Two families were reported with congenital conductive hearing loss, hyperopia, broad thumbs and broad first toes. These families fall within the spectrum of an autosomal dominant syndrome with congenital stapes ankylosis first described by Teunissen B & Cremers CWRJ in 1990. Affected members of our first family also showed to have symphalangism, syndactyly and cervical spine fusion. Clinical distinction between Teunissen-Cremers syndrome and proximal symphalngism syndrome or facio-audio-symphalangism syndrome was discussed.
The A1555G and 961delT mutations in the mitochondrial 12S ribosomal RNA (rRNA) gene were found in patients with hearing loss after aminoglycoside administration. From among 9 patients with streptomycin-induced hearing loss, the 961delT mutation was found in one (11%) and the A1555G mutation in 6 (67%). Pure-tone audiograms showed moderate symmetrical sensorineural hearing loss in a patient bearing the 961delT, and the other audiologic findings suggested cochlear impairment. Four patients with A1555G mutation, 2 of whom had the cochlear implant had profound hearing loss. Clinical consideration of family histories and molecular screening at the 1555 and the 961 positions was thought to reduce the frequency of tragic irreversible deafness due to aminoglycosides. As buccal scrapes and urinary sediments are obtained non-invasively, these methods of checking for the above mentioned mutations are suitable for children.
A new grading system for acute low-tone sensorineural hearing loss (ALHL) was proposed. Each grade was classified by the summation of hearing levels at 125, 250, 500Hz (LT3) of pure tone audiometry. Grade 1 70dB≤LT3<100dB Grade 2 100dB≤LT3<130dB Grade 3 130dB≤LT3<160dB Grade 4 160dB≤LT3 The prognosis in grade 3-4 varied in each patient, while patients of grade 1 and 2 showed good prognosis. Recurrent cases in grade 3-4 showed better prognosis than cases with a single attack. These results indicated various pathophysiology of grade 3 and 4, and some of which may cause irreversible changes of the cochlea. The results of the present study suggested that our new grading system would be helpful for the initial evaluation of hearing prognosis.
The prognosis of acute low-tone sensorineural hearing loss (ALHL) is difficult to predict. Multiple logistic regression analysis was used in this study to determine which factors were most strongly related to outcome for patients with ALHL. The study group was comprised of 61 patients (61 ears). The first analysis was performed on the poor prognosis group (no change and partial recovery groups, 15 ears) and full recovery group (46 ears). The second analysis was performed on the recurrence group (14 ears) and non-recurrence group (32 ears). The following factors were examined as explanatory variables: age, sex, vestibular symptom, number of days before the start of treatment, therapy (corticosteroids, vasodilaters, diuretics, and vitamin B12 and satellite ganglion block), and initial hearing levels at 0.125, 0.25, 0.5 and 1kHz. Factors determined to be most strongly related to outcome were age, sex, hearing level at 1kHz and use of the diuretics. And the sex was also strongly related to the recurrence of ALHL. Our results will increase the ability to prognosticate the outcome for ALHL.
Acute low-sensorineural hearing loss (ALHL) is thought to be a subtype of idiopathic sudden hearing loss. We divided ALHL patients into 2 groups of recurrent and single episode. Retrospective analysis of 91 patients revealed that 54 of them belonged to recurrent group. Thirteen out of the 54 patients were subsequently confirmed to have difinite Meniere's disease; 12 involved unilateral ear and 1 involved bilateral. We performed electrocochleography (ECochG) on these 14 ears and found that 12 of them showed increased -SP/AP ratio to a 100dBpe SPL click. The duration between an onset of ALHL and the first vertigo attack varied from 2 weeks to 68 months. There were 6 patients who had vertigo for more than a year after the onset of ALHL. The prognosis of low-tone hearing was relatively good; 10 out of the 14 ears showed the improvement of hearing. Vestibular symptoms such as vertigo and dizziness after the confirmation of Meniere's disease were not severe. We concluded that not all the ALHL patients had endolymphatic hydrops, and ECochG could be a good tool for predicting the prognosis. Otologists should carefully diagnose and manage ALHL patients because ALHL possibly had the clinical entities other than endolymphatic hydrops.
The Research Committee on Acute Profound Deafness, the Ministry of Health, Labor and Welfare in Japan proposed new diagnostic criteria for acute low tone sensorineural hearing loss. Based on these criteria, nationwide epidemiological surveys were conducted in 2000. The results of 150 cases with unilateral hearing loss, including 104 females and 46 males with a mean age of 37.9 years were analyzed. The peak incidence by age was the fourth decade in male, the third decade in female. They frequently occurred from spring to summer, and were uncommon in winter. Outcome of hearing was better in patients who visited within a week from onset and under 30 years old than in those visited after a week and over 50 years old. No significant differences were noted in the other factors i.e. sex, the severity of hearing loss at the first examination, presence or absence of causes such as common cold, stress and fatigue, and of dizzy sensation.
The following audiological findings are widely accepted for the diagnosis of low tone sudden deafness (LTSD); total hearing levels at low frequencies (125Hz, 250Hz, 500Hz) more than 70dB, and that at high frequencies (2kHz, 4kHz, 8kHz) less than 60dB. Eighty-nine patients (typical LTSD) with total hearing levels at high frequencies less than 60dB (Group A) and 33 patients (acute sensorineural deafness like LTSD) with levels more than 65dB (Group B) were studied. All patients in group B had already had the fixed hearing impairement at high frequencies. All patients visited our clinic within one week after onset, from 1992 to 1999. There was no statistical difference in the degree of hearing impairement in each frequency between the two groups. There was no difference of clinical parameters such as sex, subjective symptoms or the prognosis of patients, except for age between the two groups. Both groups were considered to belong to the same category of LTSD. Acute sensorineural deafness like LTSD may occur in elderly people and in patients with fixed hearing loss at high frequencies. Some patients with acute sensorineural deafness like LTSD may have various types of audiogram, except for the ascending type.
In patients with acute low-tone sensorineural hearing loss (ALHL), treatment progress was compared in 2 groups of prednisolone, tapering gradually from 60mg/day for 7 days, administrated group and dexamethasone, tapering gradually from 10mg/day for 7 days, administrated group. In the prednisolone group, there were some cases in which the hearing acuity was once recovered then deteriorated in the midpoint of treatment, and these hearing levels were significantly deteriorated compared to the levels at the same day in the dexamethasone group. An endolymphatic hydropse was considered as an etiology because there are some cases with ALHL later developed Meniere disease. It was known that the aldosterone which was mineral corticoids induced endolymphatic hydrops. The possibility of the mineral corticoid activity of prednisolone which might affect the liquid control of endolymph was considered.
Acute low-tone sensorineural hearing loss (ALHL) is characterized by the following features; acute or sudden onset, obscure etiology and sensorineural hearing loss limited to low frequencies (125, 250 and 500Hz) without vertigo. The sum of hearing levels at low frequencies is 70dB or more, on the other hand, that at high frequencies (2000, 4000 and 8000Hz) is 60dB or below. But the cases with the sum of hearing levels at high frequencies 65dB or more, an analogue of ALHL, have not investigated enough. We compared ALHL (group A: 37 cases) with the analogue of ALHL (group B: 17 cases) clinically. The average age of group A was 37.8 years old and that of group B was 53.1 years old. The sum of three high-tone hearing levels before treatment was 40.9dB (group A) and 89.7dB (group B) respectively, that may be caused by the difference of the average age between the two groups. The improvement of the sum of three low tone hearing levels after treatment was from 114.5dB to 45.5dB (group A) and from 112.4dB to 44.4dB (group B) respectively. There was no significant difference between two groups. They may be treated clinically as the same disease.
A 38-year-old female suffering from sudden onset of postural severe headache and neck stiffness complained bilateral tinnitus in a few days later, which were relieved by lying flat. Her audiogram showed a bilateral low-tone sensorineural hearing loss (BLSHL) manifested by 15-20dB improvement in the supine position. Lumbar puncture revealed an opening pressure of 0mmH2O and MRI showed a diffuse pachymeningeal gadolinium enhancement with a decrease in the size of the ventricles, indicating spontaneous intracranial hypotension (SIH). BLSHL in SIH may manifest a relative endolymphatic hydrops as a result of a loss of perilymphatic fluid through the cochlear aqueduct and a compression of the 8th cranial nerve by the downward displacement of brain in the upright position following CSF leak.
We examined the hearing prognosis and hearing change in 32 ears of 30 patients with acute low-tone sensorineural hearing loss (ALHL) followed up for more than 1 year at the Department of Otolaryngology, Hyogo College of Medicine. The change of hearing loss in patients with ALHL was classified into 9 types by their clinical time courses. Six ears (18.7%) were suffered only once and all of them were cured. The recurrence of hearing loss occurred in 26 ears (81.3%) of 24 patients and 14 ears (43.8%) returned to the normal hearing level at each attack. The overall curative ratio of long-term follow up of ALHL was 62.8%. Five patients (15.8%) later developed Meniere's disease. Because the minimum interval of transition to Meniere's disease was 14 months, Meniere's disease will increase with length of the follow up. Prognosis of ALHL with long-term follow up was considered to be worse than that with short-term follow up reported in the previous studies. This suggested that the long-term follow up is needed to evaluate the clinical time course and prognosis in patients with ALHL.
We examined acute low-tone sensorineural hearing loss from the results of nation-wide epidemiological study of sudden deafness in 1993. There were 252 patients with acute low-tone sensorineural hearing loss among 2, 790 patients. There were 168 wemen and 84 men. The age of patients was 34.8 years, that was 13.3 years younger than that of patients with sudden deafness. When the interval between the onset of the disease and the first visit to the hospital was short, prognosis was generally good.