Simultaneous bilateral sudden deafness. Report of a clinical case

Bilateral sudden deafness is uncommon and characterized by an acute sensorineural hearing loss in both ears of 30 dB or more in 3 consecutive frequencies. We report the case of a 57 yeras old female with this diagnosis who presented a right anacusia and a severe audiometric loss on left ear without vestibular pathology associated. She was admitted and treated by the protocol of medical therapy that we have performed in our E.N.T. Department (4 days EV and then 10 ones oral medication) with improvement in her audition 25-30 dB average. If sudden bilateral sensorineural deafnes is founded, we must consider an immuno-mediated inner ear disease (IMIED).     

Klippel-Feil syndrome and conductive deafness

The Klippel-Feil syndrome is usually associated with sensorineural deafness and reports of conductive or mixed deafness are rare. Four additional cases are presented in this paper. The middle ear anomalies found in this syndrome are discussed. The results of middle ear surgery are reviewed and found to be poor.     

Bone conductive implants in single-sided deafness

Conclusion: Bone conduction implants (BCIs) have been shown to partially restore some of the functions lost when binaural hearing is missing, such as in subjects with single-sided deafness (SSD). The use of a single BCI needs to be recommended by a clinician based on thorough counselling with the SSD subject. Objectives: To perform an overview of the present capabilities of BCIs for SSD and to evaluate the reliability of the audiological evaluation for assessing speech recognition in noise and sound localization cues, which are major problems related to the loss of binaural hearing. Methods: Nine subjects with SSD who received BCI implants underwent a preoperative audiological evaluation that included sound field speech audiometry, word recognition score (WRS) testing and sound localization testing in quiet and in noise. They were also tested for the accuracy of their directional word recognition in noise and their subjective perceptions of their hearing difficulties using the APHAB questionnaire. Results: The mean maximum accuracy of word discrimination was 65.5% in the unaided condition and 78.9% in the BCI-aided condition. Sound localization in noise was better with the BCI than in the unaided condition, especially when the stimulus and noise were presented on the same side as the implanted ear. The accuracy of directional word recognition showed an improvement with the BCI with respect to the unaided condition on the BCI side, with either the stimulus in the implanted ear and the noise in the contralateral ear or with both the stimulus and noise presented to the implanted ear.     

Diagnosis of conductive deafness by static compliance

The diagnosis of the ossicular condition, fixation or interruption, is often not achieved by the tympanometry, because the overlap of the distribution of the static compliance (SC) is so great with the normal group in these two groups. Therefore, it has been said that SC, by itself, has little diagnostic value for differentiating the ossicular lesion. The overlap of the distribution of SC, however, was much smaller between the group with ossicular fixation and the one with ossicular discontinuity. So, apart from deviation from the normal range of SC, the limiting value of SC for discriminating the two groups was determined in ears with conductive hearing loss. The subjects were fifty-one ears with ossicular fixation and seventeen ears with ossicular discontinuity, in which ossicular lesions were surgically confirmed. All ears showed intact tympanic membrane. The limiting value of SC was 0.9 cc. The internal check using this discriminant value showed high ratio of discrimination, i.e. 94.1% in the fixation group and 64.7% in the discontinuity one were diagnosed correctly. Therefore the author proposed this value as the new criteria to differentiate type As ("s" mean sclerosis) tympanogram from type Ad ("d" means discontinuity) one. In addition, the comparison of SC with the normal side in twenty-five unilaterally affected ears showed smaller SC in 75% of the fixation group and larger SC in 92.3% of the discontinuity one than the normal side. Thus, it can be said that SC have useful value in separating ossicular fixation from ossicular discontinuity, if one compares ears with both the intact tympanic membrane and conductive hearing loss.     

非感染性传导性聋动物模型

目的 构建非感染性传导性聋的动物模型,并观察听骨链活动与膜迷路积水的相关性.方法 选用健康白色豚鼠20只,右耳为手术耳,左耳为对照耳.手术方法经上鼓室进路,通过填塞骨水泥、磷酸锌牙科水泥固定听骨链.术后1、2个月,观察听神经复合动作电位阈值的变化.留取耳蜗做冰冻切片观察.结果 手术耳听泡内无炎症表现,手术耳较未手术耳复合动作电位阈值明显提高,冰冻切片显示耳蜗无炎症细胞浸润,电生理检查无膜迷路积水的电生理表现.结论 成功构建非感染性传导性聋动物模型,听骨链固定短期内不能引起膜迷路积水.     

Acute bilateral total deafness complicating mumps

While it is well known that mumps is a potential cause of deafness, it is not generally appreciated that it can produce total deafness. The damage is usually unilateral but bilateral disease has been recorded. A case is presented of severe bilateral hearing loss during a mumps epidemic in which, in spite of intensive conventional as well as lesser known therapy, no improvement occurred. In the absence of a successful therapeutic regime it is clearly advisable to intensify immunization in non-developed countries.     

Sudden-onset bilateral deafness revealing early neurosyphilis

IntroductionRapid-onset of sensorineural hearing loss in a patient at risk of genital or oral exposure to treponema can be secondary to early neurosyphilis, for which delayed treatment may result in irreversible sequelae.Summary of the case reportA 40-year-old, HIV seropositive man with a CD4 lymphocyte count greater than 500/mm³ presented with rapid-onset of bilateral sensorineural hearing loss over a period of one week. Otorhinolaryngological examination was normal. The audiogram showed bilateral hearing loss of 25 and 30 decibels, respectively. He subsequently developed loss of visual acuity, leading to the diagnosis of syphilitic meningitis affecting the optic and auditory nerves.DiscussionIn about one half of cases, neurosyphilis is an early manifestation of the disease occurring several weeks or months after contamination. Rapid- or even sudden-onset of hearing loss may be due to auditory neuritis. Clinical interview and syphilis serology in a patient at risk of exposure can allow rapid diagnosis and treatment, consisting of two weeks parenteral penicillin. Recovery of hearing loss is inconstant but can be complete.     

Spontaneous CSF otorrhoea — Presenting as conductive deafness

Spontaneous CSF otorrhea specially in older age is a rare condition. Congenital inner ear malformation is one of the commonest causes in pediatric age group and either because of trauma or spontaneously it comes out into middle ear. In pediatric age group usual presentation is with history of meningitis while adult patients may present as watery discharge from nose or ear. This is a case, presented with conducted deafness with negative middle ear pressure. Grommet was put presuming secretory otitis media but B2-transferrin levels of the secretion confirmed it as a CSF otorrhea. He was operated with past aural transmasioid approach. The defect was sealed in layers. Patient is in regular follow up is asymptomatic and without any recurrence.     

Aetiology of bilateral sensorineural deafness in children

The study was carried out in children born during the four year period 1981-1984 in the Greater Manchester County. The children were investigated for possible congenital infections. Perinatal assessment was carried out for adverse aetiological factors. Family histories were obtained and parents and siblings were examined for any hearing impairment. A total of 164 cases were investigated. Various aetiological groups were formed which showed the following distribution: cause unknown 36.5 per cent; genetic group 20.1 per cent; adverse perinatal factors 14.6 per cent; congenital infections 9.8 per cent; meningitis 6.1 per cent; chromosomal abnormalities 3.7 per cent; syndromal group 3.7 per cent; and miscellaneous 5.5 per cent. The findings are discussed in the light of previous studies.     

Involvement of the incudostapedial joint anomaly in conductive deafness

CONCLUSION: The outcome of surgery depends on complexities of middle ear anomalies and definite diagnosis can only be achieved during exploratory tympanotomy. We must be aware that the pathology of the congenital ossicular anomalies is variable and careful surgery is needed for hearing improvement. OBJECTIVE: This study aimed to investigate congenital ossicular malformation. SUBJECTS AND METHOds: Fifteen cases of ossicular anomalies without external ear malformation were studied. The anomaly of the incus-stapes complex was the most frequent. There were two cases of fused incudostapedial (IS) joint, which is an extremely rare occurrence. Case 1 was a 33-year-old man, in whom otosclerosis was suspected and exploratory tympanotomy was performed. The IS joint was fused, and the stapes was immobile. Small fenestra stapedectomy was performed. In case 2, a 52-year-old woman, otosclerosis was also suspected and exploratory tympanotomy was performed. The IS joint was fused and the incus was fixed. After the long process of the incus was cut, the stapes became mobile. However, since the incus remained immobile, it was removed and was placed on the stapes. In both cases, the hearing improved after surgery. The averaged hearing gain of 15 cases was 28.8 dB.     

Sudden unilateral deafness after bilateral knee replacement

This case report presents profound unremitting hearing loss in a 76-year-old man after having a bilateral knee replacement. There have been no other cases of permanent post-operative deafness after joint replacement in the world literature. Sudden post-operative deafness is rare. We conclude that this man's deafness is due to a fat embolism in his auditory circulation.