BACKGROUND AND OBJECTIVES: Unilateral auditory dysfunction such as tinnitus and hearing loss could be a warning sign of a retrocochlear lesion. Auditory brainstem response (ABR) and internal auditory canal magnetic resonance image (MRI) are suggested as novel diagnostic tools for retrocochlear lesions. However, the high cost of MRI and the low sensitivity of the ABR test could be an obstacle when assessing patients with unilateral ear symptoms. The purpose of this study was to introduce the clinical usefulness of three-dimensional fast imaging employing steady-state acquisition (3D-FIESTA) MRI in patients with unilateral ear symptoms. SUBJECTS AND METHODS: Two hundred and fifty-three patients with unilateral tinnitus or unilateral hearing loss who underwent 3D-FIESTA temporal bone MRI as a screening test were enrolled. We reviewed the abnormal findings in the 3D-FIESTA images and ear symptoms using the medical records. RESULTS: In patients with unilateral ear symptoms, 51.0% of the patients had tinnitus and 32.8% patients were assessed to have sudden sensory neural hearing loss. With 3D-FIESTA imaging, twelve patients were diagnosed with acoustic neuroma, four with enlarged vestibular aqueduct syndrome, and two with posterior inferior cerebellar artery aneurysm. Inner ear anomalies and vestibulocochlear nerve aplasia could be diagnosed with 3D-FIESTA imaging. CONCLUSIONS: 3D-FIESTA imaging is a highly sensitive method for the diagnosis of cochlear or retrocochlear lesions. 3D-FIESTA imaging is a useful screening tool for patients with unilateral ear symptoms.
Aneurysm ; Arteries ; Diagnosis ; Ear ; Ear, Inner* ; Evoked Potentials, Auditory, Brain Stem ; Hearing Loss ; Hearing Loss, Unilateral ; Humans ; Magnetic Resonance Imaging ; Mass Screening ; Medical Records ; Methods ; Neuroma, Acoustic ; Retrocochlear Diseases ; Temporal Bone ; Tinnitus ; Vestibular Aqueduct ; Vestibulocochlear Nerve

Child ; Cochlear Implantation ; Cochlear Implants ; Evoked Potentials, Auditory, Brain Stem ; Hearing Loss, Central ; physiopathology ; Humans ; Vestibulocochlear Nerve Diseases ; physiopathology

OBJECTIVE: The goal was to know more about the characteristic of auditory neuropathy spectrum disorder (ANSD) for high risk infants. METHOD: The newborn hearing screening was performed with automatic auditory brainstem response (AABR) and transient evoked otoacoustic emission(TEOAE) for the infants in the NICUs from August 2007 to January 2011. After subsequent rescreening, children with AABR test referred were perform hearing test set including high frequency (1000 Hz) tympanometry, ABR, DPOAE and/or Cochlear Microphonics (CMs) in 3 months old. Only infants demonstrated severely abnormal ABRs along with preserved DPOAEs and/or CMs were scheduled for re-examination in 6-8 months old and Behavior audiometry in 8-12 months old. RESULT: Eighteen infants (14 cases were bilateral and 4 cases were unilateral) considered as suffering from AN in 3 months old. All of them showed ABR thresholds > or = 80 dB nHL or absent at maximum test intensity. Follow-up examination revealed 9 cases (18 ears) with restoration of ABR to normal or a lower ABR thresholds and a resolution of ANSI) in 11 out of 18 infants retested in 6-8 months old. CMs were present in all ears but DPOAE were not present in 4 ears with middle ear pathology in 3 months test. Behavioral hearing of 10 cases ranged from mild (n = 2), moderate(n = 4) to severe and profound loss (n = 4). CONCLUSION ANSD in high risk neonates could show the temporary character. It was too difficult to forecast the prognosis, they would be to follow up to at least 3 years old for newborn ANSD.
Child, Preschool ; Evoked Potentials, Auditory, Brain Stem ; Follow-Up Studies ; Hearing Loss, Central ; diagnosis ; physiopathology ; Humans ; Infant ; Infant, Newborn ; Vestibulocochlear Nerve Diseases ; diagnosis ; physiopathology

Neurofibromatosis type II (NF2) is a genetic disease and is transmitted as an autosomal dominant trait. NF2 is characterized by bilateral acoustic neuroma with multiple tumors of central nervous system. Progressive hearing loss is a significant problem in patients with NF2, and hearing preservation is an important factor for choice of treatment plan, especially in a NF2 patient with only one hearing ear. We report an experience of preservation of hearing in a NF2 patient with only one hearing ear through decompression of the internal auditory canal via middle cranial fossa approach.
Central Nervous System ; Cranial Fossa, Middle ; Decompression* ; Ear* ; Hearing Loss ; Hearing* ; Humans ; Neurofibromatoses* ; Neurofibromatosis 2* ; Neuroma, Acoustic

Hearing Loss, Central ; genetics ; Humans ; Vestibulocochlear Nerve Diseases

A speech discrimination test is a test using a list of 25 phonetically balanced monosyllables. It is often overlooked but significant enough for pure tone audiometry. Many physicians have performed pure tone audiometry but without a speech discrimination test. A 73-year-old woman visited our clinic complaining of sudden bilateral hearing loss. Pure tone audiometry showed only bilateral high frequency loss. However, speech discrimination had decreased markedly. We decided to follow-up after 1 week of Ginexin-F(R) (ginkgo leaf extract) and Nafril(R) (nafronyl oxalate). She felt a gait disturbance within 2 days. Magnetic resonance imaging revealed a left thalamic hemorrhage. After a 1 month hospitalization, the hematoma subsided, and speech discrimination recovered 3 months later. Acute hearing loss due to thalamic hemorrhage that recovered has never been reported. We report the first case of retrocochlear hearing loss that occurred with a thalamic hemorrhage in a patient who recovered.
Aged ; Audiometry ; Female ; Follow-Up Studies ; Gait ; Hearing Loss ; Hearing Loss, Bilateral ; Hematoma ; Hemorrhage* ; Hospitalization ; Humans ; Magnetic Resonance Imaging ; Retrocochlear Diseases* ; Speech Discrimination Tests ; Speech Perception ; Thalamus

Auditory neuropathy is a term used to describe abnormal auditory brain stem response (ABR) in the presence of preserved cochlear outer hair cell functions which can be measured by otoacoustic emissions (OAE). We report a case of auditory neuropathy accompanying unilateral vestibular hypofunction and benign paroxysmal positional vertigo. The patient was a 50-year-old man who had experienced hearing loss and tinnitus which started two weeks ago. He had taken several medicines for the last few months due to his lung cancer and tuberculosis. ABR and OAE were checked and the results were compatible with auditory neuropathy. To evaluate his vestibular function, video nystagmography, rotatory chair and oculomotor test were checked. The results were compatible with left unilateral vestibular loss and left lateral canal cupulolithiasis. But the patient experienced nearly no vertigo during his daily life. As presented in this case, most of the auditory neuropathy patients do not complain of vertigo. This is probably due to long term central compensation or maybe due to the decreased nerve conduction of the vertiginous sensation. Vestibular evaluation may be crucial in order to detect masked vestibular dysfunction and to protect these patients from imbalance accidents.
Compensation and Redress ; Evoked Potentials, Auditory, Brain Stem ; Hair ; Hearing Loss ; Hearing Loss, Central ; Humans ; Lung Neoplasms ; Masks ; Middle Aged ; Neural Conduction ; Sensation ; Tinnitus ; Tuberculosis ; Vertigo ; Vestibular Neuronitis

BACKGROUND AND OBJECTIVES: There are many audiological tests to distinguish between the hearing loss patients of cochlear lesion and the retrocochlear lesion. We started this research to compare the efficacy of the audiological tests and to make helpful selections of the auditory hearing tests in diagnosis of the sensorineural hearing loss patients. MATERIALS AND METHOD: Our study was conducted on 198 patients with hearing loss. They received otologic physical examination, neurological examination, and MRI. We evaluated SISI, STAT, ARDT, DPOAE, and ABR data values and analysed their sensitivity and specificity. RESULTS: Sensitivity of SISI was 55% and specificity was 74%. Sensitivity of STAT was 58% and specificity was 67%. Sensitivity of ARDT was 44% and specificity was 71%. Sensitivity of DPOAE was 50% and specificity was 64%. Sensitivity of ABR was 90% and specificity was 74%. CONCLUSION: Among the tests performed above, ABR showed the highest sensitivity and specificity value, which implies it to be the most meaningful screening test in the sensorineural hearing loss patients. But for the patients with severe hearing loss, such that the auditory hearing test results are suspicious in its accuracy, or for the patients who are suspicious of retrocochlear lesions based upon multiple tests, MRI should be performed to make the final diagnosis.
Diagnosis ; Diagnosis, Differential* ; Evoked Potentials, Auditory, Brain Stem ; Hearing Loss ; Hearing Loss, Sensorineural ; Hearing Tests ; Humans ; Magnetic Resonance Imaging ; Mass Screening ; Neurologic Examination ; Physical Examination ; Retrocochlear Diseases* ; Sensitivity and Specificity

Vertigo/dizziness is a common complaint in patients who are seeking a primary health clinic. Vertigo is traditionally attributed to damage of the vestibular system. Many peripheral and central vestibular disorders are usually presented with vertigo. However, patients with benign paroxysmal positional vertigo (BPPV), a leading cause of vertigo, may present with postural lightheadedness, near faint, imbalance rather than true vertigo. On the contrary, patients with orthostatic hypotension may present with true spinning vertigo, not dizziness. Persistent postural perceptual dizziness, a second most common cause of dizziness (after BPPV), is mainly occurred after organic vestibular disorders such as BPPV or vestibular neuritis, and classified as a chronic functional vestibular disorder. This article describes non-vestibular disorders presenting dizziness and/or vertigos, which conditions may be misdiagnosed as structural vestibular disorders.
Benign Paroxysmal Positional Vertigo ; Diagnosis ; Dizziness ; Humans ; Hypotension, Orthostatic ; Orthostatic Intolerance ; Vertigo ; Vestibular Neuronitis ; Vestibulocochlear Nerve Diseases

Neurofibromatosis type 2(NF-2) is a dominantly inherited disorder characterized by the occurrence of bilateral acoustic neurinomas and the frequent association of other central nervous system tumors. We present a retrospective review of 16 patients with NF-2 who were treated at our hospital from 1984 to 1995. In 13 cases, the diagnoses of NF-2 were based on the criteria developed at the Consensus Development Conference of National Institute of Health in the United States, and in another 3 cases, the criterias of NF-2 were not fully satisfied, but the diagnoses of NF-2 were highly suspected. The average age of the patients was 27.6 years, ranging from 13 years to 56 years. The most common symptom was hearing difficulty;intervals between symptom onset and deafness ranged from 8 months to 6 years(mean:2.9 years.) One family of NF-2 was documented, consisting of a sister, a brother and their mother. Nine patients underwent operations on unilateral acoustic neurinomas;these were subtotally removed in eight patient s and totally in one patient. Among these patients, five were deaf on the ipsilateral side at surgery. Among the other four patients with useful hearing before surgery, hearing was preserved to preoperative status in two patients. Four patients with diagnoses of meningioma, received operations to relieve mass effect with subtotal removal in two patients and total removal in the other two. Early diagnosis and treatment are the most important in the management planning of patients with NF-2 for reasons of early manifestation and rapid progression of the disease.
Acoustics ; Central Nervous System Neoplasms ; Deafness ; Diagnosis* ; Early Diagnosis ; Hearing ; Hearing Loss ; Humans ; Meningioma ; Mothers ; Neurofibromatoses* ; Neurofibromatosis 2* ; Retrospective Studies ; Siblings ; United States