' working memory capacity. Audiovisual presentation resulted in shortened isolation points and improved accuracy for consonants and vowels relative to auditory-only presentation. This benefit was more evident for consonants than vowels. In addition, correlations and subsequent analyses revealed that listeners with higher scores on the reading span test identified both consonants and vowels earlier in auditory-only presentation, but only vowels (not consonants) in audiovisual presentation. Consonants and vowels differed in terms of the benefits afforded from their associative visual cues, as indicated by the degree of audiovisual benefit and reduction in cognitive demands linked to the identification of consonants and vowels presented audiovisually.

[Phenotypic trends and breeding values for canine congenital sensorineural deafness in Dalmatian dogs].

PubMed

Blum, Meike; Distl, Ottmar

2014-01-01

In the present study, breeding values for canine congenital sensorineural deafness, the presence of blue eyes and patches have been predicted using multivariate animal models to test the reliability of the breeding values for planned matings. The dataset consisted of 6669 German Dalmatian dogs born between 1988 and 2009. Data were provided by the Dalmatian kennel clubs which are members of the German Association for Dog Breeding and Husbandry (VDH). The hearing status for all dogs was evaluated using brainstem auditory evoked potentials. The reliability using the prediction error variance of breeding values and the realized reliability of the prediction of the phenotype of future progeny born in each one year between 2006 and 2009 were used as parameters to evaluate the goodness of prediction through breeding values. All animals from the previous birth years were used for prediction of the breeding values of the progeny in each of the up-coming birth years. The breeding values based on pedigree records achieved an average reliability of 0.19 for the future 1951 progeny. The predictive accuracy (R2) for the hearing status of single future progeny was at 1.3%. Combining breeding values for littermates increased the predictive accuracy to 3.5%. Corresponding values for maternal and paternal half-sib groups were at 3.2 and 7.3%. The use of breeding values for planned matings increases the phenotypic selection response over mass selection. The breeding values of sires may be used for planned matings because reliabilities and predictive accuracies for future paternal progeny groups were highest.

Idiopathic ophthalmodynia and idiopathic rhinalgia: two topographic facial pain syndromes.

PubMed

Pareja, Juan A; Cuadrado, María L; Porta-Etessam, Jesús; Fernández-de-las-Peñas, César; Gili, Pablo; Caminero, Ana B; Cebrián, José L

2010-09-01

To describe 2 topographic facial pain conditions with the pain clearly localized in the eye (idiopathic ophthalmodynia) or in the nose (idiopathic rhinalgia), and to propose their distinction from persistent idiopathic facial pain. Persistent idiopathic facial pain, burning mouth syndrome, atypical odontalgia, and facial arthromyalgia are idiopathic facial pain syndromes that have been separated according to topographical criteria. Still, some other facial pain syndromes might have been veiled under the broad term of persistent idiopathic facial pain. Through a 10-year period we have studied all patients referred to our neurological clinic because of facial pain of unknown etiology that might deviate from all well-characterized facial pain syndromes. In a group of patients we have identified 2 consistent clinical pictures with pain precisely located either in the eye (n=11) or in the nose (n=7). Clinical features resembled those of other localized idiopathic facial syndromes, the key differences relying on the topographic distribution of the pain. Both idiopathic ophthalmodynia and idiopathic rhinalgia seem specific pain syndromes with a distinctive location, and may deserve a nosologic status just as other focal pain syndromes of the face. Whether all such focal syndromes are topographic variants of persistent idiopathic facial pain or independent disorders remains a controversial issue.

Excitatory, inhibitory and facilitatory frequency response areas in the inferior colliculus of hearing impaired mice.

PubMed

Felix, Richard A; Portfors, Christine V

2007-06-01

Individuals with age-related hearing loss often have difficulty understanding complex sounds such as basic speech. The C57BL/6 mouse suffers from progressive sensorineural hearing loss and thus is an effective tool for dissecting the neural mechanisms underlying changes in complex sound processing observed in humans. Neural mechanisms important for processing complex sounds include multiple tuning and combination sensitivity, and these responses are common in the inferior colliculus (IC) of normal hearing mice. We examined neural responses in the IC of C57Bl/6 mice to single and combinations of tones to examine the extent of spectral integration in the IC after age-related high frequency hearing loss. Ten percent of the neurons were tuned to multiple frequency bands and an additional 10% displayed non-linear facilitation to the combination of two different tones (combination sensitivity). No combination-sensitive inhibition was observed. By comparing these findings to spectral integration properties in the IC of normal hearing CBA/CaJ mice, we suggest that high frequency hearing loss affects some of the neural mechanisms in the IC that underlie the processing of complex sounds. The loss of spectral integration properties in the IC during aging likely impairs the central auditory system's ability to process complex sounds such as speech.

Novel missense mutations in MYO7A underlying postlingual high- or low-frequency non-syndromic hearing impairment in two large families from China.

PubMed

Sun, Yi; Chen, Jing; Sun, Hanjun; Cheng, Jing; Li, Jianzhong; Lu, Yu; Lu, Yanping; Jin, Zhanguo; Zhu, Yuhua; Ouyang, Xiaomei; Yan, Denise; Dai, Pu; Han, Dongyi; Yang, Weiyan; Wang, Rongguang; Liu, Xuezhong; Yuan, Huijun

2011-01-01

The myosin VIIA (MYO7A) gene encodes a protein classified as an unconventional myosin. Mutations within MYO7A can lead to both syndromic and non-syndromic hearing impairment in humans. Among different mutations reported in MYO7A, only five led to non-syndromic sensorineural deafness autosomal dominant type 11 (DFNA11). Here, we present the clinical, genetic and molecular characteristics of two large Chinese DFNA11 families with either high- or low-frequency hearing loss. Affected individuals of family DX-J033 have a sloping audiogram at young ages with high frequency are most affected. With increasing age, all test frequencies are affected. Affected members of family HB-S037 present with an ascending audiogram affecting low frequencies at young ages, and then all frequencies are involved with increasing age. Genome-wide linkage analysis mapped the disease loci within the DFNA11 interval in both families. DNA sequencing of MYO7A revealed two novel nucleotide variations, c.652G > A (p.D218N) and c.2011G > A (p.G671S), in the two families. It is for the first time that the mutations identified in MYO7A in the present study are being implicated in DFNA11 in a Chinese population. For the first time, we tested electrocochleography (ECochG) in a DFNA11 family with low-frequency hearing loss. We speculate that the low-frequency sensorineural hearing loss in this DFNA11 family was not associated with endolymphatic hydrops.

Conductive hearing loss and middle ear pathology in young infants referred through a newborn universal hearing screening program in Australia.

PubMed

Aithal, Sreedevi; Aithal, Venkatesh; Kei, Joseph; Driscoll, Carlie

2012-10-01

over time with 66.67% of ATSI infants reviewed showing persistent conductive hearing loss compared to 17.86% of non-ATSI infants. Medical management of 17 infants with persistent conductive hearing loss included monitoring, antibiotic treatment, examination under anesthesia, and grommet insertion. Conductive hearing loss was found to be a common diagnosis among infants referred through screening. ATSI infants had significantly higher rates of middle ear pathology and conductive hearing loss at birth and showed poor resolution of middle ear pathology over time compared to non-ATSI infants. Future research using a direct measure of middle ear function as an adjunct to the automated auditory brainstem response screening tool to distinguish conductive from sensorineural hearing loss may facilitate prioritization of infants for assessment, thus reducing parental anxiety and streamlining the management strategies for the respective types of hearing loss. American Academy of Audiology.

Assessment of Appalachian region pediatric hearing healthcare disparities and delays.

PubMed

Bush, Matthew L; Osetinsky, Mariel; Shinn, Jennifer B; Gal, Thomas J; Ding, Xiuhua; Fardo, David W; Schoenberg, Nancy

2014-07-01

The purpose of this study was to examine the timing of diagnostic and therapeutic services in cochlear implant recipients from a rural Appalachian region with healthcare disparity. Retrospective analysis. Cochlear implant recipients from a tertiary referral center born with severe congenital sensorineural hearing loss were examined. Rural status and Appalachian status of their county of origin were recorded. A log-rank test was used to examine differences in the distributions of time to definitive diagnosis of hearing loss, initial amplification fitting, and cochlear implantation in these children. Correlation analysis of the rural status of each county and the timing of services was assessed. A total of 53 children born with congenital hearing loss were included in the study (36 from rural counties and 17 from urban/suburban counties). The distribution of weeks after birth to diagnosis (P=.006), amplification (P=.030), and cochlear implantation (P=.002) was delayed in rural children compared with urban children. An analysis factoring in the effect of implementation of mandatory infant hearing screening in 2000 demonstrated a similar delay in rural children for weeks to diagnosis (P=.028), amplification (P=.087), and cochlear implantation (P<.0001). Children with severe hearing loss in very rural areas, such as Appalachia, may have significant delays in diagnostic and rehabilitative services. Further investigation is warranted to assess causative factors in delays of cochlear implantation and to develop interventions to promote timely diagnosis and care. 3b. © 2014 The American Laryngological, Rhinological and Otological Society, Inc.

Clinical study of a digital vs an analogue hearing aid.

PubMed

Bille, M; Jensen, A M; Kjaerbøl, E; Vesterager, V; Sibelle, P; Nielsen, H

1999-01-01

Digital signal processing in hearing instruments has brought new perspectives to the compensation of hearing impairment and may result in alleviation of the adverse effects of hearing problems. This study compares a commercially available digital signal processing hearing aid (HA) (Senso) with a modern analogue HA with programmable fitting (Logo). The HAs tested are identical in appearance and, in spite of a different mode of operation, the study design ensured blinding of the test subjects. Outcome parameters were: improvements in speech recognition score in noise (deltaSRSN) with the HAs; overall preference for HA; overall satisfaction; and various measures of HA performance evaluated by a self-assessment questionnaire. A total of 28 experienced HA users with sensorineural hearing impairment were included and 25 completed the trial. No significant differences were found in deltaSRSN between the two HAs. Eleven subjects indicated an overall preference for the digital HA, 10 preferred the analogue HA and 4 had no preference. Concerning overall satisfaction, 8 subjects rated the digital HA superior to the analogue one, whereas 7 indicated a superior rating for the analogue HA and 10 rated the HAs equal. Acceptability of noise from traffic was the only outcome parameter which gave a significant difference between the HAs in favour of the digital HA. It is concluded that there are no significant differences in outcome between the digital and analogue signal processing HAs tested by these experienced HA-users.

Hearing Loss in HIV-Infected Children in Lilongwe, Malawi

PubMed Central

Hrapcak, Susan; Kuper, Hannah; Bartlett, Peter; Devendra, Akash; Makawa, Atupele; Kim, Maria; Kazembe, Peter; Ahmed, Saeed

2016-01-01

Introduction With improved access to antiretroviral therapy (ART), HIV infection is becoming a chronic illness. Preliminary data suggest that HIV-infected children have a higher risk of disabilities, including hearing impairment, although data are sparse. This study aimed to estimate the prevalence and types of hearing loss in HIV-infected children in Lilongwe, Malawi. Methods This was a cross-sectional survey of 380 HIV-infected children aged 4–14 years attending ART clinic in Lilongwe between December 2013-March 2014. Data was collected through pediatric quality of life and sociodemographic questionnaires, electronic medical record review, and detailed audiologic testing. Hearing loss was defined as >20 decibels hearing level (dBHL) in either ear. Predictors of hearing loss were explored by regression analysis generating age- and sex-adjusted odds ratios. Children with significant hearing loss were fitted with hearing aids. Results Of 380 patients, 24% had hearing loss: 82% conductive, 14% sensorineural, and 4% mixed. Twenty-one patients (23% of those with hearing loss) were referred for hearing aid fitting. There was a higher prevalence of hearing loss in children with history of frequent ear infections (OR 7.4, 4.2–13.0) and ear drainage (OR 6.4, 3.6–11.6). Hearing loss was linked to history of WHO Stage 3 (OR 2.4, 1.2–4.5) or Stage 4 (OR 6.4, 2.7–15.2) and history of malnutrition (OR 2.1, 1.3–3.5), but not to duration of ART or CD4. Only 40% of caregivers accurately perceived their child’s hearing loss. Children with hearing impairment were less likely to attend school and had poorer emotional (p = 0.02) and school functioning (p = 0.04). Conclusions There is an urgent need for improved screening tools, identification and treatment of hearing problems in HIV-infected children, as hearing loss was common in this group and affected school functioning and quality of life. Clear strategies were identified for prevention and treatment, since most

Cochlear hearing loss in patients with Laron syndrome.

PubMed

Attias, Joseph; Zarchi, Omer; Nageris, Ben I; Laron, Zvi

2012-02-01

The aim of this prospective clinical study was to test auditory function in patients with Laron syndrome, either untreated or treated with insulin-like growth factor I (IGF-I). The study group consisted of 11 patients with Laron syndrome: 5 untreated adults, 5 children and young adults treated with replacement IGF-I starting at bone age <2 years, and 1 adolescent who started replacement therapy at bone age 4.6 years. The auditory evaluation included pure tone and speech audiometry, tympanometry and acoustic reflexes, otoacoustic emissions, loudness dynamics, auditory brain stem responses and a hyperacusis questionnaire. All untreated patients and the patient who started treatment late had various degrees of sensorineural hearing loss and auditory hypersensitivity; acoustic middle ear reflexes were absent in most of them. All treated children had normal hearing and no auditory hypersensitivity; most had recordable middle ear acoustic reflexes. In conclusion, auditory defects seem to be associated with Laron syndrome and may be prevented by starting treatment with IGF-I at an early developmental age.

An Objective Estimation of Air-Bone-Gap in Cochlear Implant Recipients with Residual Hearing Using Electrocochleography.

PubMed

Koka, Kanthaiah; Saoji, Aniket A; Attias, Joseph; Litvak, Leonid M

2017-01-01

Although, cochlear implants (CI) traditionally have been used to treat individuals with bilateral profound sensorineural hearing loss, a recent trend is to implant individuals with residual low-frequency hearing. Notably, many of these individuals demonstrate an air-bone gap (ABG) in low-frequency, pure-tone thresholds following implantation. An ABG is the difference between audiometric thresholds measured using air conduction (AC) and bone conduction (BC) stimulation. Although, behavioral AC thresholds are straightforward to assess, BC thresholds can be difficult to measure in individuals with severe-to-profound hearing loss because of vibrotactile responses to high-level, low-frequency stimulation and the potential contribution of hearing in the contralateral ear. Because of these technical barriers to measuring behavioral BC thresholds in implanted patients with residual hearing, it would be helpful to have an objective method for determining ABG. This study evaluated an innovative technique for measuring electrocochleographic (ECochG) responses using the cochlear microphonic (CM) response to assess AC and BC thresholds in implanted patients with residual hearing. Results showed high correlations between CM thresholds and behavioral audiograms for AC and BC conditions, thereby demonstrating the feasibility of using ECochG as an objective tool for quantifying ABG in CI recipients.

Suppression of Tinnitus in a Patient with Unilateral Sudden Hearing Loss: A Case Report

PubMed Central

Fioretti, Alessandra; Peri, Giorgia; Eibenstein, Alberto

2012-01-01

We describe a case of a 67-year-old woman with severe disabling right-sided tinnitus, mild hyperacusis, and headache. The tinnitus was associated with sudden right hearing loss and vertigo, which occurred about 18 months before. Magnetic resonance imaging (MRI) resulted in normal anatomical structures of the cochlea and of the cranial nerves showing a partial empty sella syndrome with suprasellar cistern hernia. Angio-MR revealed a bilateral contact between the anterior-inferior cerebellar artery (AICA) and the acoustic-facial nerve with a potential neurovascular conflict. Surgery was considered unnecessary after further evaluations. The right ear was successfully treated with a combination device (hearing aid plus sound generator). Shortly after a standard fitting procedure, the patient reported a reduction of tinnitus, hyperacusis, and headache which completely disappeared at the follow-up evaluation after 3, 6, and 12 months. This paper demonstrates that the combination device resulted in a complete tinnitus and hyperacusis suppression in a patient with unilateral sensorineural sudden hearing loss. Our paper further supports the restoration of peripheral sensory input for the treatment of tinnitus associated with hearing loss in selected patients. PMID:23227400

A crossover trial comparing wide dynamic range compression and frequency compression in hearing aids for tinnitus therapy.

PubMed

Hodgson, Shirley-Anne; Herdering, Regina; Singh Shekhawat, Giriraj; Searchfield, Grant D

2017-01-01

It has been suggested that frequency lowering may be a superior tinnitus reducing digital signal processing (DSP) strategy in hearing aids than conventional amplification. A crossover trial was undertaken to determine if frequency compression (FC) was superior to wide dynamic range compression (WDRC) in reducing tinnitus. A 6-8-week crossover trial of two digital signal-processing techniques (WDRC and 2 WDRC with FC) was undertaken in 16 persons with high-frequency sensorineural hearing loss and chronic tinnitus. WDRC resulted in larger improvements in Tinnitus Functional Index and rating scale scores than WDRC with FC. The tinnitus improvements obtained with both processing types appear to be due to reduced hearing handicap and possibly decreased tinnitus audibility. Hearing aids are useful assistive devices in the rehabilitation of tinnitus. FC was very successful in a few individuals but was not superior to WDRC across the sample. It is recommended that WDRC remain as the default first choice tinnitus hearing aid processing strategy for tinnitus. FC should be considered as one of the many other options for selection based on individual hearing needs. Implications of Rehabilitation Hearing aids can significantly reduce the effects of tinnitus after 6-8 weeks of use. Addition of frequency compression digital signal processing does not appear superior to standard amplitude compression alone. Improvements in tinnitus were correlated with reductions in hearing handicap.

Speech perception and quality of life of open-fit hearing aid users

PubMed Central

GARCIA, Tatiana Manfrini; JACOB, Regina Tangerino de Souza; MONDELLI, Maria Fernanda Capoani Garcia

2016-01-01

ABSTRACT Objective To relate the performance of individuals with hearing loss at high frequencies in speech perception with the quality of life before and after the fitting of an open-fit hearing aid (HA). Methods The WHOQOL-BREF had been used before the fitting and 90 days after the use of HA. The Hearing in Noise Test (HINT) had been conducted in two phases: (1) at the time of fitting without an HA (situation A) and with an HA (situation B); (2) with an HA 90 days after fitting (situation C). Study Sample Thirty subjects with sensorineural hearing loss at high frequencies. Results By using an analysis of variance and the Tukey’s test comparing the three HINT situations in quiet and noisy environments, an improvement has been observed after the HA fitting. The results of the WHOQOL-BREF have showed an improvement in the quality of life after the HA fitting (paired t-test). The relationship between speech perception and quality of life before the HA fitting indicated a significant relationship between speech recognition in noisy environments and in the domain of social relations after the HA fitting (Pearson’s correlation coefficient). Conclusions The auditory stimulation has improved speech perception and the quality of life of individuals. PMID:27383708

‘Ecstasy’ Enhances Noise-Induced Hearing Loss

PubMed Central

Church, Michael W.; Zhang, Jinsheng S.; Langford, Megan M.; Perrine, Shane A.

2013-01-01

‘Ecstasy’ or 3,4-methylenedioxy-N-methamphetamine (MDMA) is an amphetamine abused for its euphoric, empathogenic, hallucinatory, and stimulant effects. It is also used to treat certain psychiatric disorders. Common settings for Ecstasy use are nightclubs and “rave” parties where participants consume MDMA and dance to loud music. One concern with the club setting is that exposure to loud sounds can cause permanent sensorineural hearing loss. Another concern is that consumption of MDMA may enhance such hearing loss. Whereas this latter possibility has not been investigated, this study tested the hypothesis that MDMA enhances noise-induced hearing loss (NIHL) by exposing rats to either MDMA, noise trauma, both MDMA and noise, or neither treatment. MDMA was given in a binge pattern of 5 mg/kg per intraperitoneal injections every 2 h for a total of four injections to animals in the two MDMA-treated groups (MDMA-only and Noise+MDMA). Saline injections were given to the animals in the two non-MDMA groups (Control and Noise-only). Following the final injection, noise trauma was induced by a 10 kHz tone at 120 dB SPL for 1 h to animals in the two noise trauma-treated groups (Noise-only and Noise+MDMA). Hearing loss was assessed by the auditory brainstem response (ABR) and cochlear histology. Results showed that MDMA enhanced NIHL compared to Noise-only and that MDMA alone caused no hearing loss. This implies that “clubbers” and “rave-goers” are exacerbating the amount of NIHL when they consume MDMA and listen to loud sounds. In contrast to earlier reports, the present study found that MDMA by itself caused no changes in the click-evoked ABR’s wave latencies or amplitudes. PMID:23711768

Hearing loss screening tool (COBRA score) for newborns in primary care setting

PubMed Central

Poonual, Watcharapol; Navacharoen, Niramon; Kangsanarak, Jaran; Namwongprom, Sirianong

2017-01-01

Purpose To develop and evaluate a simple screening tool to assess hearing loss in newborns. A derived score was compared with the standard clinical practice tool. Methods This cohort study was designed to screen the hearing of newborns using transiently evoked otoacoustic emission and auditory brain stem response, and to determine the risk factors associated with hearing loss of newborns in 3 tertiary hospitals in Northern Thailand. Data were prospectively collected from November 1, 2010 to May 31, 2012. To develop the risk score, clinical-risk indicators were measured by Poisson risk regression. The regression coefficients were transformed into item scores dividing each regression-coefficient with the smallest coefficient in the model, rounding the number to its nearest integer, and adding up to a total score. Results Five clinical risk factors (Craniofacial anomaly, Ototoxicity, Birth weight, family history [Relative] of congenital sensorineural hearing loss, and Apgar score) were included in our COBRA score. The screening tool detected, by area under the receiver operating characteristic curve, more than 80% of existing hearing loss. The positive-likelihood ratio of hearing loss in patients with scores of 4, 6, and 8 were 25.21 (95% confidence interval [CI], 14.69–43.26), 58.52 (95% CI, 36.26–94.44), and 51.56 (95% CI, 33.74–78.82), respectively. This result was similar to the standard tool (The Joint Committee on Infant Hearing) of 26.72 (95% CI, 20.59–34.66). Conclusion A simple screening tool of five predictors provides good prediction indices for newborn hearing loss, which may motivate parents to bring children for further appropriate testing and investigations. PMID:29234358

The Relationship between the Behavioral Hearing Thresholds and Maximum Bilirubin Levels at Birth in Children with a History of Neonatal Hyperbilirubinemia

PubMed Central

Panahi, Rasool; Jafari, Zahra; Sheibanizade, Abdoreza; Salehi, Masoud; Esteghamati, Abdoreza; Hasani, Sara

2013-01-01

Introduction: Neonatal hyperbilirubinemia is one of the most important factors affecting the auditory system and can cause sensorineural hearing loss. This study investigated the relationship between behavioral hearing thresholds in children with a history of jaundice and the maximum level of bilirubin concentration in the blood. Materials and Methods: This study was performed on 18 children with a mean age of 5.6 years and with a history of neonatal hyperbilirubinemia. Behavioral hearing thresholds, transient evoked emissions and brainstem evoked responses were evaluated in all children. Results: Six children (33.3%) had normal hearing thresholds and the remaining (66.7%) had some degree of hearing loss. There was no significant relationship (r=-0.28, P=0.09) between the mean total bilirubin levels and behavioral hearing thresholds in all samples. A transient evoked emission was seen only in children with normal hearing thresholds however in eight cases brainstem evoked responses had not detected. Conclusion: Increased blood levels of bilirubin at the neonatal period were potentially one of the causes of hearing loss. There was a lack of a direct relationship between neonatal bilirubin levels and the average hearing thresholds which emphasizes on the necessity of monitoring the various amounts of bilirubin levels. PMID:24303432

Targeted surveillance for postnatal hearing loss: a program evaluation.

PubMed

Beswick, Rachael; Driscoll, Carlie; Kei, Joseph; Glennon, Shirley

2012-07-01

The importance of monitoring hearing throughout early childhood cannot be understated. However, there is a lack of evidence available regarding the most effective method of monitoring hearing following the newborn screen. The goal of this study was to describe a targeted surveillance program using a risk factor registry to identify children with a postnatal hearing loss. All children who were born in Queensland, Australia between September 2004 and December 2009, received a bilateral 'pass' on newborn hearing screening, and had at least one risk factor, were referred for targeted surveillance and were included in this study. The cohort was assessed throughout early childhood in accordance with Queensland's diagnostic assessment protocols. During the study period, 7320 (2.8% of 261,328) children were referred for targeted surveillance, of which 56 were identified with a postnatal hearing loss (0.77%). Of these, half (50.0%) were identified with a mild hearing loss, and 64.3% were identified with a sensorineural hearing loss. In regards to risk factors, syndrome, craniofacial anomalies, and severe asphyxia had the highest yield of positive cases of postnatal hearing loss for children referred for targeted surveillance, whereas, low birth weight, bacterial meningitis, and professional concern had a particularly low yield. Limitations of the targeted surveillance program were noted and include: (1) a lost contact rate of 32.4%; (2) delays in first surveillance assessment; (3) a large number of children who required on-going monitoring; and (4) extensive diagnostic assessments were completed on children with normal hearing. Examination of the lost contact rate revealed indigenous children were more likely to be documented as lost contact. In addition, children with one risk factor only were significantly more likely to not attend a surveillance appointment. Positive cases of postnatal hearing loss were detected through the targeted surveillance program. However, the

[Natural history of occupational hearing loss induced by noise].

PubMed

de Almeida, S I; Albernaz, P L; Zaia, P A; Xavier, O G; Karazawa, E H

2000-01-01

To evaluate the clinical and audiometric characteristics of occupational hearing loss induced by noise, according to age and time of exposition in years. 222 patients with occupational sensorineural hearing loss induced by noise were studied retrospectively, correlating the auditive clinical claims, alterations of audiometric thresholds at frequencies of 250 Hz to 8000 Hz, speech discrimination indicator with age and time of exposure. As a control group were used the audiometric threshold of a population of same medium age, without morbid antecedents of hearing illness, as preconized by ISO 1999 (1990). The group were divided into subgroups and three decades of exposure were analyzed. It was verified that the clinical claims of hipoacusia increases according to the age and time of exposure. The frequency of tinnitus is constant. The audiometric thresholds in the second decade of exposure present variations that depend on the age. The several audiometric curves are parallel, but they are not horizontal. The worst thresholds were found in the high frequencies from 3000 Hz to 8000 Hz, as a clinical and physiopathological consequences of the commitment of basal areas of cochlea. The speech discrimination showed to be worst according to the increase of age and time of exposure. Patients with hearing loss disacusia induced by occupational noise present characteristic audiometric thresholds that vary according to age and time of exposure to noise. These characteristics defined and resumed in audiometric curves can constitute a standard of comparison, evaluation and control for exposed populations.

Reprogramming Glia Into Neurons in the Peripheral Auditory System as a Solution for Sensorineural Hearing Loss: Lessons From the Central Nervous System

PubMed Central

Meas, Steven J.; Zhang, Chun-Li; Dabdoub, Alain

2018-01-01

Disabling hearing loss affects over 5% of the world’s population and impacts the lives of individuals from all age groups. Within the next three decades, the worldwide incidence of hearing impairment is expected to double. Since a leading cause of hearing loss is the degeneration of primary auditory neurons (PANs), the sensory neurons of the auditory system that receive input from mechanosensory hair cells in the cochlea, it may be possible to restore hearing by regenerating PANs. A direct reprogramming approach can be used to convert the resident spiral ganglion glial cells into induced neurons to restore hearing. This review summarizes recent advances in reprogramming glia in the CNS to suggest future steps for regenerating the peripheral auditory system. In the coming years, direct reprogramming of spiral ganglion glial cells has the potential to become one of the leading biological strategies to treat hearing impairment. PMID:29593497

The benefits of hearing aids and closed captioning for television viewing by older adults with hearing loss.

PubMed

Gordon-Salant, Sandra; Callahan, Julia S

2009-08-01

Although watching television is a common leisure activity of older adults, the ability to understand televised speech may be compromised by age-related hearing loss. Two potential assistive devices for improving television viewing are hearing aids (HAs) and closed captioning (CC), but their use and benefit by older adults with hearing loss are unknown. The primary purpose of this initial investigation was to determine if older hearing-impaired adults show improvements in understanding televised speech with the use of these two assistive devices (HAs and CC) compared with conditions without these devices. A secondary purpose was to examine the frequency of HA and CC use among a sample of older HA wearers. The investigation entailed a randomized, repeated-measures design of 15 older adults (59 to 82 yr) with bilateral sensorineural hearing losses who wore HAs. Participants viewed three types of televised programs (news, drama, and game show) that were each edited into lists of speech segments and provided an identification response. Each participant was tested in four conditions: baseline (no HA or CC), HA only, CC only, and HA + CC. Also, pilot testing with young normal-hearing listeners was conducted to establish list equivalence and stimulus intelligibility with a control group. All testing was conducted in a quiet room to simulate a living room, using a 20 in flat screen television. Questionnaires were also administered to participants to determine the frequency of HA and CC use while watching television. A significant effect of viewing condition was observed for all programs. Participants exhibited significantly better speech recognition scores in conditions with CC than those without CC (p < 0.01). Use of personal HAs did not significantly improve recognition of televised speech compared with the unaided condition. The condition effect was similar across the three different programs. Most of the participants (73%) regularly wore their HAs while watching

Prospective study of the effects of ventilation tubes on hearing after radiotherapy for carcinoma of nasopharynx

DOE Office of Scientific and Technical Information (OSTI.GOV)

Chowdhury, C.R.; Ho, J.H.; Wright, A.

1988-03-01

In patients with nasopharyngeal carcinoma, deafness sometimes occurs following radiotherapy. It is usually conductive, but may be sensorineural. Tinnitus is present frequently and usually is distressing. The role of ventilation tubes (grommets) in relieving these problems was assessed in a prospective randomized controlled trial of 115 patients. In the group with grommets, there was an improvement in hearing, with a reduction of the averaged air-bone gap (p less than .01). This was not found in the control group without ventilation tubes, who developed a larger conductive loss (p less than .01) and, in addition, a slight deterioration of the averagedmore » bone conduction threshold (p less than .01). Surprisingly, the group with grommets did not develop this sensorineural loss (p less than .01). In addition, tinnitus was improved significantly by the insertion of ventilation tubes (p less than .01). Neither necrosis nor stenosis of the external auditory meatus was found in either group.« less

Prospective study of the effects of ventilation tubes on hearing after radiotherapy for carcinoma of nasopharynx

DOE Office of Scientific and Technical Information (OSTI.GOV)

Chowdhury, C.R.; Ho, J.H.; Wright, A.

In patients with nasopharyngeal carcinoma, deafness sometimes occurs following radiotherapy. It is usually conductive, but may be sensorineural. Tinnitus is present frequently and usually is distressing. The role of ventilation tubes (grommets) in relieving these problems was assessed in a prospective randomized controlled trial of 115 patients. In the group with grommets, there was an improvement in hearing, with a reduction of the averaged air-bone gap (p less than .01). This was not found in the control group without ventilation tubes, who developed a larger conductive loss (p less than .01) and, in addition, a slight deterioration of the averagedmore » bone conduction threshold (p less than .01). Surprisingly, the group with grommets did not develop this sensorineural loss (p less than .01). In addition, tinnitus was improved significantly by the insertion of ventilation tubes (p less than .01). Neither necrosis nor stenosis of the external auditory meatus was found in either group.« less

[Hearing aids at the International Center of Auditory rehabilitation in Abidjan: Prosthetics gains and satisfaction in patients].

PubMed

Mobio, M N A; Ilé, S; Yavo, N; Koffi-Aka, V; Kouassi-Ndjeundo, J; Tea, B

To evaluate patients wearing hearing aid at the International Center of Auditory Correction in Abidjan. It is a descriptif and transversal study from 07/01/99 to 06/30/10. We have included the files of patients completely filled. We have stu­died the indications, prosthetics gains and the satisfaction after hearing aid. We have achieved 536 files. The ave­rage was 36 years. The indications have been in 76.1% cases of sensorineural hearing loss. The hearing loss has been associa­ted in 13.2% cases to language disorder. For all patient we have noticed bilateral hearing loss in 496 cases (92.5%). The behind the ear aids have been chosen in 69% cases. The type was analogical or digital respectively in 65% and 35% des cas. The prosthetic pure tonal gain was more than 30 dB in 66.8% cases and the prosthetic speech reception threshold gain more than 30 dB in 55.3% cases. The patients have been respectively satisfied less satisfacted, no satisfacted in 68.47%, 22.76% and 8.7% cases. The hearing aids have improved the audition in most of the indications. The proportion of patients satisfied was proportionally equivalent to the audiometric results.

Consumer preferences for hearing aid attributes: a comparison of rating and conjoint analysis methods.

PubMed

Bridges, John F P; Lataille, Angela T; Buttorff, Christine; White, Sharon; Niparko, John K

2012-03-01

Low utilization of hearing aids has drawn increased attention to the study of consumer preferences using both simple ratings (e.g., Likert scale) and conjoint analyses, but these two approaches often produce inconsistent results. The study aims to directly compare Likert scales and conjoint analysis in identifying important attributes associated with hearing aids among those with hearing loss. Seven attributes of hearing aids were identified through qualitative research: performance in quiet settings, comfort, feedback, frequency of battery replacement, purchase price, water and sweat resistance, and performance in noisy settings. The preferences of 75 outpatients with hearing loss were measured with both a 5-point Likert scale and with 8 paired-comparison conjoint tasks (the latter being analyzed using OLS [ordinary least squares] and logistic regression). Results were compared by examining implied willingness-to-pay and Pearson's Rho. A total of 56 respondents (75%) provided complete responses. Two thirds of respondents were male, most had sensorineural hearing loss, and most were older than 50; 44% of respondents had never used a hearing aid. Both methods identified improved performance in noisy settings as the most valued attribute. Respondents were twice as likely to buy a hearing aid with better functionality in noisy environments (p < .001), and willingness to pay for this attribute ranged from US$2674 on the Likert to US$9000 in the conjoint analysis. The authors find a high level of concordance between the methods-a result that is in stark contrast with previous research. The authors conclude that their result stems from constraining the levels on the Likert scale.

[Norrie-Wardburg syndrome].

PubMed

Skevas, A; Kastanioudakis, I; Daniilidis, B; Exarchakos, G

1992-10-01

We describe a case of a 25-year old patient with typical Norrie-Warburg Syndrome. From the first year of his life he was found to be blind, with bilateral sensorineural loss of hearing. Audiological examination showed symmetrical moderate bilateral sensorineural hearing loss. His hearing loss was refractory to treatment for the last eight years. Because of timely diagnosis of hearing loss and timely fitting of a hearing aid, the patient could study at school and graduate from university education. Disease carriers who are clinically healthy can be identified only via chromosome analysis.

Idiopathic anaphylaxis.

PubMed

Fenny, Nana; Grammer, Leslie C

2015-05-01

Idiopathic anaphylaxis is a diagnosis of exclusion after other causes have been thoroughly evaluated and excluded. The pathogenesis of idiopathic anaphylaxis remains uncertain, although increased numbers of activated lymphocytes and circulating histamine-releasing factors have been implicated. Signs and symptoms of patients diagnosed with idiopathic anaphylaxis are indistinguishable from the manifestations of other forms of anaphylaxis. Treatment regimens are implemented based on the frequency and severity of patient symptoms and generally include the use of epinephrine autoinjectors, antihistamines, and steroids. The prognosis of idiopathic anaphylaxis is generally favorable with well-established treatment regimens and effective patient education. Copyright © 2015 Elsevier Inc. All rights reserved.

Rapid word-learning in normal-hearing and hearing-impaired children: effects of age, receptive vocabulary, and high-frequency amplification.

PubMed

Pittman, A L; Lewis, D E; Hoover, B M; Stelmachowicz, P G

2005-12-01

This study examined rapid word-learning in 5- to 14-year-old children with normal and impaired hearing. The effects of age and receptive vocabulary were examined as well as those of high-frequency amplification. Novel words were low-pass filtered at 4 kHz (typical of current amplification devices) and at 9 kHz. It was hypothesized that (1) the children with normal hearing would learn more words than the children with hearing loss, (2) word-learning would increase with age and receptive vocabulary for both groups, and (3) both groups would benefit from a broader frequency bandwidth. Sixty children with normal hearing and 37 children with moderate sensorineural hearing losses participated in this study. Each child viewed a 4-minute animated slideshow containing 8 nonsense words created using the 24 English consonant phonemes (3 consonants per word). Each word was repeated 3 times. Half of the 8 words were low-pass filtered at 4 kHz and half were filtered at 9 kHz. After viewing the story twice, each child was asked to identify the words from among pictures in the slide show. Before testing, a measure of current receptive vocabulary was obtained using the Peabody Picture Vocabulary Test (PPVT-III). The PPVT-III scores of the hearing-impaired children were consistently poorer than those of the normal-hearing children across the age range tested. A similar pattern of results was observed for word-learning in that the performance of the hearing-impaired children was significantly poorer than that of the normal-hearing children. Further analysis of the PPVT and word-learning scores suggested that although word-learning was reduced in the hearing-impaired children, their performance was consistent with their receptive vocabularies. Additionally, no correlation was found between overall performance and the age of identification, age of amplification, or years of amplification in the children with hearing loss. Results also revealed a small increase in performance for both

Rotatory and collic vestibular evoked myogenic potential testing in normal-hearing and hearing-impaired children.

PubMed

Maes, Leen; De Kegel, Alexandra; Van Waelvelde, Hilde; Dhooge, Ingeborg

2014-01-01

Vertigo and imbalance are often underestimated in the pediatric population, due to limited communication abilities, atypical symptoms, and relatively quick adaptation and compensation in children. Moreover, examination and interpretation of vestibular tests are very challenging, because of difficulties with cooperation and maintenance of alertness, and because of the sometimes nauseatic reactions. Therefore, it is of great importance for each vestibular laboratory to implement a child-friendly test protocol with age-appropriate normative data. Because of the often masked appearance of vestibular problems in young children, the vestibular organ should be routinely examined in high-risk pediatric groups, such as children with a hearing impairment. Purposes of the present study were (1) to determine age-appropriate normative data for two child-friendly vestibular laboratory techniques (rotatory and collic vestibular evoked myogenic potential [cVEMP] test) in a group of children without auditory or vestibular complaints, and (2) to examine vestibular function in a group of children presenting with bilateral hearing impairment. Forty-eight typically developing children (mean age 8 years 0 months; range: 4 years 1 month to 12 years 11 months) without any auditory or vestibular complaints as well as 39 children (mean age 7 years 8 months; range: 3 years 8 months to 12 years 10 months) with a bilateral sensorineural hearing loss were included in this study. All children underwent three sinusoidal rotations (0.01, 0.05, and 0.1 Hz at 50 degrees/s) and bilateral cVEMP testing. No significant age differences were found for the rotatory test, whereas a significant increase of N1 latency and a significant threshold decrease was noticeable for the cVEMP, resulting in age-appropriate normative data. Hearing-impaired children demonstrated significantly lower gain values at the 0.01 Hz rotation and a larger percentage of absent cVEMP responses compared with normal-hearing children

JS-X syndrome: A multiple congenital malformation with vocal cord paralysis, ear deformity, hearing loss, shoulder musculature underdevelopment, and X-linked recessive inheritance.

PubMed

Hoeve, Hans L J; Brooks, Alice S; Smit, Liesbeth S

2015-07-01

We report on a family with a not earlier described multiple congenital malformation. Several male family members suffer from laryngeal obstruction caused by bilateral vocal cord paralysis, outer and middle ear deformity with conductive and sensorineural hearing loss, facial dysmorphisms, and underdeveloped shoulder musculature. The affected female members only have middle ear deformity and hearing loss. The pedigree is suggestive of an X-linked recessive inheritance pattern. SNP-array revealed a deletion and duplication on Xq28 in the affected family members. A possible aetiology is a neurocristopathy with most symptoms expressed in structures derived from branchial arches. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

In-flight speech intelligibility evaluation of a service member with sensorineural hearing loss: case report.

PubMed

Casto, Kristen L; Cho, Timothy H

2012-09-01

This case report describes the in-flight speech intelligibility evaluation of an aircraft crewmember with pure tone audiometric thresholds that exceed the U.S. Army's flight standards. Results of in-flight speech intelligibility testing highlight the inability to predict functional auditory abilities from pure tone audiometry and underscore the importance of conducting validated functional hearing evaluations to determine aviation fitness-for-duty.

Ear health and hearing surveillance in girls and women with Turner's syndrome: recommendations from the Turner's Syndrome Support Society.

PubMed

Kubba, H; Smyth, A; Wong, S C; Mason, A

2017-06-01

Turner's syndrome (TS) is a common chromosomal disorder, affecting one in 2000 newborn girls, in which part or all of one X chromosome is missing. Ear and hearing problems are very common in girls and women with TS. The aim of this review was to review the published literature to suggest recommendations for otological health surveillance. A keyword search of Ovid Medline was performed for published literature on the subject and evidence rated according to the GRADE criteria. Middle ear disorders are very common and persistent in girls and women with TS as are progressive sensorineural hearing loss and balance disorders. Otolaryngologists should be aware of the high prevalence and challenging nature of all forms of ear disease in individuals with TS. Early intervention may offer benefits to health and education, and we advocate routine lifelong annual hearing screening in this group. © 2016 John Wiley & Sons Ltd.

Hearing status in adult individuals with lifetime, untreated isolated growth hormone deficiency.

PubMed

Prado-Barreto, Valéria M; Salvatori, Roberto; Santos Júnior, Ronaldo C; Brandão-Martins, Mariane B; Correa, Eric A; Garcez, Flávia B; Valença, Eugênia H O; Souza, Anita H O; Pereira, Rossana M C; Nunes, Marco A P; D'Avila, Jeferson S; Aguiar-Oliveira, Manuel H

2014-03-01

To evaluate the hearing status of growth hormone (GH)-naive adults with isolated GH deficiency (IGHD) belonging to an extended Brazilian kindred with a homozygous mutation in the GH-releasing hormone receptor gene. Cross-sectional. Divisions of Endocrinology and Otorhinolaryngology of the Federal University of Sergipe. Twenty-six individuals with IGHD (age, 47.6 ± 15.1 years; 13 women) and 25 controls (age, 46.3 ± 14.3 years; 15 women) were administered a questionnaire on hearing complaints and hearing health history. We performed pure-tone audiometry, logoaudiometry, electroacoustic immittance, and stapedial reflex. To assess outer hair cell function in the cochlea, we completed transient evoked otoacoustic emissions (TEOAEs). To assess the auditory nerve and auditory brainstem, we obtained auditory brainstem responses (ABRs). Misophonia and dizziness complaints were more frequent in those with IGHD than in controls (P = .011). Patients with IGHD had higher thresholds at 250 Hz (P = .005), 500 Hz (P = .006), 3 KHz (P = .008), 4 KHz (P = .038), 6 KHz (P = .008), and 8 KHz (P = .048) and mild high-tones hearing loss (P = .029). Stapedial reflex (P < .001) and TEOAEs (P = .025) were more frequent in controls. There were no differences in ABR latencies. Hearing loss in patients with IGHD occurred earlier than in controls (P < .001). Compared with controls of the same area, subjects with untreated, congenital lifetime IGHD report more misophonia and dizziness, have predominance of mild high-tones sensorineural hearing loss, and have an absence of stapedial reflex and TEOAEs.

Hearing results in otosclerosis surgery after partial stapedectomy, total stapedectomy and stapedotomy.

PubMed

Persson, P; Harder, H; Magnuson, B

1997-01-01

Hearing results in a consecutive series of 407 patients with otosclerosis undergoing primary stapes surgery were analysed (437 operated ears). Partial stapedectomy was performed in 70 ears (16%), total stapedectomy in 205 ears (47%), in both groups using the House steel wire prosthesis on fascia in the oval window. The remaining 162 ears (37%) underwent stapedotomy using the Fisch 0.4 mm teflon-platinum piston. No case of cochlear loss (> 15 dB) occurred in the total series. The comparison between the three groups one year postoperatively showed that the air-bone gap was smaller for partial and total stapedectomy for all frequencies except 4 kHz. The air-bone gap was calculated as the difference between the preoperative bone conduction and the postoperative air conduction thresholds. Partial and total stapedectomy also showed larger improvements of bone conduction thresholds compared with stapedotomy for all frequencies but 4 kHz. At the 3-year follow-up, the hearing gain for all frequencies (250-8000 Hz) was larger for partial and total stapedectomy. Yet, when comparing the decline of hearing from 1 to 3 year postoperatively, the hearing gain achieved with partial and total stapedectomy seemed to deteriorate at a higher rate, which was considered to be caused by impaired sensorineural function. Our results show that in the short-term perspective partial or total stapedectomy can still compete for better hearing results even at higher frequencies, but stapedotomy seems to yield more stable hearing results over time and should therefore be considered as the method of choice.

Localization of A Novel Autosomal Recessive Non-Syndromic Hearing Impairment Locus (DFNB38) to 6q26–q27 in a Consanguineous Kindred from Pakistan

PubMed Central

Ansar, Muhammad; Ramzan, Mohammad; Pham, Thanh L.; Yan, Kai; Jamal, Syed Muhammad; Haque, Sayedul; Ahmad, Wasim; Leal, Suzanne M.

2010-01-01

For autosomal recessive nonsyndromic hearing impairment over 30 loci have been mapped and 19 genes have been identified. DFNB38, a novel locus for autosomal recessive nonsyndromic hearing impairment, was localized in a consanguineous Pakistani kindred to 6q26–q27. The affected family members present with profound prelingual sensorineural hearing impairment and use sign language for communications. Linkage was established to microsatellite markers located on chromosome 6q26–q27 (Multipoint lod score 3.6). The genetic region for DFNB38 spans 10.1 cM according to the Marshfield genetic map and is bounded by markers D6S980 and D6S1719. This genetic region corresponds to 3.4 MB on the sequence-based physical map. PMID:12890929

Rubella in Sub-Saharan Africa and sensorineural hearing loss: a case control study.

PubMed

Caroça, Cristina; Vicente, Vera; Campelo, Paula; Chasqueira, Maria; Caria, Helena; Silva, Susana; Paixão, Paulo; Paço, João

2017-02-01

Rubella infection can affect several organs and cause birth defects that are responsible for congenital rubella syndrome (CRS). Congenital hearing loss is the most common symptom of this syndrome, occurring in approximately 60% of CRS cases. Worldwide, over 100 000 babies are born with CRS every year. There is no specific treatment for rubella, but the disease is preventable by vaccination. Since 1969, the rubella vaccine has been implemented in many countries, but in Africa, only a few countries routinely immunize against rubella. The aim of this study was to estimate the rate of infection from the wild-type rubella virus in São Tomé and Príncipe by determining rubella seroprevalence with a DBS method. The goal of this study was to reinforce the need for implementation of the rubella vaccine in this country. As secondary objectives, the validation of a DBS method was first attempted and an association between seroprevalence and hearing loss was assessed. We collected samples from individuals observed during humanitarian missions in São Tomé and Príncipe. All individuals underwent an audiometric evaluation, and a drop of blood was collected for the dried blood spot (DBS). We define two groups: the case group (individuals with unilateral or bilateral hearing loss (HL)) and the control group (individuals with two normal ears). Patients were excluded if they suffered from conductive HL, if they showed evidence of possible causes of HL, if they had developmental delay or if they refused to participate in the study. Among the 315 subjects, we found 64.1% individuals with IgG for the rubella virus, 32.1% without immunity for the rubella virus and 3.8% who were borderline. In the control group, 62.6% were positive for the rubella IgG, whereas in the case group, 72% were positive. Analyzing both groups, with ages ranging from 2 to 14 years of age and from 15 to 35 years of age, we found a seroprevalence of 50.3% to rubella in the younger group and 82.1% in the

Bisphosphonate-Linked TrkB Agonist: Cochlea-Targeted Delivery of a Neurotrophic Agent as a Strategy for the Treatment of Hearing Loss.

PubMed

Kempfle, Judith S; Nguyen, Kim; Hamadani, Christine; Koen, Nicholas; Edge, Albert S; Kashemirov, Boris A; Jung, David H; McKenna, Charles E

2018-04-18

Hearing loss affects more than two-thirds of the elderly population, and more than 17% of all adults in the U.S. Sensorineural hearing loss related to noise exposure or aging is associated with loss of inner ear sensory hair cells (HCs), cochlear spiral ganglion neurons (SGNs), and ribbon synapses between HCs and SGNs, stimulating intense interest in therapies to regenerate synaptic function. 7,8-Dihydroxyflavone (DHF) is a selective and potent agonist of tropomyosin receptor kinase B (TrkB) and protects the neuron from apoptosis. Despite evidence that TrkB agonists can promote survival of SGNs, local delivery of drugs such as DHF to the inner ear remains a challenge. We previously demonstrated in an animal model that a fluorescently labeled bisphosphonate, 6-FAM-Zol, administered to the round window membrane penetrated the membrane and diffused throughout the cochlea. Given their affinity for bone mineral, including cochlear bone, bisphosphonates offer an intriguing modality for targeted delivery of neurotrophic agents to the SGNs to promote survival, neurite outgrowth, and, potentially, regeneration of synapses between HCs and SGNs. The design and synthesis of a bisphosphonate conjugate of DHF (Ris-DHF) is presented, with a preliminary evaluation of its neurotrophic activity. Ris-DHF increases neurite outgrowth in vitro, maintains this ability after binding to hydroxyapatite, and regenerates synapses in kainic acid-damaged cochlear organ of Corti explants dissected in vitro with attached SGNs. The results suggest that bisphosphonate-TrkB agonist conjugates have promise as a novel approach to targeted delivery of drugs to treat sensorineural hearing loss.

Exome Sequencing Identifies a Founder Frameshift Mutation in an Alternative Exon of USH1C as the Cause of Autosomal Recessive Retinitis Pigmentosa with Late-Onset Hearing Loss

PubMed Central

Khateb, Samer; Zelinger, Lina; Ben-Yosef, Tamar; Crystal-Shalit, Ornit; Gross, Menachem; Banin, Eyal; Sharon, Dror

2012-01-01

We used a combined approach of homozygosity mapping and whole exome sequencing (WES) to search for the genetic cause of autosomal recessive retinitis pigmentosa (arRP) in families of Yemenite Jewish origin. Homozygosity mapping of two arRP Yemenite Jewish families revealed a few homozygous regions. A subsequent WES analysis of the two index cases revealed a shared homozygous novel nucleotide deletion (c.1220delG) leading to a frameshift (p.Gly407Glufs*56) in an alternative exon (#15) of USH1C. Screening of additional Yemenite Jewish patients revealed a total of 16 homozygous RP patients (with a carrier frequency of 0.008 in controls). Funduscopic and electroretinography findings were within the spectrum of typical RP. While other USH1C mutations usually cause Usher type I (including RP, vestibular dysfunction and congenital deafness), audiometric screening of 10 patients who are homozygous for c.1220delG revealed that patients under 40 years of age had normal hearing while older patients showed mild to severe high tone sensorineural hearing loss. This is the first report of a mutation in a known USH1 gene that causes late onset rather than congenital sensorineural hearing loss. The c.1220delG mutation of USH1C accounts for 23% of RP among Yemenite Jewish patients in our cohort. PMID:23251578

[Effects on hearing due to the occupational noise exposure of marble industry workers in the Federal District, Brazil].

PubMed

Harger, Marília Rabelo Holanda Camarano; Barbosa-Branco, Anadergh

2004-01-01

To evaluate the prevalence of hearing loss, its degree and type, among workers in the marble industry in the Brazilian Federal District (FD). Workers from eight marble industries in the FD were evaluated by means of a cross sectional epidemiological study. An audiometry screening test (air conduction) was performed. Workers with hearing loss were submitted to liminal tonal audiometry air & bone conduction and speech audiometry tests using an audiometer AD-28 (Interacoustics). All subjects studied were submitted to a visual inspection of the external acoustic meatus. One hundred and fifty two workers were examined; mean age was 32 years (SD = 8.6); average occupational noise exposure was of 8.3 years (SD = 6.8). Audiometries demonstrated that 48.0% (n = 73) had some type of hearing loss. Among the workers with hearing loss, 50.0% had results compatible with noise-induced hearing loss (NIHL); 41.0% with incipient noise induced hearing loss, 5.0% with sensorineural hearing loss (all except NIHL) and 4.0% with conductive and mixed hearing losses. Among workers with NIHL, 57.1% had bilateral involvement, 17.1% in the right ear and 25.7% in the left ear. Among those with incipient NIHL, 13.9% were bilateral, 19.4% were only in the right ear and 66.7% were only in the left ear. Abnormal audiograms were found in 48.0% of the sample. Among those with hearing loss, the predominant cause was NIHL, followed by those classified as having incipient noise induced hearing loss. Hearing loss usually started at 6 kHz, frequently in the left ear.

Encoding of speech sounds at auditory brainstem level in good and poor hearing aid performers.

PubMed

Shetty, Hemanth Narayan; Puttabasappa, Manjula

Hearing aids are prescribed to alleviate loss of audibility. It has been reported that about 31% of hearing aid users reject their own hearing aid because of annoyance towards background noise. The reason for dissatisfaction can be located anywhere from the hearing aid microphone till the integrity of neurons along the auditory pathway. To measure spectra from the output of hearing aid at the ear canal level and frequency following response recorded at the auditory brainstem from individuals with hearing impairment. A total of sixty participants having moderate sensorineural hearing impairment with age range from 15 to 65 years were involved. Each participant was classified as either Good or Poor Hearing aid Performers based on acceptable noise level measure. Stimuli /da/ and /si/ were presented through loudspeaker at 65dB SPL. At the ear canal, the spectra were measured in the unaided and aided conditions. At auditory brainstem, frequency following response were recorded to the same stimuli from the participants. Spectrum measured in each condition at ear canal was same in good hearing aid performers and poor hearing aid performers. At brainstem level, better F 0 encoding; F 0 and F 1 energies were significantly higher in good hearing aid performers than in poor hearing aid performers. Though the hearing aid spectra were almost same between good hearing aid performers and poor hearing aid performers, subtle physiological variations exist at the auditory brainstem. The result of the present study suggests that neural encoding of speech sound at the brainstem level might be mediated distinctly in good hearing aid performers from that of poor hearing aid performers. Thus, it can be inferred that subtle physiological changes are evident at the auditory brainstem in a person who is willing to accept noise from those who are not willing to accept noise. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier

Effects of noise and working memory capacity on memory processing of speech for hearing-aid users.

PubMed

Ng, Elaine Hoi Ning; Rudner, Mary; Lunner, Thomas; Pedersen, Michael Syskind; Rönnberg, Jerker

2013-07-01

It has been shown that noise reduction algorithms can reduce the negative effects of noise on memory processing in persons with normal hearing. The objective of the present study was to investigate whether a similar effect can be obtained for persons with hearing impairment and whether such an effect is dependent on individual differences in working memory capacity. A sentence-final word identification and recall (SWIR) test was conducted in two noise backgrounds with and without noise reduction as well as in quiet. Working memory capacity was measured using a reading span (RS) test. Twenty-six experienced hearing-aid users with moderate to moderately severe sensorineural hearing loss. Noise impaired recall performance. Competing speech disrupted memory performance more than speech-shaped noise. For late list items the disruptive effect of the competing speech background was virtually cancelled out by noise reduction for persons with high working memory capacity. Noise reduction can reduce the adverse effect of noise on memory for speech for persons with good working memory capacity. We argue that the mechanism behind this is faster word identification that enhances encoding into working memory.

Newly recognized recessive syndrome characterized by dysmorphic features, hypogonadotropic hypogonadism, severe microcephaly, and sensorineural hearing loss maps to 3p21.3.

PubMed

Jenkinson, Emma M; Kingston, Helen; Urquhart, Jill; Khan, Naz; Melville, Athalie; Swinton, Martin; Crow, Yanick J; Davis, Julian R E; Trump, Dorothy; Newman, William G

2011-12-01

We present a newly recognized, likely autosomal recessive, pleiotropic disorder seen in four individuals (three siblings and their nephew) from a consanguineous family of Pakistani origin. The condition is characterized by hypogonadotropic hypogonadism, severe microcephaly, sensorineural deafness, moderate learning disability, and distinctive facial dysmorphic features. Autozygosity mapping using SNP array genotyping defined a single, large autozygous region of 13.1 Mb on chromosome 3p21 common to the affected individuals. The critical region contains 227 genes and initial sequence analysis of a functional candidate gene has not identified causative mutations. Copyright © 2011 Wiley Periodicals, Inc.

Sleep apnea, daytime somnolence, and idiopathic dizziness--a novel association.

PubMed

Sowerby, Leigh J; Rotenberg, Brian; Brine, Meggan; George, Charles F P; Parnes, Lorne S

2010-06-01

To determine if an association exists between sleep apnea, daytime somnolence, and chronic idiopathic dizziness. Case-control study of new patients presenting to a tertiary neuro-otologic practice. A total of 46 subjects with idiopathic dizziness (ID), 20 positive controls with dizziness (benign paroxysmal positional vertigo [BPV]), and 69 negative controls with hearing loss (HL) but no dizziness were enrolled. Participants who were patients diagnosed with the above conditions and who met all other inclusion criteria completed a sleep questionnaire and had a complete physical exam and investigations to establish or exclude a neuro-otologic diagnosis. They were subsequently evaluated for risk of symptomatic sleep disturbance based on the Epworth Sleepiness Scale (ESS), the Berlin Questionnaire, and the Multivariable Apnea Risk Index (MAP). Statistical analysis was carried out using SPSS (SPSS Inc., Chicago, IL). There was no significant demographic difference among the groups in terms of age, sex, body mass index, neck size, alcohol consumption, or smoking. Using a cutoff of both 10 and 12 on the ESS, the ID were more likely to have significant daytime somnolence than the HL group, with a likelihood ratio (LR) of 7.8 for the ESS 12 score (P = .021) and 7.1 for the ESS 10 score (P = .029). Using the MAP score, a statistically significant difference between the ID group and both the BPV group (LR 3.99, P = .046) and the HL group (LR 5.46, P = .019) was found. This study suggests that a previously undescribed link between idiopathic dizziness, daytime somnolence, and sleep apnea might exist. Prospective investigation is warranted to determine whether treatment of any sleep issues resolves symptoms of idiopathic dizziness.

B3GALNT2-Related Dystroglycanopathy: Expansion of the Phenotype with Novel Mutation Associated with Muscle-Eye-Brain Disease, Walker-Warburg Syndrome, Epileptic Encephalopathy-West Syndrome, and Sensorineural Hearing Loss.

PubMed

Al Dhaibani, Muna A; El-Hattab, Ayman W; Ismayl, Omar; Suleiman, Jehan

2018-05-23

Mutations in B3GALNT2 , encoding a glycosyltransferase enzyme involved in α-dystroglycan glycosylation, have been recently associated with dystroglycanopathy, a well-recognized subtype of congenital muscular dystrophy (CMD). Only a few cases have been reported with B3GALNT2 -related dystroglycanopathy with variable severity ranging from mild CMD to severe muscle-eye-brain disease. Here, we describe a child with a novel homozygous nonsense mutation in B3GALNT2 . The affected child has severe neurological disease since birth, including muscle disease manifested as hypotonia, muscle weakness, and wasting with elevated creatine kinase, eye disease including microphthalmia and blindness, brain disease with extensive brain malformations including massive hydrocephalus, diffuse cobblestone-lissencephaly, deformed craniocervical junction, and pontocerebellar hypoplasia. The clinical and radiologic findings are compatible with a diagnosis of severe muscle-eye-brain disease and more specifically Walker-Warburg syndrome. A more distinct aspect of the clinical phenotype in this child is the presence of refractory epilepsy in the form of epileptic spasms, epileptic encephalopathy, and West syndrome, as well as sensorineural hearing loss. These findings could expand the phenotype of B3GALNT2 -related dystroglycanopathy. In this report, we also provide a detailed review of previously reported cases with B3GALNT2 -related dystroglycanopathy and compare them to our reported child. In addition, we study the genotype-phenotype correlation in these cases. Georg Thieme Verlag KG Stuttgart · New York.

Cochlear implantation in adults with asymmetric hearing loss.

PubMed

Firszt, Jill B; Holden, Laura K; Reeder, Ruth M; Cowdrey, Lisa; King, Sarah

2012-01-01

Bilateral severe to profound sensorineural hearing loss is a standard criterion for cochlear implantation. Increasingly, patients are implanted in one ear and continue to use a hearing aid in the nonimplanted ear to improve abilities such as sound localization and speech understanding in noise. Patients with severe to profound hearing loss in one ear and a more moderate hearing loss in the other ear (i.e., asymmetric hearing) are not typically considered candidates for cochlear implantation. Amplification in the poorer ear is often unsuccessful because of limited benefit, restricting the patient to unilateral listening from the better ear alone. The purpose of this study was to determine whether patients with asymmetric hearing loss could benefit from cochlear implantation in the poorer ear with continued use of a hearing aid in the better ear. Ten adults with asymmetric hearing between ears participated. In the poorer ear, all participants met cochlear implant candidacy guidelines; seven had postlingual onset, and three had pre/perilingual onset of severe to profound hearing loss. All had open-set speech recognition in the better-hearing ear. Assessment measures included word and sentence recognition in quiet, sentence recognition in fixed noise (four-talker babble) and in diffuse restaurant noise using an adaptive procedure, localization of word stimuli, and a hearing handicap scale. Participants were evaluated preimplant with hearing aids and postimplant with the implant alone, the hearing aid alone in the better ear, and bimodally (the implant and hearing aid in combination). Postlingual participants were evaluated at 6 mo postimplant, and pre/perilingual participants were evaluated at 6 and 12 mo postimplant. Data analysis compared the following results: (1) the poorer-hearing ear preimplant (with hearing aid) and postimplant (with cochlear implant); (2) the device(s) used for everyday listening pre- and postimplant; and (3) the hearing aid-alone and bimodal

Cochlear Implantation in Adults with Asymmetric Hearing Loss

PubMed Central

Firszt, Jill B.; Holden, Laura K.; Reeder, Ruth M.; Cowdrey, Lisa; King, Sarah

2012-01-01

Objective Bilateral severe-to-profound sensorineural hearing loss is a standard criterion for cochlear implantation. Increasingly, patients are implanted in one ear and continue to use a hearing aid in the non-implanted ear to improve abilities such as sound localization and speech understanding in noise. Patients with severe-to-profound hearing loss in one ear and a more moderate hearing loss in the other ear (i.e., asymmetric hearing) are not typically considered candidates for cochlear implantation. Amplification in the poorer ear is often unsuccessful due to limited benefit, restricting the patient to unilateral listening from the better ear alone. The purpose of this study was to determine if patients with asymmetric hearing loss could benefit from cochlear implantation in the poorer ear with continued use of a hearing aid in the better ear. Design Ten adults with asymmetric hearing between ears participated. In the poorer ear, all participants met cochlear implant candidacy guidelines; seven had postlingual onset and three had pre/perilingual onset of severe-to-profound hearing loss. All had open-set speech recognition in the better hearing ear. Assessment measures included word and sentence recognition in quiet, sentence recognition in fixed noise (four-talker babble) and in diffuse restaurant noise using an adaptive procedure, localization of word stimuli and a hearing handicap scale. Participants were evaluated pre-implant with hearing aids and post-implant with the implant alone, the hearing aid alone in the better ear and bimodally (the implant and hearing aid in combination). Postlingual participants were evaluated at six months post-implant and pre/perilingual participants were evaluated at six and 12 months post-implant. Data analysis compared results 1) of the poorer hearing ear pre-implant (with hearing aid) and post-implant (with cochlear implant), 2) with the device(s) used for everyday listening pre- and post-implant and, 3) between the hearing