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Sensorineural hearing loss in child has a profound impact on their neurodevelopment and on language acquisition. Thus, the diagnosis and early intervention are critical, namely in unilateral or bilateral asymmetric hearing losses, which are diagnosed later than symmetric bilateral hearing losses.This study aims to examine the progression of asymmetric SNHL in children, and the link between hearing thresholds and the technological level of the hearing aid fitted.Eighteen children with asymmetric SNHL were submitted to a pure tone audiogram at the age of 5 years (T0) and reassessed at the age of 10 years (T1). Children were divided according to the technological level of the hearing aid fitted: basic, medium, and advanced.A positive correlation was established between the hearing thresholds at T0 and T1, revealing an accelerated progression of hearing thresholds in the better ear after a 5-year period.Hearing thresholds in the worse ear influence the progression of the SNHL. Moreover, technologically advanced hearing aids led to a slower progression of the hearing thresholds over time.

Bilateral cochlear implants are seen to improve hearing capabilities.To assess the auditory outcome of paediatric bilateral cochlear implant in Universiti Kebangsaan Malaysia.This was a cross-sectional and descriptive study single centre analysis. Categories of Auditory Performance (CAP-II) scale and Speech, Spatial and Qualities (SSQ) of Hearing questionnaire were used.Forty-six patients were recruited. Majority of the children (30.4%) rated 7 and 23.9% scored perfectly (9) based on the CAP-II Scale. The least performing children were rated 5 (average). Children that were implanted sequentially within 24 months showed median CAP-II scale of 7. No significant correlation seen between CAP-II and the duration interval, use and age of 1st CI (Bilateral CI have shown benefits in auditory performance even though majority were performed sequentially. Promising outcome was better with prolonged usage of the device.Hearing in noise is significantly better in bilateral CI.

Background: Demographic data of patients with sensorineural hearing loss (SNHL) in super-aged societies are still limited.Aims/objectives: To report audiometric statistics of SNHL and hearing aid (...

No cochlear otosclerosis in infants with congenital bilateral SNHL has been reported.We report an infant male with bilateral cochlear otosclerosis, suggesting that cochlear otosclerosis may be a congenital disease and to further analyze the etiology of and genetic expression in congenital bilateral cochlear otosclerosis. We also describe the clinical characteristics and experience of patients with bilateral cochlear otosclerosis treated with cochlear implants (CIs).Seven patients, including an infant, who were diagnosed with cochlear otosclerosis underwent CI surgery. Their medical records, audiological and radiological results, surgical procedures, and CI outcomes were collected and reviewed.The median age at hearing loss was 38 years, ranging from 0 to 47 years. The child had bilateral hearing loss at birth and received a CI at 1 year of age. He also had growth retardation and was diagnosed with 3q+/3p- syndrome. All patients (8 ears) had better postoperative auditory performance than that preoperatively.Although cochlear otosclerosis often starts at middle age and progresses slowly, it may be a congenital disease that is related to chromosome abnormality. This disease presents with SNHL or MHL, and treatment with a CI is beneficial.

To determine speech perception in quiet and noise of adult cochlear implant listeners retaining a hearing aid contralaterally. Second, to investigate the influence of contralateral hearing thresholds and speech perception on bimodal hearing.Sentence recognition with hearing aid alone, cochlear implant alone and bimodally at 6 months after cochlear implantation were assessed in 148 postlingually deafened adults. Data were analyzed for bimodal summation using measures of speech perception in quiet and in noise.Most of the subjects showed improved sentence recognition in quiet and in noise in the bimodal condition compared to the hearing aid-only or cochlear implant-only mode. The large variability of bimodal benefit in quiet can be partially explained by the degree of pure tone loss. Also, subjects with better hearing on the acoustic side experience significant benefit from the additional electrical input.Bimodal summation shows different characteristics in quiet and noise. Bimodal benefit in quiet depends on hearing thresholds at higher frequencies as well as in the lower- and middle-frequency ranges. For the bimodal benefit in noise, no correlation with hearing threshold in any frequency range was found.

The long-term stability of low-frequency residual hearing found in a significant number of bilateral-bimodal users (cochlear implant (CI) in one ear and a hearing aid (HA) on the other ear; CI/HA) with severe-to-profound or profound hearing loss in the non-implanted ear justifies bilateral-bimodal fitting efforts in this group. Since low-frequency residual hearing tends to deteriorate in some of these CI/HA users, periodic evaluation, which includes pure-tone thresholds and speech perception tests, is mandatory for determining the point in time at which CI/HA hearing is no longer effective, and the patient should accordingly be considered as a candidate for contralateral cochlear implantation.To determine, in bilateral-bimodal (CI/HA) users with severe-to-profound or profound hearing loss in the non-implanted ear, the rate of residual hearing deterioration in the non-implanted ear after cochlear implantation.Pure-tone aided and unaided thresholds in the non-implanted ears of 39 CI/HA users at 0.25-4.0 kHz were recorded prospectively up to 6 years after implantation.Group mean threshold values in the non-implanted ears remained stable over 3, 4, 5 and 6 years post-implantation, except for significant deterioration at 4.0 kHz of both unaided (4.2, 5.2, 9.0 and 8.2 dB, respectively) and aided thresholds (8.1, 4.6, 6.1 and 8.3 dB, respectively).

The use of a hearing aid (HA) in combination with a cochlear implant (CI) significantly improved performance for speech perception in quiet, in noise, and for localization compared with monaural conditions. No significant differences in functional performance were observed following optimization of HA fitting.To evaluate the binaural benefits derived from using a contralateral HA in conjunction with a CI in subjects with significant functional hearing in the nonimplanted ear and the effects of HA fitting optimization.Fifteen adult CI users, intra-subject controls, were enrolled in a prospective repeated-measure multicenter study. Evaluation of performance for speech understanding, localization, and subjective impressions was conducted before and following HA fitting optimization for CI alone, HA alone, and CI + HA.For speech testing in quiet, bimodal scores were significantly better than for HA alone and CI alone conditions (p0.01). For speech and noise (S0N0) at 0° azimuth the scores were significantly better in the bimodal condition than for CI alone (p = 0.01), indicating binaural summation. When noise was presented to the HA side (S0NHA) bimodal scores were significantly better than for CI alone (p0.01 and p0.05, respectively), suggesting a significant binaural squelch effect. Sound localization ability was significantly improved in the bimodal condition compared with the CI alone condition (p = 0.002).

There is no significant difference in speech recognition scores obtained with the Vibrant Soundbridge and the open-fit hearing aid. However, the Vibrant Soundbridge may be superior to open-fit hearing aids in improving hearing at high frequencies (4-8 kHz).To assess whether an improvement in speech recognition conferred by Vibrant Soundbridge is more marked than that afforded by open-fit hearing aids in patients with sloping high frequency sensorineural hearing loss (SNHL).This study had a self-control prospective design. Seven patients aged 21-62 years with sloping high frequency SNHL were recruited into the study. Each patient received a Vibrant Soundbridge middle ear implant (Vibrant MED-EL) and wore an open-fit hearing aid (Danavox, DOT 10). Speech recognition tests were performed according to protocols suggested by árpád Götze's speech definition test in Hungarian language. In the first session, conventional hearing thresholds (unaided pure tone thresholds) were measured. In the second session, the aided sound-field threshold, speech understanding score and functional gain obtained using middle ear implants and open-fit hearing aids were determined after programming of the devices.Regarding speech recognition scores, there were no significant differences between data obtained with the middle ear implant and those with the open-fit hearing aid.

The range of evaluation tools used in deciding which ear to implant and which to designate for a hearing aid (HA) should be expanded to include additional aspects to those tested by audiometry and basic speech perception. Residual hearing in non-implanted ears remains stable for at least 3 years after unilateral cochlear implantation, but regular refitting and monitoring of the HA function combined with cochlear implant (CI) mapping are mandatory for maximizing benefit from binaural-bimodal hearing.To examine whether the clinical decision-making tools currently used to assess hearing are reliable guides when choosing the preferred ear for CI, and to determine the rate of residual hearing deterioration in the non-implanted ear over 36 months post-CI as a guide to recommending subsequent continued use of a contralateral HA as opposed to CI.This was a retrospective evaluation of patients' charts. The pre-CI choice of the ear for implantation in a group of 37 binaural-bimodal users was re-evaluated. In a second group of 22 patients, residual hearing deterioration was followed for 36 months post-implantation.In the group of 37 patients, subjective identification of the worse-hearing ear was in agreement with audiometric results in 28 cases, but disagreed with the unaided audiometric results in the other 9. Mean threshold values for the group of 22 patients remained stable over 36 months post-CI, except for the aided threshold at 4.0 kHz, which deteriorated by 10.9 dB (p=0.003).

Conclusions. Steroid treatment was effective in improving hearing in most episodes in our and previously reported patients with acute hearing loss associated with aortitis syndrome. This suggests that inflammatory changes due to autoimmune responses may play an important role in the manifestation of hearing loss in aortitis syndrome. Objectives. To clarify clinical features in patients with aortitis syndrome who exhibited hearing loss. Case reports. We describe two patients, 39-year-old and 53-year old women, who developed several episodes of acute sensorineural hearing loss associated with aortitis syndrome. Results. In both patients, hearing loss involved the cochlea and hearing recovered following corticosteroid treatment. A literature review revealed that hearing loss was commonly manifested in middle-aged females and involved bilateral ears. Corticosteroid treatment was effective in improving hearing in most episodes in the previously reported cases as in our patients.

Cochlear implantation in the poorer ear of an elderly patient does not predict poorer post-operative audiological, quality-of-life (QoL), and quality of sound results.To determine whether choosing the 'better' or the 'poorer' ear for cochlear implantation impacts performance outcome in an elderly population.Forty-two out of 73 elderly (≥ 60 years) cochlear implant users with some residual hearing in at least one ear were selected. Three criteria were used to group the patients as 'better' or 'poorer' ear implanted; (C1) based on previous use of hearing aid, (C2) pre-operative PTA, and (C3) pre-operative speech discrimination. The Glasgow Benefit Inventory (GBI) and the Hearing Implant Sound Quality Index (HISQUI) were used to measure QoL and quality of sound, respectively.The mean post-operative PTA was 38.7 ± 7.2 dBHL. In quiet, the mean disyllabic SDS at 65dBSPL was 75.4 ± 18.5, whereas the discrimination of sentences was 95.0% ± 6.9. The mean total GBI score was 30.9 ± 21.8, 92.9% of patients reporting a positive score. The mean HISQUI score was 111.3 ± 36.0, which corresponds to 'moderate' sound quality. No significant differences were found between both groups in terms of audiological outcomes, HISQUI scores or GBI scores when considering each of the three criteria.

This prospective study shows that working performance, quality of life (QoL), and quality of hearing (QoH) are better with two compared with a single cochlear implant (CI). The impact of the second CI on the patient's QoL is as significant as the impact of the first CI.To evaluate the benefits of sequential bilateral cochlear implantation in working, QoL, and QoH.We studied working performance, work-related stress, QoL, and QoH with specific questionnaires in 15 patients with unilateral CI scheduled for sequential CI of another ear. Sound localization performance and speech perception in noise were measured with specific tests. All questionnaires and tests were performed before the second CI surgery and 6 and 12 months after its activation.Bilateral CIs increased patients' working performance and their work-related stress and fatigue decreased. Communication with co-workers was easier and patients were more active in their working environment. Sequential bilateral cochlear implantation improved QoL, QoH, sound localization, and speech perception in noise statistically significantly.

The objective of this study was to evaluate the benefit of cochlear implantation in adults aged 60 years and over. Twenty-eight patients, older than 60 years and with profound bilateral sensorineural hearing loss, received a cochlear implant between 1991 and 2001. The mean age was 66 years and the median follow-up was 22.5 months. Speech perception scores before and after implantation were analyzed retrospectively in order to evaluate the benefit of cochlear implantation. There was a significant improvement of the disyllabic words and sentences scores after implantation. The patients who were over 70 years performed as well as those who were younger. The surgical procedure was well tolerated in all patients. One patient developed a postoperative vertigo due to a perilymphatic fistula. In conclusion, cochlear implantation offers improvement in speech perception to the elderly population, as in the younger population. A careful assessment of the physical status of these patients remains essential in order to evaluate the risk-benefit of this procedure.

When vestibular function is reduced in the rotational chair test in children with severe hearing loss, the vestibular function may be acquired later due to maturing vestibular sensory cells and vestibular nerve of the inner ear along with physical growth.To examine the relationship between acquisition of motor function and vestibular function in children with bilateral severe hearing loss.A total of 97 children under 4 years old with hearing loss defined as a hearing threshold of both ears greater than 80 dB were included in this study. For evaluation of vestibular function, a damped-rotational chair test was performed and the horizontal nystagmus was recorded using electronystagmography (ENG).Head control and independent walking were delayed in 28 of 97 children with severe hearing loss. Reduced response to the rotational chair test was observed in 16 of 97 children (16.5%), with 11 of these children having inner ear anomalies and reduced vestibular function. Of the 10 children who were followed up by the rotational chair test, 2 children with idiopathic congenital hearing loss without inner ear anomalies (100%) and 6 of 8 children with bilateral inner ear anomalies (75%) showed more obvious nystagmus during rotation compared with the initial examination.

Bone-conducted sound lateralization tests to determine interaural time difference (ITD) and interaural intensity difference (IID) were conducted by means of a self-recording apparatus in 20 children and a young adult with bilateral microtia and atresia of the ear. This apparatus changes ITD automatically from 0 to 2,000 micros at 50 micros/s and IID from 1 to 40 dB at 1 dB's. When ITD exceeds approximately 200 micros/s and IID exceeds 5 dB in normal subjects the sounds are recognized separately. The test stimulus was a continuous narrow-band noise at 500 Hz and 30 dB SL applied to the right and left mastoids through bone vibrators. In the patients with bilateral atresia of the ears, ITD results revealed approximately normal thresholds of discrimination in half the patients and IID results revealed threshold elevation in only 10%. It is noted that bone-conducted sound lateralization abilities of ITD or IID are maintained in many of these patients.

During a period of 5 years, a total of 47 patients, 16-60 years of age, with major closed head injury (CHI) were admitted to 2 Swedish medical centres. Seven to 11 years after injury, 25 of them (mean age 40.8 years; range 25-59 years) were evaluated using peripheral and central auditory tests, and most of them also completed 2 questionnaires concerning self-assessed hearing ability and quality of life. Twenty-two patients did not participate in the long-term follow-up tests: 15 were excluded for medical reasons and 4 did not wish to participate; an additional 2 were impossible to trace and 1 had died. As many as 68% of patients (17/25) demonstrated abnormalities on 1 or more of the audiometric tests, 14 on pure-tone audiometry (p < 0.02) and/or central audiometric tests (3 on central tests only and 2 on pure-tone audiometry only). Four out of six patients with available early post-traumatic audiograms showed a significant progressive deterioration. As a group, their assessments of hearing ability and quality of life were equal to or better than those of the controls. These results may indicate that a higher priority should be given to obtaining an early audiologic evaluation of every CHI patient than is the rule today. The impact of post-traumatic progress and central lesions on social hearing at an advanced age is highlighted.

Acoustic neurinoma affects the acousticofacial nerve and therefore in many cases is a contraindication for cochlear implantation and an indication for brainstem implant. Nevertheless benefit in these patients has been shown after tumour removal and cochlear implantation. The first case of bilateral cochlear implantation in a patient with single-sided untreated acoustic neurinoma is described here. In a 49-year-old woman with progressive hearing loss during the last 12 years we preoperatively diagnosed an acoustic neurinoma of the left side. After cochlear implantation of the right side she was sequentially implanted on the affected side as well. Before surgery radiological control of the tumour for signs of growth was performed and the patient was thoroughly informed of the situation and possible therapies and dangers. Speech discrimination scores obtained in the second implanted ear came up to the performance of the first implant after 6 months.

The aim of this study was to evaluate the pathological significance of antibodies against cornea and inner ear tissue in the development of audiovestibular and ocular symptoms in patients with Cogan's syndrome (CS). We analysed the serum of 5 CS patients for binding of IgM and IgG to fresh cryosections of rat labyrinth (semicircular canals, ampulla, utricle, saccule) and cornea by indirect immunofluorescence (IF). The predominant pattern of anti-corneal IgM was staining of the superficial cell layer of the non-keratinizing squamous epithelium. IgM against cornea was found in 3 patients, all of whom had bilateral inflammatory eye signs at the start of the disease. However, IgM was also detected in the chronic stage of the disease when no clinical signs of eye involvement were apparent. The study includes the first follow-up examination of anti-corneal IgM and IgG antibodies during a complete episode of active CS. During the first episode of CS in 1 patient, anti-corneal IgM became detectable 1 week after the onset of interstitial keratitis and 3 weeks after the onset of audiovestibular symptoms. It increased over several weeks and then fell to very low levels. However, at no time was anti-corneal IgG found. In the course of follow-up examinations, the serum of 4 patients intermittently contained low titre IgG antibodies against inner ear labyrinthine tissue, but without any clear correlation with the active stages of CS. In addition, high-resolution MRI (HR-MRI) of the inner ear was performed in the acute and chronic stages of CS to evaluate the activity of CS. In the acute stage, HR-MRI revealed abnormal MRI signals in the vestibule, semicircular canals, vestibular nerve, or cochlea. In the chronic stage, patients showed narrowing or occlusion of semicircular canals and the cochlea on the 3D-CISS images, but no high signal lesions (T1) and no enhancement. Antibodies against cornea or labyrinthine tissue were not consistently detected in CS and the level of organ-specific antibodies did not correlate with the activity of the disease.

The objective of this study was to examine the outcome of unilateral stapes surgery in one patient group with bilateral hearing loss and one group with unilateral hearing loss. The patients' own estimations of improvement in hearing ability and the occurrence of other ear-related symptoms were examined retrospectively and in a follow-up study. Ninety-five of 123 patients operated for otosclerosis in only one ear between 1987 and 1992 responded to a follow-up examination. Observed audiometric findings and changes thereof, along with the patients' own estimations of their hearing handicap pre- and postoperatively, and the occurrence of other ear-related symptoms were studied. Despite good surgical results (closure of air-bone gap within 20 dB in 94%), 33% of the patients had severe hearing disabilities postoperatively, and many of these patients needed further amplification with a hearing aid. Mild dizziness occurred in 33% of the patients postoperatively and did not decrease over time. Discomfort in the operated ear due to strong sounds was reported in 20%. Change in sound quality occurred in 80% of the operated ears, but tended to disappear over time. From the results of this study it may be concluded that surgery in one ear only, leaving the other ear with poor hearing, is not an optimal hearing rehabilitation of patients with otosclerosis. It is important endevour to achieve bilateral hearing in order to give the patient good social hearing. Postoperative dizziness and unpleasant hearing quality do occur frequently, and the patients need to be informed about these problems preoperatively.

We report the case of a young male patient with ankylosing spondylitis treated with sulphasalazine who developed bilateral sensorineural hearing loss with tinnitus. Discontinuation of sulphasalazine led to complete resolution of tinnitus and an improvement in hearing loss. We discuss the features of this case and also review the relevant literature.

Cochlear ossification following bacterial meningitis is related to causative pathogen, but not age at disease or time point of evaluation. However, progression may occur over time, especially in case of primary signs of ossification.To investigate the occurrence and degree of cochlear ossification on CT and MRI in patients with bilateral profound hearing loss following bacterial meningitis, in relation to causative pathogen, age at disease, and time point of evaluation. Progression of ossification in cases that underwent more than one scan was evaluated.In the period 1982-2008, 47 cochlear implantations were performed in 34 consecutive candidates suffering from bilateral profound hearing loss following bacterial meningitis. A retrospective review of patient files and preoperative CT and MR images was performed.Cochlear ossification was observed in 35% of patients and 26% of ears on CT. The corresponding values for MRI were 44 and 30% (difference not significant). Streptococcus pneumoniae infection caused ossification more frequently than Neisseria meningitidis. No difference was found between pediatric and adult cases, and the occurrence of ossification was not related to the time point of evaluation. Signs of progressive ossification were found in cases with two CT scans, especially if ossification was present at the first scan.

We mailed questionnaires to 98 university hospitals and 939 hospitals with 200 beds or more throughout Japan asking for information on patients treated for sensorineural hearing loss between January 1 and December 31, 1987. The number of patients treated for sensorineural hearing loss in 1987 in Japan was estimated 13,900-19,600, ii) idiopathic bilateral progressive sensorineural hearing loss 13,900-19,600, ii) idiopathic bilateral progressive sensorineural hearing loss in 800-2,000, iii) perilymphatic fistula in 200-310, iv) mumps deafness in 200-400, and v) unilateral profound deafness in 2,000-3,000. Sex ratio (men over women) was 0.9 in sudden deafness and mumps deafness, 1.1 in idiopathic bilateral progressive sensorineural hearing loss and 1.5 in perilymphatic fistula. Patients were distributed with a definite peak from 50 to 59 years old in sudden deafness, from 30 to 39 years old in perilymphatic fistula, and two apparent peaks in teenagers and those aged 60-69 years in idiopathic bilateral progressive sensorineural hearing loss.

Using a soundproof pressure chamber, we performed Békésy air conduction audiometry at 1000 Hz under the following pressure conditions: 1) from atmospheric pressure (AP) to 200 mmH2O below AP, 2) AP to 200 mmH2O above AP, 3) AP to 700 mmH2O below AP, and 4) AP to 700 mmH2O above AP. In conditions 1) and 2), the patient was instructed not to swallow until the pressure change was complete, then to swallow once every 15 seconds. The number of swallows required for hearing to return to the baseline level was counted. In conditions 3) and 4), the patient was told to swallow as many times as possible both before and after completion of the pressure change; maximum hearing level and the time needed for return to the baseline level were measured. Although all except one of the seven patients with suspected tubal dysfunction showed normal results with catheter insufflation and tympanometry, six of the seven showed abnormality and one reported the disappearance of ear stuffiness after the ventilation capacity test. We concluded that this test is useful not only for the diagnosis of tubal dysfunction but also as a possible therapy for aiding adaptation to changing environmental pressure.

We used a three-dimensional audiogram which displays duration of the disease on the X-axis, frequency on the X-axis, and hearing level on the Z-axis, to aid observation of hearing changes during the course of various otologic diseases. An overall, quantitative view of the changes in a patient's hearing level with time was easily obtainable with this technique, as the temporal element is shown on the X-axis. The basal view position of the three-dimensional audiogram displays most of the information necessary to form a diagnosis, determine treatment, and evaluate its efficacy in various otologic diseases. The three-dimensional audiogram was found to be most useful in observing changes in hearing level in sudden deafness, with its often rapid recovery period, and Meniere's disease, with its long-term fluctuating hearing loss. In some patients, a better understanding of the hearing changes could be obtained from a view angle other than the basal view position.

This universal newborn hearing-screening (UNHS) programme revealed high efficacy. The proportion of congenital sensorineural hearing loss was higher in left ears and in males than in right ears and females, which was in line with the systematic ear asymmetries and sex differences in transient-evoked otoacoustic emission (TEOAE) pass percentage.To study the long-term outcome of a UNHS programme based on multiple TEOAEs and clinical click-evoked auditory brainstem response (ABR).The study included all the newborns that were screened during a 6-year period (n = 31 092). TEOAE pass/fail was analysed in detail. In an assessment performed 10 years after the start of the 6-year UNHS, prevalence, degree and type of congenital hearing loss were studied.The proportion of screened newborns was high, i.e. 98%. Multiple TEOAE recordings minimized the need for clinical ABR. Fifty-seven (0.18%) subjects showed bilateral hearing loss (exceeding ≈ 30 dB HL); median ABR threshold = 60 dB nHL (at 2.5 months of age). Bilateral and unilateral sensorineural hearing loss was found in 0.17% (n = 52; 56% males) and 0.06% (n = 18; 61% left ears, 56% males) of the screened newborns, respectively. Higher TEOAE pass percentages (p0.01) were demonstrated in right ears and in females than in left ears and males.

A reported mutation in SIX1 was identified in a patient with familial hearing loss (HL), a left preauricular pit, and bilateral enlarged vestibular aqueducts (EVA). Although the characteristic symptoms of EVA including fluctuating HL and repetitive vertigo were not seen in the patient, further studies are needed to clarify the association between EVA and such symptoms.To study the audiovestibular functions, and to identify the causative gene in a patient with branchio-oto syndrome.We enrolled a 30-year-old female in whom HL was pointed out at the age of 6 years. She visited our department at the age of 21 years, and had not experienced any progression of her HL, tinnitus, or vertigo. Pure-tone audiograms showed bilateral moderate mixed HL with no apparent progression during a 9-year follow-up period. Audiovestibular examinations included distortion product otoacoustic emissions (DPOAEs), electrocochleography (ECochG), and electronystagmography (ENG). Direct sequencing was utilized to screen for SIX1, EYA1, SLC26A4, GJB2, and mitochondrial DNA MTRNR1 including 1555 position.The findings of DPOAEs, ECochG, and ENG indicated cochlear HL with no vestibular dysfunction. A previously reported mutation of a heterozygous c.386AG (p.Y129C) in SIX1 was detected. No mutation was identified in EYA1, SLC26A4, GJB2, or MTRNR1.

Auditory and vestibular investigations were carried out in 19 affected men and 13 obligate female carriers of 7 pedigrees with nonsyndromic hearing loss segregating as an X-linked trait. In addition, high resolution computerised tomographic scanning was carried out in 24 affected males and 12 obligate female carriers. The neuro-otological results confirm that non syndromic X-linked hearing loss is a clinically heterogeneous condition, but radiological assessment of the cochlea revealed two distinct groups: a normal group, and an abnormal group characterised by a bulbous internal auditory meatus, a dilated facial nerve canal and incomplete separation of the basal coil of the cochlea from the internal auditory meatus. Within a given pedigree there was marked consistency of the presence or absence of the CT scan abnormality in the affected males. One third of the obligate female carriers of the radiologically abnormal pedigrees were shown to have a similar abnormal finding, but as two thirds were normal, radiological examination did not predict carrier status. In the affected men, pure tone audiometric data did not correlate with the radiological abnormality, whereas vestibular function was strikingly correlated, being normal in all but one case in pedigrees with normal radiology and absent, or grossly impaired, in the pedigrees with abnormal radiology. Neuro-otological abnormalities were documented in approximately two thirds of the obligate female carriers, but were insufficiently frequent in occurrence or specific in type to be of predictive value.

Conclusions. The results support bilateral sequential implantation for patients who are not completely satisfied after implantation of one side. Objective. To evaluate the benefit of bilateral Vibrant Soundbridge middle ear implantation as compared with unilateral implantation in quiet and noisy environments. Methods. This was a multicentric and retrospective study of 15 patients with symmetrical sensorineural hearing loss who were implanted sequentially in both ears. The performance of each subject was compared under three conditions: with the right implant activated, with the left implant activated, and with both implants activated. Audiometric tests were compared with self-assessment subjective evaluation by questionnaire. Results. Both qualitative and quantitative assessments demonstrated improvement in speech intelligibility, especially in background noise, but also for low voice intensity in quiet.

Codeine shows a similar association with profound deafness to other opiates. Good cochlear implant outcomes suggest a sensory disorder. Although macrocytosis could be due to confounding factors, the lack of other consistent findings may signify a vascular pathology.To describe a series of patients identified as codeine users after referral for cochlear implantation.This was a retrospective case series review. Patients were identified by the senior audiologist. Information regarding mode of onset of deafness, past medical history, drug and alcohol history, investigations and audiological outcomes following cochlear implantation was collected from hospital records and patient questionnaires.Ten patients were included in the study. All patients had taken codeine phosphate and paracetamol in combination for several years, usually at greater than recommended daily dose. All patients presented with sudden or rapidly progressive bilateral deafness. All patients had a significant macrocytosis at the time of deafness (mean cell volume (MCV): mean 115 fL; range 105-132 fL). No other investigation was consistently abnormal. Four patients had a history of alcoholism. Seven patients had abnormal liver function tests. Patients usually performed well with cochlear implants (CUNY sentence scores without lip reading90% in 9 of 10 patients).

A retrospective study of 161 patients with Menière's disease followed up for 9 years or more is presented. The development, with respect to hearing, caloric response, vertigo, and ability to work, was analyzed. The main functional loss was found to take place early in the course of the disease. There was an increase in bilaterality with time. A spontaneous relief in vertigo over the years has been noticed.

Conclusions: New technological developments will most probably improve the efficiency of auditory brainstem implantation (ABI). Meanwhile, cochlear implantation in patients who have undergone prior reductive surgery, and who have maintained a positive electric stimulation, is an excellent alternative for rehabilitating complete and bilateral hearing loss in patients with neurofibromatosis type 2 (NF2). Auditory results are far better than those reported after ABI. Long-term follow-up will be necessary to demonstrate the validity of this strategy. Objectives: ABIs restore some degree of auditory perception in NF2 patients with bilateral and complete hearing loss, but results are often inadequate for maintaining social and professional activities. The aim of this study was to report the results of auditory rehabilitation by cochlear implantation in three cases of NF2. Patients and methods: This was a retrospective study undertaken in a tertiary referral center. The first patient had undergone previous surgery for a left grade III vestibular schwannoma (VS) and then underwent irradiation for a right grade I VS. Two years after irradiation, he suddenly lost his remaining hearing. Electric promontory stimulation was positive and cochlear implantation was performed. The second patient had undergone surgery for a left grade III VS and followed for a right grade II VS. She suddenly lost her remaining hearing. A cytoreductive surgery was performed and the cochlear nerve was preserved. Postoperative electric stimulation was positive. She was then implanted with a cochlear implant. The third patient presented with a right stage III and a left stage I VS. She first underwent a subtotal removal of the left VS with immediate cochlear implantation. She then underwent removal of the right VS stage III with no possible preservation of the cochlear nerve. Results: All three patients had excellent postoperative speech performance and were back to work 3 months after implantation. Imaging follow-ups at 4, 2, and 1 year, respectively, do not show any evolution of the tumor.

Patients with large vestibular aqueduct syndrome (LVAS) have disturbed morphogenesis of bony labyrinth. Semicircular canal anomalies are common in LVAS.To describe the additional inner ear anomalies on CT imaging in pediatric patients with LVAS, and to investigate the lateral semicircular canal (LSCC) anomalies associated with LVAS by measurement of the LSCC bony island width.We retrospectively reviewed the digitally stored temporal bone CT imaging obtained for 23 patients with LVAS, additional inner ear anomalies were noted, and measurements of the LSCC bony island width were made on axial CT scans on the workstation. Measurements were compared to the normative data obtained from 20 patients without sensorineural hearing loss.Of the 23 patients (bilateral in 22 and unilateral in 1), additional inner ear malformations were identified in 21 cases presenting either singly or in combination. A small LSCC bony island (3 mm in diameter) appeared highly typical; vestibule and LSCC anomalies were identified in 13 cases (26 ears) based on visual inspection combined with abnormal measurements. Dehiscence of the superior and/or posterior semicircular canal was identified in 19 ears, and Mondini deformity was identified in 6 ears.

The objective of this report is to emphasize the clinical features of delayed endolymphatic hydrops in comparison with Meniere's disease. Four cases were selected from a series of 16 examined between the years 1993 and 2005, due to their unusual characteristics. A short clinical history and significant tests are presented for each of the four cases. The cases of delayed endolymphatic hydrops discussed in this article suggest that hydrops is the most important underlying pathology that causes the hearing loss and the vestibular symptoms both in the better ear and in the ear with severe hearing loss. They also suggest that this condition probably occurs in patients with congenitally sensitive ears that make them prone to the development of the late hydrops.

Evoked otoacoustic emission (OAE) and auditory brainstem response (ABR) results for hearing screening among infants have good concordance. However, good correlation with the Griffiths Developmental Scales remains to be desired.To correlate hearing screening outcomes of a cohort of infants with developmental outcomes at 6 and 12 months.A cohort of pregnant women was identified in several communities in a rural area (Bulacan province) from April 2002 to February 2003 as part of a population-based study determining maternal exposure to pollutants and infant outcomes, with a total follow-up of 2 years. Pregnant mothers were identified and followed up until delivery at a secondary, provincial hospital. Hearing screening was performed with OAEs and ABR. Mental development of infants was assessed at 6 and 12 months using Griffiths Mental Developmental Scales - locomotor, personal-social, hearing and speech, hand and eye coordination, performance tests.Among the 1086 babies recruited, there were 724 with hearing assessment. Of these 724 babies, 565 had both OAE testing and ABR. Overall in 1130 ears, OAE and ABR testing showed an observed agreement of 99%, agreement due to chance of 96%, and kappa agreement of 79% (p=0.00) in diagnosing bilateral hearing losses. OAEs had a sensitivity of 86.4% (95% CI 64-96.4%) and a specificity of 99.4% (95% CI 98.6-99.7%). At the end of the study, there were 708/724 (97.8%) infants with normal hearing, 7/724 (1.0%) with unilateral hearing loss, 8/724 (1.1%) with bilateral mild hearing loss, and 1/724 (0.1%) with bilateral profound hearing loss, who demonstrated consistent mental delay throughout. Follow-up rates for developmental examinations at 6 and 12 months were 98% and 81.25%, respectively. In these groups, there were 8 (1%) infants at 6 months and 18 (2.4%) at 12 months with developmental delay (Griffiths Mental Developmental Scales).

Although superficial siderosis (SS) has been clinically characterized as a combination of sensorineural hearing impairment, cerebellar ataxia and pyramidal signs, precise evaluation of the function of the eighth cranial nerve has rarely been reported. The purpose of this study was to evaluate the audiological and vestibular function. We present a patient with complaints of progressive bilateral hearing loss and gait difficulty. We evaluated the audiological and vestibular functions with auditory brainstem responses and vestibular evoked myogenic potentials (VEMPs) by clicks and galvanic stimuli. The patient showed linear hypointensities surrounding the brainstem, cerebellum and the eighth cranial nerve on T2-weighted MRI images, which is characteristic of SS. Auditory brainstem response showed only wave I in the right ear and no response in the left ear. Click VEMPs and galvanic VEMPs showed no response on either side. The results of a neuro-otological examination suggested that both audiological and vestibular dysfunction in the patient with SS is of retrolabyrinthine origin.

Conclusion. It is suggested that a holistic factor – such as psychological stress – is involved in Meniere's disease (MD) and that the pathological changes in MD may be a result not only of endolymphatic hydrops but also of disorders affecting the entire cochlea. Patients and methods. Changes in the hearing of 51 patients with unilateral MD were investigated to ascertain the correlation between changes in hearing loss (a) in the affected ear vs the contralateral ear and (b) at low frequencies vs high frequencies. Results. About half of the MD patients showed a significant positive correlation between the hearing level in the affected ear and that in the contralateral ear and also between the average hearing level at lower frequencies and that at 8 kHz. These tendencies were more pronounced in patients with severe fluctuation of hearing and/or severe hearing loss.

Spontaneous musical auditory perceptions commonly occur in patients who develop abrupt bilateral sensorineural hearing loss. The findings in both subjects who were image tested while having these perceptions are suggestive of a biological substrate for this process and of a central locus for auditory memory seemingly located in and around area 39 of Brodmann. When an individual has abrupt bilateral sensorineural hearing loss, stored musical memory can be released and this person can have musical perceptions without an external source. It is likely that an abrupt bilateral loss of inner ear function might uninhibit neuronal groups storing auditory memory.The objectives of this study were as follows. (1) To determine if spontaneous musical auditory perceptions occur in patients who develop abrupt bilateral severe sensorineural hearing loss (not necessarily simultaneously). (2) To determine if there is a biological substrate to the process of recalling previous auditory perceptions. (3) To compare these findings with our normal databases of unstimulated and pure tone-stimulated volunteers. (4) To establish a hypothesis for the mechanisms of these occurrences.Thirty two patients who had had abrupt bilateral severe sensorineural hearing loss (the interval between the losses of both ears could have been years) were contacted. They were asked if they had ever had the sensation of having musical auditory perceptions without external auditory stimuli. Two of these patients were image tested with single photon computerized emission tomography (neuroSPECT) while they were having these perceptions.All of the 32 subjects had musical auditory perceptions following their hearing loss in the second ear or when hearing loss in both ears occurred simultaneously. The two patients who were image tested with neuroSPECT had similar findings. There was a statistically significant increase in perfusion in area 39 of Brodmann, more intense on the right side, with increased perfusion also in both frontal lobes at the middle gyrus, with bilateral hypoperfusion in area 38 of Brodmann. These findings are similar to those observed in normal volunteers stimulated with pure tones.

Objective-To assess the advantages of binaural hearing for cochlear implant (Cl) users using a hearing aid (HA) for the contralateral ear. Material and Methods-The subjects comprised 3 males and 3 females (age range 48-84 years). All of them had been using a CI and HA for > 6 months. Their speech perception was examined in quiet using monosyllables and Japanese Hearing in Noise Test (J-HINT) sentences. Speech perception in noise was examined using J-HINT sentences Late cortical waves were measured while subjects listened to 1 kHz frequent and 2 kHz target tone stimuli. The latency of the event-related potential (P300) wave was compared for monaural and binaural hearing conditions. Results-Three subjects showed significantly better results for binaural than monaural (Cl alone) hearing for monosyllables and HINT sentences (p < 0.05; paired t-test). Subjects with better speech perception had been using an HA for longer than those with poor performance (18.3 vs 4.0 years). The overall average score was better for binaural than monaural hearing in the speech perception test under quiet and noisy conditions. Comparison of the latency of the P300 wave under monaural and binaural hearing conditions showed a significantly shorter latency for the latter (p = 0.02; paired t-test). Conclusions-Although the use of an HA alone showed marginal benefit for CI users, binaural hearing (CI +HA) resulted in a significant improvement in speech perception under various circumstances.

To evaluate the treatment efficacy of an electromechanical middle ear amplifier implant (AI) in patients with chronic moderate-to-severe sensorineural hearing loss (SNHL). The AI is a piezoelectric system with a sound processor and a rechargeable battery within a hermetically sealed titanium canister. Its titanium-sealed microphone is placed in the bony region of the ear canal. The incus-coupled transducer (actuator), which is also inside a titanium casing, is fastened to the adjacent bone.This was a phase III study comprising 20 intention-to-treat patients. Telemetrical adjustments followed electromechanical amplifier implantations. We used a word recognition test as our primary efficacy measure (Freiburg Speech Recognition Test: DIN 45621). Secondary efficacy measures were the sentence comprehension test (Goettinger Satztest, 1996) for auditory orientation within noisy and quiet environments and a psychosocial adjustment test (Gothenburg Profile Test, 1998). The 6-month follow-up comprised a complete medical examination. Nineteen patients completed the study (per-protocol patients; 100% reference).Seventeen patients (89%) demonstrated improved binaural recognition of phonetically balanced monosyllables. Fourteen postoperative patients (74%) attained a perfect score (100%) on this test, compared to only 3 preoperative patients (16%). Thirteen patients (68%) reached the sentence recognition threshold at a 2:1 dB signal-to-noise ratio during noisy trials. Correct identification of the noise source direction in the horizontal plane occurred in 89% of the trials. The Gothenburg Profile Test scores showed that the subjective evaluation of hearing, orientation, social behavior and self-confidence increased from 48% to 88%. Three patients did not benefit from the implant.Treatment of SNHL with a totally implantable hearing system can be an efficient method for those patients unable to wear hearing aids. However, in order to avoid implantation in non-responders, there is a need for more specific audiological indication criteria.

In this study, bilateral cochlear microphonics (CM) were evoked by tone burst simultaneously. A speaker was put in head-food axis 2 m from the mid-point of a given line connecting the bilateral external meatus. Five normal persons and 68 cases (34 cases of Meniere's disease, 27 cases of sudden hearing loss, and 7 cases of low-tone sensory hearing loss without vertigo) with unilateral sensory hearing loss and recruitment, in addition to 2 cases of bilateral Meniere's disease with recruitment were examined. CM shifted in normal and hearing loss ears and was absent in profound and totally deaf ears. When recruitment was present, CM at corresponding frequencies were enlarged and prolongated in 60 cases. Some of the enlarged and prolongated CM decayed slowly, others quickly. Meanwhile the CM of the opposite normal ear decreased obviously. The presence of enlarged and prolongated CM may indicate an increase of abnormal excitability of the hair cells caused by some pathological stimulations. This would cause excitability of the hair cells in the opposite cochlea to be inhibited by the effect of the efferent system. In such a condition, the patients complained that the stimulating sound was heard louder in the disordered ear than that in the opposite normal ear. CM was slightly enlarged during sleep.

The present paper investigates the hearing function in chronic otitis media vs. tympanoplasty in 41 patients with bilateral chronic otitis media, operated on unilaterally. the auditory evaluation was performed immediately before surgery and at a follow-up time varying from 1 to 14 years (mean = 5 years). Results were expressed in terms of relative gain (in respect to preoperative hearing levels) and total gain (in comparison with the contralateral ear). a progressive reduction of the hearing threshold as a function of time was observed both in non-operated and operated ears. the comparison of the threshold shifts between the two ears indicated that the long-term functional results of tympanoplasty can be considered quite satisfactory.

In order to achieve consensus and maintain consistency within the Research Committee, diagnostic criteria were established for the types of hearing loss under investigation. All research was conducted according to these criteria. The criteria for the diagnosis of sudden deafness, assessment of hearing recovery, and diagnosis of mumps deafness, all previously published (1), are reproduced below. The criteria for perilymphatic fistula were revised in 1990. The criteria for immune-mediated sensorineural hearing loss are tentative and may be revised in the future.

Nine cases of acoustic neurinoma were treated by stereotactic radiosurgery between 1969 and 1974. The follow-up period can now be regarded as sufficiently long for a preliminary evaluation of the results. An arrest of growth or shrinkage of the tumour was observed in 8 of the 9 cases. In one case open surgery was performed 2 years after irradiation and histological examination showed regressive changes of the type expected after irradiation. Audiological examination revealed that in the majority of cases radiosurgery could be performed without causing serious damage to the hearing function. The average hearing loss present before treatment increased on average only 20.0 dB in the most successful 7 cases. None of the patients suffered facial nerve involvement. In small and medium-sized acoustic tumours this method offers a satisfactory therapeutic alternative worthy of consideration.

The audiometric configurations of a randomly selected group of 360 patients with clinical Meniere's disease were analysed in conjunction with their clinical manifestations. Although 78.6% of the patients had an abnormal pure-tone audiogram in the opposite ear from that which was initially diagnosed as Meniere's disease, based on the entire clinical picture, the disease was found to be definitely bilateral in 32% of the patients. A peaking audiogram type was found to be most common, occurring in about half of the involved ears. In approximately half of those with bilateral disease the second ear became involved within two years of onset of involvement of the first ear, and in another 27 % the second ear became involved after a period of 5 years or more. These observations stress the importance of conservatism in managing Meniere's disease, especially when considering surgical intervention for intractable disease, with the hope of retaining or restoring labyrinthine function.

Meningitis is one of the leading causes of acquired sensorineural hearing loss in childhood, and many retrospective and prospective studies and case reports have been published. However, they have seldom discussed the nature of postmeningitic hearing loss from the audiological point of view. This study reports the results of audiological examinations performed on 5 patients suffering from hearing loss following meningitis. Extremely poor word discrimination scores as compared with pure-tone audiograms were common in all 5 cases. Type IV Békésy tracings were observed in 2 cases. Discrepancies between pure-tone audiograms and subjective hearing sensation and between pure-tone audiograms and ABR recordings were noted in 2 cases. These results suggest that varying degrees of retrocochlear involvement complicating the inner ear damage are the audiological characteristics of hearing loss following meningitis.

Electrophysiological measures were collected over 3 years in 61 cases of sudden deafness. Electrocochleography (ECochG) and Auditory Brainstem Responses (ABR) using click stimuli were recorded down to threshold and compared with pure-tone and speech audiometry data. This study was intended to precise 1) localization of the disorder, 2) prognosis, 3) analysis of the contribution of different frequencies in click-evoked responses. The following results are: 1) In most cases the observations indicated that the inner ear was affected. 2) However, in 19 cases electrophysiological measures revealed a retrocochlear involvement. Four acoustic neuromas were evidenced by CT scan. In 3 of these, audiometric and electrophysiological thresholds returned merely to normal levels after medical treatment, while brainstem responses remained delayed. Thus in these cases recovery would seem to be related to release of vascular compression in the auditory internal meatus. 3) When the latencies of the VII nerve potential (N1) and wave V (PV) were short, and the therapy undertaken early, a good recovery was often obtained. 4) When recovery occurred on low frequencies only, the electrophysiological responses to the click showed slight latency increases.

We described 3 patients who, from early infancy, evidenced attacks of vomiting associated with fluctuating hearing loss, culminating in bilateral severe sensorineural hearing loss. The patients suffered from Meniere's disease, according to all clinical yardsticks. The patients described by us were at first diagnosed and treated for gastroenteritis or meningitis. Meniere's disease was not suspected. We want to point out that Meniere's disease, though usually presenting itself at middle age, may well start in childhood as well as in infancy, and should be suspected whenever vomiting, without diarrhea, is associated with some hearing loss at any age--no matter how young the patient is. It is also possible that some sensorineural hearing losses in late childhood or adulthood are in effect the end result of burnt-out Meniere's disease--as our second case presented himself.

Studies were made on 100 pupils, recruited from occupational rehabilitation schools for the deaf in Kraków. They underwent general medical as well as specialist examinations. Deafness was found up to the age of 3 years in 75% of those examined. In 30% of the subjects, their vestibular system was paralysed, ophthalmological disorders were found in 95% of cases, neurological disturbances in 15% and pathological changes of the central nervous system in 65% of cases were recorded by EEG. We did not look for evidence that deafness causes anatomical changes in the central nervous system, but the disturbances observed nevertheless indicate a correlation with congenital or early acquired deafness.

The cochlear and vestibular functions were investigated in a sample of 36 patients with unilateral Meniere's disease. Caloric reactions and hearing thresholds were compared separately at several frequencies. A topodiagnostic relationship between the cochlear and vestibular function was discovered. Using four qualitative categories, a significantly high correlation was obtained between the basal hearing loss (4000 Hz) and unilateral weakness, whereas no correlation was obtained when evaluating the more apical hearing loss (lower frequencies 250-1 000 Hz). A normal caloric reaction can be reasonably expected in cases of unilateral Meniere's disease if the hearing loss is less than approx. 40 dB HL at 4 000 Hz.

A vibratory stimulus at a frequency of about 100 Hz was transmitted through a bone conductor on the mastoid in 26 profoundly deaf subjects. In all of them, the function of the vestibular apparatus was tested by means of the Fitzgerald-Hallpike caloric test. In some of the deaf subjects, evoked potentials were recorded, which following the frequency of the stimulus and which according to amplitude and latency characteristics, corresponded to the frequency following response (FFR). A close correlation was found between the normal function of the vestibular apparatus and the appearance of FFR in deaf subjects. The possible receptors of the stimulus and the afferent pathways which generate the recorded response are discussed.