Sudden Hearing Research Articles

Conservative management of endolymphatic sac tumors in von Hippel–Lindau disease: case report

In endolymphatic sac tumors associated with von Hippel-Lindau disease, early detection and surgery have been warranted to avoid associated neurological morbidity. However, in lately discovered tumors, hearing preserving surgery is often impossible and timing of surgical resection is difficult to define. We report two cases of tumors revealed by a sudden and profound hearing loss and managed conservatively for more than 15 years without worsening of the neurological symptoms associated with the endolymphatic sac tumor. Tumor size remained stable for the first patient and a stuttering growth pattern was observed for the second patient. Initial observation may be considered a not unreasonable management paradigm in these cases.

Sudden Profound Bilateral Hearing Loss Associated with Polysubstance Narcotic Overdose

Sudden Profound Bilateral Hearing Loss Associated with Polysubstance Narcotic Overdose

めまいと急性感音難聴で発症した小脳橋角部髄膜腫例

めまいと急性感音難聴で発症した小脳橋角部髄膜腫例

A case of hearing loss associated with pegylated interferon and ribavirin treatment ameliorated by prednisone

A 51-year-old white man with chronic obstructive pulmonary disease who was infected with HCV genotype 2b presented with sudden left-sided hearing loss. He had been taking pegylated interferon (PEG-IFN) and ribavirin treatment for 6 weeks without any notable adverse effects. He had also been taking naproxen for 2 years for the treatment of chronic back pain. Physical examination, laboratory tests, including complete blood counts and liver function tests, otoscopic examination, audiogram, MRI of the middle ear, objective causality assessment using the Naranjo probability scale. PEG-IFN-induced acute sensorineural hearing loss. Prednisone while maintaining treatment with PEG-IFN.

Fluctuating hearing loss associated with Halo vest application

We report the unique case of a 16-year-old man presenting with sudden and profound left-sided hearing loss following application of a Halo vest. Case report and review of the world literature concerning spinal realignment procedures, spinal manipulation and Halo vest application. A 16-year-old man presented to the ENT clinic with sudden and profound left-sided hearing loss after undergoing posterior release of a fixed flexion extension deformity of the cervical spine and Halo vest application. The hearing loss slowly improved until the patient disrupted the Halo vest while boarding a bus. Regular audiometry documented the progress of the patient's hearing loss. Procedures that significantly alter the established bony anatomy of the neck can be associated with profound audiological deficit through disturbance of the vertebrobasilar arterial system. Such a phenomenon may be associated with application or disruption of a Halo vest.

Sudden-Onset Bilateral Hearing Loss: Case Report

An ischaemic infarction of the anterior inferior cerebellar artery (AICA) is usually associated with vertigo, hearing loss, facial palsy, nystagmus, or truncal ataxia; it is often accompanied by other brainstem or cerebellar signs. Sudden- onset bilateral hearing loss without associated neurological symptoms is infrequent in the literature. We report a case of sudden bilateral hearing loss, later diagnosed as AICA infarction without other symptoms.

Transient auditory dysfunction - a description and study of prevalence

Background. Transient Auditory Dysfunction (TAD) is a previously undescribed symptom complex of unknown cause. It is characterised by sensoneural hearing loss (unilateral or bilateral) and tinnitus, which develops over a minute, resolves completely within minutes and is not accompanied by vestibular symptoms. The condition was brought to our attention as the senior author himself experiences the symptoms. The aim of the current study was to define TAD, find its prevalence and discuss its significance. Method. 200 healthy subjects between the ages of 16 and 49 years with no pre existing medical or otological conditions were surveyed using a questionnaire. Results. 41 subjects (20.5% ± 2.86% (mean ± se; n = 200) reported experiencing symptoms of TAD. The mean number of episodes was 5.9 times per month, the mean duration was 41 s and 80% experienced concomitant tinnitus. 50% had bilateral symptoms. Conclusion. TAD is a common finding in a healthy population. This may have implications on the pathogenesis of sudden onset sensoneural hearing loss. Further longitudinal studies and detailed audiological evaluation of patients with TAD is required to ascertain the significance, aetiology and pathophysiology of this condition.

Implications of Deafblindness on Visual Assessment Procedures: Considerations for Audiologists, Ophthalmologists, and Interpreters

Visual assessment of deaf persons presents a challenge to the ophthalmologist and the optometrist. At the time they want to measure visual function, the interpreter uses visual communication, competing for the patient's attention. Important rules of good assessment of visual functioning include taking turns and giving the interpreter sufficient time to convey the questions related to each test. This has implications for audiologists working with this population as well. Communication should be tailored to the varying needs of people who may have long-standing deafness and good sign language, may have lost their hearing at an advanced age and have limited or no sign language, or may be young persons or children who have congenital or progressive loss of hearing combined with congenital or progressive vision loss. Individuals with sudden hearing and vision loss, as well as those with intellectual disabilities and limited language, are particularly challenging groups for the assessment of vision and require special communication skills for ophthalmologists and audiologists. The ophthalmologist usually knows the nature of vision changes typical of disorders that the patient has. For the clinical follow-up of a condition, a few measurements are usually taken. For a comprehensive assessment of visual functioning for rehabilitation and education, many more measurements, observations, and questions are needed so that vision for communication, orientation in the environment, and performance of near-vision tasks can be reported and the effects of the environment can be evaluated. Testing should cover ocular disorders and vision loss caused by brain damage.

Spontaneous Recovery of Sudden Sensorineural Hearing Loss: Possible Association with Autoimmune Disorders

A 46-year-old white male diagnosed with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS) was seen for audiological testing 15 minutes following a sudden onset hearing loss in the right ear. The test battery included pure-tone audiometry, word-recognition testing, speech-recognition threshold (SRT) testing, immittance testing, and distortion-product otoacoustic emissions (DPOAE) testing. Testing revealed a sensorineural hearing loss in the right ear. Shortly after testing, the patient indicated that his condition had improved. Testing was repeated, and the second round of tests revealed normal hearing in both ears. Four days later, a follow-up test again indicated normal hearing in both ears. Possible connections of this brief occurrence of idiopathic hearing loss with the patient's medical conditions are discussed. Specifically, symptoms were consistent with a transient ischemic attack (TIA) affecting his right cochlea in the stria vascularis region, resulting in a temporary, sensorineural hearing loss. No residual effects were observed clinically.

Sudden bilateral hearing loss: Case report of an audiological emergency

Hypertensive encephalopathy (HE) is characterized by headache, confusion, seizures and visual disturbance and may progress to coma and death. It is an acute neurological picture caused by sudden increases of arterial pressure. Bilateral deafness is a possible symptom of HE. We describe the first case of a patient affected by a hypertensive attack associated with sudden headache and bilateral hearing loss, refractory to medical treatment, whose condition evolved into coma and rapid expiry. Sudden bilateral deafness can be a warning sign of hypertensive encephalopathy. Prompt and intensive management for HE is needed for patients with sudden bilateral deafness.

Digital subtraction angiography abnormalities in sudden unilateral profound hearing loss

Conclusion. The reduced caliber of the anterior inferior cerebellar artery (AICA) on the affected side in patients with sudden unilateral profound hearing loss (SUPHL) may reflect a perfusion deficit which, combined to a lesser degree with anatomic abnormalities in the vertebrobasilar system, would be implicated in the pathogenesis of SUPHL. Objective. To identify existing hemodynamic disturbances and anatomic abnormalities in patients with SUPHL using digital subtraction angiography. Material and methods. Ten patients aged 18–66 years and a control group of 25 subjects were included in the study. Both vertebral arteries (VAs) were selectively catheterized in all the patients. Results. In 7/10 patients (70%) the AICA on the side affected by SUPHL was smaller in caliber than the contralateral one after injection of contrast medium into the VA on the affected side. The situation was significantly different in the control group, whose angiography results demonstrated similarly sized AICAs in 84% of cases. Tortuosity of the VA on the affected side was encountered in two patients and important VA asymmetry in one.

Use of Mammalian Inner Ear Antigens for the Diagnosis of Autoimmune Sudden Loss of Vestibular Function

It has been postulated that bilateral sensorineural hearing loss (SNHL) may be the result of an ongoing autoimmune process against the inner ear and a pattern of progressive bilateral SNHL linked to an autoimmune inner ear disorder has been reported. Various attempts have been made to develop an assay to confirm the diagnosis of autoimmune inner ear disease. In this study we used a Western blot assay to determine the presence of IgG antibodies directed against a PØ antigen (30 kDa) of the guinea pig in the sera of patients affected by sudden loss of vestibular function (SLVF). Ten patients affected by vestibular neuritis were enrolled: eight with unilateral vestibular loss and two with sequential bilateral impairment. We also tested nine patients with sudden unilateral hearing loss, five with benign paroxysmal positional vertigo and six normal subjects. In the present study only one patient, a woman affected by bilateral vestibular impairment, had IgG antibodies against the PØ protein. Our results indicate either that the antigen PØ marker for autoimmune unilateral SLVF or that our patients did not have an immunological basis for their disease. However, we can suggest that bilateral impairment of vestibular function and bilateral progressive SNHL are more likely to be immune-mediated disorders and that PØ could be a valid marker for these diseases. As bilateral vestibular neuritis is an uncommon disease, a multicentre study is required to confirm our suggestions.

Inner ear damage in TORP-operated ears: experimental study on danger from environmental air pressure changes.

In some cases of sudden inner ear hearing loss in ears with a total ossicular replacement prosthesis, the prosthesis has been found to be penetrating the footplate. Some authors have assumed an external pressure increase for this event. In this study I tried to estimate experimentally the pressure needed for perforating a normal footplate in a temporal bone model. From the data I concluded that "cracking" the footplate can hardly be due to 1 major event of increased pressure. On the other hand, different mechanisms making the footplate a "weak spot" must be discussed and further investigated. In those cases, secondary to thinned or even perforated footplates, environmental pressure changes may be dangerous.

Cochlear Implantation in Sudden Bilateral Sensorineural Hearing Loss

Although 1-4% of all cases of sudden sensorineural hearing loss (SSHL) are bilateral, all such patients reported to date have experienced significant recovery of hearing in at least one ear. We report a case of profound, bilateral idiopathic SSHL without recovery which was treated with cochlear implantation; the first such report to our knowledge. The patient achieved open-set spondee recognition. Individuals with sudden bilateral hearing loss in whom treatable causes have been eliminated may benefit from cochlear implantation.

Azathioprine with Cortisone in Treatment of Hearing Loss in only Hearing Ear

A sudden or progressive hearing loss in the only hearing ear is an otologic emergency, in which therapeutic possibilities are limited. We describe results of immunosuppressive therapy in 10 patients with one deafened ear either due to cochlear hydrops or Meniere's disease, who developed progressive or fluctuant hearing loss in the only hearing ear. The perilymphatic fistula was excluded with tympanoscopy. Azathioprine (25 mg t.i.d.) with prednisone (initial dose 20–40 mg with daily dose of 5–15 mg/day) was used. The hearing of 6 subjects responded to treatment. The initial pure tone audiogram (PTA) at speech frequencies of the “only hearing ear” was 57.3 dB HL with speech discrimination score (SDS) of 85%. The hearing improvement occurred successively within days or weeks. The average improvement of PTA was 22.4 dB HL after the mean treatment period of 5 weeks. In contralateral ear (the average PTA and SDS were 72.5 dB HL and 28% respectively) the mean improvement in PTA was insignificant (4.4 dB). In 5 patients with vertigo it was improved in 3 cases. Cortisone alone or with combination of cyclophosphamide or ciclosporine was not effective in our hands.

突発性難聴聴力の年代別検討

突発性難聴聴力の年代別検討

低音障害型の感音難聴の検討

低音障害型の感音難聴の検討

The detrimental effect of aminohydroxypropylidene bisphosphonate (APD) in otospongiosis

Two middle-aged female patients with active stapedial otosclerosis and sensorineural hearing loss have been treated with aminohydroxypropylidene bisphosphonate (APD). No effect on the Schwartze sign was observed either during or following therapy. No changes were seen in the audiometric data of the patients during the 1st year. Then, within a few weeks, both patients complained of sudden deterioration of the existing hearing losses in both ears, with an alteration of tinnitus. One patient became totally, bilaterally deaf, while the other retained minimal auditory function in the low frequencies in one ear. A sudden bilateral hearing loss in cochlear otosclerosis never develops under normal circumstances and we are convinced that in the cases that we have observed it is due to the treatment with APD.

Idiopathic sudden progressive hearing loss and round window membrane rupture.

We investigated 24 cases of idiopathic sudden progressive deafness (ISPD) in which worsening of sensorineural hearing loss after initial examination was confirmed by audiometry. Exploratory tympanotomy was performed in 10 of 24 patients, and revealed perilymphatic leak in 4 patients. However, there was a higher incidence and a more severe degree of vertigo and imbalance in the group that was operated on than in the non-operated group. Our study has shown that there was no clear difference in the degree of hearing improvement between the operated group and the conservatively treated group. In both of these groups, an abruptly falling audiometric configuration was frequently found in the audiogram obtained at the stage of fixed hearing perception, suggesting the possibility that both groups may have a common lesion at the basal turn of the cochlea.

Die Ruptur der runden Fenstermembran ? eine Ursache des akuten H�rsturzes

The rupture of the round window has been well described as an occasional lesion with sudden deafness and vertigo after diving, flying and physical stress mostly according to patients history. Apparently it also seems being the cause for sudden progressive hearing loss. Therefore in our clinic the transtympanic endoscopic evaluation of the round window is routinely carried out in patients with acute complete sudden deafness or severely progressive hearing loss even without any outpointing history of a possible trauma. The methods and the results are described.