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[The diagnosis and treatment of bilateral congenital absence of stapes and oval window in two members of a family].

OBJECTIVE: To date, bilateral congenital absence of stapes and oval window in two members (elder brother and younger sister) of a family has not been reported previously in the literature to our knowledge. The etiology, diagnosis and management are hereby introduced and discussed. METHODS: The diagnosis was based on history, audiological examinations, high resolution CT scanning and/or the operation. The modified Lempert's fenestration operation of the horizontal semicircular canal was performed on one ear each of the elder brother and the younger sister. RESULTS: Postoperative hearing threshold was 25 dB for speech frequencies with a gain of 47dB for the elder brother, and hearing threshold of 28 dB with a gain of 52 dB for the younger sister. Following up one to eight years individually showed that their hearing gains were steady. CONCLUSION: Patients' parents are relatives of first cousin and have two sons and one daughter. However, there are two family members suffered from bilateral congenital absence of stapes and oval window, and these malformations might be related to inheritance. Good bone conduction and Carhart's notches revealed bilaterally in the patients' audiograms. These are the important indications for diagnosis of this disease. The modified Lempert's fenestration operation of the horizontal semicircular canal is a safe and good choice for treatment.

Adolescent↗

[Failure of regain full function after surgery for otosclerosis: causes, diagnosis and treatment].

OBJECTIVE: The aim of this study was to evaluate the causes, the diagnosis, the treatment and the results obtained by revision surgery, in cases of stapedectomy failures. MATERIALS AND METHODS: Retrospective study of 50 recent cases operated on between January 1997 and December 2001. DIAGNOSIS OF THE FAILURE: All reoperated patients had clinical and audiological assessment; CT scan with virtual endoscopy. RESULTS: The mean time of onset of failure was 23 months. The mean preoperative air bone gap was 25.5 dB. Otoscopy revealed a retraction pocket caused by poor eustachian tube function in 9 cases. CT scan proved to be very effective at differentiating the cause of the failure. OPERATIVE FINDINGS: In 11 cases the prosthesis was too short, in 8 cases the prosthesis had migrated out of the hole of stapedotomy and in 6 cases the piston was fixed in the stapedotomy hole. A partial or complete lysis of the long process of the incus was frequently associated, but in 9 cases it was the only cause of the failure. In all the cases when the piston was displaced, the stapedotomy was found to be covered by a thin mucous membrane, avoiding labyrinthine fistula. In 3 cases, the failure was due to recurrent otosclerosis. In 5 cases the failure was due to a local anomaly at the level of the oval window niche, 2 cases of failure were due to a malleus ankylosis. In 5 cases fibrous adhesion was found between the incus and the mucosa of the promontory. In one case a reparative granuloma was found at the level of the oval window. TREATMENT: In cases of partial lysis of the long process of the incus, a new prosthesis was placed in a 0.4 mm diameter stapedotomy, performed using a KTP laser. A 0.4 mm diameter piston was extended 0.2 mm below stapedotomy to avoid a new extrusion. Indeed some prosthesis extrusion could be due to increased movements of the ossicular chain in cases of eustachian tube dysfunction. In cases of complete lysis of the long process of the incus, or in cases of a very short long process of the incus, a piston was put in the stapedotomy and attached to the malleus manubrium. The results of revision stapedotomy were favorable in the absence of associated fibrous tissue adhesion or local malformation. The air bone gap was found to be less than 10 dB in 40 cases and between 10 and 20 dB in 8 cases. An impairment of the air bone gap was found in 2 cases. No case of bone conduction impairment was found in this series.

Adult↗

[The measurement of cochlear flow by laser Doppler in man: the preliminary results].

Laser-Doppler flowmetry is presently one of the methods of choice in measuring cochlear blood flow. The techniques is non-invasive and is based on the frequency shift of the laser beam induced by the red blood cell movement. Previous studies of cochlear blood flow carried out on animals and humans demonstrated the reliability of laser-Doppler flowmetry and its usefulness in understanding inner ear microcirculation physiology. In this paper we present preliminary data obtained from three patients examined under general anesthesia while undergoing tympanoplasty. Results showed that tracings, whose baseline is proportional with the blood flow, are characterized by waves correlated to pulse beat and automatic ventilation. Moreover, intrinsic contractions of inner ear vessels (waves of vasomotion) as exist in cerebral microcirculation, were observed. Pharmacological hypotension hypocapnia and the application of epinephryne determine a significant reduction of cochlear blood flow. These results suggest that while cochlear blood flow is related to systemic pressure, it has an intrinsic control system. As well since we did not obtain any modification in bone conduction threshold after surgery, we conclude that laser-Doppler flowmetry is a safe technique.

Adolescent↗

[Experiences with incus autografts and preserved incus homografts in ear surgery (author's transl)].

The healing process of free-transplantated incus autografts and preserved homografts in the middle-ear is largely non-irritating. They are suited for sound transmission. Under the osteoinductive influence of the non-vital graft, ingrowing vascular connective tissue of the host differentiates itself in osteoblasts, osteoclasts and chondroblasts. The grafts are revitalised in relation to time. Rarely a marked resorption of preserved incus homografts was noticed in consequence of an antigen-antibody-reaction between the host and the implant which, however, never occurred in autografts.

Antigen-Antibody Reactions↗

Noise-induced low- and high-frequency hearing losses in Finnish conscripts.

Thirty-nine otologically healthy military conscripts were examined at the beginning and at the end of their 1-year service. On entry, they all showed normal findings during clinical otolaryngological examinations. The test battery included both conventional (0.25-8 kHz) and high-frequency "electric bone-conduction" (0.5-20 kHz) audiometry (EBC). The median pure-tone right ear thresholds at the end of service were 5 dB worse over the frequency range of 2-8 kHz compared with the thresholds at the beginning of the service. The difference was statistically highly significant (p = 0.00035). The median left ear pure-tone thresholds at frequencies of 0.25, 2, and 8 kHz were 5 dB worse at the end of the service compared to the thresholds at the beginning of the service. However, the difference was not statistically significant (p = 0.23). The median curves for the high-frequency (EBC) thresholds measured after the service showed worse thresholds over the whole frequency range compared to the median curves measured before the service. The greatest difference was seen in the highest frequencies (15-17 kHz) in both ears. The differences were statistically significant in both ears (p = 0.03 in the right ears; p = 0.01 in the left ears) when threshold values over the whole frequency range were analyzed. Since the otolaryngological history of these conscripts was uneventful during their service, the hearing deteriorations were considered to be caused by shooting practice.

Adolescent↗

Long-term inhibition of tinnitus by UltraQuiet therapy: preliminary report.

Masking of tinnitus by noise can produce residual inhibition, a persistence in the reduction in tinnitus after the noise is removed. Typically, this relief is very short-lived, on the order of minutes. This report highlights long-term inhibition of tinnitus by UltraQuiet therapy, a new technique that employs patterned sound in the 10- to 20-kHz range, presented through bone conduction. Nine subjects participated in a study of the efficacy of this tinnitus suppression technique. Eight reported improvement in tinnitus symptoms; one did not complete the study. The duration of the improvement ranged from days to weeks. This long-term inhibition may involve a truly plastic change in the brain at the central level.

Adult↗

Use of high-frequency and muscle vibration in the treatment of tinnitus.

Although tinnitus is defined as an internal auditory sensation, external auditory stimuli can mask tinnitus under some circumstances. High-frequency vibration delivered as bone conduction stimulation is effective in masking high-pitched tinnitus. In this preliminary report, somatosensory stimulation in the form of low-frequency muscle vibration can also mask high-frequency tinnitus. Somatosensory stimulation provides fast, immediate relief, whereas high-frequency vibration provides longer-lasting benefit. Either modality can stand alone or can be used in conjunction for tinnitus treatment. A clinically feasible technique has been identified for more wide-scale evaluation.

Adult↗

[Labyrinthine fistulas: surgical management].

The authors presented management rules and the results of surgical treatment in perilymphatic fistulas. Materials comprise 7 cases of cholesteatoma and 6 cases after previously performed so called conservative modified operation of the ear. In 3 cases cholesteatomas were destroying the membranous structures or were being adherent to them. In these cases labyrinthectomy was performed with partial conservation of hearing in one patient. In the remained 10 cases the fistulas were cleaned and sealed with 3 layers of tissues--fascia, cartilage and fascia. After the ossicular chain was reconstructed in one stage. Discharge, and vertigo were stabilised in all cases. It was stated that cleaning of the labyrinthine fistula during cholesteatoma removal does not influence on bone conduction thresholds.

Adolescent↗

Hearing results in stapes surgery using two different prosthesis.

AIM OF THE STUDY: Evaluation of hearing results after implantation of a fluoroplastic-platinum piston (FP) and of a titanium piston (T) with a shaft diameter of respectively 0.6 mm and 0.4 mm, in cases of otosclerosis requiring stapedotomy. MATERIAL AND METHODS: Pre-operative and post-operative hearing results obtained after primary stapedotomy by implantation of 30 FP and 30 T performed by the same expert author (C.Z.) were reviewed. In each patient we evaluated pre- and post-operative auditory thresholds, as recommended. RESULTS: All patients of both groups showed a significant air-bone gap (ABG) improvement for all frequencies after surgery ( P < 0. 001). Post-operative ABG comparison between the two groups showed a better ABG for lower frequencies in the FP group and for higher frequencies in the T group, but the difference was not significant. No post-operative complications, including sensorineural hearing loss, were found. Bone conduction improvement was better in the FP group than in the T one and this difference was statistically significant at 1000 and 2000 Hz. There was no statistically significant difference in the post-operative outcomes between the two prosthesis. Better results of FP for lower frequencies suggest that an increase in diameter of the prosthesis results in a greater improvement in the hearing threshold at low frequencies, while a decrease of diameter results in a greater improvement in the hearing threshold at high frequencies, as indicated by previous international studies. CONCLUSION: Our data shows that titanium piston is a as good as fluoroplastic piston in stapes surgery for otosclerosis.

Adult↗

[The evaluation of cartilage and perichondrium grafts for the reconstruction of the tympanic membrane].

Since the publication of Miodoński, Zollner and Wullstein different methods and grafting materials have been promoted in tympanoplastic surgery. The purpose of this study was to demonstrate the anatomical and functional results of tympanoplastic surgery with the use of cartilage and perichondrium grafts. The studies included a selected group of 112 patients who were operated on because of perforation of tympanic membrane. The analysed group consisted of 112 patients when perichondrium and cartilage were used to reconstruct the tympanic membrane. In all cases before and after operation there was made tonal audiometry with indication for air and bone conduction within range from 500 to 4000 Hz, verbal audiometry after operation with indication of speech detection and speech reception threshold using mono-syllable NLA-93 test, tympanometry after operation and evaluation of anatomical results after surgery.

Adolescent↗

[Results of myringoplasty and type I tympanoplasty with the use of fascia, cartilage and perichondrium grafts].

Since the publication of Miodoński, Zollner and Wullstein different methods and grafting materials have been promoted in tympanoplasty. The purpose of this study was to demonstrate the anatomical and functional results of tympanoplasty in comparison with the material used. The studies included a selected group of 142 patients who were operated on because of perforation of tympanic membrane. The analysed group consisted of 112 patients when perichondrium and cartilage were used to reconstruct the tympanic membrane. The comparison group consisted of 30 patients when fascia of the musculus temporalis was used to close a defect of the eardrum. In all cases before and after operation there was made tonal audiometry with indication for air and bone conduction within range from 500 to 4000 Hz, verbal audiometry after operation with indication of speech detection and speech reception threshold using mono-syllable NLA-93 test, tympanometry after operation and evaluation of anatomical results after surgery. The comparison of operation results showed that there was no significant difference between the two groups.

Adolescent↗

Preservation of tap vestibular evoked myogenic potentials despite resection of the inferior vestibular nerve.

Sound and skull-tap induced vestibular evoked myogenic potentials (VEMP) were studied in a 43-year-old man following inferior vestibular neurectomy. Surgery was performed because of a small acoustic neuroma. Postoperative caloric testing suggested sparing of superior vestibular nerve function on the operated side. In response to sound stimulation there were no VEMP on the operated side, irrespective of whether sounds were presented by air- or bone-conduction. This suggests sound-induced VEMP to be critically dependent on inferior vestibular nerve function and this is in agreement with present knowledge. However, VEMP were obtained in response to forehead skull taps, i.e. positive-negative VEMP not only on the healthy side but also on the operated side. This suggests remnant vestibular function on the operated side of importance for forehead skull tap VEMP, because with complete unilateral vestibular loss there are no (positive-negative) VEMP on the lesioned side. Thus, forehead skull-tap VEMP depend, at least partly, on the superior vestibular nerve function.

Acoustic Stimulation↗

Depressive disorders in relation to neurootological complaints.

Depression is a state of depressed mood characterized by feelings of sadness, despair, and discouragement. Depression ranges from normal feelings of "the blues" through dysthymia to major depression. Endogenous depression has been identified with a specific symptom complex: psychomotor retardation, early morning awakening, weight loss, excessive guilt, and lack of reactivity to the environment. Reactive depression is precipitated by a stressful life event. In the field of depression, we found an overlapping activity between psychiatry and neurootology. Our sample comprises 134 patients (53 [39.55%] male, 81 [60.45%] female) who were classified either by psychiatrists or by neurologists as suffering from depression. By evaluating our neurootological history data bank (Neurootological Data Evaluation-Claussen [NODEC]) as regards 6 important vertigo symptoms, we found that patients presented with a frequency of 2.10 signs per patient. When we extended the list to 11 vertigo and nausea signs, we found 2.93 signs per patients. All patients underwent an objective and quantitative neurootometric analysis. The following rates of abnormal findings were observed: butterfly calorigram of polygraphic electronystagmography, 69.40%; stepping craniocorpograms, 69.40%; and bone-conduction pure-tone audiometry of the right ear, 28.36%, and of the left ear, 36.57%.

Audiometry, Pure-Tone↗

[The evaluation of distortion product otoacoustic emissions after stapedotomy].

34 patients with otosclerosis operated at the ENT Clinic, were enrolled in this study. Prior and 4 months after stapedotomy the signal of DPOAE and the threshold levels for air and bone conduction were evaluated. No signal of otoacoustic emissions was registered in any of the patients prior stapedotomy. After operation DPOAE was registered in 23 out of 34 examined patients (67.6%).

Adolescent↗

Hearing loss in children with type 1 diabetes.

OBJECTIVE: To examine the auditory function in a group of children with type 1 diabetes, and to study the association between hearing impairment and duration of illness, metabolic control and diabetic complication. METHODS: Sixty-three diabetic patients below the age of 18 attending the university hospital in Khartoum, Sudan were investigated together with 63 age and sex matched non-diabetic controls. Pure-tone audiometric tests were performed using an Amplaid 300 clinical audiometer in a soundproof room. Both air and bone conduction were tested at frequencies between 250-8000 Hz and 250-4000 Hz respectively. Hearing impairment was noted at auditory threshold above 25 dB in any frequency and the magnitude of hearing loss was assessed according to auditory threshold in conversational frequencies only. RESULTS: The hearing acuity was lower in the diabetic patients than in the control subjects in all tested frequencies, but the differences achieve statistical significance only at middle and high frequencies. The hearing loss was symmetrical, generally mild, and affects both sexes equally. Duration of diabetes, HbA1c concentration, and angiopathic complications showed positive correlation with the increased hearing thresholds; while, age at onset, insulin dose per day, presence of neuropathy, and frequency of DKA and hypoglycaemic episodes were not associated. CONCLUSION: Hearing loss occurs early in diabetic children and is related to the duration of the disease and the degree of metabolic control. Strict glycemic control might prevent or delay this complication.

Adolescent↗

[Ossicular reconstruction with autograft in type III].

OBJECTIVE: The prostheses known as biocompatible are usually proposed for columellar repair in absence of stapes but at which cost and which long-term tolerance? It appeared useful to study the possibilities of autograft ossicular reconstruction (incus and cortical bone) in absence of suprastructure of the stapes. MATERIAL AND METHODS: Retrospective study for 82 operated patients for cholesteatoma with lysis of the cruras of the stapes. Columellar repair was obtained by prosthesis, columella of cortical mastoid bone, and more often autograft of incus (54 cases). The technique of Autogreffe Tympanum-Cartilage-Os-Platinum (ATCOP) (Autograft Tympanum-Cartilage-Bone-Footplate is described: tympanic repair by fascia and cartilage from the concha is made at the first surgical step. Type III ossiculoplasty is performed at the second look. The distance tympanum-footplate is then lower than 6 mm and the body of the incus, preserved as a spare ossicle in the mastoid, has a sufficient length to be interposed in between new drum and footplate. RESULTS: 78% of the patients have final air bone gap less or equal than 30 decibels. The average post op air bone gap is 23 decibels with incus while it was 42 decibels before surgery. Average gain is 19 decibels. The cost of autograft is null and tolerance is excellent. CONCLUSION: Patient's incus is usable in type III ossiculoplasty thanks to a cartilage graft of the tympanic membrane. Patient's ossicle is a material of choice for columellar repair even in absence of the suprastructure of the stapes. Prostheses in biomaterial appear justified in case of absent or destroyed incus.

Adolescent↗

[Results of surgical treatment for advanced otosclerosis].

OBJECTIVE: To assess the effect of stapes surgery on advanced otosclerosis (AO). METHODS: In 300 cases randomly collected of otosclerosis in 1970 to 1999, 68 cases (77ears) were selected for retrospective analysis, which met the criteria of advanced otosclerosis (mixed deafness, with bone-conduction levels exceeding 40 dB and air-conduction levels exceeding 70 dB in 500 - 2000 Hz). RESULTS: The air-conduction of sixty-eight cases (77 ears) were from 77.32 dB to 53.7 dB (500-2000 Hz) after operation, mean decreased 23.62 dB. Of 71 ears (92.21%) obtained air-conduction improved over 10 dB, of 46 ears (59.74%) gained A-B Gap closure in 10 dB. Air-conduction were from 79.01 dB to 58.23 dB (500 - 4000 Hz) after operation and mean decreased 20.78 dB. Of 68 ears (88.31%) obtained air-conduction improved over 10 dB, of 32 ears (41.56%) gained A-B gap closure in 10 dB. After followed up 5-25 years, of 67 ears were retained stabilization, of 28 ears obtained more improve than postoperation. But 4 ears had drop (all was stapes-elevation and re-improvement after them was reviewed). CONCLUSION: Stapes surgery is effective operation to cured otosclerosis, advanced otosclerosis or far-advanced otosclerosis had greater help to improved hearing.

Adult↗

[Diagnostic methods and clinic application for mtDNA A1555G and GJB2 and SLC26A4 genes in deaf patients].

OBJECTIVE: To establish the method of clinic genetic testing for common deaf genes such as mtDNA nt1555, GJB2 gene and SLC26A4 (Pendrin's syndrome gene, PDS) gene. METHODS: Three hundred and sixty seven sporadic patients with hearing loss from out-patient department of General Hospital of Chinese People's Liberation Army, 60 patients with history of maternal inherited hearing loss from 27 family, 20 congenital deaf patients from special educational school for deaf and dumb, 3 deaf patients with enlarged vestibular aqueduct (EVA) confirmed by CT scan, 50 control individuals with normal bone conductive hearing were analyzed. The genetic testing kit for mtDNA A1555G mutation was used to detect mtDNA A1555G mutation. The whole gene sequencing were accomplished in 20 congenital deaf patients. In 3 patients with EVA, fragments covering all exons of PDS gene were analyzed by denatured high productive liquid chromatogram and special exons were sequenced when DHPLC showed abnormal wave patterns of amplicons covering these exons. RESULTS: Fifty nine patients from 26 family and 5 sporadic patients were found to carry mtDNA A1555G mutation. Among 20 congenital deaf patients, 2 cases were found to have homozygous GJB2 235 del C mutation, 1 case had compound 235del C and 299-300 del AT mutation. Other 2 cases carried heterozygous 109 A-G mutation. Among 3 patients with EVA, 1 case was found to have heterozygous PDS G316X mutation and other 2 cases had homozygous 919-2 A-G mutation. CONCLUSIONS Genetic testing for deafness is feasible procedure with remarkable clinic significance.

Adolescent↗