scholarly journals Cochlear Implantation in Patients with Keratitis-Ichthyosis-Deafness Syndrome: A Report of Two Cases

2017 ◽  
Vol 2017 ◽  
pp. 1-5
Author(s):  
Birgul Gumus ◽  
Armagan Incesulu ◽  
Mehmet Ozgur Pinarbasli
Keyword(s):  
Hearing Loss ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Pure Tone ◽  
Pure Tone Audiometry ◽  
External Ear ◽  
Ear Canal ◽  
External Ear Canal ◽  
Brainstem Response ◽  
Visual Disturbances

Background.Keratitis-ichthyosis-deafness (KID) syndrome is a syndrome which presents with hearing loss and visual and keratinization disorders. In such patients, hearing aids cannot be effectively used in the rehabilitation of hearing loss because of the frequent blockage of the external ear canal with epithelial debris and due to dry and tense skin of the external ear canal. Moreover, severe or profound hearing loss also limits the benefits gained from the conventional hearing aids. On the other hand, cochlear implantation is a method that has been used in limited cases in the literature.Case Report.This study presents the results of cochlear implantation applied in our clinic to two children who had been diagnosed with KID. Audiological assessments before and after the cochlear implant operation were performed using pure-tone audiometry, immittance audiometry, and auditory brainstem response (ABR), and the postoperative follow-up was conducted using pure-tone audiometry.Conclusion.Skin problems, visual disturbances, and other additional problems complicate the short-term and long-term rehabilitation after implantation in individuals with KID syndrome. Close monitoring should be exercised due to possible skin complications that may develop during the postoperative period. The families and rehabilitation teams should be warned about the possible visual disturbances and skin complications.

scholarly journals Audiological findings of a patient with H syndrome: case report

2021 ◽  
Vol 37 (1) ◽  
Author(s):  
Diala Hussein ◽  
Büşra Altın ◽  
Münir Demir Bajin
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Auditory Brainstem Response ◽  
Pure Tone Audiometry ◽  
Auditory Brainstem ◽  
Sensorineural Hearing ◽  
Brainstem Response ◽  
The Right

Abstract Background H syndrome is an autosomal recessive disorder caused by mutations in SLC29A3. Hyperpigmentation, hypertrichosis, hyperglycemia, and hearing loss are some characteristics of this disorder, and it has a prevalence of < 1/1000. The aim of this report is to spread awareness among otologists, audiologists, and pediatricians about this syndrome and its audiological features. Case presentation An 8-year-old male with a diagnosed H syndrome registered to our clinic with a complaint of hearing loss. The patient was diagnosed with hearing loss in a different clinic using only the air-conducted click auditory brainstem response test which showed wave V at 60 dB nHL for the right ear and at 80 dB nHL for the left ear. The initially performed pure tone audiometry (PTA) test in our clinic revealed a bilateral asymmetric hearing loss with a moderate sensorineural hearing loss in the right ear and a profound mixed hearing loss in the left ear. The performed air conducted click auditory brainstem response (ABR) result showed wave V at 55 dB nHL for the right ear and at 70 dB nHL for the left ear. Then, the repeated PTA test revealed a mild-severe sensorineural sloping hearing loss in the right ear and a severe sensorineural hearing loss in the left ear. Conclusion Although hearing thresholds in H syndrome could be within normal limits in some patients, sensorineural hearing loss is an important characteristic feature for this syndrome. Sensorineural hearing loss could be progressive or of sudden onset and ranges from mild to profound. Thus, it must be taken into consideration to apply the audiological follow-up regularly and paying attention to the patient’s complaints; also, a regular follow-up for language development of children with H syndrome and for the hearing aids is advised.

scholarly journals Contralateral Occlusion Test (COT): the effect of external ear canal occlusion with aging

CoDAS ◽  
2019 ◽  
Vol 31 (3) ◽  
Author(s):  
Luís Roque Reis ◽  
Luís Castelhano ◽  
Filipe Correia ◽  
Pedro Escada
Keyword(s):  
Hearing Loss ◽  
Tuning Fork ◽  
Age Groups ◽  
Sound Field ◽  
External Ear ◽  
Ear Canal ◽  
Occlusion Test ◽  
External Ear Canal ◽  
Hearing Thresholds ◽  
And Gender

ABSTRACT Purpose This study aimed to evaluate the effects of complete external ear canal occlusion on hearing thresholds with aging. The goal was to decide which tuning fork is more appropriate to use for the contralateral occlusion test (COT), in individuals of different ages. Methods Forty-two normal hearing subjects between 21 and 67 years were divided into three age groups (20-30 years, 40-50 years, and 60-70 years). Participants underwent sound field audiometry tests with warble tones, with and without ear canal occlusion. Each ear was tested with the standard frequencies (250, 500, 1000, and 2000 Hz). The contralateral ear was suppressed by masking. Results Hearing thresholds showed an increase as the frequency increased from 20.85 dB (250 Hz, 20-30 years group) to 48 dB (2000 Hz, 60-70 years group). The threshold differences between occlusion and no occlusion conditions were statistically significant and increased ranging from 11.1 dB (250 Hz, 20-30 years group) to 32 dB (2000 Hz, 20-30 years group). We found statistically significant differences for the three age groups and for all evaluations except to 500 Hz difference and average difference. The mean hearing loss produced by occlusion at 500 Hz was approximately 19 dB. We found no statistically significant differences between right and left ears and gender for all measurements. Conclusion We conclude that the use of the 512 Hz tuning fork is the most suitable for COT, and its use may allow clinicians to distinguish mild from moderate unilateral conductive hearing loss.

Outcome of 22 cases of perforated tympanic membrane caused by otomycosis

2001 ◽  
Vol 115 (11) ◽  
pp. 879-880 ◽  
Author(s):  
William B. Hurst
Keyword(s):  
Hearing Loss ◽  
Tympanic Membrane ◽  
Otitis Externa ◽  
Residual Hearing ◽  
External Ear ◽  
Ear Canal ◽  
Trichloracetic Acid ◽  
External Ear Canal ◽  
Discrete Area ◽  
Perforated Tympanic Membrane

Twenty-two cases of perforated tympanic membrane due to fungal otitis externa were observed over a five-year period.The diagnosis of fungal otitis externa was made on clinical grounds due to the obvious presence of fungal bloom in the external ear canal. Some perforations were noted at the first treatment after the fungal debris had been removed from the external ear canal using a microscope. Other perforations were observed to develop over a few days. Initially, a discrete area of the tympanic membrane appeared white and opaque. As time progressed the white area disintegrated, forming a perforation. Once the otitis externa had resolved most perforations healed spontaneously. Two that were observed to develop during treatment required a myringoplasty. Another one closed significantly but a tiny persistent perforation required cauterization with trichloracetic acid to encourage it to close over completely. The only residual hearing loss was in a case with almost total disintegration of the tympanic membrane requiring a myringoplasty.Treatment of fungal otitis externa for the patients in this series was aural toilet using suction under a microscope and insertion of a gauze wick saturated in a combination of hydrocortisone, clotrimazole, framycetin and gramicidin.

Current indications for cochlear implantation in adults and children

2019 ◽  
Vol 73 (3) ◽  
pp. 1-5 ◽  
Author(s):  
Witold Szyfter ◽  
Michał Karlik ◽  
Alicja Sekula ◽  
Simon Harris ◽  
Wojciech Gawęcki
Keyword(s):  
Hearing Loss ◽  
Sensorineural Hearing Loss ◽  
Hearing Aids ◽  
Cochlear Implantation ◽  
Pure Tone Audiometry ◽  
Sensorineural Hearing ◽  
Profound Hearing Loss ◽  
Unilateral Deafness ◽  
Bilateral Sensorineural Hearing Loss ◽  
Asymmetric Hearing Loss

Introduction: Surgical treatment of deafness by cochlear implants is used for more than 40 years, and during this period permanently, gradual and significant expansion of indications for this surgery has been observed. Material and methods: In our Department in the years 1994-2018 1480 cochlear implantations were performed, both in adults (647) and in children (883). In this study current indications and the rules for eligibility of patients based on 25 years of experience are presented. Results: Indications for cochlear implantation in adults are: 1) bilateral postlingual deafness, 2) bilateral sensorineural hearing loss - in pure tone audiometry > 70 dB HL (average 500-4000 Hz) and in speech audiometry in hearing aids understanding < 50% of words for the intensity of the stimulus 65 dB, in the absence of the benefits of hearing aids, 3) bilateral profound hearing loss for high frequency with good hearing for low frequency, in the absence of the benefits of hearing aids, 4) some cases of asymmetric hearing loss with intensive tinnitus in the deaf ear. An indication in children is bilateral sensorineural hearing loss > 80dB HL confirmed by hearing tests, after about 6 months of rehabilitation with the use of hearing aids. Discussion: Although cochlear implantation is used for more than 40 years, the indications for this treatment underlies constant modifications. They concern the age of eligible patients, implantation in patients with partially preserved hearing, as well as treatment for patients with difficult anatomical conditions. In many countries, bilateral implantations are commonly performed, and more and more centers recommend this treatment in the case of unilateral deafness or asymmetric hearing loss, especially with the accompanying tinnitus in the deaf ear.

scholarly journals Metastasis of Colonic Adenocarcinoma to the External Ear Canal: An Unusual Case with a Complex Pattern of Disease Progression

2005 ◽  
Vol 84 (1) ◽  
pp. 36-38 ◽  
Author(s):  
Henry J. Carson ◽  
Jeffrey S. Krivit ◽  
Stanley G. Eilers
Keyword(s):  
Hearing Loss ◽  
White Male ◽  
Previous History ◽  
External Ear ◽  
Ear Canal ◽  
External Ear Canal ◽  
History Of ◽  
Adenocarcinoma Of The Colon ◽  
Imminent Death ◽  
The Right

We report on a patient who developed far-ranging metastases of adenocarcinoma of the colon that followed a gradual cephalad progression, including the right external ear canal, and led to hearing loss. The patient was a 63-year-old white male with stage III adenocarcinoma of the colon. After 2 years with metastases elsewhere, he developed hearing loss on the right side. Physical examination of the head and neck showed a mass in the external ear canal, and biopsy confirmed adenocarcinoma. After removal, the patient's hearing improved. This case is interesting not only because of the unusual metastasis to the external ear canal, but also because of the patient's previous history of metastasis over the course of more than 2 years. The significance of such wide-ranging metastases is that metastasis of adenocarcinoma to the ear did not signal imminent death, and relief of the hearing loss it caused was possible.

scholarly journals The Role of Occlusion of the External Ear Canal in Hearing Loss

2019 ◽  
Vol 57 (3) ◽  
pp. 122-126
Author(s):  
Dakshaini A. Narayanan ◽  
◽  
Rajagopalan Raman ◽  
Aun Wee Chong ◽  
◽  
...  
Keyword(s):  
Hearing Loss ◽  
External Ear ◽  
Ear Canal ◽  
External Ear Canal

scholarly journals Threshold Mismatches between Pure-Tone Audiometry and Auditory Steady State Response in Non-Auditory Neuropathy Adults

2020 ◽  
Vol 63 (8) ◽  
pp. 350-357
Author(s):  
Heil Noh ◽  
Hyesook Lee
Keyword(s):  
Hearing Loss ◽  
Steady State ◽  
Pure Tone ◽  
Pure Tone Audiometry ◽  
Brain Lesions ◽  
Auditory Neuropathy ◽  
Steady State Response ◽  
State Response ◽  
Auditory Steady State Response ◽  
Brainstem Response

Background and Objectives To evaluate mismatches between pure-tone audiometry (PTA) and auditory steady-state response (ASSR) tests in non-auditory neuropathy adults and investigate brain lesions that may explain the mismatches, especially in cases where the ASSR threshold was worse than the estimated PTA threshold.Subjects and Method PTA, speech audiometry, auditory brainstem response, ASSR, and neuroimaging tests were carried out on individuals selected. Among them, medical records of 30 subjects (16 males, 14 females; mean age=54.4±13.2 years) with significant mismatches between PTA and estimated ASSR thresholds were analyzed retrospectively. All neuroimaging tests were reviewed to identify any neurologic abnormalities.Results Pathologic brain lesions were found in 19 cases (63.3%) in the study group, all of which showed significant mismatch in hearing threshold between PTA and ASSR. Seven case of ischemic brain lesions (23.3%), five tumorous lesions (16.6%), and four brain vessel anomalies (13.3%) were found. Brain hemorrhage due to trauma were found in two cases (6.6%) as well as one case (3.3%) of unruptured aneurysm. Central auditory pathway disorder was suspected in two cases. The 11 cases showing normal results in the imaging studies included one mental retardation, one brain concussion, and seven cases (23.3%) with no specific causes. Six tinnitus cases included two subjects showing discrepancies between frequency matching in the tinnitogram.Conclusion In adults with a mismatch between PTA thresholds and estimated ASSR thresholds, especially for those with moderate to profound hearing loss, additional neuroimaging tests and other audiologic tests may reveal other possible causes of hearing loss.

scholarly journals Impact of Hearing Loss and Amplification on Performance on a Cognitive Screening Test

2018 ◽  
Vol 29 (07) ◽  
pp. 648-655 ◽  
Author(s):  
Gabrielle H. Saunders ◽  
Ian Odgear ◽  
Anna Cosgrove ◽  
Melissa T. Frederick
Keyword(s):  
Hearing Loss ◽  
Hearing Impairment ◽  
Hearing Aids ◽  
Pure Tone ◽  
Pure Tone Audiometry ◽  
Cognitive Screening ◽  
Strong Correlations ◽  
Speech In Noise ◽  
Hearing Ability ◽  
The Individual

AbstractThere have been numerous recent reports on the association between hearing impairment and cognitive function, such that the cognition of adults with hearing loss is poorer relative to the cognition of adults with normal hearing (NH), even when amplification is used. However, it is not clear the extent to which this is testing artifact due to the individual with hearing loss being unable to accurately hear the test stimuli.The primary purpose of this study was to examine whether use of amplification during cognitive screening with the Montreal Cognitive Assessment (MoCA) improves performance on the MoCA. Secondarily, we investigated the effects of hearing ability on MoCA performance, by comparing the performance of individuals with and without hearing impairment.Participants were 42 individuals with hearing impairment and 19 individuals with NH. Of the individuals with hearing impairment, 22 routinely used hearing aids; 20 did not use hearing aids.Following a written informec consent process, all participants completed pure tone audiometry, speech testing in quiet (Maryland consonant-nucleus-consonant [CNC] words) and in noise (Quick Speech in Noise [QuickSIN] test), and the MoCA. The speech testing and MoCA were completed twice. Individuals with hearing impairment completed testing once unaided and once with amplification, whereas individuals with NH completed unaided testing twice.The individuals with hearing impairment performed significantly less well on the MoCA than those without hearing impairment for unaided testing, and the use of amplification did not significantly change performance. This is despite the finding that amplification significantly improved the performance of the hearing aid users on the measures of speech in quiet and speech in noise. Furthermore, there were strong correlations between MoCA score and the four frequency pure tone average, Maryland CNC score and QuickSIN, which remain moderate to strong when the analyses were adjusted for age.It is concluded that the individuals with hearing loss here performed less well on the MoCA than individuals with NH and that the use of amplification did not compensate for this performance deficit. Nonetheless, this should not be taken to suggest the use of amplification during testing is unnecessary because it might be that other unmeasured factors, such as effort required to perform or fatigue, were decreased with the use of amplification.

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