Sensorineural deafness due to cochlear otospongiosis: pathogenesis, clinical diagnosis, and therapy.
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Animals↗
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[Surgery of juvenile external ear abnormalities].
Surgery of moderate cup ear deformities, severe cup ear deformities and microtia is described. In cases of unilateral atresia auris we usually do not perform middle ear surgery. In cases of bilateral atresia the child is fitted with a bone conducting hearing aid as soon as possible and operated on when the child is 4 to 5 years old. After bilateral middle ear surgery we reconstruct the auricle at the age of 5 or 6 years.
Child↗
[Neuro-otologic findings in the Wernicke-Korsakoff syndrome].
The Wernicke-Korsakoff syndrome is induced by thiamine deficiency. It occurs mainly in subjects with chronic alcoholism. It begins with an acute phase (Wernicke's encephalopathy) and changes to a chronic phase, which is characterized by a symptomatology varying from subclinical findings to a fully developed Korsakoff psychosis. We examined otoneurologically eight patients with Wernicke-Korsakoff syndrome. The examination included: clinical status, caloric stimulation, optokinetic nystagmus, pure tone audiogram (air- and bone conduction), speech audiogram, and brain-stem auditory evoked potentials. The typical findings of the chronic phase are: gaze nystagmus and reduced gain of the optokinetic nystagmus. In five of seven patients OK gain was reduced by more than two standard-deviations. The other examinations including brain-stem potentials did not reveal any specific findings. The symptomatology of the acute phase is well known: nystagmus (mostly lateral gaze nystagmus) and reduced by caloric responses. Early diagnosis of a Wernicke-Korsakoff syndrome is of great importance since its treatment is simple and efficient. The results of a thorough neuro-otologic examination are of considerable diagnostic value, particularly during the chronic phase of the disease.
Adult↗
Inner ear hearing loss in acute and chronic otitis media.
Temporary or permanent threshold shift of bone conduction occurs frequently in acute or chronic otitis media. The sensorineural hearing loss is dependent on the age of the patient and the duration of the illness. Fluid in the middle ear may impede sound transmission and oxygen transport to the inner ear. In middle ear inflammation noxious substances may pass across the round window membrane leading to serous labyrinthitis. In therapy ototoxic drugs and operations (tympanoplasty) can cause sensorineural deafness.
Acute Disease↗
[Prescription of hearing aids - examples for limits and misindication (author's transl)].
For example there are described forms of hearing disorders in which the prescription of a hearing aid is inadvisible, ineffective or seeming unexpected good. However, an explanation for this resulted only from making use of all possibilities of audiometric diagnostic. This is illustrated in patients with endocranial tumors, with diffuse neural hearing disorder, and with psychogenic or psychogenic superimposed hearing impairment. The term of "inner ear hearing disorder", therefore, even in the prescription of hearing aids should not comprise all those impairments of hearing in which the values of air and bone conduction are identical; i.e. the medical audiologic examination cannot be restricted to the pure tone threshold. The speech-audiogram may give decisive hints; often, however, versatile additional examinations are necessary to get an impression of the patient's subjective auditory picture.
Acoustic Impedance Tests↗
[Acoustic variation after surgery for severe Bell's palsy].
Auditory thresholds were studied before and after surgical decompression of the facial nerve in 35 patients following either transmastoidal (20 cases) or transmastoidal and suprapetrosal (15 cases) operations. Seventeen of the patients were under 40 years of age at the time of surgery, which did not affect hearing in the opposite ear. However, auditory threshold levels fell in the operated ear. These were of the bone conduction type, and possessed characteristics of an acute hearing lesion (maximal in the 4000 frequency range and improving progressively) of 0 to 35 dB (mean 5 dB). Possible causes of the hearing loss are discussed.
Adult↗
[Hearing disorders in industrial workers: clinical analysis of the cases observed in the audiological laboratory of the Lódź Institute of Occupational Medicine during the years 1976-1980].
The documentation of 697 cases with suspected, as suggested by regional health service units, occupational aetiology, was analysed. Within consultation those cases underwent complex audiological evaluation at the Laboratory of Audiology, using following examinations: pure tone threshold and suprathreshold audiometry, malingering tests, speech audiometry and electronystagmographic tests. The findings revealed that only 30% of subjects confirmed noise-induced hearing loss. 65.2% of those subjects exhibited cochlear localization, whereas in the remaining 34.8% the results of audiological tests showed retrocochlear impairment. The calculated mean hearing losses were characterized by symmetrical changes both in the right and left ear, as well as by directly proportional dependence on subjects' duration and exposure and age, men having more advanced hearing loss than women, despite identical mean parameters of age and exposure of both sexes. In 70% cases hearing losses were not regarded as occupational diseases, which resulted from: functional disturbances found in form of aggravation and simulation (15.3%), the presence of hearing loss lesser than the one specified by the certification rule (15.1%), post--inflammatory and adhesional changes in the middle ear (12.3%) and lack of significant exposure to noise (8.3%). It has been settled that the major causes of the diagnostic errors that we had detected were: deficient audiometric apparatus, erroneous technics of hearing measurements (among others--without masking), lack of the determination of hearing thresholds for bone conduction, drawing conclusions from single examinations, giving up simulating tests, improper evaluation of the data on occupational exposure. The considerations summing up the results of analysis include an optimum methodology of audiological studies, required standard conditions of audiometry and principles of differential diagnostics as indispensable elements of proper certification procedures in setting up the hearing impairments etiology in industrial workers.
Adult↗
Evidence for auditory localization ability in the turtle.
Evidence is presented that the semiaquatic turtle Chrysemys scripta and the terrestrial turtle Terrapene carolina major can detect the direction of a tone within their sensitive area of hearing. It is further suggested that not only can these species respond behaviorally to sound without extensive manipulation but can use limited hearing in a problem-solving situation of maze learning. Adult emydid turtles (5 C. scripta, 3 T. carolina) learned a Y-maze with a 500-c/s signal to an invisible open goal box to avoid bright light. All animals performed above chance levels, but it required over 240 trials on the average to reach 60%-correct performance. Computations suggest that binaural cues used by mammals would not be adequately encoded by the primitive auditory systems of the species studied. It is further suggested that these turtles use bone conduction by coupling their ears to the substrate to hear vibrations in the immediate area. This would appear to be a carryover from the ancient reptile stem stock. The poor middle-ear impedance system relegates air-borne sound processing to be a somewhat insensitive limited low-pass system, depending heavily on monaural cues derived from head scanning. vocal output in these species appears to be spectrally imbalanced with their auditory sensitivity. The role of species-specific vocal signalling is unclear from the present data.
Animals↗
Clinical applications of the auditory brain stem response.
The auditory brain stem response is a powerful new tool for the detection and quantification of hearing impairment, especially in the pediatric population. It gives exact information about the functional status of the cochlea and brain stem pathways. The technique distinguishes recruiting from nonrecruiting losses, predominantly high frequency from flat losses, and retrocochlear from peripheral disorders. The recent introduction of bone conducted stimuli should soon permit the unambiguous separation of conductive and sensorineural losses.
Adult↗
[Reconstruction of the ossicular chain: Plastipore prosthesis compared with autogenous and allogenic ossicles (author's transl)].
Morphological and audiological early and late results after reconstruction of the ossicular chain with Plastipore prosthesis (TORP's and PORP's) were compared with those achieved by using autogenous or allogenic ossicles. In particular 32 tympanoplasties with TORP's were compared with 25 cases of columella and 29 PORP cases with 75 interpositions. Only Plastipore prostheses were extruded, up to now in 11.4% of cases. The final extrusion rate will be even higher since perforations of the fascia graft or the drum over the head of the prostheses as signs of imminent extrusions were noted in 9.8% of tympanoplasties at the end of the 2-year follow-up period. However, only in one third of tympanoplasties cartilage had been interposed between the head of the prosthesis and the fascia graft or drum. Audiological results, early as well as late ones, using the mean postoperative air-bone gap as criterion of success, showed no statistically significant differences between the compared groups. Only the comparison of the mean air-bone gaps in late results versus the preoperative values seemed to favour the PORP group over the interposition group. In some PORP cases, however, greater reduction of the air-bone gap was caused by postoperative deterioration of the bone conduction threshold level. In addition, hearing gains were more lasting in the interposition than in the PORP group. Because of the high extrusion rate and the fact that the audiological results were not significantly better than in the compared groups we will cease using PORP's and will employ TORP's only with careful interposition of cartilage and then only in selected cases with good tubal function.
Audiometry↗
Homograft tympanoplasty: graft material effects on otoadmittance and audiometric measurements.
Otoadmittance measurements and air- and bone-conduction thresholds were obtained for 20 subjects who underwent type I tympanoplasty. Component tympanometry, particularly with a high-frequency probe tone, demonstrated that homograft tympanic membrane transplants exhibited more normal function than did temporalis fascia grafts. Audiometric results revealed greater hearing improvement by the homograft transplant group.
Acoustic Impedance Tests↗
[Otosclerosis: stapedectomy or stapedotomy. A long-term comparative study. Apropos of 1279 cases].
The authors report 1279 surgical operations of otosclerosis performed between 1980 and 1992 in 959 operated patients. They chose the autegenous vein or perichondrium interposition TeflonR piston; however this technique has improved over the past few years. The size of the stapedectomy has been diminishing; from total, then partial stapedectomy, at last to 0.8 mm across stapedotomy. The graft has become exclusively from venous origin and the diameter of the piston has been reduced from 0.8 to 0.6 then to 0.4 mm. Seven groups of patients have been examined according to the size of the incision for stapedotomy or stapedectomy and the size of the piston. The audiometric study was realized after one month, one year, three years, fine or even ten years after surgery. Comparative tests were made considering the sex and the age of the patient, the thinness of the graft, and surgical revisions. The audiometric study lied not only upon the audiometric Rinne's closing but also upon the bone conduction variation (postoperative bc-preoperative bc) in the course of time. The evolution of tinnitus, of vertigo has been, as well, the subject of a careful study in time according to the surgical techniques. The study shows that the audiometric results (Rinne's closing, bc evolution) are statically much better with total stapedectomy, then with partial stapedectomy, at last with stapedotomy in the first three postoperative years. The best audiometric results are obtained with wider pistons (0.8 mm diameter) and venous approach. The results regarding tinnitus and vertigos are dissimilar especially during the first operative year. After three years of evolution, the significant audiometric differences tend to reduce and the audiometric results become the same (no significant difference) whatever the surgical technique may be. After three years, simple, calibrated stapedotomy without interposition statically gives similar results in literature. However, each surgical technique may, though rather infrequently, produce some incidents or complications that undoubtedly influence the operator as for the choice of the surgical technique to use.
Audiometry↗
[Tympanosclerosis--clinical and pathological investigation].
Fifty-nine cases of tympanosclerosis were investigated clinically and pathologically, and the following results were obtained: 1) The male to female ratio was about 1:1.8 2) Calcification in the tympanic membrane was most common in the upper quadrants of the pars tensa, and never seen in the pars flaccida. 3) Calcification in the middle ear cavity was most common around the malleus. 4) Chronic otitis media was the most common complication of tympanosclerosis. 5) Preoperative audiometry revealed a stiffness curve with elevated bone conduction thresholds. 6) 54% of the patient had chronic otitis media in the contralateral ear. 7) Microscopic examination revealed calcification in the submucosa of the middle ear. 8) Postoperative hearing was improved in 49 ears (79.7%). Because there was no difference in the average postoperative hearing gain after type I and type III tympanoplasty, type III tympanoplasty is recommended to remove sclerotic masses completely.
Adolescent↗
The validity of tuning fork tests in diagnosing hearing loss.
Tuning fork tests are subjective and response bias must be accounted for when determining their validity as diagnostic tools. A significant proportion of patients who present for otologic evaluation have mixed hearing loss and it is important to know how this group responds to tuning fork testing. The need for masking in audiometry is indisputable but its role in tuning fork testing has never been established. The purpose of this study was to determine the sensitivity of tuning fork tests. The Weber, unmasked and masked Rinne, and Bing tests were administered in a prospective, random, and blind manner to 68 patients referred for audiologic assessment. Patients found to have normal hearing served as the control group. Signal Detection Theory was used to compare the results to pure-tone air- and bone-conduction audiometry. Overall the Rinne is an unbiased test with a sensitivity of 0.84 regardless of the type, severity, or frequency of hearing loss. This is not improved by masking. There is no role for either the Weber or Bing as independent tests. Mixed hearing loss behaves as conductive hearing loss. The Rinne without masking should be administered to patients suspected of having a hearing loss.
Adolescent↗
New techniques of hearing assessment.
This article reviews current literature in the areas of otoacoustic emissions and auditory brain stem responses (ABRs) and their application to the evaluation of peripheral auditory function in infants and children. The different types of otoacoustic emissions are described along with their incidence, development, clinical applications, and interpretation in the pediatric group. The development of the auditory system, reflected by ABRs, is presented in detail with previously unpublished results from three-channel Lissajous' trajectory studies performed in infants. Clinical application of frequency-specific ABRs, both air and bone conducted, are presented. Finally, a discussion of the need to develop objective estimators of signal quality and threshold detection for otoacoustic emissions and ABRs in infants and children is presented, which includes previously unpublished results evaluating a new technique.
Auditory Perception↗
Management of labyrinthine fistulas in cholesteatoma.
Surgical management of labyrinthine fistulas caused by cholesteatoma remains controversial. In 213 patients with middle ear surgery for cholesteatoma, 18 patients (18 ears-8.5%) presented a labyrinthine fistula. These cases are reviewed in the present paper. Symptoms, audiometry, pre-operative imaging, surgical procedure and results are discussed. In all cases total removal of the pathology was pursued with preservation of cochlear function. A two-stage closed technique was used. During the first stage reconstruction with tympano-ossicular allografts, according to the technique of J. Marquet, was performed. A second look followed one year later. Three ears were deaf pre-operatively. Improvement of bone conduction was observed in more than 50% and hearing preservation in all other cases but one. In one ear total deafness occurred from extensive intralabyrinthine invasion by cholesteatoma, demanding a labyrinthectomy. The hearing was preserved in four cases in which an amputation of a large part of the membranous labyrinth could be observed. Since neither pre-operative clinical examination nor imaging can be relied on to reveal a fistula, the surgeon needs to be prepared for unexpected fistulas.
Adolescent↗
A communication from the People's Republic of China: management of labyrinthine fistula with cholesteatoma.
Management and results in 43 cases of labyrinthine fistula with cholesteatoma are presented and discussed. The choice of surgical procedure is based on the presence or absence of infection; the size and location of the fistula; condition of the other ear; cochlear function, and condition of the stapes. Unchanged bone conduction has been obtained in 88% of our 43 surgical cases. The planned staged operation has produced good functional results.
Adolescent↗
The otologic manifestations of mandibulofacial dysostosis.
Sixteen patients (32 ears) with diagnoses of mandibulofacial dysostosis were reviewed. The characteristic otologic manifestations of the syndrome were delineated, found to be bilateral, and consist of the following: 1. Mild symmetric deformity of the auricle (grade 1 microtia). 2. Agenesis or hypoplastic development of the mastoid and mastoid antrum. 3. Absence of the external auditory canal. 4. Marked narrowing or agenesis of the middle ear cleft. 5. Agenesis or severe malformation of the malleus and incus. When present, the malleus and incus are most often rudimentary, fused to form a conglomerate mass, and ankylosed to either the atretic plate, epitympanum, or both. 6. Stapedial malformations which usually consist of a deformed suprastructure. 7. Frequently the tegmen assumes a more inferior (low lying) position than normal. 8. Occasionally, the facial nerve pursues an abnormal course and is located more anteriorly than would be expected. 9. A normal inner ear. 10. Normal bone conduction with a marked (greater than 50 dB) conductive hearing loss. 11. Marked disparity between the degree of auricular deformity (mild; grade 1 microtia) and the degree of deformity of the remaining first and second branchial arch derivatives that constitute the external and middle ears (severe).
Adolescent↗